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Síndrome de Kawasaki

Síndrome de Kawasaky Vall d´Hebron

Kawasaki disease is a rare condition that causes blood vessel inflammation. It mainly affects breastfeeding babies and children under the age of five.

Diagnosis is based on clinical examination. Treatment consists of administering aspirin and immune globulin intravenous (IGIV).

To avoid possible heart sequelae, early diagnosis and correct treatment are essential.

Description

Kawasaki disease is a form of systemic vasculitis that affects small and medium-sized blood vessels. The main complication of the condition is the formation of aneurysms in the coronary arteries. It is an acute and self-limiting inflammatory process.

 

Symptoms:

  • Irritability is a very common sign (100% of cases).
  • Fever(temperature over 39ºC) that cannot be controlled using antipyretics and lasts 5-25 days, with an average of 10 days.
  • Conjunctivitis, bilateral non-exudative.
  • Exanthema: Different types of skin rash.
  • Skin alterations in extremities: redness and hardening on the palms of the hands and bottoms of feet. Peeling on fingertips 10-15 days from the start of the illness.
  • Alterations to oral cavity: redness, cracking and formation of scabs on the lips. There may also be widespread redness in the mouth or so-called strawberry” tongue.
  • Enlargement of lymph node in neck on one side, painful, hard and more than 1.5 cm in diameter. Reduces as fever reduces.
  • Other symptoms, depending on each specific case.

 

Things that should be done when symptoms appear:

1. Avoid using soap as this may cause the skin to dry further. Wear soft, flannel or cotton clothing.

2. Massage the skin with neutral hydrating cream.

3. Stop the child rubbing their eyes and protect them from light due to light sensitivity.

4. Ensure the child gets plenty of rest as it reduces irritability.

5. Apply passive exercises with soft toys and calm games.

 

Epidemiological data:

Kawasaki disease is the main cause of acquired heart disease in children in developing countries. Greater incidence of the disease is observed in Asian countries, but distribution is currently universal and it is found in all races.  It is more common in middle and upper socio-economic classes and in winter and spring.

 

Diagnosis:

Diagnosis of Kawasaki disease requires the presence of a fever for five days along with four criteria, or the presence of fever with three criteria if there is no other cause. The criteria are: bilateral conjunctival injection; alteration of labial mucosa and/or pharynx or “strawberry” tongue; alterations in hands and feet; skin rash; lymph nodes in the neck greater than 1.5 cm. 

There is no specific laboratory test to diagnose this disorder.

Given that the symptoms of Kawasaki disease are non-specific and common to other conditions, a differential diagnosis must be conducted to correctly assess the treatment required.

 

Treatment:

The aim of treatment is to reduce inflammation in the myocardial coronary arteries, alleviate the symptoms and prevent clotting by inhibiting platelet aggregation. Immune globulin intravenous allows the disease to be cured in most cases and avoids heart sequelae. Treatment with acetylsalicylic acid (aspirin) is also used due to its anti-clotting properties.

Treatment with immune globulin is effective in reducing symptoms and avoiding coronary aneurysms. It is essential to start treatment between the fifth and tenth day of the disease.

 

 

Hospital o serveis complementaris relacionats

Children's Hospital and Woman's Hospital

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Dr. Sebastià
González Peris
Medical Coordinator
Pediatric Emergency
 
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