Aquifer end-of-module Questions

Question 1

A 33-year-old G1P0 female with a history of medically controlled seizures gives birth vaginally to a boy with IUGR at 38 weeks’ gestation. The newborn is noted to have dysmorphic cranial features and his head circumference is 28.5 cm (< 5th percentile). What is another associated abnormality you might expect to see in this newborn?

Answer 1

Cardiac defects

Taking anticonvulsants during pregnancy may lead to cardiac defects, dysmorphic craniofacial features, hypoplastic nails and distal phalanges, IUGR, and microcephaly. Mental retardation may be seen. A rare neonatal side effect is methemoglobinuria.

NOT:
A. Hepatosplenomegaly
C. Absent red reflex
D. Chorioretinitis
E. Tremors

Question 2

A 19-year-old female in her 38th week of pregnancy goes into active labor. Shortly after birth her baby is noted to have a high-pitched cry, tremulousness, hypertonicity, and feeding difficulties. The baby is otherwise developmentally normal and the remainder of the physical exam also is normal. What is the drug the baby’s mother likely used during her pregnancy?

Answer 2

Heroin

Maternal opiate use during pregnancy places the newborn at risk for neonatal abstinence syndrome. Affected newborns may exhibit CNS findings (irritability, hyperactivity, hypertonicity, incessant high-pitched cry, tremors, seizures), GI symptoms (vomiting, diarrhea, weight loss, poor feeding, incessant hunger, excessive salivation), and respiratory findings (including nasal stuffiness, sneezing, and yawning).

NOT:
B. Alcohol
C. Marijuana
D. Cocaine
E. Tobacco

Question 3

A 19-year-old G1P0 female presents in labor to the ED at 38 gestational weeks. On interview it is discovered that the patient has had irregular prenatal care, drank a couple of beers every weekend, and smoked 4 cigarettes a day during her pregnancy. She delivers a baby boy who is small for gestational age. On exam, it is noted the baby has microcephaly, a smooth philtrum, and a thin upper lip. What do you suspect caused these features in the baby?

Answer 3

Alcohol exposure

Fetal alcohol syndrome has very characteristic facial features, including a smooth philtrum, thinning of the upper lip, and small palpebral fissures.

NOT:
A. Tobacco exposure
C. Congenital rubella
D. Vertically transmitted HIV
E. Congenital CMV infection

Question 4

A 36-month-old presents for a well child visit. The parents would like to know if the child’s development is progressing appropriately. The child passed a hearing test at birth, and other than a few URIs, has been generally healthy. The child has not had any hospitalizations or serious illnesses. The child is able to run well, walk up stairs, walk slowly down stairs, uses more words than the parents are able to count, but can only use them in short, two or three-word sentences. The child’s speech is understandable and the child can draw a circle, but not a cross. Neurological examination reveals normal cranial nerves, normal sensitivity, normal motor reflexes, and no Babinski sign. Which of the following is the next best step in management of this child?

Answer 4

Reassure the parents that the boy’s development appears normal

The developmental milestones mentioned in the vignette are within the range of normal for a 36-month-old child. In the absence of any other evidence of significant impairment, there is no indication for referral at this point.

NOT:
A. Perform a brain-stem auditory evoked potential hearing screen
B. Perform a screening exam for autism
D. Refer the child to a developmental specialist for comprehensive evaluation
E. Refer the child to a specialist for evaluation of his delayed motor development

Question 5

Sammy is a healthy male child brought into your office by his mother for a well-child examination. As part of your evaluation you assess his developmental milestones. He is able to run, make a tower of 2 cubes, has 6 words in his vocabulary, and can remove his own garments. What would you estimate Sammy’s age to be based upon his developmental milestones?

Answer 5

18 months

At 18 months, a child should be able to walk backward, and 50-90% of children can run at this stage. An 18-month-old should be able to scribble, build a tower of 2 cubes, have 3-6 words in her or his vocabulary, and be able to help in the house and remove garments.

NOT:
A. 12 months
B. 15 months
D. 30 months
E. 36 months

Question 6

A 5-month-old male is brought to the urgent care clinic with a 3 day history of rhinorrhea and non-productive cough. At birth the baby was large for gestational age and exam at the time was notable for macrocephaly, macroglossia, and hypospadias. Vital signs are stable on physical exam at this time. There is copious nasal discharge, but lungs are clear to auscultation. On abdominal exam, you palpate an abdominal mass on the right side just below the subcostal margin. It is 7 cm in diameter and does not cross the midline. The abdomen is soft and non-tender with active bowel sounds. What is the most likely cause of his mass?

Answer 6

Wilms’ tumor

Wilms’ tumor is commonly associated with Beckwith-Wiedemann syndrome, a genetic overgrowth syndrome. Other features that may be seen in children with this syndrome include omphalocele, hemihypertrophy, hypoglycemia, large for gestational age, and other dysmorphic features.

NOT
A. Hepatoblastoma
B. Renal cell carcinoma
C. Teratoma

Question 7

An asymptomatic, healthy 9-month-old is found to have a palpable RUQ mass on exam. After further imaging and lab studies, the mass is diagnosed as a neuroblastoma that has involvement in the bone marrow as well. The mother is worried about the prognosis. Which of the following is true about the prognosis of neuroblastoma in this child?

A. Children who are older than 12 months have a better prognosis than younger children
B. Favorable histology does not play a role in prognosis
C. Lymph node involvement is a poor prognostic factor
D. Non-amplification of the n-myc gene is a favorable prognostic factor
E. Prognosis of neuroblastoma is predictable

Answer 7

Non-amplification of the n-myc gene is a favorable prognostic factor

NOT:
A. Children who are older than 12 months have a better prognosis than younger children
B. Favorable histology does not play a role in prognosis
C. Lymph node involvement is a poor prognostic factor
E. Prognosis of neuroblastoma is predictable

Question 8

A 9-month old comes to the clinic for a well-child visit. The child is at the 50th percentile for weight, length, and head circumference and is reaching all developmental milestones appropriately. The mother has no concerns at this visit. The child has previously received the following vaccines: 3 doses of DTaP, 3 doses of Hib, 2 doses of HepB, 3 doses of RotaV, 2 doses of IPV and 3 doses of PCV13, and no influenza vaccines. Which vaccines should the child receive at today’s visit?

A. Hep B, DTaP, IPV
B. Hep B, IPV, and MMR
C. Influenza, Hep B, IPV
D. Influenza, Hep B, IPV, DTaP
E. Influenza, IPV

Answer 8

C. Influenza, Hep B, IPV

Question 9

A 10-month-old asymptomatic infant presents with a RUQ mass. Work-up reveals a normocytic anemia, elevated urinary HVA/VMA, and a large heterogeneous mass with scant calcifications on CT. A bone marrow biopsy is performed. Which of the following histologic findings on bone marrow biopsy is most consistent with your suspected diagnosis?

A. Enlarged cells with intranuclear inclusion bodies
B. Hypersegmented neutrophils
C. Sheets of lymphocytes with interspersed macrophages
D. Small round blue cells with dense nuclei forming small rosettes
E. Stacks of RBCs

Answer 9

D. Small round blue cells with dense nuclei forming small rosettes

In addition to neuroblastoma, other tumors associated with small blue cells include Ewing’s sarcoma and medulloblastoma, both of which tumors are seen in children.

Question 10

A 3-year-old boy is brought to the clinic by his parents for follow-up of iron deficiency anemia. His diet consists mostly of sweet, bland, low-texture foods. He drinks 32 ounces of milk daily from a bottle. In addition to prescribing oral iron supplementation, what is the best advice to give the parents concerning this patient’s diet?

Answer 10

D. Gradually introduce new foods and slowly decrease his old favorites

NOT:
A. Bribe the patient to eat healthy food
B. Continue bottle-feeding
C. Encourage eating small amounts of food throughout the day
D. Gradually introduce new foods and slowly decrease his old favorites
E. No change is needed since he is on the optimal diet for his age

Question 11

A 2-year-old girl is brought to the clinic by her mother for a health maintenance visit. While waiting for the pediatrician, her mother reads her a short book. When you enter the room, her mother asks her to take the book and return it to a bookshelf. Developmental history reveals that the patient can scribble with a pencil but cannot write her name. She can kick and throw a ball, but cannot jump in place. Which of the following best describes this child’s development?

Answer 11

C. Age-appropriate development

NOT:
A. Advanced fine motor skills
B. Advanced gross motor skills
C. Age-appropriate development
D. Delayed language
E. Delayed social skills

Question 12

A 4-year-old boy is brought to the clinic by his mother for developmental evaluation. She is concerned that he is delayed when compared to the children of her friends. Although he can throw a ball and copy a circle, he cannot brush his teeth on his own, tie his shoes, or hop on one foot. Which of the following set of developmental milestones most closely matches those expected of a 4 year old child?

A. Hop on 1 foot, copy a cross, brush teeth
B. Mature pencil grasp, print some letters and numbers
C. Skip, draw a person with 6 or more body parts
D. Throw a ball overhand, ride a tricycle, build tower of 6-8 cubes
E. Tie a knot, copy squares

Answer 12

A. Hop on 1 foot, copy a cross, brush teeth

A normally developing 4-year-old should be able to hop on 1 foot, copy a cross, pour/cut/mash their own food, and brush teeth.

Question 13

A 5-year-old girl is brought by her mother to the clinic for a well-child visit. Medical history is significant for three upper-respiratory infections in the past year. She does well in preschool, is toilet trained, and enjoys eating mostly pasta, bread, and milk. Review of systems is otherwise unremarkable. She lives with her mother and father in a home built in 1985. Her height is at the 50%tile, weight is at the 50%tile and BMI is at 60%tile. Vital signs are normal. Physical examination is normal. Lab studies today show a hemoglobin of 10.0 g/dL. Her hemoglobin was in the normal range at her 3-year-old visit. Which of the following is the most likely cause of her anemia?

Answer 13

D. Iron deficiency
Given the patient’s age and preference for pasta and milk, the most likely cause of anemia is iron deficiency. Treatment would include oral iron supplementation and increased dietary iron intake.

NOT:
A. Chronic blood loss
B. Chronic illness
C. Hemoglobinopathy
E. Lead poisoning

Question 14

4-1: An 8-year-old boy is brought to clinic by his parents because they are concerned that he has not been doing his homework. His teacher recently called the parents to say that their son seems distracted in class, constantly interrupts other children when they are speaking, and is very fidgety. When you speak with the boy, he tells you that he did not know about the homework assignments and that he tries hard to pay attention in class. What is the next best step in management?

Answer 14

Find out more about his behavior at home and at school

Contacting the teacher to find out more about the child’s behavior at school and learning more about his behavior at home are the best ways to determine if six of the symptoms are present in two or more settings, which is required to make the diagnosis of ADHD. It also will be important to learn more about other aspects of this child’s life, as there are several factors that can lead to acting out (including learning disability, hearing disability, family stress, and abuse).

NOT:

• Do nothing, as this child’s behavior is normal
• Prescribe a stimulant medication for ADHD
• Recommend group therapy for the child
• Suggest behavior modification for the child and parenting classes

Question 15

An 8-year-old obese male with no past medical history is found to have a mildly elevated blood pressure during a routine health maintenance visit. Review of systems is negative. His physical exam is normal, with the exception of his blood pressure and obesity. What is the most likely diagnosis?

Answer 15

D. Primary hypertension

The most likely diagnosis is primary hypertension because the only physical findings are hypertension and obesity. Given the mild hypertension and the patient’s age, symptoms are unlikely to be present. Other etiologies should be ruled out, but review of systems and physical examination do not currently suggest other etiologies.

NOT:
A. Coarctation of the aorta
B. Hyperthyroidism
C. Pheochromocytoma
E. Renal artery stenosis

Question 16

A 7-year-old male presents to the clinic with complaints of headaches and episodes of feeling sweaty and flushed. He also reports that at times he feels as if his heart is racing. He was full term, had an uncomplicated birth, and has been otherwise healthy until now. On exam, his BP is 120/80 mmHg and is the same in his upper and lower extremities. His weight and height are in the 50th percentile for his age. What is the most likely cause of this patient’s hypertension?

Answer 16

Catecholamine excess

Catecholamine excess (pheochromocytoma or neuroblastoma) should be suspected in a child who is hypertensive and has episodes of sudden sweating, flushing, or feels that his heart is racing. This patient is exhibiting these signs and a urine catecholamine testing would be appropriate in this case.

NOT:
B. Coarctation of the aorta
C. Primary hypertension
D. Renal insufficiency due to renal scarring
E. Renal vascular disease

Question 17

An 8-year-old female presents to your clinic for follow-up after being hospitalized for status asthmaticus. She is completing a five-day course of systemic steroids. Given her history of moderate persistent asthma, her outpatient regimen includes daily use of inhaled fluticasone / salmeterol. She was also diagnosed with ADHD one year ago and was started on Adderall XR daily. Her BMI today is at the 83rd percentile for her age, and her blood pressure is at the 98th percentile for her age. What is the most likely cause of her stage I hypertension?

Answer 17

A. Medications

Both steroids and CNS stimulants can cause an increase in blood pressure, especially when used in combination. Steroids increase blood pressure by mimicking endogenous cortisol and the sympathetic fight or flight response. Stimulants mimic norepinephrine, stimulating alpha and beta adrenergic receptors, causing an overall increase in blood pressure.

NOT:
B. Neurofibromatosis 1
C. Obesity
D. Renal insufficiency
E. The blood pressure cuff is too big

Question 18

4-5: A 7-year-old male frequently gets in trouble at school for being disruptive and talking inappropriately in class. He does not follow directions and does not work well with classmates during group exercises. His mother relates that at home, he is always on the go, sleeping only six to seven hours each night, and he does not follow the rules at home. He often skips his homework and sometimes puts himself in danger, such as by running away unaccompanied. Which of the following is the most likely diagnosis?

Answer 18

B. Attention deficit hyperactivity disorder

ADHD is characterized by the triad of impulsivity, hyperactivity, and inattention- all of which are present in the child described above. Other symptoms include motor impairment and emotional lability. ADHD is typically diagnosed before the age of 7 but persists into adulthood. Intelligence is usually normal, but individuals with ADHD commonly perform more poorly academically than would be expected for their IQ.

NOT:
A. Antisocial personality disorder
C. Bipolar mood disorder
D. Functional neurologic symptom
E. Rett syndrome

Question 19

5-1: A 15-year-old female comes to the clinic with a chief complaint of feeling tired for one month. She has also been complaining of frequent nosebleeds while at school and bruising easily. She had menarche at the age of 9 and her periods have always been heavy and irregular. Her mother and grandmother also have heavy periods and easy bruising. What is the pattern inheritance of the bleeding disorder in this patient?

Answer 19

A. Autosomal dominant (AD) inheritance is the correct choice. In AD disorders males and females are equally affected within each generation. These include conditions such as von Willebrand disease, Marfan syndrome, neurofibromatosis, and Huntington’s disease.

NOT:
B. Autosomal recessive
C. Mitochondrial
D. X-linked recessive

Question 20

5-2: A 14-year-old girl presents to your office wondering why she has not had her period yet. Her mother states that she and the patient’s grandmother reached menarche at 13 years of age. The patient is concerned she is behind her friends in terms of development. She is doing well in school and has not had developmental problems in the past. On physical examination, her breasts are elevated without a secondary mound, and curly, coarse pubic hair is present on the labia majora in a triangular shape but does not reach the mons pubis. What Tanner stage would you assign this girl?

Answer 20

Tanner Stage III

The patient in the vignette is at Tanner Stage III of development. Her breast buds are elevated but do not have the secondary mound characteristic of Tanner Stage IV. Her pubic hair distribution extends more laterally than Stage II but is not adult-like in hair quality and does not extend onto the mons pubis.

Question 21

A 16-year-old female presents to clinic complaining of worsening fatigue. Family history is significant for hypothyroidism and heavy periods in the grandmother. Her exam reveals mild tachycardia and oozing around a recent piercing, but is otherwise normal. Labs reveal Hgb 8.5 g/dL, MCV 58, PT 12.5, PTT 44, and low von Willebrand factor activity. Which of the following is the most appropriate treatment for her underlying disorder?

Answer 21

C. Desmopressin

C. Von Willebrand disease is the underlying cause of this patient’s anemia, as indicated by the low von Willebrand factor activity. This is the most common hereditary bleeding disorder, occurring in roughly 1% of the population. Intranasal or intravenous desmopressin is appropriate treatment for most bleeding problems. Desmopressin works by causing release of von Willebrand factor from vessel endothelial cells.

NOT:
A. Blood transfusion and iron supplementation
B. Cryoprecipitate
C. Desmopressin
D. Factor VIII concentrate
E. Vitamin K

Question 22

A 10-year-old female comes to the clinic for a well child exam. Her mom asks about puberty and wants to know in what order she should expect to see normal pubertal changes in her daughter. Which of the following sequences is correct?

A. breast bud -> pubic hair -> growth spurt -> menarche
B. breast bud -> pubic hair -> menarche -> growth spurt
C. pubic hair -> breast bud -> growth spurt -> menarche
D. pubic hair -> breast bud -> menarche -> growth spurt
E. pubic hair -> menarche -> breast bud -> growth spurt

Answer 22

A. breast bud -> pubic hair -> growth spurt -> menarche

This choice is correct because breast buds are the first sign (10-11 years), followed by pubic hair (10-11 years), then a growth spurt (12 years), and then menarche (defined as the first occurrence of menstruation, 12-13 years). Most girls reach adult height by approximately 15 years.

Question 23

5-5: A 16-year-old male comes to clinic because of concerns about being one of the smallest students in his class. He says that people think that he looks like he should be in middle school rather than high school. His mother says that until about four years ago, she did not notice much difference between the patient and his friends. However, in the past two years, he has become the shortest person in his class. When assessing for pubertal development, what is the first physical exam finding expected in this patient if puberty has started?

Answer 23

E. The first sign of puberty in a boy is testicular enlargement. The onset of puberty is quite variable, but usually occurs between 10 and 15 years for boys. It is rare for boys not to have begun puberty by the age of 16. To assess whether or not a male has entered puberty, one must know the order of the appearance of secondary sexual characteristics.

Testicular enlargement –> pubic hair appearance –> growth of penis and scrotum –> first ejaculations –> growth spurt

Question 24

6-1: John is a 17-year-old presenting today for a pre-participation physical exam. During the interview, he reports a low-grade fever, malaise, and headache for one week. In the past few days, his fever has gotten worse and he complains of a sore throat. He denies cough or chest pain. On physical examination, he is found to have a temperature of 101.3° F, and cervical lymphadenopathy and oropharyngeal erythema with exudate are noted. His participation would be most likely affected by which of the following tests?

Answer 24

C. EBV serologies
C. This choice is correct because the patient’s symptoms are suggestive of infectious mononucleosis. These include complaints of low-grade fever and malaise and findings of cervical lymphadenopathy and pharyngeal exudate. If testing is positive, the patient should be restricted from strenuous activity or contact sports during his illness due to the risk of splenic rupture.

NOT: 
A. Chest x-ray
B. CT head/neck
D. Throat culture
E. No further workup

Question 25

6-2: A 17-year-old girl presents for a sports pre-participation physical. She reports that she occasionally gets short of breath and feels light-headed with exercise, and sometimes she experiences chest pain as well. She lost consciousness once last season during a playoff basketball game, but attributed it to feeling sick at the time. Her grandfather died suddenly at age 35 of unknown etiology. Which of the following is the most likely diagnosis?

Answer 25

D. Prolonged QT syndrome
D. Prolonged QT syndrome can cause syncopal episodes in late childhood or adolescence. QT intervals are elongated on ECG and lead to arrhythmias, like ventricular fibrillation. This condition is often associated with other abnormalities, including severe congenital sensorineural deafness.

NOT: 
A. Hypoglycemia
B. Congenital heart block
C. Postural hypotension
E. Ventricular septal defect

Note on CHB: Congenital heart block can be caused by congenital heart defects and autoimmune disease in pregnant women (lupus). This condition is a rare cause of arrhythmia and is often diagnosed early in life. Many patients require a pacemaker.