Advances in the diagnosis of renal angiomyolipoma.
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Biomedical subjects
Publications and source records attributed to M Khalil.
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Five patients with retinal macroaneurysms are described. The aneurysms may be an incidental finding or they may cause visual loss from retinal, preretinal and vitreous hemorrhages or from macular edema or exudation. The prognosis is good in those cases presenting with hemorrhage but is poorer in those with macular problems. Acquired aneurysms are found in older patients who are hypertensive or have vascular disease. We believe that retinal emboli play an important part in their development.
Twenty-two cases with communication of an artery and the portal vein or one of its tributaries are discussed. Four conditions in which relatively significant arterio-portal shunts may exist can be differentiated: (1) angiodysplasias or arteriovenous malformations, (2) cirrhosis of the liver and inflammatory lesions, (3) traumatic and postoperative lesions, and (4) benign and malignant tumors. The significance of the portal vein's early opacification during arteriographic examinations of the abdominal organs is discussed, and the findings are compared to those reported in the literature.
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Two hundred angiocardiograms were analysed. Twenty-two patients had mural intraventricular thrombi. The radiological appearances are described. Systemic emboli occurred in four patients. Elucidation of the source of emboli following the demonstration of peripheral embolic occlusions by means of an angiocardiogram of the left ventricle is not difficult and should be performed as an additional examination.
The angiographic and morphologic findings of angiomyolipomas of the kidney are described in three patients. These benign tumours contain three types of vessel in addition to fat and smooth muscle. One vessel type is thick-walled with fibrosis of its walls and no internal elastic lamina. The second type consists of sinuses with considerable variations in calibre. The third type resembles capillaries and is found in the muscular parts of the tumour. Because of the abnormal structure of their walls, these vessels are easily damaged and may cause massive bleeding.
Two hundred angio-cardiograms of patients with confirmed myodardial infarcts or angina pectoris were analysed. Patients with rheumatic heart disease were not included. In each patient the left ventricule and coronary vessels had been demonstrated and pressure measurements taken. In 8.5% there was prolapse of a mitral valve leaflet. In two thirds of these produced mitral insufficiency.
Sphingomyelinase was purified about 1700-fold from human placenta. The major steps in the procedure included chromatography on Concanavalin A-Sepharose, Sepharose 6B, and carboxymethyl-Sepharose (CM-Sepharose). The final preparation was stable for at least 3 months when stored at 4 degrees C. The enzyme was found to be heterogeneous on CM-Sepharose and isoelectric focusing. Triton X-100 which was present in most buffers used during the purification appears to be partially responsible for the heterogeneity. When Triton X-100 is removed by treatment with Bio Beads, heterogeneity was reduced. However, removal of the detergent also leads to loss of enzyme activity which could not be restored by readdition of Triton X-100. The data suggest that sphingomyelinase has a high hydrophobic character and that both its stability and electrofocusing behaviour are influenced by interaction with the nonionic detergent.
A TOTAL of 204 cases with acute viral hepatitis were studied: 93 bilharzial and 111 non-bilharzial. This study is concerned with the 106 cases showing positive HBS Ag test. Follow up of the cases after discharge from the Fever Hospital was carried out by repeated clinical and laboratory examinations 3 montly in the first year and 6 monthly subsequently. The bilharzial and non-bilharzial patients were found to be equally susceptible to HBS Ag positive hepatitis (p=0.25). However, antigenaemia tended to last longer as a whole in the bilharzial patients (p=0. 005) and was not affected by successful specific antibilharzial treatment. It seems that bilharzial infection predisposes to "retention" of HBs antigen, after subsidance of the acute attack, thus increasing the volume of reservoir for HB virus in our rural community.
We present a case of iris-nevus or Cogan-Reese syndrome with its characteristic pathological findings. Although a rare entity, it should be considered in the differential diagnosis of heterochromia associated with elevated intra-ocular pressure. To avoid unnecessary enucleation, it should not be confused with diffuse iris melanoma.
The value of early films during a urogram in the investigation of arterial hypertension was investigated (402 urograms on unselected hypertensives, with arteriographic examination in 80). The diagnostic accuracy in cases of proven unilateral reno-vascular disease was 58%, signs suggestive of renal artery stenosis were found in 6.5% of the total material. Possible causes of the low degree of accuracy are discussed.
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Twenty cases of thalassemia major and 20 children with glucose-6-phosphate dehydrogenase deficiency were studied for the protein content of their red cell membrane as well as for its fractions, compared to 20 normal controls. The results showed that the total membrane proteins per red cell and the various fraction percentages in both conditions were not significantly different from normal controls. Also the acrylamide gel electrophoretic studies of the red cell membrane proteins gave band patterns identical with those of normal controls. These results are discussed.
Serum protein components were evaluated by the specific radial immunodiffusion technique in a number of protein energy malnourished infants and young children, as well as in a group of parallel controls. Results show a drop in both serum prealbumin and albumin in concordance with the severity of the condition in both KWO and marasmus. Serum level of varies as 1-acidic glycoprotein was elevated, being more marked in KWO than in marasmus. Serum oc1-antitrypsin, haemopexin, ceruloplasmin and transferrin were all reduced but to a varying extent in both KWO and marasmus. The levels of serum IgA and IgM were above normal in all disease entities except for a slight decrease of IgA in 2nd grade marasmus. On the other hand, serum IgG was reduced in the majority of cases. The ratio of serum cerutoplasmin to transferrin in KWO was found to be of diagnostic value in different grades of the disease.
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Two observations of mammographic findings in axillary vein thrombosis showed the main similarities of alterations in the soft parts of the arm and/or breast (vein dilation, thickening of the skin, edema). Radiological findings can simulate the possibility of a tumor or even a total carcinoma.
Sphingomyelinase was obtained in excellent yield from liver and brain by homogenization with 0.05 M citrate-phosphate buffer, pH 4.5, containing 0.25% Triton-X-100 (v/v) followed by dialysis of the supernatant fluids against 1% glycine. Total recovery of enzyme was slightly less with tissue from Niemann-Pick disease compared with control tissue. Isoelectric focusing of liver and brain extracts was successfully used to resolve several species of sphingomyelinase. Three (I-III) of the five species were partially characterized. Enzyme I (pI 4.6) had a pH optimum of 4.8-5.0 in acetate buffer and a Km value of 0.026 mM. Both sphingomyelinases I and II were the major enzymes, whereas III, IV, and V were found at lower levels. Of the two major species in normal liver and brain (I and II), species I alone persisted in liver from the two cases of type C, while species III, IV, and V were present. In brain, only species II was decreased but the resolution of the brain enzymes was less satisfactory.
Sphingomyelinase was effectively extracted cultured fibroblasts homogenized in 1% glycine. The average specific activity was 24.6 nmol substrate hydrolyzed per hr per mg protein. Cultured cells from two cases of Niemann-Pick disease type A and one case of type B had markedly reduced enzyme activity, whereas, in type E cells, total activity was twice normal. Sphingomyelinase was resolved by isoelectric focusing into three peaks of activity (I-II), where I and II were the major forms. Species I had a pI of 4.6, II a pI of 4.8, and III a pI of 5.0. All three peaks, although markedly reduced, were present in cell extracts from both types A and B. All species of enzyme in B cells were found at higher levels than in A cells. Cultured cells from a case of type E contained sphingomyelinase I at a level several fold higher than normal, species II was absent, whereas III was found at near normal levels.