Pulmonary Involvement in Osler- Weber-Rendu Syndrome 

Efrat Bron-Harlev, Benjamin Zeevi, Ben-Zion Garty

Dept. of Medicine B and Cardiac Catheterization Unit, Schneider Children's Medical Center, Petah Tikva

Hereditary hemorrhagic telangiectasia (Osler-Weber-Ren-du syndrome) is a group of autosomal dominant diseases with variable penetration, characterized by vascular malformations. Recently hereditary hemorrhagic telangiectasia has been found to be a phenotypic expression of mutations in genes located on chromosomes 9 and 12, and possibly of other genes located on other chromosomes.

We describe 2 patients with hereditary hemorrhagic telangiectasia and pulmonary involvement who presented with repeated complaints of dyspnea and cyanosis and were diagnosed as having long-standing asthma. Both were treated with therapeutic catheterization and embolization with good clinical outcomes.

Cortical Blindness Following Coronary Angiography 

Addie Ron, Simcha Meisel, Myra Shapiro-Feinberg,Herman O. Klein

Depts. of Medicine, Cardiology and Diagnostic Imaging,Meir Medical Center, Kfar Saba

Cortical blindness has been occasionally reported as a complication after cerebral angiography, but is rare after coronary angiography. The contrast agent is believed to be responsible for the sudden development of blindness. Although the exact mechanism is unknown, it appears that the contrast agent disrupts the blood brain barrier, mostly in the occipital areas.

We report a 77-year-old man who suddenly developed transient, bilateral cortical blindness 4 hours after coronary angiography. Using contrast enhancement, the CT scan showed typical, symmetrical involvement of both occipital lobes. There were no other neurological deficits. Vision and CT findings returned to normal within 48 hours.

Causes of Blindness in Israel 

Y. Hod, Y. Corcia, Y. Yassur, O. Geyer

Depts. of Ophthalmology, Carmel Medical Center, Haifa and Rabin Medical Center, Petah Tikva; Israel Ministry of Labor and Social Affairs, Jerusalem; and Rehabilitation Services Administration, Services for the Blind

Of the world population, 38 million are blind and another 110 million are visually impaired. Even in the developed countries there are 3.5 million who are blind.

This study of blindness in Israel is based on the National Blind Registry. At the end of 1998, 15,937 were registered as blind, 0.3% of the total population; 776 (5%) of them were 18 years old or younger; 6,426 (40%) 18-65 years old; and 8,735 (55%) 65 years or older.

The leading causes of blindness in Israel are glaucoma (2,074, 13%), macular degeneration (1,954, 12%) and diabetes mellitus (1,680, 11%). Since glaucoma and diabetes, and to a lesser extent glaucoma, respond to treatment, blindness could have been avoided in most cases. National screening programs for early diagnosis and treatment of these diseases would reduce prevalence of the newly blind.

Endoscopic Sinus Surgery for Extracranial Complications of Sinusitis

E. Deutsch, I. Hevron

ENT Dept., Bikur Cholim Hospital, Jerusalem

Orbital subperiosteal abscess (SPA) and Pott's puffy tumor (PPT) are the major extracranial complications of acute sinusitis. These complications are aggravated by the close anatomic relationships between the nasal sinuses and the orbits and frontal bone. Furthermore, the rich diploic venous drainage of the region enhances the spread of the infection.

Between 1992 and 1997, 16 patients (mean age 12 years, range 2-15, 10 of them males), 11 of them with SPA and 4 with PPT were operated on by the senior author by means of endoscopic sinus surgery (ESS). Indications for operation included: CT findings of abscess formation and lack of clinical improvement after 48 hours of IV antibiotic therapy. Clinical resolution of symptoms was achieved in all and there were no operative or postoperative complications.

We emphasize and explain the advantages of ESS over external approaches in surgery for extracranial complications of acute sinusitis by several facts: the technique treats the source of the disease, clinical success rate is high, morbidity is low, and facial distortion and poor cosmetic results completely avoided.

Cost-Effectiveness of Imaging Procedures in Knee Injuries 

Moshe Leshno, Hagai Amir, Dalia Leshno

School of Business Administration, Hebrew University of Jerusalem; Dept. of Orthopedics, Tel Aviv Medical Center; Israel Defense Forces, and Faculty of Management, Tel Aviv University

It is clear to experts in the health care industry that with regard to medical decision-making, considering costs of medical devices is not a good index, but it is highly important to include medical aspects. This approach guarantees an advance and an appropriate method.

We present a cost-effectiveness analysis to target the optimal strategy of how to use medical diagnostic devices in cases of knee injuries. Today, one of the more common medical emergency referrals seen worldwide is in the area of orthopedics, knee injuries in particular. They can harm soft tissue, bone tissue, or both simultaneously. The diagnosis of soft tissue injury is difficult at best. Among the devices helping the clinician make an accurate diagnosis are MRI and ultrasound (US).

We conclude that US is superior to MRI in this condition. This is especially true when the orthopedic examination leads to the suggestion of arthroscopy. The results were not modified, and a sensitive study of the parameters that influence the decision- making process was undertaken. Decision-makers should not consider medical outcome alone, but should take into account the costs. A clinical study should validate the assumption on which this study was based.

Progressive Ptosis in Children as a Presenting Sign of Kearns-Sayre Syndrome 

Ron Gal, Eli Lahat

Pediatric Neurology Unit, Assaf Harofeh Medical Center and Sackler Faculty of Medicine, Tel Aviv University

Mitochondrial encephalopathies represent a heterogeneous group of various neurological syndromes caused by defects in mitochondrial metabolism. All clinical syndromes can be subdivided by type of biochemical defect into 3 subgroups: defective oxidation, defects in pyruvate metabolism and various defects in the respiratory chain.

We present a 12-year-old girl admitted for evaluation of progressive ptosis over a period of 3 years, diagnosed as having the rare mitochondrial encephalopathy, Kearns-Sayre syndrome.

Acute and Chronic Hepatitis C in Hemodialysis Patients 

Nir Hilzenrat, Marcus Mostoslavsky, David Tovbin

Liver Disease Service - Division of Gastroenterology, Dialysis Unit, Dept. of Nephrology and Division of Internal Medicine, Soroka Medical Center and Ben-Gurion University of the Negev, Beer Sheba

Acquired infection with hepatitis C virus (HCV) in hemodialysis patients has been described lately. In dialysis units in Italy and France, the prevalence and incidence of HCV are 20-60% and 1-2%, respectively. Most infected patients develop chronic hepatitis. The clinical presentation of acute HCV in hemodialysis patients is very mild and therefore the diagnosis is often made only by laboratory tests. Acute infection is usually followed by mild elevation of liver enzymes and the presence of HCV-RNA and anti-HCV in serum.

We report a 48-year-old man on hemodialysis who developed acute hepatitis C. The diagnosis was made by finding mild elevation of liver enzymes and the presence of HCV-RNA in his serum. A few months later, he developed severe hepatitis which was followed by rapid deterioration in liver function. However, the virus was eradicated and liver function tests became normal. Surprisingly, serum anti-HCV antibodies were detected 5 months later.

Follicular Splenic Lymphoid Hyperplasia Associated with EBV Infection

Boleslaw Knobel, Enrique Melamud, Sharon Nofech-Moses, Liliana Zeidel

Dept. of Medicine B and Institute of Pathology, Edith Wolfson Medical Center, Holon and Sackler Faculty of Medicine, Tel Aviv University

Massive splenomegaly is defined as a spleen weighing about 10 times normal weight. We describe a 36-year-old man who had huge splenomegaly and secondary pancytopenia simulating malignant lymphoma for about 3 months. Splenectomy was necessary because of the suspicion of hematologic malignancy, especially isolated lymphoma of the spleen, and pain and mechanical abdominal disturbances.

On operation, the spleen was 25 cm long and weighed 250 g. There was florid, reactive follicular lymphoid hyperplasia. Immunohistochemical staining with CD-20(L26), CD-45Ro(UCHL), bcl-2 oncoprotein (Dakopatts), EBV (anti-EBV mol weight 60 KD, Dakopatts) was consistent with reaction to EBV infection and not with follicular lymphoma. Lack of PCR amplification using DNA extracted from paraffin-embedded splenic tissue indicated absence of a monoclonal B cell population carrying rearranged immunoglobulin genes. The lymphocytic population was proven polyclonal by the negative results of PCR for the bcl-2 gene rearrangement. EBV seroconversion from high titer antibodies of anti-EBV-VCA-IgM to negative, and from negative EBNA to positive was consistent with an apparent primary EBV infection.

We have not found on computerized search a previous report of reactive follicular splenic hyperplasia to EBV infection causing huge splenomegaly, with or without EBV-induced infectious mononucleosis.

Apomorphine for "Off-Periods" in Parkinson's Disease

J. Zoldan, D. Merims, A. Kuritzky, I. Ziv, E. Melamed

Dept. of Neurology, Rabin Medical Center, Beilinson Campus, Petah Tikva and Sackler Faculty of Medicine, Tel Aviv University

After 3-5 years of continuous use of 1-dopa preparations for Parkinson's disease, 25%-50% of patients develop side-effects such as the "on-off" phenomenon and involuntary movements that markedly impair function. One cause of these manifestations is evidently a disturbance in the absorption of 1-dopa.

We attempted to avoid this problem by using subcutaneous injections. Apomorphine is a rapid-acting dopamine agonist which causes a return from "off" to "on" within minutes.

We present the results of a trial of subcutaneous injections of apomorphine in 22 Parkinsonian patients (12 males, 10 females) with severe motor fluctuations. During 5 days prior to the apomorphine all received Motilium (domperidone, 60 mg/d) to prevent nausea and vomiting. All were hospitalized initially to determine optimal dosage and to teach them the technique of self-injection.

2 to 4 mg of apomorphine were injected 1 to 3 times daily for 2 to 12 months. In 17 patients (80%) "off" periods were reduced without significant side-effects. Apomorphine seems to be effective, tolerable treatment for shortening 1-dopa induced "off" periods.

Listeria Monocytogenes Infections - Ten Years' Experience

Tova Rainis, Israel Potasman

Infectious Disease Unit, Bnai Zion Medical Center, and Faculty of Medicine, Technion, Haifa

7 cases of listeriosis were diagnosed here between 1988-1997 (6 in last 3 years), or 2.94/100,000 admissions. 2 elderly patients suffered from meningitis and 2 pregnant women presented with premature contractions, 1 of whom delivered a premature, infected baby. 2 other patients had fever and gastroenteritis.

Listeria monocytogenes was isolated from blood in 4, CSF in 2 and the placenta in 1. It was isolated from those with bacterial meningitis. All patients recovered. Both increased awareness for prevention and better diagnosis are essential to reduce morbidity from this unusual pathogen.

Evaluation of Asthmatic Children Presenting at Emergency Rooms

Haim Bibi, Elena Shoychet, David Shoseyov, Michael Armoni, Emil Chai, Dorit Ater

Pediatric Pulmonary Clinic and Pediatric Dept., Barzilai Medical Center, Ashkelon; Pediatric Pulmonary Clinic, Bikur Cholim Hospital, Jerusalem; Emergency Room, Barzilai Medical Center, Ashkelon; and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Bronchial asthma in the pediatric age group has become prevalent recently. Many children who suffer from asthma arrive at the emergency room (ER) with exacerbations which did not respond to medical treatment at home.

Between July and December 1997, 136 children 8 months to 14 years of age (61% below 3 years), were studied in our pediatric ER. Investigation included physical examination and pulse oximetry, which were used as guidelines for scoring the children on arrival and post-treatment. Spirometry was done in those who could cooperate. For each patient a detailed questionnaire about medical and sociodemographic factors was filled.

Primary pediatricians used mainly beta-agonist and corticosteroid inhalators, while pediatric pulmonologists used mainly inhaled steroids. There was no relationship between severity of attack on arrival at the ER, mode of treatment and speed of recovery in the ER. More children treated by a general pediatrician more were admitted to hospital. Low parental education and paternal smoking were risk factors for recurrent hospital admissions.

Our results indicate that parents must be educated to stop smoking, especially those with asthmatic children, and primary pediatricians should be updated with regard to proper treatment and follow-up of asthma.

Fulminant Meningococcemia Presenting as a Gastroenteritis - Like Syndrome

A. Hussein, Y. Naschitz, D. Yeshurun

Depts. of Emergency Medicine and of Medicine A, Bnai Zion Medical Center and Bruce Rapport Faculty of Medicine, Haifa

Neisseria meningitidis infection (meningococcemia) is very common throughout the world. It usually presents as meningitis or sometimes pharyngitis. A gastroenteritis-like syndrome, with diarrhea, vomiting and abdominal pain, may occur in children but is very rare in adults. Search of the medical literature revealed only 3 such cases, all in young adults.

We report an 80-year-old woman who presented with fever, diarrhea and abdominal pain. Meningococcus infection was later suspected, and proved by culture. Although treatment was intensive and included ceftriaxone (Rocephin) and garamycin, she did not respond and died 40 hours after admission. We draw attention to the possibility that what is usually a common symptom can be the first presentation of a serious, often fatal condition.

Elastofibroma, a Rare Cause of Snapping Scapula Syndrome

Ilan Cohen, Yehuda Kolender, Josephine Isakov, Aaron Chechick, Yitzhak Meller

Dept. of Orthopedic Surgery, Sheba Medical Center, Tel Hashomer and Depts. of Orthopedic Oncology and Pathology, Sourasky Medical Center, Tel Aviv

Scapular pain is a common complaint in daily orthopedic practice. A different type of scapular discomfort, the snapping scapula syndrome that occurs when smooth gliding motion of the scapula upon the chest wall is interfered with is much less common.

We studied the syndrome of periscapular pain and discomfort, and present a rare etiology: elastofibroma dorsi, a unique, benign, soft tissue-tumor with unique characteristics.

Elastofibroma appears deep to the lower scapular pole, is often bilateral, and consists of a mixture of collagen, elastic fibers and fibroblasts. We present 6 cases, in 3 men and 3 women aged 51-65.

Knowledge, Attitudes and Training of Family Physicians with Regard to Addiction to Illicit Drugs

Aya Peleg, Roni Peleg, Pesach Shvartzman

Health Promotion and Disease Prevention Unit, and Dept. of Family Medicine, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Substance abuse is one of the most serious problems in Israel. Recent studies show the situation is getting worse. The deleterious outcomes of psychoactive substance abuse include crime, car accidents, physical and mental illness, violence and work injuries.

48 family physicians in the Negev completed a questionnaire which included training, diagnostic and treatment skills, attitudes, knowledge and need for training in this field. 38 family physicians (81%) had had no training, most (96%) indicated the importance of such training. 34 (71%) said that their medical skills could not cope with the problems of addiction, and 29 (64.5%) claimed that the quality of care of addict patients is reduced because of lack of knowledge and diagnostic skills. These findings support the conclusion that efforts should be invested in training family physicians in the field of psychoactive drugs, licit and illicit.

Severe Hypercalcemia Due to Renal Transitional Cell Carcinoma

D. Zamir, J. Weizman, N. Erlich, M. Amar, P. Weiner

Depts. of Medicine A, Urology and Pathology, Hillel Yaffe Medical Center, Hadera

Hypercalcemia is a common metabolic disorder, especially in the elderly. The most common etioloare hyperparathy-roidism or malignancy, most often of the lung, breast, kidney or hematological system. Because hypercalcemia is an uncommon manifestation in urinary tract epithelial tumors, especially those of the renal pelvis, we present a man aged 62 years with hypercalcemia due to renal transitional cell carcinoma.