PURPOSE: This study was undertaken to identify the frequencies and the risk factors of microvascular complications in subjects with type 1 diabetes mellitus METHODS: The frequencies and their relation to risk factors of microvascular complications were analyzed in 29 type 1 diabetes mellitus subjects with duration of disease more than 5 years. Microvascular disease was defined as the presence of either retinopathy, microalbuminuria or neuropathy. RESULTS: The overall prevalence rate of microvascular disease was 8/29(27.6%). Retinopathy has developed in 3 patients(10.3%), microalbuminuria in 7 patients(24.0 %) and neuropathy in 5 patients(17.2%). The mean HbA1C was significantly higher in the patients with microvascular complications(11.6+/-.2% in microvascular complication group vs 9.3+/-.6% in control group). CONCLUSION: In childhood onset type 1 diabetes mellitus, poor glycemic control is an important risk factor for microvascular complications.
Diabetes Mellitus, Type 1* ; Humans ; Prevalence ; Risk Factors*

Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.
Child ; Male ; Female ; Humans

Primary bronchial neoplasms in children, especially malignant lesions, are extremely rare. We report a case of a 10-year old boy with intermediate-grade mucoepidermoid carcinoma of the right intermediate bronchus, presenting as collapse and bronchiectasis after pneumonia. He was diagnosed by bronchoscopic biopsy. The lobectomy was performed. The patient is in good condition 1 year after operation. All reported cases of bronchial mucoepidermoid tumors in children had histologically low or intermediate grade(well differentiated) variety with a benign clinical course. The optimal surgical therapy for bronchial mucoepidermoid tumors is identical. There must be total removal of either mass with the sacrifice of as little normal lung as possible.
Biopsy ; Bronchi* ; Bronchiectasis ; Carcinoma, Mucoepidermoid* ; Child ; Humans ; Lung ; Male ; Mucoepidermoid Tumor ; Pneumonia

In order to perform large-scale genetic studies of Kawasaki disease (KD) in Korea, the Korean Kawasaki Disease Genetics Consortium (KKDGC) was formed in 2008 with 10 hospitals. Since the establishment of KKDGC, there has been a collection of clinical data from a total of 1198 patients, and approximately 5 mL of blood samples per patient (for genomic deoxyribonucleic acid and plasma isolation), using a standard clinical data collection form and a nation-wide networking system for blood sample pick-up. In the clinical risk factor analysis using the collected clinical data of 478 KD patients, it was found that incomplete KD type, intravenous immunoglobulin (IVIG) non-responsiveness, and long febrile days are major risk factors for coronary artery lesions development, whereas low serum albumin concentration is an independent risk factor for IVIG non-responsiveness. In addition, we identified a KD susceptibility locus at 1p31, a coronary artery aneurysm locus (KCNN2 gene), and the causal variant in the C-reactive protein (CRP) promoter region, as determining the increased CRP levels in KD patients, by means of genome-wide association studies. Currently, this consortium is continually collecting more clinical data and genomic samples to identify the clinical and genetic risk factors via a single nucleotide polymorphism chip and exome sequencing, as well as collaborating with several international KD genetics teams. The consortium-based approach for genetic studies of KD in Korea will be a very effective way to understand the unknown etiology and causal mechanism of KD, which may be affected by multiple genes and environmental factors.
Aneurysm ; C-Reactive Protein ; Coronary Vessels ; Data Collection ; DNA ; Exome ; Genetics* ; Genome-Wide Association Study ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Korea* ; Mucocutaneous Lymph Node Syndrome* ; Plasma ; Polymorphism, Single Nucleotide ; Promoter Regions, Genetic ; Risk Factors ; Serum Albumin

BACKGROUND/AIMS: The aims of this study were to determine subgoups of functional dyspesia and to evaluate the short-term effect of cisapride in patients with functional dyspepsia in Korea. METHODS: 1025 patients, with a mean age of 42.6 years, with symptoms of functional dyspepsia, were recruited consecutively and upper gastrointestinal symptoms were investigated by interview in 41 hospitals in Korea. In an open, multicenter trial, 1025 patients received Smg of cisapride three times a day (TID) for at least .2 weeks for the treatment of symptoms of functional dyspepsia. When necessary, the dose of cisapride was increased to 10mg TID and the duration of therapy was extended to 4 weeks. RESULTS: The most frequently reported symptoms of functional dyspepsia were epigastric discomfort or fullness (85%), bloating (70%), belching (53%), early satiety (52%) and epigastric pain (46%) retrospectively. Subgroups of functional dyspepsia were as follows; dysmotility-like 73.5%, ulcer-like 39.7%, reflux-like 13.0%, and unspecified dyspepsia 14.0%. However, 33.2% of subjects with functional dyspepsia could be classified into more than one subgroup. Upper gastrointestinal symptoms were decreased to average 50.3% (range; 42.2 to 59.2%) after 2 weeks of cisapride treatment and to 25% (19.2 to 29.9%) after 4 weeks. cisapride therapy resulted in good or excellent improvement in 59.0% of the patients after two weeks, in 75% of patients after 4 weeks. Adverse events were occurred in 52 patients (5.8% of all patients), most commonly, loose stools or diarrhea (3.5%), abdominal pain (1.1%), and dizziness (0.3%). The majority of adverse events was mild and transient in nature and led to premature discontinuation of treatment in 4 patients. CONCLUSIONS: Although the majorities of patients with functional dyspepsia have dysmotility like symptoms in Korea, there is such overlap among the dyspepsia subgroups. Most patients responded well to a short therapeutic trial with cisapride without significant side effects.
Abdominal Pain ; Cisapride* ; Diarrhea ; Dizziness ; Dyspepsia* ; Eructation ; Humans ; Korea* ; Retrospective Studies