Subscribe to RSS
![](/products/assets/desktop/img/oa-logo.png)
DOI: 10.1055/s-0032-1329682
Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome
Publication History
21 April 2012
11 July 2012
Publication Date:
03 December 2012 (online)
![](https://www.thieme-connect.de/media/ajpr/201301/lookinside/thumbnails/10.1055-s-0032-1329682-1.jpg)
Abstract
Neonatal Bartter syndrome (NBS) is an inherited renal tubular disorder associated with hypokalemic alkalosis. Here we report a case of genetically diagnosed NBS. Polyhydramnios was noted at 26 weeks. A boy was born at 31 weeks and 1 day, weighed 1344 g, and had an Apgar score of 8/8. We initiated indomethacin (IND) at a dose of 0.2 mg/kg/d on day 31, and increased it to approximately 3 mg/kg/d. However, his urinary calcium (Ca) levels remained unchanged. At 4 months of age, nephrocalcinosis was detected by ultrasound. The placenta weighed 700 g (+2.7 standard deviations). Although the proportion of terminal villi was consistent with the gestational age, many of them exhibited poorly dilated capillaries. Hemosiderin pigment was seen throughout the amniochorionic connective tissue and along about 50% of the trophoblast basement membrane (TBM). Von Kossa stain revealed the corresponding area of mineralization along the TBM. In our opinion, urinary Ca levels were high and did not change after IND initiation, indicating that nephrocalcinosis may be inevitable. Enhanced inflow of maternal plasma through the basement membrane would cause Ca deposition, given that the same finding was obtained in the case with polyhydramnios. The same mechanism would also explain the hemosiderin pigment distribution.
-
References
- 1 Bartter FC, Gill Jr JR, Frolich JC , et al. Prostaglandins are overproduced by the kidneys and mediate hyperreninemia in Bartter's syndrome. Trans Assoc Am Physicians 1976; 89: 77-91
- 2 Bartter FC, Pronove P, Gill Jr JR, MacCardle RC. Hyperplasia of the juxtaglomerular complex with hyperaldosteronism and hypokalemic alkalosis. A new syndrome. Am J Med 1962; 33: 811-828
- 3 Gill Jr JR, Frölich JC, Bowden RE , et al. Bartter's syndrome: a disorder characterized by high urinary prostaglandins and a dependence of hyperreninemia on prostaglandin synthesis. Am J Med 1976; 61: 43-51
- 4 Adachi M, Asakura Y, Sato Y , et al. Novel SLC12A1 (NKCC2) mutations in two families with Bartter syndrome type 1. Endocr J 2007; 54: 1003-1007
- 5 Dane B, Dane C, Aksoy F, Cetin A, Yayla M. Antenatal Bartter syndrome: analysis of two cases with placental findings. Fetal Pediatr Pathol 2010; 29: 121-126
- 6 Konrad M, Leonhardt A, Hensen P, Seyberth HW, Köckerling A. Prenatal and postnatal management of hyperprostaglandin E syndrome after genetic diagnosis from amniocytes. Pediatrics 1999; 103: 678-683
- 7 Murakami M, Kudo I. Prostaglandin E synthase: a novel drug target for inflammation and cancer. Curr Pharm Des 2006; 12: 943-954
- 8 Nakayama M, Arai H, Takeuchi M , et al. Organ weights, cardiac size in infancy among with placental size and weight [in Japanese]. The Journal of Osaka Medical Center and Research Institute of Maternal and Child Health 1997; 13: 103-115
- 9 Sood BG, Lulic-Botica M, Holzhausen KA , et al. The risk of necrotizing enterocolitis after indomethacin tocolysis. Pediatrics 2011; 128: e54-e62
- 10 Nakagawa Y, Toya K, Natsume H , et al. Long-term follow-up of a girl with the neonatal form of Bartter's syndrome. Endocr J 1997; 44: 275-281
- 11 Ernst LM, Parkash V. Placental pathology in fetal Bartter syndrome. Pediatr Dev Pathol 2002; 5: 76-79
- 12 Grigsby PL, Sooranna SR, Brockman DE, Johnson MR, Myatt L. Localization and expression of prostaglandin E2 receptors in human placenta and corresponding fetal membranes with labor. Am J Obstet Gynecol 2006; 195: 260-269
- 13 Williams PJ, Mistry HD, Innes BA, Bulmer JN, Broughton Pipkin F. Expression of AT1R, AT2R and AT4R and their roles in extravillous trophoblast invasion in the human. Placenta 2010; 31: 448-455
- 14 Krohn K, Ljungqvist A, Robertson B. Trophoblastic and subtrophoblastic mineral salt deposition in hydramnios. Acta Pathol Microbiol Scand 1967; 69: 514-520