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Kawasaki Disease

Mucocutaneous Lymph Node Syndrome

Kawasaki disease (Kawasaki syndrome, mucocutaneous lymph node syndrome) is a systemic vasculitis of unknown etiology that primarily affects infants and children. Symptoms include a fever lasting more than 5 days, macular-papular erythematous rash, adenopathy, bilateral conjunctival injection and swelling of the hands and feet.

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Presentation

Most cases of Kawasaki begins with the onset of prolonged fever as the children are brought for medical care after unrelenting fever. For cases to be definitively diagnosed, the fever is expected to have at least lasted for 5 days before medical reporting.

In affected children, there is a higher case of irritability than should be expected of fever of the magnitude often presnted. The fever may continue in the midst of antibiotic therapy for other conditions suspected earlier.

Many parents often suggest an abrupt onset of the fever symptoms. Some non-specific symptoms follow the onset of fever [7]. In order of decreasing frequency, the symptoms that have been noted in the past include the following:

Cervical Lymphadenopathy
  • Jung Ok Kim, Yeo Hyang Kim and Myung Chul Hyun, Comparison between Kawasaki disease with lymph-node-first presentation and Kawasaki disease without cervical lymphadenopathy, Korean Journal of Pediatrics, 59, 2, (54), (2016).[doi.org]
  • Incomplete Kawasaki disease may therefore be characterized by a less frequent association of rash, cervical lymphadenopathy and coronary involvement.[ncbi.nlm.nih.gov]
  • Initial presentation with only cervical lymphadenopathy and fever is one of the pitfalls in the diagnosis of Kawasaki disease (KD). As the number of such patients is small, their clinical features have remained uncertain.[ncbi.nlm.nih.gov]
  • Its typical symptoms include fever, rash, oropharyngeal mucosal erythema, bilateral non-exudative conjunctivitis, cervical lymphadenopathy, extremity changes, and membranous desquamation of the fingers and toes.[ncbi.nlm.nih.gov]
  • Physical examinations revealed fever (38.5 C), fingertips desquamation of the skin, and left cervical lymphadenopathies.[ncbi.nlm.nih.gov]
Fever
  • Patients treated on day 5 or less of fever had a shorter total fever duration (5.2 /- 1.9 days vs 8.0 /- 1.8 days, P .0001), longer fever after IVIG treatment (1.5 /- 1.9 days vs 0.8 /- 1.3 days, P .008), and less coronary artery ectasia at 1 year after[ncbi.nlm.nih.gov]
  • Most cases of Kawasaki begins with the onset of prolonged fever as the children are brought for medical care after unrelenting fever.[symptoma.com]
  • On admission, she still had fever and was irritable. She was diagnosed with sepsis and given another broad-spectrum antibiotic for 2 days. However, her fever still persisted.[ncbi.nlm.nih.gov]
  • Fever was defined as an oral or rectal temperature of 38 C or greater, and antipyretic medication such as acetaminophen was used to control fever. After the fever subsided, low-dose aspirin (3–5 mg/kg per day) was prescribed.[doi.org]
Anemia
  • The hyperinflammatory condition associated with Kawasaki disease may have contributed to the severity of anemia.[ncbi.nlm.nih.gov]
  • Moreover, overt bleeding and anemia are associated with the use of aspirin, and anemia is common in patients with KD.[ncbi.nlm.nih.gov]
  • The typical laboratory features of Kawasaki disease, such as elevated erythrocyte sedimentation rate, leukocytosis, anemia, positive C-reactive protein and thrombocytosis were also seen in the incomplete cases.[ncbi.nlm.nih.gov]
  • The earlier the diagnosis and therapeutic intervention with IV IgG administration are, the lower will be the occurrence of complications; the presence of thrombocytosis, anemia and elevated and extended inflammatory activity are risk factors for complication[ncbi.nlm.nih.gov]
Congestive Heart Failure
  • Congestive heart failure rapidly developed and double valve replacement was performed. Pathologic study of the excised valve tissue disclosed active valvulitis.[ncbi.nlm.nih.gov]
  • Kawasaki disease occasionally causes inflammation of the heart muscle (myocarditis) or weakening of the heart muscle (cardiomyopathy) or decreases the heart's ability to pump blood (congestive heart failure).[cnn.com]
  • The two groups had a similar incidence of adverse effects (including new or worsening congestive heart failure in nine children), which occurred in 2.7 percent of the children overall. All the adverse effects were transient.[ncbi.nlm.nih.gov]
  • heart failure, is commonly manifested by tachycardia out of proportion to the degree of fever, a gallop rhythm or arrhythmias.[web.archive.org]
  • Nine children (three in the four-infusion group and six in the single-infusion group) had new or worsening congestive heart failure. Only one of these children, in the four-infusion group, required treatment with pressor agents.[doi.org]
Edema of the Hands and Feet
  • […] of the hands and feet, and swollen lymph nodes in the neck Kawasaki disease affects children of all ethnic backgrounds throughout the world, although susceptibility is shaped by genetic influences.[merriam-webster.com]
  • Kawasaki disease (KD) is an acute illness of early childhood characterized by prolonged fever, diffuse mucosal inflammation, indurative edema of the hands and feet, a polymorphous skin rash, and nonsuppurative lymphadenopathy. 1 KD is also called mucocutaneous[doi.org]
  • […] of the hands or feet, or generalized or periungual desquamation; 4) rash; and 5) cervical lymphadenopathy (with 1 node 1.5 cm). 5 It is recognized, however, that not all children with KD, diagnosed on the basis of development of virtually pathognomonic[doi.org]
  • […] of the hands and feet • Pallor of the fingernails and/or toenails • Cervical lymphadenopathy (typically unilateral) • Conjunctival infection without exudate (bilateral) • Changes in the oral mucous membranes, such as erythema, dryness or cracking of[physio-pedia.com]
Edema of the Hand
  • […] of the hands and feet, and swollen lymph nodes in the neck Kawasaki disease affects children of all ethnic backgrounds throughout the world, although susceptibility is shaped by genetic influences.[merriam-webster.com]
  • Kawasaki disease (KD) is an acute illness of early childhood characterized by prolonged fever, diffuse mucosal inflammation, indurative edema of the hands and feet, a polymorphous skin rash, and nonsuppurative lymphadenopathy. 1 KD is also called mucocutaneous[doi.org]
  • […] of the hands or feet, or generalized or periungual desquamation; 4) rash; and 5) cervical lymphadenopathy (with 1 node 1.5 cm). 5 It is recognized, however, that not all children with KD, diagnosed on the basis of development of virtually pathognomonic[doi.org]
  • […] of the hands and feet • Pallor of the fingernails and/or toenails • Cervical lymphadenopathy (typically unilateral) • Conjunctival infection without exudate (bilateral) • Changes in the oral mucous membranes, such as erythema, dryness or cracking of[physio-pedia.com]
Abdominal Pain
  • In older children with fever, rash, and acute abdominal pain or hematemesis, KD should be considered in the differential diagnosis.[ncbi.nlm.nih.gov]
  • Progressive bowel oedema was detected in the patient presenting with severe abdominal pain and distension.[ncbi.nlm.nih.gov]
  • We found that there are 9 associated symptoms, namely cough, rhinorrhea, sputum, abdominal pain, vomiting, diarrhea, arthralgia, headache and seizure, which occur in patients with Kawasaki disease.[ncbi.nlm.nih.gov]
  • Although gastrointestinal involvement does not belong to the classic diagnostic criteria; diarrhea, abdominal pain, hepatic dysfunction, hydrops of gallbladder, and acute febrile cholestatic jaundice are reported in patients with Kawasaki disease.[ncbi.nlm.nih.gov]
  • In this report, we describe a case of KD with colonal wall edema, occurred in 5-yr-old boy who complained of severe abdominal pain and vomiting.[ncbi.nlm.nih.gov]
Abdominal Cramps
  • Sometimes, children have abdominal cramps. After a few days, they also get a rash that can appear anywhere. The rash does not have a specific pattern and may only last a short while.[medbroadcast.com]
Strawberry Tongue
  • Principal features of this syndrome are (1) fever lasting more than seven days; (2) conjunctival injection; (3) changes in the mouth consisting of erythema of the oropharynx, "strawberry tongue", and erythema of the lips; (4) indurative edema of hands[ncbi.nlm.nih.gov]
  • Principal features of this syndrome are (1) fever lasting more than seven days; (2) conjunctival injection; (3) changes in the mouth consisting of erythema of the oropharynx, "strawberry tongue," and erythema of the lips; (4) indurative edema of hands[doi.org]
  • By this time she also displayed three of five characteristic features of KD in form of fever, strawberry tongue and cervical adenopathy. Investigations showed high ESR, high CRP, thrombocythemia and dilated coronary vessels on echocardiography.[ncbi.nlm.nih.gov]
  • Other symptoms include: Rash, often in the torso and genital areas Eye inflammation or conjunctivitis without discharge Swelling and redness of the hands and feet followed by peeling of the skin on the fingers and toes Red, chapped lips "Strawberry tongue[ucsfchildrenshospital.org]
  • Within KD, oral mucositis - represented by diffuse mucous membrane erythema, lip and tongue reddening and lingual papillae hypertrophy with subsequent development of strawberry tongue - can occur both in the acute stage of the disease (0-9 days), and[ncbi.nlm.nih.gov]
Cracked Lips
  • Symptoms include high fever swollen lymph nodes in the neck a rash on the mid-section and genital area red, dry, cracked lips and a red, swollen tongue red, swollen palms of the hands and soles of the feet redness of the eyes kawasaki disease can't be[icd9data.com]
  • Until the appearance of red cracked lips, a diagnosis of IKD was considered. A rise in periungual desquamation of the hands confirmed the diagnosis. Intravenous immunoglobulins were administered and aspirin and dipyridamole were used orally.[ncbi.nlm.nih.gov]
  • The cracking lips are a classic sign of the disease. He also has a slight rash over his face, arms, and legs. Red Cracked Lips from Kawasaki Disease. Kawasaki causes very red lips that tend to crack and peel.[newhealthadvisor.com]
  • Symptoms include High fever that lasts longer than 5 days Swollen lymph nodes in the neck A rash on the mid-section and genital area Red, dry, cracked lips and a red, swollen tongue Red, swollen palms of the hands and soles of the feet Redness of the[medlineplus.gov]
Tachycardia
  • We report the case of a 41-year-old man with symptomatic and haemodynamically unstable ventricular tachycardia in whom substrate ablation was performed for the ventricular tachycardia before insertion of an implantable cardioverter-defibrillator.[ncbi.nlm.nih.gov]
  • A 1-year-old girl presented in the hospital with ventricular premature beats and ventricular tachycardia after 2 days of fever. On the fifth day of fever, the diagnosis of Kawasaki disease was achieved. Immunoglobulin was administered.[ncbi.nlm.nih.gov]
  • Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during acute stage, Kawasaki disease shock syndrome has been recently described.[ncbi.nlm.nih.gov]
  • Paroxysmal supraventricular tachycardia(PSVT) is a severe and rare cardiovascular complication of Kawasaki disease. A case of Kawasaki disease presenting with unusual findings, including subdural effusion and PSVT is reported.[ncbi.nlm.nih.gov]
  • Examination revealed fever (39.6C), tachycardia, tachypnea, extreme irritability, and a generalized maculopapular rash, but was otherwise normal. His complete blood count, CRP and ESR were normal.[ncbi.nlm.nih.gov]
Vascular Disease
  • The TM C1418T (rs1042579) polymorphism is associated with a high risk of cardiac-cerebral vascular diseases.[ncbi.nlm.nih.gov]
  • Electronic searches of the Cochrane Peripheral Vascular Disease Group Specialised Register, CENTRAL, MEDLINE, EMBASE, and CINAHL were performed (last searched April 2003).[ncbi.nlm.nih.gov]
  • diseases Autoimmune diseases and disorders Connective tissue diseases with dermal involvement Diseases and disorders named after people Paediatric pathology Non-topical/index: Uses of Wikidata Infobox[commons.wikimedia.org]
  • Kei Takahashi, Toshiaki Oharaseki, Yuki Yokouchi, Nobuyuki Hiruta and Shiro Naoe , Kawasaki Disease as a Systemic Vasculitis in Childhood , Annals of Vascular Diseases , 3 , 3 , (173) , (2010) .[doi.org]
  • Diseases Cardiovascular Diseases Lymphatic Diseases Skin Diseases, Vascular Skin Diseases Etanercept Anti-Inflammatory Agents, Non-Steroidal Analgesics, Non-Narcotic Analgesics Sensory System Agents Peripheral Nervous System Agents Physiological Effects[clinicaltrials.gov]
Hypertension
  • IVMP was well tolerated; transient hypertension developed in one child, but it did not require treatment.[ncbi.nlm.nih.gov]
  • Aortic stiffness is an independent predictor of all-cause and cardiovascular mortality in hypertensive patients. Hypertension. 2001 ; 37 : 1236–1241.[doi.org]
  • Popat and Elif Seda Selamet Tierney, Arterial Applanation Tonometry: Feasibility and Reproducibility in Children and Adolescents, American Journal of Hypertension, 27, 9, (1218), (2014).[doi.org]
  • […] tuberculosis, such as family member with TB or taking INH Untreated Lyme disease Severe comorbidities (diabetes mellitus requiring insulin, CHF of any severity, MI, CVA or TIA within 3 months of screening visit, unstable angina pectoris, uncontrolled hypertension[clinicaltrials.gov]
  • These patients should be followed up carefully and educated to avoid other atherosclerogenic risk factors, such as smoking, obesity, hypertension, and hypercholesterolemia.[doi.org]
Red Eye
  • Thus the problem of a red eye and febrile responses may suggest the above-mentioned serious diagnosis, which is at present, however, well-curable and recognisable with stress put on the time factor.[ncbi.nlm.nih.gov]
  • By the following Saturday morning, the little boy was weak with red eyes, a high fever and sore muscles. The mom became worried, having never seen her son this sick before.[dailymail.co.uk]
  • You can see red eyes, which is known as conjunctivitis. A more prominent redness of the mouth, this is oral erythema. You may see redness of the palms and redness of the soles and you may also see a red body rash.[khanacademy.org]
  • Features of Kawasaki disease can include: Fever lasting for at least 5 days Irritability Red eyes without discharge Redness or cracking of the lips, tongue, or throat Swelling and/or redness, and/or peeling of the skin, usually beginning around the nails[my.clevelandclinic.org]
Exanthema
  • Among them, 25 (10%) patients demonstrated incomplete Kawasaki disease and 17 of these 25 (68%) lacked two of the six principal symptoms of Kawasaki disease, with the most frequently missing symptoms being cervical lymphadenopathy and polymorphous exanthema[ncbi.nlm.nih.gov]
  • Kawasaki disease is characterized by fever, bilateral non-exudative conjunctivitis, redness and swelling of the tongue, lips and oral mucosa, abnormalities in the extremities, cervical lymph node, and polymorphic exanthema.[ncbi.nlm.nih.gov]
  • We report the case of a 26-year-old paediatric resident with fever, exanthema, ocular changes, arthralgia, and desquamation of palms and soles.[ncbi.nlm.nih.gov]
  • The patients met the diagnostic criteria for KD based on the findings of persistent fever, polymorphous exanthema, unilateral cervical lymphadenopathy, non-purulent palpebral conjunctivitis and membranous desquamation.[ncbi.nlm.nih.gov]
  • The diagnosis of KD is based on the presence of clinical features of persistent fever ( 5 days) together with polymorphous exanthema, cervical lymphadenopathy, non-purulent conjunctival injection, changes of the lips, oral cavity, and extremities.[ncbi.nlm.nih.gov]
Rash of the Hands and Feet
  • Further problems are rare Systemic disease primarily of infants and young children, characterized by skin rash, swelling of hands and feet, enlarged cervical lymph nodes, "strawberry tongue", dry and cracked lips, high fevers, and coronary artery disease[icd9data.com]
  • Making the Diagnosis To be diagnosed with Kawasaki disease, a child must have a fever lasting at least 5 days, along with 4 of the following symptoms: rash red, swollen hands or feet changes in the lips (dry, cracked) or mouth ( strawberry tongue ) reddened[medbroadcast.com]
  • Definition (CSP) systemic disease primarily of infants and young children, characterized by skin rash, swelling of hands and feet, enlarged cervical lymph nodes, "strawberry tongue", dry and cracked lips, high fevers, and coronary artery disease.[fpnotebook.com]
Palpable Purpura
  • Palpable purpura is manifested in 46% of the patients at the time the disease is presented and about 90% of the patients have positive ANCA in serum 3,4,6,11.[scielo.br]
Arthritis
  • Key words Kawasaki Disease adult coronary artery aneurysms vasculitis adenopathy arthritis mucocutaneous lymph node syndrome This is a preview of subscription content, log in to check access.[doi.org]
  • ( Septic arthritis, Reactive arthritis, Rheumatoid arthritis, Psoriatic arthritis, Felty's syndrome, Juvenile idiopathic arthritis, Still's disease ) - crystal ( Gout, Chondrocalcinosis ) - Osteoarthritis ( Heberden's node, Bouchard's nodes ) acquired[wikidoc.org]
  • Associated features were lymphadenopathy, pyuria, aseptic meningitis, diarrhea, arthritis, and arthralgia. Although usually a self-limited illness, one patient died with massive coronary artery thrombosis on the 19th day of illness.[ncbi.nlm.nih.gov]
  • We report a case of acute Kawasaki Disease in a 24-year-old man who presented with rash, fever, and arthritis. He was successfully treated with high-dose aspirin and intravenous immunoglobulin (IVIG).[ncbi.nlm.nih.gov]
Arthralgia
  • However, his fever and arthralgia persisted. He was administered single 5 mg/kg doses of IFX. He became afebrile the next day and his arthralgia improved.[ncbi.nlm.nih.gov]
  • Associated features were lymphadenopathy, pyuria, aseptic meningitis, diarrhea, arthritis, and arthralgia. Although usually a self-limited illness, one patient died with massive coronary artery thrombosis on the 19th day of illness.[ncbi.nlm.nih.gov]
  • We report the case of a 26-year-old paediatric resident with fever, exanthema, ocular changes, arthralgia, and desquamation of palms and soles.[ncbi.nlm.nih.gov]
  • Adults more frequently present with cervical adenopathy (93% of adults vs. 15% of children), hepatitis (65% vs. 10%), and arthralgia (61% vs. 24-38%).[ncbi.nlm.nih.gov]
Irritability
  • The first signs of Kawasaki disease include fever and irritability. In most patients, the fever comes on rapidly and is higher than 101.3 F.[ucsfchildrenshospital.org]
  • A previously healthy full term 15 day old Caucasian male with an unremarkable antenatal and perinatal history, presented on Day 2 of illness with fever, rash, irritability, and poor feeding.[ncbi.nlm.nih.gov]
  • The symptoms of Kawasaki disease include... fever rash swollen hands and feet irritation and redness of the whites of the eyes swollen lymph glands in the neck irritation and inflammation of the mouth, lips and throat Doctors don't know what causes Kawasaki[web.archive.org]
  • Pyrexia and irritability continued despite appropriate antibiotic and antiviral therapy suggesting a vasculitic process. The mucocutaneous signs normally associated with Kawasaki disease were subtle and fleeting in this infant.[ncbi.nlm.nih.gov]
  • On admission, she still had fever and was irritable. She was diagnosed with sepsis and given another broad-spectrum antibiotic for 2 days. However, her fever still persisted.[ncbi.nlm.nih.gov]
Lethargy
  • Although most patients had irritability or lethargy, none of them had significant neurological symptoms or signs during the acute phase, except one who showed neck stiffness.[ncbi.nlm.nih.gov]
  • Central nervous system findings, especially irritability, lethargy, and aseptic meningitis, occur in 1-30 % of KD patients (1). Cranial nerve palsies are seen rarely, and abducens nerve palsy has been reported in only three children.[ncbi.nlm.nih.gov]
  • There can be fever, lethargy, breathing difficulty, vomiting and diarrhea. Patients also usually have a red skin rash, swollen lymph nodes and inflammation of the inside surfaces of the mouth and the conjunctiva of the eyes.[cnn.com]
  • Symptoms and Signs The illness tends to progress in stages, beginning with fever lasting at least 5 days, usually remittent and 39 C, associated with irritability, occasional lethargy, or intermittent colicky abdominal pain.[web.archive.org]
  • Symptoms and Signs The illness tends to progress in stages, beginning with fever lasting at least 5 days, usually unremittent and 39 C (about 102.2 F), and is associated with irritability, occasional lethargy, or intermittent colicky abdominal pain.[merck.com]
Seizure
  • This indicated acute encephalopathy with biphasic seizures and late reduced diffusion (AESD); severe neurological sequelae remained. This is the first report of AESD as a complication of KD.[ncbi.nlm.nih.gov]
  • We found that there are 9 associated symptoms, namely cough, rhinorrhea, sputum, abdominal pain, vomiting, diarrhea, arthralgia, headache and seizure, which occur in patients with Kawasaki disease.[ncbi.nlm.nih.gov]
  • The clinical manifestations included disturbance of consciousness and seizures; disturbance of consciousness developed in all patients in the early acute stage. The duration was between 2 and 11 days; seizures developed as status convulsions in two.[ncbi.nlm.nih.gov]
  • A 6-month-old boy was admitted to hospital following a prolonged partial seizure. He showed signs of worsening encephalopathy and was treated for presumed infective meningoencephalitis. Imaging revealed bilateral subdural collections.[ncbi.nlm.nih.gov]
  • A 2-month-20-day-old girl presented to our hospital with a chief complaint of intermittent fever for 1 day and 1 episode of seizure, with poor feeding and swelling of feet. Until the appearance of red cracked lips, a diagnosis of IKD was considered.[ncbi.nlm.nih.gov]
Neurologic Manifestation
  • PubMed Abstract Steinlin M, Blaser S, Gilday D: Neurological manifestations of paediatric systemic lupus erythematous. Pediatric Neurology 1995, 13 : 191-7.[web.archive.org]
  • View Article PubMed Google Scholar Steinlin M, Blaser S, Gilday D: Neurological manifestations of paediatric systemic lupus erythematous. Pediatric Neurology. 1995, 13: 191-7. 10.1016/0887-8994(95)00110-2.[doi.org]
  • Tervaert JW, Kallenberg C (1993) Neurologic manifestations of systemic vasculitides. Rheum Dis Clin North Am 19:913–940 PubMed Google Scholar 2. Jennette JC, Falk RJ (1997) Small-vessel vasculitis.[doi.org]

Workup

There are no specific laboratory tests for diagnosing the Kawasaki disease. However, diagnosis is dependent on the occurrence of certain abnormalities commonly seen at various stages of the disease. At first, acute-phase reactants (ie, C-reactive protein levels (CRP) erythrocyte sedimentation rate (ESR), and alpha1-antitrypsin levels) are often elevated. They often return to baseline between 6 and 10 weeks after the onset of the illness [8].

In the acute stage of the disease, mild-to-moderate normochromic anaemia is seen following complete blood counts (CBCs).

Pericardial Effusion
  • The presence of early desquamating perineal erythema led to the consideration of KD diagnosis, confirmed by the echocardiographic assessment of right and left coronary artery dilatations with pericardial effusion on the fifth day of hospital stay.[ncbi.nlm.nih.gov]
  • The initial presence of any echocardiographic abnormality (coronary artery dilatation, CAA, pericardial effusion, perivascular brightness of the coronary arteries, left-ventricular dysfunction and mitral insufficiency) was strongly associated with resistance[ncbi.nlm.nih.gov]
  • Something more severe that might be seen is known as pericardial effusion. Pericardial effusion is a collection of blood outside of the heart. This is caused by blood vessel rupture.[khanacademy.org]
  • effusion History and etymology It is named after the Japanese pediatrician Tomisaku Kawasaki (1925-fl. 2017) 9 who saw his first case in 1961 and initially described it in a case series of 50 children in 1967 7,9.[radiopaedia.org]
  • Common cardiovascular manifestations of the illness include myocarditis, pericardial effusion, and coronary artery aneurysm formation.[ncbi.nlm.nih.gov]
Pyuria
  • Associated features were lymphadenopathy, pyuria, aseptic meningitis, diarrhea, arthritis, and arthralgia. Although usually a self-limited illness, one patient died with massive coronary artery thrombosis on the 19th day of illness.[ncbi.nlm.nih.gov]
  • In addition to the cardiac problem, hypoalbuminaemia, electrolyte imbalance, sterile pyuria, hepatosplenomegaly, and hydrops of the gallbladder were observed in the case.[ncbi.nlm.nih.gov]
  • In the early stage, Kawasaki disease is often manifested by uncommon symptoms, such as pyuria, meningitis, shock, and retropharyngeal or parapharyngeal abscess, which may delay diagnosis and treatment.[ncbi.nlm.nih.gov]
  • Atypical manifestations include myocarditis pericarditis, valvular regurgitation, hepatitis, diarrhea, abdominal pains, hydrops of gallbladder, arthralgia, arthritis, myalgia, aseptic meningitis, sensorineural hearing loss, urethritis and sterile pyuria[orpha.net]
Echocardiogram Abnormal
  • abnormalities Anticoagulation management To determine whether other studies to assess cardiac function (including coronary artery angiography) are required Long term follow up and monitoring of patient with confirmed or suspected KD Other consulting[emedicine.medscape.com]
Thrombocytosis
  • The typical laboratory features of Kawasaki disease, such as elevated erythrocyte sedimentation rate, leukocytosis, anemia, positive C-reactive protein and thrombocytosis were also seen in the incomplete cases.[ncbi.nlm.nih.gov]
  • In contrast, adults are less frequently affected by meningitis (10% vs. 34%), thrombocytosis (55% vs. 100%), and coronary artery aneurysms (5% vs. 18-25%).[ncbi.nlm.nih.gov]
  • Blood analysis showed alterations of high ESR (88% of cases), C-reactive protein (62%), leukocytosis (82%) and thrombocytosis (96%).[ncbi.nlm.nih.gov]
  • On week 2 after onset of symptoms, the patient developed desquamation of the extremities and thrombocytosis. Echocardiography was normal during the acute illness and remained unchanged at 2-year follow-up.[ncbi.nlm.nih.gov]
  • Persistent fever in spite of antimicrobials, thrombocytosis, and elevated erythrocyte sedimentation rate and C-reactive protein levels pointed toward a diagnosis of KD in our patients.[ncbi.nlm.nih.gov]
Elevated Sedimentation Rate
  • An elevated sedimentation rate and elevations in the levels of other acute-phase reactants are almost universally present in the first week of illness and may persist for 4 to 6 weeks.[cmr.asm.org]
Hepatic Necrosis
  • necrosis, splenic infarct, intestinal pseudo-obstruction, colitis, and colon oedema are also reported.[ncbi.nlm.nih.gov]

Treatment

Children with Kawasaki disease are cared for as inpatients on paediatric/paediatric cardiology units and then put on bed rest. This is as a result of risk of myocardial events [9].

The major foundation of the managing the condition is the use of intravenous immunoglobulin (IVIg) and aspirin to reduce fever, myocardial inflammation and also prevent or reduce cardiac sequelae which is often the major cause of morbidity and mortality associated with this illness.

Prognosis

If treatment is prompt, prognosis is good. There is a dearth of data but in the United States, death is recorded in less than 1% of children infected [6]. In children younger than younger than a year, the death rate is approximately 4%. For those aged 1 year and older, the death rate is approximately less that 1%. On average, the mortality rate in Japan is between 0.1-0.3%. The highest mortality cases are seen 15-45 days after fever kicks in. Till today, there are no cases of adult death as a result of Kawasaki disease.

Etiology

The etiology of the Kawasaki disease is still unclear. However, immunologic and epidemiologic evidence suggest a causative agent that is infectious [3]. Genetic predisposition and autoimmune reactions are possible etiological factors that have been suggested as well.

Kawasaki disease is believed to have an infectious etiology because there is record of consistence occurrence of epidemics especially in spring and in late winter. The epidemics occur at 3 year intervals and there is a wide geographic spread of the epidemics when they occur. Other causes of suspicion of infectious etiology include adenopathy, eye signs, characteristic fever and the self-limited nature of the disease.

It is also believed that maternal antibodies may provide passive immunity as the condition is unusual among infants younger than 4 months of age. With all the infectious etiology suspicions, epidemiologic data suggests that person-to-person transmission of the disease is very much unlikely.

Epidemiology

The highest annual incidence of the Kawasaki disease is seen in Japan with 120-180 cases reported for children aged 5 years and under. The incidence of this condition is on the rise each year in the country [4].

In Europe, surveys show that there is a peak annual incidence of 90 cases for every 100,000 children of Asian descent aged 5 years and below.

Statistical analysis of hospital records in England show an incidence of 8 cases amongst 100,000 children aged 5 and below.

As pointed out above, incidence appears to be variable and local outbreaks occur in winter and spring, while following a three-year cycle. This suggests an infective aetiology.

In the USA, the incidence is 9.1 cases per 100,000 children of white descent and 32.5 cases per 100,000 children of Asian and Pacific Island descent.

Sex distribution
Age distribution

Pathophysiology

Even with the visible mucocutaneous clinical findings that the disease is defined with, the disease is still regarded best as generalised vasculitis involving small arteries and medium sized arteries in some cases. The vascular inflammation seen with the disease is most common in the coronary vessels but the vasculitis is also present in capillaries, small arterioles and the larger arteries.

In the early stages of the Kawasaki disease, the endothelial cells and the vascular media become edematous with the internal elastic lamina remaining intact. 7-9 days after fever kicks in, there is an influx of neutrophils and this is almost immediately followed by a proliferation of immunoglobulin A–producing plasma cells and CD8+ (cytotoxic) lymphocytes [5].

A concomitant progressive increase in the serum platelet count brings about the greatest vascular damage. When the illness progresses to this stage, the risk of death is very significant.

Prevention

Kawasaki disease can't be prevented.

Summary

Kawasaki disease is a self-limiting systemic vasculitis often regarded as idiopathic. It is seen mostly in children between 6 months and 5 years of age [1]. The condition is seen mostly among the general Asian population with China and Japan posting the greater numbers. However, Kawasaki disease has been reported around the world.

A Japanese paediatrician named Tomisaku Kawasaki was the first to describe the case in 1967. The disease was not described until the 1950s so it is thought to be a relatively new disease.

Originally, the condition was regarded as a benign illness that troubles patients suffering from it a great deal but it was discovered later that its complication of the coronary artery aneurysm formation was a major cause of death and significant morbidity [2]. Kawasaki is now the most common cause of heart disease in children, taking over from rheumatic fever. Before the delineation of the disease, cases of it were regarded as periarteritis/polyarteritis nodosa. To reduce its attendant complications, early diagnosis is recommended.

Patient Information

Kawasaki disease is a condition that affects the walls of the medium sized arteries in the body. It also involves the artery that is responsible for supplying blood to the heart muscle. The disease can also affect the skin and the membranes found inside the mouth, throat and nose. 

This condition is rare and is seen in children the most especailly those in Japan or have Asian ancestry. 

The main signs of Kawasaki disease are high fever and peeling of the skin and this can be very frightening for the patient and everyone around them. 

Fortunately, Kawasaki can be treated and most of the time children affected with the condition recover from it without any problems in the future. 

There is no way to prevent the disease but the chances of an individual developing it is very low as long as they don't have any Asian roots. 

References

Article

  1. Kushner HI, Bastian JF, Turner CL, et al; The two emergencies of Kawasaki syndrome and the implications for the developing world. Pediatr Infect Dis J. 2008 May;27(5):377-83.
  2. Satou GM, Giamelli J, Gewitz MH; Kawasaki disease: diagnosis, management, and long-term implications. Cardiol Rev. 2007 Jul-Aug;15(4):163-9.
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Last updated: 2019-06-28 12:07