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首页> 外文期刊>Transplant international : >Maturity onset diabetes of the young: Clinical characteristics and outcome after kidney and pancreas transplantation in MODY3 and RCAD patients: A single center experience
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Maturity onset diabetes of the young: Clinical characteristics and outcome after kidney and pancreas transplantation in MODY3 and RCAD patients: A single center experience

机译：年轻人的成熟期糖尿病：MODY3和RCAD患者肾脏和胰腺移植后的临床特征和预后：单中心经验

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The diabetes and renal phenotype of patients with maturity-onset diabetes of the young (MODY) on a transplantation waiting list is not known; neither is their outcome after pancreas (PT) and/or kidney transplantation (KT). Between 2002 and 2009, we screened 50 of 150 patients referred for kidney and pancreas transplantation to the Kremlin-Bicêtre center for HNF1B and HNF1A mutations if one or more of the following criteria was present (i) an atypical history of diabetes (ii) diabetes with at least one affected parent or two affected relatives, (iii) an absence of auto-antibodies at diagnosis (iv) a persistent secretion of fasting C peptide (v) a personal or a family history of renal cysts or dysplasia. Their phenotype and their outcome were analyzed. Four HNF1A (MODY3) and eight HNF1B mutations [renal cysts and diabetes (RCAD)] were identified. All MODY3 patients had diabetic nephropathy, but only 50% of RCAD patients. Four patients underwent a kidney and pancreas transplantation and two a kidney transplant alone. After 4.1 ± 1.1 years of follow-up, 83% of patients still have a functioning kidney and 75% a functioning pancreas. PT can be proposed with good results for MODY3 and RCAD patients.
机译：尚不知道移植等待名单上的年轻成年糖尿病患者（MODY）的糖尿病和肾表型;胰腺（PT）和/或肾脏移植（KT）后的结局也不是。在2002年至2009年之间，我们筛选了150例接受肾脏和胰腺移植的患者中的50例，如果存在以下一项或多项标准，则将其移植到克里姆林宫-比克特中心的HNF1B和HNF1A突变（i）非典型糖尿病史（ii）糖尿病至少有一名受影响的父母或两名受影响的亲戚，（iii）诊断时不存在自身抗体（iv）禁食C肽的持续分泌（v）肾囊肿或不典型增生的个人或家族病史。他们的表型和结果进行了分析。确定了四个HNF1A（MODY3）和八个HNF1B突变[肾囊肿和糖尿病（RCAD）]。所有MODY3患者均患有糖尿病肾病，但只有50％的RCAD患者。四名患者接受了肾脏和胰腺移植，另外两名接受了肾脏移植。经过4.1±1.1年的随访，仍有83％的患者的肾脏功能正常，而75％的胰腺功能正常。可以为MODY3和RCAD患者建议PT，效果良好。

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