תוצאת חיפוש

Gyrate Atrophy of Choroid and Retina, and Hyperornithinemia 

B-A. Sela, J. Zlotnik, T. Masos, G. Yablonski, F. Abraham

Institute of Chemical Pathology and Goldschlager Eye Institute, Sheba Medical Center, Tel Hashomer; and Sackler Faculty of Medicine, Tel Aviv University

Gyrate atrophy of the choroid and retina is a rare degenerative disease, characterized biochemically by a marked increase in blood ornithine levels, due to deficiency of ornithine S-amino transferase. 4 men aged 35, 36, 48 and 62 years are described with different stages of myopia, night blindness and loss of peripheral vision, which progressed to tunnel vision and partial blindness. Onset of the disease was at ages 3, 10 and 15 years, respectively, while in the 4th patient there was delayed expression starting at about age 50. Most had posterior subcapsular cataracts, and the ocular fundus exhibited demarcated circular areas of chorioretinal degeneration. So far the only patients described in Israel have been of Iraqui origin. Our fourth patient originated from Istanbul, and he may represent a hitherto undescribed variant with a much delayed expression of the disease.

Treatment of Velopharyngeal Insufficiency

Abraham Amir, Ram Silfen, Daniel J. Hauben

Dept. of Plastic and Reconstructive Surgery, Rabin Medical Center, Beilinson Campus, Petah Tikvah

Weak and hypernasal speech, along with nasal escape of air, are the main characteristics of velo-pharyngeal incompetence (VPI). We describe 10 years of experience (1989-1998) with surgical treatment of VPI.

51 patients underwent pharyngeal flap elevation. 37 had cleft palate (8 of them submucous), 7 had neuromuscular disorders and another 7 were idiopathic. All underwent evaluation by a speech therapist before and after operation. 25 had further nasal endoscopy and/or videofluroscopy.

There was significant speech improvement in volume and clarity in 35 (73%), mild improvement in 13 (27%) and none in 3. The complication rate was 15% and included sleep apnea, wound infection and dehiscence, stridor and bleeding. Complications were correlated with advanced age.

VPI should be diagnosed as early as possible to achieve good results and to prevent social problems.

Left Ventricular Outflow Tract Obstruction without Left Ventric-Ular Hypertrophy Treated with Ace Inhibitors

Jacob Feldman, Irit Laxer, Abraham Yaretzky

Geriatric Dept., Meir Hospital, Sapir Medical Center, Kfar Saba, and Sackler Faculty of Medicine, Tel Aviv University

We describe a very unusual case of sudden, severe worsening of congestive heart failure which was caused by ACE inhibitors. Diagnosis was made by echocardiogram showing a typical picture of dynamic, left ventricular outflow tract obstruction without left ventricular hypertrophy, which disappeared on discontinuing ACE inhibitors. This phenomenon has already been described as a complication of other drugs such as nitrates, commonly used as provocative tests for latent obstructive cardiomyopathy. To our knowledge ACE inhibihave not been described as a causative factor.

Breast Conservation: Safe for Early Breast Cancer

Gil Bar-Sella, Georgetta Fried, Zipora Brotman, Anna Ravkin, Riva Borovik, Abraham Kuten

Dept. of Oncology, Rambam Medical Center; Dept. of Oncology, Lin Medical Center; and Rappaport Faculty of Medicine, Technion, Haifa

Between 1981-1993 581 women with primary breast cancer were treated by breast conservation. Their mean age was 56‏12 years and 63% were postmenopausal and 37% pre- or perimenopausal. The median follow-up time was 56 months. 45% had pathological Stage I disease, 49% Stage II, 2.5% Stage III and 3.5% clinical Stage I-II disease. 54% of lesions were excised with good margins, 10% with close margins (<0.5 cm), 9% with microscopic residual, 3% with macroscopic residual, and in 24% margins were not reported. Adjuvant therapy, consisting of combination chemotherapy and/or hormones, was given to 69%.

Radiotherapy, usually 50 Gy tangential photon irradiation to the whole breast, was given to 564 (97%); an electron or photon "boost" to the tumor with a median dose of 17.5 Gy was given to 378 (65%). Most of those with positive nodes received 50 Gy to the lymphatic drainage system.

1 year after radiotherapy cosmetic results were rated as "good" or "excellent" in 80%, "moderate" in 17% and "poor" in 3%. The 5-year actuarial survival was 97% in Stage I and 88% in Stage II. 37 patients (6.5%) developed breast recurrence; 11 of these (2%) had simultaneous distant metastases. 5 (<1%) developed axillary or supraclavicular lymph node metastases, and 81 (14%) developed distant metastases. Most local recurrences were in those younger than 40, and in those with primary tumors >1.75 cm.

The satisfactory level of local control achieved is attributed to the high doses of radiation (up to 75 Gy) administered to those with high risk lesions.

Infective Endocarditis Presenting as Polyarticular Septic Arthritis

Abraham Yaretzky, Jacob Feldman, Carola Vigder

Geriatric Dept., Meir Hospital, Kfar-Saba and Sackler Faculty of Medicine, Tel Aviv University

We report acute polyarthritis in an 86-year-old man which was initially considered to be an exacerbation of osteoarthritis. After a delay of a few days a diagnosis of septic polyarthritis as an initial symptom of infective endocarditis was made. Although rheumatic manifestations in infective endocarditis are common, septic polyarthritis is rarely seen. In our patient the course of the disease was rapidly fatal.

Malignant Eccrine Poroma

R. Silfen, A. Amir, M. Feinmesser, D.J. Hauben

Dept. of Plastic and Reconstructive Surgery, and Pathology Dept., Rabin Medical Center (Beilinson Campus), Petah Tikva

Malignant eccrine poroma (MEP) is rare and both clinical and histologic diagnosis is often difficult. Therefore, diagnosis is sometimes delayed or even incorrect. We report a case in a 70-year old man with MEP of the leg. He demonstrated typical MEP behavior and the problems of differential diagnosis.

אברהם דומב

המח' לכימיה תרופתית, ביה"ס לרוקחות, הפקולטה לרפואה, האוניברסיטה העברית, ירושלים

Limited Percutaneous Surgical Drainage in Severe Neonatal Necrotizing Enterocolitis in Low Birth Weight Prematures

Robert Finaly, Zahavi Cohen, Vadim Kapuller, Agneta Golan, Edna Kurtzbart, Abraham Mares

Depts. of Pediatric Surgery and Neonatology, Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

The usual treatment of complicated neonatal necrotizing enterocolitis (NEC) is resection of the necrotic bowel, lavage of the peritoneal cavity and diversion enterostomy. Low-birth-weight premature neonates with this condition are in special danger if general anesthesia and full exploratory surgery is contemplated.

A relatively simple alternate procedure is percutaneous insertion under local anesthesia of a soft abdominal drain, most often in the right lower quadrant. The procedure is done in the neonatal intensive care unit without moving the whole set-up to the operating room.

4 such cases have been treated within the past year. 3 were discharged home as they did not require additional surgical treatment, not having developed intestinal stenosis or obstruction. 1 recovered from the acute episode, but succumbed to a severe intraventricular hemorrhage and respiratory failure 7 days after the procedure.

Our limited but most gratifying experience, in addition to similar experience of others, encourages us to recommend this simple surgical approach in the very sick low-birth-weight premature with fulminant NEC.

Clinical, Social and Economic Aspects of Comprehensive Geriatric Assessment

Jacob Feldman, Liora Peleg, Abraham Yaretzky

Geriatric Dept., Sapir Medical Center, Kfar Saba, and Sackler Faculty of Medicine, Tel Aviv University

It is well known that maelderly patients are referred to nursing homes because of "functional decline" without being thoroughly in. We studied 9 elderly patients, all referred to hospital due to functional decline and diagnosed as follows: spinal stenosis - 2 cases, depression - 3, thyrotoxicosis -1, Parkinson -1, polypharmacy and congestive heart failure -1 patient each. Proper diagnosis and appropriate treatment prevent unnecessary hospitalization in nursing homes. Our study is meant to draw attention to this crucial aspect of geriatric medicine.

Prognostic Implications in Pediatric Head Injuries

Gideon Paret, Ron Ben Abraham, Susana Berman, Amir Vardi, Rami Harel, Yossi Manisterski, Zohar Barzilay

Depts. of Pediatric Intensive Care and of Anesthesiology and Intensive Care, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

An unselected series of 200 consecutive cases of major head trauma in children aged 6 months to 16 years, seen during 4 years, was studied. Injuries were due to road accidents (40%), falls (30.5%) and other causes (29.5%), and were assessed clinically and by cranial CT.

On admission the Glasgow Coma Score ranged from 4.72-11.65 and in addition to pupillary responses and brain stem reflexes, was a significant predictor of outcome. Brain edema, midline shift, intracranial hemorrhage and also hyperglycemia, hypokalemia and coagulopathy, were associated with poor outcome. While 17% died, 53% were discharged in good functional condition.

Early identification of clinical features related to prognosis can help the caring team provide maximal support for patient and family.

Polyneuropathy in Critical Illness

M. Mouallem, A. Adunsky, H. Semo, M. Dolgopiat

Depts. of Medicine E, Geriatrics, and Neurological Rehabilitation, Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv University

Critical illness polyneuropathy developed in 8 patients aged 22-84 years in our intensive care units. This acute polyneuropathy, predominantly axonal and motor, develops in the setting of the systemic inflammatory response syndrome and multi-organ failure. It is found in about 50% of patients treated in intensive care units for more than 2 weeks. In those who survive, neurological and functional recovery is the rule.

Laparoscopic Spermatic Vein Ligation for Varicocele in Adolescents

Zahavi Cohen, Alon Yulevich, Vadim Kapuler, Niza Newman, Abraham J. Mares

Pediatric Surgery Dept., Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

We report our initial experience with laparoscopic ligation of spermatic veins in the treatment of varicocele in adolescence. 19 boys, 13 to 18 years old, underwent this treatment between I 1997 and III 98. The varicocele was always on the left side. 12 complained of scrotal discomfort and pain, but in 7 it was found incidentally during routine medical examination by the family or school physician. The diagnosis was based only on physical examination.

There has been no morbidity related to the laparoscopic procedure and all returned to normal activity within a few days. Follow-up 2-12 months after surgery showed no varicocele in any. We conclude that laparoscopy is useful in the treatment of varicocele in adolescents.

Intrahospital Transport of Critically Ill Children

Gideon Paret, Ron Ben Abraham, Oshrat Yativ, Amir Vardi, Zohar Barzilay

Dept. of Pediatric Intensive Care and of Anesthesiology, Sheba Medical Center, Tel Hashomer and University of Tel Aviv

Prospective evaluation of intrahospittransportation of 33 critically ill children to and from the pediatric intensive care unit was conducted over the course of a month. Factors contributing to risk of transport were assessed.

There were 33 children (25 boys and 8 girls), 3 days to 15 years of age. Reasons for admission included: disease and trauma in 19, and status post operation in 11. The pretransport PRISM score was 4.84. 22 children (66.6%) were being mechanically ventilated and 10 (30.3%) were being treated with amines. Transport time ranged from 8-150 minutes. 15 of the transports (45.4%) were urgent and a special intensive care team escorted 22 (66.6%). Equipment mishaps and physiolog-ical deterioration occured in 12 (36.3%) and 11 (30.3%) of the cases, respectively. The use of amines, mechanical ventilation, longer transport time and high PRISM score were all associated with physiological deterioration on transport.

Operative Laparoscopy in Pregnancy

Eylon Lachman, Abraham Schienfeld, R. Boldes, Samuel Levin, Michael Burstein, Michael Stark

Depts. of Obstetrics and Gynecology, Misgav Ladach Hospital, Jerusalem

The development of laparoscopic surgery in gynecology and general surgery has greatly advanced over the past decade, and recently has been successfully performed in pregnancy. In the English literature we found that of the 518 cases reported (mean age 32 years) the most common was for cholecystectomy (45%), followed by operations on the adnexae (34%), appendectomy (15%) and others (6%). To these we add 3 cases of cholecystectomy and 1 of an adnexal tumor. Of all the reported cases, 33% were performed in the 1st trimester, 56% in the 2nd and 11% in the 3rd.

This review demonstrates a definite trend to laparoscopy in pregnancy. It appears to be safe when performed by experienced surgeons, but further studies addressing safety of laparoscopic surgery during pregnancy are needed to reach definitive conclusions.

Diagnostic and Therapeutic Laparoscopy for Non-Palpable Testis

Zahavi Cohen, Niza Newman, Edna Kurzbart, Vadim Kapuller, Abraham J. Mares

Dept. of Pediatric Surgery, Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

From January 1994 to July 1997, 20 boys 1.5-13 years of age underwent laparoscopic examination and treatment for non-palpable testis. In 1 there was bilateral nonpalpable testis. Only 3 had intra-abdominal testes viable for orchiopexy. In 8 atrophic testicles were removed and in 9 laparoscopic examination revealed intra-abdominal blind ending of the spermatic cord and no testicular tissue.

We conclude that laparoscopy is a useful and safe technique for accurate diagnosis and may avoid additional intervention in treating non-palpable testes. Furthermore, intra-abdominal testes may be managed laparoscopically under the same anesthetic.