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Biomedical subjects

A Silverman

Publications and source records attributed to A Silverman.

At least 91 records · Page 5Linked to original sources
PubMed1985

Frequency and clinical progression of the vitamin E deficiency neurologic disorder in children with prolonged neonatal cholestasis.

To determine the frequency of biochemical vitamin E deficiency and of the clinical signs of the vitamin E deficiency neurologic syndrome in children with prolonged neonatal cholestatic disorders, we studied 46 children (aged 1 month to 17.0 years) with chronic forms of intrahepatic neonatal cholestasis and 47 children (aged 4 months to 8.0 years) with extrahepatic biliary atresia. Based on serum vitamin E concentrations and the ratios of serum vitamin E concentration to total serum lipid concentration, 64% of the intrahepatic and 77% of the extrahepatic cholestasis groups were vitamin E deficient. Prior to age 1 year, neurologic function was normal in all children. Between ages 1 and 3 years, neurologic abnormalities were present in approximately 50% of the vitamin E-deficient children; after age 3 years, neurologic abnormalities were present in all vitamin E-deficient children. Areflexia was the first abnormality to develop between ages 1 and 4 years; truncal and limb ataxia, peripheral neuropathy, and ophthalmoplegia developed between ages 3 and 6 years. Neurologic dysfunction progressed to a disabling combination of findings by ages 8 to 10 years in the majority of vitamin E-deficient children. Neurologic function was normal in the vitamin E-sufficient children. We conclude that vitamin E status should be evaluated in infants in whom cholestasis is diagnosed, and effective therapy should be initiated to prevent or treat vitamin E deficiency at an early age.

Adolescent↗
PubMed1983

Diagnosis of biliary atresia with radionuclide hepatobiliary imaging.

Sixteen patients with biliary atresia and 11 patients with neonatal hepatitis were studied preoperatively with either Tc-99m-diethyl-IDA or TC-99m-diisopropyl-IDA. Two parameters were evaluated: hepatocyte clearance and time to appearance of radioactivity in the intestine. Two observers, using a visual grading system of 1 to 4, gave the 16 patients with biliary atresia a hepatocyte clearance grade of 1.7 +/- 0.6 (mean +/- SD); intestinal radioactivity was not seen through 24 hours. The hepatocyte clearance grade of the 11 patients with neonatal hepatitis was 2.1 +/- 0.9 (mean +/- SD) (p greater than 0.05); intestinal radioactivity was seen in nine of 11 patients (p less than 0.001). Using both parameters, 91% of the patients were classified correctly, 4% were misclassified, and 6% were classified as indeterminate; sensitivity and specificity for biliary atresia were 97% and 82%, respectively. Radionuclide imaging with the newer technetium-99m-labeled hepatobiliary radiopharmaceuticals appears promising for the noninvasive diagnosis of biliary atresia.

Bile Ducts↗
PubMed1982

Progressive neuromuscular disease in children with chronic cholestasis and vitamin E deficiency: diagnosis and treatment with alpha tocopherol.

We have studied four children (ages 6 to 17 years) with chronic cholestasis who developed a slowly progressive neuromuscular disease characterized by ataxia, dysmetria, areflexia, loss of vibratory sensation, and a variable ophthalmoplegia. Serum vitamin E concentration were low in all patients prior to treatment. Muscle histochemical studies in all four patients showed autofluorescent basophilic esterase and acid phosphatase-positive cytoplasmic inclusions and occasional necrotic fibers. These distinctive muscle changes are similar to those described in vitamin E-deficient animals. Intramuscular injections of alpha tocopherol were required in three patients to achieve normal serum vitamin E values. High-dose oral supplementation was effective in one patient. After normalization of serum vitamin E concentrations for six to 14 months, the neurologic disease has improved in all four patients.

Adolescent↗
PubMed1982

Progressive neuromuscular disease in children with chronic cholestasis and vitamin E deficiency: clinical and muscle biopsy findings and treatment with alpha-tocopherol.

We have studied four children (ages 6 to 17 years) with chronic cholestasis who developed a slowly progressive neuromuscular disease characterized by ataxia, dysmetria, areflexia, loss of vibratory sensation, and a variable ophthalmoplegia. Serum vitamin E concentrations were low in all patients prior to treatment (0.17-2.0 mg/g cholesterol, normal greater than 3 mg/g). Muscle histochemical studies showed prominent yellow autofluorescence, basophilic cytoplasmic inclusions which stain with esterase and acid phosphatase, and occasional necrotic fibers. Ultrastructural findings consisted of increased number and size of membrane-bound dense bodies (lysosomes), membranous whorls, and autophagic vacuoles. Intramuscular injections of all-rac-alpha-tocopherol (0.55-1.42 mg/kg per 24 hours based on individualized pharmacokinetic data) were required in three patients to achieve normal serum vitamin E values. High-dose (32 mg/kg per 24 hours) oral supplementation was effective in one patient. After normalization of serum vitamin E concentrations for 12 to 20 months, the neurologic disease has improved in all four patients.

Adolescent↗
PubMed1979

A computerized three-dimensional treatment planning system utilizing interactive colour graphics.

A new computerized radiation treatment planning system has been developed to aid in three-dimensional treatment planning. Using interactive colour graphics in conjunction with a DPD 11/45 computer, the system can take multiple transverse contours and construct a perspective display of the treatment region showing organ surfaces as well as cross-sectional contours. With interactively selected orientations, the display allows easy perception of the relative positioning of the treatment volume and neighbouring anatomy. For external beam treatment planning, interactive computer simulation is used to select diaphragm sizes which best conform to the target area while avoiding sensitive structures. Dose calculations for the selected beams are carried out on multiple transverse planes. The calculational planes and surfaces are displayed in perspective with radiation dosage displayed in an interactively manipulated colour display. Altogether the system provides an easy assessment of the volume to be irradiated, interactive selection of optimal arrangements of treatment fields and a means for visualizing and evaluating the resulting dose distributions.

Color↗
PubMed1979

Primary pulmonary hypertension presenting as portal hypertension.

A two and a half-year old child is described who presented with signs of portal hypertension (hematemesis, hepatosplenomegaly, ascites). Her subsequent work-up revealed that the "pressure-head" originated within the pulmonary arterial bed. Indeed, severe changes of primary pulmonary hypertension were found at autopsy. What is unique about this case is the absence of cardiopulmonary symptoms prior to the development of suprahepatic portal hypertension. In addition, the pulmonary disease developed in the absence of underlying chronic hepatic disease or extrahepatic portal vein thrombosis which, reportedly, can lead to pulmonary hypertension.

Autopsy↗
PubMed1978

Homotransplantation of the liver in a patient with hepatoma and hereditary tyrosinemia.

A girl with hereditary tyrosinemia, diagnosed at 6 months of age, was treated with a diet restricted in phenylalanine and tyrosine. At 9 1/2 years of age she developed an acutely enlarged liver and spleen, and the diagnosis of hepatocarcinoma was made. The patient received a liver transplant and tyrosine metabolites became normal while she was receiving a regular diet. Three months later, an infected thrombosis of the portal vein caused her death. Liver transplant appears to be an effective method of enzyme replacement in tyrosinemia and should be considered for prevention of hepatoma.

Aminolevulinic Acid↗