Abstract：
OBJECTIVE: Aim: To analyse laboratory and biochemical features of the severe persistent course of asthma in patients with undifferentiated connective tissue dysplasia (UCTD) syndrome, and their phenotypic and visceral stigmas of dysembryogenesis.
METHODS: Materials and Methods: We enrolled 60 male patients with asthma, aged from 23 to 62 years (mean age (46.83 ±0.85) years): 30 patients with the background of UCTD, and 30 - without UCTD. We analysed clinical, somatometric, surveying (original questionnaire based on the phenotypic map of Glesby), instrumental (spirography, echocardiography, endoscopy, esophagofibrogastroduodenoscopy) and laboratory (including eosinophilic granulocytes and aldosterone levels) data.
RESULTS: Results: Correlations were found in men with UCTD between the number of UCTD markers and rate of earlobe diagonal fold (r=+0.75; р<0.05), asthenic constitution (r=+0.72; р<0.05), easy bruising (r=+0.7; p<0.05) and straight abdominal line hernia (r=+0.52; p<0.05). Average aldosterone serum level in patients with UCTD (176,10 ±11,22) was significantly higher than in those without UCTD (142,77 ±9,43), (p<0.05), as well as average eosinophils levels (1.3 ±0.25 vs. 0.57 ±0.12, p<0.05). In the absolute majority of patients with UCTD (93.3%) asthma onset was confirmed after pneumonia, and their age of asthma manifestation was significantly higher (37.2 ±1.21) than in patients without UCTD (21.4 ±1.13). Also, in patients with UCTD there was a high number of severe exacerbations during the last year (2.7 ±0.12 per year) on the background of high doses of combined inhaled glucocorticosteroids use.
CONCLUSIONS: Conclusions: Identified \"phenotypic profile\", clinical and biochemical features of patients with asthma on the background of UCTD syndrome, which determine the severe course and early formation of asthma complications, will further accelerate the diagnosis of this asthma phenotype and improve approaches to the selection of treatment regimens for these patients.
METHODS: Materials and Methods: We enrolled 60 male patients with asthma, aged from 23 to 62 years (mean age (46.83 ±0.85) years): 30 patients with the background of UCTD, and 30 - without UCTD. We analysed clinical, somatometric, surveying (original questionnaire based on the phenotypic map of Glesby), instrumental (spirography, echocardiography, endoscopy, esophagofibrogastroduodenoscopy) and laboratory (including eosinophilic granulocytes and aldosterone levels) data.
RESULTS: Results: Correlations were found in men with UCTD between the number of UCTD markers and rate of earlobe diagonal fold (r=+0.75; р<0.05), asthenic constitution (r=+0.72; р<0.05), easy bruising (r=+0.7; p<0.05) and straight abdominal line hernia (r=+0.52; p<0.05). Average aldosterone serum level in patients with UCTD (176,10 ±11,22) was significantly higher than in those without UCTD (142,77 ±9,43), (p<0.05), as well as average eosinophils levels (1.3 ±0.25 vs. 0.57 ±0.12, p<0.05). In the absolute majority of patients with UCTD (93.3%) asthma onset was confirmed after pneumonia, and their age of asthma manifestation was significantly higher (37.2 ±1.21) than in patients without UCTD (21.4 ±1.13). Also, in patients with UCTD there was a high number of severe exacerbations during the last year (2.7 ±0.12 per year) on the background of high doses of combined inhaled glucocorticosteroids use.
CONCLUSIONS: Conclusions: Identified \"phenotypic profile\", clinical and biochemical features of patients with asthma on the background of UCTD syndrome, which determine the severe course and early formation of asthma complications, will further accelerate the diagnosis of this asthma phenotype and improve approaches to the selection of treatment regimens for these patients.
摘要：
目的：目的：分析未分化结缔组织发育不良（UCTD）综合征患者哮喘严重持续病程的实验室和生化特征，以及它们的表型和内脏柱头发育异常。
方法：材料和方法：我们招募了60名男性哮喘患者，年龄23~62岁(平均年龄(46.83±0.85)岁):30例UCTD患者,和30-没有UCTD。我们分析了临床，躯体测量,调查(基于格莱斯比表型图的原始问卷)，仪器(肺活量描记术，超声心动图,内窥镜检查,食管纤维胃十二指肠镜检查）和实验室（包括嗜酸性粒细胞和醛固酮水平）数据。
结果：结果：在UCTD患者中，UCTD标记的数量与耳垂对角折叠率之间存在相关性（r=0.75；C.2<0.05），虚弱体质(r=+0.72;C.2<0.05),易擦伤(r=+0.7;p<0.05)和直腹线疝(r=+0.52;p<0.05)。UCTD患者血清醛固酮平均水平(176,10±11,22)明显高于无UCTD患者(142,77±9,43),（p<0.05），以及平均嗜酸性粒细胞水平(1.3±0.25vs.0.57±0.12，p<0.05)。在绝大多数UCTD患者（93.3%）中，哮喘发作是在肺炎后确诊的，他们的哮喘表现年龄（37.2±1.21）明显高于无UCTD的患者（21.4±1.13）。此外，在UCTD患者中,在高剂量联合吸入型糖皮质激素使用的背景下,去年出现了大量严重加重(每年2.7±0.12).
结论：结论：确定的“表型概况”，UCTD综合征背景下哮喘患者的临床和生化特征，这决定了哮喘并发症的严重病程和早期形成，将进一步加速这种哮喘表型的诊断,并改进这些患者治疗方案的选择.
方法：材料和方法：我们招募了60名男性哮喘患者，年龄23~62岁(平均年龄(46.83±0.85)岁):30例UCTD患者,和30-没有UCTD。我们分析了临床，躯体测量,调查(基于格莱斯比表型图的原始问卷)，仪器(肺活量描记术，超声心动图,内窥镜检查,食管纤维胃十二指肠镜检查）和实验室（包括嗜酸性粒细胞和醛固酮水平）数据。
结果：结果：在UCTD患者中，UCTD标记的数量与耳垂对角折叠率之间存在相关性（r=0.75；C.2<0.05），虚弱体质(r=+0.72;C.2<0.05),易擦伤(r=+0.7;p<0.05)和直腹线疝(r=+0.52;p<0.05)。UCTD患者血清醛固酮平均水平(176,10±11,22)明显高于无UCTD患者(142,77±9,43),（p<0.05），以及平均嗜酸性粒细胞水平(1.3±0.25vs.0.57±0.12，p<0.05)。在绝大多数UCTD患者（93.3%）中，哮喘发作是在肺炎后确诊的，他们的哮喘表现年龄（37.2±1.21）明显高于无UCTD的患者（21.4±1.13）。此外，在UCTD患者中,在高剂量联合吸入型糖皮质激素使用的背景下,去年出现了大量严重加重(每年2.7±0.12).
结论：结论：确定的“表型概况”，UCTD综合征背景下哮喘患者的临床和生化特征，这决定了哮喘并发症的严重病程和早期形成，将进一步加速这种哮喘表型的诊断,并改进这些患者治疗方案的选择.
