White or black cloud
Newborn child with ambiguous genitalia. One month later has vomiting, ↓Na, ↑K, and acidosis. Best lab test for diagnosis?
congenital adrenal hyperplasia. Most commonly due to 21 hydroxylase deficiency. Elevated 17 hydroxyprogesterone.
Newborn with Type 1 diabetic mother is cyanotic @ birth and does not improve with O2. CXR shows "egg on a string. What medicine to initiate?
Transposition of the great arteries. PGE1 to keep PDA patent until surgery.
9-year-old girl is seen for evaluation of abdominal pain and vomiting. Her symptoms began 5 months ago with onset of epigastric pain, for which she tried histamine-2 receptor antagonists for several weeks without improvement. She has lost 3 kg in 5 months. This week, she developed intermittent bilious emesis and has had an additional 2-kg weight loss. She has no other medical history and reports no medication use. She appears pale and thin (weight at the 10th percentile for age, height at the 50th percentile for age, and body mass index at the 5th percentile for age). Abdominal and rectal examination findings are normal, as is the remainder of the physical examination.
Of the following, the test MOST likely to establish the cause of the bilious emesis in this girl is
A.abdominal ultrasonography
B.hepatobiliary scan
C.magnetic resonance imaging of the brain
D.upper gastrointestinal series
D
The girl in this vignette has superior mesenteric artery (SMA) syndrome, a complication of significant weight loss. It occurs after loss of adipose tissue anterior to the duodenum. Normally, adipose tissue separates the SMA from the third portion of the duodenum; however, when the loss of this tissue occurs, the SMA compresses the duodenum, creating a partial or complete small bowel obstruction. Superior mesenteric artery syndrome is diagnosed with upper gastrointestinal series, demonstrating an obstruction in the third portion of the duodenum with contrast “to and fro” flow present.
What is malrotation and volvulus? Doesn’t rotate 270 ccw around SMA. Ladd’s bands can kink the duodenum.
An 8-year-old boy with a 3-day history of low-grade fever, sore throat, nasal discharge, cough, and malaise is seen in the office. He recently returned to school after summer break. He appears well and has a temperature of 38.2°C, a heart rate of 102 beats/min, a respiratory rate of 18 breaths/min, and a blood pressure of 108/67 mm Hg. There is redness of the pharyngeal mucosa without exudate. There is nontender, shotty, anterior cervical adenopathy. His right tympanic membrane is erythematous. The remainder of his physical examination findings are normal.
Of the following, the MOST likely etiology of this child’s illness is
A.Adenovirus
B.Influenza
C.Respiratory Syncytial Virus
D.Rhinovirus
D
Patient has clinical features that are consistent with the diagnosis of the common cold (or rhinosinusitis). Although all the community-acquired respiratory viruses noted in the responses can cause a common cold, rhinovirus is the most frequently implicated pathogen in children and adults, accounting for almost 50% of cases of upper respiratory tract infections. Rhinovirus infection occurs throughout the year, with peak activity from autumn through spring. There are many serotypes (>150) of rhinovirus and although immunity is type-specific, the duration of protection is temporary and short-lived. The serotypes of rhinovirus in any community vary from season to season. Children often experience at least two episodes of rhinovirus infections annually, and more than 90% of individuals have at least one rhinovirus infection annually. Transmission of rhinovirus infection occurs via person-to-person contact by respiratory tract secretions or large-particle aerosol spread.
Baby girl presents DOL 3-5 with bilateral purulent conjunctivitis. Mom had no prenatal labs done. Ddx?
gonococcal conjunctivitis
A 3 y/o child is brought in with petechiae, abdominal pain, vomiting and lethargy. He had bloody diarrhea 5 days ago after eating hamburgers at a family picinic. Labs reveal thrombocytopenia and ↑creatinine. Diagnosis? What do you NOT want to give?
Don’t give antibiotics!
A 16-year-old adolescent girl is evaluated in the office for left knee pain that started a couple of days ago. She has no history of trauma or known injury. The pain improves with rest and is exacerbated by walking. She had right knee pain a few days prior that improved with ibuprofen. She has otherwise been well aside from an urgent care visit for an upper respiratory infection with a sore throat a few weeks ago that has since resolved. On physical examination, her heart rate is 120 beats/min, respiratory rate is 15 breaths/min, blood pressure is 100/50 mm Hg, and oxygen saturation by pulse oximetry is 97% in room air. Her auscultatory examination demonstrates bilaterally clear breath sounds and a normal S1, physiologically split S2, with a 2/4 blowing diastolic murmur noted best at the second intercostal space with radiation to the right upper sternal border. This murmur has not been noted before. Her left knee is painful with range of motion, red, and slightly swollen. She has a pink rash on her back with a clear center that blanches on palpation. The girl is up to date on immunizations and has no allergies.
Of the following, after resolution of the acute symptoms, the BEST long-term medication to treat this adolescent’s condition is
A.intramuscular ceftriaxone monthly
B.intramuscular penicillin G monthly
C.oral amoxicillin daily
D.oral azithromycin daily
The adolescent in the vignette has polyarthritis, carditis, and a red rash with central clearing after a recent sore throat. She has a new murmur that is consistent with aortic regurgitation. Her history and physical examination findings are consistent with acute rheumatic fever (ARF) for which she should receive long-term treatment with intramuscular penicillin G. There is no role for ceftriaxone, amoxicillin, or azithromycin in the long-term chemoprophylaxis of rheumatic fever.
A 2-year-old boy is brought to the emergency department for evaluation of rectal bleeding. He has a 1-month history of intermittent rectal prolapse, which occurs every 2 to 3 days and is easily reducible at home, with no rectal bleeding noted. His first episode of prolapse was easily reduced by a physician in the emergency department. He is an otherwise healthy boy with normal growth, who is not yet toilet trained. He has 1 to 2 stools daily which his parents describe as soft blobs. He has never had a history of infrequent or hard stools. Because of his history of prolapse, the boy has been taking polyethylene glycol 3350 daily for the last month. Physical examination reveals a hemodynamically stable child. Digital rectal examination finds dark red blood and a palpable rectal mass; the remainder of his examination findings are normal. Laboratory evaluation reveals a normal hemoglobin level for age.
Of the following, the test MOST likely to confirm the etiology of this boy’s condition is
A.Colonic manometry
B.Colonoscopy
C.Meckel scan
D.Sweat chloride
B
The child in the vignette has recurrent rectal prolapse without a history of constipation or cystic fibrosis. Given the finding of a palpable rectal mass, the most likely because of his recurrent rectal prolapse and rectal bleeding would be a rectal polyp. This could be confirmed on colonoscopy.
A 4-year-old boy is brought for a health supervision visit. He has had a lifelong persistent cough and intermittent wheezing. He becomes short of breath and has increased cough when running, and cannot keep up with his peers while playing. He had surgical repair of a complete vascular ring at 2 years of age. There is a strong family history of asthma.
On physical examination, he looks well. Oxygen saturation on pulse oximetry is 99% on room air. Respiratory rate is 20 breaths/min. Heart sounds are normal, and breath sounds are clear to tidal breathing. He has monophonic expiratory wheezing that is heard best centrally and transmitted throughout the chest. There is no inspiratory stridor. There is no change after 4 puffs of an albuterol inhaler with a holding chamber and mask. The boy is diagnosed with tracheomalacia.
Of the following, the boy’s findings MOST consistent with this diagnosis include
A. exercise intolerance, family history of asthma, no response to albuterol
B.family history of asthma, history of vascular ring, normal oxygen saturation
C.history of vascular ring, central monophonic wheezing, exercise intolerance
D.no stridor, normal heart sounds, normal oxygen saturation
C. The boy in the vignette has tracheomalacia after the repair of a complete vascular ring. The history of a congenital anomaly producing compression of the trachea with either failure to develop ring cartilages or injury to them, plus the monophonic wheezing and exercise intolerance are characteristic findings of tracheomalacia. Although he has a family history of asthma and may be at risk for the development of asthma in the future, the monophonic wheezing is characteristic of a single point of obstruction of the airway, rather than the polyphonic wheezing with multiple sites of small airway obstruction heard in asthma. The failure to respond to albuterol also suggests that his airway obstruction is not that of the small, muscular airways. Because the problem is one of variable central airway obstruction, there is no small airway component and ventilation/perfusion mismatch, resulting in no effect on oxygen saturation.
A term male neonate born to a mother with limited prenatal care has a positive newborn screening result for IgM antibodies specific for Toxoplasma gondii. Further testing in conjunction with an infectious disease specialist confirms congenital toxoplasmosis. The original source of the infection is determined to be undercooked meat consumed by the boy’s mother and his 7-year-old sister. The sister has no medical problems and has remained well with no symptoms but is also found to have been infected with T gondii.
Of the following, the BEST advice regarding the management of the sister’s infection is that she should receive
A.prophylaxis if she becomes immunocompromised
B. reassurance that she does not require treatment
C. treatment if she becomes pregnant
D. treatment in conjunction with her brother
A.
She is currently healthy and asymptomatic but is infected with Toxoplasma gondii; this acute infection will likely become latent. If she later becomes immunocompromised, her latent infection may reactivate and cause severe symptoms. She should therefore receive prophylaxis if she becomes immunocompromised.
What are the subclassifications of Von Willebrand Disease , and which category presents with elevated PT and PTT?
1
2A
2B
2N
2M
3
Platelet-type vWD
Acquired vWD
33333
A 5-year-old boy is seen in the office for a health supervision visit. His past medical history is significant for an atrioventricular septal defect that was repaired at 6 months of age; there is residual left atrioventricular valve regurgitation that is being monitored by his pediatric cardiologist. His activity and diet have been normal. His mother has no concerns. The boy’s physical examination findings are normal aside from his murmur, which is unchanged from prior visits. At the most recent cardiologist visit, his heart function was depressed and the boy was started on a new medication; the mother cannot remember its name.
Of the following, the MOST likely medication prescribed for this child is
A.atenolol
B.carvedilol
C.enalapril
D.losartan
he boy in the vignette, who is asymptomatic with decreased systolic function, would be classified as being in stage B. Clinical guidelines recommend starting an angiotensin-converting enzyme (ACE) inhibitor, such as enalapril, for patients in stage B. While ꞵ-blockers (carvedilol) and angiotensin receptor blockers (losartan) have a place in heart failure management, they would not be the first-line agent in this situation. Atenolol is a ꞵ-blocker typically used for hypertension or arrhythmia management, but not heart failure specifically.
A 16-month-old boy is brought to the emergency department for trouble breathing. His mother states that he was well until approximately 48 hours ago when nasal congestion and wet cough began. On physical examination, the boy has a heart rate of 160 beats/min, respiratory rate of 60 breaths/min, oxygen saturation of 88% on room air, and blood pressure of 98/55 mm Hg. He is in moderate respiratory distress with intercostal and subcostal retractions. Lung examination reveals coarse rhonchi and scant wheezing with good aeration throughout. Although somnolent, the boy interacts with his mother appropriately and cries when a blood specimen is obtained via arterial puncture. The remainder of his physical examination findings are unremarkable. Arterial blood gas reveals a pH of 7.30, PCO2 of 55 mm Hg, and PO2 of 60 mm Hg.
Of the following, the BEST next management step for this boy is to initiate
A.bilevel positive airway pressure ventilation
B.intubation and mechanical ventilation
C.low-flow nasal cannula oxygen
D.non-rebreather face mask oxygen
A. The boy in the vignette has acute respiratory failure and is in immediate need of supplemental respiratory support. Given that the boy has evidence of hypoxia (low PO2 and low oxygen saturation) and hypercapnia (elevated PCO2 and respiratory acidosis), management must address both acute hypoxic and hypercapnic respiratory failure simultaneously. Noninvasive positive-pressure ventilation (NIPPV) for acute respiratory failure in children is associated with rapid clinical improvement and a decreased need for intubation. Thus, a trial of NIPPV with bilevel positive airway pressure (BiPAP) would be the next best step in management. A nasal or full face mask is used to administer BiPAP and allows for independent adjustment of inspiratory positive airway pressure (iPAP) and expiratory positive airway pressure (ePAP). Inspiratory positive airway pressure promotes lung inflation during inspiration, leading to alveolar recruitment, and ePAP prevents end-expiratory alveolar collapse with the application of continuous end-expiratory pressure.
A 4-month-old infant born with myelomeningocele is seen for a health supervision visit. The infant underwent an uncomplicated repair 3 days after birth. Brain magnetic resonance imaging in the neonatal period demonstrated a Chiari II malformation with accompanying ventriculomegaly. Ventricular size has been stable on serial head ultrasonography. Anticipatory guidance regarding symptoms caused by Chiari II malformation is provided.
Of the following, the sign or symptom MOST likely to occur with the condition identified on this child’s brain magnetic resonance imaging results is
A.Constipation
B.Scoliosis
C.Swallowing difficulties
D.Urinary retention
C
Among the response choices, the sign or symptom most likely to occur in a child with Chiari II malformation is swallowing difficulties.
Myelomenigocele is a complex multisystem congenital malformation due to incomplete closure of the posterior neural tube, resulting in an exposed meningeal sac that contains a portion of the spinal cord and nerves. This primary defect leads to secondary defects in brain development, including Chiari II malformation and hydrocephalus. Additional associated brain anomalies may include agenesis of the corpus callosum, polymicrogyria, heterotopia, or structural micro-anomalies that can be associated with later development of learning difficulties and attention deficit/hyperactivity disorder.
A recent study on the use of corticosteroids for the treatment of a newly described multisystem inflammatory syndrome in children revealed that corticosteroids may confer a statistically significant reduction in hospital length of stay. In addition, a mortality benefit was seen for patients receiving corticosteroids, but the authors noted that the study was not powered to detect a difference in mortality. The researchers wish to replicate the study with a specific focus on the question of mortality and plan to increase the sample size so that if present, a statistically significant difference in mortality can be detected between those receiving and not receiving corticosteroids.
Of the following, the CHANGE in the study design will result in an increase in
A.Effect size
B.Power
C.Random Error
D.Significance level
B.
The power of a study relates to the ability to correctly accept or reject the null hypothesis. To that end, a type I error occurs when the investigator rejects the null hypothesis when it is in fact true, and a type II error is failing to reject the null hypothesis (accepting the null hypothesis) when it is in fact false. The probability of a type I error is typically known as alpha (α), while the probability of a type II error is known as beta (β). The power of a study is defined as (1 – β). As the probability of committing a type II error decreases, the power of the study increases. The statistical power can be thought of as the probability of accepting an alternative hypothesis, when the alternative hypothesis is true. The higher the power, the less likely it is that the investigators will fail to detect a difference between the groups when a difference actually exists. A larger sample size will result in a larger power (a lower likelihood of failing to detect a difference between groups if one exists). None of the other choices are directly affected by the new sample size, however effect size, random error, and significance level may affect the sample size required in the new study.
In cardiac arrest, when do you give the Amiodarone? and what is the dose? and for how many doses?
For shockable rhythm (VF/pVT), after 3 shocks.
5mg/kg
Repeat up tp 3 total boluses.
A 2-year-old boy is admitted to the hospital with newly diagnosed high risk neuroblastoma. He has had a documented 2 kg weight loss over the last 2 months and has been refusing to eat for several weeks. On physical examination, his weight is now 10 kg. He has evident muscle wasting and weakness. His laboratory evaluation is remarkable for hypoproteinemia and hypoalbuminemia. Nasogastric tube feedings are initiated and he has tolerated a gradual increase in calories. Of the following, the BEST caloric goal for this patient is:
A. 800 kcal per day
B. 900 kcal per day
C. 1,000 kcal per day
D. 1,100 kcal per day
E. 1,500 kcal per day
E
The child in this vignette currently weighs 10 kg and has experienced a 2 kg weight loss. A 12 kg child requires approximately 1,100 kcal per day for normal growth and development. The presence of malignancy increases caloric needs. This child would require more calories per day to maintain growth and development than other 12 kg children without a malignancy. Of the choices given, the best caloric goal for the child in the vignette is 1,500 kcal per day.
Children with cancer are at risk for malnutrition and weight loss. Multiple interacting factors are responsible for the malnutrition seen in children with malignancy (Item C30).
Despite the nutritional challenges faced by children with cancer, there are several methods to supplement nutrition during treatment. Oral caloric supplements can be added to the food or drink patients are already consuming; enteral nutrition can be provided via nasogastric, gastric, or gastro-jejunal tube; or nutrition can be provided parenterally through total parenteral nutrition (TPN). Despite TPN’s ability to provide caloric supplementation, it has significant risks, including increased risk of infection and liver damage. Whenever possible, it is preferable to supplement caloric intake through enteral feeds. This allows for the administration of a balanced feeding formula providing adequate lipid, protein, and carbohydrate support, as well as permitting the child to eat as he wishes. For the child described in the vignette, nasogastric tube feeds providing more than the basic calories needed for growth and development would be most appropriate.
A 12-year-old girl has had a chronic dry cough for 6 months. She also notes recent onset of shortness of breath with exercise. A therapeutic trial of inhaled albuterol has not changed her cough or exercise symptoms. Spirometry shows forced vital capacity (FVC) to be 79% of that predicted for age and height, forced expiratory volume in 1 second (FEV1) 85% predicted, and the FEV1/FVC ratio 0.94. Restrictive lung disease is suspected.
Of the following, the BEST next study to confirm the suspected diagnosis for this girl is
A.carbon monoxide diffusing capacity
B.fraction of exhaled nitric Oxide
C.lung volumes with body plethysmography
D.methacholine inhalation challenge test
C. he girl in the vignette has a mild restrictive pulmonary process with decreased forced vital capacity (FVC) but normal forced expiratory volume in 1 second (FEV1) and FEV1/FVC ratio. Measurement of total lung capacity (TLC) will confirm a restrictive process. Lung volumes can be measured with body plethysmography (body box) or nitrogen or helium dilution techniques.
Carbon monoxide diffusing capacity represents the ease with which gases are transported across alveolar membranes. It is reduced in diseases involving inflammation or thickening of the alveolar membrane, including sickle cell disease, radiation therapy and cancer chemotherapy, and collagen vascular diseases, and also is reduced in young adults with a history of prematurity.
The fraction of exhaled nitric oxide (FeNO) is a measure of eosinophilic airway inflammation.
What are the components of Treacher Collins Syndrome?
ØCraniofacial abnormalities
ØAirway abnormalities
ØOphthalmic abnormalities (defect in the eye lid)
ØDental abnormalities
ØEar anomalies
-Renal anomalies
Give me the 7S's for the innocent murmur.
Sensitive (with position and respiration)
Short duration (not holosystolic)
Single (no gallops or clicks)
Small (non-radiating)
Soft (low amplitude)
Sweet (not harsh)
Systolic
Dose of defibrillator?
Dose of Synchronized cardioversion?
Dose of Adenosine?
PALS
3 day old newborn has still not passed meconium. What are you worried about? Differential? How to treat?
What is meconium ileus? Dx and tx with gastrograffin enema. Get family history for CF. What is Hirschsprung’s disease? DRE --> explosion of poo. Gold standard = bx showing no ganglia in distal colon due to failure of neural crest cells to migrate properly.
rrived at the emergency department in active labor. The neonate’s Apgar scores were 5 and 8 at 1 at 5 minutes, respectively. His birth weight was 2,950 g. After delivery, the neonate developed tachycardia, tachypnea, nasal flaring, and intercostal retraction requiring intubation and mechanical ventilation.
On physical examination, the neonate is afebrile. He has diminished breath sounds in the lateral and bases of his lung fields with fair aeration centrally and no adventitious breath sounds. The remainder of his physical examination findings are unremarkable.
A point-of-care blood glucose level is 75 mg/dL (4.2 mmol/L). Chest radiography shows bilateral pleural effusions; the left is greater than the right. Echocardiography reveals normal cardiac anatomy and function. Chest ultrasonography demonstrates nonloculated pleural effusions bilaterally.
Diagnostic and therapeutic thoracentesis is performed. Analysis of the pleural fluid reveals the following:
Pleural Fluid Test
Result
Appearance
Thin, pale, milky
Pleural:serum protein ratio
0.8
Pleural:serum lactic dehydrogenase ratio
0.7
White blood cells
12,000/μL (12 × 109/L), 85% lymphocytes
pH
7.1
Glucose
70 mg/dL (3.9 mmol/L)
Of the following, the MOST likely cause of this neonate’s pleural effusion is
A.decreased colloid oncotic pressure
B.decreased pulmonary lymphatic drainage
C.increased pulmonary capillary permeability
D.increased pulmonary vascular hydrostatic pressure
B. The neonate in the vignette had respiratory distress and abnormal lung examination and chest radiography findings. He was evaluated for cardiac and pulmonary parenchymal sources of his chest opacification and was found to have only an exudative effusion. His likely diagnosis is congenital chylothorax, the most common cause of neonatal pleural effusion. Chylothorax results from interruption of pleural lymphatic drainage and is characterized by an exudative effusion with high levels of chylomicrons, triglycerides, and lymphocytes (>80% of the total white blood cell [WBC] count in the pleural fluid). Chylous effusions typically appear thin and milky, as opposed to empyemas, which appear cloudy. Triglycerides and chylomicrons in the fluid are the source of the characteristic milky appearance. Chylous effusions may be caused by thoracic duct trauma (eg, during surgery), thoracic duct obstruction, or lymphangiectasia, or may be idiopathic. Most cases of congenital chylothorax are idiopathic.
A newborn is seen for a first health supervision visit. There is a family history of retinoblastoma, and his mother carries one wild-type RB1 allele and one mutated RB1 allele. Of the following, this newborn’s risk for developing retinoblastoma is:
•A. between 25% and 50%
•B. between 75% and 100%
•C. dependent on the father’s genotype
•D. no different than the general population
A
The mother of the neonate in this vignette is a carrier of an abnormal RB1 allele. There is therefore a 50% chance that the neonate inherited this abnormal allele. As germline mutations are approximately 90% penetrant, his chance of developing retinoblastoma is calculated as: (chance of inheriting the abnormal allele) × (penetrance)
= 0.5 × 0.9
= 0.45
Therefore, the chance that this neonate will develop retinoblastoma is less than 50% but more than 25%. If it was certain that the neonate had inherited the abnormal RB1 allele from his mother, then his chances of developing retinoblastoma would be 75% to 100%. However, it is not know if he inherited the mutated gene. Retinoblastoma as a whole is very rare with a prevalence of 11 cases per million children under 5 years, and 80% of heritable retinoblastoma is caused by de novo mutations; therefore, it is reasonable to assume that the father does not carry a germline RB1mutation.