Biomedical subjects
Z Ackerman
Publications and source records attributed to Z Ackerman.
Pulmonary valve stenosis and hemophilia A. Report of three cases and discussion of a possible genetic linkage.
We treated three unrelated patients with hemophilia A and congenital pulmonary valve stenosis. In two patients, the occurrence of the cardiac malformation was sporadic and in one familial. The coexistence of hemophilia A and pulmonary valve stenosis might suggest a genetic linkage for both disorders. Review of the literature supports the hypothesis that the inheritance of pulmonary valve stenosis is dominant and X-linked.
Skin discoloration in chronic idiopathic thrombocytopenic purpura: detection of local iron deposition by X-ray spectrometry.
A forty-six-year-old woman with a 32 years' history of idiopathic thrombocytopenic purpura presented with black-brown discoloration of her lower limbs. Noninvasive diagnostic X-ray spectrometry (DXS) for determination of trace elements in external tissues revealed striking local iron deposition confined to the skin area with the pigmentary changes. Iron deposition in the skin was probably due to recurrent local purpura caused by longstanding bleeding disorder. Hence DXS proved useful in the detection of local iron load and eliminated the need for skin biopsy in this patient with bleeding tendency.
Transbronchial biopsies in Wegener's granulomatosis.
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[Methods of individualizing phenytoin dosage].
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Amiodarone-associated hypothyroidism--a possible cause of digoxin intoxication.
The problem of a possible interaction between amiodarone and digoxin is still unsettled. We have recently treated two patients with digoxin intoxication who had received amiodarone for eight and 36 months respectively. Both developed extreme bradycardia requiring temporary pacemakers. The presence of hypothyroidism was confirmed in both cases by laboratory data. Judging by present knowledge concerning the interaction between amiodarone, thyroid function, and digoxin, it is suggested that digoxin intoxication was not the result of its direct interaction with amiodarone. The possibility that amiodarone-induced hypothyroidism precipitated digoxin intoxication seems to be more plausible. Prevention of digitalis toxicity in amiodarone-treated patients would therefore require monitoring of thyroid function every three to six months. Frequent monitoring of digitalis blood levels is also indicated in patients with amiodarone associated hypothyroidism. Early detection of hypothyroidism and digitalis intoxication is necessary in view of the severity of the course of the disease.
Bioavailability of phenytoin: comparison between three preparations.
Crossover studies, following single oral doses given to 19 healthy volunteers were conducted to compare the bioavailability of three phenytoin preparations: DANTOIN and IDANTOIN (Teva, Israel) and EPANUTIN (Parke-Davis, USA). The first has recently replaced the second as the only preparation available in Israel for alleged better bioavailability. The area under the serum concentration time curve (AUCo----48), however, did not significantly differ between preparations, indicating equivalent absorption of the three preparations. These results suggest that no overall adjustments are required when switching from one preparation to another.
Quinidine-induced vasculitis.
Four patients developed nonthrombocytopenic purpura two to three weeks after initiation of quinidine therapy. The skin lesions disappeared and did not recur after cessation of quinidine therapy. Histologic examination revealed leukocytoclastic vasculitis with deposition of C3, IgA, and/or IgM in the small dermal vessels. Since quinidine purpura is usually associated with thrombocytopenia, the possibility of leukocytoclastic vasculitis as an additional cause of purpura is stressed.
Immunoglobulin E response during measles.
Serum IgE levels were determined in 41 patients with measles infection. 23 patients (56%) had an increased level (greater than 20%) during the acute phase of the disease in comparison with the recovery phase. 14 showed no change, and decreased levels were observed in 4 patients only. In contrast, both IgG and IgA levels decreased or remained unchanged in the majority of the patients during disease activity. The changes in the pattern of serum immunoglobulins during measles might be attributed to selective depletion of T-cell subpopulations.
Extent of measles hepatitis in various ages.
To assess extent of hepatic involvement in measles, we evaluated prospectively 144 patients (ages 0.2 to 43 years) during an outbreak. Liver function parameters (AST, ALT, serum gamma-glutamyl transpeptidase and bilirubin) were determined on presentation and at 2 and 4 weeks. The study comprised 52 pediatric (less than or equal to 14 years) and 92 adult patients. Liver dysfunction was evident quite often (56 to 66%) in adult patients. However, in the pediatric age group, these abnormalities were less frequent and less extensive. Moreover, a significant correlation was noted between age and each of the following parameters: AST (r = 0.61), ALT (r = 0.56) and serum gamma-glutamyl transpeptidase (r = 0.39). In all subjects all parameters normalized after 2 to 4 weeks. The data presented suggest that hepatic dysfunction in measles is probably not rare and is more frequent and more extensive in adults. However, these abnormalities seem to be subclinical, self-limited and probably with no long-term sequelae.
Quantitative P-31 MR spectroscopy of the liver in alcoholic cirrhosis.
To determine the cause of reduced urea synthesis in cirrhosis, absolute concentrations of phosphorus metabolites in the human liver have been measured in vivo with magnetic resonance (MR) spectroscopy. One-dimensional chemical shift imaging was used to obtain phosphorus-31 spectra from five healthy volunteers and five patients with alcoholic cirrhosis. A reference standard included in all studies enabled the calculation of absolute concentrations. In contrast to hepatic metabolite ratios, absolute concentrations reveal that in the cirrhotic patients, concentrations of adenosine triphosphate (ATP) were significantly reduced and concentrations of phosphomonoesters slightly reduced. Intracellular pH was unchanged. Histologic evidence suggests that the amount of ATP per cell was unchanged and could not account for the reduced urea production. Instead, urea synthesis depends on the functional liver cell mass, which was reduced by 31% in alcoholic cirrhosis. Quantitative in vivo P-31 MR spectroscopy of liver has potential clinical applications and can supplement the more generally used P-31 metabolite ratios.
Cerebral toxoplasmosis complicating bone marrow transplantation.
Cerebral toxoplasmosis occurred in a 34-year-old patient with chronic myelogenous leukemia following bone marrow transplantation. The clinical picture included headaches, nuchal rigidity, and right-sided hemiparesis during a course of disseminated cutaneous herpes zoster. The diagnosis of toxoplasmosis was based on serologic evidence and typical computed tomography scan of the brain as well as clinical improvement following specific anti-Toxoplasma treatment. To the best of our knowledge, this is the first report of cerebral toxoplasmosis in Israel.