05-03-2011, 06:27 PM
1. A 6-yr-old girl has had intermittent fever, decreased appetite,
and weight loss since starting first grade 4 mo ago. Physical examination
reveals generalized lymphadenopathy, and a chest film shows prominent paraaortic
nodes. Past medical history is negative for infection except for
Salmonella paratyphi septicemia and liver abscess at 3 yr of age, successfully
treated with antibiotics. Cervical lymph node biopsy shows marked histiocytic
infiltration but no granulomas or giant cells; the acid-fast stain is positive. The
most likely diagnosis is:
A.Congenital hypogammaglobulinemia
B.Leukocyte mycobactericidal defect
C.Severe combined immunodeficiency disease
D.Normal child
E.Langerhans cell histiocytosis
A 1-yr-old child presents with a history of recurrent fevers for several weeks. On
physical examination you find that the pupils fail to constrict. This finding is associated with:
A.Toxoplasmosis
B.Ectodermal dysplasia
C.Juvenile rheumatoid arthritis
D.Hypothalamic dysfunction
E.Thyrotoxicosis
2. All of the following are typically associated with an eosinophilic
response and eosinophilia except:
A.Allergic rhinitis
B.Hypersensitivity drug reactions
C.Trichinosis (Trichinella spiralis)
D.Pinworms (Enterobius vermicularis
3.All of the following are associated with chronic granulomatous
disease (CGD) except:
A.X-linked and autosomal recessive inheritance
B.Pyloric outlet obstruction
C.Aspergillus pneumonia
D.Perianal abscess
E.Hypogammaglobulinemia
4 a.A 5-yr-old boy presents with his third episode of painful cervical
lymphadenitis. Each was treated with incision and drainage, and cultures grew
Staphylococcus aureus. At the age of 2 yr, he required surgical aspiration of a
liver abscess. The most important laboratory test is:
A.PCR assay for ADA deficiency
B.Fluorescence assay using dihydrorhodamine 123
C.MAC-1 assay
D.Neutrophil count
E.Bone marrow aspiration
4 b.The most likely diagnosis for the patient described in Question
24 is:
A.Bruton agammaglobulinemia
B.AIDS
C.Chronic granulomatous disease
D.Kostmann disease
E.Cyclic neutropenia
5.Neutropenia is noted in children in all of the following
conditions except:
A.Kostmann disease
B.Viral infection
C.Maternal preeclampsia
D.Hunter syndrome
6.A 5-mo-old girl presents with diarrhea and malabsorption, and
on initial laboratory testing has a WBC count of 900/mm3. Which of the
following is the most likely diagnosis?
A.Cystic fibrosis
B.Shwachman-Diamond syndrome
C.Cyclic neutropenia
D.Chronic granulomatous disease
E.Severe combined immunodeficiency
7.All of the following may be associated with neutropenia except
A.Leukocyte adhesion deficiency
B.Shwachman-Diamond syndrome
C.Cartilage-hair hypoplasia
D.Chédiak-Higashi syndrome
E.Glycogen storage disease type Ib
8.Features associated with cyclic neutropenia include:
A.An oscillatory period of 21 ± 3 days
B.Oral ulcerations and stomatitis
C.Serious infections including pneumonia
D.Septicemia, notably with Clostridium perfringens
E.All of the above
9.A 6-mo-old child presents with recurrent cellulitis and
bacteremia due to Staphylococcus aureus. The white blood cell count is
2500/mm3 with 5% neutrophils, 10% eosinophils, 35% monocytes, and 50%
lymphocytes. The platelet count is 650,000/mm3. A brother and a female
cousin died at the ages of 18 mo and 2 yr, respectively. The most likely
diagnosis is:
A.AIDS
B.Severe combined immunodeficiency
C.Kostmann disease
D.Cyclic neutropenia
E.Chronic granulomatous disease
9b.Long-term treatment of the disease described in above question is
best accomplished with:
A.Prophylactic antibiotics
B.Intravenous immunoglobulin monthly
C.IFNRecombinant
D.Human G-CSF
10.A 12-yr-old boy has a total white blood cell count of
13,000/mm3, with 60% neutrophils, 14% bands, 25% lymphocytes, and 1%
monocytes. Which of the following describes this result?
A.Neutrophilia
B.Leukocytosis
C.Shift to the left
D.Leukocytosis and shift to the left
E.Neutrophilia, shift to the left, and lymphopenia
11.Most evidence suggests that fever is an adaptive response, and that
antipyretics provide symptomatic relief but do not change the course of diseases
except in selected circumstances. Patients with which of the following underlying
conditions should be treated with antipyretics for fever as an essential part of
treatment?
A.Osteomyelitis
B.Diabetes mellitus
C.Inflammatory bowel disease
D.Chronic lung disease
E.Meningitis
12.An 8-yr-old boy with sickle cell disease presents with fever and symptoms of an
acute illness. You consider treating him as an outpatient with intramuscular
ceftriaxone. Which of the following findings should lead to hospitalization?
A.Temperature > 40°C
B.WBC count < 5,000 cells/μL
C.WBC count > 30,000>cells/μL
D.Pulmonary infiltrates
E.Any of the above
13.A 22-day-old infant is noted by his mother to have a rectal temperature of 38.3°C.
She reports that he has been acting normal and appears generally well. He was born
at 37 wk of gestation, went home with his mother at 24 hr of life, and has done well
since. There is no known underlying illness. The physical examination is normal.
The infant's WBC count is 19,500/mm3 and the absolute band count is 850/mm3.
There are 4 WBCs/mm3 in an unspun urine sample, and results of a Gram stain of
the urine are negative. This infant fails to meet criteria for low risk of serious
bacterial infection because:
A.He was born prematurely.
B.The WBC count is elevated.
C.The absolute band count is elevated.
D.There is a urinary tract infection.
E.He is less than 1 mo of age.
14.The triage history of a 9-mo-old child presenting with a temperature of 39.2°C
shows the immunization history as "up-to-date." Further review of the medical
record shows that the patient has had three doses of Hib conjugate vaccine. Of the
following immunizations, which is the most important to document before
determining any management decisions?
A.Hepatitis B
B.DTaP
C.IPV
D.Pneumococcal conjugate vaccine
E.Meningococcal conjugate vaccine
15.Thirty minutes after admission, an 18-yr-old girl with meningococcemia has just finished
receiving the last of her 60 mL/kg infusion of normal saline. The nurse tells you that the
patient's heart rate is still 120/min, that her blood pressure is 70/30 mm Hg, and that pink,
frothy material is being suctioned from the patient's endotracheal tube. The patient's
extremities are warm, with bounding pulses. The next step in the resuscitation is to:
A.Administer 20 mL/kg of lactated Ringer's solution
B.Administer 10 mL/kg of 25% albumin
C.Administer NaHCO3
D.Administer nitroprusside
E.Administer norepinephrine
enjoy!
and weight loss since starting first grade 4 mo ago. Physical examination
reveals generalized lymphadenopathy, and a chest film shows prominent paraaortic
nodes. Past medical history is negative for infection except for
Salmonella paratyphi septicemia and liver abscess at 3 yr of age, successfully
treated with antibiotics. Cervical lymph node biopsy shows marked histiocytic
infiltration but no granulomas or giant cells; the acid-fast stain is positive. The
most likely diagnosis is:
A.Congenital hypogammaglobulinemia
B.Leukocyte mycobactericidal defect
C.Severe combined immunodeficiency disease
D.Normal child
E.Langerhans cell histiocytosis
A 1-yr-old child presents with a history of recurrent fevers for several weeks. On
physical examination you find that the pupils fail to constrict. This finding is associated with:
A.Toxoplasmosis
B.Ectodermal dysplasia
C.Juvenile rheumatoid arthritis
D.Hypothalamic dysfunction
E.Thyrotoxicosis
2. All of the following are typically associated with an eosinophilic
response and eosinophilia except:
A.Allergic rhinitis
B.Hypersensitivity drug reactions
C.Trichinosis (Trichinella spiralis)
D.Pinworms (Enterobius vermicularis
3.All of the following are associated with chronic granulomatous
disease (CGD) except:
A.X-linked and autosomal recessive inheritance
B.Pyloric outlet obstruction
C.Aspergillus pneumonia
D.Perianal abscess
E.Hypogammaglobulinemia
4 a.A 5-yr-old boy presents with his third episode of painful cervical
lymphadenitis. Each was treated with incision and drainage, and cultures grew
Staphylococcus aureus. At the age of 2 yr, he required surgical aspiration of a
liver abscess. The most important laboratory test is:
A.PCR assay for ADA deficiency
B.Fluorescence assay using dihydrorhodamine 123
C.MAC-1 assay
D.Neutrophil count
E.Bone marrow aspiration
4 b.The most likely diagnosis for the patient described in Question
24 is:
A.Bruton agammaglobulinemia
B.AIDS
C.Chronic granulomatous disease
D.Kostmann disease
E.Cyclic neutropenia
5.Neutropenia is noted in children in all of the following
conditions except:
A.Kostmann disease
B.Viral infection
C.Maternal preeclampsia
D.Hunter syndrome
6.A 5-mo-old girl presents with diarrhea and malabsorption, and
on initial laboratory testing has a WBC count of 900/mm3. Which of the
following is the most likely diagnosis?
A.Cystic fibrosis
B.Shwachman-Diamond syndrome
C.Cyclic neutropenia
D.Chronic granulomatous disease
E.Severe combined immunodeficiency
7.All of the following may be associated with neutropenia except
A.Leukocyte adhesion deficiency
B.Shwachman-Diamond syndrome
C.Cartilage-hair hypoplasia
D.Chédiak-Higashi syndrome
E.Glycogen storage disease type Ib
8.Features associated with cyclic neutropenia include:
A.An oscillatory period of 21 ± 3 days
B.Oral ulcerations and stomatitis
C.Serious infections including pneumonia
D.Septicemia, notably with Clostridium perfringens
E.All of the above
9.A 6-mo-old child presents with recurrent cellulitis and
bacteremia due to Staphylococcus aureus. The white blood cell count is
2500/mm3 with 5% neutrophils, 10% eosinophils, 35% monocytes, and 50%
lymphocytes. The platelet count is 650,000/mm3. A brother and a female
cousin died at the ages of 18 mo and 2 yr, respectively. The most likely
diagnosis is:
A.AIDS
B.Severe combined immunodeficiency
C.Kostmann disease
D.Cyclic neutropenia
E.Chronic granulomatous disease
9b.Long-term treatment of the disease described in above question is
best accomplished with:
A.Prophylactic antibiotics
B.Intravenous immunoglobulin monthly
C.IFNRecombinant
D.Human G-CSF
10.A 12-yr-old boy has a total white blood cell count of
13,000/mm3, with 60% neutrophils, 14% bands, 25% lymphocytes, and 1%
monocytes. Which of the following describes this result?
A.Neutrophilia
B.Leukocytosis
C.Shift to the left
D.Leukocytosis and shift to the left
E.Neutrophilia, shift to the left, and lymphopenia
11.Most evidence suggests that fever is an adaptive response, and that
antipyretics provide symptomatic relief but do not change the course of diseases
except in selected circumstances. Patients with which of the following underlying
conditions should be treated with antipyretics for fever as an essential part of
treatment?
A.Osteomyelitis
B.Diabetes mellitus
C.Inflammatory bowel disease
D.Chronic lung disease
E.Meningitis
12.An 8-yr-old boy with sickle cell disease presents with fever and symptoms of an
acute illness. You consider treating him as an outpatient with intramuscular
ceftriaxone. Which of the following findings should lead to hospitalization?
A.Temperature > 40°C
B.WBC count < 5,000 cells/μL
C.WBC count > 30,000>cells/μL
D.Pulmonary infiltrates
E.Any of the above
13.A 22-day-old infant is noted by his mother to have a rectal temperature of 38.3°C.
She reports that he has been acting normal and appears generally well. He was born
at 37 wk of gestation, went home with his mother at 24 hr of life, and has done well
since. There is no known underlying illness. The physical examination is normal.
The infant's WBC count is 19,500/mm3 and the absolute band count is 850/mm3.
There are 4 WBCs/mm3 in an unspun urine sample, and results of a Gram stain of
the urine are negative. This infant fails to meet criteria for low risk of serious
bacterial infection because:
A.He was born prematurely.
B.The WBC count is elevated.
C.The absolute band count is elevated.
D.There is a urinary tract infection.
E.He is less than 1 mo of age.
14.The triage history of a 9-mo-old child presenting with a temperature of 39.2°C
shows the immunization history as "up-to-date." Further review of the medical
record shows that the patient has had three doses of Hib conjugate vaccine. Of the
following immunizations, which is the most important to document before
determining any management decisions?
A.Hepatitis B
B.DTaP
C.IPV
D.Pneumococcal conjugate vaccine
E.Meningococcal conjugate vaccine
15.Thirty minutes after admission, an 18-yr-old girl with meningococcemia has just finished
receiving the last of her 60 mL/kg infusion of normal saline. The nurse tells you that the
patient's heart rate is still 120/min, that her blood pressure is 70/30 mm Hg, and that pink,
frothy material is being suctioned from the patient's endotracheal tube. The patient's
extremities are warm, with bounding pulses. The next step in the resuscitation is to:
A.Administer 20 mL/kg of lactated Ringer's solution
B.Administer 10 mL/kg of 25% albumin
C.Administer NaHCO3
D.Administer nitroprusside
E.Administer norepinephrine
enjoy!