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.
Humans ; Pepsinogen A*

No abstract available.
Diabetes Insipidus, Nephrogenic* ; Urinary Bladder*

PURPOSE: Emergency colorectal surgery has high rates of complications and mortality because of incomplete bowel preparation and bacterial contamination. The authors aimed to evaluate the surgical outcomes and the risk factors for the mortality and the complication rates of patients who underwent emergency surgery to treat colorectal diseases. METHODS: This is a prospective study from January 2014 to April 2016, and the results are based on a retrospective analysis of the clinical results for patients who underwent emergency colorectal surgery at Chosun University Hospital. RESULTS: A total of 99 patients underwent emergency colorectal surgery during the study period. The most frequent indication of surgery was perforation (75.8%). The causes of disease were colorectal cancer (19.2%), complicated diverticulitis (21.2%), and ischemia (27.2%). There were 27 mortalities (27.3%). The major morbidity was 39.5%. Preoperative hypotension and perioperative blood transfusion were independent risk factors for both morbidity and mortality. CONCLUSION: These results revealed that emergency colorectal surgeries are associated with significant morbidity and mortality. Furthermore, the independent risk factors for both morbidity and mortality in such patiients were preoperative hypotension and perioperative transfusion.
Blood Transfusion ; Colorectal Neoplasms ; Colorectal Surgery* ; Diverticulitis ; Emergencies* ; Humans ; Hypotension ; Ischemia ; Mortality ; Prospective Studies ; Retrospective Studies ; Risk Factors*

PURPOSE: Although there is no favoring etiology and no definitive treatment modality for interstitial cystitis(IC), the search for the cause of IC continues in hope of identifying a target for treatment. Recently nitric oxide(NO) is speculated to be associated with the pathogenesis and the treatment of IC. But, the exact mechanisms are poorly known and the experimental and clinical data about NO in IC are conflicting. We attempted to determine that urine NO level evaluated in patients with interstitial cystitis(IC) and compared with those from control patients with bacterial cystitis. MATERIALS AND METHODS: Fifty six patients were involved in this study, including control 22, patients with IC 8, patients with bacterial cystitis 17, and recovery state from bacterial cystitis 8. Urine samples were collected by clean-catch midstream in men and from urethral catheterization in women. Urinary nitrite which was considered to be an urinary NO in this study was measured by Titerek Multiscan MCC/340 ELISA reader. RESULTS: Urinary NO levels were 6.16+/-1.09 pM/mg.creatinine in control, 5.15+/-0.87 pM/mg.creatinine in IC, 79.58+/-24.69 pM/mg. creatinine in bacterial cystitis, and 32.96+/-8.38 pM/mg.creatinine in recovery state from bacterial cystitis, respectively. Urinary NO levels in bacterial cystitis and recovery state from bacterial cystitis were significantly higher than those in control and IC(p<0.0001). CONCLUSION: Urinary NO in bacterial cystitis was higher than that in control and maintained for some period. Urinary NO in IC was lower than that in bacterial cystitis.
Creatinine ; Cystitis* ; Cystitis, Interstitial ; Enzyme-Linked Immunosorbent Assay ; Female ; Hope ; Humans ; Male ; Nitric Oxide ; Urinary Catheterization ; Urinary Catheters

PURPOSE: Many clinical guidelines recommend apolipoprotein B (apoB) measurement, particularly in subjects with metabolic syndrome or type 2 diabetes. Recently, we developed a new equation to estimate serum apoB (apoBE). We validated the clinical relevance of apoBE and compared the performance of the equation with conventional lipid measurements and direct measurement of apoB. MATERIALS AND METHODS: Study subjects were recruited from patients who visited the Health Screening Center at Kangbuk Samsung Hospital between January and December 2009 for routine medical examinations (n=78125). For analysis of coronary calcium score, we recruited study subjects from the same institution between January 2007 and December 2010 (n=16493). RESULTS: apoBE was significantly correlated with serum high-sensitivity C-reactive level {r=0.18 [95% confidence interval (CI), 0.18–0.19]} in partial correlation analysis adjusted for age, sex, and body mass index. apoBE was associated with a Framingham risk score indicating more than moderate risk (10-year risk ≥10%), the presence of microalbuminuria, and the presence of coronary artery calcium in multivariate logistic regression analysis. These associations were comparable to those of directly-measured serum apoB [odds ratio per 1 SD 3.02 (2.75–3.27) vs. 2.70 (2.42–3.02) for a Framingham risk score indicating more than moderate risk, 1.31 (1.21–1.41) vs. 1.35 (1.25–1.45) for the presence of microalbuminuria, and 1.33 (1.26–1.41) vs. 1.31 (1.23–1.38) for the presence of coronary calcium score respectively]. These findings were also consistently observed in subgroup analysis for subjects with type 2 diabetes. CONCLUSION: The associations between cardiovascular surrogate markers and apoBE were comparable to those of directly-measured apoB.
Apolipoproteins B ; Apolipoproteins* ; Biomarkers* ; Body Mass Index ; Calcium ; Cardiovascular Diseases* ; Coronary Vessels ; Humans ; Logistic Models ; Mass Screening

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

Type 2 diabetes can produce various sexual dysfunctions in both men and women. Prevalence of sexual dysfunction is 25~75% in type 2 diabetes, which is three times that of the general population. As hyperglycemia persists, atherosclerosis progresses, and macrovascular and microvascular complications can occur. Autonomic neuropathy and hypogonadism are principal causes of various sexual dysfunctions such as erectile dysfunction, retrograde ejaculation, and premature ejaculation in males and loss of libido, vaginal dryness, anorgasmia, and dyspareunia in females. Although erectile dysfunction is reversible in early stages, it is more difficult to control as diabetes and associated autonomic dysfunction and microvascular complications progress. Sexual dysfunction can decrease quality of life in type 2 diabetes patients and is a marker of vascular dysfunction. Sexual dysfunction has prognostic value for cardiovascular events in type 2 diabetes. This illustrates the importance of sexual function evaluation in type 2 diabetes patients.

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