Pediatrics/Clinical_COMLEX_Level_2/Lecturio

Over the course of a year, five children with similar congenital heart defects were referred to a pediatric cardiac surgeon for evaluation. All five children had stable vital signs and were on appropriate medication. Upon reviewing their medical records, the children were noted to have a loud holosystolic murmur over the third intercostal space at the left sternal border. The surgeon ordered echocardiograms for all five children and recommended surgical closure of the defect in one of them. Which of the following patients required surgical repair of the defect?

A 9-month-old boy is brought to the pediatrician for evaluation of blue discoloration of the fingernails. His parents recently immigrated from Venezuela. No prior medical records are available. His mother states that during breastfeeding, he sweats, and his lips turn blue. Recently, he has begun to crawl, and she has noticed a blue discoloration in his fingers. Temperature is 37°C (98.6°F), heart rate is 100/min, respiratory rate is 26/min, and blood pressure is 90/60 mm Hg. On examination, he appears to be in mild distress and cyanotic. Both fontanelles are soft and non-depressed. There are normal breath sounds and a grade 2/6 systolic ejection murmur at the left upper sternal border with a single S2. He is placed in the knee-chest position. This maneuver attempts to improve this patient's condition by which of the following mechanisms?

A 9-year-old boy is brought to the physician by his father, who is concerned because his son has been less interested in playing soccer with him recently. The father and son used to play every weekend, but the son now tires easily and complains of pain in his lower legs while running around on the soccer field. The patient has no personal or family history of serious illness. A cardiac examination reveals a systolic ejection murmur best heard over the left sternal border that radiates to the left paravertebral region. The fermoral pulse is delayed after the radial pulse when both are palpated simultaneously. A chest X-ray shows erosions on the posterior aspects of the 6th to 8th ribs. If left untreated, this patient is at the greatest risk for which of the following?

A 23-year-old woman presents to her provider for an antenatal appointment. She is at the 24th week of gestation. She has had type 1 diabetes mellitus for five years and is on insulin. She also has mitral stenosis, for which she has been advised to undergo a balloon valvotomy. Her temperature is 37.1°C (98.7°F), blood pressure is 120/60 mm Hg, and pulse is 90/minute. Her random blood glucose is 220 mg/dL and HbA1C is 8.5%. She wants to discuss possible complications concerning her pregnancy. Which of the following cardiac complications is her infant at high risk for?

An 8-year-old girl is brought to the emergency department by her parents because she complained of very fast heartbeats. The patient has previously been healthy without any childhood illnesses and has not needed to go to a provider besides well-visits in the past two years. On examination, the pulse is 198/min. Further examination by the provider reveals a systolic murmur over the anterior chest wall. ECG is immediately performed after her heart rate is reduced, and shows a short P-R interval with a slow upstroke of the QRS complex. Which of the following would most likely be found in this patient?

A 5-year-old girl is brought to her pediatrician for vaccinations and a physical examination. She is a generally healthy child, but she has no thumb on her right hand and she has a shortened and deformed left thumb. She was bom at 39 weeks' gestation via spontaneous vaginal delivery and is up to date on all vaccines and is meeting all developmental milestones. On physical examination, her vital signs are stable. On auscultation of the heart, the pediatrician notes a wide fixed splitting of the second heart sound and a medium-pitched systolic ejection murmur at the left sternal border. The murmur is not harsh in quality and is not accompanied by a thrill. Her echocardiogram confirms the diagnosis of an acyanotic congenital heart defect with a left-to-right shunt. Which of the following genetic syndromes is most consistent with this girl's congenital defects?

A 12-year-old girl presents to a pediatrician because she fails to show signs of breast development and hasn't started menstruating yet. The pediatrician notes that her stature is shorter than expected for her age and sex on physical examination. She has a webbed neck, a low posterior hairline, and a broad chest with widely spaced nipples. Non-pitting bilateral pedal edema is present. The pediatrician orders a karyotype analysis, which is shown below. Which of the following findings is most likely on chost auscultation, correlating to one of the potential cardiac malformations seen with her diagnosis?

A 2-hour-old newborn boy is admitted to the emergency room because of progressively worsening cyanosis. His mother had very poor follow-up during the prenatal period, but his prenatal ultrasound was within normal limits. The patient's temperature is 36°C (96.8°F), heart rate is 120/min, respiratory rate is 36/min, blood pressure is 88/55 mm Hg, and oxygen saturation is 82% on room air. On physical examination, the patient is severely cyanotic with nasal flaring and difficulty breathing. No significant heart murmur is noted. Chest X-ray shows cardiomegaly with an "egg-on-a-string" appearance. What is the most likely cause of this congenital heart defect?

A 1-week-old infant is brought to the pediatric clinic with concerns of poor feeding, slow weight gain, and noticeable cyanosis since birth. On physical examination, the infant's temperature is 36.9°C (98.4°F), pulse is 160/min, and respirations are 55/min. The baby appears irritable, with mild cyanosis around the lips and nail beds. There is a single loud heart sound on auscultation. chest X-ray shows an enlarged heart with increased pulmonary vascular markings. An echocardiogram reveals a single arterial trunk arising from both ventricles, with no clear separation between the aorta and pulmonary artery, Additionally, there is a large ventricular septal defect (VSD), and the arterial trunk overrides this defect. What congenital defect is associated with cyanosis but not dependent upon a patent ductus arteriosus?

A newborn is diagnosed with a large membranous ventricular septal defect. The child has poor weight gain and feeding difficulties. He requires furosemide and spironolactone to prevent dyspnea. On physical examination, his temperature is 36.9°C (98.4"F), pulse is 158/min, respirations are 30/min, and blood pressure is 94/62 mm Hg. Chest auscultation reveals a holosystolic murmur along the left lower sternal border and a mid-diastolic low-pitched rumble at the apex. Abdominal examination reveals hepatomegaly. Which of the following is the best management option for this patient?

A 16-year-old male adolescent presents to his healthcare provider with increasing fatigue and breathlessness with exercise. His parents inform the provider that they have recently migrated from a developing country, where he was diagnosed with a large ventricular septal defect (VSD). However, due to their poor economic condition and scarce medical facilities, surgical repair was not performed in that country. The provider explains to the patient’s parents that patients with large VSDs are at increased risk for several complications, including Eisenmenger syndrome. If the patient has developed this complication, he would not be a good candidate for surgical closure of the defect. Which of the following clinical signs, if present on physical examination, would mostly strongly suggest the presence of this complication?

A 5-day-old boy is brought to the emergency department because of altered mental status. After finding him grey and unarousable in his crib, his mother called an ambulance. The patient was born via cesarean section due to preterm premature rupture of membranes (PPROM). Since birth, the infant has gained little weight and has been generally fussy. His temperature is 37.0°C (98.6°F), pulse is 180/min, respirations are 80/min, blood pressure is 50/30 mm Hg, and oxygen saturation is 80% on room air. Physical examination shows a mottled, cyanotic infant who is unresponsive to stimulation. Cardiopulmonary examination shows prominent heart sounds, wet rales in the inferior lungs bilaterally, strong brachial pulses, and absent femoral pulses. Endotracheal intubation is performed immediately and successfully. Which of the following signs would a chest X-ray likely show?

A 7-year-old boy is brought by his parents to his pediatrician due to progressive fatigue and shortness of breath while playing sports. He is otherwise healthy with no known medical disorders and no other symptoms. The boy was born at 39 weeks' gestation via spontaneous vaginal delivery. He is up to date on all vaccines and is meeting all developmental milestones. On physical examination, his temperature is 36.9ºC (98.4ºF), pulse is 90/min, blood pressure is 100/70 mm Hg, and respirations are 18/min. Pulses in all four extremities are equal and normally palpated; there is no radio-femoral delay. Normal S1 with a fixed split S2 is heard on exam. The pediatrician suspects a congenital heart disease after auscultation of the heart. Which of the following congenital heart diseases is most likely to be present with the clinical features listed above?

A 10-day-old infant born at full term via uncomplicated spontaneous vaginal delivery is noted to have cyanosis of the oral mucosa. The baby appears comfortable otherwise. His respirations are 70/min, and pulse oximetry is 80% on the examination. A right ventricular lift is palpated, S1 is normal, S2 is single, and a harsh 3/6 systolic ejection murmur is heard at the left upper sternal border. Which of the following is the most likely diagnosis in this patient?

A 24-hour-old girl is found to be cyanotic in the newborn nursery. She was born via spontaneous vaginal delivery at 38 weeks’ gestation to a gravida 1, para 0 healthy mother who received routine prenatal care. The patient is small for her gestational age. She has lower-extremity cyanosis along with a mesh-like mass on the back of her neck. Her pulse is 150/min, and respirations are 48/min. Blood pressure is 120/80 mm Hg in the right arm, 124/82 mm Hg in the left arm, 80/40 mm Hg in the right leg, and 85/45 mm Hg in the left leg. Femoral pulses are 1+ and delayed. A cardiac examination shows a continuous murmur in the interscapular area. Auscultation of the lung reveals faint crackles at the base of the lung fields bilaterally. Which of the following is the most appropriate next step in management?

A newborn boy, delivered by emergency Cesarean section during the 28th week of gestation, has a birth weight of 1.2 kg (2.5 lb). He develops rapid breathing four hours after birth. Examination of the respiratory system reveals a respiratory rate of 80/min, expiratory grunting, and intercostal and subcostal retractions with nasal flaring. A chest radiograph shows bilateral diffuse reticulogranular opacities and poor lung expansion. Echocardiography suggests a diagnosis of patent ductus arteriosus with left-to-right shunt and signs of fluid overload. The pediatrician administers intravenous indomethacin to facilitate the closure of the duct. Which of the following effects best explains the mechanism of action of this drug in the management of this neonate?

A 6-month-old infant is brought to a pediatrician for his scheduled immunizations. The parents have no specific current complaints, but his facial features are different from those of his siblings. During the physical examination, the pediatrician notes that the infant’s vital signs are stable. His facial features include a medial epicanthic fold, a face that appears flat, and a flat occiput with low-set ears. The pediatrician also notes a single transverse palmar crease on both hands. An echocardiogram is performed. This suggests that the infant has the most common form of heart disease seen in children with this particular genetic disorder. Which of the following congenital heart diseases is this infant most likely presenting with?

A 15-year-old boy presents to his pediatrician with worsening fatigue and exercise intolerance over the last several months. Per his mother, who accompanies him, he was born at 39 weeks’ gestation via spontaneous vaginal delivery. He is up to date on all vaccines and met all developmental milestones on time. The patient denies palpitations, shortness of breath, or leg swelling. On physical examination, his vital signs are within the normal ranges. On cardiac exam, a right ventricular heave is noted. There is a wide fixed split in the second heart sound during inspiration and expiration and a systolic flow murmur in the pulmonary valve area. Which of the following findings would most likely be found on his echocardiogram?

A 6-week-old girl is brought to a pediatrician due to feeding difficulty for the last four days. Her mother mentions that the infant breathes rapidly and sweats profusely while nursing. She has been drinking very little breast milk and stops feeding as if she is tired, only to start sucking again after a few minutes. There is no history of cough, sneezing, nasal congestion, or fever. She was born at full term, and her birth weight was 3.2 kg (7.0 lb). Her temperature is 37.0°C (98.6°F), pulse is 190/min, and respirations are 64/min. On chest auscultation, bilateral wheezing is present. A precordial murmur starts immediately after the onset of the first heart sound (S1), reaching its maximal intensity at the end of systole and waning during late diastole. The murmur is best heard over the second left intercostal space and radiates to the left clavicle. The first heart sound (S1) is normal, while the murmur obscures the second heart sound (S2). Which of the following is the most likely diagnosis?

An 18-month-old boy is brought to the emergency department after losing consciousness. His mother states that he was running with other kids in the park when he suddenly fell and became unresponsive for less than one minute. He has not had any immunizations due to their religious beliefs. The parents report that he plays with other children but tires easily. He has had difficulty feeding, but there was no follow-up with a pediatrician. His height is in the 40th percentile, and his weight is in the 50th percentile. Heart rate is 120/min, and oxygen saturation is 91% on room air. The boy is crying and has perioral cyanosis. The lungs are clear. S1 is normal, and there is a single S2 sound without a split. A grade 2/6 systolic ejection murmur is appreciated at the left upper sternal border. When the child squats, the murmur intensifies, and the cyanosis improves. What is the most appropriate next step in the management of this patient?

One day after doctors helped a 28-year-old primigravid woman deliver a 3,700 g (8 lb 3 oz) boy, the newborn had bluish discoloration of the lips and fingernails. His temperature is 37.3°C (99.1°F), pulse is 166/min, respirations are 63/min, and blood pressure is 68/44 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 81%. Examination shows central cyanosis. A grade 2/6 holosystolic murmur is heard over the left lower sternal border. A single second heart sound is present. Supplemental oxygen does not improve cyanosis. An X-ray of the chest shows an enlarged cardiac silhouette with a narrowed mediastinum. Which of the following cardiac defects would be associated with this newborn’s diagnosis?

A male neonate is being examined by a healthcare provider. His mother informs the provider that she was not immunized as a child and was ill with a fever, rash, joint pain, and swollen lymph nodes in her neck when she was 2 to 3 months pregnant. She did not have any prenatal care, and the boy was born at 39 weeks' gestation via spontaneous vaginal delivery. On physical examination, the neonate has normal vital signs. Retinal examination reveals the findings shown in the image. Which of the following congenital heart defects is most likely in this neonate?

A 5-year-old boy presents to his pediatrician for a well-child visit. His mother reports that he is doing well and that she has no concerns. The boy was born at 39 weeks' gestation via spontaneous vaginal delivery. He is up to date on all vaccines and is meeting all developmental milestones. On physical examination, the patient is noted to have a blood pressure of 150/80 mm Hg, measured in both arms. In addition, the radial pulses are 2+ but the femoral pulses are barely palpable. Cardiac auscultation reveals a regular heart rate and rhythm with normal S1 and S2. A 2/6 long systolic murmur with a systolic ejection click is heard over the left sternal border and the back. The point of maximal impact is normal. Which of the following is the most likely diagnosis in this patient?

A 28-year-old woman gives birth to a male infant. During her third-trimester antenatal sonogram, the radiologist noted a suspected congenital heart defect but the exact nature of the defect was not clear. The pediatrician orders an echocardiogram after making sure that the baby’s vital signs are stable, which reveals the following findings: atresia of the muscular tricuspid valve, pulmonary outflow tract obstruction (POTO), patent ductus arteriosus (PDA), a small ventricular septal defect (VSD), and transposition of the great arteries (TGA). The pediatrician explains the nature of the congenital heart defects to the infant's parents. He also informs them about the probable clinical features that are likely to develop in the infant, the proposed management plan, and the prognosis. Which of the following signs is most likely to manifest first in this infant?

A 3-week-old boy is brought by his mother to the pediatrician because of poor feeding, poor weight gain, and he appears to have rapid breathing. There was no prenatal ultrasound done and he born at home with a midwife. He is in the 10th percentile for weight. His heart rate is 160/min, and respiratory rate is 60/min. Cardiac auscultation reveals clear lungs and a grade 2/6 holosystolic murmur that is loudest at the left lower sternal border. The physical examination is negative for clubbing, cyanosis, and peripheral edema. What is the most likely diagnosis in this patient?

A two-hour-old neonate is found to have a bluish discoloration throughout his body, including his lips and tongue. The boy was born at 39 weeks gestation via spontaneous vaginal delivery with no prenatal care. Maternal history is positive for an 11-year history of type 2 diabetes mellitus. On physical examination, his blood pressure is 55/33 mm Hg, pulse is 170/min, respirations are 50/min, temperature is 37°C (98.6°F), and oxygen saturation is 84% on room air. Auscultation of the chest reveals a single second heart sound without a murmur. The chest x-ray image is shown below. Which of the following is the most likely diagnosis in this patient?

A one-day-old infant born at full term via uncomplicated cesarean section delivery is noted to have a murmur, although the patient appears well otherwise. The infant's respirations are 40/min, and the pulse oximetry is 96% on room air. The precordium is normoactive. With auscultation, S1 is normal, S2 is single, and a 2/6 systolic ejection murmur is heard at the left upper sternal border. Echocardiography shows infundibular pulmonary stenosis, an overriding aorta, a ventricular septal defect, and concentric right ventricular hypertrophy. Which of the following correlates with the presence or absence of cyanosis in this baby?

A 9-month-old infant is brought to the pediatrician for immunizations and assessment. His parents report that he is eating well and produces several wet diapers every day. He is a happy and curious child. The boy was born at 39 weeks' gestation via spontaneous vaginal delivery. He is up to date on all vaccines and is meeting all developmental milestones. The infant’s vital signs are normal. His physical growth is appropriate for his age. The physician notes a loud holosystolic murmur at the left sternal border (grade IV) and orders an echocardiogram, which confirms the diagnosis of a congenital heart defect. Based on echocardiogram findings, the pediatrician reassures the parents that the infant will be monitored, but will most likely not require surgical intervention. Which of the following is the most common congenital cardiac anomaly in children?

A four-week-old girl develops poor feeding and respiratory distress while in the NICU. She was born at 29 weeks’ gestation via cesarean section due to reduced movement and a non-reassuring fetal heart tracing. APGAR scores were 6 and 8 at one and five minutes, respectively. The vital signs today include:

A 15-month-old boy is brought to the pediatrician for immunizations and assessment. His parents report that he is eating well and produces several wet diapers every day. He is occasionally fussy but is overall a happy and curious child. The boy was born at 39 weeks' gestation via spontaneous vaginal delivery. On physical examination, his vital signs are stable. His weight and height are above the 85th percentile for age and sex. On chest auscultation, the pediatrician detects a loud, harsh holosystolic murmur over the left lower sternal border. The first and second heart sounds are normal. An echocardiogram confirms the diagnosis of a muscular ventricular septal defect without pulmonary hypertension. Which of the following is the best management strategy for this patient?

A newborn is brought to the pediatric clinic by his mother because she has noticed a swelling in the belly while dressing her baby. On physical examination, the newborn is found to have a non-tender upper abdominal mass. The clinician also noticed absent irises and undescended testes in this baby. A magnetic resonance image (MRI) scan of the abdomen shows a mass of intra-renal origin. Which one of the following genetic disorders is most probably the cause of this neonate's symptoms and signs?

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A 10-year-old girl with a rash is brought to the clinic by her mother. The patient's mother says that the onset of the rash occurred two days ago. The rash is pruritic, red, and initially it was localized to the cheeks with circumoral pallor, and it has gradually spread to the arms and trunk. The patient's mother also says her daughter had been reporting a high fever to 39.4 ℃ (102.9 ℉) headaches, myalgia, and flu-like symptoms about a week ago, which resolved in two days with acetaminophen. The patient has no significant past medical history. Her temperature is 37 ℃ (98.6 ℉) pulse is 90/min, blood pressure is 105/85 mm Hg, and respirations are 20/min. Physical examination shows a symmetric erythematous maculopapular rash on both cheeks with circumoral pallor, which extended to the patient's trunk, arms, and buttocks on the day of presentation. Laboratory findings are significant for a leukocyte count of 7, 100/mm³ and platelet count of 325000/mm³ Which of the following is the next best step in the management of this patient?

A 15-year-old boy presents with a sore throat and difficulty swallowing. He has had a sore throat for the last three weeks. Two days ago, his sore throat became acutely more painful. He has no significant past medical history and takes no medications. His temperature is 38.2°C (100.8°F), blood pressure is 100/70 mm Hg, pulse is 101/min, respirations are 26/min, and oxygen saturation is 99% on room air. The patient cannot fully open his mouth, and excessive drooling is noted. His voice has a muffled quality. CT of the head is significant for the findings shown in the image. Which of the following is the best initial treatment for this patient?

An 18-month-old girl is brought to the emergency department because of a cough her parents are worried about. She has had a runny nose and a low-grade fever for the past two days, with some hoarseness and a rough-sounding cough that started this afternoon. This evening she began making high-pitched sounds when taking breaths, and she seemed to be having trouble breathing. She is alert and does not appear to be in acute distress. Her temperature is 38.0°C (100.4 "F), respirations are 18/min, and O, saturation is 97%. There is audible inspiratory stridor that worsens when she starts to cry during the examination, and mild to moderate retractions. She has an occasional barking cough. Her pharynx is mildly erythematous with normal tonsils and no exudate. Which of the following is the next best step in management?

A 16-year-old boy presents to the clinic with his mother with complaints of fever, fatigue, lack of appetite, and sore throat for the past four days. Several other students at his high school have had similar symptoms. A physical examination shows a whitish membrane in his oropharynx, bilateral enlarged cervical lymphadenopathy, and mild splenomegaly. Which of the following studies is most likely to diagnose his condition?

A 10-year-old girl who did not receive the usual childhood vaccines presents to her pediatrician because of a rash. Her mother reports that she has had a fever, runny nose, and headache for the last four days. The girl appears sick but not toxic on physical examination. Her heart has a regular rate without murmurs, her lungs are clear to auscultation bilaterally, and her abdomen is soft without hepatosplenomegaly. She has red checks with circumoral palior and no other skin findings. Which of the following is true regarding the child's most likely illness?

A 3-year-old boy with no significant past medical or family history is brought to his pediatrician for fever associated with diarrhea and conjunctivitis over the past week. His parents state he has never had an episode of diarrhea like this before, but several other children at his daycare have been ill. His blood pressure is 97/70 mm Hg, pulse is 94/min, respirations are 14/min, and temperature is 37.0°C (98.6°F). His physical exam is significant for preauricular adenopathy Which of the following interventions would have been most effective in preventing this condition?

A 9-year-old girl presents with a 16-day history of cough. Her mother reports that initially, she had a runny nose and was tired, with a slight cough, but as the runny nose resolved, the cough seemed to get worse. Her little brother is currently asymptomatic. She further states that the cough is dry-sounding and occurs during the day and night. She describes having coughing spasms that occasionally end in vomiting, but between episodes of coughing, she is fine. She reports that during a coughing spasm, her daughter will gasp for air and sometimes make a "whooping" noise. A nasopharyngeal swab confirms a diagnosis of Bordetella pertussis. Which of the following statements apply to this patient?

A 18-year-old woman presents with a sore throat. The patient says that symptoms started acutely three days ago and have progressively worsened. She denies any history of cough, nasal congestion, or rhinorrhea. No significant past medical history is noted and she takes no current medications. Her temperature is 38.0°C (100.4°F), blood pressure is 110/70 mm Hg. pulse is 74/min, respirations are 16/min, and oxygen saturation is 99% on room air. Physical examination is significant for anterior cervical lymphadenopathy. There is edema of the oropharynx and tonsillar swelling but no tonsillar exudate. Which of the following is the most appropriate next step in the management of this patient?

A neonate born at 33 weeks is transferred to the NICU after a complicated pregnancy and C-section. A week after being admitted, he developed a fever and became lethargic and minimally responsive to stimuli. A lumbar puncture is performed that reveals the following:

A 12-year-old boy presents with complaints of a swollen face. The patient says that symptoms started acutely 3 days ago and have not improved. His past medical history is significant for a self-limiting episode of pharyngitis 3 weeks ago. There is no other significant past medical history or current medications. His vital signs include temperature 37.0°C (98.6°F), blood pressure 160/100 mm Hg, pulse 74/min, respiratory rate 22/min, and oxygen saturation 99% on room air. Physical examination is significant for a 2+ pitting edema of the face and the bilateral lower extremities. Results of a CMP, CBC, and UA show:

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