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Kawasaki disease
ICD-10 M30.3
ICD-9 446.1
OMIM 611775
DiseasesDB 7121
MedlinePlus 000989
eMedicine ped/1236
MeSH D009080
Kawasaki disease (KD), also known as Kawasaki syndrome, lymph node syndrome
and Mucocutaneous lymph node syndrome,[1] is an autoimmune disease that manifests
as a systemic necrotizing medium-sized vessel vasculitis and is largely seen in children
under 5 years of age. It affects many organ systems, mainly those including the blood
vessels, skin, mucous membranes and lymph nodes; however, its most serious effect is on
the heart where it can cause severe coronary artery aneurysms in untreated children.
Without treatment, mortality may approach 1%, usually within 6 weeks of onset. With
treatment, the mortality rate is less than 0.01% in the U.S.[2] There is often a pre-existing
viral infection that may play a role in its pathogenesis.[3] The conjunctival and oral
mucosa, along with the epidermis (skin), become erythematous (red and inflamed).
Edema is often seen in the hands and feet and the cervical lymph nodes are often
enlarged. Also, a remittant fever, often 40℃ (104°F) or higher, is characteristic of the
acute phase of the disease.[4] In untreated children, the febrile period lasts on average
approximately ten days, but may range from 5 to 25 days.[4] The disorder was first
described in 1967 by Dr. Tomisaku Kawasaki in Japan.[5]
Contents
[hide]
• 1 Classification
• 2 Signs and symptoms
o 2.1 Complications
• 3 Causes
• 4 Diagnosis
o 4.1 Investigations
• 5 Treatment
• 6 Prognosis
• 7 Epidemiology
• 8 See also
• 9 References
• 10 External links
[edit] Classification
Systemic vasculitis is an inflammatory condition affecting both veins and arteries
throughout the body, and is usually caused by a proliferation of cells associated with an
immune response to a pathogen, or autoimmunity.[6] Systemic vasculitides may be
classified according to the type of cells involved in the proliferation, as well as the
specific type of tissue damage occurring within the vein or arterial walls.[6] Under this
classification scheme for systemic vasculitis, Kawasaki disease is considered to be a
necrotizing vasculitis (also called necrotizing angeititis), which may be identified
histologically by the occurrence of necrosis (tissue death), fibrosis, and proliferation of
cells associated with inflammation in the inner layer of the vascular wall.[6][7] Other
diseases featuring necrotizing vasculitis include Polyarteritis nodosa, Wegener's
granulomatosis, Henoch-Schönlein purpura and Churg-Strauss syndrome.[6] Kawasaki
disease may be further classified as a medium-sized-vessel vasculitis, affecting medium
and small sized blood vessels,[8][9][10] such as the smaller cutaneous vasculature (veins and
arteries in the skin) that range from 50 to 100µm in diameter.[11][12] KD is also considered
to be a primary childhood vasculitis, a disorder associated with vasculitis that mainly
affects children under the age of 18.[13][14] A recent, consensus-based evaluation of
vasculitides occurring primarily in children resulted in a classification scheme for these
disorders, to both distinguish them and suggest a more concrete set of diagnostic criteria
for each.[14] Within this classification of childhood vasculitides, Kawasaki disease is,
again, a predominantly medium-sized vessel vasculitis.[14]
Kawasaki disease often begins with a high and persistent fever that is not very responsive
to normal treatment with paracetamol (acetaminophen) or ibuprofen.[17][18] The fever may
persist steadily for up to two weeks and is normally accompanied by irritability.[17][18]
Affected children develop red eyes because of non-suppurative conjunctivitis, iritis[19] and
bilateral anterior uveitis.[20] Inflammation of the mucous membranes in the mouth,[4] along
with erythema (redness), edema (swelling) with fissures (cracks in the lip surface),
desquamation (peeling) and exsudation of the lips are also evident. The oropharynx
mucosa has enanthema and the tongue maintains an unusual red appearance termed
"strawberry tongue" (marked erythema with prominent gustative papillae).[12] Keratic
precipitates (detectable by a slit lamp but usually too small to be seen by the unaided
eye), and swollen lymph nodes may also be present and can be the first manifestation of
the disease.[17][21] Rashes occur early in the disease, and the cutaneous rash observed in
patients with KD is non-specific, polymorphic, non-itchy and normally observed up to
the 5th day of fever. Cutaneous exanthema may comprise macular-papular erythematous
and fissure lesions, the most common type, in addition to urticariform type rash, purpuric,
multiform-like erythema.[22] and peeling of the skin in the genital area, hands, and feet
(especially around the nails and on the palms and soles) may occur in later phases.[17][23]
Some of these symptoms may come and go during the course of the illness. It is a
syndrome affecting multiple organ systems, and in the acute stage of KD, systemic
inflammatory changes are evident in many organs.[9] Myocarditis,[24] pericarditis,
valvulitis, aseptic meningitis, pneumonitis, lymphadenitis, and hepatitis may be present
and are manifested by the presence of inflammatory cells in the affected tissues.[9] If left
untreated, some symptoms will eventually relent, but coronary artery aneurysms will not
improve, resulting in a significant risk of death or disability due to myocardial infarction
(heart attack).[12] If treated in a timely fashion, this risk can be mostly avoided and the
course of illness cut short.[25]
• High-grade fever (greater than 39 °C or 102 °F; often as high as 40 °C or 104 °F),
[12]
The duration of fever is on average one to two weeks; in the absence of
treatment, it may extend for three to four weeks.[12] However, when appropriate
therapy is started the fever is gone after two days.[17]
• Red eyes (conjunctivitis) bilateral without pus or drainage, also known as
"conjunctival injection".[19]
• Anterior uveitis.[19]
• Bright red, chapped, or cracked lips.[12]
• Red mucous membranes in the mouth.[12]
• Strawberry tongue, white coating on the tongue or prominent red bumps (papillae)
on the back of the tongue.[12]
• Red palms of the hands and the soles of the feet.[12]
• Peeling (desquamation) palms and soles (later in the illness); peeling may begin
around the nails.[4][17]
• Rash which may take many forms, non-specific, polymorphic, non-itchy, but not
vesicle-bullous lesions, and appears on the trunk.[12]
• Swollen lymph nodes (frequently only one lymph node is swollen, and is usually
on onc side), particularly in the neck area.[23]
• Joint pain (arthralgia) and swelling, frequently symmetrical, Also arthritis can
occur.[12]
• Irritability.[12]
• Tachycardia (rapid heart beat).[12]
• Beau's lines (transverse grooves on nails).[12]
• May find breathing difficult.[12]
[edit] Complications
X-ray showing Aneurysmal enlargement of the coronary arteries, which is the most
feared complication in a Kawasaki syndrome
The cardiac complications are the most important aspect of the disease. Kawasaki disease
can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and
subsequent coronary artery aneurysms. These aneurysms can lead to myocardial
infarction (heart attack) even in young children. Overall, about 10–18% of children with
Kawasaki disease develop coronary artery aneurysms with much higher prevalence
among patients who are not treated early in the course of illness. Kawasaki disease and
rheumatic fever are the most common causes of acquired heart disease among children in
the United States.[27][28]
[edit] Causes
Like all autoimmune diseases, the cause of Kawasaki disease is presumably the
interaction of genetic and environmental factors, possibly including an infection. The
specific cause is unknown,[29][30][31] but current theories center primarily on immunological
causes for the disease. Evidence increasingly points to an infectious etiology,[32] but
debate continues on whether the cause is a conventional antigenic substance or a
superantigen.[33] Children's Hospital Boston reports that "[s]ome studies have found
associations between the occurrence of Kawasaki disease and recent exposure to carpet
cleaning or residence near a body of stagnant water; however, cause and effect have not
been established."[28]
An association has been identified with a SNP in the ITPKC gene, which codes an
enzyme that negatively regulates T-cell activation.[34] An additional factor that suggests
genetic susceptibility is the fact that regardless of where they are living, Japanese
children are more likely than other children to contract the disease.[28] The HLA-B51
serotype has been found to be associated with endemic instances of the disease.[35]
[edit] Diagnosis
Criteria for Diagnosis of
Kawasaki Disease
Fever of ≥5 days' duration
associated with at least 4† of
the following 5 changes
Bilateral nonsuppurative
conjunctivitis
One of more changes of the
mucous membranes of the
upper respiratory tract,
including pharyngeal injection,
dry fissured lips, injected lips,
and "strawberry" tongue
One or more changes of the
extremities, including
peripheral erythema, peripheral
edema, periungual
desquamation, and generalized
desquamation
Polymorphous rash, primarily
truncal
Cervical lymphadenopathy
>1.5 cm in diameter
Disease cannot be explained by
some other known disease
process
†A diagnosis of Kawasaki
disease can be made if fever
and only 3 changes are present
in conjunction with coronary
artery disease documented by
two-dimensional
echocardiography or coronary
angiography.
Source: Nelson's essentials of
pediatrics,[36] Review[37]
Kawasaki disease can only be diagnosed clinically (i.e. by medical signs and symptoms).
There exists no specific laboratory test for this condition. It is difficult to establish the
diagnosis, especially early in the course of the illness, and frequently children are not
diagnosed until they have seen several health care providers. Many other serious illnesses
can cause similar symptoms, and must be considered in the differential diagnosis,
including scarlet fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood
mercury poisoning (infantile acrodynia).[citation needed]
Classically, five days of fever[38] plus four of five diagnostic criteria must be met in order
to establish the diagnosis. The criteria are: (1) erythema of the lips or oral cavity or
cracking of the lips; (2) rash on the trunk; (3) swelling or erythema of the hands or feet;
(4) red eyes (conjunctival injection) (5) swollen lymph node in the neck of at least 15
millimeters.
Many children, especially infants, eventually diagnosed with Kawasaki disease do not
exhibit all of the above criteria. In fact, many experts now recommend treating for
Kawasaki disease even if only three days of fever have passed and at least three
diagnostic criteria are present, especially if other tests reveal abnormalities consistent
with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of
coronary artery aneurysms in the proper clinical setting.
[edit] Investigations
• Complete blood count (CBC) may reveal normocytic anemia and eventually
thrombocytosis
• Erythrocyte sedimentation rate (ESR) will be elevated
• C-reactive protein (CRP) will be elevated
• Liver function tests may show evidence of hepatic inflammation and low serum
albumin
[edit] Treatment
Children with Kawasaki disease should be hospitalized and cared for by a physician who
has experience with this disease. When in an academic medical center, care is often
shared between pediatric cardiology and pediatric infectious disease specialists (although
no specific infectious agent has been identified yet).[28] It is imperative that treatment be
started as soon as the diagnosis is made to prevent damage to the coronary arteries.
Corticosteroids have also been used,[42] especially when other treatments fail or symptoms
recur, but in a randomized controlled trial, the addition of corticosteroid to immune
globulin and aspirin did not improve outcome.[43] In cases of kawasaki disease refractory
to IVIG, cyclophosphamide and plasma exchange have been investigated as possible
treatments, with variable outcomes.
There are also treatments for iritis and other eye symptoms. Another treatment may
include the use of Infliximab (Remicade). Infliximab works by binding tumour necrosis
factor alpha.[citation needed]
[edit] Prognosis
With early treatment, rapid recovery from the acute symptoms can be expected and the
risk of coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of
Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of
coronary artery involvement is much greater. Overall, about 2% of patients die from
complications of coronary vasculitis. Patients who have had Kawasaki disease should
have an echocardiogram initially every few weeks, and then every one or two years to
screen for progression of cardiac involvement.
It is also not uncommon that a relapse of symptoms may occur soon after initial treatment
with IVIG. This usually requires re-hospitalization and re-treatment. Treatment with
IVIG can cause allergic and non-allergic acute reactions, aseptic meningitis, fluid
overload and, rarely, other serious reactions. Overall, life-threatening complications
resulting from therapy for Kawasaki disease are exceedingly rare, especially compared
with the risk of non-treatment.
[edit] Epidemiology
By far the highest incidence of Kawasaki disease occurs in Japan (175 per 100,000),
though its incidence in the United States is increasing. Kawasaki disease is
predominantly a disease of young children, with 80% of patients younger than five years
of age. The disease affects boys more than girls. Kawasaki was extremely uncommon in
Caucasians until the last few decades. Approximately 2,000-4,000 cases are identified in
the United States each year
Teaching points
•
Take extra precautions to keep this drug out of the reach of children;
this drug can be very dangerous for children.
•
Use the drug only as suggested; avoid overdose. Avoid the use of other over-the-counter
drugs while taking this drug. Many of these drugs contain aspirin, and serious overdose
can occur.
•
Take the drug with food or after meals if GI upset occurs.
•
Do not cut, crush, or chew sustained-release products.
•
Over-the-counter aspirins are equivalent. Price does not reflect effectiveness.
•
You may experience these side effects: Nausea, GI upset, heartburn (take drug with
food); easy bruising, gum bleeding (related to aspirin's effects on blood clotting).
•
Report ringing in the ears; dizziness, confusion; abdominal pain; rapid or difficult
The main undesirable side effects of aspirin are gastrointestinal ulcers, stomach bleeding,
and tinnitus, especially in higher doses. In children and adolescents, aspirin is no longer
used to control flu-like symptoms or the symptoms of chickenpox or other viral
illnesses, due to the risk of Reye’s syndrome.
Aspirin was the first discovered member of the class of drugs known as non-steroidal
anti-inflammatory drugs (NSAIDs), not all of which are salicylates, although they all
have similar effects and most have inhibition of the enzyme cyclooxygenase as their
mechanism of action. Today, aspirin is one of the most widely used medications in the
world, with an estimated 40,000 metric tons of it being consumed each year. In countries
where Aspirin is a registered trademark owned by Bayer, the generic term is
acetylsalicylic acid (ASA).
PEDIATRIC PATIENTS
•
Analgesic and antipyretic: 65 mg/kg per 24 hr in four to six divided doses, not to exceed
3.6 g/day. Dosage recommendations by age:
Age (yr)
Dosage (mg q 4 hr)2–31624–52436–83249–1040511486³ 12648
•
Juvenile rheumatoid arthritis: 60–110 mg/kg per 24 hr in divided doses at 6- to 8-hr
intervals. Maintain a serum level of 150–300 mcg/mL.
•
Acute rheumatic fever: Initially, 100 mg/kg/day, then decrease to 75 mg/kg/day for 4–6
wk. Therapeutic serum salicylate level is 150–300 mg/dL.
•
Kawasaki disease: 80–180 mg/kg/day; very high doses may be needed during acute
febrile period; after fever resolves, dosage may be adjusted to 10 mg/kg/day.
Ranitdine
Action:
Potent anti-ulcer drug that competitively and reversibly inhibits histamine action at H2-
receptor sites on parietal cells, thus blocking gastric acid secretion. Indirectly reduces
pepsin secretion but appears to have minimal effect on fasting and postprandial serum
gastrin concentrations or secretion of gastric intrinsic factor or mucus
Classifications:
Indication:
Short-term treatment of active duodenal ulcer; maintenance therapy for duodenal ulcer
patient after healing of acute ulcer; treatment of gastroesophageal reflux disease; short-
term treatment of active, benign gastric ulcer; treatment of pathologic GI hypersecretory
conditions (e.g., Zollinger-Ellison syndrome, systemic mastocytosis, and postoperative
hypersecretion); heartburn.
CEFUROXIME AXETIL
Ceftin
Classifications: antiinfective; antibiotic; second-generation cephalosporin
Prototype: Cefonicid sodium
Pregnancy Category: B
NURSING IMPLICATIONS