A Case of Kawasaki Disease Presenting with Aseptic Meningitis Aseptik Menenjit Başlangıçlı Kawasaki Hastalığı Olgusu
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34 Case Report / Olgu Sunumu A Case of Kawasaki Disease Presenting with Aseptic Meningitis Aseptik Menenjit Başlangıçlı Kawasaki Hastalığı Olgusu Emin Özkaya, Yeùim Coùkun, Betül Sezgin, Özgül Yiüit, Nedim SamancÕ VakÕf Gureba Hastanesi, Çocuk SaülÕüÕ ve HastalÕklarÕ Kliniüi, ûstanbul, Turkey Abstract Özet Early recognition and prompt treatment of Kawasaki Kawasaki hastalÕüÕnÕn erken tanÕsÕ ve hÕzlÕ tedavisi, disease(KD) are essential to ensure a succesful out- koroner arter tutuluùu üzerinde olumlu sonuçlarÕn come of the coronary artery involvement. However, elde edilmesi için esastÕr. HastalÕüÕn özgül bir tanÕsÕ some patients lack sufficient clinical signs to fulfill the yoktur ve tanÕ karakteristik klinik bulgular ile konur. diagnostic criteria, and this may lead to problems in BazÕ hastalarda klinik tanÕ için yeterli sayÕda kriter diagnosing children presenting with atypical symp- olmadÕüÕndan atipik bulgularla baùlangÕç gösteren toms. Central nervous system involvement, including olgularda hastalÕüÕn teùhisi güçleùebilmektedir. Ender aseptic meningitis, can be a presenting feature of KD bulgular ile baùvuran hastalarda tanÕ güçlükleri olabil- itself. In this article, we describe a 9-year-old boy mektedir. Merkezi sinir sistemi tutulumunu gösteren who presented with an unusual onset of disease, aseptik menenjit tablosu, hastalÕüÕn atipik bir baùlan- characterized by fever, erythematous maculopapular gÕcÕ olabilmektedir. Bu makalede, ateù, eritematöz cilt rash, vomiting and abdominal pain. He was diag- döküntüleri, kusma ve karÕn aürÕsÕ ùikayetlerini taki- nosed as aseptic meningitis with an unusual onset of ben aseptik menenjit tanÕsÕ alÕp sonrasÕnda klinik ve Kawasaki Disease (KD) clinical pattern together with ekokardiyografik olarak Kawasaki HastalÕüÕ teùhisi the echocardiographic coronary arteries anomalies. alan olan 9 yaùÕnda bir erkek olgu sunulmuùtur. Since the cardiovascular sequelaes of the disease HastalÕüa baülÕ kardiyolojik sekeller hastalÕüÕn erken could be prevented by early diagnosis and effective tanÕ ve tedavisi ile önlendiüinden, ateù, döküntü, drug therapy, KD should be considered in the differ- kusma gibi menenjit bulgularÕnÕn ön planda olduüu ential diagnosis in children with fever, erythematous olgularda ayÕrÕcÕ tanÕda Kawasaki HastalÕüÕ da akÕlda maculopapular rash and vomiting that may be asso- bulundurulmalÕdÕr. ciated with clinical features of aseptic meningitis. (Çocuk Enf Derg 2010; 4: 34-7) (J Pediatr Inf 2010; 4: 34-7) Anahtar Kelimeler: Kawasaki hastalÕüÕ, ateù, aseptik Key Words: Kawasaki disease; fever; aseptic menin- menenjit gitis Geliù Tarihi: 12.06.2009 Kawasaki disease is an acute systemic vas- specific laboratory test. In addition to fever, Kabul Tarihi: 13.07.2009 culitis which effects the small and moderate- there are bilateral non-purulent conjunctival Correspondence Address: sized arteries (1). This disease is also known as injection, cervical lympadenopathy (LAP), Yazışma Adresi: mucocutaneous lymph node syndrome and it edema in hands and feet, erythema and desq- Emin Özkaya, MD, was first reported in Japan in 1967. The etiology uamation (4). Central nervous system involve- VakÕf Gureba Hastanesi, is still unknown (2). Kawasaki disease can be Çocuk SaülÕüÕ ve ment, including aseptic meningitis, can be a HastalÕklarÕ Kliniüi, seen in every ethnic group, also in Japan, and it ûstanbul, Turkey is seen mostly in infants, between 6-12 months presenting feature of KD. Approximately one- Tel.: +90 532 442 42 22 and more commonly in males (3). third to one-half of patients with KD who under- Fax: +90 212 621 75 80 go lumbar puncture will have a cerebrospinal E-mail: The typical property of Kawasaki disease is [email protected] fever, lasting for at least 5 days. There is no fluid (CSF) pleocytosis (5). Çocuk Enf Derg 2010; 4: 34-7 Özkaya et al. J Pediatr Inf 2010; 4: 34-7 A Case of Kawasaki Disease 35 With early diagnosis and treatment, especially the Samples were taken for culture and the patient was development of cardiovascular sequelae can be pre- started with ceftriaxone 100 mg/kg/day. With the antibi- vented. Therefore, the aim of this article is to focus on the otic treatment the fever was not taken under control and importance of early diagnosis and treatment of atypical on the second day of the treatment, the patient had bilat- onset of the Kawasaki disease. eral conjunctivitis. We consulted the patient with the ophthalmology department and the case was diagnosed Case with non-purulent conjunctivitis. Thus, at the ninth day of fever, the patients had erythematose rashes, oral symp- A 9-year-old male patient, admitted to our emergency toms, unilateral cervical LAP (2 cm) in addition to fever, room with fever. The case was considered as an upper and thereafter he had bilateral conjunctivitis, mild respiratory tract infection; thus, amoxicillin-clavulanate hepatomegaly with ultrasonography, and minimal dilata- threapy was initiated. Two days after the antibiotic thera- tion in the coronary arteries with echocardiography py, the patient visited the emergency room again with (ECHO). With these signs and symptoms, the patient was fever at 38.6ºC. Thus, therapy with another class of anti- diagnosed with Kawasaki disease. The antibiotic therapy biotic (clarithromycin) was initiated. Although still febrile was stopped and the patient was started with aspirine 80 and there was no meningismus, he was discharged with- mg/kg/day and intravenous immunoglobulin (IVIG) 2 g/ out a diagnosis. The next day, the patient visited the kg. His ESR was 105 mm at 1-hour and platelet count emergency room again with skin rash and continuining of was 788,000/mm3. fever. After consultation with the dermatology clinic, the During the follow-up, he experienced bradycardia (3 patient was diagnosed with multiformed erythema. Then, times) which was recovered without any treatment. Our he was hospitalized in the dermatology clinic for further patient’s fever was under control and one week after the examination. The patients’ fever was still high as above treatment he had desquamations in his hands and feet. 38ºC after 6 days of antibiotic therapy. On the second After 14 days, the dose of aspirine was reduced to 5 mg/ day after his hospitalization, he suffered from vomiting, kg/day. ESR was 35 mm at 1-hour, platelet count was headache, diarrhea and gastric pain. Since meningeal 273,000/mm3, and ECHO was normal. The aspirine treat- irritation signs were present in the patient, he was trans- ment was ended after 8 weeks, because of normal ferred to the pediatrics clinic with the prediagnosis of echocardiographic finding without any cardiac sequles, meningitis. and normal sedimentation rate. The patient had no prob- On physical examination, he was conscious, active lems during the follow-up as assessed in both our clinic and cooperated. Axillar temperature was 38.3ºC, and and the pediatric cardiology clinic. arterial blood pressure was 80/60 mmHg. His oropharynx and tonsills were hyperemic and hypertrophic, lips were Discussion red, there was a red strawberry appearance in his tongue, and a mobile, 2 cm cervical lymphadenopathy (LAP). For diagnosis of classical KD four of five main criteria Examination of the abdomen showed tenderness and should be appear (6). Incomple or atypical forms of KD defence with no rebound. He had hepatomegaly (2 cm), are related with higher risk of coronary abnormalities nuchal rigidity, Kernig and Brudzinski signs were present. because diagnosis presents more difficulties and therapy Skin examination revealed erythematous maculopapular starts after 10 days of illness (1, 7). In the present case. rash particularly on the legs. the full diagnostic criteria for KD were present, and the The laboratory findings were as follows; leukocyte diagnosis of atypical onset of KD was based on the pres- count: 8600/mm3; hemoglobin: 11 g/dL; hematocrit: ence of prolonged fever without a focus infection, skin 24%; platelet count: 305,000/mm3; C-reactive pro- rash, vomiting and meningeal irritation signs in the suba- tein:14.81 mg/dL; erythrocyte sedimentation rate (ESR): cute phase of the illnesss. There was no other underlying 64 mm at 1-hour; aspartate aminotransferase: 66 U/L; cause fort his childs presentation of aseptic meningitis alanine aminotransferase: 49 U/dL; urea: 20 mg/dL; cre- because all investigations were negative. Because of atinine: 0,66 mg/dL; antistreptolysin-O titer: 1001 IU/mL; this, in our case, coronary involvement may be associ- anti-nuclear antibody, anti-DNA and rheumatoid factor ated with delaying the accurate diagnosis (7). were negative. Prothrombin time, activated partial throm- There is no specific laboratory tests for the diagnosis boplastin time, international normalised ratios were nor- of this disease. Thus, the diagnosis primarily depends on mal, and TORCH indicators were negative. Total urinan- the clinical findings (8, 9). The presence of prolonged alysis was normal. Cerebrospinal fluid (CSF) glucose and fever, especially in the absence of bacterial infection, and protein levels were within normal limits, Pandy test was the presence of some clinical and laboratory findings negative. In CSF leukocyte count was 40/mm3, and might suggest incomple KD (7). Fever and erythematosis erythorocyte count was 160/mm3. rash are frequently confused with staphylococcal or Özkaya et al. Çocuk Enf Derg 2010; 4: 34-7 36 A Case of Kawasaki Disease J Pediatr Inf 2010; 4: 34-7 Tab le 1. Kawasaki disease, diagnostic criterias (8, 9) Additional to the fever, lasting for at least