1. CASE 2 Roque -Simbulan

2. HISTORY Pertinent Findings

3. General Data 2 yr old female from San Pablo, Laguna Sought consult last June 8, 2009 CC: FEVER

4. History of Present Illness 7 days PTA  On and off fever, Tmax 39 o C temporarily relieved after intake of Paracetamol. 6 days PTA  Appearance of maculopapular rashes at the head progressing to the abdomen and to the extremities. Fever persisted. 5 days PTA  Dry fissured lips. Rashes and fever persisted.

5. History of Present Illness 4 days PTA  Signs and symptoms persisted. 3 days PTA  Patient was brought to Immaculate Concepcion Hospital in San Pablo, Laguna. Assesment was T/C Systemic Viral Infection and was given Co-trimoxazole, Paracetamol, and ascorbic acid. Mother noted edema of the finger tips and desquamation of the skin at the buttocks area with persistence of fever. Hence, transferred to PGH and subsequently admitted.

6. Review of Systems (-) abdominal pain, (-) seizure, (-) chest pain, (-) diarrhea, (-) cough, (-) nausea, (-) colds, (-) vomiting, (-) conjunctival suffusion

7. Past Medical History January 2009 – Pneumonia at Immaculate Concepcion Hospital

8. Birth and Maternal History Born FT to a then 24 year old G2P1(1001) mother via SVD delivered at home by a midwife. Mother had regular PNCU c/o local health center with intake of MVT and FeSO4. No fetomaternal complications.

9. Family History (+) DM – maternal side

10. Personal and Social History Mother is a 26 year old housewife. Father is a 28 year old security guard. Patient is the second child in a family of 2 kids. Older child is 5 y/o male, healthy.

11. Immunization History

12. IMMUNIZATION HISTORY The patient has the ff. :The ff. are expected: (+) BCG (+) OPV3 (+) DPT3 (+) Hepa B3 (+) Measles Birth: BCG, Hepa B 6 weeks: DPT1, OPV1, Hepa B 10 weeks: DPT2, OPV2, (Hepa B*) 14 weeks: DPT3, OPV3, Hepa B 6 months (and yearly thereafter): Influenza 9 months: measles 12 months: MMR, Varicella, Hepa A (2 doses 6 months apart)

13. Recommended Immunizations The patient should also be getting yearly Influenza shots, as well as MMR, Varicella, and Hepa A shots. MMR should be taken at 12 mos; the 2 nd dose should be taken through 4-6 years (can be taken before 4 mos, provided at least 28 days have elapsed since the 1 st dose)

14. Recommended Immunizations Varicella: min age is 12 mos; 2 nd dose should be taken from 4-6 yrs (can be taken before 4 yrs provided at least 3 mos have elapsed); min interval bet doses is 3 mos for children aged 12 mos to 12 years Hepa A: min age is 12 mos; administer 2 doses at least 6 mos apart

15. Nutritional status

16. Nutritional Status Patient HistoryWHO Recommendation Breastfed for 6 monthsExclusive breastfeeding for 6 months, followed by continued breastfeeding w/ complementary food up to 2 yrs Shifted to milk formula at 6 months, consumes 4 ounces/feeding every 3 hours (900 ml/day) At 6-12 months and without other animal food sources, milk formula intake should be 400-550 ml/day Semi-solid food started at 9 monthsSemi-solid food started at 6 months Presently eats table foodTable food permitted at 12 months *Patient’s weight (10kg) is less than the 5 th percentile for her age. Diet needs to be improved and growth further monitored. (source: Nelson’s)

17. Developmental History

18. The patient is at par with her age ( 2 years old) Developmental milestones of a 2 year old child: Physical Development - Child reaches about ½ of his maximum adult height. -90% of adult head circumference is reached, with just an additional of 5 cm gain for the next few years. Motor Development: - Runs well -walks up and down the stairs, one step at a time -climbs on furnitures -jumps

19. Adaptive Development -Makes tower of 7 cubes -Scribbles in circular pattern -Imitates horizontal strokes -Folds paper once, imitatively Cognitive Development -Object permanence -Improved problem solving skills, better understands cause and effect -Language: -Puts 3 words together into simple sentences (subject, verb, object) -Vocabulary comprises of about 50-100 words -Can understand and follow 2-step commands

20. Socio-Emotional Development -Often tells about his immediate experiences -self awareness and internalized standards of behavior sfirst appears at this age Self-Help Skills -Handles spoon well -Helps in undressing Source: Nelson Textbook of Pediatrics

21. PHYSICAL EXAMINATION

22. Awake, not in cardio-respiratory distress Wt 10kgHt 91 cm BP 90/60HR 11RR 20T 38.3 o C Pink conjunctivae, anicteric sclerae, no eye discharge, hyperemic tonsillopharyngeal walls, (+) CLAD, left >1.5 cm, dry cracked lips Adynamic precordium, distinct heart sounds, tachycardic, regular rhythm, (-) murmur Equal chest expansion, clear breath sounds, (-) crackles, wheezes, retractions Abdomen flat, soft, (-) tenderness, normoactive bowel sounds, (-) hepatosplenomegaly Pink nailbeds, full pulses, (-) cyanosis, (+) edematous finger tips, (+) sacral desquamation, (+) generalized maculopapular rashes Normal external genitalia

23. PRIMARY IMPRESSION Based on History and PE Findings

24. KAWASAKI DISEASE RULE INLESS LIKELY BECAUSE (+) fever ≥ 5 days, persistently high, despite medication (+) hyperemic tonsillopharyngeal walls (+) unilateral (left) cervical lymph adenopathy, >1.5cm (+) edema of extremities (+) dry fissured lips (+) maculopapular rash (+) desquamation in the sacral area with rash (+) tachycardia (-) conjunctivitis

25. DIFFERENTIAL DIAGNOSES Based on History and PE Findings

26. SCARLET FEVER RULE INLESS LIKELY BECAUSE (+) fever (+) rash w/c appeared a day after fever and which spread from the head  abdomen and extremities (+) desquamation Persistence of rash

27. MEASLES RULE INLESS LIKELY BECAUSE (+) fever (+) maculopapular rash head  abdomen & extremities (+) desquamation (+) Measles Vaccination Rash appeared a day after fever (-) cough (-) coryza (-) conjunctivitis (-) Koplik spots

28. RUBELLA RULE INLESS LIKELY BECAUSE (+) maculopapular rash head  abdomen & extremities (+) cervical lymph adenopathy (+) fever (+) desquamation Rash persisted > 3 days

29. INFECTIOUS MONONUCLEOSIS RULE INLESS LIKELY BECAUSE (+) fever (+) hyperemic tonsillopharyngeal walls (+) cervical lymph adenopathy (-) hepatosplenomegaly

30. ROSEOLA RULE INLESS LIKELY BECAUSE Acute Presentation of fever (+) CLAD Likely for the Px’s age group Facial sparing of the rash in roseola Patient’s fever is persistent and is less than 39 o C

31. TOXIC SHOCK SYNDROME RULE INLESS LIKELY BECAUSE (+) acute fever > 38.9°C (+) progressive rash: generalized maculopapular (+) hypotension : BP less than 5% 2 y/o (+) oropharyngeal hyperemia Desquamation in the sacral area started 4 days after onset [  TSS desquamation occurs 1-2 weeks after onset, particularly palms and soles] (-) vomiting (-) diarrhea (-) conjunctivitis (-) focal Staphylococcal infection Mucous membranes usually spared Rashes usually present as bullous impetigo, scarlantiform lesions or diffuse erythema

32. DIAGNOSTICS

33. The ff. diagnostic procedures can be done to make a more definite diagnosis and rule out differentials: ( tests and expected results) CBC  In measles: Leukopenia; Normal ESR/CRP  In roseola: Leukopenia  In KD: WBC normal or elevated, predominantly neutrophils; platelet normal in wk 1, elevated in wk 2-3, elevated ESR and CRP Renal/Hepatic Tests  In toxic shock syndrome: Elevated AST, ALT, or creatinine (>2x) Serology  Rubella: (+) rubella IgM antibodies  Roseola: (+) roseola specific antibodies Antibiotic therapy  Scarlet fever: rapid clinical response (24-48 hr)

34. PERTINENT LABORATORY FINDINGS LABORATORY FINDINGCOMMENT WBC = 20.20 with Seg 0.667 LEUKOCYTOSIS WITH NEUTORPHIL PREDOMINANCE (LEFT SHIFT)  Rules in Kawasaki Disease  Rules out Measles and Rubella Plt 346PLATECOUNT WITHIN NORMAL RANGE BUT HIGH  Rules in Kawasaki Disease  Rules out Toxic Shock Syndrome CRP > 12 ESR QNS ELEVATED CRP & ESR  Rules in Kawasaki Disease  Rules out Measles Hgb 100 Hct 0.31 ANEMIA  Rules in Kawasaki Disease 2d Echo: Dilated Right Coronary Artery IMPENDING SIGN OF RCA ANEURYSM  Rules in Kawasaki Disease

35. Diagnostics There is no diagnostic test for Kawasaki disease, but certain laboratory findings are characteristic. Normal to elevated WBC; predominance of neutrophils Elevated ESR and CRP Normocytic, normochromic anemia is common Platelet count normal during 1 st week, increases by 2 nd - 3 rd week

36. Patient’s Lab results CBCResultsNormal Values Remarks HgB10090-140 g/LNormal Hct0.310.28-0.42Normal WBC20.206-17.5 x 10 9 Elevated Seg 0.6670.54-0.62Elevated Plt346150-400Normal CRP>120.8-7.9Elevated ESRQNS0-10 mm/hr---

37. Patient’s Lab results Urinalysis: Normal except for slightly hazy appearance  Sterile pyuria may be present in KD Chest X-Ray: No significant chest findings 2d echo: Dilated Right Coronary Artery, Trace PR, good biventricular contractility  Cardiac involvement is the most important manifestation of KD Blood CS: No growth after 2 days

38. Other possible diagnostic tests: Other characteristic laboratory findings of KD: (-) Antinuclear antibody (-) Rheumatoid Factor Mild elevations of the hepatic transaminases and CSF pleocytosis may be present

39. PRIMARY IMPRESSION Based on History, PE and Labs

40. KAWASAKI DISEASE

41. Natural History of the Disease It is childhood vasculitis manifests with fever and a blanching rash. Most cases occur in children < 5 years although children aged 2 years are most commonly affected. The most serious complication of Kawasaki disease is coronary artery aneurysm secondary to the acute coronary arteritis. Intravenous immunoglobulin and aspirin therapy given within the first 10 days of the illness can decrease the risk.

42. Pathophysiology Etiology is unknown however it is commonly related to a staph,strep or systemic viral infection Inflammation due to previous infection causes intimal proliferation and infiltration of vessel wall with mononuclear cells causing vasculitis

43. THERAPEUTICS

44. TREATMENT High dose IV gammaglobulins - treatment of choice for Kawasaki disease - administered as a single bolus of 2 g/kg or as a 400-mg/kg infusion daily for 4 days - most effective in preventing development of coronary artery aneurysm when used within the first seven days of onset of fever Aspirin - used together with IV gammaglobulin - administered at a dose of 80-100 mg/kg/day for the first 2 weeks - administration is continued until fever subsides, after which a lose dose of 3- 5mg/kg/day is continued for 6-8 weeks or until there are no more signs of coronary artery disease Anti inflammatory drugs - for pain management Other Options - Plasmapheresis - TNF-blocking drugs to minimize inflammation - Steroids for those who failed to respond to initial treatment

45. Treatment for Complications 1. Coronary Artery Disease - Aspirin and dipyridamole - Anticoagulatant therapy - Fibrinolytic therapy - Surgical management - Interventional Cardiac Catheterization Techniques 2. Chronic Myocardial Ischemia - Transluminal coronary angioplasty - Coronary artery bypass-graft surgery - Cardiac transplantation 3. Pneumonia - amantadine, rimantadine, osetamivir

46. Non Pharmacological Treatment  Since the exact cause of Kawasaki disease is not yet fully understood, there are still no known preventive measures for the disease  Treatment is generally safe and effective  Education on early treatment is important to prevent further complications

47. MANAGEMENT

48. Management Further Outpatient Care careful follow up for cardiac complications (CAD)  pediatric cardiologist  long-term implications for coronary artery disease are unknown at this time.

49. Management Deterrence/Prevention unknown etiology, no method of deterrence. Therapy is directed at prevention of coronary artery aneurysm formation.

50. Management Others Immunization with MMR, varicella and Hep A Advise parents about proper diet for the patient to improve weight, make sure that diet is balanced and healthy. Monitor weight and other growth parameters.