Kawasaki disease

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]

Bilateral, non-exudativ conjunctival injection
Changes to mucouses - red and swollen lips with vertical cracks and haemorrhaging, raspberry tongue
Erythema and tough swelling of hands and feet + swelling of PIP hand joints
Erythematous exanthema affecting perineum

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]

Reference[edit | edit source]

↑ ^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.
↑ ^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]

[Nelson-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.

[ppp-2] 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.

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