®®®® SIIA Público

Título del libro: Renal Tubular Acidosis In Children: New Insights In Diagnosis And Treatment
Título del capítulo: Proximal renal tubular acidosis (Type II)

Autores UNAM:
MARA MEDEIROS DOMINGO;
Autores externos:

Idioma:

Año de publicación:
2022
Palabras clave:

Fanconi's syndrome; Hyperchloremia; Hypokalemia; Hypophosphatemic rickets; Metabolic acidosis; Secondary RTAs


Resumen:

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. © The Author(s), under exclusive license to Springer Nature Switzerland AG 2022.


Entidades citadas de la UNAM: