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Her baseline laboratory work up was sent. OBJECTIVES: Kawasaki syndrome (KS) is an acute systemic vasculitis of unknown origin predominantly affecting young children. Some patients who do not fulfill the criteria outlined in Table 1 have been diagnosed as having “incomplete” or “atypical” Kawasaki disease, a diagnosis that often is based on echocardiographic findings of coronary artery abnormalities. However, it is also well recognized that some Patients do not fulfill the classic diagnostic criteria for the diagnosis of kawasaki disease. in incomplete/atypical Kawasaki disease, children present with persistent fever and some of typical clinical features of Kawasaki disease, but not enough to meet criteria for classic Kawasaki disease 1; infants ≤ 6 months may present with prolonged fever (≥ 7 days) with or without other typical features of Kawasaki disease 1 This patient was refractory to two doses of intravenous immune globulin and therefore was started on methylprednisolone, to which she responded dramatically. C-reactive protein and Erythrocyte Sedimentation Rate were raised (24.4 mg/dl and 100 mm/hr respectively). Kawasaki disease is a condition that mainly affects children under the age of 5. The characteristic symptoms are a high temperature that lasts for 5 days or more, with: Kawasaki disease is an acute, systemic vasculitis that predominantly affects patients younger than five years. All her recent follow ups have been unremarkable in terms of any fever, joint pain, swelling or limitation of activity. A Single Intravenous Infusion of Gamma globulin as Compared with Four Infusions in the Treatment of Acute Kawasaki Syndrome. Therefore, physicians are always in a dilemma over whether to first make an accurate diagnosis or begin early treatment to prevent the development of CAA. This case report describes an atypical or incomplete presentation of Kawasaki Disease. Kawasaki disease is an acute febrile condition seen in children. The ePub format is best viewed in the iBooks reader. Shakeel Shaikh, Sidra Ishaque, and Taimur Saleem. Early treatment with intravenous immunoglobulin (IVIg) has been shown to reduce morbidity and mortality. Toole KP, Frank C. Atypical or Incomplete Kawasaki Disease in a Young Child: A Case Report. Numerous efforts to produce a diagnostic algorithm have been made, but without success. Discrimination between incomplete and atypical Kawasaki syndrome versus other febrile diseases in childhood: results from an international registry-based study. The main dilemma is that neither KD nor SJIA have absolutely specific diagnostic laboratory tests. Kawasaki disease is an uncommon illness in children that causes fever, swollen lymph nodes, sore throat, rash, redness or swelling of the hands or feet, and conjunctivitis. IVIG therapy is not only a costly intervention but it also exposes the patients to unnecessary risks related to receiving a blood product [18], the diagnosis of incomplete KD requires sufficient clinical evidence. Echocardiographic images showing right coronary artery dilatation. High dose intravenous Gamma-globulin for kawasaki disease. Until a gold standard for diagnosing KD is available, these therapeutic decisions will continue to be made on an individual basis. She showed significant clinical improvement after institution of steroid therapy with resolution of fever spikes and normalization of inflammatory markers (ESR = 55, CRP = 3.7 and platelet = 660 × 109/L). 1Department of Pediatrics and Child Health, The Aga Khan University(Stadium Road), Karachi, 74800, Pakistan, 2Medical College, The Aga Khan University(Stadium Road), Karachi, 74800, Pakistan. J Pediatr Health Care. The Adobe Flash plugin is needed to view this content. There could be two considerations in the light of the stated scenario. She has been improving clinically over the last few months. Given a second dose of IVIG while there is ongoing inflammation ( usually taken as ongoing or. 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