Bartter syndrome natural history, complications and prognosis

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Main article: Bartter syndrome

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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

Bartter Syndrome usually occurs in childhood. 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. Common complications of Bartter syndrome include Gallstones, Rhabdomyolysis, Prolonged QT interval, Life-threatening arrhythmia, Syncope, Sudden death, weakening of the bones and Renal failure. 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.

Natural History

Complications

Prognosis

  • The prognosis is based on mutations or depends on the degree of the receptor dysfunction.[8]
  • 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 3.2 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.
  4. Hacihamdioglu DO, Fidanci K, Kilic A, Gok F, Topaloglu R (2013). "QT and JT dispersion and cardiac performance in children with neonatal Bartter syndrome: a pilot study". Pediatr Nephrol. 28 (10): 1969–74. doi:10.1007/s00467-013-2517-5. PMID 23760993.
  5. Scognamiglio R, Negut C, Calò LA (2007). "Aborted sudden cardiac death in two patients with Bartter's/Gitelman's syndromes". Clin Nephrol. 67 (3): 193–7. doi:10.5414/cnp67193. PMID 17390745.
  6. Reungjui S, Roncal CA, Sato W, Glushakova OY, Croker BP, Suga S; et al. (2008). "Hypokalemic nephropathy is associated with impaired angiogenesis". J Am Soc Nephrol. 19 (1): 125–34. doi:10.1681/ASN.2007030261. PMC 2391040. PMID 18178802.
  7. "Bartter syndrome - Genetics Home Reference - NIH".
  8. 8.0 8.1 Al Shibli A, Narchi H (2015). "Bartter and Gitelman syndromes: Spectrum of clinical manifestations caused by different mutations". World J Methodol. 5 (2): 55–61. doi:10.5662/wjm.v5.i2.55. PMC 4482822. PMID 26140272.


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