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Pediatrics Flashcards

1
Q

Eisenmenger syndrome

A

left to right shunts lead to pulmonary HTN and and shunt reversal

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2
Q

PDA is associated with

A

congenital rubella

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3
Q

Ebstein abnormality is associated with

A

maternal lithium use

*** ebstein abnormality is apical displacement of the tricupsid valve

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4
Q

MC cyanotic heart defect in children

A

Tetralogy of fallot

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5
Q

Tetralogy of fallot components

A
  1. )pulmonary stenosis
  2. )overriding aorta
  3. )VSD
  4. ) right ventricular hypertrophy (becauce increased pressure to push into the left ventricle and the pulmonary stenosis)
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6
Q

3 holosystolic murmurs

A

mitral regurgitation
tricuspid regurgitation
ventricular septal defect

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7
Q

MC cyanotic heart defect in neonates

A

transposition of the great vessels

risk factors: diabetic mothers and DiGeorge syndrome

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8
Q

Hypoplastic left heart syndrome Sxs

A

Sxs: absent pulses, right ventricular heave (from Right ventricular hypertrophy), cyanosis
***dependent on PDA

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9
Q

Truncus arteriosus

A

major problem: pulmonary HTN

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10
Q

PDA dependent cyanotic heart defects

A

transposition of the great vessels and hypoplastic left heart syndrome
***must get prostaglandin E1

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11
Q

MC heart defect in children

A

VSD

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12
Q

VSD presentation

A
  • dyspnea with distress (because the pulmonary arteries are already maximally dialted)
  • loud pulmonic S2 (because the increased pressure in the pulmonary artery slams the pulmonary valve shut)
  • High-pitched holosystolic murmur
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13
Q

VSD and ASD best initial test and best diagnostic test

A

best initial: echo

most diagnostic: cardiac catheterization.

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14
Q 
2 month developmental milestones:
gross motor:
Fine motor:
language:
social/cognitive:
A

gross motor: lifts head/ chest when prone
Fine motor: tracks past midline
language: alerts to sound and coos
social/cognitive: recongized parent; exhibits social smile

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15
Q 
4 month developmental milestones:
gross motor:
Fine motor:
language:
social/cognitive:
A

gross motor: roll
Fine motor:grasps rattle
language:laugh and squeals
social/cognitive: enjoys looking around, laughs

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16
Q 
6 month developmental milestones:
gross motor:
Fine motor:
language:
social/cognitive:
A

gross motor: sits unassisted
Fine motor: transfers objects, raking grasp
language: babbles
social/cognitive:STRANGER ANXIETY

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17
Q 
9 month developmental milestones:
gross motor:
Fine motor:
language:
social/cognitive:
A

gross motor: crawls, pulls to stand
Fine motor: Uses 3-finger pincer grasp
language: mama/dada
social/cognitive:waves bye bye; plays pat-a-cake

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18
Q 
1 year developmental milestones:
gross motor:
Fine motor:
language:
social/cognitive:
A

gross motor: walks alone, throws objects
Fine motor:uses 2-finger grasp
language: uses 1-3 words
SEPARATION ANXIETY, follows one step commands

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19
Q 
2 year developmental milestones:
gross motor:
Fine motor:
language:
social/cognitive:
A

gross motor: walks up and down steps; jumps
Fine motor: builds a tower of 6 cubes
language: uses 2 word phrases
social/cognitive: follows 2 step commands; removes clothes

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20
Q 
3 year developmental milestones:
gross motor:
Fine motor:
language:
social/cognitive:
A

gross motor:rides tricycle
Fine motor: copies a circle; uses utensils
language: uses 3 word sentences
social/cognitive: brushes teeth with help; washes/dries hands

21
Q

Kallmann syndrome

A

look up

22
Q

Causes of pathologic puberty delay

A

malnutrition, klinefelter syndrome, turner syndrome, hypopituitarism, hypothyroidism, Kallmann syndrome, androgen insensitivity syndrome, prader-willi syndrome.

23
Q

Fabry disease
mechanism:
Sxs:

A

mechanism: deficiency of alpha-galactosidase A
Sxs: Neuropathic limb pain
angiokeratomas and telangiectasias
renal failure and increased risk of thromboembolic events
***X linked recessive

24
Q

Krabbe disease
mechanism:
Sxs:

A

mechanism: absence of galactosylceramide
Sxs: progressive CNS degeneration, optic atropy, spasticity and death within first 3 years of life

25
Q

Gaucher disease
mechanism:
Sxs:

A

mechanism: deficiency of glucocerebrosidease
Sxs: anemia and thrombocytopenia

26
Q

Niemann-Pick disease
mechanism:
Sxs:

A

mechanism: deficiency of sphingomyelinase
Sxs: cherry-red spot and hepatosplenomegaly

27
Q

Tay-Sachs disease
mechanism:
Sxs:

A

mechanism: absence of hexoaminidase
Sxs: Normal until 3 to 6 months, weakness and begins and development slows and regressess
cherry-red spot, no hepatosplenomegaly

28
Q

Metachromatic leukodystrophy
mechanism:
Sxs:

A

mechanism: deficiency of arylsulfatase A
Sxs: Demyelination leads to progressive ataxia and dementia

29
Q

Hurler syndrome
mechanism:
Sxs:

A

mechanism:deficiency of alpha-l-iduronidase
Sxs: corneal cloudy, intelectual disabilities, and gargoylism

30
Q

Hunter syndrome
mechanism:
Sxs:

A

mechanism: deficiency of iduronate sulfatase
Sxs: milder form of hurler syndrome that doesn’t have corneal cloudy and has only mild intellectual disabilities.
*** X-linked recessive (unlike hurler which is autosomal recessive)

31
Q

MC cause of pulmonary infections in CF

A

pseudomonas and staph aureus

32
Q 
pyloric stenosis
correlations: 
timeline:
Dx:
TX:
A

correlations: first born infant boys, associated with tracheoesophagral fistula, formula feeding and maternal erythromycin use
timeline: 3 to 12 weeks old
Dx: Abdominal ultrasound is method of choice
TX: make NPO, establish IV access, correct dehydration, hypochloremia, and hypokalemic metabolic alkalosis. surgical correction

33
Q

Hirschsprung disease

Dx:

A

Dx: Barium enema, with rectal biopsy for confirmation

34
Q

malrotation with volvulus

Sxs:

A

presents in first month of life with bilious emesis, abdominal pain, distention, and passage of blood or mucus in the stool.

35
Q

pneumatosis intestinalis

A

intramural airbubble representing gas produced by bacteria within the bowel wall
-pathognomonic for necrotizing enterocolitis

36
Q

kowasaki diagnosis

A 
Symptoms:
5 days of fever + 4/5
1.) conjunctivitis
2.) strawberry tongue
3.) truncal polymorphous erythmatous rash
4.) desquamating rash on hands and feet
5.) cervical lymphadenopathy 
*** children with scarlet fever have norma lips and no conjunctivitis

Labs:
Acute phase: normochromic anemia, leukocytosis with a left shift, high ESR and CRP
Subacute phase: thrombocytosis. ESR and CRP gradually decrease

37
Q

Still disease

A

recurrent fever >102.2, hepatosplenomegaly, and salmon colored macular rash
joint inflammation may not occur for months to years later

38
Q

Chediak higashi

A

defect in neutrophil chemotaxis
Sxs: partial albinism, peripheral neuropathy, and neutropenia
increased incidence of overwhelming pyogenic infections with stroptococcus pyogenes, s aureus, and pseudomonas species

39
Q

croup
age:
pathogen:
Tx:

A

age: 3 months to 3 years
pathogen: parainfluenza virus
Tx: racemic epineprine

40
Q

epiglottitis
age:
pathogen:
Tx:

A

age: 3 to 7 years
pathogens: H influenzae, S pneumoniae
Sxs: drooling and tripod positioning
Tx: SECURE AIRWAY, then give antibiotics

41
Q

menengitis TX for neonates vs children

A

neonates: ampicillin and cefotaxime or gentamicin
children: ceftriaxone and vancomycin
* **both: acyclovir

42
Q

chlamydial conjunctivits Tx

A

Topical AND oral erythromycin

43
Q

gonococcal conjunctivitis Tx

A

IV/IM third generation cephalosporin ( corneal ulceration and scaring can occur in 24 to 48 hours if left untreated)

44
Q

pertussis tx and close contact prophylaxis

A

Tx: 10 days of azithromycin
prophylaxis: 2 days of azithromycin

45
Q

Increased bilirubin production in newborns, causes

A 
ABO or Rh incompatability
G6PD deficiency
sickle cell anemia, hereditary spherocytosis
thalassemias
sepsis with DIC
46
Q

increased conjugated bilirubin in newborns causes

A

Dubin-johnson syndrome

rotor syndrome

47
Q

MC childhood maignancy

A

ALL
general malaise, thenbone pain with limp, fever (from neutropenia), anemia, ecchymoses, petechiae, and / or hepatosplenomegaly

48
Q

Ewing sarcoma vs ostesarcoma

A

ewing sarcoma: systmeic symptoms (fever, anorexia fatgiue) midshaft of long bones, “onion skin”

osteosarcoma: systemic symptoms are rare, metaphyses of long bones, increased alkaline phosphatase (from osteoblast origin), “sunburst” lytic bone lesions

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