Soliman A T, Alsalmi I, Darwish A, Asfour M G
Department of Paediatrics, Royal Hospital, Muscat, Oman.
Arch Dis Child. 1996 May;74(5):379-85. doi: 10.1136/adc.74.5.379.
Seven children, with a mean (SD) age of 4.6 (2.1) years, who as infants (21 (7.5) days) underwent near total (95-98%) pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) were studied. At birth all the infants were macrosomic. Four infants had been born after a difficult labour, of whom three had moderate birth asphyxia and respiratory distress. All had normal thyroid function. After surgery transient hyperglycaemia was manifest in six of the children and required insulin treatment for 5.8 (3.8) weeks, and transient hypoglycaemia was encountered in one child and responded well to increased carbohydrate intake and diazoxide for three weeks. Six of the children rapidly crossed down their length and weight centiles during the first year after surgery. At the end of the first year these children were at or below the 5th centile of height and weight for their age and gender. After a period of 4.6 (2.1) years, their mean (SD) height score was -2.57 (0.5), growth velocity 3.9 (0.75) cm/year, and growth velocity SD score -2.1 (0.55)l these were significantly low and denoted significant growth retardation. The growth hormone peak responses to provocation with clonidine were normal (13.5 (2.8) micrograms/l). However, the circulating insulin-like growth factor-I (IGF-I) concentrations were significantly decreased (79 (34) ng/ml). Three of the children developed diabetes at two and a half, five, and seven years after surgery, two others had impaired oral glucose tolerance and six out of the seven children had an impaired C peptide response to glucagon. Defective insulin secretion in these children might directly inhibit IGF-I synthesis in the liver. The body mass index of the pancreatectomised children was 14.9 (0.5) and was normal for age and gender; they had a normal 72 hour faecal fat content and normal serum albumin concentration. These data indicated grossly adequate exocrine pancreatic function. It appears that children requiring near total pancreatectomy for PHHI have normal developmental milestones but defective linear growth with impaired insulin secretion and low IGF-I production despite normal growth hormone response to provocation.
对7名平均（标准差）年龄为4.6（2.1）岁的儿童进行了研究，这些儿童在婴儿期（21（7.5）天）因持续性婴儿高胰岛素血症性低血糖症（PHHI）接受了近乎全胰腺切除术（95%-98%）。所有婴儿出生时均为巨大儿。4名婴儿分娩困难，其中3名有中度出生窒息和呼吸窘迫。所有儿童甲状腺功能均正常。术后6名儿童出现短暂性高血糖，需要胰岛素治疗5.8（3.8）周，1名儿童出现短暂性低血糖，增加碳水化合物摄入量并使用二氮嗪治疗3周后反应良好。6名儿童在术后第一年身高和体重百分位数迅速下降。在第一年末，这些儿童的身高和体重处于或低于其年龄和性别的第5百分位数。经过4.6（2.1）年，他们的平均（标准差）身高评分是-2.57（0.5），生长速度为3.9（0.75）厘米/年，生长速度标准差评分是-2.1（0.55），这些数值显著偏低，表明存在显著的生长发育迟缓。可乐定激发试验后生长激素峰值反应正常（13.5（2.8）微克/升）。然而，循环胰岛素样生长因子-I（IGF-I）浓度显著降低（79（34）纳克/毫升）。3名儿童在术后2.5年、5年和7年患糖尿病，另外2名儿童口服葡萄糖耐量受损，7名儿童中有6名对胰高血糖素的C肽反应受损。这些儿童胰岛素分泌缺陷可能直接抑制肝脏中IGF-I的合成。接受胰腺切除术的儿童体重指数为14.9（0.5），按年龄和性别属于正常；他们72小时粪便脂肪含量正常，血清白蛋白浓度正常。这些数据表明胰腺外分泌功能总体充足。似乎因PHHI需要近乎全胰腺切除术的儿童发育里程碑正常，但线性生长存在缺陷，胰岛素分泌受损，尽管生长激素对激发试验反应正常，但IGF-I产生量较低。
