No abstract available.
Animals ; Mice* ; T-Lymphocytes*

The May-Hegglin anomaly is a rare autosomal dominant trait characterized by platelet abnormalities in the peripheral blood and large (up to 5mu) pale blue staining inclusions in the cytoplasm of neutrophils, eosinophils, basophils, and monocytes. We experienced a case of May-Hegglin anomaly in a 7 year old male and another case out of his family. His initial complaints at the admission were petechiae and intermittent epistaxis. Diagnosis was confirmed by peripheral blood smear and family study. We report the case with brief review of related literature.
Basophils ; Blood Platelets ; Child ; Cytoplasm ; Diagnosis ; Eosinophils ; Epistaxis ; Humans ; Male ; Monocytes ; Neutrophils ; Purpura

No abstract available.
Lissencephaly* ; Walker-Warburg Syndrome

BACKGROUND: It has been demonstrated that brief periods of calcium depletion and repletion (calcium-free preconditioning, CP) have cardioprotective effects as seen in ischemic preconditioning(IP) which enhances the recovery of post-ischemic contractile dysfunction and reduces the incidence of reperfusion-induced arrhythmia or infarct size after a prolonged ischemia. In the present study, we tested this paradoxical phenomenon in isolated rabbit hearts. MATERIAL AND METHOD: Hearts isolated from New Zealand white rabbits(1.5~2.0 Kg body weight) were perfused with Tyrode solution using the Langendorff technique. After stabilizing the baseline hemodynamics, the hearts were subjected to 45 minutes of global ischemia followed by 120 minutes of reperfusion with IP(IP group, n=7) or without IP (ischemic control group, n=7). IP was induced by a single episode of 5 minutes global ischemia and 10 minutes reperfusion. In the CP group(n=7), the hearts were subjected to perfusion with Tyrode solution with calcium depletion for 5 minutes and repletion for 10 minutes, and 45 minutes of ischemia and 120 minutes of reperfusion. Left ventricular function including developed pressure, dP/dt, heart rate, left ventricular end-diastolic pressure and coronary flow was measured. Infarct size was determined by staining with 1% triphenyltetrazolium chloride and planimetry. Data were analyzed by a one-way analysis of variance and Tukey's post-hoc test. RESULT: In comparison with the ischemic control group, IP significantly enhanced the recovery of the left ventricular function including the left ventricular developed pressure, contractility, and coronary flow; in contrast, these functional parameters of the CP group tended to be lower than those of the ischemic control group. However, the infarct size was significantly reduced by IP or CP(p<0.05). CONCLUSION: These results suggest that in isolated Langendorff-perfused rabbit heart model, CP(induced by single episode of 5 minutes calcium depletion and 10 minutes repletion) could not improve.
Arrhythmias, Cardiac ; Calcium ; Heart ; Heart Rate ; Hemodynamics ; Incidence ; Ischemia ; New Zealand ; Perfusion ; Reperfusion ; Ventricular Function, Left

A 47-years-old woman presented with a 2-month history of a dry mouth and dry cough. The patient had been taking medication for Sjogren's syndrome for approximately 7 years. The chest radiography showed multiple cystic lesions and a hazy density in both lower lung fields. The HRCT showed a diffuse ground glass like appearance and multiple variable sized cystic lesions in both lung fields. After medication, the symptoms were aggravated. Bronchoscopy was preformed with a transbronchial lung biopsy. The biopsies showed an infiltration of lymphocytes, neutrophils, monocytes and histiocytes through the interstitial space of the alveola and a widening of the alveolar septa. However, the histological findings of the cysts were not obtained. Sjogren's syndrome is a slowly progressive inflammatory autoimmune disease, which is characterized by lymphocyte mediated destruction of the exocrine glands, with pulmonary involvement in approximately 19-65%, High-resolution CT is a sensitive technique for assessing the pulmonary involvement in patients with Sjogren's syndrome. Although a lung biopsy is not always necessary for establishing a diagnosis of an interstitial lung disease in Sjogren's syndrome. A lung biopsy may reveal a wide spectrum of changes ranging from a mild inflammatory response to end stage fibrosis with honeycombing. Because of the predominantly peribronchiolar inflammatory infiltration and inspissated secretions the cysts were suspected to have been formed by the ballvalve phenomenon. However, no definite evidence was obtained.
Autoimmune Diseases ; Biopsy ; Bronchoscopy ; Cough ; Diagnosis ; Exocrine Glands ; Female ; Fibrosis ; Glass ; Histiocytes ; Humans ; Lung Diseases* ; Lung Diseases, Interstitial* ; Lung* ; Lymphocytes ; Monocytes ; Mouth ; Neutrophils ; Radiography ; Sjogren's Syndrome* ; Thorax

We report a case of uncrossed complete ureteral duplication with the orifice of the upper system ureter opening in the cranial and lateral positionin the bladder. There was reflux only to the upper system of the duplex kidney and the orifice of the lower system ureter in caudal position was associated with a ureterocele, and the lower pole system was large and non-functioning. The patient was treated successfully by lower pole heminephrectomy and ureterectomy.
Humans ; Kidney ; Ureter* ; Ureterocele* ; Urinary Bladder

OBJECTIVE: To investigate the frequency, type and severity of cardiac abnormalities in the patients with ankylosing spondylitis and undifferentiated spondyloarthopathy. METHODS: A history, clinical examination, standard 12 lead electrocardiography, two dimensional, M mode, and Doppler echocardiographies were performed on 19 patients with ankylosing spondylitis, 15 patients with undifferentiated spondyloarthropathy and 21 normal controls. RESULTS: 1) Cardiac abnormalities were detected in 8 patients(42.1%) with ankylosing spondylitis. 2) Cardiac abnormalities were detected in 8 patients(53.3%) with undifferentiated spondyloarthropathy including 2 patients with aortic valve abnormalities(mild aortic insufficiency, aortic valve thickening. 3) Cardiac abnormalities were detected in one (4. 8%) among normal controls (mild tricuspid regurgitation). 4) There were sinus bradycardias on electrocardiography in 2 patients among patients witn anl;ylosing spond!litis and in 1 patient among undifferentiated spondyloarthropathy. But there was no conduction disturbance in both groups. 5) The frequency of cardiac abnormality was higher in patients with ankylosing spondylitis and undifferentiated spondyloarthropathy than in normal controls. 6) The mean age, mean disease duration, presence of uveitis, peripheral arthritis, HLA-B27, enthesopathy, Schober test and chest expansion in the patients with ankylosing spondylitis and undifferentiated spondyloarthropathy with cardiac abnormalities were not different from those in the patients without cardiac abnormalities. CONCLUSION: The frequency of cardiac abnormality was higher in patients with ankylosing spondylitis and undifferentiated spondyloarthropathy than in normal controls. The frequency, type and severity of cardiac involvement in patients with ankylosing spondylitis were not different from those in patients with undifferentiated spondyloarthropathy.
Aortic Valve ; Aortic Valve Insufficiency ; Arthritis ; Bradycardia ; Electrocardiography ; HLA-B27 Antigen ; Humans ; Rheumatic Diseases ; Spondylarthropathies* ; Spondylitis, Ankylosing* ; Thorax ; Uveitis

It is well known that many Korean patients with type 2 diabetes mellitus (T2DM) were non-obese and had decreased insulin secretion in past. However, during the past three decades, lifestyles in Korea have been westernized. As a result, the prevalence of obesity, the main cause of diabetes has increased. Thus, there is still a question as to whether the main pathophysiology of current Korean T2DM is insulin resistance or an insulin secretion defect. Because various anti-diabetes medications having different mechanisms of action are currently used as therapeutics, it is important to understand which of these factors is the main physiology in the development of diabetes in Koreans. In this review, we review changes in obesity prevalence, insulin resistance and insulin secretion defects in Korean T2DM during three decades.
Asian Continental Ancestry Group ; Diabetes Mellitus, Type 2* ; Humans ; Insulin Resistance* ; Insulin* ; Korea ; Life Style ; Obesity ; Physiology ; Prevalence

Clonorchis sinensis habitating in the bile duct of mammals causes clonorchiasis endemic in East Asian countries. Parkin is a RING-between-RING protein and has E3-ubiquitin ligase activity catalyzing ubiquitination and degradation of substrate proteins. A cDNA clone of C. sinensis was predicted to encode a polypeptide homologous to parkin (CsParkin) including 5 domains (Ubl, RING0, RING1, IBR, and RING2). The cysteine and histidine residues binding to Zn2+ were all conserved and participated in formation of tertiary structural RINGs. Conserved residues were also an E2-binding site in RING1 domain and a catalytic cysteine residue in the RING2 domain. Native CsParkin was determined to have an estimated molecular weight of 45.7 kDa from C. sinensis adults by immunoblotting. CsParkin revealed E3-ubiquitin ligase activity and higher expression in metacercariae than in adults. CsParkin was localized in the locomotive and male reproductive organs of C. sinensis adults, and extensively in metacercariae. Parkin has been found to participate in regulating mitochondrial function and energy metabolism in mammalian cells. From these results, it is suggested that CsParkin play roles in energy metabolism of the locomotive organs, and possibly in protein metabolism of the reproductive organs of C. sinensis.
Amino Acid Sequence ; Animals ; Clonorchis sinensis/*enzymology ; Cluster Analysis ; Conserved Sequence ; DNA, Complementary/genetics ; Energy Metabolism ; Gene Expression Profiling ; Mitochondria/metabolism ; Models, Molecular ; Molecular Weight ; Phylogeny ; Protein Conformation ; Sequence Homology, Amino Acid ; Ubiquitin-Protein Ligases/chemistry/*genetics/*metabolism

Clonorchis sinensis habitating in the bile duct of mammals causes clonorchiasis endemic in East Asian countries. Parkin is a RING-between-RING protein and has E3-ubiquitin ligase activity catalyzing ubiquitination and degradation of substrate proteins. A cDNA clone of C. sinensis was predicted to encode a polypeptide homologous to parkin (CsParkin) including 5 domains (Ubl, RING0, RING1, IBR, and RING2). The cysteine and histidine residues binding to Zn2+ were all conserved and participated in formation of tertiary structural RINGs. Conserved residues were also an E2-binding site in RING1 domain and a catalytic cysteine residue in the RING2 domain. Native CsParkin was determined to have an estimated molecular weight of 45.7 kDa from C. sinensis adults by immunoblotting. CsParkin revealed E3-ubiquitin ligase activity and higher expression in metacercariae than in adults. CsParkin was localized in the locomotive and male reproductive organs of C. sinensis adults, and extensively in metacercariae. Parkin has been found to participate in regulating mitochondrial function and energy metabolism in mammalian cells. From these results, it is suggested that CsParkin play roles in energy metabolism of the locomotive organs, and possibly in protein metabolism of the reproductive organs of C. sinensis.
Amino Acid Sequence ; Animals ; Clonorchis sinensis/*enzymology ; Cluster Analysis ; Conserved Sequence ; DNA, Complementary/genetics ; Energy Metabolism ; Gene Expression Profiling ; Mitochondria/metabolism ; Models, Molecular ; Molecular Weight ; Phylogeny ; Protein Conformation ; Sequence Homology, Amino Acid ; Ubiquitin-Protein Ligases/chemistry/*genetics/*metabolism