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      Consumptive Hypothyroidism: Case Report of Hepatic Hemangioendotheliomas Successfully Treated with Vincristine and Systematic Review of the Syndrome

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          Abstract

          Objectives: To provide a comprehensive description of consumptive hypothyroidism syndrome (CHS), a severe form of hypothyroidism that occurs due to the high expression levels of thyroid hormone inactivation enzyme type 3 deiodinase (D3) in tumors. Study Design: Case report and systematic review. Results: A 7-month-old girl with a diagnosis of massive hepatic hemangioendotheliomas was treated with high doses of thyroid hormones and tumor-directed chemotherapy with vincristine. The tumor displayed excellent response, and euthyroid status was regained. A systematic review on the databases PubMed/Medline and Embase was performed, using the term “Consumptive AND “Hypothyroidism.” From the 33 selected references, we extracted 42 case reports of CHS: 36 children and 6 adults. The laboratory profile at diagnosis displayed high TSH and low T<sub>4</sub> and T<sub>3</sub> serum levels. The serum reverse T<sub>3</sub> and D3 activity levels were high in all patients tested. In children, 97% had vascular tumors, whereas in adults 33% were vascular tumors, 33% fibrous tumors, and 33% gastrointestinal stromal tumors. The conservative treatment was predominant in children, while in adults all cases were treated with surgery. Death occurred in 16% of children and 33% of adults. Conclusions: CHS is a rare form of hypothyroidism that occurs in children and adults, usually linked to hepatic vascular tumors. The condition is associated with high lethality. Prompt diagnosis, followed by high-dose thyroid hormone replacement and tumor-directed therapy are the keys to optimize outcomes.

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          Most cited references26

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          Severe hypothyroidism caused by type 3 iodothyronine deiodinase in infantile hemangiomas.

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            Deiodinases: the balance of thyroid hormone: type 1 iodothyronine deiodinase in human physiology and disease.

            Thyroid hormone is essential for the normal function of virtually all tissues. The iodothyronine deiodinases catalyze the removal of an iodine residue from the pro-hormone thyroxine (T(4)) molecule, thus producing either the active form triiodothyronine (T(3); activation) or inactive metabolites (reverse T(3); inactivation). Type I deiodinase (D1) catalyzes both reactions. Over the last years, several studies have attempted to understand the mechanisms of D1 function, underlying its effects on normal thyroid hormone metabolism and pathological processes. Although peripheral D1-generated T(3) production contributes to a portion of plasma T(3) in euthyroid state, pathologically increased thyroidal D1 activity seems to be the main cause of the elevated T(3) concentrations observed in hyperthyroid patients. On the other hand, D1-deficient mouse models show that, in the absence of D1, inactive and lesser iodothyronines are excreted in feces with the loss of associated iodine, demonstrating the scavenging function for D1 that might be particularly important in an iodine deficiency setting. Polymorphisms in the DIO1 gene have been associated with changes in serum thyroid hormone levels, whereas decreased D1 activity has been reported in the nonthyroid illness syndrome and in several human neoplasias. The current review aims at presenting an updated picture of the recent advances made in the biochemical and molecular properties of D1 as well as its role in human physiology.
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              Thyroid hormone inactivation in gastrointestinal stromal tumors.

              Gastrointestinal stromal tumors (GISTs) are resistant to traditional chemotherapy but are responsive to the tyrosine kinase inhibitors imatinib and sunitinib. The use of these agents has improved the outcome for patients but is associated with adverse effects, including hypothyroidism. Multiple mechanisms of this effect have been proposed, including decreased iodine organification and glandular capillary regression. Here we report the finding of consumptive hypothyroidism caused by marked overexpression of the thyroid hormone-inactivating enzyme type 3 iodothyronine deiodinase (D3) within the tumor. Affected patients warrant increased monitoring and may require supernormal thyroid hormone supplementation.
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                Author and article information

                Journal
                ETJ
                ETJ
                10.1159/issn.2235-0640
                European Thyroid Journal
                S. Karger AG
                2235-0640
                2235-0802
                2017
                November 2017
                13 October 2017
                : 6
                : 6
                : 321-327
                Affiliations
                Thyroid Section, Endocrine Division, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil
                Author notes
                *José Miguel Dora, MD, PhD, Thyroid Unit, Endocrine Division, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcelos 2350, Porto Alegre, RS 90035-003 (Brazil), E-Mail jdora@hcpa.edu.br
                Article
                481253 PMC5704697 Eur Thyroid J 2017;6:321–327
                10.1159/000481253
                PMC5704697
                29234626
                3d794b9e-0bd9-47d0-95fa-109ad98909e7
                © 2017 European Thyroid Association. Published by S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 21 July 2017
                : 05 September 2017
                Page count
                Figures: 1, Tables: 2, Pages: 7
                Categories
                Clinical Thyroidology / Original Paper

                Endocrinology & Diabetes,Neurology,Nutrition & Dietetics,Sexual medicine,Internal medicine,Pharmacology & Pharmaceutical medicine
                Type 3 deiodinase,Vincristine,Hepatic hemangioendothelioma,Consumptive hypothyroidism,Infantile hypothyroidism

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