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Biomedical subjects

A Adam

Publications and source records attributed to A Adam.

At least 325 records · Page 18Linked to original sources

X-linked dyskeratosis congenita with pancytopenia.

Two maternal male cousins in a Jewish Iraqi kindred were affected with dyskeratosis congenita and had a megaloblastic bone marrow. One cousin had pancytopenia and the other had thrombocytopenia. The kindred displays a deficiency of glucose-6-phosphate dehydrogenase (G6PD) and a beta-thalassemia trait. The following genetic "markers" of the X chromosome were studied: G6PD, the X-linked blood groups Xg, and color vision. Linkage analysis indicated that dyskeratosis, G6PD, and Xg are far apart on the X chromosome. Chromosomal studies showed a 46XY karyotype in both cases; however, nonspecific numerical aberrations and structural abnormalities were found in the first and in the second case, polyploidy was seen in four of 60 cells. The proband's cultured fibroblasts did not show increased susceptibility to malignant transformation by simian virus 40, an oncogenic virus.

Adolescent↗

[Electric burns in the operating room. Apropos of 4 cases].

Four cases of patients suffering from peroperative skin burns at electrocardioscope electrode sites are reported. They were the result of incorrect function of the cutting diathermy, (error of internal or external connection). Use of the electrocardioscope with a "floating input" protects the patient against this type of electrical accident.

Adult↗

Heterogeneity of Wilson's disease in Israel.

In a survey in Israel of 50 patients with Wilson's disease, it was found that this disease occurred in all ethnic groups. In the Arab patients there was a significantly early age of onset and the disease followed a more severe course than that in the Jewish patients. The overall sex ratio of patients was nearly 1:1, and genetic analysis of 20 families confirmed an autosomal recessive mode of inheritance. The very similar age of onset and type of disease within sibships and the varying ages of onset noted between the Arab and Jewish patients suggest that the disease is genetically heterogeneous.

Adolescent↗

Kinetics of absorption, equilibration (or distribution), and excretion of orally and intraperitoneally administered cholesterol in the rat.

Resorption and metabolism of cholesterol administered intraperitoneally and per os have been described in the rat utilizing a technique of double isotopic labeling. Depending upon the route of administration, these parameter vary to a large degree. Alimentary cholesterol is progressively resorbed over a period of 10 hr, while the maximal blood level of cholesterol is attained after the 6th hr following intraperitoneal injection. On the other hand, cholesterol administered per os is more rapidly utilized in biliary acid synthesis than cholesterol administered intraperitoneally. In the range of concentrations utilized in this work (10-300 mug and 0.2-300 mug, respectively, administered to the rat, orally and intraperitoneally), the rate of cholesterol resorption remained constant.

Administration, Oral↗

A controlled surgical approach to annulo-aortic ectasia.

Annulo-aortic ectasia has attracted much surgical attention in the last 20 years. Replacement of the aortic valve and ascending aorta from the valve ring to just proximal to the innominate artery eliminates most, if not all, the pathologically involved tissue. Composite valve-Dacron tube grafting, plus elective saphenous vein grafts from the coronary orifices to the Dacron tube or distal aortic wall, provide a safe systematic approach to this entity. A review of surgical techniques and a description of a successful case employing this method are presented.

Adult↗