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.

Congenital Syphilis: Need for Adequate Antenatal Care

Mark Friedberg

Pediatrics B Dept., Soroka Medical Center, Beer Sheba

Congenital syphilis is well-known and treatable with penicillin. Diagnosis in the neonate and young child may be difficult and consequently morbidity and mortality can be high. Prevention in children is of utmost importance and can be achieved by proper antenatal care and adequate follow-up of pregnant women. This includes identification of pregnant women at risk for contracting syphilis. The case presented demonstrates this need.

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.

One-Stage Surgery for Hirschsprung's Disease in Children

Mark Weinberg, Baruch Klin, Itzhak Vinograd

Dept. of Pediatric Surgery, Assaf Harofeh Medical Center, Zerifin, and Sackler School of Medicine, Tel Aviv University

Traditionally Hirschsprung's disease has been treated by 2-or 3-stage procedures. During the past 6 years a 1-stage Duhamel procedure without stoma has become our treatment of choice for Hirschsprung's disease in neonates and young infants. Over a 6-year period, 15 infants and children with colonic Hirschsprung's disease were treated with the 1-stage Duhamel retro-rectal pull-through procedure without a stoma, with the Lester-Martin modification. All patients had the usual short segment aganglionosis, but 1 had a long segment which included the splenic flexure.

Early complications included wound infection in 1 and minor rectal bleeding in 3. Late complications included constipation in 1 and enterocolitis in 4. Long-term functional results were very good in all those operated except for 1 with rectal achalasia.

We conclude that Hirschsprung's disease can be successfully treated with a 1-stage pull-through operation, the child usually benefitting from the shorter hospital stay and the avoidance of a colostomy.

Computerization in a Community Health Service Provider

Boaz Porter

Maccabi Health Services, Beer Sheba

As the second largest health service provider organization in Israel, we have been progressively computerized. The process was begun in 1988, focusing on improving administrative and financial processes.

Today there is a single centralized database for 6,000 users. The system monitors member eligibility, accounting procedures and clinical processes, including diagnoses, laboratory tests, imaging procedures and drug-prescribing. The potential of the computer for physician support is now being realized through integration of clinical guidelines and reminder systems into the computerized clinical record. In addition, the centralized database is used for quality improvement, facilitating cost-effective drug-prescribing and efficient use of technology.

The establishment of a computerized working environment for 2,000 physicians and 4,000 other healthcare workers serving 1.3 million patients is a unique model for the development of community health services. Data regarding demographics, disease patterns, drug-prescribing, use of new technology and costs are now readily available to all, from senior management to the individual physician in independent practice.

The computer revolution has also presented a new set of problems such patient-record confidentiality and the effect of the computer on the physician-patient encounter.

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.

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.

Evaluation of Clinical Findings in Occupational Health Examinations

Rafael Carel, Eynat Scheiner

Dept. of Occupational Medicine, Kupat Holim, Negev Region and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Findings in occupational health examinations of a regional service are analyzed. The service provides pre-employment, surveillance and work-capacity evaluations for about 150,000 workers from many types of work-sites and occupations. The average number of pre-employment examinations (during 1993-97) was 4,800/year, and there were relevant pathological findings in about 6.6%. Most findings involved the respiratory (11%) and cardiovascular systems (10%) or eyes (10%).

The average annual number of surveillance system examinations was 10,750. There were relevant findings in about 16%, mainly of the respiratory system (17%) and noise-induced hearing damage (about 80%).

Work-capacity examinations constituted about 35% of the work-load (9,250 examinees/year). In 53% a significant health problem affecting an individual's job performance was identified. Pathological findings were found in the musculoskeletal (21%), cardiovascular (7.5%), respiratory (3%), and neurological systems (2%).

Occupational health examinations can identify areas in which prevention and intervention or cooperation with other medical specialties are indicated, as well as specific topics requiring further training of occupational physicians.

Burns in Children in Israel: Epidemiology, Prevention and Treatment

Arieh Eldad

Medical Corps, Israel Defense Forces and Burns Unit, Hadassah University Hospital, Ein Kerem, Jerusalem

45% of all hospitalized burn casualties in Israel are children younger than 16 years old. In various hospitals they make up 30-60% of all burn casualties, depending on the proportion of children in the area of hospital intake, social and economic factors and the type of hospital. Length of hospitalization of children is shorter than that of the general population (7.3 vs 9.0 days). Scalding is the main cause of thermal injuries among babies and infants (70%), while fire burns are the most common causes among adolescents (56.5%); 90% of babies are injured at home; only 40% of adolescents are burned in home accidents.

In Israel, burned children are treated in 25 different hospitals and in departments of pediatric surgery, plastic surgery, general surgery, pediatrics or burn departments. There is no pediatric burn unit in Israel.

Tension-Free Vaginal Tape: A New Surgical Treatment of Female Urinary Stress Incontinence

M. Neuman, B. Zuckerman, M. Zilberman, A. Farkash, U. Beller

Division of Gynecologic Surgery and Oncology, Shaare Zedek Medical Center, Jerusalem

Female urinary stress incontinence diminishes the quality of life of about 5% of women. It is usually dealt with by surgery to correct the relaxed pelvic floor, the cause of the incontinence.

Tension-free vaginal tape is used in a newly described procedure. It consists of the vaginal introduction of a prolene needle-guided mid-urethral sling. The procedure is easy to perform under local anesthesia, recovery is rapid, and results are similar to those of other effective operations.

We report 44 patients who underwent this type of surgery. There were no significant complications. The early results were good and although the follow-up has been short, we believe that experience with this operation will play an important role in the treatment of urinary stress incontinence.

Plication of Diaphragm for Postoperative, Phrenic Nerve Injury in Infants and Young Children

Yael Refaely, David A. Simansky, Michael Paley, Alon Yellin

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

Paralysis of the diaphragm may cause life-threatening respiratory distress in infants and young children because of paradoxical motion of the affected diaphragm and contralateral shift of the mediastinum during expiration. Phrenic nerve injury (PNI) may follow chest operations.

10 children with diaphragmatic paralysis and severe respiratory distress underwent plication of the diaphragm. Ages ranged from 14 days to 5 years. 9 had PNI after operations for congenital heart disease and 1 after resection of an intraspinal cervical lipoma. The right side was affected in 7, the left in 3.

Indication for surgery was inability to wean from mechanical ventilation, which had ranged from 11 to 152 days (median 35). 8 underwent plication via a thoracic approach and 2 via an abdominal approach. There were no complications directly related to the operation.

The interval from plication to weaning from mechanical ventilation ranged from 2 to 140 days (median 4). 1 patient died 2 hours after plication due to severe heart failure and 2 after prolonged hospitalization due to sepsis and multi-organ failure. 6 were extubated 2-8 days (median 4) after plication and 1 only after 40 days.

Early diaphragmatic plication is simple and avoids more serious surgery. While effective in ventilator-dependent infants and young children, it should not be used in those with multi-organ failure. Early plication may prevent the complications of prolonged mechanical ventilation.

When are Menopausal Symptoms Psychiatric?

Helen Shoenfeld

Talbia Mental Health Center, Jerusalem

Nervousness, sleep disorders, mood instability and sexual dysfunction are frequent symptoms during menopause. It is commonly believed that they are due to the characteristic menopausal hormonal changes. However, they also commonly occur in psychiatric disorders unrelated to the menopause. The literature deals with the characteristic psychological aspects and studies have examined the ways in which menopausal phenomena are related to the hormonal background and its effect on the brain.

The clinical menopausal picture may be complicated by secondary psychiatric disease. Also, menopausal symptoms and symptoms of previous psychiatric disease may coexist. In addition, menopause may precipitate psychiatric disorders in women predisposed to them.

Accumulated personal myths and expectations related to the menopause are likely to affect the way in which a woman copes with it. Important factors in this connection include education, culture and certain life events. In all such cases precise evaluation of the etiology, with its intermingled gynecological and psychiatric factors is required. Such women should be treated by both a gynecologist and a psychiatrist.

We present 5 cases, 4 of which were sent to the menopause clinic and were then referred for psychiatric evaluation and treatment. The other case presented at a psychiatric clinic due to a first acute psychostate. Laboratory tests showed typical changes of menopause.
 

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.

Equine Rhodococcus Equi Pneumonia: First Report in Israel and its Significance for Man

A. Steinman, G.A. Sutton, D. Elad

Koret School of Veterinary Medicine, Hebrew University of Jerusalem; and Kimron Veterinary Institute, Beit Dagan

Rhodococcus equi is an important pathogen in young horses. In recent years it has been increasingly recognized as an opportunistic infectious agent in patients with immune deficiency. The increase in recognized cases may be related to the increased prevalence of AIDS. However, more cases may have been recognized lately due to increased awareness of the pathogenicity of this bacterium. Based on medical reports, there appears to be an association between Rhodococcus equi infections and exposure to animals, horses in particular.

During the past year, 2 cases of rhodococcus pneumonia were diagnosed in foals in Israel. This information deserves the attention of the local medical community because of the zoonotic potential of Rhodococcus equi and the danger of its becoming more common. The climate in Israel is conducive to its growth and viability and since it flourishes in an environment with horses, the significant growth in our equine industry in recent years may contribute to an increase in Rhodococcus infections.

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.