BCPPS Practice Questions Set #4

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You're processing chemotherapy orders for a 2 year old girl with a newly diagnosed brain tumor. She has the co-morbidity of hydrocephalus with increased intracranial pressure. You recognized that the admixture for her etoposide dose of 90 mg will need to have a concentration of no more than 0.4 mg/mL to give it adequate stability. Due to desire for limited fluid load in the patient, the oncologist says her increased fluids should be no more than 115 ml/hr. What would you present as a strategy to satisfy the pharmaceutical limitations and the clinical management of the patient?

90 mg / 0.4 mg/mL = 225 mL; 225 mL/ 115 mL/hr = ~2 hours; run etoposide over 2 hours (usually run over 1 hour, but can extend if needed)

An 11 year old girl with Hgb SS has had recurrent admissions to the hospital mostly due to acute painful episodes, but also due to pneumonia, and worsening avascular necrosis of her hip. A recent scrrening test for cerebral vascular health indicates she's at risk for stroke. Which of the complications above could she expect to lessen after a month of 2 of hydroxyurea?

Acute painful episodes · Hydroxyurea reduces painful episodes, need for RBC transfusions, acute chest syndrome, and dactylitis · Deferasirox is an iron chelator used in patients that need chronic RBC transfusions that helps prevent strokes

A 5 year old boy weighing 28 kg with severe hemophilia A has come into the ED with signs and symptoms of a major muscle bleed after blunt trauma. Knowing that an early goal of therapy with a factor VIII infusion is to raise the circulating factor VIII to 100%, you are asked to calculate a dose in units. Patient is not known to have inhibitors to FVIII. Available vial sizes of FVIII are 1050 units and 230 units. Dosing of FVIII in patients without inhibitors: 1 unit/kg is expected to raise serum level by 2%.

Dosing of FVIII in patients w/o inhibitors: 1 unit/kg is expected to raise serum level by 2%. 50 units/kg would raise by 100%. Dose =1400 units, rounded down to nearest vial size of 1050 + 230 = 1280 units. · Severe hemophilia: <1% circulating clotting factor compared to normal individual · Moderate: 1-5% circulating clotting factor compared to normal individual · Mild: 6-50% circulating clotting factor compared to normal individual

A 2 year old patient with ALL has finished her month of induction therapy and the marrow testing done yesterday showed 0.1% lymphoblasts. When she began therapy, her WBC was 45,000 and she initially presented with significant bleeding. Which feature(s) of the patient indicate that her prognosis is worse than a patient with standard risk?

Marrow results of 0.1% blasts · Poorer outcomes with ALL: <1 yo or >10 yo, WBC at diagnosis >50,000, disease outside bone marrow/bloodstream (CNS, testicles), less than full remission at end of induction (full remission=<0.01% blasts in marrow), presence of unfavorable genetic alterations (mixed lineage rearrangements, Philadelphia chromosome positive)

A 14 year old boy with osteosarcoma is set to start a cycle of chemotherapy including doxorubicin, target dose 37.5 mg/m2. His weight is 61 kg and height is 180 cm. Calculate the dose of dexrazoxane which would precede his doxorubicin knowing the dose is based on a 10:1 ratio with doxorubicin.

Mostellar method BSA (m2) =√(ht (cm) x wt (kg)/3600) · √(61x180)/3600 = BSA of 1.75 · 10:1 ratio of dexrazoxane to doxorubicin - dexrazoxane dose 10x higher than doxorubicin · 1.75 m2 x 10 x 37.5 mg/m2 = 656 mg dexrazoxane · Key treatment drugs in osteosarcoma: cisplatin, doxorubicin, high-dose methotrexate

An 8 year old boy (30 kg) diagnosed with Burkitt lymphoma has just transferred out of the PICU to your unit. He is strict NPO except for medications. After reviewing his medication profile, you see the following: hydration with D5-1/2NS + 20 mEq of KCl @ 175 mL/hr, sevelamer 400 mg TID, allopurinol 100 mg PO TID, and cefepime 1.5 gm IV q8hr. What changes, if any, do you recommend?

Remove KCl from IVF, D/c sevelamer until taking nutrition by mouth (phosphate binders pull phosphorous from food) · Burkitt lymphoma has a large tumor burden. Tumor lysis syndrome is an early cause of morbidity in newly diagnosed, newly treatment malignancies, especially with acute leukemias and Burkitt lymphoma. · Manifestations of tumor lysis syndrome: ↑K, Phos, uric acid, SCr; ↓Ca · Prevention: hyperhydration, restriction/elimination of K in fluids, allopurinol (prevents further formation of uric acid) · Treatment: hydration, K binders/dextrose-insulin boluses, P binders, rasburicase (drop uric acid from extremely high levels to undetectable), infrequently Ca supplementation (risk for complexing of Ca and phos in bloodstream, even if phos isn't super high), dialysis

A 15 year old boy is receiving a 5-day course of ifosfamide 2200 mg and etoposide 140 mg daily. The daily mesna total given along with ifosfamide is meant to equal 120% of the ifosfamide dose. If specified, that mesna is to be mixed in the ifosfamide bag, then given separately at hrs 4 & 8. What dose of mesna do you calculate for each fraction?

a. (2200 x 1.2)/3=880 mg

D.K. is a 14-year-old male adolescent with no pertinent medical history. He presents to the emergency department obtunded and in hypovolemic shock. His serum glucose is 1210 mg/dL and HCO3 - is 16 mEq/L. He is given a diagnosis of hyperglycemic hyperosmolar state (HHS). He receives 20 mL/kg of 0.9% sodium chloride, followed by fluid deficit therapy with 0.75% sodium chloride. Which best depicts the desired rate of glucose decline during the first several hours of fluid therapy? a. 75-100 mg/dL/hour. b. 50-75 mg/dL/hour. c. Greater than 100 mg/dL/hour. d. Less than 50 mg/dL/hour.

a. 75-100 mg/dL/hour. · Initial glucose correction with fluid therapy in HHS is generally 75-100 mg/dL/hour. · A decrease of less than 50 mg/dL/hour would require the addition of insulin therapy, which would be used to maintain a decrease of 50-75 mg/dL/hour once initiated. · A decrease of greater than 100 mg/dL/hour would be too rapid and would require the addition of dextrose 2.5%-5% to the replacement fluids.

A.J. is a 16-year-old male adolescent who presents to the ED with swelling and tenderness around his right knee. He was riding his horse earlier in the day when he was unexpectedly thrown off. He has been unable to put pressure on his right leg and currently rates his pain as 10 out of 10. Radiography of the leg reveals a Codman triangle with a fracture in the distal femur. He is given a preliminary differential diagnosis of osteosarcoma and is sent for a biopsy. The treating physician opts to initiate therapy with a course of "AP" chemotherapy (doxorubicin 37.5 mg/m2 / day and cisplatin 60 mg/m2 /day for 2 days). The patient has no known medication allergies, takes no other medications at home, and has no contraindications to the receipt of corticosteroids. What is the appropriate antiemetic regimen for this patient? a. Aprepitant, dexamethasone, and ondansetron. b. Ondansetron, dexamethasone, and promethazine. c. Dexamethasone, palonosetron, and promethazine. d. Dexamethasone, ondansetron, and nabilone.

a. Aprepitant, dexamethasone, and ondansetron. · This adolescent patient is receiving a highly emetogenic chemotherapy regimen (cisplatin is one of most highly emetogenic chemotherapy agents). According to the POGO guidelines, the patient should receive aprepitant, dexamethasone, and ondansetron (Answer A is correct). · Promethazine is not a guideline-supported agent (Answers B and C are incorrect). · Answer D is inappropriate because aprepitant is not included.

R.M. is a 14-month-old girl (weight 11 kg) admitted to the PICU and intubated for mechanical ventilation 72 hours earlier for respiratory failure and acute mental status changes. She has a medical history of autism, bronchopulmonary dysplasia, and gastroesophageal reflux disease. She is receiving ranitidine for stress-related mucosal disease (SRMD) prophylaxis, midazolam 0.2 mg/kg/hour, and fentanyl 2 mcg/kg/hour. She has two peripheral intravenous lines and a Foley catheter. Currently, the head of the bed is elevated to 35 degrees, and she is receiving oral care with chlorhexidine. The hospital infection control supervisor is reviewing R.M. for her risk of ventilator-associated pneumonia (VAP). Which other prevention strategy would be best for this patient? a. Daily sedation holidays with assessment of extubation readiness. b. Discontinuation of SRMD prophylaxis. c. Venous thromboembolism prophylaxis with enoxaparin. d. Proton pump inhibitor (PPI) for SRMD prophylaxis.

a. Daily sedation holidays with assessment of extubation readiness.

M.C. is a 4-year-old girl who presents to her pediatrician with fever, pallor, and bone pain. The pediatrician does a complete blood cell count in the clinic and refers her to the cancer center at the local children's hospital. Further diagnostic analysis reveals a white blood cell count (WBC) of 89,000 cells/mm3 with 47% blasts on peripheral blood smear. The results of a bone marrow aspirate and biopsy are conclusive for acute lymphoblastic leukemia (ALL) with no known genetic abnormalities. Which statement best describes the corticosteroid that M.C. should receive during induction therapy and why? a. Dexamethasone, because this is the standard of care for children 1-10 years of age. b. Hydrocortisone, because patients with newly diagnosed ALL are adrenally suppressed and require hydrocortisone supplementation. c. Methylprednisolone injection, because M.C. is 4 years old and cannot swallow oral tablets. d. Prednisone, because M.C. is female and does not need the extramedullary protection offered with dexamethasone.

a. Dexamethasone, because this is the standard of care for children 1-10 years of age. · Dexamethasone is the preferred corticosteroid for children 1-10 years of age with newly diagnosed ALL (Answer A is correct). · Hydrocortisone has no role as a therapeutic corticosteroid for patients with newly diagnosed ALL (Answer B is incorrect). · There is no indication that the patient cannot swallow tablets or suspension. If she were unable to swallow oral dosage forms, she should receive intravenous dexamethasone (the preferred corticosteroid), not methylprednisolone (Answer C is incorrect). · Prednisone is used in adolescents because of the increased risk of toxicity (osteonecrosis, infection) from dexamethasone (Answer D is incorrect).

A 6-month-old child undergoes intubation for respiratory syncytial virus pneumonia. She is given fentanyl 1 mcg/kg/hour and midazolam 1 mcg/kg/minute with good sedation. On the second day of sedation, she is recovering from pneumonia and ready for extubation. Which is the most appropriate course for her sedation? a. Discontinue the sedatives. b. Continue mechanical ventilation for another 24 hours while the drips are weaned off. c. Initiate oral methadone and lorazepam. d. Initiate a clonidine patch.

a. Discontinue the sedatives. · This patient has been on a low level of sedation and has been on continuous infusions for only 2 days. Withdrawal is unlikely, so discontinuing the sedatives would be appropriate. · Dependence occurs after ~5 days of exposure to continuous infusions of opioids, benzodiazepines, dexmedetomidine

A 5-year-old boy presents in septic shock with a temperature of 102.5°F (39.2°C). His blood pressure is 60/40 mm Hg, his heart rate is 100 beats/ minute, and he is cool to the touch. Which is the most appropriate vasopressor to initiate first? a. Dopamine. b. Norepinephrine. c. Milrinone. d. Epinephrine.

a. Dopamine. · This patient is in cold shock, for which dopamine is preferred. · Norepinephrine is for warm shock. · Milrinone is reserved for patients with decreased cardiac function and/or pulmonary hypertension. Because this patient is already hypotensive, milrinone would cause an even further decrease in SVR. · Epinephrine is an alternative if effects are inadequate with dopamine. Because there is some clinical controversy, some centers will use epinephrine as a first-line agent in this scenario.

1. A 4-month-old child with no significant past medical history presents with respiratory failure secondary to respiratory syncytial virus. Mechanical ventilation has been maximized, with little improvement in the patient's clinical status. The decision is made to start ECMO. Which is the most appropriate anticoagulant to use during ECMO? a. Heparin. b. Warfarin. c. Enoxaparin. d. Aspirin.

a. Heparin. · During ECMO, blood flow to the gastrointestinal tract is compromised, making the oral route of administration inappropriate for medications, which eliminates warfarin and aspirin as appropriate. Warfarin also takes days to work, which is another reason not to use this agent in ECMO. · Because there is an extremely fine line between clotting and bleeding in ECMO, an agent that can be easily titrated is best. Heparin is a continuous infusion that can be titrated easily, especially with bedside testing. · Enoxaparin, which lasts for extensive periods, would not be appropriate in ECMO.

A 5-year-old patient with no significant medical history presents to the emergency department in January with tachycardia (120 beats/minute), a temperature of 102°F (39°C), and an elevated white blood cell count of 25 x 103 cells/mm3 . Both the basic metabolic panel and the liver function tests are normal. The team initiates two rounds of normal saline 20 mL/kg and is almost ready to initiate the third when the medical resident hears rales in the lungs. The patient's blood pressure is 65/45 mm Hg, and he has cold extremities. Which is the next most appropriate step for this patient? a. Initiate dopamine at 9 mcg/kg/minute. b. Initiate milrinone at 0.5 mcg/kg/minute. c. Initiate norepinephrine at 0.1 mcg/kg/minute. d. Initiate normal saline 10 mL/kg.

a. Initiate dopamine at 9 mcg/kg/minute.

J.W. is a 2-year-old girl who was referred to the clinic from her primary pediatrician. One week ago, her grandmother was bathing her and noticed a nontender mass in her abdomen. J.W. is afebrile and otherwise well appearing. Ultrasonography revealed a large mass arising from the right kidney that appeared to be a Wilms tumor. There was no involvement of the inferior vena cava and no visible metastases. Biopsy of the tumor after surgical excision confirmed a stage II, favorable histology, Wilms tumor (weighing 600 g), with LOH at 1p and 16q. Which statement is most accurate regarding the late outcomes that J.W. might experience after the treatment selected in the previous question? a. Late cardiotoxicity (heart failure) from doxorubicin. b. Chronic diarrhea from irinotecan. c. Hearing loss from carboplatin. d. Secondary AML from etoposide.

a. Late cardiotoxicity (heart failure) from doxorubicin. · Survivors of Wilms tumor are at a high risk of developing late effects of chemotherapy, namely cardiotoxicity, secondary malignancies, and pregnancy-related complications. This patient would have received doxorubicin as part of DD4A (Answer A is correct). · She would not have received irinotecan (UH-2), carboplatin (UH-1, UH-2), or etoposide (M, UH-1, UH-2; Answers B-D are incorrect).

J.R. is a 16-year-old female adolescent (weight 52 kg) with no known significant medical history. As reported by her track coach, she fell down during practice, and her left leg began shaking uncontrollably, followed by uncontrolled shaking of her entire body. The shaking stopped without intervention in about 5 minutes, but J.R. still could not be awakened. Her coach called 911, and J.R. was immediately transferred by ambulance to the hospital, where she was admitted to the PICU. On arrival to the PICU, she had another brief seizure that was treated with lorazepam. Her vital signs in the PICU included temperature 100.7°F, respiratory rate 20 breaths/minute, heart rate 128 beats/minute, and blood pressure 130/70 mm Hg. Significant laboratory values included Na 129 mEq/L, K 5.1 mEq/L, Cl 105 mEq/L, CO2 25 mEq/L, BUN 22 mg/dL, SCr 1.4 mg/ dL, lactic acid 8 mg/dL, and albumin 2.5 g/dL. About an hour later, J.R. begins to seize again. Which is the best treatment option for this patient? a. Lorazepam 4 mg intravenously. b. 0.9% sodium chloride at a rate of 50 mL/hour. c. Phenobarbital 20 mg intravenously. d. Propofol infusion at 1 mg/kg/hour.

a. Lorazepam 4 mg intravenously.

M.K. is a 16-year-old female adolescent recently given a diagnosis of stage IIIB Hodgkin lymphoma (HL). She received conventional chemotherapy and radiation. Which statement is the most accurate description of the outcomes she may experience after therapy? a. M.K. is at risk of cardiac dysfunction, including heart failure, because of the receipt of doxorubicin and radiation. b. M.K. is at risk of developing pulmonary fibrosis because of receiving vincristine therapy. c. M.K. is at risk of developing a secondary malignancy; however, there is currently no recommendation for long-term follow-up. d. M.K. should expect to have impaired fertility, and she should be advised never to become pregnant because of the risk to the fetus.

a. M.K. is at risk of cardiac dysfunction, including heart failure, because of the receipt of doxorubicin and radiation. · This patient is at risk of developing cardiac dysfunction because of the receipt of doxorubicin and radiation (Answer A is correct). · Pulmonary fibrosis is often the result of bleomycin therapy, not vincristine therapy (Answer B is incorrect). · She is at risk of developing a secondary malignancy (caused by radiation and chemotherapy) and should be monitored closely long term for the development of another malignancy or other late effects (Answer C is incorrect). · Although this patient may have impaired fertility, there is no clear evidence that a fetus born after a successful cure will be affected by the prior receipt of chemotherapy (Answer D is incorrect).

T.N. is a 5-year-old girl (weight 20 kg) who is in the PICU for status epilepticus (SE). Seizures lasted about 15 minutes before cessation after two doses of benzodiazepines. Her current laboratory values include the following: sodium (Na) 137 mEq/L, potassium (K) 4.2 mEq/L, HCO3 - 18 mEq/L, chloride (Cl) 100 mEq/L, blood urea nitrogen (BUN) 20 mg/dL, serum creatinine (SCr) 0.3 mg/dL, and lactate 6 mmol/L. Her ABG includes pH 7.3 and Pco2 30 mm Hg. Which primary acid-base disorder is most consistent with T.N.'s laboratory and clinical data? a. Metabolic acidosis. b. Respiratory acidosis. c. Metabolic alkalosis. d. Respiratory alkalosis.

a. Metabolic acidosis. · pH low @ 7.3 (normal 7.35-7.45) · Pco2 low @ 30 (normal 35-45) · HCO3 low @ 18 (normal 22-28) · Anion gap: (Na - (Cl-HCO3): 19 · pH low, HCO3 low: metabolic acidosis

A.J. is a 16-year-old male adolescent who presents to the ED with swelling and tenderness around his right knee. He was riding his horse earlier in the day when he was unexpectedly thrown off. He has been unable to put pressure on his right leg and currently rates his pain as 10 out of 10. Radiography of the leg reveals a Codman triangle with a fracture in the distal femur. He is given a preliminary differential diagnosis of osteosarcoma and is sent for a biopsy. Which is the most likely treatment approach for a patient with newly diagnosed osteosarcoma? a. Neoadjuvant chemotherapy, followed by surgery as local control; then adjuvant chemotherapy. b. Neoadjuvant chemotherapy, followed by radiation as local control; then adjuvant chemotherapy. c. Chemotherapy followed by radiation. d. Palliative radiation therapy alone.

a. Neoadjuvant chemotherapy, followed by surgery as local control; then adjuvant chemotherapy. · Children with newly diagnosed osteosarcoma should receive neoadjuvant chemotherapy, followed by surgery for local control; then adjuvant chemotherapy (Answer A is correct). Osteosarcoma is considered a relatively radiation-resistant tumor (Answers B and C are incorrect). · Palliative therapy would not be appropriate at this time (Answer D is incorrect).

Which pair most closely links the toxicity with a causative chemotherapeutic agent? a. Neuropathy and vinca alkaloids/cisplatin. b. Sinusoidal obstruction syndrome and carboplatin. c. Ototoxicity and cyclophosphamide. d. SIADH and ifosfamide.

a. Neuropathy and vinca alkaloids/cisplatin. · Neuropathy associated with vinca alkaloids and cisplatin. Treatment: supportive care - neuropathic analgesics, laxatives in case of vinca effects on peristalsis (neuropathy b/c of constipation)); dose reduction or delay may be considered in severe cases · Sinuosal obstruction syndrome (liver issue): busulfan and dactinomycin · Ototoxicity: high-dose carboplatin, cisplatin · SIADH: vincristine and cyclophosphamide

T.H. is a 2-year-old girl with sickle cell anemia. She has been admitted to the hospital three times in the past 12 months for vaso-occlusive crises associated with sickle cell anemia. She has no history of receiving any red blood cell transfusions, and her hemoglobin is at baseline levels. She has no known allergies. Two of T.H.'s older siblings with sickle cell anemia are adherent to their office visits and preventive treatment. Which treatment would be considered best, given the severity of T.H.'s disease? a. Oral hydroxyurea therapy. b. Oral deferasirox prophylaxis. c. Human leukocyte antigen (HLA)-matched sibling transplantation. d. Long-term red blood cell transfusions.

a. Oral hydroxyurea therapy. · Oral hydroxyurea therapy should be considered for this patient to minimize the complications of her disease and help prevent hospitalizations (Answer A is correct). · The other options are not appropriate for this patient because there is no indication that she is yet in need of chelation (i.e., no history of transfusions to date; Answer B is incorrect), she has no HLA-matched donor available (both siblings are affected with sickle cell disease; Answer C is incorrect), and she has no indication yet for chronic red blood cell transfusions (i.e., no known history of stroke; Answer D is incorrect). · Hydroxyurea is preventative therapy aimed to reducing painful episodes, need for RBC transfusions, acute chest syndrome, and dactylitis (inflammation of toe/finger joints). MOA=increases fetal HgB --> improved health of RBCs

a. SIRS. · This patient has a fever with a leukocytosis, classifying him as having SIRS (Answer A is correct). · If there were laboratory abnormalities or the presence of an infection, he would be classified as having sepsis. · Hypotension would indicate septic shock. · Severe shock would be correct if there were other organ system dysfunction.

You are working with the interdisciplinary medical team on the chemotherapy orders for an 8-month-old patient (weight 7 kg) with newly diagnosed stage I favorable histology Wilms tumor (tumor weight 600 g, no loss of heterozygosity at 1p and 16q). The team would like to initiate therapy with a two-drug regimen, EE4A, containing vincristine and dactinomycin. Which set of doses is most appropriate? a. Vincristine 0.05 mg/kg; dactinomycin 0.045 mg/kg. b. Vincristine 0.05 mg/kg; dactinomycin 2.5 mg. c. Vincristine 1.5 mg/m2 ; dactinomycin 0.045 mg/kg. d. Vincristine 2.5 mg; dactinomycin 0.045 mg/kg.

a. Vincristine 0.05 mg/kg; dactinomycin 0.045 mg/kg. · Chemotherapy for infants younger than 12 months is almost always dosed according to body weight in kilograms. Dosing infants by BSA will result in a relative overdose and is unwarranted (Answer A is correct). · Infants younger than about 12 months have a disproportionately higher BSA relative to older children. Historical trials of children with Wilms tumor helped to contribute to the understanding of this issue where younger, smaller children exposed to dactinomycin experienced untoward adverse events (e.g., sinusoidal obstruction syndrome) when dosed by BSA instead of weight in kilograms (Answers B and D are incorrect). · The traditional maximum single dose of dactinomycin is 2.5 mg, but this dose would be much too high for this patient when calculated using body weight (Answer B is incorrect). · Answer A provides both drugs in milligram per kilogram values; Answer C is incorrect because the dose of vincristine is provided according to BSA. Vincristine doses for patients with most tumors are capped at 2 mg to prevent excess neurotoxicity; a dose of 2.5 mg would be too high for this patient (Answer D is incorrect). · Doses in milligrams per square meter can be divided by a factor of 30 to produce doses in milligrams per kilogram.

a. Vincristine, dexamethasone, and pegaspargase. · This patient has been given a diagnosis of standard-risk ALL. Children with standard-risk ALL should receive a three-drug induction consisting of vincristine, dexamethasone, and pegaspargase (Answer A is correct; Answers C and D are incorrect; they are four-drug inductions). · Dexamethasone is used instead of prednisone because this patient is younger than 10 years and at a lower risk of osteonecrosis (Answer B is incorrect). Key treatment drugs: vincristine, corticosteroid, asparaginase, +/- daunorubicin, methotrexate (IV, intrathecal, PO), mercaptopurine (PO maintenance tx)

A 5-year-old girl was an unrestrained passenger in a motor vehicle collision. Having sustained a significant TBI with a Glasgow Coma Scale score of 3, she is brought to the emergency department after undergoing intubation at the scene. An intracranial monitor is placed, which reveals an ICP of 30 mm Hg. She is hypotensive and tachycardic. After initial fluid resuscitation, her blood pressure normalizes, but she remains tachycardic. She will likely require intubation for more than 7 days. Laboratory values are as follows: sodium 137 mEq/L, potassium 4.1 mEq/L, chloride 100 mEq/L, bicarbonate 25 mEq/L, blood urea nitrogen 20 mg/dL, and SCr 0.6 mg/dL. Her ICP is still 30 mm Hg. Which is the most appropriate initial therapy for managing her ICP? a. Mannitol 0.5 g/kg every 6 hours. b. 10-mL/kg bolus of 3% saline. c. 0.2-mL/kg/hour infusion of 3% saline. d. 5-mg/kg pentobarbital bolus.

b. 10-mL/kg bolus of 3% saline. · According to these laboratory values, all of the patient's tests are within normal range. However, her sodium concentration, though normal, is too low for a patient with TBI. · Strategies to increase the sodium to 160 mEq/L or higher should be used. A bolus is a quick way to increase the patient's sodium and lower the increase in ICP initially. · She could be placed on a 0.2-mL/kg/hour infusion of 3% saline after the saline bolus because it would not rapidly lower her ICP. Mannitol is an appropriate choice; however, evidence points toward using saline over mannitol. · Using a pentobarbital bolus would be appropriate when the other options have failed.

An 8-year-old boy (weight 30 kg) sustained burns to 40% of his body after accidentally pulling boiling water off the stove. Which is the most appropriate way to replace his fluids? a. 300 mL/hour for 24 hours. b. 450 mL/hour for 8 hours; then 225 mL/hour for 16 hours. c. 225 mL/hour for 8 hours; then 450 mL/hour for 16 hours. d. 225 mL/hour for 8 hours; then 112 mL/hour for 16 hours.

b. 450 mL/hour for 8 hours; then 225 mL/hour for 16 hours. 6 mL/kg per percent TBSA: 6 mL/kg x 30 kg x 40(TBSA) = 7200 mL/day. 7200 mL/day divided in half: 3600 mL/8 hrs = 450 mL/hr x 8 hrs. 3600 mL/16 hrs = 225 mL/hr x 16 hrs Reasons to use 6 mL/kg/TBSA instead of 2-4 mL/kg/TBSA: inhalation injury, larger burns >30% TBSA, deeper burns, children <2 yro, delayed treatment

Which patient would be the most appropriate candidate for HSCT? a. A 10-month-old boy with intermediate-risk neuroblastoma who has responded to conventional chemotherapy. b. A 7-year-old girl with AML with abnormal cytogenetic features in first remission. c. A 16-year-old girl with sickle cell disease who has no matched sibling donors. d. A 5-year-old boy with average-risk ALL who has just achieved remission after induction therapy.

b. A 7-year-old girl with AML with abnormal cytogenetic features in first remission. · A child with AML in first remission with abnormal cytogenetic features can undergo a transplant if a matched related donor is identified in a timely manner (Answer B is correct). · A 10-month-old with intermediate-risk neuroblastoma should be treated with chemotherapy alone; transplantation plays no role in therapy (Answer A is incorrect). · Children with sickle cell disease may be eligible for a transplant if they have an unaffected matched sibling donor (which this patient does not have; Answer C is incorrect). · Children with average-risk ALL in remission after induction should continue directly to consolidation and should not undergo transplantation (Answer D is incorrect).

Which of the following accurately reflect guidelines for the management of pediatric patients with febrile neutropenia? a. An antibiotic with coverage vs bacteria producing ESBL should be started empirically b. A diagnosis of pneumonia with no causative organism is a valid reason to add vancomycin coverage c. Detection of rhino/enterovirus is a criteria for high risk status d. >72 hours of unexplained fevers should prompt consideration of amphotericin B

b. A diagnosis of pneumonia with no causative organism is a valid reason to add vancomycin coverage · Should have antipseudomonal coverage, not ESBL coverage · Vancomycin should be considered if hemodynamic instability, pneumonia, suspected catheter-related infection or skin/soft tissue infection · Risk factors: presence of central line catheter, dx of acute leukemia (especially if relapsed), focus for possible infection or mucosal barrier breakdown, absence of viral respiratory infection which could explain fevers · >96 hrs of unexplained fevers should prompt consideration of fungal antigen testing, start of mold-active empiric antifungal (amphotericin B or caspofungin) and imaging for occult fungus in those at high risk for invasive fungus

A 7-year-old patient is currently on a fentanyl infusion at 1 mcg/kg/hour and is in the process of being deployed on extracorporeal membrane oxygenation (ECMO). Which best describes the pharmacokinetic change that is expected to occur with fentanyl while the patient is on ECMO? a. Decreased volume of distribution. b. Adsorption to the circuit. c. Increased clearance. d. No changes to fentanyl pharmacokinetics.

b. Adsorption to the circuit. · Volume of distribution increases, clearance decreases in ECMO · Drugs that adsorb a lot to the circuit (almost all do, but some adsorb more than others): fentanyl, furosemide, midazolam, propofol, voriconazole · If a patient on a fentanyl drip gets placed on ECMO, change to morphine drip

M.C. is a 4-year-old girl who presents to her pediatrician with fever, pallor, and bone pain. The pediatrician does a complete blood cell count in the clinic and refers her to the cancer center at the local children's hospital. Further diagnostic analysis reveals a white blood cell count (WBC) of 89,000 cells/mm3 with 47% blasts on peripheral blood smear. The results of a bone marrow aspirate and biopsy are conclusive for acute lymphoblastic leukemia (ALL) with no known genetic abnormalities. Which best explains why M.C. should receive CNS chemoprophylaxis? a. All female patients should receive CNS chemoprophylaxis because of an increased risk of CNS-relapsed ALL compared with male patients. b. All children with newly diagnosed ALL should receive CNS prophylaxis to eradicate disease in the CNS. c. CNS chemoprophylaxis enhances the efficacy of cranial irradiation and improves cognitive outcomes in children with newly diagnosed ALL. d. Children with newly diagnosed ALL do not require CNS chemoprophylaxis.

b. All children with newly diagnosed ALL should receive CNS prophylaxis to eradicate disease in the CNS. · The general approach to treating pediatric ALL includes induction, consolidation, and maintenance chemotherapy plus CNS prophylaxis.

A 3-year-old involved in a motor vehicle collision sustained a severe traumatic brain injury (TBI). Mannitol is used to help control the patient's intracranial pressure (ICP). Which best matches the mechanism of action of ICP reduction with mannitol's duration or onset? a. Blood viscosity reduction lasts 6 hours. b. Blood viscosity reduction lasts 1 hour. c. Osmolar agent starts working within 2 minutes. d. Osmolar agent lasts 1 hour.

b. Blood viscosity reduction lasts 1 hour. · Mannitol is an osmolar agent that also decreases blood viscosity. The onset of decreased blood viscosity is quick but does not last long (1 hour). · The effects on osmolarity take about 15-20 minutes but can last for 6 hours. · Answer A is incorrect because 6 hours is the duration of action as an osmolar agent.

J.W. is a 2-year-old girl who was referred to the clinic from her primary pediatrician. One week ago, her grandmother was bathing her and noticed a nontender mass in her abdomen. J.W. is afebrile and otherwise well appearing. Ultrasonography revealed a large mass arising from the right kidney that appeared to be a Wilms tumor. There was no involvement of the inferior vena cava and no visible metastases. Biopsy of the tumor after surgical excision confirmed a stage II, favorable histology, Wilms tumor (weighing 600 g), with LOH at 1p and 16q. What is the most appropriate approach to the treatment of this patient, using the abbreviations in Table 2? a. EE4A for 19 weeks. b. DD4A for 25 weeks. c. Regimen M for 31 weeks. d. UH-1 plus the VI window (UH-2) for 30 weeks.

b. DD4A for 25 weeks. · This 2-year-old patient has stage II, favorable histology Wilms tumor with LOH at 1p and 16q. The LOH at 1p and 16q upstages her tumor and thus necessitates her receipt of DD4A ("three-drug therapy": vincristine, dactinomycin, and doxorubicin) over EE4A ("two-drug therapy"; Answer B is correct; Answer A is incorrect). · Regimens M and UH-1 would be indicated for children with higher-stage tumors and diffuse anaplasia, respectively (see Table 2 in the text; Answers C and D are incorrect).

B.L. is a 17-year-old male adolescent (weight 62 kg) with no pertinent medical history. He presents with serum glucose 885 mg/dL, venous pH 7.19, HCO3 - 10 mEq/L, serum osmolality 325 mOsm/kg, and moderate ketones. He is pale with a moderately prolonged capillary refill time, but his peripheral pulses are good. His mental status is altered. Which is the best diagnosis and initial therapy? a. HHS; administer 1200 mL of 0.9% sodium chloride over 1 hour, continuous insulin infusion 0.05 unit/ kg/hour after completion of fluids. b. DKA; administer 1200 mL of 0.9% sodium chloride over 1 hour, continuous insulin infusion 0.05 units/ kg/hour after completion of fluids. c. HHS; administer 650 mL of 0.9% sodium chloride over 10 minutes, regular insulin bolus 6 units. d. DKA; administer 650 mL of 0.9% sodium chloride over 10 minutes, regular insulin bolus 6 units.

b. DKA; administer 1200 mL of 0.9% sodium chloride over 1 hour, continuous insulin infusion 0.05 units/ kg/hour after completion of fluids. · The patient has DKA, given his decreased venous pH and HCO3 - values as well as his moderate ketones. Resuscitation fluids should be administered at a dose of 10-20 mL/kg over 1-2 hours. · Resuscitation fluids with an isotonic agent (NS) in HHS should be administered at 10-20 mL/kg over 10-20 min. · Fluid replenishment should happen first in both HHS and DKA. · With HHS, continuous insulin would be initiated once the rate of glucose decrease was less than 50 mg/dL/hour with fluids alone.

T.H. is a 2-year-old girl with sickle cell anemia. She has been admitted to the hospital three times in the past 12 months for vaso-occlusive crises associated with sickle cell anemia. She has no history of receiving any red blood cell transfusions, and her hemoglobin is at baseline levels. She has no known allergies. Two of T.H.'s older siblings with sickle cell anemia are adherent to their office visits and preventive treatment. T.H. develops a fever with pain, swelling, and decreased range of movement in her hand. Which clinical scenario would you most likely suspect? a. Dactylitis; she should be treated with antibiotics that provide coverage against Enterococcus species. b. Dactylitis; she should be treated with antibiotics that provide coverage against Salmonella and Staphylococcus species. c. Dactylitis; she should be treated with antibiotics that provide coverage against Streptococcus species. d. Dactylitis; antibiotics are not clinically indicated.

b. Dactylitis; she should be treated with antibiotics that provide coverage against Salmonella and Staphylococcus species. · Dactylitis is a common presenting symptom in children with sickle cell disease. Dactylitis can be caused by infection with Salmonella or Staphylococcus species; a patient with sickle cell disease presenting with fever and symptoms of dactylitis should receive antibiotics that cover these organisms (Answer B is correct; Answer D is incorrect). · Streptococcus, a potential infectious agent in children with sickle cell disease, is not one of the top infectious organisms in the setting of dactylitis (Answer C is incorrect). · Enterococcus would not normally be a causative infectious organism for dactylitis (Answer A is incorrect).

A 9-year-old mechanically ventilated child goes into cardiac arrest in the intensive care unit (ICU). Earlier that day, the child lost intravenous access, and the team is unable to obtain a new line. Which drug would be most appropriate to give endotracheally to this child? a. Calcium chloride. b. Epinephrine. c. Amiodarone. d. Vasopressin.

b. Epinephrine. · Acronym for medications that can be administered by an endotracheal tube is LEAN: lidocaine, epinephrine, atropine, and naloxone.

A code is called on a 7-year-old child who was extubated yesterday after a 6-day stint of respiratory syncytial virus pneumonia. The team arrives at the scene and finds no pulse. Chest compressions are begun, and the patient is placed on a monitor. The monitor reveals PEA. The nurses cannot establish peripheral intravenous access after three attempts (10 minutes), and the patient does not have a central line. They are setting up for an emergency intubation, but they have not yet begun. Access is obtained, and the monitor now reveals VF. Which is the most appropriate initial drug therapy? a. Adenosine. b. Epinephrine. c. Atropine. d. Amiodarone.

b. Epinephrine. · VF/pVT: Shock -> shock -> epi -> shock -> amiodarone/lidocaine

The oncology fellows at your institution have asked for a review of the appropriate use of antifungal prophylaxis in children with cancer or those receiving HSCT. Which statement best represents a guideline-supported recommendation for children receiving an autologous HSCT with an anticipated duration of neutropenia of more than 7 days? a. Prophylaxis is not recommended. b. Give fluconazole 6-12 mg/kg/day (maximum 400 mg/day) intravenously or orally from the start of conditioning until engraftment. c. Give posaconazole 200 mg orally three times daily from the start of conditioning until engraftment. d. Give voriconazole 7 mg/kg orally/intravenously every 12 hours from the start of conditioning until engraftment.

b. Give fluconazole 6-12 mg/kg/day (maximum 400 mg/day) intravenously or orally from the start of conditioning until engraftment. · The most appropriate answer for this case is fluconazole, according to the C17 guideline for antifungal prophylaxis in children with cancer or those receiving HSCT (Answer B is correct; Answer A is incorrect). · Voriconazole is not guideline-recommended for antifungal prophylaxis in children (Answer D is incorrect). · Posaconazole is guideline-recommended for patients older than 13 years receiving an allogeneic HSCT with acute grade II-IV GVHD or chronic extensive GVHD (Answer C is incorrect). Posaconazole is also recommended as an alternative to fluconazole prophylaxis for patients with AML/myelodysplastic syndrome in an institution where there is a high rate of invasive mold disease.

M.C. is a 4-year-old girl who presents to her pediatrician with fever, pallor, and bone pain. The pediatrician does a complete blood cell count in the clinic and refers her to the cancer center at the local children's hospital. Further diagnostic analysis reveals a white blood cell count (WBC) of 89,000 cells/mm3 with 47% blasts on peripheral blood smear. The results of a bone marrow aspirate and biopsy are conclusive for acute lymphoblastic leukemia (ALL) with no known genetic abnormalities. Which most accurately describes the broad approach to the treatment of M.C.'s disease? a. Neoadjuvant chemotherapy, radiation, and adjuvant chemotherapy. b. Induction, consolidation, and maintenance chemotherapy plus central nervous system (CNS) prophylaxis. c. Two cycles of induction chemotherapy, followed by two cycles of intensification chemotherapy. d. Myeloablative chemotherapy, followed by an allogeneic stem cell transplant.

b. Induction, consolidation, and maintenance chemotherapy plus central nervous system (CNS) prophylaxis. · The general approach to treating pediatric ALL includes induction, consolidation, and maintenance chemotherapy plus CNS prophylaxis (Answer B is correct). · Key treatment drugs: vincristine, corticosteroid (dexamethasone if <10 yro & at lower risk of osteonecrosis; pred otherwise), asparaginase + daunorubicin, methotrexate (intrathecal, IV, PO), mercaptopurine (PO - maintenance) · 2 yrs for girls, 3 yrs for boys - historically boy would relapse in testicles if they only received treatment for 2 yrs, so go 1 more year · Several other subsets of treatment are within these major categories. The other selections include treatment approaches that apply to other pediatric malignancies (Answers A, C, and D are incorrect).

N.M. is a 3-month-old African American boy (height 24 inches [60 cm], weight 4 kg) admitted to the PICU for dehydration and sepsis. He was born full term and has no significant birth or medical history. He was electively intubated in the PICU 2 days earlier because of altered mental status. He is currently receiving milrinone 0.25 mcg/kg/minute, midazolam 0.1 mg/kg/hour, and fentanyl 1 mcg/ kg/hour. His most recent laboratory values include the following: Na 140 mEq/L, K 3.5 mEq/L, Cl 105 mEq/L, HCO3 - 22 mEq/L, BUN 20 mg/dL, SCr 0.7 mg/dL, glucose 90 mg/dL, and international normalized ratio (INR) 1.82. Which would best for prevention of bleeding from SRMD in this patient? a. Ranitidine 4 mg intravenously every 6 hours. b. Lansoprazole 4 mg by nasogastric tube daily. c. Sucralfate 5 mL by nasogastric tube every 6 hours. d. No stress ulcer prophylaxis needed.

b. Lansoprazole 4 mg by nasogastric tube daily. · This patient is at risk of SRMD secondary to an elevated INR, mechanical ventilation, shock, and acute renal failure (estimated creatinine clearance 30-32 mL/minute/1.73 m2 ). Thus, pharmacologic prophylaxis should be administered. · Either an H2RA or a PPI provided enterally or intravenously would be an appropriate first-line choice. If ranitidine is chosen, it should be renally dose adjusted because of the patient's acute kidney injury; thus, it should not be dosed at 1 mg/kg/dose intravenously every 6 hours. A PPI such as lansoprazole would be an appropriate first-line choice and would not require renal dose adjustment. · Sucralfate is not recommended as a first-line choice, and aluminum accumulation can occur in patients with renal insufficiency.

A 4 year old 15 kg patient presents to the ER after a 12 minute generalized-complex seizure that was treated at home with Diastat. During transport by EMS, the patient had another seizure that was treated by IV lorazepam. The patient is not seizing again and neurology would like you to choose the next step in therapy. Vitals: HR 135, RR 24, BP 72/50, SPO2: 98% a. Propofol 2 mg/kg then 150 mcg/kg/min b. Levetiracetam 40 mg/kg over 15 minutes c. Supportive care including bag mask ventilation and fluid boluses d. Fosphenytoin 20 mg/kg PE IV over 20 minutes

b. Levetiracetam 40 mg/kg over 15 minutes · Fosphenytoin can be associated with hypotension and the patient's BP is already a little soft

L.T. is a 14-year-old male adolescent (weight 65 kg) with a history of seizure disorder. He was at a friend's house for the weekend and forgot his seizure medication of topiramate. After returning home, he develops a tonic-clonic seizure lasting about 5 minutes, according to his mother. She administered 15 mg of rectal diazepam and called emergency medical services. Diazepam initially resulted in cessation of seizure activity, but on PICU admission, he began seizing again. He has peripheral intravenous access. Which would be the best emergent initial therapy for generalized clonic SE in this patient? a. Another 5-mg dose of rectal diazepam. b. Lorazepam 4 mg intravenously. c. Phenobarbital 1300 mg intravenously. d. Midazolam continuous infusion at 0.1 mg/kg/ hour.

b. Lorazepam 4 mg intravenously. · The best emergent initial option for this patient would be lorazepam 4 mg IV because the patient has IV access, and his initial response to benzodiazepines makes repeat dosing an appropriate plan. · Although another dose of rectal diazepam could be given, 5 mg is not the correct dose for this patient; also, rectal diazepam has a slower onset than IV lorazepam, and alternative routes need only be used when IV access is not available. · Either phenobarbital or continuous infusion midazolam may be considered if the patient does not respond to an intermittent IV dose of lorazepam.

A 5-year-old girl was an unrestrained passenger in a motor vehicle collision. Having sustained a significant TBI with a Glasgow Coma Scale score of 3, she is brought to the emergency department after undergoing intubation at the scene. An intracranial monitor is placed, which reveals an ICP of 30 mm Hg. She is hypotensive and tachycardic. After initial fluid resuscitation, her blood pressure normalizes, but she remains tachycardic. She will likely require intubation for more than 7 days. Which would be the most appropriate therapy for managing her tachycardia? a. Propofol infusion for length of intubation. b. Midazolam and fentanyl infusions for length of intubation. c. Ketamine infusion for length of intubation. d. Vecuronium infusion for length of intubation.

b. Midazolam and fentanyl infusions for length of intubation. · This patient has been adequately resuscitated with fluids but remains tachycardic likely because of her pain and sedation needs. · Propofol should not be used for more than 24 hours in children. · Ketamine could be an option, but in TBI, it has been studied only in procedural sedation as an adjunct. · Vecuronium is incorrect because paralyzing a patient without adequate sedation is inappropriate.

A 6-year-old girl presents in shock. She has a past medical history significant for a dilated viral cardiomyopathy with a baseline ejection fraction of 45%. Her current ejection fraction is 35%. Her current systemic vascular resistance (SVR) is 130/80 mm Hg. Which is the most appropriate agent to use in this child for cardiac support? a. Norepinephrine. b. Milrinone. c. Dopamine. d. Phenylephrine.

b. Milrinone. · Cold shock: dopamine, epinephrine if resistant (stimulate beta receptors to increase CO & alpha receptors to vasoconstrict) · Warm shock: norepinephrine (stimulate alpha receptors to vasoconstrict) · Low CO and low SVR: epinephrine (stimulate beta receptors to increase CO & alpha receptors to vasoconstrict) · Low CO and high SVR: milrinone (PDE-3 inhibitor to increase vasocontractility and vasodilate) · High CO and low SVR: norepi (stimulate alpha receptors to vasoconstrict - increase BP without adding to cardiac work) · Phenylephrine is an α1 -agonist only, which would make the patient hypertensive but would not affect the cardiac function.

D.P. is an 8-month-old male infant (weight 6 kg) with a medical history of chronic lung disease of prematurity, for which he is receiving furosemide at home. He is now in the PICU with a diagnosis of ARDS. He is mechanically ventilated with Fio2 100%, PEEP 7 cm H2 O, and respiratory rate 24 breaths/minute. His oxygenation continues to worsen, despite advancing his mechanical ventilation. An ECHO reveals suprasystemic right ventricular pressures consistent with pulmonary hypertension. Which pulmonary-specific treatment would best be considered to potentially improve his oxygenation? a. Surfactant. b. Nitric oxide. c. Albumin. d. Methylprednisolone.

b. Nitric oxide. · Many ARDS therapies have limited supportive evidence, and large controlled randomized trials either have not been conducted or have shown no meaningful difference. · Because this patient's ECHO findings are consistent with pulmonary hypertension and poor oxygenation, a course of inhaled nitric oxide would be reasonable. · No pediatric trials have evaluated corticosteroids, and the data from adult studies are contradictory; therefore, corticosteroids are not recommended in pediatric patients with ARDS. · Data are insufficient to recommend the use of bronchodilators.

A 16 year old patient with 40% blasts reported on his CBC has had diagnostic bone marrow sampling and initial word from the pathologist is that there appear to be myeloid features to the blasts. In reviewing lab work ordered for the patient, you see that a key lab parameter has not yet been ordered. Which of the following would you identify as especially important? a. Serum magnesium b. Prothrombin time c. Serum lactic dehydrogenase

b. Prothrombin time · Common presenting signs/symptoms for AML: similar to ALL, though medialstinal masses less common and coagulopathies more common

S.R. is a 10-year-old girl (weight 25 kg) who has been in the PICU for more than 2 weeks. Her medical history includes premature birth at 30 weeks' gestation, retinopathy of prematurity, laryngomalacia, cerebral palsy, developmental delay, and decreased gastrointestinal (GI) motility. She remains intubated and mechanically ventilated. During this admission, she has been treated for septic shock and has required an endoscopic intervention for an upper gastrointestinal bleed (UGIB). Which of S.R.'s risk factors has most consistently been identified as an independent risk factor for UGIB? a. Born prematurely. b. Respiratory failure. c. Previous GI bleed. d. Septic shock.

b. Respiratory failure. · Respiratory failure has been identified as an independent risk factor in both adult and pediatric patients (Answer B is correct). · A history of a GI bleed and septic shock are both factors that contribute cumulative risk, making them risk factors in this patient, but they have not been identified as independent predictors of risk in pediatric patients. The patient's septic shock also has resolved; therefore, this would no longer be considered a risk. · Prematurity is not a known risk predictor.

S.S. is a 7-year-old girl (weight 20 kg) who has had polydipsia, polyuria, and nausea/vomiting for the past week. She has no significant medical history. On admission to the emergency department, her point-of-care blood glucose was 578 mg/dL. She has a Kussmaul breathing pattern and is slightly obtunded; she also has extremely dry mucous membranes and skin tenting. After receiving 100 mL of 0.9% normal saline, she is transferred to the PICU. While awaiting her chemistry results, which would be the best initial treatment for her? a. Obtain an ABG. b. Resuscitate with 300 mL of 0.9% sodium chloride over 1 hour. c. Resuscitate with 300 mL of 0.9% sodium chloride plus 20 mEq/L of potassium chloride over 1 hour. d. Initiate continuous regular insulin at 0.1 unit/ kg/hour intravenously.

b. Resuscitate with 300 mL of 0.9% sodium chloride over 1 hour. · This patient has new-onset diabetes mellitus presenting with DKA. Adequate resuscitation is the initial treatment phase for all patients with DKA and should occur while awaiting laboratory values. Even if the laboratory values are not yet available, the patient can be rehydrated. · Resuscitation fluids are 10-20 mL/kg intravenously over 1-2 hours (for this patient, 200-400 mL). This patient has received only 5 mL/kg; therefore, administering the remainder of the fluid resuscitation, 100-300 mL over 1 hour, would be best.

T.H. is a 2-year-old girl with sickle cell anemia. She has been admitted to the hospital three times in the past 12 months for vaso-occlusive crises associated with sickle cell anemia. She has no history of receiving any red blood cell transfusions, and her hemoglobin is at baseline levels. She has no known allergies. Two of T.H.'s older siblings with sickle cell anemia are adherent to their office visits and preventive treatment. Which combination most accurately describes the preventive measures T.H. should receive? a. Routine childhood vaccinations, erythromycin prophylaxis, and folic acid supplementation. b. Routine childhood vaccinations, penicillin prophylaxis, and folic acid supplementation. c. Routine childhood vaccinations, erythromycin prophylaxis, and vitamin B12 supplementation. d. Routine childhood vaccinations, penicillin prophylaxis, and iron supplementation.

b. Routine childhood vaccinations, penicillin prophylaxis, and folic acid supplementation. · Vaccination and penicillin prophylaxis are well-known interventions to minimize the risk of invasive pneumococcal disease. Folic acid supplementation is important in patients with chronic hemolysis and red blood cell turnover. · Erythromycin should be used only in patients with a known penicillin hypersensitivity reaction (Answers A and C are incorrect). · Iron supplementation should not be used because many patients with sickle cell disease will in fact have iron overload because of transfusion therapy (Answer D is incorrect). · Vitamin B12 supplementation has no routine role in the treatment of sickle cell disease (Answer C is incorrect).

E.G. is a 3-year-old girl who presents to the emergency department with lethargy, increased respiratory rate, hypotension, weak pulses, and poor capillary refill. Her heart rate is 200 beats/minute. Her medical history is significant for tuberous sclerosis with a large rhabdomyoma on the left ventricular septum. An electrocardiogram (ECG) shows monomorphic wide QRS complexes (greater than 0.09 seconds) with absent P waves. Which would be best for treating E.G.? a. Amiodarone. b. Synchronized cardioversion. c. Lidocaine. d. Magnesium.

b. Synchronized cardioversion. · This patient has VT, probably caused by her rhabdomyoma interfering with normal conduction. She has a pulse but poor perfusion, making her extremely unstable. Cardioversion is the best option to quickly return her to a perfusing rhythm. · After the patient becomes less symptomatic and if she is still in a wide tachycardia, amiodarone would be appropriate. · Magnesium is inappropriate because she has a monomorphic tachycardia, and the rhythm is not torsades de pointes. · Lidocaine is used in pulseless VT or VF.

Which chemotherapy agents do not have myelosuppression as a side effect (select all that apply)? a. Cisplatin b. Vincristine c. Doxorubicin d. Asparaginase e. Monoclonal antibodies f. Tyrosine Kinase Inhibitors

b. Vincristine d. Asparaginase e. Monoclonal antibodies f. Tyrosine Kinase Inhibitors When do to certain things: · Transfusions of RBCs &/or platelets: Hbg 7, platelet 10,000 · Typical thresholds for proceeding with next cycle of chemotherapy: ANC>750, plt >75,000 · Common endpoint for stopping filgrastim after a given cycle is ANC>1500

T.N. is a 5-year-old girl (weight 20 kg) who is in the PICU for status epilepticus (SE). Seizures lasted about 15 minutes before cessation after two doses of benzodiazepines. Her current laboratory values include the following: sodium (Na) 137 mEq/L, potassium (K) 4.2 mEq/L, HCO3 - 18 mEq/L, chloride (Cl) 100 mEq/L, blood urea nitrogen (BUN) 20 mg/dL, serum creatinine (SCr) 0.3 mg/dL, and lactate 6 mmol/L. Her ABG includes pH 7.3 and Pco2 30 mm Hg. Which best represents the calculated anion gap for T.N.? a. 5. b. 11. c. 19. d. 40.

c. 19. · Anion gap: Na - (Cl - HCO3) · Anion gap normal range: 7-14 · HCO3 normal range: 22-28

Cardiac arrest is called on a 4-year-old girl on the floor. The team begins cardiopulmonary resuscitation (CPR), and the monitor reveals ventricular fibrillation (VF). The team does three cycles of CPR, defibrillation, and epinephrine with no return to spontaneous circulation. Which drug would be best to consider next? a. Atropine. b. Adenosine. c. Amiodarone. d. Procainamide.

c. Amiodarone. · The standard sequence of treatments for VF: shock, CPR, shock, CPR, epinephrine every 3-5 minutes, shock, and then amiodarone/lidocaine

A.P. is a 6-week-old girl (weight 3.5 kg) who was admitted to the PICU 16 days for group B Streptococcus sepsis, has now completed a course of therapy for this infection. She is receiving mechanical ventilation for respiratory failure. Overnight, her O2 saturation worsened, decreasing to 88%, and she has developed an increase in respiratory secretions according to the respiratory therapist. Her morning complete blood cell count (CBC) includes a white blood cell (count) (WBC) of 3 x 103 cells/mm3 (78 polymorphonuclear leukocytes, 12 band neutrophils, 9 lymphocytes, and 3 monocytes), hemoglobin (Hgb) of 9 g/dL, hematocrit (Hct) of 30%, and platelet count of 100,000/mm3 . Her vital signs include temperature 97°F, heart rate 180 beats/minute, respiratory rate 24 breaths/minute, and blood pressure 100/60 mm Hg. Her chest radiograph reveals new infiltrates. A mini-BAL (bronchoalveolar lavage) is sent, and antibiotics are initiated. Which antibiotic regimen would provide the best empiric coverage for this patient? a. Ampicillin/sulbactam 50 mg/kg/dose (of ampicillin component) every 6 hours plus gentamicin 5 mg/kg/dose intravenously every 24 hours plus vancomycin 15 mg/kg/dose every 6 hours. b. Ampicillin 100 mg/kg/dose every 6 hours plus gentamicin 5 mg/kg/dose intravenously every 24 hours plus vancomycin 15 mg/kg/dose every 6 hours. c. Cefepime 50 mg/kg/dose intravenously every 8 hours plus gentamicin 5 mg/kg/dose intravenously every 24 hours plus vancomycin 15 mg/ kg/dose every 6 hours. d. Ceftriaxone 50 mg/kg intravenously every 24 hours plus gentamicin 5 mg/kg/dose every 24 hours plus vancomycin 15 mg/kg/dose every 6 hours.

c. Cefepime 50 mg/kg/dose intravenously every 8 hours plus gentamicin 5 mg/kg/dose intravenously every 24 hours plus vancomycin 15 mg/ kg/dose every 6 hours. · This patient, who has signs and symptoms consistent with VAP, has been intubated for more than 2 weeks; therefore, empiric treatment should provide coverage for organisms common in VAP and for those with risk factors for resistant organisms, including Pseudomonas and MRSA. · 2 antipseudmonal agents (cephalosporin + aminoglycoside/fluoroquinolone) if: septic shock, ARDS, prolonged hospitalization (>5 days), acute renal replacement therapy (don't use extended interval gent if patient receiving CRRT or if AKI present)

A 2-year-old is 5 days post-severe scald injury burn to 45% of her body. Which most accurately describes how propranolol would help? a. Increase heart rate. b. Increase resting energy expenditure. c. Decrease resting energy expenditure. d. Decrease lean body mass.

c. Decrease resting energy expenditure

A 5 year old boy with a neuroblastoma is approaching his phase of treatment after autologous HSCT which includes immune therapy agents. Which of the following are included in this cycle of treatment (select all that apply)? a. Bevacizumab b. Rituximab c. Dinutuximab d. Interferon-alfa e. Aldesleukin

c. Dinutuximab e. Aldesleukin · Stage 4 patients with neuroblastoma are patients that have received HSCT. They should receive dinutuximab, aldesleukin, isotretinoin

R.M. is a 7-month-old boy (weight 8 kg) admitted to the PICU for new-onset GCSE. He has no pertinent birth or medical history. He has a social history of two sisters in day care and a dog and a cat. He presented to the PICU seizing which had been ongoing for 10 minutes. He was given two doses of lorazepam 0.8 mg intravenously, but they did not stop the seizures. Which would be the next best option for this patient? a. Diazepam 1 mg rectally. b. Phenytoin 160 mg intravenously over 2 minutes. c. Fosphenytoin 160 mg phenytoin equivalents (PE) intravenously over 6 minutes. d. Valproic acid 320 mg intravenously over 10 minutes.

c. Fosphenytoin 160 mg phenytoin equivalents (PE) intravenously over 6 minutes. · Fosphenytoin 20 mg PE/kg (160 mg) or phenytoin 20 mg/kg (160 mg) would be the next best choice. · The fosphenytoin dose can be administered at a rate of 3 mg/ kg/minute (24 mg/minute) over 5-6 minutes (Answer C is correct). The phenytoin dose can be administered at 1 mg/kg/minute (8 mg/minute) over 15-20 minutes (Answer B is incorrect).

Your patient will receive ifosfamide dosed at 1800 mg/m2 per day for 5 consecutive days. Which statement best describes the most appropriate approach to the prevention of hemorrhagic cystitis? a. Hyperhydration alone is sufficient for this dose of ifosfamide. b. Give mesna continuous infusion starting at the completion of the fifth day of ifosfamide. c. Give hydration together with mesna 360 mg/m2 intravenously at hours 0, 4, and 8, respective to the start of the ifosfamide dose. d. Give mesna 240 mg/m2 intravenously at hours 0, 3, 6, 9, and 12, respective to the start of the ifosfamide dose.

c. Give hydration together with mesna 360 mg/m2 intravenously at hours 0, 4, and 8, respective to the start of the ifosfamide dose. · Ifosfamide should always be given with hydration and mesna; hydration alone is insufficient (Answer C is correct; Answer A is incorrect). · When given as a continuous infusion, mesna should be given with ifosfamide and through at least 12 hours after the last dose, not starting after the last dose (Answer B is incorrect). · Answer D uses an appropriate schedule (i.e., hours 0, 3, 6, 9, and 12), but there is no mention of hydration, which is important in the prevention of hemorrhagic cystitis (Answer D is incorrect).

Among the following agents, which should receive special attention due to higher risk of drug-drug interactions (select all that apply)? a. Cisplatin b. Rituximab c. Methotrexate d. Daunorubicin e. Imatinib f. Vincristine

c. Methotrexate e. Imatinib f. Vincristine · Imatinib is a tyrosine kinase inhibitor which is a major substrate of CYP3A4

16 year old patient presents after intestinal ingestion of unknown substance approximately 6 hours ago. Patient is semi-comatose and has a respiratory rate of 42. Useful laboratory data: pH 7.48, HCO3: 13 mmol, Anion gap 23, PCO2 20 mmHg. Classify the acid base disorder. a. Primary metabolic alkalosis b. Primary metabolic acidosis c. Mixed respiratory alkalosis with metabolic acidosis d. Primary respiratory alkalosis with compensatory metabolic acidosis

c. Mixed respiratory alkalosis with metabolic acidosis · Increased RR: hyperventilating and blowing of CO2 · Decreased PCO2 @ 20 (normal 35-45): respiratory alkalosis · Decreased HCO3 @ 13 (normal 22-28): metabolic acidosis · Increased anion gap @ 23 (normal 7-14) · Increased pH @ 7.48 (normal 7.35-7.45)

Which statement is most accurate regarding iron chelation for children with sickle cell disease? a. Iron chelation should be considered when a patient has received 60 mL/kg of packed red blood cells. b. Iron chelation with deferiprone should be the first choice because it is the only oral iron chelator labeled for use in children. c. Oral deferasirox can be used in children with iron overload resulting from long-term treatment with transfusions. d. Deferoxamine, which is only indicated for acute iron toxicity, should not be used for oral iron chelation.

c. Oral deferasirox can be used in children with iron overload resulting from long-term treatment with transfusions. · Oral deferasirox can be used in children at least 2 years of age for chronic transfusion-related iron overload (Answer C is correct). Iron chelation should be considered when the patient has received about 120 mL/ kg of packed red blood cells (Answer A is incorrect). · Deferiprone is indicated only in adults (Answer B is incorrect). · Deferoxamine can be used for both acute and chronic iron overload (Answer D is incorrect).

Which statement most accurately describes the current standard-of-care approach to treating diffuse intrinsic pontine gliomas (DIPGs)? a. Surgery alone. b. Surgery with palliative chemotherapy. c. Palliative radiation. d. Radiation with radiosensitizing chemotherapy.

c. Palliative radiation. · Palliative radiation is often provided for children with a newly diagnosed DIPG (Answer C is correct). · Surgery is not routinely done for DIPG because of the location of the primary tumor and the potential for significantly morbidity and even mortality with such a surgery (Answers A and B are incorrect). · Recent clinical trials have investigated the utility of radiosensitizing agents; however, their use is not routine (Answer D is incorrect).

G.I. is a 7-year-old boy (weight 25 kg) who presents to the PICU intubated and mechanically ventilated for respiratory failure secondary to status asthmaticus. His pertinent medical history includes asthma and gastroesophageal reflux disease. Before intubation, he received albuterol/ipratropium x 3 doses, methylprednisolone 50 mg intravenously once, and magnesium sulfate 2 g intravenously once. On the patient's presentation to the PICU, the nurse reports red-tinged secretions after suctioning and coffee-ground nasogastric output. His laboratory values include K 3.1 mEq/L, SCr 1.6 mg/dL, Hgb 10.5 g/dL, and Hct 35%. Which is the best initial pharmacotherapy option for this patient? a. Octreotide 25 mcg intravenously x 1. b. Ranitidine 25 mg intravenously every 6 hours. c. Pantoprazole 20 mg intravenously once daily. d. Sucralfate 1 g by nasogastric tube every 8 hours.

c. Pantoprazole 20 mg intravenously once daily. · This patient likely has clinically nonsignificant UGIB, potentially from SRMD. He does not have a clinically overt UGIB, as shown by his normal Hgb/Hct, and has no history consistent with concern for variceal bleeding, for which octreotide might be indicated. · He does not have an acute GI bleed because his Hgb and Hct are moderately reduced. He should be treated for the prevention of clinically overt UGIB, for which the best option would be either an H2 RA or a PPI. The patient's SCr is higher than normal for his age; therefore, the ranitidine dose would need to be adjusted for decreased renal function. · Of the options, pantoprazole would be best, and if the patient has an ulcer that is the source of his bleeding, pantoprazole would provide sufficient acid suppression, which should allow for platelet aggregation and healing. · Sucralfate is not a first-line choice and could clog the patient's enteral feeding tubes.

A 5-kg patient is receiving adequate sedation with fentanyl and midazolam, scheduled mannitol 5 g every 6 hours, and 3% saline continuous infusion at 5 mL/hour for ICP management. Osmolarity is 350 mOsm/L, and serum sodium is 175 mEq/L. Which is the next most appropriate agent for management of an increased ICP? a. A 7-mL/kg bolus of 3% saline. b. Mannitol 0.5 g/kg x 1 now (regardless of timing of previous mannitol). c. Pentobarbital bolus, followed by continuous infusion. d. Phenytoin every 8-12 hours.

c. Pentobarbital bolus, followed by continuous infusion. · Barbiturate-induced coma would be the next step for this patient. · Mannitol scheduled every 6 hours will help reduce blood viscosity for a short while and will work as an osmolar agent. Hypertonic saline is at an appropriate dose and is optimized, given the osmolar and sodium limits (350 mOsm/L and 175 mEq/L, respectively). If an increased ICP were to occur, an extra dose of mannitol would probably have no effect. · Phenytoin, which has little effect on ICP, should be used as seizure prophylaxis, if indicated.

M.B. is a 17-year-old male adolescent who presents to the emergency department (ED) from the scene of a motor vehicle crash. He has uncontrolled bleeding from several sites of trauma (including head trauma) and is unable to communicate because he was sedated and intubated in the field. His mother, who was in the accident with him, states that she knows that M.B. has hemophilia but that she cannot remember which type. You are working in the ED, and the trauma physician asks you to recommend a pharmacologic factor replacement product for this patient. What is the most appropriate intervention at this time for M.B.? a. Send a serum sample to the laboratory for analysis and wait for the results before selecting a blood or factor replacement product. b. Suggest a recombinant factor VIII product because hemophilia A is the most common bleeding disorder in children. c. Recommend recombinant factor VIIa until the laboratory can provide more information about the type of hemophilia and the presence or absence of inhibitors. d. Provide a factor IX product because it will work for hemophilia A and hemophilia B.

c. Recommend recombinant factor VIIa until the laboratory can provide more information about the type of hemophilia and the presence or absence of inhibitors. · This patient has a known history of hemophilia but has no available medical records and is unconscious and critically bleeding. The type of hemophilia and the presence of inhibitors are unknown. The medical team will be using blood products, but the team is also inquiring about a pharmacologic option. Recombinant factor VIIa would be the most appropriate choice from the selections provided because factor VIIa is often indicated for patients with inhibitors to FVIII or FIX, so it can be used in both types of hemophilia (Answer C is correct). · Waiting for laboratory analysis could delay control of the bleeding, which could worsen this patient's clinical presentation (Answer A is incorrect). · Recombinant factor VIII might not be the best choice at this time because the patient may have another type of hemophilia, such as hemophilia B (which would require factor IX) or hemophilia A or B with inhibitors (which would be most benefited by factor VIIa; Answer B is incorrect). · Factor IX is effective only for hemophilia B, not hemophilia A (Answer D is incorrect).

T.K. is a 5-year-old girl (weight 20 kg) with a history of recurrent otitis media who presented to the PICU 2 days earlier with community-acquired pneumonia and respiratory failure. Her respiratory status continues to worsen, and she is given a diagnosis of acute respiratory distress syndrome (ARDS). She is currently intubated on fraction of inspired oxygen (Fio2 ) 0.8 (80% O2 ) and positive endexpiratory pressure (PEEP) 12 cm H2 O. Despite optimizing her midazolam and fentanyl infusions and bolus doses, she continues to be hypoxemic. Echocardiography (ECHO) reveals no signs of right ventricular dysfunction. Which would be the best medication therapy for this patient? a. Methylprednisolone 20 mg intravenously every 6 hours. b. Lorazepam 4 mg intravenously every 6 hours as needed for agitation. c. Rocuronium infusion initiated at 0.6 mg/kg/ hour. d. Inhaled nitric oxide at 20 ppm.

c. Rocuronium infusion initiated at 0.6 mg/kg/ hour. · There is little or no definitive evidence for medication therapy in pediatric patients. Adding neuromuscular blockade can be considered when adequate oxygenation is not achieved by sedation optimization. · Studies using corticosteroids for ARDS in adults have had inconsistent results on its effect on outcomes, and pediatric data are currently available only in case reports/case series; thus, corticosteroids cannot be recommended. · Inhaled nitric oxide is indicated in patients with known pulmonary hypertension or right ventricular failure, which this patient does not currently have.

S.K. is a 6-year-old girl with a diagnosis of ALK-positive high-risk neuroblastoma who is referred to your institution from another pediatric hospital. She received one cycle of chemotherapy before transfer to your institution. Which statement best describes the optimal approach to the management of her disease? a. S.K. should receive an additional five cycles of multiagent, conventional chemotherapy; surgery; and radiation. b. S.K. should receive an additional five cycles of multiagent, conventional chemotherapy; surgery; radiation; high-dose chemotherapy with autologous stem cell rescue; and maintenance isotretinoin. c. S.K. should receive an additional five cycles of multiagent, conventional chemotherapy; surgery; radiation; high-dose chemotherapy with autologous stem cell rescue; and maintenance immunotherapy with sargramostim, aldesleukin, dinutuximab, and isotretinoin. d. The standard treatment for children with high-risk neuroblastoma is typically limited to palliative chemotherapy and radiation.

c. S.K. should receive an additional five cycles of multiagent, conventional chemotherapy; surgery; radiation; high-dose chemotherapy with autologous stem cell rescue; and maintenance immunotherapy with sargramostim, aldesleukin, dinutuximab, and isotretinoin. · Treatment of high-risk neuroblastoma is aggressive and includes multiagent chemotherapy, surgery, radiation, high-dose chemotherapy with autologous stem cell rescue, and maintenance immunotherapy with sargramostim, aldesleukin, dinutuximab, and isotretinoin (Answer C is correct; Answers A and B are incomplete and thus incorrect). · In the United States, palliative care would not be appropriate for a patient with recently diagnosed high-risk neuroblastoma on frontline therapy (Answer D is incorrect).

12 year old female involved in a roll-over MCV with resultant depressed temporal fracture and subdural hematoma. The hematoma was evacuated in the OR during a 2 hour case and the patient returned to the unit intubated on low settings. The patient was started on epinephrine for hypotension. Choose the answer that most appropriately identified the risk factors for SRMD based on the information provided: a. Trauma, head or spinal injury, hypotension, mechanical ventilation b. Trauma, hypotension, head or spinal injury, coagulopathy c. Trauma, hypotension, head or spinal injury d. The patient does not exhibit any risk factors for SRMD

c. Trauma, hypotension, head or spinal injury · She wasn't in a major surgery (>4 hours), mechanical ventilation settings need to be higher, coagulopathy wasn't mentioned in the case · Contributory risk factors: acute hepatic/renal failure, anticoagulation, significant burn injury, high dose corticosteroid, history of GI bleed, hypotension, major surgery (>4 hours), sepsis, head or spinal injury

A 3 year old is mechanically ventilated in the PICU after a laryngeal tracheal reduction. On day 3 of mechanical ventilation, the patient develops fever, increased CRP, and increased WBC. Chest-X ray shows a new consolidation in the left lower lobe. A tracheal aspirate is obtained, but the intensivist would like to start empiric antibiotics now. Use the CMH 2017 antibiogram to help guide therapy. Choose the most appropriate empiric antibiotic therapy. a. Vancomycin 15 mg/kg IV q6hr and ceftriaxone 75 mg/kg IV q24h b. Cefepime 50 mg/kg IV q8hr c. Vancomycin 15 mg/kg IV q6hr and cefepime 50 mg/kg IV q8hr d. Vancomycin 15 mg/kg IV q6hr and cefepime 50 mg/kg IV q8hr and tobramycin 7.5 mg/kg IV q24hr

c. Vancomycin 15 mg/kg IV q6hr and cefepime 50 mg/kg IV q8hr · No septic shock, no ARDS, no CRRT, no prolonged hospitalization (>5 days): don't need to have two antipseudomonal agents (cephalosporin + aminoglycoside/ fluoroquinolone) - shouldn't do extended interval aminoglycoside if patient on CRRT or in AKI · CMH antibiogram: 467/1618 staph aureus isolates are MRSA - 29%. Need MRSA coverage in VAP if >10% of staph aureus isolates are MRSA (>20% in HAP)

A 2-year-old child, on long-term total parenteral nutrition secondary to short gut, presents with an acute viral endocarditis and is subsequently placed on ECMO for cardiac support. Sedation is achieved with fentanyl 5 mcg/kg/hour and midazolam 6 mcg/kg/minute (0.36 mg/kg/hour). He is to remain on ECMO for 14 days until his cardiac function recovers. As his heart improves, a decannulation and extubation is planned in 24-36 hours. Which is the most appropriate course for his sedation? a. Discontinue fentanyl and midazolam, and start morphine and lorazepam continuous infusions. b. Wean off fentanyl and midazolam by time of extubation. c. Wean off fentanyl and midazolam by time of extubation, and start lorazepam and methadone intravenously. d. Wean off fentanyl and midazolam by time of extubation, and start lorazepam and methadone nasogastrically.

c. Wean off fentanyl and midazolam by time of extubation, and start lorazepam and methadone intravenously. · Only weaning off fentanyl and versed by the time of extubation and not initiating other therapies is incorrect because a good level of sedation should be achieved before extubation to prevent the child from self-extubation or, worse, self-decannulation. · Should wean off fentanyl and versed and start IV lorazepam and methadone. · Administering NG options is also incorrect. During ECMO, this patient probably had poor blood flow to the gut, and it will take time for the gut to recover. This may in turn decrease drug absorption. · Goals with lorazepam and methadone weans: decrease by 10-20% daily or every other day (frequency can be less frequent) based on patient's clinical status

A 3-year-old girl (weight 15 kg) had a severe scald injury to 60% of her TBSA. Which is the most appropriate replacement rate for fluids during the next 24 hours? a. 113 mL/hour for 24 hours. b. 169 mL/hour for 8 hours; then 84 mL/hour for 16 hours. c. 225 mL/hour for 24 hours. d. 338 mL/hour for 8 hours; then 169 mL/hour for 16 hours.

d. 338 mL/hour for 8 hours; then 169 mL/hour for 16 hours. · 6 mL x 15 kg x 60 TBSA = 5400 mL/day. 2700 mL in the first 8 hours = 338 mL/hour for 8 hours. For the second half, 2700 mL in the next 16 hours = 169 mL/hour for 16 hours · When to do 6 mL/kg/TBSA over 2-4 mL/kg/TSBA: inhalation injury, larger burns >30% TBSA, deeper burns, children <2 yro, delayed treatment

A code is called on a 7-year-old child who was extubated yesterday after a 6-day stint of respiratory syncytial virus pneumonia. The team arrives at the scene and finds no pulse. Chest compressions are begun, and the patient is placed on a monitor. The monitor reveals PEA. The nurses cannot establish peripheral intravenous access after three attempts (10 minutes), and the patient does not have a central line. They are setting up for an emergency intubation, but they have not yet begun. The team is able to resuscitate the patient and has increased perfusion and pulses. The next day, the child begins to have some mental status changes, and the monitor reveals a wide QRS complex tachycardia. Which is the most appropriate therapy for this child? a. Adenosine. b. Amiodarone. c. Procainamide. d. Cardioversion.

d. Cardioversion. · This patient is having some type of VT. Because the patient is also having mental status changes, the condition is considered cardiopulmonary compromise. The immediate option is synchronized cardioversion. · If the patient were not having signs of compromise, adenosine (if regular rhythm and QRS monomorphic), amiodarone, or procainamide would be options, depending on the situation and rhythm.

T.D. is a 6-year-old girl (weight 22 kg) with a medical history pertinent for developmental delay, constipation, and chronic lung disease of prematurity. Admitted to the PICU 3 days ago after a traumatic brain injury, she underwent emergency neurosurgical intervention, and an intraventricular drain remains in place. A double-lumen temporary central line was placed during the surgical procedure and remains in place. She has been intubated and receiving mechanical ventilation since the day of admission. Today, her maximum temperature is 102.5°F, respiratory rate is 42 breaths/minute, and heart rate is 120 beats/minute. A tracheal aspirate obtained by a protected brush specimen this morning shows many gram-negative rods and many WBCs on stain. Which is the best empirical therapy for T.D.? a. Levofloxacin 10 mg/kg/dose intravenously twice daily and clindamycin 15 mg/kg intravenously every 8 hours. b. Ampicillin/sulbactam 50 mg/kg/dose (of ampicillin component) intravenously every 6 hours and vancomycin 15 mg/kg/dose intravenously every 6 hours. c. Gentamicin 7.5 mg/kg/dose intravenously every 24 hours and vancomycin 15 mg/kg/dose intravenously every 6 hours. d. Cefepime 50 mg/kg/dose intravenously every 8 hours and vancomycin 15 mg/kg/dose intravenously every 6 hours.

d. Cefepime 50 mg/kg/dose intravenously every 8 hours and vancomycin 15 mg/kg/dose intravenously every 6 hours. · Because this patient was admitted after a trauma and has been intubated and receiving mechanical ventilation for more than 2 days, she is at increased risk of VAP. · The most likely organisms for which this patient should empirically be covered include gram-negative bacilli and S. aureus. Despite having Gram stain information from the tracheal aspirate, broad empiric coverage should be initiated pending culture results. · Of the available options, cefepime, which will provide coverage for susceptible gram-negative bacilli and susceptible Staphylococcus, together with vancomycin for coverage of resistant gram-positive organisms, would be best, pending culture results.

E.J. is a 14-year-old male adolescent who was recently given a diagnosis of stage III anaplastic large cell lymphoma (ALCL). Which statement is most accurate regarding the therapeutic approach for E.J.'s disease? a. Brentuximab vedotin should be added to standard conventional chemotherapy in all children with ALCL. b. Crizotinib, which has shown significant efficacy in children and adolescents with newly diagnosed ALCL, should be given in lieu of conventional chemotherapy. c. Crizotinib, together with conventional chemotherapy, can increase overall survival (OS) rates to almost 95%. d. Conventional chemotherapy is the mainstay of treatment and can result in a 70%-85% OS rate.

d. Conventional chemotherapy is the mainstay of treatment and can result in a 70%-85% OS rate. · Conventional chemotherapy is the mainstay of treatment and can result in a 70%-85% OS rate (Answer D is correct). · Brentuximab vedotin and crizotinib are intriguing investigational agents that have yet to be proven efficacious as frontline agents, together with conventional chemotherapy in children with ALCL. Crizotinib as a single agent showed success in relapsed and refractory patients with ALCL, but it has not been studied as a single agent for newly diagnosed ALCL (Answers A-C are incorrect).

Which pair most closely links the toxicity with a causative chemotherapeutic agent? a. Nephrotoxicity and vincristine. b. Hemorrhagic cystitis and busulfan. c. Ototoxicity and doxorubicin. d. Electrolyte disturbances and cisplatin.

d. Electrolyte disturbances and cisplatin. · Nephrotoxicity is a hallmark adverse event associated with cisplatin and methotrexate, not vincristine. Vincristine is more commonly associated with peripheral neuropathy (Answer A is incorrect). · Hemorrhagic cystitis is more often associated with cyclophosphamide or ifosfamide. · busulfan may be more often associated with sinusoidal obstructive disease or seizures (Answer B is incorrect). · Anthracyclines such as doxorubicin are not commonly associated with ototoxicity; rather, they are associated with late cardiotoxicity (Answer C is incorrect). · Ototoxicity is a possible result of cisplatin or carboplatin therapy (Answer D is correct).

A code is called on a 7-year-old child who was extubated yesterday after a 6-day stint of respiratory syncytial virus pneumonia. The team arrives at the scene and finds no pulse. Chest compressions are begun, and the patient is placed on a monitor. The monitor reveals PEA. The nurses cannot establish peripheral intravenous access after three attempts (10 minutes), and the patient does not have a central line. They are setting up for an emergency intubation, but they have not yet begun. Which is the most appropriate next course of action for this patient? a. Continue chest compressions until intravenous access is obtained. b. Interrupt the first round of compressions to establish ET access for drugs. c. Administer 2-10 times the dose sublingually. d. Establish intraosseous access and administer same dose.

d. Establish intraosseous access and administer same dose.

A 13-year-old female patient with a long history of viral cardiomyopathy is in the PICU and on mechanical ventilation secondary to community-acquired pneumonia. All laboratory values are normal (SCr 0.7 mg/ dL). The patient's medications include fentanyl continuous infusion 1 mcg/kg/hour, midazolam 0.1 mg/kg/ hour, and vancomycin 20 mg/kg/dose every 6 hours. The vancomycin trough this morning was 16 mcg/mL (obtained at the appropriate time and before the fourth dose). The next day, the patient's ejection fraction decreased to 3% and was unable to perfuse her organs. The decision was made to initiate ECMO. Which is the most appropriate management strategy for this patient's medications now that she is on ECMO? (Assume monitoring of vancomycin concentrations.) a. Decrease midazolam and fentanyl dose; keep vancomycin the same. b. Increase midazolam and fentanyl dose; keep vancomycin the same. c. Increase midazolam and fentanyl dose; decrease vancomycin dose. d. Increase midazolam and fentanyl dose; increase vancomycin interval.

d. Increase midazolam and fentanyl dose; increase vancomycin interval. · Midazolam and fentanyl adsorb to circuit a lot (midazolam - 70% loss adsorption; fentanyl - 70-80% loss adsorption). Vancomycin also adsorbs to the circuit (30%). · Other PK changes with ECMO: volume of distribution increases, clearance decreases

T.N. is a 5-year-old girl (weight 20 kg) who is in the PICU for status epilepticus (SE). Seizures lasted about 15 minutes before cessation after two doses of benzodiazepines. Her current laboratory values include the following: sodium (Na) 137 mEq/L, potassium (K) 4.2 mEq/L, HCO3 - 18 mEq/L, chloride (Cl) 100 mEq/L, blood urea nitrogen (BUN) 20 mg/dL, serum creatinine (SCr) 0.3 mg/dL, and lactate 6 mmol/L. Her ABG includes pH 7.3 and Pco2 30 mm Hg. Which is the most likely cause of T.N.'s acid-base disorder? a. Hypoxemia secondary to the seizure. b. Hyperglycemia secondary to the seizure. c. Renal dysfunction secondary to the seizure. d. Lactic acidosis secondary to the seizure.

d. Lactic acidosis secondary to the seizure. · She was seizing for about 15 minutes, which can result in muscle breakdown and lactate production. Elevated lactate concentrations result in an elevated anion gap metabolic acidosis. · Hypoxemia secondary to seizures can result in acid-base disturbances, but the primary disturbance would be respiratory alkalosis (Answer A is incorrect). · Although DKA can result in an elevated anion gap metabolic acidosis, hyperglycemia without ketosis is not a cause of metabolic acidosis; also, prolonged seizures generally lead to hypoglycemia (Answer B is incorrect). · This patient's SCr is 0.3 mg/dL, which is not consistent with renal dysfunction (Answer C is incorrect).

After initial intervention, E.G.'s ECG now shows wide, polymorphic QRS complexes with a QRS axis shifting back and forth around the isoelectric baseline (i.e., torsades de pointes). Which would be best for treating E.G.? a. Amiodarone. b. Synchronized cardioversion. c. Lidocaine. d. Magnesium.

d. Magnesium.

M.C. is a 4-year-old girl who presents to her pediatrician with fever, pallor, and bone pain. The pediatrician does a complete blood cell count in the clinic and refers her to the cancer center at the local children's hospital. Further diagnostic analysis reveals a white blood cell count (WBC) of 89,000 cells/mm3 with 47% blasts on peripheral blood smear. The results of a bone marrow aspirate and biopsy are conclusive for acute lymphoblastic leukemia (ALL) with no known genetic abnormalities. Which statement about M.C.'s diagnosis is most accurate? a. Mortality from ALL has increased substantially during the past 4 decades because of increased environmental exposures and a lack of new therapeutic agents. b. Mortality from ALL has decreased substantially during the past 4 decades because of new, targeted agents such as monoclonal antibodies. c. Mortality from ALL has increased substantially during the past 4 decades despite a cooperative group approach to treatment and research. d. Mortality from ALL has decreased substantially during the past 4 decades because of the coordinated efforts of cooperative group treatment and research.

d. Mortality from ALL has decreased substantially during the past 4 decades because of the coordinated efforts of cooperative group treatment and research. · Acute lymphoblastic leukemia is the most common malignancy in children with cancer. · Although targeted therapies have proven beneficial in select populations (i.e., Ph+ ALL), their utility has not been proven in most patients with ALL.

Which of the following is not part of the definition of ARDS? a. Pulmonary edema of noncardiac origin due to damaged alveolar epithelial-endothelial barrier b. Occurs within 7 days of clinical insult (infection, trauma, burn, inhalation injury, pancreatitis, drowning, toxicity, etc.) c. New alveolar infiltrates appear on imaging d. Pulmonary edema of cardiac origin due to damaged alveolar epithelial-endothelial barrier

d. Pulmonary edema of cardiac origin due to damaged alveolar epithelial-endothelial barrier

A 3-year-old is in supraventricular tachycardia (SVT). Which is the most appropriate way to administer adenosine? a. Intravenous push over 2-5 minutes, followed by a saline flush over 2-5 minutes. b. Diluted infusion over 30 minutes. c. Rapid intravenous push, followed by a saline flush over 2-5 minutes. d. Rapid intravenous push, followed by a rapid saline flush.

d. Rapid intravenous push, followed by a rapid saline flush.

A.S. is a 3-year-old boy (weight 13 kg) admitted to the pediatric intensive care unit (PICU) for pneumonia. He is not currently receiving any form of positive pressure ventilation. His most recent arterial blood gas (ABG) is a pH of 7.48, with partial pressure of carbon dioxide (Pco2) 30 mm Hg and serum bicarbonate (HCO3 -) 22 mEq/L. Which primary acid-base disorder is most consistent with A.S.'s laboratory data? a. Metabolic acidosis. b. Respiratory acidosis. c. Metabolic alkalosis. d. Respiratory alkalosis.

d. Respiratory alkalosis. · pH high @ 7.48 (normal 7.35-7.45) · Pco2 low @ 30 (normal 35-45) · HCO3 normal @ 22 (22-28)

A 7 year old boy is about to begin his 1st cycle treatment for Burkitt lymphoma and a medical student knows that 1 of his treatment agents can be considered targeted due to its ability to selectively act on cells with the CD20 antigen. Which of the following agents would you identify for the student as meeting that description? a. Doxorubicin b. Methotrexate c. Vincristine d. Rituximab

d. Rituximab · Rituximab acts selectively on CD20 · Key treatment drugs: vincristine, prednisone, cyclophosphamide, doxorubicin, methotrexate (high-dose), rituximab

S.R. is a 3-year-old boy with trisomy 21 who presents to the ED with fatigue, pallor, petechiae, and pancytopenia. The team does a complete blood cell count with differential, a bone marrow aspirate and biopsy, and a lumbar puncture. His WBC is 12,000 cells/mm3. He receives a diagnosis of standard-risk ALL. Which approach is the most choice for administering methotrexate in this patient? a. S.R. should not receive any methotrexate because of the increased risk of severe mucositis in children with trisomy 21. b. S.R. should receive high-dose methotrexate and leucovorin rescue during consolidation because children with trisomy 21 have increased sensitivity to methotrexate. c. S.R. should receive intrathecal methotrexate with two doses of leucovorin rescue after each dose throughout therapy and 6 courses of high-dose methotrexate with leucovorin rescue during consolidation or interim maintenance. d. S.R. should receive intrathecal methotrexate throughout therapy plus two doses of leucovorin rescue after each dose during all phases except maintenance.

d. S.R. should receive intrathecal methotrexate throughout therapy plus two doses of leucovorin rescue after each dose during all phases except maintenance. · Children with Down syndrome (trisomy 21) are sensitive to the toxic effects of methotrexate. As such, they should receive intermediate-dose or Capizzistyle methotrexate in lieu of high-dose methotrexate (Answers B and C are incorrect). · All children with ALL should receive some intrathecal chemotherapy (Answer A is incorrect). In addition, they should be given two doses of oral leucovorin at hours 48 and 60 after a methotrexate-containing intrathecal injection during all phases of therapy except for maintenance (Answer D is correct).

A 5-year-old patient with no significant medical history presents to the emergency department in January with tachycardia (120 beats/minute), a temperature of 102°F (39°C), and an elevated white blood cell count of 25 x 103 cells/mm3 . Both the basic metabolic panel and the liver function tests are normal. While the team is examining the patient, a nurse notices a warm red area around the child's ankle. On questioning, the mother explains that he was cut in that area 5 days ago and that it did not heal correctly. A blood pressure reading is taken, which is 60/40 mm Hg. Which best represents the patient's stage of sepsis now? a. SIRS. b. Sepsis. c. Severe sepsis. d. Septic shock.

d. Septic shock. · The child's ankle has a warm area around it now, which appears to be an infection, placing him in sepsis. He is also hypotensive now, which signifies decompensated septic shock. · Severe sepsis would be correct IF the patient had other organ system dysfunction.

Match the chemotherapy agent used in HSCT conditioning regimens with its key monitoring parameter: a. Busulfan, thiotepa, rATG b. Vital signs during infusion, serum levels, regular skin inspection

· Busulfan: serum levels, SOS, seizures (pre-emptive antiepileptic) · Thiotepa: regular skin inspection and cleaning · rATG: vital signs during infusion

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