Lang S M, Fischer R, Stratakis D F, Huber R M
Klinik Bad Reichenhall der LVA Niederbayern Oberpfalz, Fachklinik für Erkrankungen der Atemwege für Allegien und für Orthopädie, Bad Reichenhall.
Dtsch Med Wochenschr. 2004 Jul 9;129(28-29):1551-5. doi: 10.1055/s-2004-828988.
In adult patients with cystic fibrosis bone metabolism may be altered by multiple mechanisms, such as abnormal calcium homeostasis, malnutrition, chronic inflammation or inactivity in the course of respiratory failure. In contrast to the high prevalence of osteoporosis in CF patients before lung transplantation, data from different CF collectives show great variation. It was the purpose of our cross-sectional study to determine changes in bone metabolism by measuring bone mineral density, and assessing calcium metabolism and clinical characteristics in adult patients (mean age 32 years) with cystic fibrosis.
Bone mineral density (quantitative digital radiography), parameters of calcium homeostasis and clinical characteristics were determined in 34 adult patients with cystic fibrosis.
The mean age of the study population was 32 years (range 20 to 47; 21m: 13f). 13 patients had normal T-values (mean bone mineral density in young adults), whereas 11 patients (32 %) had osteopenia and 10 (29 %) had osteoporosis. Calcium homeostasis was abnormal in only one case. In contrast T-values were positively correlated with a low body mass index (p = 0.01) and a low one-second forced expiratory volume (FEV1) (p < 0.05).
Decreased mineral bone density is a frequent complication in adult patients with cystic fibrosis, but does not occur inevitably even in long-standing disease (up to 47 years). In our cohort measurable alterations of calcium homeostasis could be avoided by consistent substitution policy. The main determinants of a low T-value were poor nutritional status, lowered serum calcium or phosphate concentrations and severely impaired lung function as indicator of the progression of the disease. Screening of adult patients with CF can be recommended especially in presence of malnutrition or poor lung function.
在成年囊性纤维化患者中,骨代谢可能通过多种机制发生改变,如钙稳态异常、营养不良、慢性炎症或呼吸衰竭过程中的活动减少。与肺移植前囊性纤维化患者骨质疏松的高患病率相反,来自不同囊性纤维化群体的数据显示出很大差异。我们这项横断面研究的目的是通过测量骨密度、评估钙代谢和临床特征,来确定成年囊性纤维化患者(平均年龄32岁)骨代谢的变化。
对34例成年囊性纤维化患者测定骨密度(定量数字X线摄影)、钙稳态参数和临床特征。
研究人群的平均年龄为32岁(范围20至47岁;21名男性,13名女性)。13例患者的T值正常(年轻成年人的平均骨密度),而11例患者(32%)患有骨质减少,10例(29%)患有骨质疏松症。仅1例患者钙稳态异常。相反,T值与低体重指数(p = 0.01)和低一秒用力呼气量(FEV1)(p < 0.05)呈正相关。
骨矿物质密度降低是成年囊性纤维化患者常见的并发症,但即使在病程长达47年的长期疾病中也并非不可避免。在我们的队列中,通过一致的替代策略可以避免可测量的钙稳态改变。低T值的主要决定因素是营养状况差、血清钙或磷酸盐浓度降低以及作为疾病进展指标的严重肺功能受损。建议对成年囊性纤维化患者进行筛查,尤其是在存在营养不良或肺功能差的情况下。