Antenatal Bartter Syndrome: A Review
Antenatal Bartter syndrome (ABS) is a rare autosomal recessive renal tubular disorder. The defective chloride transport in the loop of Henle leads to fetal polyuria resulting in severe hydramnios and premature delivery. Early onset, unexplained maternal polyhydramnios often challenges the treating o...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | International Journal of Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2012/857136 |
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| author | Y. Ramesh Bhat G. Vinayaka K. Sreelakshmi |
| author_facet | Y. Ramesh Bhat G. Vinayaka K. Sreelakshmi |
| author_sort | Y. Ramesh Bhat |
| collection | DOAJ |
| description | Antenatal Bartter syndrome (ABS) is a rare autosomal recessive renal tubular disorder. The defective chloride transport in the loop of Henle leads to fetal polyuria resulting in severe hydramnios and premature delivery. Early onset, unexplained maternal polyhydramnios often challenges the treating obstetrician. Increasing polyhydramnios without apparent fetal or placental abnormalities should lead to the suspicion of this entity. Biochemical analysis of amniotic fluid is suggested as elevated chloride level is usually diagnostic. Awareness, early recognition, maternal treatment with indomethacin, and amniocentesis allow the pregnancy to continue. Affected neonates are usually born premature, have postnatal polyuria, vomiting, failure to thrive, hypercalciuria, and subsequently nephrocalcinosis. Hypokalemia, metabolic alkalosis, secondary hyperaldosteronism and hyperreninaemia are other characteristic features. Volume depletion due to excessive salt and water loss on long term stimulates renin-angiotensin-aldosterone system resulting in juxtaglomerular hyperplasia. Clinical features and electrolyte abnormalities may also depend on the subtype of the syndrome. Prenatal diagnosis and timely indomethacin administration prevent electrolyte imbalance, restitute normal growth, and improve activity. In this paper, authors present classification, pathophysiology, clinical manifestations, laboratory findings, complications, and prognosis of ABS. |
| format | Article |
| id | doaj-art-2d6d6ce6b40642f5bfe81597273c9047 |
| institution | Kabale University |
| issn | 1687-9740 1687-9759 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Pediatrics |
| spelling | doaj-art-2d6d6ce6b40642f5bfe81597273c90472025-02-03T06:42:03ZengWileyInternational Journal of Pediatrics1687-97401687-97592012-01-01201210.1155/2012/857136857136Antenatal Bartter Syndrome: A ReviewY. Ramesh Bhat0G. Vinayaka1K. Sreelakshmi2Department of Paediatrics, Kasturba Medical College, Manipal University, Udupi District, Manipal 576104, IndiaDepartment of Paediatrics, Kasturba Medical College, Manipal University, Udupi District, Manipal 576104, IndiaDepartment of Obstetrics and Gynecology, Kasturba Medical College, Manipal University, Udupi District, Manipal 576104, IndiaAntenatal Bartter syndrome (ABS) is a rare autosomal recessive renal tubular disorder. The defective chloride transport in the loop of Henle leads to fetal polyuria resulting in severe hydramnios and premature delivery. Early onset, unexplained maternal polyhydramnios often challenges the treating obstetrician. Increasing polyhydramnios without apparent fetal or placental abnormalities should lead to the suspicion of this entity. Biochemical analysis of amniotic fluid is suggested as elevated chloride level is usually diagnostic. Awareness, early recognition, maternal treatment with indomethacin, and amniocentesis allow the pregnancy to continue. Affected neonates are usually born premature, have postnatal polyuria, vomiting, failure to thrive, hypercalciuria, and subsequently nephrocalcinosis. Hypokalemia, metabolic alkalosis, secondary hyperaldosteronism and hyperreninaemia are other characteristic features. Volume depletion due to excessive salt and water loss on long term stimulates renin-angiotensin-aldosterone system resulting in juxtaglomerular hyperplasia. Clinical features and electrolyte abnormalities may also depend on the subtype of the syndrome. Prenatal diagnosis and timely indomethacin administration prevent electrolyte imbalance, restitute normal growth, and improve activity. In this paper, authors present classification, pathophysiology, clinical manifestations, laboratory findings, complications, and prognosis of ABS.http://dx.doi.org/10.1155/2012/857136 |
| spellingShingle | Y. Ramesh Bhat G. Vinayaka K. Sreelakshmi Antenatal Bartter Syndrome: A Review International Journal of Pediatrics |
| title | Antenatal Bartter Syndrome: A Review |
| title_full | Antenatal Bartter Syndrome: A Review |
| title_fullStr | Antenatal Bartter Syndrome: A Review |
| title_full_unstemmed | Antenatal Bartter Syndrome: A Review |
| title_short | Antenatal Bartter Syndrome: A Review |
| title_sort | antenatal bartter syndrome a review |
| url | http://dx.doi.org/10.1155/2012/857136 |
| work_keys_str_mv | AT yrameshbhat antenatalbarttersyndromeareview AT gvinayaka antenatalbarttersyndromeareview AT ksreelakshmi antenatalbarttersyndromeareview |