Juvenile generalized XXXX with @entity34693 mutation treated with short-term infliximab.

@entity1 @entity1667 @entity404 @entity1569 @entity66 @entity2905 @entity34693 @entity205 @entity1499 @entity71442
UNASSIGNED: A 8-year-old Chinese @entity1 with generalized @entity1569 (GPP) refractory to @entity205 and @entity1667 was treated successfully with two infusions of infliximab 3.3 mg/kg. He remained in remission for 21 months. Direct sequencing of @entity34693 gene showed a homozygous mutation, @entity71442 . Juvenile GPP is a rare severe form of @entity1569 occasionally associated with life-threatening complications. Like acitretin, @entity205 and @entity1499 , infliximab has been reported to be effective for juvenile GPP in case reports. However, there is a lack of data in the optimal treatment course of infliximab for juvenile GPP. Prolonged administration of these medications may cause @entity66 . We suggest that short-term infliximab regimen should be recommended as a choice for acute juvenile GPP refractory to traditional systemic therapies. WBC count and CRP are sensitive parameters to reflect the disease activity and evaluate the effectiveness of treatment. Monitoring @entity404 T lymphocyte count, preventing and correcting @entity404 @entity2905 are important in the treatment course of juvenile GPP.
