No abstract available.
Child ; Health Promotion ; Humans ; Immunization* ; Child Health

Kawasaki disease (KD) is an immune-mediated disease which is a leading cause of acquired cardiovascular disease in developed country. Recently, tumor necrosis factor-alpha (TNF-alpha) blocker, infliximab has been considered a promising option for patients with refractory KD. Although chronic use of a TNF-alpha blocker could increase risk of opportunistic infections, a few studies have documented that use of infliximab was safe without serious adverse effects in patients with KD. We observed serious bacterial infection after infliximab treatment in an infant with refractory KD. Our patient was a 5-month-old male infant diagnosed with KD who did not respond to repeated doses of intravenous immunoglobulin. We effectively treated him with a single infusion of infliximab (5 mg/kg), but gram-negative (Acinetobacter lwoffii) septicemia developed after infliximab infusion. Therefore, we report a case of serious septicemia after treatment with infliximab, and suggest considering the risk of severe infection when deciding whether to prescribe infliximab to an infant with refractory KD.
Bacterial Infections ; Cardiovascular Diseases ; Developed Countries ; Humans ; Immunoglobulins ; Infant* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Opportunistic Infections ; Sepsis* ; Tumor Necrosis Factor-alpha ; Infliximab

BACKGROUND: Chronic cough is a common clinical problem to which various etiologies are attributable; postnasal drip, asthma, gastroesophageal reflux (GER) and chronic bronchitis, However, in some cases, no particular etiology can be confirmed. OBJECTIVE: This study was aimed to evaluate cough sensitivity and the clinical manifestations of patients with idiopathic chronic cough. MATERIALS AND METHODS: We recruited 70 non-smoking patients who had been coughing for more than 3 weeks without any history, symptoms or signs of rhinitis, sinusitis, typical asthma, GER or recent upper respiratory infection episodes. Nineteen healthy controls were also enrolled. Bronchial provocation tests with methacholine, capsaicin and distilled water (DW) were performed. RESULTS: Thirteen patients were positive to rnethacholine test (PC20 < 25mg/ml) and defined as cough variant asthma. The others are classified into idiopathic chronic cough. By capsaicin and DW challenge tests, idiopathic cough patients could be divided into two groups, i.e., increased cough sensitivity (ICS) and normal cough sensitivity (NCS) groups. DW- induced cough counts were negatively correlated with C7 (lowest capsaicin concentration inducing continuous 7 or more coughs) (r=-0.739, p<0.001). No difference was found in clinical features such as age, sex, atopy, or peripheral eosinophil counts between these two groups but cough counts per cough burst were significantly higher in the ICS group. CONCLUSION: The heterogeneity in cough sensitivity of idiopathic chronic cough patients may refiect different pathophysiologic mechanisms. It is unclear whether patients with NCS merely have habitual or psychogenic cough or not. These suggest that different therapentic strategies should be established on idiopathic chronic cough patients according to cough sensitivity.
Asthma ; Bronchial Provocation Tests ; Bronchitis, Chronic ; Capsaicin ; Cough* ; Eosinophils ; Gastroesophageal Reflux ; Humans ; Methacholine Chloride ; Population Characteristics ; Rhinitis ; Sinusitis ; Water

Congenital absence of the gall bladder without extrahepatic biliary atresia is an extremely rare congenital malformation with a reported incidence ranging between 0.01 and 0.02%. It is thought to be occurred from failure of the gallbladder bud to develop in utero and frequent association with other malformations. Agenesis can be randomly discovered at autopsy or operations for symptoms suggestive of biliary tract disease. The authors report a case of agenesis of the gallbladder without extrahepatic biliary atresia in a neonate which was incidentally found at laparotomy for presumed duodenal obstruction, with a review of the literature.
Autopsy ; Biliary Atresia* ; Biliary Tract Diseases ; Duodenal Obstruction ; Gallbladder* ; Humans ; Incidence ; Infant, Newborn ; Laparotomy ; Urinary Bladder

PURPOSE: The aim of this study was to investigate the pathophysiologic role of serum E-selectin, vascular endothelial growth factor(VEGF)-induced cell adhesion mollecule in Kawasaki disease(KD) and to look for the evidence of direct relationship between the plasma levels of soluble E-selectin and the incidence of coronary artery lesion(CAL). METHODS: Changes in plasma levels of sE-selectin(n=98) over time were measured by enzyme-linked immunosorbent assay(ELISA) in 23 patients with acute KD and 25 age-matched febrile children. RESULTS: Compared with control values, the peak levels of plasma sE-selectin were significantly elevated(mean+/-S.E.:22.89+/-12.53 ng/mL vs 10.65+/-3.42 ng/mL, P=0.01) in KD. 5 patients with CAL, plasma sE-selectin levels before treatment were higher than in 18 patients without CAL(mean+/-S.E.:39.43+/-15.08 ng/mL and 19.00+/-8.32 ng/mL, respectively; P=0.01). Plasma sE-selectin declined rapidly in the majority of KD patients regardless of the presence of CAL. Plasma sE-selectin levels after treatment and convalesent period were similar in KD patients with and without CAL. The plasma levels sE-selectin were correlated with those of white blood cell count(r=0.299, P<0.05), CRP(r=0.430, P<0.05), serum albumin(r=-0.483, P<0.05), serum protein(r=-0.502, P<0.05) and hemoglobin(r=-0.372, P<0.05) not with those of ESR, platelet, or duration of fever. There were significant differences in the initial level of serum sE-selectin between KD with and without CAL(mean+/-S.E.:39.44+/-15.08 ng/mL vs. 19.00+/-17.18 ng/mL) in multivariated linear tests. CONCLUSION: Plasma sE-selectin levels were significantly higher in KD than in other febrile illness. Higher plamsa levels of sE-selectin may have potential as a predictor of CAL in patients with KD.
Blood Platelets ; Cell Adhesion ; Child ; Coronary Vessels ; E-Selectin* ; Fever ; Humans ; Incidence ; Leukocytes ; Mucocutaneous Lymph Node Syndrome* ; Plasma

We report a case of thymic hyperplasia accompanied by pericardial lipomatosis and right facial hemihypertrophy in an 8-year-old boy. On imaging studies, the hyperplastic thymus had prominent curvilinear and nodular fatty areas simulating a fat-containing anterior mediastinal mass, which is an unusual finding in children. To our knowledge, this is the first report on a child with a combination of thymic hyperplasia, pericardial lipomatosis, and right facial hemihypertrophy. The radiologic findings are presented with a brief discussion.
Child ; Diagnosis, Differential ; Facial Asymmetry/complications/*diagnosis ; Heart Diseases/complications/*diagnosis/surgery ; Humans ; Hypertrophy/pathology ; Lipomatosis/complications/*diagnosis/surgery ; Magnetic Resonance Imaging ; Male ; Pericardium/*pathology ; Thymus Hyperplasia/complications/*diagnosis/surgery ; Tomography, X-Ray Computed

Wilson s disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson s disease. However, nephrotoxicity can occur after D-penicillamine treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syndrome in a 3-year-old girl with Wilson s disease 3 weeks after initiation of D-penicillamine.
Brain ; Child, Preschool ; Cornea ; Female ; Glomerulonephritis, Membranous ; Humans ; Liver ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Penicillamine* ; Trientine ; Zinc Sulfate

No abstract available.
Gastrointestinal Tract* ; Radionuclide Imaging*

Bickerstaff's brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. It is similar to Miller Fisher syndrome, a variant of Guillain-Barre syndrome, in that they share features such as ophthalmoplegia and ataxia. The difference is that patients with Bickerstaff's brainstem encephalitis have impaired consciousness, whereas patients with Miller Fisher syndrome have alert consciousness and areflexia. Here, we report the case of a 3-year-old child who was diagnosed with Bickerstaff's brainstem encephalitis presenting typical clinical features and interesting radiological findings. The patient showed ophthalmoplegia, ataxia, and subsequent stuporous mentality. Brain magnetic resonance imaging revealed high signal intensity in the pons and cerebellum around the 4th ventricle on a T2-weighted image. He was successfully treated with intravenous immunoglobulin. Differentiation of Bickerstaff's brainstem encephalitis and Miller Fisher syndrome is often difficult because they possess many overlapping features. Brain magnetic resonance imaging may be helpful in diagnosing Bickerstaff's brainstem encephalitis, especially when lesions are definitely found.
Ataxia ; Brain ; Brain Stem* ; Cerebellum ; Child ; Child, Preschool ; Consciousness ; Encephalitis* ; Guillain-Barre Syndrome ; Humans ; Immunoglobulins ; Magnetic Resonance Imaging ; Miller Fisher Syndrome ; Ophthalmoplegia ; Pons ; Stupor

Seckel syndrome is a rare autosomal recessive disease. The characteristic features of Seckel syndrome include Intrauterine and postnatal growth deficiency, severe microcephaly, and a bird-like head. General appearance of our patient was characterized by Pre- and postnatal growth retardation, microcephaly, large eye, and a beaked nose. The patient had mental retardation with CNS anomalies. The CNS anomalies seen in the patient included cerebral dysgenesis and cerebellar atrophy. So, we report the case of CNS anomalies in Seckel syndrome.
Animals ; Atrophy ; Beak ; Head ; Humans ; Intellectual Disability ; Microcephaly ; Nose