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

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

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

PURPOSE: The purpose of this study was to find out cognitive function of the patients with subjective memory complaint. MATERIAL AND METHODS: From March 1st 2005 to May 31st 2009, 155 normal individuals without any medical illness who visited Yeungnam University Hospital to undergo medical checkup with neurocognitive test was enrolled, and checked by using Cognitive Assessment & Reference Diagnostic System. RESULTS: 107 of the patients had normal cognitive function, 21 patients (about 15%) were diagnosed with dementia, and 10 patients (about 7%) were diagnosed with considerable psychiatric illness, such as depression, anxiety disorder, adjustment disorder. CONCLUSION: Because the patients with subjective memory complaint can be diagnosed as any psychiatric illness as well as dementia, sensitive screening test and early psychiatric approach is needed.
Adjustment Disorders ; Anxiety Disorders ; Dementia ; Depression ; Humans ; Mass Screening ; Memory

BACKGROUND: It has been well documented that transient occlusion of the coronary artery causes myocardial ischemia and finally cell death when ischemia is sustained for more than 20 minutes. Extensive studies have revealed that ischemic myocardium cannot recover without reperfusion by adequate restoration of blood flow, however, reperfusion can cause long-lasting cardiac dysfunction and aggravation of structural damage. The author therefore attempted to examine the effect of postischemic reperfusion on myocardial ultrastructure and to determine the rationales for recanalization therapy to salvage ischemic myocardium. MATERIALS AND METHODS: Young Holstein-Friesian cows (130~140 Kg body weight; n=40) of both sexes, maintained with nutritionally balanced diet and under constant conditions, were used. The left anterior descending coronary artery (LAD) was occluded by ligation with 4-0 silk snare for 20 minutes and recanalized by release of the ligation under continuous intravenous drip anesthesia with sodium pentobarbital (0.15 mg/Kg/min). Drill biopsies of the risk area (antero-lateral wall) were performed at just on reperfusion (5 minutes), 1-, 2-, 3-, 6-, 12-hours after recanalization, and at 1-hour assist (only with mechanical respiration and fluid replacement) after 12-hour recanalization. The materials were subdivided into subepicardial and subendocardial tissues. Tissue samples were examined with a transmission electron microscope (Philips EM 300) at the accelerating voltage of 60 KeV. RESULTS: After a 20-minute ligation of the LAD, myocytes showed slight to moderate degree of ultrastructural changes including subsarcolemmal bleb formation, loss of nuclear matrix, clumping of chromatin and margination, mitochondrial destruction, and contracture of sarcomeres. However, microvascular structures were relatively well preserved. After 1-hour reperfusion, nuclear and mitochondrial matrices reappeared and intravascular plugging by polymorphonuclear leukocytes or platelets was observed. However, nucleoli and intramitochondrial granules reappeared within 3 hours of reperfusion and a large number of myocytes were recovered progressively within 6 hours of reperfusion. Recovery was apparent in the subepicardial myocytes and there were no distinct changes in the ultrastructure except narrowed lumen of the microvessels in the later period of reperfusion. CONCLUSIONS: It is likely that the ischemic myocardium could not be salvaged without adequate restoration of coronary flow and that the microvasculature is more resistant to reversible period of ischemia than subendocardium and subepicardium. Therefore, thrombolysis and/or angioplasty may be a rational method of therapy for coronarogenic myocardial ischemia. However, it may take a relatively longer period of time to recover from ischemic insult and reperfusion injury should be considered.
Anesthesia ; Angioplasty ; Biopsy ; Blister ; Body Weight ; Cell Death ; Chromatin ; Contracture ; Coronary Vessels* ; Diet ; Heart* ; Infusions, Intravenous ; Ischemia ; Ligation* ; Microvessels ; Muscle Cells ; Myocardial Ischemia ; Myocardium ; Neutrophils ; Nuclear Matrix ; Pentobarbital ; Reperfusion Injury ; Reperfusion* ; Respiration ; Sarcomeres ; Silk ; SNARE Proteins ; Sodium

No abstract available.
Cardioplegic Solutions* ; Ischemia*

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