Functional significance of the cells in the pars anterior of the pituitary gland of the musk shrew (Suncus murinus L.). Seven morphologically and tinctorially distinct types of cells (types 1--7) have been distinguished in the pars anterior of the pituitary gland of the musk shrew (Suncus murinus L.). On the basis of their responses to various experimental stimuli, these cell types were correlated with the secretion of various trophic hormones. Type 1 cells exhibited conspicuous changes after thyroidectomy or inactivation of the thyroid gland and hence appeared to be the source of TSH. Types 2 and 3 cells responded to gonadectomy and administration of androgens, which suggests that they were associated with gonadotrophin secretion. The granules of the type 2, but not the type 3 cells could be extracted with 10% trichloroacetic acid, which may indicate that type 2 and 3 cells secrete FSH and LH respectively. After the administration of either reserpine or oestrogen, the type 4 cells underwent hypertrophy and hyperplasia, which suggests that they were the likely source of prolactin. Type 6 cells, which are distinguishable from type 4 cells by their thinly dispersed erythrosinophilic granulation, showed conspicuous changes after unilateral adrenalectomy, administration of metyrapone or exposure to stress and may therefore be responsible for secretion of ACTH. Type 5 cells tinctorially resembled the somatotrophic cells of other mammalian species and did not respond to any of the experimental treatments used in the present study. It is therefore possible that these cells have a somatotrophic function. The possible significance of type 7 cells has been discussed previously.


An electromyographic analysis of forelimb muscles during overground stepping in the cat. The patterns of activity of 33 forelimb muscles during unrestrained overground stepping in eight adult cats were analysed using intramuscular electromyography. Three general patterns were found. Some muscles began activity during the first extension epoch (E1) and ceased near the end of the third extension epoch (E3) and were considered extensors. Others, considered flexors, began activity just prior to the flexion (F) epoch and ceased at or just after the onset of E1. Other muscles showed a biphasic pattern of activation; one period of activity occurring during F, the other during the extension epochs. In all regions of the limb, individual muscles displayed variation in the onset and time course of activity. The results are interpreted in terms of a model of locomotor generation which proposes specific neural output to individual muscles. Muscles of the shoulder region are proposed to act mainly to produce translatory and rotatory movements of the scapula associated with lengthening the step. Muscles of the elbow region and antebrachium are interpreted as playing roles both in producing flexion-extension movements and in the absorption of energy. The latter group are considered especially suitable to energy absorption because of their pennate arrangement of muscle fasciculi and their long tendons.


Patterns of activity and the effects of activation of the fast conducting system on the behaviour of unrestrained leeches. 1. The neural activity of the nerve cord of Hirudo medicinalis has been recorded in unrestrained animals by means of chronically implanted electrodes. 2. The Fast Conducting System (FCS) is inactive both in motionless animals and during various kinds of active behaviour (creeping, swimming, ventilation). 3. Photic and tactile stimuli applied to a motionless animal elicit a FCS discharge, which may be followed by generalized shortening. 4. Photic and tactile stimuli applied during ventilation are followed by a reversible blockade of the ongoing activity only if they are able to elicit a FCS discharge. No such effect is observed on swimming. 5. An explanation of these findings in terms of the known connexions of leech neurones is offered and a role in the control of reafferent inputs is attributed to the rectifying synapses made by FCS and T cells on the L motor neurones.


Quantitative mapping of cutaneous receptive fields in normal and operated leeches, Limnobdella. 1. The receptive fields and physiological properties of the sensitive cutaneous mechanoreceptive neurones in the leech Limnobdella australis were found to be very similar to those previously described in Hirudo medicinalis. 2. Following separation from the central nervous system (C.N.S.), the distal dendrite stump and cutaneous receptive field remained unchanged for at least 160 days. 3. There was little spreading of receptive fields into regions of skin isolated from the C.N.S. for at least 184 days. 4. Cutting one dendrite of a mechanoreceptive neurone which has two major dendrites produced little change in the receptive field of the intact dendrite. 5. Abnormalities were found in most of the receptive fields of operated leeches, irrespective of the site of operation. These abnormalities were not seen in normal leeches.


Axonal adaptations to osmotic and ionic stress in an invertebrate osmoconformer (Mercierella enigmatica Fauvel). I. Ultrastructural and electrophysiological observations on axonal accessibility. The giant axons in Mercierella are overlaid by narrow glial processes which provide an incomplete covering of the axonal surface. Where more complete covering occurs the intercellular clefts are not sealed by tight junctional complexes. Ionic lanthanum penetrates to the surfaces of axons from sea-water-adapted animals (in normal saline and during initial exposure to hyposmotic saline) and, also, to the surface of hyposmotically adapted axons. A relatively free intercellular access to the axon surfaces is also indicated by the rapid electrical responses of sea-water-adapted axons to hyposmotic dilution and of hyposmotically adapted axons to sodium-deficient saline. The giant axon possesses an unusual ultrastructural specialization: hemidesmosome-like structures (associated with the axon membrane) which are connected to a network of neurofilaments within the axon. Theoretical considerations suggest that these structures could enable the axons to withstand appreciable excesses in intracellular hydrostatic pressure resulting from osmotic imbalance during hyposmotic stress.


Axonal adaptations to osmotic and ionic stress in an invertebrate osmoconformer (Mercierella enigmatica Fauvel). II. Effects of ionic dilution on the resting and action potentials. The giant axon of this extreme euryhaline osmoconformer possess an unusual ability to produce action potentials of large amplitude over a wide range of ionic dilution when constant osmotic concentration is maintained by the addition of mannitol to the bathing medium. Ionic dilution under these circumstances causes a decline in the overshoot of the action potential (resulting largely from reduction in [Na+]o) and an appreciable axonal hyperpolarization (primarily as a result of decrease in [K+]O). This hyperpolarization tends to compensate for the reduction in the extent of the overshoot and so maintains the amplitude of the sodium-mediated action potentials during isosmotic dilution of the bathing medium. The axonal hyperpolarization also appears to reduce sodium inactivation so as to maintain a rapid rate of rise of the action potential despite drastic reduction in the ionic concentration of the bathing medium. Prolonged exposure to reduced ionic concentrations appears to induce a ouabain sensitive reduction in intracellular sodium concentration which increases the sodium gradient across the axon membrane during isosmotic dilution of the external medium.


Axonal adaptation to osmotic and ionic stress in an invertebrate osmoconformer (Mercierella enigmatica Fauvel). III. Adaptations to hyposmotic dilution. The giant axons of this extreme osmoconformer were adapted, in vitro, to progressive hyposmotic dilution of the bathing medium (from 1024 m-Osmol to concentrations as low as 76.8 m-Osmol). Hyposmotic adaptation is associated with reductions in the intracellular concentrations of both sodium and potassium ions. These reductions do not appear to result from appreciable axonal swelling. The different electrical responses to isosmotic and hyposmotic dilution suggest that reduction in [Na+]1 results from ouabain-dependent sodium extrusion, in response to ionic dilution, and that reduction in [K+]1 is induced by a combination of ionic and osmotic dilution. The reduced level of intracellular potassium achieved during hyposmotic adaptation represents a balance between the necessity to contribute to osmotic equilibration and to maintain a potassium gradient across the axon membrane sufficient to produce appreciable axonal hyperpolarization during dilution of the bathing medium. This hyperpolarization tends to maintain the amplitude of the action potential, by compensating for reduction in overshoot (with decline in E(Na), and by reducing sodium inactivation. This, together with the reduction in [Na+]1, enables overshooting action potentials of relatively large amplitude and rapid rise time to be maintained during more than tenfold dilution of the ionic and osmotic concentration of the bathing medium.


Cilioexcitatory and cilioinhibitory processes initiated by light on two identifiable neuronal pigments in Mytilus edulis. Two neuronal chromoproteins (red-haemoprotein and yellow-carotenoprotein) initiate changes in ciliary activity on the gill of Mytilus. Photic, electrical and chemical stimulation of these chromoproteins correlate with fluctuations in ionic (calcium and iron) deposits in gill bulk tissue samples. Microspectrophotometric measurements of chromoprotein spectral band patterns reveal two or three absorption maxima for carotenoprotein and haemoprotein respectively. Under aerobic conditions, cilioinhibition and cilioexcitation results after activation of carotenoprotein or haemoprotein respectively. Carotenoproteins are only engaged during gradual anoxia. Immediate anoxia renders both chromoproteins inoperative. Changes in ciliary activity initiated by photic stimulation of neuronal chromoproteins are abolished by denervation of the gill. It is postulated that photoactivation and/or release of specific neurotransmitters lead to transformations in chromoproteins with subsequent neuronal regulation of gill ciliary activity.


A role for conditioned stimulus duration in toxiphobia conditioning. The effect of conditioned stimulus (CS) exposure duration on toxiphobia conditioning was investigated. When a relatively long (20--30 min) CS exposure was administered on the conditioning trial, rats subsequently displayed a reduced conditioned aversion in comparison with when CS exposure was relatively brief (5--10 min). This result was obtained in an odor-aversion learning task with neonatal rats as subjects (Experiments 1 and 2) and in a flavor-aversion learning task with adult rats (Experiment 3). This phenomenon was further investigated in the odor-aversion task by presenting neonates with two discrete 10-min exposures to the odor CS and by varying both the temporal proximity of the two CS presentations to each other and to the occurrence of the unconditioned stimulus (US). Two exposures to the CS reduced conditioning in comparison with a single exposure but only when the second CS presentation preceded US occurrence by at least 5 min (Experiments 4 and 5). Both nonassociative and associative interpretations of the data were discussed.


Urinary isoamylases in juvenile diabetics. An anomalous ratio of salivary to pancreatic amylase activities has been observed in urine from juvenile diabetics. Decreased pancreatic amylase activity in urine from these subjects appears to be a characteristic trait.


[Diagnostic value of the lipoprotein-X determination (author's transl)]. LP-X was investigated in the serum of 221 patients with and without cholestasis. The diagnostic sensitivity and the diagnostic specificity of the test were 0.9 and 0.88, respectively. When this test is used on a non-selected collective, however, the predictive value of the positive test is very low, whereas negative results have a high diagnostic value. Thus, in practice, LP-X is more suitable for the exclusion, rather than the detection of cholestasis.


[Enzymatic determination of xylitol and sorbitol (author's transl)]. An enzymatic endpoint determination of xylitol and sorbitol by the use of L-iditol dehydrogenase is described. Despite the use of pH 9 and the addition of semicarbazide, the reaction goes only 80% to completion. Therefore a standard value has to be used. The lower limit for measurement of concentrations in serum is around 0.1 mmol/l. The most favorable range is between 0.4 and 1.2 mmol/l with a coefficient of variation of below 4%. Sensitivity and precision is higher than with the kinetic method described earlier. Further simplification for the routine control of infusion therapy with polyols is suggested.


Investigation of reference values of components of cerebrospinal fluid. In order to obtain reference values, random samples of CSF from 70 women and 69 men were analyzed for various components, including total protein and protein fractions. Using statistical methods, data were also collected for sex differences and the influence of age. Small variations in the values of total protein, albumin and gamma-globulin between males and females were found, and slightly higher values for the gamma-globulin were found with increasing age.


[Dependence of the serum concentrations of creatinine and urea on the time of day, with normal and impaired kidney function (author's transl)]. Daily variations in the serum concentrations of creatinine, urea and cortisol were studied in 7 patients without kidney or muscle disease, and in 8 patients with impaired kidney function (creatinine clearance less than 40 ml/min). Neither creatinine nor urea showed a circadian rhythm; the daily variations lay within the limits of the methodological scatter. Serum cortisol showed its known circadian rhythm in high amplitude.


[Fluorometric method for the determination of naproxen and demethylnaproxen in serum and urine (author's transl)]. Naproxen and demethylnaproxen can be determined fluorometrically in serum and urine after extraction with dichlorethane, and without prior separation. The detection limit of naproxen and demethylnaproxen is below 0,2 mg/l in serum and below 0,5 mg/l in urine. After incubation of urine with beta-glucuronidase/arylsulfatase, most of the conjugated naproxen and demethylnaproxen can be determined.


In vitro conversion of estradiol-17beta into estrone in normal human myometrium and leiomyoma. The specific activity of 17beta-hydroxysteroid dehydrogenase was measured in normal human myometrium, and in leiomyoma specimens obtained from the same tumor-bearing uterus. In all cases the normal tissue showed greater conversion of estradiol-17beta into estrone than the neoplastic tissues. In normal myometrium of fertile women, the specific enzyme activity depended on the phase of the menstrual cycle, the highest values of 17beta-hydroxysteroid dehydrogenase activity being found in the early secretory phase. To determine the intracellular distribution of the 17beta-hydroxysteroid dehydrogenase, purified microsomes, mitochondria, nuclei and cytosol fractions were prepared. The purity of each fraction was monitored by marker enzymes. It was found that the enzyme was mainly located in mitochondria and microsomes. Furthermore it was demonstrated that the microsomal enzyme was bound tightly to the membranes of the endoplasmic reticulum, while the mitochondrial 17beta-hydroxysteroid dehydrogenase was mainly associated with the outer membranes of the organelle. Kinetic parameters (Km-values, coenzyme requirements, temperature and pH-optima) of the cytoplasmic, nuclear, mitochondrial and microsomal enzyme of normal and neoplastic tissue were compared.


Estrogen and progesterone binding proteins in normal human myometrium and leiomyoma tissue. The occurrence and characteristics of macromolecular components of normal human myometrium and leiomyoma which bind [3H]estradiol and [3H]progesterone were investigated, employing dextran coated charcoal, density gradient centrifugation and gel filtration techniques. On sucrose density gradient centrigugation, [3H]progesterone was bound by macromolecules with sedimentation rates of about 4 S and 8 S. The major [3H]progesterone binding component had a sedimentation coefficient of about 4 S, which contained specific and nonspecific binding sites. Sedimentation patterns as well as elution profiles from agarose gel revealed a striking similarity between biochemical properties of the progesterone receptors from normal myometrium and leiomyomas of the same organ. Both progesterone and estradiol receptor change in concentration during the normal menstrual cycle. During the early proliferative phase the number of estradiol receptor binding sites was highest; after ovulation, a rapip decrease of estradiol receptor level was seen. On the other hand, using [3H]progesterone as the ligand, the highest receptor concentration was found at midcycle. The leiomyoma steroid hormone receptor levels were compared with those in normal myometrium. Whereas leiomyoma exhibited higher estradiol binding capacity, the concentration of progesterone receptors was low in fibroid tumors.


[Uroporphyrinogen decarboxylase in erythrocytes: studies on the primary genetic enzyme defect in chronic hepatic porphyria (author's transl)]. In chronic hepatic porphyria, including the clinical phase, porphyria cutanea tarda, the activity of uroporphyrinogen decarboxylase is decreased not only in the liver, but also in the erythrocytes. The synonomous decrease in the enzymic activity in liver and erythrocytes in both familial and sporadic hepatic porphyria shows that the disturbance of this enzyme is the primary genetic defect of this condition; inheritance of the defect is probably autosomal and dominant. The clinical manifestation of disturbances of porphyrin metabolism are precipitated, however, by additional factors, such as liver damage, alcohol, oestrogens and neoplastic growths. In the absence of these other pathogenic influences, the enzyme defect is compensated and does not result in disturbances of haem or haemoglobin synthesis, either in the liver or the bone marrow.


Evaluation of the Bradford method for protein determination in body fluids. The applicability of a dye binding method for protein determination described by Bradford (1976), Anal. Biochem. 72, 248--254) for use in the clinical chemical laboratory was studied. The simple, fast and sensitive method is well suitable for protein determination of cerebrospinal fluid but not for urinary protein. Polymer carbohydrates (Macrodex) do not interfere with dye binding.


Facial development in normal and mutant chick embryos. I. Scanning electron microscopy of primary palate formation. Early facial development in normal chick embryos was studied by scanning electron microscopy, and compared to the abnormal facial development of a mutant in which primary palate formation does not occur, thus resulting in bilateral cleft lip. In both normal and "cleft primary palate" mutant embryos, subsequent to the appearance of the nasal placodes, the surrounding tissues elevate to give rise to the presumptive facial primordia. As the facial primordia grow forward, they gradually assume the configuration of a square which is most pronounced at five days development. In normal embryos, the square configuration is then lost as the facial primordia become aligned in preparation for primary palate formation. The primary palate is formed at six days development by fusion of the "free-ended" medial nasal processes with the combined lateral nasal and maxillary processes across the nasal grooves. Just prior to fusion, long, slender filaments extend from the apposing surfaces of the facial primordia in the regions of prefusion contact. It is speculated that these "prefusion filaments" may function in alignment or adhesion of the facial primordia. In "cleft primary palate" embryos, facial morphogenesis appears to arrest at five days development, so that the square configuration persists. The medial nasal processes never contact the lateral nasal and maxillary processes, but instead remain separated from them by wide nasal grooves. Furthermore, facial primordia of mutant embryos do not exhibit the "prefusion filaments" characteristic of normal embryos.


Nerve trophic effects: an in vitro assay for factors involved in regulation of protein synthesis in regenerating amphibian limbs. We have developed a rapid sensitive test for factors that mimic the trophic effects of nerves by maintaining normal rates of protein synthesis in denervated forelimb blastemata of adult newts (Notophthalmus viridescens). Rates of protein synthesis in secondary blastemata are similar. However, after they are denervated and explanted into organ culture the rates of protein synthesis first increase and later fall below control values. Similar changes occur after denervation in vivo. The alterations in the rates of protein synthesis are prevented by adding to the culture medium aqueous extracts from brains of adult newts or chicken embryos. The active material is either a peptide or a protein.


Studies on oocyte maturation of the medaka, Oryzias latipes. VI. Relationship between the circadian cycle of oocyte maturation and activity of the pituitary gland. The relationship between pituitary activity and oocyte maturation was examined in Oryzias latipes (medaka), which has a circadian cycle of oviposition. Throughout the circadian cycle of oviposition, females possessed a population of large oocytes more than 800 micronmeter in diameter that could mature in the presence of gonadotropin. Oocyte maturation was observed in vitro in females hypophysectomized between three and ten hours after the beginning of the light period with the number of maturing oocytes increasing as hypophysectomy was delayed. Although in vivo oocyte maturation was blocked by hypophysectomy within two hours after the beginning of the light period, it was restored by a single injection of synthetic or mammalian pituitary hormones (gonadotropic, corticotropic and thyrotropic hormones) within ten hours after hypophysectomy. Of these pituitary hormones, FSH, LH and TSH could induce in vitro maturation of isolated oocytes. Oocytes matured in vitro in the absence of exogeneous hormones if they were isolated nine or more hours after the onset of light. The present study indicates that the circadian cycle of maturation of Oryzias oocytes is controlled by the release of pituitary hormone between three and nine hours after the beginning of the light period.


Satellite cells are mitotically quiescent in mature mouse muscle: an EM and radioautographic study. Normal adult mouse tibialis anterior muscles were perfused continuously with 3H-thymidine for nine days. Quantitation of the satellite cell population in these muscles reveals that not only is the frequency of satellite cell nuclei low, but that those present are mitotically quiescent.


Scanning electron microscopy of primordial germ cells in early chick embryos. Primordial germ cells (PGCs) of the early chick embryo were examined using scanning electron microscopy. Temporal changes in the form and distribution of surface projections were found to be correlated with migratory phases of PGCs. Non-migrating PGCs were spherical to ovoid with relatively smooth surfaces. Their transition to the migratory phase was first evidenced by a burst of membrane activity. Migrating PGCs became somewhat flattened against the underlying hypoblast (which serves as the substratum for their migration) and exhibited blebs and lamellar processes. The lamellar processes were most prominent at the leading edges of actively migrating PGCs. Overall results of the present study indicates that PGCs found in the germinal crescent area of early chick embryos actively migrate on the dorsal surface of the hypoblast towards posterior embryonic regions.


Calcium-dependent increase in spike duration during repetitive firing of Aplysia axon in the presence of TEA. Repetitive stimulation was studied in the axon of the giant neuron, R2, of Aplysia in the presence of TEA. In 25 or 50 mM extracellular TEA, a plateau develops on the axon spike during repetitive stimulation at frequencies greater than 3/sec. The plateau in extracellular TEA is inhibited by 30 mM CoCl2 or 1 mM CdCl2, and is enhanced by raising the Ca concentration. Intracellular TEA induces a plateau on the axon spike at frequencies less than 1/30sec. This plateau increases in duration with repetitive stimulation at higher frequencies and is inhibited by 30 mM CoCl2 or 1 mM CdCl2. The increase in spike duration during repetitive firing in the presence of TEA is indicative of an increased entry of Ca during the spike train.


Changes in synthesis of specific proteins following axotomy: detection with two-dimensional gel electrophoresis. Changes in protein synthesis during development and following axotomy were analyzed by two-dimensional gel electrophoresis. The two major postganglionic nerves emerging from the superior cervical sympathetic ganglia (SCSG) of adult rats were either cut or crushed unilaterally. At intervals ranging from 1 to 112 days after surgery both SCSG were removed and incubated for 1 hr in the presence of 14C-leucine. Proteins were extracted and subjected to two-dimensional electrophoretic separation and autoradiography. With this technique, proteins are separated on the basis of isoelectric point and molecular weight. Also, intact SCSG from 1, 2, 7, and 14 day old rats were labeled and analyzed. It was found that a minority of the separated proteins exhibited some detectable change in relative rate of synthesis following axotomy. Actin exhibited a slight (less than 20%) increase in relative synthesis rate while tubulin did not change significantly. There were small but significant differences in the protein patterns following nerve crush, as opposed to nerve cut. Comparison of protein synthesis patterns from developing rat SCSG with those from intact and from axotomized adult SCSG failed to demonstrate any marked similarity between the developmental and the axotomy patterns.


The caudal ganglion of the leech, with particular reference to homologues of segmental touch receptors. The caudal ganglion of the leech, which provides sensory and motor innervation to the posterior sucker, represents the fusion of seven embryonic segmental ganglia. Although fused, each of the seven contributing ganglia ("subganglia") of the caudal ganglion can be distinguished morphologically and functionally. The roots from each subganglion carry the axons of mechanoreceptors homologous to "touch" cells found in the segmental ganglia and the subesophageal compound ganglion. The receptive fields supplied by the touch cells of the caudal ganglion are uniquely arranged and reveal the modified segmentation of the circular posterior sucker. Extensive overlap of sensory innervation occurs between adjacent segments of the sucker, beyond the overlap characteristic of the homologous cells of body segments. It thus appears that the touch receptors of the caudal ganglion are less restricted than receptors of the segmental ganglia with regard to their territories of innervation. The caudal ganglion has additional unique properties that establish it as a distinct integrative center of the leech CNS.


Hypothyroidism with true myotonia.

Hypothyroidism with true myotonia. A patient with subclinical hypothyroidism who presented with true myotonia is described. There was no evidence that either he or members of his family had dystrophia myotonica or myotonia congenita. Treatment with thyroxine resolved his symptoms completely.


Reflexes evoked in human thenar muscles during voluntary activity and their conduction pathways. Responses evoked by an electrical stimulus to the median nerve in a small muscle of the human hand during a voluntary contraction have been examined. Two of these responses have been shown to be evoked through reflex pathways. The first, with a mean afferent conduction velocity of 64 m/s and estimated central delay of about 0.8 ms, is identified as an H reflex. The second response, which has an undefined central pathway, has a mean afferent conduction velocity of 43 m/s and an estimated central delay of about 17 ms.


Disturbances of ocular movements and blinking in schizophrenia. Neurological examination and electroencephalograms and electro-oculograms, recorded by telemetry, from unmedicated patients with acute and chronic schizophrenia demonstrate a number of abnormalities of extraocular movement including staring, abnormal blink rate, absent glabellar reflex, and increase in horizontal eye movements. As potential clues to the pathophysiology of schizophrenia, these disturbances are analysed in relation to anatomical substrate and dopamine modulation of ocular movement, rapid eye movement sleep, and the neurological disorders in which similar disturbances of ocular movement occur.


Ewing's sarcoma of the spinal epidural space: report of two cases. Two new cases of primary extraosseous Ewing's sarcoma of the spinal epidural space, and their histogenesis and differential diagnosis are described. The diagnosis of Ewing's sarcoma, which is essentially an undifferentiated tumour, depends largely on the exclusion of several other neoplasms with morphological similarities. With these two cases, 43 extraosseous Ewing's sarcomas have been reported to date, seven of which were epidural in location.


Assessment of recovery from serious head injury. A procedure for serial assessment of neuropsychological recovery after serious head injury was designed. The assessment procedure consists of four segments, each appropriate for different phases of the recovery process. Recovery can be traced from early in the period of post-traumatic amnesia until it reaches an asymptote. The course of recovery of several patients has been observed. The procedure is shown to be practical and appears to be valid. The recovery process is compared to ontogenesis, and is shown to be generally similar though differing in important particulars.


Brain abscesses in Northern Ireland: a 30 year community review. One hundred and seventy-two cases of intracranial abscesses, obtained from Neurosurgical and Centralised Autopsy Records for Northern Ireland for the 30 years, 1947--1976, have been reviewed. The incidence of the disease has fallen from five to three per million of population per year over the past three decades. Intracranial abscesses were three times as common in males as in females. Twenty-nine per cent of the abscesses were in the temporal lobe, 25% frontal, 10% parietal, 6% cerebellar, 3% occipital, and 7% were either subdural or in deep sites such as the thalamus; the remainder (20%) were multiple. Multiple and occipital abscesses were all fatal, temporal and parietal abscesses were associated with a 65% mortality, and 45% of patients with frontal abscesses died. Chronic suppurative otitis media was the single largest cause, and it was the only aetiological factor to have shown a progressive decline over 30 years. For those seen and treated in the neurosurgical unit the mortality was 53%, but if those obtained from the necropsy records were included the overall mortality was 70%.


Coincidental aneurysms with tumours of pituitary origin. Angiographic studies on 150 pituitary adenomas and 33 craniopharyngiomas presenting for surgical treatment are reviewed. Eleven incidental silent aneurysms (four arising from the intracavernous and four from the supraclinoid carotid artery, and three from the anterior cerebral artery complex) are shown. Intracavernous aneurysms were also present in two acromegalic patients who had been treated previously with yttrium implantation. Although encasement of vessels by these tumours is unusual, the relevance of vascular abnormalities to surgical treatment is sufficient to justify routine magnification angiography.


Periodic respiration in erect posture in Shy-Drager syndrome. Tilt-table polygraphic study in four patients with Shy-Drager syndrome demonstrated periodic apnoea in the erect posture. In one patient reduced hypercapneic ventilatory response and necropsy findings of neuronal loss and astrocytosis in the pontine tegmentum suggested dysfunctional respiratory neurones in the brainstem. One patient had Cheyne-Stokes respiration during the late stage of the illness.


Blue-domed cyst with optic nerve compression. A patient presenting with persistent headache of vascular quality and an intermittently progressive chiasmal syndrome had a blue-domed haemorrhagic cyst beneath the right optic nerve and underwent surgical drainage with good results. As prompt surgical intervention may proivde significant return of visual function, detailed diagnostic evaluation of similar cases is recommended.


Removal of an arteriovenous malformation from the basal ganglion. A case is reported in which an arteriovenous malformation was removed from a portion of the basal ganglion and region of the lenticulostriate arteries. The patient made an excellent recovery. The importance of stereoangiography and the operating microscope are emphasised.


Treatment of carotid-cavernous fistula using a balloon-tipped intra-arterial catheter. The use of a balloon-tipped intra-arterial catheter for occlusion of the carotid-cavernous fistula seems to be a simple  and, by the reports published to date, very effective procedure, and its usage has been without any sequelae. Successful treatment of carotid-cavernous fistulae by this method is reported in two patients.


Observations on the development of muscle hypersensitivity following chronic nerve conduction blockage and recovery. Agar-sleeves containing 0.01%, 0.015% and 0.02% Tetrodotoxin were placed onto the sciatic nerve of the rat. The time-course of the conduction block and the full recovery of the nerve were studied; correlations were drawn with the hypersensitivity developed on the innervated muscles. The earliest sign of a TTX-produced conduction block was a decrease in the amplitude of the faster conducting fibres appearing 3 min later. Complete block was fully established 35 min later. The duration of a complete conduction block was a dose-dependent phenomenon and lasted from 1--4 days. The recovery process was gradual, simulating the reverse pattern of the acute TTX-block but spread over a much longer period with complete conduction recovery occuring 12 to 13 days later. Innervated muscles behaved as paralytic even before the complete establishment of a conduction block and remained so for 2--6 days after which clinical recovery was prompt. Muscles innervated by the TTX-treated nerves developed hypersensitivity to acetylcholine which could be seen within two days. This hypersensitivity continued to increase over the following days, despite some recovery of conduction. Its maximum appeared six to seven days later and then declined to return to normal at the time when nerve conduction properties had fully recovered. A similar degree of partial conduction block when acutely established always resulted in paralysis but when chronically present, the clinical picture of paralysis was fully compensated, due to the hypersensitivity of the muscle and possibly to collateral nerve sprouting.


Neurological manifestations of Paget's disease. The neurological features of 96 patients whose skull and spinal X-rays showed the features of Paget's disease of bone were reviewed. The clinical phenomena were compared with those in another group of patients, identified by having a normal skull X-ray. Cranial nerve lesions and spinal syndromes could be attributed to the Paget's disease in many cases but the evidence did not suggest that other clinical phenomena such as dementia and epilepsy were related to the bony changes. Amongst cranial nerve lesions deafness is certainly attributable to Paget's disease, hemifacial spasm when combined with other signs is probably related, and possibly also trigeminal neuralgia. Basilar invagination can be asymptomatic, but deafness, hemifacial spasm and pyramidal signs were more prevalent in its presence. The importance of reviewing the cause and effect relationship between Paget's disease and neurological problems is discussed in the context of the therapeutic use of calcitonin.


Multicystic encephalomalacia of infancy: clinico-pathological report of 7 cases. Clinical follow up and complete neuropathological examination was made on seven cases of multicystic encephalomalacia of infancy. Etiological factors were carefully studied in all the cases. They consisted of prenatal injuries presenting as a cord prolapse, in 3 cases; prolonged labour with marked cyanosis; abdominal trauma during gestation, and various maternal infections at different stages of pregnancy. Pathological interest is centred on the variable involvement of different areas of the brain, generally sparing the cerebellum and brain stem, and being minimal or absent in the occipito-temporal areas. This distribution may be explained by a different effect of the "causal agency" on these different areas, or by a different capacity of these regions to react against injury. Among the etiological factors reviewed in the literature, the anoxic theory appears the most probable, as there was a close parallelism between lesions and vascular areas, mainly the carotid and vertebro-basilar systems.


Peroxidase in ceroid-lipofuscinosis.

Peroxidase in ceroid-lipofuscinosis. Peroxidase determination in leucocyte homogenates of 3 patients with ceroid-lipofuscinosis the infantile, late-infantile, and juvenile form, was not different from normal control values.


Abnormal epinephrine urinary excretion in Parkinsonians: correction of the disorder by levodopa administration. Epinephrine (E) and norepinephrine (NE) as well as vanilmandelic acid (VMA) urinary excretion was investigated in 54 patient with Parkinsonism and 29 controls. In Parkinsonians the mean value of NE excretion was normal, while that of E was significantly higher than in controls (P less than 0.001). Analysis of results in terms of age showed that only the patients in the 5th and 6th decades displayed significant increases in E excretion when compared to age-matched controls. No age-group differences were found for NE excretion. VMA was significantly higher in Parkinsonians than in controls. Both in Parkinsonians and in controls VMA excretion correlated significantly with that of NE but not with the E excretion. Ten-day treatment with levodopa (1.5 g/day) of Parkinsonians below the age of 60 was followed by a constant decrease in the excretion of E and a less consistent, nonsignificant increase in the excretion of NE. The abnormality of E excretion noticed in younger Parkinsonians suggests involvement in the pathologic process, of adrenal medulla, the main site of E synthesis.


Aspergillosis of the central nervous system. Aspergillosis of the central nervous system is rare. The spread to the CNS is usually from the lungs and uncommonly from paranasal sinuses. Four cases of CNS aspergillosis with spread of infection from the paranasal sinuses are described. Two patients had meningitis, one intracranial granuloma and one presented with subarachnoid haemorrhage resulting from a fungal mycotic aneurysm. Difficulties in establishing the diagnosis are discussed.


Autosomal dominant spinal muscular atrophy: a clinical and genetic study. A clinical and genetic study of 6 kindreds (13 patients) with autosomal dominant spinal muscular atrophy is presented. Evidence is presented to indicate that two separate autosomal dominant genes are involved. One of these causes clinical disease with onset in early childhood (birth--8 years), which is relatively benign and in which proximal selectively of muscle involvement is not marked. A separate autosomal dominant gene causes a disease with onset in adult life (median age 37 years), showing marked initial proximal selectively; this disease may be more rapid in its clinical progression. Penetrance of both genes approaches 100%. Incidence figures are presented; less than 2% of all cases of childhood onset spinal muscular atrophy, but 30% of adult onset cases, are due to an autosomal dominant gene transmitted from an affected parent. Implications for prognosis, diagnosis and genetic counselling are discussed. A review of 11 kindreds of dominant spinal muscular atrophy in the literature is presented.


Detachable balloon and calibrated-leak balloon techniques in the treatment of cerebral vascular lesions. Of the cerebral vascular lesions that can be treated with intravascular detachable balloon techniques, carotid-cavernous sinus fistulas and vertebro-vertebral fistulas have the best results. The great advantage of this technique is that the cerebral blood flow can usually be preserved after the occlusion of the fistula. The authors report 17 postraumatic carotid-cavernous sinus fistulas successfully treated with preservation of the carotid blood flow in 12 cases. None of the patients died, and the morbidity was limited to one case of third nerve palsy. The treatment of aneurysms by this method is, however, much more difficult and dangerous. Of 14 cases treated, seven good results were obtained. Two patients died and two had a poor outcome. The embolization of certain brain angiomas with calibrated-leak balloons using bucrylate promises to be important in the future.


Decreased mortality from brain abscesses since advent of computerized tomography. No deaths have occurred among 20 consecutive patients with intraparenchyma brain abscesses treated at the University of California, San Francisco, since computerized tomographic (CT) brain scanning became a routine diagnostic procedure (study period: July, 1974, to June, 1977). These patients have been compared to 18 consecutive cases treated without benefit of CT analysis (January, 1970, to June, 1974) in order to determine the factors responsible for the recently improved prognosis. The mortality rate was 44% for all cases and 36% for all operated patients treated before the availability of CT. Similar morbidity (about 33%) was seen in survivors from both series. No significant differences in the two groups were noted with respect to patient population and antibiotic or corticosteroid therapy. Among the factors that may have contributed to the improved results for patients diagnosed with CT are: less frequent occurrence of multiple abscesses, fewer patients with poor preoperative clinical status, and a greater incidence of total abscess removal. In addition, CT scanning provided more accurate diagnosis and localization of abscesses and aided in the rapid detection of postoperative complications that probably accounted for six out of eight deaths in the earlier series. Serial CT studies provide a means to optimize the timing for surgical intervention and plan appropriate medical therapy. It is noteworthy that two patients have been followed by serial CT scans to non-surgical cures.


Microsurgical relationships of the superior cerebellar artery and the trigeminal nerve. Comparison and distortion of the trigeminal nerve by a tortuous and elongated superior cerebellar artery (SCA) is postulated to be a frequent cause of trigeminal neuralgia. This theory and the use of operative therapy in which the offending arterial loop is separated from the trigeminal nerve has created a need for more detailed information on the relationship of the SCA and the trigeminal nerve. In order to meet this need, 50 trigeminal nerves and the adjacent SCA were examined in 25 adult cadavers. Twenty-six of the 50 nerves examined had a point of contact with the SCA, but it was uncommon for the arterial contact to produce distortion of the nerve. In six instances, the contact was at the pontine entry zone of the trigeminal nerve, the site of arterial compression postulated to be associated with trigeminal neuralgia. Four trigeminal nerves (8%) had a point of contact with the anterior inferior cerebellar artery (AICA). The fact that large arteries are commonly in contact with the trigeminal nerve is important not only because of the controversial relationship of neurovascular contact to trigeminal neuralgia, but because of the possibility that major vessels may be encountered and injured during rhizotomy and other posterior fossa operations on the trigeminal nerve.


Treatment of moyamoya disease with STA-MCA anastomosis. Moyamoya disease is a chronic occlusive cerebrovascular disease of unknown etiology for which no effective treatment has been found. The authors report the result of 23 superficial temporal-middle cerebral artery (STA-MCA) anastomoses and seven encephalomyosynangioses, performed on 13 cases with moyamoya disease and on four additional atypical cases. There were 10 children and seven adults in this study. The follow-up period ranged from 1 year and 4 months to 4 years and 1 month postoperatively; nine patients had excellent results, five good, and one fair; two patients were unchanged. The anastomotic procedure was most effective for transient ischemic attacks, reversible ischemic neurological deficits, and even minor or moderate neurological symptoms. The STA-MCA anastomosis appears to be an effective treatment for moyamoya disease.


Symptomatic subependymoma. Report of 21 cases with review of the literature. Of 48 cases of subependymoma reported to date, 22 were associated with symptoms. In a personal series of 47 additional cases of subependymoma reviewed by the author, 21 were symptomatic. The mean age of patients with symptom-producing subependymomas was 39 years, that is, 20 years younger than that of patients with asymptomatic tumors. Symptoms were most often produced by large tumors, particularly those arising from the septum pellucidum (100%), the floor of the fourth ventricle (65%), and the lateral ventricular walls (55%). The majority of symptomatic tumors displayed the classic gross and microscopic features of subependymomas; however, large tumors more frequently demonstrated cyst formation, microcalcification, and vessel degeneration accompanied by hemorrhage. Of all subependymomas, 15% were microscopically composed of an admixture of classic subependymoma and cellular ependymoma; of these, one-half occurred within the first decade, all were situated in the fourth ventricle, and 80% were symptomatic. In contrast to pure subependymomas, the mortality rate of patients with mixed tumors was 80%, reflecting their growth potential, large size, and dangerous location.


Immediate correction of sagittal synostosis. A technique for correction of sagittal synostosis with achievement of an immediately pleasing cosmetic result is presented. Even with replacement of bone and no attempt to inhibit bone union, premature reclosure does not occur. Moss' theory of dural tensions is discussed to explain the effect.


Myelomeningocele before birth.

Myelomeningocele before birth. The authors report a study of 92 human embryos and four fetuses with myeloschisis. The characteristics of embryonic myeloschisis compared with spina bifida cystica in infants are: 1) the lesion is often more diffuse, involving the whole spinal cord (12 embryos); 2) the cervical cord is frequently affected (23 of the remaining 80 embryos); 3) holoprosencephaly is frequently associated (18 embryos); 4) meningocele is not found; and 5) hydrocephalus and Arnold-Chiari malformation are not yet developed. Hydrocephalus and Arnold-Chiari malformation are found in myeloschistic fetuses. Almost all embryos with diffuse and cervical myeloschisis or with holoprosencephaly are extruded before birth by spontaneous abortion. Absence of meningocele in the embryonic period implies that its appearance is deferred to the fetal period. The development of hydrocephalus and Arnold-Chiari malformation also seems to be delayed until the fetal period. Our observation implies that myelomeningocele is induced by non-closure of the neural tube, not by rupture once it was closed. "Neural overgrowth" and disturbed "recanalization process" are discussed in relation to the pathogenesis of myelomeningocele.


Aneurysmal bone cyst of the skull. Case report. A case of an aneurysmal bone cyst of the frontal bone is presented. This lesion is rare in the calvaria. It is a benign condition, and may extend intracranially. Total surgical removal is recommended whenever possible either in one stage or in multiple stages. The operation is comparatively easier in the calvaria than it is in the vertebral column or the long bones, where no more than thorough curettage is usually possible. The fact that this is a definite, readily recognizable pathological entity is stressed. Prognosis is excellent and total removal will effect a cure.


Blow-in fracture of both orbital roofs caused by shear strain to the skull. Case report. The authors report a case with blow-in fractures of both orbital roofs caused by horizontal shear strain to the skull. The mechanism of the injury is discussed. Ophthalmic symptoms were improved by removal of intraorbital bone fragments and by repair of the orbital roof defects with methyl methacrylate.


Intracerebral hemorrhage due to ruptured venous aneurysm. Report of two cases. Documented rupture of venous aneurysms associated with arteriovenous fistulas is uncommon, and reports of the formation and rupture of venous aneurysms that are not associated with fistulas are distinctly rare. One case is presented in which spontaneous rupture of a saccular aneurysm on a vessel draining an arteriovenous fistula produced an intracerebral hematoma. A second case in described in which fatal intracerebral hemorrhage was produced by a traumatic aneurysm of an otherwise normal superficial cerebral vein.


Bilateral intracerebellar calcification associated with cerebellar hematoma. Case report. A case of bilateral intracerebellar calcificaiton associated with cerebellar hematoma on the left side is reported. Clinical and microscopic examination failed to clarify the causes of calcification and hematoma. It is postulated that hemorrhage occurred from time to time through the fragile calcified vessel walls, since some portions of the organized hematoma were composed of massive erythrocytes.


Computerized tomographic definition of mesencephalic hematoma with evacuation through pedunculotomy. Case report. A 10-year-old boy, who presented with symptoms of a progressive hemorrhagic stroke, had suffered a spontaneous midbrain hematoma. The extent of the clot was detailed by computerized tomography and the lesion was surgically removed through pedunculotomy.


Cerebral aneurysm in an infant with fibromuscular hyperplasia of the renal arteries. Case report. The authors report a case of subarachnoid hemorrhage in an 11-month-old infant with tragic outcome. Radiological investigation showed an anterior communicating aneurysm, and postmortem examination confirmed the aneurysm to be a so-called "berry" aneurysm. There were also typical signs of fibromuscular hyperplasia of the renal arteries. The microscopic findings are discussed. In view of the rarity of both aneurysms and fibromuscular hyperplasia in such a small child, a possible association of these entities suggested by several earlier investigators is reviewed.


Superficial temporal-middle cerebral artery anastomoses in the treatment of a carotid-cavernous fistula. Case report. The treatment of patient with a carotid-cavernous fistula and complete occlusion of the contralateral internal carotid artery is reported. Bilateral superficial temporal-middle cerebral artery anastomoses were performed to create collateral circulation prior to trapping the fistula. The patient made an excellent recovery.


Intradural sacral nerve root metastasis mimicking herniated disc. Case report. Spinal tumors may mimic herniated discs but the neurological findings and radiographs usually indicate the correct diagnosis. A case of metastasis to the S-1 intradural nerve rootlets is reported, with symptoms closely simulating a herniated disc. The correct diagnosis could not be made preoperatively in spite of clinical suspicion of a tumor.


Asymptomatic persistence of infundibularis recessus. Case report. A congenital malformation of the anteroinferior aspect of the third ventricle is described. This anomaly has been found only rarely in anatomical descriptions. The embryological development of the pituitary region is described.


The effects of chronic undernutrition over generations on rat development. Experimental rats were fed 2/3 (10g/24 hours) of adlibitum diet throughout pregnancy and post-weaning, thus far for six generations; their brain and body development was compared with those of controls fed ad libitum (15.5 g/24 hours). As expected from previous reports, neonatal F1 offspring exhibited highly significant decreases in body weight, cerebral wet weight, cerebral DNA and cerebral protein. However, neonatal decreases were not greater in F2 through F6 than in F1 indicating that there was no cumulative effect of this undernutrition on offspring's parameters over generations. Maternal body weight at mating (90 days) and percentage of females that did not litter steadily decreased over generations. The observed high mortality in F1 through F6 and the resulting strong natural selection in favor of best mothers and weanlings could explain these findings. The phenomena contributing to high mortality are multiple and involve maternal factors during pregnancy and before weaning, as well as offspring factors.


The relationship of choline and carnitine in the choline deficient rat. Levels of carnitine in choline deficient and choline supplemented rats were measured in heart, skeletal muscle, plasma, and liver after various treatments. A decreased hepatic concentration of carnitine was shown to be closely correlated to the deficiency in dietary choline. No change in the concentration of plasma ketone bodies after a 48 hour fast was observed in the choline deficient rats when compared to choline supplemented controls, even though the level of hepatic carnitine subsequent to the fast was lower in the deficient rats. The concentration of hepatic carnitine was increased to normal values 1.5 hours after a single injection of 100 mumoles of choline, but was not affected by other "methyl" donors. From experiments presented in this communication it is concluded that the increase in hepatic carnitine is probably due to increased transport and uptake of this compound into the liver rather than by "de novo" synthesis.


Use of -l-ascorbic acid, ethocel coated ascorbic acid and ascorbate 2-sulfate in diets for channel catfish, Ictalurus punctatus. Purified diets with five levels (25, 50, 75, 100, and 200 mg/kg) of supplemental L-ascorbic acid (LAA), and equimolar levels of ethylcellulose coated L-ascorbic acid (EAA) and dipotassium L-ascorbate 2-sulfate dihydrate (AS) were pelleted and fed to 7.9 +/- 0.2 g channel catfish fingerlings for 20 weeks. A dietary level of 23 mg/kg of all three forms of vitamin C prevented spinal abnormalities. Approximately 50 mg/kg diet of either LAA or EAA was sufficient for maximal growth and feed efficiency. Growth response to AS was similar to a Michaelis-Menten type curve and 200 mg/kg diet of AS was necessary to achieve maximal growth. Blood and liver ascorbic acid levels were positively correlated with supplemental levels of LAA, EAA, and AS up to 200 mg/kg; however, blood and liver ascorbic acid levels of fish fed AS were considerably less than those fed LAA and EAA. Weight gains were positively correlated with blood ascorbate levels up to 7 microgram/ml. No measurable level of AS was detected in blood or liver. These results suggest that the rate of enzymatic hydrolysis of AS to LAA or rapid excretion of AS may have been the limiting factor.


Diet-hormone interrelationships in the rat. Twenty-eight-day old male rats were fed, either ad libitum or in restricted amounts, isoenergetic diets containing 2, 5, 10, 15, 25, or 50% lactalbumin and 5, 11.9, or 21.1% fat for 8 weeks. They were then killed and the plasma levels of triiodothyronine (T3), thyroxine (T4), insulin, glucagon, corticosterone, norepinephrine (NE), and epinephrine (E) were measured. Dietary changes explained most of the variation in the plasma concentrations of T3, T4, insulin, and glucagon but less than 20% of the variation in the plasma concentrations of the adrenal hormones. Dietary protein level was directly related to plasma T4, insulin and corticosterone and inversely related to plasma T3, glucagon, NE, and E. Dietary fat level had its most significant effect on the plasma glucagon concentration to which it was inversely related whereas the most noteworthy effect of a low energy intake was to reduce plasma E and thereby to increase the NE/E ratio. A refeeding study confirmed the effects of dietary protein level on plasma hormone concentrations and showed that the changes in diet-hormone interrelationships in 12-week old male rats have been derived by multiple regression analyses of the data.


Sulfate metabolism in rat calvaria cultured under vitamin A deficient conditions. The metabolism of sulfated glycosaminoglycans (GAG) in bone has been found to be altered in vitamin A deficiency. A neonatal rat calvarium model system was used to determine if these changes are related to catabolic defects. Two day old rat pup calvaria were cultured in media containing serum from A- or A+ rats and radiolabeled sulfate or glucosamine. The incorporation of 35S-sulfate into the GAG fraction of calvaria cultured with A- serum for 48 hours was significantly increased compared to the values found in calvaria cultured with A+ serum (A-, 1,970 +/- 300; A+, 940 +/- 177; X +/- SD). The uptake of 35S-sulfate into the GAG fraction of calvaria cultured with A- serum showed continuous increase over 96 hours,whereas, 35S-sulfate uptake leveled off after 24 hours in the A+ group. There was also a significant increase in [14C]glucosamine uptake into the GAG fractions of calvaria cultured with A-serum (A-, 1,966 +/- 537; A+, 1,662 +/- 244; X +/- SD). To determine if the alteration in metabolism of sulfated GAG was in the biosynthetic or degradative pathways, a chase study was performed in which the calvaria were prelabeled with 35SO4. The rate of tissue loss of 35S-sulfate was lower in the total digests and the GAG fractions from calvaria cultured with A- serum than those cultured with A+ serum. Thus, the alteration in the metabolism of the sulfated GAG resulting from A- culture conditions seems to be defect in the degradative process.


Late effects of premature weaning to different diets in the rat. Female rats were prematurely weaned on the 18th postnatal day to a high fat (HF) or high carbohydrate (HG) diet. Twelve days later they were all given free access to a cereal based stock diet. When aged 10 months, they were all fed an atherogenic diet for the next 2 months and were then killed. Blood cholesterol levels were higher in the HG group at 11 months but not 1 month later. After 2 months of feeding the atherogenic diet, injected 14C-cholesterol disappeared more slowly from the HF group during the first 24 hours after injection but more rapidly during the next 48 hours than the HG group. At that time, serum glucagon levels in the HF group had doubled but remained unchanged in the HG group. Serum insulin levels decreased significantly during that same period in the HF group but not the HG group. Consequently, the I/G molar ratio was lower in the HF than the HG group after 2 months of feeding the atherogenic diet. At 12 months, selected enzyme activities in white adipose tissue and liver did not differ between the two groups, except for phosphoenolpyruvate carboxykinase, the activity of which was higher in the HG


The influence of dietary fat and meal frequency on lipoprotein lipase and hormone-sensitive lipase in rat adipose tissue. Activities of NaCl-inactivated lipoprotein lipase (LPL) and protamine-resistant hormone-sensitive lipase (HSL) in adipose tissue, accumulation of carcass fat, and serum triglycerides (TG) were determined in meal-fed (MF) and ad libitum-fed (AD) rats. At each feeding frequency, diets provided total fat as 15 or 30% of calories and polyunsaturated fatty acids (PUFA) as 2.5 or 11% of calories. The average energy intake of MF rats was 67% that of AD rats. Total weight gained by MF rats was only 60% that of the AD rats. Significantly greater activities of LPL, HSL, and LPL:HSL in adipose of MF rats suggested a greater capacity for fat accumulation which was not realized at the limited energy intake. In AD rats, the percentage of body fat was significantly correlated  with LPL:HSL and with serum TG, suggesting that the relative enzyme activities and fat deposition may be influenced by the concentration of circulating TG. Mean body fat of rats receiving the 30% fat diet was significantly greater than that of rats fed 15% fat. Both serum TG and adipose LPL activity were significantly reduced when the diet contained high levels of PUFA.


Lactate as a precursor of fatty acids in bovine adipose tissue. In vitro experiments were conducted to determine the rates of lactate, acetate, and glucose conversions to glycerol, CO2, and fatty acids by subcutaneous adipose tissue of cattle. To study the pathway for lactate utilization as a carbon source for lipogenesis, the effect of each of four substates (lactate, acetate, pyruvate, and glucose) on the rate of conversion of the other three was determined. The rates of lactate conversion to fatty acids and to glycerol were greater than those for acetate and glucose. Acetate, however, was oxidized to CO2 at greater rates than either lactate or glucose. Acetate was used for fatty acid synthesis at greater rates than pyruvate was less than that from lactate but greater than that from glucose. Acetate additions to the incubation media decreased the synthetic rates from lactate, pyruvate, and glucose. Pyruvate decreased rates of fatty acid synthesis from glucose but slightly (not statistically significant) stimulated synthesis from lactate and acetate. Lactate decreased glucose and pyruvate utilization but had no effect upon that of acetate. Our results clearly demonstrated that lactate can be used for fatty acid synthesis as well as for glycerogenesis by bovine adipose tissue.


Effect of dietary fiber on egg yolk, liver, and plasma cholesterol concentrations of the laying hen. Two experiments were conducted to determine the effect of dietary fiber source and level on egg yolk, liver, and plasma cholesterol concentrations of White Leghorn laying hens. Initially, dietary fiber levels of 2.05, 4.41, 6.68, and 8.79% furnished mainly by sunflower meal were fed to laying hens for 140 days. In the second experiment, alfalfa meal, ground whole oats, sunflower meal, rice mill feed, or wood shavings was added to a corn-soybean meal basal diet to furnish 2.00% added crude fiber and fed to laying hens for 84 days. Yolk cholesterol decreased 4.39, 10.38, and 13.29% by feeding crude dietary fiber levels of 4.41, 6.68, and 8.79%, respectively, to hens as compared to a corn-soybean meal basal diet containing 2.05% crude fiber. Egg yolk cholesterol was significantly decreased by feeding alfalfa meal, oats, sunflower meal, rice mill feed, or wood shavings to laying hens when compared to yolk cholesterol of hens fed the basal diet. The greatest reduction in egg yolk cholesterol was found by feeding either oats or wood shavings. No significant differences were found in plasma cholesterol due to dietary fiber level. Plasma triglycerides decreased and liver cholesterol increased as dietary fiber level increased in diets fed to laying hens. When laying hens were fed alfalfa meal, oats, rice mill feed, or wood shavings, plasma cholesterol significantly decreased. Liver cholesterol increased when hens were fed either alfalfa meal or rice mill feed as the primary fiber source.


Zinc absorption and metabolism by isolated, vascularly perfused rat intestine. An isolated vascularly perfused rat intestine system was utilized to examine various aspects of zinc absorption in an attempt to more clearly examine the mechanisms involved. The lumen was perfused with a modified tissue culture medium containing 65Zn. The vascular system was perfused from the superior mesenteric artery to the portal vein, with Krebs-Ringer bicarbonate buffer containing 5% rat serum. The criterion for absorption was the amount of radioactivity transferred to the vascular perfusate. When the intestines were obtained from rats that had consumed a zinc-deficient diet the amount of zinc absorbed increased markedly. Conversely, elevation of zinc status decreased the amount of 65Zn that could be transferred to the vascular perfusate. These data strongly suggest that the isolated, perfused rat intestine retains the ability to exercise homeostatic control over 65Zn absorption. Transfer of infused 65Zn to the vascular perfusate was significantly decreased by aspirin, phytate, and prostaglandin E2. Uptake of 65Zn from the lumen into the intestinal cells was significantly increased by histidine and significantly decreased by phytate and prostaglandin E2. Thus, the isolated, vascularly perfused rat intestine appears to be capable of differentiating between the cellular uptake and cell to plasma transfer phases of zinc absorption.


Vitamin E, antioxidants and lipid peroxidation in experimental atherosclerosis of rabbits. The purpose of this study was to evaluate the effects of large amounts of dietary vitamin E and butylated hydroxyanisole (BHA) and butylated hydroxytoluene (BHT) in rabbits fed a low-cholesterol, atherogenic diet, and to seek for evidence of lipid peroxidation in the atherosclerotic lesions. Rabbits were fed a purified atherogenic diet, containing butter or the basal diet supplemented with either 1.0% of vitamin E or 0.1% each of BHA and BHT for periods up to 3 years; a negative control group was fed the basal diet with corn oil replacing butter. Aortic and coronary atherosclerosis were more frequent and extensive in rabbits fed either the basal diet or the basal diet supplemented with BHA and BHT than in rabbits fed either the basal diet supplemented with vitamin E or the negative control diet. Dietary vitamin E inhibited atherogenesis by preventing hypercholesterolemia. No evidence of lipid peroxidation was detected in the arterial lesions.


"Protected" polyunsaturated fatty acid in the diet of the ewe and the essential fatty acid status of the neonatal lamb. A polyunsaturated fatty acid supplement, protected from biohydrogenation in the rumen by a formaldehyde-treated protein coat, was fed to ewes during the last 8 weeks of pregnancy as a possible method of improving the poor essential fatty acid status of the newborn lambs. Significant increases were observed in the concentrations of 18:2 (n-6) in both the cholesteryl ester and phospholipid fractions of the plasma and in the phospholipid fraction of the liver of the lamb at birth. Concomitantly, there were significant increases in 20:4 (n-6) concentrations and reductions in the concentrations of 20:3 (n-9). In addition, the concentration of 18:2 (n-6) in the colostrum of the ewes was increased some eightfold thereby providing a secondary source of this essential fatty acid to the lamb. A marked decrease in erythrocyte fragility was observed in lambs whose ewes had received the "protected" polyunsaturated fatty acid diet. These findings are discussed in relation to the known metabolic roles of essential fatty acids in the animal body, with particular reference to the essential fatty acid status of the lamb at birth.


Active transport of thiamine by freshly isolated rat hepatocytes. Rat hepatocytes were freshly prepared from adult animals using the collagenase-perfusion technique. The hepatic transport of thiamine was studied in isolated liver cells. The process was found to be saturable with an apparent Kt of 0.31 mM and a V max of 0.7 mumoles/ml intracellular fluid/5 minutes. However, at higher substrate concentrations, the process proceeded in a linear fashion. Both pyrithiamine and oxythiamine were inhibitory on the hepatic uptake of thiamine, the latter showed much weaker activity than the former. The system required the presence of sodium ions and was sensitive to ouabain. Anaerobic condition and metabolic inhibitors, e.g., 2,4-dinitrophenol, cyanide, and iodoacetate suppressed the uptake rate of thiamine. Addition of ethanol in the incubation medium also caused significant reduction of thiamine uptake. Efflux studies indicated that a portion of intracellular thiamine is readily available for exodus. Chromatographic analyses showed that thiamine was only slightly metabolically altered during the transport process. It is suggested that thiamine is transported into isolated hepatic cells by an active, sodium-dependent process.


Effects of riboflavin deficiency on the lipids of rat liver mitochondria and microsomes. Weanling male rats were fed a riboflavin deficient diet for 5 weeks and enlargement of the liver mitochondria and discontinuity of the outer membrane were observed. The content of phospholipids was slightly increased in the deficient mitochondria, and decrease in phosphatidylcholine and increase in phosphatidylethanolamine were shown respectively. In comparison with the mitochondria, the content and distribution of phospholipids in microsomes were not affected. The fatty acid composition of phosphatidylcholine in mitochondria and microsomes was remarkably altered by the deficiency, and increases in palmitic and linoleic acids and decrease in arachidonic acid were demonstrated. The incorporation of 32P into diphosphatidylglycerol in mitochondria was reduced by the deficiency. The incorporation into other phospholipids was not significantly altered, whereas the incorporation into the subspecies of phosphatidylcholine was variously affected. By the intraperitoneal injection of riboflavin to the deficient rats, normalization of the mitochondrial size and fatty acid composition of liver mitochondrial lipids was observed. However, decreased incorporation of 32P into diphosphatidylglycerol in mitochondria was not recovered COMPLETELY AT 40 Hours after the injection, and in the mitochondrial lipids linoleic acid was higher and arachidonic acid was lower than respective controls at 60 hours.


Studies on the factors influencing the hydrogen peroxide hemolysis test. There seems to be a greater variation between laboratories in hydrogen peroxide hemolysis techniques than in other clinical laboratory procedures. In this report, factors influencing hemolytic values were examined and the effect of the combination of these factors on the results was analysed statistically by using the orthogonal array. Factors influencing hemolysis induced by hydrogen peroxide were as follows; the concentration of hydrogen peroxide, temperature in the peroxide reagent when added to the red cell suspension, the red cell concentration in the cell suspension and the addition of charcoal to the reaction mixture. However, addition of charcoal may not be essential to stabilize the hemolytic values and other factors such as keeping blood for 4 hours at room temperature before testing, the difference between investigators, a reaction time 2 or 3 hours and the technique of adding peroxide reagent to the cell suspension, had little effect on hemolysis. The most important factor was the temperature in hydrogen peroxide solution. The estimated hemolytic values by the orthogonal array linearly correlated with the plasma tocopherol levels.


Relationship between gamma-aminobutyric acid metabolism and antivitamin B6-induced convulsions. The correlation between the gamma-aminobutyric acid (GABA) metabolism and convulsions by some vitamin B6 antagonists, DL-penicillamine (PeA), hydrazine (Hyd), thiosemicarbazide (TSC) were investigated. Glutamic acid decarboxylase (GAD) and gamma-aminobutyric acid transaminase (GABA-T) activities were inhibited during convulsions by three antagonists, and GABA content was not changed by PeA, increased by Hyd and decreased by TSC in mice whole brain. In subcellular fractions of brain, GAD activity was inhibited and GABA content decreased in synaptosomes during convulsions by the above three drugs. Aminooxyacetic acid (AOAA), a potent GABA-elevating agent, showed an anticonvulsant property against convulsions by TSC for several hours after the injection of AOAA, but lost this property 16hr after treatment. During the convulsions by TSC 16hr after the AOAA-pretreatment, the GABA content in synaptosomes was less than that from the group treated with AOAA alone, though its GABA level was higher than the normal level. From the above results, the GABA content and GAD activity in synaptosomes might be deeply associated with convulsions by B6 antagonists.


Comparative susceptibility to amylases of starch granules of several single endosperm mutants representative of floury-opaque, starch-deficient, and modified starch types and their double-mutant combinations with opaque-2 in four inbred lines of maize. Starch granules were prepared from kernels of eight single endosperm mutants, brittle-1, (bt1), brittle-2 (bt2), floury-1, floury-2, soft starch, opaque-1 (o1), shrunken-2 (sh2), and sugary-2 (su2), and their double-mutant combinations with opaque-2 (o2) of four inbred lines of maize (Zea mays L.), B37, C103, Oh43 and W64A. We compared the susceptibility of various starch granules to Rhizopus glucoamylase and pancreatin. Starch granules of the su2 and su2o2 mutants were digested by amylases much faster than those of the normal counterparts. Starch granules of the bt1, bt2, o1 and sh2 mutants tended to be digested by amylases faster than those of normal maize. Starch granules of double-mutant combinations with the o2 gene were, in general, digested to an extent very comparable to their respective non-opaque single mutant counterparts in each of their four inbred backgrounds. We followed the relative digestion of starch granules by using scanning electron microscopy. Starch granules of endosperm mutants susceptible to amylases showed numerous pin holes on the surface layer and the pores penetrated into the inner layers of the granules during the attack by amylases. In some of the granules the inner portion, which appeared terraced or step-shaped, could be seen. This may be indicative of layered internal structures of the granules.


Dietary cholesterol influences fasting serum free amino acids in rats fed diets containing different sugars. Fasting serum aminograms were studied in rats fed a commercial stock diet or purified diets supplemented with or without 1% cholesterol and 0.25% sodium cholate. Sucrose, glucose or fructose served as a carbohydrate source for each purified diet. Accompanied by marked rises in serum cholesterol, the serum amino acid profile of rats fed glucose or fructose diets was modified significantly by dietary cholesterol. On feeding a glucose diet, dietary cholesterol caused decreases in Trp, Thr and Tyr and increase in Pro and Met. However, the concentration of total essential amino acids remained unchanged. Feeding a fructose diet resulted in a significant reduction of the amino acid level in comparison with that observed with glucose. This decrease was routinely compensated by the inclusion of cholesterol in the diet with the concentration of a number of amino acids being increased. Only Trp was decreased by this dietary manipulation. The serum aminogram of rats fed either a commercial stock diet or a sucrose diet was inconsiderably modified by dietary cholesterol. These data denote that dietary cholesterol influences the metabolic process of amino acids and that the response to cholesterol is modified by the carbohydrate source of the diet.


The preplacement medical evaluation of hospital personnel. Preemployment physical examinations necessarily focus on problems bearing on an individual's ability to safely perform in his work setting. A review of 3,599 examinations of prospective hospital workers reveals that the most frequent observations of significance involved susceptibility to communicable disease--specifically, inadequate diphtheria immunization in 52% and inadequate rubella antibodies in 16% of those tested--and the potential for disease transmission, including tuberculin reactivity in 14% and intestinal protozoa in 13 prospective food handlers. Other findings included 14 individuals with positive serological tests for syphilis, but only two of whom required anti-luetic therapy. An additional 14 were found to be hepatitis B surface antigen carriers. Only 2.3% of those examined were found to exhibit diastolic hypertension at the time of their examination, possibly because of the relative youth of the group studied.


Chromosome investigations on workers exposed to styrene/polystyrene. Chromosomes investigations were undertaken on 12 employees with three to 34 years possible exposure to styrene in order to determine if there was a possibility of chromosome impairment. The data did not reveal any significant differences between persons exposed to styrene and members of the control group. The concentrations of styrene at the workplace were far below the MAC value and also the mandelic acid content of the urine was far below the norm.


Increased lead absorption in children of workers in a lead storage battery plant. Exposure to toxic materials originating in the workplace has been recognized in families of workers in several industries. However, to date this problem has not been studied in the storage battery production industry. Twenty-seven one- to six-year-old children of 22 workers at a storage battery plant at high risk of exposure to lead oxide were compared with 32 one- to six-year-old children in 22 neighborhood control families for evidence of increased lead absorption. Workers' children had significantly higher blood lead (p less than 0.001) and erythrocyte protoporphyrin (p less than 0.003) values than control children. Household exposures to lead in paint and water were similar for the two groups, but workers' homes had significantly higher concentrations of lead in dust (p less than 0.001). It is postulated that lead is brought home on the skin, hair and clothing of the workers and that their children are ingesting or inhaling the lead in household dust. This is the first report of increased lead absorption in children of workers in this industry.


Magnetite pneumoconiosis.

Magnetite pneumoconiosis. A subject is described who was exposed to fine particles of magnetite (Fe3O4). His chest radiograph demonstrated the presence of multiple small rounded opacities; however, his lung function remained unaffected.


Movement-selective mechanisms in human vision sensitive to high spatial frequencies. Evidence for motion-selective mechanisms sensitive to high spatial frequencies (e.g., 15 c/deg) was obtained via direction-specific adaptation and measurements of the threshold ratios for moving and counterphase flickering gratings.


The unspecified role of cones and rods in grating detection: a theoretical note. Detection and resolution thresholds for two 50 ms square-wave gratings (1.5 and 6.7 cps) were determined as a function of background luminance. Results indicated differential cone and rod involvement in resolution of the two targets at low background levels. Implications for other studies employing similar stimuli in terms of retinal inhomogeneity were suggested.


Spectral response of the human eye.

Spectral response of the human eye. A thermal activation energy of 1.5 eV for the activation of rhodopsin is needed to reduce the rate of thermal activations below the rate of optical activation at the luminance of the absolute threshold. The thermal energy of 1.5 eV is not inconsistent with the optical threshold energy of about 1.8 eV.


A novel rapid scanning microspectrophotometer and its use in measuring rhodopsin photoproduct pathways and kinetics in frog retinas. A novel rapid scanning microspectrophotometer is described which utilizes a cathode ray tube as a measuring light source. Spectral scanning is accomplished electronically with a sampling time of 600 mus for each waveband. The cathode ray tube emission is chopped electronically into two separate beams, 180 degrees out of phase, resulting in a dual-beam configuration. A lock-in amplifier functions as a coherent detector to recover separately the signals from the two beams. The instrument generates separate voltage outputs, one proportional to the transmittance of a single sample and the other to the difference between two samples. A computer calculates both absorption and difference spectra directly from voltage measurements. A demonstration of the instrument's use to study kinetics of visual pigment photoproducts is presented. Two models of photoproduct sequence and kinetics were examined to determine which better represents the experimental data. The experiments show that environmental factors, such as pH, metabolic and respiratory state, interact in complex ways to determine the pathways and kinetics of photoproducts of rhodopsin in intact vertebrate eyes.


Luminance-brightness comparisons of LED alpha-numeric sources at suprathreshold levels. An experiment was conducted in which observers adjusted the luminance of  2856 K white comparison source to appear equally as bright as a red, yellow, or yellowish-green LED alpha-numeric source. Although the present CIE photometric system predicts brightness-luminance ratios of 1.0, the ratios obtained in this experiment, for the mean observer, were 1.13 for the yellowish-green, 1.28 for the yellow, and 2.54 for the red. These results demonstrate that there can be substantial differences between photometric luminance based on the V(lambda) function and brightness as perceived visually.


Use of the waveguide parameter V to determine the difference in the index of refraction between the rat rod outer segment and the interstitial matrix. The difference in the index of refraction between the receptor outer segment and the interstitial matrix has been determined to be 0.06 by making use of the waveguide properties of rat rod photoreceptors. This was done by determining the cutoff wavelength for the transition between the bilobe or annulus modal patterns and the single-lobe H E 11 modal pattern, and by measuring the diameter of the transmitting cell. These measurements were made on bleached receptors. Enoch, Scandrett, and Tobey had previously shown that bleaching only slightly alters the outer-segment index of refraction and diameter in the frog. Assuming interspecies transfer, and using Sidman's prior estimate of bleached rat rod outer-segment index of refraction, then the index of refraction of the interstitial matrix of the albino rat is estimated to be 1.3476. This estimate was made using two different media in contact with the retinal preparation, a miscible mountant tissue culture medium believed to be compatible with the rat retina, and an encapsulating nonmiscible silicone oil which allows only ocular media from the same eye in contact with the outer segments.


The corneal polarization cross.

The corneal polarization cross. The corneal polarization cross can be seen at the human cornea using crossed polaroids. The optical mechanisms for this phenomena include rotation and retardation of the light at the surface of the cornea due to the corneal curvature, as well as retardation due to an average circumferential orientation of the birefringent corneal collagen.


Luminance and opponent color contributions to visual detection and to temporal and spatial integration: comment. King-Smith and Carden have postulated a "luminance system" or achromatic channel in the visual system which has a temporal response better than  the other channels, and which also responds to high spatial frequencies better than the other channels. All evidence, both psychophysical and electrophysiological, indicates that these properties are contradictory.


Helping the parents of children with malignancy. The parents of children with cancer experience a uniquely stressful situation that poses a profound threat to the equilibrium of the entire family. As health professionals, we recognize, with the parents, that this is a crisis and an ordeal, but that it can be resolved; we mobilize them to broaden their understanding of their situation and find constructive solutions to the problems that develop. For our patients with good prognoses, we hope to ensure a future for the whole family that is as healthy emotionally as it is physically; for those who become fatally ill, we hope to help their families support them and emerge from this experience with new adaptive abilities. Our group program is open to parents of patients who have various diagnoses, with a format that is unstructured and focuses on problems of daily living as well as the possibility of death. We have found that this is an effective approach for the delivery of mental health services to our growing population, early in the course of treatment, and for the identification of families that need ongoing counseling. Our experience with the large number of parents who have participated demonstrates that the program provides the support that the parents need and that it plays a vital role in resolving the problems that confront them as they struggle with this very difficult experience.


Bronchial hyperreactivity in cystic fibrosis and asthma. We studied 113 patients with CF and compared their responsiveness to inhaled methacholine to that of 50 asthmatic children and 26 normal children. Positive responses to MCh occurred in 51% of the patients with CF and 98% of those with asthma. There was a significant correlation between a positive response to MCh in CF and poor pulmonary function. There was no relationship between allergic rhinitis or positive allergen skin tests and a positive MCh response. Dose-response curves and time course to MCh challenge differed in CF and asthma. Bronchial hyperreactivity is thus common in CF but is different from that in asthma.


Bayley-Pinneau, Roche-Wainer-Thissen, and Tanner height predictions in normal children and in patients with various pathologic conditions. Bayley-Pinneau, Roche-Wainer-Thissen, and Tanner height predictions at various chronologic ages were compared with final adult height in 56 normal subjects and in 34 patients with abnormal growth pattern (11 with familial tall stature, 7 with idiopathic precicious puberty, 6 with Turner syndrome, and 10 with primordial small stature or Silver-Russell syndrome). The two recent methods (Roche-Wainer-Thissen and Tanner) gave very accurate results and were superior to the Bayley-Pinneau method in normal subjects and in patients with familial tall stature. However, they overestimated adult height grossly in precocious puberty and moderately in Turner syndrome and in primordial small stature. It is concluded that calculations based on coefficients and regression equations obtained from normal children (as in the Roche-Wainer-Thissen and Tanner methods) can only be used in normal children or in patients with normal growth potential under adequate treatment. Calculations based on percentages of adult height (as in the Bayley-Pinneau method) are preferable in conditions in which the growth potential in relation to bone maturation is inherently reduced and cannot be corrected by treatment.


Acute renal failure in infants and children: outcome of 53 patients requiring hemodialysis treatment. Fifty-three children, ages one day to 15 years, were treated with hemodialysis for acute renal failure between 1968 and 1977. Twenty-three had acute tubular necrosis. Nine had ATN associated with catastrophic medical illnesses; all died. Fourteen had ATN following major surgical procedures; ten died. Thirty had ARF due to primary nephrologic disorders; 27 survived. Thus it was not the ARF per se but the underlying and concomitant disorders which had the major influences on survival. As prognostic indications of survival in patients with postoperative ATN cannot be clearly defined, these patients almost always deserve aggressive management, including dialysis therapy. Patients with ATN associated with severe medical illness often have fatal underlying conditions which cannot be influenced by presently available technologies.


Focal glomerulosclerosis in children: correlation of histology with prognosis. The clinical and pathologic data of 32 nephrotic children diagnosed as having focal glomerulosclerosis were retrospectively analyzed to determine what factors were responsible for progression to renal failure in 12 of these children. The patients were classified into three groups based on the histologic findings in their initial renal biopsies: Group I (n = 19) had a combination of global and segmental lesions; Group II (n = 8) had only globally sclerotic or obsolescent glomeruli; and Group III (n = 5) had only segmentally sclerosed glomeruli. Ten of the 12 patients with end-stage renal failure came from Group I and two from Group II. The median period from recognition of symptoms to renal failure was four years. Clinical features were of little prognostic value. However, comparison of the histopathologic data of the ten patients in Group I with renal failure (Group Ia) and Groups Ib, II, and III revealed that the risk of progressing to renal failure was significantly higher in patients having greater than 20% of their glomeruli involved by both global and segmental lesions (P = 0.005). Furthermore, patients in Group I and III had a lower probability of responding to treatment as compared to patients in Group II (P less than 0.025).


A simple method for measuring separate glomerular filtration rate using a single injection of 99mTc-DTPA and the scintillation camera. A new technique for measuring glomerular filtration in each kidney has been developed using a scintillation camera and the 99mTc-DTPA complex. The technique has been applied to 101 children with various uropathies. Correlations with the total and separate creatinine clearance, the HgCl2 uptake test, and the maximal urinary concentration have been good. The reproducibility of the method has been satisfactory in a small number of patients. The test is particularly adapted to children because of its simplicity, reduced trauma, short duration (20 minutes) and low-radiation dose. In contrast to some other radioisotope techniques, it can be carried out in infants.


Hyperglycemia associated with the therapeutic use of L-asparaginase: possible role of insulin receptors. A study of four children, two with acute lymphocytic leukemia and two with non-Hodgkin lymphoma, treated for three days with L-asparaginase, suggests that insulin receptors may be involved in the hyperglycemia associated with the use of L-asparaginase. Comparison of insulin receptors of circulating monocytes revealed a marked decrease in their number after treatment. This might also explain the lack of response to insulin treatment in one of the patients with symptomatic hyperglycemia.


Carpal-tarsal osteolysis.

Carpal-tarsal osteolysis. A mother-daughter kindred with carpal-tarsal osteolysis and evidence of renal involvement is presented. There was a similarity between the early clinical manifestations of this syndrome and those of rheumatoid arthritis. The distinction made in previous reports between the dominantly inherited form and the sporadic form with nephropathy is questioned.


The association of chromosome 3 duplication and the Cornelia de Lange syndrome. Two unrelated children with features of the Cornelia de Lange syndrome, including mental retardation, growth retardation, glaucoma, and a similar facies, had duplications corresponding to bands q25 leads to q29 of chromosome 3. These patients were compared to others with duplications of chromosome 3 and to a large series of patients with Cornelia de Lange syndrome. Diseases that sometimes involve characteristic chromosomal changes but have normal chromosomes in other instances are discussed.


Sodium balance in very low-birth-weight infants. Sodium balance was studied in 17 consecutively admitted neonates weighing less than 1,200 gm at birth. Infants whose gestation was less than or equal to 30 weeks were sicker and were in markedly negative sodium balance on day 3 (-9.25 mEq/kg day), despite a high sodium intake (7.22 mEq/kg/day). This negative balance was the result of a high fractional sodium excretion and resulted in hyponatremia in six (50%) of the patients. By day 8 these immature infants were in positive sodium balance, although fractional sodium excretion and daily sodium requirements remained high. More mature infants (greater than 30 weeks gestational age) were in positive sodium balance on both days 3 and 8. Creatinine clearance did not differ significantly between groups on either day 3 or 8 but increased within each group during the study period. These data suggest that the daily sodium requirement of immature sick infants may be much higher than was previously suggested.


Long-term colchicine therapy of familial Mediterranean fever. Familial Mediterranean fever is a disorder characterized by recurrent fever and polyserositis. Continuous prophylactic colchicine therapy has been effective in suppressing attacks in affected adults. From 30 children with FMF, 14 were selected for colchicine therapy. Eight children continued prophylactic colchicine therapy for 29 months (mean) and experienced a marked decrease in the frequency of attacks. Six other children did not comply with the treatment regimen. Although no deleterious side effects were noted, the safety of long-term colchicine administration in childhood is unknown.


The risks of small intestinal transplantation for the recipient: experimental results in young minipigs. In a previous series of 36 small intestinal transplants in young minipigs, a monitoring system, based on a special transplantation model, clinical and histologic criteria has been worked out. The spontaneous evolution was observed and no attempt was made to save the animal when complications on the graft occurred. Most of the animals died. In the present series of 32 small intestinal transplants the monitoring system and explanation criteria are now tested. Twenty-three primarily successfully transplanted animals had complications of the graft, i.e., hemorrhagic infarction, deficient arterial circulation, or rejection. Only 1 of the 23 died before explanation could be done. Twenty-two were explanted according to the previously established criteria. Two died of complications not directly related to the graft, the other 20 animals survived for 1-5 mo. All 22 explanted grafts showed irreversible damages both macroscopically and on histologic examination. The present series confirms that early recognition of complications of the graft and timely removal with survival of the recipient animal is possible. This could be an important step forward to the clinical application of small intestinal transplantation in man.


Antenatal intestinal adaptation with experimental jejunoileal atresia. Jejunoileal atresia without significant shortening of total intestinal length, was created in five date-bred Dorset ewes by devascularizing a 15 cm segment of fetal intestine at 90-100 days gestation. Light microscopy showed mucosal hypertrophy distal to the atresia with almost total obliteration of the lumen by tail, feathery-shaped villi similar to those observed in newborns with jejunal atresia. Scanning electron microscopic views of the proximal mucosa showed the villi to be thicker, more segmented and tended to congregate compared to normal villi and those distal to the atresia. No evidence of microvillus, intracellular or capillary membrane change or injury could be identified to explain the functional secretory state.


Neonatal meconium obstruction in the ileum without mucoviscidosis. Two newborns with intestinal obstruction of the terminal ileum without mucoviscidosis were cured by Gastrografin enema. A further case of a premature infant showed, at autopsy, a large intestine containing normal meconium, but an ileum which was plugged with tenacious meconium containing PAS-positive and alcian blue-negative mucus. Our 3 cases were similar to cases of "meconium disease" reported by Rickham and of "meconium plug in the small intestine" reported by Emery. The primary cause of this disease lies in the large amount of PAS-positive mucus secreted from goblet cells of the ileal mucosa and the hypertrophy of lymph nodules in the ileal submucosa.


Low molecular weight dextran and reexploration in the management of ischemic midgut-volvulus. Administration of low molecular weight dextran and reexploration in 36-48 hr, is recommended in cases of ischemic midgut volvulus. Five cases are presented utilizing this technique and many centimeters of small bowel were saved that would have been resected at time of the original exploration. One patient died as a result of sepsis due to central intravenous nutrition: the others are alive and well.


Pseudomembranous colitis associated with antibiotic therapy in a child: report of a case and review of the literature. A case of pseudomembranous colitis is presented in a child with multiple medical problems who received intraoperative antibiotics. Despite aggressive medical management and operative intervention, the patient died. All patients who develop diarrhea after receiving antibiotics should be suspected of having pseudomembranous colitis. Sigmoidoscopy and hyaque enema should be performed to confirm the diagnosis and all antibiotics should be discontinued. Aggressive medical management consisting of intravenous fluids, albumin, lactinex granules, fecal enemas, and cholestyramine should be instituted. Surgery should be considered only in the face of perforation, toxic megacolon, peritonitis, and failure of medical management with disease limited to the colon.


Pancreaticocolonic fistula: a complication of pancreatic pseudocysts in childhood. Two patients had an unusual complication of pancreatitis: pancreaticocolonic fistula, frequently associated with life-threatening gastrointestinal hemorrhage and sepsis. To avoid these complications, early diagnosis is important, but it may be difficult. Treatment consists of external drainage of the pancreatic pseudocyst or abscess and colonic diversion.


Demonstration of rectourinary fistulas by pressure gastrografin enema. Rectourethral and vesical fistulas in patients with high anorectal atresia can be demonstrated by using a pressure Gastrografin enema through the distal colostomy opening. A technique is described where contrast medium is forced by gentle pressure, filling the fistula and the urethra. This picture is of great value during the repair of these anomalies.