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Growth and Endocrinal Abnormalities in Pediatric Langerhans Cell Histiocytosis
Aashima Dabas1, Atul Batra2, Rajesh Khadgawat1
1Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
Insights
Pediatric Langerhans Cell Histiocytosis (LCH) frequently causes growth failure and endocrinal disturbances like central Diabetes Insipidus (DI). Regular growth monitoring and DI screening are crucial for managing children with LCH.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Histiocytosis Research
Background:
- Pediatric Langerhans Cell Histiocytosis (LCH) is a rare disorder affecting children.
- Growth disturbances and endocrine issues are known complications of LCH.
Purpose of the Study:
- To investigate the incidence and impact of growth disturbances and endocrinal complications in pediatric LCH patients.
- To identify specific risk factors and outcomes associated with these complications.
Main Methods:
- Retrospective analysis of 62 pediatric LCH patient records.
- Classification into groups based on disease severity (bone, soft tissue, organ dysfunction).
- Statistical comparison of growth parameters and endocrine status at baseline and follow-up.
Main Results:
- Growth failure (44%) and central Diabetes Insipidus (19%) were common in pediatric LCH.
- Patients with organ dysfunction (Group C) exhibited poorer growth outcomes.
- Children with DI showed impaired height catch-up compared to those without DI.
Conclusions:
- Growth monitoring and screening for central Diabetes Insipidus are essential components of LCH patient care.
- Early detection and management of these complications can improve long-term outcomes.
- Further research into the specific mechanisms of growth failure in LCH is warranted.
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.
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