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Bartter syndrome

MedGen UID:
2172
Concept ID:
C0004775
Disease or Syndrome
Synonyms: Bartter's syndrome; Potassium wasting
SNOMED CT: Bartter's syndrome (707742001); Bartter syndrome (707742001); Bartter syndrome with hypercalciuria and nephrocalcinosis (707742001)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
X-linked recessive inheritance
MedGen UID:
375779
Concept ID:
C1845977
Finding
Source: Orphanet
A mode of inheritance that is observed for recessive traits related to a gene encoded on the X chromosome. In the context of medical genetics, X-linked recessive disorders manifest in males (who have one copy of the X chromosome and are thus hemizygotes), but generally not in female heterozygotes who have one mutant and one normal allele.
 
Related genes: MAGED2, BSND, SLC12A1, KCNJ1, CLCNKB, CLCNKA
 
Monarch Initiative: MONDO:0015231
OMIM® Phenotypic series: PS601678
Orphanet: ORPHA112

Definition

Bartter syndrome is a group of very similar kidney disorders that cause an imbalance of potassium, sodium, chloride, and related molecules in the body.

In some cases, Bartter syndrome becomes apparent before birth. The disorder can cause polyhydramnios, which is an increased volume of fluid surrounding the fetus (amniotic fluid). Polyhydramnios increases the risk of premature birth.

Two major forms of Bartter syndrome are distinguished by their age of onset and severity. One form begins before birth (antenatal) and is often life-threatening. The other form, often called the classical form, begins in early childhood and tends to be less severe. Once the genetic causes of Bartter syndrome were identified, researchers also split the disorder into different types based on the genes involved. Types I, II, and IV have the features of antenatal Bartter syndrome. Because type IV is also associated with hearing loss, it is sometimes called antenatal Bartter syndrome with sensorineural deafness. Type III usually has the features of classical Bartter syndrome.

Beginning in infancy, affected individuals often fail to grow and gain weight at the expected rate (failure to thrive). They lose excess amounts of salt (sodium chloride) in their urine, which leads to dehydration, constipation, and increased urine production (polyuria). In addition, large amounts of calcium are lost through the urine (hypercalciuria), which can cause weakening of the bones (osteopenia). Some of the calcium is deposited in the kidneys as they are concentrating urine, leading to hardening of the kidney tissue (nephrocalcinosis). Bartter syndrome is also characterized by low levels of potassium in the blood (hypokalemia), which can result in muscle weakness, cramping, and fatigue. Rarely, affected children develop hearing loss caused by abnormalities in the inner ear (sensorineural deafness). [from MedlinePlus Genetics]

Professional guidelines

PubMed

Do C, Vasquez PC, Soleimani M
Am J Kidney Dis 2022 Oct;80(4):536-551. Epub 2022 May 5 doi: 10.1053/j.ajkd.2021.12.016. PMID: 35525634Free PMC Article
Konrad M, Nijenhuis T, Ariceta G, Bertholet-Thomas A, Calo LA, Capasso G, Emma F, Schlingmann KP, Singh M, Trepiccione F, Walsh SB, Whitton K, Vargas-Poussou R, Bockenhauer D
Kidney Int 2021 Feb;99(2):324-335. doi: 10.1016/j.kint.2020.10.035. PMID: 33509356
Assadi F
Iran J Kidney Dis 2008 Jul;2(3):115-22. PMID: 19377223

Recent clinical studies

Etiology

Konrad M, Nijenhuis T, Ariceta G, Bertholet-Thomas A, Calo LA, Capasso G, Emma F, Schlingmann KP, Singh M, Trepiccione F, Walsh SB, Whitton K, Vargas-Poussou R, Bockenhauer D
Kidney Int 2021 Feb;99(2):324-335. doi: 10.1016/j.kint.2020.10.035. PMID: 33509356
Mrad FCC, Soares SBM, de Menezes Silva LAW, Dos Anjos Menezes PV, Simões-E-Silva AC
World J Pediatr 2021 Feb;17(1):31-39. Epub 2020 Jun 1 doi: 10.1007/s12519-020-00370-4. PMID: 32488762
Howles SA, Thakker RV
Nat Rev Urol 2020 Jul;17(7):407-421. Epub 2020 Jun 12 doi: 10.1038/s41585-020-0332-x. PMID: 32533118
Mehta L, Jim B
Semin Nephrol 2017 Jul;37(4):354-361. doi: 10.1016/j.semnephrol.2017.05.007. PMID: 28711074
Seyberth HW, Weber S, Kömhoff M
Curr Opin Pediatr 2017 Apr;29(2):179-186. doi: 10.1097/MOP.0000000000000447. PMID: 27906863

Diagnosis

Nuñez-Gonzalez L, Carrera N, Garcia-Gonzalez MA
Int J Mol Sci 2021 Oct 22;22(21) doi: 10.3390/ijms222111414. PMID: 34768847Free PMC Article
Konrad M, Nijenhuis T, Ariceta G, Bertholet-Thomas A, Calo LA, Capasso G, Emma F, Schlingmann KP, Singh M, Trepiccione F, Walsh SB, Whitton K, Vargas-Poussou R, Bockenhauer D
Kidney Int 2021 Feb;99(2):324-335. doi: 10.1016/j.kint.2020.10.035. PMID: 33509356
Mrad FCC, Soares SBM, de Menezes Silva LAW, Dos Anjos Menezes PV, Simões-E-Silva AC
World J Pediatr 2021 Feb;17(1):31-39. Epub 2020 Jun 1 doi: 10.1007/s12519-020-00370-4. PMID: 32488762
Fulchiero R, Seo-Mayer P
Pediatr Clin North Am 2019 Feb;66(1):121-134. doi: 10.1016/j.pcl.2018.08.010. PMID: 30454738
Mehta L, Jim B
Semin Nephrol 2017 Jul;37(4):354-361. doi: 10.1016/j.semnephrol.2017.05.007. PMID: 28711074

Therapy

Do C, Vasquez PC, Soleimani M
Am J Kidney Dis 2022 Oct;80(4):536-551. Epub 2022 May 5 doi: 10.1053/j.ajkd.2021.12.016. PMID: 35525634Free PMC Article
Nuñez-Gonzalez L, Carrera N, Garcia-Gonzalez MA
Int J Mol Sci 2021 Oct 22;22(21) doi: 10.3390/ijms222111414. PMID: 34768847Free PMC Article
Blanchard A, Bockenhauer D, Bolignano D, Calò LA, Cosyns E, Devuyst O, Ellison DH, Karet Frankl FE, Knoers NV, Konrad M, Lin SH, Vargas-Poussou R
Kidney Int 2017 Jan;91(1):24-33. doi: 10.1016/j.kint.2016.09.046. PMID: 28003083
Assadi F
Iran J Kidney Dis 2008 Jul;2(3):115-22. PMID: 19377223
Bartter FC
Urol Clin North Am 1977 Jun;4(2):253-61. PMID: 331616

Prognosis

Palazzo V, Raglianti V, Landini S, Cirillo L, Errichiello C, Buti E, Artuso R, Tiberi L, Vergani D, Dirupo E, Romagnani P, Mazzinghi B, Becherucci F
Int J Mol Sci 2022 May 18;23(10) doi: 10.3390/ijms23105641. PMID: 35628451Free PMC Article
Capolongo G, Zacchia M, Perna A, Viggiano D, Capasso G
Urolithiasis 2019 Feb;47(1):91-98. Epub 2018 Dec 18 doi: 10.1007/s00240-018-01104-y. PMID: 30564846
Bockenhauer D, Bichet DG
Curr Opin Pediatr 2017 Apr;29(2):199-205. doi: 10.1097/MOP.0000000000000473. PMID: 28134709
Blanchard A, Bockenhauer D, Bolignano D, Calò LA, Cosyns E, Devuyst O, Ellison DH, Karet Frankl FE, Knoers NV, Konrad M, Lin SH, Vargas-Poussou R
Kidney Int 2017 Jan;91(1):24-33. doi: 10.1016/j.kint.2016.09.046. PMID: 28003083
Knoers NV, Levtchenko EN
Orphanet J Rare Dis 2008 Jul 30;3:22. doi: 10.1186/1750-1172-3-22. PMID: 18667063Free PMC Article

Clinical prediction guides

Güven S, Gökçe İ, Alavanda C, Çiçek N, Demirci EB, Sak M, Pul S, Türkkan ÖN, Yıldız N, Ata P, Alpay H
Turk J Pediatr 2022;64(5):825-838. doi: 10.24953/turkjped.2021.4697. PMID: 36305432
Ravarotto V, Bertoldi G, Stefanelli LF, Nalesso F, Calò LA
Kidney Blood Press Res 2022;47(9):556-564. Epub 2022 Jul 20 doi: 10.1159/000526070. PMID: 35858584Free PMC Article
Sismanlar Eyuboglu T, Dogru D, Çakır E, Cobanoglu N, Pekcan S, Cinel G, Yalçın E, Kiper N, Sen V, Selimoglu Sen H, Ercan O, Keskin O, Bilgic Eltan S, Alshadfan L, Yazan H, Altıntas DU, Sasihuseyinoglu AS, Sapan N, Cekic S, Cokugraş H, Kılınc AA, Ramaslı Gursoy T, Aslan AT, Bingol A, Başaran AE, Ozdemir A, Kose M, Hangul M, Emiralioglu N, Tugcu G, Yuksel H, Yılmaz O, Orhan F, Gayretli Aydın ZG, Topal E, Tamay Z, Suleyman A, Can D, Bal CM, Caltepe G, Ozcelik U
Pediatr Pulmonol 2020 Aug;55(8):2011-2016. Epub 2020 May 4 doi: 10.1002/ppul.24805. PMID: 32364312
Capolongo G, Zacchia M, Perna A, Viggiano D, Capasso G
Urolithiasis 2019 Feb;47(1):91-98. Epub 2018 Dec 18 doi: 10.1007/s00240-018-01104-y. PMID: 30564846
Kleta R, Bockenhauer D
J Am Soc Nephrol 2018 Mar;29(3):727-739. Epub 2017 Dec 13 doi: 10.1681/ASN.2017060600. PMID: 29237739Free PMC Article

Recent systematic reviews

Qasba RK, Bucharles ACF, Piccoli MVF, Sharma P, Banga A, Kamaraj B, Nawaz FA, Kumar HJ, Happy MA, Qasba RK, Kogilathota Jagirdhar GS, Essar MY, Garg P, Reddy ST, Rama K, Surani S, Kashyap R
Medicina (Kaunas) 2023 Sep 11;59(9) doi: 10.3390/medicina59091638. PMID: 37763757Free PMC Article
Vaisbich MH, Messa ACHL, Rangel-Santos AC, Ferreira JCOA, Nunes FAMDF, Watanabe A
Nephron 2023;147(8):478-495. Epub 2023 Mar 7 doi: 10.1159/000528557. PMID: 36882007
von Vigier RO, Ortisi MT, La Manna A, Bianchetti MG, Bettinelli A
Pediatr Nephrol 2010 May;25(5):861-6. Epub 2009 Dec 22 doi: 10.1007/s00467-009-1388-2. PMID: 20033223

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