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Evaluating renin and aldosterone levels in children with organic acidemia—therapeutic experience with fludrocortisone

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Abstract

Hyporeninemic hypoaldosteronism has been reported in only a few cases with methylmalonic acidemia (MMA) and has been attributed to the renal involvement. This study aims to investigate renin-aldosterone levels along with the renal functions of the patients with organic acidemia. This is a cross-sectional study conducted in patients with MMA, propionic acidemia (PA), and isovaleric acidemia (IVA). Serum renin, aldosterone, sodium, and potassium levels were measured, and glomerular filtration rates (GFR) were calculated. Comparisons were made between the MMA and non-MMA (PA+IVA) groups. Thirty-two patients (MMA:PA:IVA = 14:13:5) were included. The median GFR was significantly lower in the MMA group than in the non-MMA group (p < 0.001). MMA patients had the highest incidence of kidney damage (71.4%), followed by PA patients (23%), while none of the IVA patients had reduced GFR. GFR positively correlated with renin levels (p = 0.015, r = 0.433). Although renin levels were significantly lower in the MMA group than the non-MMA group (p = 0.026), no significant difference in aldosterone levels was found between the two groups. Hyporeninemic hypoaldosteronism was found in 3 patients with MMA who had different stages of kidney damage, and fludrocortisone was initiated, which normalized serum sodium and potassium levels.

  Conclusions: This study, which has the largest number of patients among the studies investigating the renin-angiotensin system in organic acidemias to date, has demonstrated that hyporeninemic hypoaldosteronism is not a rare entity in the etiology of hyperkalemia in patients with MMA, and the use of fludrocortisone is an effective treatment of choice in selected cases.

What is Known:

• Hyperkalemia may be observed in cases of methylmalonic acidemia due to renal involvement and can be particularly prominent during metabolic decompensation.

• Hyporeninemic hypoaldosteronism has been reported to be associated with hyperkalemia in only a few cases of methylmalonic acidemia.

What is New:

• Hyporeninemic hypoaldosteronism was found in one-fifth of cases with methylmalonic acidemia.

• Fludrocortisone therapy leads to the normalization of serum sodium and potassium levels.

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Data availability

Data are available on request only due to ethical or legal reasons.

Abbreviations

CKD:

Chronic kidney disease

GFR:

Glomerular filtration rate

IVA:

Isovaleric acidemia

KDIGO:

Kidney Disease Improving Global Outcomes

MMA:

Methylmalonic acidemia

PA:

Propionic acidemia

PRA:

Plasma renin activity

UNaK:

Urine sodium to potassium ratio

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Funding

This work was funded by Istanbul University-Cerrahpasa, Scientific Research Projects Coordination Unit with the project number 35848.

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Authors and Affiliations

  1. Department of Pediatric Endocrinology, Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Türkiye

    Gürkan Tarçın & Oya Ercan

  2. Department of Pediatric Metabolic Diseases and Nutrition, Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Türkiye

    Saffa Ahmadzada & Ayşe Çiğdem Aktuğlu Zeybek

  3. Department of Pediatric Nephrology, Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Türkiye

    Seha Saygılı

  4. Department of Biochemistry, Institute of Cardiology, Istanbul University-Cerrahpaşa, Istanbul, Türkiye

    Ayşem Kaya

Authors
  1. Gürkan Tarçın
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Contributions

G.T. wrote the main manuscript text, G.T. and S.A. collected the samples, all authors reviewed the manuscript.

Corresponding author

Correspondence to Gürkan Tarçın.

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Ethics approval

This study was conducted in accordance with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of Istanbul University-Cerrahpaşa (Date: 3.3.2021 / No: 43399). Written informed consent was obtained from the parents.

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Written informed consent was obtained from the parents.

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The authors declare no competing interests.

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Communicated by Peter de Winter

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Tarçın, G., Ahmadzada, S., Saygılı, S. et al. Evaluating renin and aldosterone levels in children with organic acidemia—therapeutic experience with fludrocortisone. Eur J Pediatr 182, 5447–5453 (2023). https://doi.org/10.1007/s00431-023-05221-8

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  • DOI: https://doi.org/10.1007/s00431-023-05221-8

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