תוצאת חיפוש

Oncogenetic Counseling and Genetic Testing of Those at High Risk for Breast and Ovarian Cancer

Livia Theodor, Ronit Shiri-Sverdlov, Galit Hirsch Yechezkel, Revital Bruchim Bar-Sade, Eva Gak, Irit Friedman, Anna Kruglikova, Gilad Ben-Baruch, Shulamit Risel, Moshe Z. Papa, Boleslav Goldman, Eitan Friedman

Oncogenetics Unit, Dept. of Clinical Epidemiology, Institute of Genetics, and Gynecology, Oncology, and Surgical Depts., Chaim Sheba Medical Center, Tel Hashomer

There is inherited predisposition to breast and ovarian cancer in 5-10% of all women with these diseases. Germline mutations in BRCA1 and BRCA2 presumably account for most of the genetically susceptible individuals. We summarize 2 years of experience in counseling and testing for inherited predisposition to these cancers.

597 women (from 320 families) have been evaluated since August 1995. 242 were evaluated for inherited predisposition to breast and ovarian cancer. One-third had clear-cut evidence of familial background. 74 families were of Ashkenazi origin; the age range of breast cancer was 30-35, of ovarian cancer 40-45. In 80% of families other cancers were also noted in first degree family members, including lung, colon, and prostate cancer and leukemia.

Genetic testing revealed that 45% of affected and 25% of unaffected women were carriers of a mutation in BRCA1 or BRCA2: 67/90 185delAG (BRCA1), 12/90 6174delT (BRCA2), and 4/90 of 5382insC (BRCA1). In addition, a novel mutation in exon 11 of BRCA1 was detected, carried by 7/90 women. The experience gained in oncogenetic counseling and genetic testing for inherited cancer predisposition will eventually enable determining an optimal, rational therapeutic regimen in carriers of mutations.

Cyclic Vomiting Syndrome in Children

G. Ben-Amitay, Y. Nevo, D. Lieberman, R. Mester, S. Harel

Ness Ziona Mental Health Center, Institute for Child Development and Pediatric Neurology Unit, Tel Aviv-Sourasky Medical Center, and Tel Aviv University Medical School

Cyclic vomiting syndrome in children is a manifestation of various etiologies, including gastroenterological and renal disorders, central and autonomic nervous system abnormalities, as well as metabolic and endocrine dysfunction. Frequently no organic cause is found. Personality profiles of children with cyclic vomiting reveal perfectionism, competitiveness, and aggressive behavior. Vomiting attacks have been induced by anxiety and excitement in patients with cyclic vomiting. We describe an 8-year-old girl with cyclic vomiting, frequently associated with occipital headaches, photophobia or dizziness. Psychiatric evaluation indicated a generalized anxiety disorder.

Changes in Structure and Process Components of Trauma Care in Emergedepartments

Rephael Joseph Heruti, Ron Ben-Abraham, Yanir Abramovitch, Michael Stein, Joshua Shemer, Baruch Marganit

Trauma Control, Israeli Center for Disease Control (ICDC), Israel Ministry of Health and Sackler School of Medicine, Tel Aviv University

In recent years there have been tremendous efforts to improve primary trauma care. The Ministry of Health and other authorities have invested in new trauma facilities in various hospitals. A nationwide survey with regard to structure and function of emergency departments was carried out. Compared to a similar survey conducted in 1992, significant progress in quality and quantity of equipment at various emergency departments was demonstrated. However, there are still differences between various hospitals. A drive to standardize trauma care will undoubtedly contribute to improvement in care of the injured.

Abdominal Compartment Syndrome

D. Rosin, M. Ben Haim, A. Yudich, A. Ayalon

Dept. of General Surgery and Transplantation, Chaim Sheba Medical Center, Tel Hashomer and SacSchool of Medicine, Tel Aviv University

Abdominal compartment syndrome refers to a complex of negative effects of intra-abdominal hypertension. Its most common cause is complicated abdominal trauma. The syndrome includes mainly hemodynamic and respiratory manifestations but may involve other systems as well. It may present as a life-threatening emergency in the multi-trauma patient. Awareness of the syndrome may enable the surgeon to take preventive measures or to diagnose it earlier and to treat it effectively. We describe a 21-year old man who developed this syndrome after multiple gunshot wounds, with severe liver injury. After 2 operations the typical manifestations of the syndrome were diagnosed. He was re-operated to release intra-abdominal hypertension and then slowly recovered.

Israel's ATLS Program: Summary and Outlook

Ron Ben Abraham, Michael Stein, Yoram Kluger, Gideon Paret, Avraham Rivkind, Joshua Shemer

Medical Dept., Israel Defense Forces and Israel Trauma Society

In recent years a vigorous effort has been made to improve quality of primary trauma care in Israel. Advanced trauma life support courses (ATLS) were given to physicians throughout the country, regardless of their specialties. In 7 years 4229 physicians participated in 202 courses with an average success rate of 76%. In the future, issues such as mandatory vs. voluntary training, language barriers, and continuous decline of knowledge should be addressed if momentum is to be maintained.

Skeletonized Internal Mammary Arteries for Coronary Bypass Grafting

Jacob Gurevitch, Yosef Paz, Menachem Matsa, Amir Kramer, Dimitri Pevni, Oren Lev-Ran, H. Locker, Raphael Mohr

Dept. of Thoracic and Cardiovascular Surgery, Sourasky-Tel Aviv Medical Center

The skeletonized internal mammary artery (IMA) is longer, and its immediate spontaneous blood flow is greater than that of the pedicled IMA, thus providing increased versatility for complete, arterial myocardial revascularization without the use of saphenous vein grafts. From April 1996 to May 1997, 583 patients underwent coronary artery bypass grafting here and in 415 (71%) complete arterial revascularization was achieved using bilateral skeletonized IMA. The right gastroepiploic artery was used in 57 (13%); there were 329 males (79%) and 86 women (21%); average age was 64 (30-87) and 175 (36%) were older than 70; 131 (32%) were diabetics. Average number of grafts was 3.2 (range 2-6 grafts). At 30 days, 5 (1.2%) had died and there had been 6 perioperative infarcts (1.4%), 5 CVA's (1.2%), and 6 had sternal wound infections (1.4%). Up to 1-12 months of follow-up was achieved in 409 (99%). Late mortality was 1.4% (of which 3 were noncardiac). 394 (97%) were angina-free at latest follow-up. We conclude that arterial revascularization using bilateral skeletonized IMA is safe, as postoperative morbidity and mortality are low, even in old and diabetic patients.

Effect of Alpha-Alpha Esterol on Plasma Cholesterol and Antioxidants

Y. Levy, E. Paster, G. Dankner, A. Ben-Amotz, J.G. Brook

Lipid Research Unit, Medical Dept. D, Rambam Medical Center; Rappaport Faculty of Medicine, The Technion; and Israel National Oceanographic Research Institute, Haifa

16 patients with hypercholesterolemia were treated with an extract of alpha-alpha leaves (esterol) while on a low-fat, low-cholesterol diet. Esterol is believed to inhibit the absorption of cholesterol and bile acids and may interfere with the absorption of essential nutrients. As oxidative modification of lipoproteins is required for the process of atherosclerosis, plasma antioxidant vitamins were followed. After 4 months of treatment, plasma cholesterol decreased by 10% from 282 to 250 mg/dl (p<0.001) and LDL cholesterol by 13%, from 203 to 177 mg/dl (p<0.001). Plasma antioxidant vitamins E, A and β-carotene were unchanged. Thus, esterol has a cholesterol-lowering effect but apparently does not lower fat-soluble, plasma antioxidant vitamins. Both cholesterol-lowering and plasma antioxidant vitamins are important for the primary prevention of coronary artery disease in hypercholesterolemia.

Recurrent Tuberculosis in a Psychiatric Hospital

A. Zeenreich, B. Gochstein, A. Grinshpoon, M. Miron, J. Rosenman, I. Ben-Dov

Pulmonary and Radiology Institutes, Chaim Sheba Medical Center, Tel Hashomer and Tel Aviv University; Israel Ministry of Health; and Gan Meged Hospital

During 1987-1996, 39 of 720 patients hospitalized (most for severe schizophrenia) were diagnosed as having active pulmonary tuberculosis (5.4%, 975 per 105 per year). In 1992-1993, after a cluster of 5 cases was found, all patients were screened by PPD skin test and chest X-ray and 16 more cases were identified. Diagnosis was confirmed bacteriologically in only 10 of them but there were typical radiological findings in the others. 39 were treated with a multi-drug regimen. In addition, 333 exposed patients and 21% who had converted their skin tests were given isoniazid preventive therapy. A small increase in levels of liver enzymes was common, but significant abnormality (over 4 times the upper limit of normal) was found in only 7 patients, in whom therapy was therefor stopped or changed. During a follow-up period of 4 years, 2 more developed tuberculosis and 33 converted their PPD reactivity status. We conclude that an outbreak of tuberculosis in a psychiatric hospital can be controlled with a relatively low rate of side-effects by using systematic diagnostic and therapeutic measures. However, single step screening is not sufficient. Routine screening of all new patients, a high index of suspicion and contact investigation are needed.

Propofol Anesthesia for Craniotomy in Patients who are Awake

Ron Ben Abraham, Noah Lieberman, Zvi Ram, Sylvia Klempner, Azriel Perel

Depts. of Anesthesia and Intensive Care and of Neurosurgery, Chaim Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv University

During craniotomy, the patient's cooperation is needed during procedures in which continuous neurological examination and mapping of crucial regions close to the area to be resected area are required. We report our experience in 9 patients who underwent such procedures under intravenous propofol as the main sedating agent. This short-acting hypnotic was administered prior to and during the painful stages of the procedure. Patients were fully asleep when the skull was opened and the dural flap raised or excised. During the rest of the operation patients were lightly sedated but remained responsive and cooperative. This enabled precise intra-operative mapping of the brain and surgery-related neurological deficits were avoided. Respiratory depression or hemodynamic compromise were not encountered. All patients were comfortable during the operation and there were no additional neurological deficits after operation. We believe that propofol should be the main sedating agent used for these procedures.

Paroxysmal Nocturnal Hemoglobinuria

Haim Ben-Ami, Yehuda Edoute

Medical Dept. C, Rambam Medical Center and Bruce Rappaport Faculty of Medicine, The Technion, Haifa

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disorder of blood cells which originate from an abnormal hematopoietic stem cell. The condition is characterized by nocturnal hemoglobinuria, chronic hemolytic anemia, and thrombosis. We describe a 60-year-old woman with PNH admitted with abdominal pain and jaundice, who had dark urine on arising after a night's sleep. The diagnosis was established by the typical clinical story and a positive Ham test. She was successfully treated with Halotestin and folic acid. Although PNH is rare, it should be considered in the differential diagnosis of hemolytic anemia. Early diagnosis and treatment are important.

Local Treatment of Purulent Chronic Otitis Media with Ciprofloxacin

Ludwig Podoshin, Alexander Brodzki, Milo Fradis, Jacob Ben-David, Josef Larboni, Isaac Srugo

Dept. of Otolaryngology, Head and Neck Surgery, Microbiology Unit and Dept. of Pharmacology, Bnai-Zion Medical Center, and Bruce Rappaport Faculty of Medicine, The Technion, Haifa

We evaluated the efficacy of ciprofloxacin eardrops compared to tobramycin and to a placebo in the treatment of chronic suppurative otitis media. 60 ears were randomly assigned to treatment for 3 weeks with ciprofloxacin, tobramycin or placebo eardrops. The organism most commonly isolated from the ear discharge was Pseudomonas aeruginosa. The clinical responses were 78.9% and 72.2%, respectively, in the ciprofloxacin and tobramycin groups, while it was only 41.2% in the placebo group. Treatment with ciprofloxacin eardrops seemed to be at least as efficient as treatment with tobramycin. Considering the lack of ototoxicity of ciprofloxacin, this treatment may be best for chronic otitis media.

Surgical Approach to Benign Cecal Ulcer

J. Haik, A. Judich, I. Barshack, M. Ben-Haim, M. Shabtai, A. Ayalon

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

Benign cecal ulcer is a rare lesion, usually diagnosed during operation for suspected acute appendicitis or peritonitis of unknown origin. In the past, right hemicolectomy was recommended as the treatment of choice because of the difficulty in differentiating malignant lesions from benign cecal ulcers. However, in recent reports a more conservative approach has been suggested, consisting of selective colectomy followed by frozen section biopsy. This approach is aimed at preventing unnecessary excision of the colon and conserving the ileocecal valve. We present a 47-year-old woman operated for right lower quadrant peritonitis, believed to be due to acute appendicitis. On exploration, a biopsy-proven benign cecal ulcer was found and resected.

ATLS Course for Surgery Residents - Should it be Mandatory?

Ron Ben-Abraham, Michael Stein, Yoram Kluger, Gideon Paret, Avraham Rivkind, Joshua Shemer

Medical Corps, Israel Defense Forces; Sourasky Medical Center and Chaim Sheba Medical Center, Sackler School of Medicine, Tel Aviv University; and Hadassah Medical Center, Jerusalem

Senior surgeons were asked about mandatory participation of general surgery residents in the advanced trauma life support (ATLS) course. Although trauma care in Israel is given by surgical residents, in the opinion of their senior mentors the course should continue to be mandatory for them.

Carnitine Deficiency in Inborn Errors of Metabolism

B.-A. Sela, T. Lerman-Sagie, M. Berkovitz

Institute of Chemical Pathology, Chaim Sheba Medical Center and Section of Clinical Biochemistry, Sackler School of Medicine, Tel Aviv University; Pediatric Neurology Unit, Wolfsohn Medical Center, Holon; and Children's Ambulatory Clinic, Assaf Harofeh Medical Center, Zrifin

Several conditions, considered as inborn errors of metabolism, involve severe deficiencies in carnitine in both plasma and muscle. In the absence of evidence suggesting primary carnitine deficiency due to a biosynthetic enzymatic defect in the liver, the various diseases with carnitine deficiency are related to genetic defects in organic acid metabolism leading to blocked mitochondrial b oxidation. We describe a 4.5-year-old boy and 2 female infants with glutaric aciduria type I, isovaleric acidemia, and long-chain acid dehydrogenase deficiency, in whom severe carnitine deficiency was apparent. In all 3, long-term carnitine treatment proved to be vital and eliminated most of the symptoms.

Penetrating Ocular Injuries: a Retrospective Study

M. Schneck, S. Weitzman, T. Lifshitz, B. Biedner

Dept. of Ophthalmology, and Epidemiology and Health Services Evaluation Unit, Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Penetrating injuries of the eye are an important cause of unilateral visual loss. We studied a series of 82 cases of penetrating injuries treated here from 1987 through 1993. The injuries were caused by sharp objects in 66% and blunt trauma in 6%. The prognosis after a penetrating injury is greatly influenced by the nature of the injury and the extent of the initial drainage. Among factors associated with an unfavorable visual outcome were diminished preoperative visual acuity and scleral wounds with dense vitreous hemorrhage.