目的通过研究腹腔镜胰腺部分切除术治疗婴儿持续性高胰岛素血症性低血糖（per-sistent hyperinsulinemic hypoglycemia of infancy，PHHI）的技术和效果，探讨该技术的可行性和有效性。方法分析2008年9月至2011年4月4例PHHI患儿接受腹...目的通过研究腹腔镜胰腺部分切除术治疗婴儿持续性高胰岛素血症性低血糖（per-sistent hyperinsulinemic hypoglycemia of infancy，PHHI）的技术和效果，探讨该技术的可行性和有效性。方法分析2008年9月至2011年4月4例PHHI患儿接受腹腔镜胰腺部分切除术治疗的临床资料。术后对所有患儿进行跟踪随访，内容包括术后低血糖症状缓解与否，定期复查血胰岛素、血糖浓度等。结果手术时间170～190min，术中出血量较少，无输血者。术后腹腔引流时间为2～7d，术后住院时问为12-24d。术后空腹血糖浓度较术前升高（术前：0．4～8．0mmol／L，平均2．8retool／L；术后：2．6～15．2mmol／L，平均7．5mmol／L），术后空腹血胰岛素浓度较术前降低（术前：52．9～102．3mU／L，平均77．4mU／L；术后：3．7～13．3mU／L，平均7．2mU／L）。迄今随访2～32个月，此间行胰腺大部切除的3例血糖浓度和血胰岛素浓度恢复正常，未出现复发。行腹腔镜局灶性病变切除1例术后7个月，因低血糖症状复发，手术探查发现胰头结节，再次行胰腺结节切除术，术后至今已25个月，恢复良好，未出现低血糖症状。结论腹腔镜胰腺部分切除术治疗PHHI是安全、有效的。展开更多
Objectives:To characterize the clinical features and insulin regulation in in fants with hypoglycemia due to prolonged neonatal hyperinsulinism.Study design:Data were collected on 26 infants with hypoglycemia due to n...Objectives:To characterize the clinical features and insulin regulation in in fants with hypoglycemia due to prolonged neonatal hyperinsulinism.Study design:Data were collected on 26 infants with hypoglycemia due to neonatal hyperinsuli nism that later resolved.Acute insulin response(AIR)tests to calcium,leucine,glucose,and tolbutamide were performed in 11 neonates.Results were compared to children with genetic hyperinsulinism due to mutations of the adenosine triph osphate-dependent potassium(KATP)channel and glutamate dehydrogenase(GDH).R esults:Among the 26 neonates,there were significantly more males,small-for-gestational-age infants,and cesarean deliveries.Only 5 of the 26 had no ident ifiable risk factor.Hyperinsulinism was diagnosed at a median age of 13 days(r ange,2 to 180 days)and resolved by a median age of 181 days(range,18 to 403 d ays).Diazoxide was effective in 19 of the 21 neonates treated.In the 11 neonat es tested,the AIRs to calcium,leucine,glucose,and tolbutamide resembled thos e in normal controls and differed from genetic hyperinsulinism due to KATP chann el and GDH mutations.Conclusions:We define a syndrome of prolonged neonatal hy perinsulinism that is responsive to diazoxide,persists for several months,and resolves spontaneously.AIR tests suggest that both the KATP channel and GDH hav e normal function.展开更多
文摘目的通过研究腹腔镜胰腺部分切除术治疗婴儿持续性高胰岛素血症性低血糖（per-sistent hyperinsulinemic hypoglycemia of infancy，PHHI）的技术和效果，探讨该技术的可行性和有效性。方法分析2008年9月至2011年4月4例PHHI患儿接受腹腔镜胰腺部分切除术治疗的临床资料。术后对所有患儿进行跟踪随访，内容包括术后低血糖症状缓解与否，定期复查血胰岛素、血糖浓度等。结果手术时间170～190min，术中出血量较少，无输血者。术后腹腔引流时间为2～7d，术后住院时问为12-24d。术后空腹血糖浓度较术前升高（术前：0．4～8．0mmol／L，平均2．8retool／L；术后：2．6～15．2mmol／L，平均7．5mmol／L），术后空腹血胰岛素浓度较术前降低（术前：52．9～102．3mU／L，平均77．4mU／L；术后：3．7～13．3mU／L，平均7．2mU／L）。迄今随访2～32个月，此间行胰腺大部切除的3例血糖浓度和血胰岛素浓度恢复正常，未出现复发。行腹腔镜局灶性病变切除1例术后7个月，因低血糖症状复发，手术探查发现胰头结节，再次行胰腺结节切除术，术后至今已25个月，恢复良好，未出现低血糖症状。结论腹腔镜胰腺部分切除术治疗PHHI是安全、有效的。
文摘Objectives:To characterize the clinical features and insulin regulation in in fants with hypoglycemia due to prolonged neonatal hyperinsulinism.Study design:Data were collected on 26 infants with hypoglycemia due to neonatal hyperinsuli nism that later resolved.Acute insulin response(AIR)tests to calcium,leucine,glucose,and tolbutamide were performed in 11 neonates.Results were compared to children with genetic hyperinsulinism due to mutations of the adenosine triph osphate-dependent potassium(KATP)channel and glutamate dehydrogenase(GDH).R esults:Among the 26 neonates,there were significantly more males,small-for-gestational-age infants,and cesarean deliveries.Only 5 of the 26 had no ident ifiable risk factor.Hyperinsulinism was diagnosed at a median age of 13 days(r ange,2 to 180 days)and resolved by a median age of 181 days(range,18 to 403 d ays).Diazoxide was effective in 19 of the 21 neonates treated.In the 11 neonat es tested,the AIRs to calcium,leucine,glucose,and tolbutamide resembled thos e in normal controls and differed from genetic hyperinsulinism due to KATP chann el and GDH mutations.Conclusions:We define a syndrome of prolonged neonatal hy perinsulinism that is responsive to diazoxide,persists for several months,and resolves spontaneously.AIR tests suggest that both the KATP channel and GDH hav e normal function.
