Etiology and clinical manifestations of renal tubular acidosis in infants and children
- Tej K Mattoo, MD, DCH, FRCP
Tej K Mattoo, MD, DCH, FRCP
- Section Editor — Pediatric Nephrology
- Professor of Pediatrics
- Wayne State University School of Medicine
In children, renal tubular acidosis (RTA) is due to either an inherited or acquired defect that affects the kidney's ability to absorb filtered bicarbonate, or excrete ammonia or titratable acid. RTA is characterized by a normal anion gap (hyperchloremic) metabolic acidosis caused by the net retention of hydrogen or loss of bicarbonate.
There are four subgroups of RTA (table 1):
●Distal (type 1) RTA
●Proximal (type 2) RTA
●Mixed (type 3) RTA
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- DISTAL (TYPE 1) RTA
- - Genetic causes
- - Acquired causes
- Clinical manifestations
- - Recessive form
- - Dominant form
- - Chronic kidney disease
- PROXIMAL (TYPE 2) RTA
- - Isolated proximal RTA
- - Fanconi syndrome
- Clinical features
- - Clinical findings of Fanconi syndrome
- MIXED (TYPE 3) RTA
- ALDOSTERONE DEFICIENCY OR RESISTANCE (TYPE 4 RTA)