title: An unusual presentation of galactosemia: hemophagocytic lymphohistiocytosis.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition. Uncontrolled proliferation of activated lymphocytes secreting high amounts of inflammatory cytokines seems to be the main pathogenesis. The diagnosis of HLH can often be difficult. It may presents in many forms such as fever of unknown origin, hepatitis, acute liver failure, and sepsis-like illness. Here we present a newborn galactosemia case presented with HLH. Close monitoring of the diagnostic criteria of HLH during the course of galactosemia-associated hemophagocytosis, both before and after dietary treatment, should be performed in order to fully determine if the triggering mechanism is infection or accumulation of metabolites.
  
  
  BACKGROUND
  None declared.
authors.affiliation: Dr.Sami Ulus Hospital, Neonatology Department, Ankara, Turkey.
authors.forename: Ahmet Afşin
authors.initials: AA
authors.lastname: Kundak
authors.affiliation: Dr.Sami Ulus Hospital, Neonatology Department, Ankara, Turkey.
authors.forename: Ayşegül
authors.initials: A
authors.lastname: Zenciroğlu
authors.affiliation: Ankara Pediatric Hematology Oncology Training and Research Hospital, Ankara, Turkey.
authors.forename: Neşe
authors.initials: N
authors.lastname: Yaralı
authors.affiliation: Dr.Sami Ulus Hospital, Neonatology Department, Ankara, Turkey.
authors.forename: Belma
authors.initials: B
authors.lastname: Saygılı Karagöl
authors.affiliation: Dr.Sami Ulus Hospital, Neonatology Department, Ankara, Turkey.
authors.forename: Arzu
authors.initials: A
authors.lastname: Dursun
authors.affiliation: Dr.Sami Ulus Hospital, Gastroenterology Department, Ankara, Turkey.
authors.forename: Selim
authors.initials: S
authors.lastname: Gökçe
authors.affiliation: Dr.Sami Ulus Hospital, Neonatology Department, Ankara, Turkey.
authors.forename: Nilgün
authors.initials: N
authors.lastname: Karadağ
authors.affiliation: Dr.Sami Ulus Hospital, Neonatology Department, Ankara, Turkey.
authors.forename: Nurullah
authors.initials: N
authors.lastname: Okumuş
keywords: Galactosemia; Hemophagocytic lymphohistiocytosis; newborn