Histiocytosis X; follow-up of 43 cases.

  title={Histiocytosis X; follow-up of 43 cases.},
  author={Douglas G Sims},
  journal={Archives of disease in childhood},
  volume={52 6},
Over a 29-year period, 43 cases of histiocytosis X presented in children under the age of 12 years. 29 patients (67%) have survived, and of these, 15 (52%) have a detectable disability. It was confirmed that young age at presentation and evidence of soft tissue involvement were associated with a worse prognosis. The majority of deaths were associated with pulmonary involvement. 14 patients developed diabetes insipidus. 5 of the surviving adults have heights below the 3rd centile. Puberty… CONTINUE READING