Growth and endocrine disorders in multisystem Langerhans' cell histiocytosis.
BACKGROUND
Langerhans' cell histiocytosis is a rare disorder, with diabetes insipidus occurring in up to half of patients. Causes of growth failure include the illness itself, treatments used and growth hormone insufficiency.


METHODS
We identified all patients with an endocrinopathy secondary to Langerhans' cell histiocytosis (LCH). Growth data were analysed from 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 present 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, the development of which appeared to be independent of pituitary radiation, occurred at a median age of 8.3 years (4.7-18 years) and at a median interval 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 patients with growth hormone insufficiency attained final height with a median height SDS of -1.2. The final height SDS of 15 patients without GH insufficiency was closer to target height SDS, but not statistically different from that of the GH insufficient group.


CONCLUSIONS
GH therapy significantly improves growth in GH insufficient patients 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 target height.
Nanduri
V R
VR
Department of, Endocrinology, Great Ormond Street Hospital, Department of Surgery, The Institute of Child Health, London, UK.
Bareille
P
P
Pritchard
J
J
Stanhope
R
R
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003919:Diabetes Insipidus / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006065:Gonadotropins, Pituitary / Q000172:deficiency; D006130:Growth Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D013006:Growth Hormone / Q000172:deficiency / Q000627:therapeutic use; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D010901:Pituitary Function Tests; D010902:Pituitary Gland / Q000473:pathology; D010910:Pituitary Irradiation; D018709:Statistics, Nonparametric; D013972:Thyrotropin / Q000172:deficiency
D006065:Gonadotropins, Pituitary; D013972:Thyrotropin; D013006:Growth Hormone