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.