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1) . A neonate is noted to have an abnormally shaped face with a very small jaw. Several hours after birth, the baby develops convulsions and tetany.
Serum chemistries show the following: Sodium 1 40 mEq/L, Potassium 4 mEq/L, Chloride 100 mEq/L, Bicarbonate 24 mEq/L, Magnesium 2 mEq/L,
Calcium 5 mg/dL, Glucose 100 mg/dL. This child's disorder is associated with aplasia or hypoplasia of which of the following organs?
Ovaries
Pancreas
Pituitary
Thymus
Thyroid
2) 16 An 18-month-old child is brought to the emergency department by his mother due to a one-day history of lethargy and anorexia. He had a fever
the whole day yesterday, which responded to Tylenol (Acetaminophen). He then developed a petechial rash over his entire body, which worsened in
the last few hours. He is up-to-date with his immunizations, and is an otherwise healthy baby. On examination, he is drowsy and lethargic. He has neck
stiffness and appears septic. He flexes his hips when his neck is flexed. What is the most likely organism responsible for the patient's symptoms?
Meningococcus
Haemophilus influenza
Cytomegalo virus
Borrelia burgdorferi
Listeria monocytogenes
3) 6-month-old male is brought to the office due to fussiness and tugging at his right ear for the past 2 days. He has had a fever of 39.4° C (103° F) for
the past 2 days. His past medical history is significant for recurrent ear and lung infections, oral candidiasis, and persistent diarrhea by rotavirus. His
temperature is 39.4° C (103° F), pulse rate is 150/min, respirations are 28/min, and blood pressure is 80/60mm Hg. Physical examination reveals an
erythematous, bulging right tympanic membrane with poor mobility on pneumatic otoscopy. His lymph nodes are not palpable, and his tonsils are not
visualized. His B and T lymphocyte levels are markedly reduced. The chest x-ray reveals an absent thymic shadow. What is the most likely etiology of
this patient's condition?
. Severe combined immune deficiency
. Common variable immunodeficiency
. Bruton's agammaglobulinemia
. Wiskott-Aldrich syndrome
. Chronic granulomatous disease
4) A 1-day-old boy is evaluated in the nursery for minimal right arm movement. He was born at 41 weeks gestation to a woman with poorly controlled
type 1 diabetes mellitus. Attempted vaginal delivery was complicated by shoulder dystocia and became a cesarean section delivery. Family history is
positive for obesity in the boy's father and osteoporosis in both grandmothers. Birth weight was 4.5 kg. Examination shows crepitus and irregularity
over the right clavicle. Moro reflex is absent on the right. Bilateral biceps and grasp reflexes are intact and symmetric. The infant is plethoric and has
excessive fat accumulation in the abdominal and scapular regions. The remainder of the examination is normal. Which of the following is the most
significant risk factor for this patient's condition?
Brachial plexus injury
Family history of osteoporosis
In utero cerebrovascular accident
Maternal history of diabetes
Osteogenesis imperfecta
5) A 1-day-old infant appears dusky in the newborn nursery during feeding. Oxygen is immediately administered by nasal cannula. Shortly afterward,
she develops tachypnea. On physical examination, her blood pressure from the right upper arm is 50/30 mm Hg, her pulse is 180/min, and her
respirations are 60/min. An echocardiogram is consistent with hypoplastic left heart syndrome. Which of the following would likely be found on
auscultation?
. Continuous ductal murmur, bounding pulses
. Continuous ductal murmur, poor peripheral pulses
. Holosystolic murmur, poor peripheral pulses, quiet second heart sound
. No murmur, precordial hyperactivity, loud second heart sound
. No murmur, precordial hyperactivity, quiet second heart sound
6) A 1-week-old female infant is brought to the office by her 30-year-old African-American mother because she has been "crying a lot." She was born
at term. Her mother was diagnosed with gestational diabetes mellitus (GDM) at 24 weeks gestation and had been on insulin injections since. During
labor, there was a prolonged second stage due to difficulty in delivering the shoulders. Her Apgar scores were 8 and 10 at 1 and 5 minutes,
respectively. Her birth weight is 3.8 kg (8.5 Ib). On examination, the infant is active. On sudden extension of the head, there is extension of all the
extremities, except for the left upper extremity. There is crepitus over the left clavicular bone. Which of the following statements is true for this baby?
This infant has clavicular fracture and should be treated with a figure of eight bandage
This is a clavicular fracture and the infant should be evaluated for child abuse
This is Erb Duchenne palsy due to difficult shoulder delivery
This is a common outcome with large babies and related to gestational diabetes
This infant has clavicular fracture and should be treated with a surgery
7) A 1-week-old infant is brought to the emergency department due to vomiting and poor feeding. The prenatal and birth histories are unremarkable.
The infant's pulse is 140/min, temperature is 37 C (98.7 F), and capillary refill is 2 sec. He has sunken eyes, depressed anterior fontanel, and dry
,mucus membranes; skin elasticity is reduced. Heart and lungs are clear. There is no abdominal mass. Genital examination shows enlarged clitoris, and
fusion of the labioscrotal folds. The initial laboratory work-up reveals the following: Sodium 128 mEq/L, Potassium 5.8 mEq/L, BUN 25 mg/dl. If this
patient's karyotype is 46 XX, which of the following is most likely to be increased in this patient's serum?
17 -alpha-hydroxyprogesterone
18-hydroxycorticosterone
11-deoxycorticosterone
11-deoxycortisol
Corticosterone
8) A 1-week-old male newborn is seen in the office for "noisy breathing." The mother says that the "noisy breathing" is more prominent when the
infant is lying on his back, and improves when he is in a prone position with his chin up. The newborn is afebrile and has no cough, vomiting, or
cyanosis. There are no inspiratory retractions or wheezes. On direct laryngoscopy, the epiglottis is rolled in from side to side. Which of the following
statements is true about the child's condition?
. The child should be held in an upright position for 30 minutes after feeding and never fed while lying down
. The child requires immediate tracheostomy
. The child should be treated with intravenous ceftriaxone
. The child's condition is likely to deteriorate as the age advances
. In this condition the stridor improves when the child cries
9) A 1-year-old boy is brought to the physician by his parents for evaluation of bruising and blood in his stool. The child has had multiple episodes of
otitis media and has been hospitalized twice with pneumonia, but has never had bleeding or easy bruising in the past. He has been eating and drinking
well with no vomiting or diarrhea. He is afebrile with a heart rate of 150/min and a blood pressure of 80/40 mmHg. On examination, he is
well-developed, well-nourished, and has a fair complexion. There are dry, scaly patches on his cheeks and lower extremities. He also has bruising and
purpura on his lower extremities. His diaper contains a small amount of occult blood positive stool, but no fissures are seen on rectal examination. The
remainder of the physical examination is unremarkable. Laboratory studies show: Complete blood count: Hemoglobin 11 .5 g/dL, Platelet count
20,000/mm3, Leukocyte count 6,500/mm3, Neutrophils 76%, Eosinophils 1%, Lymphocytes 13%, Monocytes 10%. A peripheral smear reveals a low
number of platelets, and the platelets that are seen are small. Which of the following is the most likely cause of his bleeding?
. Bone marrow infiltration
. Platelet antibodies
. Nutritional deficiency
. Platelet activation and consumption
. Impaired platelet production
10) A 1-year-old boy presents with the complaint from his parents of “not developing normally.” He was the product of an uneventful term pregnancy
and delivery, and reportedly was normal at birth. His previous health-care provider noted his developmental delay, and also noted that the child seemed
to have an enlarged spleen and liver. On your examination, you confirm the developmental delay and the hepatosplenomegaly, and also notice that the
child has short stature, macrocephaly, hirsutism, a coarse facies, and decreased joint mobility. Which of the following is the most likely etiology of his
condition?
. Beckwith-Wiedemann syndrome
. Crouzon syndrome
. Trisomy 18 (Edwards syndrome)
. Jeune syndrome
. Hurler syndrome
11) A 1-year-old child with ALL in remission for 3 months is in the office for a health maintenance visit. He is due for multiple vaccinations including
hepatitis B vaccine, inactivated polio vaccine (IPV), varicella vaccine H. influenzae B vaccine (Hib), and pneumococcal vaccine (PCV). You remember
that some of these vaccines are live attenuated viruses and are contraindicated in immunocompromised patients. Which vaccine will you not give to this
patient?
PCV
varicella vaccine
hepatitis B vaccine
Hib
IPV
12) A 1-year-old patient is in the office for a health maintenance visit and is ready for immunizations. The child has a mild upper respiratory infection
and a low-grade fever. The mother does not want the child to receive vaccine because she has been told that the vaccine could make the illness worse.
You tell her the only true contraindication to vaccination is which of the following?
if the child has a skin rash
if there is an immunosuppressed adult in the householdif
the child has hypersensitivity to a vaccine componentif
a pregnant woman is in the household
if the mother is breast-feeding
13) A 10-year-old African American boy is brought to the office for the evaluation of worsening fatigue for the past few weeks. He has sickle cell
anemia, and has had several hospitalizations for painful crises. His vital signs are stable. He appears pale. He has a hemoglobin level of 7. 7 g/dl and
hematocrit of 22.5%. Which is the most likely type of anemia of this patient?
Iron deficiency anemia
Anemia of chronic disease
Megaloblastic anemia
Hemolytic anemia
Sideroblastic anemia
14) A 10-year-old boy comes to the office with fever and chills for 5 days and myalgia. He has recently returned from a 2-week vacation to New
,England with his family. On physical examination he has mild splenomegaly. Which of the following is the most likely cause of his symptoms?
Kawasaki disease
pneumococcus
babesiosis
leptospirosis
psittacosis
15) A 10-year-old boy is admitted to the hospital because of bleeding. Pertinent laboratory findings include a platelet count of 50,000/µL, prothrombin
time (PT) of 15 seconds (control 11.5 seconds), activated partial thromboplastin time (aPTT) of 51 seconds (control 36 seconds), thrombin time (TT)
of 13.7 seconds (control 10.5 seconds), and factor VIII level of 14% (normal 38%-178%). Which of the following is the most likely cause of his
bleeding?
. Immune thrombocytopenic purpura (ITP)
. Vitamin K deficiency
. Disseminated intravascular coagulation (DIC)
. Hemophilia A
. Hemophilia B
16) A 10-year-old boy presents with a 1-day history of fever, cough, and chest pain. He has not been eating and has been listless. He does not have
any previous history of health problems. On physical examination, his temperature is 40°C, and he is tachypneic. He looks ill. He has rales on his left
posterior lower lung fields. You order a chest x-ray. Which of the following organisms is most likely responsible for his pneumonia?
Haemophilus influenzae
Mycoplasma pneumoniae
Pneumocystis carinii
Staphylococcus aureus
Streptococcus pneumoniae
17) A 10-year-old boy presents with fever, headache, photophobia, and neck discomfort in the middle of summer. He is alert and oriented, but has
neck pain with flexion and extension of the head. His fundi are normal, and there are no focal neurologic findings or skin changes. A lumbar puncture
reveals normal protein and glucose with a cell count of 240/mL (90% lymphocytes). Which of the following is the most likely causative organism?
enterovirus (coxsackievirus or echovirus)
Streptococcus pneumoniae
Neisseria meningitides
Listeria monocytogenes
HSV-1
18) A 10-year-old boy was healthy until about 10 days ago when he developed 7 days of fever, chills, severe muscle pain, pharyngitis, headache,
scleral injection, photophobia, and cervical adenopathy. After 7 days of symptoms he seemed to get better, but yesterday he developed fever, nausea,
emesis, headache and mild nuchal rigidity. Cerebrospinal fluid (CSF) shows 200 white blood cells (WBC) per microliter (all monocytes) and an
elevated protein. Correct statements about this infection include which of the following?
. The condition is obtained from arthropod vectors
. CNS involvement is uncommon
. Most cases are mild or subclinical
. Appropriate treatment includes intravenous (IV) immune globulin (IVIG) and aspirin
. Hepatic and renal involvement occurs in the majority of cases
19) A 10-year-old girl has bullous target lesions and mucosal erythema, which developed after her third dose of trimethoprim-sulfamethoxazole for a
urinary tract infection. Which of the following is a likely associated clinical finding?
Diarrhea
Fever
Lymphadenopathy
Vomiting
Nausea
20) A 10-year-old male fell while riding his scooter down a steep hill. In the Emergency Department, his injuries included a fractured wrist and a
lacerated spleen, which required surgical removal. Two years later he is diagnosed with bacterial pneumonia. Which of the following bacterial agents is
the most likely pathogen for this patient's pneumonia?
. Escherichia coli
. Klebsiella pneumoniae
. Neisseria meningitidis
. Streptococcus pneumoniae
. Staphylococcus aureus
, 21) A 10-year-old presents with 2 months history of heavy menstrual-like bleeding. Menarche occurred 6 months ago and this first menses consisted of
spotting for 3-4 days without cramps. Subsequent periods were light in flow but lasted 6 to 8 days. Which of the following is the most likely cause of
her bleeding?
. Von Willebrand disease
. Ovarian tumor
. Thyroid disease
. Dysfunctional uterine bleeding
. Pregnancy
22) A 12-month-old patient has allergies to multiple foods. The child’s mother has eliminated the foods from the diet and wants to know if these
allergies will be lifelong. You tell her that some allergies do get better if the food is eliminated for 1–2 years. In which of the following is the allergy
most likely to resolve, with elimination of the food from the diet?
peanuts
milk
nuts
fish
shellfish
23) A 12-year-old African American boy is brought to the office due to a 2-day history of high-grade fever and chills. He was apparently well before
the onset of fever. He has no bone pain. He has sickle cell disease and has had 4 hospitalizations for painful crises and one episode of osteomyelitis.
His blood pressure is 90/60 mm Hg, pulse is 100/min, respirations are 22/min and temperature is 38.9 C (102F). He appears drowsy. His laboratory
report shows a total WBC count of 16,000/mm3 with 12% bands and Hb of 9.0 g/dl. Which of the following is the most likely cause of this patient's
condition?
Streptococcus pneumoniae
Staphylococcus aureus
Salmonella
Escherichia coli
Pseudomonas aeruginosa
24) A 12-year-old African American male is found to have a murmur during a routine sports physical. He has a family history of sudden death at a
young age. It is a harsh crescendo-decrescendo murmur that begins after S1 and is best heard at the left lower sternal border. Valsalva maneuver
intensifies the murmur. Which of the following is the most likely mitral valve abnormality in this patient?
. Dilated mitral valve annulus
. Rupture of chordae tendinae
. Abnormal mitral leaflet motion
. Mitral annulus calcifications
. Prolapse of the mitral valve
25) A 12-year-old boy is brought to the clinic by his parents because "he cannot walk anymore." Yesterday, he became irritable, uneasy, and
complained of tingling and weakness in both his legs. This morning, he couldn't stand up or move his legs, and complained of pain in his leg and thigh
muscles. He denies any headaches, nausea or vomiting. He had an episode of febrile diarrhea 10 days ago. His family history is unremarkable.
Physical examination reveals symmetric flaccid paralysis of both legs, absent deep tendon reflexes, and decreased superficial touch and vibratory
sense. There is no nuchal rigidity. What structure is most likely affected in this patient's condition?
Cerebral cortex
Medial lemniscus
Corticospinal tract
Gracile fasciculus
Peripheral nerves
26) A 12-year-old boy is brought to the emergency department because of severe pain near his left knee. He has sickle cell disease and has been
hospitalized previously for sickle cell crisis. Vital signs are notable for persistent fever. Examination of the left lower extremity reveals a normal knee
joint with marked tenderness and swelling over the proximal tibia. Laboratory studies show leukocytosis and elevated ESR. Imaging studies confirm the
diagnosis of osteomyelitis. Which of the following organisms is the most likely cause of his condition?
. Escherichia coli
. Pseudomonasspecies
. Salmonella species
. Streptococcus pneumoniae
. Group B streptococcus
27) A 12-year-old boy is brought to the emergency department because of severe pain near his left knee. He has sickle cell disease, and has been
hospitalized previously for sickle cell crisis. Vital signs are notable for mild fever. Examination of the left lower extremity reveals a normal knee joint
with marked tenderness and swelling over the proximal tibia. Labs show leukocytosis and elevated ESR. He is subsequently diagnosed with
osteomyelitis. Which of the following organisms is the most likely cause of his condition?
. Escherichia coli
. Pseudomonas species
. Salmonella species
. Staphylococcus aureus
. Group B streptococcus