Kawasaki disease

From WikiLectures

Mucocutaneus lymph node syndrome otherwise known as Kawasaki disease is a Vasculitis unknown etiologic. It causes multisystem inflammatory disease targeting small and medium size arteries, leading to a creation of aneurysms.The disease targets mainly children up to the age of 5 with a maximum between second and third year of age. The highest incidence is in Japan.[1] Asian children are affected 6 times more often than Caucasian ones. In the Czech Republic the incidence is guessed to be 1.6/100 000 children up to 5 years.[2]

Clinical picture[edit | edit source]

Acute phase[edit | edit source]

  • Sudden fevers (over 40°C);
  • bilateral non-purulent conjunctivitis;
  • changes to mucous (dry, cracked lips, erythema of buccal mucosa and strawberry tongue);
  • cervical Lymphadenopathy (more than 1,5cm in diameter) – in 70% of cases[1];
  • erythema and tough swelling of hands and feet + swelling of PIP hand joints[2];
  • polymorphic exanthem mostly in inguinal region and on thorax- in 80% of cases[1];
  • symptoms of acute myocarditis (sinus tachycardia, gallop rhythm, weak heart sounds)[2];
  • other: stomach pains, gallbladder hydrops, pleocytosis in CSF, arthritis (middle size and large size joints);
  • lasts 1–2 weeks[1].

Subacute phase[edit | edit source]

  • Desquamation of skin (mainly of fingers);
  • thrombocytosis(as much as 1012/liter)[2];
  • creation of aneurysms of coronary arteries - risk of Sudden death;
    • risk factors: prolonged fevers, prolonged elevation of inflammation markers, age under 1year, male sex;
  • lasts till the fourth week[1].

Recovery phase[edit | edit source]

  • From disappearance of clinical symptoms to decline of inflammation markers to normal (usually 6.-8. weeks from the first symptoms);
  • the Beau's lines can appear on nails during this phase [1].

Diagnostics[edit | edit source]

  • Exclude other causes of fever (infection);
  • Hemoculture, cultivation of urine, thorax X-RAY;
  • heightened inflammation markers, thrombocytosis;
  • Lumbar puncture (to exclude infection) – pleocytosis;
  • echo – evidence of aneurysms of coronary arteries[1].

Diagnostic criteria[edit | edit source]

  • Fever lasting longer than 5 days and minimally 4 of the following:
    • bilateral non-purulent conjunctivitis;
    • reddened, dry cracked lips, raspberry tongue, …;
    • peripheral erythema, peripheral edema, pealing of skin on fingers, generalized desquamation;
    • polymorphic exanthema on the abdomen, the thorax and the area of genitals;
    • cervical lymphadenopathy (over 1,5 cm in average)[1].

Treatment[edit | edit source]

  • i. v. imunoglobulin (IVIG);
  • Acetylsalicylic acid (Aspirin) in anti-inflammation dose (80–100mg/kg/den) in acute phase and in antiaggregating dose (3–5mg/kg/den) in other phases[1].

References[edit | edit source]

Related articles[edit | edit source]

Reference[edit | edit source]

  1. a b c d e f g h i KLIEGMAN, Robert M. – MARCDANTE, Karen J. – JENSON, Hal B.. Nelson Essentials of Pediatrics. 1. edition. China : Elsevier Saunders, 2006. 5; pp. 430-432. ISBN 978-0-8089-2325-1.
  2. a b c d JEHLIČKA, Petr – LÁD, Václav – SEDLÁČEK, Dalibor. Kawasakiho syndrom. Pediatrie pro praxi [online]2008, y. 9, p. 12-14, Available from <http://www.pediatriepropraxi.cz/artkey/ped-200801-0003.php>. ISSN 1803-5264. 

External links[edit | edit source]