exam 2, deck 4 Flashcards
1
Q
liver mass and elevated alpha fetoprotein
A
Hepatoblastoma
2
Q
Azathioprine is used in what rheum
Class
SE
A
SLE
DMARD
Check TMPT enzyme to make sure med can be metabolized;
monitor for cytopenia
Can check 6-MMPN and 6TGN levels (presence of toxic metabolites
3
Q
Cyclosporine is used in what rheum
Class
SE
A
Membranous lupus nephritis
JDMS
DMARD
Grapefruit juice increases levels
pancytopenia
can check levels and monitor for renal disease
4
Q
side effects in glucocorticoids (methylprednisolone, prednisone, prednisolone)
A
HTN
atrophy of skin
impaired wound healing
body fluid retention
decreased body growth
hypernatremia
hypokalemia
peptic ulcer disease
liver function tests abnormal
increases risk of infection
muscle weakness
osteopenia/porosis
glaucoma
cataracts
depression
euphoria
5
Q
cyclophosphamide is used in what rheum
Class
SE
A
cytotoxic agent
SLE (severe nephritis or CNS involvement)
severe vasculitis
Hemorrhagic cystitis
fertility concerns
pancytopenia
malignancy
6
Q
Infliximab
etanercept
adalimumab
golimumab
is used in what rheum
Class
SE
A
Biologic - TNF alpha inhibitors
Spondyloarthropathy
JIA
Uveitis when firs/second line meds are ineffective
Heart failure (esp infliximab)
must check PPD prior to starting med (TB activation)
malignancy
demyelinating diseae
development of ANA/autoimmunity
7
Q
Anakinra (kineret)
canakinumab (LLaris)
Rilonacept (arcalyst)
is used in what rheum
Class
SE
A
SJIA
Biologic
IL-1 inhibitors
Immunosuppression
Anakinra not approved for sJIA
LLaris is monthly injection and approved for sJIA
8
Q
Abatacept
Orencia
is used in what rheum
Class
SE
A
Biologic
CTLa-4 Ig
JIA
SLE
no tox info listed
9
Q
Rituximab
is used in what rheum
Class
SE
A
SLE
JIA
JDMS
Biologic
immunize with pneumococcal and meningococcal vaccine prior
higher risk of infusion reaction
may need to administer IgG replacement after
10
Q
Actemra (tocilizumab)
is used in what rheum
Class
SE
A
JIA
Biologic
IL-6 antagonist
Need to monitor cell counts and liver function and adjust dose for abnormalities
11
Q
child with mild to moderate eczema history when is earliest age of peanut intro
A
around 6 months
12
Q
Child with no eczema or any food allergy earliest age of peanut intro
A
age appropriate and in accordance with family pref
13
Q
Cefepime dosing
how often
A
50mg/kg/dose
q 8
14
Q
Zosyn dosing
how often
A
80mg-100mg/kg
q 8
15
Q
meropenem dosing
how often
A
20-40mg/kg
q 8 hr
16
Q
how often do you give Flagyl
A
q6
17
Q
Tephilis med and dose
A
7.5mg/kg Flagyl q 6
18
Q
Clindamycin is given q
A
6
19
Q
clindamycin dosing and how often
A
40mg/kg
q 6
20
Q
normal ANC
A
> 1500
21
Q
what virus can cause lymphoma
A
EBV
22
Q
term used to describe the process by which a minor can participate in a decision r/t their health care.
A
Assent
23
Q
goal of oral immunotherapy (OIT)
A
to build tolerance
24
Q
Management of food allergy includes which of the following elements
a) strict avoidance
b) developing and communicating a food allergy action plan
c) dietary education
D) all of the above
A
D) all of the above
25
For a child weighing 20kg, the appropriate dose PRN allergic reaction would be
25 mg PO diphenhydramine
0.15mg IM epinephrine
26
electrolyte disturbances associated with Amphotericin B
Hypomagnesemia
Hypokalemia
Hyponatremia
27
What anemia has cafe au lait spots
Fanconi anemia
28
Leukocoria is associated with
Retinoblastoma
29
DiGeorge is associated with what electrolyte imbalance
hypocalcemia. usually needs dietary supplement
30
chromosome 22q11.2 deletion is
DiGeorge
31
electrolyte derangements associated with massive blood transfusion
hypocalcemia
Hyperkalemia
32
what lobe of brain
thought/memory/motor movements
frontal lobe
33
electrolyte derangements associated with HUS
hyperkalemia
hyponatremia
hyperphosphatemia
metabolic acidosis
34
infant being fed formula mixed with well water presents with acute hypoxia and no other resp symptoms. what are you thinking
Methemoglobinemia
35
what type of anemia is associated with iron tox
B thalassemia
36
what vasculitis can cause temp abnormal peristalsis that poses a risk for intussusception
HSP
37
most common gram neg organisms causing resp symptoms in a febrile neutropenic pt
Pseudomonas Aeruginosa
Pneumocystis jiroveci
Cag neg staph
38
what genetic condition is associated with leukemia
Trisomy 21
39
An 8-year-old African American girl is evaluated for complaints of fatigue, shortness of breath, rash, and cough for 3 weeks. A chest radiograph reveals small bilateral pleural effusions, pneumonitis, and cardiomegaly. Which of the following diagnosis should be considered in this child? a. Mycoplasma (e.g., walking) pneumonia. b. SLE. c. Cardiomyopathy. d. Localized scleroderma.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1358). Wolters Kluwer Health. Kindle Edition.
Answer: B A diagnosis of SLE is made by meeting at least 4 out of 11 criteria that are set forth by the American College of Rheumatology. SLE must be considered in early stages of presentation to prevent risk for increased organ damage when diagnosed late.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1358). Wolters Kluwer Health. Kindle Edition.
40
A 17-year-old with the diagnosis of SLE is admitted with chest pain, fatigue, dyspnea, and tachycardia. A murmur is noted on auscultation. Which of the following should be considered? a. “Shrinking lung” syndrome. b. Libman–Sacks endocarditis. c. Sepsis. d. Pericarditis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1358). Wolters Kluwer Health. Kindle Edition.
Libman–Sacks endocarditis is a form of nonbacterial endocarditis that should be considered in patients with SLE when a murmur is noted on examination. In conjunction with cardiac symptoms, the murmur may indicate progression of mitral valve disease.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1358). Wolters Kluwer Health. Kindle Edition.
41
A 15-month-old female toddler is seen in clinic with fever of 104°F for 2 days, generalized morbilliform rash, and an otherwise normal examination. Which of the following is the most appropriate course of action? a. Prescribe antipyretics and request follow-up in 3 days if no resolution of fever and/or rash. b. Prescribe antipyretics and reassure parents the most likely cause is viral, with fever and rash resolving spontaneously. c. Obtain a urinalysis, urine culture, and CBC. d. Obtain cerebrospinal fluid (CSF) and blood cultures.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1360). Wolters Kluwer Health. Kindle Edition.
Answer: A Prompt recognition is key to the diagnosis and treatment of Kawasaki disease. Typical and atypical classifications require 2 to 6 criteria be met, which include fever for at least 4 days in addition to the other manifestations. Antipyretics should be prescribed for comfort as well as for control of high fever, and reevaluation should be completed after 4 days of persistent fever to evaluate for other potential causes of fever and rash. Laboratory studies can assist in the evaluation of Kawasaki disease when the fever persists for 4 or more days.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1360). Wolters Kluwer Health. Kindle Edition.
42
A 4-year-old is seen in the emergency department with a history of fever for 1 week up to 106°F, conjunctivitis, complaints of generalized pain, nausea, and vomiting. The clinician suspects Kawasaki disease. Which of the following tests should be ordered first to assist in determining the diagnosis? a. Chest radiography, pancreatic function tests, and CSF cultures. b. CBC; blood, urine, and CSF cultures. c. Echocardiography, urine and blood cultures. d. CBC; liver and pancreatic function tests and inflammatory markers.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 1360-1361). Wolters Kluwer Health. Kindle Edition.
Answer: D Laboratory findings consistent for Kawasaki disease include elevated CRP and ESR, thrombocytosis, anemia, elevated transaminases, as well as pancreatitis. Aseptic meningitis is also seen, although less frequently. Echocardiography should be completed to evaluate for coronary aneurysms and to rule out pericardial effusion, although the absence of coronary abnormalities does not rule out Kawasaki disease. Echocardiography will be needed if the laboratory evaluation and clinical signs suggest Kawasaki disease; however, it is not needed in this stage of the evaluation.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1361). Wolters Kluwer Health. Kindle Edition.
43
a life-threatening disease of bone marrow failure resulting in decreased production of hematopoietic stem cells that results in peripheral pancytopenia and bone marrow aplasia.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 785). Wolters Kluwer Health. Kindle Edition.
aplastic anemia
44
treatment in aplastic anemia
Transfusions of RBCs and platelets.
* Antibiotic therapy.
* Bone marrow transplantation (BMT). * Immunosuppressive therapy if unable to receive a BMT.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 786). Wolters Kluwer Health. Kindle Edition.
45
A rare congenital hypoplastic anemia resulting in constitutional bone marrow failure.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 786). Wolters Kluwer Health. Kindle Edition.
Diamond–Blackfan Anemia (Diamond-Blackfan Anemia)
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 786). Wolters Kluwer Health. Kindle Edition.
46
mutation for Diamond-Blackfan is on chromosome ____ which encodes for a ribosomal protein known as _____
19
RPS19
47
Symptoms: pallor, fatigue, irritability, syncope, and dyspnea during feeding. * Physical examination: irregular heartbeat, hypotonia, short stature, and evidence of failure to thrive.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 787). Wolters Kluwer Health. Kindle Edition.
Diamond-Blackfan Anemia
48
* Associated with physical defects including craniofacial, hands, upper limbs, cardiac, or genitourinary.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 787). Wolters Kluwer Health. Kindle Edition.
Diamond-Blackfan Anemia
49
labs and dx for Diamond Blackfan Anemia
* Profound macrocytic anemia; WBCs and platelet count generally normal.
* Reticulocytopenia.
* Increased percentage of hemoglobin F for age. * Elevated erythrocyte adenosine deaminase activity.
* Decreased or absent erythroid precursors in bone marrow aspirate.
* Genetic screening; Diamond–Blackfan anemia—mutation in RPS19.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 787). Wolters Kluwer Health. Kindle Edition.
50
Treatment in Diamond Blackfan anemia
* Corticosteroids, frequent blood transfusion, BMT in some cases.
* Hematology, BMT, and endocrinology team involvement.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 787). Wolters Kluwer Health. Kindle Edition.
51
Triggers for DIC
* Gram-negative (most commonly associated with DIC) or gram-positive sepsis. * Viral processes. * Fungal infection. * Severe pancreatitis. * Trauma; penetrating brain injury, burns, or multiple trauma. * Hematologic malignancies; hemolytic processes. * Acute respiratory distress syndrome. * Obstetrical complication. * Necrotizing enterocolitis. * Extra Corporeal Membrane Oxygenation. * Graft-versus-host disease (GVHD).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 788). Wolters Kluwer Health. Kindle Edition.
52
s/s DIC
Symptoms: headache, altered level of consciousness, bleeding, disproportionate bruising. * Findings: diffuse bleeding; often initial symptom, petechiae, ecchymosis, purpura, and hematoma, gingival bleeding and epistaxis, hematuria, hematemesis and melena, intrahepatic hemorrhage, signs and symptoms of shock, thrombosis can be present, cool mottled skin, pallor, poor perfusion, tissue necrosis, and gangrene.
Multiorgan failure
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 789). Wolters Kluwer Health. Kindle Edition.
53
Lab findings DIC
* Prolonged PT. * Prolonged activated partial thromboblastin time. * Increased international normalized ratio (INR). * Decreased fibrinogen and platelet count. * Schistocytes (fragmented RBCs) on complete blood count (CBC) smear. * Elevated fibrin split product (FSP). * Positive/elevated D-dimer (marker is sensitive to endogenous generation of thrombin and plasmin).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 789-790). Wolters Kluwer Health. Kindle Edition.
54
mgmt DIC
* Supportive therapy should target specific organ symptoms affected while maintaining perfusion of vital organs until DIC is controlled. * Monitor vital signs, central venous pressure, and oxygen saturation. * Administer oxygen as needed; additional respiratory strategies as needed. * Antibiotics—organism specific for presumed or identified infectious etiology. * Fluid and electrolyte balance; correct acidosis and shock. * Administer vitamin K as indicated. * Frequent evaluation of laboratory studies—coagulopathy profile, CBC, chemistry, and acid–base balance (arterial or venous blood gases). * Blood product administration and replacement. * Cryoprecipitate—provides fibrinogen, factor VIII, and vonWillebrand factor. * Consider anticoagulation (e.g., heparin); controversial and not widely recommended; inhibits thrombin generation; efficacy has not been demonstrated in clinical trials. * Antithrombin III (ATIII)—An α2-globulin that inhibits coagulation. * Consider Aprotinin—slows fibrinolysis, not routinely used
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 790). Wolters Kluwer Health. Kindle Edition.
55
most common lab finding in DIC
Thrombocytopenia
56
In liver disease what does Factor VII and VIII look like?
Factor VII significantly decreased, Factor VIII normal or increased
57
Postdiarrheal or typical HUS; occurs in previously healthy children who have had recent gastroenteritis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 791). Wolters Kluwer Health. Kindle Edition.
D+ HUS
58
Atypical or sporadic HUS is less common and more severe
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 791). Wolters Kluwer Health. Kindle Edition.
D- HUS
59
HUS caused by inherited factor H deficiency
D- HUS
60
most common cause of D+ HUS
E.coli 0157:H7
61
causes of D- HUS
include Inherited factor H deficiency (10%–20%); inhibits complement activation, Membrane cofactor protein mutations, Streptococcus pneumoniae infection, medications including Cyclosporine and Tacrolimus.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 792). Wolters Kluwer Health. Kindle Edition.
62
diagnostic findings in HUS
microangiopathic hemolytic anemia, thrombocytopenia, and ARF. * Reticulocytosis and abnormal RBC morphology. * Schistocytes, burr, and helmet cells on smear; fragmented erythrocytes. * Anemia; decreased plasma haptoglobin. * Thromobocytopenia. * Leukocytosis is common. * Coagulation profile is often normal. * Stool cultures are often positive for E. coli 0157:H7, or other toxin-producing bacteria; however, not always detected. * Serum ELISA testing—should be done at diagnosis and repeated 2 weeks later (determines presence of antibodies to Shiga toxin E. coli serotypes). * Elevated BUN, serum creatinine, bilirubin, and potassium. * Coombs negative. * Microscopic hematuria, proteinuria, and casts on urinalysis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 793). Wolters Kluwer Health. Kindle Edition.
63
management D+ HUS
* Supportive therapy—90% of patients survive the acute phase. * Greater than 50% recover full renal function. * Antibiotics are not indicated (may stimulate the bacteria to release more toxins that can damage platelets, blood vessels, and kidneys). * Dialysis—(approximately 50% of patients). * Correct electrolyte imbalances, azotemia, manage fluid overload. * Maintain adequate nutrition and caloric intake while observing renal protective diet. * Correct anemia—75% of patients require PRBC transfusion. * Control hypertension. * Oral calcium channel blocker (e.g., nifedipine). * Intravenous (IV) calcium channel blocker (e.g., nicardipine) or nitroprusside.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 793-794). Wolters Kluwer Health. Kindle Edition.
64
treatment D- HUS
Plasmapheresis—consider for patients with factor H deficiency; may limit renal involvement temporarily, but does NOT prevent progression to end-stage renal disease and has not been shown to prevent recurrence of D−HUS. * Kidney transplantation—8% to 30% if recurrence persists. * Long-term follow-up with monitoring of BP and urinalysis. * Proteinuria, decreased glomerular filtration rate, and hypertension may recur up to 1 year later.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 794). Wolters Kluwer Health. Kindle Edition.
65
* Syndrome of immune overactivation leading to systemic inflammation and resulting damage to any organ(s) in the body.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 794). Wolters Kluwer Health. Kindle Edition.
Hemophagocytic Lymphohistiocytosis
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 794). Wolters Kluwer Health. Kindle Edition.
66
Gene defects associated with increased risk of HLH
Perforin (PRF), MUNC (UNC13D), Syntaxin 11 (STX11), and SAP (SH2-D1A).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 794-795). Wolters Kluwer Health. Kindle Edition.
67
what can trigger secondary HLH
Conditions associated with malignancy, autoimmune disease, immune deficiency, and specific infections (e.g., Ebstein–Barr virus, cytomegalovirus [CMV]).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 795). Wolters Kluwer Health. Kindle Edition.
68
s/s of HLH
Variable presentation; often appear quite ill. * Most “common” presentation is prolonged high fever (≥38.5ºC), hepatosplenomegaly, hepatitis, and cytopenias (at least two cell lines). * Can affect any organ system; notable systems include the skin, pulmonary, and CNS. * A high index of suspicion is needed; the diagnosis can often be confused with malignancy, Kawasaki Disease, and severe infection.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 795). Wolters Kluwer Health. Kindle Edition.
69
diagnostic for HLH
Five out of the eight criteria listed below are fulfilled:
* Fever ≥38.5°C.
* Splenomegaly.
* Cytopenias (affecting at least two of three lineages in the peripheral blood):
* Hemoglobin <9 g/dL (in infants <4 weeks: hemoglobin <10 g/dL).
* Platelets <100 × 103 cells/mL.
* Neutrophils <1 × 103 cells/mL.
* Hypertriglyceridemia (fasting >265 mg/dL) and/or hypofibrinogenemia (<150 mg/dL).
* Hemophagocytosis in bone marrow or spleen or lymph nodes or liver.
* Low or absent NK-cell activity.
* Ferritin >500 ng/mL (usually much higher in HLH).
* Elevated Soluble CD25 (alpha chain of soluble IL-2 receptor).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 796). Wolters Kluwer Health. Kindle Edition.
70
management of HLH
* Human leukocyte antigen (HLA) typing is usually sent early in the treatment course for possible stem cell transplantation. * Induction therapy is generally with dexamethasone, etoposide +/− cyclosporine, with treatment weaning over 8 weeks.
* In patients with genetic/familial predisposition or recurrent/refractory disease, an allogeneic stem cell transplant with a HLA-matched donor is indicated.
* Risk of recurrence is much higher in this patient population.
* Caution must be used with a HLA-matched sibling, as they may also have the genetic/familial predisposition to HLH.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 796-797). Wolters Kluwer Health. Kindle Edition.
71
an uncommon cause of cyanosis in infants and children that is a result of the heme iron being in the ferric rather than ferrous state. Under these conditions, oxygen binding to hemoglobin is severely impaired.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 807). Wolters Kluwer Health. Kindle Edition.
Methemoglobinemia
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 807). Wolters Kluwer Health. Kindle Edition.
72
Ewing Sarcoma most frequent location
axial skeleton
73
osteosarcoma is more commonly found in
long bones
74
Associated with somatic translocation 22q11—EWS-FLI1 fusion gene.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 844). Wolters Kluwer Health. Kindle Edition.
Ewing Sarcoma
75
“Starburst” or “bone in bone” formation noted in radiographs, lytic lesion, or “Codman triangle” suggests
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 844). Wolters Kluwer Health. Kindle Edition.
malignancy
76
Tumor arising from mesenchymal cells, likely skeletal in origin, although may present where skeletal muscle is not otherwise present.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 845). Wolters Kluwer Health. Kindle Edition.
Rhabdomyosarcoma
77
malignancy with Increased risk for children with RB mutation for retinoblastoma, neurofibromatosis-type 1, and other congenital anomalies.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 845). Wolters Kluwer Health. Kindle Edition.
Rhabdomyosarcoma
78
Rare childhood tumor arising from retina.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 847). Wolters Kluwer Health. Kindle Edition.
Retinoblastoma
79
inherited form of retinoblastoma with germline mutation in
RB-1 gene
80
lack of normal red reflex of the eye. Typically, white mass visualized in vitreous during ophthalmic examination.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 848). Wolters Kluwer Health. Kindle Edition.
Leukocoria
(Retinoblastoma)
81
malignancy Can be associated with congenital cancer predisposition syndromes including Beckwith-Wiedemann, WAGR syndrome
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 849). Wolters Kluwer Health. Kindle Edition.
Wilms tumor
82
WAGR syndrome
(Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation).
83
* Mouth sores and/or ulcerations from esophagus to rectum. * May cause severe pain, anorexia, dehydration, bleeding, or infection.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 854). Wolters Kluwer Health. Kindle Edition.
Graft vs Host disease
84
mgmt graft vs host disease
* Diligent oral care—may decrease severity. * Mouth rinses such as sodium chloride—may prevent infections. * Pain management—lidocaine mouth rinses, opioids. * Can be severe—may require continuous opioid infusions/PCA, TPN. * Usually resolves with return of bone marrow activity or with engraftment following HSCT.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 855). Wolters Kluwer Health. Kindle Edition.
85
A potentially life-threatening disorder, defined by the triad of neutropenia, abdominal pain, and fever.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 863). Wolters Kluwer Health. Kindle Edition.
Typhlitis
86
A 5-month-old female infant recently adopted from Southeast Asia presents to the Emergency Department (ED) with pallor, failure to thrive, frontal bossing, hepatosplenomegaly, and jaundice. In considering differential diagnoses, the most helpful diagnostic study results to indicate β-thalassemia would be: a. Absence of hemoglobin A on electrophoresis and microcytic anemia. b. Presence of hemoglobin SS on electrophoresis with normal RBC indices. c. Normal hemoglobin electrophoresis with hypochromic/microcytic anemia. d. Presence of fetal hemoglobin on electrophoresis with normal MCV.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 879). Wolters Kluwer Health. Kindle Edition.
Answer: A A hallmark finding of β-thalassemia is absence of hemoglobin A on electrophoresis, which is the study of choice for diagnosis along with microcytic anemia.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 879). Wolters Kluwer Health. Kindle Edition.
87
the peripheral blood smear reveals RBCs that are normocytic or macrocytic, and the remaining cellular elements are reduced in number, but morphologically normal.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 880). Wolters Kluwer Health. Kindle Edition.
aplastic anemia
88
8. A 9-year-old patient is referred to the ED for fatigue, pallor, and petechiae. She is clinically stable; however, a complete blood count reveals pancytopenia. The priority intervention for this patient is: a. Blood transfusion with PRBCs and platelets. b. Immediate consult to hematology. c. Administration of rasburicase and initiation of IVF at 1.5 times maintenance. d. Placement of a central venous catheter for administration of chemotherapy.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 881-882). Wolters Kluwer Health. Kindle Edition.
Answer: B Early evaluation of a patient with pancytopenia by pediatric oncology will allow for timely diagnosis and intervention. Although transfusion may be necessary, the consult is a priority as this patient is described as clinically stable.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 882). Wolters Kluwer Health. Kindle Edition.
89
the radiographic finding of a “starburst” mass makes the diagnosis of
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 882). Wolters Kluwer Health. Kindle Edition.
osteosarcoma
90
the radiographic finding of a “moth-eaten” or “onion-skin” appearance described in
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 882). Wolters Kluwer Health. Kindle Edition.
Ewing Sarcoma
91
Aggressive fluids to prevent tumor lysis syndrome in a new diagnosis starting chemo. IV fluids at ___ -__ L/m2/day
2-3L/m2/day
92
meds used in prevention and treatment of tumor lysis syndrome
Rasburicase and/or Allopurinol
93
A noninvasive test that can be ordered to confirm a suspicion of Neuroblastoma is:
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 883). Wolters Kluwer Health. Kindle Edition.
Urine for HVA and VMA (catecholamines).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 883). Wolters Kluwer Health. Kindle Edition.
94
Tachycardia, pallor, and a flow murmur in a child who is fatigued are indications of
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 884). Wolters Kluwer Health. Kindle Edition.
acute anemia
95
A four-year old presents to the emergency room (ED) with constipation, abdominal pain and distension, and general malaise. Of note, she also has periorbital ecchymosis, although her caregiver does not recall trauma. The child is hypertensive and ill appearing on initial examination. Which of the following is highest on the differential diagnosis? a. Wilm tumor. b. Ewing sarcoma. c. Hodgkin Lymphoma. d. Neuroblastoma.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 885-886). Wolters Kluwer Health. Kindle Edition.
Answer: D Although this patient could have Wilm tumor, the description portrays an ill child with a mass that is impairing her bowel habits. Additionally, she has periorbital ecchymosis, which is likely secondary to metastasis. The hypertension is the result of a sympathetic nervous system tumor arising from the adrenal medulla. Based on this patient’s age and the description of her clinically, Ewing sarcoma and Hodgkin Lymphoma are less likely.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 886). Wolters Kluwer Health. Kindle Edition.
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Children receiving anticoagulation with unfractionated heparin should be monitored: a. Using PT/INR with a goal of 2 to 3. b. With Factor X level with a goal of 0.5 to 1. c. For heparin-induced thrombocytopenia. d. With Anti-Xa with a goal of 0.5.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 886). Wolters Kluwer Health. Kindle Edition.
Answer: D Unfractionated heparin such as Enoxaparin is used for anticoagulation in many instances instead of heparin or warfarin as it is just as effective with consistent therapy. Factor Xa level is the monitor for effect, but is not always warranted for every patient.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 886). Wolters Kluwer Health. Kindle Edition.
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19. A 14-year-old with known sickle cell disease has a history of avascular necrosis of his shoulder a year ago. His current hemoglobin is 11.8 g/dL. Which type of SCD does the patient most likely have? a. HbSS. b. HbSC. c. HbSβ0 Thal. d. Sickle cell trait.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 886). Wolters Kluwer Health. Kindle Edition.
Answer: B Patients with HbSC typically have milder disease, mild anemia, or normal hemoglobin levels. They are also more commonly affected by vascular necrosis than the other types of SCD.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 886-887). Wolters Kluwer Health. Kindle Edition.
98
The parents of an 18-month-old female are concerned that her abdomen was distended while bathing her last night. She has no other signs or symptoms. On examination, a large right-sided abdominal mass is palpated. Which of the following findings would lead to a higher suspicion of Wilms’ Tumor? a. Multiple café-au-lait lesions. b. Hemihypertrophy. c. Ptosis of the right eye. d. Multiple petechiae and ecchymoses.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 887). Wolters Kluwer Health. Kindle Edition.
Answer: B Congenital hemihypertrophy is a condition of unknown etiology characterized by unilateral overgrowth of part or all of one side of the body, including limbs. Hemihypertrophy is a finding associated with Beckwith–Weidemann Syndrome (BWS). Patients with BWS have a higher risk of developing WIlms’ tumor.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 887). Wolters Kluwer Health. Kindle Edition.
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23. Which of the following therapies is most effective in managing vasoocclusive crisis in sickle cell disease? a. NSAIDs. b. Blood transfusion. c. Antibiotics. d. Cold packs.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 888). Wolters Kluwer Health. Kindle Edition.
Answer: A NSAIDs are used to manage vasoocclusive crisis. Other ways to manage vasoocclusive crisis include acetaminophen, opiates, heat, distraction, massage, hydration. Cold can trigger vasoocclusive crisis. Blood transfusions are used to treat other complications such as acute chest syndrome, splenic sequestration, and aplastic crisis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 888). Wolters Kluwer Health. Kindle Edition.
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Hyperleukocytosis is often seen in
new onset leukemia
101
Which test provides the most accurate diagnosis of methemoglobinemia? a. Spectrophotometric assay. b. CBC. c. Peripheral Blood Smear. d. Reticulocyte Count.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 889-890). Wolters Kluwer Health. Kindle Edition.
Answer: A Spectrophotometric assays are used to confirm the presence of methemoglobinemia and to determine the level of methemoglobin. Typically, methemoglobinemia occurs when there is a higher than 1% content in the RBCs.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 890). Wolters Kluwer Health. Kindle Edition.
102
A child with cyanosis, dyspnea, and tachycardia was diagnosed with methemoglobinemia secondary to sulfonamide antibiotics. The antibiotics were discontinued. The most appropriate next intervention is: a. Supportive care. b. Vitamin K administration. c. Blood transfusion.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 890). Wolters Kluwer Health. Kindle Edition.
d. Methylene blue administration. Answer: D Once the causative agent is removed or addressed, Methylene blue administration is the treatment of choice for children with methemoglobinemia who are symptomatic.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 890-891). Wolters Kluwer Health. Kindle Edition.
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31. A 9-month-old with a history of prematurity and chronic lung disease was admitted for acute respiratory failure due to RSV bronchiolitis. History includes a 1-month NICU stay with a diagnosis of heparin induced thrombocytopenia during that time. An ultrasound for edema of one lower extremity confirmed an acute nonocclusive thrombus in the left common femoral vein. Best choice for anticoagulation will be: a. Heparin infusion. b. Enoxaparin. c. Aspirin. d. Bivalirudin.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 892). Wolters Kluwer Health. Kindle Edition.
Answer: D Bivalirudin is a direct thrombin inhibitor and preferred choice of anticoagulation in a patient with a history of HIT. Enoxaparin is a low molecular weight heparin, and aspirin would not be an appropriate anticoagulant for deep vein thrombosis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 892). Wolters Kluwer Health. Kindle Edition.
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32. Analysis of a bone marrow aspirate specimen to identify chromosomal abnormalities is known as a. Cytogenetics. b. Flow cytometry. c. Polymerase chain reaction. d. Fluorescent in situ hybridization.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 892). Wolters Kluwer Health. Kindle Edition.
Answer: A Cytogenetics is the part of genetics that is involved with the study of the cell. Bone marrow aspirate can be evaluated for chromosomal abnormalities through this process.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 892-893). Wolters Kluwer Health. Kindle Edition.
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33. Type 1 Von Willebrand Disease is inherited as a a. X-linked disorder. b. Recessive disorder. c. Autosomal disorder. d. Y-linked disorder.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 893). Wolters Kluwer Health. Kindle Edition.
C
106
An irritable 6-month-old presents with a history of fatigue with feeds, pallor, and low tone. The physical examination indicates that the infant is less than the 3rd percentile for weight and height, tachycardic, and difficult to console. The initial CBC shows a profound macrocytic anemia, normal WBC, and normal platelet count. The reticulocyte count is very low. Which of the following diagnoses is highest on the differential list? a. Diamond–Blackfan Anemia. b. Aplastic Anemia. c. β-Thalassemia. d. DIC.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 893). Wolters Kluwer Health. Kindle Edition.
d. DIC. Answer: A Diamond–Blackfan anemia is a rare congenital hypoplastic anemia resulting in constitutional bone marrow failure characterized by a macrocytic anemia, normal WBC count, and normal platelet count. It is associated with an inadequate reticulocyte response.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 893). Wolters Kluwer Health. Kindle Edition.
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A child with β-thalassemia receives frequent blood transfusions. What is the best way to evaluate the need for iron chelation therapy? a. Serum ferritin levels and abdominal CT. b. Serum ferritin levels, superconducting quantum interference device and MRI. c. Serum iron levels and brain MRI. d. Serum iron levels and superconducting quantum interference device and CT.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 893-894). Wolters Kluwer Health. Kindle Edition. .
Answer: B Body iron burdens need to be assessed in children who receive frequent blood transfusions. Even though ferritin levels are an appropriate method of evaluation, levels may not reliably indicate total body iron stores. Superconducting quantum interference device and modified MRI is a noninvasive and effective method of determining
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 894). Wolters Kluwer Health. Kindle Edition.
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Which of the following tests would be reasonable to perform in the initial diagnostic evaluation HLH of this patient? a. Karyotype of the patient. b. Genetic testing for gene defects associated HLH, such as Perforin and Syntaxin-11. c. HLA typing of the patient. d. Bone marrow biopsy.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 894). Wolters Kluwer Health. Kindle Edition.
Answer: C This patient has a family history suspicious for a genetic/familial predisposition to HLH. It is reasonable to assume that this patient will ultimately proceed to a stem cell transplantation, in which case obtaining HLA typing early is critical in order to have time to find a proper HLA match. Genetic testing for gene defects in Perforin, Syntaxin-11 are appropriate, but not in the initial evaluation, and should be performed with the guidance of a pediatric hematologist/oncologist. Karyotype of the patient is not indicated in the evaluation for HLH.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 894-895). Wolters Kluwer Health. Kindle Edition.
109
The patient in question #2 (above) is started on HLH therapy. The caregivers ask which therapy will be used, and what the following steps will be? Which of the following is the correct response to these questions? a. Therapy will be with dexamethasone for 1 month, followed by etoposide for another month, then reevaluation. b. Therapy will be with dexamethasone and etoposide for 2 months, then if in remission, will likely proceed to a stem cell transplantation if a donor is available. c. As soon as the patient is stable, a stem cell transplantation with a HLA-matched donor is the next step. d. The patient’s underlying infection will be treated, and then the body will be allowed to “correct itself.”
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 895). Wolters Kluwer Health. Kindle Edition.
Answer: B Steroids are seldom used alone in treating HLH. Treatment duration is 8 weeks of upfront therapy, with stem cell transplantation as an option to those with a high risk of HLH recurrence. Treating any concurrent infection will seldom make the immune activation in HLH completely “correct itself.”
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 895). Wolters Kluwer Health. Kindle Edition.
