Acquired acrodermatitis enteropathica and Hirschsprung's disease Edinsel Akrodermatit Enteropatika ve Hirschsprung Hastaliǧi


ŞİKAR AKTÜRK A., Imanli Ü., Aktürk H., EKİNGEN YILDIZ G.

Turkiye Klinikleri Pediatri, cilt.27, sa.3, ss.163-166, 2018 (Scopus, TRDizin) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 27 Sayı: 3
  • Basım Tarihi: 2018
  • Doi Numarası: 10.5336/pediatr.2018-60091
  • Dergi Adı: Turkiye Klinikleri Pediatri
  • Derginin Tarandığı İndeksler: Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.163-166
  • Anahtar Kelimeler: Acquired, Acrodermatitis enteropathy, Hirschsprung's disease
  • Kocaeli Üniversitesi Adresli: Evet

Özet

Acrodermatitis enteropathica (AE) is a rare genetic disorder characterized by diarrhea, periorificial and acral dermatitis and alopecia due to zinc deficiency. It may also occur as a secondary with insufficient zinc intake, liver and kidney disease, dietary habits and malabsorption. Genetic and secondary AE have similar clinical presentations. Untreated infants exhibit severe growth retardation, secondary bacterial and fungal infections, and neurologic changes; however, all symptoms are reversible with zinc therapy. Diagnosis of AE can be confirmed by detection of low serum zinc levels and rapid clinical response to systemic zinc supplementation. In this article, we present a case of acquired AE developing after total colectomy and subtotal gastroectomy due to Hirschsprung's disease.