Imaging findings of wolman disease.

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Aslan M., Kalyoncu Uçar A., Arıöz Habibi H., Namdar Y., Kuruğoğlu S., Adaletli İ.

The International Pediatric Radiology (IPR) 7th Conjoint Meeting and Exhibition, Illinois, Amerika Birleşik Devletleri, 15 - 20 Mayıs 2016, ss.372, (Özet Bildiri)

• Yayın Türü: Bildiri / Özet Bildiri
• Basıldığı Şehir: Illinois
• Basıldığı Ülke: Amerika Birleşik Devletleri
• Sayfa Sayıları: ss.372
• İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet

Özet

Purpose or Case Report: Wolman disease is a rare autosomal recessive inherited disease characterized by storage of cholesterol esters and triglycerides in lysosomes due to a deficiency of lysosomal acid lipase. Clinical signs such as persistent vomiting, diarrhea, hepatosplenomegaly, growth retardation and liver dysfunction occur in the first weeks of life. Most cases die in the first year of life. Here, we aim to present radiological findings of Wolman disease.