Growth and Endocrinal Abnormalities in Pediatric Langerhans Cell Histiocytosis

Objective: To ascertain the growth and endocrinal disturbances associated with Pediatric Langerhans Cell Histiocytosis (LCH).

Methods: Retrospective analysis of hospital records of subjects with LCH, aged 1 mo to 18 y was performed. The diagnosis of LCH was made as per Histiocyte Society criteria. Subjects were classified as group A: multifocal bone disease; B: soft tissue involvement without organ dysfunction; and C: organ dysfunction and treated as per DAL-HX-83 protocol of the Histiocyte Society LCH treatment guidelines. Paired t-test was used to compare the baseline and follow-up data.

Results: Total 62 records (group A- 18, B-32 and C-12) were identified with median follow-up of 5.3 ± 3.3 y. Growth failure [measured as weight/ height Standard deviation score (SDS) ≤-2] was the commonest disorder seen in 27 (44 %) subjects. Central Diabetes Insipidus (DI) was seen in 12 (19 %) subjects. Subjects with group C of LCH had poorer weight and height at baseline and follow-up than subjects with group A or B. Height SDS were lower in subjects with concomitant DI than those without DI at baseline (-2.35 ± 1.9 and -1.69 ± 1.4; P 0.18). Subjects with DI did not show significant catch-up in their height (P 0.12) unlike those without DI who showed a catch-up in height (P 0.03) on follow-up.

Conclusions: Growth monitoring and screening for DI should be essential part of follow-up in all subjects with LCH.