תוצאת חיפוש

Paroxysmal Nocturnal Hemoglobinuria

Haim Ben-Ami, Yehuda Edoute

Medical Dept. C, Rambam Medical Center and Bruce Rappaport Faculty of Medicine, The Technion, Haifa

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disorder of blood cells which originate from an abnormal hematopoietic stem cell. The condition is characterized by nocturnal hemoglobinuria, chronic hemolytic anemia, and thrombosis. We describe a 60-year-old woman with PNH admitted with abdominal pain and jaundice, who had dark urine on arising after a night's sleep. The diagnosis was established by the typical clinical story and a positive Ham test. She was successfully treated with Halotestin and folic acid. Although PNH is rare, it should be considered in the differential diagnosis of hemolytic anemia. Early diagnosis and treatment are important.

Experience with Under-Water Birth

Eylon Lachman, Zvi Finelt

Dept. of Obstetrics and Gynecology, Yoseftal Hospital, Eilat

Underwater birth is now deemed an acceptable type of delivery. Safety is a recurring consideration, the main concern being that of drowning. But in reports of 19,000 underwater births no untoward events were noted. Also, need for pain relief and of intervention during labor were both reduced. The short term indicators of neonatal outcome were good. A recent review explains 5 different physiological factors which inhibit initiation of fetal breathing under warm water. Many studies have shown no increase in risk of infection of either mother or baby. We report our experience with 26 women, 23 of whom actually delivered in the pool with excellent results. We believe that birth under water is safe and beneficial if done properly for low-risk patients.

Six Cases of Acute Rheumatic Fever in One Year

George Habib, Ramiz Abu-Ahmad

Rheumatology Clinic and Dept. of Medicine, Nazareth Hospital; and Medical Dept. B, Carmel Medical Center, Haifa

During 1995, 6 cases of acute rheumatic fever were diagnosed here. Taking into account differences in total admissions, this appears to represent an increase over 1994. Most of the cases were males, with average age at diagnosis 19.5 years. All were of low socioeconomic status. 50% had cardiac involvement, and 1 needed treatment with corticosteroids. Most had pharyngeal symptoms prior to the acute attack, and 1 patient had 2 prior episodes of rheumatic fever. A thorough epidemiological study should be done in the Nazareth area to assess the real incidence of acute rheumatic fever, and to determine whether there is a true increase in incidence.

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.

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.

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.

Hospitalization of the Elderly for Stroke Rehabilitation

D. Gottlieb, M. Kipnis, E. Sister, N. Lipkin, M. Medvedev, S. Brill

Stroke Rehabilitation Unit, Neurogeriatric Dept., Beit Rivka Geriatric Hospital, Petah Tikva

This is a descriptive, longitudinal study of 400 elderly patients admitted for stroke rehabilitation to a geriatric department dedicated to this purpose. We present the demographic, medical, and impairment and disability characteristics on admission, and the outcome of rehabilitation. Mean stay was 54 days and 2/3 became independent and returned home. Functional score on admission was the best predictor of outcome, but age and social support were also significant.

Bacterial Culture of Chip Tissue of Enucleated Prostates

I.Z. Ben-Zion, P.M. Dagtyar, J. Kaneti

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

To assess the prevalence of infection and colonization of the prostate by bacteria, chip tissue samples from 166 patients undergoing retropubic prostatectomy were submitted for bacterial tissue culture. In 28 patients with an indwelling catheter before surgery, E. coli, Klebsiella, Pseudomonas and Enterobacter were the commonest species encountered, the first the most common. In only 7 patients (20%) who didn't have an indwelling catheter before operation was the culture positive. We confirmed that the longer the time the catheter was indwelling before surgery, the greater the likelihood of positive cultures. However, postoperative outcome and morbidity were not related to culture results. We conclude that even though it is worth trying to sterilize the urine and prostate before prostatectomy, the effect on the postoperative outcome is minimal when proper antimicrobial therapy is given perioperatively.

Hyponatremia due to Prolonged Excessive Ingestion of Water

I. Shavit, J. Diment, S. Ravid, N. Shehadeh

Dept. of Pediatrics A, Rambam Medical Center and Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

We report a 1.5-year-old boy admitted for restlessness and constipation. He was found to have hyponatremia caused by voluntary drinking of excessive amounts of water. Although unusual in children, intoxication by oral water is a recognized clinical syndrome in infants, 3-6 months old, fed with dilute formula. Water intoxication in older children is rare. The diagnosis was established by the water deprivation test.

Effect of the Yom Kippur Fast on Parturition

A. Wiser, E. Maymon, M. Mazor, I. Shoham-Vardi, T. Silberstein, A. Wiznitzer, M. Katz

Depts. of Obstetrics and Gynecology and of Epidemiology, Soroka Medical Center, and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Food-withdrawal has been proposed as a possible mechanism for initiating the onset of labor in animals and humans. The hypothesis was based upon the reported increase in deliveries of infants during the Yom Kippur fast. We studied the effect of the fast on full term deliveries of Jewish women, with non-fasting Bedouin women as controls (1988-1995, 1,313 Jewish and 1,091 Bedouin deliveries). To determine the effect of Yom Kippur itself, delivery rates on Sukkot and Yom Kippur were compared in both groups. The mean delivery rate in the Jewish population was significantly higher during Yom Kippur and the day after, than during the 7 days before Yom Kippur (15.1±5.1 and 14.6±4.7 vs 10.7±3.5, p<0.04 and p<0.01, respectively). There was an increase in delivery rate during the 6 hours before the end of the fast. In the Bedouin women there were no changes in delivery rate during any of these periods. There were no significant differences in the rates of deliveries during the Sukkot festival between Jewish and Bedouin women. We conclude that fasting is associated with a significant increase in the rate of deliveries at term.

Attention Deficit Hyperactivity Disorder, Facilitating Alcohol and Drug Abuse in an Adult

R. Durst, P. Rebaudengo-Rosca

Talbieh Mental Health Center, Jerusalem (Affiliated with the Hebrew University-Hadassah Medical School)

Attention-deficit hyperactivity disorder (ADHD) has been considered a mental and behavioral disorder of childhood and adolescence. It is being increasingly recognized in adults, who may have psychiatric co-morbidity with secondary depression, or a tendency to drug and alcohol abuse. We describe a 32-year-old woman known for years as suffering from borderline personality disorder and drug dependence (including hasshish, marijuana, LSD and "exstasy'") and alcohol abuse that did not respond to treatment. Only when correctly diagnosed as ADHD and appropriately treated with the psychotropic stimulant, methylphenidate (Ritalin), was there significant improvement. She succeeded academically, which had not been possible previously, the craving for drugs diminished and a drug-free state was reached. Although administration of psychostimulants to drug abusers is controversial, as they are addictive, in cases of ADHD they have promoted drug abstinence.

Red Cell Na+/H+ Exchange and Role of Protein Kinase C in its Stimu-Lation in Diabetes Mellitus, Essential Hypertension and Nephropathy

Wladimir Koren, Robert Koldanov, Edna Peleg, Eva Izsak, Meir Berezin, Talma Rosenthal

Dept. of Medicine C, Hypertension Unit and Endocrinology Institute, Chaim Sheba Medical Center, Tel Hashomer

Na+/H+ exchange (NHE) was measured as maximal initial velocity of pH-dependent H+ efflux from red cells into an alkaline medium containing Na+ in patients with insulin-dependent or noninsulin-dependent diabetes, with and without hypertension and in normoglycemic, essential hypertensives and normal controls (50 subjects in each subgroup). Maximal velocities of NHE were found in microalbuminuric patients in all subgroups, and NHE correlated with the rate of microalbuminuria (r=0.61, p=0.02). Daily insulin requirements were greater in those with elevated NHE (84±8 vs 42±4 U/day). There was no correlation between NHE and levels of plasma glucose, HbA1 and plasma aldosterone and lipid profile and PRA. NHE was correlated with plasma prolactin (r=0.51, p=0.02) and PTH r=0.24, p=0.05). In uremic patients, NHE was inversively correlated with creatinine clearance (r=-0.48, p=0.03). Since calphostin C, a selective inhibitor of protein kinase C, lowered increased NHE in vitro, the protein kinase C-dependent pathway of the exchanger regulation was concluded to be responsible for NHE activation in diabetes mellitus and essential hypertension.

Water Intoxication following Desmopressin Overdose

D. Segal-Kuperschmit, O. Dali-Gotfrid, A. Luder

Pediatric Dept., Rebecca Sieff Hospital, Safed

Water intoxication is a serious condition which may be caused by desmopressin overdose, with reversible or irreversible neurological complications. In the past, desmopressin was used in endocrinological centers for the treatment of anti-diuretic hormone deficiency (central diabetes insipidus). Indications for hormone treatment have since widened, especially as an effective solution for nocturnal enuresis. It is now often prescribed in community clinics, and its use has been encouraged by extensive promotion. We describe a 15-year-old boy with primary nocturnal enuresis who started treatment with desmopressin 1 year prior to admission. He was allowed to use the drug without supervision, and drank excessively. The result was water intoxication which required admission for intensive care because of loss of consciousness and convulsions for 36 hours.

Separation of Conjoined Twins: The Anesthesiologist's Perspective

Zvi Grunwald, Yacov Katz

Dept. of Anesthesiology, Rabin Medical Center, Beilinson Campus and Schneider Children's Hospital, Petah Tikva

Managing anesthesia care for separation of conjoined twins poses a special challenge for the anesthesia team. Early preparation, thorough understanding of the anatomical and the physiological consequences of this complex anomaly, as well as careful coordination with the teams of surgeons, nurses and operating room personnel are mandatory to assure successful separation of the conjoined twins. It is recommended that the mother be transferred to and the babies be delivered at a tertiary care medical center, like the Children's Hospital. Special attention is devoted during the surgical procedure to cardiovascular stability, drug disposition, fluid balance and temperature control. The multidisciplinary team approach makes this complex surgery a success. When postoperative survival cannot be guaranteed for both twins, close contact and consultation is mandated with the parents, medical personnel and religious and legal advisors.

Prognostic Factors And Surgical Results In Traumatic Cataract

Yuval Gelfand, Joseph Pikkel, Benjamin Miller

Ophthalmology Dept., Rambam Medical Center and Bruce Rappoport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

The visual outcome in 23 men and 2 women with traumatic cataracts was analyzed retrospectively. Their average age was 33 and they ranged from 10 to 69 years. Surgical results were either very good or very poor. Associated retinal injuries significantly decreased final visual acuity (p = 0.001). Those with initial visual acuity restricted to finger counting had better visual results than those with initial visual acuity restricted to light perception (p = 0.01) and hand motions (p = 0.02). Usually the lens was removed via the pars plana; the most common mode of optical correction was contact lenses.