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Proximal Renal Tubular Acidosis (Type II)

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Renal Tubular Acidosis in Children

Abstract

The main functions of the proximal tubule are sodium-bicarbonate Na+HCO3− reabsorption (90% of filtered) and ammonia production. Na+HCO3− reabsorption is via NHE3 antiporter (exchanger) at the apical membrane, (Na+/H+ ions). Na+K+ATPase exchanges Na+/K+ (basolateral membrane). Na++HCO3− get reabsorbed at the basolateral membrane (NBCe1 cotransporter). Carbonic anhydrase (CA) favors Na+/H+ exchange. Chromosomal mutations (NHE3, NBCe1, CA) lead to hereditary RTA. Secondary RTAs to systemic diseases are several: nephropathic cystinosis, hypophosphatemic rickets, lupus nephropathy, and drug intoxication. Clinical manifestations are mainly polyuria, polydipsia failure to thrive, rickets, Fanconi’s syndrome (anorexia, vomiting, aminoaciduria, hyperchloremic RTA (normal anion gap), hypokalemia, glycosuria, phosphaturia). Treatment is based mainly on bicarbonate administration, and management of the primary disease in cases of secondary RTAs.

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Authors and Affiliations

  1. Research and Diagnosis of Nephrology and, Bone Mineral Metabolism, Hospital Infantil de México Federico Gómez, Mexico City, Mexico

    Mara Medeiros & Omar Guadarrama

  2. Faculty of Medicine, National Autonomous University of Mexico, Mexico City, Mexico

    Mara Medeiros

  3. National System of Investigators, Level III, Mexico City, Mexico

    Mara Medeiros

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

    Ricardo Muñoz

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

    Ricardo Muñoz

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

    Ricardo Muñoz

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

    Ricardo Muñoz

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

    Ricardo Muñoz

Authors
  1. Mara Medeiros
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  2. Omar Guadarrama
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Editors and Affiliations

  1. Mexico City, Distrito Federal, Mexico

    Ricardo Muñoz

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Medeiros, M., Guadarrama, O., Muñoz, R. (2022). Proximal Renal Tubular Acidosis (Type II). In: Muñoz, R. (eds) Renal Tubular Acidosis in Children. Springer, Cham. https://doi.org/10.1007/978-3-030-91940-5_8

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  • DOI: https://doi.org/10.1007/978-3-030-91940-5_8

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-91939-9

  • Online ISBN: 978-3-030-91940-5

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