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Kendigs > Lung In Sickle Cell > Flashcards

Lung In Sickle Cell Flashcards

1
Q

leading cause of death

A

acute chest syndrome

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2
Q

major risk factor for sickle chronic lung disease

A

recurrent ACS

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3
Q

PFT of children with sickle cell

A

obstructive

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4
Q

elevated pulmonary capillary blood volume is a consequence of _____ which correlates to _______ airway obstruction

A

chronic anemia

increase

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5
Q

most severe form of SCD

A

HbSS

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6
Q

onset of symptoms in newborn

A

10-12 weeks

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7
Q

Acute chest syndrome
highest incidence

A

2 to 4 yo

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8
Q

50% of children will present with ACS before

A

10 yo

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9
Q

ACS is directly proportional to _______ count

inversely proportional to hgB ____

A

WBC

hgF

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10
Q

high risk factors in children

A

ANC >9 x 10^9/L
hgb <8.6 g/dL

previous acs
urti
non compliance to PENICILLIN

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11
Q

most frequent pathogen

A

chlamydia pneumonia

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12
Q

Features of acs

A

Chest pain
PRODUCTIVE cough
Dyspnea

others: fever, tachypnea, crackles and wheeze

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13
Q

essential to the diagnosis of acs

A

new infiltrate on cxr (lower & middle lobe)

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14
Q

pulmo function of sickle chronic lung disease

A

restrictive

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15
Q

common cause of sudden death in scld px with pulmonary hypertension

A

Pulmo embo
Cardiac arrythmia

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16
Q

causes of Sleep d/o breathing in scld

A

functional asplenia

extramedullary hematopoiesis

17
Q

diagnostic test for scld

A

HbS solubility
(unreliable in first 6 months)

18
Q

primary cause of death

A

respiratory failure from Pulmo embo or pneumonia

19
Q

management of acute chest syndrome

A
20
Q

tx for fev 1 less than 70%

A

bronchodilator

21
Q

more than 70% fev1

A

Exercise challenge test
cold air

22
Q

it is a ribonuclease inhibitor
blocks the synthesis of DNA
raising hgBF level

a NO donor also
reducing sickle cell adhesion
by reducing VCAM 1

A

hydroxyurea

Kendigs (24 decks)

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