Search PubMed⌕ Search

Biomedical subjects

J Herman

Publications and source records attributed to J Herman.

At least 217 records · Page 12Linked to original sources

Association between thyrotoxicosis and thrombocytopenia. A case report and review of the literature.

In a young woman who presented with hyperthyroidism and autoimmune thrombocytopenic purpura, the platelet count returned to normal following successful treatment of the hyperthyroidism. Thromboagglutinins were present, the titer declining as the patient became euthyroid. A survey of the literature revealed 48 reports of hyperthyroidism and thrombocytopenia, these disorders coexisting in 37 patients and there being no apparent cause for the lowered platelet counts in 28 of them. The clinical features and response to treatment in the latter group and in our patient are reviewed, comprising a series of 29. Of 22 patients whose hyperthyroidism was adequately treated, platelet counts returned to normal in 18 (82%). In three patients the purpura remitted despite persistent thrombocytopenia. It is estimated that in 7% of patients with autoimmune thrombocytopenic purpura, thrombocytopenia responds to treatment of an underlying thyrotoxicosis and in many of these patients the thrombocytopenia will prove resistant to other forms of therapy.

Adult↗

Recurrent myoglobinuria and muscle carnitine palmityltransferase deficiency.

A 16-year-old boy with a two-year history of recurrent attacks of myalgia, muscle cramps without weakness, and myoglobinuria was shown to have a deficiency in muscle carnitine palmityltransferase. Serum concentrations of creatinine phosphokinase, serum glutamic oxalacetic transaminase, and aldolase were elevated. An electromyogram was consistent with a nonspecific myopathy as were microscopic and ultrastructural examinations of biopsied muscle. Venous lactic acid response to ischemic exercise was compatible with paroxysmal idiopathic myoglobinuria. Activities of muscle phosphorylase A and B, phosphofructokinase, muscle palmityl CoA synthetase, carnitine, and serum carnitine were normal as was the glycogen content. Activity of muscle carnitine palmityltransferase (2.7 microM/minute/mg protein), as measured by a spectrophotometric method and by radioactive assay, was significantly reduced when compared to normal control subjects (14.5 microM/minute/mg protein) and ischemic control subjects (13.8 microM/minute/mg protein). Muscle carnitine acetyltransferase (13.4 microM/minute/mg protein) was approximately 50% of normal control values (25.5 microM/minute/mg protein). This is the third reported case of myoglobinuria in a patient associated with a deficiency of muscle carnitine palmityltransferase activity.

Acyltransferases↗

Overt hemolysis in patients with glucose-6-phosphate dehydrogenase deficiency: a survey in general practice.

The records of 129 patients with glucose-6-phosphate dehydrogenase deficiency from two general practices were reviewed for overt hemolysis. There were 25 such cases: 14 were diagnosed as favism; 10 were associated with various combinations of drugs, or with fever, infection, acidosis or the neonatal state; and one was of undetermined cause. Over the years 1952-72, these patients had been challenged on 756 occasions with potentially hemolytic drugs. The low incidence of overt hemolysis after antipyretic preparation is noted, as is the danger of nitrofurantoin. The clinical setting was an important determinant of whether hemolysis would occur after drug administration. Fever seemed to be a particularly significant factor. Quantitative determinations of enzyme activity in 18 patients confirmed the reliability of the screening method used. There was no correlation between the enzyme levels and the occurrence of overt hemolysis.

Adolescent↗

Absence of nystagmus during REM sleep in a patient with waking nystagmus and oscillopsia.

Polygraphic recording is presented of the sleep pattern in a young male who developed nystagmus and oscillopsia associated with a remittent CNS demyelinating disease. The vestibular nystagmus observed during wakefulness disappeared during all stages of sleep, including rapid eye movement sleep (REM). Since vestibular nystagmus experimentally induced in wakefulness is also absent during all phases of sleep, these findings suggest that during sleep similar suppressive mechanisms are operative.

Adult↗