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Objective To analyze the clinical features of children with Kawasaki disease(KD) in order to find out the trend of its clinical features and to provide guidance for clinical diagnosis and treatment.Methods Children with KD hospitalized in the Pediatric Ward of Peking University Third Hospital from January 2007 to December 2016 were collected and analyzed for their characteristics of onset,clinical features and treatment.The children were divided into 2 groups according to the time:2007 to 2011 group,2012 to 2016 group.The changes in clinical characteristics between the 2 stages were analyzed.Results A total of 337 children with KD were enrolled in this study,including 212 males and 125 females,with a ratio of 1.69 ∶ 1.00.The age of onset was from 2 months to 12 years old.The median age was 24 months.A total of 325(96.4％) children were under 6 years of age.There were 114(33.8％) cases of KD children from 2007 to 2011,among whom incomplete KD (IKD),intravenous gamma globulin non-response and coronary artery injury cases accounted for 23.7％ (27 cases),4.5％ (5 cases),38.6％ (44 cases),respectively;there were 223 (66.2％) cases of KD children from 2012 to 2016,among whom IKD,intravenous gamma globulin non-response and coronary artery injury cases accounted for 38.6％ (86 cases),11.9％ (26 cases),31.4％ (70 cases),respectively.The group of 2012 to 2016 was compared with the the group of 2007 to 2011 and it was found that the proportion of IKD and intravenous gamma globulin non-response cases increased,and the differences were significant (x2 =7.495,4.654,P =0.006,0.031),but the proportion of coronary artery injury cases decreased with no statistical difference (x2 =1.750,P =0.186).Conclusion With the incidence of KD increasing,the proportion of incomplete KD and intravenous gamma globulin non-response KD also showed an upward trend,which should be paid more attention to by clinicians in order to promptly diagnose and implement more targeted treatment.
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Objective To investigate the advantages of glucocorticoid combined with traditional meth-ods and to explore glucocorticoid use in appropriate time in the whole course of the disease and the appropriate dose.Methods With reference to Kawasaki disease(KD)diagnostic criteria,363 cases of patients with KD were enrolled,and divided into conventional treatment group and combined hormone group randomly. Combined hormone group according to clinical manifestations can be divided into sub groups as typical KD and atypical KD group. We analyzed respectively before and after 1 week treatment in two groups of children with the onset of age,gender,heating duration after treatment, IVIG treatment time, hospitalization days and total hospitalization expenses,tips and course of acute stage,recovery period the relevant indexes to the test,and coronary artery le-sion( CAL) in one month. Results Thermal process after treatment,hospitalization duration and hospitalization expenses decreased significantly in combined hormone group compared with conventional treatment group. The difference between the two,in the clinical laboratory indexes and CAL have no obvious difference. In different types of KD, the thermal process after treatment, hospitalization days and hospitalization expenses, have de-creased significantly in typical KD sub group when treating with combined hormone therapy comparing with hor-mone therapy atypical sub group. Other clinical laboratory indexes and CAL have no obvious differences in com-pared between the sub groups. Conclusion Combined hormone therapy for KD patients,plays an important role in shortening the thermal process after treatment and reducing the hospitalization expense.
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Objective To investigate the advantages of glucocorticoid combined with traditional meth-ods and to explore glucocorticoid use in appropriate time in the whole course of the disease and the appropriate dose.Methods With reference to Kawasaki disease(KD)diagnostic criteria,363 cases of patients with KD were enrolled,and divided into conventional treatment group and combined hormone group randomly. Combined hormone group according to clinical manifestations can be divided into sub groups as typical KD and atypical KD group. We analyzed respectively before and after 1 week treatment in two groups of children with the onset of age,gender,heating duration after treatment, IVIG treatment time, hospitalization days and total hospitalization expenses,tips and course of acute stage,recovery period the relevant indexes to the test,and coronary artery le-sion( CAL) in one month. Results Thermal process after treatment,hospitalization duration and hospitalization expenses decreased significantly in combined hormone group compared with conventional treatment group. The difference between the two,in the clinical laboratory indexes and CAL have no obvious difference. In different types of KD, the thermal process after treatment, hospitalization days and hospitalization expenses, have de-creased significantly in typical KD sub group when treating with combined hormone therapy comparing with hor-mone therapy atypical sub group. Other clinical laboratory indexes and CAL have no obvious differences in com-pared between the sub groups. Conclusion Combined hormone therapy for KD patients,plays an important role in shortening the thermal process after treatment and reducing the hospitalization expense.
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High-dose intravenous gamma globulin (2 g/kg) combined with oral aspirin is currently considered as standard strategy for Kawasaki disease (KD) in initial treatment.However,there are still 15％-25％ of KD patients with no response to the treatment,thus leading to persistent fever,or even further damage of the coronary arteries.International literature have also mentioned other methods of treatment,such as corticosteroids,TNF-α blockade,lyclosporin,IL-1 receptor blockade,methotrexate,anti-CD20 rituximab,plasma exchange.But fixed standard for non-reactive gamma globulin require for further treatment.
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Purpose: Many patients who have an acceptable living- kidney donor do not undergo transplantation because of the presence of antibodies against the donor cells resulting in a positive lymphocyte-crossmatch (LCX). Recently, the combination therapy of plasmapheresis, intravenous gamma- globulin and potent immunosuppression to induce negative conversion of LCX in patients who had positive LCX to their living donors was reported. Our institute gave these patients the combination therapy and reported the results of follow-up done 1~3 years after kidney transplantation. Methods: Eleven patients, who showed positive LCX to their living donors, underwent the conversion trials between January 1, 2002 and March 31, 2004. Combination therapy consisting of plasmapheresis, intravenous gamma globulin injection, tacrolimus, mycophenolate mofetil (MMF) and steroids was used. Plasmapheresis had been done every other day up to 6 times. Kidney transplantations were performed immediately after negative conversion was achieved. Five to ten day-courses of ATG (or OKT3) were used as an induction immunosuppression and tacrolimus, MMF, and steroids as a maintenance immunosuppression. Results: Negative conversions in ten out of eleven patients were achieved. Kidney transplantations in these 10 patients were successfully performed. No hyperacute rejection transpired, although four patients developed acute rejection, whose grafts were all rescued with steroid pulse therapy. Serum creatinine level was 1.57+/-0.12 mg/dL (mean+/-SD) during follow-up periods except for one whose graft was lost to Polyoma virus nephropathy. Conclusion: Nine of the 10 grafts are functioning well for 15~41 months after transplantations. Our results suggest that selected crossmatch positive patients can be transplanted successfully with living donor kidney allograft.
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Humans , Allografts , Antibodies , Creatinine , Follow-Up Studies , gamma-Globulins , Immunosuppression Therapy , Kidney , Kidney Transplantation , Living Donors , Lymphocytes , Plasmapheresis , Polyomavirus , Steroids , Tacrolimus , Tissue Donors , TransplantsABSTRACT
Objective To study the dose of IVGG in treating atypical Kawasaki disease(KD) at defervescence and the necessity.Methods Clinical data of 54 cases of atypical Kawasaski disease without coronary artery lesion (CAL) but with the abatement of fever in 10 days were retrospectively analyzed. They were divided into there groups according to IVGG dosage ;group A (1g/kg IVGG n=11 ),group B(2g/kg n=24 ) and group C (blank n=19 ) .CAL was observed to at 14~21 days and 0.5year. Results Compared with group A and B, The rate of CAL was significantly higher (P0.05) . Conclusions In order to prevent CAL ,atypical Kawasaki disease(KD) children with confirmed diagnosis in 10 days should all be treated with IVGG whether with abatement of fever or not .The dose of 1g/kg IVGG would have satisfactory effects on atypical KD children at defervescence.
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PURPOSE: It is well-known that kidney transplantation cannot be done if recipient has circulating antibodies showing positive lymphocyte cross-match (LCX) to organ donor. In the United States and European countries, the incidence of positive LCX to cadaveric donors in patients who are on the waiting list is up to 20~40%. Unfortunately, these patients also show high rate of positive LCX to live donors when they have donor candidates in their family members and have to be on dialysis until compatible donor comes up. Recently, Eugene J Schweitzer and his associates at the University of Maryland used the combination therapy with plasmapheresis, intravenous gamma globulin and potent immunosuppression to induce negative conversion of LCX in patients who were LCX positive to their living donors and reported the good results after the trial. We did the combination therapy in patients who had positive LCX to their living donors and reported the results. METHODS: Seven patients, four women and three men who showed positive LCX to their living donors, underwent the conversion trials between January 1 and July 31, 2002. The mean age of patients was 43.86 (35~60) and the duration of dialyses varies from 9 to 120 months. We used combination therapy with plasmapheresis, intravenous gamma globulin injection, tacrolimus, mycophenolate mofetil (MMF) and steroids. Plasmapheresis had been done on every other day up to 6 times to induce negative conversion of LCX. If patient continue to show positive LCX to donor after 6 times of plasmapheresis, we stopped the therapy. The numbers of plasmapheresis varies from two to six times. Kidney transplantations were preformed immediately after negative conversion of LCX as a semi-elective procedures. Five to ten day courses of ATG (or OKT3) were used as an induction immunosuppression after transplantation and tacrolimus, MMF, and steroids were used as a maintenance immunosuppression. RESULTS: We could achieve negative conversion of LCX in six out of seven patients, and kidney transplantations were performed in these 6 patients successfully. There was no hyperacute rejection during the operations, but three patients developed acute rejection episodes during their early postoperative periods. Steroid pulse therapies were used as a primary therapy to treat acute rejection and all three patients showed complete recovery of their graft function after the treatments. Baseline serum creatinine level varies from 1.0 mg/dl to 1.9 mg/dl with 3 to 6 months follow-up periods after transplantations. We could not induce negative conversion in one patient and he remained on hemodialysis. CONCLUSION: We did successful kidney transplantations in six patients who achieved negative conversion of LCX to their donors after the combination therapy with plasmapheresis and potent immunosuppression. All patients showed excellent graft function since their operations and did not have any significant complications except three reversible acute rejection episodes. According to the results, although it is preliminary, we recommend the use of the combination therapy in patient who has LCX positive living donor. Further long-term study with more numbers of patients is needed for the evaluation of the efficacy of this trial.
Subject(s)
Female , Humans , Male , Antibodies , Cadaver , Creatinine , Dialysis , Follow-Up Studies , gamma-Globulins , Immunosuppression Therapy , Incidence , Kidney Transplantation , Living Donors , Lymphocytes , Maryland , Plasmapheresis , Postoperative Period , Renal Dialysis , Steroids , Tacrolimus , Tissue Donors , Transplants , United States , Waiting ListsABSTRACT
PURPOSE: Treatment of Kawasaki disease with intravenous gamma globulin(IVGG), together with aspirin, has been dernonstrated to be safe and effective in preventing coronary artery lesion and systemic inflarnmation, but optimal IVGG dosage and administration method are still controversial. We compared the therapeutic efficacy and clinical response of single IVGG 1g/kg to that of IVGG lg/kg for comparable risk group of Kawasaki disease. METHODS: We conducted a prospective study involving 63 children with Kawasaki disease requiring IVGG treatment(Harada score> or =4) at Chungnam National University Hospital from February 1996 to January 1999. The children were assigned to receive IVGG either as a single infusion of 1g/kg(A group, 32 person) or 2g/kg(B group, 31 person) and aspirn(100mg/kg/day through acute phase, then 3 to 5mg/kg/day for 8 weeks of duration). RESULTS: There were no significant difference between the two groups according to clinical and laboratry data, including coronary artery lesions(group A, 31.3% and group B, 29.0%) before treatment. After IVGG treatment ratio of complication with coronary artery lesion(group A 1/32=3.1% and group B, 2/31=6.5%) and that of retreatment(group A, 4/32=12.5%, group B, 2/31=6.5%), duration of fever(group A, 1.3+/-1.6 days and group B, 0.7+/-1.4 days), hospital stay(group A, 7.0+/-1.4 days and group B, 6.5+/-2.0 days), laboratory finding and side effects of IVGG were not significantly different(P>0.05). The total dosage of IVGG was significantly lower in group A than group B(group A, 1.16+/-0.37g/kg, 375,421+/-207,351won and group B, 2.10+/-0.40g/kg, 641,498+/-274,750won (P<0.05). CONCLUSION: The therapeutic efficacy and clinical response of single 1g/kg therapy are comparable to that of single 2g/kg therapy.
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Child , Humans , Aspirin , Coronary Vessels , gamma-Globulins , Mucocutaneous Lymph Node Syndrome , Prospective StudiesABSTRACT
PURPOSE: This study was designed to determine the outcome and safety of intravenous gamma-globulin(IVGG) retreatment in Kawasaki disease. METHODS: A clinical observation of the therapeutic effects, laboratory findings and echocardiograms was carried out on 72 patients with Kawasaki disease in Kosin University Hospital from 1991 to 1999. 27 patients were treated with 1g/kg/day IVGG for 2 days, 45 patients were treated with 2 g/kg for 10hours. The clinical indication for retreatment was fever. Persistent fever was defined as a temperature> or =38.3degrees C persisting beyond 48hrs after the completion of the infusion. Recrudescent fever was defined as a temperature> or =38.3degrees C for 48hrs after the completion of the infusion, followed by a temperatureup. CONCLUSION: We concluded that the IVGG retreatment of Kwasaki disease may improve the clinical course and coronary artery outcome.
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Child , Humans , Coronary Vessels , Fever , gamma-Globulins , Mucocutaneous Lymph Node Syndrome , Retreatment , Treatment FailureABSTRACT
We compared the efficacy of each modality of treatment group in reducing the frequency of coronary artery abnormalities and change of clinical courses in children with Kawasaki disease in the children of 81 cases who were admitted in pediatric department of Yeungnam University Hospital from September 1985 to August 1990. Group A (37 cases)-aspirin alone, Group B (44 cases)-intravenous gammaglobulin (400 mmg/kg/day) for 5 consecutive days, plus aspirin. We studied the frequency of echocardiographic abnormalities, the duration of fever, and changes in the total white blood cell counts, platelet counts, ESR and CRP value at 1, 2 and 3 weeks of the illness and compared the results between the two groups. The results were as follows. 1) There was no significant intergroup difference in age and sex ratio. 2)The duration of the febrile period after the initiation of the therapy was significant shorter in group B (2.5±1.2days) than in group A: (5.2±3.5 days) (p<0.01). 3) No significant difference was noticed in the WBC and platelet counts in two groups as measured at admission day, 1 and 2 weeks of the illness, however, at 3weeks of illness significant difference was noted. 4) The CRP values measured at 1,2 and 3 weeks after treatment were significantly lower in group B (2.42±1.8, 2.00±1.2, 1.16±1.0) than in group A (7.22±5.3, 5.25±3.9, 1.85±1.2) respectively (p<0.01). 5) In 2D-Echocardiogram, coronary artery dilatation was more frequent in Group A than in Group B at 6month of illness (p<0.01). In conclusion, intravenous gammaglobulin therapy was effective in the shortening of the duration of fever and in the anti-inflammatory action and somewhat effective in prevention of coronary artery aneurysm.