Extreme insulin-resistance syndrome associated with alpha-1 antitrypsin deficiency
Tipo
Artigo de revisão
Data de publicação
2010
Periódico
Endocrinologist
Citações (Scopus)
1
Autores
Gama M.P.R.
Jonasson T.H.
Gama J.D.
Teixeira De Freitas P.Z.L.
Ossowski A.C.
Perraro R.C.
De Souza B.V.
Custodio G.
Jonasson T.H.
Gama J.D.
Teixeira De Freitas P.Z.L.
Ossowski A.C.
Perraro R.C.
De Souza B.V.
Custodio G.
Orientador
Título da Revista
ISSN da Revista
Título de Volume
Membros da banca
Programa
Resumo
The 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.