RoshReview

Question 1

AC joint injury

1) MC mechanism of action
2) exam
3) Tx
4) Prognosis

Answer 1

1) fall to lateral shoulder, or blow to AC joint
2) pain over AC joint, Pain with adduction of the arm across the chest (clavicle displacement can be visible with higher grade injuries)
3) anywhere from sling to surgery
4) type 1- heal in 1 to 2 weeks
type II or III- heal in 1 to 3 months

Question 2

Bleeding since birth with normal PT, PTT, von willebrand activity and plt number, think_______

Answer 2

plt function disorders, like Bernard-Soulier syndrome (a disorder of platelet adhesion) and Glanzmann thrombasthenia (a disorder of platelet aggregation).

Question 3

most common congenital bleeding disorder

Answer 3

Von willebrand

Von Willebrand factor is a linking factor that allows functional platelets to bind to fibrin to form a clot

Question 4

Bloody diarrhea and pharyngitis

pathogen?

Answer 4

Yersinia enterocolitica

Question 5

Yersinia enterocolitica
duration?
Tx?

Answer 5

1) Up to 3 weeks

2) Antibiotics are not beneficial in the treatment of acute uncomplicated yersiniosis.

Question 6

•Recurrent otitis media

-Definition?

Answer 6

episodes of acute otitis media (AOM) in the past 6 months or 4 episodes of AOM in the past 12 months

Question 7

•Incontinentia pigmenti

-presentation?

Answer 7

vesicles in a linear arrangement at birth or within the first weeks of life.

Question 8

Neonatal lupus

1) What is the pathogenesis?
2) Presentation
3) Course

Answer 8

1) tranference of maternal lupus antibodies (Ro and SSA) across the placenta
2) heart block starting in utero, discoid rash started from birth to starting 1-2 months after birth, Hepatosplenomegaly and Cholestasis
3) self-limited

Question 9

NF type 1

Answer 9

café-au-lait macules, axillary and inguinal freckling, Lisch nodules, and neurofibromas and cognitive disability

Question 10

Galactosemia

1) presentation

Answer 10

1) occurs the first few days after birth and symptoms vary in frequency. The most common findings are jaundice, vomiting, hepatomegaly, failure to thrive, poor feeding, lethargy, diarrhea, and sepsis. Gram-negative sepsis due to microorganisms like Escherichia coli is one of the key findings in galactosemia.

Question 11

Drug of choice once the diagnosis of neonatal Gram-negative meningitis is confirmed?

Answer 11

Cefotaxime

Question 12

Benign childhood epilepsy with centrotemporal spikes (BCECTS)

• When does it first appear?
• When does it occur?
• what does the seizure look like?

Answer 12

• most common epilepsy syndrome in childhood
• Most children will outgrow the syndrome (it starts around the age of 3-13 with a peak around 8–9 years and stops around age 14-18)
• occur shortly after falling asleep or before wakening
• The seizures typically consist of unilateral facial sensorimotor symptoms, oropharyngeal manifestations, speech arrest, and hypersalivation

Question 13

Digeorge syndrome findings

Answer 13

Catch 22

• Cardiac abnormalities
• Abnormal facies (small mouth, a submucous cleft palate, abnormal and low set ears, upturned nose, and a widened distance between the inner canthi with short palpebral fissures)
• Thymic absense
• Cleft palate
• Hypocalcemia

Chromosome 22

Question 14

Hypocalcemia what do you need to stabilize the heart

Answer 14

Calcium gluconate

Question 15

Parkland formula

Answer 15

fluids in first eight hours=1/2[4 x %area of burn x kg+ maintenance fluids]

Question 16

Lymphogranuloma venereum vs Chancroid

Answer 16

Lymphogranuloma venereum has a painLESS ulcer

Chancroid has a painFULL ulcer

Question 17

Pertussis Tx

Answer 17

Azithromycin

Question 18

Hepatoblastoma

1) who gets it?
2) sxs and labs

Answer 18

1) asymptomatic abdominal mass in children < 3. Associated with prematurity, hemihypertrophy, Beckwith-Wiedemann, familial adenomatous polyposis, Li-Fraumeni, and Trisomy 18
2) Anemia, thrombocytosis, elevated AST, ALT, and bilirubin levels. AFP is elevated in more than 90 percent of cases

Question 19

scabies treatment

Answer 19

permethrin 5% cream

The entire family should be treated as well as all caretakers of the infested child. Clothing, bed linens, and towels should be washed in hot water and items that cannot be washed must be stored in bags for a week. If there are severe manifestations or the patient is immunocompromised, oral ivermectin given orally for two doses, two weeks apart, is recommended.

Question 20

WAGR syndrome

Answer 20

Wilms tumor
Aniridia (no iris)
Genitourinary abnormalities
Retardation (mental)

Question 21

thrombocytopenia skin findings vs vasculitis skin findings

Answer 21

Thrombocytopenia causes flat, non-palpable purpura, while vasculitis causes palpable purpura.

Question 22

antidote for a tricyclic antidepressant overdose

Answer 22

Sodium bicarbonate

Question 23

tx of otitis media

Answer 23

Amoxicillin 90 mg/kg per day orally in two divided doses

Question 24

Testing for pheochromocytoma

Answer 24

Plasma metanephrines

Question 25

Screening for patients with HSP

Answer 25

Needs a urinalysis to evaluate for glomerulonephritis, specifically to evaluate for blood, casts, and protein. Renal manifestations of HSP vary widely. While 20-30% of children develop gross hematuria, others develop a range of manifestations from asymptomatic hematuria or non-nephrotic range proteinuria to acute renal insufficiency. The child must be screened for development of renal disease over at least six months following presentation, as renal manifestations of HSP can continue to develop after other signs and symptoms have resolved.

Question 26

Kerion

Answer 26

Patient with a history of tinea capitis (scalp ringworm) Complaining of scalp rash PE will show a scalp that is boggy with inflamed purulent nodules and plaques Most commonly caused by delayed-type hypersensitivity reaction to the dermatophyte causing the fungal infection Treatment is long-term oral griseofulvin Comments: hair follicle is frequently destroyed leading to scarring alopecia

Question 27

Hypocalcemia manifestations

Answer 27

Prolonged QTc, tetany, seizures

Question 28

rickets and precocious puberty

Answer 28

McCune-Albright Syndrome

Question 29

Fanconi syndrome 1) confirmatory test 2) labs 3) sxs

Answer 29

1) leukocyte cystine level 2) hyperchloremic metabolic acidosis and a low to low-normal serum potassium 3) present between 3 and 6 months of age with polyuria, polydipsia, acute dehydration, poor weight gain and poor growth. They may also have signs of rickets, such as frontal bossing.

Question 30

macrophage activation syndrome 1) what is it? 2) symptoms 3) how to confirm dx?

Answer 30

1) rare but potentially fatal complication of systemic-onset juvenile idiopathic arthritis (JIA) 2) thrombocytopenia, elevated AST, leukopenia, hypofibrinogenemia, CNS dysfunction, hemorrhage, and hepatomegaly 3) bone marrow biopsy demonstrating macrophage hemophagocytosis

Question 31

congenital hypopituitarism

Answer 31

* Hypoglycemia * Direct hyperbilirubinemia - Relative TSH deficiency results in a low free thyroxine level with a low or inappropriately normal TSH level. - Due to gonadotropin deficiency, males may have a small penis and/or cryptorchidism.

Question 32

patellofemoral pain syndrome 1) Hx 2) exam

Answer 32

1) chronic anterior knee pain without an inciting injury, pain with prolonged sitting, a feeling of instability with running, pain going up and down stairs 2) tenderness over the medial patellar facet, pain with patellar compression, and mild swelling

Question 33

Anterior vs posterior mediastinal mass differentials

Answer 33

anterior- 4 Ts:teratomas, thymomas, thyroid cancers, and “terrible lymphomas.” Posterior- likely a neuroblastoma

Question 34

Allergic bronchopulmonary aspergillosis 1) who gets it? 2) symptoms 3) tx

Answer 34

1) 1% to 5% of patients with persistent asthma and less than 10% of cystic fibrosis patients will have ABPA 2) Recurrent asthma exacerbations, complicated by fever, malaise, bronchial obstruction, productive cough with dark brown mucus plugs, and even hemoptysis 3) The treatment for ABPA focuses on control of the inflammation to prevent lung injury with long-term systemic glucocorticosteroids followed by a very slow taper over 6 months. A triazole antifungal may be used.

Question 35

DUKE criteria

Answer 35

Criteria for endocarditis (need 2 major or 1 major and 3 minor or 5 minor) Major: - 2 separate blood cultures with typical endocarditis organisms - ECHO evidence Minor: - temp >38 - predisposing heart condition or IV drug use - vascular phenomenon - immunologic phenomenon - blood culture

Question 36

Empiric treatment of endocarditis

Answer 36

Vanco and gentamicin

Question 37

initial genetic testing for mental delay

Answer 37

fragile X testing and chromosomal microarray

Question 38

Spinal epidural abscess Sxs Dx Treatment

Answer 38

1. ) fever, back pain, nerve compression sxs 2. ) MRI , contrasted CT if MRI not available 3. ) Vanco (most common cause is staph aureus in US) + 3rd gen cephalosporin

Question 39

Empiric therapy for brain abscess

Answer 39

Vancomycin+cefotaxime+ metronidazole * if has had recent neurosurgery need to cover for MRSA and pseudomonas, so do vancomycin + meropenem or cefepime

Question 40

presentation for spinal muscular atrophy

Answer 40

presents in infancy with head lag; weak, hypotonic limbs; areflexia; paradoxical breathing; tongue fasciculations; and finger trembling. The muscles of facial expression are spared.