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Biomedical subjects

S Epstein

Publications and source records attributed to S Epstein.

At least 19 recordsLinked to original sources

Endocrine function in sclerosteosis.

Sclerosteosis is a rare autosomal recessive condition which is characterized by excessive skeletal overgrowth, distortion of the facies, cranial nerve abnormalities and raised intracranial pressure. Syndactyly and digital malformation are associated features. Radiological examination reveals thickened sclerotic bone maximally involving the skull, including the pituitary fossa. Sclerosis and hyperostosis are present throughout the skeleton. Biochemical and endocrine tests were carred out on 3 patients with sclerosteosis in an attempt to detect any dysfunction of calcium regulation of the pituitary. Results revealed no abnormality of basal parathyroid or calcitonin secretion. Histological examination revealed quantitatively increased bone resorption in comparison with normal subjects, although the pattern resembled osteosclerosis. Regulation of growth hormone, adrenocorticotrophin, gonadotrophin and thyrotrophin function were intact. We conclude that pituitary function and calcium 'homeostasis' are normal in this disorder.

Adult

Menstrual disturbances in chronic renal failure.

Twelve female patients undergoing intermittent hemodialysis (HD) and 5 females posttransplantation (PT) were studied. All the HD patients had menstrual disturbances and 5 had galactorrhea. The mean basal LH level was significantly elevated (p less than .05) in patients on HD compared to normal controls, but the mean LH response to luteinizing hormone releasing hormone (LRH) was not significantly different from the control group. Mean basal FSH and the FSH response to LRH was normal. In the PT pateints the LH response to LRH was significantly greater at 120 min when compared to normal females. In the HD group the serum 17B estradiol, progesterone and testosterone levels were significantly lower than in the controls but in the PT group only testosterone levels were significantly lower. These results differ from those previously found in uremic males. Elevated prolactin levels were found in the patients on hemodialysis and correlated well with the presence of galactorrhea. These was no correlation between the elevated prolactin levels and amenorrhea in the patients on hemodialysis but one PT patient with amenorrhea had elevated prolactin levels.

Adolescent

Natural healing processes of the mind: I. Acute schizophrenic disorganization.

It is proposed that there are three fundamental adaptive systems that have developed in the course of evolution. One of these, learning, is the only one that is widely recognized. The other two consist of regulation of the energetic aspect of stimulation, and integration of the data of experience into an organized conceptual system. Corresponding to each of the three basic adaptive systems are three basic types of disorder, namely maladaptive learning, which is by far the most common source of behavioral problems; overstimulation, as in the traumatic neurosis; and collapse of the individual's conceptual integrative system, as in acute schizophrenic disorganization. Just as relearning is a natural adaptive process for correcting problems produced by learning, there are inherent processes for correcting the disorders produced by overstimulation and by a maladaptive integrative system. The present article is concerned with the natural process for correcting a poorly organized conceptual system. A second article (Epstein 1978) will be devoted to the natural process of coping with the energic aspects of stimulation. An acute disorganizational state can provide an opportunity for a new and more effective conceptual system to develop. As a desperate remedy, it is often unsuccessful. Nevertheless, an orderly process can be discerned in such states that can enhance the likelihood that new, and dissociated old, material will be assimilated into a new organization. If all goes well, the new organization will be more resilient than the old one; it will be better able to accomplish the functions of an implicit theory of reality, which are to integrate the data of experience, to maintain a favorable pleasure-pain balance, and to maintain self-esteem.

Acute Disease

N-alkaline phosphatase: a potential disease marker for lymphoproliferative disorders.

A characteristic alkaline phosphatase (orthophosphoric monoester hydrolase, alkaline pH optimum, EC 3.1.3.1) was detected in the sera of most patients with infectious mononucleosis, acute and chronic lymphatic leukaemia, non-Hodgkin's lymphoma, Burkitt's lymphoma and nasopharyngeal carcinoma. The enzyme was also present in the sera of nine out of 26 patients with cancer of the cervix. N-APase in these cases counted 30-100% of the total alkaline phosphatase activity. N-APase was absent from the sera of healthy individuals and of patients with acute and chronic granulocytic leukaemia, breast cancer, colon cancer, rheumatoid arthritis, ulcerative colitis, systemic lupus erythematosis, hepatitis and obstructive jaundice. Only three of 22 patients with Hodgkin's disease showed n-apase activity in the serum. In infectious mononucleosis the presence of N-APase activity was well correlated with the clinical course. In 13 cases studied, the clinical improvement was associated with the decrease or disappearance of N-APase activity. N-APase activity could not be detected in white cells of acute myeloid leukaemic patients, nor in the cells of myeloid blastic crisis of chronic granulocytic leukaemia. It was present in the cells of lymphoid blastic crisis of chronic granulocytic leukaemia.

Adult

Mechanism of carbon isotope fractionation associated with lipid synthesis.

The low carbon-13/carbon-12 ratio of lipids is shown to result from isotopic fractionation during the oxidation of pyruvate to acetyl coenzyme A. In vitro analysis of the kinetic isotope effects of this reaction indicates that there will be a large, temperature-dependent difference in the carbon-13/carbon-12 ratio between the methyl and carbonyl carbon atoms of acetyl coenzyme A and between those carbon atoms of lipid components which derive from them.

Acetates

Paget's disease of bone (osteitis deformans) treated with human synthetic calcitonin.

Thirteen patients with symptoms of active Paget's disease of bone, confirmed biochemically and radiologically, were studied. Each patient received a 6 months' course of therapy with human synthetic calcitonin (HCT), starting with 1 mg daily subcutaneously or intramuscularly for the first month and thereafter 3 times per week for 5 months. Twelve patients completed the course of therapy. Ten of the 12 patients derived moderate to great symptomatic improvement from their treatment. The improvement usually became evident 6 weeks after the start of treatment and this improvement persisted in 4 patients from 6 months to 1 year after treatment was stopped. The plasma alkaline phosphatase had fallen from the initial values by a mean of 26% after 6 weeks and 31% after 6 months of treatment. The urinary hydroxyproline likewise had fallen by 42% at 6 weeks and 46% at 6 months. Side-effects were generally mild and self-limiting, with flushing being the most common. It is concluded that human calcitonin is beneficial in the treatment of Paget's disease of bone and may prevent the problems of antibody resistance and allergic phenomena encountered with other preparations of calcitonin.

Aged

The effects of TRH on prolactin, plasma renin activity, water and electrolyte excretion in normal males.

The effect of TRH induced secretion of TSH and prolactin (hPrl) on plasma renin activity (PRA), water and electrolyte excretion, was studied in 7 normal males before and after an intravenous injection of 2 ml normal saline or 200 microgram TRH. Plasma hPrl and TSH rose significantly (p less than 0.01) in all 7 subjects after TRH but not after saline injection. No significant differences in the hourly excretion of sodium, potassium and free water clearance were noted before and after either saline or TRH injection. Mean PRA values of the 7 subjects were similar after either the 2 ml saline of TRH injection. Our results indicate that despite a correlation between basal hPrl and sodium excretion as well as free water clearance, acute TRH induced elevation of hPrl is not associated with changes of urinary sodium and potassium excretion, free water clearance and PRA in normal males. These findings provide some evidence against a direct osmoregulatory role of hPrl in man.

Adult

Increasing growth with raised circulating somatomedin but normal immunoassayable growth hormone.

Two patients are described with elevation of circulating somatomedin A concentration but normal growth hormone levels. One,a young male, appeared clinically acromegalic; the other, a young female, was tall with kyphoscoliosis, and her appearance resembled Marfan's syndrome. In both patients plasma growth hormone concentration was suppressible by hyperglycaemia. It is suggested that their clinical syndromes resulted from excessive somatomedin activity.

Acromegaly

Metabolic clearance and plasma half disappearance time of exogenous gonadotropin releasing hormone in normal subjects and in patients with liver disease and chronic renal failure.

The metabolic clearance rate (MCR) and half disappearance time (t 1/2) of gonadotropin releasing hormone (GnRH) has been measured during and after cessation of constant infusion of exogenous GnRH. Studies were performed on normal subjects and patients with severe renal and liver disease. GnRH was quantified by a sensitive and specific radioimmunoassay which does not measure GnRH fragments. The MCR of GnRH in normal subjects was 1640+/-59.7 ml/min (23.7+/-1.8 ml/min/kg), similar to values found in 4 patients with liver disease. However in chronic renal failure an MCR of only 631+/-62 ml/min (9.1+/-0.7 ml/min/kg) was obtained. The t1/2 of GnRH after infusion was linear for 8-10 min, after which a much slower component was observed. The t1/2 of the first component ranged from 5.5 to 8 min in normal subjects, 6.5-8 min in patients with liver disease but prolonged (12-16.5 min) in patients with renal failure. It would appear that GnRH is cleared rapidly in normal subjects, that moderate liver dysfunction does not alter this, but that impaired renal function significantly prolongs the t1/2 and lowers the MCR. The kidney might be an important catabolic organ for infused GnRH; alternatively, uremia might impair catabolism non-specifically.

Adult