Growth and endocrine disorders in multisystem Langerhans' cell histiocytosis

INTRODUCTION Langerhans' cell histiocytosis is a rare disorder, with diabet es insipidus occurring in up to half of patients. Causes of growth failure include the illness itself, treatments used and growth hormone insufficienc y. PATIENTS AND METHODS We identified all patients with an endocrinopathy seco ndary to Langerhans' cell histiocytosis (LCH). Growth data were analysed fr om all patients with multisystem involvement. RESULTS Of 144 patients with multisystem LCH, 50 had an endocrinopathy, 49 of whom had diabetes insipidus. Growth hormone insufficiency (GHI) was pres ent in 21 patients, seven of whom had other anterior pituitary deficiencies as well (gonadotrophin deficiency + GHI n = 2, gonadotrophin deficiency TSH deficiency + GHI n = 2, panhypopituitarism n = 3). GH insufficiency, th e development of which appeared to be independent of pituitary radiation, o ccurred at a median age of 8.3 years (4.7-18 years) and at a median interva l of 3.5 years (0-11.8 years) after diagnosis of LCH. The median height SDS at diagnosis of growth hormone insufficiency was -2.9. Thirteen of the pat ients with growth hormone insufficiency attained final height with a median height SDS of -1.2. The final height SDS of 15 patients without GH insuffi ciency was closer to target height SDS, but not statistically different fro m that of the GH insufficient group. CONCLUSIONS GH therapy significantly improves growth in GH insufficient pat ients with Langerhans' cell histiocytosis. Early institution of GH therapy may further improve height outcome. However, most children with Langerhans' cell histiocytosis regardless of endocrine function, failed to reach targe t height.