ABSTRACT
Objective To analyze the clinical and laboratory characteristics,treatment,and outcomes of Ka-wasaki disease (KD)patients associated with macrophage activation syndrome (MAS)(KD - MAS)and to compare three diagnostic standards. Methods Twelve cases of KD - MAS were reviewed retrospectively,who had been treated and therapied at the Children′s Hospital of Chongqing Medical University from September 2007 to September 2017. The clinical data were analyzed. And,the efficacy of different MAS diagnostic criteria for KD - MAS was evaluated. Results The subjects included 8 males and 4 females,with a median age of 25 months. The capital trigger of MAS was infection(8 cases,66. 7％). Unabating high fever had been the initial manifestation for 12 patients(100％),other com-mon clinical features including hepatomegaly(11 cases,91. 6％),splenomegaly(8 cases,66. 7％)and lymphadenectasis (7 cases,58. 3％). Besides,8 patients (66. 7％)had different degrees of central nervous system symptoms. Laboratory examination showed a decrease in hemoglobin (11 cases,91. 6％),in thrombocytopenia (8 cases,66. 7％),and white blood cells (4 cases,33. 3％);while there was an increase were found in serum transaminase (11 cases,91. 6％), triglyceride(72. 7％,8 / 11 cases)and serum ferritin (100％,9 / 9 cases). Eleven patients (91. 6％)had decreased erythrocyte sedimentation rates (ESR). Bone marrow cytology was performed in 10 cases,and 8 cases of them showed hemophagocytic phenomenon. All the patients were diagnosed by SoJIA - MAS(2005)criteria. All patients were treated with high - dose intravenous immunoglobulin (IVIG)treatment,among whom 3 cases were combined with methylpred-nisolone treatment,and 2 cases received with more than 2 kinds of immunosuppressive drugs (Dexamethasone and Ciclosporin or Etoposide). Among the 12 patients,2 patients lost to follow - up,4 cases(33. 3％)died due to hepatic encephalopathy,including 2 cases who withdrawn treatment the remaining 6 cases (50. 0％)improved. Conclusions Prolonged high fever is the first manifestation of MAS in KD. Hemogram and ESR will decrease,elevated serum transaminase and ferritin may increase,which indicates MAS occurrence. If a high dose of IVIG therapy does not work,the combination of glucocorticoid and immunosuppressive for therapy may improve the remission rate. Severe cen-tral nervous system involvement may indicate a terrible prognosis. SoJIA - MAS (2005)can diagnose earliler by using preliminary diagnostic guidelines for macrophage activation system complicating systemic juvenile idiopathic arthritis.
ABSTRACT
Objective To evaluate the effect of breathing exercise program (shrinking lips abdominal breathing combined with vertical breathing gymnastics) on dyspnea, pulmonary function, exercise tolerance and quality of life in patients with moderate to severe chronic obstructive pulmonary disease(COPD). Methods A total of 90 hospitalized patients with moderate to severe COPD were randomly divided into the experimental group and the control group with 45 cases in each group. The experimental group lost one case and the control group lost three cases, 86 patients finished the experiment. During hospitalization, on the base of conventional treatment and care, the experimental group received training of shrinking lips abdominal breathing combined with vertical breathing gymnastics after medical staff′s care, with folk music as the background. Exercise frequency:morning, afternoon, 2 times per day, 15 min per time and continue to exercise with researchers′ guidance after discharge. In the control group:take exercises by self, take routine follow-up after discharge. The intervention continued for three months. The indicators such as dyspnea, pulmonary function, exercise tolerance and life quality of both groups were assessed respectively before and after the intervention. Results There were no significant differences in dyspnea symptoms, lung function, exercise endurance, quality of life between two groups before intervention (P>0.05). The dyspnea scored 1.43±0.87 after intervention in the experimental group, and 1.93 ±0.97 in the control group, there was significant difference (Z=-2.293, P=0.022). The 6 min walking test distance was (371.34 ± 67.74) m after intervention in the experimental group, and (301.57 ± 61.67) m in the control group, there was significant difference(t = 4.988, P =0.000). The St. George′s Respiratory Questionnaire (respiratory symptoms, limited activity, influence disease) score and total score were 54.73 ± 11.96, 52.55 ± 14.48, 55.45 ± 10.01, 54.56 ± 10.79 after intervention in experimental group respectively, and 61.19 ± 10.72, 61.35 ± 14.66, 60.48 ± 9.39, 60.93 ± 10.16 in the control group, there were significant differences(t=-2.815--2.397, P0.05). Conclusions The program of shrinking lips abdominal breathing combined with vertical breathing gymnastics can relieve dyspnea in patients with moderate to severe COPD, improve exercise tolerance and quality of life, which is a safe and effective rehabilitation for patients with COPD.