ABSTRACT
C3 glomerulopathy is a rare disease of glomeruli mediated by abnormal activation of alternative complement pathway secondary to congenital genetic defects and acquired autoantibodies.Renal biopsy is the gold standard for diagnosing C3 glomerulopathy.C3 glomerulopathy encompasses both dense deposit disease and C3 glomerulonephritis.The main glomerular immunofluorescence staining is C3, with few or without immunoglobulins deposition, which is the obvious pathological feature.The clinical manifestations of C3 glomerulopathy are usually various, with limited detection methods and therapies and poor prognosis.This article mainly reviews the progress of C3 glomerulopathy in recent years, in order to improve clinical understanding of C3 glomerulopathy, and choose individualized therapy.
ABSTRACT
Objective: To investigate the clinical symptom improvement time of the children with non-diarrhea-related hemolytic uremic syndrome (HUS) treated with glucocorticoids, and to evaluate its efficacy. Methods: A total of 22 children with non-diarrhea-related HUS were selected as the subjects. According to whether glucocorticoid was used in combination of plasma in the actue stage of the children, the children were divided into simple plasma group (n=ll) and plasma combined with glucocorticoid treatment group (n=ll). The average hospital stays, the time of hematuria, the time of platelets and serum creatinine to recover to the normal levels of the children in two groups were analyzed. The clinical material of children in two groups after hospital discharge were collected, and the prognosis of the children in two groups was evaluated according to the follow-up results. Results: Compared with simple plasma group, the average hospital stays, and the time of hematuria and the time of serum creatinine to recover to the normal level of the children in plasma combined with glucocorticoid treatment group were shortened, but there were no significant differences (P>0. 05). The time of platelets rising to the normal level was similar had no significant difference between two groups (P>0.05). Ten cases completed the follow-up in simple plasma group; one children suffered hypertension during follow-up, and one case replapsed 1 year after operation. In plasma combined with glucocorticoid treatment group, nine cases completed the follow-up; one children persistented urinalysis abnormalities and hearing loss, with the growth and development being slower than their peers; the remaining children all achieved the clinical cure standards. The clinical cure rate of the chidren in plasma combined with glucocorticoid treatment group was 88. 9%, while it was 80. 0% in simple plasma group; there was no significant difference in the clinical cure rate between two groups (P> 0.05). Conclusion: Using glucocorticoids in the acute stage of non-diarrhea-related hemolytic uremic syndrome in the children could not shorten the acute course and improve the prognosis of the children.