[A disseminated form of histiocytosis X in infants].
Over the last twelve years, only three cases of a dissiminated form of histiocytosis X, in infancy, have been treated at the Institute for Mother and Child Health Care in Novi Sad. The clinical picture exhibits a very characteristic, prolonged (from two to nine months) first stage of the illness. Its general symptoms are: fever, general decline of the infant, seborrheic dermatitis, coughing and recurrent purulent otitis. In the second stage the symptoms are even more pronounced but they depend on localization and the functional deterioration of the organs involved: the lungs, liver, pancreas, skin, bone marrow and lymph nodes. The article underlines the importance of hematological, cytological and histiopathological analyses in diagnosis, and it gives the results for the cases in question. Particular emphasis is placed on the importance of a cytological analysis of the skin scarificate. Two of the patients in question were treated with antibiotics and corticosteroids, while the third received antibiotics, corticosteroids and cytostatics, yet the outcome of all three cases was fatal. This is attributed, in part, to the late beginning of treatment and, in part, to the early age of the patients.
D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant
Diseminovani oblik histiocitoze X odoj 56ceta (Letterer-Siwe-ova bolest)