A 4-year-old male has a fever of 1 week’s duration. It has been at or slightly above 38°C (101°F) and has responded poorly to antipyretics. The patient complains of photophobia, burning in his eyes, and a sore throat. His mother also notes that his eyes look red, his lips are red and cracked, and he has a “strawberry tongue”. The child’s palms and soles are erythematous and the peri-ungal regions show desquamation of the skin. He has minimally painful nodes located in the anterior cervical region, about 2 x 2 cm in size. A Streptococcus screen is negative.
The most appropriate management at this time would be:
Correct Answer C:
Kawaski disease, or mucocutaneous lymph node syndrome, is a common form of vasculitis in childhood. It is typically self-limited, with fever and acute inflammation lasting 12 days on average without therapy. However, if untreated, this illness can result in heart failure, coronary artery aneurysm, myocardial infarction, arrhythmias, or occlusion of peripheral arteries. It is most common in those under the age of 5 years. To diagnose this disease, fever must be present for 5 days or more with no other explanation.
In addition, at least four of the following symptoms must be present:
The disease must be diagnosed from toxic shock syndrome, streptococcal scarlet fever, Stevens-Johnson syndrome, juvenile rheumatoid arthritis, measles, adenovirus infection, echovirus infection, and drug reactions.
Treatment significantly diminishes the risk of complications. Current recommendations are to hospitalize the patient for treatment with intravenous immune globulin. In addition, aspirin is used for both its anti-inflammatory and antithrombolitic effects. While prednisone is used to treat other forms of vasculitis, it is considered unsafe in Kawaski disease, as a previous study showed an extraordinarily high rate of coronary artery aneurysm with its use.
You are seeing a previously healthy 4-year-old girl with a 5-day history of fever, itching truncal rash, and bilateral foot pain. Her fever has been as high as 39.5°C. The rash is described by her mother as “feeling rough”. She complains of pain in her feet when she is wearing shoes, and has refused to walk today. She has not had any recent URI symptoms and there has been no emesis or diarrhea. There have been no known ill contacts; however, she is in daycare. Physical examination is significant for a mildly dehydrated female who appears ill, but not toxic. Her temperature is 38.5°C. There is bilateral bulbar and conjunctival injection without exudates. Her lips are dry and cracked. Her tongue is erythematous and without lesions. There are no palatal petechia and her posterior pharynx is benign without exudates. She has tender anterior cervical lymph nodes measuring 2-3 cm. Lungs are clear. There are no murmurs, and extremities are well perfused. Examination of the skin reveals a fine mildly erythematous sandpaper-like truncal rash with areas of excoriation. Both hands and feet are edematous. A rapid strep test is negative.
The most appropriate next step would be:
Correct Answer D:
This child has Kawasaki disease, as characterized by the non-exudate conjunctival injection, polymorphous non-vesicular rash, mucosal involvement, edema of the hands and feet, and cervical lymphadenopathy. Due to the cardiac complications (coronary aneurysms), it is essential to consult a cardiologist to evaluate for myocarditis and possible aneurysm development.
A. This patient should be admitted for a complete evaluation.
B. This treatment plan is incomplete.
C. Although streptococcal pharyngitis and scarlet fever are in the differential, this plan does not address other diagnostic possibilities.
E. Cardiology should be notified and involved as early as possible when Kawaski’s disease is suspected, to assist with diagnosis, treatment, and long-term follow-up.
A 4-year-old boy was brought to hospital with fever for the last 6 days. The boy looked ill but not pale or jaundiced and his body temperature was 38.6°C. Examination of the mouth showed generalized erythema of oropharynx, strawberry tongue and red fissured lips. Polymorphous, confluent maculo-papular erythematous skin rash was noted. Two enlarged cervical lymph nodes could be palpated. Laboratory examination showed mild anemia and raised ESR. Kawasaki disease was strongly suspected. Although, scarlet fever and toxic shock syndrome should be considered in the differential diagnosis of this presentation, they are less likely because of:
Correct Answer B:
Fissuring of the lips is a strong clue for differentiating Kawasaki disease from scarlet fever and toxic shock syndrome.
→ Scarlet fever and toxic shock syndrome might have the same type of scarlatiniform rash as Kawasaki disease described in this case.
→ Mild anemia, the ill look and high ESR might all accompany severe scarlet fever and toxic shock syndrome.
Careful physical examination (fissuring of the lips in this case) is a strong tool in differentiating between diseases with similar presentations.
An 11-year-old patient presents with petechiae and mucosal bleeding. His initial labs show a low platelet count. He is eventually diagnosed with idiopathic thrombocytopenic purpura (ITP).
What is the most appropriate treatment?
Correct Answer A: Idiopathic thrombocytopenic purpura is a bleeding disorder caused by thrombocytopenia not associated with a systemic disease. Typically, it is chronic in adults but is usually acute and self-limited in children. Spleen size is normal. Diagnosis requires that other disorders be excluded through selective tests. Treatment includes corticosteroids, splenectomy, and, for life-threatening bleeding, platelet transfusions, and IV immune globulin.
A 12-year-old boy comes to your office because of unexplained bruising. There is no history of previous bruising or excessive bleeding. The child has had no fever or respiratory symptoms. Three weeks ago, the child had chickenpox (varicella). On examination today, the patient is cooperative and in no acute distress. A complete CBC has the following results:
Large platelets are seen on the smear. Prothrombin time and activated partial thromboplastin time (APPT) are normal.
Treatment of this child should include:
This patient has idiopathic thrombocytopenic pupura (ITP), a condition that often follows viral infection or immunization. Most cases resolve spontaneously without therapy. Children who have platelet counts > 30 x 109/L and are asymptomatic or have only minor purpura do not require routine treatment. Children who have platelet counts < 20 x 109/L and significant mucous membrane bleeding and those who have platelet counts < 10x10^9/L and minor purpura should receive specific treatment.
Usually choose to treat because spontaneous recovery takes a few months, and risk of bleeding (especially intracranial hemorrhage with platelets < 20 x109 /L).
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