Genetic effects of PR toxin in eukaryotic microorganisms. The genetic activity of PR toxin, a mycotoxin from Penicillium roqueforti, was studied in Saccharomyces cerevisiae and Neurospora crassa. The results show that PR toxin, without enzymic activation, causes gene conversion in S. cerevisiae strains D4 and D7, reverse mutation in S. cerevisiae strain D7 and N. crassa strain N24, and mitotic crossing-over in S. cerevisiae strain D7, In the log phase cells of S. cerevisiae the effects are more pronounced at alkaline than at acid pH. The active site responsible for the genetic activity and toxicity is known. The carbonyl groups appear to play an important role in the biologic activity of this molecule.


A colorimetric assay of lysogenic induction designed for screening potential carcinogenic and carcinostatic agents. Simple, rapid colorimetric tests for lysogenic induction (the derepression of a latent bacterial virus) are described. A quantitative test and a more rapid semiquantitative test are based on the assay of the beta-galactosidase synthesized from lacZ gene fused to an operon under lambda repressor control. These biochemical "inductests" are suitable for screening programs designed to detect agents that damage DNA and that are of potential interest in carcinogenesis and cancer chemotherapy.


7-Substituted theophyllines. Part I. Nicotinamide derivatives. Some nicotinamides derived from 7-substituted theophyllines were prepared and pharmacologically screened. They showed a very low coronarodilatory activity, a remarkable antispastic activity and a low toxicity.


Larsen's syndrome. Case report and discussion. The writers present the case of a small girl affected by Larsen's syndrome. Her brother had the same syndrome. Surgical reduction of the dislocated knees was performed at the age of two months, followed by immobilisation in a spica plaster. At operation it was found that both patellae, which are cartilaginous at that age, were absent. On the basis of recent literature and the heterogeneous manifestations of the syndrome, the authors discuss the pathogenetic theories, the differential diagnosis, and the current therapeutic possibilities.


[The advantages and disadvantages of ultrasound scanning in the diagnosis of ectopic pregnancies (author's transl)]. There is no need any more to demonstrate the value of echotomography in the diagnosis of extra-uterine pregnancies. All the same, the authors want to point out in the light of two cases they have had the rare, but real risk of errors made because of deficiencies in echography, which because they have excluded extra-uterine pregnancy lead to a delay which can be considerable in diagnosis. The first section deals with the features of diagnosis to be considered in extra-uterine pregnancy (an empty uterine cavity or, on the other hand, the presence of a pregnancy sac in the uterus; rarely the picture of an ovum in an ectopic position, but more often the picture of a haematosalpinx or a haematocoele). The risks of making mistakes because of insufficient information are described. These mistakes can be of serious consequence because they delay using the laparoscope (particularly likely to occur if there is the presence of a pseudo-sac of pregnancy due to separation of the decidua). Finally, the risks of making mistakes by having too much information are spelt out. These are less serious because they do lead to the use of the laparoscope. (They are wrongly diagnosed ovarian cyst, hydrosalpinx and double uterus.) In the last section the statistics given by various authors are reviewed.


[Severe acute genital infections (Based on 100 cases). A clinical study and the contribution of laparoscopy (author's transl)]. A continuous prospective series of 100 cases of severe acute genital infections which were collected over a period of 16 months at the Gynaecological and Obstetrical Clinic of the Saint Antoine Hospital has been studies using the same protocol. Analysing the history given by these patients before the onset of the illness shows how young they were and how frequently they were nulliparous, as well as the role of intra-uterine contraceptive devices. The correlation between the clinical features and those found on laparoscopy which was carried out almost routinely shows the great advantages of this examination. The authors in their discussion have tried, by analysing groups of symptoms, to show that to make an early correct diagnosis laparoscopy has to be used in a majority of cases. It allows treatment to be instituted quickly and unhesitatingly. Furthermore, it is justified because: It is necessary to find out all lesions that are present and which are unpredictable clinically. It renders it possible to carry out therapeutic manoeuvres (such as cutting adhesions and giving antibiotics locally) and to take swabs for bacteriological examination (this will be discussed in the second part of this article).


[Retardation of intra-uterine growth and its connexion with chromosome abnormalities (author's transl)]. 17 chromosome abnormalities were found out of 571 small-for-dates newborn babies. 7 of these were trisomy 18, 5 trisomy 21, and 2 trisomy 13. The obstetric and paediatric implications of the fact that 3 percent of small-for-dates babies are carriers of a chromosome abnormality are discussed.


[Perforating injuries of the colon: primary surgery in their management (author's transl)]. Thirty four penetrating wounds of the colon in 23 patients were treated by primary surgery. Location of the injuries on the colon, associated injuries and the nature of the missile had not affected the good quality of the results. These are encouraging an attitude towards primary surgery in the management of the colon injuries. Colostomies are still indicated for patients seen lately with severe peritoneal contamination or important lesions of the posterior abdominal wall.


[Refractory sideroblastic anaemia of primary and acquired appearance (author's transl)]. This anaemia begun at the age of 40 years. The patient died 17 years later. There was no leukaemic transformation. The authors describe the enzyme abnormalities found in the red cells (increased urosynthetase) and/for of raised serum iron levels in relatives, such that the truly acquired and apparently primary nature of this blood disease must be brought into question.


[Prognosis of infantile scoliosis (author's transl)]. 35 cases of infantile scoliosis were studied most of whom were followed for more than 10 years. In 11 instances, the scoliosis was present in the new born. The results are compared with those obtained by other authors. The authors consider that the signs found at the first examination as described by Mehta are not reliable enough and that the subsequent evolution of the curve is important. They have observed totally regressive and partially regressive cases. They are also found that right thoraco-lumbar curves in girls were common. Criteria for a more reliable prognosis are put forward.


[The treatment of chronic haematogenous osteomyelitis (author's transl)]. The authors have operated on 106 cases of chronic haematogenous osteomyelitis. On 83 occasions the surgical procedure was on the bone itself and in 23 only on the soft tissues. After a one-stage bone drilling with sequestrectomy and saucerisation the results wee satisfactory in 60 p. 100 of cases. Healing was achieved in 93 p. 100 of cases after a second, third or fourth surgical procedure. An analysis of the results has led to the following conclusion; the prognosis is better in girls, in the humerus, when the onset of the disease was subacute or when it had been present for less than 1 year.


[Lumbar venography in the diagnosis of disc herniation (author's transl)]. The authors have performed lumbar venography on 120 occasions in patients with low back pain. In 58 patients in whom the herniated disc was confirmed, the result of venography was positive and the localisation was precise in 67 cases. It is concluded that the method is safe, reliable and allows precision in diagnosis to allow better selection for surgical treatment. The examination is particularly useful in recurrent sciatica, fractures of the lumbar spine, recent or old, and in spinal stenosis. It may be associated with discography when the result is not clear cut.


[Recurrent posterior dislocation of the elbow following malunion of supracondylar fracture of the humerus. Report of a case (author's transl)]. One case of recurrent posterior dislocation of the elbow after malunion of a supracondylar fracture is reported. The malunion was a posterior angulation of the epiphysis of about 30 degrees. A good result was obtained after osteotomy to correct the malunion.


[Infected total knee prostheses treated by Papineau's technique and arthrodesis (author's transl)]. The technique of arthrodesis of the knee after removal of an infected total prostheses is described. It was used in four cases. In the first stage, the prostheses and cement were removed, the wound was left open and knee immobilized by Hoffman external fixation. Details of the fixation are given. The second stage of filling of the wound by cancellous chips was performed two weeks later. On average, the fixation was removed after seven months. Some mechanical experiments were made to establish the best type of fixation device.


[Angulation--lengthening by cylindrical osteotomy (author's transl)]. The theory and technique of cylindrical osteotomy are described. It allows correction of angulation with some lengthening of the bone. It does not allow the correction of rotational deformities. 18 cases were operated on, 4 in the tibia and 14 in the femur.


[Simultaneous bilateral traumatic dislocation of the hip. Report of a case (author's transl)]. A case of simultaneous bilateral traumatic dislocation of the hip is reported. A review of the literature shows that this lesion is rare and impressive but that its treatment is not particularly difficult.


Endotoxin fever in the newborn kitten. The role of prostaglandins and monoamines. In 5--10 day-old kittens at thermoneutral environmental temperature cerebroventricular injections of 10 microgram serotonin or noradrenaline caused hyperthermia and hypothermia, respectively. Central injections of 20 and 200 ng prostaglandin E1 induced hyperthermia. Monophasic fever followed the cerebroventricular injections of 0.2 or 0.002 microgram E. coli endotoxin, both in thermoneutral and moderately cool environments. In kittens pretreated with para-chlorophenylalanine (PCPA) the endotoxin induced rise in body temperature was attenuated within 60 to 90 min after the endotoxin. Indomethacin pretreatment prevented the first part of the febrile response and only a slight temperature rise occurred after a long latency. Central injections of phentolamine did not modify the fever response, while centrally applied propranolol modified the fever course so that it resembled that seen in PCPA treated kittens. The central mediation of endotoxin fever in the kitten is complex, despite that the pattern of the temperature change is simple (monophasic). Arachidonic acid metabolites and serotonin of the central nervous system may be involved in the reaction, while the activation of central noradrenergic mechanisms does not seem to be indispensable for the response. The changes in mediators are similar to those in newborn guinea pigs, although the fever course is different in the two species.


Visualization of collateral venous circulation during intravenous urography secondary to superior vena cava syndrome. Transient visualization of superficial collateral abdominal veins can occur in intravenous urography in some patients with the superior vena cava syndrome. The findings are characteristic enough to lead the radiologist to the correct diagnosis. A presentation and explanation of this finding is made.


Prune belly syndrome in young females.

Prune belly syndrome in young females. The cases of three females with deficiency of the abdominal muscles are reported. In one, severe urinary tract changes, typical of the prune belly syndrome in males was found. Twenty-three cases of prune belly syndrome in young females are reviewed. In over half, urinary tract changes were present. Intravenous urography and micturating cystourethrography are indicated in all young females with reduced abdominal musculature.


[Down syndrome: cardiotocographic alterations (author's transl)]. The Authors describe the results of a study on the cardiotocographic tracings during labour of pregnant women, whose fetuses were affected by Down syndrome. The high incidence of cardiotocographic alterations in these fetuses does not seem to be in relation with congenital cardiac disease.


Effect of ubiquinone-homologs on the sensitivity of mitochondrial ATPase to energy transfer inhibitors. Short-chain ubiquinone (UQ-3) abolishes oligomycin sensitivity of ATPase in submitochondrial particles and the effect is reversed by long-chain ubiquinone (UQ-7). Ubiquinone-3 also abolishes DCCD sensitivity of ATPase in submitochondrial particles but the effect is not reversed by long-chain ubiquinones. These data suggest that ubiquinone interferes with energy transfer process by interaction with mitochondrial ATPase.


The clinical significance of an elevated excretion of xanthurenic acid in psychiatric patients. This study investigations whether the synthesis of nicotinamide out of L-tryptophan is disordered in depression or in anxiety. To this end the excretion of xanthurenic acid (XA) in 24 hours urine was measured after administration of an oral loading dose of 5 grams L-tryptophan. The subjects were depressive, anxious, and alcoholic patients, while other psychiatric patients, served as control group. Anxiety and not depression is the clinical correlate of an elevated excretion of XA. Liver disorder and vitamin B-6 deficiency have to be excluded. A psychiatric control group is necessary.


The effect of glycosaminoglycans (GAG) on the intramolecular bindings of collagen. The subfractions of collagen (alpha, beta and gamma components) precipitated from neutral collagen solution by gelation corresponded to those of collagen precipitated in the same conditions (ion environments) and in the presence of chondroitin-4-sulphate. Collagen fibrils precipitated delayed in the presence of heparin poor in beta chains and rich in alpha chains. The delaying effect of oversulphated GAG on the in vitro fibril formation can be explained by the release or prevention of ion-type intramolecular bonds of collagen. The possible in vivo significance of the above bonds is discussed.


Effect of the fungal toxin (zearalenone) on the reproductive system and fertility of male and female rats. The fertility-inhibiting effects of long-term (8 weeks) consumption of maize infected with a fungus producing F2 toxin (zearalenone) was studied in adult male and female albino rats. The fertility rate was further decreased by 25-30% if the animals were kept on contaminated diet up to 14 weeks. The gonadal weight was decreased, follicular maturation and spermatogenesis were disturbed. The toxic diet consumed by mothers during pregnancy and lactation induced permanent changes in reproductive organs, disorders in vaginal cyclicity and disturbed fertility in the offspring. Neonatal administration of purified F2 toxin provoked similar changes. It is suggested that this fungal toxin may cause sterility syndrome in the offspring, similar to that produced by androgen or estrogen administration.


ATPase activity of sulfhydryl-modified cardiac myosin from normal and isoproterenol-treated rats. The possible role of sulfhydryl groups in the adaptation of cardiac myosin to work overload has been examined. The functional integrity of sulfhydryl groups was evaluated by measurement of Ca2+- and K+-(EDTA)-ATPase activities of myosins following sulfhydryl modification. No activation of Ca2+-ATPase of normal rat cardiac myosin was observed after pMB or NEM pretreatment. The decrease in Ca2+-ATPase of myosin from hypertrophied hearts was eliminated following sulfhydryl modification: moreover, slight stimulation of Ca2+-ATPase was observed. An increase in KCl concentration did not stimulate the Ca2+-ATPase of NEM-modified myosins obtained from either control or hypertrophied hearts. The sulfhydryl content of rat cardiac myosin expressed as moles of SH per 10(5) g of myosin was 6.99 +/- 0.30 and in IPR-induced hypertrophy did not change it significantly. In the authors' opinion an alteration in the integrity of the sulfhydryl groups may be responsible for the functional partition (decreased Ca2+-ATpase with unchanged K+-[EDTA]-ATPase activity) of myosin from hypertrophied hearts.


Hybridization of DNA's from Neurospora crassa strains may indicate base sequence alterations as a consequence of genetic transformation. Deoxyribonucleic acids of Neurospora crassa strains involved in genetic transformation experiments were studied by means of DNA-DNA hybridization. No significant difference was detected in the extent of hybridization reassociating 32P-DNA of an inositol-requiring recipient strain with an excess amount of unlabelled homologous DNA and that of the transformed, spontaneous revertant and wild-type strains. Studies on the thermal stability of hybrids revealed 1.2-1.7% heterology between the recipient and transformant DNA's. The spontaneous revertant and wild-type strains proved to be homologous with the recipient strain. We suppose that the heterology we measured is the result of the alteration of the nucleotide sequences caused by the multilocal integration of transforming DNA into the recipient genome.


[Immunomechanisms in drug-induced hemolytic anemias]. Among the acquired hemolytic anemias, drug-induced immune hemolytic anemias (IHA) play a significant role. Many different drugs are capable of causing IHA, alphamethyldopa and penicillins have been responsible for the greatest number of cases. For the majority of the other drugs single cases of IHA are reported. Drug-induced immune hemolysis may be mediated by different immune mechanisms including the immune cytotoxicity type (penicillins and cephalosporins) with predominantly extravascular hemolysis. Immune hemolysis of most other drugs is due to immune complexes formed by the drug and specific antibodies leading to intravascular hemolysis in the presence of complement activation. So far, the autoimmune type of IHA caused by alphamethyldopa is unexplained, serologically this form cannot be distinguished from warm antibody auto-IHA. The clinical importance of drug-induced IHA depends on the severity of anemia and the problem of diagnosis leading to adequate therapeutic measures. Only a small number of cases showing a positive direct antiglobulin (Coombs') test caused by drugs will develop IHA. Therefore this finding does not require absolutely withdrawal of the drug, but careful observation of the patient. The diagnosis of drug-induced IHA is necessary for its distinction from auto-IHA and other secondary IHA. The study of such IHA has great importance for the understanding of other drug-induced cytopenias and may give explanations of the pathogenetic mechanisms of yet unexplained autoimmune phenomena.


[The hemolytic syndrome in subacute bacterial endocarditis]. Long lasting subacute bacterial endocarditis often presents with marked anemia. Infective mechanisms and hemolysis are considered as most important pathophysiological mechanisms. Pronounced hemolytic anemia, thrombocytopenia and edema observed in two cases are possible misleading symptoms in the diagnosis of subacute bacterial endocarditis.


[Clinical relevance of circulating immune complexes in patients with systemic lupus erythematosus and chronic polyarthritis]. Using a solid phase C1q assay, circulating immune complexes (IC) were detected in 15 of 55 patients with rheumatoid arthritis (RA) and 6 of 14 patients with systemic lupus erythematosus (SLE). In contrast to RA, presence of IC correlated to clinical disease activity in SLE patients. Preliminary short term follow up studies revealed additional differences between RA and SLE: whereas persistence of IC was noted in the patients with SLE, rapid changes of IC levels were observed in RA.


[Essential cryoglobulinemia with accompanying angioneurotic edema]. A case report of a patient with essential cryoglobulinemia and episodes of angioedema caused by an acquired C1 inhibitor deficiency is presented. The patient had high levels of circulating immune complexes. In the course of complement activation C1 inhibitor may be consumed and the resulting C1 inhibitor deficiency led to the occurrence of angioedema.


[A method for the detection of suppressor lymphocytes and its clinical use]. Suppressor cells play a major role in immune regulation and supervision. Unspecific suppressor cells can be stimulated by Concanavalin A (ConA) in a concentration of 1-50 micrograms/ml. Absorbed pooled human AB serum is the optimal serum source. Monocytes are necessary to induce suppressor cells. Preincubation of lymphocytes abolishes the suppressor cell inducibility. Different ConA incubation periods show two suppressor peaks, the first on day 0-1, the second on day 4-6. It is concluded, therefore, that we deal with two populations of suppressor cells. The first of these seems to resemble the spontaneous in vivo suppressor level, needs not to be activated by ConA but is rapidly inactivated without permanent antigeneic stimulus. The second peak consists of suppressor cells which are activated by a ConA stimulation of at least 3-4 days. Another conclusion can be drawn from these data: The conventional control system for the ConA induction of suppressor cells is not the optimal one, because the spontaneous suppressor cells are not included within this system, although they perhaps give a better insight into the actual immune status.


[Effect of motor stimulation and stretching on afferent activity of the neuromuscular spindle isolated from the frog]. The arrangement of muscle spindles in m. ext. long. dig. IV has been examined by microdissection. It is confirmed that spindle systems generally appear to consist of individual receptors. Stimulation effects of fast motor fibres (conduction velocities greater than 12 m/sec) on the spindles of the same muscle were studied. Receptors were isolated with their nerves and the appropriate spinal roots, the latter ones were used for stimulating efferent fibres and recording sensory discharges. Single shocks to the ventral root filaments caused afferent responses ranging from a single action potential to a train of impulses. During repetitive stimulation (train of stimuli at frequency of 10 to 150/sec) a marked increase in afferent activity was found. Afferent activity could be driven by the frequency of stimuli ("driving") and the stimulus/action potentials ratio varied from 1:1 to 1:3 or more. The rate of sensory discharge depended on the frequency of stimuli: the maximum effect, was attained at 30 to 50 stimuli/sec and, in the most responsive receptors, up to 80 stimuli/sec. Slight increases of the initial lengths of the receptors caused facilitation of sensory responses to motor stimulation. Moreover, impairing effects, which appear during sustained or high-frequency stimulation, possibly related to fatigue in intrafusal neuromuscular transmission, could be relieved by increasing the initial length. The repetitive stimulation of fast fusimotor fibres increased both dynamic and static responses and also raised the afferent activity after a period of stretching, when usually a depression occurs; these effects varied according to the preparation, its initial tension and the frequency of stimulation. The main feature of the examined motor fibres, when stimulated, is the constant excitatory action on muscle spindle static response. Results are discussed. It is suggested that the different characteristics of intrafusal muscle fibres, the receptor initial tension and the frequency of motor units discharges, may together affect muscle spindles static or dynamic performance.


[Effects of acetylcholine and succinylcholine on isolated frog neuromuscular spindle]. The mode of action of acetylcholine (ACh) and succinylcholine (SCh) on the isolated frog's muscle spindle has been studied. Receptor afferent nervous supply was maintained; the appropriate spinal roots were dissected for stimulating motor axons and recording from sensory fibres. Excitatory effects on the afferent activity, when the receptor was held still and during stretching, were found with ACh or SCh concentrations of 10(-8) to 10(-3); 10(-6) g/ml being usually effective. These effects are similar to those obtained by stimulating fusimotor nerve fibres. The contractile activity of intrafusal muscle fibres which occurred during these effects was observed. Seldom, and only for high concentrations of ACh and SCh, a decrease in afferent activity following the excitatory effects was found. Tubocurarine chloride (10(-5)-10(04) g/ml) in the bath prevented both motor fibres and drugs effects. Sometimes slight transient excitation occurred at very high concentrations of the two tested substances; however, this effect was prevented by stronger curarization. The observed blocking effects were always reversed by removing tubocurarine from the bath. No more excitatory effects by motor fibres stimulation and by ACh and SCh action could be found after destruction of intrafusal muscle fibres, by pinching them as close as possible to the ends of the spindle. It is suggested that ACh and SCh act indirectly by causing mechanical changes in intrafusal muscle fibres, and that a direct action on sensory nerve endings, if any, cannot, by itself, increase the afferent activity of the receptor.


Circadian periodicity of plasma prolactin in some neurological diseases. The circadian rhythmicity of plasma PRL has been studied in some neurological diseases in which hypothalamic involvement or abnormalities of brain neurotransmitters has been postulated. 11 patients with Steinert's myotonic dystrophy, 7 with cluster headache and 10 with Huntington's chorea have been studied. By the mean cosinor procedure, a significant circadian rhythm of plasma PRL has been observed both in Steinert's disease and in cluster headache, whereas a circadian periodicity is not detectable in Huntington's chorea, a degenerative disorder affecting the basal ganglia, the hypothalamus and many other areas of the CNS.


The stimulation of photophosphorylation and ATPase by artificial redox mediators in chromatophores of Rhodopseudomonas capsulata at different redox potentials. (1) Inhibition of cyclic phosphorylation in chromatophores of Rhodopseudomonas capsulata by antimycin A can be fully reversed by artificial redox mediators, provided the ambient redox potential is maintained around 200 mV. The redox mediator need not be a hydrogen carrier in its reduced form, N-methyl-phenazonium methosulfate and N,N,N',N'-tetramethyl-p-phenylenediamine being equally effective. However, the mediator needs to be lipophilic. Endogenous cyclic phosphorylation is fastest around 130 mV. A shift to 200 mV can also be observed if high concentrations of artificial redox mediator are present in the absence of antimycin. (2) ATPase activity of Rhodopseudomonas capsulata, in the light as well as in the dark, activated or not activated by inorganic phosphate, can also be stimulated by N-methylphenazonium methosulfate. This stimulation is highest at redox potentials between 60 to 80 mV and is sensitive to antimycin A. In this case N,N,N',N-tetramethyl-p-phenylenediamine is much less effective.


Low-back pain among electric power supply workers and their attitude toward its prevention and the treatment. Low-back pain among electric power supply workers and their opinions about the ways to prevent and relieve the disorder were investigated. A questionnaire study revealed that 38.7% of 6,272 electric power supply workers were suffering from low-back pain, including severe pain resulting in frequent absenteeism or requiring occasional rest from work. Chronological analysis of the occurrence of the disorder revealed that the incidence of gradually developing pain has increased and that improper working posture and overwork have become more important as etiological factors. Rest from work was found to be the most effective way of relieving pain, while any medical treatment which did not include rest was useless. Low-back pain among these workers is believed to have resulted from rapid and intensified work as required by increasing demand and mechanization, greater mental stress and improper working posture. As preventive measures, it is necessary to improve working hours and to lighten the rate of work. In order to relieve pain, the employers should permit the workers to absent themselves from work quickly following an attack. Discrepancy between workers' opinions and both actual causation of low-back pain and actual effectiveness of medical treatment is discussed.


[Behavior of certain acid hydrolases in the central nervous systems of various mammals]. The activity of several acid hydrolases in different sections of the C.N.S. of various mammalian has been evaluated. The enzymes which have been studied are: beta-glucosidase, beta-galactosidase, beta-N-acetylglucosaminidase, beta-N-acetylgalactosaminidase, beta-glucuronidase, alpha-mannosidase. The enzymes show a higher activity in the gray matter than in the white matter. The results are interpreted on the assumption that glyco-lipoprotein turnover is higher in the gray matter than in the white matter, since myelin, which is the major component of the white matter, is a relatively stable structures.


[A case of relapsing mesenchymatous tumour of the abdominal wall with metastases (author's transl)]. It is classical to consider desmoid tumours as an entity with only a local prognosis, conditioned by the frequency of relapses. In this respect, they are different from other mesenchymatous tumours, fibrosarcomas in particular which have a poorer prognosis. The authors report the case of a 23 years old man with a tumour of the abdominal wall, which in the early stages resembled a desmoid tumour, but the development of metastases suggested a sarcoma. This observation seems to make doubtful the distinction between desmoid tumour and fibrosarcoma, and thus the prognosis of tumours with a desmoid appearance. Under these conditions, this term should only be used for descriptive purposes and has no prognostic value.


[Epidemiological aspects of Huntington chorea in the Genoa region from 1930 to 1977 (author's transl)]. As a preliminary approach to an epidemiological investigation of Huntington's disease, the authors report the incidence of admissions of these patients to both Neurological and Psychiatric Departments in Genoa and surrounding region from 1930 to 1977. The number of patients (84 cases) and their family history (225 cases of Chorea in the ancestors) suggest a prevalence of Huntington's disease remarkably higher (about 28 cases out of 100,000 inhabitants) than it is usually estimated. Further studies are however required to assess the distribution of the disease in the whole population of the region.


[Early control laparoscopy after tubal microsurgery (author's transl)]. It is essential to carry out laparoscopic control to determine the result of plastic tubal surgery. This is classically done 12 to 18 months after the operation, yet it has been suggested that this control should be carried out earlier between the 4th and the 8th weeks or even on the 8th day following the operation. The authors have carried out 63 early control laparoscopies after microsurgical operations on the tube at different dates: 33 times between 4 and 8 weeks after the operation, 5 times between the 10th and the 13th day, and 25 times on the 8th day. They are in favour of early laparoscopic control with the reserve that it will require much more experience to tell whether the procedure is harmless.


R.H.I.S.A. -- cysternography study in sporadic choreo-athetotic syndrome accompanied with dementia (sporadic Huntington disease). Seven patients who suffered from choreo-athetotic movements, accompanied by slowly progressive mental and affective decline over several years, without hereditary background, were admitted for clinical and psychodiagnostic tests. Laboratory examinations and anamnestic data were negative, and therefore a presumed diagnosis of sporadic Huntington disease was made. In order to verify the diagnosis, we had done P.E.G. -- Contrast study, and R.H.I.S.A. -- Cysternography. The P.E.G. -- Contrast demonstrated an enlargement of the sub-arachnoidal space and a symmetrical enlargement of the ventricular System, that may represent cortical and sub-cortical atrophy. The R.H.I.S.A. -- Cysternography Study showed in our patients the characteristics of the Mixed type Pattern: combined ventricular penetration with delayed para-sagittal absorption. Considering the results of the R.H.I.S.A. Studies, that correspond to the P.E.G. -- Contrast Studies, and its invulnerability towards patients, we presume that R.H.I.S.A. may be preferred as a diagnostic tool in Sporadic Huntington Disease.


Carcinogenic activity in Sprague-Dawley rats of 2-[3-(2-chloroethyl)-3-nitrosoureido]-D-gluco-pyranose (chlorozotocin). Sprague--Dawley rats of both sexes received 2 mg/kg body wt (group 1) or 0.4 mg/kg body wt (group 2) of 2-[3-(2-chloroethyl)-3-nitrosoureido]-D-glucopyranose (chlorozotocin) intraperitoneally in weekly injections for life. A dose-dependent carcinogenic effect of chlorozotocin was found (animals with malignant tumors in group 1: males 88%, females 89%, in group 2: males 83%, females 63%, in controls: males 10%, females 24%). Chlorozotocin reduced the life expectancy of treated animals significantly, compared to solvent controls. Histologically, the chlorozotocin-induced tumors were shown to be mesotheliomas or sarcomas of the peritoneal cavity (undifferentiated type, fibrosarcomas, myosarcomas).


The fate of [14C]streptozotocin in nicotinamide-pretreated mice: observations on pancreatic islet radioactivity and urinary N1-methyl-14C]nicotinamide-excretion. A high labelling of the pancreatic islets was found 3 and 24 h after a diabetogenic dose of [14C]streptozotocin to mice in which the acids islet injury had been prevented by nicotinamide-pretreatment. In non-pretreated [14C]streptozotocin-injected mice, a much lower radioactivity was observed in the pancreatic islets; at 3 h and at 24 h, there was no detectable radioactivity in the islets. No evidence was found to indicate that nicotinamide-pretreatment had any marked effect on the uptake or retention of radioactivity in other tissues. N1-[methyl-14C]nicotinamide was not found in the urine of non-pretreated [14C]streptozotocin-injected mice. When the animals were pretreated with nicotinamide, N1-[methyl-14C]nicotinamide was detected in the urine, but this represented only a small fraction of the injected radioactivity and of the excreted N1-methylnicotinamide. This result does not support the hypothesis that the disturbance of the NAD-metabolism, which streptozotocin causes, is due to a methylation of nicotinamide.


[Circulating immune complexes in patients with malignant diseases]. Circulating immune complexes were determined by a solid phase C1q assay in patients with divers malignancies. In Hodgkin's disease (n = 20), immune complexes were detected only in two patients during chemotherapy. Both patients succumbed shortly thereafter. In patients with testicular malignancies (n = 18), immune complexes were detected especially in patients without evidence of metastatic disease. Sequential investigations did not reveal prognostic insights. Summing up these data and the data obtained in additional patients with malignancies, it is interesting to note, that patients with carcinomas have a significant higher incidence of circulating immune complexes with malignancies of the lymphatic system. Our data, however, do not exclude presence of other than C1q binding immune complexes.


[A new insulin radioimmunotest and its use in the determination of anti-insulin antibodies]. Acetylation of insulin with 3H-acetic anhydride resulted in the predominant 3H-labeling at the aminoterminal position of the B-chain (B1). The labeled insulin was unchanged in its immunologic reactivity as compared to native insulin. It was used in a second antibody radioimmunoassay for the detection of antiinsulin-antibodies in insulin-dependent patients with Diabetes mellitus.


[P wave analysis for the electrocardiographic diagnosis of left ventricular hypertrophy. A study of a population with arterial hypertension (author's transl)]. The electrocardiogram of 100 normal subjects and 216 patients with arterial hypertension in different stages have been analysed. P wave especially has been taken into consideration: it is often abnormal in the presence of left ventricular hypertrophy (LVI). The point-score system elaborated by Romhilt and Estes allows, if only QRS-T is considered, to diagnose LVI in 32 of the hypertensive patients (15%) and in 41 cases (19%) if the increase of P wave terminal forces is emphasized. Nevertheless this P wave characteristic does not appear more useful than others (axis less than or equal to 40 degrees; duration greater than or equal to 0.12 sec; presence of notch). The best results are achieved by considering the coexistence of two or more abnormalities of the P wave. Thus LVI is diagnosed in 52 (24%) of our cases, without false positive.


Correlated effects of cigarette smoke components on alveolar macrophage adenosine triphosphatase activity and phagocytosis. An initial examination was made of the hypothesis that one action of cigarette smoke components on pulmonary alveolar macrophage function involves the inhibition of contractile protein adenosine triphosphatase activity. Pulmonary alveolar macrophage calcium-dependent adenosine triphosphatase activity, magnesium-dependent adenosine triphosphatase activity, sodium-potassium-dependent adenosine triphosphatase activity, phagocytosis, and cell adhesiveness were measured in the presence of cigarette smoke, acrolein, ouabain, and ethacrynic acid. Calcium-dependent adenosine triphosphatase activity, magnesium-dependent adenosine triphosphatase activity, phagocytosis, and adhesiveness were inhibited by smoke and ethacrynic acid, but not by ouabain. Acrolein, a component of smoke, inhibited phagocytosis, adhesiveness, and calcium-dependent adenosine triphosphatase activity, indicating that another component of smoke must be effective at inhibiting magnesium-dependent adenosine triphosphatase activity. Sodium-potassium-dependent adenosine triphosphatase activity was inhibited by ouabain and ethacrynic acid, but not by smoke or acrolein. Finally, sulfhydryl reagents at least partially protected the macrophages against the inhibitory actions of each of the agents. The results are in accord with recently obtained experimental evidence that calcium-dependent adenosine triphosphatase and, perhaps, magnesium-dependent adenosine triphosphatase play a role in phagocytosis. The data also suggest that smoke components affect a number of macrophage activities, including adhesion and phagocytosis, by altering the cell's contractile apparatus.


Erythrocyte enzyme deficiencies assessed with a miniature centrifugal analyzer. Methods for assaying 16 erythrocyte enzymes have been adapted to the miniature centrifugal analyzer. Less than 15 micro L of whole blood is required for all 16 assays. Variation attributable to temporal effects, rotor effects, and random residual error is minor. Initial population studies of blood from adults and cord-blood samples suggest a CV of less than 12% for 12 of the 16 enzymes; thus it should be possible to identify the heterozygous deficient individual. Preliminary data suggest that three such individuals, with enzyme activity (adenylate kinase, pyruvate kinase, phosphoglycerate kinase) about half the expected, have been identified, as well as two individuals deficient in glucose-6-phosphate dehydrogenase.


Psychiatric liaison nurse for neurosurgery: an innovative approach to management of chronic pain. A fundamental aim of the Neurosurgical Unit at Georgia Baptist Medical Center is to enable each individual to return to normal and meaningful functioning. The problem of chronic pain almost always results in a steady decrease in those activities, interests, and concerns which are essential to the normal process of living. When the process is disrupted, the result is usually a feeling of despair and uselessness. These problems will almost inevitably complicate the pain experience. In addition, the ever present stress that accompanies severe and chronic problems of any sort tends to result in related psychological difficulties such as depression, anxiety, feelings of inadequacy and a multitude of other family and personal problems. These difficulties often become major features of an individual's pain problem. A holistic approach to patient care is based on the concept that each individual needs to be considered physically, psychologically and spiritually. The active participation of the psychiatric liaison nurse as a member of the neurosurgical team helps integrate the forces that enable such an approach. By focusing on a thorough patient assessment, improved staff morale and improved well being of patients, the psychiatric liaison nurse assists the team in focusing its energy on total comprehensive patient care. The combination of clinical neurosurgical treatment and psychological care has proven effective in helping our neurosurgical team achieve the fundamental goal toward which we all work.


[Acute neurogenic pulmonary edema].

[Acute neurogenic pulmonary edema]. A case of acute pulmonary edema after subarachnoid hemorrhage is presented. A supra and infratentorial arteriovenous malformation was revealed by serial cerebral angiography. Sequential chest films documented regression of both, pulmonary edema and cardiac enlargment. The literature is reviewed and a possible physiopathology is discussed.


[Vectorcardiographic findings in atrial enlargement]. Rational interpretation of changes of the P loop, due to atrial enlargements, must be based on the magnitude and spatial orientation of the main resulting vectors of the atrial activation phenomenon. Under normal conditions, these ones give origin to a mean vector oriented to the left, downward and slightly forward. Right atrial enlargement can be recognized by P loops of more than 100 mcv. The main axis of the elongated PF is comprised between + 60 degrees and + 90 degrees; that of the open PH between + 70 degrees and + 80 degrees and that of PS is located around + 135 degrees. Left atrial enlargement can be diagnosed by a characteristic box glove configuration of the PF loop as well as by an eight-shaped PH. In some cases, the PS loop also becomes eight-shaped. Biatrial enlargement gives rise to slurrings and notchings of the distal portion of the PF loop, a diphasic aspect and frequently an eight-shaped configuration of the PH loop and a triangular morphology of PS, whose base lasts 30 msec. or more.


Bend propagation in flagella. II. Incorporation of dynein cross-bridge kinetics into the equations of motion. The cross-bridge formalism of T. Hill has been incorporated into the nonlinear differential equations describing planar flagellar motion in an external viscous medium. A stable numerical procedure for solution of these equations is presented. A self-consistent two-state diagram with curvature-dependent rate functions is sufficient to generate stable propagating waves with frequencies and amplitudes typical of sperm flagella. For a particular choice of attachment and detachment rate functions, reasonable variation of frequency and wave speed with increasing viscosity is also obtained. The method can easily be extended to study more realistic state diagrams.


Zeaxanthin ([3R,3'R]-beta, beta-carotene-3-3'diol) as a resonance Raman and visible absorption probe of membrane structure. When zeaxanthin ([3R,3R']-beta, beta-carotene-3,3'diol) is inserted into phospholipid dispersions and the latter heated through their gel-liquid crystal phase transitions, large changes are noted in the resonance Raman and absorption spectra of the carotenoid molecule. By analogy with the data of Carey and co-workers (J. Raman Spectrosc. 6:282) who studied the aggregation of zeaxanthin in acetone-water solutions, it is suggested that the carotenoid aggregates in the phospholipid gel state while forming a monomer in liquid crystal phases. The alterations in both the visible absorption and resonance Raman data have been used to monitor phospholipid phase behavior in dipalmitoylphosphatidylcholine and distearoylphosphatidylcholine, (DSPC) one-component systems and binary mixtures. The phase diagram obtained for the binary system, as constructed from visible absorption and resonance Raman data, is compared with that of Shimshick and McConnell (Biochemistry. 12:2351) obtained from electron spin resonance (ESR) studies. Although the agreement between absorption and ESR data is generally satisfactory, onset temperatures for phase separation at low DSPC mole fractions deduced from resonance Raman measurements are several degrees lower than those from the other methods. Nevertheless, the use of zeaxanthin as a resonance Raman and visible absorption probe behavior will be useful in some situations where ordinary Raman spectroscopic data cannot be obtained easily. The advantage of the resonance Raman approach is illustrated in a study of the phase behavior of a phospholipid extract of a cel- mutant of Neurospora crassa. A phase separation region is observed with onset and completion temperatures of -19 and -6 degrees C, respectively.


Inhibition of the humoral response by spleen cells from cyclophosphamide-treated mice. Spleen cells obtained from mice injected with cyclophosphamide (200 mg/kg body weight) suppressed the secondary IgG antibody response of memory cells to a T-dependent antigen, DNP-HGG, in Millipore diffusion chambers. Significant suppression (greater than 50%) was found from 5 to 14 days following cyclophosphamide treatment, with peak suppression (86%) on day 7. The primary IgM antibody response to DNP-Ficoll, a T-independent antigen, was not suppressed by these cells. In contrast, suppression was observed in the primary IgM response to sheep red blood cells, a T-dependent antigen. In addition, treatment of the suppressor cell population with anti-Thy-1 serum and complement did not inhibit suppressor activity. We concluded that the suppressor activity was not attributable to a typical T cell, and that the target of suppression was not a B cell. Preliminary evidence suggests that the suppressor activity is regulated, directly or indirectly, by a T cell.


Initial experience with transluminal recanalization of the recently occluded infarct-related coronary artery in acute myocardial infarction -- comparison with conventionally treated patients. In 7 patients, the recently occluded infarct-related vessel was recanalized by transluminal catheter techniques during acute myocardial infarction (Group A). 4 patients had single-vessel disease, 2 patients two-vessels disease and one, involvement of three vessels. Control angiography was performed in 6 patients, 8 days to 7 months later. Changes of coronary artery anatomy and left ventricular function were compared with a group of 9 conventionally treated patients, who were found to have occlusion of the infarct-related vessel in the acute stage (Group B). Five Group B patients had one-vessel disease, 3 patients two-vessel disease and 1 patient, involvement of all three vessels. In the chronic stage, all transluminally recanalized vessels were found to be patent in Group A. There was spontaneous recanalization of the infarct vessel in 4 of 9 Group B patients. In Group A, the length of the akinetic segment (AKS) decreased significantly (p less than 0.05) from 145.4 +/- 48.5 mm to 73.2 +/- 73.4 mm (mean +/- SD). Volume parameters did not change significantly. In Group B, length of the AKS did not change significantly, EDVI increased significantly from 81.1 +/- 19.8 to 106.8 +/- 4.6 ml/m2 (p less than 0.05); ESVI increased significantly from 41.7 +/- 13.7; ml/m2 to 66.8 +/- 37.9 ml/m2 (p less than 0.01). In the acute stage, length of the AKS and volume parameters did not differ significantly between the two groups. In the chronic stage, AKS was significantly shorter (A: 73.2 +/- 63.4 mm; 144.9 +/- 59 mm (p less than 0.0025) and EF was significantly higher (A: 54.6 +/- 11.6%; B: 40.9 +/- 14.5% (p less than 0.05) in Group A. Peak CPK was lower in Group A (A: 1009 +/- 827 U/l; B: 1324 +/- 655 U/l), but this difference did not achieve statistical significance. Results of this pilot study suggest that transluminal recanalization in the early phases of acute myocardial infarction might result in limitation of myocardial injury. However, further research will be needed to improve the technique and to test its results.


Role of hCG in regulation of the fetal zone of the human fetal adrenal gland. It has been suggested that hCG is a trophic hormone for the fetal zone of the human fetal adrenal gland. To test this hypothesis, the isolated fetal zones of adrenals from eight fetuses (12-17-week gestation age) were superfused in the presence or absence of hCG. Dehydroepiandrosterone sulfate (DHAS) was measured in the superfusion effluent. A significant increase in DHAS production was observed in the presence of hCG. DHAS secretion decreased during the first 60 min in the control and experimental superfusions from 83 +/- 10.0 (mean +/- SE) to 71 +/- 8.0, and from 90 +/- 9.0 to 70 +/- 6.0 ng/100 mg/ml, respectively. In the presence of hCG (250 ng/ml), DHAS secretion increased significantly (P less than 0.01) over the controls to 116 +/- 12.0 at 120 min, and remained above the controls thereafter. These results support the hypothesis that hCG is one of the regulators of DHAS production by the human fetal adrenal gland early in gestation. As we found that ACTh stimulated DHAS secretion in a previous study and as there is indirect evidence for a role of ACTH in DHAS regulation late in pregnancy, these observations suggest dual regulation by hCG and ACTH early in pregnancy, and a possible transition to ACTH regulation of the fetal zone of the human fetal adrenal after midgestation.


Direct animal and indirect human evidence of altered platelet function in diabetics. All agree on altered platelet function in vitro (and increasingly in vivo) in diabetics of substantial duration and/or with clinical evidence of angiopathy. However, a platelet abnormality earlier in the disease remains uncertain. Three sets of data from Oxford will be reviewed: (1) Observations of Honour on platelet aggregation at sites of minimal injury within blood vessels of anesthetized rabbits, with greater sensitivity to superfused ADP when hyperglycemia has followed alloxan only days previously. This increased aggregatability (not hyperglycemia determined) is reversed by a few days of insulin treatment or by dipyrimadole (alone or with synergistic acetyl salicylic acid): (2) Beta-thromboglobulin is released from platelets and is increased in venesected blood from diabetics after a standardized procedure (no prostaglandin E1 in anticoagulant) with final radioimmunoassay. Results in diabetics after surgery, etc., will also be presented, and (3) in a prospective study of newly-diagnosed, mostly maturity-onset type diabetics, an increase in plasma fibrinogen (thrombin coagulation of plasma, controlled against normals) was observed during the first 3 yr, largely due to males treated with sulfonylureas; decreases in platelet count and in prothrombin concentration were also statistically significant.


Augmented venous return: protection of the ischemic myocardium during endotoxemia. The effects of gram-negative endotoxin (Escherichia coli 4 mg/kg) induced myocardial failure in the pentobarbital-anesthesized dog were examined by monitoring its influence on coronary and systemic hemodynamics and correlating these results with myofibrillar ATPase activity. An identical series of studies was performed incorporating a femoral-femoral arterial-venous shunt to augment venous return. Over a five-hour period, gram-negative endotoxemia was associated with a progressive fall in arterial pressure, stroke volume, cardiac output, coronary flow, and a rise in total peripheral resistance and diastolic coronary vascular resistance. Augmenting venous return by 313 +/- 71 ml/min significantly increased cardiac output, stroke volume, and coronary flow and substantially reduced total peripheral resistance and coronary vascular resistance. Myofibrillar ATPase activity from both endocardium and epicardium was significantly depressed in the endotoxin-shocked preparations. However, with the augmentation of venous return with the A-V shunt in the endotoxin-treated animal, myofibrillar ATPase activity is normal. It appears that endotoxin causes a decrease both in venous return and in cardiac contractility by increasing total peripheral resistance, coronary vascular resistance, and impedance to left ventricular ejection. Augmenting venous return by optimizing preload and reducing afterload prevents any significant increase in total peripheral resistance, coronary vascular resistance, or impedance to left ventricular ejection. This is manifested by a rise in cardiac output, stroke volume, and coronary flow and the preservation of myocardial function. This is the first successful application of left ventricular afterload reduction in noncardiogenic shock.


[Vectorcardiographic manifestations of right ventricular enlargement]. The basic criteria for the vectorcardiographic diagnosis of right ventricular enlargement are discussed, in context with the course of myocardial activation. Right ventricular dilatation, secondary to isolated diastolic overloading (atrial septal defect) shows basically different degrees of dextrorotation. The ventricular curve starts to the left on the frontal and horizontal planes, and forward on the last one. Cases with right ventricular hypertrophy, produced by sustained systolic overload, are also evaluated. When the hypertrophy is generalized (pulmonary valvular stenosis), there is an increase in the manifestation of all the resulting vectors of activation of this ventricle: IIs, IIr, and IIIr. As a resultant of these changes, the ventricular curve presents a clockwise rotation in the three planes, and is oriented to the right and forward, with its terminal portions generally located above the E point. When the right ventricular hypertrophy is of the segmentary type, there is an increase of the manifestation of only some of the resulting vectors of the activation of this ventricle. For example, the vector IIr will be increased in cases of tetralogy of Fallot, while the IIIr will be increased in some cases of obstructive chronic pulmonary hypertensive cardiopathy. The T loop, of secondary type, generally opposes the vector IIr on the horizontal plane, and the IIIr on the frontal plane. When an important right ventricular dilatation is associated to a right bundle branch block of intermediate degree, owing to their proximity, the manifestation of the electromotive parietal forces is increased at the expense of the septal ones. This phenomenon produces a characteristic appearance of the SH loop, narrow and with a clockwise rotation.


[Vectorcardiographic manifestations of left ventricular and biventricular enlargement]. The basic criteria for the vectorcardiographic diagnosis of left ventricular and biventricular enlargements are discussed on the basis of the myocardial activation sequence. Left ventricular dilatation, secondary to isolated diastolic overloading, increases the manifestation of all the vectors resulting of the activation of this ventricle. These changes reflect the proximity of the left ventricular walls to the exploring electrodes. The vectors above mentioned project themselves as wide ventricular curves with counterclockwise rotation on the three planes. The T loop, of secondary type, is concordant in its orientation with the R loop. Cases with left ventricular hypertrophy, produced by a sustained systolic overloading, are also described. In the presence of global left ventricular hypertrophy without LBBB, the manifestation of all the vectors resulting from the depolarization of this ventricle (I, IIl, IIIl), is increased. This is due to a prolonged duration of the corresponding activation fronts. These vectors are projected on the different segments of the ventricular curves and they show a counterclockwise rotation on the three planes. When LBBB is also present, the first septal vector is not evident. The T loop, of secondary type, opposes the R loop on the frontal and horizontal planes. The presence of left ventricular hypertrophy of the segmentary type, generally increases the manifestation of the vector I, and sometimes, also that of the vector IIIl. When both ventricles are hypertrophied, the electromotive forces of the chamber more severely affected predominate in the vectorcardiographic records.


Isoenzyme characterization of Leishmania spp from Algeria. Five Algerian stocks of Leishmania were compared with two stocks from USSR and one from Sudan by means of isoenzyme electrophoresis of two enzymes: glucose-phosphate isomerase (GPI) and phosphoglucomutase (PGM). Three stocks isolated from patients suffering from cutaneous leishmaniasis appeared to be L. tropica major. This correlated well the epidemiological features of the endemic area from where the stocks originated. Two stocks isolated from patients suffering from visceral leishmaniasis appeared to be different from the Sudanese stock of L. donovani.


Psychosis as a predictor of response to lithium maintenance treatment in bipolar affective disorder. Sixty-six bipolar I lithium clinic patients were studied for a history of psychotic symptoms at some time during the course of their illness. Agreement between different sources of information was calculated, and the patient population was divided into psychotic and non-psychotic subgroups. Probability of remaining well on lithium for the different subgroups was analyzed by the life table method. Psychosis during mania appeared to be associated with especially good early lithium prophylaxis.


Assortative mating and affective disorders. Seventy-two spouses of subjects with recurrent primary affective disorders (PAD), were investigated for the presence of psychiatric disorders in their lives and in those of their first degree relatives, and compared with 71 spouses of non-psychiatrically ill control subjects. No difference was found in the risk for PAD; on the other hand spouses of affective patients manifested a greater occurrence of psychiatric disorders belonging to the affective spectrum, as did their respective first-degree relatives.


Detecting depressive disorders in drug abusers: a comparison of screening instruments. Previous investigators have reported a high prevalence of depressive symptoms in drug-dependent patients. Given the responsiveness of depressive disorders to both psychological and pharmacological treatments, it is desirable to find an economical, efficient screening instrument to detect depressive disorders in this population. In this study, 6 depression symptom screening scales (Beck Depression Inventory, Hamilton Depression Scale, Raskin Depression Scale, Degree of Illness Rating, Symptom Checklist 90 Overall, and Depression Subscale) based on either clinician interview or patient self report, were compared according to their utility in detecting cases of depression among 64 applicants for treatment at a substance abuse treatment unit of a community mental health center. The criteria for a case of depression were the Research Diagnostic Criteria (RDC) which are specified and operationalized. Cases identified using previously described cutoff scores on the screening scales were compared to rates based on the RDC and sensitivity and specificity were determined. The results showed that: (1) although the sensitivity of the symptom scales was applicable, ranging from 65--94%, the specificity was less impressive, ranging from 39--61%, and (2) the Beck Depression Inventory, a 13-item patient self report was the most sensitive and specific and is recommended for screening drug-dependent populations for depression.


Depressive symptoms in rape victims.

Depressive symptoms in rape victims. Thirty-four recent rape victims were assessed for depressive symptomatology using a well-validated self-report instrument in combination with formal psychiatric evaluation. Fifteen subjects were found to be moderately or severely depressed when measured on the self-report questionnaire. A closer examination of these 15 subjects revealed that 8 were suffering from a major depressive disorder. The authors emphasize that all clinicians working with rape victims should be alert to the emergence of depression in this population.


The familial prevalence in second-degree relatives of patients with anxiety neurosis (panic disorder). A family history study of second-degree relatives of 19 patients with anxiety neurosis (panic disorder) and 19 controls showed a morbidity risk of 9.5% among the former compared with 1.4% among the latter. These risks were approximately half those found among first-degree relatives. Female relatives were at higher risk for anxiety neurosis. The risk for other psychiatric illnesses did not differ between the relatives of anxiety neurosis and controls.


Distribution of ancestral secondary cases in anxiety neurosis (panic disorder). The Slater computational model for use in distinguishing between polygenic inheritance and the effects of a single dominant gene with incomplete penetrance was applied to 15 kindreds of anxiety neurosis (panic disorder). The number of observed unilateral pairs (two ancestral cases from one side of the kindred) was significantly greater than the number expected. Therefore, these results suggest a dominant mode of transmission for anxiety neurosis.


The relationship of family history of alcoholism to primary affective disorder. One hundred sixty-eight patients with primary affective disorder were studied regarding history of alcohol-related problems. Alcohol-related problems were identified in 19 patients. We found that the morbid risk for alcoholism was significantly increased among relatives of male patients with drinking problems as compared to relatives of male patients without drinking problems. Our data suggest that psychiatric illness in relatives of probands with severe bipolar illness tends to be affective disorder tends to include alcoholism plus affective disorder.


Treatment of unipolar depression following electroconvulsive therapy. Relapse rate comparisons between lithium and tricyclics therapies following ECT. A restrospective chart study and follow-up telephone interviews were used to compare the relapse rates of unipolar depressives who had received either lithium or tricyclics following ECT. The results showed no difference between the two treatment groups. The literature on ECT was reviewed in the light of the results from the present study. We concluded that ECT followed by either lithium or a tricyclic antidepressant is a more effective treatment for unipolar depression than ECT alone.


Schizoaffective disorders. Results of a genetic investigation, I. 1004 first degree relatives fo 150 schizoaffective patients (41 males, 109 females) were studied and a total morbidity risk of 29.6% of schizoaffective spectrum disorders were found. The relatives show an increased morbidity risk for schizophrenia (5.26%) and affective disorder (6.55%) with a high incidence of catatonia and unipolar depression; schizoaffective secondary cases were only found in 3%. There is no significant difference in morbidity between parents, siblings and children. The morbidity risk of neuroses is 5.3%, for personality disorders 7.2% and for suicides without spectrum diagnosis 1.8%. Off-spring of affected parents show a morbidity risk twice as high as that of off-spring of non-affected parents. The findings do not support the present concept of the ICD (International Classification of Disorders) of WHO, which subsumes schizoaffective disorders under the major rubric of schizophrenia. From a genetic viewpoint schizoaffective disorder takes an intermediate position between schizophrenia and affective disorders. None of the present hypotheses of the mode of inheritance is supported by the findings.


Are schizoaffective psychoses heterogeneous? Results of a genetic investigation, II. 150 schizoaffective probands and their 1029 first-degree relatives were examined in search of the heterogeneity of the disorder. The sample of probands was split by several criteria. Among the various subgroups the morbidity risk of relatives was analysed as an external criterion for heterogeneity. Female relatives show a higher risk for affective disorders that male relatives. This is true for relatives of male and female index patients. Schizophrenia is equally frequent in male and female relatives. Schizoaffective psychoses take an intermediate position. The further analysis included the following characteristics of the probands: age at first episode, number of episodes, psychopathological subtypes (affective, schizophrenic, undifferentiated: manic, non-manic). None of these criteria proved to distinguish subgroups significantly, therefore, the search for heterogeneity was negative, although some results show a trend to the expected direction.


Affect and the menstrual cycle.

Affect and the menstrual cycle. Evidence from both retrospective and prospective studies suggests that many women do show cyclical changes in affect. Negative changes such as irritability, headache, tension, anxiety, sleep disturbance and depression are more frequent in the premenstrual and menstrual phases. Positive changes, pleasantness, increased vigour and elation are reported more often in the follicular or mid-cycle phases. Various theories have been suggested to explain these changes. These include psychodynamic, sociological and biological explanations. Although there is some evidence to support each theory no definite conclusions can be reached about the aetiological basis of most affective changes. Interpretation of studies attempting to link hormonal and affective fluctuations in limited by the many methodological problems outlined. Only one study appeared able to conclusively demonstrate a hormonal basis for a cyclical symptom. Suggestions are made for further research in this area.


Relationship between mood changes and adrenal cortical activity in a patient with 48-hour unipolar-depressive cycles. A 66-year-old patient with 48-hour unipolar-depressive cycles was studied in the hospital for one month and in an isolation unit for two weeks. At 3-hour intervals during the day and once at night the mood state was assessed by two self-rating scales and urine was collected for the determination of urinary free cortisol (UFC). In the hospital there was a regular alternation between 'good' and 'bad' days. Without exception the switch from normal to depressive mood occurred between 22:00 and 2:30, when the patient was sleeping. The switch from depressive to good mood was variable and occurred in the late morning or in the afternoon. On a 24-hour basis a highly significant correlation was seen between the mood state and the UFC excretion: r = 0.73, P less than 0.001 (hospital); r = 0.82, P less than 0.01 (isolation unit). The shape of the 24-hour profile for the UFC excretion was similar on good and bad days, with a maximum between 2:30 and 7:00 and a minimum between 19:00 and 2:30. However, on bad days UFC was elevated at all clock times. A decrease in adrenal cortical activity preceded or was parallel to substantial improvement in the mood state. Internal desynchronization occurred in the isolation unit but not in the hospital, with a rest-activity period of 18.6 h and a period close to 24 h for UFC excretion. Changes in mood were associated with both cycles, and a very close link between the switch from normal to depressive mood and the sleep state could be demonstrated.


Further validation of a questionnaire method for classifying depressive illness. A total of 367 patients admitted to a psychiatric facility completed the LPD questionnaire. By application a decision rule to their responses, they were classified as 'non-endogenous depression', 'endogenous depression' and 'non-depressive syndrome'. This classification was found to be associated significantly with their categorization on the basis of clinical diagnosis. The findings suggest that this method of classification on the basis of responses to the LPD may have a useful research and clinical role.


Factor patterns in depression. A replication study. A principal component analysis was carried out on symptom ratings and selected history items from 79 depressed patients. Three interpretable factors were obtained. The first was a general factor reflecting severity. The second a bipolar factor contrasting endogenous and neurotic depression, the third a bipolar contrasting anxiety and depressive symptoms. These replicate similar factors found previously in a different sample. The stability and predictive value are emphasized of other factors as well as the endogenous-neurotic dimension.


Psychotic and neurotic depression. Part 3. Aetiological and background factors. Patients receiving diagnoses of 'psychotic' and 'neurotic' depression do not differ significantly in their pre-morbid experience of life events and difficulties of aetiological significance. Some aetiological differences emerge when the two groups are subdivided into their more and less extreme halves along a distribution of discriminant function scores, but these differences are not substantial. Reallocation of the extreme 20% of each diagnostic group (who are likely misclassifications) into the other group tends to enlarge these 'aetiological' differences but they are still slight. Comparison of 'endogenous' patients (those without severe life events or major difficulties) with 'reactive' patients reveals a slight tendency for the 'endogenous' group to have a higher frequency of psychotic-type symptoms. Apart from age and experience of 'past loss', no further significant differences were found in the background characteristics of depressed patients diagnosed as 'psychotic' and those diagnosed 'neurotic'.


The diagnosis of depression in children. Operational criteria for depression in children were derived from diagnostic criteria designed for adults. These criteria were applied to a series of outpatients and inpatients on a Child Psychiatry Service. Thirteen per cent of girls and 5% of boys admitted to the clinic met the criteria for depression. (For this calculation autistic, brain-damaged and seriously retarded were excluded from the clinic population.) The frequencies of criterion symptoms and some related symptoms are reported. Generally the data suggest that the significant depression does occur as an independent syndrome in children, it is relatively common in a clinic population and it does not differ obviously from depression in adults.


An analysis of lumbar intervertebral disc prolapse. Clinical findings and operative results of 212 operated cases of disc protrusion are analyzed in this paper. The maximum age incidence is between 21 and 40 years. In our series disc prolapse was most common between the L4-L5 disc space, mostly on the left side, whereas the L5-S1 is more common in white people. Anthropologic differences may account for the discrepancy. A careful search should be made for disc protrusion by flexing the spine and also exploring the intervertebral foramen during surgery.


General effects of beryllium sulfate on isolated rat heart muscle mitochondrial fraction. The effects caused by Be2+ on the properties of isolated rat heart muscle mitochondria were investigated. It has been shown that Be2+ enhances oxygen uptake by the mitochondrial fraction in the presence of oxidizable substrates. This is due to the uncoupling effect caused by Be2+ on the mitochondria, which also undergoes a swelling effect by this divalent ion that can be accounted by the retention of Be2+ by the mitochondria. The saturation of the organellae binding sites occurs when the concentration of the ion reaches a maximum of 6.2 mumoles/mg protein.


Anomaly of the gallbladder lying extraperitoneally in the internal oblique muscle: report of an unusual case. A gallbladder occupying internal oblique muscle rather low down in the right lower abdomen, is reported. The preoperative diagnosis was an abdominal wall tumor. The operation showed that we were dealing with a gallbladder which contained stones, and which had a relatively long cystic duct. The gallbladder was very loosely attached to the liver-bed with a long mesentery. Such a variation in the gallbladder position has only once been described in the previous literature.


Radiation therapy of the oral cavity: sequelae and management, part 2. This is the concluding portion of a two-part series dealing with the effects and manifestations in the oral cavity of radiation therapy of head and neck tumors. Preradiation and postradiation extractions in dentulous patients, as well as dental maintenance of such patients (including fluoride treatments follow-up, and restorative care), are discussed. Guidelines for the dental management of edentulous patients are also presented at length (this section covers risk of bone necrosis, soft liners, timing of denture placement, dentures and preexisting bone necrosis, soft-tissue necrosis and dentures, morbidity, and prosthodontic procedures). The article concludes with a brief discussion of osteoradionecrosis and soft-tissue necrosis.


11 beta-Hydroxydehydroepiandrosterone in a case of virilizing adrenal adenoma: isolation from urine and mitochondrial conversion from dehydroepiandrosterone. 11 beta-Hydroxydehydroepiandrosterone has been isolated from the urine of a 14-yr-old girl with a virilizing adrenal adenoma. Its excretion was estimated to be 0.4 mg/24 h by gas chromatography and the compound was further identified by mass spectrometry. When [7-3H]dehydroepiandrosterone was incubated with mitochondria prepared from the adenoma, approximately 10% was converted to 11 beta-hydroxydehydroepiandrosterone. The identity of the radioactive 11 beta-hydroxydehydroepiandrosterone was verified by reversed isotopic dilution, its conversion to 11 beta-hydroxyandrostenedione, and its mobility in several chromatographic systems. This is the first demonstration of an 11 beta-hydroxylase from a human source having an affinity for dehydroepiandrosterone.


Biological characters and bacteriophage typing of Staphylococcus aureus isolated from chicken staphylococcosis and commercial balanced chicken food in Argentine. During 1976-78, 33 S. aureus strains were isolated from chickens suffering from dermatitis and septicemia, omphalitis, pneumonia, arthritis and tenosynovitis; and from a commercial balanced chicken food. All the strains were classified into the biotype B of Hájek and Marsálek. Twenty-nine (87.8%) strains could be classified into Sato et al biological type 4 although they differed from it in its ability to grow at 45 degrees C. The remaining 4 strains differed from the others because they failed in producing hemolysis in bovine and ovine blood agar, egg yolk reaction and splitting of Tween 80. Thirty (90.9%) strains were lysed by avian phages: 28 strains at 1 x RTD and 2 at 100 x RTD. Twenty seven (93.1% strains of the Sato et al biotype 4 were sensitive to the phages CH42/CH43/CH63/CH37 of avian group II, and 2 strains to the phage CHA1 at 100 x RTD. Only one of the 4 unclassified strains could be typed by phage CH4 of avian group I at 1 x RTD. Twenty seven (81,8%) strains were typable at 100 x RTD by the basic set of human phages: 26 strains belonged to group III (53 and 53/77) and 1 to a mixed group I/III (79/53/77). The typable strains belonged only to Sato et al biotype 4. None of 33 strains were sensitive to any of the bovine phages. The biological characters and bacteriophage typing of the S. aureus showed that almost all the strains belong to chicken types. The strains isolated from food belong to the same chicken biotype and phagetype as the strains isolated from dermatitis, septicemia, omphalitis and pneumonia. The strains isolated from arthritis and tenosynovitis were different from the ones isolated from other pathologies.


Pattern of plasma dehydroepiandrosterone sulfate levels in humans from birth to adulthood: evidence for testicular production. Plasma levels of dehydroepiandrosterone sulfate (DHAS) were measured in 513 normal full term newborns, infants, children, adolescents, and adults and the results were expressed in micrograms per dl. In infancy and childhood, DHAS levels were similar in both sexes. In 74 neonates, mixed cord blood mean values /+- SD were 134.6 +/- 64. During the first day of life, plasma DHAS levels were 140 +/- 125 in 33 neonates. During the first month of life, DHAS decreased drastically, then more progressively until the 6th month of life. Between 1-6 months of age, mean levels were 5.9 +/- 4.7 in 40 children. DHAS was very low between 1-6 yr of life (2.3 +/- 1.6) and rose abruptly at the 7th year of life. Thereafter, DHAS continued to increase correlatively with age and pubertal stages in both sexes, a further increase after age 16 or pubertal stage P5 was noted only in male subjects. In adults, DHAS was significantly higher in male (224 +/- 93) than in female (138.3 +/- 51) subjects. DHAS levels were compared to those of dehydroepiandrosterone; at two periods of life, early infancy and adulthood, their patterns differed. After long term hCG stimulation, DHAS increased significantly in 45 normal prepubertal boys and in 2 boys with adrenal insufficiency. These data would suggest a direct testicular production of DHAS.


Decline of adrenal androgen production as measured by radioimmunoassay of urinary unconjugated dehyroepiandrosterone. Dehydroepiandrosterone (DHA), in large part a measure of adrenal androgen secretion, previously has been measured in urine after hydrolysis or solvolysis of the glucuronide and sulfate conjugates. These procedures are time consuming; they often require several days and variable recoveries are a source of error. A method is presented here for determination of unconjugated DHA in 24-h urine specimens which requires less time, labor, and sample volume than necessary for the assay of DHA derived from conjugates. In 76 men and women, age 20-96 yr, total 24-h urinary unconjugated DHA showed no sex differences. However, the mean unconjugated DHA excretion decreased, which may indicate decreased zona reticularis function with respect to relatively constant zona fasiculata function in advancing age.


Adrenal suppression with aminoglutethimide. III. Comparison of plasma delta 4- and delta 5-steroids in postmenopausal women treated for breast carcinoma. A regimen or aminoglutethimide in combination with replacement glucocorticoid has been used to suppress adrenal steroidogenesis in postmenopausal women with metastatic breast carcinoma. During acute and chronic treatment with aminoglutethimide, the levels of the delta 4-steroids [progesterone (P), 17 alpha-hydroxyprogesterone (17-delta 4-P), and androstenedione (delta 4-A)] and the delta 5-steroids [dehydroepiandrosterone (DHEA), dehydroepiandrosterone-sulfate (DHEA-S), and 17 alpha-hydroxypregnenolone (17-delta 5-P)] were determine. In the total group of women, the plasma levels of P and delta 4-A increased 2- to 3-fold (P less than 0.05) while 17-delta 4-P rose 10-fold (P less than 0.01) from basal concentrations of 0.65 +/- 0.07 to 6.48 +/- 1.46 ng/ml during the initial 2 weeks of therapy with aminoglutethimide (AG) and dexamethasone. These three steroids then fell to basal levels during chronic treatment (P and 17-delta 4-P) or were suppressed (delta 4-A; P less than 0.001). In contrast, the levels of delta 5-steroids (17-delta 5-P, DHEA, and DHEA-S) were reduced 3- to 5-fold during the initial 2 weeks of therapy and remained suppressed throughout. The relative levels of certain delta 5- and delta 4-steroids pairs were then examined. The ratio of 17-delta 5-P to 17-delta 4-P decreased from baseline values of 2.15 +/- 0.35 to 0.38 +/- 0.21 ng/ml (P less .02) with the initiation of therapy and remained low thereafter. A similar pattern for the ratios between DHEA and delta 4-A, and DHEA-S and delta 4-A was observed. This may indicate that the regimen of AG treatment utilized may facilitate the activity of the 3 beta-ol-dehydrogenase, delta 5- to delta 4-isomerase, and accelerate the conversion of delta 5- to delta 4-steroids. The patterns of suppression of the plasma delta 4- and delta 5-steroids in oophorectomized and spontaneously postmenopausal patients with intact ovaries were analyzed separately. The plasma levels of progesterone were higher during the first 2 weeks of therapy in surgically castrate women than in spontaneously postmenopausal women (0.72 +/- 0.25 vs. 0.47 +/- 0.20 ng/ml). A similar pattern was observed for 17-delta 4-P, DHEA, and DHEA-S indicating that the adrenals might contribute to this increase. In contrast, during chronic treatment the levels of all steroids were lower in surgically castrate women than in those with intact ovaries. This suggested residual ovarian steroid during AG administration.


Dehydroepiandrosterone: kinetics of metabolism in normal men and women. The single injection and constant infusion techniques were utilized to study the kinetics of dehydroepiandrosterone (DHEA) metabolism and its peripheral conversion to several other C19-steroids including C19-steroid sulfates. The MCRs (mean +/- SEM) for normal men and normal women were 1866 +/- 144 and 1901 +/- 87 liters/24 h, respectively. The single injection technique yielded values for rate constants (units) and volumes of distribution (1) as follows: K1, 42.6 +/- 7.7 for men and 37.1 +/- 5.0 for women; K2, 64.3 +/- 11.2 for men and 55.5 +/- 5.0 for women; K2, 64.3 +/- 11.2 for men and 55.5 +/- 5.0 for women; V1, 38.5 +/- 6.0 for men and 33.7 +/- 2.5 for women; V2, 30.4 +/- 7.3 for men and 27.5 +/- 9.9 for women. The constant infusion technique yielded values for the conversion ratios for the transformation of DHEA to several products: delta 5-androstene-3 beta, 17 beta-diol to DHEA of 0.10 +/- 0.01 for men and 0.16 +/- 0.03 for women, delta 4-androstenedione to DHEA of 0.04 +/- 0.01 for men and 0.07 +/- 0.02 for women, DHEA sulfate (DHEAS) to DHEA of 6.36 +/- 0.81 for men and 10.09 +/- 0.87 for women, delta 5-androstene-3 beta, 17 beta-diol sulfate to DHEA of 0.42 +/- 0.06 for men and 0.50 +/- 0.04 for women, and androsterone sulfate to DHEA of 1.11 +/- 0.13 for men and 2.06 +/- 0.18 for women. The ratios for the conversion to DHEA sulfate and androsterone sulfate were significantly higher for women than men. The plasma concentrations of DHEA were 8.50 +/- 0.95 and 8.75 +/- 1.01 ng/ml for men and women, respectively. The calculated production rates for DHEA were 16.34 +/- 2.66 and 16.19 +/- 1.78 mg/24 h for men and women, respectively. There was no sex difference in the binding of DHEA to plasma proteins and this is reflected in the lack of sex difference in the MCRs. Calculations indicate that DHEA is a major precursor of circulating delta 5-diol.


Plasma levels of gonadotropins, prolactin, thyroxine, and adrenal and gonadal steroids in obese prepubertal girls. Plasma levels of gonadotropins, PRL, T4, and adrenal and gonadal steroids were measured in two groups of 7- to 9-yr-old and 10- to 11-yr-old obese prepubertal girls, and were compared to those found in groups of nonobese girls of the same age. The data found in normal weight subjects confirm the data reported in the literature, showing a significant rise between the 7- to 9- and 10- to 11-yr groups, of FSH, pregnenolone, dehydroepiandrosterone, testosterone, and estradiol plasma levels, while LH, PRL, T4, cortisol, progesterone, 17-hydroxyprogesterone (17P), and androstenedione remained constant. In the obese subjects, pregnenolone and dehydroepiandrosterone levels are notably higher than in the normal girls, in the same range as those found in adult women; furthermore, they show no rise between the two age groups. The obese prepubertal groups had significantly higher progesterone, androstenedione, and PRL levels in comparison with those observed in girls of normal weight, but 17-hydroxyprogesterone, cortisol, testosterone, LH, and T4 were similar in both groups. Estradiol levels were markedly depressed in the obese girls; FSH levels were higher in the younger girls than in normal subjects. These data indicate that in prepubertal obesity, maturation of adrenal gland function (chiefly the delta 5 pathway), is notably enhanced, whereas gonadal secretion of estradiol is impaired in the presence of high levels of FSH and PRL.


Stimulation of prolactin and growth hormone secretion by muscimol, a gamma-aminobutyric acid agonist. The alteration in circulating levels of PRL, GH, TSH, and cortisol was studied after the oral administration of muscimol (3-hydroxy-5-aminomethylisoxazole) to human subjects with Huntington's disease (n = 4) and chronic schizophrenia (n = 5). PRL levels rose significantly in a dose-dependent fashion within a 120-min time interval. GH rose significantly but modestly over the same time interval, whereas TSH and cortisol levels remained unchanged. Since muscimol is thought to be a potent and specific gamma-aminobutyric acid agonist, these data indicate that gamma-aminobutyric acid-mediated neural transmission may function to stimulate the release of plasma PRL and GH in human subjects.


Studies on human sexual development. VI. Concentrations of unconjugated dehydroepiandrosterone, estradiol, and estriol in amniotic fluid throughout gestation. Concentrations of unconjugated dehydroepiandrosterone, estradiol, and estriol were measured in samples of amniotic fluid from uneventful pregnancies of 9-40 weeks conceptual age. There was no apparent influence of fetal sex upon the levels of these steroids. Dehydroepiandrosterone concentrations rose slightly from 9-20 weeks, and then showed little further change. Estradiol concentrations declined slightly from 9-20 weeks; after 32 weeks gestation, there was a 2-fold rise to term. Estriol levels rose in almost exponential fashion throughout gestation.


The ventricular A wave: a new echocardiographic index of late diastolic filling of the left ventricle. Echocardiography was used to evaluate the late diastolic filling period of the left ventricle in 30 normal individuals, 25 patients with left ventricular hypertrophy, normal sinus rhythm, and either a normal or delayed mitral valve closure, and 15 patients with atrial fibrillation. The echocardiographic ventricular A wave (VAW) was defined as the difference between the end-diastolic and an earlier late-diastolic internal left ventricular dimension, and it was felt to primarily reflect the atrial contribution to late diastolic filling of the left ventricle. It disappeared during ventricular pacing and was significantly smaller than normal in patients with atrial fibrillation. The VAW was significantly larger in patients with left ventricular hypertrophy and normal mitral valve closure reflecting the greater contribution by atrial contraction to late diastolic filling in these patients. In patients with left ventricular hypertrophy and delayed mitral valve closure, the VAW was normal reflecting lesser ventricular enlargement with atrial contraction than in the other patients with left ventricular hypertrophy. This suggested a greater impairment to left ventricular filling in these patients. Therefore, the VAW appears to be an indicator of abnormalities of late diastolic filling caused by left ventricular hypertrophy.


Left ventricular function in beta-thalassemia and the effect of multiple transfusions. Left ventricular performance was studied in 23 young patients with severe chronic anemia due to beta-thalassemia major and intermedia. The patients were divided into three groups according to the number of blood transfusions they had received. The left ventricle (LV) was enlarged in patients who had not received blood and larger still in patients who had received multiple transfusions. Echocardiography and systolic time interval measurements showed that systolic function of the LV was good in all the patients and that there was no statistical difference in systolic function in patients who had and those who had not received multiple transfusions. Heart rate was increased in the latter group. Stroke index and cardiac index were high, especially in patients in Group 3. The diastolic closure rate (EF slope) of the anterior mitral leaflet and its amplitude of movement were increased, but less so in Group 3; this may reflect an alteration in diastolic LV distensibility. The results indicate that despite the presence of cardiomegaly and severe clinical congestive heart failure, LV performance is well preserved in patients with beta-thalassemia, even in those who have received repeated blood transfusions. Clinical cardiac failure is the consequence of volume overload and abnormal chamber compliance. There was no evidence in this of a congestive cardiomyopathy.


Congenital tricuspid incompetence simulating pulmonary atresia with intact ventricular septum: a report of two cases. Two infants with isolated congenital tricuspid incompetence appeared to have associated right ventricular outflow tract obstruction at diagnostic evaluation, including catheterization and cineangiography. One infant died during surgery; the other improved rapidly and survived with medical therapy. We discuss the problem of establishing the presence of patency of the right ventricular outflow tract in infants with massive tricuspid incompetence and outline an approach to the management of infants whose diagnosis remains unclear even after careful evaluation.


A genetic analysis of the Papillon-Lefèvre syndrome in a Jewish family from Cochin. The Papillon-Lefèvre syndrome (PLS) is segregating in a large kindred of a Jewish isolate originating from Cochin, India. The frequency of the gene responsible for PLS among the Cochin Jews, 0.1, was estimated from the number of unrelated carriers in the isolate who married into the kindred. The obvious discrepancy between this apparently high gene frequency and the total absence of PLS in other kindreds of the isolate suggests that the syndrome may not behave as a simple autosomal recessive trait.


A detailed description of mothers' knowledge before genetic counseling for Down syndrome: Part I. This study focuses on counselees' knowledge of the Down syndrome before receiving genetic counseling. Data were collected from 47 mothers of children with the Down syndrome using a structured interview of 13 open-ended questions. This instrument was found to be both internally reliable and consistent. Results of this study document the enormous variation of counselees' knowledge of the Down syndrome before genetic counseling and show that this is positively associated with their educational background. Counselees with more than a high school education knew about 60% of the genetic information pertaining to the diagnosis before genetic counseling, while those with less than a high school education knew only 23% of this information before counseling. These results indicate that the better educated counselees are less apt to need to learn basic genetic information and may seek out genetic counseling services for other reasons. Possible motives are seeking knowledge confirmation, emotional support, and personalization of the information.


Prenatal cytogenetic diagnosis: first 1,000 successful cases. From February 1969 to August 1976, we studied 1,048 amniotic fluids. Of these, 958 (91.4%) were primarily for prenatal cytogenetic diagnosis. Cytogenetic studies were attempted in 1,021 cases; the diagnosis was successful in 1,000 of these. The failure rate of obtaining a diagnosis from the amniotic fluid cell culture of the first amniocentesis was 5% (50 cases); 29 cases had a repeat tap and successful diagnosis was achieved in all. In 21 cases, a repeat tap was refused. Thus, the overall failure rate of obtaining a final cytogenetic diagnosis was 2.06% (21/1,021). There were 32 fetal losses after amniocentesis including 16 spontaneous second trimester abortions, 7 fetal deaths in utero and 9 stillbirths. In two additional cases, fetal death had occurred before amniocentesis. This number of fetal losses does not exceed the number that would be expected in the same maternal age group without amniocentesis. In our series, the frequencies of trisomy in maternal age groups 40 years and over, 37-39 years, 35-36 years, and under 35 years were 4.5, 3.14, 0 and 0% respectively. These frequencies are comparable to those reported from other prospective prenatal studies and higher than those of retrospective live born studies. Various problems and pitfalls in prenatal cytogenetic diagnosis are discussed.


[Incorporation of H3-uridine in vitro into normal lymphocytes and those in cases of aberration of acrocentric chromosomes]. Literature data indicate that the formation of ribosomes is a basic function of the nucleolus. The formation of the nucleolus depends upon the function of the nucleolus organizing region (NOR). It was shown that in man the NOR locus is situated in secondary constrictions and adjacent segments of the short arms of acrocentric chromosomes of the D and G group. The presence of DNA sequences coding for 18 S and 28 S RNA i.e. ribosomal RNA (r-RNA), was demonstrated in the secondary constriction of acrocentric chromosomes using the RNA-DNA hybridization in situ method. The genes coding for 5 S RNA which is also a component of ribosomes, are located on other chromosomes. Therefore ribosome production should be considered a polygenic process, the genes situated in the NOR locus playing a basic role in its initiation. In cases of acrocentric chromosome aberration NOR "dosage" changes; in cases of regular trisomy there are 11 NOR "doses" instead of the normal 10, occurring in 5 pairs of group D and G chromosomes, in cases of translocation trisomy there are 9 such "doses" and in cases of balanced translocation carriers there are 8 "doses". Hence the following question: do changes in the NOR "dose" cause changes in ribosome production? Eventual changes should influence the intensity and/or the time course of r-RNA synthesis in cells stimulated to growth and differentiation . An approximate index for the study of this phenomenon is the 3H-uridine incorporation in an adequate experimental system. It seems that the most convenient model to study this phenomenon is the blastic transformation of lymphocytes induced by phytohaemagglutinin. This process has already been investigated in depth and is well known, the material being easily accessible. Venous blood was taken from 10 patients with 21 regular trisomy, 2 cases of 21 translocation trisomy and from 21 carriers of balanced translocations. Lymphocytes from 12 healthy persons with a normal karyotype served as controls. Lymphocytes in plasma were separated from whole blood and a routine macroculture was set up adding phaseoline of standardized mitogenic activity in the quantity 0.02 ml/ml of medium. The cells were incubated for 3, 6, 12, 24 and 48 hours. One hour before termination of the incubation period 1 microCi/ml 3H-uridine was added to the medium.(ABSTRACT TRUNCATED AT 400 WORDS)


[Incorporation of C-14-glycine in vitro into normal human lymphocytes and those in cases of aberration of chromosome 21]. Since Caspersson 's and Brachet 's investigations it has been very well known that the nucleolus is a cellular center of protein synthesis regulation. Later it has been shown that the nucleolus is the site of ribosome production, that means that it produces one of the basic elements of the cytoplasmatic apparatus of protein synthesis. Morphologic observations demonstrated that the nucleolus in man is formed in close connection with secondary constrictions of acrocentric chromosomes of the D and G groups. These regions contain the nucleolus organizing region (locus NOR). By means of DNA-RNA hybridization in situ it has been established that the secondary constrictions of acrocentric chromosomes contain sequences coding ribosomal RNA synthesis. RNA synthetized on r-DNA matrix contained in NOR is transported to the nucleolus where ribonucleoprotein complexes are formed. Ribosome formation is the result of a complex process. Ribosomes are transported to the cytoplasm where they aggregate into polyribosomes on the rough ergastoplasmic reticulum. In this way the cytoplasmatic aparatus of protein synthesis is formed. Its activity depends on ribosome formation. In view of the above the question arises: will the presence of an additional chromosome coding r-RNA synthesis as in Down's syndrome, i.e. 21 trisomy, influence the course of protein synthesis? Blastic transformation of lymphocytes in vitro induced by phytohaemagglutinin is a convenient model for the study of protein synthesis in cells stimulated to growth and differentiation. This phenomenon has been very well investigated. It is well known that blastic transformation is accompanied by intensification of RNA synthesis, ribosome formation and intensification of protein synthesis. The existing literature data allow to choose adequate methods and time points for the examination of the course of protein synthesis in this model. Venous blood was obtained from 17 healthy humans with normal karyotype and from 15 cases of trisomy 21. After separation of plasma with lymphocytes routine cultures with addition of phaseoline were set up and were incubated 6, 12, 24, 48 and 72 hours. 14C-glycine was added one hour before the termination of the incubation period. Following this the number and differential count of lymphocytes were determined.(ABSTRACT TRUNCATED AT 400 WORDS)