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.

Endoscopic Parathyroidectomy - Preliminary Experience 

Joseph Kuriansky, Laureano Fernandez-Cruz

Dept. of General Surgery and Transplantation, Sheba Medical Center and Sackler School of Medicine, Tel Aviv University; and Dept. of Surgery, Hospital Clinic, University of Barcelona

Since the advent of extraperitoneal approaches for laparoscopic surgery, the creation of extraperitoneal virtual spaces has spread rapidly. We describe our preliminary experience with selective neck exploration and endoscopic parathyroidectomy in 4 women, aged 57-74, with primary hyperparathyroidism.

Preoperative localization of parathyroid adenoma was assessed by technetium-sestamibi scanning and cervical ultrasonography. A cervical work space was created by the introduction of a trocar with an inflatable balloon-tip and maintained with low-pressure CO² insufflation. All procedures were completed endoscopically. There were no intraoperative complications. Mean operative time was 40 minutes and cosmetic results were very satisfactory.

We found endoscopic parathyroidectomy a feasible and safe option and particularly appropriate for the surgical treatment of primary hyperparathyroidism. Further refinements in technique will enhance its practicability in exploring the mediastinum for ectopic parathyroid glands and in those with secondary hyperparathyroidism.

Extraperitoneal Laparoscopic Adrenalectomy

J. Kuriansky, A. Saenz, D. Rosin, E. Astudillo, L. Fernandez-Cruz

Dept. of General Surgery and Transplantation, Sheba Medical Center, Tel Hashomer, and Dept. of Surgery, Hospital Clinic, Barcelona

Laparoscopic adrenalectomy has recently been shown to be safe and effective for a variety of benign adrenal tumors, such as aldosterone adenoma, pheochromocytoma, nonfunctioning adenoma, Cushing's adenoma and Cushing's syndrome. Recently, laparoscopic surgeons adopted an extraperitoneal approach in which a space is created by the introduction of a trocar with an inflatable balloon tip. This technique with a minimal access procedure provides direct access to the adrenal glands without interfering with intraperitoneal organs.

Laparoscopic adrenalectomy using the extraperitoneal approach was completed in 18/20 consecutive patients. Mean operative time was 95 min (range 80-120) and median hospital stay 3 days. Blood transfusion was not required, and there were no postoperative complications.

Thoracoscopic Splanchnicectomy for Intractable Pancreatic Pain

Joseph Kuriansky, Alejandro Saenz, Laureano Fernandez-Cruz

Dept. of Surgery, Sheba Medical Center, Tel Hashomer, and Sackler School of Medicine, Tel Aviv University; and Dept. of Surgery, Clinic Hospital, Barcelona

Intractable pain is the most distressing symptom in nonresectable pancreatic carcinoma and in chronic pancreatitis. Recently, thoracoscopic splanchnicectomy has been advocated as a minimally invasive method of pain control in these patients. Between May 1995 and April 1998, 24 patients with nonresectable pancreatic cancer and 4 with chronic pancreatitis, underwent 43 thoracoscopic splanchnicectomies. All suffered from intractable pain, were opiate-dependent and unable to perform normal daily activities. Unilateral left splanchnicectomy was done in 13 and bilateral in 15.

All procedures were completed thoracoscopically. Operative time ranged from 25 to 60 min and mean hospital stay was 3 days. Median pain intensity was reduced by 50% in 24 and no further narcotics or analgesics were required. We found thoracoscopic splanchnicectomy a safe and effective procedure for intractable pancreatic pain.

Alpha-Amino Adipic Aciduria: a Rare Psycho-Motor Syndrome

Ben-Ami Sela, Tamar Massos, Dov Fogel, Joseph Zlotnik

Pathological Chemistry and Child Development Institutes, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

A 3.5-year-old boy with developmental motor retardation, hypotonicity, and severe speech disturbance had alpha-amino adipic acid in his blood and very high levels in his urine. In only 20 cases has this catabolite of lysine and hydroxylysine been found in high concentrations in urine, due to enzymatic block.

The clinical features associated with alpha-amino adipic aciduria may include mental retardation, developmental and motor delay, learning difficulties, convulsions, speech problems and ataxia. 3 siblings had milder symptoms of psychomotor delay and intermediate degrees of alpha amino-adipic aciduria, suggesting that the described developmental deficits could be related to this metabolite or its derivatives.

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.

Tension-Free Vaginal Tape Procedure for Female Stress Incontinence

David Gordon, Asnat Groutz, Joseph Lessing

Lis Maternity Hospital, Sourasky-Tel Aviv Medical Center

We evaluated the safety and efficacy of the tension-free vaginal tape procedure (TVT) in 20 consecutive women with urodynamically proven genuine stress incontinence. Mean operating time under spinal analgesia was 37.2‏13 minutes and 80% were discharged within 24 hours. There were 5 uncomplicated, intraoperative bladder perforations, but no defects in healing nor tape rejection. The objective cure rate was 95%, while the subjective rate was 100%. Although follow-up was short (9-16 months), the TVT method seems to be safe and effective for stress urinary incontinence.

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.

Violence in the Emergency Department

H. Derazon, S. Nissimian, C. Yosefy, R. Peled, E. Hay

Dept. of Emergency Medicine and Epidemiology Unit, Barzilai Medical Center, Ashkelon

There is an international epidemic of violence in the emergency department (ED) which threatens medical staff daily. The problem is underestimated in Israel and there are as yet no regulations of the Ministry of Health and the Bureau of Security and Safety that deal with the problem.

At the beginning of 1997 we conducted a retrospective survey to estimate the extent of this problem and to define its causes and the various options for management. An anony-mous questionnaire was given to all permanent workers of our ED: physicians, nurses and reception clerks, as well as physicians who worked in the ED during evening and night shifts at least twice a week.

74% of (questionnaire) responders experienced violent events, most of them 5 or more times during the previous 2 years. Violence was experienced by 90% of nurses, 70% of physicians and 64% of clerks. The main reason for violence was prolonged waiting in the ED. Other causes were dissatisfaction with treatment, refusal to leave the ED, and language that displeased the patient.

Most violent patients were middle-aged men, of whom alcohol and drug users were only a small proportion. Most victims of physical violence called hospital security personnel, but only a third of the victims of verbal violence pressed charges. Half of the staff who were physically attacked called the police and most pressed charges. Only 2 attackers were convicted; charges were dropped against 3 because of "lack of public concern." We were unable to ascertain the results of the other charges. Most victims of violence didn't press charges because of fear resulting from threats of the patient and/or family.

The violent patient was usually characterized by responders as a middle-aged man, sober, of low socioeconomic level, impatient, with a bad previous hospital experience, dissatisfied with treatment and who insisted on being admitted to hospital.

Suggestions for violence management by questionnaire- responders included 24-hour police protection and a training program in violence management for hospital security and medical staff.

Multicenter Community-Based Trial of Amlodipine in Hypertension

C. Yosefy, J.R. Viskoper, Y. Leshem, Y. Rav-Hon, G.I. Rosenberg, E. Yaskil

(Representing the 39 Investigators of Project AML-IL-95-001, WHO Collaborative Center for Prevention of CV Diseases) Ben-Gurion University of the Negev, Beer Sheba; Barzilai Medical Center, Ashkelon; Hypertension Clinic, Kupat Holim Afula; Statistics Consulting Unit, Haifa University; and Promedico Ltd., Petah Tikva

The safety and efficacy of Amlodipine (AML) for mild to moderate hypertension was evaluated in a "real life" setting. This open non-comparative trial included 123 men and 143 women (age 30-91 years, mean 59.4). All had sitting diastolic blood pressure (DBP) between 95 and 115 mmHg, confirmed in most by 2 baseline measurements, 2 weeks apart.

Eligible patients were given AML 5 mg daily as add-on or monotherapy and were evaluated 4 weeks later. If DBP was then >90 mmHg, the daily dose was raised to 10 mg; those with <90 mmHg remained on 5 mg. AML was continued for 8 weeks. Other BP-lowering drugs were unchanged.

Of the original 266 patients 22 (8.2%) withdrew due to adverse events (AE), and others were protocol violators, lost to follow-up or withdrew, leaving 211 available for efficacy analysis. In this major group BP was reduced from 165±15/101±4 to 139±11/83±5 after 12 weeks of AML (p<0.05). The reduction was greater in those under 70 years, from 173±12/100±5 to 142±12/80±4 (p<0.05). In those with BMI>30 kg/m², BP decreased from 165±15/101±5 to 140±12/83±5 (p<0.05).

Mean change in heart rate was -1.5 bpm (p<0.05). Mean final AML dose was 5.5 mg/day. The most common AML-related AE requiring cessation of the drug was pedal edema in 2.6% of the 266 patients; in 3.7% it persisted during therapy. Other AE occurring in >1% were dizziness in 1.8%, headache 1.5%, flushing 1.1% and fatigue 1.1%.

We conclude that AML is an effective and well-tolerated antihypertensive suitable for most hypertensive patients.

Mesalamine-Induced Hyper- Sensitivity Pneumonitis

D. Zamir, J. Weizman, C. Zamir, Z. Fireman, P. Weiner

Dept. of Medicine A and Gastroenterology Unit, Hillel Yaffe Medical Center, Hadera and Hadera Subdistrict Health Office

A 23-year-old woman was admitted with a history of 2 weeks of cough, fever and bilateral lung infiltrates. She had been diagnosed 2 months before as having ulcerative proctitis and was treated with mesalamine, which induced a full remission, but 3 antibiotic regimens failed to improve her lung disease.

Since computerized tomography revealed bilateral peripheral lung infiltrates and her eosinophile count was elevated, the diagnosis of drug-induced eosinophilic pneumonia was suggested. Mesalamine and antibiotics were stopped and oral corticosteroids begun. She became almost asymptomatic a week after mesalamine withdrawal, and the x-ray became normal.

Laparoscopic Donor Nephrectomy: Initial Experience

Amir Szold, Joseph M. Klausner, Richard Nakache

Advanced Endoscopic Surgery Unit, Transplantation Unit and Depts. of Surgery B-C, Tel Aviv-Sourasky Medical Center and Sackler Faculty of Medicine, Tel Aviv University

We present our initial experience in laparoscopic nephrectomy. 6 patients were operated for a kidney donation and 2 for treatment of a benign disease. All procedures were completed successfully, with no conversion to laparotomy or intra-operative complications. Mean operating time was 210 minutes, and in the donor kidneys the mean warm-ischemic time was 165 seconds. There were 3 postoperative complications, and mean hospitalization was 3.5 days. The transplanted kidneys are all functioning.

From our initial experience, laparoscopic nephrectomy appears to be both feasible and safe. Its implementation requires a combined team with experience in donor surgery and advanced laparoscopic skills. The procedure may increase the availability of living donor kidneys, due to the smaller impact on the donor compared to conventional donor nephrectomy.

Effect of Specific Inspiratory Muscle Training on Dyspnea and Exercise Tolerance in Congestive Heart Failure

Paltiel Weiner, Joseph Waizman, Rasmi Magadle, Noa Berar-Yanay, Benny Pelled

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

It has been shown that the inspiratory muscles of patients with congestive heart failure (CHF) are weaker than normal. This weakness may contribute to dyspnea and limit exercise capacity. But respiratory muscles can be trained for increase in both strength and endurance. This study was designed to evaluate the effect of specific inspiratory muscle training (SIMT) on muscular performance, lung function, dyspnea and exercise capacity in moderate heart failure.

10 patients with CHF (NYHA functional class II-III) received 1/2 hour of SIMT daily, 6 times/week, for 3 months. They started breathing at a resistance 15% of their Pimax for 1 week and the resistance was then increased incrementally to 60%. Spirometry, inspiratory muscle strength and endurance, and the 12-minute walk test were performed before and after the training period. All showed an increase in inspiratory muscle strength and endurance. This was associated with a small but significant increase in FVC, a significant increase in the distance walked (458±29 to 562±32 m, p<0.01), and improvement in the dyspnea index score.

SIMT resulted in increased inspiratory muscle strength and endurance. This increase was associated with decreased dyspnea and an increase in submaximal exercise capacity. SIMT may prove to be useful complementary therapy in CHF.

Treatment of Retroperitoneal Sarcoma

Dina Lev-Chelouche, Subhi Abu-Abeid, Mordechai Gutman, Yoram Kluger, Moshe Michovitch, Isaac Meller, Moshe Inbar, Joseph M. Klausner

Depts. of Surgery B, Orthopedic Oncology and Oncology, Tel Aviv - Sourasky Medical Center (Affiliated with Sackler Faculty of Medicine, Tel Aviv University)

Soft tissue sarcomas are exceedingly rare, making up less than 1% of all solid malignancies. In the retroperitoneum, they tend to be large when diagnosed and are a therapeutic challenge to the surgical oncologist. Our experience with 51 patients with retroperitoneal sarcomas operated on during the past 4 years is presented. 37 were primary and 26 presented as recurrent tumors. The group included many different histological sub-types, the majority being high grade tumors.

Complete resection was achieved in 84%, necessitating extensive surgery, but was not possible in 8 patients (16%) who underwent partial resection or biopsy only. There was 1 perioperative fatality (2%). 18 (35%) suffered complications, all of which were reversible. The estimated 5-year survival in the complete resection group is 40%, while none of those who underwent partial resection survived more than 2 years. There was significantly better survival in patients with primary, low grade sarcomas which were smaller than 8 cm, compared to those with high-grade, recurrent sarcomas larger than 8 cm. Local recurrence developed in 8 patients of the complete resection group (18%), 2 months to 3 years after surgery.

These data show that despite the concept of retroperitoneal sarcomas as being aggressive, invasive tumors with a poor prognosis, the prognosis is not unusually bad. With proper surgical technique, resectability may be high, with improved overall survival.

A-V Junctional Arrhythmia Due to Oral Phenytoin Intoxication

Rasmi Magadle, Paltiel Weiner, Joseph Waizman

Dept. of Medicine A, Hillel Yaffe Medical Center, Hadera

Phenytoin (Dilantin) is the drug of choice for most of the convulsive disorders. However, even when given orally it causes many adverse reactions, mainly affecting the nervous system, both central and peripheral. When administered intravenously, severe cardiovascular reactions may occur, including conduction disorders, sinus arrest, atrioventricular block or hypotension.

Medline screening did not reveal previous reports of arrhythmias induced by oral phenytoin. We present a case in which oral phenytoin administered chronically for a convulsive disorder caused atrioventricular junctional arrhythmia.