Skip to main content
SpringerLink
Log in

Endocrine Hypertension

Pediatric Hypertension

  • 12 Accesses

Abstract

Hypertension may be caused by abnormal synthesis of, or response to, various hormones. The proportion of pediatric hypertension cases resulting from such problems probably represents at most a small percentage of cases overall, but a higher fraction of cases of severe hypertension, those occurring in the very young, or cases clustering in families. Most endocrine hypertension involves the adrenal gland and its hormones. The adrenal gland is composed of two endocrine tissues: the medulla (secreting catecholamines) and the cortex (synthesizing cortisol and aldosterone). Pheochromocytoma is mainly a disease of the adrenal medulla, although extramedullary sites may be involved. Many different diseases affecting the adrenal cortex can cause hypertension. These include hypertensive forms of congenital adrenal hyperplasia, primary aldosteronism due to hyperplasia of the zona glomerulosa or to adenomas, and Cushing syndrome (excessive glucocorticoid exposure) due to iatrogenic etiologies, to pituitary or adrenal adenomas, or other tumors secreting excessive ACTH. Hypertension can also be caused by thyrotoxicosis due to Graves’ disease or to the thyrotoxic phase of Hashimoto’s thyroiditis. It is important to accurately diagnose these disorders because the associated hypertension requires and usually responds well to specific treatment of the underlying hormonal abnormality.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

Similar content being viewed by others

References

  • Aglony M, Cristian A. Martínez-Aguayo A Fau – Carvajal, Carmen Carvajal Ca Fau – Campino, Hernán Campino C Fau – García, Rodrigo García H Fau – Bancalari, Lillian Bancalari R Fau – Bolte, Carolina Bolte L Fau – Avalos, Carolina Avalos C Fau – Loureiro, Pamela Loureiro C Fau – Trejo, Karin Trejo P Fau – Brinkmann, Vinka Brinkmann K Fau – Giadrosich, Verónica Giadrosich V Fau – Mericq, Ana Mericq V Fau – Rocha, Alejandra Rocha A Fau – Avila, Viviana Avila A Fau – Perez, Andrea Perez V Fau – Inostroza, Carlos E. Inostroza A Fau – Fardella, Fardella CE (2011) Frequency of familial hyperaldosteronism type 1 in a hypertensive pediatric population: clinical and biochemical presentation Hypertension 57:1117–1121

    Google Scholar 

  • Akamizu T, Satoh T, Isozaki O, Suzuki A, Wakino S, Iburi T, Tsuboi K, Monden T, Kouki T, Otani H, Teramukai S, Uehara R, Nakamura Y, Nagai M, Mori M (2012) Diagnostic criteria, clinical features, and incidence of thyroid storm based on nationwide surveys. Thyroid 22:661–679

    Article  CAS  PubMed  Google Scholar 

  • Auchus RJ (2001) The genetics, pathophysiology, and management of human deficiencies of P450c17. [Review] [77 refs]. Endocrinol Metab Clin N Am 30:101–119

    Article  CAS  Google Scholar 

  • Bahn RS, Burch HB, Cooper DS, Garber JR, Greenlee MC, Klein I, Laurberg P, McDougall IR, Montori VM, Rivkees SA, Ross DS, Sosa JA, Stan MN (2011) Hyperthyroidism and other causes of thyrotoxicosis: management guidelines of the American Thyroid Association and American Association of Clinical Endocrinologists. Thyroid 21:593–646

    Article  CAS  Google Scholar 

  • Barrett B, Hawkes CP, Isaza A, Bauer AJ (2019) The effects of amiodarone on thyroid function in pediatric and young adult patients. J Clin Endocrinol Metab 104:5540–5546

    Article  PubMed  Google Scholar 

  • Beuschlein F, Fassnacht M, Assie G, Calebiro D, Stratakis CA, Osswald A, Ronchi CL, Wieland T, Sbiera S, Faucz FR, Schaak K, Schmittfull A, Schwarzmayr T, Barreau O, Vezzosi D, Rizk-Rabin M, Zabel U, Szarek E, Salpea P, Forlino A, Vetro A, Zuffardi O, Kisker C, Diener S, Meitinger T, Lohse MJ, Reincke M, Bertherat J, Strom TM, Allolio B (2014) Constitutive activation of PKA catalytic subunit in adrenal Cushing’s syndrome. N Engl J Med 370:1019–1028

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Brenta G, Danzi S, Klein I (2007) Potential therapeutic applications of thyroid hormone analogs. Nat Clin Pract Endocrinol Metab 3:632–640

    Article  CAS  PubMed  Google Scholar 

  • Buffet A, Ben Aim L, Leboulleux S, Drui D, Vezzosi D, Libé R, Ajzenberg C, Bernardeschi D, Cariou B, Chabolle F, Chabre O, Darrouzet V, Delemer B, Desailloud R, Goichot B, Esvant A, Offredo L, Herman P, Laboureau S, Lefebvre H, Pierre P, Raingeard I, Reznik Y, Sadoul JL, Hadoux J, Tabarin A, Tauveron I, Zenaty D, Favier J, Bertherat J, Baudin E, Amar L, Gimenez-Roqueplo AP (2019) Positive impact of genetic test on the management and outcome of patients with paraganglioma and/or pheochromocytoma. J Clin Endocrinol Metab 104:1109–1118

    Article  PubMed  Google Scholar 

  • Castinetti F, Nieman LK, Reincke M, Newell-Price J (2021) Approach to the patient treated with steroidogenesis inhibitors. J Clin Endocrinol Metab 106:2114–2123

    Article  PubMed  PubMed Central  Google Scholar 

  • Charmandari E, Kino T, Chrousos GP (2013) Primary generalized familial and sporadic glucocorticoid resistance (Chrousos syndrome) and hypersensitivity. Endocr Dev 24:67–85. https://doi.org/10.1159/000342505. Epub 2013 Feb 1

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Choi M, Scholl UI, Yue P, Bjorklund P, Zhao B, Nelson-Williams C, Ji W, Cho Y, Patel A, Men CJ, Lolis E, Wisgerhof MV, Geller DS, Mane S, Hellman P, Westin G, Akerstrom G, Wang W, Carling T, Lifton RP (2011) K+ channel mutations in adrenal aldosterone-producing adenomas and hereditary hypertension. Science 331:768–772

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Claahsen-van der Grinten HL, Speiser PW, Ahmed SF, Arlt W, Auchus RJ, Falhammar H, Flück CE, Guasti L, Huebner A, Kortmann BBM, Krone N, Merke DP, Miller WL, Nordenström A, Reisch N, Sandberg DE, Stikkelbroeck N, Touraine P, Utari A, Wudy SA, White PC (2022) Congenital adrenal hyperplasia – current insights in pathophysiology, diagnostics and management. Endocr Rev 43:91–159

    Google Scholar 

  • Clark AJ, Metherell LA (2006) Mechanisms of disease: the adrenocorticotropin receptor and disease. Nat Clin Pract Endocrinol Metab 2:282–290

    Article  CAS  PubMed  Google Scholar 

  • Cooper DS (2021) Long-term antithyroid drug therapy. Curr Opin Endocrinol Diabetes Obes 28:510–516

    Article  CAS  PubMed  Google Scholar 

  • Dumitrescu AM, Refetoff S (2007) Novel biological and clinical aspects of thyroid hormone metabolism. Endocr Dev 10:127–139

    Article  CAS  PubMed  Google Scholar 

  • Feelders RA, Newell-Price J, Pivonello R, Nieman LK, Hofland LJ, Lacroix A (2019) Advances in the medical treatment of Cushing’s syndrome. Lancet Diabetes Endocrinol 7:300–312

    Article  PubMed  Google Scholar 

  • Fleseriu M, Auchus RJ, Pivonello R, Salvatori R, Zacharieva S, Biller BMK (2021) Levoketoconazole: a novel treatment for endogenous Cushing’s syndrome. Expert Rev Endocrinol Metab 16:159–174

    Article  CAS  PubMed  Google Scholar 

  • Funder J (2001) Mineralocorticoids and cardiac fibrosis: the decade in review. Clin Exp Pharmacol Physiol 28:1002–1006

    Article  CAS  PubMed  Google Scholar 

  • Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, Stowasser M, Young WF Jr (2016) The management of primary aldosteronism: case detection, diagnosis, and treatment: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 101:1889–1916

    Article  CAS  PubMed  Google Scholar 

  • Geller DS, Farhi A, Pinkerton N, Fradley M, Moritz M, Spitzer A, Meinke G, Tsai FTF, Sigler PB, Lifton RP (2000) Activating mineralocorticoid receptor mutation in hypertension exacerbated by pregnancy. Science 289:119–123

    Article  CAS  PubMed  Google Scholar 

  • Gershengorn MC, Neumann S (2012) Update in TSH receptor agonists and antagonists. J Clin Endocrinol Metab 97:4287–4292

    Google Scholar 

  • Hamidi O (2019) Metastatic pheochromocytoma and paraganglioma: recent advances in prognosis and management. Curr Opin Endocrinol Diabetes Obes 26:146–154

    Article  PubMed  Google Scholar 

  • Hattangady NG, Olala LO, Bollag WB, Rainey WE (2012) Acute and chronic regulation of aldosterone production. Mol Cell Endocrinol 350:151–162

    Article  CAS  PubMed  Google Scholar 

  • Higuchi S, Ohtsu H, Suzuki H, Shirai H, Frank GD, Eguchi S (2007) Angiotensin II signal transduction through the AT1 receptor: novel insights into mechanisms and pathophysiology. Clin Sci (Lond) 112:417–428

    Article  CAS  Google Scholar 

  • Hurt DE, Suzuki S, Mayama T, Charmandari E, Kino T (2016) Structural analysis on the pathologic mutant glucocorticoid receptor ligand-binding domains. Mol Endocrinol 30:173–188

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kamilaris CDC, Stratakis CA, Hannah-Shmouni F (2021) Molecular genetic and genomic alterations in Cushing’s syndrome and primary aldosteronism. Front Endocrinol (Lausanne) 12:632543

    Article  Google Scholar 

  • Kaplowitz PB, Vaidyanathan P (2020) Update on pediatric hyperthyroidism. Curr Opin Endocrinol Diabetes Obes 27:70–76

    Article  PubMed  Google Scholar 

  • Kino T (2018) Single nucleotide variations of the human GR gene manifested as pathologic mutations or polymorphisms. Endocrinology 159:2506–2519

    Article  CAS  PubMed  Google Scholar 

  • Korzyńska W, Jodkowska A, Gosławska K, Bogunia-Kubik K, Mazur G (2018) Genetic aspects of primary hyperaldosteronism. Adv Clin Exp Med 27:1149–1158

    Article  PubMed  Google Scholar 

  • Lane LC, Cheetham TD, Perros P, Pearce SHS (2020) New therapeutic horizons for graves’ hyperthyroidism. Endocr Rev 41:873–884

    Article  Google Scholar 

  • Léger J, Carel JC (2017) Management of endocrine disease: arguments for the prolonged use of antithyroid drugs in children with Graves’ disease. Eur J Endocrinol 177:R59–r67

    Article  PubMed  Google Scholar 

  • Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, Naruse M, Pacak K, Young WF Jr (2014) Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 99:1915–1942

    Article  CAS  PubMed  Google Scholar 

  • Lenders JWM, Eisenhofer G, Reincke M (2017) Subtyping of patients with primary aldosteronism: an update. Horm Metab Res 49:922–928

    Article  CAS  PubMed  Google Scholar 

  • Li JS, Flynn JT, Portman R, Davis I, Ogawa M, Shi H, Pressler ML (2010) The efficacy and safety of the novel aldosterone antagonist eplerenone in children with hypertension: a randomized, double-blind, dose-response study. J Pediatr 157:282–287

    Article  CAS  PubMed  Google Scholar 

  • Lodish M, Stratakis CA (2016) A genetic and molecular update on adrenocortical causes of Cushing syndrome. Nat Rev Endocrinol 12:255–262

    Article  CAS  PubMed  Google Scholar 

  • Lutterman SL, Zwaveling-Soonawala N, Verberne HJ, Verburg FA, van Trotsenburg ASP, Mooij CF (2021) The efficacy and short- and long-term side effects of radioactive iodine treatment in pediatric graves’ disease: a systematic review. Eur Thyroid J 10:353–363

    Article  PubMed  PubMed Central  Google Scholar 

  • Martins CS, de Castro M (2021) Generalized and tissue specific glucocorticoid resistance. Mol Cell Endocrinol 530:111277

    Article  CAS  PubMed  Google Scholar 

  • Miller WL, Auchus RJ (2011) The molecular biology, biochemistry, and physiology of human steroidogenesis and its disorders. Endocr Rev 32:81–151

    Article  CAS  PubMed  Google Scholar 

  • Miller WL, Auchus RJ (2019) The “backdoor pathway” of androgen synthesis in human male sexual development. PLoS Biol 17:e3000198

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Mulatero P, Monticone S, Rainey WE, Veglio F, Williams TA (2013) Role of KCNJ5 in familial and sporadic primary aldosteronism. Nat Rev Endocrinol 9:104–112

    Article  CAS  PubMed  Google Scholar 

  • Nieman LK, Biller BM, Findling JW, Murad MH, Newell-Price J, Savage MO, Tabarin A (2015) Treatment of Cushing’s syndrome: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 100:2807–2831

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Papakokkinou E, Piasecka M, Carlsen HK, Chantzichristos D, Olsson DS, Dahlqvist P, Petersson M, Berinder K, Bensing S, Höybye C, Engström BE, Burman P, Follin C, Petranek D, Erfurth EM, Wahlberg J, Ekman B, Åkerman AK, Schwarcz E, Johannsson G, Falhammar H, Ragnarsson O (2021) Prevalence of Nelson’s syndrome after bilateral adrenalectomy in patients with Cushing’s disease: a systematic review and meta-analysis. Pituitary 24:797–809

    Google Scholar 

  • Petenuci J, Guimaraes AG, Fagundes GFC, Benedetti AFF, Afonso ACF, Pereira MAA, Zerbini MCN, Siqueira S, Yamauchi F, Soares SC, Srougi V, Tanno FY, Chambo JL, Lopes RI, Denes FT, Hoff AO, Latronico AC, Mendonca BB, Fragoso MCBV, Almeida MQ (2021) Genetic and clinical aspects of paediatric pheochromocytomas and paragangliomas. Clin Endocrinol 95:117–124

    Article  CAS  Google Scholar 

  • Rabon S, Burton AM, White PC (2016) Graves’ disease in children: long-term outcomes of medical therapy. Clin Endocrinol 85:632–635

    Article  CAS  Google Scholar 

  • Redlich A, Pamporaki C, Lessel L, Frühwald MC, Vorwerk P, Kuhlen M (2021) Pseudohypoxic pheochromocytomas and paragangliomas dominate in children. Pediatr Blood Cancer 68:e28981

    Article  CAS  PubMed  Google Scholar 

  • Rivkees SA, Mattison DR (2009) Ending propylthiouracil-induced liver failure in children. N Engl J Med 360:1574–1575

    Article  CAS  PubMed  Google Scholar 

  • Ross DS, Burch HB, Cooper DS, Greenlee MC, Laurberg P, Maia AL, Rivkees SA, Samuels M, Sosa JA, Stan MN, Walter MA (2016) 2016 American Thyroid Association guidelines for diagnosis and management of hyperthyroidism and other causes of thyrotoxicosis. Thyroid 26:1343–1421

    Article  PubMed  Google Scholar 

  • Santos IN, Spadari-Bratfisch RC (2006) Stress and cardiac beta adrenoceptors. Stress 9:69–84

    Article  CAS  PubMed  Google Scholar 

  • Seamon ML, Yamaguchi I (2021) Hypertension in pheochromocytoma and paraganglioma: evaluation and management in pediatric patients. Curr Hypertens Rep 23:32

    Article  CAS  PubMed  Google Scholar 

  • Seidel E, Schewe J, Scholl UI (2019) Genetic causes of primary aldosteronism. Exp Mol Med 51:1–12

    Article  CAS  PubMed  Google Scholar 

  • Silva JE, Bianco SD (2008) Thyroid-adrenergic interactions: physiological and clinical implications. Thyroid 18:157–165

    Article  CAS  PubMed  Google Scholar 

  • Soinio M, Luukkonen AK, Seppänen M, Kemppainen J, Seppänen J, Pienimäki JP, Leijon H, Vesterinen T, Arola J, Lantto E, Helin S, Tikkanen I, Metso S, Mirtti T, Heiskanen I, Norvio L, Tiikkainen M, Tikkanen T, Sane T, Välimäki M, Gomez-Sanchez CE, Pörsti I, Nuutila P, Nevalainen PI, Matikainen N (2020) Functional imaging with 11C-metomidate PET for subtype diagnosis in primary aldosteronism. Eur J Endocrinol 183:539–550

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Soundararajan R, Pearce D, Hughey RP, Kleyman TR (2010) Role of epithelial sodium channels and their regulators in hypertension. J Biol Chem 285:30363–30369

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Stocco DM (2001) StAR protein and the regulation of steroid hormone biosynthesis. [Review] [162 refs]. Annu Rev Physiol 63:193–213

    Article  CAS  PubMed  Google Scholar 

  • Stratakis CA, Berthon A (2019) Molecular mechanisms of ARMC5 mutations in adrenal pathophysiology. Curr Opin Endocr Metab Res 8:104–111

    Article  PubMed  PubMed Central  Google Scholar 

  • Subramonian D, Wu YJ, Amed S, Sanatani S (2021) Hyperthyroidism with atrial fibrillation in children: a case report and review of the literature. Front Endocrinol (Lausanne) 12:689497

    Article  Google Scholar 

  • Tomaschitz A, Pilz S, Ritz E, Obermayer-Pietsch B, Pieber TR (2010) Aldosterone and arterial hypertension. Nat Rev Endocrinol 6:83–93

    Article  CAS  PubMed  Google Scholar 

  • Tomlinson JW, Stewart PM (2005) Mechanisms of disease: selective inhibition of 11beta-hydroxysteroid dehydrogenase type 1 as a novel treatment for the metabolic syndrome. Nat Clin Pract Endocrinol Metab 1:92–99

    Article  CAS  PubMed  Google Scholar 

  • Turkova H, Prodanov T, Maly M, Martucci V, Adams K, Widimsky J Jr, Chen CC, Ling A, Kebebew E, Stratakis CA, Fojo T, Pacak K (2016) Characteristics and outcomes of metastatic SDHB and sporadic pheochromocytoma/paraganglioma: an National Institutes of Health study. Endocr Pract 22:302–314

    Article  PubMed  Google Scholar 

  • Uslar T, San Francisco IF, Olmos R, Macchiavelo S, Zuñiga A, Rojas P, Garrido M, Huete A, Mendez GP, Cortinez I, Zemelman JT, Cifuentes J, Castro F, Olivari D, Domínguez JM, Arteaga E, Fardella CE, Valdés G, Tagle R, Baudrand R (2021) Clinical presentation and perioperative management of pheochromocytomas and paragangliomas: a 4-decade experience. J Endocr Soc 5:bvab073

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • van Lieshout JM, Mooij CF, van Trotsenburg ASP, Zwaveling-Soonawala N (2021) Methimazole-induced remission rates in pediatric Graves’ disease: a systematic review. Eur J Endocrinol 185:219–229

    Article  PubMed  Google Scholar 

  • Vassiliadi DA, Mourelatos P, Kratimenos T, Tsagarakis S (2021) Inferior petrosal sinus sampling in Cushing’s syndrome: usefulness and pitfalls. Endocrine 73:530–539

    Article  CAS  PubMed  Google Scholar 

  • White PC (2001) Steroid 11 beta-hydroxylase deficiency and related disorders. [Review] [60 refs]. Endocrinol Metab Clin N Am 30:61–79

    Article  CAS  Google Scholar 

  • White PC (2018) Alterations of cortisol metabolism in human disorders. Horm Res Paediatr 89:320–330

    Article  CAS  PubMed  Google Scholar 

  • Yang S, Zhang L (2004) Glucocorticoids and vascular reactivity. Curr Vasc Pharmacol 2:1–12

    Article  PubMed  Google Scholar 

  • Yen K, Lodish M (2021) Pheochromocytomas and paragangliomas. Curr Opin Pediatr 33:430–435

    Article  CAS  PubMed  Google Scholar 

  • Yuen KCJ (2021) Osilodrostat: a review of recent clinical studies and practical recommendations for its use in the treatment of Cushing disease. Endocr Pract 27:956–965

    Google Scholar 

  • Zaat AS, Derikx JPM, Zwaveling-Soonawala N, van Trotsenburg ASP, Mooij CF (2021) Thyroidectomy in pediatric patients with Graves’ disease: a systematic review of postoperative morbidity. Eur Thyroid J 10:39–51

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Pediatric Endocrinology, Department of Pediatrics, UT Southwestern Medical Center and Children’s Medical Center, Dallas, TX, USA

    Perrin C. White

Authors
  1. Perrin C. White
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Perrin C. White .

Editor information

Editors and Affiliations

  1. Division of Nephrology, Seattle Children's Hospital, Seattle, WA, USA

    Joseph T. Flynn

  2. Massachusetts General Hospital, Boston, MA, USA

    Julie R. Ingelfinger

  3. Johns Hopkins University, Baltimore, MD, USA

    Tammy Brady

Section Editor information

  1. University of Washington School of Medicine, Seattle, WA, USA

    Joseph T. Flynn MD, MS

  2. Divison of Nephrology, Seattle Children’s Hospital, Seattle, WA, USA

    Joseph T. Flynn MD, MS

Rights and permissions

Reprints and permissions

Copyright information

© 2022 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

White, P.C. (2022). Endocrine Hypertension. In: Flynn, J.T., Ingelfinger, J.R., Brady, T. (eds) Pediatric Hypertension. Springer, Cham. https://doi.org/10.1007/978-3-319-31420-4_25-2

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-31420-4_25-2

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-31420-4

  • Online ISBN: 978-3-319-31420-4

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Chapter history

  1. Latest

    Endocrine Hypertension
    Published:
    01 May 2022

    DOI: https://doi.org/10.1007/978-3-319-31420-4_25-2

  2. Original

    Endocrine Hypertension
    Published:
    21 December 2016

    DOI: https://doi.org/10.1007/978-3-319-31420-4_25-1

Search

Navigation