title: Type 3 deiodinase and consumptive hypothyroidism: a common mechanism for a rare disease.
abstract: The major product secreted by the thyroid is thyroxine (T4), whereas most of the biologically active triiodothyronine (T3) derives from the peripheral conversion of T4 into T3. The deiodinase enzymes are involved in activation and inactivation of thyroid hormones (THs). Type 1 and type 2 deiodinase (D1 and D2) convert T4 into T3 whereas D3 degrades T4 and T3 into inactive metabolites and is thus the major physiological TH inactivator. The hypothalamic-pituitary-thyroid axis maintains circulating TH levels constant, while the deiodinases tissue-specifically regulate intracellular thyroid status by controlling TH action in a precise spatio-temporal fashion. Here we review the data related to the recent identification of a paraneoplastic syndrome called "consumptive hypothyroidism," which exemplifies how deiodinases alter substantially the concentration of TH in blood. This syndrome results from the aberrant uncontrolled expression of D3 that can induce a severe form of hypothyroidism by inactivating T4 and T3 in defined tumor tissue. This rare TH insufficiency generally affects patients in the first years of life, and has distinct features in terms of diagnosis, treatment, and prognosis with respect to other forms of hypothyroidism.
authors.affiliation: Department of Clinical Medicine and Surgery, University of Naples "Federico II," Naples , Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Luongo
authors.forename: Luigi
authors.initials: L
authors.lastname: Trivisano
authors.forename: Fausta
authors.initials: F
authors.lastname: Alfano
authors.forename: Domenico
authors.initials: D
authors.lastname: Salvatore
keywords: deiodinase; hypothyroidism; thyroid gland; thyroid hormones; thyroid neoplasms
references.citation: Paediatr Anaesth. 2001 Mar;11(2):228-32
references.pmid: 11240884
references.citation: Gastroenterology. 2012 Oct;143(4):1037-47
references.pmid: 22771508
references.citation: J Pediatr. 2001 Jun;138(6):932-5
references.pmid: 11391345
references.citation: J Clin Endocrinol Metab. 1991 Mar;72(3):523-9
references.pmid: 1997508
references.citation: J Endocrinol Invest. 2011 May;34(5):395-407
references.pmid: 21427525
references.citation: Endocrinology. 2009 Jan;150(1):540-5
references.pmid: 18787028
references.citation: Endocrinology. 1997 Jun;138(6):2559-68
references.pmid: 9165049
references.citation: Thyroid. 2005 Dec;15(12):1369-72
references.pmid: 16405410
references.citation: Proc Natl Acad Sci U S A. 2007 Sep 4;104(36):14466-71
references.pmid: 17720805
references.citation: Endocr Rev. 2002 Feb;23(1):38-89
references.pmid: 11844744
references.citation: Horm Res. 2005;63(2):86-9
references.pmid: 15711094
references.citation: J Pediatr Surg. 2006 Oct;41(10):1783-5
references.pmid: 17011291
references.citation: Gynecol Endocrinol. 2012 Dec;28(12):993-8
references.pmid: 22686167
references.citation: Hum Mol Genet. 2013 Feb 1;22(3):531-43
references.pmid: 23108156
references.citation: Pediatr Blood Cancer. 2007 Dec;49(7):1021-4
references.pmid: 16544297
references.citation: J Pediatr Surg. 2007 Jan;42(1):62-7; discussion 67-8
references.pmid: 17208542
references.citation: J Pediatr Endocrinol Metab. 2012;25(1-2):153-5
references.pmid: 22570966
references.citation: Thyroid. 1992 Fall;2(3):207-17
references.pmid: 1422233
references.citation: J Endocrinol. 2011 Jun;209(3):273-82
references.pmid: 21398344
references.citation: J Clin Endocrinol Metab. 2011 Oct;96(10):3087-94
references.pmid: 21816788
references.citation: Biochim Biophys Acta. 2013 Jul;1830(7):3900-7
references.pmid: 22704954
references.citation: Biochim Biophys Acta. 2013 Jul;1830(7):3946-55
references.pmid: 22498139
references.citation: Endocrinology. 2002 Nov;143(11):4483-6
references.pmid: 12399446
references.citation: Mol Cell Endocrinol. 2009 Dec 10;313(1-2):36-49
references.pmid: 19737599
references.citation: N Engl J Med. 2000 Jul 20;343(3):185-9
references.pmid: 10900278
references.citation: N Engl J Med. 1990 Jul 12;323(2):91-6
references.pmid: 2359428
references.citation: J Clin Endocrinol Metab. 1993 Dec;77(6):1719-22
references.pmid: 8263162
references.citation: Curr Opin Pediatr. 2008 Apr;20(2):213-5
references.pmid: 18332720
references.citation: Horm Res Paediatr. 2010;74(2):149-52
references.pmid: 20516650
references.citation: Endocrinology. 2003 Mar;144(3):937-46
references.pmid: 12586771
references.citation: Placenta. 2013 May;34(5):395-400
references.pmid: 23518454
references.citation: Ann Clin Lab Sci. 2007 Summer;37(3):280-4
references.pmid: 17709695
references.citation: Horm Res Paediatr. 2012;78(4):256-60
references.pmid: 22907522
references.citation: Endocrinology. 2005 Apr;146(4):1701-6
references.pmid: 15661862
references.citation: J Clin Endocrinol Metab. 2002 Oct;87(10):4457-61
references.pmid: 12364418
references.citation: J Clin Invest. 1995 Nov;96(5):2421-30
references.pmid: 7593630
references.citation: Endocr Rev. 2010 Apr;31(2):139-70
references.pmid: 20051527
references.citation: J Med Case Rep. 2013 Feb 18;7:48
references.pmid: 23418754
references.citation: Trends Endocrinol Metab. 2008 Mar;19(2):50-6
references.pmid: 18291666
references.citation: J Clin Endocrinol Metab. 2011 Jul;96(7):1966-70
references.pmid: 21508133
references.citation: J Pediatr Gastroenterol Nutr. 2012 May;54(5):692-5
references.pmid: 21716131
references.citation: Endocrinology. 2003 Oct;144(10):4253-61
references.pmid: 12959985
references.citation: J Clin Endocrinol Metab. 1978 Nov;47(5):1034-7
references.pmid: 263334
references.citation: J Clin Endocrinol Metab. 2003 Mar;88(3):1384-8
references.pmid: 12629133
references.citation: Endocrinology. 2006 Dec;147(12):5845-54
references.pmid: 16935842
references.citation: Ann Clin Biochem. 2011 Mar;48(Pt 2):186-9
references.pmid: 21382982
references.citation: Endocrinology. 2005 Dec;146(12):5128-34
references.pmid: 16150911
references.citation: J Clin Endocrinol Metab. 2005 Jul;90(7):4322-34
references.pmid: 15840737
references.citation: Pediatrics. 2003 Dec;112(6 Pt 1):1424-6
references.pmid: 14654623
references.citation: J Biol Chem. 2003 Jan 10;278(2):1206-11
references.pmid: 12419801
references.citation: Eur J Pediatr. 2010 Feb;169(2):215-21
references.pmid: 19548001
references.citation: Biochim Biophys Acta. 2013 Jul;1830(7):3937-45
references.pmid: 22634734
references.citation: J Pediatr Endocrinol Metab. 2008 Jul;21(7):701-3
references.pmid: 18780606
references.citation: Endocrinology. 2003 Dec;144(12):5411-21
references.pmid: 12960017