10.2 Kawasaki Disease, Sickle Cell Disease, G6PD Flashcards
(38 cards)
KD
Most common cause of __
Inflammation in __
Affect _______
Kawasaki disease, mucocutaneous lymph node syndrome
Acquired heart disease
Blood vessel, esp. coronary arteries
Mucous membranes, lymph nodes, walls of blood vessels, heart
Avg age of KD
2 years old
Most <5
Pathophysiology of KD
Initial stage:
Extensive inflammation of arteries, venules and capillaries
Later stage:
Formation of coronary artery aneurysms
Complication of KD
Develop coronary artery aneurysm
Disrupted BF, thrombotic occlusion
Myocardial infarction
Stenosis of aneurysm
Myocardial ischemia
Symptoms of acute MI
Abdominal pain
Vomiting
Restlessness
Inconsolable crying
Pallor
Shock
Diagnostic criteria of KD
Presence of fever >= 5 days + 4/5
- Bilateral conjunctival inflammation without exudation
- Change in extremity such as peeling, erythema, edema
- Change in oral mucous membrane such as erythema lips, reddening oropharyngeal, strawberry tongue
- Polymorphous rash
- Cervical lymphadenopathy (at least one node >1.5cm)
KD:
Clinical manifestations and phase
- Acute phase (wk 1-2)
- Abrupt onset of high fever
- Very irritable - Subacute phase (wk 2-4)
- Resolution of fever, until all signs disappear
- Irritability
- Risk of developing coronary artery aneurysm (EchoCG) - Convalescent phase (wk 4-6)
- all sign resolve
- lab result still abnormal
Diagnostic test for KD
*No single test, involve ruling out other disease
CBC, WBC
Inflammation: CPR, ESR
Serum albumin
Urinalysis
CXR
Echocardiogram (how blood flow inside heart and valves, coronary artery, myocarditis)
ECG (irregular heartbeat)
Therapeutic management for KD
- High dose IVIG
Reduce risk of coronary artery abnormalities
-Aspirin
Anti-inflammatory and antiplatelet effects
+/- Prednisone/ prednisolone (Steroid)
Nursing management for KD
-Monitor cardiac status
IO, daily weight
- Monitor congestive heart failure sign
Decrease UO, Abnormal heart sound
TF, respiratory distress - administration of IVIG and aspirin
- pain relief and promote comfort
Distraction method
-skin care
Tepid water bath, Cool, soft, loose clothing, Use unscented lotions
- mouth care
Lubricating ointment to lip, clear fluid and soft food - quiet environment reduce irritability
- respite care for family
Discharge teaching for KD
- Monitor Body temp
- Management of irritability
- Continue aspirin
- Monitor side effect e.g. potential bleeding, avoid contact activities
- monitor complication e.g. passive ROM if arthritis, follow-up echoCG for s/s cardiac ischemia and heart-related problem
- defer live immunizations for 11 months after IVIG
G6PD deficiency
The most common _____ in human
__ linked disorder
Function of G6PD
Enzymatic disorder of RBC
*X-linked disorder
To maintain integrity of RBCs
Pathophysiology of G6PD
Susceptible to oxidant-induced hemolysis
Display signs until trigger
- RBC breakdown, haemolytic (not chronic)
- Bilirubin produce, jaundice
- Excessive bilirubin accumulate in brain (irreversible damage, hearing loss, intellectual disabilities, death)
G6PD: trigger for haemolytic
Stress
Infection
Drug
Chemical (naphthalene in mothballs)
Food (fava beans)
Clinical manifestations of G6PD
Majority are asymptomatic
Depend degree of enzyme deficiency
Likehood develops NNJ / chronic hemolysis
- Jaundice
- hemolytic crisis
- Anemia
- Pale looking
- sclera turns yellow
- dark-color urine
Newborn: refuse feeding, lacking energy, excessively sleepy
Child Adult: pale, complain tiredness, SOB, rapid HR RR
Diagnostic test for G6PD
G6PD deficiency: peripheral blood smear
Hemolysis: reticulocyte count, indirect/ direct bilirubin count, urinalysis for hematuria
Splenomegaly: UGS
Neonatal screening programs screen for
Congenital hypothyroidism
G6PD deficiency
Assessment for G6PD deficiency
Skin, urine color, spleen palpation, neurological status, VS O2
Management for G6PD deficiency
Lifelong
Avoidance of oxidative stress to RBC
Treat jaundice
Transfusions for severe anemia
- Avoidance of unsafe drugs and chemical (Chinese herbal medicines, mothballs)
- Dietary restrictions
- Family education (alert card)
Sickle cell disease
Atypical haemolytic molecule called __
RBC = __ shaped
Chronic deterioration of multiple organ systems due to ___ of RBC
Hemoglobin S
Sickle, crescent, half moon
Sickling
*Sickling aggravated by
Stress, infection, fever, acidosis, dehydration, physical exertion, excessive hot/ cold exposure, hypoxia
Pathophysiology of sickle cell disease
- RBC hard and inflexible, blood become viscous
- clump together,
block small capillaries and venules,
Local tissue hypoxia/ anoxia
Ischemia, may infraction
Complication of sickle cell disease
Limbs and bones, brain, liver, kidney, spleen, heart, lungs, blood, skin, penis, eyes, general
Splenic sequestration 脾臟阻斷
Acute chest syndrome
Vaso-occlusive pain crisis
FLACC pain assessment tools
Indication
Range
Face, legs, activity, cry, consolability
2 months to 7 years old
(0,1,2)x5
0=no pain 10=the worst pain
