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Langerhans cell histiocytosis (LCH)

It’s rare, but it is there, and will need to be considered in your differential diagnoses on a few occasions in a general paediatrician’s career. Lucina’s most recent experience has been with the common presentation of polydipsia and polyuria and an ultimate diagnosis of central diabetes insipidus (CDI) caused by LCH. Alexander A and Zacharin M [J Paediatr and Child Health 2023] have documented the incidence of endocrine features in paediatric patients with LCH from one tertiary endocrine centre in Victoria, Australia. Using a simple retrospective chart review of electronic medical records and oncology database of patients with biopsy diagnosed LCH, they identified one hundred seventy-one patients diagnosed with LCH over a 30 year period. Mean age at diagnosis was 5 years 8 months. Of these, 15% (n = 21) had CDI, 7% had growth hormone deficiency (GHD) (n = 10) and 8% (n = 11) had more than one endocrinopathy noted during follow-up. Forty percent (n = 57) were pre-pubertal at the time of audit or on discharge from tertiary services. They recommend that there should be an ongoing pituitary assessment to detect evolving deficiencies of GHD and gonadotropins as these can be subtle, late, or missed. Although 40% were discharged before puberty they recommend close follow-up of growth and progression …

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  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Provenance and peer review Commissioned; internally peer reviewed.