The guideline on the dosing of Epoprostenol sodium (PGI
2) was established based on data for adults. In order to study the dosing regimen in children in Japan, we followed-up 7 patients under 16 years old with primary pulmonary hypertension (PPH) who received PGI
2 and analyzed their doses, hemodynamic parameters and brain natriuretic peptide (BNP) levels. The mean initial dose of PGI
2 was 2.0±0.7 ng/kg/min and increases in the PGI
2 dosage occurred mostly within 2 weeks of the start of administration. Up to 28 days after starting administration, the mean interval between dose increases was 2.9±3.3 days and the mean dosage increase increment was 0.8±0.4 ng/kg/min. Up to 3 months after starting administration, there was no statistically significant difference in doses between adults and children. Among severe adverse effects reported in acute dose-ranging in Japan, 7 of 18 cases were in children and the initial doses in 4 of them were over 2 ng/kg/min. Based on these findings, appropriate dosing of PGI
2 in children is considered to be as follows : 1) The initial dose should be 0.5 ng/kg/min, 2) The dosage increase interval should be over 2 days and the dosage increase increment between 0.5 and 1 ng/kg/min, 3) Up to 3 months, the dose in children may be increased to the same extent as in adults.
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