Presented by Samuel Lee, MD
A 3 year old African-American male is brought to the ED by his mother with a fever for six days, sore throat, and a rash on his chest. The patient had been seen by his PCP 3 days ago, diagnosed with scarlet fever, and started on amoxicillin.
Since then the fever has not subsided and the mother has noticed painful swelling and skin peeling over the patient’s lips, palms, soles, and genital area. The patient cries when walking and avoids placing weight on his soles. The mother denies any sick contacts. The patient does not have a cough, nausea, vomiting ,or diarrhea. He has been eating and drinking, and the mother states that he is still producing urine.
Past Medical History: multiple past ED visits for croup
Medications: Amoxicillin X 3 days
Social Hx: lives with mother and grandmother in an apartment
Birth History: Full term SVD
Vital Signs: BP 90/56, HR 136, RR 24, Rectal Temp 38.8, Oxygen Sat. 99% on Room air; weight 18 kg
General: The patient is sitting on his mother’s lap; awake and alert, good eye contact; cries if he is made to stand on his feet
HEENT: Atraumatic/normocephalic; PERRL, bilat conjunctival injection, no discharge. The peri-oral area is swollen and erythematous and the lips are cracked. There is pharyngeal erythema with no exudate, vesicles, or lesions seen. MMM. No LAD upon palpation. Neck is supple; there is no nuchal rigidity.
Resp: Lung sounds clear to auscultation. No wheezing/rales/rhonchi.
Chest: Sandpaper rash on chest and upper abdomen which is resolving per mother. No vesicles or lesions.
CV: RRR, no murmurs, rubs, or gallops; pulses 2+ and equal in all ext.
ABD: Soft, nontender/nondistended. Positive bowel sounds, no flank tenderness.
EXT: palms and soles are swollen, erythematous, tender to palpation. No vesicles or lesions present. Cap refill less than 2 sec.
GU: There is an erythematous rash and peeling skin on the groin and penis. Testes descended.
Neurological: Awake, alert; able to ambulate but cries when weight is placed on soles.
Impression: Three year old male with 6 day hx of fever and scarlitiniform rash, initially diagnosed as scarlet fever; Condition has not improved with amoxicillin. Now with conjunctival injection, erythema and skin peeling of lips, palms, and GU area.
CBC: WBC 10, Hemoglobin 11.2, Hematocrit 32.6, Platelets 328
Diff: Neutrophils 61%, Lymphocytes 22%, Monocytes 14%, Eosin 3%, Bands 1%
Electrolytes: Na 138, K 4.8, Cl 103, CO2 20, AG 15
BUN 5, Creatinine 0.4
UA: clear, yellow, Specific Gravity 1.016, pH 7.5, WBC 5-10, no RBC, Nitrite and LE negative; 0 bacteria
CXR: mild hyperinflation, no focal opacity
Rapid Strep test neg
EKG: NSR@100 bpm, no axis deviation, no PR prolongation, no QRS widening, no atrial or ventricular hypertrophy, no ST changes, no T wave inversions, no accessory waves or ectopic beats; no previous EKG available for comparison
1. Given this patient’s history and physical exam, what is the best definitive course of action?
a. Admit the patient for treatment and arrange for an echocardiogram
b. Continue supportive treatment as an outpatient and have the patient follow up in one week for an echocardiogram
c. Initiate antibiotic treatment for Urinary Tract infection and arrange for a voiding cystourethrogram
d. Advise the patient’s mother to continue with amoxicillin and follow up with her PCP in 2 days.
2. What is a serious complication of this patient’s diagnosis?
a. Development of meningitis in the next week
b. Coronary artery aneurysm and myocardial infarction
d. Rhabdomyolysis and acute renal failure
3. What is the definitive treatment for this patient’s condition?
a. IV fluid 20cc/kg bolus
b. Nitrofurantoin 2 mg/kg PO X 10 days
c. IVIG 2 mg/kg over 12 hrs, Aspirin 100 mg/kg/day divided qid
d. Acetaminophen 15 mg/kg
1. A. The patient fulfills diagnostic criteria for Kawasaki disease and should be admitted for treatment and echocardiogram evaluation. Sterile pyuria is commonly seen, and with negative LE, nitrite, and bacteria in the urine, treatment is not warranted. Continuing amoxicillin therapy will not be effective in treating this patient.
2. B. In Kawasaki disease the most life-threatening complication is coronary artery involvement and subsequent infarction. This patient did not exhibit any meningeal signs or AMS, so meningitis would not be in your differential. The sterile pyuria and hand/foot swelling and pain can be attributed to Kawasaki disease.
3. C. IVIG and ASA is the definitive treatment for Kawasaki disease. IV hydration or acetaminophen may be used as supportive treatment but will not treat the underlying cause.
Kawasaki disease is a pediatric vasculitic syndrome which was first documented in 1967 by Dr. Tomisaku Kawasaki. He had observed that some pediatric patients with a self-limiting fever and rash would subsequently die of myocardial infarctions. Later studies showed that a significant number of patients with the particular constellation of symptoms of prolonged fever, rash, oral involvement, and conjunctivitis developed coronary artery aneurysms either during or soon after their illness. Today Kawasaki disease is the most common cause of acquired pediatric heart disease in developed nations.
Kawasaki disease outbreaks occur in three-year cycles and are typically seen during the influenza season of late-winter to early-spring. The disease causes immune-mediated damage to the body’s vasculature, with the most alarming complication being coronary artery involvement which can lead to aneurysms and myocardial infarction.
The diagnosis of Kawasaki disease is based on the clinical presentation and requires a fever greater than 39° C for at least five days in addition to four of the five following symptoms:
- Conjunctival injection, usually non-exudative
- Cervical lymphadenopathy of at least 1.5 cm diameter
- Lip and oral cavity involvement such as swollen or dry lips, erythematous tongue, or pharyngeal erythema
- Trunk or genital area erythema
- Hand or foot erythema, swelling, or desquamation
Additional clinical findings may include sterile pyuria, hepatitis, gall bladder distention and hydrops, and swelling or erythema at a previous BCG vaccination site.
The clinical course of Kawasaki disease follows three distinct phases. The acute phase begins with onset of the fever and symptoms. This phase typically lasts one to two weeks. Cardiac complications during this first phase are aneurysm, pericarditis, and myocarditis. The subacute phase begins when the fever and symptoms begin to disappear. This phase lasts approximately one to three weeks, and thrombocytosis and cardiac complications such as coronary artery aneurysms and myocardial infarction can occur. The convalescent phase begins when all symptoms disappear and ends when serum CRP and ESR levels return to normal. The majority of coronary aneurysms are discovered during this phase, which typically lasts four weeks.
Over the past few years the diagnosis of Incomplete Kawasaki disease has become established in emergency medical practice to guide practitioners on the care of patients who fit some but not all the criteria. For patients with 5-day fever and 3 of the 5 symptoms, if the patient is less than 6 months old then treatment is warranted. For those patients who are older than 6 months or those who fulfill only 2 of the additional criteria, CRP and ESR levels are drawn. If CRP ❤ mg/dL and ESR< 40 mm/h, then the patient is monitored for any progression of new symptoms. If CRP >3 and ESR >40, then a CBC, albumin, ALT, and urinalysis are all examined. If any three or more of the following studies are abnormal, then the patient is treated for Kawasaki disease:
- Elevated ALT
- Platelets>450,000 after 7 days of fever
However, if less than three of the studies are found to be abnormal, then the patient undergoes an echocardiogram. Abnormal echo results would warrant starting treatment.
The treatment of Kawasaki disease focuses on blocking the immune-mediated damage to the vasculature. IVIG at a dose of 2 mg/kg is infused over 12 hours. If the fever and other symptoms do not begin to subside, a repeat dose can be given. Aspirin 80 mg/kg/day divided into four doses Q6 is also given for its anti-platelet effects. A 12-lead ECG and echocardiogram should also be done to assess for any cardiac involvement. In addition to basic bloodwork to establish a diagnosis of Kawasaki or incomplete Kawasaki disease, baseline levels of CRP and ESR should be noted as well.
Back to our patient…
The patient was admitted and started on IVIG 36 mg over 12 hours and ASA 1800mg PO divided qid. The fever subsided and the erythema began to resolve. Echocardiogram showed a normal ventricular size and function and no effusion. The coronary arteries were well visualized and were of normal size. The patient was afebrile for 36 hours and was stable for discharge with low-dose aspirin for 8 weeks and followup with Pediatric cardiology in 2 weeks.
At the clinic two weeks later, the patient was asymptomatic. ESR was 11 and CRP was 1.85. A repeat ECG and Echocardiogram were normal. Repeat followup in 1 year.
- Kawasaki disease is diagnosed by 5-day fever greater than 39º C, plus four of the following: conjunctival injection, lip or pharyngeal erythema, cervical LAD >1.5 cm, rash or peeling of the trunk or genital area, or hand or foot erythema, swelling, or peeling.
- Complications of Kawasaki disease include coronary artery aneurysm, myocardial infarction, thrombocytosis, sterile pyuria, and gall bladder hydrops.
- Patients who do not fulfill the criteria for Kawasaki disease may warrant treatment if they are diagnosed with incomplete Kawasaki disease.
- Ensure prompt follow-up of pediatric patients with fever and URI symptoms who are being discharged from the ED and educate caretakers about Kawasaki disease symptoms.
This case discussion presented by Samuel Lee, MD
Filed under: Intern Report |