HISTORY OF PRESENT ILLNESS

AJ is a 4M 1D previously healthy female infant who presents with fever and rash. She is being transferred from an OSH TIMELINE OF EVENTS PTA: 4 days ago found to be restless and febrile to 99 F. She was seen at a clinic where she was febrile to 102.5 F. A bagged urine specimen was remarkable for 10-25 WBCs and 2+ leukocyte esterase. No culture was sent. She was diagnosed with a UTI, given a shot of Ceftriaxone and sent home on Amoxicillin The next day she developed a rash over her trunk and limbs. Her mother was concerned that the rash was related to the Amoxicillin, so she stopped it The following morning, she was increasingly fussy so her family took her to a different clinic where she was febrile to 105 with RR of 62 and HR of 200. She was given 40 mL/kg of NS because of diarrhea and poor PO intake. A catheterized urine specimen showed 3-10 WBCs. Urine and blood cultures were sent, and she was transferred to local hospital

HISTORY OF PRESENT ILLNESS

At the OSH initial work up was obtained: CBC was remarkable for white count of 9.4 CRP was 60 mg/L She was given three additional 20 mL/kg NS boluses over the course of the next 24-36 hours LP yielded clear fluid, 22 WBCs, glucose of 50, and total protein of <2. CSF gram stain showed few whites and no organisms HSV and Enterovirus PCRs of the CSF were sent She was started on Vancomycin, Acyclovir, and Ceftriaxone

She continued to be febrile to 104 overnight and developed dry, bloody, cracked lips and worsening rash. Given the ongoing high fevers and evolution of her rash, she was transferred to PCH for further treatment. The team at the OSH had been in contact with our dermatology team prior to transfer

MORE HISTORY
PAST MEDICAL HISTORY: Born at 38 weeks gestation via c-section (repeat). No problems during pregnancy. No medical problems since birth until this current illness PAST SURGICAL HISTORY: None

IMMUNIZATIONS: Up to date through 2 month vaccines

MEDICATIONS: None regularly ALLERGIES: None DIET: Similac formula and some baby foods

FAMILY HISTORY: No family history of serious illnesses in infants. Older sibling is s/p kidney transplant due to a congenital kidney problem
SOCIAL HISTORY: Lives with mom and dad. 18 year old sister does not live in the home. No cigarette smokers in the house

PHYSICAL EXAM
VITAL SIGNS: T 38.6 HR 195, which improved to 170-160s when calm. RR 30. BP 104/45. SaO2 96% on Room Air. WEIGHT - 6.89 Kg, (83%ile) HEIGHT - 63 cm, (72%ile) GENERAL: Crying infant examined lying in crib. HEAD: Normocephalic, atraumatic, anterior fontanelle open, soft, and flat. EYES: Unable to assess pupils. Extraocular movements intact, Right eye with conjunctival injection. EARS: Tympanic membranes gray bilaterally, normal light reflex and landmarks, no effusion or perforation. NOSE: Bloody discharge bilaterally. OROPHARYNX: Cracked lips with scabbing and areas of bleeding. No lesions visualized inside the mouth. Mucus membranes in mouth moist. NECK: Supple without lymphadenopathy or tenderness to palpation. CARDIOVASCULAR: Tachycardic, normal rhythm, and S1/S2, without murmur or gallop. Pulses appropriate. Capillary refill time about 3 seconds. LUNGS: Coughs occasionally during exam. Moves air well bilaterally, but does have transmitted upper airway sounds bilaterally. No retractions. ABDOMEN: Soft, non-tender, non-distended with active bowel sounds and no masses or hepatosplenomegaly. EXTREMITIES: All extremities warm and well perfused. BACK: No sacral dimple or hair tuft. GENITOURINARY: Normal Female external genitalia, Tanner stage I. Small fissure above labia, and possibly some small fissures around anus. No other mucosal lesions visualized. NEUROLOGIC: Awake and irritable. No focal deficits noted. Appropriate tone. SKIN: Rash over trunk and limbs. Rash is more pronounced on abdomen than back. Discrete patches of erythemous macules and papules, almost serpentigious. No vesicles noted. Dry skin on feet. No petechiae.

LABORATORY
CBC: WBC 9.4 HGB 11.9 HCT 35.4 PLAT 273 Neut 32% Lymph 50% Bands 9% Mono 6% UA (catheterized, per records): Blood large, Ketones neg, LE neg, Bac neg, WBCs 5, RBCs 3, Nitrites neg, Squ Epi neg CSF WBC 22 RBC 8 39% PMNs, 61% Lymphs Gram stain: No bacteria, few WBCs Glucose: 50 (ref range 40-80) Protein: <2 (ref range 12-60) HSV PCR: Pending Enterovirus PCR: Pending CRP: 60.21 (ref range 0-3, units mg/L)

Following Day: CBC: WBC 12.7 HGB 13.2 HCT 37 PLAT clotted
CMP: Na 143 K 3.8 Cl 110 HCO3 18 BUN 3 Cr 0.3 Gluc 117 Ca 9.7 Mag 2.5 Phos 4.2 Pro 6.8 Alb 3.7 Bili 0.5 AST 35 ALT 34 Alk phos 159 Vancomycin trough: 3

DIFFERENTIAL DIAGNOSIS
ID: UTI Urosepsis Renal abscess Viral meningitis DERM: Stevens-Johnson syndrome Erythema Multiforme major Drug hypersensitivity reaction

Influenza
Adenovirus Enterovirus Measles EpsteinBarr Virus Cervical Lymphadenitis

CV:
Kawasaki Disease Myocarditis RHEUM: Systemic Onset Juevenille Idiopathic Arthritis

Staphylococcal Scalded Skin

Scarlet Fever Rocky Mountain Spotted Fever Toxic Shock Syndrome

PATIENT HOSPITAL COURSE

DERM CONSULT Punch Biopsy Treatment with IVIG with marked improvement ECHO: 1. Echo findings are consistent with Kawasaki disease. 2. Dilated left main coronary artery and right main coronary artery. 3. The left main coronary artery is dilated at 2.7 mm (z score = 2.28 ). 4. The right main coronary artery is dilated at 2.1 mm (z score = 2.19). 5. Normal left ventricular size and qualitatively normal systolic function. 6. Normal right ventricular size and qualitatively normal systolic function.

KAWASAKI DISEASE (KD)

Acute febrile illness of childhood characterized by vasculitis of medium-sized extraparenchymal arteries, with a predilection for coronary arteries

Leading cause of acquired heart disease in developed countries

Etiology remains unclear Aspects of KD mimic infectious process (toxin mediated and viral illnesses) Seasonal peaks suggesting transmissible vector Efficacy of IVIG postulated to be due immunoglobulin binding of toxin Alternative hypothesis that infectious agents trigger a final common pathway in genetically susceptible hosts

CLINICAL MANIFESTATIONS
Classic Clinical Criteria: Fever of at least 5 days duration Presence of at least 4 of the following: Changes in extremities Polymorphous exanthem (bullous or vesicular lesions suggest alternative dx) Bilateral conjunctival injection (nonexudative and limbic sparing) Changes in lips and oral cavity (discrete ulcers and tonsillar exudate not seen) Cervical lymphadenopathy ( 1.5 cm) Features of KD can fluctuate so need a thorough history

OTHER CLINICAL FINDINGS

Cardiovascular Congestive heart failure, myocarditis, pericarditis, valvular regurgitation Coronary artery abnormalities Musculoskeletal Arthritis Arthralgias GI Diarrhea, vomiting, abdominal pain Hydrops of the gallbladder Hepatic dysfunction CNS Extreme irritability Aseptic meningitis Genitourinary Urethritis/meatitis

INCOMPLETE KD
Dont have atypical features, rather have some of classic features but not enough to meet case definition

More likely to be infants and older children Also at higher risk for coronary involvement (especially infants < 6 months)

Given cardiac consequences of failing to treat KD, the American Heart Association published an algorithm for the evaluation and treatment of suspected incomplete KD

Algorithm: Evaluation of suspected incomplete Kawasaki disease (KD) Excellent images of:
Bilateral nonexudative limbal sparing conjunctivitis Strawberry tongue Periungual peeling

TREATMENT
Treat with high dose IVIG (2 g/kg) and high dose Aspirin (80-100 mg/kg per day divided into 4 doses) Ideally treatment administered within the first 7 days and day 10 at the latest

Approximately 15% of children will have recurrent fevers after the 1st dose of IVIG Most clinicians will administer another dose of IVIG (2 g/kg) after 36-48 hrs after the first dose if persistent fevers

If IVIG resistant, consider treatment with Infliximab

After resolution of fevers, switch to low dose aspirin (3-5 mg/kg/day)

Remember Measles and Varicella containing vaccinations contraindicated for 11 months after IVIG administration!