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Paeds Vivasendocrinology-diabetes-and-growth

Paeds Vivas · endocrinology-diabetes-and-growth

Precocious puberty — branching viva

Branching viva on precocious puberty: recognising early sexual development, splitting central from peripheral on gonadotropins and clinical consonance, confirming with bone age and GnRH stimulation, choosing the imaging and treatment, and branching to the boy with a hypothalamic hamartoma, the girl with McCune-Albright, and the family weighing a GnRH analog — defending the central-against-peripheral distinction at every step.

branching clinical structured oral3 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Study tools

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
Outpatient clinic: a 6-year-old girl with breast budding, a growth spurt and a bone age advanced two and a half years, and a pubertal luteinising-hormone peak on GnRH stimulation. The examiner asks: what is the mechanism, what confirms it, what is the treatment, and what is the goal — then branches to a 4-year-old boy with a virilised body, a 6 mL testis and gelastic seizures on MRI, and asks you to explain the lesion and the dual management; to a 3-year-old girl with vaginal bleeding, coast-of-Maine cafe-au-lait macules and suppressed gonadotropins, and asks you to name the syndrome and why a GnRH analog will fail; and to a family weighing GnRH-analog therapy for their daughter, and asks you to counsel on height, safety and the stopping point.

Opening framework

My framework rests on one decision: who is driving the axis? Precocious puberty is secondary sexual characteristics before 8 years in girls and before 9 years in boys. If the hypothalamic–pituitary–gonadal axis is genuinely switched on, the process is central (GnRH-dependent), gonadotropins rise, and a GnRH analog will switch it back off. If a sex-steroid source is operating outside the axis, the process is peripheral (GnRH-independent), gonadotropins are suppressed, and a GnRH analog is useless. I confirm with a bone age and a basal-then-stimulated gonadotropin panel, I image the brain in every boy and in girls under 6 years, and I match the treatment to the driver. The two preventable harms are a short adult stature from unchecked sex steroids and a missed central nervous system lesion. [1] [2]

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References4ShowHide
  1. [1]Carel JC, Léger J. Clinical practice. Precocious puberty. N Engl J Med, 2008.PMID 18509122
  2. [2]Kaplowitz PB. Update on Precocious Puberty: Who Should Be Treated? Adv Pediatr, 2020.PMID 32591066
  3. [3]Bangalore Krishna K, Fuqua JS, Rogol AD, Klein KO, et al. Use of Gonadotropin-Releasing Hormone Analogs in Children: Update by an International Consortium. Horm Res Paediatr, 2019.PMID 31319416
  4. [8]Dumitrescu CE, Collins MT. McCune-Albright syndrome. Orphanet J Rare Dis, 2008.PMID 18489744
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