Division of Endocrinology & Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, 34th and Civic Center Boulevard, Philadelphia, PA 19104, USA.
Arch Dis Child Fetal Neonatal Ed. 2013 Jul;98(4):F351-4. doi: 10.1136/archdischild-2012-302546. Epub 2013 Feb 1.
Hypertrophic cardiomyopathy (HCM) is a well-recognised complication in infants of diabetic mothers and is attributed to a compensatory increase in fetal insulin secretion. Infants with congenital hyperinsulinism have excessive prenatal and postnatal insulin secretion due to defects in pathways of insulin secretion (most commonly the KATP channel). HCM has been reported in a few neonates with hyperinsulinism, but its extent and risk factors for its development have not been evaluated.
Retrospective chart review of infants, age <3 months, with congenital hyperinsulinism managed by Children's Hospital of Philadelphia over a 3.5-year period.
Gestational age, birth weight, hyperinsulinism form and treatments, echocardiogram results, cardiac/respiratory complications.
68 infants were included, 58 requiring pancreatectomy for diffuse (n=28) or focal (n=30) disease, 10 were diazoxide-sensitive. Twenty-five had echocardiograms performed. Ten had HCM, all of whom required pancreatectomy and eight of whom had confirmed ATP-sensitive potassium-hyperinsulinism. Subjects with HCM had younger gestational age 36(32, 38) than their surgical counterparts without HCM 38 (31.6, 43), p=0.02.
HCM appears common in infants with severe hyperinsulinism. Routine echocardiogram and EKG of at-risk newborns should be considered. Fetal hyperinsulinism is the likely mediating factor for HCM in HI infants.
肥厚型心肌病（HCM）是糖尿病母亲婴儿的一种公认并发症，归因于胎儿胰岛素分泌的代偿性增加。患有先天性高胰岛素血症的婴儿由于胰岛素分泌途径（最常见的是 KATP 通道）的缺陷而导致产前和产后胰岛素分泌过多。已有少数患有高胰岛素血症的新生儿报道有 HCM，但尚未评估其发病程度及其危险因素。
对在费城儿童医院接受治疗的年龄<3 个月的先天性高胰岛素血症婴儿进行回顾性图表审查，时间跨度为 3.5 年。
胎龄、出生体重、高胰岛素血症的形式和治疗方法、超声心动图结果、心脏/呼吸并发症。
共纳入 68 例婴儿，58 例因弥漫性（n=28）或局灶性（n=30）疾病需要行胰腺切除术，10 例对二氮嗪敏感。25 例行超声心动图检查。10 例患有 HCM，均需行胰腺切除术，其中 8 例被证实为 ATP 敏感性钾通道高胰岛素血症。患有 HCM 的患者的胎龄为 36（32，38），明显小于无 HCM 的手术对照组患者 38（31.6，43），p=0.02。
HCM 在严重高胰岛素血症的婴儿中似乎很常见。应考虑对高危新生儿进行常规超声心动图和心电图检查。胎儿高胰岛素血症可能是 HI 婴儿 HCM 的中介因素。
