Amedco - Quiz
April 2018 JPGN
1 - You have been consulted by the newborn nursery on a 3-day old infant with marked unconjugated hyperbilirubinemia with most recent measurement of 32 mg/dL. You suspect Crigler-Najjar type I. The genetic defect(s) most likely to be found in this child is(are)?
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
2 - The parents of a 11-year-old child with CNI present to your office as part of a pre-transplant evaluation. Current treatment for the child include 14-16 hours per day of phototherapy although recent bilirubin levels have been increasing from ~14 mg/dL to ~20 mg/dL. The main indication to consider liver transplant in this child is?
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
3 - Which of the following describes the use providing donor liver cells to a recipient to replace a critical mass of metabolically normal cells with a functioning gene to support appropriate metabolic processes in children with monogenetic liver disorders?
A - Auxiliary partial orthotopic liver transplant
B - Hepatocyte transplant
C - Liver-directed gene therapy
D - Patient derived induced pluripotent stem cells
4 - Which disease processes would be least likely to benefit from hepatocyte transplantation?
A - Ornithine transcarbamylase (OTC) deficiency
B - Citrullinemia
C - Crigler-Najjar type I
D - Tyrosinemia
E - Biliary atresia
5 - Which of the following is a potential bias of the study?
A - Response bias
B - Funding bias
C - Sampling bias
D - Recall bias
6 - Which of the following statements regarding biologic infusion reactions in children is false?
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
7 - Which of the following statements is true regarding biologic infusions in non-hospital based vs hospital-based settings:
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
8 - A 13 year old girl with ulcerative colitis (UC) received her 4th maintenance infliximab infusion at home. During the infusion, she developed shortness of breath and throat swelling. The home care nurse administered epinephrine & diphenhydramine. Which of the following can ensure appropriate shared liability between provider and IHSA?
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
9 - A 17 year old girl with UC reported a sore throat and oral temperature of 101 to her IHSA nurse on the day of her maintenance biologic home infusion. The nurse noted pharyngeal erythema and was hesitant to start the infusion. What is the next best step to ensure patient safety?
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
10 - Which of the following pediatric patients would be the best candidate for consideration for transitioning to home-based infusions?
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
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