Section of Pediatric Surgery, Children's Hospital, Helsinki University Central Hospital and University of Helsinki, Helsinki, Finland.
Horm Res Paediatr. 2013;79(4):227-35. doi: 10.1159/000350616. Epub 2013 Apr 26.
To assess bone health in pediatric intestinal failure (IF).
A population-based cohort of 41 IF patients (age 9.9 years) underwent evaluation of bone mineral density (BMD), bone biochemistry, nutritional status and growth. Eleven patients remained on parenteral nutrition (PN) after 69 months. Thirty had weaned off PN 9.0 years earlier (mean), i.e. after 30 months on PN.
The majority of patients had lumbar spine or femoral BMD Z-score ≤-1.0 (70%), vitamin D deficiency (serum 25-hydroxyvitamin-D, S-25-OHD, <50 nmol/l, 41%) or secondary hyperparathyroidism (plasma parathyroid hormone >47 ng/l, 44%), equally during and after weaning off PN. Hyperparathyroidism was absent when S-25-OHD was >80 nmol/l. Until puberty, height (-1.4 to -0.8, age 1-12) and weight Z-scores (-1.3 to -0.5, age 1-16) were below the normal mean (p < 0.05). Small bowel length associated with S-25-OHD levels (r = 0.489, p = 0.013). In a multivariate model, time after weaning off PN (β = -0.597, p = 0.001), duration of PN (β = -0.466, p = 0.006) and calcium intake (β = -0.331, p = 0.035) predicted decreased lumbar spine BMD.
In pediatric IF, vitamin D insufficiency, secondary hyperparathyroidism and decreased BMD are common. BMD, vitamin D, calcium and nutritional status should be closely monitored during and after weaning off PN to ensure sufficient vitamin D and mineral substitution for normal growth and bone mass attainment.
评估儿科肠衰竭（IF）患者的骨骼健康状况。
对 41 例 IF 患者（年龄 9.9 岁）进行了骨矿物质密度（BMD）、骨生化、营养状况和生长评估。11 例患者在 69 个月后仍接受肠外营养（PN）治疗。30 例患者在 PN 前 9.0 年（平均）即 30 个月时已停用 PN。
大多数患者的腰椎或股骨 BMD Z 评分≤-1.0（70%），维生素 D 缺乏（血清 25-羟维生素 D，S-25-OHD<50nmol/l，41%）或继发性甲状旁腺功能亢进症（血浆甲状旁腺激素>47ng/l，44%），在停用 PN 期间和之后均相等。当 S-25-OHD>80nmol/l 时，甲状旁腺功能亢进症不存在。直到青春期，身高（1-12 岁为-1.4 至-0.8，1-16 岁为-1.3 至-0.5）和体重 Z 评分（1-16 岁为-1.3 至-0.5）均低于正常平均值（p<0.05）。小肠长度与 S-25-OHD 水平相关（r=0.489，p=0.013）。在多变量模型中，停用 PN 后的时间（β=-0.597，p=0.001）、PN 持续时间（β=-0.466，p=0.006）和钙摄入量（β=-0.331，p=0.035）预测腰椎 BMD 降低。
在儿科 IF 中，维生素 D 不足、继发性甲状旁腺功能亢进症和 BMD 降低很常见。在停用 PN 期间和之后，应密切监测 BMD、维生素 D、钙和营养状况，以确保充足的维生素 D 和矿物质替代，以实现正常生长和获得骨量。
