The clinical records of 55 consecutive pediatric patients with biopsy-proven MCN treated in our hospital from 1984 to 2004 were reviewed to evaluate the disease pattern, treatment regimen, and the development of treatment-related complications.
Of the 55 patients treated, 35 were followed after age 18 years; 13 (37 % ) had relapses during adulthood. Treatment-related complications were observed in 20 patients (57 % ), including being overweight (23 % ), impaired fasting glucose (14 % ), infertility (14 % ), persistent low grade proteinuria (11 % ), fracture (9 % ), and hypertension (9 % ). All patients had normal renal function when last assessed.
A substantial proportion of patients with childhood-onset MCN continued to have relapse after they became adults. Although almost all patients have normal renal function, prolonged use of steroid and immunosuppressive agents results in a considerable risk of treatment-related complications. Life-long follow-up seems advisable for this group of patients, not only for detecting relapses, but also to allow timely diagnosis of treatment-related complications.