Advertisement

Springer Nature is making SARS-CoV-2 and COVID-19 research free. View research | View latest news | Sign up for updates

A neonate with idiopathic hyperaldosteronism

Abstract

A boy with functional abnormalities of the gastro-intestinal tract, hyponatraemia, hypokalaemia and hypertension is described. All symptoms developed within the first 2 months of life. Increased aldosterone levels were associated with suppressed values in the renin-angiotensin system. The diagnosis of idiopathic hyperaldosteronism was made because of adrenal hyperplasia and the failure to suppress aldosterone to undetectable levels with glucocorticoids. Treatment with spironolactone alone, or in combination with either intravenous dopamine or ibopamine orally, amiloride, enalapril, hydralazine or clonidine corrected serum potassium values but failed to normalize blood pressure and to correct plasma renin activity and plasma aldosterone. However, the combination of spironolactone with nifedipine decreased blood pressure. Abnormal gastro-intestinal motility was corrected by low doses of oral magnesium hydroxide. To assess intracellular calcium homeostasis, the patient's peripheral blood mononuclear cells were incubated with increasing concentrations of calcium. As these cells failed to maintain physiological calcium concentration, a defect in intracellular calcium homeostasis was suspected.

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

References

  1. 1.

    Swiet M de, Fayers P, Shinebourne EA (1980) Systolic blood pressure in a population of infants in the first year of life the Brompton study. Pediatrics 65: 1028–1035

  2. 2.

    Wit JM, Roermund HP van, Oostelijk W, Benraad TH, Thyssen JH, Boer P, Jansen M, Spit M, Brande JL van den (1988) Heterozygotes for 17a-hydroxylase deficiency can be detected with a short ACTH test. Clin Endocrinol (Oxf) 28: 657–664

  3. 3.

    Ploeg KR van der Wolthers GB, Nagel GT, Volmer M, Drayer NM (1982) The diagnosis of 21-hydroxylase deficiency in a prematurely born infant on the basis of the urinary steroid excretion pattern. Clin Chim Acta 120: 341–353

  4. 4.

    Koopman BJ, Lokerse IJG, Verwey H, Nagel GT, Molen JC van der (1986) Improved gas chromatographic-mass fragmentographic assay for tetrahydroaldosterone and aldosterone in urine. J Chromatogr 378: 283–292

  5. 5.

    Boer P, Hene RJ, Koomans HA, Nieuwenhuis MG, Geyskes GG, Dorhout Mees EJ (1983) Blood and extracellular fluid volume in patients with Bartter's syndrome. Arch Intern Med 143: 1902–1905

  6. 6.

    Derkx FHM, Stuenkel C, Schalekamp MPA, Visser, W, Huisveld IH, Schalekamp MADH (1986) Immunoreactive renin, prorenin, and enzymatically active renin in plasma during pregnancy and in women taking oral contraceptives. J Clin Endocrinol Metab 63: 1008–1015

  7. 7.

    Menard J, Guyenne, Corvol P, Pau B, Simon D, Roncucci R (1985) Direct immunometric assay of active renin in human plasma. J Hypertens 3: 5275–5278

  8. 8.

    Boomsma F, Schalekamp MADH (1983) Evaluation of a test kit for the rapid and simple colorimetric measurement of angiotensin I-converting enzyme in serum. J Clin Chem Biochem 21: 845–849

  9. 9.

    Nussbeger, J, Brunnet DB, Waeber B, Brunner HR (1985) True versus immunoreactive angiotensin II in human plasma. Hypertension 7: 11–17

  10. 10.

    Admiraal PJJ, Derkx FHM, Danser AHJ, Pieterman H, Schalekamp MADH (1990) Metabolism and production of angiotensin I in different vascular beds in subjects with hypertension. Hypertension 15: 44–55

  11. 11.

    Rosmalen FMA, Tan ACITL, Tan HS, Benraad THJ (1987) A sensible radioimmunoassay of atrial natriuretic peptide in human plasma, using a tracer with an immobilized glycouril agent. Clin Chim Acta 165: 331

  12. 12.

    Hoorn FAJ van der, Boomsma F, Man in't Veld AJ, Schalekamp MADH (1989) Determination of catecholamines in human plasma by high-performance liquid chromatography: comparison between a new method with fluorescence detection and an established method with electrochemical detection. J Chromatogr 487: 17–28

  13. 13.

    Milla PJ (1991) Introduction. In: Milla PJ (ed) Disorders of gastrointestinal motility in childhood. Wiley, New York, pp 1–14

  14. 14.

    Griffioen AW, Rijkers GT, Keij J, Zegers BJM (1989) Measurement of cytoplasmatic calcium in lymphocytes using flow cytometry. Kinetic studies and single cell analysis. J Immunol Methods 120: 23–27

  15. 15.

    Keij JF, Griffioen AW, The TH, Rijkers GT (1989) INCA: software forflow cytometric measurement of changes in cytolasmic calcium. Cytometry 6: 814–819

  16. 16.

    Sippel WG (1980) Plasma levels of aldosterone, corticosterone, 11-deoxycorticosterone, 17-hedroxyprogesterone, cortisol, and cortisone during infancy and childhood. Pediatr Res 14: 39–46

  17. 17.

    Stark P, Beckerhoff R, Leumann EP, Vetter W, Siegenthaler, W (1975) Control of plasma aldosterone in infancy and childhood. Helv Paediatr Acta 30: 349–356

  18. 18.

    Fiselier TJW, Lijnen P, Monnens L Munster P van, Jansen M, Peer P (1983) Levels of renin, angiotensin I and II, angiotensin-converting enzyme and aldosterone in infancy and childhood. Eur J Pediatr 141: 3–7

  19. 19.

    Bisset WM (1991) The development of motor control systems in the gastro-intestinal tract of the preterm infant. Intestinal motor activity. In: Milla PJ (ed) Disorders of gastro-intestinal motility, in childhood. Wiley, New York pp 17–37

  20. 20.

    Shackleton CHL, Honour JW, Dillon MJ, Chantler C, Jones RW (1980) Hypertension in a four year old child: gas chromatographic and mass spectometric examination. Deficient hepatic metabolism of steroids. J Clin Endocrinol Metab 50: 786–792

  21. 21.

    Oberfield SE, Levine LS, Carey RM, Greig F, Ulick S, New MI (1983) Metabolic and blood pressure responses to hydrocortisone in the syndrome of apparent mineralocorticoid excess. J Clin Endocrinol Metab 56: 332–339

  22. 22.

    Young WF, Klee GG (1988) Primary aldosteronism. Diagnostic evaluation. Endocrinol Metab Clin North Am 17: 367–395

  23. 23.

    Melby JC, (1984) Primary aldosteronism. Kidney Int 26: 769–778

  24. 24.

    DiMartino-Nardi J, New, MI (1987) Low-renin hypertension of childhood. Pediatr Nephrol 1: 99–108

  25. 25.

    Holland OB, Thomas C, Brown H, Schindewolf D, Hillier Y, Gomez-Sanchez C (1983) Aldosterone suppression with dopamine infusion in low-renin hypertension. J Clin Invest 72: 754–766

  26. 26.

    Nadler JL, Hsueh W, Horton R (1985) Therapeutic effect of calcium channel blockade in primary aldosteronism. J Clin Endocrinol Metab 60: 896–899

Download references

Author information

Correspondence to Raymond A. Donckerwolcke.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Veenhoven, R.H., Vande Walle, J.G., Donckerwolcke, R.A. et al. A neonate with idiopathic hyperaldosteronism. Pediatr Nephrol 5, 680–684 (1991). https://doi.org/10.1007/BF00857870

Download citation

Key words

  • Hypertension
  • Hypokalaemia
  • Gastro-intestinal motility disorder