青少年发病的成人型糖尿病诊治进展3

图片来源网络
6预后
所有的MODY类型中，MODY2是预后好的一种，MODY2患者的血糖不会明显地进行性恶化，尽管发病年 龄很轻，病程很长，也不会导致并发症的出现。其他MODY的预后除与其高血糖的程度及病程进展相关外， 还与MODY的突变基因在哪些脏器表达及影响其功能的严重程度相关，如MODY5，突变基因HNF-1B在肝脏、胰腺、肠道、子宫和肾脏中表达，MODY5患者可以有多囊肾、蛋白尿、终末期肾病，发生与高血糖不相关的进行性肾功能损害。大多数MODY糖尿程度轻，进展不快，并发症出现晚，预后相对较好。

综上，不同MODY的分子病理学、临床表现和治疗方法各有不同。对于近年来新发现的MODY类型，临床资料仍很局限。通过对MODY的遗传和分子生物学的深入研究，将更好地为患者提供个体化治疗，并对遗传咨询及患者家属风险评估发挥有益的指导作用。

参考文献

[1] Thanabalasingham G,Owen KR. Diagnosis and management of matu“ty onset diabetes of the young( MODY)[J].BMJ,2011,343:d6044.
[2] 向红丁．威廉姆斯内分泌学[M]．11版，北京：人民军医出版社，2011:1294 - 1296．

[3] American Diabetes Association. Diagnosis and classification of(liabetcs mellitus[J].Diabetes Care ,2011 ,34( Suppl l):S62 - 69.
[4] Thomas CC, Philipson LH.Update on diabetes classification[J] Med Clin North Am,2015 ,99(1):1-16.
[5] Bonnefond A,Philippe J,Durand E,et alt Whole-exome sequencing and high throughput gcnotyping identified KCNJ11 as the thirteenth MODY gene[J].PLoS One,2012,7 (6): e37423.
[6] Johansson BB,Torsvik J, BjOrkhaug L,et al.Diabetes and pancreatic exocrine dysfunction due to mutations in the carboxyl ester lipase genematurity onset diabetes of the young(CEL-MODY) :a prolein misfolding disease [J].J Biol Chen,2011,286(40):34593 - 34605.
[7] Raeder H,Mcallister FE,Tjora E,et al.Carboxyl-ester lipase maturityonset diabetes of the young is associated with devclopment of' pancrcatic cysts and upregulatcd MAPK signaling in secretin-stimulated duodenal fluid [ J] . Diabetes,2014 ,63 ( 1 ) :259 - 269.
[8] Bonnefond A,Lomberk C,Butlar N,et al. Disruption of a novel Kruppel like transc[x]ription factor p300-regulated pathway for insulin biosynthesis revealed by studies of the c. -331 INS mutation found in neonatal diabetes mellitus [ J] . J Biol Chem,2011 ,286 (32) :28414 - 28424.