UWorld Facts Flashcards
1
Q
Intermittent knee pain in a teenager partially responsive to NSAIDS. Location is at proximal tibia.
A
Osgood-Schlatters Disease (Traction apophysitis of the tibial tubercle). XR shows anterior soft tissue swelling, lifting of the tubercle from the shaft, and irregularity/ fragmentation of the tubercle.
Males 13-14, Females 10-11. Trt: decreased activity, stretch, NSAIDs.
2
Q
Intermittent knee pain in a teenager partially responsive to NSAIDs. Location at inferior pole of patella.
A
Patellar tendonitis (overuse). Rest and NSAIDs.
3
Q
Visible hyper intensity in the A-P radiograph of a 2 year old located in the right upper hilum. Shows a scalloped border in a triangular shape with uniform density.
A
Thymus - “sail sign.” Kids
4
Q
Risk factors for Vit D. Deficiency?
A
Increased skin pigmentation, exclusive breastfeeding, inadequate sun exposure, maternal vit. D. deficiency.
5
Q
Baby with large anterior fontanel, lethargy, difficulties with feeding, big tongue. Dx?
A
Congenital Hypothyroidism
6
Q
3 hour old with choking, coughing, and regurgitation on first feed, tachycardia, tachypneic, and hypoxic. She is in marked respiratory distress, and has abdominal distension. She has an Et and orogastric tube inserted, and each ends above the cardiac shadow on XR. What other anomalies are more likely to be found than the general population?
A
This patient has Tracheo-Esophageal Fistula. She should be worked up for VACTERL (vertebral, anal atresia, cardiac, TEF, renal, limb). On prenatal ultrasound, polyhydramnios would be seen due to inability to swallow amniotic fluid.
7
Q
Patient with severe paroxysms of cough shows subcutaneous emphysema in the anterior chest. What is next step and why?
A
subQ emphysema = air in the subQ tissues due to coughs. XR the child to rule out pneumothorax, which can also be caused by the forceful coughing.
8
Q
Mom shows up with a 6 week old, complaining of 3-4 hours of crying each evening for the past three weeks. He is not consolable by pacifiers or normal measures, feeds and stools regularly, and is unremarkable on exam. Dx?
A
Colic. Happens for >2 hrs daily, usually in the evening, or >2 times/wk for ~3weeks. Ddx = Colic, GERD, Corneal abrasion, Hair tourniquet, milk protein allergy, normal crying. Teach parents soothing techniques.
9
Q
Kid with Hepatosplenomegaly, bright red macula, cervical lymphadenopathy, areflexia, hypotonia.
A
Niemann-Pick Disease (Sphingomyelinase deficiency). Tay-Sachs (B-Hexosaminidase A deficiency) does not have a large liver and has hyperreflexia.
10
Q
Risk Factors for Cerebral Pasly?
A
1 Prematurity, IUGR, IU-infection, antepartum hemorrhage, placental pathology, multiple gestation, maternal EtOH/ tobacco.
11
Q
Unvaccinated child with with a blanching, reddish-brown maculopapular rash that spreads from the head to the body and then extremities and spares the palms and soles. Also cough, coryza, and conjunctivitis.
A
Measles (rubeola) - give Vitamin A.
12
Q
Child comes in with rapidly enlarged, unilateral fluctuant mass on the right neck in the anterior lymph node chain. Name the bug (two most likely) and the best medication.
A
Staph A. or Strep Pyogenes - give clindamycin due to LN penetration and MRSA coverage.
13
Q
8 month old with fever, vomiting, sleepiness, full fontanelle, crying with head mvmt, fever of 104, decreased PO, and CBC with cultures pending. In what order should Head CT, IV abx, LP go?
A
IV Abx, LP, no Head CT in Infants due to decreased risk of herniaton
14
Q
Risk factors for Intussusception.
A
Meckel’s diverticulum, HSP, celiac, intestinal tumor, polyps, recent viral illness/Rota vaccine, inspissated stool in CF. Give Air Enema.
15
Q
Kid with a headache that warrants an MRI or CT; indications include?
A
Hx of coordination issues, numbness, tingling, focal neuro deficits, HA awakens from sleep, increasing frequency.
16
Q
Most common cardiac abnormalities in Trisomy 21?
A
50% have Heart Disease: complete AV canal defect (~40%), VSD (~30%), ASD (~15%). Also Hypothyroidism, Hirschsprung, duodenal atresia, ALL/AML
17
Q
Contraindications to Pertussis?
A
Anaphylaxis to vaccine ingredients, progressive neuro disorder (epilepsy/infantile spasms), encephalopathy within a week of previous dose of vaccine.
18
Q
Prophylactic Tylenol before vaccination. Good or bad idea?
A
Bad - can reduce antibody response to vaccines!
19
Q
A 4 year old girl comes in with smelly vaginal discharge and bleeding for 1 week. On exam, she has a whitish foreign body in her vaginal introitus. What do you do?
A
Removal with a calcium alginate swab or irrigation with warmed fluid will likely remove the foreign body - most likely toilet paper. Sedation/anesthesia may be necessary.
20
Q
Patient is born with a defect in the abdominal wall to the right side of the cord insertion. You lost the phone call after that - is it covered with peritoneum or not?
A
No - gastroschisis is not covered by membrane or skin, and always occurs to the right of the umbilicus. Omphalocele (covered by peritoneum) is midline. These patients (omphalocele) have increased risk of cardiac disease, NTDs, and trisomy (1/2 of patients), but gastroschisis does not.
21
Q
Patient with right shoulder pain is found to have a single lytic lesion on Right humerus and mild hypercalcemia - all other labs are WNL. Dx?
A
Langerhan’s Cell Histiocytosis; bone lesions can be locally destructive, but are typically regarded as benign and treated conservatively. Ddx = metastasis, Ewing Sarcoma.
22
Q
4 month old w/ history of NICU for hypoglycemia and macroglossia. 99th% for weight, length, head circ. Reducible umbilical hernia, Right extremities are larger than left. What does he have, and whats the next step?
A
Beckwith-Wiedemann Syndrome - check TSH. Caused by 11p15 imprinting. Often have umbilical hernia or omphalocele. Screen for AFP and do an Abd US /3mo for first 4 years, then just US /3mo 4-8 years, then renal US 8yrs-adolescence due to Wilms and Hepatoblastoma risk.
23
Q
Unvaccinated minor with an urgent need for tetanus vaccination. Divorced parents disagree over vaccine - what do you do.
A
You only need one parent to consent, no consent from the patient, only parents with custody can consent. Treat the kid. If both parents refuse, this is an urgent situation and life-saving treatment (vaccination) is warranted without court approval. Chemo in a stable patient (non-urgent) would need court approval, however.
24
Q
Young boy with eczema, TTP, and has an infection with Neisseria Meningitiditis. He is fully vaccinated including Neisseria, and has a history of Strep Progenies and Strep Pneumo infections. He bled excessively from his circumcision. What is his diagnosis?
A
Wiskott-Aldrich Syndrome; X-R, low IgM, high IgA and IgE, poor Ab response to polysaccharide Ag and moderately low platelets and T-cells.
25
Hepatosplenomegaly, partial oculocutaneous albinism, frequent Staph A. infections, and pancytopenia with mild coagulopathy. Dx?
Chediak-Higashi syndrome. Decreased degranulation, chemotaxis and granulopoiesis leads to giant lysosymes in neutrophils in the setting of neutropenia.
26
Coarse facial features, chronic pruritic dermatitis, eosinophilia, and high IgE with dental abnormalities. Dx?
Hyper-IgE (Job) syndrome. Look for recurrent skin and respiratory staph infections. bone fractures are also common.
27
Patient with a bug bite to the face has pain with extra ocular movements as well as eyelid edema, erythema, and tenderness. Does he have Pre or Post-septal cellulitis?
Post-Septal (orbital) cellulitis has the same features as perceptual (perioribital), including eyelid edema, erythema, tenderness and possibly fever/leukocytosis. It also has ophthalmoplegia, pain with EOM, and possibly vision impairment and proptosis.
28
Young girl with irregular menses varying from 3-6 weeks in length. UPT is negative, PRL and TSH are normal. What is the most likely cause?
Immature Hypothalamic-Pituitary-Gonadal axis causing insufficient gonadotropin secretion, anovulatory cycles, and breakthrough bleeding.
29
Patient with family history of migrane headaches comes in with complaints of stereotyped vomiting episodes with many bouts of emesis per episode. Upper GI series was normal. Dx?
Cyclic vomiting syndrome; >2 episodes/6 months, stereotyped, lasts 1-10 days, vomits >3/hr at peak, no symptoms between vomiting episodes, no underlying dz. Give anti-emetics ad migraine agents.
30
5 year old with sudden-onset weakness on her left with slurred speech. She has developmental delay, eye issues, and is a tall, fair-skinned, thin girl with decreased upper-to-lower segment ratio. She has an upgoing babinski on the left and has joint hyper laxity and skin hyperelasticity. Dx?
Homocysteinuria. Marfanoid body habitis with intellectual disability, fair complexion, and thrombosis (stroke). This is AR vs AD Marfan syndrome. There is less Lens dislocation in homocysteinuria, though it isn't uncommon. There is also a risk of megaloblastic anemia.
31
What is the most common cause of congenital hypothyroidism?
Thyroid dysgenesis.
32
What do the labs look like in Pyloric Stenosis? Note pH, PaCO2, HCO3, K, and Cl.
High pH, PaCO2, and HCO3; Low K and Cl. Its a Metabolic Alkalosis with a respiratory compensation (CO2) and hypochloremia due to loss of gastric HCl. Hypovolemia causes a contraction hypokalemia due to RAAS.
33
Vaccinations that prevent Cancer?
HBV - prevents Hepatocellular carcinoma.
34
Patient with Croup desaturates in front of you from 98% on 2L to 92%. What should be the first step?
Start racemic epinephrine.
35
Patient with presumed (no XR evidence) epiglottitis. Clinically presenting with Distress (inspiratory stridor), Dysphagia, and Drooling. What do you do
Endotracheal intubation in the OR. Steroids and epic are not useful for these patients.
36
A 7 year old boy comes in with aplastic anemia, short stature, hypogonadism, hypo pigmentation, large freckles, low set ears, strabismus, and middle-ear abnormalities. What is the cause of his disorder?
Chromosomal breaks - leads to Fanconi Anemia, leading to loss of DNA repair. Abnormal thumbs are common. Increased risk of cancers. Can have bleeding issues, fatigue.
37
Acquired Causes of Aplastic Anemia
Drugs (NSAIDs, Sulfas), Benzene, Glue, Idiopathic, Viral infections (HIV, EBV), Immune disorders, thymoma.
38
Stroke symptoms in a kid with Sickle Cell Anemia. What do you do?
Exchange transfusion; doesn't remove the issue, but helps to avoid additional strokes. Fibrinolytics don't work on Sickled RBC blockages.
39
Child with rapidly growing head circumference and bulging anterior fontanelle comes in for poor feeding and fussiness for the last month. What is the next step?
Head CT or MRI. Hydrocephalus - give a shunt.
40
Patient shows up for a 3 week WCC. She has a negative ortolani and barlow, but seems to have one leg longer than the other. The left inguinal fold extends posteriorly beyond the anal orifice. What is the next step?
Hip Ultrasound for Hip Laxity. Age 2wks to 6 months = hip US, ages >4-6months = hip XR. If positive Ortolani or Barlow (Developmental Dysplasia of the Hip or DDH), refer directly to orthopedics.
41
How often should a kid have AOM before you should consider tubes?
>2 / 6months, >3 /12months.
42
Scaly erythematous lessons around the eyebrows and sides of the nose in a 6month old, as well as a scaly scalp that improves with shampoo. Dx?
Seborrheic dermatitis (Cradle cap). Moisturizers, topical antifungals, anti-dandruff-shampoo, topical steroids.
43
1 day old; blue when he feeds, pink when he cries. Not fixed by bottle-feed. No retractions, clear lung sounds, normal rate and rhythm, though tachycardic.
Choanal atresia = failure of the posterior nasal passage to canalize, leaving bony (90%) or membranous (10%) obstruction. Part of CHARGE syndrome; Coloboma, Heart defects, Atresia of Choanae, Renal anomalies, Growth impairment, and Ear abnormalities/deafness. Dx with failure to pass a catheter through nose to oropharynx.
44
Mother comes in with her 6 y/o due to hemiplegia of cut onset. He was playing alone, and when she returned, he was unconscious. He slowly regained consciousness, but could not move his right arm and leg. He regains full function after 24hrs in the ED. CT scan is normal. Dx?
Seizure with postictal paralysis (Todd paralysis). This is an indication that a structural abnormality underlies the seizure.
45
Patient with constipation and a UTI. What is the cause of the UTI?
Urinary stasis caused by fecal retention compressing the bladder. Treat the constipation with laxatives, limit cows milk, and increase fiber. Fear, milk, and school entry often contribute.
46
A 12 year old girl comes in complaining of left-sided ear drainage for the last three weeks. She completed two courses of Abx on her previous two visits without improvement, and feels like she is loosing some hearing on the left. Left TM has peripheral granulation with skin debris. Dx?
Cholesteatoma; acquired form, as here, presents with chronic ear drainage, congenital shows up at 5 years old. Complication include hearing loss, CN palsies, vertigo, and infections (abscess, meningitis).
47
A girl presents with annorexia nervosa with a low K and low phos. What should you do with her? Hospitalize, CBT outpatient, or Olanazapine?
Hospitalize for patients with electrolyte disturbances, bradycardia, severe weight loss, or dehydration. Watch for refeeding syndrome; electrolyte depletion, arrhythmias, and HF. Also look for Vitamin deficiencies. Olanazapine for patients who fail CBT and nutritional rehab.
48
Neonate presents with respiratory distress after an uncomplicated vaginal delivery. The pregnancy was complicated with pre-eclampsia. The patient was born SGA (4lb. 4oz), and has been unable to feed due to tachypnea. CBC is significant for a Hemoglobin of 22.8, HCT of 69%, glucose of 50, and a WBC of 5.5. Treatment?
This patient has polycythemia - treat with partial exchange transfusion (remove blood, infuse NS). Caused by IUGR, maternal DM, maternal HTN, smoking, twin-twin transfusion, or delayed cord clamping. Patients are often distended and cyanotic as well as in resp. distress. HCT>65% is diagnostic.
49
What do we treat Enterobius Vermicularis (pinworm) with?
Albendazole! Can also use Pyrantel Pamoate (pregnant),
50
Migratory arthralgia after a trip to NE - synovial fluid is ~25k cells/uL.
Lyme Arthritis, still in early localized (first month) disease. Next is Carditis (5%), Neuro (15%), Muscular (60%), conjunctive (10%) and LAD all possible. Late disease is Arthritis (60%) and Encephalomyelitis or Peripheral neuropathy.
51
Girl with primary amennorhea with an US confirming uterus is present. Next step?
FSH. High FSH -> Karyotype for XO, decreased -> MRI. If Uterus was absent, it would be karyotype. Female = abnormal mullerian development, male = AIS.
52
Gray vesicles/ulcers on posterior oropharynx. Dx?
Herpangina (Coxsackie A); occurs in summer/early fall, fever pharyngitis, ulcers. Supportive treatment only. Differentiate from HSV - anterior oropharynx/lips.
53
Birth to a HBV positive mother (acutely), with HBsAG positivity, what do you do to manage the infant?
Hep B IgG followed by HBV vaccine.
54
Kid with a recent head trauma, headache, 2X emesis, and "isn't acting right" according to his parents. His CT is positive for Epidural Hematoma. What do you do?
Indications for emergent craniotomy include GCS
55
Patient with a constellation of symptoms c/w trisomy 21, an NG tube, and a clearly enlarged stomach and duodenum on XR is consistent with...?
Duodenal Atresia (double bubble sign). Check for ASD/VSD, then surgery.
56
Down syndrome with symptoms of dizziness, hyperreflexity, urinary incontinence, and a positive babinski. Dx?
Atlantoaxial instability (10-15% of Downs patients). Dx with lateral XR of neck. Can present with behavioral change, torticollis, urinary incont., vertebrobasilar symptoms (dizziness, vertigo), hyperreflexia, clonus, positive babinski. Treat with cervical fusion.
57
Patient who yells in her sleep, is found crying, sweating, and looks frightened, but falls back asleep when consoled and does not remember in the morning. Dx?
Night terrors; these are parasomnia in non-REM sleep. The patient is not awake during the episode; episodes are triggered by stress, sleep deprivation, illness, or CNS meds. Nightmares occur during REM, and can be remembered.
58
5 y/o Patient shows up for post-viral hip/knee pain that has not resolved for 2 months. He has an antalgic gate and has limited internal rotation and abduction of the hip. Dx?
Legg-Calvé-Perthes disease; Boys 4-10yrs with avascular necrosis of the femoral head. Shows up like transient synovitis that lasts >1 month. XR shows flattened/fragmented femoral head.
59
A 7 day old is in the NICU with poor feeding and bloody stool. She was term and AGA, but is awaiting weight gain to get her surgery for trunks arteriosus. Vitals are 60/30, HR 164, RR 30. Pulse ox is 82% on RA. Baby is lethargic, cyanotic, and distended with decreased bowel sounds. Blood is visible in her stool. Dx?
Necrotizing enterocolitis (NEC); risk factors are prematurity, birth weight breast milk). Early XR shows pneumatosis intestinal, laid shows pneumoperitoneum. Use abx, bowel rest, IV hydration/nutrition, and possibly surgery.
60
3 year old with abdominal swelling discovered during bathing; recently treated for strep pharyngitis with abx, well appearing, w/ firm non-tender mass in left abdomen. Blood is found on UA. Dx?
Wilms Tumor; Related syndromes include WAGR, Beckwith-Wiedemann and Denys-Drash. Average age 2-5yrs, US first, then contrast CT for confirm/pulm metastasis search. Treat with surgery and chemo. Differentiate from Neuroblastoma which crosses the midline (kidneys are retroperitoneal).
61
Howell-Jolly bodies are seen on peripheral blood smear. What is the cause?
Splenectomy.
62
Brain tumor found in right parietal lobe on MRI secondary to UMN signs on left and recent seizure activity. Most likely tumor?
Benign Astrocytoma (most common supratentorial and infratentorial lesions.
63
Posterior fossa tumor in a 15 year old found to be located in the vermis of the cerebellum. Most likely?
Medulloblastoma (90% are in the vermis).
64
Patient with small face and jaw, overlapping fingers bilaterally without a palmar crease, and rocker bottom feet. Heart murmur is most likely?
Patient has Trisomy 18 - VSD is most common.
65
Prevention/treatment for Chlamydial conjunctivitis (5-14days post birth)?
Maternal testing/treatment; oral erythromycin for 14d. Topical erythromycin is only effective for gonorrhea.
66
A 20 year old female comes to your clinic with a history of facial weakness, difficulty swallowing and delayed relaxation of her achilles tendon reflexes, bilaterally. What Autosomal dominant genetic defect is involved, and what comorbidities should be screened for?
AD CTG trinucleotide repeat in DMPK gene on 19q 13.3. Check for arrhythmias, cataracts, balding, and testicular atrophy/infertility in males.
67
Young girl with pertussis lives in a household with her parents and 1 y/o sister. Her family is fully vaccinated. What is the best way to limit risk of infection in the family?
Age 1 month= Azithromycin x 5 days, Clarithromycin x 7 days, or Erythromycin x 14 days.
Erythromycin is assoc. with pyloric stenosis in neonates. Do not use.
68
Who should be screened for dyslipidemia?
9-11, 17-21, people at high risk (any time) and men >35yrs. This is because lipids are stable before and after puberty.
69
A sexually active female comes to your clinic at 22 years old. She takes OCPs and has a menstrual cycle regularly at 3-4 days long each 29 days. What should you do to evaluate her?
NAAT testing for GC/Chlamydia; Cervicitis/PID are dangerous and often are silent!
70
A 13 y/o girl is brought in for primary amenorrhea. She shows Tanner 2 breasts, no axillary or pubic hair, and a non-reducible mass in the left inguinal region. US shows cryptorchid gonads w/o uterus. Dx/Treatment?
Androgen Insensitivity Syndrome. Her Karyotype is 46, XY. She would have a 1-5% risk of dysgerminoma or gonadoblastoma after puberty, but should allow puberty to occur to attain full height before gonadectomy is performed.
71
15y/o with right tibial pain with XR significant for spiculated "sunburst" pattern with periosteal elevation. Elevated Alk Phos, ESR, and LDH. Dx?
Osteosarcoma = most common primary bone tumor in children/young adults. Usually no constitutional symptoms, Boys 13-16 are most likely, and distal femur, proximal tibia, and proximal humerus are likely sites. Excise and Chemo. Distinguish from Ewing sarcoma with "onion skin" appearance.
72
Impetigo is a clinical diagnosis from golden-yellow crusted pustules on the skin. What is the treatment of bullous vs. non-bullous impetigo?
```
Non-Bullous = strep or staph; Mupirocin topical
Bullous = staph A; Oral abx (cephalexin, diclox, clinda)
```
73
Your doc tells you - patient has Mumps! How cool! What should you see when you walk in; think what nodes/glands are enlarged, rash, etc.
Fever, Malaise, painful enlarged parotids (most often), orchitis (20%); treat with cold complexes, watch for meningitis/encephalitis.
74
Young girl comes in with a fever for the past 6 days. She has a fever of 103, bilateral injected conjunctiva, a 1.9 cm LN on the right neck, and a blanching erythematous rash on her face, trunk, and extremities, including palms and soles. Most common complication without treatment?
Treatment is IVIG and ASA; pt has Kawasaki disease. Worry about coronary artery aneurysm (~20% of untreated pts) and MI. Criteria; Fever>4 days, 4+ of (nonexudative conjunctivitis, mucositis, cervical LAD w/ 1> 1.5cm, erythematous polymorphous rash, extremity changes).
75
What is the gold standard for diagnosing a kid with DMD?
Genetic testing with deletion of Xp21. Also see elevated CK and aldolase before clinical manifestations.
76
Mom presents with a 5 month old with new onset floppiness. Mom says it started with just her neck, but now its her body and legs, too. They recently moved to suburban Utah where a small community was built just for mormons. Examination reveals bilateral ptosis, sluggish pupillary reaction to light, drooling, weak suck and gag, and head lag. Dx/trt?
This infant has botulism; consider it in Utah, PA, California (highest amounts of soil botulism) in environmental dust, or in raw honey. Descending flaccid paralysis is the key. Treat with Botulism immune globulin. Differentiate from ingested toxin (give antitoxin) and GBS (ascending flaccid paralysis). Also consider polio in specific populations.
77
Impaired adaptation to darkness, photophobia, dry kin, dry conjuntiva/cornea, keratomalacia, bitot spots (silver-gray plaques on the bulbar conjunctiva), follicular hyperkeratosis of the shoulders, buttocks, extensor surfaces.
Vitamin A deficiency.
78
Patient presents with a recent history of AOM currently on treatment day 8/10 with amoxicillin. The patient presents with a sore throat, large tonsils, and tonsillar exudates as well as cervical lymphadenopathy. A polymorphous maculopapular rash is noted on the body. Dx?
EBV Mononucleosis. Monospot them and confirm with IgM and IgG. Council them no contact sports for 1 month. distinguish from GAS (doesn't have rash from amox., which is not a real allergy and wouldn't show up while on amox.), Herpangina, HSV, aphthous stomatitis.
79
9 year old girl with instead gait and lower limb weakness. She has a wide-based git and balance issues. Decreased vibratory/position sense and absent ankle jerks. MRI shows atrophy of the cervical spinal cord and some cerebellar atrophy. T-wave inversions are seen on inferior/lateral leads on EKG. Dx?
AR Friedrich Ataxia. Wheelchair bound by 25, death by 35. Cardiomyopathy, CHF.
80
Benefits of Breastfeeding?
Mom: Reduced Risk of breast and ovarian cancer, faster return to prepartum weight, decreased postpartum bleeding. Infant: immunity, better GI function, prevention of AOM, gastroenteritis, resp. illness, UTIs; decreased risk of T1 DM, Necrotizing enterocolitis, childhood cancer.
81
1 month old with 2/6 harsh holosystolic mumur at left lower sternal border. ECG Wnl, 2+ extremity pulses. Next?
Echo. 75% of small VSD close by 2 years.
82
2 month old turns blue with feeds and crying. BP 80/50, HR 150, RR 55. He has a 2/6 crescendo-decrescendo systolic ejection murmur at the left upper sternal border and a single S2. Dx? Treatment is knee-chest position; pathophysiology?
Tetrology of Fallot; RVOT obstruction, RVH, overriding aorta, VSD. Knee-Chest position increases systemic vascular resistance and changes a right-left shunt into a left-right shunt. Give O2, IVF, and morphine.
83
22 month old with easy fatigue and SOB with mild activity. 2/6 holosystolic murmur on Left lower sternal border and diastolic rumble at apex. Cause?
VSD - reversal into eisenmenger syndrome (pulm. overcirculation).
84
9 year old with fever, hives, joint pain. Itchy rash began 2 days ago, pain in wrists and ankles. He is currently on oral Penicillin for Strep pharyngitis diagnosed 9 days ago, and he has a fever of 101.5. He has diffuse lymphadenopathy and a diffuse urticarial rash. Dx?
Serum sickness; B-lactams and sulfas most common. Polyarthralgia 1-2 weeks after exposure, Fever, urticaria, HA, edema, lad, splenomegaly.
85
Kiddo with fever, dysphagia, inability to extend the neck, muffled voice, lateral XR with widened prevertebral space.
Petropharyngeal abscess
86
Brick-red urate crystals in the diaper of a 4 day old with 3 diapers per day. Dx?
Breastfeeding failure jaundice; suboptimal breastfeeding and dehydration show low diapers (
87
Threshold for phototherapy in a full-term, 4 day old?
>20 total; exchange transfusion >25.
88
Child with ITP (TTP, peripheral smear with megakaryocytes) Treatment for skin only vs bleeding?
```
Skin only (petechiae, ecchymosis) = Observe
Bleeding (epistaxis, hematuria, GI) = IVIg or GCs.
```
89
FSH and LH findings in a Turners patient?
High FSH due to lack of feedback, low E, relatively nl LH.
90
Most common predisposing factor for a bacterial sinusitis ( purulent nasal drainage, congestion, cough, fevers >39C)?
Viral Upper resp. infection. Treat with Amox/ Clav.
91
Patient with inspiratory stridor, worsens with laying down, crying, feeding; dx via? and treatment with ?
Flexible laryngoscopy showing omega-shaped epiglottis and collapse of supraglottic structures = larnygomalacia; reassurance. Give PPIs for GERD, as there is increased airway collapse. Self-resolution at 18months.
92
Poison Ivy = what type of hypersensitivity?
Type 4, Delayed-type; cell-mediated.
93
Vit K. Dependant bleeding cofactors?
Factors 2, 7, 9, 10, C, S. Prolonged PT.
94
Risk Factors for brain abscess?
Otitis Media, Mastoiditis -> cerebellum or temp. lobe
Frontal/Ethmoid sinusitius -> Frontal lobe
Dental infection -> Frontal Lobe
Bacteremia/ cyanotic heart disease -> multiple along MCA gray-white matter junction.
95
Uterine contractions triggered by prostaglandin in the absence of other pathology. Dx?
Primary Dysmenorrhea. Up to 90% of young women. NSAIDS are first line. VERY common. dyspareunia, think endometriosis; Adeno = > 35 years.
96
Obsessive Compulsive Disorder is treated how?
CBT +/- SSRI (high dose); Clomipramine for refractory, Deep-brain stim (Nucleus Accumbens) for severe refractory. Rare post-group A strep rapid onset OCD = PANDAS - Pediatric Autoimmune Neuropsychiatric Disorders Associated with Strep.
97
Little boy who doesn't respond to sounds and failed bilateral newborn hearing screen, has bilateral white reflexes, has continuous systolic-diastolic murmur. Dx?
Rubella (german measles):
Congenital = sensorineural hearing loss, intellectual disability, cardiac anomalies, cataracts/glaucoma.
Children = low grade fever, conjunctivitis, coryza, cervical LAD, cephalocaudal spread of blanching erythematous maculopapular rash.
Adults - kids symptoms + arthritis.
98
8 year old with abdominal pain and dark urine. Scleral icterus and mild abdominal tenderness, mass in RUQ. Total bill is 6.5, direct is 3.4, Lipase is 152, amylase 91. US shows extra hepatic cystic mass, normal gallbladder. Dx?
Biliary, or choledochal cyst. Pain, Jaundice, Palpable mass
99
Floppy baby syndrome, no exposure to botulism. Dx?
Werdnig-Hoffman syndrome. AR; anterior horn cells and CN motor nuclei degenerate.
100
Diagnosis of CF is via what methodology?
Quantitative Pilocarpine Intophoresis = sweat test.
101
15 year old with scoliosis and feet deformities with 'hammer toes' has a neuro exam significant for dysarthria, dysmetria, nystagmus, and absent deep plantar reflexes. Dx?
Friedrich ataxia = spinocerebellar ataxias including gait, falls, etc. Spinocerebellar tract, posterior columns, pyramidal tracts affected. Watch for hypertrophic cardamypathy (90%), diabetes, skeletal deformities.
102
4 week old with forceful emesis for 1 week that is non-bilious. Patient seems dehydrated. Dx?
Infantile hypertrophic pyloric stenosis or gastric outlet obstruction. Risk increased with first born, erythromycin, and formula feeds. Find hypochloremic metabolic alkalosis.
103
Rubella vs Rubeola... differences
Measles (rubeola) = more severe, higher fevers (~104 vs 101), slower moving and darker rash, and coryza and malaise are more severe. Rubella often has Forschheimer spots (patchy erythema of the soft palate).
104
Grip myotonia, facial weakness, foot drop, dysphagia, and cardiac conduction abnormalities in an 18 year old female. Dx?
AD Myotonic Muscular dystrophy. Trinucleotide expansion of CTG on 19q13.3.
105
18 month old with history of frequent infections here for labs of: CBC wnl, low IgG and IgA, high IgM; dx?
Hyper IgM syndrome. X-linked CD40 ligand defect. Recurrent sinopulmonary infections (AOM, PNA, sinusitis).
106
NF1 vs NF2; gene mutations?
NF1 = neurofibromin; NF2 = merlin.
107
15 year old with history of sickle cell crises comes in with Temp of 99, nl pressure, hr, resp, with pain in right hip starting several weeks ago and gradually progressing He has no local tenderness, but restricted abduction and internal rotation of the hip. dx?
Avascular necrosis of the femoral head. Lack of Fever = not osteomyelitis, no local warmth/tenderness = not septic arthritis. Other causes of avascular necrosis are chronic corticosteroids, SLE, EtoH, Gaucher's.
108
What hormone underlies refeeding syndrome?
Insulin! Rapid increased insulin due to switch from ketone use for energy leads to increased uptake of phase, K, Mg, b1; leads to CHF, seizures, Wernicke encephalopathy, and arrhythmias.
109
Infant with tarsometatarsal deformity. Based upon correction, you treat differently. Overcorrects vs correctors vs rigid feet (no correction); treatment?
Overcorrects are self-limited; reassure the family
Correctors get orthotics
Rigid feet get serial casts.
110
Child with recent hematuria and proteinuria and history of sensorineural deafness gets a renal biopsy with thinned and thickened capillary loops with splitting of the GBM. Dx?
Alports; family history is important.
111
Bone Pain, LAD with nontender, rubbery lymph nodes, hepatosplenomegaly, pallor, petechiae and Lymphocytosis to 30K. Dx?
Bone Marrow Biopsy for >25% blasts = ALL; Male>female, peak 2-5 years, most common childhood cancer.
112
Most common complication of sickle cell anemia?
Hematuria then impaired concentrating ability (polyuria/nocturia).
113
Sepsis in setting of Sickle Cell Anemia. Other than vaccinations, what could have been done to prevent this?
Penicillin prophylaxis until 5 years old. Pneumococcus is the most common bug for sepsis, and is also a cause of encapsulated sepsis (asplenics, beware!).
114
Treatment for Tourette disorder?
Antipsychotics, alpha agonists, behavioral therapy. Risperidone is the best studied, and often preferred over 1st gen anti psych due to decreased EPSEs.
115
Prader-willi syndrome = loss of which copy of 15p11?
Loss of the Prader (Father). Complications include OSA (70%), T2dM (25%), Gastric issues, and often death by choking (8%).
116
13 year old with BMI of 31 comes with a limp favoring his right log. AP frog-leg XR shows slight sliding movement of the femoral neck and head. Dx?
SCFE. Obesity 10-16 years, Boys>girls. Children with endocrine issues (hypothyroid, GH deficiency) have bilateral disease, present earlier. Treat with immediate surgical screw fixation.
117
Strong apical impulse with systolic ejection murmur heard on left sternal border. Carotid pulse has a dual upstroke. Dx? How do we make it louder?
HOCM; Make louder with decreased preload via valsalva, abrupt standing, or Nitro; quieter with increased after load of preload via squatting, passive leg raise, sustained hand grip.
118
inattentiveness in a 6 year old who seems to ignore people and has 'trouble following directions because he doesn't listen' with concurrent language delay and prefers to be alone. Dx?
Undetected hearing impairment.
119
Guillain-Barré syndrome; pathology?
demyelination of peripheral motor nerves; flaccid paralysis with absent DTRs. CSF = increased protein, nl cells. IGg or plasmapheresis.
120
2 month old presents with poor feeding. Lab shows CBC with low red cells, low reticulocytes, but normal WBC and platelet counts. PE shows pallor.
Diamond-Blackfan anemia (or congenital pure red cell aplasia).
121
Limited upward gaze, upper eyelid retraction, HA, vomiting, ataxia, and sluggish pupillary reaction to light. Workup? Dx?
Get MRI, serum and CSF AFP and B-HCG; this is a Pinealoma; Pineal gland puts pressure on the pretectal region of the midbrain and CN3, leading to ptosis, limited upward gaze, eyelid retraction and minimal reaction to light with normal accommodation (Parinaud syndrome). Obstructive Hydrocephalus due to blocked CSF flow in the aqueduct of selves causes HA, vomiting, and ataxia. Surgery, CSF shunt, and/or radiation treatments.
122
In a certain type of seizure, hyperventilation during the EEG simulates an episode. Which type?
Absence seizures (no post-ictal state, spike and wave activity). Normal EEG with hyper vent is likely a complex partial = LOC, failure to respond to stimulus, staring spells, autospasms, post-octal confusion.
123
8 y/o female with Hx two bone fractures, moonlike face, two light-brown spots with irregular contours on the shoulders and neck. She has bruising on the right arm and breast budding and enlargement of the areola. Tanner 4 hair is present. Dx?
McCune-Albright; females, precocious puberty, cafe au-lait spots, bony defects (polyostotic fibrous dysplasia). Assoc. with hypercortisolism. 3 P's; precocious puberty, pigmentation, polyostotic fibrous dysplasia.
124
First time seizure in a 1 year old. Temp of 101.4, No previous seizure history and patient returned to normal within minutes following. Patient has full ROM of neck. Dx?
Febrile Seizure = 6 months-6 years with Temp >38, no history of afebrile seizures, no CNS infection, no acute systemic metabolic cause. Reassurance.
125
6 year old with fever, joint pain, inability to bear weight, ESR elevated, leukocytosis. Synovial fluid is aspirated and sent for culture. What would indicate surgical drainage?
>100,000 leukocytes, >90% PMNs, purulent fluid; this can lead to femoral head necrosis, so send to ortho immediately.
126
Patient has daydreaming episodes noted by his teacher with declining school performance. He does not remember these episodes, and is non-responsive during them. Trt?
Absence seizures (no post-ictal state mentioned). Valproic Acid or Ethosuximide. Phenobarbital in refractory.
127
SGA infants are at risk for what complications?
hypoxia, perinatal asphyxia, meconium aspiration, hypothermia, hypoglycemia, hypocalcemia, polycythemia.
128
A 3 year old comes to the office with an erythematous rash and superficial flaccid bull all over the body. Nikolsky sign is positive, there is edema in her face, and a fever of 102. Symptoms started this morning. Dx?
Staph scalded skin syndrome. erythema starts on face and generalizes within 24-48 hours. Usually resolves in 1-2 weeks. Sterile cultures from bullae; give anti-staph abx.
129
Vesicoureteral reflux in the setting of recurrent UTIs is most likely to cause what complication?
Renal Scarring - can lead to renal insufficiency and ESRD.
130
A young boy comes in after failing a 10 day course of antibiotics for otitis media two days ago. He has been awakened by headaches for the last two nights, and has been vomiting in the early morning. Next step?
MRI or CT; CT is faster, available, gives better bone pictures. MRI is better for soft tissue details is more sensitive for early cerebritis, and can distinguish edema from necrosis. Dx; brain abscess.
131
A 6 year old patient comes to the clinic for looking yellow. He has generalized jaundice and scleral icterus, has a palpable spleen, has hemoglobin of 9.7g/dL and an MCHC of 37%. What is the gold standard for diagnosing his condition, and how is it treated?
MCHC >36%, spherocytosis, coombs-negative anemia = Hereditary Spherocytosis, and is diagnosed with Eosin-5-maleimide binding test and acidified glycerol lysis test. Treat with Folic Acid, blood transfusions, and splenectomy. Can be complicated by gallstones and Parvo B19 crises.
132
Causes of acquired torticollis etiologies?
URIs, minor trauma, cervical lymphadenitis, retropharyngeal abscess, atlantoaxial subluxation.
133
Edematous swelling of face, including the lips, hands, arms, legs, genitals without a rash. Patient has recently been sick with bronchitis, but has new onset colicky abdominal pain.
Hereditary Angioedema = C1 inhibitor deficiency with elevated C2b and bradykinin; Rapid onset of noninflammatory edema of the face, limbs, genitals, Laryngeal edema, and colicky abdominal pain without urticaria. Acquired due to ACEI use = C1q elevated.
134
Palpable purpura of lower extremities, arthralgia, abdominal pain, and renal disease (hematuria, and some proteinuria).
HSP vasculitis. Renal = IgA deposition in the mesangium. Treat with supportive or steroids if severe.
135
How do we treat urinary incontinence in a 5 year old?
Behavior modifications = avoid sugary/caffeinated beverages, void immediately before bed, drink ample fluids in morning/afternoon and less before bed, and use a reward system. Enuresis alarm is 1st line for failed behavior modifications, with best long-term outcomes. Pharm = desmopressin (1st), TCAs (2nd).
