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.

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.

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.

Irritable Bowel Syndrome: Biofeedback Treatment

N. Barak, R. Ishai, E. Lev-Ran

Biofeedback Unit, Psychiatric Ward, Sheba Medical Center, Tel Hashomer

Irritable bowel syndrome is a group of heterogenic complaints of functional bowel disorder in the absence of organic pathology. The pathophysiology is unclear. In most cases treatment includes symptomatic remedies, antidepressants, psychotherapy and hypnotherapy.

Biofeedback has recently been introduced as a therapeutic modality. Treatment also includes relaxation techniques and guided imagery, together with computer-assisted monitoring of sympathetic arousal. Biofeedback requires active participation of patients in their healing progress and leads to symptom reduction in 2/3.

Outcome in Fetuses with Increased Nuchal Translucency Thickness

Ron Maymon, Eli Dreazen, Yosi Tovbin, Zwi Weinraub, Arie Herman

Ultrasound Unit, Dept. of Obstetrics and Gynecology, Assaf Harofeh Medical Center, Zerifin and Sackler Faculty of Medicine, Tel Aviv University

Increased thickness of fetal nuchal translucency (TNT) measured at 10-14 weeks of gestation, may suggest underlying fetal chromosomal defects, structural abnormalities or genetic syndromes. We examined the relationship between increased TNT and pregnancy outcome, especially in fetuses with normal karyotypes.

1400 pregnant women underwent first trimester scanning and screening for chromosomal abnormalities and measurement of fetal TNT. 25 fetuses (2%) with increased TNT (>3 mm) were identified. 8 (30%) had an abnormal karyotype. Of these, 5 pregnancies were aborted, 3 ended in spontaneous abortions before karyotyping, and 2 were terminated, all before detailed cardiac scanning. There was a high association between increased TNT and karyotype abnormalities. The total incidence of favorable outcome in fetuses with normal chromosomal and cardiac features but enlarged TNT was 56%.

Acute, Painful, Swollen Testis

Dan Leibovici, Simon Strauss, Ahuva Sharon

Dept. of Urology, Ultrasound Unit, Institute of Diagnostic Imaging and Medical Dept., Assaf Harofeh Medical Center and Sackler Faculty of Medicine, Tel Aviv University

An acutely painful and swollen testis mandates urgent diagnostic and therapeutic measures since this symptom complex may indicate torsion of the testis. Prompt scrotal exploration is necessary if the testis is to be saved from ischemic necrosis.

Polyarteritis nodosa (PAN) is a vasculitis involving mainly medium and small sized arteries and may damage any organ. In PAN, the presentation of an acutely painful and swollen testis raises a perplexing diagnostic problem since the symptoms may be related to vasculitis involving the testis on the one hand, or represent primary testicular pathology unrelated to the underlying PAN. A 31-year-old man with PAN who presented with acute pain and swelling in a solitary testis is reported.

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.

Effect of Specific Inspiratory Muscle Training on Dyspnea and Exercise Tolerance in Congestive Heart Failure

Paltiel Weiner, Joseph Waizman, Rasmi Magadle, Noa Berar-Yanay, Benny Pelled

Depts. of Medicine A and Cardiology, Hillel Yaffe Medical Center, Hadera

It has been shown that the inspiratory muscles of patients with congestive heart failure (CHF) are weaker than normal. This weakness may contribute to dyspnea and limit exercise capacity. But respiratory muscles can be trained for increase in both strength and endurance. This study was designed to evaluate the effect of specific inspiratory muscle training (SIMT) on muscular performance, lung function, dyspnea and exercise capacity in moderate heart failure.

10 patients with CHF (NYHA functional class II-III) received 1/2 hour of SIMT daily, 6 times/week, for 3 months. They started breathing at a resistance 15% of their Pimax for 1 week and the resistance was then increased incrementally to 60%. Spirometry, inspiratory muscle strength and endurance, and the 12-minute walk test were performed before and after the training period. All showed an increase in inspiratory muscle strength and endurance. This was associated with a small but significant increase in FVC, a significant increase in the distance walked (458±29 to 562±32 m, p<0.01), and improvement in the dyspnea index score.

SIMT resulted in increased inspiratory muscle strength and endurance. This increase was associated with decreased dyspnea and an increase in submaximal exercise capacity. SIMT may prove to be useful complementary therapy in CHF.

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.

Intrahospital Transport of Critically Ill Children

Gideon Paret, Ron Ben Abraham, Oshrat Yativ, Amir Vardi, Zohar Barzilay

Dept. of Pediatric Intensive Care and of Anesthesiology, Sheba Medical Center, Tel Hashomer and University of Tel Aviv

Prospective evaluation of intrahospittransportation of 33 critically ill children to and from the pediatric intensive care unit was conducted over the course of a month. Factors contributing to risk of transport were assessed.

There were 33 children (25 boys and 8 girls), 3 days to 15 years of age. Reasons for admission included: disease and trauma in 19, and status post operation in 11. The pretransport PRISM score was 4.84. 22 children (66.6%) were being mechanically ventilated and 10 (30.3%) were being treated with amines. Transport time ranged from 8-150 minutes. 15 of the transports (45.4%) were urgent and a special intensive care team escorted 22 (66.6%). Equipment mishaps and physiolog-ical deterioration occured in 12 (36.3%) and 11 (30.3%) of the cases, respectively. The use of amines, mechanical ventilation, longer transport time and high PRISM score were all associated with physiological deterioration on transport.

Preliminary Report of Experience with Laparoscopic Splenectomy

Joseph Kuriansky, Ron Bilik, Moshe Shabtai, Itamar Avigad, Amram Ayalon

Depts. of General Surgery and Transplantation and Pediatric Surgery, Sheba Medical Center, Tel Hashomer; and Sackler School of Medicine, Tel Aviv University

Laparoscopic splenectomy is effective and technically feasible for treating various hematological diseases such as idiopathic thrombocytopenic purpura (ITP), congenital sphero„cytosis, hemolytic anemia, and Hodgkin's lymphoma. An anterior approach to the vascular pedicle is usually described. However, in this approach to the splenic hilum, the dissection of the splenic artery is frequently difficult. The laparoscopic posterolateral approach involves dissection of the posterolateral attachments to the diaphragm, followed by the disection and ligation of all splenic branches near the splenic parenchyma. We used it in 9 adults and 4 children: 11 patients had ITP, 1 spherocytosis and 1 Hodgkin's lymphoma.

This procedure was completed in 13 patients, but in 2 it had to be converted to open surgery. Mean operating time was 3 hours and mean postoperative stay 3 days. Blood transfusion was not required and there were no postoperative complications.

Laparoscopic splenectomy is safe in both adults and children, and the posterolateral approach provides excellent visualization and allows control of the branches of the splenic vein and artery in the splenic hilum.

Danaparoid-Sodium for Dialysis in Heparin-Associated Thrombocytopenia

Ronen Ben Ami, Ruth Rachmimov, Shlomo Berliner

Medicine Dept. D and Anticoagulation Therapy Unit, Tel Aviv-Souraski Medical Center, and Nephrology Institute, Sheba Medical Center, Tel Hashomer

Danaparoid sodium is an anticomposed of 3 glycosaminoglycans: heparan sulfate, dermatan sulfate and chondroitin sulfate. Similar to heparin, operates by activating antithrombin 3, but does not contain heparin or heparin fragments, and is therefore antigenically distinct.

Danaparoid has been advocated as a safe and effective anticoagulant for heparin-associated thrombocytopenia. However, there is little experience in its use as a substitute for heparin in hemodialysis.

We report 2 men, aged 82 and 73 years, respectively, who developed thrombocytopenia while undergoing hemodialysis with heparin, and who subsequently underwent successful dialysis with danaparoid. There was a rise in platelet levels in both while receiving danaparoid, and dialysis was completed without hemorrhagic or thrombotic complications. Danaparoid is a safe and effective substitute for heparin, and may be used as an anticoagulant in hemodialysis.

Recording Nocturnal Erections and Insurance Claims: Cost-Effectiveness

Ron Peled, Giora Pillar, Y. Berger, Naveh Tov, Nir Peled, Peretz Lavie

Sleep Lab, Gutwirth Building, Technion Medical School, Haifa

Road accidents, work accidents, or other trauma can cause impotence and are frequently followed by insurance claims. During 1990-97 we examined 230 males with such a complaint. All underwent full polysomnographic recordings in the sleep laboratory for 2 nights, during the course of which NPT (nocturnal penile tumescence) was examined with special equipment. It was assessed by an experienced technician following planned awakenings from REM sleep.

In 75 of the 230 subjects (33%), satisfactory erections were observed. In 100 (43%), who experienced at least 3 periods of REM sleep, no erections occurred. These patients were categorized as suffering from organic impotence. In the remaining 55 (24%), the results were inconclusive, with only partial erections or not enough REM sleep periods.

Since a man recognized as suffering from impotence may be awarded large monthly payments for life, these examinations, in our opinion, are an important tool to prevent unjustified claims, and can save the state unnecessary expenses.
 

Reactive Increase in Blood Pressure on Immobilization, but not Hypertension, Prevents Pressure Ulcers

Vladimir Shats, Silvio Kozacov

Geriatric Dept., Rebecca Sieff Hospital, Safed

Of 135 geriatric patients immobilized for at least 2 days, 37 (27.4%) had pressure ulcers (PU). Those without PU were the control comparison group. Gender, length of immobilization, number of blood pressure determinations and proportion with hypertension were similar in those with and without PU. Those with PU were slightly older than those in the comparison group: 75.5±8.8 and 74.7±9.6 years, respectively (p>0.05).

Of 66 patients with acute ischemic stroke, reactive increase of systolic or diastolic blood pressure to 140/90 mm Hg or above following immobilization, was seen in 60.6% and 22.7% of patients, respectively, and there were PU in 12.1%. Of 17 with recurrent ischemic stroke, corresponding figures were: 41.2%, 23.5% (p>0.05), and 47.1% (p<0.01). In 7 patients with previous ischemic stroke corresponding figures were: 14.3% and 0% (p<0.01) and 100% (p<0.001). In 36 operated for fracture of the femur, corresponding figures were: 50%, 11.1% (p>0.05), and 27.8% (p>0.05). For 9 patients with severe infections, sepsis or pneumonia, the corresponding figures were: 22.2% and 0.0% (p>0.05), and 44.4% (p<0.04).

The proportion of patients with reactive increase in systolic blood pressure on immobilization was lower in the PU group than in the controls, 27% vs 59.2%, (p<0.001). The corresponding figures for reactive increase in diastolic blood pressure were similar, 8.1% and 20.4%, respectively (p>0.05).

The mean systolic blood pressure on immobilization was higher in the control than in the PU group, 145.4±21.7 and 130.8±14.9 mm Hg, respectively (p<0.001). The corresponding figures for the mean diastolic blood pressure were similar, 81.2±10.5 and 75.7±8.9 mm Hg, respectively (p<0.01). An increase in systolic blood pressure on immobilization reduced the risk of developing PU (p<0.05).

There was no significant statistical relation between diagnosis of hypertension and proportion of patients with PU (p>0.05). Of 67 patients with hypertension, in 23.9% and 74.6% of them there was no increase in systolic or diastolic blood pressure, respectively. Statistical difference between lack of diastolic or systolic response was very significant (p<0.001).

Reactive increase of blood pressure, but not hypertension, predicts reduced risk of PU on immobilization in the hospitalized elderly. Diminished reactive increase of blood pressure in response to stress of any kind may be a criterion of frailty and reduced physiological reserves. Efforts to reduce elevated blood pressure when a patient is immobilized appear irrational.

Proper use of Pressurized Hand-Held Inhalers in Patients with Chronic Airway Obstruction 

Shabtai Varsano, Ilana Jacoby, Mila Garenkin

Asthma Care and Education Unit, Dept. of Pulmonary Medicine, and Epidemiology Unit, Meir General Hospital, Sapir Medical Center, Kfar Saba; and Sackler School of Medicine, Tel Aviv University

Inhaling drugs via hand-held inhalers in recommended for those with chronic obstructive airway disease (COPD). Approximately 8%-9% of Israel's population use hand-held inhalers, many of them pressurized. Skill in using them and ability of chronic users to learn their proper use have not been assessed.

During 1993 and 1994 we studied 200 patients with bronchial asthma or COPD who regularly used a pressurized hand-held inhaler (PI), but were not trained to use it in our out-patient pulmonary clinic. Only a third were found to be skilled in its use. About half were completely unable to use it properly, and 17% used it in a suboptimal way. Remarkably, only 40% had been taught anything with regard to its use. About 75% of the suboptimal users significantly improved their skill in its use immediately after receiving a single individual teaching and corrective demonstration session. While 15% failed to learn the proper use of the PI, many of those who improved immediately after a single teaching session retained the learned skills for months.

We conclude that the physician who recommends the use of a PI is responsible for the patient's being taught its proper use in a demonstration session. Skill in its use should be reassessed periodically during the entire treatment period.