Paeds Vivas · endocrinology-diabetes-and-growth
Phaeochromocytoma and endocrine hypertension — branching viva
Branching viva from the hypertensive adolescent with spells, through the metanephrines-first diagnostic rule, the alpha-before-beta preoperative trap and the hereditary genetics, to the hypokalaemic child with suppressed renin and aldosterone whose gene dictates the drug.
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RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
You are the paediatric registrar in the outpatient clinic and on the ward. The consultant asks you to assess three children: a hypertensive adolescent with episodic headache, sweating and palpitations; an eight-year-old with an adrenal mass spotted on ultrasound; and a ten-year-old with hypertension, hypokalaemia and a suppressed renin and aldosterone. The examiner releases information in stages.
Station opening
The consultant presents a 14-year-old boy with sustained hypertension and episodes of severe headache, sweating and palpitations lasting minutes, with pallor. Between episodes he feels well. The examiner asks: "What is the most likely diagnosis, and what single biochemical test would you order first?" [4]
References9ShowHide
- [1]Lenders JW; Duh QY; Eisenhofer G; et al Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab, 2014.PMID 24893135
- [2]Casey RT; Hendriks E; Deal C; et al International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents. Nat Rev Endocrinol, 2024.PMID 39147856
- [3]Lenders JW; Pacak K; Walther MM; et al Biochemical diagnosis of pheochromocytoma: which test is best? JAMA, 2002.PMID 11903030
- [4]Barontini M; Levin G; Sanso G Characteristics of pheochromocytoma in a 4- to 20-year-old population. Ann N Y Acad Sci, 2006.PMID 17102069
- [5]Havekes B; Romijn JA; Eisenhofer G; et al Update on pediatric pheochromocytoma. Pediatr Nephrol, 2009.PMID 18566838
- [7]Muth A; Crona J; Gimm O; et al Genetic testing and surveillance guidelines in hereditary pheochromocytoma and paraganglioma. J Intern Med, 2019.PMID 30536464
- [8]Castinetti F; Waguespack SG; Machens A; et al Natural history, treatment, and long-term follow up of patients with multiple endocrine neoplasia type 2B: an international, multicentre, retrospective study. Lancet Diabetes Endocrinol, 2019.PMID 30660595
- [11]New MI; Geller DS; Fallo F; et al Monogenic low renin hypertension. Trends Endocrinol Metab, 2005.PMID 15808805
- [12]Garovic VD; Hilliard AA; Turner ST Monogenic forms of low-renin hypertension. Nat Clin Pract Nephrol, 2006.PMID 17066054