Konjenital kalp hastalığı bulunan Down sendromlu olguların asimetrik dimetilarjinin düzeylerinin değerlendirilmesi

Amaç: Konjenital kalp hastalığı bulunan Down sendromlu hastalarda pulmoner hipertansiyon önemli bir morbidite ve mortalite nedenidir. Asimetrik dimetil arjinin (ADMA) nitrik oksit sentezinin endojen inhibitörüdür. Artmış plazma ADMA seviyeleri pulmoner hipertansiyonla ilişkili olabilir. Bu çalışmanın amacı konjenital kalp hastalığı bulunan Down sendromlu hastalarda pulmoner hipertansiyon ve ADMA arasındaki ilişkinin değerlendirilmesidir.Gereç ve Yöntem: Çalışma grubunu konjenital kalp hastalığı bulunan 21 Down sendromlu çocuk oluşturmaktadır. Plazma ADMA konsantrasyonları pulmoner hipertansiyon olup olmamasına göre analiz edildi.Bulgular: 21 hastanın ortalama yaşı 29,7±23,5 ay (3-88 ay) idi. Konjenital kalp hastalıklı altı hastaya pulmoner hipertansiyon eşlik etmekte idi. On beş hastada pulmoner hipertansiyon yoktu. ADMA seviyeleri açısından gruplar arasında istatistiksel olarak fark saptanmadı (p >0,05).Sonuçlar: Konjenital kalp hastalığı bulunan Down sendromlu çocuklarda pulmoner hipertansiyon ile ADMA seviyeleri arasında ilişki görülmedi. Bununla birlikte, Down sendromlu çocuklarda ADMA ve pulmoner hipertansiyon arasındaki ilişkinin saptanmasında daha gerçekçi sonuçlar elde etmek için geniş örneklem içeren prospektif, çok merkezli çalışmalara ihtiyaç vardır.

Anahtar Kelimeler:

ADMA, pulmoner hipertansiyon, asimetrik dimetil arjinin, down sendromu

Evaluation of asymmethric dimethylarginine levels in Down syndrome patients with congenital heart disease

Aim: Pulmonary hypertension is a major cause of morbidity and mortality among Down syndrome patients with congenital heart disease. Asymmethric dimethyl arginine (ADMA) is an endogenous inhibitor of nitric oxide synthesis. Increased plasma levels of ADMA may be related with pulmonary hypertension. The aim of this study was to evaluate the relationship between ADMA and pulmonary hypertension in Down syndrome patients with congenital heart disease.Material and Method: The study group consisted of 21 children with Down syndrome with congenital heart disease. Plasma ADMA concentrations were analyzed according to the presence or absence of pulmonary hypertension.Results: The mean age of the 21 patients was 29.7±23.5 months (range: 3-88 months). Six patients with congenital heart disease had accompanied with pulmonary hypertension. Fifteen patients had no pulmonary hypertension. The levels of ADMA showed no statistically significant difference between the groups (p >0.05).Conclusion: No correlation was shown between ADMA levels and pulmonary hypertension in children with Down syndrome with congenital heart disease. However, multicenter prospective studies with enlarged sample sized are needed to provide more realistic results about the connection between ADMA and pulmonary hypertension in Down syndrome children.

Keywords:

ADMA, Down syndrome, pulmonary hypertension, asymmethric dimethyl arginine,

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