Department of Pediatric Gastroenterology, Hepatology and Nutrition, National Reference Center for Rare Digestive Diseases, Necker Enfants Malades University Hospital, 75015, Paris, France; Université de Paris, Faculty of Medicine, 75006, Paris, France.
Department of Pediatric Gastroenterology, Hepatology and Nutrition, National Reference Center for Rare Digestive Diseases, Necker Enfants Malades University Hospital, 75015, Paris, France.
Clin Nutr. 2021 Apr;40(4):1974-1982. doi: 10.1016/j.clnu.2020.09.014. Epub 2020 Sep 15.
BACKGROUND & AIMS: Children on long-term home parenteral nutrition (HPN) are at increased risk of suboptimal growth and metabolic bone disease (MBD) i.e. decreased bone mineral density (BMD). The aims of this cross-sectional study were to assess growth and bone health in children on long term HPN and to identify risk factors for MBD.
Children above the age of 5 years, stable on HPN for more than 2 years were included. Medical files were reviewed retrospectively and included demographics, gestational age, birth weight and height, indication for PN, age at PN start, duration of PN, number of weekly PN infusions, weight-for-age and height-for-age (SD), body mass index (BMI, kg/m) as well as blood and urine analyses at the time of Dual X-ray absorptiometry (DXA) measurements. All BMD values were adjusted to statural age which corresponds to the 50th percentile of height. Growth failure (height-for-age ≤ -2SD) and MBD (at least one BMD measurement ≤ -2SD) were analyzed according to the indication of PN, duration of PN and PN dependency index (PNDI) by comparing means and performing logistic regression analysis. PNDI is the ratio of non-protein energy intake in HPN to resting energy expenditure using Schofield equations.
Forty children were assessed at 12.4 ± 4.5 years of age. Mean age at PN start was 1.1 ± 3.6 y (median 0.5). The indications for PN were short bowel syndrome (SBS, n = 21), chronic intestinal pseudo-obstruction syndrome (CIPOS, n = 10) and congenital enteropathies (CE, n = 9). The mean number of PN perfusions was 6 ± 1/week. PNDI was 110 ± 30%. The mean serum level of 25-OHD3 was suboptimal at 26.5 ± 9.1 ng/mL (66.2 ± 22.8 nmol/L). The mean concentrations of calcium, phosphorus, and parathyroid hormone (PTH) were in the normal ranges. Eight children (20%) had PTH levels above normal with low 25-OHD3 levels. The mean weight-for-age and height-for-age Z-scores SDS were 0.4 ± 0.9 and -0.5 ± 1.1 respectively. The actual height was lower than genetic target height (p < 0.001). The BMD Z-scores, adjusted to the 50th percentile of height, of the spine, the left femur and the whole body were: -1.1 ± 1.7, -1.2 ± 1.5 and -1.5 ± 1.8 SDS respectively. Children with CE had significantly lower BMD values than those with SBS and CIPOS (p = 0.01). Only two children had bone fractures after a mild trauma (5%).
All children on long-term PN, are at risk of low BMD. High dependency on PN (PNDI>120%) and very long-term PN (>10 years) do not appear to increase the risk of growth failure nor MBD. PN-related bone fractures were rare. Close follow-up remains mandatory.
长期接受家庭肠外营养(HPN)的儿童存在生长不良和代谢性骨病(MBD)的风险增加,即骨矿物质密度(BMD)降低。本横断面研究的目的是评估长期接受 HPN 的儿童的生长和骨骼健康状况,并确定 MBD 的危险因素。
纳入年龄超过 5 岁,HPN 稳定 2 年以上的儿童。回顾性审查病历,包括人口统计学资料、胎龄、出生体重和身高、PN 适应证、PN 开始年龄、PN 持续时间、每周 PN 输注次数、体重-年龄和身高-年龄(SD)、体质指数(BMI,kg/m)以及双能 X 线吸收法(DXA)测量时的血液和尿液分析。所有 BMD 值均调整为身高年龄,对应身高的第 50 个百分位。根据 PN 的适应证、PN 持续时间和 PN 依赖指数(PNDI),通过比较均值和进行逻辑回归分析,分析生长不良(身高年龄≤-2SD)和 MBD(至少有一个 BMD 测量值≤-2SD)。PNDI 是 Schofield 方程中 HPN 中非蛋白能量摄入与静息能量消耗的比值。
40 名儿童在 12.4±4.5 岁时进行评估。PN 开始的平均年龄为 1.1±3.6 岁(中位数为 0.5 岁)。PN 的适应证为短肠综合征(SBS,n=21)、慢性肠假性梗阻综合征(CIPOS,n=10)和先天性肠病(CE,n=9)。平均 PN 输注次数为 6±1/周。PNDI 为 110±30%。血清 25-OHD3 水平平均为 26.5±9.1ng/mL(66.2±22.8nmol/L),低于最佳水平。钙、磷和甲状旁腺激素(PTH)的平均浓度在正常范围内。8 名儿童(20%)的 PTH 水平升高,同时伴有低 25-OHD3 水平。体重年龄和身高年龄 Z 评分 SDS 分别为 0.4±0.9 和-0.5±1.1。实际身高低于遗传靶身高(p<0.001)。脊柱、左侧股骨和全身 BMD Z 评分分别为-1.1±1.7、-1.2±1.5 和-1.5±1.8 SDS。CE 患儿的 BMD 值明显低于 SBS 和 CIPOS 患儿(p=0.01)。仅有 2 名儿童在轻微外伤后发生骨折(5%)。
所有长期接受 PN 的儿童均存在低 BMD 风险。对 PN 的高度依赖(PNDI>120%)和长期接受 PN(>10 年)似乎不会增加生长不良或 MBD 的风险。PN 相关的骨折很少见。仍需密切随访。