Kawasakii

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Kawasakii

  1. 1. M.D Pediatrics Ph.D Pediatric special need and nutrition consultant Mansoura national Hospital Insurance H Fever H and Egyptian liver H
  2. 2. •”‫منكم‬ ‫آمنوا‬ ‫الذين‬ ‫ا‬ ‫يرفع‬ .“‫درجات‬ ‫العلم‬ ‫أوتوا‬ ‫والذين‬ •”‫الذين‬ ‫يستوى‬ ‫هل‬ ‫قل‬ .“‫يعلمون‬ ‫ل‬ ‫والذين‬ ‫يعلمون‬
  3. 3. M.D Pediatrics Ph.D Pediatric special need child health and nutrition consultant Mansoura national Hospital Insurance H Fever H and Egyptian liver H
  4. 4. male patient 13 years old from Mehalet Damna ,he is the first kid of the family and the older brother of two healthy sisters
  5. 5.  The conditon stareted 1.5 months ago when fever developed and increased gradually .the child was feverish all the day and fever was more at night but without significant diurnal variations and it was oscillating around 40º C . The fever was altered temporarily by antipyretics .
  6. 6. The family sought medical advice and the child received a lot of medications including different classes of antibiotics without any improvement. Then the child was admitted to hospital and was fully investigated but yet undiagnosed.
  7. 7.  One week later the child developed oral ulcers which markedly interfered with oral feeding  These ulcers were multiple and persisted the whole duration of illness.
  8. 8.  Also 2 days later the the child developed simultaneous events.  There was edema , erythema , pain in the hands and feet.this pain was associated with limitation of movements.
  9. 9.  At the same time the patient developed multiple neck swellings bilaterally.  These neck swellings were variable in size the largest of them was about lemon size.They gradually decreased in size
  10. 10.  One of the cervical lymph nodes was excised and sent to biopsy  The child developed redness in both eyes without marked discharge which continued the whole month.
  11. 11. Thre was no history of other skin rash There was no history suggestive of bleeding tendency or purpuric eruption . No history of abdominal distension  No history of big joint affection
  12. 12.  No history of jaundice or changes of the colour of urine or stool  No history of convulsions or abnormal movements or limb weakness.  No history of disturbed conscious level or increased intracranial tension
  13. 13.  There is no history of dyspnea or chest pain Or significant chest symptoms  There is no history of other system affection
  14. 14.  There is no significant past medical history or family history as well
  15. 15.  General Examination  The patient is fully conscious well oriented for time place and person , he is co-operative and of average intelligence  The patient has no special decubitus
  16. 16.  The temperature during examination was 39º C .  Pulse was 145 BPM of average volume , reglar , equal bilaterally with intact peripheral pulsations and no special character.  Blood pressure 100/70 mmhg . Respiratory rate 30.
  17. 17.  Head and Neck examination revealed bilateral non purulent conjuctival congestion , multiple mouth ulceration , red strawberry tongue ,multiple small lymph nodes the largest of them 2 cm in diameter , the lymph nodes are mobile ,non- tender and firm in consistency.
  18. 18.  Neck veins are 3 cm above the clavicle measured at the same level to that of Angle of Louis .Neck veins showed normal pulsations with no inspiratory filling.  There was no goitre , complexion abnormalities or any other significant abnormality.
  19. 19.  UL examination revealed bilateral distal skin peeling , no arthritis , no clubbing, no sc nodules , bilateral epitrochlear lymphadenopathy 1.5 cm , firm and non tender. There was no axillary lymph nodes.
  20. 20.  LL examination revealed erythema and skin peeling in the sole of the foot but no oedema , clubbing or arthritis.
  21. 21.  Cardiac examination showed an evident 3rd heart sound with protodiastolic gallop but without significant murmurs.  A monocomponent pericardial rub is heared
  22. 22.  Abdominal examination revealed no abnormities apart from mild tender hepatomegaly ( liver span 11 cm in MCL ) with rounded border and soft consistency .  Chest and neurological examination were normal.
  23. 23.  Laboratory :  ESR 70 mm in the 1st hour and 110 mm in the 2nd hour  CBC  WBCs 8000/ cmm with normal differential cont  Hb 11gm/dl  Platelets 485.000 / cmm
  24. 24.  CRP 18 mg / dl  ASOT 100  Normal liver and kiney functions  Urine analysis is free
  25. 25.  ANA and RF negative  EBV Ig M negative  CMV Ig M and Ig G –ve  Widal test and blood culture negative
  26. 26.  Bone marrow examination is within normal  Microscopic examination of the lymph nodes showed only reactive hyperplasia
  27. 27.  Normal CXR initially  Normal initial pelviabdominal ultrasound  Echocardiography revealed moderate pericardial effusion  ECG is low voltage sinus tachycardia t wave inversion in antrolateral leads
  28. 28. What is Kawasaki disease?
  29. 29.  Kawasaki disease (KD) (ie, Kawasaki syndrome [KS]) is a febrile illness of childhood. It is a self-limited acute vasculitic syndrome of unknown etiology, first described by Tomisaku Kawasaki in 1967.
  30. 30.  The diagnosis of classic Kawasaki disease (KD) requires fever of at least 5 days duration and the presence of 4 of the following :
  31. 31. 1-Changes in extremities (eg, erythema, edema, desquamation): This may limit movement and cause the child to refuse to bear weight. Desquamation of the fingers and toes begins in the periungual region, may involve the palms and soles
  32. 32. 2-Bilateral conjunctivitis (not associated with exudates) 3- Polymorphous rash (not vesicular) 4- Cervical lymphadenopathy 5- Changes in the lips and oral cavity (eg, pharyngeal erythema, dry/fissured or swollen lips, strawberry tongue)
  33. 33.  Cardiac involvement is the most important manifestation of Kawasaki disease. Myocarditis manifested by tachycardia and decreased ventricular function occurs in at least 50% of patients.
  34. 34. Is Kawasaki disease present in Egypt?
  35. 35.  It is estimated that at least 3,000 cases are diagnosed annually in the United States. The incidence of Kawasaki disease in Asian children is substantially higher than in other racial groups, but the illness occurs worldwide in all ethnic groups.
  36. 36. Kawasaki disease in Northern Africa (Extrapolated Statistics) Country/Region Extrapolated Incidence Population Estimated Used Egypt 280 76,117,4212 Libya 20 5,631,5852 Sudan 144 39,148,1622
  37. 37.  Pericarditis with a small pericardial effusion is common during the acute illness. Coronary artery aneurysms generally develop in up to 25% of untreated patients during the 2nd–3rd wk of illness.
  38. 38.  Patients with acute Kawasaki disease should be treated with intravenous immunoglobulin (IVIG) and high-dose aspirin as soon as possible after diagnosis
  39. 39. ACUTE STAGE  Intravenous immunoglobulin 2 g/kg over 10–12hr with aspirin 80–100 mg/kg/24 hr divided every 6 hr orally until 14th illness day CONVALESCENT STAGE  Aspirin 3–5 mg/kg once daily orally until 6–8 wk after illness onset
  40. 40. LONG-TERM THERAPY FOR THOSE WITH CORONARY ABNORMALITIES  Aspirin 3–5 mg/kg once daily orally ± dipyridamole 4–6 mg/kg/24 hr divided in two or three doses orally (most experts add warfarin for those patients at particularly high risk of thrombosis)
  41. 41. ACUTE CORONARY THROMBOSIS  Prompt fibrinolytic therapy with tissue plasminogen activator, streptokinase, or urokinase under supervision of a pediatric cardiologist
  42. 42.  IVIG also should be administered to children presenting after the 10th day of illness (ie, children in whom the diagnosis was missed earlier) if they have either persistent fever without other explanation or aneurysms and ongoing systemic inflammation, as manifested by elevated ESR or CRP
  43. 43.  Steroids Although corticosteroids are the treatment of choice in other forms of vasculitis, their use has been limited in children with Kawasaki disease  Corticosteroids also have been used to treat patients who have failed to respond to initial therapy for Kawasaki disease
  44. 44.  Abciximab, a platelet glycoprotein IIb/IIIa receptor inhibitor, has been used to treat patients in the acute or subacute phase of Kawasaki disease who have large coronary aneurysms.  A new class of agents that may play a role in the treatment of patients with refractory Kawasaki disease is monoclonal antibodies to various proinflammatory cytokines.
  45. 45.  A humanized monoclonal antibody against TNF-, infliximab, is being studied in a clinical trial of treatment for children who fail to become afebrile after initial IVIG treatment.  Cytotoxic agents like cyclophosphamide, in conjunction with oral steroids, have been suggested as useful for the treatment of exceptional patients with particularly refractory acute Kawasaki disease

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