Clinical Analysis Of 68 Children With Hemolytic Uremic Syndrome In 25 Years Of Single Center

Background and Purpose Hemolytic uremic syndrome occurs predominantly in children,with rapid onset,rapid progression,high acute mortality,poor prognosis,and easy recurrence.About 25%-30%of survivors still have permanent kidney damage.At present,there is little research data on the characteristics of the general population of hemolytic uremic syndrome(HUS)and the recovery of hematological indicators in the acute phase at home and abroad.The purpose is to explore the clinical characteristics,treatment and recovery of patients with HUS in the acute phase and their influencing factors.Meanwhile,a comparative analysis of the effects was taken in the change of the treatment plan on the acute phase outcomes in the first 13 years(1994.5?2007.10)and the last 13 years(2007.11?2019.9).Objects and Methods A retrospective analysis of 68 children with HUS who were admitted to the Provincial Hospital of Shandong University from May 1994 to September 2019 was conducted.The general data,clinical manifestations,laboratory examinations,treatment and recovery were summarized.And analyze the possible risk factors that affect the outcome and the effect of 13 years of treatment changes on the outcome of the acute phase.Results1.General information:The sex ratio is 5:2 for male to famale,age from 0.08 to 13.5(median 6.04)years.The disease season is distributed throughout the year,and it is more common from May to August and November to December.2.Clinical manifestations(1)Prodromal infections:53 of 68 children had symptoms of prodromal infections,50 of which had respiratory symptoms such as fever and cough,21 had digestive symptoms such as bloody stoolsand diarrhea,16 had symptoms of combined infection.(2)Clinical manifestations on the acute phase:47 of 68 children showed pale and yellow staining of skin and mucous membranes;42 showed skin bleeding spots or ecchymosis;43 had gross hematuria,31 cases had oliguria or anuria,27 cases had edema,and 26 cases had hypertension;another 44 cases showed complications of extrarenal system.3.Laboratory examination(1)Blood routine,urine routine and bone marrow examination:At acute phase,blood routine showed that 68 patients had anemia,65 of them showed moderate to severe anemia,61 cases of reticulocytes increased,54 cases of thrombocytopenia.Urine examination showed that 39 children showed Nephrotic proteinuria.36 cases of bone marrow aspiration examination showed hemolytic anemia and thrombocytopenia.(2)Biochemical tests:Liver and kidney function showed 68 cases showed acute kidney damage;38 cases showed hyperbilirubinemia,mainly indirect bilirubin elevation;52 cases showed hyperlactide dehydrogenaseemia.(3)Immune index:37 cases had reduced C3 and normal C4,and 9 cases had reduced C3 and C4.9 cases were tested for factor H and ADAMTS13 activity.AD AMTS 13 activity was normal,7 cases were positive for factor H antibody,2 cases were normal for factor H concentration,and 5 cases were reduced.(4)Film degree exam:41 cases underwent urinary ultrasound examination,mainly showing enhanced echo of both kidney parenchyma.24 patients underwent chest imaging examination,mainly showing pneumonia,bronchitis or pleural effusion.13 cases underwent cranial MRI or CT examination.The results showed that 9 cases showed abnormal signals in the brain,and 2 cases showed reversible posterior encephalopathy.(5)Renal biopsy:12 children underwent renal biopsy.Light microscopy mainly manifested as mesangial hyperplasia,endothelial cell swelling,balloon adhesion,double basement membrane contour,diffuse or segmental glomerulosclerosis,capillary microthrombosis,onion skin-like changes,etc.Immunofluorescence showed immune complex was distributed in a granular form along the mesentery area or capillary wall.Electron microscopy mainly showed swelling of glomerular endothelial cells,widening of the subendothelial space,diffuse or segmental foot process fusion,swelling of renal tubular epithelial cells,etc.The results were consistent with the performance of hemolytic uremic syndrome.(6)DNA tests:Six patients underwent genetic testing,with one case of CFHR5 gene mutation,one case of MMACHC gene mutation,and the remaining four cases no disease-related mutations.4.Treatment and recovery(1)Treatment:28 cases of HUS patients before November 2007 lacked a specific treatment plan in the acute phase,and only general symptomatic support treatment was given;40 cases of HUS patients after November 2007 began to adopt individualized Treatment options,including low-dose glucocorticoids,high-dose prednisolone shock,cyclophosphamide shock,and gamma globulin,etc.(2)Recovery:The recovery of the child was evaluated at 1 month of acute treatment.Before November 2007.the remission rate was 28.57%(8/28);the non-remission rate was 42.86%(12/28),including 10 deaths and illness Aggravated 2 cases.After November 2007,the treatment remission rate was 77.5%(31/40);the non-remission rate was 10%(4/40),including 1 death and 3 aggravated conditions.Compared with the recovery of 13 years before and after,the remission rate after adopting the individualized comprehensive treatment program was significantly improved(28.57%vs 77.5%).(3)Influencing factors analysis:A comparative analysis of the outcome at 1 month of the acute phase was taken.It was found that there were no statistical differences at the age of onset,days of onset,diarrhea,bloody stools,hypertension,convulsions,nervous system symptoms,coinfection,hemoglobin.C-reactive protein,erythrocyte sedimentation rate,aspartate aminotransferase alanine aminotransferase,albumin,urea nitrogen,blood creatinine,lactate dehydrogenase,Creatine kinase,creatine kinase isoenzyme,D-2 polymer and fibrinogen between the treatment remission group(39 cases)and the non-remission group(16 cases)(P>0.05).There was a statistically significant difference in the oliguria,complement C3,and the use of individualized comprehensive treatment plan(P<0.05).(4)Comparative analysis of 13 years before and after:Removing the influence of gender,age of onset,diarrhea,oliguria,convulsions,hypertension,hemoglobin,platelets,reticulocyte ratio,complement C3,indirect bilirubin,urea nitrogen,blood creatinine and lactate dehydrogenase(P>0.05),the impact of treatment changes in the acute phase on the outcome at 1 month was analyzed.It was concluded that the treatment remission rate of the individual comprehensive treatment plan in the past 1 3 years(2007.11?2019.9)was obvious higher than the first 13 years(1994.5?2007.10)with symptomatic support therapy,and the difference is statistically significant(P<0.05).Conclusion There are more boys than girls with HUS,and they are more common in preschool and school age.The onset season is distributed throughout the year and more common in summer and winter.Most children with HUS have symptoms of prodromal infection,with diverse clinical manifestations,and common damage to the extrarenal system.Comparing the outcomes in the 13 years before and after,it is found that the treatment efficiency has improved significantly and the mortality rate has decreased significantly,after using the individualized comprehensive treatment plan.Through statistical analysis,it is concluded that oliguria and hypocomplement C3emia may be risk factors affecting the recovery of children in the acute phase of treatment at 1 month.The use of individualized comprehensive treatment programs can improve the remission rate of HUS children at the acute stage of 1 month.