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Question flashcards

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
Q

Minimal change disease

A

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
Q

Rotavirus vaccine is given….

A

given between 2-8 months

27
Q

McCune-Albright Syndrome

A

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
Q

Immune Thrombocytopenia

A
  • 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
Q

Laryngomalacia

A

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
Q

Riboflavin deficiency (B2)

A
  • due to poor intake, poor absoption, probenacid, oral contraceptive use.
  • SX: cheliosis, glossitis, ocular problems (keratitis, conjunctivits, corneal vascularization), and seborrheic dermatitis
31
Q

cheliosis, glossitis, ocular problems (keratitis, conjunctivits, corneal vascularization), and seborrheic dermatitis

which deficiency am I?

A

B2, riboflavin

32
Q

Vitamin B6 (pyridoxine) deficiency

A

-enzyme deficiency vs. poor absorption vs. isoniazid, steroids, anticonvulsants

SX: seizures, peripheral neuritis, dermatitis, microcytic anemia

33
Q

seizures, peripheral neuritis, dermatitis, microcytic anemia

which deficiency am I?

A

Vitamin B6 (pyridoxine) deficiency

34
Q

Folate deficiency

A

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
Q

megaloblastic anemia, glossitis, pharyngeal ulcers, impaired immunity

which deficiency am I?

A

Folate deficiency

36
Q

Kawasaki disease

A

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
Q

evaluation of Malrotation –> _____

A

upper GI series abd XR

38
Q

DDX of bilious emesis

A

meconium ileus
Hirschsprung disease
malrotation
duodenal atresia

39
Q

Evaluation of meconium ileus and hirschsprung disease?

A

contrast enema

40
Q

evaluation of duodenal atreasia

A

abd XR –> double bubble sign

41
Q

Iron poisoning –> what sx?

A

abdominal pain, vomiting, diarrhea, hypotensive shock

  • Anion gap metabolic acidosis
  • -> hepatic necrosis and pyloric stenosis

TX: deferoxamine, whole bowel irrigation

42
Q

Di George Syndrome

A

CATCH -22

Conotruncal cardiac defects
Abnormal facies
Thympic aplasia/hypoplasia
Cleft palate
Hypocalcemia

*Defective development of pharyngeal pouches
Chr 22 q11.2 deletion

43
Q

Sickle cell disease patients are at high risk for what bugs causing sepsis?

A

1) Strep pneumoniae
2) Haemophilus influenzae
3) Neisseria meningitidis

44
Q

Wilms tumor is associated with what syndromes?

A

WAGR
Beckwith Wiedemann syndrome
Denys-Drash syndrome

45
Q

WAGR

A

wilms tumor, aniridia, GU abnormalities, intellectual disability (retardation)

46
Q

Wilms tumor sx

A

asymptomatic, firm, smooth abdominal mass that does not cross midline

47
Q

Types of renal tubular acidosis

A

1) distal - poor hydrogen secretion into urine
2) proximal - poor bicarbonate resorption
4) aldosterone resistance

48
Q

Type 1 distal RTA

A

poor hydrogen secretion into urine

urine pH > 5.5
low-normal serum potassium

CAUSES:

  • genetic disorders
  • med toxicity
  • autoimmunie disorders
49
Q

Type 2 (proximal) RTA

A

poor bicarbonate resorption

urine pH < 5.5
low normal potassium

CAUSES:
-fanconi sydrome (glucosuria, phosphaturia, aminoaciduria)

50
Q

Type 4 RTA

A

aldosterone resistance

urine pH < 5.5

HIGH serum potassium

CAUSES:

  • obstructive uropathy
  • CAH
51
Q

Vitamin A deificiency

A 
impaired adaptation to darkness
photophobia
dry scaly skin
dry conjunctiva (zerosis conjunctiva)
dry cornia (xerosis cornea)
keratomalacia
bitot spots
follicular hyperkeratosis
52
Q

Major Jones Criteria for rheumatic fever

A 
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
Q

murmur of VSD vs. ASD

A

VSD = harsh, blowing holosystolic murmur best heard alonf the left lower sternum

ASD = fixed splitting of S2

54
Q

Fanconi syndrome

A

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
Q

Minimal change disease

sx?
what does renal bx look like?

A

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
Q

Thickened BM and subepithelial “spikes”

what am I?

A

Membranous glomerulonephritis

-common nephrotic syndrome in adults, rare in young children

57
Q

McCune-Albright Syndrome

A

gonadotropin-independent precocious pubery

presents with irregular cafe-au-lait macules and fibrous dysplasia of bone

58
Q

Presentation of transposition of the great vessels in a new born

A

SINGLE S2 +/- VSD

cyanosis within 24hrs of live

59
Q

Trachoma

A

chlamydia serotypes A, B, and C

-leading cause of blindness world wide

SX: follicular conjunctivitis with concomitant nasopharyngeal infection

TX: azithromycin

60
Q

EBV infection

A 
fever
tonsillitis/pharyngitis +/- exudates
posterior or diffuse cervical lymphadenopathy
fatigue
\+/- hepatosplenomegaly
\+/- rash after AMOXiCILLIN

Peds (2 decks)

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