Progressive transmural electrographic, myocardial potassium ion/sodium ion ratio and ultrastructural changes as a function of time after acute coronary occlusion. The progressive transmural electrographic, biochemical and ultrastructural changes as a function of time after acute coronary occlusion were systematically assessed in eight dogs. Transmural plunge electrodes with poles 1 mm apart were placed in the ischemic and nonischemic zones, and coronary occlusion was maintained for 4 hours. Transmural full thickness biopsy specimens were obtained from each zone for electron microscopy before, and 1 and 4 hours after occlusion. Endocardial and epicardial layers were also obtained for assessment of myocardial potassium ion (K+) and sodium ion (Na+) concentrations. Before coronary occlusion, local Q waves were recorded an average depth of 1.0 +/- 0.34 mm from the endocardial surface. After 1 hour of occlusion, Q waves appeared at an average depth of 3.8 +/- 0.67 mm and progressed to a depth of 5.2 +/- 0.7 mm at 2 hours, 6.2 +/- 0.5 mm at 3 hours and 7.0 +/- 0.5 mm at 4 hours. After 1 hour, ultrastructural changes of early ischemia, including a decrease in glycogen and mild mitochondrial swelling, were seen in the endocardial layer; the epicardial layer showed normal morphologic features. After 4 hours, the endocardial layer showed  well developed ischemic changes marked by the loss of mitochondrial cristae, vacuolization, the appearance of amorhopous mitochondrial cristae, vacuolization, the appearance of amorphous mitochondrial densities, an increase in interfibrillary space and the appearance of I bands. In contrast, the epicardial layer at this time showed only early ischemic changes. At the end of 4 hours, the endocardial layer showed a marked decrease in myocardial K+  concentration and an increase in Na+ concentration leading to complete reversal of K+/Na+ ratio (0.7 +/- 1.0; P less than 0.001). In the epicardial layer, a smaller decrease in K+ concentration and an increase in Na+ concentration occurred, resulting in a diminution but not a reversal of K+/Na+ ratio (1.4 +/- 0.2; P less than 0.005). Thus, the dynamic evolution of an acute myocardal infarction involves a sequential progression from endocardium to epicardium as a function of time, resulting in an epicardial "border zone" in the early stages after acute coronary occlusion.


Computer-assisted reporting system for the follow-up of patients with prosthetic heart valves. The implantation of large numbers of prosthetic heart valves carries with it the responsibility for continual reassessment of all aspects of patient management. Experience with more than 2,000 prosthetic valve operations since 1963 led to the development of a comprehensive computer-assisted data collection, management and reporting system. Over a 5 year period, data forms were developed for the detailed documentation of preoperative, intraoperative and postoperative information. These were designed in the form of checklists suitable for direct computer entry with use of mark-sense document readers. Special emphasis was placed on preoperative assessment of ventricular function, valve selection, intraoperative myocardial preservation, postoperative rehabilitation and prosthetic valve-related complications. This system makes possible rapid computer generation of a variety of reports to the referring physician regarding the individual patient and to the clinical investigator in relation to patient group statistics. Also, questionnaires to patients of physicians, or both, to update patient data can be produced by the computer at appropriate intervals after valve surgery. Experience indicated that a computer-assisted methodology is the only practical way to provide adequate follow-up of large groups of patients. Additionally direct access to relevant information helps to create an environment in which essential research can be carried out in the face of a demanding clinical practice.


Transvascular endomyocardial biopsy in infants and small children: description of a new technique. A miniaturized technique for transcatheter endomyocardial biopsy has been developed in the belief that myocardial biopsy performed in infancy, when the disease process in cardiomyopathy may be most active, should yield important etiologic and nosologic information. To obtain six biopsy specimens, three from each ventricle, adds about 1 hour to a diagnostic right and left heart catheterization. A no. 4 or 5 French forceps with a modified soft shaft is guided to the site in theapical septum of the right and left ventricles through a previously molded to measure guide tube of ultrathin radiopaque Teflon. With biplane fluoroscopy the guide tube of ultrathin radiopaque Telflon. With biplane fluorsocopy the guide tube is introduced as a sheath over a matching catheter and the catheter is removed. Contrast medium in injected to verify position, the forceps is introduced and the biopsy specimen is taken. If the forceps is sharp and pressure on the endocardium in light, evidence of biopsy is not discrenible on examination of the heart 1 week later. The method was developed in small dogs and proved safe and effective in rabbits weighing 3 kg. Biopsy has been performed safely in children aged 4 1/2 months to 5 1/2 years and weighing 4.5 to to 19.6 kg.


Life expectancy without surgery in tetralogy of Fallot. All published autopsy cases of patients with tetralogy of Fallot who died without surgical treatment were studied to determine the life expectancy of such persons. In addition, the data from a study of persons with tetralogy alive in Denmark in 1949 were reanalyzed. The survival data from these two sources were remarkably similar, indicating that 66 percent of persons with tetralogy of Fallot not treated surgically live to age 1 year, 49 percent to age 3 years and 24 percent to age 10 years; thereafter, the hazard function (or instantaneous risk of death) remains constant. The chance of survival is significantly less when pulmonary atresia, rather than stenosis, is present.


Association of interrupted aortic arch with malformations producing reduced blood flow to the fourth aortic arches. Interrupted aortic arch is a rare congenital malformation of the great arteries with loss of continuity between the proximal portion of the aortic arch and the descending aorta, which is usually supplied by a patent ductus arteriosus. The hearts of 12 patients with interrupted aortic arch listed in the autopsy files of The Johns Hopkins Hospital were evaluated. Four categories were identified. Seven hearts with normal outflow tract relations had significant pulmonary arterial override of a ventricular septal defect and thus recevived part of left ventricular and all of right ventricular ejection flow; flow into the aorta was correspondingly reduced. Two hearts had mitral valve obstruction with decreased left heart flow. Two hearts had double inlet left ventricle and transposed great arteries with the aorta originating from a small anterior conal chamber. One heart had a truncus malformation with a disproportionately large flow entering the pulmonary arterial division. In each of these hearts, the pattern of the outflow tracts and associated malformations suggested that there was a preferential flow of blood into the sixth arches and correspondingly a reduced flow into the fourth arches at the time of normal disappearance of components of the embryonic aortic arch system. Thus, interrupted aortic arch may simply be disappearance of the normally persisting connection between the left fourth and sixth arches as a result of flow imbalance in early cardiogenesis.


Antiarrhythmic drug effect assessed from ventricular arrhythmia reduction in the ambulatory electrocardiogram and treadmill test: comparison of propranolol, procainamide and quinidine. A 5 week study was performed in 17 patients with frequent ventricular ectopic complexes. The study design comprised an initial control period, 1 week each of treatment with propranolol (240 mg daily), procainamide (3.0 g daily) and quinidine (1.8 g daily) and a final control period. Twenty-four hour ambulatory electrocardiograms and maximal exercise tests were performed each week. For the group, the total number and qualitative types of ventricular ectopic complexes were similar during the two control periods; however, there were large variations among individual patients. Each drug reduced the total number of ventricular ectopic impulses and the percent of patients with each qualitative type. There was agreement between the ambulatory electrocardiogram and treadmill test in three quarters of the drug evaluations. Although it is possible to determine antiarrhythmic drug effects for a group, spontaneous variability in the occurrence of ventricular arrhythmias makes it difficult to evaluate the effects in individual patients.


Retrograde block during dual pathway  atrioventricular nodal reentrant paroxysmal tachycardia. There are limited reported data regarding the occurrence of retrograde block during dual pathway atrioventricular (A-V) nodal reentrant paroxysmal tachycardia. This study describes two patients with this phenomenon. The first patient had 2:1 and type 1 retrograde ventriculoatrial block during the common variety of A-V nodal reentrance (slow pathway for anterograde and fast pathway for retrograde conduction). Fractionated atrial electrograms suggested that the site of block was within the atria. The second patient had type 1 retrograde block (between the A-V node and the low septal right atrium) during the unusual variety of A-V nodal reentrance (slow pathway for retrograde and fast pathway for anterograde conduction). The abolition of retrograde block by atropine suggested that the site of block was within A-V nodal tissue. Both cases demonstrate that intact retrograde conduction is not necessary for the continuation of A-V nodal reentrant paroxysymal tachycardia. Case 2 supports the hypothesis that the atria are not a requisite part of the A-V nodal reentrant pathway.


Electrophysiologic observations in a patient with bradycardia-dependent atrioventricular block. In a patient with atrioventricular (A-V) block distal to the His bundle (H), 1:1 A-V conduction with right bundle branch block and H-V interval of 70 msec was established with atrial pacing at rates of 120 to 150/min, suggesting that the A-V block was bradycardia-dependent. Advanced second degree A-V block distal to the H deflection occurred with atrial pacing at 160/min after completion of A-V nodal Wenckebach periodicity proximal to the H deflection because of the long H-H encompassing the blocked P wave. Atrial extrastimulus testing coupled with sinus rhythm (with A-V block) demonstrated that critical H1-H2 intervals of less than 545 msec allowed conduction to the ventricles. The H2-V2 interval shortened progressively from 290 to 70 msec with shortening of these critical H1-H2 intervals. Atrial extrastimulus testing coupled with an atrial driven cycle lenght of 500 mesc (with intact A-V conduction) revealed block of the H2 deflection with an H1-H2 interval longer than 540 msec. In conclusion, at critical diastolic intervals, impulses were blocked, creating a state of decreased responsiveness. If a cycle length of subsequent impulses was shorter than the critical diastolic blocking interval, membrane responsiveness gradually improved and conduction resumed. If a cycle length of subsequent impulses was longer than the critical blocking diastolic interval, A-V block was sustained. Blocked impulses continually penetrated to the site of block and reset the state of membrane responsiveness.


Changes in the crude fiber content of the American diet. The intake of crude fiber in the American diet was assessed for 7 time periods between 1909 and 1975 using food consumption and composition tables. Crude fiber intake dropped 28% from 6.8 g/day in 1909 to 4.9 g/day in 1957 to 1959 and has remained at that level until the present. The intake of fiber from vegetables has remained relatively constant from 1909 to 1975 while potatoes, fruit, cereals, dry peas, and dry bean consumption have declined. The trends shown for crude fiber consumption in the United States support the hypothesis that fiber intake has decreased coincidentally with increases in degenerative diseases.


Urolithiasis in Manipur (north eastern region of India). Incidence and chemical composition of stones. The incidence of urolithiasis in Manipur is very high. From hospital records for a period of 7 years and 3 months, it was observed to be 11.6% of all general surgery cases in the General Hospital, Imphal. This is alarmingly high. The social, eating, drinking, and living habits are different among the three major populations in this state. The prevalence was minimal among Tribals. Compared to them the prevalence was about one and one half times higher among Muslims (also called Pangals) and seven times higher among Hindus. Surprisingly, the incidence of renal calcalus was higher in females. One hundred ninety-six stones were studied by wet chemical analysis. Calcium and oxalate were present in all stones. Phosphate was present in 194 stones and uric acid (including urate) was present in 146 stones.


Amino acid and protein metabolism in renal failure. There are many cAUSES OF ALTERED AMINO ACID AND PROTEIN METABOLISM IN UREMIA WHICH MAY Lead to impaired growth, wasting, malnutrition, and other aspects of the uremic syndrome. These causes have complex interrelationships that are not well understood. The factors include altered nutrition due to poor intake, losses of nutrients during dialysis, and abnormal metabolism of many nutrients. Uremic toxins, superimposed catabolic illnesses, elevated or reduced serum hormone levels, reduced capacity of the kidney to synthesize certain amino acids and to degrade other amino acids, peptides, and small proteins, and decreased excretion of certain amino acids and peptides may also contribute to altered amino acid and protein metabolism. The response of certain plasma amino acids to protein restriction appears to differ in uremic patients as compared to normal subjects. Increased plasma levels of many products of amino acids and proteins in renal failure are due primarily to decreased urinary clearance by the kidney. However, for some metabolites, increased synthesis or decreased degradation may also contribute to elevated levels. These latter compounds include guanidinosuccinic acid, methylguanidine, certain middle molecules, and in some patients, phenylpyruvic acid.


Effects of catabolic stress in acute and chronic renal failure. The degree of catabolism was studied in a group of seven patients with postsurgical acute renal failure, and net protein catabolism of 43.1 to 927 g/day. In 70% of a group of 30 patients with stable chronic renal failure receiving and 18 g high biological value protein diet, urea synthesis was equivalent to what could be accounted for by the protein content of the food. This equivalence was not found in studies in both adults and children who were treated for catabolic chronic renal failure. Data obtained show the superiority of feeding essential amino acids with an adequated caloric supply. The need for studies aimed at adapting the uremic patient to protein depletive mechanisms is strongly stressed.


Nitrogen metabolism in the gut.

Nitrogen metabolism in the gut. All three of the major human nitrogenous waste products--urea,, creatinine, and uric acid--are significantly degraded by intestinal bacteria. The breakdown products of creatinine and uric acid are not fully known, but metabolites of these complex heterocyclic nitrogen compounds may well turn out to play a role in uremic toxicity. Urea degradation is almost certainly by way of ammonia, but the exact site of urea hydrolysis in the alimentary tract is not known, and it is uncertain whether urea is the major source of intestinal ammonia. Ammonia is absorbed from the colon predominantly in unionized form, and the bicarbonate ion secreted by the colonic mucosa plays an important role in facilitating this absorption.


Effects of intestinal flora on nitrogen metabolism in patients with chronic renal failure. Gastrointestinal bacteria have been thought to be beneficial to uremic patients since they could provide an internal source of nitrogen by degrading urea and could function as an alternative means of clearing waste products. Data are presented to indicate that the bacteria of uremic patients do not clear significantly more urea than do the intestinal flora of normal subjects. Analysis of urea metabolism before and during oral administration of aminoglycoside antibiotics demonstrated that nitrogen derived from urea is not used by uremic patients for amino acid synthesis. In addition, it was found that nitrogen balance, on the average -0.98 +/- 0.41, significantly improved to -0.18 +/- 0.29 g N per day during the antibiotic period. The possible explanation for this are discussed. It is concluded that intestinal bacteria adversely affect uremic patients by promoting catabolism and by producing toxins which accumulate in body fluids.


Anabolic role of urea in renal failure.

Anabolic role of urea in renal failure. The amount of urea nitrogen released and the amount reincorporated into albumin has been measured in healthy and uremic individuals on both normal and low-protein diets. The albumin synthesis rate was measured simultaneously. Gut urea breakdown was only 50% higher in renal failure than in health, but the efficiency of utilization of the nitrogen thus released was increased more than 6-fold in renal failure and was higher on a low protein than on a normal protein diet. The lower the albumin synthetic rate, the greater was the efficiency of incorporation of urea nitrogen into albumin. The rate of urea nitrogen incorporation into albumin increased on average 14-fold in chronic renal failure. The absolute rate of utilization (84 mumole/hr) was, however, small and comprised on average only 2.4% of the nitrogen used in albumin synthesis. These findings suggest that although some urea derived nitrogen is incorporated into albumin, the amount is not nutritionally significant even under conditions of protein deprivation and high urea availability.


The metabolism of human serum albumin in renal failure on conservative and dialysis therapy. The pathophysiology of albumin metabolism in uremia was investigated by turnover measurements in a large series of uremic patients, either on conservative management or on dialysis therapy. A total of 62 turnover studies were performed in patients on dietary treatment, divided into two groups according to the duration of the low protein diet: 35 subjects from 6 to 30 days, 27 subjects from 6 months to 5 years. Albumin catabolism and distribution were measured by the two-tracer technique (131I-albumin and 125I-iodide, simultaneously injected iv), while albumin synthesis was directly determined in 10 patients by the use of 14C-carbonate and 131I-albumin. Sixteen turnover studies were also performed in a group of end-stage uremics on dialysis therapy by a two-tracer procedure especially designed to determine albumin catabolism in the course of a single peritoneal or hemodialytic treatment. The main features of albumin metabolism observed in the patients on dietary management were: normal intravascular albumin mass, marked reduction of the extravascular and total albumin pools, with proportionally reduced catabolism. No significant turnover difference was found between the short-term diet group and the patients on low-protein diet from 6 months to 5 years. As to the uremics on dialysis therapy, catabolic rate of albumin was 3-fold increased in three patients showing clinical features of "hypercatabolism" in the early phase of uremia, or during relapse from it. Albumin turnover rate returned to normal when measured during clinical steady-state conditions. All these findings suggest that a marked body protein depletion exists in chronic uremia, and that dietary treatment per se is not responsible for such a depleted state. Instead, the depletion of protein stores observed in the steady phase of chronic uremia may have been originated by the exaggerated increased catabolism in the early phase of renal failure, not compensated by a proportional increase of the synthetic rate, due to both the state of uremic intoxication and to the reduced dietary protein intake during the early phase.


Pharmacokinetics of essential amino acids in chronic dialysis-patients. Possible disorders of essential amino acid (EAA) metabolism in maintenance dialysis patients (D) were studied by measuring plasma amino acids before and sequentially after administering a mixture of 8 EAA po and iv. The EAA were in a ratio similar to that required for optimal utilization, and the total dose given was within physiological range. Ten D and six normals (N) received 150 mg/kg po as a 10.5 g/dl solution and 117 mg/kg iv as a 5.1 g/dl solution infused at a constant rate of mg/kg per min. Blood glucose and immunoreactive insulin were also measured. Both D and N were postabsorptive and at least 18 hr postdialysis. The fraction of the oral dose appearing in the systemic circulation was variable for each EAA in both N and D. Total body clearance was significantly lower in D (P less than 0.05) for theronine, phenylalanine, valine, leucine, and isoleucine, and this difference could not be explained by changes in renal excretion. The apparent volume of distribution did not differ between N and D for all EAA except for valine and phenylalanine. Blood glucose insulin varied only slightly in both N and D for all EAA except for valine and phenylalanine. Blood glucose and insulin varied only slightly in both N and D. These studies indicate that there are a variety of significant abnormalities in the metabolism of specific EAA in D. Decreased total body clearance of the branched-chain amino acids, since they are primarily metabolized total body clearance of the brnached-chain amino acids, since they are primarily metabolized by muscle, may result from a defect in muscle metabolism in D.


Valine metabolism in normal and chronically uremic man. Valine metabolism was investigated in five normal and three nondialyzed chronically uremic subjects eating 40 +/- SEM 1 and 53 (range 40 to 80) protein diets respectively, in a metabolic research unit. Subjects were injected iv with a tracer dose of L-valine-1-14C while they fasted, and specific activity of plasma valine-14C and expiration of 14CO2 were monitored for two hours. Plasma valine was significantly lower in the uremic patients than in the normal subjects (P less than 0.05). In the uremic patients, specific activity of plasma valine fell less rapidly and remained higher, and expiration of 14CO2 was not different from normal subjects. A two-pool model for valine metabolism was derived which indicated that in uremic patients there was a significant decrease in both valine pools and in the rate of irreversible loss, i.e., valine incorporated into larger molecules, degraded, or excreted. Valine degradation was estimated to be decreased in the uremic patients.


Protein restriction in the conservative management of uremia. Protein-restricted diets are widely used in the dietary management of uremia. These diets are undoubtedly effective in ameliorating many aspects of the uremic syndrome. However, there is no consensus as to whether diets providing less than 0.6 g/kg per day of protein are nutritionally adequate and capable of preventing the wasting syndrome. Wasting is common in the adult patient with renal insufficiency as is growth failure in the uremic child. There is some evidence that wasted patients do less well on hemodialysis and are more prone to infection. Experimental studies in uremic animals point ot diminihsed efficiency of utilization of protein, increased gluconeogenesis from animo acids, and increased catabolism of protein in the fasting state; in addition, the metabolism of a number of individual amino acids is altered in uremia. In view of these multiple abnormalities, it would seem unwise to routinely provide less than the Recommended Daily Allowances of protein. More recent developments, i.e., supplementation of essential amino acids and perhaps alpha keto acids, may provide useful alternatives. One important aspect of dietary management, i.e. prevention of hyperlipidemia, has attracted surprisingly little attention so far. Therapy with protein restricted diets in nondialyzed uremic patients has to compete with other modalities of treatment currently available, i.e., hemodialysis and transplantation, in providing optimal medical rehabilitaiton of the patient.


Control of measles and rubella through use of attenuated vaccines. A summary report of the current status of morbidity and mortality associated with measles and rubella vis á vis immunization practices in the United States since these vaccines were licensed in 1963 (measles) and 1969 (rubella) is presented.


Review of existing vaccines for influenza. The problems of producing and distributing influenza vaccines are described as well as effectiveness and adverse reactions. It appears that Guillain-Barré (GBS) is likely to be encountered with the use of any of the inactivated influenza vaccines.


Studies with live attenuated influenza vaccines. Inhibitor-resistant and temperature-sensitive live attenuated influenza virus vaccines were administered to normal volunteers and elderly and chronically ill persons to assess safety, antigenicity, and transmissibility. Recombinants of both types of vaccine were made using currently prevalent influenza virus strains (A/Hong Kong/68, A/England/42/72, A/Scotland/74, A/Victoria/75) over a five-year period to keep the vaccine relevant with regard to antigenic drift. The results indicate that both vaccine types are safe, antigenic and non-transmissible and that for both types of vaccines, recombinants can be made to new antigenic drifts. The inhibitor-resistant vaccine replicates to lower titers in the nasopharynx than the temperature-sensitive virus, and this is considered an advantage, whereas the genetic defect of the temperature-sensitive virus is better defined than that of the inhibitor-resistant vaccine.


Skeletal trauma in skateboard injuries.

Skeletal trauma in skateboard injuries. Thirty fractures resulting from skateboarding have been treated in the past six months at the Henry Ford Hospital, Detroit. Although there have been no serious or unusual injuries, this represents a substantial increase over the previous year. Awareness of the hazards of this sport is the first step in reducing the frequency and seriousness of these injuries. As a result of our experience, safety guidelines for skateboarding are recommended for the parents of enthusiasts. We hope adherence to these guidelines will greatly reduce the number of injuries.


Erythroid aplasia in juvenile rheumatoid arthritis. A 9-year old girl had clinical manifestations of juvenile rheumatoid arthritis that were further complicated by severe anemia. Subsequent studies showed the anemia to be due to erythroid aplasia. Hematologic remission was induced with prednisone, 60 mg/day.


Effect of prednisone on growth and bone mineral content in childhood glomerular disease. Children with acquired glomerular disease were divided into two groups: Group 1 patients received short-term daily or long-term alternate-day prednisone (up to 2.7 mg/kg/48 hr); group 2 patients received no corticosteroids. Height, bone mineral content (BMC), and bone density were evaluated in the two groups and compared to those of 800 sex- and age-matched controls; BMC and bone density were assessed by the photon absorption technique. Significant demineralization was present in 18 of 25 prednisone-treated and none of the 17 nonprednisone-treated patients (P less than .001). Group 1 patients were 5.3 +/- 0.7% shorter than controls, while group 2 patients were only 1.9 +/- .8% shorter (P less than .02). Height velocity was 2.6 +/- 0.8 cm/yr in group 1 and 5.1 +/- 0.8 cm/yr in group 2 patients (P less than .05). When prednisone therapy was discontinued, six patients had an increase in height and BMC toward normal values. This study suggests that BMC and height velocity are correlated. Both appear to be influenced by alternate-day prednisone therapy rather than by glomerular disease per se.


Diagnostic value of the muscle biopsy in the neonatal period. Muscle biopsy specimens of 20 full-term neonates (13 surgical and seven necropsy specimens) with clinical evidence of neuromuscular disease were studied to determine the diagnostic usefulness of this procedure in the newborn. Characteristic pathologic alterations were identified in specific diseases. Some findings were similar to those seen later in life, but others differed from those expected in the same diseases at older ages. Persistence of fetal muscle cells was a characteristic common to several congenital myopathies and neuropathies. Lymphocytic infiltrates, muscle fiber necrosis, and architectural alterations of the muscle fibers were not seen at birth. Extramedullary hematopoiesis may involve newborn muscle. Muscle biopsy is a safe and simple procedure in the neonatal period and has a diagnostic reliability as good as at older ages, but histochemistry and sometimes electron microscopy are essential supplements to classical histology for interpretation. Recommended indications for muscle biopsy in the neonatal period are multiple joint contractures at birth or hypotonia and weakness, of unknown origin.


Child abuse. Its relationship to birthweight, apgar score, and developmental testing. Fifty-two abused and 23 nonabused children from a low socioeconomic group were studied with respect to birthweight, five-minute Apgar score, and developmental quotients. An association was found between low Apgar scores, low birthweight, poor performance on developmental testing, and child abuse. Furthermore, it was found that among children with normal birthweights, abused children tended to have lower Apgar scores and significantly lower developmental quotients. This study suggests that among this group of children, abuse and subsequent poor performance may be related not only to the socioeconomic status of the children but also to the characteristics the child brings to the parent-child relationship and to the abusive environment.


Obesity in urban black adolescents of high and low relative weight at 1 year of age. The prevalence of obesity was determined in 789 9 to 15-year-old Philadelphia subjects who had either a high (+1 SD) or a low (-1, SD) relative weight at 1 year of age. During the adolescent years, obesity was assessed by the triceps skin-fold thickness and by the relative weight, using national reference standards from the US Health Examination Survey. The prevalence of obesity for the high relative weight group at 1 year of age was three to four times higher than in the low relative weight group at 1 year of age. Compared with all urban youth of this age range, regardless of their status at 1 year of age, it is estimated that the risk ratio associated with a high relative weight at 1 year of age is approximately 1.6.


Consumptive coagulopathy. A complication of therapy of solid tumors in childhood. The association of consumptive coagulopathy and malignancy is thought to be rare in pediatrics. In one patient with Ewing's sarcoma and in two with rhabdomyosarcoma consumptive coagulopathy developed in the presence of either diffuse metastatic disease or an extensive primary tumor. The coagulopathy was a  major clinical problem, developing within five days of the onset of chemotherapy. Resolution coincided with the clinical response of the tumor.


Limitations of emergency room evaluations of sexually abused children. A retrospective review was performed on charts of all children who were treated in 1975 in the Strong Memorial Hospital (Rochester, NY) emergency room for alleged sexual abuse, suspicious conditions, or complaints related to the genitourinary system, and on a randomly selected comparison sample of children treated for acute otitis media during the same period. In 44 cases of otitis media, pediatric house officers' charting uniformly evidenced adequate care. However, in ten cases of alleged sexual abuse and 29 cases considered suspicious, the evaluations and care were significantly (.01 greater than P less than .001) less adequate. The young pediatric house officer deals with these problems, which are complicated by emotional overtones and psychosocial implications, less effectively than those with more clear-cut, routine diagnoses.


Guide to oral theophylline therapy for the treatment of chronic asthma. Theophylline, when used appropriately, seems to be the most effective noncorticosteroid prophylactic medication for chronic asthma. Dosage, however, requires consideration of the relationship between serum concentration, efficacy, and toxicity, in addition to the variability in rates of elimination. Therapy is initiated with 16 mg/kg/day or 400 mg/day (whichever is less) in divided doses at appropriate intervals depending on the rate of absorption of the product used. If side effects are absent, the dosage is increased at three-day intervals until the age-related mean dosage necessary to produce a therapeutic level, is reached. Final dosage adjustment is generally based on a single determination of serum concentration; dosage requirements generally then remain constant for six to 12 months depending upon the child's growth rate. Dosage adjustment in this manner optimizes benefit, minimizes risk of toxic effect, and avoids excessive numbers of serum theophylline measurements.


Critical malnutrition in breast-fed infants. Three case reports. Three breast-fed infants of primiparous women had hypothermia, azotemia, and severe dehydration and malnutrition. No disease entities were identified. Although the cause of inadequate breast nutrition was unclear, these cases underscore the necessity for close follow-up and support of first-born breast-feeding babies.


Staphylococcus aureus colonization and infection in newborn nursery patients. The correlation between Staphylococcus aureus colonization and infection among newborns was studied during a four-year period. Of the 9,423 newborns cultured, 24% were colonized at the time of discharge and in 2% of these an infection developed, whereas only 0.2% of the noncolonized newborns experienced a staphylococcal infection. The weekly colonization rates ranged from 0% to 62%, and outbreaks of infections (two or more concurrent) occurred periodically when the colonization rates ranged from 11% to 57%. Colonization rates per se did not serve as an indicator of an actual or potential outbreak of infection. Instead, the occurrence of two or more concurrent cases of staphylococcal infection or presumptive evidence of an outbreak seems to be a more reliable indicator than colonization monitoring.


Gluteal necrosis with foot-drop. Complications associated with umbilical artery catheterization. A newborn infatn developed gluteal necrosis and foot-drop associated with umbilical artery catheterization. Clinical course of the infant's illness illustrates that the etiology was multifactorial, being related to the site of catheter tip placement, hypotension, and infusion of hypertonic glucose and other drugs via umbilical catheter.


Hyperuricemia in congenital heart disease. Gout is rarely noted as a clinical problem in secondary polycythemia-- even if profound polycythemia exists, as in cyanotic congenital heart disease. A retrospective study of 81 patients with congenital heart disease was done to assess the incidence of hyperuricemia. Twenty of 46 patients with cyanotic congenital heart disease had serum levels of uric acid greater than 8 mg/dl. Thirteen of 16 (81%) cyanotic male patients more than 15 years old had serum levels greater than 8 mg/dl. For cyanotic patients, serum levels of uric acid were related directly to the degree of polycythemia (r = .44; P less than .02). Impaired renal function or drug therapy did not seem to account for the hyperuricemia. Because levels of uric acid greater than 10 mg/dl probably are nephropathic, many of these patients may be incurring subclinical uric acid nephropathy.


Pyloric atresia.

Pyloric atresia. A case of pyloric atresia was treated successfully with side-to-side gastroduodenostomy. The mortality in treated cases is high, due to delay in diagnosis and inadequate relief of the obstruction. Side-to-side gastroduodenostomy or excision of the atresia with pyloroplasty offer the greatest opportunity for a successful outcome, as indicated by a review of the world literature.


Intraspinal enterogenous cysts. A case report and review of pediatric cases in the literature. A patient with an intraspinal enterogenous cyst is described. A review of the English literature disclosed 26 previous pediatric cases reported. These cases were studied in regard to clinical presentation, laboratory findings, histology of the lesion, and neurological follow-up. Although rare, enterogenous cysts should be considered where there is evidence of spinal cord compression.


Cholangiographic abnormalities in ulcerative colitis associated pericholangitis which resemble sclerosing cholangitis. Cholangiographic abnormalities in asymptomatic patients with pericholangitis and long-standing ulcerative colitis, which resemble sclerosing cholangitis, have not been previously reported. Endoscopic retrograde cholangiography (ERC) performed in one such patient suggesting intrahepatic sclerosing cholangitis stimulated the study of seven additional patients with largely asymptomatic pericholangitis. In seven of these eight patients, ERC demonstrated abnormalities which resembled sclerosing cholangitis. These consisted of beading and strictures mainly of the intrahepatic biliary tree (IHB). In two of the eight, the common bile duct was involved. In one, this was associated with histologic progression to cirrhosis and frank cholangitic episodes even though the initial clinical presentation and hepatic histology 2 1/2 years earlier suggested only pericholangitis. We therefore conclude that bile duct abnormalities resembling sclerosing cholangitis may be demonstrated cholangiographically in patients with ulcerative colitis who present with the typical picture of pericholangitis.


The normal cholangiogram.

The normal cholangiogram. This paper describes the measurements of the normal cholangiogram as demonstrated by endoscopic retrograde cholangiopancreatography. Two groups of patients were studied. There were 49 patients who had normal livers and no evidence of biliary tract disease. In addition, there were 25 patients with known liver disease and an apparent normal extra-hepatic biliary tract. The extrahepatic bile duct was measured in three regions: (1) the common hepatic duct (above the cystic duct and below the bifurcation); (2) the prepancreatic portion (below the cystic duct and above the pancreas); and (3) the interpancreatic portion (bile duct within the pancreas). These measurements ranged from 2.1 to 9.2 mm. Although the common bile ducts were generally quite uniform in caliber, there were instances where each portion was the largest diameter in an individual case. There was slight increase in bile-duct caliber with age. This study showed somewhat smaller measurements than previously reported and may relate to case selection in this or previous studies.


Plasma amino acids imbalance in patients with liver disease. The venous plasma amino acid patterns have been determined in 12 normal individuals and in 71 shunted and nonshunted cirrhotics in various grades of hepatic encephalopathy. The free amino acids have been determined by an amino autoanalyzer; the total and free tryptophan have been measured by a spectrophotofluorimetric method. In 14 instances, arterial plasma amino acid patterns have been measured simultaneously. High levels of aromatic and sulfurated amino acids and low levels of branched-chain amino acids have been constantly found in all cirrhotics. Methionine, phenylalanine, valine, leucine, tyrosine, and free tryptophan showed a statistical difference between controls and all other groups. These altered patterns did not correlate either with the grade or the evolution of the coma or with the presence of the surgical anastomosis. No statistical differences were found between arterial and venous values. The only amino acid which statistically correlated with the grade and evolution of the hepatic encephalopathy was free tryptophan. The molar ratios between the amino acids sharing the same transport system across the blood-brain-barrier have been considered. A very good correlation with the grade of the mental disorder was found with the ratios free tryptophan/phenylalanine-tyrosine-methionine-valine-leucine-isoleucine and free tryptophan/branched-chain amino acids.


Associated liver disease in alcoholic pancreatitis. Two studies investigating the association of liver disease with acute and chronic pancreatitis in alcoholics are presented. In a retrospective study of 50 patients, no clinical liver disease was found in 9 patients with acute pancreatitis, while 23 (56%) of 41 patients with chronic pancreatitis had liver disease by clinical criteria. Of this latter group, 8 were confirmed histologically; thus 19% of patients with chronic pancreatitis had biopsy-proven cirrhosis. Fifty alcoholic patients with pancreatitis were prospectively evaluated. All who had clinical evidence of liver disease were biopsied. No cases of liver disease were encountered in the 4 patients with acute pancreatitis. Although 28 (60%) cases of clinically diagnosed liver disease were present in 46 patients with chronic pancreatitis, only 20 of these seemed significant (cirrhosis, alcoholic hepatitis, severe fatty liver), for an incidence of 43%. Thus, clinically significant alcoholic liver disease occurs quite frequently in association with alcoholic pancreatitis. This association is meaningful in more effective management of these patients in general and in preoperative assessment of the risk of surgery in particular.


Pancreatic retention cyst secondary to chronic pancreatitis. A cause of hemobilia. We describe a 47-year-old male with chronic calcific pancreatitis who had a four-year history of occult gastrointestinal bleeding with three prior hospitalizations. At endoscopy on the fourth admission, hemobilia was identified. Arteriography showed a 3-cm-diameter dye blush in the head of the pancreas. A retention cyst had eroded into an adjacent artery, causing bleeding. A stone at the ampulla of Vater apparently served as a ball valve to prevent massive hemorrhage. We discuss the pathogenesis of hemobilia in chronic pancreatitis and suggested therapy.


Colonic motility in domestic turkeys.

Colonic motility in domestic turkeys. This study was performed to determine (1) if electrical slow waves could be recorded from the colon of turkeys, and (2) how ingesta moves into and through the colon despite colonic antiperistalsis. Electrical activity and contractile forces were monitored via implanted bipolar electrodes and strain gage transducers (SGT), respectively. Two types of slow waves, small (sSW) and large (lSW) were recorded simultaneously. The former were correlated with antiperistaltic contractions observed radiographically and with small contractions recorded with SGT, the latter were correlated with large contractions recorded via SGT. The sSW had higher frequencies distally than proximally while the frequency gradient for the lSW was just the opposite. The sSWs were believed to be involved in regulation of antiperistalsis while the lSW were believed to be involved in regulation of the large contractions which, on the basis of the lSW frequency gradient, appeared to be peristaltic and to be primarily responsible for aborad movement of colonic digesta. The small contractions were believed to be responsible for reflux of urine from the cloaca into the colon and ceca and for cecal filling.


Postoperative intestinal motility in dogs and sheep. Electrical activity of the small intestine was recorded from chronically implanted electrodes in dogs and sheep. The effects of laparotomy and surgical procedures were identified under general anesthesia. Laparotomy induced in both species a complete inhibition of electrical spiking activity during the stage of muscular and peritoneal incision. The inhibitory period due to laparotomy was shorter in dogs than in sheep and in both species less marked at the duodenal level. A secondary period of inhibition (ileus) lasting from 6 hr for a resection of the colon to 24 hr for a transection of the small bowel was observed in dogs after a transient phase of normal activity during 3-6 hr. The period of inhibition due to abdominal surgery lasted from 48 to 72 hr and occurred at the time of surgery in sheep. In both species, inhibition due to laparotomy and surgical procedures was prevented by splanchnicectomy. The results showed that myoelectric activity of the different parts of the small intestine is specifically inhibited and/or disorganized through peripheral reflex pathways. They are activated at different degrees by laparotomy and abdominal surgery.


Effects of anesthesia and surgical procedures on intestinal myoelectric activity in rats. Electrical spiking activity of the duodenum and jejunum was recorded from chronically implanted electrodes in rats during volatile or barbiturate anesthesia and following laparotomy. The normal pattern of electrical spiking activity in the fasted rat, with myoelectric complexes at 15-min intervals, was transiently replaced by quiescence during ethyl ether anesthesia. A slight increase in irregular spiking activity occurred after induction with pentobarbital, and the only effect of thiopental anesthesia was a reduction in the velocity of propagation of the complexes by 20%. Under barbiturate anesthesia, incision of the skin did not inhibit myoelectric activity, but incision of each abdominal muscle layer had an immediate and transient inhibitory effect; the deeper the layer, the longer was the inhibition. Peritoneal incision consistently produced inhibition of spiking activity which was prolonged by exposure of the bowel to air and intestine handling. The inhibitory effects produced by surgery persisted after vagotomy or transection of the spinal cord at the thoracic level but disappeared after splanchnicectomy. The above results suggest that a somatovegetative reflex with efferent pathways in the splanchnic nerves is involved in the first stage of operative inhibition of intestinal myoelectric complexes.


Potent reduction of basal acid output produced by meperidine. Meperidine is a potent narcotic related chemically and pharmacologically to morphine and atropine. To examine its gastric antisecretory activity a study designed to test the effect of meperidine on basal acid secretion was carried out: 100 mg of meperidine given intramuscularly reduced basal acid output (BAO) by 66.3% at 1 hr (P less than 0.001), 64.9% at 2 hr (P less than 0.001), and 44.9% at 3 hr (P = 0.005). This degree of reduction of BAO by this dose of meperidine did not differ from that produced by 30 mg of propantheline intramuscularly. These results demonstrate that meperidine in standard classical doses is a potent inhibitor of BAO in man.


Bile lipid composition and bile acid pool size in diabetes. Since the prevalence of gallstones is higher in diabetics than in controls and since cholelithiasis is often associated with supersaturated bile, we measured bile lipid composition and bile acid pool size in 8 patients with juvenile diabetes, 16 with maturity-onset diabetes, and 10 control subjects. Bile lipid composition was expressed as "saturation index." In the maturity-onset diabetics the saturation index (1.60 +/- 0.45 SDM) was significantly higher (P less than 0.005) than that in the controls (0.82 +/- 0.20) and in patients with juvenile diabetes (0.75 +/- 0.24). The absolute values for biliary bile acid concentration were significantly lower (P less than 0.01) in the maturity-onset diabetics than in the other two groups. There were no differences in either the proportion of the individual biliary bile acids or the size of the bile acid pool between the three groups. The results suggest that the incidence of cholelithiasis in diabetes is associated with the secretion of a supersaturated bile only in the maturity-onset subgroup.


Gastric bezoars. A technique of endoscopic removal. Gastric bezoar formation is an uncommon sequela of gastroduodenal surgery or unusual eating habits. Because they generally produce severe symptoms, their removal is always necessary. Previously, this required surgical extirpation or slow enzymatic dissolution. We present here an endoscopic procedure for bezoar removal utilized successfully in five patients with vegetable-mucus bezoars. This technique employs a jet spray of water under direct vision to mechanically disrupt the bezoar, which may then be removed using a large gastric lavage tube. This procedure is simple, safe, and rapid and is therefore recommended as an alternative to surgical removal or enzymatic dissolution of gastric bezoars.


Validation of the use of a tube with a proximal occlusive balloon for measurement of intestinal absorption in man. Because of its simplicity, a tube with a proximal occlusive balloon is widely used to measure segmental intestinal absorption in man. Yet, the possibility that the presence of an inflated balloon might alter the absorptive capacity of the underlying segment of intestine has never been tested. In the present work the jejunal absorption of water and ions has been measured in 13 healthy subjects with the proximally occlusive tube and with the triple lumen nonocclusive method; precautions were taken to achieve similar flow rate and ionic composition of the fluid entering the test segment with both techniques in each subject. Under these conditions net water and ion movements measured by the two methods were identical. This finding validates the use of the proximal occlusive tube to measure intestinal absorption in man.


A comparison between intragastric titration and aspiration technique under basal conditions and after food or pentagastrin stimulation. A total of 16 healthy subjects had gastric secretory studies where acid was either measured by the conventional aspiration technique (AT) or by intragastric titration at a pH of 5.0 (IT). In a first study food- and pentagastrin-stimulated PAO levels, both measured by IT, were within similar ranges. However, as compared to the respective AT data of the same subjects, the mean IT results were 35% higher, although both values were well correlated. In a second study a similar difference was observed between IT and AT data throughout the whole range of the pentagastrin dose-response curve. Both curves followed Michaelis-Menten kinetics and had similar Km values, while Vmax values were significantly higher at IT. This constellation excludes an increase of the gastrin sensitivity of the parietal cells as a cause for the higher IT data. It is, however, in keeping with the hypothesis that the distension stimulus present at IT potentiates the pentagastrin-stimulated acid secretion. It is likely that methodological problems such as overtitration of acid at IT and transpyloric loss of acid at AT additively contribute to the relatively large difference between AT and IT values. It is therefore not possible to fully interchange AT and IT data, despite their good correlation.


Colonic varices. A complication of pancreatitis with splenic vein thrombosis. A patient with recurrent gastrointestinal bleeding was found to have varices at the splenic flexure at colonoscopy. Angiography revealed complete occlusion of the splenic vein. Although the patient did not have cirrhosis, he did have a history of pancreatitis which presumably was responsible for the splenic vein thrombosis. This case represents a compartmentalized form of portal hypertension which requires careful endoscopic and radiographic studies for proper evaluation. Successful treatment was accomplished by splenectomy.


Methyldopa-induced pancreatitis.

Methyldopa-induced pancreatitis. An acute febrile illness associated with gastrointestinal upset developed in a patient within one week after starting treatment with methyldopa. The illness was characterized by prompt subsidence of symptoms when the patient withdrew therapy secondary to gastrointestinal upset, and recrudescence of symptoms when methyldopa therapy was reinitiated. This was associated with hyperamylasemia, hyperlipasemia, hyperpyrexia, and epigastric pain, both on admission to the hospital and upon rechallenge with methyldopa. Although gastrointestinal upset has been reported as an untoward side effect of methyldopa, this is the first report to our knowledge of documented methyldopa-induced hyperamylasemia and hyperlipasemia secondary to pancreatitis.


Pancreatic insufficiency and Ménétrier's disease. Report of a case with clinical response to pancreatic enzyme replacement. A patient with exocrine pancreatic insufficiency and hypoalbuminemic edema was shown also to have a protein-losing gastropathy secondary to giant hypertrophic gastritis (Ménétrier's disease). Despite continuation of excessive gastric protein loss, the manifestations of hypoproteinemia were reversed by oral pancreatic enzyme replacement therapy. We speculate that impaired intraluminal proteolysis exaggerated the consequences of gastric protein loss.


On the appearance, levels and regulation of pancreatic enzyme serum concentrations. The evaluation of serum pancreatic enzyme activity on whole and diluted serum permits the estimation of serum enzyme inhibitor activity via a ADS/AWS ratio. This has diagnostic value in chronic pancreatic disease. The serum pancreatic ferment activity appears to be regulated in part by serum steroid levels. It is responsive to high protein diet. A physiologic rationale is presented for the use of steroids and high protein diet in the treatment of acute and chronic pancreatic inflammation.


Metabolism of bile acids by strains of Acinetobacter anitratum and Acinetobacter lwoffii. The activity of two species of bacteria: Acinetobacter anitratum and Acinetobacter lwoffii, isolated from jejunum of patients with achlorhydria and Reichel-Polya resection towards bile salts was investigated. These species were not previously tested from this view point. All strains tested hydrolyzed bile salts; conjugates of deoxycholic acid were hydrolyzed more readily than those of cholic acid. This feature may be used as a bacteriological taxonomic criterion.


Vibriocidal antibody titer rise after rectal or anal administration of Vibrio cholerae in dogs. In many developing countries and ablution with surface water is a daily habit. This water is heavily contaminated with microbes, raising the possibility that and contamination may lead to immune responses. This hypothesis was experimentally tested in 21 dogs by anal or rectal application of Vibrio cholerae suspension V. cholerae was recovered from rectal swab cultures for a mean of one day after rectal contamination and 2.5 days after anal application (P less than .02). Significant increases occurred in the geometric mean titer of serum vibriocidal antibody; this suggests the need for study of the possible role of anal ablution in maintaining serum vibriocidal antibody levels in endemic cholera areas.


Barrett's esophagus. Clinical review of 26 cases. Barrett's esophagus was diagnosed in 26 men in a five-year period by demonstrating esophageal specialized columnar epithelium in target biopsies obtained at endoscopy or in peroral suction biopsies of the esophageal mucosa. The clinical, radiologic and manometric features of these patients were reviewed retrospectively. Esophageal lesions associated with this epithelium included distal and midesophageal strictures and ulcers, alone or in combination, or simply esophagitis. One patient had an associated adenocarcinoma. Twenty of 26 (77%) had heartburn or regurgitation, 16 (62%) had easily elicited reflux of barium while supine and 16 of 17 tested had lower esophageal sphincter pressure in the incompetent range. Ninety-six percent had one or more of these parameters positive. This series demonstrates a wide spectrum of esophageal lesions in Barrett's esophagus, and supports the concept that this lesion occurs as a consequence of gastroesophageal reflux and erosive esophagitis. The case of adenocarcinoma in this series adds to the concern that the columnar lined lower esophagus may be a premalignant lesion.


Mucoepidermoid carcinoma of the esophagus. Report of an unoperated autopsy case and review of literature. The esophageal tumor which occurred in a 53-year old female and was histologically similar to the salivary mucoepidermoid carcinoma, showed local invasion at autopsy. No distant metastasis was noted. Our literature review showed invasive and metastatic nature of the tumor. Submucosal location of the tumor in our case supported the proposed origin of the neoplasm from the esophageal gland.


Improvement in esophageal abnormalities in a patient with scleroderma and features of polymyositis. A patient with clinical features of both scleroderma and polymyositis, including esophageal dysfunction typical of scleroderma is described. Regression of her illness occurred along with manometric improvement in the esophageal dysfunction. Improvement in esophageal abnormalities in scleroderma has not been previously reported.


Systemic mercury intoxication following rupture of a Miller-Abbott tube. Following rupture of a Miller-Abbott tube mercury bag a 39-year old woman, postoperative for resection of the right colon for diverticulosis and diverticulitis, developed signs and symptoms of systemic mercury intoxication. A small bowel fistula allowed the metallic mercury to aggregate in retroperitoneal tissues setting up an environment which was conductive to conversion of metallic mercury to divalent mercury, an absorbable product. Analysis of brain, kidney and urine following her demise showed markedly elevated mercury levels. To our knowledge this is the only reported case of such a complication. Clinicians should be wary of elemental mercury in the gastrointestinal tract in patients with suspected or proven enteric fistulas.


Retroperitoneal lymphangioma. A case report with review of the literature. A case of retroperitoneal cystic lymphangioma is reported. Added to the 42 cases of retroperitoneal lymphangioma found from the English literature, this brings up the total of cases reported to date to 43. A critical review of the clinical picture and management of this condition is presented on the basis of these reported cases.


The effect of Imuran on pancreatic secretion. A preliminary report. The acute administration of Imuran and hydrocortisone significantly decrease pancreatic flow and protein output in canines. This observed phenomenon may explain the gastrointestinal side reactions encountered in clinical usage of these potent immunosuppresants.


Spontaneous pneumoperitoneum with Pneumatosis cystoides intestinalis in a patient with mixed connective tissue disease. Pneumatosis cystoides intestinalis (PCI), a disorder in which gas filled cysts develop within the bowel wall, has been associated with collagen vascular disease, usually progressive systemic sclerosis (PSS). We have extended the spectrum of collagen vascular disease associated with PCI by this report of a patient with mixed connective tissue disease (MCTD) who developed PCI and recurrent spontaneous pneumoperitoneum (SP). Possible pathogenetic mechanisms are reviewed and the management of the asymptomatic patient with pneumoperitoneum, PCI and collagen vascular disease is discussed.


The lower esophageal ring and esophageal reflux. Although the lower esophageal ring is an important cause of dysphagia in adults, its pathogenesis is unknown. To better define the relationship between the lower esophageal ring and esophageal reflux, we analyzed, retrospectively, the records of 18 hospitalized patients with radiologically demonstrated rings (16 confirmed by endoscopy and/or surgery). In 16 of these patients, evidence of esophageal reflux including at least one of the following was present: overt esophagitis noted at endoscopy or surgery (14 cases), histologic evidence of esophagitis (3 cases), and/or symptoms of pyrosis or free reflux (13 cases). These findings suggest that 1. reflux is frequently associated with lower esophageal rings and may play a role in their pathogenesis; 2. the radiologic finding of a ring should serve as a clue to the possibility of esophageal reflux and 3. long-term control of dysphagia in these patients may depend upon control of the associated reflux as well as mechanical disruption of the ring.


Jejunal bile salts and microflora in patients with partial gastrectomy. Bile acids and microflora were studied in the proximal jejunum in 31 patients with Billroth I or II gastrectomy. At least 10(5) bacteria/ml., most frequently aerobic or facultative aerobic organisms, were isolated in 25 patients. Deconjugation diminished considerably in only three of seven patients with bile salt deconjugation treated with clindamycin. The relationship between bacterial overgrowth, deconjugation, steatorrhea, cholesterolemia and the relationship between bile salt concentration after testmeal and steatorrhea, are discussed. Bacterial overgrowth and bile salt deconjugation are only minor causes of steatorrhea in this series.


An unusual case of portasystemic encephalopathy caused by splenic vein occlusion following gastrectomy. A patient with recurrent episodes of consciousness disturbance following partial gastrectomy is presented. Angiographic examination revealed a great portasystemic shunt, which probably developed following the postoperative splenic vein thrombosis. This is suspected a rare cause of the condition. In chronic liver disease, recurrent encephalopathy which is caused by portasystemic shunt is well known as one of the most frequent complications. It is very uncommon in adults, however, when the liver is not concerned in the shunt formation. In the present paper the authors report an unusual case of recurrent consciousness confusion which is due to the great portasystemic shunt probably caused by postgastrectomy splenic vein thrombosis.


Squamous cell carcinoma of the stomach.

Squamous cell carcinoma of the stomach. Squamous cell carcinoma of the stomach is a rare, unusual lesion which tends to occur at a somewhat earlier age than adenocarcinoma. Grossly and radiologically the tumor is indistinguishable from adenocarcinoma and may involve any portion of the stomach. Although the pathogenesis of this lesion remains unclear, most authors favor the "metaplastic theory" for the development of the tumor. In this case it is felt that the tumor probably arose from gastric squamous mucosa present congenitally in the lesser curvature.


Bronchial asthma associated with intestinal parasites. Fifty patients with bronchial asthma and 50 normal controls were studied in Hacettepe University Medical Center. Forty per cent of the patients with bronchial asthma and 14% of the controls had ascarides in the stool examinations. The significant difference in these two groups indicates that intestinal parasites, should be looked for in the patients with bronchial asthma.


Localized giant pseudopolyposis of the colon. Localized giant pseudopolyposis is a rare complication in patients with inflammatory diseases of the colon which produces a huge intraluminal polypoid mass. Examples are described in a child with chronic ulcerative colitis in whom the lesion filled the rectum and in an adult with recurrent granulomatous ileocolitis in whom the mass was present in the transverse colon adjacent to a stenotic anastomosis. The clinical presentation, radiologic features and pathologic findings of this unusual sequela to inflammation are reviewed.


Energy expenditure, cigarette smoking, and blood pressure level as related to death from specific diseases. In a 22-year followup of 3686 San Francisco longshoremen, the roles of physical activity, cigarette smoking habit, and systolic blood pressure level were evaluated independently in relation to risk of death from a broad range of diseases. Smoking pattern and blood pressure status were established in 1951 and job activity was assessed annually during the followup period. Lower levels of energy expenditure predicted increased risk of fatal heart attack and perhaps of stroke. Heavy cigarette smoking predicted increased risk of death from heart attack, cancer, chronic obstructive respiratory disease, and pneumonia. Higher levels of systolic blood pressure were associated with death from all cardiovascular diseases, diabetes mellitus, and cirrhosis. Tacit to these findings: sedentary living takes its toll largely through heart disease and stroke; the toxicity of cigarette smoking is associated with a broader range of diseases, including heart attack, cancer, and respiratory disease; and higher level of blood pressure related to an even broader range of cardiovascular disease than either of the other characteristics studied.


A cluster of Hodgkin's disease in a small community: evidence for environmental factors. A cluster of Hodgkin's disease (HD) cases occurred in a small rural town of 1250 people. Ten cases of HD and three cases of non-Hodgkin's lymphoma were identified within or linked to this town since 1954. This town therefore had an average annual incidence and mortality for HD of 29.3 and 16.7 cases per 100,000 population, respectively. Most cases of HD demonstrated case-contact associations, and a distinct geographic distribution. The data suggested that there was an environmental agent responsible for the elevated rates of HD. One unique aspect of this cluster is that this town has only one industry, a large grain elevator. The cases closely surrounded this elevator. We postulate that residents of the town are subject to chronic immune stimulation from mitogenic substances in this environment. These agents may alter immunity in the residents of this community and predispose them to acquiring HD.


The relation of social factors to physical and psychiatric illness. A study in social factors associated with illness status was conducted in an Australian suburban community. Forty six per cent of adults reported a chronic physical condition and twenty four per cent were psychiatrically impaired. Life event stress, adverse childhood experience and poor social support were related to both physical psychiatric illness. Low occupation status was associated with poor physical health and a poor coping style was associated with psychiatric illness. Twenty per cent of the physical illness and thirty seven per cent of the psychiatric impairment could be attributed to the presence of social factors.


An outbreak of histoplasmosis at an Arkansas courthouse with five cases of probable reinfection. An outbreak of histoplasmosis occurred at a county courthouse in Arkansas in July, 1975. Fifty cases were identified by symptoms and positive complement fixation (CF) tests and 18 others by the clinical findings alone. Only three positive CF tests (greater than or equal to 1:8) were found in a control group of 55 unexposed persons of the same age. The attack rate for 84 courthouse employees was 52%. Histoplasma capsulatum was isolated from the sputum of two cases, and from bird droppings which had been dumped from the roof of the courthouse two weeks before the epidemic's peak. Window air conditioners apparently helped to distribute infectious particles throughout the building. Nine exposed people had pulmonary calcifications and a negative tuberculin test that were taken as presumptive evidence of previous histoplasmosis. Five (55%) of these became ill. Exposed persons without pulmonary calcifications had an attack rate of 85%. The reinfected cases during convalescence had higher serum CF titers but not larger histoplasmin skin test diameters than the rest of the cases. A microdroplet aerosol of cooking oil was used to simulate air flow patterns during the exposure, and the results agreed with the hypothesized means of infection.


Alternatives to Rothman's approach for assessing synergism (or antagonism) in cohort studies. K.J. Rothman has explored in some detail the issue of assessing the potential presence of synergism (or antagonism) in data generated from either a cohort or a case-control study. Arguing that the "natural" scale for quantifying the joint effects of two or more factors acting in combination is the probability scale, he has proposed a procedure based on a ratio-type index for evaluating two-factor interaction in the presence of non-zero background effects. In this paper, the authors review the rationale underlying Rothman's approach for a cohort study. They then present what they maintain is a simpler and more appropriate test procedure (utilizing a linear contrast of the observed risks) for the additive approximation to his basic probabilistic model of "no interaction." A likelihood ratio test based on his original model is also proposed, as well as a closed form approximation to it. Finally, the assessment of interaction in cohort studies involving exposure factors measured at more than two levels is addressed.


Health care institution labor law: case law developments, 1974-78. This Article examines the key National Labor Relations Board and federal court interpretations of the much-heralded 1974 Health Care Institution Amendments to the National Labor Relations Act. It analyzes NLRB decisions that have resulted in a proliferation of separate employee bargaining units at health care facilities, and that have applied the strike notice provisions of the NLRA to labor disputes involving building trade employees engaged in construction at operating health care facilities. In addition, the Article examines the Board's decision to decline jurisdiction over employee unions representing interns and residents, and summarizes both the special NLRB rules designed to apply to union solicitation of employees at health care facilities and the conflicting circuit court decisions that have modified the Board's specialized solicitation rules.


Governmental regulation of health care: a response to some criticisms voiced by proponents of a "free market". In this Comment, the Massachusetts Rate Setting Commissioner takes issue with the criticism of health care cost-containment regulation that was expressed by Professor Clark C. Havighurst in a recent edition of the Journal, and argues that instead of abandoning regulation in favor of various "free market" alternatives recommended by Professor Havighurst, the nation should find ways to make regulation work more effectively in the public interest. The author challenges Professor Havighurst on the ground that he fails to recognize (1) that the free market model is inadequate for evaluating regulatory activity and (2) that regulation is essentially a political process, and therefore regulatory objectives cannot and should not be defined in economic terms alone. What is needed, suggests Mr. Weiner, is acceptance of the need for, and validity of, regulation, and an examination of how regulation can best achieve its economic and political objectives. The key challenge for policy makers in the health care regulatory field, he asserts, is the clarification and implementation of appropriate relationships (1) between health care regulation and health care rationing; (2) between health care regulation and health care planning; and (3) between health care regulation and health care competition.


Managing public health risks: the swine flu immunization program revisited. In this Comment, the Massachusetts Commissioner of Public Health views the federal government's 1976-77 Swine Flu Immunization Program, which was sharply criticized by Dr. Cyril Wecht in a recent Article in this journal, as a classic example of a public policy decision made under conditions of stress and uncertainty. Once the responsible government officials had made a public commitment to immunize the entire populace of the United States, he contends, they found it very difficult to reformulate the program in response to changing information concerning its relative costs and benefits. Dr Fielding offers suggestions for avoiding in the future some of the problems that surrounded the Swine Flu Program and for preventing further erosion of public confidence in essential preventive medicine programs.


Federal preclusion of state certificate-of-need exemptions for research and education expenditures. The National Health Planning and Resources Development Act of 1974 requires each state to enact a certificate-of-need program in compliance with federal standards in order to remain eligible for continued receipt of federal funds for health resource development after 1980. This Note contends that the Act and related HEW regulations preclude states from exempting health care facilities' research expenditures and education expenditures from the scope of the states' certificate-of-need programs. The Note recommends that, as an alternative to such state exemptions, each state develop a streamlined certificate-of-need procedure that fulfills federal requirements while efficiently meeting the special needs of research and education projects.


Hemodynamic responsiveness to short- and long-acting vasodilators in left ventricular failure. Vasodilators acutely reduce afterload and improve hemodynamics in congestive heart failure. Intravenous nitroprusside reduces left ventricular filling pressure and increases cardiac output while modestly reducing blood pressure and not changing heart rate in patients with heart failure in whom this response is characteristic. Comparably reduced blood pressure during nitroprusside infusion in normal subjects or hypertensive patients without failure results in a decrease in cardiac output and tachycardia. Long-acting vasodilators are also effective in patients with congestive heart failure. Nitrates, predominant venodilators, decrease left ventricular filling pressure as much as nitroprusside does, but increase cardiac output less. Hydralazine, an arterial dilator, increases cardiac output similarly to nitroprusside but decreases filling pressure less. Combining hydralazine with nitrates results in hemodynamic effects almost identical to those of nitroprusside. The quinazoline derivatives, trimazosin and prazosin, are also effective vasodilators, which act on both arteries and veins in patients with congestive heart failure. The hemodynamic response to vasodilators is influenced by the underlying hemodynamic status, as the change in cardiac output is directly related to base line ventricular filling pressure as well as systemic vascular resistance, and inversely related to the base line cardiac output. Response to vasodilators does not appear to be altered by age, diabetes, acute myocardial infarction or the cause of congestive myocardiopathy.


Hospital treatment of congestive heart failure. Management according to hemodynamic profile. Selection of therapy for subjects with acute congestive dardiac failure usually involves a choice among a diuretic, a vasodilator and an inotropic agent. Three principal questions are involved in the decision: (1) Is cardiac out normal or depressed? (2) Is blood pressure normal or depressed? (3) is regional myocardial ischemia present? Diuretics are safe and easy to administer, but they do not increase cardiac output or relieve hypoperfusion. Inotropic agents increase cardiac output but differ widely in their effects on blood pressure: selection of specific agents is influenced by their blood pressure effect. All inotropic agents, however, potentially aggravate regional myocardial ischemia. In ischemic heart failure, therefore, vasodilators which also increase cardiac output, may be chosen. Vasodilator administration is in turn limited by the decrease in arterial pressure which accompanies increasing infusion rate. When these three questions are considered in combination, an effective therapeutic regimen can be identified. Thus, congestion without hypoperfusion requires a diuretic if blood pressure is normal; and a vasodilator when blood pressure is increased. In the presence of congestion with hypoperfusion, a vasodilator is employed if blood pressure is normal; and a positive inotropic drug when blood pressure is depressed.


Selection of vasodilator, inotropic or combined therapy for the management of heart failure. Vasodilator and inotropic drugs work through independent mechanisms in augmenting left ventricular pump function in patients with heart failure. The selection between these two classes of pharmacologic agents for an individual patient may be based on the control blood pressure as well as the underlying disease. Although vasodilator drugs are easiest and safest to employ in patients with normal or high arterial presure levels, even in relatively hypotensive subjects (systolic arterial pressure less than 105 mm Hg), a salutary hemodynamic effect can be achieved without an undue decrease in pressure. Inotropic drugs may be safest to administer to patients without coronary artery disease, but the oxygen-consuming effect of these drugs need not necessarily have an adverse effect on patients with ischemic heart disease. Combined vasodilator and inotropic drug therapy is the most potent pharmacologic means of restoring pump function in patients with severe heart failure. The long-term use of vasodilator and inotropic drugs in the treatment of heart failure is dependent on the availability of agents that will produce a sustained hemodynamic effect. Hydralazine, nitrates and prazosin have been employed alone or in combination and provide a promising approach to vasodilator treatment of heart failure. Better and more selective oral inotropic agents are needed to allow this therapeutic modality to be employed optimally.


Mechanisms of beneficial effects of vasodilators and inotropic stimulation in the experimental failing ischemic heart. Both vasodilator and inotropic agents improve cardiac function in ischemic heart failure. However, since vasodilators may reduce coronary perfusion pressure and inotropic interventions may increase myocardial oxygen consumption (MVO2), both may increase myocardial ischemia. Accordingly, we determined myocardial blood flow and MVO2 in a canine model of failure induced by propranolol and volume load combined with acute coronary ligation. Both nitroprusside and digitalis reduced ventricular diastolic pressure (LVDP) and increased myocardial blood flow in the ischemic subendocardium. Decreased systolic wall tension also caused a significant reduction MVO2. The benefit of nitroprusside in failing hearts was obtained even with the addition of critical obstruction of the main left coronary artery (LCA). The role of preload reduction is emphasized by the contrasting results with nitroprusside in hearts with low LVDP: (1) decreased myocardial blood flow in ischemic subendocardium, and (2) left ventricular decompensation in animals with critical LCA obstruction. Thus, reduction of LVDP, which decreases subendocardial ischemia, is essential for the beneficial effects of vasodilators and inotropic interventions in ischemic heart failure. Decreased MVO2 caused by reduced heart size may also have a salutary role.


Recurrent hepatitis following halothane exposures. The existence of hepatotoxicity due to halothane remains subject to debate. Three cases are presented in which multiple exposures to halothane were followed in several instances by evidence of postoperative fever and/or hepatitis. In one case, hepatitis with bridging necrosis developed; the patient responded to long-term steroid therapy. In another, fulminant hepatic necrosis proved fatal. A brief review of halothane hepatitis is given. Otherwise unexplained fever and hepatitis following the use of halothane, especially if recurrent or accompanied by evidence of hypersensitivity, should contraindicate subsequent use of that drug.


Renal failure following major angiography. Acute renal failure following angiography with contrast agents is known to occur, but the circumstances and frequency of its occurrence are not well described. A retrospective review of consecutive angiographic procedures performed over a six month interval revealed a 12 per cent incidence of renal failure following angiography. The degree of failure was severe in approximately 30 per cent of these cases and was associated with a significant mortality even though renal function usually recovered. The occurrence of renal failure was associated with the presence of renal insufficiency, impaired liver function, diabetes mellitus, hypoalbuminemia and proteinuria at the time of angiography to a statistically significant level. Furthermore, combinations of these factors, particularly preexisting combined renal insufficiency and impaired liver function, were associated with an increased incidence of acute renal failure. It is concluded that angiography poses a significant hazard to patients with underlying medical problems, particularly those involving the excretory routes of the contrast agent.


Abstinence following detoxification and methadone maintenance treatment. During an 11 year overview of methadone treatment, 161 (72 per cent) of 225 patients who completed detoxification were followed up to eight years. Fifty-one (22.6 per cent) of those were classified as stable and narcotic free 2.9 years after detoxification. Of 89 self-selected patients who had undergone a planned, supported, "therapeutic" detoxification, 37 (42 per cent) were classified narcotic-free; whereas many fewer were so judged after other methods of detoxification. Relapse to nonprescribed opioid use, detected in 34 (38.7 per cent) of those traced, was inversely related to time since detoxification. Relapse potential was very low after three years of apparent continuous narcotic-free existence; three years should be a minimal time for successful detoxification. Some subjects had several cycles of methadone treatment and detoxification. Most patients with combined alcohol-methadone dependencies did poorly, whether or not detoxification from opioids was undertaken. Since the frequency of enduring narcotic-free state was only 9.7 per cent of 522 patients in the treatment sample, detoxification should not be a realistic goal for all patients who enter treatment.


Hemodynamic compromise associated with ventriculoatrial conduction following transvenous pacemaker placement. Ventriculoatrial conduction complicating ventricular pacing in a patient with ischemic heart disease led to severe clinical disability and hemodynamic compromise. Data obtained at cardiac catheterization documented that ventriculoatrial conduction of the patients' paced beats led to inadequate left ventricular filling pressures, pulmonary congestion and depressed cardiac output. This complication of ventricular pacing can be suspected at bedside examination and emphasizes the importance of considering the possible hemodynamic effects of ventricular pacing when long-term pacemaker therapy is contemplated.


Bilateral diaphragmatic paralysis with hypercapnic respiratory failure. A physiologic assessment. Bilateral diaphragmatic paralysis was suspected in a patient presenting with hypercapnic respiratory failure who exhibited paradoxic (i.e., inward) abdominal movement on inspiration during tidal breathing in the supine posture; no paradoxic abdominal motion was observed at the bedside with the patient upright. Transdiaphragmatic pressure measurements established the diagnosis of diaphragmatic paralysis, although 20 cm H2O pressure developed across the diaphragm during the latter part of a forced expiration, presumably due to the development of passive tension in the diaphragm as it was stretched near residual volume. Analysis of the relative motion of the rib cage and abdomen during breathing by the use of magnetometers confirmed the presence of abdominal paradox throughout the breathing cycle when the patient was supine, and established that paradoxic motion of the abdomen also occurred when the patient was in the erect posture but only in the latter half of inspiration. Our findings confirm that the use of transdiaphragmatic pressure measurements and magnetometry will help to quantify diaphragmatic function, that passive tension develops in the paralyzed diaphragm near residual volume and should not be confused with active contraction, and that paradoxic motion of the abdomen may be masked from the clinician when the patient is erect.


Morphologic and functional substrate of thyrotoxicosis caused by nodular goiters. The pathogenesis of nonimmunogenic thyrotoxicosis caused by nodular goiters--with the exception of true toxic adenoma--was investigated in 11 patients by means of scintigraphic, morphologic and autoradiographic technics. The basic event is the appearance, for unknown reasons, of autonomously functioning follicles which are morphologically indistinguishable from normal follicles. Four basic patterns of intrathyroidal distribution of autonomously functioning follicles are individualized: Type I = multiple individual autonomously functioning follicles scattered throughout the goiter. Type II = clustered autonomous follicles without demarcation from less active parenchyma. Type III = multiple microadenomas. Type IV = autonomous function of the majority of all follicles. In all four types, the autonomous follicles occur without recognizable relation to nodule boundaries. Scintiscans cannot predict the microstructure of these types of goiters. More than one pattern of distribution of autonomously functioning follicles may occur within a single goiter. The growth of thyroid nodules is independent of, and certainly not a prerequisite to, thyrotoxicosis. Rather, the appearance of thyrotoxicosis in this type of multinodular goiter depends on (1) the number of autonomous follicles throughout the gland and (2) their mean hormone-producing capacity per unit of time.


Clinical implications of a "nonspecific" transbronchial biopsy. The diagnostic accuracy of transbronchial biopsy via fiberoptic bronchoscope was reviewed in 127 noncritically ill patients. Biopsy results were analyzed according to whether a "specific" pathologic diagnosis of neoplasm, granuloma or pneumonia, or a "nonspecific" diagnosis of inflammation, fibrosis or normal lung was made. The clinical significance of a "nonspecific" biospy specimen was evaluated by clinical follow-up of at least 12 months (mean 15 months) and by grouping patients according to the type of abnormality found on chest roentgenography. Clinical follow-up was available in 119 of these patients. The over-all "specific" diagnostic yield for biopsy with secretions was 49 per cent, with transbronchial biopsy being the sole means of specific diagnosis in 14 per cent of the patients with a peripheral mass lesion, in 18 per cent of the patients with localized infiltrative processes and in 52 per cent of the patients with diffuse infiltrative processes. In 64 (52 per cent) patients both biopsy specimens and secretions were diagnostically nonspecific. In 16 (77 per cent) patients with peripheral mass lesions but nonspecific biopsy findings and secretions, neoplasm was diagnosed by more invasive procedures. However, 22 (91 per cent) patients with localized and 12 (75 per cent) patients with diffuse infiltrative processes had benign clinical follow-up suggesting that open lung biopsy in such patients should be reserved for patients with obvious clinical or roentgenographic evidence of deterioration.


Rest angina with transient S-T segment elevation. Correlation of clinical features with coronary anatomy. In 44 consecutive patients with angina at rest associated with transient S-T segment elevation, clinical features were correlated with angiographic coronary anatomy. Patients were divided into three groups depending on the number of major vessels having greater than or equal to 70 per cent luminal narrowing: Group I = no or minimal disease (six patients); group II = single vessel disease (13 patients); and group III = multiple vessel disease (25 patients). The following features did not differ significantly among groups I, II or III: age, sex, risk factors, time from onset of episodes of pain at rest to study or arrhythmias during ischemic episodes. Patients in group III were more likely to have angina on effort (p less than 0.001) and an abnormal base line electrocardiogram (p less than 0.001) than patients in groups I or II. However, the absence of these features did not separate patients in group I from those in group II. In patients with angina at rest associated with transient S-T segment elevation, clinical features identify patients with multiple vessel disease but do not allow differentiation of patients with no or minimal coronary disease from patients with single vessel disease.


Infections caused by Rhodochrous.

Infections caused by Rhodochrous. Organisms conforming to "rhodochrous taxon" were isolated from three immunocompromised patients, suggesting a pathogenic role for the organisms. The organisms are partially acid-fast, gram-positive catalase positive rods which form orange or red colonies aerobically in three or four days on Sabouraud, Mueller-Hinton and Middlebrook 7H-10 agars. They are differenitated from Nocardia by morphology and ability to degrade ethylene glycol in 7H-10 media. Two of these clinical isolates and a reference strain were injected intraperitoneally into guinea pigs, half of which received methylprednisolone intramuscularly beginning three days prior to inoculation. Steroid-treated animals exhibited clinical illness, diffuse peritonitis and recovery of inoculated organisms whereas one of three nonsteroid-treated animals exhibited a localized abscess without recovery of organisms. This study suggests that rhodochrous may be pathogenic under conditions of immune compromise.


The association between Staphylococcus aureus bacteremia and bacteriuria. The relationship between Staphylococcus aureus bacteremia and bacteriuria was studied over a five year period in three hospitals. In a Veterans Administration Hospital, 59 patients with Staph, aureus bacteremia had a urine culture within 48 hours of a positive blood culture. In 16 of 59 (27 per cent), greater than 10(5) Staph. aureus was recovered from the urine in pure culture. Six of these patients had apparent primary staphylococcal urinary tract infection. Clinical and laboratory parameters in the patients with staphylococcal bacteremia and bacteriuria were compared with those in 31 patients with staphylococcal bacteremia and sterile urine cultures. The two groups differed only in the more frequent occurrence of pyuria and proteinuria in the bacteriuric patients. In two other hospitals, staphylococcal bacteriuria occurred in 7 per cent of patients with Staph. aureus bacteremia and in 13 per cent of cases of staphylococcal endocarditis. Review of autopsy records for 33 patients who died within one month of their bacteremia failed to show a correlation between bacteriuria and the presence of renal abscess. Staphylococcal bacteriuria is a frequent and unexplained concomitant of Staph. aureus bactremia.


Autoimmune hemolytic anemia and periodic pure red cell aplasia in systemic lupus erythematosus. A patient with systemic lupus erythematosus and autoimmune hemolytic anemia complicated by periodic episodes of red cell hypoplasia is described. Using a plasma clot culture system a serum inhibitor of erythropoiesis was detected. In addition, heat eluates of the red cells of this patient were capable of impairing erythroid colony formation. The possibility that the autoantibodies of acquired autoimmune hemolytic anemia might influence the proliferation and/or maturation of erythroid progenitor cells is raised by these findings.


Progressive systemic sclerosis with eosinophilia and a fulminating course. A case of progressive systemic sclerosis, with blood and pleural fluid eosinophilia and a fulminating course, is presented. Wide-mouth colonic diverticula developed within 10 weeks. Death from renal failure occured five and a half months after the onset of symptoms. The possibility of eosinophilia as a marker of severe disease in progressive systemic sclerosis is raised.


Tuberculoid tenosynovitis and carpal tunnel syndrome caused by Mycobacterium szulgai. Mycobacterium szulgai, a scotochromogenic mycobacterium, is a newly recognized pathogen of man and has been reported to cause pulmonary infections, olecranon bursitis and cervical adenitis. We isolated M. szulfai from granulomatous tissue removed at surgery from a young florist with the carpal tunnel syndrome. The organism was susceptible to ethambutol and rifampin but resistant to isoniazid. Cure was achieved by debridement and chemotherapy with ethambutol and rifampin. Neither the source in our patient nor the natural habitat of M. szulgai is known. Because it resembles M. gordonae and M. flavescens, common scotochromogenic mycobacteria in tapwater, care must be taken to avoid dismissing M. szulgai as a contaminant when it is isolated from tissue.


Unusual cardiac, renal and pulmonary involvement in Gaucher's disease. Intersitial glucocerebroside accumulation, pulmonary hypertension and fatal bone marrow embolization. A 25 year old black woman who had manifestations of Gaucher's disease since one year of age is described. This patient had clinically significant cardiac, renal and pulmonary involvement with Gaucher's disease. Interstitial infiltration of the myocardium by Gaucher cells caused decreased left ventricular compliance and decreased cardiac output. In the kidney, Gaucher cells were present in the mesangium of the glomeruli and the interstitium of the cortex. Also, electron dense, intramembranous granular deposits were seen in glomeruli on electron microscopy. The pulmonary findings included pulmonary arterial hypertension, accentuated basilar deposition of glucocerebroside in the interstitium of alveolar septums and fatal bone marrow embolization.


Bilateral atrial myxomas. Echocardiographic considerations. In this report we describe a patient with bilateral atrial myxomas, which were diagnosed preoperatively by echocardiography and angiography, and successfully removed. The excised tumor mass consisted of mobile right and left atrial myxomas connected by a common stalk which passed through the atrial septum, collectively resembling the shape of a dumbbell. Preoperative echocardiographic and angiographic observations were instrumental in planning the surgical approach, and correlated well with intraoperative findings and with the anatomic configuration of the intact pathologic specimen. Diagnostic aspects of echocardiography are emphasized as they relate to both isolated and bilaterally-occurring atrial myxomas.


Massive osteolysis. An unusual cause of bone destruction. A patient who presented with multiple lytic lesions of bone was found to have massive osteolysis. The clinical course of this patient is described and the literature on massive osteolysis reviewed. This disease of uncertain etiology is pathologically marked by resorption of bone, and replacement by angiomas and fibrosis. X-ray findings are usually more severe than expected from the patients' clinical presentation. Laboratory studies, including attempts at biopsy, are generally unrevealing. Drug therapy is of no value, and success of surgical attempts at bone union are unpredictable. Massive osteolysis should be considered in the differential diagnosis of osteolytic lesions, particularly since this disorder can result in serious morbidity.


Reduced drug elimination in congestive heart failure. Studies using aminopyrine as a model drug. Aminopyrine disposition was studied in 11 patients with congestive heart failure (CHF) and 15 control patients. The aminopyrine metabolic clearance rate was 29.7 +/- 7.1 ml/min (mean +/- SEM) in the patients with CHF and 125.1 +/- 5.7 ml/min (mean +/- SEM) in the control patients (p less than 0.01). The aminopyrine breath test was 2.6 +/- 0.4 per cent (mean +/- SEM) in the patients with CHF and 5.6 +/- 0.3 per cent (mean +/- SEM) in the control subjects (p less than 0.01). Probably due to fluid retention in CHF, the apparent volume of distribution of aminopyrine increased to 63.3 +/- 4.9 liters (mean +/- SEM) in patients with CHF from 43.1 +/- 1.9 liters (mean +/- SEM) in control patients, thereby further impairing aminopyrine elimination in patients with CHF (p less than 0.01). The aminopyrine breath test was measured in a group of eight patients before treatment for an acute episode of CHF and seven to 10 days after initiation of therapy: in each patient clinical improvement was associated with an increased aminopyrine breath test, mean values of aminopyrine breath test increasing from 2.8 per cent before treatment to 5.2 per cent after initiation of treatment (p less than 0.01). These results suggest that in patients with CHF hepatic drug-metabolizing activity is imparied, and the volume of distribution of drugs is increased, with consequent retardation in rates of drug elimination.


Noonan syndrome in an adult family presenting with chronic lymphedema. A 27 year old man with multiple findings of the Noonan syndrome ("male Turner" phenotype) presented with chronic lymphedema which was also present in his mother. Noonan syndrome should be considered in the differential diagnosis of chronic or familial lymphedema.


Congestive heart failure as cause of fulminant hepatic failure. In this patient with long-standing cardiomyopathy and congestive heart failure the syndrome of fulminant hepatic failure developed on two occasions; he recovered both times. There was no evidence of viral or toxic hepatitis as a cause of his liver failure. We conclude that in this case, aggravation of long-standing congestive heart failure may have led to severe hepatocellular necrosis with signs of encephalopathy not commonly observed.


Primary amenorrhea associated with hirsutism, acanthosis nigricans, dermoid cysts of the ovaries and a new type of insulin resistance. We describe a 15 1/2 year old presenting with primary amenorrhea, hirsutism, acanthosis nigricans and insulin resistance. Ovarian vein catheterization studies revealed bilateral excess plasma testosterone and androstenedione secretion, and at surgery multiple dermoid cysts of both ovaries were found in association with polycystic ovaries. The suggestion that the dermoid cysts may be causative in the evolution of the polycystic ovarian disease has been made. The mechanism of the insulin resistance appears to be at the post receptor level. The acanthosis nigricans diminished following surgery with normalization of the plasma androgens.