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Hyperkalemic Renal Tubular Acidosis (RTA Type IV)

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Renal Tubular Acidosis in Children

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Abstract

Contrary to adults, Type IV RTA is the least frequent type of RTA in children, and the only one showing hyperkalemia. Etiology: primary hypoaldosteronism or pseudohypoaldosteronism. Primary hypoaldosteronism (adrenal insufficiency, Addison’s disease) presents in young children. In older children, the etiology is autoimmune dysplasia, adrenal hemorrhage or infections, amyloidosis, and systemic lupus. Deficiency of 21-α-hydroxylase causes neonatal adrenogenital syndrome. Pseudo-hypoaldosteronism is a lack of response of the kidney to aldosterone actions, secondary to diverse entities with tubulointerstitial nephritis or toxic drugs. Hereditary pseudohypoaldosteronism presents as two types: Type I (PHAI) and Type II (PHAII), with hyperkalemic RTA and different clinical features. Determination of the renal transtubular potassium gradient (GGTK) is used to differentiate hypoaldosteronism from pseudohypoaldosteronism. Treatment aims to correct the hyperkalemia and the metabolic acidosis, and also it depends on the primary disease involved or aldosterone alteration.

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Authors and Affiliations

  1. Department of Pediatric Nephrology, National Medical Center La Raza, Mexican Institute of Social Security, Mexico City, Mexico

    Jesús Lagunas-Muñoz

  2. High Specialty Medical Unit, General Hospital “Dr. Gaudencio González Garza.” National Medical Center La Raza, Mexican Institute of Social Security, Mexico City, Mexico

    Jesús Lagunas-Muñoz

  3. Retired from: Department of Pediatric Nephrology, Hemodialysis Unit, The Children’s Hospital at Albany Medical Center, Albany, NY, USA

    Ricardo Muñoz

  4. Retired from: Pediatric Nephrology Department, Hospital Infantil de México Federico Gómez, Mexico City, Mexico

    Ricardo Muñoz

  5. Retired from: Research Division, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico

    Ricardo Muñoz

  6. Retired from: National System of Investigators, Level I, Mexico City, Mexico

    Ricardo Muñoz

  7. Retired from: North American Pediatric Renal Transplantation Collaborative Study (NAPRTCS) Study, Woburn, MA, USA

    Ricardo Muñoz

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    Ricardo Muñoz

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Lagunas-Muñoz, J., Muñoz, R. (2022). Hyperkalemic Renal Tubular Acidosis (RTA Type IV). In: Muñoz, R. (eds) Renal Tubular Acidosis in Children. Springer, Cham. https://doi.org/10.1007/978-3-030-91940-5_10

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