Re-treatment regimens for acute stage of Kawasaki disease patients who failed to respond to initial intravenous immunoglobulin therapy: Analysis from the 17th nationwide survey

BACKGROUND: Current regimens for re-treatment of patients with Kawasaki disease who failed to respond to the initial intravenous immunoglobulin (IVIG) therapy are still uncertain. The purpose of this study is to reveal what regimens were used as the initial therapy and re-treatment for acute stage of Kawasaki disease in the current Japanese medical setting. METHODS: The 17th nationwide survey on Kawasaki disease covered patients whose onset was in 2001 and 2002. In questionnaires sent to all hospitals with a bed capacity of 100 or more and a pediatric department, several questions related to therapeutic regimens for Kawasaki disease were posed. The authors observed the proportions of hospitals that had regimens for patients who failed to respond to the initial therapy. RESULTS: Among those hospitals that responded to the survey, 1052 (64.1%) reported that at least one patient with Kawasaki disease visited the hospital. Among these 1052 hospitals, 73.3% had a regimen to administer 30-39 mg/kg per day of oral aspirin with initial IVIG. The proportion of hospitals that used 1 g/kg per day of IVIG for 2 days was the largest among the options for the initial treatment. For those patients who fail to respond to the initial therapy, 464 hospitals (44.1%) reported that their pediatricians would use additional IVIG only. The number of hospitals that planned to administer high-dose IVIG and ulinastatin was 185 (17.6%). The number of hospitals having regimens of additional IVIG and steroids was 54 (5.1%). CONCLUSIONS: The current status of the treatment for patients with Kawasaki disease not responding to the initial IVIG therapy in Japan was revealed. A randomized trial of a large sample is needed to ascertain the effectiveness of several options for re-treating Kawasaki disease.     

Improvement of stone comminution by slow delivery rate of shock waves in extracorporeal lithotripsy

AIM: The aim of this study was to investigate the effect of delivery rate of shockwaves (SW) on stone comminution and treatment outcomes in patients with renal and ureteral stones. METHODS: Patients with radio-opaque stones in the upper urinary tract that were treated by extracorporeal shock wave lithotripsy (ESWL) were divided into two groups according to delivery rate (120 or 60 SW/min). The effective fragmentation after one ESWL session and treatment success at 3 months after ESWL was compared between the two groups. RESULTS: Of 134 patients (84 men and 50 women), 68 patients were treated at a fast rate and 66 were treated at a slow rate. Thirty and 38 patients in the fast rate group and 28 and 38 in the slow rate group had renal and ureteral stones, respectively. After one ESWL session, effective fragmentation was noted more often in the slow group (65.2%) than the fast group (47.1%) (P = 0.035), particularly for smaller stones (stone area <100 mm(2)) (P = 0.005) and renal stones (p = 0.005). However, there was no significant difference in treatment success at 3 months after ESWL between the two groups. In univariate logistic regression analysis, slow SW rate and smaller stones were significant factors for effective fragmentation after one ESWL session. In multivariate analysis, slow SW rate and smaller stones were also independent factors. CONCLUSIONS: Slow SW rate contributed to better stone comminution than fast rate, particularly for small stones and renal stones. ESWL treatment at a slow SW rate is recommended to obtain efficient stone fragmentation.     

Urinary pregnanetriol-3-glucuronide in children: Age-related change and application to the management of 21-hydroxylase deficiency

Urinary concentrations of pregnanetriol-3-glucuronide (PT-3-G) were determined in 485 normal Japanese subjects (277 males and 208 females), aged 5 days to 20 years, using an enzyme-linked immunosorbent assay (ELISA). The usefulness of urinary PT-3-G concentrations before giving the morning dose of medications in monitoring the adequacy of glucocorticoid treatment was assessed in eight patients with 21-hydroxylase deficiency (21-OHD). The ratio of PT-3-G to excreted creatinine (PT-3-G/Cre ratio) increased significantly during the first month and did not change from age 1 month to 1 year of life. The ratio decreased to a nadir at age 3 or 4 years followed by continuous, significant increase until late adolescence. In the subjects treated with corticosteroids for 21-OHD, PT-3-G/Cre ratios at the 50th percentile or below suggested a risk of excessive treatment, as judged by the patients' growth. Measurement of the PT-3-G/Cre ratio enabled recognition of corticosteroid overtreatment, which was not demonstrated by determining the serum concentrations of 17α-hydroxyprogesterone (17-OHP) On the other hand, ratios at the upper 95–99% tolerance limits seemed to be required for optimal control. The present study revealed the normal age-related changes in urinary excretion of PT-3-G and showed it to be a reliable marker for evaluating glucocorticoid treatment in young children with 21-OHD.