Lung In Sickle Cell Flashcards
leading cause of death
acute chest syndrome
major risk factor for sickle chronic lung disease
recurrent ACS
PFT of children with sickle cell
obstructive
elevated pulmonary capillary blood volume is a consequence of _____ which correlates to _______ airway obstruction
chronic anemia
increase
most severe form of SCD
HbSS
onset of symptoms in newborn
10-12 weeks
Acute chest syndrome
highest incidence
2 to 4 yo
50% of children will present with ACS before
10 yo
ACS is directly proportional to _______ count
inversely proportional to hgB ____
WBC
hgF
high risk factors in children
ANC >9 x 10^9/L
hgb <8.6 g/dL
previous acs
urti
non compliance to PENICILLIN
most frequent pathogen
chlamydia pneumonia
Features of acs
Chest pain
PRODUCTIVE cough
Dyspnea
others: fever, tachypnea, crackles and wheeze
essential to the diagnosis of acs
new infiltrate on cxr (lower & middle lobe)
pulmo function of sickle chronic lung disease
restrictive
common cause of sudden death in scld px with pulmonary hypertension
Pulmo embo
Cardiac arrythmia
causes of Sleep d/o breathing in scld
functional asplenia
extramedullary hematopoiesis
diagnostic test for scld
HbS solubility
(unreliable in first 6 months)
primary cause of death
respiratory failure from Pulmo embo or pneumonia
management of acute chest syndrome
tx for fev 1 less than 70%
bronchodilator
more than 70% fev1
Exercise challenge test
cold air
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
hydroxyurea
