Kawasaki_Disease_Flashcards
What is Kawasaki disease?
Kawasaki disease is a type of vasculitis predominantly seen in children, notable for its potential to cause coronary artery aneurysms.
What are the key features of Kawasaki disease?
Features include high-grade fever lasting more than 5 days and resistant to antipyretics, conjunctival injection, bright red cracked lips, strawberry tongue, cervical lymphadenopathy, and red palms and soles that later peel.
How is Kawasaki disease diagnosed?
Kawasaki disease is diagnosed clinically, as there is no specific diagnostic test available.
What is the management for Kawasaki disease?
Management includes high-dose aspirin, intravenous immunoglobulin, and an echocardiogram to screen for coronary artery aneurysms.
Why is aspirin used in children with Kawasaki disease despite general contraindications?
Aspirin is used in Kawasaki disease because it is one of the few conditions where the benefits outweigh the risks, such as Reye’s syndrome, which is why aspirin is generally avoided in other pediatric conditions.
What are the complications associated with Kawasaki disease?
The major complication of Kawasaki disease is coronary artery aneurysms.
summarise KAWASAKI
Kawasaki disease
Kawasaki disease is a type of vasculitis which is predominately seen in children. Whilst Kawasaki disease is uncommon it is important to recognise as it may cause potentially serious complications, including coronary artery aneurysms.
Features
high-grade fever which lasts for > 5 days. Fever is characteristically resistant to antipyretics
conjunctival injection
bright red, cracked lips
strawberry tongue
cervical lymphadenopathy
red palms of the hands and the soles of the feet which later peel
Kawasaki disease is a clinical diagnosis as there is no specific diagnostic test.
Management
high-dose aspirin
Kawasaki disease is one of the few indications for the use of aspirin in children. Due to the risk of Reye’s syndrome aspirin is normally contraindicated in children
intravenous immunoglobulin
echocardiogram (rather than angiography) is used as the initial screening test for coronary artery aneurysms
Complications
coronary artery aneurysm
A 2-year-old child presents to the emergency department accompanied by her mother. She has suffered from a fever in the past six days, accompanied by a sore throat.
On examination, the child appears febrile and is crying. There is evidence of an inflamed throat, crimson red oral mucosa and tongue, cervical lymphadenopathy and injected conjunctivae. The child was previously healthy with no past medical history.
What is the most appropriate treatment?
Administer varicella-zoster immunoglobulin
Prescribe analgesia and ask to come back in 5 days for review
Prescribe oral azithromycin for 10 days
Prescribe oral high-dose aspirin for 14 days
Prescribe oral penicillin V for 10 days
Prescribe oral high-dose aspirin for 14 days
High dose aspirin is indicated in Kawasaki disease, despite it usually being contraindicated in children
The correct answer is to prescribe oral high-dose aspirin for 14 days. The patient is presenting with Kawasaki disease, a form of vasculitis that primarily affects children. The diagnosis is supported by the presence of fever persisting for more than six days, conjunctival injection, erythematous and fissured lips, strawberry tongue, cervical lymphadenopathy, and pharyngitis which may be related to the initial illness that triggered the vasculitis.
Despite the association between aspirin use in children and Reye’s syndrome—a rare but serious condition characterised by acute encephalopathy and fatty liver degeneration—the benefits of treating Kawasaki disease outweigh the risks. High-dose aspirin is indicated due to the potential complications of untreated Kawasaki disease, such as coronary artery aneurysms.
Administer varicella-zoster immunoglobulin is not an appropriate option in this scenario. This treatment is reserved for immunocompromised individuals or neonates exposed to varicella peripartum. It would not be suitable for this patient who does not exhibit signs of varicella infection, such as an evolving rash from macules to papules to vesicles.
Prescribe analgesia and ask to come back in 5 days for review is incorrect. Given the severity of Kawasaki disease and its potentially life-threatening complications like coronary artery aneurysms, this management approach would be insufficiently proactive.
Prescribing oral azithromycin for 10 days is also incorrect. Azithromycin may be indicated for patients with scarlet fever who are allergic to penicillin. However, this patient does not present with scarlet fever; although they exhibit pharyngitis and strawberry tongue, they lack the pathognomonic ‘sandpaper’ rash and display signs consistent with vasculitis including conjunctival injection.
Prescribing oral penicillin V for 10 days is similarly inappropriate. While it is the antibiotic of choice for scarlet fever, no indications are suggesting that this patient has scarlet fever given the absence of a ‘sandpaper’ rash and the presence of vasculitic features such as conjunctival injection.
A mother arrives at the paediatric emergency department with her 4-year-old boy. He has a fever and she has noticed raised nodes on his neck. She has given him paracetamol and ibuprofen but his temperature is not reducing. His lips have become extremely dry and cracked and his tongue red and slightly swollen. She has noticed that his feet are also red and puffy now, and he is developing a widespread fine rash. What is the most likely diagnosis?
Hand, foot and mouth disease
Kawasaki’s disease
Measles
Parvovirus B19
Scarlet Fever
Kawasaki’s disease
High fever lasting >5 days, red palms with desquamation and strawberry tongue are indicative of Kawasaki disease
This presentation is typical of Kawasaki’s disease. It presents with a high fever that is not very responsive to paracetamol or ibuprofen. The patient then develops a ‘strawberry tongue,’ dry cracked lips and inflamed mucosa. Erythema and oedema are followed by desquamation of the extremities. Hand, foot and mouth disease starts with general malaise and pyrexia, however skin lesions in the form of vesicles appear in the mouth. 75% develop an eruption on the hands and feet with tender papule and vesicles. Measles presents with a fever and coryzal symptoms. Koplick’s spots can develop on the oral mucosa however these are bright red with a bluish white speck at the centre. A maculo-papular rash arrives 3-5 days later. Parvovirus B19 is also known as ‘slapped cheek syndrome. Scarlet fever presents with an inflamed tongue also, however would not explain the red and puffy feet which later desquamate.
A 3-year-old boy presents to the emergency department with a 6-day history of fever peaking at 39.0ºC. His mother reports that he also has a swollen, red tongue. On clinical examination, palpable lymph nodes were noted on the left anterior neck, his lips were dry and cracked, and a maculopapular rash was concentrated around the trunk.
What is the most appropriate management for this patient based on the likely diagnosis?
IV immunoglobulins
IV immunoglobulins and oral high-dose aspirin
IV immunoglobulins and oral ibuprofen
IV immunoglobulins and oral low-dose aspirin
Observation
IV immunoglobulins and oral high-dose aspirin
High dose aspirin is indicated in Kawasaki disease, despite it usually being contraindicated in children
IV immunoglobulins and oral high-dose aspirin is correct. The clinical presentation of a fever exceeding 38 degrees Celsius for more than five days, accompanied by a strawberry tongue, cervical lymphadenopathy, and a maculopapular rash, is indicative of Kawasaki disease—a vasculitis that predominantly affects medium-sized vessels, including the coronary arteries. Intravenous immunoglobulins, specifically gamma globulins, are administered primarily to prevent cardiac complications. Aspirin is prescribed at high doses due to its anti-inflammatory properties that assist in reducing fever and alleviating pain; this is despite it being generally contraindicated in children under 16 years due to the risk of Reye’s syndrome.
IV immunoglobulins is incorrect. The six-day history of fever combined with symptoms such as a strawberry tongue, cervical lymphadenopathy, and a maculopapular rash suggests Kawasaki disease as the most likely diagnosis. Although IV immunoglobulins are critical in treating Kawasaki disease to reduce the risk of cardiac complications, optimal management also necessitates the use of oral high-dose aspirin for its anti-inflammatory effects. Consequently, this option is less suitable.
IV immunoglobulins and oral ibuprofen is incorrect. The six-day history of fever along with features like a strawberry tongue, cervical lymphadenopathy, and a maculopapular rash points towards Kawasaki disease as the probable diagnosis. While ibuprofen possesses anti-inflammatory properties, it does not constitute part of the standard treatment regimen for Kawasaki disease; instead, high-dose aspirin is utilised.
IV immunoglobulins and oral low-dose aspirin is incorrect. The presence of a six-day fever together with signs such as a strawberry tongue, cervical lymphadenopathy, and a maculopapular rash supports the diagnosis of Kawasaki disease. Oral low-dose aspirin does not form part of acute phase management in Kawasaki disease; however, it becomes integral to standard care after fever resolution. Low-dose aspirin therapy continues until 6-8 weeks post-onset of symptoms as an antiplatelet agent to prevent clot formation in the coronary arteries.
Observation is incorrect. Given the six-day duration of fever coupled with findings such as a strawberry tongue, cervical lymphadenopathy and a maculopapular rash indicative of Kawasaki disease, adopting a conservative approach would be inappropriate due to potential severe cardiac complications or mortality if left untreated.
buzz words
A 2-year-old child presents to the emergency department accompanied by her mother. She has suffered from a fever in the past six days, accompanied by a sore throat.
On examination, the child appears febrile and is crying. There is evidence of an inflamed throat, crimson red oral mucosa and tongue, cervical lymphadenopathy and injected conjunctivae. The child was previously healthy with no past medical history.
A 1-year-old boy of Asian ethnicity is admitted to the hospital with a history of fevers reaching 39.9°C and marked irritability for the past seven days. Oral ibuprofen, paracetamol and antibiotics have had no effect.
On examination, he presents with a widespread blanching maculopapular rash, bilateral conjunctival injection, and enlarged cervical lymph nodes. Further examination reveals dry, erythematous, fissured lips and a strawberry tongue. Both Kernig’s and Brudzinski’s signs are negative.
What is the most appropriate definitive management for this patient?
High dose prednisolone
High-dose aspirin
Intravenous ceftriaxone
Intravenous fluids
Intravenous paracetamol
High-dose aspirin
High dose aspirin is indicated in Kawasaki disease, despite it usually being contraindicated in children
The clinical presentation of this patient strongly indicates Kawasaki disease, which is the second most common form of vasculitis in children, with IgA vasculitis being the most common. The risk factors for Kawasaki disease, which are present in this patient, include male gender, age between three months and four years, and Asian ethnicity. Other classic features of Kawasaki disease are:
High-grade fever, which lasts for > 5 days. Fever is characteristically resistant to antipyretics
Conjunctival injection
Bright red, cracked lips
Strawberry tongue
Cervical lymphadenopathy
Red palms of the hands and the soles of the feet, which later peel
High-dose aspirin is the correct answer. High-dose aspirin is indicated in Kawasaki disease despite being usually contraindicated in children. The most serious sequelae of Kawasaki disease is coronary artery aneurysm. Although aspirin does not reduce the risk of developing coronary artery aneurysms, administration of intravenous immunoglobulins (IVIG) has been shown to lower this risk. Consequently, aspirin should be administered alongside IVIG therapy in this patient’s case.
High-dose prednisolone is incorrect. However, corticosteroids could be considered if this patient presented with severe Kawasaki disease, had an increased risk of coronary complications, or proved resistant to initial treatment. It is best to treat this patient with aspirin at this early stage, so corticosteroids are not indicated yet.
Intravenous ceftriaxone is incorrect. This would be an appropriate therapeutic choice for meningococcal meningitis but is not indicated here. Meningitis is less likely given that both Kernig’s and Brudzinski’s signs are absent in this patient. Furthermore, this patient’s clinical features align more closely with Kawasaki disease.
Intravenous fluids is incorrect. While intravenous fluids may be administered as supportive care during treatment, they do not constitute definitive management for Kawasaki disease. High-dose aspirin remains the superior option for this patient.
Intravenous paracetamol is incorrect. This has proven ineffective in the vignette (albeit as an oral tablet). Fevers linked to Kawasaki disease typically do not respond to paracetamol therapy. Therefore, treating this patient with paracetamol is unlikely to represent the definitive management of the patient.
A 4-year-old boy is reviewed in the Paediatric Admissions Unit. He has had a fever for the past week. On examination he has red, sore lips and conjunctival injection. He also has swollen, red hands. Blood tests show:
Hb 13.1 g/dl
WBC 12.7 *109/l
Platelets 520 *109/l
CRP 96 mg/L
What is the likely diagnosis?
Rheumatic fever
Dermatomyositis
Lyme disease
Still’s disease
Kawasaki disease
Kawasaki disease
High fever lasting >5 days, red palms with desquamation and strawberry tongue are indicative of Kawasaki disease
The correct answer is Kawasaki disease. Kawasaki disease is an acute febrile vasculitis of childhood that predominantly affects children under the age of 5. It presents with fever lasting for at least five days, along with other clinical features such as bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities (e.g., swelling or redness), rash, and cervical lymphadenopathy. The patient’s age, fever duration, and presence of characteristic findings (red, sore lips; conjunctival injection; swollen, red hands) are consistent with this diagnosis. Additionally, elevated CRP and thrombocytosis are common laboratory findings in Kawasaki disease.
Rheumatic fever is a less likely diagnosis because it usually presents with migratory polyarthritis affecting large joints, carditis (including valvular involvement), chorea, erythema marginatum (a non-itchy rash), and subcutaneous nodules. Although fever can be present in rheumatic fever, the clinical features described in the question do not support this diagnosis.
Dermatomyositis typically manifests as a progressive proximal muscle weakness accompanied by a characteristic skin rash involving the face, neck and upper trunk. While dermatomyositis may present with fever and skin manifestations such as Gottron’s papules or heliotrope rash around eyes or on knuckles/elbows/knees/ankles/toes respectively, it does not involve lip erythema or conjunctival injection commonly seen in Kawasaki disease.
Lyme disease is caused by infection with the spirochete Borrelia burgdorferi, transmitted through the bite of infected Ixodes ticks. Early Lyme disease can present with a characteristic erythema migrans rash, fever, and malaise. However, the clinical features described in the question, such as lip erythema and conjunctival injection, are not typical of Lyme disease. Additionally, Lyme disease is less common in children aged 4 years.
Still’s disease, also known as systemic juvenile idiopathic arthritis (sJIA), is a rare form of chronic arthritis in children characterized by high spiking fevers, an evanescent salmon-pink rash, and arthritis. Although the patient has fever and rash, the absence of joint involvement makes Still’s disease less likely than Kawasaki disease given the clinical presentation.
