Kawasaki Disease details explain in this , what else to say in forty words.pptx

Kawasaki

1. Kawasaki Disease Azhar Mahmood 28Bi

2. What is Kawasaki Disease? Kawasaki disease or mucocutaneous lymph node syndrome is an acute, febrile disease that is most often seen in boys younger than 5 years. Kawasaki disease (KD) is an acute febrile vasculitic syndrome of early childhood that, although it has a good prognosis with treatment, can lead to  death  from coronary artery  aneurysm (CAA) in a very small percentage of patients. The disorder has also been called mucocutaneous lymph node syndrome and infantile periarteritis nodosa.

3. Pathophysiology

4. CAUSE Genetic factors.  Siblings of affected children have a 10-20 times higher probability of developing Kawasaki disease than the general population, and children in Japan whose parents had Kawasaki disease seem to have a more severe form of the disease and to be more susceptible to recurrence. Infection.  Features of Kawasaki disease that are consistent with an infectious etiology include the occurrence of epidemics primarily in late winter and spring with 3-year intervals and the wavelike geographic spread of those epidemics; the self-limited nature of the disease; and the characteristic fever, adenopathy , and  eye  signs.

5. Clinical Manifestations The clinical presentation of Kawasaki disease varies over time, with the clinical course conventionally divided into three stages: acute, subacute, and convalescent.

6. Acute Febrile Stage The acute stage begins with an abrupt onset of fever and lasts approximately 7-14 days. Irritability Non exudative bilateral conjunctivitis (90%) Anterior uveitis (70%) Perianal erythema (70%) Erythema and  edema  on the hands and feet; the latter impedes  ambulation Strawberry tongue and lip fissures Hepatic, renal, and GI dysfunction Myocarditis and pericarditis Lymphadenopathy (75%), generally a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm

7. Subacute Stage The subacute stage begins when the fevers have abated, and it continues until week 4-6; the hallmarks of this stage are desquamation of the digits, thrombocytosis (the platelet count may exceed 1 million/ μ L), and the development of coronary aneurysms; the risk for sudden death is highest at this stage.

8. Convalescent stage The convalescent phase is marked by the complete resolution of clinical signs of the illness, usually within 3 months of presentation; this stage begins with the return to baseline of the acute phase reactants (eg, erythrocyte sedimentation rate, C-reactive protein) and other laboratory abnormalities; during this stage, most of the clinical findings resolve; however, deep transverse grooves across the nails (Beau lines) may become apparent 1-2 months after the onset of fever.

9. Physical Examination Changes in the peripheral extremities. Polymorphous rash (not vesicular).  Usually generalized but may be limited to the groin or lower extremities. Oropharyngeal changes.  Erythema, fissuring, and crusting of the lips; strawberry tongue; diffuse mucosal injection of the oropharynx. Conjunctivitis.  Bilateral, nonexudative , painless bulbar conjunctival injection. Lymphadenopathy.  Acute nonpurulent cervical lymphadenopathy with lymph node diameter greater than 1.5 cm, usually unilateral.

10. Assessment And Diagnostic Findings Urine  proteins.  More recently, 2 urine proteins hold promise as biomarkers of Kawasaki disease: meprin A or filamin C. CBC.  On complete blood counts (CBCs), mild-to-moderate normochromic   anemia  is observed in the acute stage; the white blood cell count (WBC) is moderate to high (50% of patients have a WBC greater than 15,000/µL), with a left shift, which is a predominant sign of immature and mature granulocytes. Platelet count.  During the subacute stage, thrombocytosis is the outstanding marker; the platelet count begins to rise in the second week and continues to rise during the third week.

11. Cholesterol .  Serum cholesterol, high-density lipoprotein, and apolipoprotein A levels are decreased. Echocardiography.  Echocardiography is the study of choice to evaluate for coronary artery aneurysms (CAAs), in both fully manifested and suspected incomplete cases of Kawasaki disease. Imaging studies.  Magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and ultrafast computed tomography (CT) scanning are other noninvasive tests that can be used to evaluate coronary artery abnormalities. Electrocardiography.  On electrocardiography ( ECG ), tachycardia, prolonged  PR interval , ST-T wave changes, and decreased voltage of R waves may indicate myocarditis; Q waves or ST-T wave changes may indicate  myocardial infarction . Cardiac enzymes.  Cardiac enzyme levels (eg, creatine kinase [CK], creatine kinase myocardial band [CK-MB], cardiac troponin, lactate dehydrogenase [LD-1 >LD-2]) are elevated during a  myocardial infarction .

12. Pharmalogical Therapy Intravenous immunoglobulin.  IVIG relieves acute inflammation and has been shown to reduce the rate of coronary aneurysms . Aspirin .  Aspirin has a synergistic effect with IVIG and has long been a standard part of therapy for Kawasaki disease. Other adjunctive agents.  In addition to their use in treatment of IVIG-resistant Kawasaki disease, corticosteroids have been proposed as part of primary therapy. Anticoagulant therapy.   Anticoagulants  such as warfarin and low molecular weight  heparin  are used in patients with large aneurysms in whom the risk of  thrombosis  is high. The goal is to maintain an international normalized ratio (INR) of 2-2.5.

13. Prognosis KD is an acute, self-limiting illness. The immediate outcome has improved dramatically, with a decrease in the frequency of coronary artery aneurysms to less than 3% following the introduction of intravenous immunoglobulin (IVIG) therapy. Overall, the mortality rate is less than 0.5%.