We reported a pediatric case of Kawasaki disease complicated with mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) from Beijing Tsinghua Changgung Hospital. The clinical data were retrospectively analyzed and the related literature was reviewed. The clinical features, treatment and prognosis of the disease were summarized to improve recognition of Kawasaki disease complicated with MERS. A 7-year-old boy was diagnosed with Kawasaki disease due to continuous high fever for 6 d, accompanied by strawberry tongue, conjunctival congestion, erythema-like hyperemia rash, and cervical enlarged lymph nodes. And treatment was started with intravenous immunoglobulin (IVIG: 2 g/kg) and oral aspirin [40 mg/(kg·d)]. Twenty-four hours after the treatment of IVIG, the patient' s fever persisted and in addition he developed headache and drowsiness. His cranial magnetic resonance imaging (MRI) demonstrated a localized lesion in the splenium of the corpus callosum with high intensity signal on diffusion-weighted images (DWI) and T2-weighted, and low intensity signal on apparent diffusion coefficient (ADC) and T1-weighted. Based on these findings, he was diagnosed with MERS-complicated Kawasaki disease. Methylprednisolone [2 mg/(kg·d)] treatment was started intravenously, and within several hours he was afebrile and the neurological symptoms disappeared. A follow-up MRI was conducted after 1 week was normal. He was discharged without any neurological sequelae and coronary artery lesions. A total of 12 qualified foreign literature were retrieved, with no Chinese literature searched. Seventeen children were reported, the median age was 6.5 years (range: 1-14 years), among them 11 cases were children over 5 years old, and 4 cases were complicated with coronary artery lesions. All children had neurological symptoms, such as consciousness disorder, visual hallucination or convulsion. MRI conformed to MERS imaging changes. After active treatment, the neurological manifestations and radiological abnormalities completely disappeared, leaving no neurological sequelae. Kawasaki disease complicated with MERS had not been reported in China by now. Literature that identified Kawasaki disease complicated with MERS mostly occurred in children over 5 years old. Cranial MRI examination is helpful for early diagnosis. Timely treatment can reverse MERS in a short time, without neurological sequelae left.
Brain Diseases/diagnosis* ; Child ; Child, Preschool ; Encephalitis/pathology* ; Fever ; Humans ; Hyperplasia/complications* ; Immunoglobulins, Intravenous/therapeutic use* ; Magnetic Resonance Imaging ; Male ; Mucocutaneous Lymph Node Syndrome/diagnosis* ; Retrospective Studies

Kawasaki disease is a common paediatric vasculitide. It is usually diagnosed by its classical constellation of mucocutaneous signs. Recurrent Kawasaki disease is a rare phenomenon that occurs in approximately 3% of all patients diagnosed with Kawasaki disease. Its presentation is usually similar to the first episode of Kawasaki disease, and early diagnosis with prompt treatment is key in preventing associated cardiovascular morbidities. Recurrent Kawasaki disease is not well reported, and atypical presentations have not been previously reported in medical literature. Here, we report the case of a young girl with recurrent Kawasaki disease who presented atypically with acute airway obstruction secondary to retropharyngeal phlegmon.
Acute Disease ; Airway Obstruction ; diagnosis ; etiology ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Lymph Nodes ; diagnostic imaging ; pathology ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; Neck ; Recurrence ; Tomography, X-Ray Computed

Aspirin ; administration & dosage ; therapeutic use ; Brain ; diagnostic imaging ; pathology ; Cerebral Infarction ; diagnosis ; drug therapy ; etiology ; Epilepsy ; diagnosis ; drug therapy ; etiology ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; therapeutic use ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; drug therapy ; Tomography, X-Ray Computed

Child ; Child, Preschool ; Coronary Vessels ; pathology ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; diagnosis ; pathology ; therapy ; Retrospective Studies

Kawasaki disease is an acute, self-limiting febrile mucocutaneous vasculitis of infants and young children. Retropharyngeal lymphadenopathy is a rare presentation of Kawasaki disease. We present a case of Kawasaki disease mimicking a retropharyngeal abscess, with upper airway obstruction resulting in delayed diagnosis.
Child ; Humans ; Male ; Mucocutaneous Lymph Node Syndrome/*diagnosis/*pathology ; Retropharyngeal Abscess/*pathology

Background:Kawasaki is acute fever disease with systematic vein infection which often seen in children under 5 years old. Objectives:Describe the clinical characteristics and laboratory, echocardiography data in children with Kawasaki between early diagnosed group and late one. Subjects and method: A descriptive, prospective study was carried out on 77 Kawasaki disease patients were selected, including 50 patients were diagnosed before day 10 of illness (group 1) and 27 patients were diagnosed on or after day 10 (group 2) in the National Pediatrics hospital from June 2004 to June 2006. Results:The mean was 13.4 months. Age under 12 months was 61 %. Male/female ratio was 1.7: 1. Fever, red lips, red eyes, skin rash, extremities edema and cervical lymphadenopathy occurred in the first week. The inflammatory response was strong (CRP: 83.6 mg/I, erythrocyte sedimentation rate (ESR) after an hour: 79.1 mm. WBC: 27800/ mm3). There were no differences between patients in the group 1 and group 2 in age, gender, time to the first medical visit, C-reactive protein (CRP) concentration, white blood cell count or erythrocyte sedimentation rate. Patients in the group 1 had significantly close clustering of symptoms onset in the first few days of illness, but patients in the group 2 had onset of symptoms scattered over 4 days. A platelet count of over 500.000/mm3 occurred more often in the group 2 (60%) than the group 1 (31.3%). Coronary involvement was observed in 23 patients (29.8%) including 17 patients who had coronary dilation and 6 patients with coronary aneurysm. Conclusion:Coronary artery abnormalities in the group 2 (48.1%) occurred significantly more than the group 1 (20%).
Mucocutaneous Lymph Node Syndrome/ diagnosis ; pathology ; Child

Background: Kawasaki is an acute fever disease with systematic vein infection and often seen in children.Objectives:This study aims to determine features and risk factors of coronary artery lesion (CAL) in Kawasaki disease in children. Subjects and method:A descriptive, prospective study was conducted on 83 patients diagnosed with Kawasaki disease whom treated at National Hospital for Pediatric from January 2005 to March 2007. They were divided into 2 groups: with and without CAL. All data from clinical characteristics, laboratory and echocardiography were analyzed to evaluate the differences between 2 groups. Univariate and multivariate analysis were used. Results: Among 83 patients diagnosed with Kawasaki disease, 27 patients (32.5%) was found with CAL. 24 patients (88.9%) had both right and left coronary artery abnormalities. The CAL in left anterior descending (LAD) and in left circumflex coronary artery (LCX) was 55.6% and 25.9% respectively. Risk level II: 7 patients (25.9%). Risk level III: 14 patients (51.9%) and risk level IV: 6 patients (22.2%). 14 patients (51.9%) with CAL were resolved at 6th month of the illness. Independent risk factors of CAL in acute stage included age under 12 months (OR = 3.97, p<0.05). IVIG treatment was within the first 10 day of the illness (OR=0.25, p<0.05). Non - responsiveness to \u03b3globulin therapy (OR=7.69, p<0.01). CRP before starting initial treatment above 90mg/1 (OR = 12.81, p<0.05). Platelets before starting \u03b3 globulin therapy ~ 557 000/ mm3 with OR=4.73 and p<0.05. Conclusion:Early detection and treatment were necessary in order to decrease CAL in patients with Kawasaki disease.
Mucocutaneous Lymph Node Syndrome/ diagnosis ; pathology ; Coronary Vessels/ pathology ; Child

Angiography ; methods ; Coronary Angiography ; methods ; Coronary Vessels ; pathology ; Humans ; Magnetic Resonance Angiography ; methods ; Mucocutaneous Lymph Node Syndrome ; diagnosis ; diagnostic imaging

Churg-Strauss Syndrome ; pathology ; Diagnosis, Differential ; Giant Cell Arteritis ; pathology ; Granulomatosis with Polyangiitis ; pathology ; Humans ; Mucocutaneous Lymph Node Syndrome ; pathology ; Polyarteritis Nodosa ; pathology ; Purpura, Schoenlein-Henoch ; pathology ; Takayasu Arteritis ; pathology ; Thromboangiitis Obliterans ; pathology ; Vasculitis ; classification ; pathology

OBJECTIVEKawasaki disease (KD) is an acute and self-limited systemic vasculitis syndrome of unknown origin that mainly affects small and medium-sized arteries, particularly the coronary arteries, which is followed by aneurysm formation. Increased levels of matrix metalloproteinase-1 (MMP-1) have been detected in aortic aneurysms in adults, suggesting an important role of MMP-1 in arterial wall destruction and resultant aneurysm formation. The aim of this study was to investigate the potential role of MMP-1 in the pathogenesis of coronary artery lesions in patients with KD.

METHODSForty patients with KD, including 23 patients without coronary artery lesions (CAL) and 17 patients with CAL, as well as age-matched 10 febrile and 10 healthy afebrile controls were studied. The duration of KD was divided into three phases: the acute phase, the subacute phase and the convalescent phase. Enzyme-linked immunosorbent assay was used to detect the protein levels of MMP-1 in the sera. MMP-1 mRNA expression in the circulating leucocytes was studied using reverse transcription-polymerase chain reaction.

RESULTSLevels of MMP-1 protein in serum and MMP-1 mRNA expression in the leucocytes were significantly elevated at the acute phase in the two groups of KD patients (CAL group: 14.91 +/- 3.88 ng/ml and 0.89 +/- 0.15 ng/ml; NO-CAL group: 11.27 +/- 3.28 ng/ml and 0.77 +/- 0.14, respectively), compared with febrile (7.05 +/- 1.98 ng/ml and 0.45 +/- 0.12 ng/ml, respectively) and afebrile (5.13 +/- 1.20 ng/ml and 0.29 +/- 0.12 ng/ml, respectively) controls (P < 0.01). Furthermore, MMP-1 protein and MMP-1 mRNA levels were significantly higher in KD patients with CAL than in KD patients without CAL (P < 0.05). There was a significantly positive correlation between the serum protein level of MMP-1 at the acute phase of KD and the circulating leucocytes counts (r = 0.750, P < 0.01). The MMP-1 serum protein level and mRNA expression in the leucocytes at the acute phase of the two KD groups decreased obviously from the subacute through the convalescent phases (P < 0.05 or P < 0.01).

CONCLUSIONThe expression of MMP-1 at the acute phase of KD was significantly elevated, especially in KD patients with CAL. MMP-1 might be involved in the formation of coronary artery lesions and pathogenesis of KD.

Acute Disease ; Child, Preschool ; Coronary Aneurysm ; enzymology ; etiology ; pathology ; Coronary Vessels ; pathology ; Enzyme-Linked Immunosorbent Assay ; Fever ; etiology ; Humans ; Infant ; Leukocytes ; enzymology ; Male ; Matrix Metalloproteinase 1 ; blood ; genetics ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; RNA, Messenger ; blood ; Reverse Transcriptase Polymerase Chain Reaction ; Severity of Illness Index