A patient with type B insulin resistance syndrome, responsive to immune therapy
A patient with type B insulin resistance syndrome, responsive to immune therapy
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DOI：
10.1038/ncpendmet0693
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发表时间：
2007-12-01
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影响因子：
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通讯作者：
Inzucchi, Silvio E.
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文献类型：
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作者：
Page, Kathleen A.;Dejardin, Stephanie;Inzucchi, Silvio E.
背景 一名 55 岁女性患有白癜风、甲状腺功能减退症、间质性肺病和糖尿病，在因呼吸衰竭入院期间出现严重的胰岛素抵抗。住院前，她的 HbA(1c) 水平为 8.1%，每天注射 100 U 的胰岛素。她的间质性肺病曾接受糖皮质激素治疗，但停药后，她的胰岛素需求急剧增加。尽管静脉注射胰岛素剂量高达 30,000 U/天，她仍然处于高血糖状态（血糖水平为 16.7-27.8 mmol/l）。检查 患者的血清肌酐水平为 301 μmol/l，肝功能检查正常。白细胞计数轻度升高。患者被诊断为铜绿假单胞菌引起的肺炎。当患者的血糖水平为22.5 mmol/l时，血浆C肽水平为0.9 nmol/l，血清胰岛素水平为294 pmol/l。当时，患者每天静脉注射门冬胰岛素 2,600 U。未检测到抗胰岛素和抗胰岛细胞抗体，但发现了抗胰岛素受体抗体。诊断 B 型胰岛素抵抗综合征。治疗 患者的胰岛素抵抗对糖皮质激素和血浆置换有反应。患者接受泼尼松（60 毫克/天）治疗后，她的胰岛素需求量在 1 周内下降至入院前剂量。当随后停用类固醇时，血糖控制再次恶化。血浆置换术开始实施，导致胰岛素需求急剧下降。维持剂量为 10 mg 强的松/天，平均每天两次 60 U 的 Isophan 胰岛素后，血糖控制得到改善（HbA(1C) 5.8%）。
Background A 55-year-old woman with vitiligo, hypothyroidism, interstitial lung disease and diabetes mellitus developed severe insulin resistance during a hospital admission for respiratory failure. Before hospitalization, her HbA(1c) level was 8.1% on similar to 100 U/day of insulin. Her interstitial lung disease had been treated with glucocorticoids, but after their withdrawal her insulin requirements had increased dramatically. She remained hyperglycemic (blood glucose levels 16.7-27.8 mmol/l), despite intravenous insulin at doses as high as 30,000 U/day.Investigations The patient's serum creatinine level was 301 mu mol/l and her liver function tests were normal. A mildly elevated white cell count was present. The patient was diagnosed with pneumonia due to Pseudomonas aeruginosa. When the patient's plasma glucose level was 22.5 mmol/l, her plasma C-peptide level was 0.9 nmol/l and her serum insulin level was 294 pmol/l. At that time the patient was on 2,600 U/day of intravenous insulin aspart. Anti-insulin and anti-islet-cell antibodies were not detected, but anti-insulin-receptor antibodies were found.Diagnosis Type B insulin resistance syndrome.Management The patient's insulin resistance responded to glucocorticoids and plasmapheresis. After the patient was treated with prednisone (60 mg/day), her insulin requirements decreased within 1 week to pre-admission doses. When steroids were subsequently discontinued, glycemic control deteriorated once again. Plasmapheresis was initiated, inducing a striking acute decline in insulin needs. On a maintenance dose of 10 mg prednisone/day, glucose control improved (HbA(1C) 5.8%) with an average of 60 U of isophane insulin twice daily.