Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.

Kawasaki Disease


Published on

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!