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Kawasaki Disease

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Common disease in japan and even in asia.

Published in: Health & Medicine

Kawasaki Disease

  1. 1. Crisbert I. Cualteros, M.D. 1 st Year Resident
  2. 2. Objectives: <ul><li>To present a case of a 2yr. old female Infant with Kawasaki Disease </li></ul><ul><li>To discuss briefly the epidemiology, pathophysiology, clinical manifestations, diagnostics, complication, management, and prognosis of Kawasaki Disease </li></ul>
  3. 3. Patient’s Profile <ul><li>P.T., 2yo and 1month, female, Infant, Filipino, admitted due to swollen lips </li></ul><ul><li>G1P0, regular PNC </li></ul><ul><li>No maternal illness. </li></ul><ul><li>Patient was delivered preterm (36 weeks AOG) via NSD at CDH. BW: 5lbs </li></ul><ul><li>admitted at the NICU for sepsis </li></ul>
  4. 4. <ul><li>BF for 2 months, then mixed fed up to 6mos. </li></ul><ul><li>Semi-solid at 6 months </li></ul><ul><li>Solid food at 9 months </li></ul><ul><li>Complete EPI </li></ul>
  5. 5. <ul><li>No medical problem </li></ul><ul><li>No food and drug allergies </li></ul><ul><li>HFD: HPN & BA </li></ul><ul><li>Jan 2009 – CDH – Acute Gastroenteritis </li></ul>
  6. 6. HPI: <ul><li>10 days PTA, patient had cough associated with fever, temporarily relieved by Paracetamol </li></ul><ul><li>9 days PTA, sought consult with private pediatrician, was given Amoxicillin, Cetirizine and & Salbutamol </li></ul><ul><li>CBC taken was unremarkable </li></ul>
  7. 7. <ul><li>3 days PTA, pain on ambulation and swelling of both ankle </li></ul><ul><li>Consult with pediatrician, was given Multivitamins and Ibuprofen which afforded relief of pain and fever </li></ul><ul><li>Repeat CBC showed thrombocytosis (581T) and elevated CRP (85 mg/dL ) </li></ul>
  8. 8. <ul><li>Night PTA, consult at CDUH-OPD due to swelling of right ankle and swollen lips </li></ul><ul><li>Ibuprofen was continued </li></ul><ul><li>Pt was referred to a Pediatric - Cardiologist for further work up </li></ul><ul><li>Admitted </li></ul>
  9. 9. Physical Examination: <ul><li>Conscious, not irritable, afebrile, not in respiratory distress: </li></ul><ul><li>BP: 100/60 HR:124 RR:20 T:37.3C </li></ul><ul><li>WT: 13.8 kgs </li></ul><ul><li>No pallor, No rashes </li></ul><ul><li>Normocephalic, PPC, AIS, no conjunctivitis, moist tongue, but pinkish & swollen lips </li></ul>
  10. 10. <ul><li>Neck: no LAD </li></ul><ul><li>C/L: Equal chest expansion, clear breath sounds </li></ul><ul><li>CVS: distinct heart sounds, NRRR, no murmur </li></ul><ul><li>ABD: flabby, NABS, soft, no tenderness </li></ul>
  11. 11. <ul><li>GUT: grossly female </li></ul><ul><li>EXT: no deformities, swollen both ankles noted, no LOM </li></ul>
  12. 12. <ul><li>Admitting Impression : </li></ul><ul><li>Atypical Kawasaki Disease </li></ul>
  13. 13. Course In the Ward <ul><li>On Admission: </li></ul><ul><li>Diet for age </li></ul><ul><li>IVF at MR </li></ul><ul><li>IVIG test dose: (2 gm/Kg/Day ) </li></ul><ul><li>To give 8cc to run in 1hr </li></ul><ul><li>To give 16cc to run in 1hr </li></ul><ul><li>To give 33cc to run in 1hr </li></ul>
  14. 14. <ul><li>After the 3 rd dose was consumed </li></ul><ul><li>IVIG was given at 60 cc/hr to run in 9hrs </li></ul><ul><li>Aspirin 60 mg PO OD mixed with water (AD: 4.3 mkD) </li></ul>
  15. 15. 1 st Hospital Day: <ul><li>S: Afebrile, lips not swollen, no headaches, </li></ul><ul><li>O: BP:90/60 HR:110 RR:21 T:36.9 </li></ul><ul><li>No conjunctivitis, no strawberry tongue, lips not swollen </li></ul><ul><li>no LOM of ankle noted </li></ul>
  16. 16. <ul><li>A: Pt is improving </li></ul><ul><li>P: Revised to Aspirin 40 mg PO OD mixed with water (2.9 mkD) </li></ul><ul><li>For 2 d echo with doppler </li></ul>
  17. 17. 2D-Echo
  18. 18. 2 nd Hospital Day: <ul><li>Terminate IVF </li></ul><ul><li>THM: </li></ul><ul><li>Aspirin 40 mg OD PO for 2 months </li></ul><ul><li>For follow up with her Pediatrician 1 month after discharge </li></ul><ul><li>For repeat 2D-echo after 2weeks </li></ul><ul><li>Repeat CBC & ECG after 2mos </li></ul>
  19. 19. Final Diagnosis <ul><li>Incomplete Kawasaki Disease </li></ul>
  20. 20. <ul><li>AKA: mucocutaneous LN syndrome or infantile polyarteritis nodosa </li></ul><ul><li>is an acute febrile vasculitis of childhood first described by Dr. Tomisaku Kawasaki in Japan in 1967 </li></ul>
  21. 21. Dr. Tomisaku Kawasaki
  22. 22. <ul><li>20% of untreated patients: </li></ul><ul><li>aneurysms, which may lead to coronary artery thrombosis or stenosis </li></ul><ul><li>myocardial infarction </li></ul><ul><li>aneurysm rupture </li></ul><ul><li>sudden death </li></ul>
  23. 23. Incidence: <ul><li>US: 3000 cases diagnosed yearly </li></ul><ul><li>Is higher in Asian than in other racial groups </li></ul><ul><li>Japan: almost 200,000 cases reported since the 1960s </li></ul>
  24. 24. Etiology: <ul><li>unknown </li></ul><ul><li>Hypothesis: Bacterial Superantigen Toxin causes KD </li></ul><ul><li>occurs only in genetically predisposed hosts </li></ul><ul><li>virtual absence of cases in adults may be due to widespread immunity. </li></ul>
  25. 25. Pathogenesis <ul><li>KD causes a severe vasculitis of all blood vessels with predilection for the coronary arteries </li></ul><ul><li>Pathologic examination of fatal cases in KD: edema of endothelial and smooth muscle cells with intense inflammatory infiltration of the vascular wall, initially by PMN cells but rapidly followed by macrophages, lymphocytes and plasma cells </li></ul>
  26. 26. <ul><li>Affected vessels involves inflammation of 3 layers of the vascular wall with destruction of the internal elastic lamina </li></ul><ul><li>Vessel loses its structural integrity and weakens resulting in dilatation or saccular/fusiform aneurysm formation </li></ul>
  27. 28. Phases of KD <ul><li>Acute febrile phase - 1-2 weeks </li></ul><ul><ul><li>Febrile </li></ul></ul><ul><ul><li>Irritable </li></ul></ul><ul><ul><li>Bilateral conjunctivitis and rash </li></ul></ul><ul><ul><li>Erythema & edema of hands & feet </li></ul></ul>
  28. 29. <ul><ul><li>The tongue and oral mucosa become red and cracked. </li></ul></ul><ul><ul><li>Hepatic dysfunction may develop. </li></ul></ul><ul><ul><li>Cardiac complications noted in the first stage include myocarditis and pericarditis. </li></ul></ul>
  29. 30. Recrudescent Kawasaki disease <ul><li>small group of patients who do not respond to therapy </li></ul><ul><li>Defined: fever beyond the 36-hour mark from completion of the 12-hour IVIG infusion. </li></ul>
  30. 31. <ul><li>Subacute phase: </li></ul><ul><ul><li>Fever resolution </li></ul></ul><ul><ul><li>end by the 4th week. </li></ul></ul><ul><ul><li>persistent irritability, anorexia & conjunctival injection </li></ul></ul>
  31. 32. <ul><ul><li>If fever persists, a greater risk of cardiac complications </li></ul></ul><ul><ul><li>Thrombocytosis (may exceed 1 million) </li></ul></ul><ul><ul><li>Desquamation of the fingertips and toes begins at this time. </li></ul></ul><ul><ul><li>Aneurysm formation may occur </li></ul></ul>
  32. 33. <ul><li>Convalescent phase: 4-6 weeks </li></ul><ul><ul><li>all signs of illness have disappeared </li></ul></ul><ul><ul><li>Acute Phase Reactants normalized </li></ul></ul><ul><ul><li>presence of coronary artery aneurysms. </li></ul></ul>
  33. 34. <ul><li>Chronic phase </li></ul><ul><ul><li>important only in patients with cardiac complications </li></ul></ul><ul><ul><li>aneurysm formed in childhood may rupture in adulthood </li></ul></ul>
  34. 35. Diagnostic Criteria for Kawasaki Disease <ul><li>Fever lasting for at least 5 days * </li></ul><ul><li>Presence of at least four of the following five signs: </li></ul><ul><li>1. Bulbar conjunctival injection </li></ul><ul><li>2.Changes in the mucosa </li></ul><ul><li>3.Edema/erythema of the hands/feet </li></ul><ul><li>4.Polymorphous, nonvesicular rash (truncal) </li></ul><ul><li>5.Cervical LAD ≥1.5 cm (unilateral)    Illness not explained by other known disease process </li></ul>
  35. 36. 1. Bilateral bulbar conjunctival injection
  36. 37. 2. Changes in the mucosa strawberry tongue Fissured/ crack lips
  37. 38. 3. Edema/erythema of the hands/feet
  38. 39. 4. Rash
  39. 40. 5. Cervical LAD ≥1.5 cm (unilateral)
  40. 42. <ul><li>Incomplete Kawasaki Disease: </li></ul><ul><li>lack sufficient clinical signs of the disease to fulfill the classic criteria </li></ul><ul><li>do not demonstrate atypical clinical features </li></ul><ul><li>more common in young infants than in older children </li></ul><ul><li>Atypical Kawasaki Disease: </li></ul><ul><li>reserved for pts with problem, such as renal impairment </li></ul><ul><li>is more common in young infants than in older children </li></ul>
  41. 43. Laboratory Findings <ul><li>No specific diagnostic test for Kawasaki disease exists </li></ul><ul><li>CBC : WBC is normal to elevated, with a predominance of neutrophils and immature forms </li></ul><ul><li>Plt is generally normal in the 1st wk of illness and rapidly increases by the 2nd–3rd wk of illness </li></ul><ul><li>Normocytic anemia is common </li></ul>
  42. 44. <ul><li>Elevated ESR, CRP are present in acute phase of illness and persist for 4–6 wk </li></ul><ul><li>Sterile pyuria </li></ul><ul><li>mild elevations of the hepatic transaminases </li></ul><ul><li>CSF pleocytosis may be present . </li></ul>
  43. 45. 2-D Echo <ul><li>Done at diagnosis and again after 2–3 wk of illness </li></ul><ul><li>If normal, a repeat study at 6–8 wk after onset of illness </li></ul><ul><li>If coronary abnormalities are not detected by 6–8 wk after onset of illness and the ESR has normalized, additional follow-up studies are optional </li></ul>
  44. 46. 2d-echo <ul><li>Some centers routinely perform again 1 yr after onset of illness </li></ul><ul><li>However, KD is an acute vasculitis; there is no convincing evidence of long-term cardiovascular sequelae in children who do not develop coronary abnormalities within 2 mo after the onset of illness </li></ul><ul><li>For patients who develop coronary artery abnormalities, more frequent 2D echo studies and potentially angiography may be indicated. </li></ul>
  45. 47. Treatment <ul><li>Patients with acute KD should be treated with IVIG and high-dose aspirin ideally within 10 days of disease onset </li></ul><ul><li>MOA of IVIG is unknown, but treatment results in rapid defervescence and resolution of clinical signs of illness in most patients </li></ul><ul><li>Decrease in coronary disease from 20–25% treated with aspirin alone to 2–4% if treated with IVIG + aspirin </li></ul>
  46. 48. Treatment of Kawasaki Disease <ul><li>ACUTE STAGE </li></ul><ul><li>   IVIG 2g/kg over 10–12hr with aspirin 80–100 mg/kg/24hr divided every 6hr orally until 14th day of illness </li></ul><ul><li>CONVALESCENT STAGE </li></ul><ul><li>    Aspirin 3–5mg/kg once daily orally until 6–8 wk after illness onset    </li></ul>
  47. 49. <ul><li>LONG-TERM THERAPY FOR THOSE WITH CORONARY ABNORMALITIES    Aspirin 3–5mg/kg OD orally ± clopidogrel 1mg/kg/Day (max:75 mg/day) </li></ul><ul><li>ACUTE CORONARY THROMBOSIS    Prompt fibrinolytic therapy with tissue plasminogen activator, streptokinase, or urokinase under supervision of a pediatric cardiologist </li></ul>
  48. 50. <ul><li>No response to an initial IVIG infusion or only a partial response </li></ul><ul><ul><li>Re-treatment: additional infusion of IVIG, 2 g/kg </li></ul></ul><ul><li>Corticosteroids should be reserved for patients with persistent fever following two 2 g/kg infusions of IVIG </li></ul><ul><ul><li>Methylprednisolone 30mg/kg/D for 3days </li></ul></ul>
  49. 51. <ul><li>Patients with a small solitary aneurysm should continue aspirin indefinitely </li></ul><ul><li>Patients with larger/numerous aneurysms may require the addition of clopidogrel, warfarin or LMWH </li></ul><ul><li>Acute thrombosis may occasionally occur in an aneurysmal coronary artery </li></ul><ul><li>Abciximab is used in some patients who develop giant coronary aneurysms or possible thrombosis </li></ul>
  50. 52. Long-term follow-up of patients with aneurysms include: <ul><li>echocardiography </li></ul><ul><li>stress testing </li></ul><ul><li>possibly angiography </li></ul><ul><li>Catheter intervention with: </li></ul><ul><li>percutaneous transluminal coronary rotational ablation </li></ul><ul><li>directional coronary atherectomy </li></ul><ul><li>stent implantation </li></ul><ul><li>are promising therapeutic strategies for the management of coronary stenosis caused by KD </li></ul>
  51. 53. <ul><li>Patients on long-term aspirin therapy are candidates for influenza vaccine to reduce the risk of Reye syndrome </li></ul><ul><li>The risk of Reye syndrome in children who take salicylates and who receive varicella vaccine is believed to be much lower than with wild-type varicella </li></ul>
  52. 54. <ul><li>Patients treated with 2 g/kg IVIG should have MMR and varicella vaccines delayed for 11 mo because the presence of specific antiviral antibody in IVIG may interfere with the immune response to parenteral live-virus vaccines. </li></ul>
  53. 55. Complications and Prognosis <ul><li>Recovery is complete and without apparent long-term effects for patients who do not develop coronary disease </li></ul><ul><li>Recurrent illness occurs in 1–3% of cases </li></ul><ul><li>Prognosis: 50% of coronary artery aneurysms resolve echocardiographically by 1–2 yr after the illness </li></ul><ul><li>Intravascular UTZ: resolved aneurysms are associated with marked intimal thickening and abnormal functional behavior of the vessel </li></ul>
  54. 56. AHA classifications of aneurysms: <ul><li>Small: <5 mm internal diameter </li></ul><ul><li>Medium: 5 to 8 mm internal diameter </li></ul><ul><li>Giant: >8 mm internal diameter </li></ul>AHA Journal by JW Newburger - 2004
  55. 57. <ul><li>Giant aneurysms are unlikely to resolve and most likely to lead to thrombosis/stenosis </li></ul><ul><li>CABG may be required if myocardial perfusion is significantly impaired </li></ul><ul><li>Heart transplantation has been required in rare cases in which revascularization is not feasible because of distal coronary stenosis or aneurysms or severe myocardial dysfunction </li></ul>
  56. 58. Risk Scores for Predicting Aneurysms (Harada score) <ul><li>used to determine whether IVIG treatment will be used </li></ul><ul><li>IVIG is given to children who fulfill 4 of the following criteria, assessed within 9 days of onset of illness: </li></ul><ul><li>(1) WBC >12 000/mm3 </li></ul><ul><li>(2) platelet count <350 000/mm3 </li></ul><ul><li>(3) CRP >3+ </li></ul>AHA Journal by JW Newburger - 2004
  57. 59. <ul><li>(4) HCT <35% </li></ul><ul><li>(5) albumin <3.5 g/dL </li></ul><ul><li>(6) age 12 months </li></ul><ul><li>(7) male sex </li></ul><ul><li>For children with <4 risk factors but continuing acute symptoms, the risk score is reassessed daily </li></ul>AHA Journal by JW Newburger - 2004
  58. 60. <ul><li>Because of the imperfect performance of scoring systems, all patients who are diagnosed with Kawasaki disease should be treated with IVIG. </li></ul>
  59. 61. THANK YOU!

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