An adolescent with fever, jaundice, and abdominal pain an unusual presentation of Kawasaki disease

Clinical observation

An Adolescent With Fever,Jaundice,and Abdominal Pain:An Unusual Presentation of Kawasaki Disease

Kelly Nicole Fradin,M.D.a ,and Hai Jung Helen Rhim,M.D.,M.P.H.b,*

a Department of Pediatrics,Children’s Hospital at Monte?ore,Bronx,New York

b

Department of Pediatrics,Children’s Hospital at Monte?ore,Albert Einstein College of Medicine,Bronx,New York

Article history:Received October 12,2011;Accepted June 11,2012

Keywords:Kawasaki disease;Adolescence;Jaundice;Coronary artery aneurysm

Kawasaki disease (KD)is a small to medium vessel vasculitis that can present insidiously with fever and a coterie of other nonspeci?c symptoms.Treatment with intravenous immuno-globulin (IVIG)early in the course of illness has been associated with a reduced incidence of coronary artery aneurysms,a poten-tially life-threatening sequela [1].The present article reports a case of KD in an adolescent and discusses aspects of KD that are unique to adolescents.Case Report

A 16-year-old boy presented with a 104?F fever for 6days without pattern.The fevers were associated with body aches,and he took ibuprofen,which provided some relief.Four days

before presentation and 2days after the fever began,he started having diffuse crampy abdominal pain associated with nausea,but no emesis.

On the day of admission,he noted periorbital swelling with clear eye discharge,and presented to his pediatrician,who noted scleral icterus and sent him to our institution’s emergency de-partment for further evaluation.On review of symptoms,he endorsed fatigue,lightheadedness,a retro-orbital headache,in-termittent dark urine,and evanescent rash for several days.He denied cough,rhinorrhea,throat pain,dysuria,joint pain,or swelling.

Although no periorbital edema was observed,his examina-tion was signi?cant for scleral icterus,dry lips,and mild diffuse abdominal tenderness without rebound or guarding.The rash

he experienced at home was not present on admission.His labora-tory studies were signi?cant for an elevated white blood count of 14,800/?L,with 86%granulocytes.He had a normal platelet count and was not anemic.Erythrocyte sedimentation rate was elevated to 25mm/hr.Several liver function test results were

*Address correspondence to:Hai Jung Helen Rhim,MD,MPH,Department of Pediatrics,Children’s Hospital at Monte?ore,Albert Einstein College of Medicine,111East 210th Street,Rosenthal-4,Bronx,NY 10467.

E-mail address:hrhim@monte?https://www.360docs.net/doc/747944616.html, (H.J.H.Rhim).

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elevated,including alkaline phosphatase,?-glutamyl trans-ferase,serum glutamic oxaloacetic transaminase,serum glu-tamic pyruvic transaminase,indirect bilirubin,and direct biliru-bin.His sodium level was 134mEq/L;bicarbonate level was 21mEq/L;and all other electrolytes,albumin,and total protein levels were normal.Autoimmune liver disease,Wilson disease,and hemolytic diseases were ruled out.Test results for human immunode?ciency virus,cytomegalovirus,Epstein-Barr virus,and hepatitis A,B,and C were negative.His respiratory viral panel was positive for rhinovirus.Abdominal ultrasonography ?ndings were normal.Given concern for possible cholangitis,he was admitted and given broad-spectrum antibiotics.

On the eighth day of illness,he developed a large posterior cervical lymph node and cracked lips.The following day,he devel-oped conjunctival injection,a desquamating rash in his groin area,and swelling in his hands and feet.Echocardiography was per-formed,which showed left main coronary artery ectasia with a z score of 2.7.This indicates that the lumen of his left main coronary artery was 2.7standard deviations above the mean or approxi-mately the 99th percentile when compared with normal values from the population without KD adjusted by body surface area.The 2004American Heart Association guidelines describe z scores of 2.5or higher as uncommon in the general population (.6%)and unlikely to be associated with febrile illnesses besides KD [2].

A diagnosis of KD was made,and the patient was treated with IVIG and acetylsalicylic acid on the ninth day of illness.The antibi-otics were discontinued.After 26hours,he defervesced and his symptoms of fatigue and malaise improved.He remained afebrile,and on the 12th day of illness,he was discharged home.After discharge,he continued receiving acetylsalicylic acid therapy,and repeat echocardiography showed a coronary artery z score of 3.0.Review of the Literature

KD is a small to medium vessel vasculitis that predominantly affects infants and young children,but can affect older children,adolescents,and adults.Older age-groups may be at risk for delayed or missed diagnosis owing to a decreased awareness of KD in these age-groups.Pannaraj showed that 52%of general pediatricians and 27%of subspecialists would not consider KD in the diagnosis of children older than age 8years [3].Further complicating this picture,a Canadian review found that older children were more likely to present with KD meeting fewer than four clinical features,also called atypical or incomplete disease [4].A series of 28cases of children older than 8years showed that only 61%received treatment before 10days of fever [5].As IVIG treatment within 10days decreases the risk for coronary artery aneurysm development,delays in diagnosis and treatment can have serious consequences.Lin showed that in a Canadian pop-ulation of children with KD,10–14-year-old children had an increased risk of coronary artery aneurysms (17%)compared with children 1–4years old (8%)and 5–9years old (6%)[6].

Although on presentation our patient did not meet criteria for KD,during the course of his hospital stay,he met all ?ve criteria for KD;in addition to fever,he had conjunctivitis,desquamating rash,mucosal changes,swelling of the hands and feet,and lymphade-nopathy (Table 1).With any patient presenting with a fever of more than 5days’duration,the diagnosis of KD should be considered.Additionally,a positive rhinovirus panel did not exclude the possi-bility of KD nor did it explain all of his symptoms.Even if the initial echocardiogram ?ndings had been normal,the patient met the criteria for KD.

KD occurs in a broad range of age-groups.A retrospective analysis of U.S.inpatient hospitalizations from a database of billing codes registered in 1997–2007showed an incidence of KD-associated hospitalization in .6per 100,00010–17year olds compared with 20per 100,0001–4year olds [7].This report illustrates that,although rare,KD does affect adolescents.

Even adults can be diagnosed with KD.One case series from France describes 10cases of suspected KD in adults aged 18–58years [8];four cases of complete KD and four cases of incomplete KD were identi?ed at clinical presentation,and two cases were identi?ed retrospectively,when patients presented with myo-cardial infarction secondary to coronary aneurysm and a history of incomplete KD was obtained.Séve et al [9]analyzed 57cases of adult KD and compared the most frequent associated symptoms with those reported in children with KD in the literature.They found that the most prominent symptoms varied;children were more likely to suffer cheilitis,meningitis,and thrombocytosis,whereas adults presented with arthralgia,adenopathy,and liver function abnormalities [9].

The prominent clinical features in our patient were gastroin-testinal,an atypical ?nding in KD.In another case report,an 18-year-old man presented with fever,jaundice,abdominal pain,diarrhea,and vomiting [10].As the authors point out,these digestive manifestations of KD lack speci?city.Although hydrops is present in 13%and abdominal pain in 20%,this case and ours suggest that severe abdominal complaints can be presenting features of KD.In a review of 28late childhood and adolescent cases,digestive symptoms were more common:vomiting was present in 64%and diarrhea in 40%[5].One patient even required laparotomy for severe abdominal pain.

This case report emphasizes the relevance of a high index of suspi-cion in older children and adolescents who may present with atypical ?ndings,and thus are at a higher risk for life-threatening complica-tions.References

[1]Baer AZ,Rubin LG,Shapiro CA,et al.Prevalence of coronary artery lesions on

the initial echocardiogram in Kawasaki syndrome.Arch Pediatr Adolesc Med 2006;160:686–90.

[2]Newburger JW,Takahashi M,Gerber MA,et al.Diagnosis,treatment,and

long-term management of Kawasaki disease:A statement for health pro-fessionals from the committee on rheumatic fever,endocarditis,and Kawa-saki disease,council on cardiovascular disease in the young,American Heart Association.Pediatrics 2004;114:1708–33.

[3]Pannaraj PS,Turner CL,Bastian JF,Burns JC.Failure to diagnose Kawasaki disease at

the extremes of the pediatric age range.Pediatr Infect Dis J 2004;23:789–91.

[4]Han RK,Sinclair B,Newman A,et al.Recognition and management of

Kawasaki disease.CMAJ 2000;162:807–12.

[5]Stockheim JA,Innocentini N,Shulman ST.Kawasaki disease in older chil-dren and adolescents.J Pediatr 2000;137:250–2.

Table 1

Clinical features of Kawasaki disease

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[6]Lin YT,Manlhiot C,Ching JC,et al.Repeated systematic surveillance of

Kawasaki disease in Ontario from1995to2006.Pediatr Int2010;52:699–706.

[7]Holman RC,Belay ED,Christensen KY,et al.Hospitalizations for Kawasaki

syndrome among children in the United States,1997–2007.Pediatr Infect Dis J2010;29:483–8.

[8]Gomard-Mennesson E,Landron C,Dauphin C,et al.Kawasaki disease in

adults:Report of10cases.Medicine(Baltimore)2010;89:149–58.

[9]Séve P,Stankovic K,Smail A,et al.Adult Kawasaki disease:Report of two

cases and a literature review.Semin Arthritis Rheum2005;34:785–92. [10]Granel B,Serratrice J,Ene N,et al.Painful jaundice revealing Kawasaki

disease in a young man.J Gastroenterol Hepatol2004;19:713–15.

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