Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome
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 in...
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| Format: | Online |
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Thieme Medical Publishers
2012
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| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3699155/ |
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pubmed-36991552013-08-13 Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome Maruyama, Hidehiko Shinno, Yoko Fujiwara, Kaori Nakamura, Akie Tajima, Toshihiro Nakamura, Makoto Kageyama, Misao Article 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. Thieme Medical Publishers 2012-12-03 2013-05 /pmc/articles/PMC3699155/ /pubmed/23943704 http://dx.doi.org/10.1055/s-0032-1329682 Text en © Thieme Medical Publishers |
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Open Access Journal |
| institution_category |
Foreign Institution |
| institution |
US National Center for Biotechnology Information |
| building |
NCBI PubMed |
| collection |
Online Access |
| language |
English |
| format |
Online |
| author |
Maruyama, Hidehiko Shinno, Yoko Fujiwara, Kaori Nakamura, Akie Tajima, Toshihiro Nakamura, Makoto Kageyama, Misao |
| spellingShingle |
Maruyama, Hidehiko Shinno, Yoko Fujiwara, Kaori Nakamura, Akie Tajima, Toshihiro Nakamura, Makoto Kageyama, Misao Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome |
| author_facet |
Maruyama, Hidehiko Shinno, Yoko Fujiwara, Kaori Nakamura, Akie Tajima, Toshihiro Nakamura, Makoto Kageyama, Misao |
| author_sort |
Maruyama, Hidehiko |
| title |
Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome |
| title_short |
Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome |
| title_full |
Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome |
| title_fullStr |
Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome |
| title_full_unstemmed |
Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome |
| title_sort |
nephrocalcinosis and placental findings in neonatal bartter syndrome |
| description |
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. |
| publisher |
Thieme Medical Publishers |
| publishDate |
2012 |
| url |
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3699155/ |
| _version_ |
1611991114662805504 |