Clinical Analysis Of 39 Children With Non-diarrhea Hemolytic Uremic Syndrome

Objective: To study clinical feature,molecular genetics,treatment and prognosis of the non-diarrhea hemolytic uremic syndrome(D-HUS).Methods: Totally 39 patients with D-HUS in children's hospital of Chong Qing Medical University were retrospectively reviewed and the clinical data were collected.Next generation sequencing and Sanger sequencing were used for complement genes screening.Quantitative data was expressed by mean value±standard deviation or median.Results: 1.D-HUS was sporadic and no familial case was found in our data.The male-female ratio was 1.29.The mean age of onset was 6.10±3.58 years old and school-aged children count most.2.Acute renalfailure,hemolysis and hemorrhage were main clinical manifestations.Gastrointestinal symptoms and neurological symptoms were common extra-renal manifestations.3.There was one patient who was diagnosed as Streptococcus pneumoniae–HUS.5 patients were screened for complement genes.Underlying genetic abnormalities CFI mutation,CFHR4 mutation and suspicious CFHR1/CFHR4 deletion were uncovered respectively in 3patients and all associated with disease.4.6 patiens received renal biopsybecause of atypical progression or continuous renal dysfunction,4 of which had pathologic changes of typical thrombotic microangiopathy.5.Plasma treatment,hemopurification,anti-infection and symptomatic treatment were the main treatments in acute phase of D-HUS in our hospital.6.Clinical outcome of hospitalization: 2 patients died in acute phase,and the causes of death were multiple organ failure and cerebral hernia respectively.1 patient was discharged with hematologic remission,and relied on occasional hemodialysis after discharge because of severe renal pathologic changes;1 patient progressed to ESRD,relying on renal replacement therapy;3 patients asked to be discharged without remission due to economic factors;The rest 32 patients were discharged with static hemolysis,anemia correction,and recovered thrombocytopenia and renal function.A total of 19 patients were followed up,14 patients had complete remission,2 patients had partial remission,1 patient had recurrence and 1patient relied on peritoneal dialysis at their last follow-up visit.1 patient died soon after hospital discharge.Conclusions: With comprehensive treatments including plasma treatment,hemopurification,symptomatic treatment and so on,most patients with D-HUS can achieve significant curative effect,and the long-term prognosis depends on the etiology.After clinical diagnosis of D-HUS,it is necessary to carry out the complement factor defect and pathogen screening as early as possible to clarify the etiology,guide thetreatment and evaluate the prognosis.