Department of Dermatology Pediatrics, Stanford University School of Medicine, Stanford, CA 94305-5168, USA.
Br J Dermatol. 2011 Nov;165(5):1081-6. doi: 10.1111/j.1365-2133.2011.10517.x. Epub 2011 Sep 29.
Reduced bone mass and fractures are known complications of generalized forms of epidermolysis bullosa (EB). However, the aetiology - inadequate bone acquisition, premature bone loss, or a combination - is unclear.
To determine patterns of bone mineral acquisition in children with EB and to identify clinical and laboratory correlates of change in areal bone mineral density (aBMD).
Seventeen subjects ≥ 6 years of age with generalized EB were studied at two visits at least 12 months apart with clinical and laboratory evaluations and dual energy X-ray absorptiometry scans of the lumbar spine. Wilcoxon signed-rank tests were used to determine if changes from baseline to follow-up were significant. Wilcoxon rank-sum tests were used to compare subjects with gains in aBMD Z-score with those who experienced no change or decreases to determine if baseline laboratory or clinical characteristics differed between the two groups.
Subjects gained height and weight at follow-up, but there was no significant improvement in mean Z-scores for height, weight or body mass index. Laboratory values did not change significantly. Mean bone mineral content and aBMD of the lumbar spine increased significantly at follow-up, but mean aBMD Z-scores remained static. No differences in clinical characteristics or laboratory values were seen between subjects with increased aBMD Z-scores vs. those whose scores decreased or did not change.
Low bone mass in children with generalized EB is due primarily to inadequate gains in aBMD. Interventions to improve overall health and to help build bone mass in this patient population are warranted.
已知广泛型大疱性表皮松解症（EB）会导致骨量减少和骨折等并发症。然而，其病因（骨量获取不足、过早的骨质流失，或两者兼而有之）尚不清楚。
确定 EB 患儿骨矿物质获取的模式，并确定其骨密度（aBMD）变化的临床和实验室相关因素。
本研究纳入了 17 名年龄≥6 岁、患有广泛型 EB 的受试者，他们在至少相隔 12 个月的两次就诊中接受了临床和实验室评估以及腰椎双能 X 射线吸收法扫描。采用 Wilcoxon 符号秩检验确定从基线到随访的变化是否具有统计学意义。采用 Wilcoxon 秩和检验比较 aBMD Z 评分增加和无变化或减少的受试者，以确定两组之间的基线实验室或临床特征是否存在差异。
受试者在随访时身高和体重增加，但身高、体重或体重指数的 Z 评分平均值没有显著改善。实验室值没有显著变化。腰椎骨矿物质含量和 aBMD 的平均值在随访时显著增加，但 aBMD Z 评分平均值保持不变。在 aBMD Z 评分增加的受试者与评分降低或无变化的受试者之间，临床特征或实验室值没有差异。
患有广泛型 EB 的儿童骨量减少主要是由于 aBMD 增加不足所致。需要采取干预措施来改善整体健康状况，并帮助该患者群体增加骨量。
