■肌肉骨骼和心理健康受损在患有低磷酸盐症(HPP)的成年人中很常见。有人建议限制磷的摄入对HPP的症状有积极影响,但缺乏干预证据.
■这项工作旨在评估磷限制的效果,钙调节饮食对HPP肌肉骨骼和心理健康的影响。
■预期,非控制,单中心介入研究(NuSTEPSII)在骨科门诊患者中进行,维尔茨堡大学,德国。共有26名确诊为HPP的成年人接受了标准化饮食,在8周内每天摄入明确的磷(1160-1240mg/d)和钙(870-930mg/d)。主要结局指标是功能测试和患者报告的结局指标。
■在8周时,在通常的步态速度(P=.028)和起椅测试(P=.019)中观察到显着改善,而在6分钟步行测试(P=0.468)和定时上行测试(P=0.230)中没有发现显着变化。根据视觉模拟量表(VAS),疼痛没有明显减轻(P=0.061),36项简短健康调查(SF-36)的疼痛子量表(P=.346),和疼痛残疾指数(P=.686)。Further,SF-36活力分量表(P=.022)有显著改善,而所有其他分量表以及下肢功能量表(P=.670)和疲劳评估量表(P=.392)没有显著变化。矿物质摄入的调整与能量摄入和能量供应营养素或身体成分的相关变化无关。
■调整磷和钙的摄入量可能会对成人HPP的个体症状产生积极影响,但是在疼痛和耐力等主要问题上的总体临床有效性似乎有限。
UNASSIGNED: Impairments in musculoskeletal and mental health are common in adults with Hypophosphatasia (HPP). Restricted phosphorus intake has been suggested to positively affect symptoms in HPP, but there is a lack of interventional evidence.
UNASSIGNED: This work aimed to evaluate the effect of a phosphorus-restricted, calcium-adjusted diet on musculoskeletal and mental health in HPP.
UNASSIGNED: A prospective, noncontrolled, single-center interventional study (NuSTEPS II) was conducted among outpatients at the Osteology Department, University of Wuerzburg, Germany. A total of 26 adults with an established HPP diagnosis received a standardized diet with a defined daily intake of phosphorus (1160-1240 mg/d) and calcium (870-930 mg/d) over 8 weeks. Main outcome measures were functional testing and patient-reported outcome measures.
UNASSIGNED: At 8 weeks, significant improvements were observed in usual gait speed (P = .028) and the chair-rise test (P = .019), while no significant changes were seen in the 6-minute walk test (P = .468) and the timed up-and-go test (P = .230). Pain was not significantly reduced according to the visual analog scale (VAS) (P = .061), pain subscale of the 36-Item Short-Form Health Survey (SF-36) (P = .346), and Pain Disability Index (P = .686). Further, there was a significant improvement in the SF-36 vitality subscale (P = .022) while all other subscales as well as the Lower Extremity Functional Scale (P = .670) and the Fatigue Assessment Scale (P = .392) did not change significantly. Adjustments of mineral intake were not associated with relevant alterations regarding the intake of energy and energy-supplying nutrients or body composition.
UNASSIGNED: Adjusting phosphorus and calcium intake may positively affect individual symptoms in adults with HPP, but overall clinical effectiveness regarding major issues like pain and endurance appears limited.