Long-term follow-up study of IgM associated nephrotic syndrome patients: clinical outcome and prognostic indicators.

RATIONALE: Even though it is the most common primary glomerular disease, the natural history and prognosis of IgM associated nephrotic syndrome have not been well established. OBJECTIVES: To determine the (1) responsiveness to prednisolone therapy, (2) long-term clinical and laboratory outcomes, and (3) prognostic indicators to prednisolone therapy in patients with IgM associated nephrotic syndrome. STUDY DESIGN: Clinical descriptive, longitudinal study. SUBJECTS: Seventy two biopsy-proved IgM associated nephrotic syndrome patients, diagnosed between 1978-1996 at Vajira Hospital, Bangkok, were included in the study. METHOD: Clinical parameters with age, sex, duration of edema, blood pressure and laboratory findings such as hematuria, BUN, creatinine, albumin, and cholesterol, 24-hour urine protein, and stool examination, were collected pre-renal biopsy. Each patient was treated with 45-60 mg of prednisolone according to body weight, for up to 8 weeks. Each patient was followed-up every 4-weeks for clinical and laboratory evaluations, and for adjusting the steroid dosage. Clinical responses were stratified into 3 groups as steroid responsive (SRP), steroid dependent (SD), and steroid resistant (SRS). MAIN OUTCOME MEASURES: (1) Frequency and types of steroid responsiveness. (2) Incidence of hypertension, hematuria, renal insufficiency, end-stage renal disease, and survival during the follow-up. (3) Prognostic indicators for initial clinical response to prednisolone, and for long-term morbidity and mortality. RESULTS: Forty eight of the 72 patients (66.67%) were responsive to prednisolone at 8-weeks, the 24 remaining patients (33.33%) were nonresponsive. High proteinuria of 7.66 +/- 4.14 g/D was the only good prognostic indicator to initial prednisolone therapy (p < 0.03). During the follow-up, 42(58.33%), 26(36.11%), and 4(5.56%) patients were SRP, SD, and SRS, respectively. There were no prognostic indicators associated with long-term steroid responsiveness. Of the 60 patients followed-up for more than one year; 34, 15 and 11 patients were followed-up for 1-5, > 5-10 and > 10 years, respectively. Hematuria and proteinuria were more frequent among the SRS group (p < 0.01 and 0.02, respectively) during the follow-up. Only one patient, initially in the SD group, and later on became SRS, died. CONCLUSION: Patients with IgM associated nephrotic syndrome had very good response to prednisolone therapy. It had a very slow progressive course, with low morbidity and mortality.

Paget's disease of bone--clinico-pathology study of the first case report in Thailand.

Paget's disease of the bone is rare in Asia. We report a case of Paget's disease with clinico-pathology study and it appears to be the first case in Thailand. The case was a 44-year-old female of Chinese descent who developed pain in the left hip and sacral area for one year prior to admission to Vachira Hospital. The earlier clinical diagnoses were either osteoporosis or metastatic tumor. Biopsy of the right iliac bone was typical for Paget's disease of the bone.