Kawasaki disease by Hassan AL-Qarni

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Kawasaki disease

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Kawasaki disease by Hassan AL-Qarni

  1. 1. HASSAN AL-QARNI MEDICAL INTERN TAIF UNIVERSTY Kawasaki Disease
  2. 2. Outcomes  Case presentation  Definition  Etiology & Epidemiology  Clinical manifestation  Diagnosis  Treatment  Complication
  3. 3. Case presentation
  4. 4. History  HajarYassin Mohammed is 3 year old female pt, admitted at 8/4/2014 with history of fever, skin rash ,mouth ulcres ,red eyes with discharge since 4 days prior to admission  No history of cough , vomiting and diarrhea  No family history of URTI  Diagnosed as URTI with stomatitis and query skin allergy
  5. 5. On examination  VS:  Temp: 39.9 PR: 135  RR: 40 SPO2: 95% W/0 O2  General examination:  Conscious, febrile ,looks ill  Erythematous lips with vesicles and ulcers  Congested throat , normal tongue  Maculopapular rash with scratch marks on upper & lower limbs (eczema like rash)  Conjunctvitis with bilateral mucupurulent disharge  NO significant lymph node enlargement
  6. 6. On examination  Systemic examination:  Chest : bilateral air entry w/0 added sounds  CVS: s1+s2+0, NO murmur , NO gallop  Abdomen : soft ,lax . NO organomegally  CNS: conscious  Case seen by dermatologist , diagnosed as: (herpes simplex infection vs erythema multiform) ?  Treated with acyclovir , Cefrixone  Case seen by ophthalmologist, diagnosed as : mucupurluent conjunctivitis
  7. 7. Investigation  CBC:  WBC: 7000 – 12000 (3 days) – 13000 - 15000  Platelet : 257000- 59700,000 (3 days) -700,000 – 815000 (after 1 wk)  Lymphocyte : 55%  Neutrophil : 38%  ESR : 95 mm/hr – 124 mm/hr  CRP: +ve  ASO ( Antistreptolysin O ) titer : -ve  LFT , RFT & Chemistry : normal  ECG & Echocardiography : normal  Blood & urine culture : -ve
  8. 8. Management  After 2 days, as fever not subsided and pt developed erythema , swelling of both hand & feet , became irritable  Diagnosed as (query Kawasaki disease) :  IVIG started as 2g/kg  Aspirin 80 mg /kg  On 6th day of admission ,pt developed peeling of skin, cracked lips.
  9. 9. Management  On 7th day of admission , fever was on & off (refractory )  Another dose of IVIG given 2g/kg  Aspirin continued as 80 mg/kg  On 10th day of admission  NO fever for more than 1 day  Pt signed discharge against medical advice(DAMA)  Given aspirin 80 mg/kg ( high dose) to complete 14 days  aspirin Prophylaxis 60 mg OD to be started after 14 days  Follow up  Pediatric Cardiology clinic  Consultant pediatric clinic
  10. 10. Tomisaku Kawasaki (centre right) at the 8th International Kawasaki Disease Symposium, 2005
  11. 11. Definition  Kawasaki disease (KD) is an acute febrile vasculitic syndrome of early childhood  previously called :  Mucocutaneous lymph node syndrome  Most common vasculitides of childhood  Typically a self-limited condition, with fever and other acute inflammatory manifestations lasting for an average of 12 days
  12. 12. EPIDEMIOLOGY  Greatest in children who lives in East Asia (eg, Japan, Korea,Taiwan) or are of Asian ancestry living in other parts of the world  In japan :  134 cases per 100,000 children younger than 5 years  10 - 20 times higher than in Western countries  Other risk factors include:  Male gender  Age between 6 months - 5years  Family history of KD
  13. 13. EPIDEMIOLOGY  In Saudi Arabia :  CITY:  Madinah region, Kingdom of Saudi Arabia (KSA).  METHODS  retrospective  Maternity and Children Hospital, Madinah  January 2007 to January 2010.  51 patients suspected cases of Kawasaki disease  RESULTS  24 patients diagnosed as Kawasaki ( 47 %)  M:F = 1.7 : 1  CONCLUSION:  High index of suspicion is mandatory for early diagnosis of Kawasaki disease  Delayed diagnosis may lead to coronary lesions Kawasaki disease in western Saudi Arabia Khalid Alharbi SMJ 2010
  14. 14. Etiology  Unknown  Theories :  Immunologic response  Infectious etiology  Genetic factors
  15. 15. Etiology  Immunologic response:  Affects medium-sized arteries  Inflammatory cell infiltration into KD vascular tissue vascular damage  Stimulus for this inflammatory infiltration has not been identified
  16. 16. Etiology  Infectious etiology:  Similarities between KD and other pediatric infectious conditions suggest that KD is caused by a transmissible agent include:  Febrile exanthem with lymphadenitis and mucositis  Seasonal increase in disease incidence in the winter and summer  No studies have convincingly identified a specific virus, bacteria or bacterial toxin, or other pathogen associated with KD
  17. 17. Etiology  Genetic factors:  Increased frequency of the disease in Asian and Asian-American populations and among family members
  18. 18. CLINICAL MANIFESTATIONS  Fever :  Most consistent manifestation of KD  above 38.5ºC during most of the illness
  19. 19. CLINICAL MANIFESTATIONS  Conjunctivitis  Bilateral nonexudative conjunctivitis is present in more than 90 % of patients Courtesy of Robert Sundel, MD. Graphic 78898Version 2.0
  20. 20. CLINICAL MANIFESTATIONS  Mucositis  Mucositis often becomes evident as KD progresses.  Cracked, red lips and a strawberry tongue
  21. 21. CLINICAL MANIFESTATIONS  Rash  Polymorphous  Begins as perineal erythema and desquamation, followed by macular, morbilliform, or targetoid skin lesions of the trunk and extremities
  22. 22. CLINICAL MANIFESTATIONS  Extremity changes  last manifestation to appear  Indurated edema of the dorsum of their hands and feet  Diffuse erythema of their palms and soles.
  23. 23. CLINICAL MANIFESTATIONS  Lymphadenopathy :  Involve primarily the anterior cervical nodes overlying the sternocleidomastoid muscles
  24. 24. CLINICAL MANIFESTATIONS  Cardiovascular findings :  During the first week to 10 days of illness include:  Tachycardia out of proportion  Gallop sounds  Muffled heart tones  Fusiform aneurysms of the brachial arteries that are easily palpable or visible in the axillae .  Young infants may have cold, pale, or cyanotic digits of the hands and feet due to reduced blood perfusion
  25. 25. Investigation  No laboratory test specific for KD
  26. 26. Investigation  CBC :  Leukocytosis, and a left-shift in the white blood cell count  Thrombocytosis: may reach to 1,000,000/mm3  Normocytic, normochromic anemia  Increased of acute phase reactants [CRP,ESR]  Urinary microscopy: white blood cells (Pyuria ) is often of urethral origin  Abnormal liver function test because of intrahepatic congestion  Echocardiography : study of choice to evaluate for coronary artery aneurysms  ECG
  27. 27. Treatment  Intravenous immune globulin (IVIG)  Single dose of (IVIG) (2 g/kg) administered over 8 to 12 hours  Aspirin  high-dose : (80 - 100 mg/kg/day) Untill resolution of fever Or 14 days of fever  Prophylaxis (3 -5 mg/kg /day)  48 hours after the resolution of fever.  continued until laboratory markers of acute inflammation (eg, platelet count and ESR) return to normal  unless coronary artery (CA) abnormalities are detected by echocardiography
  28. 28. Treatment of refractory Kawasaki disease  INCIDENCE :  ( 10% - 20%)  Significantly increased risk of developing coronary artery aneurysms  Manifested as persistent fever 36 hours after completion of initial therapy
  29. 29. Complication
  30. 30. References  Uptodate.com  Emedicine.com
  31. 31. THANK YOU 

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