infection
smooth brain
orthopedics
name the cause or disease!
dx & tx
100

newborn with "sniffles/snuffles" 

what is syphillis?

100

Given the following clinical descriptor, what is the most likely diagnosis?

-6 mo M comes for his routine Peds visit. His weight at birth was 5 Lbs. His current weight is 9 Lbs. His first bowel movement was at 47 hrs after birth.

-He has experienced 3 bouts of PNA since birth and has foul smelling, greasy stools.

-He was recently treated for rectal prolapse.


CF

dx: sweat test

tx: pul therapy, NAC, aminoglycoside, give pancreatic enzymes, fat soluble enzymes

They might say on PE "digital clubbing and recurrent nasal polyps which have not been well controlled with intranasal steroids."

100

4 yo F is brought to the ED by concerned parents. She is completely fine during the day but cries to sleep at night from bilateral calf pain. Her PE is completely normal. She runs around and plays with her friends during the day.



Growing pains. The pain classically shows up at night and disappears during the day. Consider an Osteoid Osteoma as the dx if your exam describes a kid with localized bone pain that is worse at night and relieved with analgesics. The PE is typically +ve for a bone mass.


100

5 yo F is brought to the pediatrician by her mom with complaints of sore throat and “watery eyes” that started over 3 days ago. PE is notable for severe conjunctival erythema and hyperemia.


adenovirus

100


Given the following clinical presentation, what is the most likely dx?
-Recent history of a febrile adenovirus infection.
-Home aspirin was used to treat the fevers.
-The infant presents today with copious vomiting. He ultimately becomes comatose. -Blood glucose is 45 g/dl.


This is Reye Syndrome. Avoid aspirin as tx with febrile viral infections in kids. The liver and brain stop working (hypoglycemia, confusion/coma). 

**Remember the exception of aspirin with Kawasaki’s disease.


200

how would you diagnose CMV?

urine viral cultrue 

200

name as many Down syndrome associations that you can

hirschsprungs, AV cushion defect, VSD, ASD, TOF, Duodenal atresia, congenital umbilical hernia, annular pancreas, ALL, atlantoaxial instability, early onset Alzheimers

200

7 yo M recently had an URI. He presents with a painful limp that began yesterday. WBC is wnl. ESR is marginally elevated. There is no evidence of joint erythema or effusion on PE


This is Transient Hip Synovitis. Give NSAIDs. If there is any mention of “joint findings” on the exam, get an arthrocentesis to r/o bad stuff.


200

A 3 mo presents with circumoral pallor and cyanosis. CBC is notable for severe anemia. The child has a hx of thumb and craniofacial anomalies


This is Diamond Blackfan anemia. Note the age (< 1 yo) and associated anomalies. Contrast with Fanconi anemia (chromosomal breakage) where other “blood” cell lines could be down with the addition of cafe au lait spots.


200

Given the following clinical descriptions, what is the most likely diagnosis?

-4 year old F presents with fever and pain with urination. This is her 2nd UTI in the past 6 mo. PE is notable for CVA tenderness and a palpable LUQ mass. Urine cx obtained through a suprapubic cath (or midstream clean catch) grows 120k colonies of gram -ve lactose fermenting rods.

Newborn with fullness on suprapubic palpation. Gestational hx is notable for oligohydramnios.


This is pyelonephritis more than likely from vesicoureteral reflux. For a young kid (F) with this many UTIs (commonly E. Coli), consider getting a voiding cystourethrogram to r/o anatomic causes of recurrent urinary infections. Tx with Ceftriaxone or TMP-SMX.

On your exam, certainly get a VCUG if you get a Q about any young male infant/female < 5 yo with a UTI (this should never happen). To reiterate, a urine culture is the gold standard for UTI dx.


300

A child presents with high fever for 4 days, followed by abrupt resolution of fever and onset of a maculopapular rashstarting on the trunk.

Which virus is the cause?

HHV6 Roseola

300

A 7-year-old boy presents with recurrent respiratory infections, eczema, and thrombocytopenia.

Which immunologic abnormality is most likely present?

Elevated IgE and IgA

(wiskott)

300


Given the following injury scenario related to osteomyelitis, what is the most likely bug?

Most common overall cause of osteomyelitis


Osteomyelitis in a 16 d old neonate


2 yo M w/left ankle osteomyelitis. Recent history of stepping on a “sharp substance” 

5 yo M w/right knee osteomyelitis. Spleen is not visible on abdominal imaging.



Most common overall cause of osteomyelitis-S. Aureus. 

Osteomyelitis in a 16 d old neonate-S. Agalactiae (GBS).

2 yo M w/left ankle osteomyelitis. Recent history of stepping on a “sharp substance”-P. Aeruginosa (remember association with footwear).

5 yo M w/right knee osteomyelitis. Spleen is not visible on abdominal imaging-Salmonella (remember the association with sickle cell disease).

On the exam, always get an arthrocentesis if you see signs of inflammation at a joint. If an XR is -ve and you suspect osteomyelitis, proceed to an MRI. Get cultures as well.


300

A newborn with multiple facial defects and hypocalcemic seizures is transported to the Med19 NICU for persistent cyanosis. Cardiac auscultation is notable for a single S2 heart sound


This is Truncus Arteriosus. Arises from a failure of the aorticopulmonary septum to separate the truncus arteriosus into the pulmonary artery and aorta. Very common in DiGeorge Syndrome. Pulmonary vascular markings will be increased.


300

kid with cherry red skin after falling asleep in basement with wonky space heater

CO poisoning 

TX with 100% O2

400

Several children at a daycare develop bloody diarrhea. One child later develops anemia, thrombocytopenia, and AKI.

What is the most important management principle?

avoid antibiotics!!!!


400

A toddler presents with bowing of the legs, widened wrists, and delayed closure of the fontanelles.

Which lab abnormality is most likely?

High ALP

(rickets)

400

A 2-week-old newborn has a positive Ortolani maneuver on exam.

What is the next step in management?

Pavlik harness

what if they are 6-18 months?

what if they are 18months+?

400


An 18 mo infant is brought to a pediatrician by his mom after she noticed a bulge in his abdomen when lifting him up from a crib. The presence of a left, infraumbilical mass is confirmed on a physical exam. Vital signs are notable for a BP of 138/97. All other vitals and a spot urinalysis are within normal limits. The infant has no relevant PMH and had a congenital hypospadia corrected 6 weeks after delivery. An abdominal CT scan is notable for a unilateral, non-calcified mass. The most likely pathophysiology of this child’s presentation is?

A)A tumor derived from cells of neural crest origin.

B)A tumor derived from embryonal renal cells.

C)Proliferation of catecholamine producing cells.

D)Metastases of proliferating cells found in the posterior mediastinum.



The best answer here is B. This child most likely has a Wilms Tumor. Remember the association with the WAGR complex (Wilms, Aniridia, GU anomalies, Mental Retardation). The mutation is often ascribed to Chromosome 11 on the WT1 gene (or on some exams the PAX6 gene).


400

A 12-year-old girl with a history of systemic lupus erythematosus presents to the emergency department with decreased urine output, fatigue, and nausea for 2 days. She recently had poor oral intake due to vomiting.

On exam, she is tachycardic with dry mucous membranes and delayed capillary refill.

Laboratory studies show:

  • BUN and creatinine significantly elevated from baseline

  • Potassium: 6.8 mEq/L

An ECG is shown and demonstrates tall, peaked T waves.

Which of the following is the most appropriate next step in management?

emergent administration of IV CALCIUM GLUCONATE or CALCIUM CHLORIDE to stabilize cardiac cell membrane

500

A 4-year-old has close contact with a family member with active TB. Child is asymptomatic and has a positive PPD, normal chest X-ray.

Management?

Isoniazid for 9 months

500

A 3-year-old boy is brought to the emergency department with fever, poor oral intake, and neck stiffness for 2 days. His parents report that he has been refusing to turn his head and prefers to keep his neck extended. They also note droolingand a muffled voice.

On exam, the child appears toxic. He has limited neck range of motion, particularly with extension. Oropharyngeal exam reveals posterior pharyngeal wall fullness.

Which of the following is the most appropriate next step in management?

contrast CT of neck

(RPA)

500

A newborn is found to have a foot that is rigid, plantarflexed, inverted, and adducted.

What is the first-line treatment?

serial casting

500


A 3 hr old newborn is seen by a med student in the NICU for persistent cyanosis. Cardiac auscultation is consistent with a wide, fixed, split of the second heart sound, a harsh, holosystolic murmur at the LLSB, and a “diminished” S1. A CXR is consistent with decreased pulmonary vascular markings. An EKG is notable for left axis deviation



This is tricuspid atresia. This is the only congenital cyanotic defect with LAD. I’ll discuss mechanisms behind the findings in the review. Consider initial tx with our PGE1 analog. There’s a single S2 in some types (also in TGA and truncus arteriosus).

As an aside, remember the association of congenital rubella with a PDA (continuous machine like murmur since there is always flow through the defect in all phases of the cardiac cycle). Give indomethacin/close surgically. Also note the “maneuvers” that will make tet spells in tetralogy of fallot better.


500


A 5 mo is brought to the ED by his mom. The infant’s parents have observed what appears to “jerky” movements of the head, arms, and legs for the past 2 weeks. These episodes occur multiple times a day over short time periods. His medical history is notable for gross and fine motor developmental delay. PE reveals hypopigmented macules that are more prominent on the trunk. The rest of the PE is wnl. What is the next best step in the management of this patient?

A)Supportive care.

B)IV Diphenhydramine therapy.

C)Daily oral ethosuximide therapy.

D)Neurosurgical hyperstimulation of the subthalamic nucleus.

E)Symptom control with a Proopiomelanocortin derivative.



The best answer is E. This child has Tuberous Sclerosis and the accompanying infantile spasms (also known as West Syndrome).

-Associate this dx with the classic hypsarrhythmia on an EEG. ACTH (or vigabatrin) are first line tx options.


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