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.

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.

Can Police Car Flashing-Light Induce Encephalographic Discharges and Seizures?

G. Vainstein, V. Yofe, N. Gadoth

Dept. of Neurology, Meir General Hospital, Sapir Medical Center, Kfar Saba

The new police car flashing-light device (930 Heliobe Lightbar) has recently been implicated as potentially epileptogenic. We exposed 30 epileptic patients, 30 nonepileptic patients who suffered from headache and 15 normal volunteers to this light source. All had routine EEGs with standard intermittent photic stimulation, followed by 3-minute stimulation with the Lightbar. In none were either seizures or EEG changes induced. In 1 epileptic spike-and-wave activity induced by standard photic stimulation was enhanced with the Lightbar.

We could not confirm that the Lightbar is epileptogenic.

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.

Dose-Intensive Chemotherapy with Continuous Infusion 5-Fluorouracil

T. Tichler, E. Ghodsizade, A. Katz, P. Rath, R. Berger, H. Brenner

Sheba Medical Center, Tel Hashomer, and Beilinson Medical Center, Petah Tikvah

54 patients with advanced malignancy refractory to chemotherapy were studied to evaluate efficacy and toxicity of continuous infusion of 5-fluorouracil (5FU) given for 3 weeks. We report results of the first 156 courses given in combination with other drugs.

19 (37%) of the 54 responded, including 3 (6%) with complete response. Toxicity was acceptable, with mucositis in 13 (26%) and 3 (6%) with grade II-III toxicity. Results and toxicity profile were compatible with further disease-oriented studies using this dose-intensive program.

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.

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.

Multiple Organ Damage due to Cholesterol Embolization

Ido Wolf, Meir Mouallem

Dept. of Medicine E, Sheba Medical Center, Tel Hashomer, and Sackler Faculty of Medicine, Tel Aviv University

Cholesterol crystal embolization can affect multiple organ systems and mimic other systemic diseases. We describe a 65-year-old woman who had renal failure, diarrhea, transient ischemic attacks and purple toes due to spontaneous cholesterol crystal embolization.

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.

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.

Patient Satisfaction and Hospital Services Evaluation by Regular and Private Patients

Sara Carmel, Jonathan Halevy

Dept. of the Sociology of Health, Faculty of Health Sciences, Ben-Gurion University of

the Negev, Beer Sheba; Sha'are Zedek Medical Center and Hebrew University-Hadassah Medical School, Jerusalem

"Sharap" is a private medical service integrated within our public clinic and hospital services. Clients may choose their physician by paying a fee in addition to what their health insurance agency (Kupat Holim) pays for. All other hospital services are supplied to all patients alike. The main purpose of this study was to evaluate the extent to which this declared policy is maintained in practice.

During 5 months in 1997, 198 Sharap patients and 198 regular patients were interviewed in the the general surgery, cardiac surgery, ENT, cardiology, newborn and gynecology wards of this hospital. Both groups were similar in regard to cause of hospitalization, gender, age group (10-year age ranges), and length of hospitalization (at least 24 hours).

Similar levels of satisfaction with hospitalization in general and with the nursing service and with supportive services were found in both groups. However, Sharap patients were more satisfied with their physicians than regular patients (87% vs 74%, respectively). Similar results were also found using indirect measures of satisfaction. About 86% in both groups reported having achieved the goal of improvement in health. A high proportion of respondents from both groups (82% and 88%, respectively) could not distinguish between Sharap and regular patients in the ward. However, a greater proportion of regular patients (35% vs 21%) wanted more extensive explanations from their physicians regarding their treatment.

Sharap patients belonged to higher socio-economic classes than regular patients. Our evaluation indicates that although the Sharap service enables the affluent to choose their preferred physician, resulting in a different doctor-patient relationship, the service does not create a significant feeling of discrimination among hospitalized patients, and does not interfere with the high level of health services available to the public at large.

Low Prevalence of Hepatitis G Infection in Dialysis Patients

Doron Zamir, Shimon Shtorch, Chen Zamir, Zvi Fireman, Hilkiau Zonder

Internal Medicine Dept. A, Liver Clinic, Dialysis Unit and GI Unit, Hillel Yaffe Hospital and Hadera Subdistrict Health Office

Prevalence of hepatitis G virus (HGV) infection in the general western population ranges from 0.2-1.5%. In high-risk groups, such as patients with chronic liver disease, hematologic disorders and drug addicts, prevalence is as high as 10%-15%. Dialysis patients have increased rates of HGV infection (6%-50%).

We evaluated prevalence of HGV infection among dialysis patients, and the association between HGV infection and hepatitis C virus (HCV) infection. Serum samples were screened for HGV infection by RT-PCR. Screening for HCV infection was performed by an EIA test and confirmed by RIBA and RT-PCR for HCV. Sera were also tested for HBV markers.

The study group included all 78 hemodialysis patients and 7 of the 12 peritoneal dialysis patients in our unit during September to November 1997. 4 (5.2%) were HGV-positive but none were peritoneal dialysis patients. 1 of the 12 HCV-positives was also infected with HGV. HGV infection was not associated with duration of dialysis, number of blood transfusions or levels of transaminases.

Prevalence of HGV infection among our hemodialysis patients was low (5.2%), but higher than reported for the general population. Prevalence of HGV/HCV infection in hemodialysis patients was low and unrelated to duration of dialysis, number of blood transfusions and levels of transaminases.

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.