ABSTRACT
OBJECTIVE: To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment. METHODS: Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN). RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination. Proteinuria cleared in all 11 cases through treatment with steroids and/or immunosuppressive drugs. However, half of the DEP-HSPN patients continuously had hematuria after treatment. CONCLUSION: The early diagnosis and prompt initiation of immunosuppressive treatment based on renal biopsy are important for achieving favorable outcomes.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Nephritis/drug therapy , Nephritis/pathology , IgA Vasculitis/drug therapy , IgA Vasculitis/pathology , Biopsy , Glucocorticoids/therapeutic use , Hematuria , Immunosuppressive Agents/therapeutic use , Kidney/pathology , Prednisone/therapeutic use , Proteinuria , Treatment OutcomeABSTRACT
OBJECTIVES: Familial steroid-sensitive idiopathic nephrotic syndrome is rare, and only approximately 3% of patients have affected siblings. METHODS: Herein, we report seven cases of patients with steroid-sensitive idiopathic nephrotic syndrome from three Chinese families. Mutational screening of the Nphs2 gene was performed in all the patients. RESULTS: All seven of the familial steroid-sensitive idiopathic nephrotic syndrome cases in our sample exhibited minimal change disease, and one case also presented with mesangial proliferative glomerulonephritis, according to the renal pathology. No significant was associations were found between Nphs2 gene mutations and the onset of proteinuria and nephrotic syndrome in these familial cases. CONCLUSIONS: The presence of minimal change disease is important, but it is not an unusual finding in patients with familial steroid-sensitive idiopathic nephrotic syndrome, which appears to be clinically benign and genetically distinct from other types of nephrosis. .
Subject(s)
Adolescent , Adult , Child , Child, Preschool , Female , Humans , Male , Intracellular Signaling Peptides and Proteins/genetics , Membrane Proteins/genetics , Mutation/genetics , Nephrotic Syndrome/genetics , Polymorphism, Genetic/genetics , Rare Diseases/genetics , China , Nephrotic Syndrome/pathology , Pedigree , Rare Diseases/pathologyABSTRACT
IgM nephropathy presents with refractory nephrotic syndrome and its treatment is a significant challenge for pediatricians. We present two patients with IgM nephropathy and frequently relapsing nephrotic syndrome treated with rituximab and subsequently mycophenolate mofetil. Both showed complete remission, which 24 to 30 months later, was still maintained. The role of mycophenolate mofetil therapy in maintaining remission after successful treatment of rituximab in IgM nephropathy needs to be examined.