Liver Failure with Coagulopathy in an Infant with Tyrosinemia

Moshe Nussinovitch, Gadi Campino, Rivka Shapira, Benjamin Voluvitz, Jacob Amir

Department of Pediatrics, Schneider Children's Medical Center of Israel, Petah Tikva, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

Tyrosinemia is an inherited autsomal recessive condition. We present a 5 week-old boy with this disorder. He was admitted because of a fever, vomiting and lethargy. The laboratory tests confirmed a coagulopathy with prolonged prothrombin time (PT), partial thromboplastin time (PTT) and a decreased serum fibrinogen. The a-fetoprotein level was markedly elevated. To confirm the diagnosis of tyrosinemia, quantitative urinary succinylacetone was measured. Although overt liver failure with coagulopathy may be part of the representation of tyrosinemia, a significant coagulopathy in the absence of overt signs of liver disease has not been emphasized as a clue to the diagnosis of this condition.

איברהים מרעי, יאיר לוי

המח' לרפואה פנימית ב', מרכז רפואי שיבא, תל-השומר, והפקולטה לרפואה סאקלר, אוניברסיטת תל-אביב

דופמין נמצא בטיפול נרחב למניעה וטיפול באי-ספיקת כליות. הטיפול בו מבוסס על ההנחה, שלדופמין ברירנות כלייתית כשהוא ניתן במינון נמוך. אין עדויות ליעילות דופמין במניעת אי-ספיקת כליות חדה בקרב חולים שעברו הליכים בסיכון גבוה, ברוב העבודות, דווח על חוסר יעילות ואף על סכנה במתן דומפמין ללוקים באי-ספיקת כליות חדה. לדופמין השפעות-לוואי לא מבוטלות. קיים צורך בעבודות מבוקרות ואקראיות שבהן תיבדק השפעת הטיפול בדופמין על שיעור התמותה, הצורך בדיאליזה או על משך האישפוז. בינתיים לא מומלץ מתן דופמין כטיפול מונע או כטיפול בלוקים באי-ספיקת כליות חדה.

בעקבות הדיווח הראשוני על השפעות דופמין על מערכת הלב וכלי-הדם ועל הכליות, נמצא תכשיר זה בטיפול נרחב ביחידות לטיפול נמרץ ובמחלקות לרפואה פנימית מאז 1972. מתן דופמין למניעה וטיפול בלוקים באי-ספיקת כליות חדה מבוסס על יכולתו להעלות את זרימת הדם לכליות בבעלי חיים ובבני אדם בריאים. הטיפול בדופמין למטרות אלה ניתן זה יותר מ- 25 שנים באורח שיגרתי, מבלי שלמעשה נערכו מחקרים מבוקרים המוכיחים את יעילותו והמבהירים מהן השפעות-הלוואי שלו. לאחרונה, עלו שאלות וספקות לגבי יעילות הטיפול בדופמין באי-ספיקת כליות חדה, ופורטו הסיכונים בטיפול זה.

במאמר זה, נסקרת הספרות בשנים האחרונות, המתייחסת לטיפול בדופמין באי-ספיקת כליות חדה, והאם קיימת הצדקה לטיפול זה.

אליהו לויטס

היחידה לפוריות והפריה חוץ-גופית, החטיבה למיילדות וגינקולוגיה, ביה"ח האוניברסיטאי סורוקה, הפקולטה למדעי הבריאות, אוניברסיטת בן-גוריון בנגב, באר-שבע

יותר ויותר מתבהר הצורך לאבחן ולטפל בחוסר הביוץ. אי-הפוריות היא רק תוצאה אחת, קביעת האבחנה וטיפול בתאם יכולים למנוע תופעות שליליות נוספות לבריאות האשה. תוצאות של חוסר ביוץ כוללות ירידה בצפיפות העצם, ירידה בחימוד (ליבידו), תופעות בלתי רצויות בעור ובשיער, שיגשוג בלתי מבוקר של רירית הרחם (endometrium), מצב קרדיוואסקולרי מעורער ושינויים פסיכולוגיים ופסיכיאטריים. התוצאות הסוציאליות והפסיכוסוציאליות של חוסר הביוץ לא תמיד בתודעתנו, למרות חשיבותן הרבה. על כן, כדי לייעץ נכון למטופלת, דרושה אבחנה מדויקת, ככל האפשר.

המושג הבסיסי להבנת הפתוגנזה של הפרעות ביוץ קשור בהבנת העובדה, שהפרשה פעימתית מתאימה של GnRH (Gonadotropin Releasing Hormone) מההיפותאלאמוס במוח קובעת את מועד הביוץ והווסת. פעימות של GnRH מהירות או איטיות מדי יגרמו לחוסר ביוץ או להרס הגופיף הצהוב השחלתי טרם זמנו...
 

Laparoscopic Cholecystectomy - 1,000 Procedures in a Surgical Department

Uri Freund, Ami Mayo, Ivan Schwartz, David Neufeld, Haim Paran

Dept. of Surgery A, Meir Hospital, Kfar Saba; and Sackler School of Medicine, Tel Aviv University

The first 1,000 laparoscopic cholecystectomies performed in our department were reviewed. There was no operative mortality; conversion to open cholecystectomy was necessary in 2%. In the last 600 cases the rate of conversion had decreased to 0.5%. There was common bile duct injury in 0.3%, with the injuries identified during primary surgery. This clinical experience is consistent with previous studies, which proved that laparoscopic cholecystectomy is safe and should replace open operation as the procedure of choice. 

Burch Laparoscopic Procedure for Repairing Proven Stress Incontinence

Moshe Bustan, Shabtai Romano, Raed Salim, Jacob Rosenman, Eliezer Shalev

Depts. of Obstetric and Gynecology and of Urology, HaEmek Medical Center, Afula

There are more than 200 procedures for repairing stress urinary incontinence. We evaluated the safety and efficiency of the Burch laparoscopic procedure in 32 women with urodynamically proven genuine stress incontinence.

Mean operating time was 40 minutes and mean hospitalization time after the procedure was 30 hours. The cure rate was 97%, similar to that rin other studies (80-95%). The major complications w2 cases (6.2%) of unintended bladder injury, diagnosed and repaired laparoscopically. Although follow-up has only been for 3-42 months, the high cure rate and safety and advantages of laparoscopy over laparotomy, make laparoscopic Burch colposuspension the procedure of choice for repairing stress incontinence.

Penile Prosthesis for Erectile Dysfunction: Long-Term Follow-Up 

Ofer N. Gofrit, Ofer Z. Shenfeld, Ran Katz, Amos Shapiro, Ezekiel H. Landau, Dov Pode

Urology Dept., Hadassah University Hospital, Jerusalem

Our armamentarium for the treatment of erectile dysfunction has recently been expanded by addition of Viagra and the MUSE. However, their long-term results are still unknown. The insertion of a penile prosthesis is invasive, expensive, and irreversible, but under optimal condition provides an acceptable, definitive solution for erectile dysfunction. We evaluated our long-term results with penile prosthesis insertion (PPI).

From 1987-1998, 57 patients underwent PPI in our department. Mean age was 55 years and the common causes of erectile dysfunction were atherosclerotic disease (23), radical pelvic surgery (15), and diabetes mellitus (14). Semirigid prostheses were inserted in 12 and inflatable prostheses in 45, including 42 single-component and 3 multi-component prostheses.

Recently we interviewed these patients by telephone, using a standard questionnaire. Those not satisfied with the surgical results (83% of the living patients) were examined in our clinic. Mean follow-up was 53 months. In 37 (84%) the prosthesis was mechanically functional (rates after 1, 5 and 10 years were 87.8%, 80%, and 75%, respectively). In only 2 (2.5%) had serious complications led to prosthesis removal.

All mechanical failures had occurred in those with inflatable prostheses after a mean of 48.5 months (range 4-113). At the time of the survey 68% were sexually active and 64% were satisfied with the surgical result. We conclude that PPI is safe treatment for erectile dysfunction. Although the rate of mechanically functioning prostheses decreases with time, modern multi-component prostheses may lead to better mechanical results.

Tacrolimus does not Accentuate Hepatic Damage due to Hypoperfusion 

Ron Ben Abraham, Ivgeni Isartal, Richard Nakache, Vallery Rudick, Daniel Ogorek, Gideon Paret, Avi Weinbroum

Dept. of Anesthesiology and Critical Care Medicine, Organ Transplantation and Postoperative Care Units, Tel Aviv-Sourasky Medical Center; and Pediatric Intensive Care Units, Sheba Medical Center, Tel Hashomer; and Sackler Faculty of Medicine, Tel Aviv University

Deterioration of hepatic function following liver transplantation is a known complication, sometimes attributed to the use of cyclosporin A. Reaction to tacrolimus (Prograf), a relatively new and effective immunosuppressant drug, is thought to result in a much lower grade of organ dysfunction, especially in the transplanted liver.

Using the ex-vivo rat model of isolated perfused liver, we evaluated hepatocellular damage and oxygen extraction when tacrolimus was administered following liver hypoperfusion. Tacrolimus did not worsen hepatic dysfunction caused by the hypoperfusion. Therefore using tacrolimus in the perioperative period might be safer than cyclosporin A, which tends to worsen hepatic damage in the presence of hypoperfusion.

Unconventional Treatment of Severe Heart Failure in the Elderly

Z. Fidelman, Z. Korshun

Geriatric Medical Center, Natanya

In the elderly, congestive heart failure is common and associated with significant morbidity and mortality.

Comprehensive management of heart failure improves functional status and decreases mortality. Diuretics remain the mainstay in treating congestive heart failure. However, some patients are resistant to diuretics. This is a serious problem as it seems to represent a final manifestation of congestive heart failure.

In an 85-year-old woman diuretic resistance was recognized as the cause of failure of conventional diuretic therapy. When the dose of furosemide was rapidly increased from 120 mg per os to 1000 mg IV within 4 days, diuresis began and there was dramatic improvement in her condition. The dose was then gradually decreased until a conventional maintenance dose of 200 mg/day per os was reached. This dose was sufficient to keep her heart failure under control.

Identifying the Elderly at Risk for Falling 

D. Galinsky, V. Fried, A. Biderman, J. Cwikel, Y. Ben Moshe

Geriatric Dept., Soroka University Hospital and Depts. of Family Medicine and of Social Work, Faculty of Health Sciences, Ben-Gurion University of the Negev; and ESHEL, Beer Sheba

Falling is one of the main problems affecting the health of the elderly. A community project was carried out to detect elderly people at high risk for falls. One of its aims was also to develop tools allowing primary care professionals to detect the elderly at risk for falling. Such a screening test in the community-dwelling elderly (EFST) and a protocol for diagnosis and treatment of the elderly at risk for falls is presented.

Laser Treatment of Airway Obstruction in Infants and Children

Y. Efrati, S.M. Sarfaty, S. Kromholz, G. Eshel, M. Weinberg, I. Vinograd

Depts. of Pediatric Surgery, Otolaryngology, Anesthesia and Pediatric Intensive Care, Assaf Harofeh Medical Center, Zerifin (Affiliated with Sackler Faculty of Medicine, Tel Aviv University)

Airway obstruction during infancy and childhood requiring surgical ablation is rare, and surgical intervention poses a significant challenge. During recent decades, appropriate endoscopic instrumentation, together with advanced laser beam technology have provided new operative modalities for such patients.

From 1993 to 1995 we treated 40 infants and children, 26 males and 14 females, 13 days to 11 years old (mean 3.3 years) with Nd-YAG or CO² laser. Obstructing lesions included granulation tissue or polyps (16 cases), septa or webs (27), or benign tumors (4). 7 had more than a single lesion.

All were treated endoscopically under general anesthesia without any operative or postoperative deaths. Surgical intervention removed the obstruction and related symptoms in 34. In 6, laser treatment failed, necessitating additional surgical procedures. 3 had circumferential subglottic web. Operative complications included bleeding during removal of a hemangioma in 1 and recrudescence in another. Postoperative complications were transient respiratory failure and pneumonia in 6, all of which resolved with appropriate treatment.

This series proves that laser technology is feasible in the treatment of airway obstruction during infancy and childhood, and is safe and effective.

Treatment of Velopharyngeal Insufficiency

Abraham Amir, Ram Silfen, Daniel J. Hauben

Dept. of Plastic and Reconstructive Surgery, Rabin Medical Center, Beilinson Campus, Petah Tikvah

Weak and hypernasal speech, along with nasal escape of air, are the main characteristics of velo-pharyngeal incompetence (VPI). We describe 10 years of experience (1989-1998) with surgical treatment of VPI.

51 patients underwent pharyngeal flap elevation. 37 had cleft palate (8 of them submucous), 7 had neuromuscular disorders and another 7 were idiopathic. All underwent evaluation by a speech therapist before and after operation. 25 had further nasal endoscopy and/or videofluroscopy.

There was significant speech improvement in volume and clarity in 35 (73%), mild improvement in 13 (27%) and none in 3. The complication rate was 15% and included sleep apnea, wound infection and dehiscence, stridor and bleeding. Complications were correlated with advanced age.

VPI should be diagnosed as early as possible to achieve good results and to prevent social problems.

Left Ventricular Outflow Tract Obstruction without Left Ventric-Ular Hypertrophy Treated with Ace Inhibitors

Jacob Feldman, Irit Laxer, Abraham Yaretzky

Geriatric Dept., Meir Hospital, Sapir Medical Center, Kfar Saba, and Sackler Faculty of Medicine, Tel Aviv University

We describe a very unusual case of sudden, severe worsening of congestive heart failure which was caused by ACE inhibitors. Diagnosis was made by echocardiogram showing a typical picture of dynamic, left ventricular outflow tract obstruction without left ventricular hypertrophy, which disappeared on discontinuing ACE inhibitors. This phenomenon has already been described as a complication of other drugs such as nitrates, commonly used as provocative tests for latent obstructive cardiomyopathy. To our knowledge ACE inhibihave not been described as a causative factor.

Multiple Organ Damage due to Cholesterol Embolization

Ido Wolf, Meir Mouallem

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

Cholesterol crystal embolization can affect multiple organ systems and mimic other systemic diseases. We describe a 65-year-old woman who had renal failure, diarrhea, transient ischemic attacks and purple toes due to spontaneous cholesterol crystal embolization.

Tension-Free Vaginal Tape Procedure for Female Stress Incontinence

David Gordon, Asnat Groutz, Joseph Lessing

Lis Maternity Hospital, Sourasky-Tel Aviv Medical Center

We evaluated the safety and efficacy of the tension-free vaginal tape procedure (TVT) in 20 consecutive women with urodynamically proven genuine stress incontinence. Mean operating time under spinal analgesia was 37.2‏13 minutes and 80% were discharged within 24 hours. There were 5 uncomplicated, intraoperative bladder perforations, but no defects in healing nor tape rejection. The objective cure rate was 95%, while the subjective rate was 100%. Although follow-up was short (9-16 months), the TVT method seems to be safe and effective for stress urinary incontinence.

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.