| Congenital nephrotic syndrome and persistent hypothyroidism after bilateral nephrectomy. | |
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MedLine Citation:
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PMID: 18717247 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Congenital nephrotic syndrome is commonly associated with hypothyroidism. Thyroid hormone supplementation is recommended as standard of care. The hypothyroidism is postulated to occur secondary to chronic massive proteinuria with loss of thyroid binding globulin, thyroid hormone and iodine. Previous reports have indicated that thyroxin may be discontinued following bilateral nephrectomy. We report our experience with one child with congenital nephrotic syndrome, Finnish type, and hypothyroidism who had a high requirement for thyroxin (100-150 microg/d) from infancy to 4 years of age. Hypothyroidism persisted despite bilateral nephrectomy and later following renal transplantation. However, his thyroxin requirement is now substantially lower (62.5 microg/d) at age 14 years. No goiter was detected clinically and antithyroid antibodies were negative. Thyroid ultrasound and 123I scan revealed a thyroid gland in the anatomically normal location. 123I uptake was elevated, 18% at 6 hours and 51% at 24 hours (normal values: 3-16% at 6 hours and 8-25% at 24 hours). Perchlorate was unavailable for a perchlorate washout study. We speculate that this patient may have an intrinsic problem with thyroid hormone synthesis. It is unclear whether this is related or coincidental to the Finnish nephrotic syndrome. We recommend following thyroid functions closely if thyroxin is discontinued following bilateral nephrectomies in Finnish type congenital nephrotic syndrome. |
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Authors:
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Martina Kacer; Dilys A Whyte; Ivy Boydstun; Thomas A Wilson |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of pediatric endocrinology & metabolism : JPEM Volume: 21 ISSN: 0334-018X ISO Abbreviation: J. Pediatr. Endocrinol. Metab. Publication Date: 2008 Jun |
Date Detail:
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Created Date: 2008-08-22 Completed Date: 2008-09-30 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9508900 Medline TA: J Pediatr Endocrinol Metab Country: England |
Other Details:
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Languages: eng Pagination: 597-601 Citation Subset: IM |
Affiliation:
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Division of Pediatric Endocrinology, Department of Pediatrics, State University of New York, Stony Brook, NY 11794-8111, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Congenital Hypothyroidism
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complications*,
drug therapy,
surgery Follow-Up Studies Humans Infant Male Nephrectomy / methods Nephrotic Syndrome / complications*, congenital*, surgery* Thyroxine / therapeutic use |
| Chemical | |
Reg. No./Substance:
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7488-70-2/Thyroxine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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