Naureen Akhtar, Jaida Manzoor, Shahida Iftikhar, Adeela Chaudhry.
Clinical Types and Outcome of Renal Tubular Acidosis in Children.
Pak J Med Health Sci Jan ;13(2):252-4.

Background: Renal tubular acidosis (RTA) is a tubulopathy characterized by polyuria , polydipsia and failure to thrive with hyperchloremic metabolic acidosis and normal serum anion gap. Aim: To determine the clinical types and outcome of children with RTA. Methods: This was a retrospective observational study carried out in the Department of Nephrology at The Children’s Hospital and The Institute of Child Health Lahore, Pakistan from January 2009 to December 2017. Results: 120 subjects were selected 70% distal, 29% proximal and only one with mixed type of RTA. There were 61% males and 39% females with parental consanguinity identified in 67% and family history positive in 36% cases. Cystinosis, Lowe’s syndrome and Dent disease were found to be the underlying etiologies in 12.5%, 6% and 2% participants with proximal RTA. Failure to thrive and short stature was noted in all patients at the time of diagnosis. The mean serum creatinine on the last visit after 5 years of follow-up was 1.04 +/- 0.95 mg/dl (distal) , 1.01+/-1.12 mg/dl (proximal) and 0.9 mg/dl (mixed) and 12% children were found to develop renal insufficiency . Conclusion: The overall prognosis of RTA is generally good provided the diagnosis is made early with a good compliance to alkali treatment. The findings of our study reiterate the need for early diagnosis in order to institute appropriate treatment for improving outcome regarding growth and preserving renal function.

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