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.

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.

Cryptococcal Meningitis Following Cryptococcal Pneumonia in an Immunocompetent

N. Reichman, M. Elias, R. Raz, E. Flatau

Dept. of Internal Medicine B and Infectious Disease Unit, HaEmek Hospital, Afula and Rappaport Faculty of Medicine, Technion, Haifa

Cryptococcal meningitis (CM) is common in the immunocompromised (especially due to AIDS), but also occurs in immunocompetent subjects. CM can complicate cryptococcal pneumonia (CP) not only in the immunocompromised but also in the immunocompetent. We describe a healthy 26-year-old man who developed a prolonged lung infection. Diagnosis of cryptococcal pneumonia was established from bronchoscopic washings. He recovered spontaneously, so no antifungal treatment was given.

4 months later he was admitted with cryptococcal meningitis and was treated successfully with amphotericin B. An extensive immunologic study revealed no abnormalities. Since CM can complicate cryptococcal pneumonia, it is recommended that patients with CP be followed, even if recovery is apparently complete.

Knowledge, Attitudes and Training of Family Physicians with Regard to Addiction to Illicit Drugs

Aya Peleg, Roni Peleg, Pesach Shvartzman

Health Promotion and Disease Prevention Unit, and Dept. of Family Medicine, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Substance abuse is one of the most serious problems in Israel. Recent studies show the situation is getting worse. The deleterious outcomes of psychoactive substance abuse include crime, car accidents, physical and mental illness, violence and work injuries.

48 family physicians in the Negev completed a questionnaire which included training, diagnostic and treatment skills, attitudes, knowledge and need for training in this field. 38 family physicians (81%) had had no training, most (96%) indicated the importance of such training. 34 (71%) said that their medical skills could not cope with the problems of addiction, and 29 (64.5%) claimed that the quality of care of addict patients is reduced because of lack of knowledge and diagnostic skills. These findings support the conclusion that efforts should be invested in training family physicians in the field of psychoactive drugs, licit and illicit.

Ferritin in Adult Still's Disease

E. Flatau, N. Reichman, N. Kaufman

Dept. of Medicine B, Central Hospital of the Emek, Afula and Technion Faculty of Medicine, Haifa

Adult-onset Still's disease (AOSD) is characterized by a spiking fever and diverse clinical findings; the diagnosis is often delayed for months or even years. The only positive laboratory finding is neutrophilic leukocytosis. Since 1987, the diagnostic importance of elevated serum ferritin levels has been discussed in numerous papers, but is not yet among the diagnostic criteria for AOSD. We describe a case in which the finding of extreme hyperferritinemia enabled prompt diagnosis and treatment.

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.

Multilocular Cystic Mass of the Kidney: A Diagnostic Challenge

Judy Kleinmann, Simon Negelev

Urology Dept., Assaf Harofeh Medical Center, Zerifin

Multilocular cystic masses of the kidney present a diagnostic challenge because they may be malignant. Renal cell carcinoma with multilocular cysts has been reported. We present 4 cases in which the preoperative differential diagnosis was between a benign and a malignant multilocular cystic kidney mass.

Our results, combined with those of 33 previously reported cases, reveal that only 32% of cases were diagnosed correctly preoperatively. In 21% of these patients the preoperative clinical evaluation did not correctly diagnose malignancy: 24% of them yielded false negative and 21% false positive results. Angiography was more accurate than CT, US or aspiration of cysts. Frozen section was inaccurate in 55% of cases.

There is no reliable diagnostic test to distinguish between a malignant and a benign multilocular cystic mass. Invasive angiography is more accurate than other modalities. A therapeutic decision has to be made between nephron-sparing and radical surgery in each case. When nephron-sparing surgery is considered, the possible need to perform radical nephrectomy in second-look surgery must be taken into account.

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.

The New Family Physician

Eliezer Kitai, Avraham Sandiuk, Michael Weingarten, Shlomo Vinker

Dept. of Family Medicine, Rabin Medical Center (Beilinson Campus), Petah Tikva and Sackler School of Medicine, Tel Aviv University

The content of all consultations of a family physician during the first 2 months in a new practice and again in the same 2 months, 2 years later were analyzed. The workload did not change between the 2 periods, but in the first period there were relatively more men over the age of 60 years, and a more marked predominance of women in the 45-59 year age-group.

There were more follow-ups of pre-existing conditions during the first period, but in both periods the main reason for visits was an acute condition; a quarter of visits required only simple counseling without further investigation or prescribing of medication. During the first period 9% of visits were for the explicit purpose of getting to know the new physician, usually by women.

Thus the initial period in a new practice is not excessively burdensome, but involves seeing a number of patients who come either simply to get to know the new doctor, or to present non-chronic problems without expectation of further investigation.

Severe Heat Stroke in an Intensive Care Unit

Amir Halkin, Dina Lev, Oded Szold, Philip Bidermann, Sarah Bulocnic, Pinchas Halpern, Patrick Sorkine

Depts. of Medicine and Surgery, and Intensive Care Unit, Tel Aviv Medical Center

During the August 1998 heat wave in Tel Aviv we admitted many patients for acute heat-related illness; 6 had severe heat stroke and were admitted in critical condition. We describe their clinical courses during the first 5 days of hospitalization, including response to treatment and implications for future management of this disorder.

The mean APACHE II score of the 6 was 30±3.5 and mean Glasgow Coma Scale rating 3.5±0.5; they were in hypovolemic shock and respiratory failure, necessitating mechanical ventilation. Despite early effective therapy (core temperature in all was reduced to less than 398C in less than 1 hour), there was 1 death (mortality 15%) and 4 required further intensive care for life-threatening multiple organ failure.

During severe heat waves a significant number of referrals for acute heat-related illness must be anticipated, possibly overwhelming admission capacity of regional intensive-care units. Severe heat stroke complicated by multi-organ failure is not necessarily related to prior physical activity. Although important in determining prognosis, early treatment does not prevent severe complications. Mechanisms regulating body heat may remain disturbed for days following early treatment and apparent stabilization, mandating continued hospitalization.

The Israeli Pharmaceutical Market

Segev Shani, Joshua Shemer

Pharmaceutical Policy and Economics Unit, Israeli Center for Health Technology Assessment in Health Care, Gertner Institute for Epidemiology and Health Policy Research, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

The pharmaceutical market in Israel has undergone many major changes over the past 2 years. We detail measures taken with regard to drug prices and utilization, from 1992 until 1995, when the National Health Insurance Law was implemented. This provides an information base for marketing and dispensing of drugs, economic planning in the public health system, and for physicians and pharmacists in clinical practice.

The national expenditure on pharmaceuticals in 1994 was 9.4% of the annual national health expenditure. From this fact, and the number of registered drugs and the number of clinical trials conducted, it appears that our pharmaceutical market is quite diversified and well developed compared to other markets in the western world. This size advantage enables our sick funds to purchase drugs at much lower prices than the private sector.