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Biomedical subjects

H Zinman

Publications and source records attributed to H Zinman.

8 recordsLinked to original sources
PubMed1991

Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency in muscle: a new cause for recurrent myoglobinuria and encephalopathy.

We report on a 16-year-old girl with short-chain L-3-hydroxyacyl-coenzyme A (CoA) dehydrogenase deficiency resulting in juvenile-onset recurrent myoglobinuria, hypoketotic hypoglycemic encephalopathy, and hypertrophic/dilatative cardiomyopathy. Urinary organic acids showed traces of 3-hydroxy-dodecanedioic acids and small amounts of suberic, sebacic, and adipic acids. There was a marked decrease in L-3-hydroxyacyl-CoA dehydrogenase activity in muscle with acetoacetyl-CoA as substrate (2.48 mumol/min/gm; normal = 6.90 +/- 1.80 mumol/min/gm of tissue; n = 11), contrasting with normal L-3-hydroxyacyl-CoA dehydrogenase activity with 3-ketooctanoyl-CoA and 3-ketopalmitoyl-CoA as substrates. Short-chain L-3-hydroxyacyl-CoA dehydrogenase activity was normal in fibroblasts, suggesting a tissue-specific defect.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1987

Idiopathic transient osteoporosis of the hip.

We have reviewed 11 patients with idiopathic transient osteoporosis of the hip; the six who were women all developed the condition during pregnancy. Both simultaneous and sequential bilateral involvement were seen, but biochemical studies were consistently normal and one synovial biopsy showed only non-specific inflammation. Radioisotope bone scans and CT scans were useful to aid diagnosis. Treatment by limiting weight-bearing relieved symptoms, and spontaneous resolution was paralleled by radiographic remineralisation, usually within a few months. One patient developed a stress fracture of the hip and other areas of transient osteoporosis. A hip involved by the condition should be protected from overloading until bone density has recovered.

Adult↗
PubMed1981

Fracture of the medial sesamoid bone of the hallux.

Fracture of the sesamoid bones of the hallux is rare. A case of fracture of the medial sesamoid bone is presented. The X-ray finding of an irregular line dividing the medial sesamoid bone together with typical local symptoms and signs established the diagnosis in a 17-year-old female athlete. Nonunion of the fractured sesamoid was successfully treated by extraction of the sesamoid fragments. Complete relief of pain was followed by the patients' return to full athletic activity. The diagnostic criteria and indications for operation are discussed briefly.

Adolescent↗
PubMed1980

Responses of Y1 adrenocortical tumor cells to o-nitrophenyl sulfenyl ACTH.

NPS(o-nitrophenyl sulfenyl)-ACTH stimulated steroidogenesis in Y1 cells to the same maximum level as did ACTH, but with a 60-fold lower potency than the native hormone. NPS-ACTH also stimulated the extracellular accumulation of cAMP in Y1 cells with lower potency than the unmodified hormone. The amount of cAMP accumulated in the presence of NPS-ACTH approached 75% of the maximum with ACTH. In Y1 plasma membranes, NPS-ACTH was a partial agonist of adenylate cyclase activity, witn an efficacy dependent upon the type of guanyl nucleotide present. The steroidogenic responses of two Y1(Kin) mutants and two Y1(Cyc) mutants to NPS-ACTH paralleled their responses to ACTH and reflected closely their defects in cAMP-dependent protein kinase and ACTH-sensitive adenylate cyclase activity. These data imply an obligatory role for adenylate cyclase and cAMP-dependent protein kinase activities in stimulation of steroidogenesis in Y1 cells by NPA-ACTH.

Adenylyl Cyclases↗