תוצאת חיפוש

Effect of Chlorocresol VS Caffeine on Muscle Contracture in Malignant Hyperthermia Susceptible Patients

R. Ben-Abraham, R.M. Krivosic-Horber, G. Haudcoeur, A. Perel, P.J. Adnet

Dept. of Anesthesiology and Intensive Care, Chaim Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University; and Dept. d'Anesthesie-Reanimation Chirurgicale, Laboratoire de Pharmacologie Hospitaliere, et Service des Urgence, Centre Hospitalier Universitaire, Lille Cedex, France

The phenotype of susceptibility to malignant hyperthermia (MHS); can only be detected reliably by the in vitro caffeine-halothane contracture test (CHCT). Enhanced sensitivity of the calcium-induced calcium release mechanism is responsible for the exaggerated contracture response of skeletal muscle fibers from MHS patients to halothane and caffeine. Chlorocresol was demonstrated to be a potent activator of Ca++release from skeletal muscle sarcoplasmic reticulum. This effect is probably mediated through action on a ryanodine sensitive Ca++ release channel known to be more sensitive in MH. We studied the effect of chloroscresol on the mechanical contracture response of skeletal muscle from patients presenting for the in vitro CHCT. Chlorocresol induces contracture response in a concentration 1/200 of that of caffeine in muscle strips from MH patients. By adding chlorocresol to the protocol of the CHCT, there is clearer discrimination between the responses of MH patients and normal subjects can be achieved. 

Pulmonary Alveolar Micro-Lithiasis Presenting With Prolonged Cough

I. Barshack, M. Krupsky, M. Perelman, J. Kopolovic

Institute of Pathology and Dept. of Pulmonary Diseases, Chaim Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

A 40-year-old man had been followed in the pulmonary clinic for prolonged cough. Chest X-ray showed bilateral diffuse interstitial infiltrates with accentuation toward the bases. CT-scan demonstrated a fine diffuse reticulonodular pattern. Transbronchial lung biopsy showed pulmonary alveolar microlithiasis, a rare disease characterized by the presence of concentric calcifications within the pulmonary alveoli. This is the second case of the disease reported in Israel.

Angioedema Caused by Splenectomy with Malignant Lymphoma Foll-Owed by Multiple Myeloma 7 Years Later

A. Shahar, R. Sharon, M. Lorber, S. Pollack

Institute of Allergy, Clinical Immunology and AIDS and Institute of Hematology, Rambam Medical Center and B. Rappaport Technion- Faculty of Medicine, Haifa

Acquired C1-inhibitor (C1-INH) deficiency has been reported in patients with immunoglobulin abnormalities and lymphoproliferative disorders, and angioedema has appeared simultaneously with the lymphoproliferative disease. We present a 50-year-old woman with acquired C1-INH deficiency and angioedema which preceded by 7 years the diagnosis of malignant mantle cell lymphoma. During the interval she was treated with Danazole and there were no attacks of angioedema. When routine follow-up bone marrow aspiration revealed infiltration of nonspecified lymphoma cells, exploratory laparotomy and splenectomy were performed. A month later Danazol was stopped, C1-INH levels returned to normal and there were no attacks of angioedema. Mantle cell lymphoma consisting of lymphocytes with cytoplasmic IgM-lambda was diagnosed in the excised spleen but chemotherapy was not initiated. 6 months later, a second lymphoproliferative disorder, multiple myeloma IgA kappa, was diagnosed.

Red Cell Na+/H+ Exchange and Role of Protein Kinase C in its Stimu-Lation in Diabetes Mellitus, Essential Hypertension and Nephropathy

Wladimir Koren, Robert Koldanov, Edna Peleg, Eva Izsak, Meir Berezin, Talma Rosenthal

Dept. of Medicine C, Hypertension Unit and Endocrinology Institute, Chaim Sheba Medical Center, Tel Hashomer

Na+/H+ exchange (NHE) was measured as maximal initial velocity of pH-dependent H+ efflux from red cells into an alkaline medium containing Na+ in patients with insulin-dependent or noninsulin-dependent diabetes, with and without hypertension and in normoglycemic, essential hypertensives and normal controls (50 subjects in each subgroup). Maximal velocities of NHE were found in microalbuminuric patients in all subgroups, and NHE correlated with the rate of microalbuminuria (r=0.61, p=0.02). Daily insulin requirements were greater in those with elevated NHE (84±8 vs 42±4 U/day). There was no correlation between NHE and levels of plasma glucose, HbA1 and plasma aldosterone and lipid profile and PRA. NHE was correlated with plasma prolactin (r=0.51, p=0.02) and PTH r=0.24, p=0.05). In uremic patients, NHE was inversively correlated with creatinine clearance (r=-0.48, p=0.03). Since calphostin C, a selective inhibitor of protein kinase C, lowered increased NHE in vitro, the protein kinase C-dependent pathway of the exchanger regulation was concluded to be responsible for NHE activation in diabetes mellitus and essential hypertension.