Allen J R, Humphries I R, Waters D L, Roberts D C, Lipson A H, Howman-Giles R G, Gaskin K J
James Fairfax Institute of Paediatric Clinical Nutrition, Children's Hospital, Camperdown, New South Wales, Australia.
Am J Clin Nutr. 1994 Feb;59(2):419-22. doi: 10.1093/ajcn/59.2.419.
Previous studies have suggested that children with phenylketonuria (PKU) have a reduction in bone mineralization compared with control subjects. To investigate this, bone mineral density (BMD) of the total body (TBMD) was measured in 32 prepubertal children with PKU and in 95 age-matched control subjects. Spine bone mineral density (SBMD) was also recorded in a subset, 24 with PKU and 55 control subjects. The effect of dietary intake on bone mass was assessed in 30 of the children with PKU and in 12 control subjects. In the children with PKU, TBMD and SBMD were significantly lower than in the control subjects after adjustment for height and weight (P = 0.03 and P = 0.003, respectively). The children with PKU had a higher intake of calcium (P < 0.0001), phosphorus (P = -0.0002), and magnesium (P < 0.0001), suggesting that their lower BMD occurred despite an adequate diet based on current recommendations. Further study is needed to establish the cause of this deficit in bone mass and the benefit of additional nutritional support to reverse this problem.
以往研究表明，与对照受试者相比，苯丙酮尿症（PKU）患儿的骨矿化减少。为了对此进行研究，对32名青春期前PKU患儿和95名年龄匹配的对照受试者测量了全身骨密度（TBMD）。还对其中一部分受试者记录了脊柱骨密度（SBMD），PKU组24名，对照组55名。评估了30名PKU患儿和12名对照受试者的饮食摄入对骨量的影响。在PKU患儿中，调整身高和体重后，TBMD和SBMD显著低于对照受试者（分别为P = 0.03和P = 0.003）。PKU患儿的钙（P < 0.0001）、磷（P = -0.0002）和镁（P < 0.0001）摄入量较高，这表明尽管根据当前建议饮食充足，但他们的骨密度仍较低。需要进一步研究以确定这种骨量不足的原因以及额外营养支持对改善这一问题的益处。
