תוצאת חיפוש

Muir-Torre Syndrome: Importance of Clinical Diagnosis and Genetic Investigation

Hana Strul, Paul Rozen, Tova Naiman, Ruth Shomrat

Gastroenterology Dept. and Genetics Institute, Tel Aviv Medical Center and Tel Aviv University

Muir-Torre syndrome is a relatively rare cutaneous manifestation of hereditary nonpolypous colorectal cancer (HNPCC). This autosomal dominant syndrome is characterized by a combination of sebaceous gland and malignant visceral tumors. The common sites of internal malignancies are the gastrointestinal tract and urinary system. It appears in early adult life and its clinical course is relatively slow.

In some families genetic diagnosis can identify asymptomatic carriers of the mutation. All first-degree relatives, especially mutation carriers, should be referred from the age of 20 years for routine follow-up and early treatment, as it has been proven to decrease morbidity and mortality.

We present a 51-year-old man with Muir-Torre syndrome diagnosed by the presence of multiple adenomas of sebaceous glands, colonic adenoma and adenocarcinoma of the duodenum. The family history was typical for HNPCC. A mutation in the hMSH2 gene on chromosome 2p was found in the patient and in several asymptomatic family members. The aim of this report is to increase awareness of this syndrome and emphasize the importance of referring patients and their families for clinical and genetic counseling and diagnosis.

Multisystem Disease Caused by BCG Imitating Miliary Tuberculosis

I. Tsikonov, D. Yeshurun, J.E. Naschitz

Dept. of Medicine A, Bnai Zion Medical Center and B. Rappaport Faculty of Medicine, The Technion, Haifa

As the prevalence of tuberculosis is on the rise in western countries, we present a 79-year-old man who developed a pulmonary tuberculosis-like syndrome following immunotherapy with BCG for carcinoma of the urinary bladder. The symptoms subsided following 3-drug antitubercular treatment, and the addition of steroids following negative cultures for Mycobacterium tuberculosis. The course of this disease, named BCG-osis, is much more favorable than miliary tuberculosis, even with milder treatment. It is important to keep in mind this phenomenon now that there is increasing treatment of cancers with BCG.

Combination Chemotherapy in Metastatic Urothelial Cancer

A. Sella, D. Flex, D. Gafni, O. Rabinovitz, A. Sulkes, J. Baniel

Genitourinary Medical Oncology Unit, Depts. of Oncology and Urology, Rabin Medical Center, Beilinson Campus, Petah Tikva and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv

The treatment of metastatic urothelial cancer is based on the combination of cisplatin, methotrexate, vinblastine and adriamycin (M-VAC). From November 1994 to May 1997 we treated 25 patients (51 men, 3 women, aged 50-77) with M-VAC. The tumor originated from the urinary bladder in 14 (56%) and the upper urinary tract in 11 (44%). Disease sites included: primary - 5 (25%), lymph nodes - 17 (68%), lungs - 10 (40%), bones - 8 (32%), pelvic mass and liver each - 4 (16%), with an overall median of 2 (1-5) sites per patient.

9 patients (38%) had complete responses and 8 (32%) had partial responses, for an overall response rate of 68% (95% CI 48.5%-85%). The median duration of response was 15.3 (1.6-29.6+) months. Median survival of responders was 19.1 (4.8-35.7+) months compared to 6.2 (0.7-11.2) for the non-responders (p<0.05). 13 (52%) of patients are alive, of whom 8 (32%) are free of disease and 5 with a single metastatic site on presentation at follow-up.

In the 118 treatment cycles we observed grade III-IV toxicity: myelosuppression 53 (45%), thrombocytopenia 4 (3%), stomatitis 8 (6.7%), diarrhea 3 (2.5%). There were 22 infectious episodes and 1 patient died of sepsis.

We achieved a high response rate with the combination M-VAC. However, only a third had long-term disease-free states and treatment was associated with excessive toxicity. Thera-peutic approaches with new agents are required to improve the response rate and toxicity.

Laparoscopic Repair of Inguinal Hernia Experience in 54 Consecutive Cases

S. Kyzer, I. Kristalni, M. Alis, I. Charuzi

Surgical Dept. B, Wolfson Medcial Center, Holon

We describe our experience in 54 consecutive patients who underwent laparoscopic repair of 86 inguinal hernias. Laparoscopic repair of inguinal hernia is technically feasible, does not prolong the length of the procedure nor of hospitalization and is not accompanied by increased morbidity. Although there is not yet general agreement, in our experience and that of others, it appears that laparoscopic repair will be the preferred approach to the treatment of inguinal hernia.

Intestinal Transplantation: World Experience and Future Perspective

Eytan Mor, Moshe Rubin, Yaron Niv

Depts. of Transplantation, Surgery B and Gastroenterology Institute, Rabin Medical Center, Beilinson Campus, Petah Tikva

Intestinal transplantation, which until recently has been considered an experimental procedure, is now undergoing considerable change and becoming a standard treatment for patient with intestinal failure. The major improvement in results is due to the introduction of new immunosuppressive agents that have led to significant reduction in acute rejection. Nevertheless, reduction in severe infectious complications, which are the main cause of mortality after transplant, as well as improved techniques for early diagnosis of rejection are needed before intestinal transplantation can be widely used.

We describe our experience in post-transplant follow-up of a woman, aged 32-years, who had undergone intestinal transplantation for short bowel syndrome after extensive bowel resection.

Legionellosis in Israel

Ida Boldur, Silviu Hoffmann, Regina Kazak, Batzion Benjamin

Institute of Microbiology, Assaf HaRofeh Medical Center, Zrifin and Dept. of Life Sciences, Bar-Ilan University, Ramat Gan

Infection with Legionella remains an important cause of disease and death. We analyzed our laboratory data from 1993 through 1997, augmented by our 20 years of experience. The incidence of Legionella as a cause of pneumonia varied in our study from 5%-9%, with a slight increase during the winter. Isolation of these microorganisms from different water sources was higher during the summer and ranged from 7%-70%.

Special laboratory tests are necessary to diagnose the disease and monitor these bacteria in water samples. The serologic method - indirect immunofluorescent assay -- for 41 serogroups of Legionella was the main diagnostic method used. Legionella sg. 1 was the most frequent cause of the disease, with an incidence of 52% in 1993, decreasing to 15% in 1997. An increase in the incidence of seropositivity to "other Legionellae" is characteristic for our country.

No correlation was found between the incidence of isolation of a specific strain and exposure. However, it is well known that the disease is overtreated but underdiagnosed, which requires reversal. Larger studies of Legionella colonization in water supplies and in air are needed in order to establish the risk of infection. Water sources are presently under-studied, as are respiratory devices in hospitals, or they are not studied at all in Israel, such as in mist machines in supermarkets, in dental clinics, and in ships and airplanes.

Cerebellar Infarction: Clinical Presentation, Diagnosis and Treatment

Y. Skurnik, S. Brandiner, G. Gandelman, Z. Shtoeger

Medical Dept., Kaplan Medical Center, Rehovot (Affiliated with Hebrew University-Hadassah Medical School, Jerusalem) and Dept. H, Harzfeld Hospital, Gedera

Cerebellar infarction is relatively infrequent and accounts for about 2% of all strokes. Its clinical presentation and course are variable. It may resemble vestibulitis in mild cases, but the presentation may be more dramatic in other cases. Cerebellar infarction may cause life-threatening complications such as acute hydrocephalus or brain stem compression, resulting from their mass effect in the posterior fossa or extension of the infarct to the brain stem.

Clinical features alone are insufficient for the diagnosis and for follow-up of patients with cerebellar infarction. However the advent of CT and MRI and their availability enable early diagnosis of cerebellar infarction, and early recognition of the development of acute hydrocephalus or brain stem compression which require surgical decompression. The prognosis of most cases is good when treatment is appropriate.

Alcoholic Delirium: Warning Signs and Diagnosis

Eliezer Witztum, Izhak Z. Ben-Zion, Vladimir Lerner

Community Mental Health Center; and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Lately an increasing number of physicians are asked to diagnose and treat physical and mental disorders caused by alcohol abuse, a phenomena which had been quite rare in Israel until recently.

Early diagnosis and efficient treatment are essential for the management of alcohol-dependent patients. Primary care physicians and hospital personnel should be more alert to the growing numbers of alcohol abusers and to their appropriate diagnosis and treatment. This article describes and summarizes the symptoms of alcohol withdrawal in general, and alcoholic delirium in particular. Problems in diagnosis and treatment are illustrated by typical cases, pointing out early clinical warning signs and suggesting some treatment guidelines.

Percutaneous Closure of Patent Arterial Ducts with Occluding Spring Coils

Benjamin Zeevi, Galit Bar-Mor, Michael Berant

Cardiac Catheterization Unit, Schneider Children's Medical Center, Petah Tikva and Sackler School of Medicine, Tel Aviv University

In recent years percutaneous closure of small and medium- sized patent arterial ducts has been achieved using occluding spring coils. We describe our experience in 93 patients with this tec, using a snare to facilitate the procedure in most. All patients had a clinically apparenpatent arterial duct and had undergone attempts at transcatheter closure at a mean age of 6.8 years. In 1, the duct was a residual lesion following surgical ligation, and in 5 it was a residual following attempted closure with the Rashkind double-umbrella. The mean narrowest diameter of the ducts was 2.1 mm.

In our 93 patients implantation was successful in 92 (99%), using 1 coil (82 patients), or 2 (10 patients), and in 1 by a combination of a double-umbrella device and an occluding spring coil.

The mean fluoroscopic screening time for the whole group was 22.8 minutes, which decreased to 16.8 minutes in the last 50 patients. The coil embolized in 7 patients, but was retrieved in 6 and the ducts were subsequently occluded with another coil. In 1 patient the coil was left in a distal small branch of the left pulmonary artery and the duct was successfully occluded with a double-umbrella.

Color-Doppler echocardiogram performed the morning after placement of the coils showed residual leaks in 18%. At mean follow-up of 24.6 months repeat imaging showed residual leaks in only 3 of these patients (3%).

We conclude that occlusion of small to medium-sized ducts using coils appears to be effective and is the treatment of choice. The use of a snare to hold and manipulate the coil as it is delivered improves control of the coil, the accuracy of its placement, as well as giving complete occlusion of the ducts.

Maximal Age Reflects Ignoring the Health of the Oldest in General, Geriatric, and Gerontolo- Gical Studies

Vladimir Shats

Geriatric Dept., Rebecca Sieff Government Hospital, Safed

In publications relating to the health of the elderly there are 2 ways of presenting maximal ages, collective (for example: 70+ years) and individual maximal age (for example 70 years). While enabling assessment up to a certain age, data from subjects above the maximal age stated in the research will not be included. From the literature of the past 10 years, there were selected 764 disease parameters (PD) and 177 parameters of aging (PA). Among them 667 (70.9%) and 274 (29.1%) were parameters with collective and exact maximal ages, respectively.

The lack of reference by authors to ages above 70 to 79 (or 70+ to 79+) and 80 to 89 (or 80+ to 89+) was calculated from the medical literature, and estimated as significant, and for ages above 80 to 89 was estimated as minimally significant, in regard to their focus on health data in the elderly. In different groups of parameters, 24% to 32% of maximal ages indicated significant and 25% to 65% minimal lack of reference. Maximal ages of PA were higher than those of PD (p<0.001), so lack of reference to health of the elderly was more significant when PD were studied as compared to PA. Lack of reference was more significant in studies of hospitalized and ambulatory patients and people living within the community. Usually authors checked all the populations of people living in nursing homes, but the total number of parameters relating to the latter, was very small, only 5.1% of the total number of parameters, so the populations of nursing homes did not reach the attention of the researchers.

Collective maximal ages are more often used in geriatric journals, including the Journal of the American Geriatric Society, as opposed to general and gerontological journals, although this approach seems to be too sweeping in the assessment of health of the elderly.

Cocaine "Mules"

Ornit Yanai, Jehuda Hiss

L. Greenberg Institute of Forensic Medicine, Tel Aviv (Affiliated with the Sackler Faculty of Medicine, Tel Aviv University)

We present 2 cases of "body-packer" syndrome (BPS) in which the "mules" carrying the packages of narcotics arrived at Ben-Gurion Airport presented symptoms of acute cocaine intoxication due to the bursting of packages of narcotics they were transporting within their gastro-intestinal systems.

Acute cocaine overdose due to sudden massive release of the drug into the digestive system in BPS, may cause psychosis, convulsions and eventually death. Blood levels of cocaine between 0.25 and 5 mcg/ml are toxic and occasionally lethal. When a package bursts within the digestive tract of a smuggler or blocks the intestines, an immediate laparotomy is necessary to evacuate the "ovules" and the remains of the drug from stomach and intestines. Patients merely suspected of being body packers can be diagnosed by physical examination and by means of various imaging methods. They can be conservatively treated with fluids and mild laxatives and kept under close supervision until the remaining packages are naturally discharged.

Sudden onset of psychotic behavior in travelers from South America or other drug-producing countries should raise suspicion of body-packer syndrome. The relevant authorities should therefore be aware of the symptoms and consider their potentially dangerous outcome.

Ilizarov Reconstructive Surgery in Complex Problems of the Musculoskeletal System

H. Shtarker, G. Volpin, A. Lerner, H. Stein, S. Wientroub, D. Hendel

Depts. of Orthopedic Surgery, Western Galilee Hospital, Naharyia, Rambam Medical Center, Haifa, Dana Children Hospital, Tel Aviv-Sourasky Medical Center, and Rabin Medical Center (Golda Campus, and Hasharon Hospital), Petah Tikva

The Ilizarov technique is an important modality of reconstructive surgery for limb deformities, such as malunion and nonunion of fractures, persistent osteomyelitis, and bone loss following complex limb injuries, as well as in limb- lengthening procedures. It has received wide recognition in the Western world over the past decade. In MEDLINE we found 537 articles published between 1971-1995 that describe the use of this technique. Of these, only 18 were published between 1971-1975, while 261 were published between 1991-1995.

The present paper describes the developments and updates in this method, as a result of the large experience gained by the authors in a number of centers in Israel using this technique. The Ilizarov apparatus is a circular frame that allows accurate control, much more than any other external fixator, during correction of limb deformities and limb-lengthening. It is minimally invasive, and open techniques and use of internal hardware and bone grafts are not needed.

Based on our experience, we suggest that this method requires careful preoperative planning and meticulous surgical technique. More important, cooperation between surgeon and patient throughout the lengthy treatment is a prerequisite to ensure complete success, even in the most complicated cases.

Video-Assisted Thoracoscopic Surgery for Diagnosis of Pulmonary Lesions

O. Zamir, Y. Haskel, R. Spira, D. Eimerl, H.R. Freund

Depts. of Surgery and Anesthesiology, Hadassah University Hospital, Mount Scopus, Jerusalem

23 patients (age 11-66 years) underwent video-assisted thoracoscopic biopsy for diffuse disease or peripheral nodular lesions of the lung. 12 had been previously treated for extra- pulmonary malignancy and lung biopsy was done for suspicious metastases. In all cases except 1, lesions were identified and biopsied by thoracoscopy. The postoperative course was easier and shorter as compared to thoracotomy and the mean hospital stay was only 2.5 days. Thoracoscopic lung biopsy is a safe, effective and accurate diagnostic modality for diffuse lung disease and peripheral lesions. It is associated with minimal postoperative pain and discomfort, short hospital stay, early return to normal activity, and gives good cosmetic results.

Influenza Surveillance through Sentinel Reporting Clinics

T. Shohat, N. Versano, A. Kiro, G. Golan, E. Mendelson, M. Weingarten

For the Influenza Surveillance Network: Israel Center for Disease Control, Israel National Laboratory for Influenza and Central Virology Laboratory; Netka Child Health Center and Dept. of Family Medicine, Rabin Medical Center, Beilinson Campus, Petah Tikva

In a joint effort of the Israel Center for Disease Control, the National Center for Influenza in the Central Virology Laboratory, together with a group of collaborating pediatricians and family physicians, a network for influenza surveillance was established in the winter of 1996-97. Nose and throat swabs were obtained from 571 patients with flu-like illness. 133 (23%) were positive for influenza virus. Both influenza A(H3N2) and B were isolated, predominantly influenza B during the beginning of the season. Both circulating strains were antigenically similar to those included in the vaccine for 1996-1997. Patients from whom influenza virus was isolated were significantly more likely to suffer from cough and myalgia in comparison with patients whose cultures were negative (p=0.02 and 0.003. respectively). Results of the first year of surveillance indicate that sentinel reporting clinics are useful for timely detection and identification of the viral strains circulating in the community, thus allowing prompt intervention in preventing the spread of influenza. Conclusions from the first year of the study were drawn and applied in the winter of 1997-1998.

Iatrogenic Gallstone Ileus: A New Complication of Bouveret's Syndrome

Miguel Iuchtman, Amos Sternberg, Ricardo Alfici, Ehud Sternberg, Tzvi Fireman

Depts. of Surgery and Gastroenterology, Hillel Yaffe Medical Center, Hadera, and Rappaport Medical School, Haifa

Bouveret's syndrome involves gastric outlet obstruction caused by a gallstone in the duodenum. This type of gallstone ileus can be diagnosed and treated endoscopically. Endoscopic stone removal is especially indicated in poor risk patients. A dislodged impacted stone can migrate distally and cause small bowel mechanical obstruction. We report a 51-year-old woman who underwent endoscopic duodenal stone manipulation which resulted in small bowel obstruction.