Answer: C

DiGeorge syndrome results in absence or hypoplasia of the parathyroid gland which can lead to hypocalcemia resulting in seizures. Dysmorphic facial features characteristic of DiGeorge syndrome include low-set ears, micrognathia, and hypertelorism. Cleft palate is present 70% to 80% of the time in this syndrome. Hypotonia is common in infancy in those with the syndrome.

Answer: B

Pneumothorax is a known potential complication of central line placement. Unequal breath sounds with impaired oxygenation are hallmark signs. Foreign body aspiration could have a similar presentation but is less likely in a supervised environment.

Answer: C

The recommended range for dosing of tobramycin in patients with CF is 7.5 to 10 mg/kg/24 hours. The risk of toxic side effects is real, but the dose should be increased to try to achieve therapeutic target, but not above the upper limit to minimize risk of toxicity.

Answer: C

Obtaining an inspiratory/expiratory chest radiograph assists in evaluating lung deflation on exhalation and presence of unilateral air trapping. Pleural effusions are generally not associated with foreign body aspiration. Objects most commonly position themselves on the vertical plane. Bilateral hyperinflation is common in asthma/status asthmaticus.

Answer: D

The clinical presentation of a child with suspected pulmonary edema can include tachypnea, dyspnea, tachycardia, hypoxia, and wheezing. Cough with frothy sputum (often pink), diaphoresis, orthopnea, and paroxysmal nocturnal dyspnea with increased work of breathing are other clinical findings. On auscultation, crackles may be heard along with a third heart tone or gallop, which is indicative of cardiogenic pulmonary edema.

Answer: A

Dobutamine is used to treat cardiogenic shock and works by increasing contractility and promoting peripheral vasodilation. It will also increase renal perfusion and urine output.

Answer: A

Cardiac tamponade occurs as a result of several mechanisms including trauma. Clinical presentation includes Beck triad: hypotension—from low cardiac output; distended neck (jugular) veins—from heart compression; and muffled (distant) heart sounds—from fluid in pericardial space. Patients often have pulsus paradoxus with a narrow pulse pressure, altered neurologic status, audible pericardial rub, and shock with tachycardia and tachypnea.

Answer: C

Bacterial pneumonia, while overall much more common than Pneumocystis, usually has a focal finding on chest radiograph and typically causes fever. Viral infections are usually associated with other symptoms such as rhinorrhea and cough. Tuberculosis is a reasonable concern, but usually does not cause persistent tachypnea or oxygen desaturations. Hilar adenopathy on chest radiograph would make the diagnosis more likely. Pneumocystis jirovecii is likely as it is common in immunocompromised patients, and without prophylaxis, it would be highest on the differential diagnosis.

Answer: A

There are a variety of organisms that are responsible for causing meningitis in the newborn period, but these organisms change at 1 month of age and then again between 3 months and 2 years of age. Recognizing the potential etiology assists in providing appropriate treatment. The organisms that typically cause meningitis in infants beyond the neonatal period are Haemophilus influenzae type b, Streptococcus pneumoniae, Neisseria meningitidis, late-onset group B streptococcus and Bordetella pertussis.

Answer: B

Oxygen administration, respiratory support with intubation, and mechanical ventilation as needed are the first consideration for a child who presents with septic shock. Early establishment of vascular access, fluid resuscitation up to and over 60 mL/kg within 15 minutes, correction of hypoglycemia and hypocalcemia, and prompt antibiotic administration follow soon after.

Answer: A

There are three specific types of SMA, with the first one (type I) typically diagnosed early in infancy with symptoms of hypotonia with generalized weakness, poor head control, lack of meeting milestones, paradoxical breathing, and areflexia. Once genetic testing indicates SMA type I, parents can be counseled that prognosis is poor and that there is no specific therapy or cure. Management is directed at aggressive treatment of symptoms, but palliative care is also often appropriate. SMA type II is usually diagnosed later in infancy with typically normal development until 6 months of age, with variable life expectancy, but often through adolescence. SMA type III may not be diagnosed until adulthood and these patients may live a normal life expectancy with more involvement in lower extremities, often requiring wheelchair for ambulation.

Answer: A

Typically, young children do not experience LP headaches, but by using the smallest needle possible for an LP, the patient will have less risk of postprocedure headache due to leaking of CSF outside of the spinal column. A larger needle increases the risk of fluid leakage. The position of the patient during the procedure does not change the risk of a postprocedure headache.

Answer: B

In HLH, serum ferritin is often quite elevated, and fibrinogen is often notably low (leading to a clinical coagulopathy when coupled with low platelets). Low hemoglobin and platelet count are part of the HLH-2004 diagnostic criteria, but these values are mild compared to those usually seen in HLH. Although ESR can be a marker for inflammation, it is generally nonspecific and not a part of the HLH-2004 criteria.

Answer: C

Anaphylaxis should be treated with IM or IV epinephrine, even with mild symptoms such as pruritus, urticaria, and angioedema. Delay in administration of epinephrine has been associated with poor outcomes and additional doses may be required.

Answer: C

Hepatoblastoma is the most common liver mass in children. An elevated α-fetoprotein is present in 90% of children who are diagnosed with this form of cancer. A Wilms tumor is a renal mass, not liver. Hepatocellular carcinoma is rare in young children and is usually associated with a long-standing chronic metabolic liver disease. Neuroblastoma is not a liver tumor and is most commonly of adrenal origin.

Answer: D

Elevated potassium, phosphorous, and uric acid levels are indicative of tumor lysis syndrome, a side effect of chemotherapy when tumor cells are broken down. Elevated BUN and creatinine with low calcium can indicate renal failure which occurs as a result of tumor lysis syndrome.

Answer: C

Inadequately treated exudative parapneumonic effusion can be a risk for the formation of an empyema, requiring surgical debridement and prolonged antibiotic therapy.

Answer: B

Macrolides are the treatment for pertussis. Erythromycin and clarithromycin are not recommended in the neonatal period due to the risk of infantile hypertrophic pyloric stenosis. Azithromycin has equal efficacy. Bactrim is a preferred second-line agent, but duration of treatment is 14 days. Intravenous ceftriaxone is not recommended for treating pertussis.

Answer: A

A spiral CT is rapid, requiring the least amount of transport time, and is highly sensitive and specific for pulmonary embolus which needs to be considered in any patient who has an acute onset of hypoxia and dyspnea.

Answer: C

The goal of therapy for a child with acute hypertension is to slowly bring the BP down to avoid hypoperfusion and end-organ failure. In the first 6 to 12 hours, BP should not be decreased by more than 25% to 33%.

Answer: A

Pericarditis occurs when the layers of the pericardium become inflamed as a result of many different etiologies, including bacterial and viral infectious processes, metabolic disorders, medication reactions, and Kawasaki disease, among others. The symptoms of acute pericarditis include chest pain and fever. A pericardial friction rub is often noted on auscultation.

Answer: D

An infant less than 2 months of age with fever requires a septic workup, and in this case, there is also the need to distinguish between direct and indirect hyperbilirubinemia to assist in management. Transaminases may be transiently elevated due to infection, so further evaluation is indicated.

Answer: A

In children with burns >15% TBSA, formulas such as the Parkland formula are used to calculate fluid administration. The Parkland formula recommends 4 mL/kg multiplied by percentage of TBSA burned; half is replaced in the first 8 hours and the rest over the following 16 hours. Example: 4 mL × 48 × 21 = 4,032 mL; half of that = 2,016 mL divided by 8 hours = 252 mL/hour for the first 8 hours. Isotonic crystalloid solutions, such as Lactated Ringers, are recommended for initial fluid resuscitation.

Answer: C

The risks of both accidental and intentional ingestion of acetaminophen include hepatic failure with potential for the need for liver transplant. It is important to identify the time of acetaminophen ingestion to allow more accurate evaluation of hepatotoxicity risks with serum drug levels. Obtaining serial liver function tests should continue with support from the poison control center and identification of when to stop obtaining laboratory tests based on results and clinical status.

Answer: A

According to the PECARN algorithm, a child who has any alteration in neurologic status and a potential skull fracture would warrant a head CT with first management. An MRI may be more preferable as it does not require the exposure to radiation unlike a CT. Neurologic evaluation is extremely important with access to neurosurgical care as well.

Answer: B

Due to the persistent vomiting and loss of gastric acid, infants with pyloric stenosis will develop a hypochloremic, hypokalemic metabolic alkalosis. The infant must have fluid and electrolyte balance restored and stabilized prior to surgical repair. Soon after the surgical procedure the infant will be able to feed normally again.

Answer: B

A high total bilirubin with presence of conjugated or direct bilirubin likely indicates cholestatic liver disease. These results are not physiologic jaundice of the newborn which has an associated elevated unconjugated bilirubin level. Evaluation by a pediatric gastroenterologist is most important as this child could have biliary atresia, with the best prognosis associated with timely diagnosis and treatment. The interventions of placing the infant in direct sunlight and maintaining hydration with regular bowel movements aid in the resolution of unconjugated hyperbilirubinemia, not conjugated hyperbilirubinemia. It is unlikely that repeating the laboratory studies will reveal different results.

Answer: C

No additional antibiotic therapy is needed for a child following appendectomy for appendicitis which did not include perforation. For a perforated appendix, a longer duration of IV antibiotics is indicated and is based on the child’s response to therapy.

Answer: A

There are several reasons for the placement of a tracheostomy tube, including the need for continued ventilation outside the hospital setting. For a child who has been on a ventilator for a long period of time and is not able to be successfully weaned, the tracheostomy is an acquired indication for either temporary or permanent respiratory support.

Answer: A

The Mallampati score is an assessment of the airway of a patient usually completed by an anesthesia provider prior to intubation. The patient is assessed by opening the mouth for direct visualization of the airway. A score of class 3 or 4 or higher is associated with a more difficult airway and intubation.

Answer: C

Obtaining a CT of the orbits and paranasal sinuses, with and without contrast, should be done first to differentiate the orbital cellulitis. While early treatment with IV antibiotics improves patient outcomes, it is important to obtain blood cultures prior to initiation of antibiotics. A lumbar puncture should be performed in patients who are exhibiting meningeal signs or if central nervous system dysfunction is suspected. Although an otolaryngologist and ophthalmologist may need to be consulted for further examination and treatment, this is not the first priority.

Answer: D

Absolute contraindications to lung transplantation include malignancy within past 2 years, immunodeficiency syndrome, hepatitis B or C with liver disease, severe neuromuscular disease, multiorgan system dysfunction. Relative contraindications include pleurodesis, renal insufficiency, markedly abnormal BMI, chronic airway infection with specified organisms, severe scoliosis, active collagen disease, mechanical ventilation, among others. Careful evaluation is most important in any situation.

Answer: A

A normal QTc value is 0.44 m/second or less. Prolonged QT syndrome can be inherited or acquired, so the rationale for the prolonged value must be evaluated prior to the athlete returning to any sports activities. A cardiac consult should be obtained before any decisions of management or return to play is determined. If not inherited, the most likely causes of a prolonged QT interval include certain medications, intracranial processes, hypocalcemia, and hypokalemia; hence electrolyte assessment is also important in this case.

Answer: C

Hyperkalemia typically causes peaked T waves on EKG and is the most common presentation of this abnormality in children. If a child has an elevated potassium level, an EKG can support the reliability of the laboratory result. In all cases, however, the electrolytes should be repeated.

Answer: A

Many chemotherapy agents are considered cardiotoxic and can have either short- or long-term effects on cardiac function, so a baseline EKG or echocardiography is indicated along with interval checks as the child progresses through therapy.

Answer: A

There are two types of neurofibromatosis, identified as types I and II. Type I is usually diagnosed in infancy or at least prior to age 10 and involves the presence of café au lait spots in more than 90% of patients. Lisch nodules which are hamartomas of the iris are the defining characteristic. Type II neurofibromatosis occurs in patients in adult years and along with other findings, including glioma, meningioma, and hearing loss.

Answer: C

Simple febrile seizures typically do not cause any long-term effects, occurrence of a febrile seizure lasting less than 15 minutes does not require diagnostic evaluation or treatment unless there are clinical findings of concern, including meningeal signs. Imaging is also not recommended due to the radiation exposure and intracranial structural abnormalities are not common in young children.

Answer: A

A brain tumor that obstructs the flow of cerebrospinal fluid can cause increased intracranial pressure, identified by symptoms of headache, emesis, and altered neurologic state.

Answer: A

The typical patient with a diagnosis of SCFE is an obese adolescent who presents with a limp. Complaints of pain to the hip, knee, or thigh and holding the leg in an externally rotated position are other findings. SCFE requires urgent intervention, and surgical correction is necessary to prevent further slippage. The 5-year-old with a limp is classic for Legg-Calvé-Perthes disease, which is caused by poor blood supply to the femoral head causing avascular necrosis. This occurs in prepubertal, immature children and it affects males more than females. The 6-year-old with a painful limp is common for transient synovitis or septic arthritis which is an inflammatory process often following a viral illness. It can be acute or gradual onset and is usually unilateral.

Answer: A

Gastrointestinal losses are one of the leading causes of hypomagnesemia in the hospitalized child.

Answer: C

LCPD is an avascular necrosis of the proximal femoral head resulting from compromise of the tenuous blood supply to this area. It usually occurs in prepubertal children aged 4 to 10 years and is more prominent in boys than in girls. In the vast majority of cases, the disorder is unilateral. Initial therapy for LCPD includes rest and slow restoration back to use. If not identified early and managed, a child may require surgical intervention.

Answer: C

Central DI is the inability to concentrate urine secondary to a vasopressin deficiency which can occur as a result of head trauma due to edema in the area surrounding the hypothalamus and pituitary gland. Classic findings of DI include excess urine output with low urine osmolarity (specific gravity <1.005), high serum osmolarity, and hypernatremia. Polyuria can be >4 mL/kg/hour in infants and 150 mL/kg/day in older children. Direct measurements of urine sodium excretion, in addition to serum and urine osmolality, are critical in diagnosing DI. Laboratory studies to confirm the diagnosis of DI and to differentiate it from cerebral salt wasting include a urine sodium <30 mEq/L, urine osmolarity <200 mOsm/L, serum sodium >150 mEq/L, and serum osmolarity >295 mOsm/L. DI should be identified as soon as possible to prevent profound hypovolemic hyperosmotic dehydration. SIADH results in decreased urine output due to an excess in antidiuretic hormone.

Answer: B

CAH is characterized by a 21-hydroxylase deficiency, which typically presents with a salt-wasting crisis in the first few weeks of life. This enzyme deficiency results because of insufficient adrenal production of cortisol and aldosterone, in addition to an excess of androgens, which can also cause ambiguous genitalia in female infants. Clinical manifestation of acute adrenal crisis in CAH includes lethargy, poor feeding, altered sensorium, vomiting, hypotension, and hypothermia. Laboratory findings include hypoglycemia, hyperkalemia, hyponatremia, dehydration, and metabolic acidosis.

Answer: A

Apnea is defined as cessation of airflow through the respiratory tract for 20 seconds or longer, or a shorter respiratory pause associated with bradycardia or cyanosis, significant enough to cause arterial hypoxemia and hypercapnia. There are several types of apnea; the two primary types are obstructive and central. Obstructive apnea occurs when the airway is occluded with tonsils or tissue, especially during relaxed phases, such as sleep.

Answer: D

A history associated with RPA may include endotracheal intubation, oral foreign object, dental procedures, and recent infection of any structures that drain into the retropharyngeal space. Characteristic findings associated with RPA include trismus, drooling, dysphagia, sore throat, neck swelling, and fever.

Answer: A

Components of a quality improvement plan include initially identifying a clear aim, conducting a stakeholder analysis, and assembling a team. Continue by selecting “changes” or interventions that are hypothesized resulting in improvement. Next steps include selecting measures, creating a data collection process, testing changes to determine if they result in improvement, and developing a strategy for sustaining and spreading successful changes.

Answer: B

There are some dental procedures including dental radiography, placement of orthodontic appliances, and shedding of deciduous teeth that do not require endocarditis prophylaxis. A dental extraction would qualify as an appropriate procedure prior to which patients with prosthetic heart valves should receive bacterial endocarditis prophylaxis. Based on the most recent American Heart Association guidelines, aortic root dilation and tricuspid regurgitation are not conditions that routinely require endocarditis prophylaxis.

Answer: B

CHARGE syndrome is a genetic pattern of birth defects which include coloboma of the eyes, choanal atresia or stenosis, cranial nerve involvement, outer, inner, and middle ear abnormalities, cardiac defects, cleft lip and palate, and several others that are not typically associated with other patterned abnormalities. CHARGE syndrome includes hearing loss and outer ear abnormalities, such as the outer ear appearing wide, low, and with no earlobe. Angelman syndrome features are characteristically dysmorphic, inappropriately happy, and severe mental retardation. Cystic fibrosis is characterized by poor growth and lung dysfunction. Rett syndrome is usually found in females, and between 6 and 18 months development begins to regress, and microcephaly ensues.

Answer: C

Oxygen therapy to provide support for a child with an underlying respiratory illness includes nasal cannula, providing 24% to 44% based on liter flow. The nasal cannula is typically tolerated well and can be left in place during inhalation treatments.

Answer: A

Heated high-flow oxygen delivery systems include Vapotherm. High-flow therapy is effective for any age child, particularly young children and infants. When lower liter flow is not effective, increasing the flow is appropriate prior to considering a more invasive type of therapy such as BiPAP or continuous positive airway pressure.

Answer: B

After back-to-back albuterol treatments and corticosteroids, the next intervention for a child in status asthmaticus should be continuous albuterol. A terbutaline bolus is not given before continuous albuterol is attempted. Magnesium as a bolus would also be an appropriate choice at this time. Intubation or BiPAP therapy may be needed if the child deteriorates.

Answer: A

A cricothyrotomy is an invasive surgical procedure that will secure an airway in a patient with difficult airway. The American Society of Anesthesiologists’ Difficult Airway Algorithm describes “surgical or percutaneous tracheostomy or cricothyrotomy” as invasive airway access. Use of an LMA, Wisconsin 2 blade, or a light wand is a noninvasive strategy for a difficult airway.

Answer: A

Some infants who have been mechanically ventilated in the neonatal period develop a narrowing of their tracheal tissue, as the cartilage has “molded” to the endotracheal tube. With continuous positive pressure, a set level of continuous pressure is provided throughout the entire respiratory cycle. The area of narrowing is stented open with additional pressure, improving the airway obstruction and reducing the work of breathing.

Answer: A

The American Academy of Pediatrics has indicated that one single dose of Decadron at 0.6 mg/kg/dose given orally to a young child with croup symptoms is beneficial at preventing worsening respiratory distress.

Answer: C

The bidirectional Glenn procedure is completed as the stage 2 repair for tricuspid atresia. The surgery involves disconnecting the superior vena cava (SVC) from the right atrium and connecting the SVC directly to the right pulmonary artery. This procedure is also used for single ventricle anatomy.

Answer: B

Secondary hypertension is more common in children than in adults and is most often caused by renal disease. Adrenal insufficiency, certain medications (including corticosteroids), sleep apnea, stress, and anxiety can also be causative factors. Genetic predisposition is the most common cause of primary hypertension in children.

Answer: A

Calcium channel blockers work by widening blood vessels which decreases cardiovascular resistance. They slow the movement of calcium into the cells and are recommended for children with asthma. Clonidine is a sympatholytic medication which is not first line for pediatric hypertension therapy and a diuretic may contribute to dehydration in an athlete. ACE inhibitors may not be a first choice as they also can contribute to dehydration, especially in an athlete who needs continuous hydration.

Answer: B

Typical cerebrospinal fluid results for viral meningitis include a slightly elevated WBC count (<500 × 10⁶/L), protein close to normal (<100 g/L), and a normal glucose level. Gram stainings and cultures are negative. A positive herpes simplex virus (HSV) polymerase chain reaction would indicate a HSV infection.

Answer: B

Oral formulations for invasive fungal disease are seldom appropriate, especially in an immunocompromised child. Fluconazole has no activity against Aspergillus. IV voriconazole is a reasonable choice, but would be inappropriate in light of its interactions with many immunosuppressive medications, which this patient is likely prescribed. Amphotericin is the most appropriate therapy, and urine isolates of invasive fungal species are real and should be treated as such.

Answer: A

Symptoms of headache, fever, malaise, myalgias, and cough represent influenza, especially during the winter months. It is important to determine the history of illness prior to completing laboratory testing or beginning management. Asking about history of vaccination and obtaining influenza screening are appropriate initial assessment or evaluation. It is also important to assess for risk factors, including underlying cardiac, neurologic, or respiratory conditions, which could contribute to secondary illness or worsening symptoms.

Answer: A

After management of airway and breathing, circulation should be addressed in this case of potential toxic shock syndrome (TSS). Fluid bolus of 20 mL/kg can be repeated multiple times to manage blood pressure. Placement of a central line would be appropriate if fluid therapy was not sufficient. Antibiotics for the treatment of TSS include a second- or third-generation cephalosporin such as ceftriaxone and vancomycin.

Answer: C

Malaria is a parasitic illness which is transmitted through a mosquito bite in areas that are endemic. Plasmodium vivax and Plasmodium falciparum are the most common species that affect humans. P. falciparum is prevalent in Africa and Papua New Guinea. Symptoms of malaria include high fever, with chills, rigor, sweats, and headache and are cyclic in occurrence. Infection with P. falciparum is fatal and can manifest with multisystem involvement, including neurologic findings, renal failure, nephrotic syndrome, anemia, respiratory failure, and metabolic acidosis without pulmonary edema, among other significant problems. People traveling to these areas should receive prophylaxis prior to travel with pharmacologic agents based on potential exposure.

Answer: D

An 8-year-old with headache, vomiting, and visual changes is symptomatic for increased intracranial pressure, and a head CT is indicated prior to a lumbar puncture to evaluate for cerebral edema, space-occupying lesion, or hemorrhage.

Answer: A

Phenytoin is protein bound; therefore, if the albumin is low, the phenytoin level may not be correctly interpreted. A free-phenytoin level should also be obtained prior to administering another bolus of fosphenytoin.

Answer: C

Iron overload has been reported in patients following chemotherapy and hematopoietic stem cell transplant. A prolonged elevated ferritin level can lead to liver dysfunction, endocrine disorders, and altered cardiac function.