Hussain K, Thornton P S, Otonkoski T, Aynsley-Green A
The London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children and the Institute of Child Health, University College London, UK.
J Pediatr Endocrinol Metab. 2004 Feb;17(2):203-9. doi: 10.1515/jpem.2004.17.2.203.
Transient hyperinsulinism (HI) occurs in infants born to diabetic mothers, in infants experiencing perinatal asphyxia and in infants with intrauterine growth retardation. The precise mechanism of transient HI in these different aetiologies is not fully understood. Lactic acidosis is commonly seen in neonates as a secondary phenomenon due to hypoxia, hypovolaemia, anaemia and infection. The combination of transient HI and lactic acidosis is rare. We present the clinical and biochemical features of five infants presenting with transient HI associated with hyperlactataemia in the absence of markers of perinatal stress. This combination lasted for 3-4 weeks with complete resolution except in one patient in whom the hyperinsulinism lasted until 6 months before resolution. The precise mechanism of this association is not clear but may be related either to immaturity of the pyruvate dehydrogenase complex or to the accumulation of abnormal intramitochondrial intermediary metabolites. Infants presenting with HI should have a free flowing blood sample drawn for the measurement of plasma lactate levels.
短暂性高胰岛素血症（HI）发生于糖尿病母亲所生的婴儿、经历围产期窒息的婴儿以及宫内生长受限的婴儿。这些不同病因导致的短暂性HI的确切机制尚未完全明确。乳酸酸中毒在新生儿中常见，是由缺氧、低血容量、贫血和感染引起的继发现象。短暂性HI与乳酸酸中毒同时出现的情况较为罕见。我们报告了5例婴儿的临床和生化特征，这些婴儿出现短暂性HI并伴有高乳酸血症，且无围产期应激标志物。这种情况持续了3 - 4周，除1例患者高胰岛素血症持续至6个月才消退外，其余均完全缓解。这种关联的确切机制尚不清楚，但可能与丙酮酸脱氢酶复合体不成熟或线粒体内异常中间代谢产物的积累有关。出现HI的婴儿应采集自由流动的血样以测定血浆乳酸水平。
