Bartter syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Tayyaba Ali, M.D.[2]

Overview

Natural History

  • Bartter Syndrome usually occurs in childhood. Patient presents with the history of:
    • Constipation
    • Growth failure. The rate of weight gain is much lower than that of other children of similar age and gender.
    • Increased urinary frequency. The patient needs to urinate more often than usual.
    • Low blood pressure
    • Kidney stones
    • Muscle cramping and weakness[1]

Complications

Kidney failure is a possible complication.

  • Bartter syndrome type I and type II are salt-wasting renal tubular disorders that are clinically characterized by polyhydramnios leading to premature delivery, marked polyuria, and a tendency towards nephrocalcinosis.[2]
  • Gallstones might represent a new complication of antenatal Bartter syndrome.[3]

Prognosis

  • The limited prognostic information available suggests that early diagnosis and appropriate treatment of infants and young children with classic Bartter Syndrome (type 3) may improve growth and perhaps neuro-intellectual development. On the other hand, sustained hypokalemia and hyperreninemia can cause progressive tubulointerstitial nephritis, resulting in end-stage renal disease (Kidney failure). With the early treatment of the electrolyte imbalances, the prognosis for patients with Classic Bartter Syndrome is good.
  • Patients with Bartter syndrome type I and II tend to present a satisfactory prognosis after a median follow-up of more than 10 years.[3]

References

  1. "Bartter syndrome: MedlinePlus Medical Encyclopedia".
  2. Seyberth HW (2008). "An improved terminology and classification of Bartter-like syndromes". Nat Clin Pract Nephrol. 4 (10): 560–7. doi:10.1038/ncpneph0912. PMID 18695706.
  3. 3.0 3.1 Puricelli E, Bettinelli A, Borsa N, Sironi F, Mattiello C, Tammaro F; et al. (2010). "Long-term follow-up of patients with Bartter syndrome type I and II". Nephrol Dial Transplant. 25 (9): 2976–81. doi:10.1093/ndt/gfq119. PMID 20219833.


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