Progressive Ptosis in Children as a Presenting Sign of Kearns-Sayre Syndrome 

Ron Gal, Eli Lahat

Pediatric Neurology Unit, Assaf Harofeh Medical Center and Sackler Faculty of Medicine, Tel Aviv University

Mitochondrial encephalopathies represent a heterogeneous group of various neurological syndromes caused by defects in mitochondrial metabolism. All clinical syndromes can be subdivided by type of biochemical defect into 3 subgroups: defective oxidation, defects in pyruvate metabolism and various defects in the respiratory chain.

We present a 12-year-old girl admitted for evaluation of progressive ptosis over a period of 3 years, diagnosed as having the rare mitochondrial encephalopathy, Kearns-Sayre syndrome.

Malaria in Travelers Returning from Endemic Areas

Andre Matalon, Amos Yinnon

Kupat Cholim Klalit, Dan-Petah Tikva District, and Dept. of Family Medicine, Tel Aviv University; and Infectious Disease Unit, Shaare Zedek Hospital and Hebrew University-Hadassah Medical School, Jerusalem

2 cases of malaria in family practice are described in a 26- and a 50-year-old woman. Both probably could have been prevented had common chemoprophylactic drugs been taken. The risk of malaria is greater than the risk of possible side-effects of the drugs commonly used by travelers for prevention. Family physicians must be aware of the possibility of malaria in young people with fever, especially those who have travelled to equatorial areas and special attention should be given to encourage chemoprophylaxis.

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.

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.

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.

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.

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.
 

Familial Hemiplegic Migraine of Childhood

A. Livne, E. Lahat

Pediatric Division and Pediatric Neurology Unit, Assaf Harofeh Medical Center, and Sackler Faculty of Medicine, Tel Aviv University

Familial hemiplegic migraine is a rare autosomal, dominant, migraine subtype. It is characterized by acute episodes of hemiplegia and hemisensory deficits, and other neurological abnormalities occurring either before or together with severe headache, nausea and vomiting; episodes last several hours and then spontaneously subside. Intervals between episodes are relatively prolonged. Unless there is a relevant family history suggesting this syndrome, the diagnosis is usually delayed. Recently the gene for the syndrome was identified on chromosome 19. We report 3 boys and 1 girl, 11-15 years old with hemiplegic migraine.

Combined Sclerotherapy and Surgery for Huge Cervical Lymphangioma

Zvi Steiner, Asher Pressman, Jorge Mogilner

Dept. of Pediatric Surgery, B'nei Zion Medical Center and Faculty of Medicine, Technion Institute of Technology, Haifa

Lymphangioma is a benign developmental anomaly of the lymphatic system located in about 40% of cases in the neck and usually completely resectable. In some cases it invades adjacent structures such as the larynx, pharynx, or tongue. In such cases it is almost impossible to resect completely, as this would endanger vital structures.

We describe a baby born with a huge cervical lymphangioma which invaded the tongue, larynx, pharynx and other cervical structures. He was treated with bleomycin and aethoxysklerol. The sclerotherapy shrank the lymphangioma considerably and it became resectable. At 2 years of age the cosmetic result is good and vital function, such as swallowing and facial expression, are preserved.

Accidental Severance of a Venous Catheter: Diagnostic and Therapeutic Approach

Harel Gilutz, Aharon Gavriel, Shmuel Yurfest

Cardiology, Heart and Lung, and Vascular Surgery Depts., Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

The most common invasive procedure performed in hospitals ithe insertion of a vascular access device. This procedure has the rare complication ofcatheter emboli. Accidental cutting of a peripheral catheter, the use of duplex ultrasound to locate the cut and the extraction of the catheter through a venesection is described. If peripheral extraction fails, percutaneous extraction or thoracotomy should be tried, in that order. The immediate precautions have an impact on the final results.

ACE Gene Polymorphism in a Diabetic Cohort and Diabetic Nephropathy

Orna Levinson, Shmuel Oren, Chana Yagil, Marina Sapojnikov, Alexander Wechsler, Rosanna Bloch, Yoram Yagil

Laboratory for Molecular Medicine, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba and Barzilai Medical Center, Ashkelon

The renin-angiotensin system is thought to play an important role in the pathophysiology of kidney disease in diabetes. Previous studies have shown a possible association between the D allele of the angiotensin converting enzyme (ACE) gene, known to be associated with higher circulating levels of ACE, and increased risk of developing nephropathy in NIDDM. The present study investigated the distribution of ACE gene genotypes in the general population and patients with NIDDM, the association between the D allele and diabetic nephropathy, and the association between the ACE genotype and involvement of other target organs in NIDDM. The ACE genotype (insertion/deletion I/D) was determined in all subjects, subsequently divided into 3 groups based on their polymorphism (DD, DI and II). The presence of nephropathy was defined by an albumin-creatinine ratio of 30 mg/g or greater (mean of 2 first morning urine samples).

In the general population most had the D allele (DD or ID) and a minority the II genotype. There was no association between genotype and hypertension, ischemic heart disease, hyperlipidemia, and cerebrovascular or peripheral vascular disease. In diabetics the genotype distribution was not different from that in the general population. Within the diabetic group, there was no association between genotype and hypertension, hyperlipidemia, duration of diabetes, or HbA1C levels. Nephropathy, found in 81 of the 156 with NIDDM, was not associated with genotype. Diabetic nephropathy was not associated with retinopathy, neuropathy, or ischemic heart, cerebrovascular or peripheral vascular disease. We conclude that in the population sampled, there was no association between the D allele of the ACE gene and the risk of developing nephropathy in NIDDM.

Laparoscopic Spermatic Vein Ligation for Varicocele in Adolescents

Zahavi Cohen, Alon Yulevich, Vadim Kapuler, Niza Newman, Abraham J. Mares

Pediatric Surgery Dept., Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

We report our initial experience with laparoscopic ligation of spermatic veins in the treatment of varicocele in adolescence. 19 boys, 13 to 18 years old, underwent this treatment between I 1997 and III 98. The varicocele was always on the left side. 12 complained of scrotal discomfort and pain, but in 7 it was found incidentally during routine medical examination by the family or school physician. The diagnosis was based only on physical examination.

There has been no morbidity related to the laparoscopic procedure and all returned to normal activity within a few days. Follow-up 2-12 months after surgery showed no varicocele in any. We conclude that laparoscopy is useful in the treatment of varicocele in adolescents.

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.

Maximal Age Reflects Ignoring the Health of the Oldest in General, Geriatric, and Gerontolo- Gical Studies

Vladimir Shats

Geriatric Dept., Rebecca Sieff Government Hospital, Safed

In publications relating to the health of the elderly there are 2 ways of presenting maximal ages, collective (for example: 70+ years) and individual maximal age (for example 70 years). While enabling assessment up to a certain age, data from subjects above the maximal age stated in the research will not be included. From the literature of the past 10 years, there were selected 764 disease parameters (PD) and 177 parameters of aging (PA). Among them 667 (70.9%) and 274 (29.1%) were parameters with collective and exact maximal ages, respectively.

The lack of reference by authors to ages above 70 to 79 (or 70+ to 79+) and 80 to 89 (or 80+ to 89+) was calculated from the medical literature, and estimated as significant, and for ages above 80 to 89 was estimated as minimally significant, in regard to their focus on health data in the elderly. In different groups of parameters, 24% to 32% of maximal ages indicated significant and 25% to 65% minimal lack of reference. Maximal ages of PA were higher than those of PD (p<0.001), so lack of reference to health of the elderly was more significant when PD were studied as compared to PA. Lack of reference was more significant in studies of hospitalized and ambulatory patients and people living within the community. Usually authors checked all the populations of people living in nursing homes, but the total number of parameters relating to the latter, was very small, only 5.1% of the total number of parameters, so the populations of nursing homes did not reach the attention of the researchers.

Collective maximal ages are more often used in geriatric journals, including the Journal of the American Geriatric Society, as opposed to general and gerontological journals, although this approach seems to be too sweeping in the assessment of health of the elderly.

Optic Glioma in Children with Type 1 Neurofibromatosis

M. Ben-Arush, H. Goldberg, A. Kuten, J. Guilbord, R. El-Hassid

Pediatric Hematology-Oncology Unit, The Northern Israel Oncology Center, and Division of Neurosurgery, Rambam Medical Center; and Technion School of Medicine, Haifa

During the period 1985-95 we treated 5 girls and 13 boys with optic glioma associated with Type 1 neurofibromatosis (median age 3 years, range 2-10 years). 6 were treated with radiotherapy alone, 1 with surgery and radiation therapy and 1 with chemotherapy in order to postpone irradiation to an older age; 1 is being followed with no therapy. All children are alive, 2-10 years from diagnosis (mean follow-up time 5.4 years). 3 had improvement of vision following therapy and in 4 visual ability stabilized, including the child being followed without treatment. In 1 vision deteriorated despite therapy.