Author/Editor		Novosel-Sever, M; Paro-Panjan, D; Bratanič, B
Title		Akutna diseminirana oblika Letterer-Siwejeve bolezni pri novorojencu
Translated title		Acute disseminated form of Letterer-Siwe disease in a newborn
Type		članek
Source		Slov Pediatr
Vol. and No.		Letnik 6, št. 1-2
Publication year		1999
Volume		str. 31-3
Language		slo
Abstract		The aetiology of Letterer-Siwe disease is unknown and it usually presents as LCH (Langerhans cell histiocytosis). It is most common in the first year of life and until the end of the third year. However, cases of LCH in adults have been described. 0ur patient was a 6-week-old girl with acute congenital disseminated LCH. At birth the disease presented with skin changes and later spread to all internal organs except the central nervous system. Death was the consequence of acute respiratory failure.
Summary		Letterer-Siwejeva bolezen je navadno diseminirana oblika histiocitoze Langerhansovih velikank (LCH = Langerhans cell histiocytosis). Večinoma zbolevajo otroci v prvem letu starosti, redko odrasli. Etiologija ni znana. Prikazan je primer 6-tedenske deklice z akutno kongenitalno diseminirano obliko LCH. Bolezen se je pričela ob rojstvu s kožnimi spremembami, nato je zajela vse organe, razen osrednjega živčevja. Smrt je nastopila zaradi dihalne stiske.
Descriptors		HISTIOCYTOSIS, LANGERHANS-CELL LETTERER-SIWE DISEASE INFANT, NEWBORN