CASE 2

Roque -Simbulan

HISTORY

Pertinent Findings

General Data

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

History of Present Illness 7 days PTA On and off fever, Tmax 39oC

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.

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.

Review of Systems

(-) abdominal pain, (-) seizure, (-) chest pain,

(-) diarrhea, (-) cough, (-) nausea, (-) colds,

(-) vomiting, (-) conjunctival suffusion

Past Medical History

January 2009 – Pneumonia at Immaculate Concepcion Hospital

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.

Family History

(+) DM – maternal side

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.

Immunization History

IMMUNIZATION HISTORYThe 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)

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 2nd dose should be taken through 4-6 years (can be taken before 4 mos, provided at least 28 days have elapsed since the 1st dose)

Recommended Immunizations

Varicella: min age is 12 mos; 2nd 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

Nutritional status

Nutritional Status

Patient History WHO Recommendation

Breastfed for 6 months Exclusive 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 months Semi-solid food started at 6 months

Presently eats table food Table food permitted at 12 months

*Patient’s weight (10kg) is less than the 5th percentile for her age. Diet needs to be improved and growth further monitored. (source: Nelson’s)

Developmental History

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

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

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 undressingSource: Nelson Textbook of Pediatrics

PHYSICAL EXAMINATION

Awake, not in cardio-respiratory distressWt 10kg Ht 91 cmBP 90/60 HR 11 RR 20 T

38.3oCPink 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

PRIMARY IMPRESSION

Based on History and PE Findings

KAWASAKI DISEASE

RULE IN LESS 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

DIFFERENTIAL DIAGNOSES

Based on History and PE Findings

SCARLET FEVER

RULE IN LESS LIKELY BECAUSE

(+) fever(+) rash w/c appeared a day after fever and which spread from the head abdomen and extremities (+) desquamation

Persistence of rash

MEASLES

RULE IN LESS LIKELY BECAUSE

(+) fever(+) maculopapular rash head abdomen & extremities(+) desquamation

(+) Measles Vaccination Rash appeared a day after fever (-) cough(-) coryza(-) conjunctivitis(-) Koplik spots

RUBELLA

RULE IN LESS LIKELY BECAUSE

(+) maculopapular rash head abdomen & extremities(+) cervical lymph adenopathy

(+) fever (+) desquamationRash persisted > 3 days

INFECTIOUS MONONUCLEOSIS

RULE IN LESS LIKELY BECAUSE

(+) fever(+) hyperemic tonsillopharyngeal walls(+) cervical lymph adenopathy

(-) hepatosplenomegaly

ROSEOLA

RULE IN LESS 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 39oC

TOXIC SHOCK SYNDROME

RULE IN LESS 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 infectionMucous membranes usually sparedRashes usually present as bullous impetigo, scarlantiform lesions or diffuse erythema

DIAGNOSTICS

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)

PERTINENT LABORATORY FINDINGSLABORATORY FINDING COMMENT

WBC = 20.20 with Seg 0.667

LEUKOCYTOSIS WITH NEUTORPHIL PREDOMINANCE (LEFT SHIFT)Rules in Kawasaki DiseaseRules out Measles and Rubella

Plt 346 PLATECOUNT WITHIN NORMAL RANGE BUT HIGHRules in Kawasaki DiseaseRules out Toxic Shock Syndrome

CRP > 12ESR QNS

ELEVATED CRP & ESRRules in Kawasaki DiseaseRules out Measles

Hgb 100Hct 0.31

ANEMIA Rules in Kawasaki Disease

2d Echo: Dilated Right Coronary Artery

IMPENDING SIGN OF RCA ANEURYSM Rules in Kawasaki Disease

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 1st week, increases by 2nd

- 3rd week

Patient’s Lab resultsCBC Results Normal

ValuesRemarks

HgB 100 90-140 g/L Normal

Hct 0.31 0.28-0.42 Normal

WBC 20.20 6-17.5 x 109 Elevated

Seg

0.667 0.54-0.62 Elevated

Plt 346 150-400 Normal

CRP >12 0.8-7.9 Elevated

ESR QNS 0-10 mm/hr ---

Patient’s Lab results Urinalysis: Normal except for slightly hazy

appearanceSterile pyuria may be present in KD

Chest X-Ray: No significant chest findings 2d echo: Dilated Right Coronary Artery, Trace

PR, good biventricular contractilityCardiac involvement is the most important manifestation

of KD Blood CS: No growth after 2 days

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

PRIMARY IMPRESSION

Based on History, PE and Labs

KAWASAKI DISEASE

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.

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

THERAPEUTICS

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

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

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

MANAGEMENT

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.

Management

Deterrence/Prevention unknown etiology, no method of

deterrence. Therapy is directed at prevention of coronary artery aneurysm formation.

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.