Enteropathy. X–Linked Syndrome

  • Дмитрий Олегович Иванов Saint-Petersburg State Pediatric Medical University 194100, Russia, Saint-Рetersburg, Litovskaya str., 2
  • Валерия Павловна Новикова Saint-Petersburg State Pediatric Medical University 194100, Russia, Saint-Рetersburg, Litovskaya str., 2
  • Наталья Эдуардовна Прокопьева Saint-Petersburg State Pediatric Medical University 194100, Russia, Saint-Рetersburg, Litovskaya str., 2
Keywords:
врожденная диарея с гипериммуноглобулинемией E и отсутствием островков Лангерганса X-сцепленное заболевание диарея и полиэндокринопатия congenital diarrhea with hyperimmunoglobulinemia E and absence of Langerhans Islands X‑linked disease diarrhea and polyendocrinopathy

Abstract

The review defines congenital diarrhea with hyperimmunoglobulinemia E and absence of Langerhans Islands, (X‑linked syndrome) describes its prevalence, etiology, pathogenesis, clinical picture, diagnosis and differential diagnosis, treatment and prognosis. The prognosis of this triad is unfavorable.

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