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Título del libro: Renal Tubular Acidosis In Children: New Insights In Diagnosis And Treatment
Título del capítulo: Classification of renal tubular acidosis

Autores UNAM:
NORMA ELIZABETH GUERRA HERNANDEZ;
Autores externos:

Idioma:

Año de publicación:
2022
Palabras clave:

Anion gap; Fanconi syndrome; Hyperchloremia; Primary; RTAs Types I-IV; Secondary


Resumen:

RTA is classified into four types, but Type III is nowadays considered a subtype of Type I RTA. All RTAs are subdivided into primary or secondary, frequently to cystinosis, Lowe's syndrome, hypophosphatemic rickets, considered rare diseases. Secondary RTAs are accompanied by Toni-Fanconi-Debré syndrome (glycosuria, aminoaciduria, phosphaturia, hypercalciuria, hyperuricosuria, and RTA). Primary RTAs are hereditary due to chromosomal mutations. Proximal RTA (Type II). Characterized by a defect in renal proximal tubular reabsorption of bicarbonate. Clinically presents monosymptomatic, but more frequently seen together with Fanconi's syndrome and, failure to thrive and, rickets. Distal RTA (Type I). Characterized by the inability of the A-intercalated cells (renal collecting tubules) to secrete hydrogen ions. Clinical onset is within the first year of life, with metabolic acidosis, (normal anion gap, hypercalciuria, hypocitraturia, and nephrocalcinosis), evident within the perinatal period; frequently with early or late sensorineural deafness. Type III is a mixed, proximal, and distal RTA. Type IV is a hyperkalemic RTA due to hypoaldosteronism or pseudohypoaldosteronism. © The Author(s), under exclusive license to Springer Nature Switzerland AG 2022.


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