תוצאת חיפוש

סרגיי יאלונצקי, אסעד חורי, יצחק שוורץ, אברהם לורבר

היחידה לקרדיולוגיה-ילדים ומומי לב מלידה במבוגר, בית-חולים מאייר לילדים, מרכז רפואי רמב"ם, חיפה

ההתפתחות האיבחונית והטכנולוגית מקדמת את אפשרויות הצינתור של חולים עם מומי לב מלידה. הניסיון הנרכש מאפשר יישום שיטות טיפול במומי לב משולבים מלידה. כך ניתן פיתרון חלופי ראוי לגישה הניתוחית המחייבת ניתוח לב פתוח, תוך חיבור החולה למכונת לב-ריאות. במאמר הנוכחי מובאת פרשת חולה, בת 31 שנה, אשר אובחנה לראשונה כלוקה בפגם מלידה בחיץ הבין-פרוזדורי ובהיצרות ניכרת במסתם הריאה עם כיחלון עמוק וירידה משמעותית בתיפקוד. פרשת חולה זו ממחישה את הקושי האיבחוני ואת האתגר הטיפולי במטופלים מבוגרים הלוקים במומי לב מורכבים. 

Long-Term Sequelae of Malignant Tumors in Childhood

N. M. Peretz, H. Goldberg, A. Kuten, I. Meller, E. Krivoi, A. Lorber, L. Bentur, A. Lightman, V. Gorenberg, M. Ben Arush-Weyl

Pediatric Hematology-Oncology Dept., Oncology Center, Pediatric Cardiology Unit, Pulmonology and Gynecology Depts., and Pediatric Endocrinology Unit; Rambam Medical Center and Technion-Israel Institute of Technology, Haifa; and Orthopedic-Oncology Unit, Tel Aviv-Sourasky Medical Center and Tel Aviv University

110 children with malignant diseases (leukemia excepted) who survived 5-20 years (median 9) post-therapy were followed (1996-1998). Median age during follow-up was 15 years (range 5-23). The most common malignancies were brain tumors, lymphoma, retinoblastoma and Wilm's tumor.

The 174 late side-effects included endocrine disorders (19%), cognitive impairment (14%), orthopedic dysfunction (12%), alopecia (12%), dental damage (11%), psychological (8%) and neurological (8%) disturbances, and azoospermia or amenorrhea (5%). There was no cardiac or renal damage and no second malignancy.

29% of side-effects were severe. There was significant reduction in quality of life in 54 (49%), in 27 of whom it was severe enough to require psychological intervention. Treatment of brain tumor caused 98 late side-effects in 28 patients (sequelae-to-patient ratio [SPR] 3.3). Most cognitive, endocrine and neurological disorders, and most cases of alopecia, dental and psychological difficulties were in these patients. There were frequent late complications in those treated for retinoblastoma (SPR 1.8), and bone or soft tissue sarcomas (SPR 0.8). Those treated for Wilm's tumor had few side-effects (SPR 0.4).

Late side effects were most frequent after radiation, reaching as high as SPR 2.4. It averaged only 0.5 in those treated with chemotherapy alone or in combination with surgery.

Reduction of late side-effects in these patients requires using less toxic modalities, as long as cure rate is not compromised. When considering secondary strategies, screening for early detection of late complications would enable immediate solutions, such as hormonal replacement or providing compensating skills for post-treatment disability.
 

Familial Occurrence of Ebstein Anomaly

Ruti Margalit-Stashefski, Avraham Lorber, Eyal Margalit

Family Practice Unit, Kupat Holim Klalit, Haifa, Pediatric Cardiology Unit, Rambam Hospital, Haifa and Ophthalmology Dept., Hadassah--University Hospital, Jerusalem

Ebstein anomaly is a rare congenital disease which affects location, structure and mobility of the tricuspid valve, and right atrium and ventricle. Although most cases are sporadic, familial occurrence has been reported. We report 2 brothers born with Ebstein anomaly. The parents were first degree cousins and there were 8 other children. 2 daughters were born with other congenital heart anomalies, 1 with ventricular septal defect and the other with severe pulmonary artery stenosis. We suggest that in some families, Ebstein anomaly is an autosomal dominant disease with different expression in the sexes.