Congenital Nephrotic Syndrome and Persistent Hypothyroidism After Bilateral Nephrectomy
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Abstract
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 μg/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 μg/d) at age 14 years. No goiter was detected clinically and antithyroid antibodies were negative. Thyroid ultrasound and 1231 scan revealed a thyroid gland in the anatomically normal location. 1231 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.
© 2020 by Walter de Gruyter Berlin/Boston
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Articles in the same Issue
- TITLE
- TABLE OF CONTENTS
- EDITORIAL. Why the Consensus for Consensus?
- REVIEW. Translating Transition: A Critical Review of the Diabetes Literature
- SOCIETY NEWS. VanWyk Award Acceptance Speech, May 5, 2008
- IMAGES IN PEDIATRIC ENDOCRINOLOGY. Pseudopuberty and Juvenile Hypothyroidism
- ORIGINAL CONTRIBUTIONS
- Long-term Follow-up of Patients with Congenital Hyperinsulinism in Austria
- An Analysis of Predictive Factors for the Conversion from Premature Thelarche into Complete Central Precocious Puberty
- Propylthiouracil Associated Antineutrophil Cytoplasmic Antibodies (ANCA) in Patients with Childhood Onset Graves' Disease
- Molecular Analysis of the AR and SRD5A2 Genes in Patients with 46,XY Disorders of Sex Development
- Efficacy and Safety of Inhaled Human Insulin (Exu hera®) Compared to Subcutaneous Insulin in Children Ages 6 to 11 Years with Type 1 Diabetes Mellitus: Results of a 3-Month, Randomized, Parallel Trial
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- Congenital Nephrotic Syndrome and Persistent Hypothyroidism After Bilateral Nephrectomy
- LETTER TO THE EDITOR
- Re: Relationship between endothelial dysfunction, carotid artery intima media thickness and circulating markers of vascular inflammation in obese hypertensive children and adolescents (J Pediatr Endocrinol Metab 2007; 20: 1125-1136)
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