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1
Q

Henoch-Schonlein purpura

A

IgA mediated leukocytoclastic vasculitis

sx: palpable purpura, arthritis/arthralgia, abd pain/intussusception, renal dz (IgA dephropathy)

  • -> nml plt, coags, CBC
  • -> normal or increased creatinine
  • -> hematuria +/- RBC casts and proteinuria
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2
Q

NF-1 vs. NF-2

A

NF1 = cafe au lait spots, macrocephaly, short stature, feeding problems, learning disabilities, tumors later in life

NF2 = Bilateral acoustic neuromas + cataracts

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3
Q

Pityriasis Rosea

A

+/- viral prodrome
annular, pink herald patch on trunk
-oval lesions in “christmas tree” pattern
-pruritis

TX = reassurance (resolve spontaneously in weeks to months)

tx itching with anthistamines

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4
Q

Selective Mutism

A

Refusal to speak in specific social situations, nml elsewhere

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5
Q

Congenital torticollis

A

head tilt to one side

  • postural deformity
  • palpably, well circumscribed, mass that does NOT transilluminate on inferior aspect of SCM
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6
Q

Legg-Calve-Perthes disease

A

idiopathic avascular necrosis of femur

  • boys age 3-12
  • insidious hip pain + limp
  • decreased hip ABduction and internal rotation

TX: non-weight bearing, splinting, (?) surgery

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7
Q

Slipped capital femoral epiphysis

A
  • limp + insidious hip pain
  • OBESE adolescent
  • XR –> posterior displacement of femoral head
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8
Q

Reye syndrome

A

2/2 peds ASA use w/influenza or varicella infection

  • -> Acute liver failure (microvesicular steatosis on liver bx)
  • -> elevated AST, ALT, PT, INR, PTT, and ammonia

TX: supportive

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9
Q

Eosinophilic esophagitis

A

chronic, immune-mediated esophageal inflammation

sx: dysphagia, epigastric pain, reflux/vomiting, food impaction, associated atopy

DX: endoscopy and esophageal biopsy with > 15 eos/hpf

TX: dietary modification +/- topical glucocorticoids

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10
Q

Gonnococcal vs. chlamydial neonatal conjunctivitis

A

Gonococcal: onset at 2-5 days
-marked eyelid swelling, profuse purulent discharge, corneal edema/ulceration
TX: single IM dose of 3rd gen cephalosporin

Chlamydial: mild eyelid swelling, watery/serosanguinous, or mucopurulent eye discharge
TX: PO macrolide

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11
Q

Leukocyte adhesion deficiency

A
  • recurrent skin and mucosal bacterial infections
  • -> no pus (lack of neutrophils at inflammation site)
  • -> poor wound healing
  • Delayed umbilical cord separation (>21 days)
  • Marked peripheral leukocytosis

Pathophys: defective integrins on leukocyte surface –> neutrophils can’t adhere to vascular endothelium and exit

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12
Q

Adenosine deaminase deficiency

A

AR
Severe combined immunodeficiency

  • deficient formation of mature B and T lymphocytes
  • Severe infections and failure to thrive
  • Labs show marked lymphopenia
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13
Q

X-linked agammaglobulinemia

A

Defective B lymphocyte maturation due to mutation in Bruton tyrosine kinase

  • Recurrent sinopulmonay and GI infections
  • LOW B cell and Ig concentrations
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14
Q

Patellofemoral stress syndrome

what makes it worse?

A

overuse injury seen in runners

anterior knee pain that worsens upon descending steps or hills

pain localized to patella

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15
Q

Breastfeeding failure jaundice

A

presents in 1st week of life
-Lactation failure resulting in –> decreased bilirubin elimination, increased enterohepatic circulation

–> suboptimal breastfeeding, signs of dehydration

TX: increase frequency and duration of feeds to stimulate milk production and maintain adequate hydration

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16
Q

Breast milk jaundice

A

starts at 3-5 days, peaks at 2 weeks

-High levels of B-glucuronidase in breast milk deconjugates intestinal bilirubin and increases enterohepatic circulation

–> adequate breastfeeding w/ normal exam

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17
Q

Treatment of Croup (laryngotracheitis)

A

mild (no stridor at rest) –> humidified air +/- corticosteroids

Moderate/severe (stridor at rest) –> corticosteroids + nebulized epinephrine

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18
Q

Necrotizing enterocolitis (NEC)

A

risks: prematurity, very low birth weight
- -> gut immaturity and exposure to bacteria from enteral feeds –> inflammation and damage to bowel wall

*Decreased rates of NEC in breastfed premature infants

DX: abd XR with air in bowel wall (pneumatosis intestinalis) and portal venous air –> can lead to perforation and pneumoperitoneum

w/leukocytosis and metabolic acidosis.

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19
Q

Edwards Syndrome (trisomy 18)

A

microcephaly, micrognathia, overlapping fingers, absent palmar creases, rocker-bottom feet

Increased risk of VSD (holosystolic murmur at LL sternal border)

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20
Q

Ewing’s sarcoma

A

malignant tumor in LE > UE

typically in metaphysis and diaphysis of femur

very aggressive, early mets to lungs and lymph nodes

on XR has onion skin periosteal reaction or moth-eaten/mottled appearance and extension into soft tissue

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21
Q

Causes of QT prlongation

A
  1. hypocalcemia
  2. hypokalemia
  3. hypomagnesmia
  4. Meds - abx, psychotropics, opiods, antiemetics, antiarrhythmics
  5. Inherited
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22
Q

Inherited disorders of QT prlongation

A
  1. Jervell and Lange-Nielsen

2. Romano-Ward syndrome

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23
Q

Jervell and Lange-Nielsen

A

AR, congenital long QT due to defect in potassium channels

  • associated with congenital deafness
  • increased risk of syncope, ventricular arrhythmias, sudden death, and torsades

TX: B-blockers, normal electrolyte levels, avoid QT prolonging medications, avoid rigorous exercise

*if h/o syncope, add pacemaker

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24
Q

Methemoglobinemia

A

caused by exposure to oxidizing agent (anesthetics, nitrites, dapsone)

Fe2+ –> Fe3+ (ferric state) has increased O2 affinity –> decreased oxygen delivery to peripheral tissues

SX: cyanosis, dark chocolate blood, low pulse ox, PaO2 is NORMAL

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25
Minimal change disease
most common cause of nephrotic syndrome in children (age 2-3 yrs, most <10yrs) Path: T-cell mediated injury to podocytes causes increased molecular permeability to albumin SX: edema, fatigue, no hematuria, hypoalbuminemia, proteinuria TX: corticosteroids
26
Rotavirus vaccine is given....
given between 2-8 months
27
McCune-Albright Syndrome
SX: precocious puberty (FSH, LH), irregular cafe-au-lait macules, recurrent fractures due to polyostotic fibrous dysplasia -can also cause thyrotoxicosis (TSH), acromegaly (GH), cushings (ACTH) GNAS gene mutation --> G protein activation and overproduction of pituitary hormones
28
Immune Thrombocytopenia
- antecedent viral infection - asymptomatic petechiae and ecchymosis - mucocutaneous bleeding - isolated thrombocytopenia w/peripheral smear showing megakaryocytes TX in kids: - skin manifestations only --> observe - bleeding --> IVIG or glucocorticoids
29
Laryngomalacia
increased laxity of supraglottic structures --> inspiratorry stridor that worsens when supine -peaks at age 4-8 months DX - direct laryngoscopy TX - reassurance vs. supraglottoplasty
30
Riboflavin deficiency (B2)
- due to poor intake, poor absoption, probenacid, oral contraceptive use. - SX: cheliosis, glossitis, ocular problems (keratitis, conjunctivits, corneal vascularization), and seborrheic dermatitis
31
cheliosis, glossitis, ocular problems (keratitis, conjunctivits, corneal vascularization), and seborrheic dermatitis which deficiency am I?
B2, riboflavin
32
Vitamin B6 (pyridoxine) deficiency
-enzyme deficiency vs. poor absorption vs. isoniazid, steroids, anticonvulsants SX: seizures, peripheral neuritis, dermatitis, microcytic anemia
33
seizures, peripheral neuritis, dermatitis, microcytic anemia which deficiency am I?
Vitamin B6 (pyridoxine) deficiency
34
Folate deficiency
due to poor intake/absorption, high demand (e.g. sickle cell), inborn errors of metabolism, MTX, phenytoin SX: megaloblastic anemia, glossitis, pharyngeal ulcers, impaired immunity
35
megaloblastic anemia, glossitis, pharyngeal ulcers, impaired immunity which deficiency am I?
Folate deficiency
36
Kawasaki disease
acute vasculitis of small and medium arteries - typically age <5, asian - prolonged high fever, unresponsive to antipyretics -Conjunctivitis (BL, nonexudative) -strawberry tongue -rash erythema, edema, desquamation of hands and feet -cervical lymphadenopathy -elevated ESR/CRP, thrombocytosis, sterile pyuria **can get coronary artery aneurysms TX: ASA + IVIG
37
evaluation of Malrotation --> _____
upper GI series abd XR
38
DDX of bilious emesis
meconium ileus Hirschsprung disease malrotation duodenal atresia
39
Evaluation of meconium ileus and hirschsprung disease?
contrast enema
40
evaluation of duodenal atreasia
abd XR --> double bubble sign
41
Iron poisoning --> what sx?
abdominal pain, vomiting, diarrhea, hypotensive shock * Anion gap metabolic acidosis - -> hepatic necrosis and pyloric stenosis TX: deferoxamine, whole bowel irrigation
42
Di George Syndrome
CATCH -22 ``` Conotruncal cardiac defects Abnormal facies Thympic aplasia/hypoplasia Cleft palate Hypocalcemia ``` *Defective development of pharyngeal pouches Chr 22 q11.2 deletion
43
Sickle cell disease patients are at high risk for what bugs causing sepsis?
1) Strep pneumoniae 2) Haemophilus influenzae 3) Neisseria meningitidis
44
Wilms tumor is associated with what syndromes?
WAGR Beckwith Wiedemann syndrome Denys-Drash syndrome
45
WAGR
wilms tumor, aniridia, GU abnormalities, intellectual disability (retardation)
46
Wilms tumor sx
asymptomatic, firm, smooth abdominal mass that does not cross midline
47
Types of renal tubular acidosis
1) distal - poor hydrogen secretion into urine 2) proximal - poor bicarbonate resorption 4) aldosterone resistance
48
Type 1 distal RTA
poor hydrogen secretion into urine urine pH > 5.5 low-normal serum potassium CAUSES: - genetic disorders - med toxicity - autoimmunie disorders
49
Type 2 (proximal) RTA
poor bicarbonate resorption urine pH < 5.5 low normal potassium CAUSES: -fanconi sydrome (glucosuria, phosphaturia, aminoaciduria)
50
Type 4 RTA
aldosterone resistance urine pH < 5.5 HIGH serum potassium CAUSES: - obstructive uropathy - CAH
51
Vitamin A deificiency
``` impaired adaptation to darkness photophobia dry scaly skin dry conjunctiva (zerosis conjunctiva) dry cornia (xerosis cornea) keratomalacia bitot spots follicular hyperkeratosis ```
52
Major Jones Criteria for rheumatic fever
``` Joints - polyarthritis O (Heart) - carditis Nodules - subcutaneous nodules Erythema marginatum Syndam chorea --> this is alone enough to make dx of RF ``` Polyarthritis is the most common finding
53
murmur of VSD vs. ASD
VSD = harsh, blowing holosystolic murmur best heard alonf the left lower sternum ASD = fixed splitting of S2
54
Fanconi syndrome
diminished reabsorption of solutes by the proximal tubule SX: - dehydration - polyuria, polydipsia - hypokalemia - proteinuria - hypophosphatemia, hypocalcemia - hyperchloremic metabolic acidosis (defect in bicarb reabsorption) --> high urine pH
55
Minimal change disease sx? what does renal bx look like?
T cell mediated injury to podocytes -most common cause of nephrotic syndrome in children age 2-3 DX: renal biopsy without microscopic changes --> can see diffuse effacement of foot processes of podocytes on EM TX: steroids
56
Thickened BM and subepithelial "spikes" what am I?
Membranous glomerulonephritis -common nephrotic syndrome in adults, rare in young children
57
McCune-Albright Syndrome
gonadotropin-independent precocious pubery presents with irregular cafe-au-lait macules and fibrous dysplasia of bone
58
Presentation of transposition of the great vessels in a new born
SINGLE S2 +/- VSD cyanosis within 24hrs of live
59
Trachoma
chlamydia serotypes A, B, and C -leading cause of blindness world wide SX: follicular conjunctivitis with concomitant nasopharyngeal infection TX: azithromycin
60
EBV infection
``` fever tonsillitis/pharyngitis +/- exudates posterior or diffuse cervical lymphadenopathy fatigue +/- hepatosplenomegaly +/- rash after AMOXiCILLIN ```

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