Effects of Traditional Chinese Medicine (GSJYF) in Children With Inherited Proteinuric Kidney Disease

Inclusion Criteria: Aged 3-18 years； Proteinuria associated with hereditary nephropathy, which was confirmed by mutations in podocyte genes or syndromal genes (including collagens); Urine protein more than 500 mg/24 hours and/or UPCR (in first-morning void) more than 0.5 mg/mg at time of baseline despite ACEI or ARB treatment for at least 3 months; Estimated GFR ≥ 45 ml/min/1.73m2 (estimated with Schwartz formula); Without any immunosuppressive medications such as corticosteroids, calcinrurin inhibitors, etc； On a stable dose of ACEI or ARB for at least 4 weeks； Willingness to give written consent and comply with the study protocol. Exclusion Criteria: Diagnosed with Congenital Anomalies of the Kidneys and Urinary Tracts (CAKUT), Nephronophthisis, Polycystic kidney disease etc; With abnormal liver function, ALT or AST >3.0 x upper limit of normal (ULN) at enrolment; Receiving immunosuppressive therapy (including corticosteroids, and other immune suppressive medicine) within three months prior to enrolment; Receiving other traditional Chinese medicine and/or its analogue which can reduce proteinuria within the past 2 weeks. Patients who are taking other TCM treatment could be enrolled after 2 weeks of wash out period; Has undergone major organ transplantation (e.g. heart, kidney, liver); Any medication, surgical or medical condition which might significantly alter the absorption, distribution, or metabolism including, but not limited to any of the following: active inflammatory bowel disease, received major gastrointestinal tract surgery; History of noncompliance to medical regimens or inability to comply with the study and follow-up procedures.