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      Severe hypothyroidism caused by hepatic hemangioendothelioma in an infant of a diabetic mother.

      Hormone research
      Abnormalities, Multiple, etiology, Ear, External, abnormalities, pathology, Female, Hemangioendothelioma, complications, enzymology, ultrasonography, Humans, Hypothyroidism, physiopathology, Infant, Newborn, Iodide Peroxidase, metabolism, Liver Neoplasms, Pregnancy, Pregnancy in Diabetics, Severity of Illness Index, Spine, radiography, Ultrasonography, Prenatal

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          Abstract

          Hemangioendotheliomas can express type 3 iodothyronine deiodinase and cause severe hypothyroidism. The risk of congenital malformations such as vertebral and cardiac abnormalities in infants of diabetic mothers is higher than in babies of healthy women. Here we report an infant of a diabetic mother with hypothyroidism caused by liver hemangioendothelioma. Consumptive hypothyroidism should be an indicator to search for a vascular tumor in infants. Supranormal doses of L-thyroxine might be required for normalization of thyroid function until the tumor involutes or is resected. Copyright (c) 2005 S. Karger AG, Basel.

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

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            A 21-year-old woman with consumptive hypothyroidism due to a vascular tumor expressing type 3 iodothyronine deiodinase.

            We present a 21-yr-old female with a large hepatic vascular tumor and subclinical hypothyroidism. A high level of the thyroid hormone inactivating enzyme type 3 iodothyronine deiodinase (D3) was detected in her tumor, and the TSH of 26.2 mU/liter returned to normal after surgical resection of the mass. This indicates that the vascular tumor caused this adult's hypothyroidism as has now been documented in nine infants with this syndrome. This first example of consumptive hypothyroidism in an adult indicates that the inactivation rate of thyroid hormone by D3 in a vascular tumor can stress the secretory capacity even of the TSH-stimulated normal adult thyroid gland.
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              Spontaneous regression of severe acquired infantile hypothyroidism associated with multiple liver hemangiomas.

              A 9-week-old infant presented with severe postnatal hypothyroidism. His hypothyroidism corrected only after his L-thyroxine dose was progressively increased to 28 micro g/kg/d. At 6 months of age, multiple clinically asymptomatic hepatic hemangiomas were detected and support a diagnosis of consumptive hypothyroidism as a result of increased type 3 iodothyronine deiodinase activity in the hemangiomas. Coincident with the involution of the hemangiomas, the child's hypothyroidism improved and L-thyroxin replacement could be stopped at the age of 3 years. Despite some degree of hypothyroidism for several weeks during infancy, his growth and development have been normal.
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