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先天性高胰岛素血症 被引量:18

Congenital hyperinsulinism: a difficult and complicated case study
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摘要 报道1例先天性持续性高胰岛素性低血糖症患儿的临床诊治情况。患儿,男,孕36周,因“胎儿宫内窘迫”剖宫产出生,出生体重4200g,生后即表现为反复低血糖,加用糖皮质激素治疗后患儿的血糖仍一直波动于1.2~2.8mmol/L之间,需静脉输注10%葡萄糖溶液,糖速为10~17mg/(kg·min),才能维持血糖的稳定。生后30d测定血胰岛素浓度为24.13U/L,同份标本的血糖为1.5mmol/L,血浆胰岛素(U/L)/血浆葡萄糖(mg/dL)比值为0.89,表明体内存在器质性胰岛素不适当分泌过多,诊断为先天性高胰岛素血症。 This paper reported a case of congenital hyperinsulinism and reviewed the relevant literatures regarding to the etiology, pathogenesis, clinical and pathological features, diagnosis and treatment of this disorder. The baby (male) , with gestational age of 36 weeks and birth weight 4 200 g, was delivered by caesarean section. It presented with hypoglycemia immediately after birth (0.8 mmol/L). Through the course of the disease, the baby's blood sugar manifested with 1.2-2.8 mmol/L although glucocorticoid was administered. 10% glucose solutions were intravenously infused at a speed of 10-17 mg/( kg · min) for this patient to retain a stable blood sugar level. The plasma insulin level was 24.13 U/L and blood sugar level was 1.5 mmol/L on day 30 of his life. The ratio of plasma insulin (U/L) and plasma glucose (mg/dL) was 0.89. These results suggest an inappropriate insulin secretion resulting in persistent hypoglycemia in this baby and so it was definitely diagnosed with congenital hyperinsulinism.
出处 《中国当代儿科杂志》 CAS CSCD 2006年第5期391-394,共4页 Chinese Journal of Contemporary Pediatrics
关键词 先天性高胰岛素血症 低血糖 持续性 婴儿 新生 Congenital hypefinsulinism Hypoglycemia Persistence Infant, newborn
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参考文献11

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二级参考文献8

  • 1王增贵 肖侠明 吴炎兴 等.婴儿高胰岛素血症致低血糖惊厥1例[J].中华儿科杂志,1991,29(4):255-255.
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