Kawasaki disease

by Dr Riham Arnous, Pedi­a­tri­cian, Port said Gen­er­al hos­pi­tal

Def­i­n­i­tion:

Kawasa­ki dis­ease (KD), or muco­cu­ta­neous lymph node syn­drome, is an ill­ness that caus­es inflam­ma­tion in arter­ies, veins, and cap­il­lar­ies. It also affects lymph nodes and caus­es symp­toms in  nose, mouth, and throat. It’s the most com­mon cause of heart dis­ease in chil­dren.

Mag­ni­tude Of the Prob­lem:

The Kawasa­ki Dis­ease Foun­da­tion (KDF) esti­mates that KD affects more than 4,200 chil­dren in the Unit­ed States each year. KD is also more com­mon in boys than in girls and in chil­dren of Asian and Pacif­ic Island descent. How­ev­er, KD can affect chil­dren and teenagers of all racial and eth­nic back­grounds.

In most cas­es, chil­dren will recov­er with­in a few days of treat­ment with­out any seri­ous prob­lems. Recur­rences are uncom­mon. If left untreat­ed, KD can lead to seri­ous heart dis­ease. 

What are the symp­toms of Kawasa­ki dis­ease?

Kawasa­ki dis­ease occurs in stages with tell­tale symp­toms and signs. The con­di­tion tends to appear dur­ing late win­ter and spring. In some Asian coun­tries, cas­es of KD peak dur­ing the mid­dle of sum­mer

Ear­ly stages

Ear­ly symp­toms, which can last up to two weeks, may include:

a.high fever that per­sists for five or more days

b.rash on the tor­so and groin                                          

c.bloodshot eyes, with­out crust­ing

d.bright red, swollen lips

e.“strawberry” tongue, which appears shiny and bright with red spots

f.swollen lymph nodes

g.swollen hands and feet

h.red palms and soles of the feet

Late stages

Lat­er symp­toms begin with­in two weeks of the fever. The skin on the hands and feet of your child may start to peel and come off in sheets. Some chil­dren may also devel­op tem­po­rary arthri­tis, or joint pain.

Oth­er signs and symp­toms include: abdom­i­nal pain, vom­it­ing, diar­rhea, enlarged gall­blad­der, tem­po­rary hear­ing loss

Chil­dren who are younger than 1 or old­er than 5 are more like­ly to present incom­plete symp­toms. These chil­dren make up the 25 per­cent of KD cas­es that are at a height­ened risk of expe­ri­enc­ing heart dis­ease.

Eti­ol­o­gy And Risk Fac­tors

Kawasa­ki dis­ease is most com­mon in chil­dren, par­tic­u­lar­ly those of Asian descent. About 75 per­cent of KD cas­es are chil­dren under the age of 5, accord­ing to the KDF. Researchers don’t believe that  any child  can inher­it the dis­ease, but the risk fac­tors tend to increase with­in fam­i­lies. Sib­lings of some­one who has KD are 10 times more like­ly to have the dis­ease.   

There is no spe­cif­ic diag­nos­tic test for Kawasa­ki dis­ease. A pedi­a­tri­cian will take into account the child’s symp­toms and rule out ill­ness­es with sim­i­lar symp­toms, such as:

1.scarlet fever, a bac­te­r­i­al infec­tion that caus­es fever, chills, and sore throat

2.juvenile rheuma­toid arthri­tis, a chron­ic dis­ease that caus­es joint pain and inflam­ma­tion

3.measles

4.toxic shock syn­drome

5.idiopathic juve­nile arthri­tis

6.juvenile mer­cury poi­son­ing

  7.medical reac­tion

8.Rocky Moun­tain spot­ted fever, a tick-borne ill­ness

Addi­tion­al Tests:
a.Echocardiograph: An echocar­dio­graph is a pain­less pro­ce­dure that uses sound waves to cre­ate pic­tures of the heart and its arter­ies. This test may need to be repeat­ed to show how Kawasa­ki dis­ease has affect­ed the heart over time.

 b. Blood tests: Blood tests may be ordered to rule out oth­er ill­ness­es. In KD, there may be an ele­vat­ed white blood cell count, low red blood cell count, and inflam­ma­tion.

  c.Chest X‑ray: A chest X‑ray cre­ates black and white images of the heart and lungs. A doc­tor may order this test to look for signs of heart fail­ure and inflam­ma­tion.

  d.Elec­tro­car­dio­gram: An elec­tro­car­dio­gram, or ECG, records the elec­tri­cal activ­i­ty of the heart. Irreg­u­lar­i­ties in the ECG may indi­cate that the heart has been affect­ed by KD.

 N.B. Kwasa­ki dis­ease should be con­sid­ered a pos­si­bil­i­ty in any infant or child who has a fever last­ing more than five days. This is espe­cial­ly the case if they’re show­ing oth­er clas­sic symp­toms of the dis­ease like peel­ing skin.   

  How is Kawasa­ki dis­ease treat­ed?

Chil­dren diag­nosed with KD should  start  treat­ment imme­di­ate­ly to pre­vent heart dam­age.

First-line treat­ment for KD involves an infu­sion of anti­bod­ies (intra­venous immunoglob­u­lin) over 12 hours with­in 10 days of the fever and a dai­ly dosage of aspirin over the next four days. The child may need to con­tin­ue to take low­er dos­es of aspirin for six to eight weeks after the fever goes away to pre­vent the for­ma­tion of blood clots.

One study also found that the addi­tion of pred­nisolone sig­nif­i­cant­ly reduced poten­tial heart dam­age. But this has yet to be test­ed in oth­er pop­u­la­tions.

Tim­ing is crit­i­cal for pre­vent­ing seri­ous heart prob­lems. Stud­ies also report a high­er rate of resis­tance to treat­ment when it’s giv­en before the fifth day of the fever. About 11 to 23 per­cent of chil­dren with KD will have a resis­tance.

Some chil­dren may require longer treat­ment time to pre­vent a blocked artery or a heart attack. In these cas­es, treat­ment involves dai­ly antiplatelet aspirin dos­es until they have a nor­mal echocar­dio­graph. It may take six to eight weeks for coro­nary artery abnor­mal­i­ties to reverse.

 Com­pli­ca­tions:

 KD leads to seri­ous heart prob­lems in about 25 per­cent of the chil­dren who have the dis­ease. Untreat­ed KD can lead increase risk for a heart attack and cause: myocardi­tis, dys­rhyth­mia or aneurysm. Treat­ment for this stage of the con­di­tion requires long-term dos­ing of aspirin. Patients may also need to take blood thin­ners or under­go pro­ce­dures such as coro­nary angio­plas­ty, coro­nary artery stent­ing, or coro­nary artery bypass.

     

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