תוצאת חיפוש

שי שרוט¹, יואב צפמן¹, יהודה שינפלד²

¹המח' למחלות עצבים, המרכז הרפואית תל-אביב, ²מח' לרפואה פנימית ב' והמרכז למחלות אוטואימוניות, בי"ח שיבא, תל-השומר

תיסמונת האנטיפוספוליפידיים, antiphospholipid syndrome (APS) היא מחלה רב-מערכתית שבה מעורבים נוגדנים ואנטיגנים שונים. הפרעה האוטואימונית מתבטאת קלינית באירועים פקקתיים-תסחיפיים נשנים (עורקיים וורידיים), בהפלות עצמוניות נשנות, בתרומבוציטופניה ובכייל גבוה של נוגדנים כנגד פוספוליפידים ((antiphospholipid-antibodies - aPL וכן של נוגד קרישה זאבתי (lupus anticoagulant - LA). התיסמונת יכולה להתרחש משנית למחלות אוטואימוניות אחרות (כמו זאבת אדמנתית מערכתית systemic-lupus-erythematosus (SLE), לממאירויות או לזיהומים וכן כתגובה לתרופות. כשלא נמצאת סיבה ראשונית מעין אלו להתהוות התיסמונת, היא מוגדרת כראשונית (primary APS).

הנוגדנים הנמנים עם משפחת ה- aPL מאותרים בבדיקה שבה נמדדת תגובתיות (reactivity) חיסונית כלפי קרדיוליפין (aCL) ופוספוליפידים למיניהם הטעונים שלילית, בנוכחות או ללא נוכחות  ביתא 2 – גליקופרוטאין או קופקטורים חלבוניים אחרים, או ע"י תגובתיות חיסונית כלפי ביתא 2 – גליקופרוטאין (הידוע כקופקטור חיוני לקישור ה- aPL) או קופקטורים חלבוניים אחרים לבד, או על ידי יכולתם לעכב את מנגנון הקרישה בתבחיני קרישה התלויים בפוספוליפידים (LA=).

לא כל בני-האדם עם aPL יהיו תסמיניים. בסקר פרוספקטיבי של בנק הדם התגלה, שב- 6.5% מהאוכלוסייה הכללית אובחנו בתבחין ELISA נוגדנים מסוג IgP-aPL. לעתים קרובות, יורד כיל-ה- aPLלטווח התקין עם הזמן, ללא אירועים פקקתיים.

בחולים הלוקים ב- APS, דווח על ביטויים קליניים שונים, הכוללים סיבוכים המאטולוגיים, סיבוכים בעור, מחלת מסתמי הלב והיפגעות הכליות. בנוסף, דווח על ביטויים נירולוגיים שונים המערבים בעיקר את מערכת העצבים המרכזית. בסקירה זו, נדונים ההיבטים הנירולוגיים של תיסמונת הנוגדנים האנטיפוספוליפידיים.
 

האתיקה הרפואית, כבר מימיה הראשונים, מטילה על הרופא אחריות לטובת החולה. בזמננו, ממשיכים הקודים לתמוך במסורת זו, וקובעים מחויבות להגיש טיפול יעיל ומצפוני לטובת החולה מעבר לשיקול דעת הרופא עצמו, על הצהרת ג'נבה (1948) "בריאות החולה שלי היא חשיבותי הראשונה". בין היסודות של כללי ההתנהגות האתית מצויה מחד גיסא מחויבות של הרופא להגיש לחולה מידע שיעזור לו לקבל החלטות, ומאידך גיסא הצורך לשמור על הסודיות כדי למנוע פגיעה בחולה על ידי הציבור. נשאלת השאלה, האם ההתפתחויות בכלי התקשורת והאינטרנט משרתות את החולה או פוגעות בו.

Surgical Repair of Myxomatous Mitral Valve

D. Bitran, O. Merin, I. Dzigivker, D.Rosenmann, S. Od-allah, S. Silberman

Depts. of Cardiothoracic Surgery and of Anesthesiology and Cardiology; Shaare Zedek Medical Center, Jerusalem

Myxomatous mitral valve disease is now the most common cause of mitral regurgitation in the western world. Repair of the leaking valve has become standard surgical procedure during the past 2 decades. Between 1993-1999 we performed 113 repairs of the mitral valve. In 25 patients the etiology was myxomatous degeneration (no mortality). Long-term clinical results depend on patients' functional class at surgery. Based on this fact, and the good surgical results, it is recommended to refer such patients even with severe mitral incompetence for surgery at an early stage, even if symptoms are minimal.

Antioxidant Profile of Human Saliva and its Biological Significance

R. M. Nagler, A. Z. Reznick

Dept. of Oral and Maxillofacial Surgery, Salivary Clinic and Oral Biochemistry Laboratory; Rambam Medical Center and Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

Saliva is the first biological medium to come in contact with materials entering the body through the oral cavity. These materials are contained in food and drink (or inhaled as volatile ingredients). Accordingly, saliva contains various defense mechanisms which have been thoroughly investigated. They include immunological and enzymatic systems aimed at pathological microorganisms. Saliva also can protect the mucosa against mechanical insults and when required, promote its healing via agents such as the epidermal growth factor.

Another defense mechanism in saliva which has been gaining increased attention and seems to be of paramount importance is its antioxidant system. We discuss both the molecular and enzymatic components of the salivary antioxidant system in respect to mixed, parotid, submandibular and sublingual saliva and under various physiological conditions of secretion.

Surgery for Blepharoptosis in Muscular Dystrophy

Ori Mahler, Asher Milstein, Ayala Pollack, Daniel Hauben

Eye Dept., Kaplan Medical Center, Rehovot; and Plastic Surgery Dept., Rabin Medical Center, Petah Tikva

In some muscular dystrophies there is ocular involvement characterized by blepharoptosis and ophthalmoplegia. These conditions occur in chronic progressive external ophthalmoplegia, oculopharyngeal muscular dystrophy, mitochondrial myopathy, myotonic dystrophy, and ocular myasthenia, among others. Although they differ in their systemic clinical manifestations and in genetic inheritance, ocular involvement is common to all of them. Manifestations include bilateral progressive blepharoptosis with or without extraocular muscle malfunction.

During surgical repair of the ptotic eyelid, consideration must be given to eyeball movements, in addition to maximal eyelid elevation, and to avoiding overcorrection and consequent corneal overexposure, leading to dryness and visual impairment. With these muscular dystrophic disorders, resection of the levator muscle or blepharoplasty alone does not suffice. Follow-up shows that most patients need a secondary repair after a short while. Operative correction uses a frontalis sling for eyelid elevation and support.

A series of 8 patients with these diseases, operated on by various surgical techniques during the past 7 years, is presented.

Folic Acid for Preventing Neural Tube Defects

Ziv Gil, Adi Aran, Orna Friedman, Liana Beni-Adani, Shlomo Constantini

Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba; Division of Pediatric Neurosurgery, Dana Children's Hospital, Sourasky Medical Center and Sackler Faculty of Medicine, Tel Aviv

Spina bifida and anencephaly are the most common, serious malformations in neural tube defects (NTD). Randomized trials in the last 2 decades have demonstrated that folic acid, 0.4 mg/d, reduces the incidence of NTD by more than 50%. We investigated the use of folic acid and multivitamins containing folic acid in childbearing women.

Of 221 women interviewed, 67 (30%) regularly took pills containing 0.4 mg folic acid. Women with higher educational levels were more likely to take multivitamins with folic acid than were the less educated (p=0.05). Of the women who took folic acid, only 5 (7.5%) used separate folic acid tablets, before and during their pregnancy. The rest used multivitamins containing folic acid. The 5 women who took folic acid separately were college-educated and nonreligious, and they took multivitamins in addition (p>0.05).

Of the women interviewed, 58 (26.2%) were Bedouin of the Negev. 24 (41.4%) of them took pills containing folic acid on a regular basis. This percentage is higher than that in the Jewish women in the study who took folic acid for prevention of NTD (17%; p=0.038).

Most of the women took folic acid after the first trimester. Only a minority took daily periconceptional folic acid. Multivitamins containing 0.4 mg of folic acid were more popular than folic acid tablets alone. This study emphasizes the need for continuing efforts to increase consumption of folic acid and awareness of its benefits among women of childbearing age.

Thrombocytopenic Purpura as Sole Manifestation of Brucellosis in a Child

Ronen Marom, Dan Miron, Herzel Gabriel, Yosef Horowitz

Pediatric Dept. A, Pediatric Infectious Disease Service, and Pediatric Hemato-Oncology Unit, HaEmek Medical Center, Afula and Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

Thrombocytopenic purpura associated with brucellosis has rarely been described in children. The thrombocytopenic purpura is usually part of the array of manifestations of brucellosis, such as fever, malaise, arthralgia, arthritis, hepatosplenomegaly and lymphadenopathy.

We describe a 4-year-old girl in whom severe thrombocytopenic purpura was the only manifestation of brucellosis which resolved after appropriate antibiotic therapy. We conclude that brucellosis should be included in the differential diagnosis of thrombocytopenic purpura in areas endemic for brucellosis, and when there is a history of exposure to infected food products.

Preservation of the Larynx in Advanced Cancer

Edward Rosenblatt, Nava Siegelmann-Danieli, Jemal Zidan, Nisim Haim, Abraham Kuten

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

The effectiveness of sequential chemo-radiotherapy in preserving the larynx in advanced laryngeal carcinoma was assessed. 4 Unselected patients (19 men and 2 women, mean age 60 years) with advanced squamous cell carcinoma of the larynx (T3-4/N0-3) received induction chemotherapy consisting of 2-3 cycles of cisplatin (100 mg/m²) and 5-flourouracil (1000 mg/m²/day) as a continuous infusion on days 1-5 followed by definitive radiotherapy: 50 Gy to the whole neck, 70 Gy to the larynx and clinically involved nodes, using a combination of 6 MV photons and 9-12 MeV electrons.

19 of The 21 patients responded to combined therapy but there was no response to induction therapy in 2 (10%) and 2 did not complete therapy due to severe toxicity. At a mean follow-up of 40 months, 7 had undergone total laryngectomy (33%), for an overall 5-year laryngeal preservation rate of 66%. Reasons for total laryngectomy in 2 patients were no response and in 5 tumor recurrence.

Mean survival was 39 months (range 11-46 months); at last follow-up 17 of 21 were alive and disease-free, 11 of whom had a functional larynx (65% of survivors). 2 died due to disease progression and 1 due to a cardiovascular event. Sequential chemo-radiation allows laryngeal preservation in about 2/3 of surviving patients without compromising survival.

Secondary Bone Grafting in Cleft Lip and Palate

M. Peled, D. Aisenbud, D. Goldstein, A. Rachmiel, D. Laufer

Maxillofacial and Oral Surgery Dept. and Cleft-Palate Clinic, Rambam Medical Center; and Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

Results of reconstruction of residual alveolar bone defects in 52 patients operated between 1990-1998 were evaluated clinically and radiographically in a retrospective study.

Ages ranged between 9-37; 30 were males. The donor site of bone grafts in all was particulate cancellous marrow from the anterior iliac crest. 32 had unilateral clefts and 20 bilateral. Total cleft sites treated was 72.

Best results were achieved when bone grafting was carried out prior to the eruption of the canine tooth. The cleft space was closed and oro-nasal fistulas were eliminated in 42 (80%). Success rates in unilateral and bilateral cases were significantly different.

Marked Creatine-Phosphokinase Elevation in Myopathy after Treatment with Bezafibrate

G. Slobodin, D. Yeshurun

Medicine A Dept. and Hyperlipidemia Clinic, Bnai Zion Medical Center,Bruce Rappaport Faculty of Medicine and Technion-Institute of Technology, Haifa

Bezafibrate is a fibric acid derivative which has been widely used in the past 15 years. Recent studies have elucidated much of its mechanism of action, which mainly results in reduction of VLDL and triglyceride levels and in elevation of HDL. The drug is relatively safe and its side-effects well known, mild, and reversible.

The most severe side-effect is myositis, varying from mild flu-like symptoms to rhabdomyolysis, which is extremely rare. The underlying situations most frequently associated with bezafibrate-induced myositis are renal insufficiency and concomitant treatment with certain other drugs.

We describe 2 women who developed severe myositis with bezafibrate treatment. 1, aged 43, who had moderate diabetes but no renal insufficiency, was treated with metformin and warfarin, which can interact with bezafibrate and affect its metabolism. The other, aged 54, had renal insufficiency and was on home peritoneal dialysis. Her bezafibrate dose had been increased because of very high triglyceride levels.

The aim of the study is to call attention to this significant side-effect of benzafibrate and to ways of preventing it.

High-Dose Chemotherapy and Autologous Stem Cell Trans-Plantation for Refractory and Relapsing Hodgkin's Disease

A. Avigdor, I. Hardan, O. Shpilberg, P. Raanani, I. Grotto, I. Ben-Bassat

Hematology Institute and Hemato-oncology Unit, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

High dose chemotherapy and autologous stem cell transplantation are widely used in relapsed and primary refractory Hodgkin's disease. We transplanted 42 patients with Hodgkin's disease between 1990-1998. Median follow-up was 31 months (range 1-102). 29 (69%) were transplanted after relapse and 13 (31%) were refractory to first line therapy. Median age at transplantation was 29 years (range 19-58) and 23 (55%) were males.

All were treated with the BEAM protocol (carmustine, etoposide, cytarabine and melphelan). 18 who were in remission received radiotherapy following transplantation. The source of the stem cells was bone marrow in 17% and peripheral blood in 83%. At initial diagnosis: 57% had stage III-IV disease and B symptoms were present in 52%. 75% were treated with MOPP, ABVD or with related versions. Radiotherapy followed in 52%. Prior to transplantation, 45% of the relapsed group were in the advanced stage. 33% and 12% of all patients had lung and bone involvement, respectively.

The complete remission rate was 86% for the 2 groups. 2 (5%) died from transplant-related complications and MDS/AML developed in 2 (5%) after transplantation. The 3-year overall survival (OS) and disease-free survival (DFS) were 68% and 60%, respectively. The 3-year OS for the relapsed group was 64% compared with 76% for the refractory group, and the 3-year DFS for the relapsed group was 60% vs. 42% for the refractory group (neither difference significant). Radiotherapy following transplantation did not have a beneficial effect on DFS. No prognostic factors for outcome of transplantation were found, most probably due to the limited number of patients and the high variability of disease characteristics.

We conclude that high dose chemotherapy and autologous stem cell transplantation are effective and relatively safe for relapsed or primary refractory Hodgkin's disease. The DFS at 3 years was longer for those transplanted after relapse than those with primary refractory disease, but not significantly. Patients with primary refractory disease can be salvaged with high dose chemotherapy.

נתן רוז'נסקי, אברהם בן-שושן

מח' לרפואת נשים ויולדות, ביה"ח הדסה, עיןכרם, הפקולטה לרפואה של האוניברסיטה העברית, ירושלים

אורה שובמן, בוריס גילבורד, פנינה לנגביץ', יהודה שינפלד

המח' לרפואה פנימית ב' והיחידה לחקר מחלות אוטואימוניות, המרכז הרפואי שיבא תל השומר והפקולטה לרפואה סאקלר, אוניברסיטת תל אביב

מילות מפתח:

נוגדני RA33, RA33, מחלות אוטואימוניות, אורה שובמן, בוריס גילבורד, פנינה לנגביץ', יהודה שינפלד, שובמן, גילבורד, לנגביץ', שינפלד

Familial Parinaud Oculo-Glandular Syndrome in Cat-Scratch Disease 

Nir Shoham, Dan Miron, Raul Raz, Hanna J. Garzozi

Depts. of Ophthalmology and Pediatrics A, and Infectious Diseases Unit, HaEmek Medical Center, Afula

Cat-scratch disease is manifested by subacute, regional lymphadenitis and occurs mainly in children. The causative agent is a pleomorphic, gram-negative bacillus, Bartonella henselae carried by asymptomatic cats. Parinaud oculoglandular syndrome is the most common ocular manifestation of this disease. It is characterized by unilateral conjunctivitis with polypoid granuloma, usually of the palpebral conjunctiva, and preauricular lymphadenopathy. The diagnosis is supported by a history of exposure to cats and is confirmed by positive serologic tests or positive PCR assay.

The occurrence of more than 1 case of Parinaud syndrome in a family is rare. We describe 2 sisters with Parinaud oculoglandular syndrome, proven by serologic tests. They reported that they used to cuddle with their cats, among them a kitten. Because of the refractory conjunctivitis and signs of imminent periorbital cellulitis, they were treated with oral tetracycline with apparently good responses.

We recommend asking about contacts with cats in any atypical conjunctivitis accompanied by regional lymphaden-opathy, especially in young patients. Systemic antibiotics should be given when there is any suspicion of significant ocular involvement, if the patient is immunosuppressed, or if there are systemic manifestations of cat-scratch disease.

Benign Paroxysmal Positional Vertigo: Diagnostic Pitfalls 

C.R. Gordon, O. Zur, R. Furas, E. Kott, N. Gadoth

Depts. of Neurology and of Physical Therapy, Meir General Hospital, Kfar Saba, and Sackler Faculty of Medicine, Tel Aviv University

Benign paroxysmal positional vertigo (BPPV) is a common but often unrecognized cause of treatable vertigo. Possible causes of misdiagnosis of BPPV were studied by review of the records of 191 patients referred to our neurology clinic; 36 were identified as having BPPV not previously diagnosed. On referral the patients carried the following diagnoses: unspecified dizziness/vertigo (33%), transient ischemic attacks (28%), cervicogenic vertigo (19%), psychogenic dizziness/vertigo (11%), and others (8%).

The paroxysmal nature of the vertigo and position-precipitating factors were not spontaneously reported by 31%. Atypical, even bizarre, symptoms including dizzy sensations were reported by 19%, and neck pain and headache were cardinal symptoms that accompanied vertigo.

The Dix-Hallpike maneuver, which is essential for the diagnosis of BPPV, was not performed in any of the patients prior to referral. 30 (83%) experienced complete resolution of signs and symptoms after the first physical treatment session.

We conclude that non-paroxysmal, non-positional vertigo does not rule out BPPV. Atypical and even bizarre complaints of dizziness, as well as neck pain and headache could be cardinal symptoms of BPPV. The Dix-Hallpike maneuver is mandatory in those complaining of dizziness and vertigo.