Agladıoglu Sebahat Yılmaz, Cetınkaya Semra, Savas Erdeve Senay, Onder Asan, Kendırcı Havva Nur Peltek, Bas Veysel Nijat, Aycan Zehra
J Pediatr Endocrinol Metab. 2013;26(9-10):955-8. doi: 10.1515/jpem-2012-0411.
There are different opinions concerning changes in glucose metabolism in patients with Laron syndrome. In this paper we discuss the treatment results of our patient with Laron syndrome who developed diabetes during late adolescence. A 19-year-old boy with Laron syndrome was referred to our clinic for follow-up. He had been diagnosed with Laron syndrome (LS) at 4 years old and rIGF-1 therapy was initiated. After 4 months the treatment was discontinued. At the age of 17, rIGF-1 therapy was restarted. A height gain of 8.8 cm. was observed during the 2-year treatment period. He was admitted to our hospital at the age of 19 years following discontinuation of the therapy. At that time, his height was 142 cm, and weight for height was 136%. His blood glucose was 85 mg/dL (4.72 mmol/L), insulin was 26.39 pmol/L, and HbA1c was 5.4%. At the age of 20, when he has not been receiving IGF-1 therapy for 1 year, his weight for height was 143 cm. Laboratory evaluation revealed that fasting blood glucose was 176 mg/dL (9.77 mmol/L), fasting insulin was 29.86 pmol/L, and HbA1c was 7.5%. Primary insulin therapy was then initiated. His parents both had a diagnosis of type 2 diabetes. Insulin therapy was switched to oral antidiabetic (OAD) therapy at the end of the second year because of a normal C-peptide level of 0.8 nmol/L under insulin therapy. After 6 months of OAD, HbA1c was 5.7%. The treatment was then switched to IGF-1 therapy, but his blood glucose profile was impaired and OAD therapy was restarted. In conclusion, we observed that genetic susceptibility and abdominal obesity caused type 2 diabetes in this patient. We believe that oral antidiabetic agents and life-style changes may be the appropriate approach when diabetes is developed in LS patients.
关于拉龙综合征患者葡萄糖代谢的变化存在不同观点。在本文中,我们讨论了一名在青春期末期患糖尿病的拉龙综合征患者的治疗结果。一名19岁的拉龙综合征男孩被转诊至我们诊所进行随访。他4岁时被诊断为拉龙综合征(LS),并开始接受重组胰岛素样生长因子-1(rIGF-1)治疗。4个月后治疗中断。17岁时,rIGF-1治疗重新开始。在为期2年的治疗期间,观察到身高增长了8.8厘米。19岁时,在治疗中断后他入住我院。当时,他的身高为142厘米,身高体重比为136%。他的血糖为85毫克/分升(4.72毫摩尔/升),胰岛素为26.39皮摩尔/升,糖化血红蛋白(HbA1c)为5.4%。20岁时,在未接受IGF-1治疗1年后,他的身高体重比为143厘米。实验室评估显示空腹血糖为176毫克/分升(9.77毫摩尔/升),空腹胰岛素为29.86皮摩尔/升,HbA1c为7.5%。随后开始进行初始胰岛素治疗。他的父母均被诊断为2型糖尿病。由于胰岛素治疗期间C肽水平正常,为0.8纳摩尔/升,在第二年年底胰岛素治疗改为口服抗糖尿病(OAD)治疗。OAD治疗6个月后,HbA1c为5.7%。然后治疗改为IGF-1治疗,但他的血糖情况受损,于是重新开始OAD治疗。总之,我们观察到该患者因遗传易感性和腹部肥胖导致2型糖尿病。我们认为,当拉龙综合征患者患糖尿病时,口服抗糖尿病药物和生活方式改变可能是合适的治疗方法。