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醋酸兰瑞肽(善龙)治疗先天性高胰岛素血症。

Treatment of congenital hyperinsulinism with lanreotide acetate (Somatuline Autogel).

机构信息

Pediatric Endocrinology and Diabetes Unit, The Edmond and Lily Safra Children's Hospital, Chaim Sheba Medical Center, Tel-Hashomer (affiliated with The Sackler School of Medicine, Tel-Aviv University), 52621 Israel.

出版信息

J Clin Endocrinol Metab. 2011 Aug;96(8):2312-7. doi: 10.1210/jc.2011-0605. Epub 2011 Jun 22.

Abstract

CONTEXT

Congenital hyperinsulinism (CH) may be treated conservatively in many children with octreotide given by multiple sc injections or via an insulin pump.

OBJECTIVE

We describe two children treated with a once-monthly injection of a long-acting somatostatin analog.

PATIENTS AND METHODS

Both patients presented with hypoglycemia 30 min after birth and were subsequently diagnosed with CH. Patients were initially treated with diazoxide, hydrochlorothiazide, frequent feedings, and octreotide via an insulin pump. With this therapy, they were normoglycemic with a good growth rate, normal weight gain, and excellent neurodevelopment. Treatment with the long-acting somatostatin analog lanreotide acetate (Somatuline Autogel), administered by deep sc injection of 30 mg once a month, was started at the ages of 4½ and 4 yr, respectively. Octreotide infusion was gradually weaned over 1 month. Continuous glucose monitoring after discontinuation of pump therapy showed normoglycemia. The first patient has now been treated with the lanreotide acetate for over 5 yr, and the second for 3 yr. Treatment is well-tolerated, and both the patients and their parents are satisfied with the transition from pump therapy to once-a-month injection and prefer it to pump therapy.

CONCLUSION

Lanreotide acetate may be a safe and effective alternative to octreotide pump therapy in patients with CH, offering an improved quality of life. Longer follow-up of a larger patient group is needed.

摘要

背景

许多患有先天性高胰岛素血症(CH)的儿童可通过多次皮下注射奥曲肽或胰岛素泵进行保守治疗。

目的

我们描述了两名接受长效生长抑素类似物每月一次注射治疗的儿童。

患者和方法

两名患者均在出生后 30 分钟出现低血糖,并随后被诊断为 CH。患者最初接受二氮嗪、氢氯噻嗪、频繁喂养和胰岛素泵输注奥曲肽治疗。采用这种治疗方法,他们血糖正常,生长速度良好,体重正常增加,神经发育良好。在 4 岁半和 4 岁时,分别开始使用长效生长抑素类似物兰瑞肽醋酸酯(Somatuline Autogel)经深部皮下注射 30 mg,每月一次进行治疗。奥曲肽输注逐渐在 1 个月内逐渐减少。停止泵治疗后进行连续血糖监测显示血糖正常。第一个患者现在已经接受兰瑞肽醋酸酯治疗超过 5 年,第二个患者接受治疗 3 年。治疗耐受性良好,患者及其父母均对从泵治疗转为每月一次注射治疗感到满意,并倾向于选择这种治疗方式。

结论

兰瑞肽醋酸酯可能是 CH 患者奥曲肽泵治疗的一种安全有效的替代方法,可提高生活质量。需要对更大的患者群体进行更长时间的随访。

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