Çocuklarda Kistik Fibrozis İlişkili Diyabet
Özet
Kistik fibrozis ilişkili diyabet (KFRD), kistik fibrozis (KF) hastalarının yaşam sürelerinin uzamasıyla prevalansı artan en yaygın komplikasyondur. Tip 1 diyabetin aksine otoimmün kaynaklı olmayan KFRD, ekzokrin pankreastaki viskoz salgıların neden olduğu obstrüktif hasar, ilerleyici fibrozis ve β hücre kaybı sonucu gelişen insülin eksikliği ve enfeksiyonlar veya steroid tedavileriyle tetiklenen geçici insülin direnci ile karakterizedir. Hastalık genellikle sinsi ilerler; klasik diyabet semptomları yerine çocuklarda büyüme geriliği ve kilo kaybı ile kendini gösterebilir. Tanı ve rutin taramada 10 yaşından itibaren yıllık 2 saatlik oral glukoz tolerans testi (OGTT) önerilmektedir. Tedavide temel yaklaşım insülin olup, hastaların yüksek kalorili ve yüksek yağlı özel KF diyetlerinin sürdürülmesi esastır. KFRD, mikrovasküler komplikasyonlara yol açabilse de makrovasküler tutulum izlenmez ve mortalite genellikle kardiyovasküler nedenlerden ziyade pulmoner yetmezliğe bağlıdır.
Cystic fibrosis-related diabetes (CFRD) is the most common complication with increasing prevalence as the life expectancy of cystic fibrosis (CF) patients extends. Unlike type 1 diabetes, CFRD is not autoimmune; it is characterized by insulin deficiency resulting from obstructive damage, progressive fibrosis, and β-cell loss caused by viscous secretions in the exocrine pancreas, alongside transient insulin resistance triggered by acute infections or steroid therapies. The disease progresses insidiously, presenting with growth failure and weight loss in children rather than classic diabetes symptoms. Standard screening via a 2-hour oral glucose tolerance test (OGTT) is recommended annually starting at age 10. Insulin is the primary treatment, and maintaining the high-calorie, high-fat CF diet remains essential. Although CFRD can lead to microvascular complications, macrovascular involvement is absent, and mortality is driven by pulmonary failure rather than cardiovascular causes.
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