全身型幼年特发性关节炎并发巨噬细胞活化综合征的护理诊断及干预要点

Main nursing diagnosis and intervention points of children with systemic juvenile idiopathic arthritis and macrophage activation syndrome

目的对全身型幼年特发性关节炎(systemic juvenile idiopathic arthritis,SJIA)并发巨噬细胞活化综合征(macrophage activation syndrome,MAS)患儿的护理诊断和干预要点进行探讨。方法纳入2020年5月至2021年6月7例于郑州大学附属儿童医院进行治疗的SJIA-MAS患儿为本次研究对象。其中男4例,女3例,年龄3~11(7.08±1.06)岁。根据临床特点等给予个性化的治疗措施,主要包括糖皮质激素、环孢素A、白细胞介素6(IL-6)受体拮抗剂,同时对患儿施以护理干预措施(病情监测、感染、出血、关节、口腔、发热、心理护理)。观察患儿的治疗效果、临床症状、实验室指标变化情况和随访结果,采用t检验进行统计学分析。结果应用甲泼尼龙治疗患儿3例,甲泼尼龙联合环孢素A治疗2例,IL-6受体拮抗剂2例。治疗后患儿的病情均有所缓解,并在1~3周体温逐渐恢复正常,2~3个月血常规水平逐渐恢复至正常范围,无死亡病例。1例采用IL-6受体拮抗剂治疗的患儿出现总胆固醇(TC)水平升高。治疗后4周的肿胀、压痛、活动受限关节数分别为(2.03±0.30)个、(1.58±0.23)个、(2.44±0.36)个,明显低于治疗前的(5.87±0.88)个、(5.45±0.81)个、(5.16±0.77)个(t=10.927、12.160、8.466,均P<0.001);红细胞沉降率(ESR)、C反应蛋白(CRP)、铁蛋白水平分别为(9.59±1.43)mm/h、(8.25±1.23)mg/L、(347.08±52.06)μg/L,均低于治疗前的(46.45±6.96)mm/h、(95.13±14.26)mg/L、(549.12±82.36)μg/L(t=13.933、16.059、5.486,均P<0.001),血红蛋白(Hb)水平为(131.88±19.78)g/L,高于治疗前的(110.30±16.54)g/L(t=2.214,P=0.046)。与治疗前相比较,治疗后8周的肿胀、压痛、活动受限关节数分别为(1.28±0.19)个、(0.42±0.06)个、(1.13±0.16)个,明显较少(t=13.489、16.384、13.557,均P<0.001);ESR、CRP、铁蛋白水平分别为(10.01±1.50)mm/h、(7.63±1.14)mg/L、(163.15±24.47)μg/L,明显较低(t=13.541、16.182、11.885,均P<0.001),Hb水平为(136.65±20.49)g/L,明显较高(t=2.647,P=0.021)。患儿出院后每隔1~2个月到医院门诊进行复查,均未再次出现SJIA-MAS典型临床症状和实验室指标水平异常的情况。结论SJIA-MAS属于儿科的危急重症,及时给予患儿有效的治疗和护理干预措施,能有效降低病死率并改善患儿预后。

Objective To investigate the main nursing diagnosis and intervention points of children with systemic juvenile idiopathic arthritis(SJIA)and macrophage activation syndrome(MAS).Methods Seven children with SJIA and MAS who were treated in Children's Hospital Affiliated to Zhengzhou University from May 2020 to June 2021 were included in this study.There were 4 males and 3 females;they were 3-11(7.08±1.06)years old.All the children were given personalized treatment measures according to their clinical characteristics,including glucocorticoids,cyclosporine A,interleukin 6(IL-6)receptor antagonists,and nursing interventions(disease monitoring,infection,bleeding,joint,oral,fever,and psychological care).The therapeutic effect,clinical symptoms,changes in laboratory indicators,and follow-up results were observed.t test were applied.Results Three cases were treated with methylprednisolone,2 cases with methylprednisolone and cyclosporine A,and 2 cases with IL-6 receptor antagonist.After the treatment,the conditions of all the children were relieved;their body temperature gradually returned to normal in 1-3 weeks;their blood routine levels gradually returned to normal in 2-3 months.There was no death case.However,one child treated with IL-6 receptor antagonist had an elevated total cholesterol(TC)level.Four weeks after the treatment,the numbers of swollen,tender,and restricted joints were(2.03±0.30),(1.58±0.23),and(2.44±0.36),which were significantly lower than those before the treatment[(5.87±0.88),(5.45±0.81),and(5.16±0.77)](t=10.927,12.160,and t=8.466;all P<0.001);the erythrocyte sedimentation rate(ESR)and levels of C-reactive protein(CRP)and ferritin were(9.59±1.43)mm/h,(8.25±1.23)mg/L,and(347.08±52.06)μg/L,which were lower than those before the treatment[(46.45±6.96)mm/h,(95.13±14.26)mg/L,(549.12±82.36)μg/L](t=13.933,16.059,and 5.486;all P<0.001);the hemoglobin(Hb)level was higher than that before the treatment[(131.88±19.78)g/L vs.(110.30±16.54)g/L;t=2.214,P=0.046].The numbers of swollen,tender,and motility limited joints 8 weeks after the treatment were(1.28±0.19),(0.42±0.06),and(1.13±0.16),which were significantly fewer than those before the treatment(t=13.489,16.384,and 13.557;all P<0.001).The ESR and levels of CRP and ferritin 8 weeks after the treatment were(10.01±1.50)mm/h,(7.63±1.14)mg/L,and(163.15±24.47)μg/L,which were significantly lower than those before the treatment(t=13.541,16.182,and 11.885;all P<0.001).The Hb level 8 weeks after the treatment was(136.65±20.49)g/L,which was significantly higher that before the treatment(t=2.647,P=0.021).After discharge,the children went to the outpatient department for reexamination every 1-2 months,and there were no abnormal clinical symptoms and laboratory indicators of SJIA-MAS again.Conclusion SJIA-MAS is a critical and severe disease in pediatrics.Timely and effective treatment and nursing intervention can effectively reduce the mortality rate and improve the children's prognosis.