title: Diabetes Insipidus and Sclerosing Cholangitis in a child may be a clue to the diagnosis of Langerhans' Cell Histiocytosis: A Case Report.
abstract: Langerhans cell histiocytosis (LCH) is a rare disease that usually affects children and young adults. Sclerosing cholangitis (SC) can occur in 10-15% of patients with disseminated form of the disease. Central diabetes insipidus (CDI) is a rare disorder that may be caused by a variety of diseases mainly LCH and germinoma especially in children. In this case report, a- 4-year-old girl who is a known case of CDI and a single bone lesion in the left humerus, presented with jaundice, abdominal distention and itching. The diagnosis of SC was made by histopathology on liver biopsy. In this case, we found a link between CDI and SC through LCH, the diagnosis of which was made by histopathology of the explanted liver. The combination of CDI, liver involvement with SC and a single bone lesion is remarkable, since the histological diagnosis of LCH was made outside the biliary tract in the liver parenchyma.
authors.affiliation: Department of Pathology, College of Medicine, Qassim University, Qassim, Saudi Arabia.
authors.forename: Abdulaziz A
authors.initials: AA
authors.lastname: Al Salloom
authors.affiliation: Department of Pathology, Riyadh Armed Forces Hospital, Riyadh, Saudi Arabia.
authors.forename: Salman T
authors.initials: ST
authors.lastname: Almalki
authors.affiliation: Department of Pathology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors.forename: Hadeel
authors.initials: H
authors.lastname: Almana
authors.affiliation: Department of Organ Transplant, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia burdelsk@uke.uni-hamburg.de .
authors.forename: Martin
authors.initials: M
authors.lastname: Burdelski