תוצאת חיפוש

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.

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.

Premature Birth and Cognitive Functioning in Adolescence

Ofra Lubetzky, Avi Weitzman, Izhak Gilat, Samuel Tyano

Levinsky College of Education and Geha Hospital, Tel Aviv and Sackler Faculty of Medicine, Tel Aviv University

Premature infants are considered a high-risk population for developing cognitive dysfunction. Studies have indicated lower cognitive performance among elementary school children born prematurely. We focused on cognitive functioning of such adolescents. This age was chosen because of its critical importance in the development of the individual.

50 adolescents aged 14-16 years born prematurely were compared with 50 born at full-term and matched for gender, age and socioeconomic status. All subjects attended regular schools and did not suffer severe neurological disorders. Cognitive functioning was measured by the Bender-Visual Motor Gestalt Test and by 3 subtests from the Wechsler Intelligence Scale for Children (revised WISC-R test).

Results revealed that prematurely born adolescents scored lower than those born at term on all measures of cognitive performance. The results are discussed in terms of their developmental meaning and of therapy for the prematurely born.

Retinal Lesion due to Excessive Exposure to Sunlight

Irene Krasniz, Itzchak Beiran, Benjamin Miller

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

Retinal damage caused by direct exposure to the sun's rays is well recognized by the ophthalmic community. Although functional ability in solar retinopathy is usually regained within weeks, some suffer long-term visual impairment. Anatomic damage to the retina, even in those who regain full vision, is permanent. We describe 2 cases of solar retinopathy, 1 of which remained with permanent loss of vision. The role of medical education in preventing damage from solar retinal hazards is stressed.

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.

אברהם דומב

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

Measuring Residual Urine by Portable Ultrasound Scanner

Santiago Richter, Rachel Hag'ag, Moshe Shalev, Israel Nissenkorn

Urology Dept. and Outpatient Clinic, Meir Hospital, Kfar Saba and Sackler Faculty of Medicine, Tel Aviv University

Urethral catheterization, the standard method of measuring residual urine, is uncomfortable and associated with risk of infection and trauma to the urethra. It has also been reported as inaccurate to a certain extent. We compared catheterization with ultrasound scanning in a prospective study of 52 men and 3 women.

100 measurements of postvoiding residual urine by portable ultrasound scanner, were each followed immediately by urethral catheterization (both procedures performed by an experienced nurse in our outpatient clinic). A difference of >25€ml between measurements by scanner and by catheter was considered significant.

The range of residual urine measured by scanner was 1-425 ml, and by catheter 1-410 ml. There was good matching between the 2 methods in 85 of 100 measurements (scanning accuracy 85%). In 30/85 matching was excellent while in 55 cases the mean difference was 8.5±6.2 ml, range 1-24 ml. The accuracy of scanning was 85%; there was perfect matching between the 2 methods in 30 cases. In the remaining 15 cases the mean difference was 41.8±13.6 (range 25-56).

Each catheterization took 4-5 minutes and scanning 30 seconds. There were no complications after catheterization, but all reported discomfort and dysuria for 1-2 hours thereafter. Scanning was absolutely uneventful in all.

The cost per catheterization, including medication, disposable materials and personnel time was approximately 80 NIS. Our 80-90 measurements of residual urine a month require annually about 80 hours and a budget of about NIS 80,000. Scanning requires only 8 hours, while the cost of the portable scanner is significantly less than NIS 80,000 and it can be used for more than a year.

We conclude that measuring urine residual with the noninvasive scanner instead of by catheterization is easier, more accurate, and more cost-effective.

Malignant Nodular Hidradenoma (Sweat Gland Tumor)

Anat Engel, Yaron Bar-Dayan, Santiago Engelberg, Yair Levi

Depts. of Medicine B, Pathology, and Disease Research Unit, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

Malignanhidradenoma is a very rare tumor that originates from sweat glands. We present a 61-year-old man with an ulcerated tumor in his right flank, 4 cm in diameter, that was excised with a wide free margin. Histopathologic study showed an ill-defined, epithelial neoformation, formed by lobules of clear polygonal cells in the deep dermis and subcutaneous tissue, diagnosed as malignant nodular hidradenoma.

1.5 years after excision there was enlargement of the right axillary and inguinal lymph nodes, which showed metastatic, adnexal neoplastic cells. Axillary resection and superficial dissection of the right inguinal nodes were performed. After 3 months the tumor had spread to other lymph nodes and acute obstructive renal failure required insertion of a pig-tail catheter into the right ureter. Radiotherapy was followed by chemo-therapy, but he died from end-stage metastatic disease in multi-organ failure.

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.

Laparoscopy for Common Bile Duct Stones

Ibrahim Matter, Moshe Wald, Shmuel Eldar

Depts. of Surgery and Urology, B'nai-Zion Medical Center, Haifa

We performed 75 laparoscopic cholecystectomies during July and September 1996. In 3 men and 4 women, aged 32-87 years, there was obstructive jaundice caused by choledocholithiasis. During laparoscopy in the jaundiced patients, calculi were identified by cholangioscopy and intra-operative cholangiography. They were washed into the duodenum (confirmed cholangiographically) after intravenous glucagon injections and dilation of the papilla of Vater.

Serum bilirubin and liver enzyme levels returned to normal within a few days. There was no operative or postoperative morbidity, nor any biliary-related systemic complications. Average postoperative hospitalization was 3 days.

Pelvic Floor Exercise and Biofeedback in Genuine Stress Incontinence

D. Gordon, D. Luxman, Y. Sarig, A. Groutz

Women and Children's Division, Liss Hospital, Sourasky-Tel Aviv Municipal Medical Center and Sackler Faculty of Medicine, Tel Aviv University

Stress urinary incontinence is a medical and social problem. In the past decade there has been increased awareness of this condition and the number of those affected who seek help is increasing. Treatment is usually surgical - elevation of the bladder neck. Pelvic floor exercise is an accepted conservative treatment modality used for mild to moderate cases that have not yet completed their families.

We present our results in 30 women, aged 28-71 years, av. 49% with genuine stress incontinence treated with pelvic floor exercise and biofeedback. 14 patients (46.7%) were completely cured and 15 (50%) were improved. In only 1 was there no improvement.

Our results show significant improvement in the duration and intensity of pelvic floor contractions after treatment. Pelvic floor exercise with biofeedback is a very important treatment modality, requiring a highly motivated patient and a physiotherapist specialized in pelvic floor exercise.

Malignant Lymphoma of the Bladder

Y. Mor, Z. Dotan, J.H. Pinthus, I.S. Engelberg, J. Golomb, J. Ramon

Depts. of Urology and Pathology, Chaim Sheba Medical Center, Tel Hashomer

Urinary tract lymphoma is usually reported when the secondarily stem is affected by widespread non-Hodgkin lymphoma. We describe an 83-year-old woman who presented with secondary lymphoma of the bladder 3 years after diagnosis when it initially infiltrated her breast. Treatment included local transurethral excision followed by chemotherapy, during which she died of disseminated disease.

Irradiation Injury of the Salivary Glands

R. M. Nagler

Dept. of Oral and Maxillofacial Surgery and Oral Biochemistry Laboratory, Rambam Medical Center and Technion Faculty of Medicine, Haifa

Ionizing irradiation of the salivary glands often leads to severe histological and functional alterations. Such exposure usually occurs during radiotherapy in patients with head and neck malignancy. The consequent xerostomia, often life-long, may result from even relatively low dosage irradiation, and causes a great deal of suffering. We suggest a radiobiological mechanism for this phenomenon which has been studied extensively since first described in 1911. The suggested injurious role of redox active transition metal ions and highly destructive free radicals is discussed in relation to the ultimate radiosensitive cellular target, DNA.

Exhaustion of Motor Cortex after Head Injury Revealed by Trans-Cranial Magnetic Stimulation

M. Elron, G. Levi, M. Trovnick, J.F. Soustiel, H. Hafner, A. Chistyakov, M. Feinsod

Center for Treatment and Applied Research in Head Injury, Dept. of Neurosurgery, Rambam Medical Center and Rappaport Faculty of Medicine, The Technion, Haifa

We evaluated the pattern of motor evoked potentials elicited by single-pulse and slow-rate (1 Hz) repetitive, transcranial, magnetic stimulation (RTMS) in minor head injuries. The motor response to a single magnetic stimulus in patients with minor head injury was characterized by a significantly higher threshold than in healthy subjects. However, central and peripheral motor conduction was normal in all patients. A stable pattern of MEP throughout the RTMS session was the most prominent feature in the control group. A progressive decrease in MEP amplitude and irregular alternation of large and very small MEPs over the course of RTMS was observed in minor head injury. The higher threshold of the motor response and the abnormal patterns of MEP behavior revealed by RTMS may reflect impaired excitability and enhanced exhaustion of the motor cortex in patients with minor head injuries, which improve with time.

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.