Tubular disorders form a significant proportion of pediatric
kidney diseases and are an important differential diagnosis of
failure to thrive (FTT) in children. Data regarding their outcome is scarce from India. We evaluated the clinical profile of these children and studied the outcome in terms of their growth and
renal failure. This is a retrospective longitudinal study of all children with renal tubular disorders attending a tertiary care pediatric nephrology center from 2005 to 2010. Growth and renal outcomes were assessed by Z scores and estimated glomerular filtration rate at diagnosis and. The common disorders encountered were
distal renal tubular acidosis (d-RTA) (44%), Bartter-like (Bartter's and Gitelman) syndromes (22%) followed by hereditary
Fanconi syndrome (
cystinosis and idiopathic
Fanconi syndrome) (13%) and few cases of
nephrogenic diabetes insipidus,
hypophosphatemic rickets and idiopathic
hypercalciuria. Male: female ratio was 1.22. The median age at diagnosis was 1.5 (range 0.13-11) years. Growth failure was the presenting feature in 86% of children followed by
polyuria (60%) and bone
deformities (47%). In 60% of children with hereditary
Fanconi syndrome,
nephropathic cystinosis was diagnosed, all of whom progressed to stage III
chronic kidney disease (CKD) within 3.41 ± 1.42 years. With appropriate
therapy, catch-up growth was noted in d-RTA and
Bartter syndrome. Renal tubular disorders usually present with FTT. d-RTA is the most common etiology followed by Bartter-like syndrome. Renal function is preserved in all these disorders except for
nephropathic cystinosis, who ultimately progressed to CKD. With appropriate and inexpensive
therapy, these children do grow well.