primary nephrotic syndrome
Conditions
Interventions
Case series:NA
Sponsors
Children's Hospital of Chongqing Medical University
Eligibility
Sex/Gender
All
Age
No minimum to 18 Years
Inclusion criteria
Inclusion criteria: 1. An onset age from birth to 18 years; 2. A diagnosis of PNS according to the following criteria: (1) nephrotic proteinuria: proteinuria >=50mg/kg or morning urine protein/creatinine (mg/mmol) >=200.0 in 24hours, (2) hypoproteinaemia: serum albumin less than 25g/L; (3) hyperlipidaemia: serum cholesterol higher than 5.7mmol/L; (4) different degrees of oedema. (1) and (2) of the above four items are necessary for diagnosis. Patients with SSNS or FRNS/SDNS should respond to prednisone or prednisolone within 4 weeks,and FRNS/SDNS should also meet the standard of two consecutive relapses during the reduction of corticosteroid therapy or within 2 weeks of discontinuation of corticosteroid therapy and more than two relapses within a 6month period or relapse for more than 4 of 12 months during the course of the disease. Patients with SRNS exhibit persistent proteinuria despite that 60 mg / (m2*d) dose of prednisone is administered orally for 4 weeks, followed by a dose of 40 mg / m2 intermittently for 4 weeks according to the standard International Study of Kidney Disease in Children (ISKDC).
Exclusion criteria
Exclusion criteria: (1) Patients with chronic kidney disease(CKD)caused by factors such as congenital nephrotic syndrome or secondary nephrotic syndrome (allergic purpura, lupus, hepatitis B, diabetes, hereditary nephritis, severe infection, tumour and drugs); (2) Patients with a positive genetic test associated with podocytopathies.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| WES;eGFR; | — |
Secondary
| Measure | Time frame |
|---|---|
| histopathological outcomes; | — |
Countries
China
Contacts
Public ContactQiu Li
Department of Nephrology, Key Laboratory of the Ministry of Education, Children's Hospital of Chongqing Medical University
Outcome results
None listed