[Early initiation of peritoneal dialysis for the treatment of a patient with refractory nephrotic syndrome].

ABSTRACT

A proportion of patients with refractory nephrotic syndrome (NS) due to focal segmental glomerulosclerosis (FSGS) often require long-term hospitalization, which results in the loss of quality of life (QOL) of the patients. An-11-year-old girl was brought to a regional hospital with an 8-day history of generalized edema. Laboratory studies revealed massive proteinuria without hematuria and marked hypoalbuminemia associated with hyperlipidemia. A percutaneous renal biopsy revealed lesions characteristic of FSGS. Although methylprednisolone pulse therapy was administered followed by oral prednisolone combined with cyclosporine A, heavy proteinuria persisted for the next 4 months. The patient was referred subsequenthy to our hospital for further examinations. High-dose intermittent mizoribine pulse therapy, LDL-apheresis, cyclosporine A, tacrolimus and intravenous cyclophosphamide pulse therapy proved to be partially effective. As a result, long-term hospitalization and intravenous administration of albumin, diuretics and immunoglobulin was required for this patient. Also, she developed severe steroid toxicity such as obesity, cataract and osteoporosis. A third renal biopsy revealed an advanced stage of FSGS lesions, suggesting subsequent development of end-stage renal disease (ESRD). Since the patient suffered from long-term hospitalization over 3 years and significant loss of QOL, we therefore proposed early initiation of peritoneal dialysis (PD), as in cases of congenital NS, in order to preserve the patient's general condition without intravenous drug administrations and for the preparation of a future renal transplantation. After obtaining informed consent from the patient and her family, the initiation of PD was performed at the age of 15 years before the ESRD state(serum creatinine level 2.0 mg/dL). The patient was successfully free from intravenous drug administrations and hospitalization, and the QOL was significantly improved thereafter. We, therefore, suggest that early initiation of PD might be a treatment of choice for elected patients with severe refractory nephrotic syndrome such as this presented case.