| Objective:This study aimed to investigate the characteristics of pulmonary function in patients with amyotrophic lateral sclerosis(ALS),to explore the relationship between the disease onset,time of diagnosis,FVC% and ALSFRS-R score,and to study the risk factors of survival time.Method:69 patients with ALS who visited the first Hospital of Jilin University from September 2016 to December 2018 were selected.The clinical data such as gender,age of onset,time from onset to diagnosis,area of onset,ALSFRS-R score and pulmonary function were collected,and the information of pulmonary function,arterial blood gases(ABGs)and ALSFRS-R score were followed up every 3 months for one year.The FVC% and the time of diagnosis were compared by bilateral t-test,and the correlation analysis about the influencing factors of pulmonary function in ALS was carried out.The survival curves of different groups were described by Kaplan-Meier method and tested by Log-rank method.Result:1.A total of 69 with sporadic ALS patients were recruited in this study,including 42 males(60.9%)and 27 females(39.1%),the male: female ratio was 1.56:1.The mean age of symptom onset was 53.5±9.6 years old.The age of onset was earlier in the limb-onset group(52.7 years old),as well as the age in the bulbar-onset group(60.3 years old),and the difference has statistically significant(P=0.048).The differences between the two groups were statistically significant in ALSFRS-R score(39.3 vs.26.9,P < 0.001),time from symptom onset to FVC% < 70%(18.1 vs.10.7,P=0.001),and FVC%(87.2% vs.62.3%,P < 0.001).2.Mean FVC% was 84.6%±16.3%.After adjusting for ALSFRS-R score,time of diagnosis,age of onset and gender,FVC% in the limb-onset group was 23.2% higher than the bulbar-onset group(95%CI: 5.9%,40.4%),which was statistically significant(P=0.009).Linear correlation analysis showed that there was a positive correlation between ALSFRS-R score and FVC%,suggesting that the higher the ALSFRS-R score,the higher the FVC% and the better the prognosis.3.The damage indicators of pulmonary function in ALS patients could indicate the progression of the disease,in which the decrease rate of FVC% was 1.4% per month and of ALSFRS-R score was 0.8 points per month.The Δ FVC% and Δ ALSFRS-R of patients with bulbar-onset were 3.8%(95% CI :1.9%,7.0%)and 2.3%(95% CI : 0.67,3.83),which are significantly fall faster than limb-onset group 1.2%(95% CI: 0,5.6%)and 0.7%(95% CI: 0.15,3.33)(P < 0.001;P = 0.001).4.The survival time from onset to FVC<70% was analyzed,and the overall median survival time was 17.0 months.The median survival time of bulbar-onset group was 12.0 months,which was significantly shorter than the limb-onset group(18 months)(P<0.001).Furthermore,according to the further classification of limbs,the median survival time of patients with onset of upper limbs,lower limbs and unknown limbs were 20.0 months,17.0 months and 16.0 months,longer than the bulbar-onset group.The differences were statistically significant(P=0.001).Conclusion:(1)There are more males with ALS than females,and most of the patients with bulbar-onset were females.(2)Most ALS patients were limb-onset,they were onset earlier,better overall condition,better disease development,and longer time to diagnosis.(3)The risk factors for pulmonary function in ALS patients were bulbar-onset,rapid progression and low ALSFRS-R score.(4)Δ FVC % and Δ ALSFRS-R can reflect changes in pulmonary function and disease progression,and can be used as a related index to judge therapeutic effect in patients with ALS.(5)Patients with low ALSFRS-R score,bulbar-onset and early diagnosis need to receive non-invasive ventilation earlier,which can be used as deleterious prognostic factors for prognosis. |
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