Hallucinations and hyperthermia after promethazine ingestion.
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Biomedical subjects
Publications and source records attributed to E Kerem.
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A seven-year-old girl, stung by a scorpion, was hospitalized in a confused state with signs of myocarditis and pulmonary edema. In spite of clinical improvement within 24 h, 14 serial echocardiograms and electrocardiograms performed during a four-month period showed severe changes. There have been no previously published reports of echocardiographic studies showing myocardial changes after a scorpion sting.
Five cases of persistent neonatal hyperinsulinemic hypoglycemia (PNHH) were studied by a combined immunofluorescent and Feulgen technique under a computerized microscope which was used to measure the area and absorbance of beta cell nuclei. An increase of 16% was found in the nuclear area and absorbance of the beta cells in PNHH cases, in comparison with age-matched control cases. A smaller increase (9%) was found in nuclear area and absorbance of non-endocrine ductal cells in PNHH cases. In all PNHH and control cases, higher values of nuclear area and absorbance were found for beta cells in the islets of Langerhans than for nesidioblasts. The increase in average size of beta cell nuclei in PNHH can be used as a morphologic criterion for diagnosis in these cases. The increased absorbance of the nuclei stained with the Feulgen reaction is the morphologic expression of polyploidy, and has a correlation to the metabolic activity of beta cells in this disorder. We conclude that, in contrast to the normal postnatal developmental process of nesidioblastosis, nesidiodysplasia is a well-defined histologic entity, manifested by an increase of beta cell nuclear size and its DNA content.
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A 6-month-old infant presented with failure to thrive, hyperuricaemia and renal insufficiency. The hyperuricaemia was due to uric acid over-production. The level of hypoxanthine-guanine phosphoribosyltransferase (HGPRT) activity was found to be normal. However, a two-fold increase in the Km of the enzyme to hypoxanthine as well as in the Vmax values was observed. It seems therefore, that in cases of uric acid over-production, screening tests of HGPRT activity may be insufficient and additional kinetic properties of the enzyme should be tested.
Noma is a very rare gangrenous infection of the oral cavity usually associated with debilitating diseases, immunosuppression, or malnutrition. Its development in a previously healthy 13-year-old boy with perforated appendicitis is presented. Streptococcus bovis type II and E coli were isolated from the noma and Streptococcus bovis, Bacteroides fragilis, and Bacteroides asacharolyticus from the peritoneal fluid. A transient impaired immune cellular function was found and may have contributed to the development of the noma in this child.
The syndrome of chronic pigmented purpura (CPP) consists of pigmented macular lesions, predominantly involving the lower extremities. An 11-year-old girl was diagnosed initially as suffering from vasculitic purpura, but the clinical course and the skin histology proved to be consistent with CPP. This syndrome should be included in the differential diagnosis of childhood purpura.
Very high serum levels of alkaline phosphatase were found in four children aged 13 to 24 months. No other abnormalities nor explanation for the raised enzyme levels were found. The origins of the elevated enzyme levels were shown by isoenzyme studies to be the liver in one case, the bone in two cases, and undetermined in one case. Serum alkaline phosphatase levels returned to normal after periods of 5 to 20 weeks. Awareness of these benign forms of hyperphosphatasemia will aid the physician in the differential diagnosis of elevated alkaline phosphatase levels.
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A male newborn with partial deletion of the short arm of chromosome 3 is described. The patient shares most of the features with the previously reported cases. In addition, cardiac, skeletal and gastrointestinal anomalies not previously reported are described. These characteristics may help in further delineation of the syndrome.
Pancytopenia after intramuscular iron-dextran treatment occurred in an infant with Down's syndrome. Haematological abnormalities recurred on subsequent challenge. Positive migration inhibiting factor and mast cell degranulation tests support an allergic pathogenesis for the pancytopenia. These side effects have not been reported previously.
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A male infant with multiple congenital anomalies and psychomotor retardation was found to have a translocation resulting in partial trisomy for the distal part of chromosome 3p. An older sister with similar clinical findings had an identical karyotype. Chromosome studies in the phenotypically normal parents revealed a balanced translocation in the mother involving chromosomes 3, 11, and 18. An identical translocation was found in one of the normal children.