Extreme insulin-resistance syndrome associated with alpha-1 antitrypsin deficiency

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dc.contributor.authorGama M.P.R.
dc.contributor.authorJonasson T.H.
dc.contributor.authorGama J.D.
dc.contributor.authorTeixeira De Freitas P.Z.L.
dc.contributor.authorOssowski A.C.
dc.contributor.authorPerraro R.C.
dc.contributor.authorDe Souza B.V.
dc.contributor.authorCustodio G.
dc.date.accessioned2024-03-13T01:31:43Z
dc.date.available2024-03-13T01:31:43Z
dc.date.issued2010
dc.description.abstractThe type B insulin-resistance syndrome is a rare autoimmune disease in which anti-insulin-receptor antibodies are formed. It generally affects young female patients with a history of other autoimmune diseases such as systemic lupus erythematosus. Difficult-to-control diabetes with alternating episodes of hyperglycemia and hypoglycemia suggest that the anti-insulin-receptor antibody is capable of both blocking insulin binding to its receptor and mimicking insulin action.The case of a man with preexisting diabetes and hypertension who developed liver cirrhosis is described. During hospitalization, the patient developed severe hyperglycemia, unresponsive to all available therapies, including up to 16,000 U/d of regular intravenous insulin without adequate control. A liver biopsy confirmed alpha-1-antitrypsin deficiency. Type B insulin-resistance was diagnosed concurrently. Treatment with cyclophosphamide was initiated, bringing about the characteristic presentation of the disease, with alternating episodes of hyperglycemia and hypoglycemia. When intravenous insulinotherapy was discontinued, the patient died of diabetic ketoacidosis refractory to emergency measures. This is the first described case correlating type B insulin-resistance syndrome and alpha-1-antitrypsin deficiency. © 2010 by Lippincott Williams & Wilkins.
dc.description.firstpage137
dc.description.issuenumber3
dc.description.lastpage140
dc.description.volume20
dc.identifier.doi10.1097/TEN.0b013e3181dfdcc2
dc.identifier.issn1051-2144
dc.identifier.urihttps://dspace.mackenzie.br/handle/10899/37195
dc.relation.ispartofEndocrinologist
dc.rightsAcesso Restrito
dc.subject.otherlanguageAlpha-1-antitrypsin syndrome
dc.subject.otherlanguageInsulin receptor antibodies
dc.subject.otherlanguageType B insulin resistance
dc.titleExtreme insulin-resistance syndrome associated with alpha-1 antitrypsin deficiency
dc.typeArtigo de revisão
local.scopus.citations1
local.scopus.eid2-s2.0-77952395211
local.scopus.updated2024-05-01
local.scopus.urlhttps://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=77952395211&origin=inward
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