59 ABP Pediatrics Board Exam Practice Questions

Transient tachypnea of the newborn is correct. Radiographic evidence revealing prominent perihilar streaking, correlating with the engorgement of the lymphatic system with retained lung fluid and fluid in the fissures, is diagnostic of transient tachypnea of the newborn. Partial carbon dioxide tensions are usually normal. This condition presents as respiratory distress in full-term or near-term infants with a maternal history of caesarean delivery; it resolves over a 24 to 72-hour period.

Congenital diaphragmatic hernia (CDH) is incorrect. CDH presents with variable respiratory distress and cyanosis, feeding intolerance, and tachycardia, with a scaphoid abdomen on physical examination if significant visceral herniation is present. In the antenatal period, it can manifest as polyhydramnios, an absent intra-abdominal gastric air bubble, mediastinal shift, and hydrops fetalis.

Pulmonary sequestration describes a cystic or solid mass with abnormal vascular supply (systemic rather than pulmonary) and nonfunctioning primitive tissue sequestered from the tracheobronchial tree, either within the visceral pleura of normal lung tissue or within its own pleural sac.

Congenital cystic adenomatoid malformation (CCAM) is incorrect. CCAM involves adenomatous overgrowth of the terminal bronchioles due to deranged embryogenesis. Signs and symptoms include pulmonary hypoplasia, recurrent pulmonary infections, dyspnea and chest pain, hemoptysis, cough, fever, and failure to thrive.

Neurogenic tumors are the most commonly occurring tumors or cysts in children and infants and should be apparent during CT scan of the chest and mediastinum or posteroanterior and lateral radiograph of the chest as a mediastinal mass.

8 to 12 weeks after broken contact is correct because this is the ideal time to perform TST or IGRA testing in a child on window prophylaxis for tuberculosis. A broken contact is defined as the point at which the child was removed from direct contact to a possible contagious source of tuberculosis; it is necessary to immediately break contact and initiate window prophylaxis in a child younger than 5 years as soon as the source is suspected of active tuberculosis. If at 8 to 12 weeks after broken contact, the TST is positive, then isoniazid therapy is extended. If it is negative, the therapy can be stopped.

4 to 8 weeks after exposure is incorrect because the guidelines do not recommend re-testing based on the time of exposure. The accuracy of the time period of exposure is indeterminate; therefore, clinicians usually start window prophylaxis in a child immediately after exposure, strictly recommend breaking contact with the contagious source and re-test 2 to 3 months after broken contact to increase the sensitivity of a true positive result.

8 to 12 weeks after exposure is incorrect because exposure time does not determine when to re-test after initiating window prophylaxis. The delayed hypersensitivity in response to mycobacterium develops 6 to 8 weeks after exposure, and for the accuracy of results, the re-test is performed 8 to 12 weeks after breaking contact.

4 to 8 weeks after broken contact is incorrect because it is too early to perform TST during the first month of window prophylaxis. A false negative is likely as the time period required for developing an innate immune response to mycobacterium is 4 to 8 weeks after exposure, and the results might be inconsistent in that time frame.

No need to repeat IGRA and TST testing is incorrect because repeat testing will determine whether the isoniazid therapy in window prophylaxis needs to be extended to offer treatment or stopped to prevent unnecessary side effects in TST-negative cases. The experts recommend performing TST and IGRA again, after an initial negative, 8 to 12 weeks after broken contact to increase the sensitivity and yield of the test.

Discuss the diagnosis with the patient respecting her wishes to either include her parents in the discussion or not is correct. This patient has a chronic illness; however, she has not been given the information she is seeking. Although children have limited autonomy regarding medical decisions that need to be taken, they have a right to information, especially in chronic illnesses where the child is expected to undergo a series of medical procedures and regular follow-up visits. The child’s trust in the physician and the physician’s honesty and respect towards the patient are vital for a healthy patient-physician relationship.

Respect the wishes of the parents and do not inform the patient of the actual diagnosis is incorrect. Although children have limited autonomy regarding medical decisions that need to be taken, they have a right to information, especially in chronic illnesses where the child is expected to undergo a series of medical procedures and regular follow-up visits.

Schedule an appointment with the patient to discuss the diagnosis without informing the parents is incorrect. Although the patient has the right to information and how she wants that information disclosed, the physician should make an effort to convince her to involve her parents and keep them informed to avoid communication gaps between the physician, the patient, and the patient’s parents.

Convince the parents to discuss the diagnosis in detail with the patient in a home setting is incorrect. The patient has requested information about her diagnosis from the physician, and the physician must relay that information responsibly.

Tell the patient that she has anemia that will improve with regular blood transfusions is incorrect. Her parents have told this patient that she has anemia, and she is requesting more information regarding her diagnosis. The physician must be compassionate but honest with her as children with chronic illnesses are expected to undergo a series of medical procedures and are entitled to information regarding their diagnosis.

Wilcoxon signed-rank test is the correct option. It compares 2 related samples, matched samples, or repeated measurements on a single sample to assess whether their population mean ranks differ. The above-mentioned study uses data that is paired, non-parametric, and from the same population. As this test compares 2 sets of observations on a single sample, it is the most likely choice.

Mann Whitney-U test is an incorrect option because it compares whether there is a difference in the dependent variable for 2 independent groups. This test ranks all the dependent values and then utilizes the sum of the ranks of each group in the analysis of the statistics.

Cohen’s kappa statistic is incorrect because it tests inter-rater reliability for qualitative items using Cohen’s kappa coefficient.

Chi-square test is incorrect because it can be performed when the test statistic is chi-squared distributed under the null hypothesis. It is used to compare the observed values in data to the expected values that the researcher would see if the null hypothesis is true.

Analysis of variance (ANOVA) is an incorrect option. It determines if 3 or more groups are statistically different from each other. ANOVA uses the F-test for statistical analysis. This allows for the comparison of multiple means simultaneously because the error is calculated for the whole set of comparisons rather than for each 2-way comparison.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is correct. It is associated with dyspnea, tachycardia, diaphoresis, and vomiting while feeding, secondary to anginal pain resulting from a coronary artery steal phenomenon to the anomalous origin of the left coronary artery. The classic ECG findings include deep Q waves, peaked T waves, and/or ST segment changes consistent with ischemia, injury, or infarction. Diagnosis should be confirmed via echocardiogram and/or cardiac catheterization with angiography.

Kawasaki disease can affect the pericardium, endocardium, myocardium, valvular, and conductive tissues of the heart, via extensive immune activation.

Coronary artery ostial stenosis or coronary artery kinking follows arterial switch repair of dextro-transposition of the great arteries in the neonate.

Tetralogy of Fallot encompasses positional defects such as ventricular septal defect (VSD), anterior shift of the aorta over the VSD (overriding aorta), obstruction of the right ventricular outflow tract, and right ventricular hypertrophy.

Pulmonary atresia with an intact ventricular septum involves heterogeneous right ventricular development, an imperforate pulmonary valve, with extensive ventriculocoronary connections.

Computerized provider order entry is the correct answer because paper-based systems, in which medication orders are handwritten, are prone to transcribing errors, which can result in the recipient of the order not being able to properly read and interpret the prescription. To prevent transcribing errors, most hospitals have adopted computerized provider order entry (CPOE) systems, which also have the advantage of helping to prevent ordering errors because they are usually combined with a clinical decision support system.

Medication reconciliation is incorrect because it is a measure used to prevent ordering errors during a transition of care and helps to avoid inconsistencies between the patient’s previous medication and the new medication prescribed. In this case, the medication error occurred at the transcribing stage and led to the misinterpretation of a trailing zero, which would not have been prevented by medication reconciliation.

Tall man lettering is incorrect because it is a strategy used to prevent dispensing errors related to medications that look or sound alike (buPROPion vs. busPIRone). In this case, the medication error occurred at the transcribing stage and led to the misinterpretation of a trailing zero, which would not have been prevented by tall man lettering.

Multicompartment medication device is incorrect because it is recommended for patients in ambulatory settings to take multiple medications to help prevent administration errors and improve medication safety and adherence. In this case, the medication error occurred at the transcribing stage and led to the misinterpretation of a trailing zero, which would not have been prevented by a multicompartment medication device.

Clinical decision support system (CDSS) is incorrect because it generates computerized recommendations or alerts based on patient information to prevent ordering errors. Examples of ordering errors include prescribing a drug that is contraindicated (due to drug interactions or drug allergies) and a failure to prescribe the correct dose based on weight, renal dysfunction, or hepatic dysfunction. In this case, the medication error occurred as a result of a trailing zero during transcribing, which would not have been prevented by a CDSS.

Cataracts is the correct answer because hearing loss, a likely cardiac defect, and small for gestational age in the setting of a maternal first-trimester illness has likely caused congenital rubella syndrome (CRS). CRS typically presents with a triad of leukocoria (white pupillary reflex) from cataracts, cardiac defect (machine-like systolic murmur of patent ductus arteriosus), and sensorineural hearing loss.

Chorioretinitis is incorrect because it is one of the symptoms of congenital toxoplasmosis, which classically presents with a triad of diffuse intracranial calcifications, chorioretinitis, and hydrocephalus. Congenital cytomegalovirus infection can also lead to periventricular and calcifications chorioretinitis. However, cardiac defects or cataracts are not seen.

Limb hypoplasia is incorrect because it occurs in congenital varicella syndrome, characterized by cataracts, distinctive skin lesions (scarring), and limb hypoplasia. Varicella is not associated with cardiac defects.

Rhinitis is incorrect because it is one of the presenting symptoms in congenital syphilis, which can cause maculopapular rash, hepatomegaly, osteoarticular destruction, and a nasal discharge (“snuffles”). Heart defects are not seen, and sensorineural hearing loss is a late sequela of untreated congenital syphilis.

Vesicular rash is incorrect because it typically occurs due to the herpes simplex virus, which is acquired perinatally during delivery. Transplacental (congenital) herpes infection is rare and presents with brain destruction, seizures, and vesicular skin lesions; hearing loss and heart defects are not seen.

Costochondritis is the correct answer. This patient’s history and examination are most consistent with costochondritis, a benign condition involving inflammation of the costal cartilage that is a very common cause of chest pain in children. Reproducible chest tenderness is the classic finding.

Tietze syndrome is incorrect. It is similar to costochondritis but is differentiated by visible swelling of the costal cartilages. No additional investigations or referral to a pediatric cardiologist are required. NSAID therapy and warm compresses as needed and reassurance about the benign nature of the condition are sufficient.

Exertional chest pain is incorrect. It requires referral to a pediatric cardiologist immediately for additional investigations with 12-lead ECG. Exertion can entail pressure on the aorta and pulmonary artery to squeeze the left main coronary artery or left anterior descending artery, leading to myocardial ischemia and, occasionally, sudden death in adolescents.

Left ventricular hypertrophy is incorrect. It relates to the thickening of the myocardium (muscle) of the left ventricle of the heart, which can be identified with echocardiography and ECG.

Aortic stenosis is incorrect. It involves an abnormal narrowing of the aortic valve.

Tonsillectomy is correct. Tonsillectomy may decrease the overall duration of inpatient stay and carries no increased morbidity when performed after an interval of several weeks. It is referred to as abscess tonsillectomy, quinsy tonsillectomy, cold tonsillectomy, and tonsillectomy à chaud when carried out for the acute stages of a peritonsillar abscess.

Incision and drainage are contraindicated in the event of uncertain diagnosis, uncooperative child, very low age, or risk of airway management issues.

Needle aspiration is similarly contraindicated and is associated with the risk of respiratory distress, aspiration, and hemorrhage. Uncomplicated peritonsillar abscess may be treated medically with analgesia, and antibiotics.

Vitamin A is correct because it is recommended for children with measles to reduce the severity and complications associated with the disease. Given the symptoms of high-grade fever, maculopapular rash, and palpable lymph nodes, the clinical picture is suggestive of measles. This makes Vitamin A the appropriate intervention to mitigate the risk of complications associated with measles. Vitamin A supplementation is particularly important in children who might be at risk of Vitamin A deficiency, contributing significantly to reducing morbidity and mortality from measles.

Vitamin C is incorrect because there is no specific indication for Vitamin C supplementation in the management of measles or similar viral exanthems based on the symptoms described. While Vitamin C plays a role in immune function, it does not specifically address the complications or the severity of measles.

Vitamin D is incorrect because it has no direct role in the acute management of measles or conditions with similar presentations. Although Vitamin D is crucial for bone health and immune function, it does not specifically reduce the morbidity or mortality associated with measles.

Vitamin E is incorrect because it primarily functions as an antioxidant and does not have a specific indication for treating measles or its complications. There is no evidence to suggest that Vitamin E supplementation would benefit a patient with the described presentation.

Vitamin K is incorrect because it is essential for blood clotting but does not have a role in the treatment of measles or in managing the symptoms presented by the patient. There is no indication for Vitamin K supplementation in the context of measles or the clinical scenario described.

Enjoying mirror is correct as the child is 8 months old now. A child starts enjoying mirrors at the age of 7 months. This action forms a component of the social behavior that the child develops at 7 months of age. Other components include preferring mother, babbling, and responding to changes in the emotional content of social contact.

Assisted pincer movement is incorrect as that is not developed by 8 months of age. This movement is a milestone related to adaptive behavior achieved on average by 10 months of age. At the age of 8 months, the child reaches out and grasps large objects, transfers objects from hand to hand, grasps using radial palm, and uses a raking motion.

Responds to sound of name is incorrect as this is an advanced milestone that the child would not have achieved. The child would achieve this at 10 months of age. Other milestones related to social behavior at 10 months of age include playing peek-a-boo or pat-a-cake and waving bye-bye.

Uncovers hidden toys is incorrect as this is a milestone that the child is not expected to achieve by this age. This is an adaptive behavior that the child is expected to achieve at 10 months of age. Other adaptive behaviors at 10 months include grasping objects with thumb and forefinger, poking at things with forefinger, picking up pellets with assisted pincer movement, attempting to retrieve dropped objects, and releasing objects grasped by other people.

Walking while holding furniture is incorrect as it is too early for this child to be doing this. This action develops at 10 months of age in addition to pulling to a standing position.

Building a tower of 2 cubes is correct because this is the most age-appropriate milestone for this baby’s age. Milestones have a wide range of development, and some children may achieve them before the expected age. However, the absence of such motor functions at appropriate ages should encourage increased vigilance.

Can push and pull large wheeled toy is incorrect because the average age for achieving this gross motor milestone is 18 months.

Walks upstairs with handheld is incorrect because children at this age are often only starting to walk without support and would not be expected to be able to navigate stairs even with support.

Building a tower of 3 cubes is incorrect because children usually achieve this gross motor milestone at 18 months of age.

Holding a pencil with a tripod grasp is incorrect because infants at 12 months should have mastered the pincer grasp but not be expected to be able to hold a pencil. Tripod grasp usually develops by 18 months of age.

Female’s athlete triad is correct as the patient’s history is consistent with its symptoms which are characterized by amenorrhea, low bone density and decrease calorie intake. It is more common in athletic women who indulge in strenuous activities or sports. Such patients develop functional hypothalamic amenorrhea leading to hypoestrogenemic state. Low levels of estrogen in the blood lead to secondary amenorrhea due to the absence of endometrial lining. Such patients also experience decreased bone mineral density and infertility.

Polycystic ovarian syndrome is characterized by weight gain, oligomenorrhea, and hirsutism, so this option is incorrect.

Anorexia nervosa is an eating disorder due to excessive fear of gaining weight and distorted body image. It is characterized by BMI < 18.5 kg/m2, amenorrhea, anemia, lanugo, electrolyte disturbances, and stress fractures. This patient’s BMI is 22kg/m2, so this option is incorrect.

Prolactinoma manifest as headache, visual changes, amenorrhea and galactorrhea with elevated prolactin levels. This patient’s prolactin levels are within the normal range, so this option is incorrect.

Ectopic pregnancy presents with a triad of lower abdominal pain, amenorrhea, and vaginal spotting with positive B-hCG. Ultrasound findings show gestational sac at the ectopic site. This patient’s negative B-HCG is inconsistent with ectopic pregnancy.

8 cm/year is correct. After the first year of life, the average annual growth velocity for children slows down. Preschool-aged children, between 2 and 5 years old, typically grow about 5 to 7 cm per year. An average growth of 8 cm/year is a reasonable estimate for this age group, reflecting a normal growth pattern.

6 cm/year is incorrect because it is slightly below the expected range of growth velocity for a preschool-aged child. While it might be within the broader spectrum of normal growth, it is not the optimal average figure representing normal growth velocity in this age group.

10 cm/year is incorrect because it exceeds the typical growth velocity for a child in the preschool years. This rate of growth is more characteristic of the rapid growth observed during the first year of life.

5 cm/year is incorrect as it represents the lower limit of the expected growth velocity range for preschool-aged children. It is less than the average growth velocity for this age, thereby not representing the most accurate estimate for normal growth velocity.

25 cm/year is incorrect because it far exceeds the normal growth velocity for children in this age group. This rate of growth is significantly higher than what is expected and is not typical for preschool-aged children, indicating a misunderstanding of normal growth patterns.

Upper endoscopy and colonoscopy is correct. The patient in this clinical vignette has inflammatory bowel disease (IBD), more likely Crohn’s disease than ulcerative colitis. The most appropriate next step in management would be to perform an upper endoscopy and colonoscopy to establish a diagnosis of IBD. IBD can explain the poor linear growth and ankle pain.

Hand X-ray to assess bone age is incorrect as inflammatory bowel disease (IBD) can explain the poor linear growth and ankle pain.

Ankle X-ray is incorrect as inflammatory bowel disease (IBD) can explain the poor linear growth and ankle pain.

Serologic assays to screen for inflammatory bowel disease (IBD) is incorrect as they may have false negative rates of at least 30% in children with Crohn’s disease and have false positives for IBD as well.

Small bowel follow-through (SBFT) primarily evaluates the mucosal disease in the small intestine. The SBFT has become a less favorable imaging modality over time. More recently, magnetic resonance imaging (MRI) and computed tomography (CT) enterography have become the preferred imaging modalities because they can provide information about small and large bowel and extramural disease. In addition, MRI does not expose children to ionizing radiation, whereas CT and SBFT do; this is important due to potential radiation-related malignancies later in life.

Diphtheria-tetanus-pertussis vaccine is the correct answer because low-grade fever, cough, and coryza, followed by the onset of multiple coughs during expiration and then inspiratory stridor (or the “whoop”), are characteristic of the catarrhal and paroxysmal stages of pertussis. The diphtheria-tetanus-pertussis vaccine is 95% effective against severe illness and is usually first given when a child is 2 months old. Because immunity wanes, a booster every 10 years is recommended.

H.influenza type b vaccine is incorrect because H.influenza in younger children usually presents with mild upper respiratory tract symptoms: bronchitis, bronchiolitis, or pneumonia. They can also present with laryngotracheitis, myositis, and parotitis. This patient’s signs and symptoms are more consistent with pertussis.

Hepatitis B vaccine is incorrect because acute hepatitis causes jaundice and scleral icterus. The liver may also be enlarged and tender. Hepatitis can also lead to metabolic abnormalities and portal hypertension, chronic hepatitis, causing cirrhosis and an increased risk of hepatocellular carcinoma.

Inactivated poliomyelitis vaccine is incorrect because polio causes an asymptomatic infection in 95% of patients. The other 5% may have fever, malaise, pharyngitis, nausea, and vomiting. Others may present with weakness of affected muscles, including skeletal or cranial muscles, leading to an asymmetric flaccid paralysis. It can also cause aseptic meningitis. Polio has been eradicated in the United States, the Western Hemisphere, Europe, and the Western Pacific region, including China, but is still seen in developing countries.

Measles-mumps-rubella vaccine is incorrect because measles causes a 3-day prodrome of cough that is not stridor, coryza, and conjunctivitis. Patients also may develop Koplik spots (small red spots with grey centers on the buccal mucosa), which last for 12-24 hours, and a maculopapular rash. Mumps can lead to painful swelling of the parotid glands and swelling of other salivary glands. They are not associated with cough or fever. Rubella causes a non-coalescent, erythematous, maculopapular rash lasting 3 days and is accompanied by posterior cervical, posterior occipital, and posterior auricular lymphadenopathy. Additionally, vaccination against measles doesn’t occur until a child is 12 months old.

Alkaline phosphatase is correct. Alkaline phosphatase (AP) and phosphorous are the 2 most commonly used biomarkers to assess bone mineralization in infants. The values of AP and phosphorous levels have been well correlated to dual-energy x-ray absorptiometry (DEXA) scans. In infants with MBD, changed AP is increased severalfold. Former premature infants are at very high risk for MBD due to a common history of placental insufficiency, RDS necessitating steroid and/or diuretic use, NEC, and prolonged NPO status.

Phosphorus is incorrect since phosphorous is usually low. A combination of phosphorus less than 4.6 mg/dL and alkaline phosphatase (AP) greater than 900 IU/L yielded a sensitivity of 100% and a specificity of 70% for low bone mineral density.

Calcium is incorrect. Serum calcium concentration is usually normal in infants with MBD due to the rapid secretion of parathyroid hormone.

Magnesium is incorrect since it is not used as a biomarker in the workup of MBD.

Osteocalcin is incorrect as osteocalcin has been used mainly in research purposes.

Oral polio vaccine (OPV) is the correct option because oral polio vaccine is a live attenuated vaccine and is contraindicated in patients with any condition leading to a compromised immune system, including DiGeorge syndrome. Patients with this syndrome have characteristic facial features of small mandible, low-set ears, and antimongoloid slant to the eyes. They are also prone to developing hypocalcemia leading to seizures early in the neonatal period.

Diphtheria- tetanus- acellular pertussis is incorrect because Diphtheria- tetanus- acellular pertussis is not a live vaccination. It consists of diphtheria and tetanus toxoids and acellular pertussis components that can be safely administered to immune-compromised patients

Hepatitis B vaccine is incorrect because Hepatitis B is a sub-unit vaccine and can be safely administered to immunocompromised patients.

Hemophilus influenza (type B) vaccine is incorrect because Hemophilus influenza (B) vaccine is not a live vaccination and can be safely administered to immunocompromised individuals.

Influenza vaccine is incorrect because Influenza vaccine is available in an inactivated influenza vaccine form (IIV) that can be safely administered to immunocompromised individuals.

Nonsteroidal anti-inflammatory drugs (NSAIDs), including naproxen, ibuprofen, and celecoxib, are the first line of medications used to treat primary dysmenorrhea.

Oral combined contraceptives (OCPs) are the second line of therapy for those who fail to respond to NSAIDs. OCPs are not used as the first line of treatment, so this option is incorrect.

Dilatation and curettage are not done for primary dysmenorrhea, so this option is incorrect.

Leuprolide is a gonadotropin-releasing hormone (GnRH) agonist used in the treatment of endometriosis, so this option is incorrect.

Gentamicin + clindamycin are used to treat pelvic inflammatory disease, so this option is incorrect as well.

Premenstrual dysphoric disorder is correct as this patient’s history is consistent with the symptoms which are characterized by anhedonia, mood swings, irritability, loss of concentration, sleep disturbances and changes in appetite. The symptoms start a week before the onset of menses and improve spontaneously within a few days after the beginning of menses. Selective serotonin reuptake inhibitors are used as the first line of medication in the treatment of premenstrual dysphoric disorder.

Major depressive disorder is incorrect. DSM V criteria of major depressive disorder include having 5 of the 9 symptoms (sleep disturbances, loss of interest, guilt, fatigue, loss of concentration, appetite loss, decreased psychomotor activity and suicidal ideations) for at least 2 weeks, so this option is incorrect.

Bipolar disorder is characterized by alternating periods of depression and mania, so this option is incorrect.

Generalized anxiety disorder is characterized by irritability, restlessness, palpitations, and feelings of fear lasting for more than 6 months which is inconsistent with this patient’s history.

Persistent depressive disorder is diagnosed if the symptoms of depression are chronic and last for at least 2 years, so this option is incorrect as well.

Involve a multi-disciplinary team is the correct option because the child belongs to a family struggling financially, which is affecting the child’s health. The physician is in a good position to determine the impact of the socio-economic situation on the family and thus involve social services and other agencies to mobilize resources to improve their health status. The American Academy of Pediatrics recommends pediatricians and family physicians to routinely screen for social issues so that early interventions can be made in the children’s best interest.

Alleviate poverty is incorrect because it is not the physician’s responsibility to improve the socio-economic status of society.

Arrange a job for the father is incorrect because it does not come in the jurisdiction of a physician.

Offer financial support is incorrect because it is not the physician’s responsibility to solve the family’s financial matters on his own. The correct approach involves a multi-disciplinary team including social welfare, which will look after the greater interests of the family.

Treat diarrhea and discharge is incorrect because it is the physician’s responsibility to explore the social aspects influencing the child’s chronic diarrhea.

Post-traumatic stress disorder (PTSD) is the correct option because this patient has witnessed and survived child neglect which places her at an increase risk for developing PTSD. The symptoms of PTSD in this age group include nightmares and recalling the traumatic experience. Children who have experienced prolonged exposure to traumatic experiences and subsequently go through separation from their home and family are particularly vulnerable. It is important for foster parents to identify the symptoms and seek help promptly because PTSD can make adjustment into foster care difficult.

Obsessive compulsive disorder (OCD) is incorrect because there are no symptoms of repetitive compulsive behavior in this patient.

Specific phobia is incorrect because it is characterized by an irrational excessive fear of a particular situation that is not present in this patient.

Schizophrenia is incorrect because it is characterized by hallucinations, social withdrawal, negative thoughts, and delusions. The absence of these symptoms excludes the diagnosis of schizophrenia

Bipolar disorder is incorrect because this child does not have any history of episodes of mania and depression.

Initiation of prostaglandin E2 is correct. This infant is demonstrating signs of acute circulatory shock without respiratory distress. His clinical picture is suggestive of a left heart obstructive lesion, such as subaortic obstruction secondary to hypertrophic cardiomyopathy and septal hypertrophy, critical aortic stenosis, coarctation of the aorta, interrupted aortic arch, or hypoplastic left heart syndrome. The onset of this clinical presentation at this age is consistent with the closure of the patent ductus arteriosus (PDA).

Initiation of intravenous antibiotics is incorrect. Other causes of cardiogenic shock, such as sepsis, should also be considered, but not to be the only diagnosis.

Iv fluid bolus is incorrect. An IV fluid bolus may temporarily improve the patient’s clinical status due to increased intravascular volume that forces the small closing patent ductus arteriosus (PDA) to temporarily open. Prostaglandin should be started upon suspicion of a left-sided cardiac obstructive lesion to maintain patency of the ductus arteriosus and improve systemic perfusion.

Transthoracic echocardiography is incorrect. Echocardiography is an essential tool in confirming the diagnosis, but initiation of prostaglandin (PGE) 2 should not be delayed until the transthoracic echocardiogram (TTE) is obtained.

Emergency surgery is incorrect. Given the early onset of symptoms in the neonatal period, surgery with resection of the coarctation segment and end-to-end anastomosis of the aortic segments is the best intervention once the child is stabilized from a clinical point of view.

Anticipation describes the phenomenon in which successive generations display accelerated, earlier-onset, and more severe disease manifestations. Diseases typified by anticipation are often first recognized in the most severely affected offspring, with subsequent diagnoses of milder disease forms in parents or grandparents. A molecular correlate of anticipation is seen in diseases caused by trinucleotide repeat expansions, such as Huntington’s disease, where the number of coronary artery angiography (CAG) repeats can increase with each generation, resulting in longer repeats that confer a more severe phenotype. Another example is in the short telomere syndromes, in which telomeres can become shorter with successive generations, leading to a younger age of presentation in children compared with parents (i.e. Dyskeratosis congenyta).

Mosaicism is incorrect. Mosaicism has two or more populations of genetically distinct cells in an individual who arose from a single fertilized egg. An example of mosaicism is Klinefelter syndrome.

Law of segregation is incorrect because the law of segregation states that paired parental copies of genes are separated from each other during gamete formation, with each parental copy (i.e., allele) segregating into separate gametes.

Genetic heterogeneity is incorrect. Genetic heterogeneity describes mutations in more than one gene that can cause the same disease, where penetrance and expressivity typically vary (i.e., Charcot-Marie-Tooth disease).

Incomplete penetrance is incorrect as it describes the expression of a disease, such as retinoblastoma, that is not observed among all individuals who carry the disease-associated genotype.

Increased sodium reabsorption is correct because extensive studies into the pathogenesis of cystic fibrosis have shown that mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene leads to impaired chloride secretion and increased sodium reabsorption across the respiratory epithelium. This results in dehydration of the airway surface liquid (ASL) as high sodium reabsorption, and poor chloride efflux predispose to chronic airway inflammation and recurrent infections when added with other factors.

Increased chloride secretion is incorrect because the cystic fibrosis (CF) epithelial cells cannot secrete chloride in cystic fibrosis, and there is a net decrease in chloride secretion. The cystic fibrosis transmembrane conductance regulator (CFTR) is the chief conductor of anions like chloride across respiratory epithelium to maintain mucociliary transport, and it is mutated in cystic fibrosis.

Increased bicarbonate secretion is incorrect because cystic fibrosis transmembrane conductance regulator (CFTR) mutation causes decreased secretion of anions like chloride and bicarbonate across the epithelial cells. There is increased mucus viscoelasticity from poor bicarbonate secretion and acidic pH. These factors add up to impaired mucociliary transport and compromised innate immune clearance of the bacteria.

Increased thiocyanate secretion is incorrect because there is poor thiocyanate secretion across the epithelium in cystic fibrosis. Loss of thiocyanate impairs the bacterial killing mediated by lactoperoxidase and predisposition to invasive, recurrent pneumonia.

Increased glutathione secretion is incorrect because glutathione is protective against antioxidant stress, and gross reduction is seen in cystic fibrosis (CF). Loss of glutathione secretion is an important pathology in CF that weaves the vicious cycle of chronic inflammation, increased mucus production, and enhanced mucus viscoelasticity.

Varicella (chickenpox) is the correct answer because this patient’s constitutional symptoms (fever, headache, and myalgia) along with a highly pruritic, generalized rash with multiple lesions in different stages (macules, papules, vesicles, and crusted lesions) are characteristic of chickenpox.

Eczema herpeticum is incorrect because it is a cutaneous manifestation of herpes infection (usually HSV-1 or HSV-2). This condition is associated with pre-existing skin conditions, most often atopic dermatitis.

Erythema infectiosum is incorrect because it is usually only mildly pruritic and initially pronounced on the cheeks (i.e., slapped cheek rash) before spreading to the extremities and trunk where it adopts a lace-like and reticular appearance. This patient’s pruritic rash with fluid-filled vesicles and crusted lesions is inconsistent with erythema infectiosum.

Rubella is incorrect because the rash seen in rubella is usually only mildly pruritic, maculopapular, and not associated with any vesicles or crusted lesions. Also, affected children commonly have concomitant postauricular and/or suboccipital lymphadenopathy.

Scarlet fever is incorrect because the rash seen in scarlet fever is typically fine, erythematous, and maculopapular (often described as sandpaper-like), unlike in this patient who presents with macules, papules, vesicles, and crusted lesions. Also, apart from fever, he does not have any symptoms that are characteristically seen in scarlet fever (e.g., throat pain, and cervical lymphadenopathy).

Throat culture is correct as this is a case of group A streptococcal (Streptococcus pyogenes) pharyngitis. This condition occurs due to colonization of the pharynx, after an incubation period of 2 to 5 days, rapid onset of significant sore throat and high-grade fever occurs. On examination, pharynx is red, and tonsils are enlarged with white, gray, or yellow exudate, which is sometimes blood-tinged along with white coating on the tongue. It may be associated with petechiae or doughnut lesions on the soft palate and posterior pharynx with a red and swollen uvula. For diagnosis, throat culture grown on a blood agar is gold standard.

Rapid antigen detection test (RADT) is incorrect, as even though it is another test for the diagnosis of this condition, it is not the gold standard.

Monospot test is incorrect; it is the test to diagnose infectious mononucleosis when the suspicion is high or atypical lymphocytes are isolated in the complete blood count.

Polymerase chain reaction (PCR) is incorrect as it is done for viral infections as viral culture is often unavailable and is too expensive or slow to be clinically useful; thus, viral PCR is performed for detection.

Blood culture is incorrect as it may detect organisms in the blood, in case of bacteremia; however, its presence in the throat and whether it is the causative organism for pharyngitis will still not be established.

Referral to occupational therapy is correct. Toilet training older than 33 months of age is considered a delay in adaptive functioning or self-help and can be attributed to delays in one or more developmental streams. Referral to occupational therapy is indicated because fine motor skills need to be developed enough to enable a child to use the toilet without excessive frustration or guilt, and a 36-month-old should be able to take clothes off and pull up his or her own pants and use scissors to cut side-to-side, which the child, in this case, cannot do.

Referral to speech and language therapy is incorrect. It is not indicated because this child’s expressive and receptive speech and language milestones are within normal limits.

Referral to child neurology is incorrect. It is not indicated because the child does not have global developmental delays or focal neurological findings.

Referral to developmental-behavioral pediatrics is incorrect. It is not indicated because the child’s social-emotional and behavioral development is within normal limits and further evaluation or parent counseling regarding toilet training would only delay definitive treatment in this case.

Reassurance is incorrect because the child does display some developmental delay with milestones.

Immediate decompression with needle thoracostomy is correct. It is indicated for a tension pneumothorax. A tension pneumothorax results when positive pressure due to continued air leakage into the pleural space results in the collapse of the involved lung and a shift of the mediastinal structures to the contralateral side. As the cardiac output decreases due to decreased venous return, the condition can quickly lead to shock and death. Thus, prompt recognition and management are critical and often life-saving. Diagnosis and treatment should be based on the clinical findings. Treatment should not be delayed by waiting for confirmatory radiologic studies.

Pleurodesis (intrapleural instillation of sclerosing agents such as talc, fibrin glue, and antibiotics) is incorrect, however, it may reduce the risk of future recurrence.

Video-assisted thoracoscopy (VATS) is incorrect, however, it may reduce the risk of future recurrence.

Thoracotomy is incorrect, however, it may reduce the risk of future recurrence.

Sclerotherapy is incorrect as it may be indicated for patients with cystic fibrosis with recurrent pneumothoraces.

Generalized asymmetric tonic episodes and generalized flexor spasms is the correct choice. Generalized asymmetric tonic episodes and generalized flexor spasms are completely likely epileptic events associated with metabolic and diffuse abnormalities of brain development, including neurocutaneous syndromes and inborn errors of metabolism.

Tremors is incorrect. While jitters and tremors can be stopped with a bit of pressure, a little painful stimulus can stop episodes of benign neonatal sleep myoclonus.

Benign sleep myoclonus is incorrect. It is not elicited by any stimuli.

Hyperekplexia refers to an exaggerated startle with stimuli during generalized muscular stiffness.

Benign myoclonus of infancy is incorrect. It is a recurrent nonepileptic event during waking and not elicited by any stimuli.

Open incision and drainage of the left hip is correct. This girl has septic arthritis of her left hip with a gram stain showing what appears to be Staphylococcus aureus. Septic arthritis of the hip is an orthopedic emergency and needs open incision and drainage due to the risk of developing avascular necrosis of the femoral head. S. aureus is the most common cause of bone and joint infection and can be methicillin-resistant S. aureus (MRSA) or methicillin-susceptible S. aureus (MSSA). Pending culture results, she should receive IV vancomycin, and if MSSA, could be changed to a beta-lactam antibiotic such as nafcillin. The Infectious Diseases Society of America MRSA clinical guidelines state that some experts recommend the addition of rifampin for MRSA bone and joint infections after clearance of bacteremia and that drainage or debridement of the joint space should always be performed. The neutrophil oxidative burst assay would be to test for chronic granulomatous disease (CGD) but this patient was previously healthy and does not have a history of recurrent infections consistent with CGD. In addition, the more severe forms of CGD are X-linked recessive.

Premature thelarche is correct. It is characterized by breast development in young girls in the absence of other signs of precocious puberty (e.g., growth acceleration, changes in uterine size, and vaginal mucosa), possibly due to the persistence of maternal estrogens for a year or more in some infants.

Premature pubarche is incorrect. It manifests as early pubic hair, axillary hair (adult-type axillary body odor), or both in children without other signs of puberty.

Gonadotropin-dependent precocious puberty is incorrect. It involves the premature activation of the hypothalamic-pituitary-gonadal (HPG) axis.

Gonadotropin-independent precocious puberty is incorrect. It manifests in the presence of sex steroids independent of pituitary gonadotropin release. Central precocious puberty is gonadotropin-dependent and involves the early maturation of the entire hypothalamic-pituitary-gonadal (HPG) axis, with the complete physical and hormonal changes of puberty.

Skin hyperextensibility, widened atrophic scars and joint hypermobility, and family history is correct because these are major criteria required for diagnosis. Ehlers-Danlos syndrome (EDS), classic type, is a rare connective tissue disorder. It is diagnosed by family history and physical exam. There are major and minor clinical criteria; a combination of the major criteria is sufficient to make the diagnosis, whereas the minor criteria can contribute to making the diagnosis but are not sufficient unto themselves. The major criteria are:
1. skin hyperextensibility; the skin is “stretchy” and tends to be elastic
2. widened atrophic scars
3. joint hypermobility
4. family history
Minor diagnostic criteria are:
1. smooth, velvety skin
2. molluscoid pseudotumors, which are fleshy lesions that occur over pressure points such as elbows and knees
3. subcutaneous spheres- these are small mobile nodules that are found over bony prominences
4. complications of joint hypermobility, such as dislocations of joints (e.g., knees, shoulders), flat feet
5. muscle hypotonia and delayed gross motor development
6. easy bruising
7. hiatal hernia and anal prolapse secondary to tissue hyperextensibility and fragility
8. surgical complications, such as post-op hernias

Molluscoid pseudotumors and smooth, velvety skin is incorrect as these are minor criteria, which are not sufficient to make the diagnosis

Subcutaneous spheroids representing avascular fat globule is incorrect as these are minor criteria, which are not sufficient to make the diagnosis
Muscle hypotonia and delayed gross motor development is incorrect as these are minor criteria, which are not sufficient to make the diagnosis
Hiatal hernia, childhood anal prolapse, and cervical insufficiency is incorrect as these are minor criteria, which are not sufficient to make the diagnosis

Wilms’ tumor is correct. It is the most common renal malignancy in children, with two-thirds of cases being diagnosed before 5 years of age. Most children with Wilms’ tumor present with an abdominal mass and without other symptoms or signs. However, children may have abdominal pain, hematuria, and/or hypertension.

Renal cell carcinoma is incorrect. Renal cell carcinoma is rare in young children.

Congenital mesoblastic nephroma is incorrect. It usually presents as an asymptomatic abdominal mass before the age of 1 year. Hepatoblastomas should not be associated with hematuria or hypertension. While a neuroblastoma can present as an abdominal mass and can cause hypertension, hematuria would be very unusual.

Pulpectomy or incision and drainage is correct. Surgical drainage of any pus collection is the key to successfully treating patients with dental abscesses. A pulpectomy or incision and drainage are recommended for localized acute apical abscesses in the permanent teeth to resolve any infection quickly.

Antibiotics is incorrect. Antibiotics are not indicated for a localized dental abscess.

Endotracheal intubation or tracheostomy is incorrect. They may secure the airway in patients with dental abscesses, respiratory difficulty, oropharyngeal edema, or inability to handle secretions.

Transient synovitis of the hip is correct. It is most common between 2 and 10 years old and more common in boys than girls. It typically presents as an acute onset of hip pain and limp, often following a viral upper respiratory illness. Many affected children also give a history of preceding minor trauma. It is a self-limited condition and is typically managed supportively with oral non-steroidal anti-inflammatory drugs and rest. Differentiating between toxic synovitis of the hip and septic arthritis is critical, as the former is a self-limiting condition, and the latter requires emergent treatment. When combined, factors predictive of septic arthritis include refusal to bear weight, fever higher than 101.3°F (38.5°C), a serum white blood cell count of greater than 12,000/mm3, and an erythrocyte sedimentation rate of greater than 40 mm/hr.

Septic arthritis is incorrect. A septic hip is a surgical emergency requiring IV antibiotics and drainage. Joint fluid typically reveals greater than 100,000 white blood cells per mm3 with a predominance of polymorphonuclear cells or positive gram stain.

Osteomyelitis is incorrect. It presents similar to septic arthritis of the hip with fever and pain, but children may have some passive range of motion unless the joints are infected (Staph. aureus is involved).

Pyomyositis is incorrect. It follows infection of the muscles of the pelvic girdle and lower extremities, presents with fever, swelling of the affected muscle, overlying skin changes, and guarded hip motion and pain with passive movement.

Chondrolysis is incorrect. Chondrolysis of the hip involves progressive loss of articular cartilage space due to inflammation, evident radiographically.

Congenital cholesteatoma is correct. These are characterized by a white mass behind an intact eardrum in a child with no history of otitis. Generally associated with the middle ear and mastoid, they are deposits of squamous epithelium and keratin debris. Surgical excision is indicated, as expanding cholesteatomas can destroy surrounding bone.

Tympanosclerosis is incorrect. Tympanosclerosis (myringosclerosis or intratympanic tympanosclerosis) is caused by calcification of tissues in the middle ear.

Middle ear osteoma is incorrect. Osteomas of the middle ear are rare, benign neoplasms causing conductive hearing loss.

Chronic suppurative otitis media is incorrect. It manifests as a perforation in the tympanic membrane and active bacterial infection within the middle ear space for several weeks.

Pediatric otosclerosis is incorrect. Otosclerosis is a genetically mediated metabolic bone disease affecting only the human otic capsule and ossicles.

Begin IV doxycycline is correct. This patient has Rocky Mountain spotted fever (RMSF), subsequently confirmed with a Rickettsia rickettsii IgM of at least 1:1024 and IgG 1:256. RMSF serology is often negative in the first week of illness, and a negative serology should not delay the initiation of appropriate therapy if RMSF is clinically suspected. A febrile illness with a rash starts peripherally, involves the palms and soles, and becomes petechial is concerning for RMS. A delay in treatment with doxycycline beyond 5 days from the onset of illness is associated with increased morbidity and mortality. Regardless of age, doxycycline is the drug of choice for RMSF.

Obtain measles IgM is incorrect. Measles and enterovirus rashes typically begin centrally, and measles has a prodrome of fever, cough, coryza, and conjunctivitis.

Obtain ehrlichiosis serology is incorrect. Ehrlichiosis often does not have a rash and generally does not have conjunctival injection.

Type 2 is correct. Based on silence classification, type 2 OI is lethal in the perinatal period due to pulmonary failure secondary to multiple rib fractures and skeletal deformities.

Type 1 is incorrect. Type 1 OI is the mildest form associated with a fairly small number of fractures and modest deformities.

Type 3 is incorrect. Type 3 OI is the most severe non-lethal form, presenting with white sclerae, triangular face, and associated deformities.

Type 4 is incorrect. Type 4 is heterogeneous, with white sclerae, short stature, and less severe skeletal abnormalities.

Type 5 is incorrect. Hypertrophic calluses and ossification of the interosseous membranes are the hallmarks of type 5 OI. Except for type 5, other types arise from mutations in the COL1A1 or COL1A2 genes encoding the alpha1 and alpha2 chains of type 1 collagen.

Dyshemoglobinemia is correct. With dyshemoglobinemia, it is possible to have a substantial reduction in the blood’s oxygen-carrying capacity and yet have normal functional saturation. This does not ensure adequate oxygen-carrying capacity, thereby misleading someone who is unaware of the difference between functional and fractional saturation. Dyshemoglobinemia can skew both functional and fractional pulse oximetry readings.

Effects of ambient light is incorrect. While wrapping the oximeter probe in opaque material can overcome the issues with ambient light, oximeter performance can be seriously affected by dark skin pigmentation.

Low peripheral perfusion states is incorrect as it is among the 2 most common causes of faulty oximeter readings, especially the SpO2 or oxygen saturation measured.

Motion artifact is incorrect as it is among the 2 most common causes of faulty oximeter readings, especially the SpO2 or oxygen saturation measured.

Oral anticholinesterase treatment is correct. It is generally the first-line treatment in childhood AMG, while neostigmine may be preferred in neonates or infants. Only later, oral steroids (and other immunosuppressive agents such as azathioprine, methotrexate, cyclosporine, tacrolimus, or mycophenolate mofetil), intravenous immunoglobulin, or plasmapheresis may be useful. Individuals who are anti-AChR antibody-negative most likely have an immune-mediated disorder.

Thymectomy in early childhood can lead to subsequent immunosuppression and is not indicated unless there is the presence of a thymoma, generalized anti-AChR positive myasthenia, ocular myasthenia with progressive generalized weakness, and non-thymoma-related autoimmune myasthenia that is unresponsive to treatment.

Henoch-Schönlein Purpura (HSP) is correct. The presence of rash with fever, joint involvement, and microscopic hematuria suggests the diagnosis of HSP. HSP is an IgA-mediated systemic vasculitis that involves multiple organs, such as the GI tract, kidneys, joints, and skin. It is the most common vasculitis in children. It affects the small vessels of the system and starts as an erythematous macule. The rash later spreads to involve the lower legs and assumes the form of flat purpuric lesions. Rash is the most common symptom occurring in 95% to 100% of patients, followed by joints (up to 82%) and renal involvement (20% to 60%). Renal involvement may range from an acute nephritic syndrome that resolves in a few months to persistent proteinuria and nephrotic syndrome. Most other symptoms of HSP resolve without exception for renal issues, which can result in chronic morbidity.

Septic arthritis is incorrect. The presence of multiple joint involvement and other symptomatology make septic arthritis unlikely.

Hemolytic uremic syndrome is incorrect. Hemolytic uremic syndrome presents following a diarrheal infection and causes hemolytic anemia associated with increased blood urea nitrogen.

Child abuse is incorrect. The child has a fever and multiorgan involvement and is not indicating abuse.

Thrombocytopenic purpura is incorrect. Thrombocytopenic purpura is characterized by low platelets, which, in this case, is normal.

Vitamin B12 deficiency is correct. Vegan diets do not contain vitamin B12 and require supplementation with the vitamin. Significant anemia with neurological deficits can occur in children whose vegan mothers exclusively breastfeed.

Folate deficiency is incorrect. It can cause macrocytic anemia like vitamin B12 deficiency but is unlikely in this scenario. Folate deficiency is more likely seen in infants exclusively fed goat’s milk.

Iron deficiency is incorrect. It typically causes microcytic anemia.

Zinc deficiency is incorrect. It causes a skin rash and diarrhea.

Vitamin K deficiency is incorrect. A vitamin K deficiency presents with bruising and excessive bleeding.

Vitamin D is the correct answer. This child has rickets caused by vitamin D deficiency. She is dark-skinned and was predominantly breastfed, both of which are risk factors for vitamin D deficiency. Other features of rickets include delayed closure of the anterior fontanel, enlargement of the metaphases producing widening of joints, weight-bearing deformities such as bowlegs and knock-knee, and rachitic rosary, as well as generalized muscular hypotonia. Apart from generalized osteopenia on X-rays, the metaphyses exhibit widening, cupping, and fraying.

Vitamin A is incorrect. It is associated with follicular hyperkeratosis on the extensor aspects of the limbs. In addition, keratinization of the conjunctiva and lacrimal glands leads to dryness of the eyes. This can progress to keratomalacia and blindness.

Vitamin C is incorrect. In a vitamin C deficiency, generalized tenderness causing pseudoparalysis, swelling of gums, and petechial hemorrhages can be seen.

Vitamin K is incorrect. It leads to a bleeding tendency.

Vitamin E is incorrect. It can cause neuropathy and hemolytic anemia in children with difficulty absorbing vitamin E.

Caesarean delivery is correct. The incidence of RDS increases in white male infants, infants born to diabetic mothers, infants born via Caesarean delivery, second-born twin, and infants with a family history of respiratory distress syndrome.

Use of antenatal steroids is incorrect. It’s associated with decreased incidence.

Pregnancy-induced or chronic maternal hypertension is incorrect. It’s associated with decreased incidence.

Prolonged rupture of membranes is incorrect. It’s associated with decreased incidence.

Maternal narcotic addiction is incorrect. It’s associated with decreased incidence.

Recommend the medical team prescribe olanzapine 2.5 mg as needed for nausea and irritability is correct. The most disabling symptom for this patient is nausea. Olanzapine has been shown to be among the most beneficial pharmacological strategies for nausea as it antagonizes both D2 and 5HT3. Moreover, it might help with patient’s recent irritability.

Evaluate for attention-deficit hyperactivity disorder and prescribe low-dose stimulants that may also help cancer-related fatigue is incorrect. It is an important intervention for this patient, but at this point the nausea is the most disabling symptom; the management of this nausea should not be delayed.

Call the mother and set a family meeting is incorrect. It is an important intervention for this patient, but at this point the nausea is the most disabling symptom; the management of this nausea should not be delayed.

Call psychosocial oncology and arrange follow up for psychotherapy is incorrect. It is an important intervention for this patient, but at this point the nausea is the most disabling symptom; the management of this nausea should not be delayed.

Leave the room, call the oncologist, and tell him to go back in the room to obtain consent from the father for a psychiatry consult is incorrect. It is important to discuss with the primary team if patient knows about the psychiatric consult, but in this case, the father seems to be accepting of the consult. Leaving the room to have the oncologist come back to discuss a psychiatric consultation will delay the treatment of this patient.

Maternal cannabis use can result in infant exposure and increased risk of hyperactivity, impulsivity, and neurodevelopmental delays is correct. Cannabis use during pregnancy has been linked to multiple health issues for infants, including poor executive functioning, hyperactivity, impulsivity, low birth weight, stillbirth, and other conditions. Pregnant mothers using cannabis should be counseled of the risks and given the recommendation to quit.

Evidence does not support adverse neurobehavioral outcomes for babies exposed to cannabis during pregnancy is incorrect because several studies support an increased prevalence of attention deficit hyperactivity disorder (ADHD) symptoms and aggression in children exposed to cannabis during gestation.

Concentrations of cannabis in breast milk are generally lower than maternal plasma levels while breastfeeding is incorrect because cannabis transfers into breast milk at a very high (up to 8-fold the maternal plasma) concentration, and the American Academy of Pediatrics recommends against breastfeeding with regular cannabis use.

Alcohol concentration in breast milk is relatively high, up to half of the weight-adjusted maternal dose is incorrect because alcohol concentration in breast milk is low, about 5%-6% the maternal dose.

Higher amounts of nicotine are transferred through the placenta as compared to through breast milk is incorrect because nicotine transfers at a higher rate in breast milk than transplacentally which is different than most exposures; smoking may result in altered infant sleep patterns and reduced milk production.

QTc of 530 msec on EKG is correct. Arrhythmias (including a prolonged QT interval) other than sinus indicate the need for admission and further evaluation because the patient would be at risk for continued decompensation or death without hospitalization. Hospitalization is also recommended for bradycardia less than 40 beats per minute.

Sinus bradycardia with a rate of 55 bpm is incorrect because a sinus bradycardia of less than 40 bpm is considered (by most guidelines) a reason for inpatient hospitalization.

Body Mass Index (BMI) of 16 alone is not a reason to hospitalize a patient with anorexia nervosa. Recommendations for inpatient hospitalization are if the weight is less than 70% of optimal body weight or if their BMI is less than 15 kg/m2.

Continued exercise despite weight loss alone is not enough to warrant inpatient hospitalization. The patient would need at least 1 other reason for hospitalization (like serious medical complications, unstable vitals, etc.)

A blood pressure of 100/70 mmHg is incorrect. Inpatient admission recommendations are for blood pressures less than 80/60 mmHg or for severe symptoms of lightheadedness.

Symptoms of post-traumatic stress disorder is correct. This patient’s oppositional and agitated behavior with healthcare providers and interventions could most likely be explained by a trauma response from recent intensive care.

Oppositional defiant disorder is less likely given a lack of ongoing history of symptoms.

Pain associated with acute infection is incorrect since he is comfortable with mom and not persistently in pain.

Recent corticosteroid treatment is unlikely since he is comfortable with mom and not persistently in pain.

Specific phobia of needles could be feasible but likely involve less reactivity to all health care interventions.

Endocrinology consultation is correct. This is the primary intervention to treat the underlying cause of the patient’s complaints.

Begin treatment with sertraline is incorrect. This patient most likely has opioid-induced hypogonadism from prolonged treatment with narcotics during his cancer treatment. His history is not consistent with a depressive disorder, and thus, options for treatment of depression would not be indicated.

Begin treatment with bupropion is incorrect. This patient most likely has opioid-induced hypogonadism from prolonged treatment with narcotics during his cancer treatment. His history is not consistent with a depressive disorder, and thus, options for treatment of depression would not be indicated.

Referral for psychotherapy may be appropriate, but is not the first/next step in management.

Begin treatment with sildenafil may be recommended in the interim, but the primary intervention would be endocrinology consultation to treat the underlying cause of the patient’s complaints.

Down’s syndrome (trisomy 21) is characterized by meiotic nondisjunction of the chromosomes in about 95% of children with an extra chromosome 21 found in all cells, while translocation and mosaicism account for the remaining 5% (Committee on Genetics, American Academy of Pediatrics [AAP], 2001). They have an increased risk for cardiac defects (50%), with atrioventricular septal defect the most common; eye disease (60%); hearing loss (75%); otitis media (50% to 75%); obstructive sleep apnea (50% to 75%); thyroid disease (15%); gastrointestinal abnormalities (5%), including duodenal stenosis/atresia (50%), imperforate anus (20%), Hirschsprung disease (10%); and leukemia (less than 1%).

Turner syndrome occurs in females with an XO karyotype. Some associated features of Turner syndrome include short stature, primary amenorrhea due to streak ovaries, webbed appearance of the neck, and congenital heart disease.

Fragile X syndrome is a sex-linked disorder (X-linked dominance with variable penetrance) with a folate-sensitive fragile site on the X chromosome. Fragile X syndrome is the most common form of inherited intellectual disability. Clinical features include protruding eyes, learning disabilities, poor speech, hyperactivity, shyness, social anxiety, short attention span, large testicles, and long face.

Marfan syndrome is associated with a defect in the fibrillin-1 gene (FBN1) on chromosome 15, inherited as an autosomal dominant trait with clinical features manifesting as connective tissue disorders affecting the skeletal system, cardiovascular system, eyes, and skin. Symptoms include arachnodactyly, pectus excavatum, pectus carinatum, flat feet, excessively flexible joints, micrognathia, scoliosis, and learning disabilities.

Achondroplasia is the most common form of skeletal dysplasia inherited as a mendelian autosomal dominant trait with complete penetrance; clinical features include short stature with normal trunk length, rhizomelic shortening of the extremities, macrocephaly, frontal bossing, depressed nasal bridge, bowing of the lower extremities, trident hands, lumbar lordosis, and genu varum.

Pseudohypoparathyroidism is correct. The child in the vignette presented with facial twitching secondary to low calcium. The low calcium is accompanied by elevated phosphorus, which is often seen in hypoparathyroidism. However, parathyroid hormone (PTH) levels are low in hypoparathyroidism. The elevated PTH levels associated with low calcium and high phosphorus is suggestive of pseudohypoparathyroidism (PHP).

Vitamin D deficiency usually presents as low calcium and phosphorus with elevated parathyroid hormone (PTH). The high phosphorus in this patient makes vitamin D deficiency an unlikely cause for hypocalcemia in this patient.

Parathyroid adenoma results in hyperparathyroid state with hypercalcemia and low phosphorus. It is unusual for poor intake to result in a low calcium level of this magnitude. Further phosphorus levels are also low in poor intake states.

Start her on oral contraceptive pills is correct. This girl has mild-to-moderate anovulatory uterine bleeding (AUB) and should be treated with oral contraceptives. Anovulation refers to the absence of ovarian cycles and is suggested by irregular bleeding patterns; it is common within the first 1-2 years following menarche.

No specific treatment; provide reassurance that her period should stop soon is incorrect since she is still bleeding with a low hemoglobin level.

Start her on high-dose ibuprofen is incorrect since NSAIDs are generally used for ovulatory heavy menstrual bleeding but are less commonly used for anovulatory bleeding.

Perform a speculum exam is incorrect since girls with moderate anovulatory uterine bleeding (AUB) can be managed based on a history and hemoglobin level. Girls with severe AUB should undergo evaluation for a bleeding disorder, which may warrant pelvic ultrasonography.

Obtain blood hormone studies is incorrect since hormone studies are not helpful in the management of anovulatory uterine bleeding (AUB); they would be indicated only if other features of the history, physical, or response to treatment pointed away from a diagnosis of anovulatory uterine bleeding.

Prescribe an antidepressant medication with appropriate monitoring is correct because this should be considered in the management of depression after a period of active monitoring by the primary care physician.

Refer her to a support group for teens is incorrect since support groups for teens are not recommended in the management of depression.

Recommend academic tutoring is incorrect since her primary problem is depression and not academic concerns.

Refer her to a substance abuse program is incorrect since her primary problem is not substance abuse but depression.

Prescribe lithium is incorrect since her primary problem is not bipolar disorder but depression. Lithium would be the appropriate drug for bipolar disorder.

Review behavioral (Vanderbilt) rating scales and grade reports is correct. This child is manifesting several symptoms of attention-deficit hyperactivity disorder (ADHD). This is correct since the American Academy of Pediatrics (AAP) recommends that the primary care physician (PCP) obtain and review a validated ADHD instrument and school records. The Vanderbilt rating scale is one such instrument.

Reassure mother that this is normal boy behavior is incorrect since the pediatrician should obtain more information before concluding whether the behavior is normal or abnormal.

Recommend a psychoeducational evaluation is incorrect since the Academy of Pediatrics (AAP) statement does not recommend a psychoeducational evaluation but suggests that the primary care physician (PCP) review the same if available.

Prescribe a stimulant medication is incorrect since it is important to first make a diagnosis of ADHD before considering medication. In addition, a variety of behavioral and educational changes are recommended before medication use.

Refer him to a mental health provider is incorrect since the Academy of Pediatrics (AAP) statement does not recommend referral to a mental health provider as the first intervention for a child with potential ADHD.

Exchange transfusion is correct. Individuals with sickle cell disease can develop both ischemic as well as hemorrhagic strokes. Ischemic stroke is more common than hemorrhagic stroke in children and adolescents with sickle cell disease, while hemorrhagic stroke is more common in adults. It is important to distinguish an acute ischemic versus hemorrhagic stroke in patients with sickle cell disease. Urgent exchange transfusion is used for patients with acute ischemic stroke (and patients with hemorrhagic transformation of an acute ischemic stroke), but not for those with hemorrhagic stroke.

Red blood cell (RBC) transfusion is incorrect because the goal of therapy is to lower the percentage of sickle hemoglobin to =30 percent of total hemoglobin and to increase total hemoglobin to approximately 10 g/dL. The first aim cannot be achieved by RBC transfusion. However, many centers will first given an RBC transfusion while getting the patient ready for an exchange transfusion.

Hydroxyurea is incorrect because hydroxyurea is not useful in the acute treatment of stroke.

Intravenous fluid (IVF) bolus is incorrect because intravenous fluids do not directly influence the stroke.

Pain management is incorrect because pain management does not directly influence the stroke.

CGH (comparative genomic hybridization) microarray is correct. The child in the vignette has a syndrome that has cognitive delays and autism spectrum disorder-type features (absent speech, poor eye contact). He also has a facial anomaly (the ear tags) that would make one consider a genetic syndrome. Current recommendations are that any child with autism spectrum disorder, developmental delays, or intellectual disability should have molecular testing. CGH (comparative genomic hybridization) microarray is the recommended test.

Routine chromosome analysis is incorrect. Routine chromosomal analysis only looks at the number and structure of the child’s chromosomes; although this may provide a diagnosis, it is not complete enough. A microarray allows the detection of much smaller chromosomal deletions and duplications.

Chromosome studies with fluorescence in situ hybridisation (FISH) for 7q11.23 is incorrect: 7q11.23 is Williams syndrome. Children with Williams syndrome are quite chatty and loquacious and are said to have a “cocktail party personality.”

Complete blood count (CBC), lead is incorrect. Although a child with lead intoxication would have cognitive delays, they would have speech, have no autistic features (poor eye contact), and there are no associated ear tags.

Thyroid studies is incorrect. Although a child with undiagnosed and untreated congenital hypothyroidism would have cognitive delays, they would have speech, have no autistic features (poor eye contact), and there would not be associated ear tags.

An apnea test is correct. In documenting brain death, an apnea test is the standard test performed after all other clinical criteria have been met. Note that metabolic disturbances, hypothermia, and hypotension should all be corrected prior to brain death testing.

A cerebral radionuclide study is incorrect. This is an ancillary study performed when an apnea test is not possible (e.g. because of oxygenation defect and clinical decompensation). Additionally, the use of a cerebral radionuclide study to determine cerebral blood flow (CBF) and electroencephalogram (EEG) remain the most widely used methods to support the clinical diagnosis of brain death in infants and children.

Four-vessel cerebral angiography is incorrect. This is an ancillary study performed when an apnea test is not possible (e.g. because of oxygenation defect and clinical decompensation). Four-vessel cerebral angiography is also the gold standard for determining absence of cerebral blood flow (CBF). This test can be difficult to perform in infants and small children and is not readily available at all institutions.

An electroencephalogram is incorrect. This is an ancillary study performed when an apnea test is not possible (e.g. because of oxygenation defect and clinical decompensation).

No further testing is indicated is incorrect; an apnea test should be performed to confirm brain death unless otherwise contraindicated.

Fasting blood glucose is correct as she likely has type 2 diabetes mellitus and a fasting glucose would be elevated. A hemoblobin A1C would also be a good option.

Evening cortisol and ACTH levels is incorrect as her dark skin is acanthosis nigricans, and not hyperpigmentation related to cortisol abnormalities. Her obesity is not secondary to Cushings disease.

Early morning cortisol and ACTH levels is incorrect as her presentation is not consistent with adrenal disorders.

Abdominal ultrasound is incorrect as it may show the damage secondary to the obesity (ie, a fatty liver), it would not help with the diagnosis of diabetes.

Urine culture is incorrect as her symptoms are vaginal (yeast infection) and not that of a urinary tract infection.