תוצאת חיפוש

Carnitine Deficiency in Inborn Errors of Metabolism

B.-A. Sela, T. Lerman-Sagie, M. Berkovitz

Institute of Chemical Pathology, Chaim Sheba Medical Center and Section of Clinical Biochemistry, Sackler School of Medicine, Tel Aviv University; Pediatric Neurology Unit, Wolfsohn Medical Center, Holon; and Children's Ambulatory Clinic, Assaf Harofeh Medical Center, Zrifin

Several conditions, considered as inborn errors of metabolism, involve severe deficiencies in carnitine in both plasma and muscle. In the absence of evidence suggesting primary carnitine deficiency due to a biosynthetic enzymatic defect in the liver, the various diseases with carnitine deficiency are related to genetic defects in organic acid metabolism leading to blocked mitochondrial b oxidation. We describe a 4.5-year-old boy and 2 female infants with glutaric aciduria type I, isovaleric acidemia, and long-chain acid dehydrogenase deficiency, in whom severe carnitine deficiency was apparent. In all 3, long-term carnitine treatment proved to be vital and eliminated most of the symptoms.

Identification of Sentinel and Axillary Node Involvement in Breast Cancer

M.Z. Papa, D. Bersuk, M. Koler, E. Klein, M. Sareli, G. Ben-Ari

Dept. of Surgical Oncology and Breast Unit, Chaim Sheba Medical Center, Tel Hashomer

Axillary node dissection for breast cancer is important for staging and prognosis. "Sentinel nodes" are the first nodes into which primary cancer drains. Identification, removal and pathological examination of those nodes indicates whether completion of axillary lymphadenectomy is required. The sentinel nodes are identified using a vital dye injected at the primary tumor site. With this technique we were able to identify sentinel nodes in 46 of 48 (95%) women examined. An average of 2.7‏1.2 nodes were identified as sentinel nodes. In 81% of cases there was a correlation between involvement of sentinel nodes and of other axillary nodes as well. In 10% of patients sentinel nodes were involved with tumor while other axillary nodes were negative. The major problem in routine application of this is relationship in surgical decisions is reliable real time pathological identification of lymph node involvement by tumor.

Analgesia in Breast Surgery with Interpleural Bupivacaine

Z. Crystal, Y. Katz

Dept. of Anesthesiology, HaEmek Medical Center, Afula and B. Rappaport Faculty of Medicine, Technion, Haifa

A control group of 15 patients undergoing breast surgery was given general anesthesia. In 15 other patients an interpleural block with 0.4 ml/kg bupivacaine, 0.5%, was performed 20 minutes before induction of general anesthesia for pre-emptive analgesia. This was extended further by continuous administration of bupivacaine 0.25%, 0.125 ml/kg/hr by automatic infusion pump, with supplements of opiates for postoperative pain management. The combined technique was associated with significantly reduced perioperative opiate requirement with better emergence from anesthesia, fewer side effects, a prolonged pain-free period, and overall better quality of postoperative recovery.

Fear of Personal Death among Hospital Physicians

Y. Hamama-Raz, Z. Solomon, A. Ohry

School of Social Work and Dept. of Neuro-Rehabilitation, Chaim Sheba Medical Center, Tel Hashomer (Both affiliated with Tel Aviv University)

Many studies have tried to explain why professionals experience difficulty when dealing with, and in treating efficiently situations connected with death. We studied levels of personal fear among physicians in general hospitals and addressed 2 questions: Does exposure to death on professional and personal levels, affect the level of the fear of personal death which physicians experience? Is there a relationship between personality variables, represented by the repression-sensitization dimension, and level of fear of personal death? A sample of 233 physicians from 22 general hospitals who specialized in oncology, internal medicine, surgery, psychiatry or pediatrics was studied. Each answered 4 questionnaires with regard to demographic information, fear of personal death, level of repression-sensitization and exposure to the death of relatives and significant others. There were no differences in level of fear of personal death of physicians according to specialization, but those who had been exposed to death on the personal level, feared less their own death. With respect to the personality variable, tendency to sensitization, it was found that those who were sensitized exhibited a higher level of the fear of their own death compared to those who were repressive. Of the various demographic variables examined (sex, level of religiouobservance, age, number of children, health, professional experience) it was found that those: with many years of professional experience, who were relatively older, who were nonobservant religiously and who were in good health, had lower levels of personal fear of death; gender was not a factor.

Kleptomania: Phenomenological, Clinical and Legal Aspects

R. Durst, G. Katz, K. Jabotinsky-Rubin, H.Y. Knobler

Kfar Shaul Mental Health Center, Jerusalem

Kleptomania is currently classified in psychiatric nomenclature as one of the impulse control disorders (DSM-IV, 1994). It is characterized by repeated failure to resist impulses to steal objects, not for personal use or monetary gain. The objects are therefore discarded, given away, or hoarded (ICD-10, 1992). This disorder is known since the early 18th century from the phenomenological and clinical viewpoints, yet is still debated with regard to therapeutic strategies and criminal liability. Although there are usually complications associated with the legal consequences of being caught and arrested, subjects continue to violate the law despite repeated arrests and convictions. In a 28-year old man suffering from kleptomania, years of psychodynamic psychotherapy were ineffective. Only when he was treated as suffering from an impulse control disorder or a variant of obsessive-compulsive disorder, was there significant improvement. The positive response to buspirone (5-HT1A) augmentation of fluvoxamine (SSRI) suggested that disturbed central serotonergic neurotransmission might play an important role in the pathogenesis of kleptomania. This concept is strengthened by the comorbidity of the syndrome with depression and by its compulsive traits. We stress that although kleptomaniacs cannot differentiate between right and wrong, testing shows that their sense of reality is intact, but they act under the influence of drives they cannot resist.

Fatal Multiple Organ System Dysfunction Associated with Germanium Metal

Micha Shamir, Charles L. Sprung

Dept. of Anesthesiology and Critical Care Medicine, Hadassah--University Hospital and Hebrew University-Hadassah Medical School, Jerusalem

The element germanium is widely distributed in nature. It is used in industry as a semiconductor and there have been a few attempts to use it in medicine. In the past few years 20 patients have been described in the literature as suffering from germanium overdosage. Like laboratory animals affected by the element, they suffer from renal failure and injury to other organs. We describe a 52-year old man given germanium to prevent recurrence of a brain tumor. He developed multiple organ dysfunction syndrome and died of intractable hyperdynamic shock. We call for caution regarding morbidity resulting from treatments believed safe.

Participation of Patients with Uret-Eral Calculi in Clinical Decision Making, and Level of Anxiety

Ilana Margalith, Amos Shapiro

Hadassah-Hebrew University School of Nursing, and Dept. of Urology, Hadassah Medical Center, Jerusalem

In a study examining the relationship between patient participation in clinical decision making and levels of anxiety, patients were offered a choice of treatment for ureteral calculus. 42 received information about 2 treatment options, ultrasound fragmentation of the stone through a ureteroscope and extracorporeal shock wave lithotripsy (ESWL), and were asked to choose the method that they preferred. 54 received treatment decided on by the physician without their participation in the decision making process. Anxiety was measured before meeting with the physician, immediately after the meeting and on hospitalization for treatment. The contribution of the patient's perception of participation in the decision- making process and level of education was also examined. There was a decrease in level of anxiety after meeting with the physician only among those who did not actually participate in the decision-making process (p<0.05). There was no change in the level of anxiety among those offered choice of treatment. However, a decrease in anxiety was evident among patients who perceived that they had received information about their illness and its treatment (p<0.01). This was not the case for patients who perceived themselves as participants in decision making unless they had a relatively high-level of education (p=0.05).

Macroenzymes: an Interesting Laboratory Finding, without Clinical Relevance

C. Perry, H. Peretz, E. Graf, O. Ben-Tal, A. Eldor

Hematology Dept. and Biochemistry Laboratory, Tel Aviv-Sourasky Medical Center, and Sackler Faculty of Medicine, Tel Aviv University

Macroenzymes are complexes of serum enzymes with proteins which have a higher molecular weight and longer plasma half-life than the normal enzyme. The presence of macroenzymes is suggested by finding increased serum enzyme activity, not associated with symptoms. Thus, macroenzymes can cause diagnostic errors and the performance of unnecessary tests or invasive procedures. We describe 2 patients with highly elevated serum levels of lactate dehydrogenase (LDH) and creatine kinase (CK) due to formation of complexes with immunoglobulin G. 1 patient had LDH of 4500 u/L but was otherwise normal and in the second CK was elevated with no evidence of ischemic heart disease. Awareness of the phenomenon of macroenzymes may save the patient long and sometimes invasive investigation.

Quality of Life in Younger Adults after First Stroke

Shenka Alfassa, Revital Ronen, Haim Ring, Aida Dynia, Ada Tamir, Reuben Eldar

Fleischman Unit for Study of Disability, Neurological Ward, Loewenstein Hospital, Ra'anana

To study the effect of stroke on the quality of life in younger adults, 199 patients 17-49 years of age who had sustained a first stroke between 1.11.92 and 31.10.93 were followed up. They were interviewed by telephone at 3, 6, 12 and 24 months after the event. 2 died during the first year of follow-up, and 8 had recurrent strokes. After 2 years, 8 additional patients had died and 4 had sustained recurrent events. Gradual improvement was reported within all age groups and in all areas. During the 3-6 months period, a mean of 4% improvement occurred in functional capability, 15% in social and recreational activity and 8% in return-to-work. The 6-12 month period showed an increase of 3% in improvement in mean functional capability, 10% in social and recreational activity and 2% in return-to-work. 1 year after the stroke 27% remained with moderate to severe disability, but over 86% were functionally independent in their daily living activities. There were no significant changes during the second year of follow-up in these statistics. 67% of those employed prior to their stroke returned to work and approximately 70% reported a return to prestroke social and recreational activity. These results demonstrate that the relatively high recovery rate and functional improvement during a year of follow-up were not accompanied by similar rates of improvement in emplyment and in social integration. They indicate the need for increased emphasis on long-term psychosocial rehabilitation services within the community.

Epidemiological Characteristics of Outbreaks of Diarrhea and Food Poisoning in the Israel Defense Forces

I. Grotto, Y. Mandel, I. Ashkenazi, J. Shemer

Army Health Branch, IDF Medical Corps and Sackler Faculty of Medicine, Tel Aviv University

Acute infectious diseases of the gastrointestinal tract and food poisoning are problems of great importance in the Israel Defense Force (IDF). They involve individual and epidemic morbidity, with impairment of health of individual soldiers and in the activities of units. Outbreaks of gastrointestinal infectious diseases must be reported to the IDF army health branch, which conducts epidemiological investigation. This study is based on data from yearly epidemiological reports for 1978-1989, and from a computerized database for the years 1990-1995. The incidence of outbreaks is characterized by an unstable trend, It was highest at the end of the 80's (68.3 per 100,000 soldiers on active duty) and lowest for the last 2 years (1994-1995, 36.3 per 100,000). The incidence of soldiers involved in food-borne outbreaks has been more stable, constantly declining during the course of the years. There was marked seasonality with a peak in the summer months. Sporadic morbidity was constant in 1990-1995, with a yearly attack rate of 60% in soldiers on active duty. Shigella strains were the leading cause of outbreaks until 1993, while in 1994-1995 their proportion decreased, with an increase in the proportion of Salmonella strains. As to Staphylococcus aureus, its role in causing food poisoning has been characterized by marked changes. Shigella sonnei replaced Shigella flexneri as the leading strain. 73.3% of outbreaks were small, with fewer than 40 soldiers involved, while 5.4% of outbreaks affected more than 100 soldiers. Outbreaks in which a bacterial agent was identified or which occurred in new-recruit bases were larger than those in which a bacterial agent was not identified, or which occurred in active field unit bases. In conclusion, the rates of infectious disease of the gastrointestinal tract are still high, although there has been a marked decrease since 1994. The incidence of outbreaks has also decreased, as well as the role of Shigella as a leading causative agent.

Gram-Negative Enteric Bacteremia in Children in the Negev

Michal Maimon-Greenwald, Eugene Leibovitz, Nimrod Maimon, Nechama Peled, Ron Dagan

Pediatric Infectious Disease Unit and Clinical Microbiology Laboratory, Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

During 1989-1994, there were 322 episodes of Gram-negative enteric bacteremia in 308 children. The incidence increased from 31/100,000 in children younger than 15 years of age during 1989-1991, to 50/100,000 during 1992-1994. The most common pathogens were Klebsiella, E. Coli, Salmonella and Enterobacter. 39% of episodes were nosocomial and a significant increase was recorded for each species during the last 3 years of the study. Klebsiella represented the most common pathogen causing nosocomial bacteremia, while E. coli and Salmonella were the main pathogens causing community-acquired bacteremia. In this study in southern Israel, the incidence of Gram-negative enteric bacteremia was significantly higher in Bedouin children, with the exception of bacteremia due to Salmonella, which occurred mainly in Jewish children.

Q Fever Endocarditis and Bicuspid Aortic Valve

Ora Shovman, Jacob George, Yehuda Shoenfeld

Medial Dept. B and Autoimmune Disease Research Unit, Chaim Sheba Medical Center, Tel Hashomer and Sackler of Medicine, Tel Aviv University

Q fever is caused by the rickettsia Coxiella burnetti, an obligate intracellular bacterium acquired by inhalation of infected dust from subclinically infected animals. Q fever may be acute or chronic; the chronic form mostly presents as endocarditis. Immunocompromised states and underlying heart disease are the most important risk factors. Usually the symptoms of Q fever endocarditis are nonspecific and diagnosis is often established very late. New criteria for diagnosis include a single blood culture positive for Coxiella burnetti, positive Q fever serology and characteristic echocardiographic studies. We describe a 49-year-old man with bicuspid aortic valve admitted with fever, weight loss and a new heart murmur. The diagnosis of Q fever endocarditis was established by positive Q fever serology, and an echocardiogram showing vegetations and valvular dysfunction. This case suggests that Q fever endocarditis should be considered in patients with "sterile" endocarditis.

Gradenigo Syndrome and Cavernous Sinus Thrombosis, in Fusobacterial Acute Otitis Media

S. Hananya, Y. Horowitz

Pediatrics Dept., Central Hospital of the Emek, Afula

In this era of antimicrobial medication, intracranial complications following otitis media are rare. We present a 5-year-old boy who suffered from petrositis (Gradenigo syndrome) and cavernous sinus thrombosis as combined complications of acute otitis media caused by fusobacteria. The diagnosis was made using imaging methods suited to the various structures of the skull. Cure was achieved by prolonged conservative treatment with antibiotics, with gallium scan for evaluation of the bone inflammation.

Prosthetic Heart Valve Thrombosis: A 3-Year Experience

Yaron Shapira, Rafael Hirsch, Ruth Jortner, Moshe Nili, Bernardo Vidne, Alex Sagie

Sheingarten Echocardiography Unit and Cardiology Dept., Rabin Medical Center (Beilinson Campus), Petah Tikva and Sackler Faculty of Medicine, Tel Aviv University

A series of 12 patients with 16 episodes of prosthetic heart-valve thrombosis over 3 years is presented. Most episodes affected mitral or tricuspid bileaflet prostheses. All patients were inadequately anticoagulated at the time of thrombosis. The clinical presentation was acute and severe in 6 patients, and subacute or chronic in the rest. Physical examination was suggestive of stuck valves in most cases. Transthoracic echocardiography revealed increased transvalvular gradients in most. However, clearer evidence of valve thrombosis was obtained from transesophageal echocardiography or fluoroscopy. 9 patients eventually had their valves re-replaced successfully, and the preoperative diagnosis was confirmed in all. 5 patients were operated as soon as the diagnosis was established, and an additional 4 were operated after failure of anticoagulation. In 4 patients the valve leaflets became completely mobile after a course of thrombolysis. Prosthetic valve thrombosis is a severe and potentially fatal complication in patients with mechanical heart valves. Alertness of physicians at all levels- the general practitioner, the internist and the cardiologist- to the possibility of valve thrombosis and to its clinical presentation may lead to prompt and earlier diagnosis and to comprehensive therapy.

Nissen Fundoplication by Laparoscopy

Dan Seror, Oded Zamir, Raphael Udassin, Amos Vromen, Tanir M. Allweis, Herbert R. Freund

Depts. of Surgery and Pediatric Surgery, Hadassah-University Hospital, Mount Scopus, Jerusalem

Short term results following laparoscopic Nissen fundoplication were evaluated in 31 patients with symptomatic gastroesophageal reflux. 6 were females and 26 males, and they ranged in age from 5 months to 64 years (mean: 4.9 years in 19 younger than 18 years, and 39.3 years in 12 adults). Most of the adults who complained of pain and heartburn underwent pH monitoring, endoscopy, and manometry as needed. Milk scan was the most useful diagnostic tool for the evaluation of the children, who suffered mainly from gastroesophageal-related pulmonary disease. Indications for laparoscopic operation were identical with those for conventional open Nissen fundoplication. 1 case of dysautonomia died postoperatively; the rate of complications, mostly minor, was 22.5%. 3 patients required conversion to open Nissen fundoplication due to cardiorespiratory instability secondary to pneumothorax in 2, and to esophageal perforation in the third. 5 adults developed temporary dysphagia. 3 children had only partial improvement in their pulmonary disease following the operation, while the other 15 had complete relief. The total time for the laparoscopic operation averaged 245 minutes in adults, and 228 in children. Discharge was usually on the fourth postoperative day in adults (mean: 6.0 days). Regurgitation and heartburn were cured in 10 out of 11 adults (91%). All parents of children were satisfied. Symptomatic outcomes following laparoscopic Nissen fundoplication compare favorably with those of open surgery with respect to mortality, complications, and outcome.