Amedco - Quiz

April 2018 JPGN


A   - ABCC2 mutation affecting the multidrug resistance protein 2 (MRP2)
B   - UGT1A1 exon mutations affecting the UGT1A1 protein
C   - ATP8B1 mutation affecting the FIC1 protein
D   - TATA box mutations affecting the promoter region of the UGT1A1 gene
E   - Both B and D

A   - To prevent the development of irreversible neurological injury
B   - To enable improved quality of life
C   - To prevent the development of other complications of end-stage liver disease such as coagulopathy and portal hypertension
D   - To decrease amount of needed phototherapy to manage his disease with goal of less than 10 hours a day

A   - Auxiliary partial orthotopic liver transplant
B   - Hepatocyte transplant
C   - Liver-directed gene therapy
D   - Patient derived induced pluripotent stem cells

A   - Ornithine transcarbamylase (OTC) deficiency
B   - Citrullinemia
C   - Crigler-Najjar type I
D   - Tyrosinemia
E   - Biliary atresia

A   - Response bias
B   - Funding bias
C   - Sampling bias
D   - Recall bias

A   - Mild infusion reactions occur in 5- 10% of children who receive intravenous biologics
B   - Younger children, including patients with very early onset IBD are less likely to have infusion reactions
C   - Infusion reactions are best handled through a standard clear protocol communicated and agreed upon before the infusions begin in any setting
D   - The majority of infusion reactions can be safely handled with simple practical steps and medications given in any setting including home-based infusions

A   - Existing regulations in the United States do not mandate the presence of a certified pediatric nurse to administer home infusions
B   - Data show higher risk of infusion reactions when administered at home- vs hospital-based setting
C   - Cost of biologic infusion is similar regardless of which setting the infusion is administered
D   - Home infusions are contraindicated in children

A   - Select an IHSA that is closest to the patient’s home
B   - Provide the family with on-call contact information for the ordering pediatric GI group
C   - Agree upon an infusion protocol and infusion reaction protocol with the IHSA
D   - Document reactions that are reported by the family

A   - Administer diphenhydramine
B   - Cancel the infusion and reschedule
C   - Suggest the patient start antibiotics and contact their provider
D   - Notify the ordering provider at the contact number provided to inquire about next steps

A   - 12 year old female with Crohn’s disease in remission on maintenance infliximab infusions for the last 4 years with stable infliximab level and no antibodies to infliximab (mobility shift assay)
B   - 10 year old male with IBD-unspecified with significant improvement after his first 2 induction doses now due for 3rd induction dose of infliximab
C   - 15 year old male with UC diagnosed at age 9 on maintenance infliximab who has a recent infliximab level (mobility shift assay) of 25ug/ml and antibodies to infliximab 250ug/ml
D   - 2 year old female with stable Crohn’s disease in remission on maintenance infliximab infusions