10-1.

You are seeing a 26-month-old boy with ESRD secondary to dysplastic kidneys,
currently managed on peritoneal dialysis. He is in the clinic with his parents for kidney
transplant evaluation. His parents are asking about complications of transplant and would
like to know his risk factors for development of post-transplant lymphoproliferative disorder
(PTLD). Which of the following is an associated risk factor for development of PTLD?
a. EBV seronegative status pretransplant
b. Female gender
c. Age >5 years old
d. CMV seropositive status pretransplant
e. Asian race
10-2. A 13-year-old girl presents to your clinic with low- grade fever and URI symptoms for 3
days. She has a history of end-stage kidney disease secondary to reflux nephropathy, s/p
transplant 6 years ago. Her kidney function has been stable over the past few years with
a baseline creatinine of 0.8. Her medications include prednisone, tacrolimus mycophenolate
mofetil (eg, CellCept, myfortic) and ferrous sulfate. She does not require any medicine for
blood pressure. For her acute illness, you decide to start her on treatment with
azithromycin. Four days later, her Mom calls to report that her fever and respiratory
symptoms have improved, but now she is feeling shaky and her blood pressures have
increased from her baseline of 110s/70s to 140s/90s. Her diet has not changed and
she continues to drink her usual 2.5 L of fluid daily. Her Mom is concerned about her kidney
function so you order labs with the following results: Sodium 138, potassium 6, Chloride
102, CO2 22, BUN 27, creatinine 1.2, glucose 70, calcium 9, phosphorus 4.2.
What is your next step in evaluation of this patient? a. Obtain a history of caffeine
consumption
b. Order a renal ultrasound with dopplers
c. Check a tacrolimus level
d. Check fractionated serum metanephrines
e. Check serum and urine electrolytes for a FeNa
10-3. You are seeing a 4-year-old boy in your primary care clinic for routine well child care.
He has a history of hypoplastic left heart and he received a heart transplant at 18 months of
age. His transplant is functioning well and his cardiologist is happy with his progress. His
current medications include prednisone and tacrolimus. Mom is asking whether he needs
any vaccines today. You review his chart and note that he is due for a number of vaccines.
Which of the following vaccines is contraindicated for this patient?
a. DTaP
b. MMR
c. PPSV
d. IPV
e. MCV4
10-4. All of the following medications may contribute to the development of renal
dysfunction in patients with solid organ (heart, liver, kidney, intestine) or hematopoietic cell
transplant EXCEPT...
a. Tacrolimus
b. Ganciclovir
c. Sirolimus
d. Vancomycin
e. Mycophenolate mofetil
10-5. The mother of an 8-year-old boy with a history of biliary atresia, s/p liver transplant at
7 months of age, calls your clinic. Her son was at a sleepover party 2 nights ago with a friend
who was diagnosed with chicken pox the next day. His current immunosuppression includes
tacrolimus and prednisone. The mom reports her son is well, without any signs of illness.
What is the appropriate management of this patient?
a. Monitor for chicken pox lesions and give varicella immune globulin if he develops any
b. Measure serum IgM and IgG levels to varicella and prescribe immune globulin if IgM
positive
c. Prescribe antibiotics to prevent superinfection of any developing varicella lesions
d. Administer varicella immune globulin and continue to monitor for signs of illness
e. Monitor for signs of chicken pox and treat symptoms of illness
10-6. A 7-year-old girl presents to the emergency department (ED) with severe diarrhea for
the past 4 days. She received a kidney transplant 5 years ago for dysplastic kidneys.
Her mother reports she has been maintaining her usual daily fluid goal of 2 L and drinking
above that to keep up with the diarrhea. Her urine output has not changed. Her mother
reports that she has also been more hypertensive over the past 24 hours, with blood
pressures ranging in the 130s mm Hg systolic, though she is usually stable in the 100–110s
mm Hg systolic. She has continued to take all her usual immunosuppression medications
including prednisone, tacrolimus, and cellcept. You obtain the following labs in the ED:
Sodium 134, potassium 6, chloride 94, HCO3 16, BUN 48, creatinine 0.8 (baseline 0.5),
glucose 82, calcium 11, phos 5.8. Stool studies are pending.
Which of the following pieces of information will help you diagnose the most likely cause of
this child’s acute kidney injury?
a. Serum tacrolimus level
b. Kidney biopsy
c. Transtubular potassium gradient
d. Stool studies
e. Urine culture
10-7. You are seeing a 15-year-old boy for routine well-child care. He has a history of liver
transplant 10 years ago for Alagille syndrome. He is maintained on prednisone and
tacrolimus for immunosuppression.
For this patient on chronic immunosuppression, which of the following
examinations/evaluations is NOT required on a routine basis?
a. Eye exam
b. Skin exam
c. Blood pressure measurement
d. Lipid panel
e. Hearing evaluation
10-8. A 13-year-old boy s/p liver transplant 9 years ago comes to your office with complaint
of daily right hip pain for the past few weeks. His liver function was stable when checked 1
month ago. He had a runny nose and fever a week ago, but this has resolved. His current
immunosuppression consists of daily prednisone and tacrolimus. He is on a regular daily
multivitamin. His mother is concerned that “he is short” compared to all his friends. Physical
exam is remarkable for height and weight <3rd percentile for his age and mild pain with
internal and external rotation of his right hip, otherwise it is normal.
Of the following, you are most concerned that this boy’s symptoms are being caused by the
following conditions?
a. Septic arthritis due to immunosuppression
b. Chronic steroid use
c. Chronic malnutrition
d. Medication nonadherence and graft failure
e. Growth hormone deficiency
10-9. An 11-month-old boy underwent liver transplant for cholestatic liver disease 6 days
ago. He received a deceased-donor split liver. Initially his LFTs were trending down, but in
the past day they have started to trend up and his total bilirubin level is also rising. This
morning he developed a fever and abdominal distension, thus he was made NPO. An
abdominal ultrasound is ordered, which demonstrates abdominal free fluid and minimal
arterial signal on doppler evaluation over the hepatic artery.
What is the PRIMARY process leading to these complications?
a. Acute cellular rejection
b. Acute bacterial cholangitis
c. Hepatic artery thrombosis
d. Primary graft nonfunction
e. Post-transplant coagulopathy
10-10. A 15-week-old boy presents to the emergency room for evaluation of poor appetite,
weight loss, abdominal distension, and decreased energy. Labs reveal elevated AST, ALT,
and total bilirubin. Stool patterns have changed and are now described as “pale” in color.
What is the most common reason for liver failure and need for liver transplantation in
patients this age?
a. Wilson disease
b. Autoimmune hepatitis
c. Viral hepatitis
d. Biliary atresia
e. Tylenol-induced hepatic necrosis
10-11. You are seeing a 16-year-old boy for follow-up in a transplant clinic. The patient had
a history of end- stage renal disease of unknown etiology, and had been anuric, managed on
hemodialysis for 6 months. Two weeks ago he received a living unrelated kidney transplant.
The surgery was uncomplicated and his creatinine came down from a pretransplant level of
4.5 mg/dL to 0.7 mg/dL at the time of discharge. He was discharged 1 week ago and
presents today for his first follow-up visit. He reports he is drinking 2.5 L of fluid daily,
voiding every few hours, and taking all his medications as directed. He reports that he is
feeling well without any fevers or dysuria, but he has had lower extremity edema for the
past 2 days. His labs demonstrate the following:
Serum: Na 136, K 4, Cl 101, CO2 23, BUN 27, Cr 1.0, Alb 2.4, glu 91, Ca 8, phos 3.2, C3 115
(normal) Urine: S.G. 1.015, (+) LE, 500 protein, 25 blood. Statistically, which of the following
was the most likely
cause of this patient’s initial kidney failure?
a. Membranoproliferative glomerulonephritis
b. Hemolytic uremic syndrome
c. IgA nephropathy
d. Focal segmental glomerulosclerosis
e. Anti-GBM nephritis
10-12. You are seeing a 17-year-old girl in your primary care clinic for evaluation for
depression. She has a history of end-stage kidney disease secondary to renal dysplasia and
received a deceased donor kidney transplant at age 2. Over the past few months, she has
been having increasing fatigue and depression. She agrees school has been particularly
stressful lately as she has been working on college applications, preparing for college
entrance examinations, and participating on the swim team. As a result, she had not
followed up with her nephrologist in the last 6 months. When she went back last week, her
nephrologist told her that she would have to “go on dialysis soon.” She reports that she has
been adherent with her transplant medications and meets her daily total fluid goal of
2.5 L. She has been eating, voiding, and stooling normally. She is upset and does not
understand why her kidney is failing. Statistically, which of the following is the most likely
cause for graft loss?
a. Chronic allograft nephropathy
b. Acute rejection
c. Medication nonadherence
d. Vascular thrombosis
e. BK nephropathy
10-13. A 5-year-old boy comes in with intestinal failure and evaluation for intestinal
transplant. In discussing outcomes with the family you explain that 1 year outcomes for
intestinal transplant recipients are excellent at around 90% but the 5-year survival is only
about 60%. You also explain that the leading cause of death in intestinal transplant patients
is:
a. Rejection of intestinal allograft
b. Post-transplant lymphoproliferative disease
c. Infection
d. Surgical complications
e. Cardiac arrest due to electrolyte complications
10-14. A 15-year-old girl with a history of T-cell lymphoma received a hematopoietic stem
cell transplant after a myeloablative conditioning regimen 100 days ago. Her early course
was unremarkable except for mild CMV infection. She presents to the office with a cough,
low grade fever, and dyspnea. She has tachypnea and her chest radiograph is normal. She
reports she has been missing one of her medications for the past 2 months.
This medication is most likely:
a. Acyclovir
b. Ferrous sulfate
c. Prednisone
d. Tacrolimus
e. Trimethoprim-sulfamethoxazole
10-15. A 17-year-old boy develops dyspnea and exercise intolerance 5 years after receiving
a cardiac transplant for a dilated cardiomyopathy. He and his family have been adherent
with his immunosuppressive regimen and he has had an uneventful course except for an
early CMV infection. His EKG shows ischemic changes and his ECHO shows decreased
function with focal wall abnormalities. He undergoes a cardiac catheterization and biopsy.
Which of the following processes is most likely to be present?
a. Acute cellular rejection
b. Carnitine deficiency
c. CMV cardiomyopathy
d. Graft vasculopathy
e. Recurrent disease
10-16. A 5-year-old boy with a history of ALL who failed to attain remission received an
allogeneic stem cell transplant from his non-HLA identical sibling 2 years ago. He had an
early complicated course with moderate sinusoidal obstruction and acute GVHD. At this
time, he demonstrates weight loss, rash, restrictive lung disease, and elevated liver
enzymes. Bowel biopsy shows chronic GVHD. He does not respond to methylprednisone and
is placed on mycophenolate mofetil. In talking with the family, you can tell the family that
they can expect that 1 year from now:
a. His disease may progress despite treatment b. He has over a 80% chance of being alive
c. He will be off antibiotics
d. He will be off immunosuppression
e. Other organs will not become involved
10-17. A 4-year-old with a history of acute lymphoblastic leukemia who has relapsed is
being evaluated for hematopoetic cell transplant. His parents, along with a twin sibling and
2 other older siblings, are potential donors. Which source of stem cells will present the
highest risk of graft versus host disease:
a. Bone-marrow transplant from an unrelated donor with a HLA genetic match
b. Bone-marrow transplant from an identical twin
c. Cord blood transplant from an unrelated donor with a HLA antigen match
d. Peripheral blood stem cell transplant from a sibling with a HLA antigen match
e. Peripheral blood stem cell transplant using pooled donations from parents
10-18. A 15-year-old girl who had a stem cell transplant at 20 months of age comes in for
well child care. She was transplanted for a nonmalignant condition without prior
chemotherapy. She had problems with chronic GVHD that required prolonged steroid
therapy. In reviewing the potential late effects of her transplant, the least likely
complication would be:
a. Alopecia
b. Cataracts
c. Deceased bone mineral density
d. Pulmonary disease
e. Thyroid disorder
10-19. The wait-list mortality in pediatric solid organ transplant varies by organ and age.
Overall, which organs represent the highest and lowest waitlist mortality, respectively?
a. Heart highest, liver lowest
b. Heart highest, kidney lowest
c. Intestine highest, kidney lowest
d. Intestine highest, liver lowest
e. Liver highest, intestine lowest
10-20. A 16-month-old girl with a history of liver failure due to biliary atresia, status post
liver transplant 2 months ago, presents for routine follow-up. Her post-transplant course
has been fairly unremarkable. Her routine labs are all within normal limits apart from a
haemoglobin of 8.8 and WBC 2.7. She has no evidence of active bleeding and stool
hemoccult is negative and no evidence of active infection. You suspect that one of her
medications is causing the anemia. Which of the following medications prescribed to this
patient is least likely to be responsible for this anemia?
a. Trimethoprim and sulfamethoxazole (Bactrim)
b. Fluconazole
c. Tacrolimus
d. Mycophenolate mofetil
e. Valganciclovir (Valcyte)
Key
1. A
2. C
3. B
4. E
5. D
6. A
7. E
8. B
9. C
10. D
11. D
12. A
13. C
14. E
15. D
16. A
17. E
18. A
19. C
20. B