Mukopolisakkaridozlarda kardiyak tutulum
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Date
2010-03-10
Authors
Babaoğlu, Kadir
Özsoy, Gamze
Binnetoğlu, Köksal
Journal Title
Journal ISSN
Volume Title
Publisher
Uludağ Üniversitesi
Abstract
Giriş: Mukopolisakkaridozlar (MPS), lizozomal depo hastalıklarının önemli bir grubudur. Literatürde yayınlanmış vaka serilerinde kardiyovasküler tutulumun MPS’ye sıklıkla eşlik ettiği belirtilmektedir. MPS’li hastalarda en sık görülen kardiyak bulgular, mitral kapakta kalınlaşma ve buna eşlik edebilen prolapsus, yetersizlik ve daha az sıklıkla darlıklardır. Bu çalışma ile MPS tanısı alan hastalarda mukopolisakkaridozlar ve kalp tutulumunun irdelenmesi amaçlanmıştır. Gereç ve Yöntem: Bu çalışmada 2005-2009 yılları arasında mukopolisakkaridoz tanısı nedeniyle kardiyak inceleme amacıyla bölümümüze gönderilen 11 hastanın (5 erkek, 6 kız) kardiyak bulguları geriye dönük olarak değerlendirildi. Kardiyak değerlendirme fizik muayene, elektrokardiyografi, telekardiyografi ve ekokardiyografik inceleme ile gerçekleştirildi. Bulgular: Hastaların başvuru yaşı 1,5 ile 14 yıl arasında değişiyordu ve yaş ortalaması 7,44±5,35 (ortanca 7) yıl idi. MPS tip II ve tip IV üçer hastada saptanmıştı. Tip I iki hastada tip VI ise bir hastada mevcuttu. ‹ki hastanın tiplendirilmesi henüz sonuçlanmamıştı. Hastaların tamamında mitral kapak, 4’ünde (%36,3) mitral kapak tutulumuna ilaveten aort kapak tutulumu ve ikisinde ise ilaveten triküspit kapak tutulumu vardı. Bir hastada interventriküler septumda ve sol ventrikül arka duvarda hipertrofi mevcuttu. Pulmoner hipertansiyon ise sadece 1 hastada saptandı. Hastaların hiçbirinde herhangi bir ritm problemi bulunmadı. Sonuç: Mukopolisakkaridozlarda kardiyak tutulum sıktır. Mitral kapak anormallikleri en sık görülen bulgudur. MPS tanısıyla takip edilen hastaların ekokardiyografik incelemeleri, asemptomatik olsalar dahi rutin olarak yapılmalı ve aralıklarla tekrarlanmalıdır.
Introduction: Mucopolysaccharidoses (MPS) are an important group of lysosamal storage diseases. Commonly reported cardiac involvements are mitral leaflet thickening and accompanying prolapsus, regurgitation and rarely stenosis. In this study, it was aimed to evaluate cardiac in involvements in patients with MPS. Materials and Method: We studied a total of 11 children with MPS who were admitted to our department between 2005 and 2009. Cardiac status was evaluated retrospectively with telecardiography, electrocardiography and echocardiography. Results: The ages of the patients (6 girls and 5 boys) ranged between 1.5-14 years and the mean age was 7.44±5.35 (median 7) years. Each of Type II and IV were identified in three patients; type I in two and type VI in one. In two patients, MPS type has not been identified yet. Thickening of the mitral valve with or without regurgitaion and prolapsus was the most common lesion seen in all patients. Additional involvement of the aortic valve was detected in 4 (36.3%) and additional involvement of tricuspid valve in 2 patients. Septal hypertrophy was found in one patient and pulmonary hypertension in another. No arrhythmia was found in any patient. Conclusion: Cardiac involvement is frequent in MPS. Mitral valve deformation is the most frequent finding. Echocardiographic examination should be performed periodically even if the patient has no clinical signs of cardiac disease.
Introduction: Mucopolysaccharidoses (MPS) are an important group of lysosamal storage diseases. Commonly reported cardiac involvements are mitral leaflet thickening and accompanying prolapsus, regurgitation and rarely stenosis. In this study, it was aimed to evaluate cardiac in involvements in patients with MPS. Materials and Method: We studied a total of 11 children with MPS who were admitted to our department between 2005 and 2009. Cardiac status was evaluated retrospectively with telecardiography, electrocardiography and echocardiography. Results: The ages of the patients (6 girls and 5 boys) ranged between 1.5-14 years and the mean age was 7.44±5.35 (median 7) years. Each of Type II and IV were identified in three patients; type I in two and type VI in one. In two patients, MPS type has not been identified yet. Thickening of the mitral valve with or without regurgitaion and prolapsus was the most common lesion seen in all patients. Additional involvement of the aortic valve was detected in 4 (36.3%) and additional involvement of tricuspid valve in 2 patients. Septal hypertrophy was found in one patient and pulmonary hypertension in another. No arrhythmia was found in any patient. Conclusion: Cardiac involvement is frequent in MPS. Mitral valve deformation is the most frequent finding. Echocardiographic examination should be performed periodically even if the patient has no clinical signs of cardiac disease.
Description
Keywords
Mukopolisakkaridozlar, Çocuklar, Ekokardiyografi, Kalp hastalıkları, Mucopolysaccharidoses, Children, Echocardiography, Cardiac diseases
Citation
Babaoğlu, K. vd. (2010). "Mukopolisakkaridozlarda kardiyak tutulum". Güncel Pediatri, 8(2), 48-51.