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Biomedical subjects

J B Alperin

Publications and source records attributed to J B Alperin.

At least 37 records · Page 2Linked to original sources
PubMed1981

Juvenile Niemann-Pick disease with vertical supranuclear ophthalmoplegia. Two cases reports and review of the literature.

Examination of two siblings who had histories of progressive decline in speech, intelligence, and coordination disclosed vertical supranuclear ophthalmoplegia, hepatosplenomegaly, and signs of diffuse CNS dysfunction. Niemann-Pick "foam cells" were found in the bone marrow of both patients. The features of these cases correlate in appearance and clinical findings with those of 21 other previously reported cases, which are reviewed in this article. Knowledge of the clinical manifestations of this particular variety of Niemann-Pick disease should aid in its earlier diagnosis.

Adult↗
PubMed1980

Central nervous system recurrence ten years after remission of acute lymphoblastic leukemia.

Therapy with vincristine and prednisone achieved a complete remission in a 10-year-old Caucasian girl with acute lymphoblastic leukemia. Maintenance therapy with 6-mercaptopurine was then administered for seven years, but prophylactic intrathecal chemotherapy and central nervous system (CNS) radiation were never given the patient. Ten years from the time of diagnosis, she presented with signs of increased intracranial pressure and a diagnosis of CNS leukemia was confirmed. At this time, however, no leukemia was detected on examination of her blood and bone marrow. She was successfully treated with intrathecal methotrexate and radiation therapy. After a careful search of the literature it has been concluded that this is the first reported case of such a long remission of acute lymphoblastic leukemia with subsequent relapse.

Antineoplastic Agents↗
PubMed1980

Anaphylactic reactions following infusion of factor VIII in a patient with classic hemophilia.

A 28 year old white man with hemophilia A had, for several years, been successfully maintained on a home care program utilizing self-administration of factor VIII concentrates, but one such infusion resulted in severe anaphylaxis. An immunoglobulin E (IgE)-mediated hypersensitivity response was demonstrated by (1) the release of histamine from the patient's basophils in vitro upon challenge with several different lots of factor VIII concentrate; (2) immediate cutaneous response to commercial factor VIII, factor IX and cryoprecipitate; and (3) measurement of IgE antibodies against a commercial factor VIII preparation. A subsequent life-threatening hemorrhage required cautious infusion of another commercial lot of factor VIII concentrate. Despite pretreatment with antiallergic drugs and attempted desensitization, a moderately severe anaphylactic reaction was observed.

Adult↗
PubMed1980

Serum ferritin assay and bone-marrow iron stores in patients on maintenance hemodialysis.

Fifty-five patients on maintenance hemodialysis underwent bone-marrow aspirations for evaluation of iron stores that were to be compared to concomitant measurements of hematocrit, red blood cell volume, serum iron concentration, total iron binding capacity, transferrin saturation, and serum ferritin concentration. In 42 patients (76.4%), the bone marrow iron stores were found to be absent or deficient. Mean hematocrit for the total group was 26.4%, and red blood cell volume measurement showed a mean value of 41.1% of predicted normal. Results also indicated that serum ferritin was the best predictor of iron storage levels, with diagnostic thresholds of 80 to 350 ng/ml derived from statistical analysis of the data. Other hematologic parameters studied had significantly less correlation with bone-marrow iron stores.

Adult↗
PubMed1980

Human immune interferon: induction in lymphoid cells by a calcium ionophore.

Human lymphocyte cultures produced interferon after treatment with the calcium ionophore A23187, but not after treatment with potassium ionophore nonactin. Interferon production was detectable between 3 and 6 h after ionophore treatment and reached the maximum level between 12 and 24 h. Ionophore-induced interferon appeared to be immune interferon on the basis of acid lability, lack of neutralization by antibody to leukocyte interferon, slow kinetics of activation of the antiviral state, and molecular characterization by isoelectrofocusing.

Adult↗
PubMed1980

Quinidine-induced thrombocytopenia with pulmonary hemorrhage.

Examination of an elderly man with quinidine sulfate-induced thrombocytopenia complicated by pulmonary hemorrhage failed to identify any underlying pulmonary disease contributing to the bleeding All bleeding ceased, and pulmonary infiltrates disappeared after the platelet count returned to normal. Special studies indicated a high titer quinidine-dependent IgG antibody level in the patient's serum and a strongly positive quinidine patch test result on his forearm. The pathogenesis of pulmonary hemorrhage is considered in view of these studies.

Aged↗
PubMed1979

111In chloride bone marrow scintigraphy and ferrokinetic studies in a case of sickle cell anemia with transient erythroid aplasia.

A patient with sickle cell anemia was hospitalized because of an acute erythropoietic aplastic crisis. 111In chloride bone marrow scintigraphy performed during the aplastic crisis showed markedly impaired uptake of the radionuclide and simultaneous 59Fe clearance T 1/2 studies were prolonged (420.9 min). Following recovery from the aplastic crisis 111In scintigraphy revealed an expanded marrow and the radioiron clearance T 1/2 was accelerated (20.9 min). The clinical course, hemograms and bone marrow aspirations correlated with marrow scintigraphy. 111In appears to be a valuable marrow scanning agent for assessing the presence of intramedullary erythropoietic activity.

Adult↗
PubMed1979

A comparison of hemoglobin A1c in human and baboon blood.

Cation exchange chromatography was performed on hemolysates prepared from human and baboon (Papio anubis) blood. In humans with diabetes mellitus and in baboons with pancreatectomy induced diabetes mellitus there were significant increases in Hbs A1, A1c, and A1a +A1b. The quantity of Hb A1 and Hb A1c in nondiabetic baboons was approximately one-half the quantity of Hb A1 and Hb A1c in nondiabetic humans. These differences may be explained by the differences in survival time between human and baboon red cells.

Animals↗
PubMed1978

Viral hepatitis in pregnancy with disseminated intravascular coagulation and hypoglycemia.

A case of a 26-year-old woman who presented at 38 weeks of gestation with severe hepatitis B complicated by disseminated intravascular coagulation (DIC) and hypoglycemia is reported. The clinical features of the illness suggested acute fatty liver of pregnancy. Cesarean section was followed by resolution of the coagulopathy and the hypoglycemia. Both mother and infant survived and remain well. The diagnosis of hepatitis B was confirmed by a transiently positive hepatitis B surface antigen and percutaneous liver biopsy. This case emphasizes the difficulty in distinguishing acute viral hepatitis from acute fatty liver of pregnancy. In addition, the predominant features of DIC and hypoglycemia in our case are reported.

Adult↗
PubMed1977

Hemoglobin A2 levels in health and various hematologic disorders.

Using a method involving elution of hemoglobin bands from cellulose acetate strips following electrophoresis of hemolysates, hemoglobin A2 (Ab A2) was quantitated in bloods from 300 healthy individuals and 904 patients. The percentage of Hb A2 was elevated in beta-thalassemia heterozygotes and some patients who had megaloblastic anemia. In the latter, the highest Hb A2 levels were observed in patients with the most severe anemia. Low Hb A2 percentages were found in iron-deficiency anemia, hereditary persistance of fetal hemoglobin, and Hb H disease. In iron-deficiency anemia, the lowest levels of Hb A2 were observed in association with the most severe anemia. Iron and folate deficiency each suppressed Hb A2 levels in beta-thalassemia heterozygotes; however, vitamin B12 deficiency did not alter the percentage of Hb A2 in thalassemia. Malignant tumors, renal and hepatic insufficiency, chronic infections and inflammation, hemolytic disease, lead poisoning, aplastic anemia, leukemia, myelofibrosis, and hypothyroidism did not change Hb A2 levels. The pathogenesis of altered Hb A2 levels and their clinical significance in various diseases are discussed.

Adolescent↗
PubMed1976

The effect of cytosine arabinoside on platelet aggregation.

Platelet aggregation induced by ADP, collagen, epinephrine, and thrombin was studied in patients with acute leukemia in complete remission, before and after a continuous 5-day intravenous infusion of cytosine arabinoside at a dose of 100 mg/m2 per day. Aggregation was also measured in normal subjects with cytosine arabinoside added in vitro. Cytosine arabinoside had no significant effect on platelet aggregation after either in vitro or in vivo administration.

Acute Disease↗