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Growth of children with Langerhans cell histiocytosis
A C van den Hoek1, A Karstens, R M Egeler
1Sophia Children's Hospital/Erasmus University Rotterdam, The Netherlands.
Insights
Langerhans cell histiocytosis (LCH) in children rarely causes growth hormone deficiency. Growth is typically unaffected by LCH, but skull lesions may impact height.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Growth Disorders
Background:
- Langerhans cell histiocytosis (LCH) is a rare disease affecting children, with potential impacts on growth that are not well understood.
- Growth hormone (GH) deficiency can occur in LCH due to hypothalamic or pituitary involvement, necessitating interventions like GH therapy.
Observation:
- A review of 22 pediatric LCH patients (median follow-up 7 years) assessed growth patterns using standard deviation scores (SDS).
- At diagnosis, mean SDS was 0.04 for isolated LCH and -0.37 for disseminated LCH.
- Twelve patients showed no growth impact from LCH or its treatment.
Findings:
- Ten patients experienced improved growth, reaching higher percentiles after a minimum of 3 years.
- All ten patients with improved growth had skull lesions, specifically on the facial side.
- Growth hormone deficiency is not a common LCH manifestation; testing is advised for poor or decelerating growth.
Implications:
- Growth hormone provocation tests are indicated only for children with LCH exhibiting poor or decelerating growth.
- The extent of organ involvement and treatment modality did not significantly impact growth in most LCH patients.
- Facial skull lesions may be a key factor influencing growth outcomes in pediatric LCH.
Abstract:
Diseases in childhood have an impact on growth. The influence of Langerhans cell histiocytosis (LCH) on growth has never been studied well. Recently a patient with LCH was treated with human growth hormone (GH) because of severe GH deficiency due to LCH involvement of both the hypothalamus and pituitary. This led us to review our charts from 1971 onward for evaluation of the growth patterns in patients with LCH. Here the long-term growth of 22 patients with LCH is reported, the median follow up being 7 years and 1 month. The height data were converted into standard deviation scores (SDS). At diagnosis the mean SDS of patients with isolated LCH at diagnosis was 0.04 and -0.37 in patients with disseminated LCH. Of the total group, 12 patients did not show any influence from the LCH or therapy on their growth. The remaining 10 patients reached, after a minimum of 3 years, a percentile clearly higher than that at diagnosis. However all the ten above mentioned patients, either isolated or disseminated LCH, had a lesion in the facial side of the skull. CONCLUSION. GH deficiency is not a common manifestation of LCH in childhood and GH provocation tests are only indicated when there is a poor or decelerating growth rate. In our patients the number of organs involved and/or the treatment modality did not influence the growth in all but one.