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Paeds Vivasendocrinology-diabetes-and-growth

Paeds Vivas · endocrinology-diabetes-and-growth

Endocrine late effects of cancer treatment — branching viva

Branching viva on the endocrine late effects of childhood cancer treatment: recognising the survivor with growth hormone deficiency after cranial irradiation and the survivor with combined hypothalamic-pituitary deficits, confirming with axis-specific stimulation tests, delivering hormone replacement in the correct order of hydrocortisone before levothyroxine before sex steroids with recombinant growth hormone only after magnetic resonance imaging, and counselling on the paradoxical central precocious puberty of lower-dose cranial radiation and the primary gonadal failure of alkylating chemotherapy.

branching clinical structured oral2 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
Long-term follow-up clinic: a 9-year-old boy treated for medulloblastoma at age 5 with craniospinal radiation (36 Gy with a posterior fossa boost) now has a growth velocity of 3 cm/year, a delayed bone age, and a low IGF-1. The examiner asks: why is this growth hormone deficiency and not a normal variant, what is the dose-dependent hierarchy of radiation-induced hypothalamic-pituitary damage, what test confirms the deficiency, and what imaging must precede growth hormone therapy — then branches to a second case of a 12-year-old leukaemia survivor after 18 Gy cranial prophylactic radiation with rapid pubertal progression and an accelerated bone age, asking you to explain paradoxical central precocious puberty, the GnRH-analogue management, and the threat to final height — then branches to a third case of a 16-year-old survivor after haematopoietic stem cell transplantation with primary amenorrhoea and raised gonadotropins, asking you to explain primary gonadal failure, the fertility counselling, and the transition to adult survivorship care.

Branch 1 — the brain-tumour survivor with growth failure

The candidate is expected to justify growth hormone deficiency over a normal variant from the growth velocity, the delayed bone age and the low IGF-1, and to explain that growth hormone deficiency is the earliest and most common endocrine late effect of cranial radiation because the somatotroph axis is the most radiosensitive, with a dose-dependent threshold near 18 Gy and near-universal occurrence at the 36 Gy cranial dose. The confirmation is the growth hormone stimulation test with a peak below the age-appropriate cutoff, and the imaging is the pituitary magnetic resonance imaging scan that must precede any recombinant growth hormone because growth hormone is a mitogen. [1] [2]

The examiner branches into the dose hierarchy: growth hormone deficiency at 18 Gy, gonadotropin disturbance at 24 to 30 Gy, TSH and ACTH deficiency above 30 to 40 Gy, and panhypopituitarism at the highest doses — so this survivor must be screened across the whole axis, not only for growth hormone deficiency. The replacement order is named and justified: hydrocortisone before levothyroxine before sex steroids, with recombinant growth hormone after the magnetic resonance imaging, because giving thyroxine before cortisol precipitates an adrenal crisis. [2] [6]

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References5ShowHide
  1. [1]Chemaitilly W, Sklar CA. Childhood Cancer Treatments and Associated Endocrine Late Effects: A Concise Guide for the Pediatric Endocrinologist. Horm Res Paediatr, 2019.PMID 30404091
  2. [2]Sklar CA, et al. Hypothalamic-Pituitary and Growth Disorders in Survivors of Childhood Cancer: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab, 2018.PMID 29982476
  3. [3]Chemaitilly W, et al. Endocrine Late Effects in Childhood Cancer Survivors. J Clin Oncol, 2018.PMID 29874130
  4. [6]van Iersel L, et al. Hypothalamic-Pituitary and Other Endocrine Surveillance Among Childhood Cancer Survivors. Endocr Rev, 2022.PMID 34962573
  5. [11]Vatanen A, et al. Ovarian function after allogeneic hematopoietic stem cell transplantation in childhood and adolescence. Eur J Endocrinol, 2014.PMID 24179099
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