Proximal renal tubular acidosis with primary Fanconi syndrome

Authors

  • Prijo Philip Department of Pediatrics, K. S. Hegde Medical Academy, Mangalore, Karnataka, India
  • Chinthu Sara Jacob Department of General Medicine, K. S. Hegde Medical Academy, Mangalore, Karnataka, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20181556

Keywords:

Bicarbonate wasting, Fanconi syndrome, Proximal RTA, Rickets

Abstract

Renal tubular acidosis (RTA) is associated with normal or near normal glomerular filtration rate. Proximal RTA is associated with impaired bicarbonate reabsorption. This is manifested as bicarbonate wastage in the urine, and this reflects the defect in proximal tubular transport. Osteopenia or full-blown rickets may develop. Type 2 RTA is rare and occurs in association with conditions such as Fanconi syndrome. This is manifested as glycosuria, aminoaciduria, phosphate wasting and mild proteinuria. The basis of therapy is the continuous administration of appropriate amounts of alkali in the form of either bicarbonate or citrate, as well as the treatment of the cause.

References

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Soriano JR. Renal tubular acidosis: the clinical entity. JASN. 2002;13(8):2160-70.

Haque SK, Ariceta G, Batlle D. Proximal renal tubular acidosis: a not so rare disorder of multiple etiologies. Nephrol Dial Transplant. 2012;27(12):4273-87.

Long WS, Seashore MR, Siegel NJ, Bia MJ. Idiopathic Fanconi syndrome with progressive renal failure: a case report and discussion. Yale J Biol Med. 1990;63(1):15-28.

Deshpande P, Ali U. Primary Fanconi syndrome. 1997;34:547-9.

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Published

2018-04-20

Issue

Section

Case Reports