No abstract available.

We report two cases of porphyric peripheral neuropathy in a 19-year-old male with variegate porphyria and in a 39 year-old male with intermittent acute prophyria. Clinically, there were sensory, motor disturbance and autonomic symptoms including decreased sweating, urinary and sphinctor distrubances. Variegate porphyria showed facial diplegia and positive family history inherited by autosomal dominent trait. Intermittent acute porphyria was combined-with SIADH. Both cases were expired due to respiratory failure. Nerve conduction studies were carried out in two cases and both cases showed slow motor, sensory nerve conductlon velocity ,and significant low CMAPs(Compound Muscle Action Potentials). Sural nerve biopsy was carried out in a variegate prophyria compared with one normal control. Decreased large myelinated fibers was found. In nerve fiber teased study. 8.5% of nerve fibers showed axonal degenration and only 2.3% of the segmental demyelination. There findings are suggesting that the porphyric neuro might be the axonal type.and severe neuropathy in a sign of poor prognosis.
Adult ; Axons ; Biopsy ; Demyelinating Diseases ; Humans ; Inappropriate ADH Syndrome ; Male ; Myelin Sheath ; Nerve Fibers ; Neural Conduction ; Peripheral Nervous System Diseases ; Porphyria, Acute Intermittent ; Porphyria, Variegate ; Prognosis ; Respiratory Insufficiency ; Sural Nerve ; Sweat ; Sweating ; Young Adult

OBJECTIVE: Necrotizing pneumonia (NP) is a severe complication of lobar pneumonia caused by various pathogens. The immunopathogenesis and clinical characteristics of NP in children are not clearly understood. We wanted to evaluate the clinical characteristics and suggest in part the immunopathogenesis of NP. METHODS: We reviewed retrospectively the medical charts and radiographic materials of eight patients with NP, who were diagnosed by chest radiography and chest computed tomography at the Department of Pediatrics, Soonchunhyang University Hospitals at Cheonan and Bucheon from January 2002 to December 2011. RESULTS: They were previously healthy, 2.1 to 4.6 years of ages (mean, 2.8+/-1.0 years) and three boys and five girls. All of them had pleural effusion. Five patients had pneumonic consolidations in right upper lung field. Three patients had pneumatocele. They developed leukocytosis (mean, 19,400+/-6,400/mm3), higher C-reactive protein level (mean, 25.1+/-8.0 mg/dL). The etiologic agents were revealed in two patients; Streptococcus pneumonia (S. pneumonia) was revealed in one patient and S. pneumonia and Mycoplasma pneumonia in the other patient. Three patients were treated with additional intravenous immunoglobulin. Clinical improvement was prolonged: fever lasted 10 to 23 days, and length of hospitalization was 15 to 36 days. NP or pneumatocele were completely resolved on the follow-up radiographic studies in all of the patients. CONCLUSION: Although the previously healthy young children with NP had protracted clinical course, they recovered without any problematic sequelae. Our results suggest that the immunopathogenesis of NP in children may be associated with the exaggerated immune reaction of the host to insults from initial bacterial infections, rather than the pathogen-induced cytopathies.
Bacterial Infections ; C-Reactive Protein ; Child* ; Chungcheongnam-do ; Female ; Fever ; Follow-Up Studies ; Gyeonggi-do ; Hospitalization ; Hospitals, University ; Humans ; Immunoglobulins ; Leukocytosis ; Lung ; Pediatrics ; Pleural Effusion ; Pneumonia* ; Pneumonia, Mycoplasma ; Radiography ; Retrospective Studies ; Streptococcus ; Thorax

Atopic dermatitis (AD) is an inflammatory skin disorder that is both uncomfortable and distressing to patients, and its prevalence has been steadily increasing. It is obvious that the identification of efficient markers of AD in plasma would offer the possibility of effective diagnosis, prevention, and treatment strategies. In this study, a proteomic approach was used to analyze plasma glycoproteins from both children with AD and healthy child donors. Several protein spots showing significant quantitative changes in the AD patients were identified. Through sequential studies, it was confirmed that CD5L and ApoE were significantly up-regulated or down-regulated, respectively, in the plasma from AD patients compared with that from healthy donors. In addition, we suggest that the up-regulated CD5L in AD patients causes eosinophilia by inhibiting apoptosis or promoting the proliferation of eosinophils either in combination with or without IL-5. The glycoproteomic data in this study provides clues to understanding the mechanism of atopic alterations in plasma and suggests AD-related proteins can be used as candidate markers for AD.
Apolipoproteins E/*blood ; Biological Markers/blood ; Cell Line ; Cell Proliferation ; Child ; Dermatitis, Atopic/*metabolism ; Eosinophilia/metabolism ; Eosinophils/physiology ; Female ; Glycoproteins/*blood ; Humans ; Interleukin-5/metabolism ; Male ; Proteomics ; Scavenger Receptors, Class B/*blood

PURPOSE: In Korea, tree pollens are known to be prevalent in spring, grass pollens in summer and weed pollens in autumn. However, few studies have revealed their seasonal specificity for allergic rhinitis symptoms. An ARIA (Allergic Rhinitis and its Impact on Asthma) classification of allergic rhinitis was recently introduced and its clinical validation has not been well proved. The aim of this study was to evaluate the seasonal specificity of seasonal allergens and to validate the ARIA classification with the conventional seasonal and perennial allergic rhinitis (SAR/PAR) classification. METHODS: Two hundred twenty six patients with allergic rhinitis were included in this study. The patients were classified according to the sensitized allergens and the ARIA classifications. A questionnaire survey was performed and the data on the seasonal symptom score, the severity of symptoms and the SNOT (sinonasal outcome test)-20 score was obtained and the data was analyzed and compared between the conventional SAR/PAR classification and the ARIA classification. RESULTS: Seasonal pollens (tree, grass, weed) were not specific to the pollen peak season and the patients' symptoms were severe during spring and autumn regardless of the offending pollens. More than 60% of the patients with SAR showed persistent symptoms and 33% of the patients with perennial allergic rhinitis (PAR) had intermittent symptoms, showing the lack of association between the SAR/PAR/PAR+SAR classification and the ARIA classification. The ARIA classification showed better association not only with the symptomatic score, but also with the SNOT-20 score, which showed better validity than the conventional SAR/PAR classifications. CONCLUSIONS: Seasonal pollens were not specific to their season of prevalence in terms of the severity of symptoms, and the ARIA classification showed better representation of allergic symptoms and quality of life (SNOT-20 score) than did the SAR/PAR classification.
Allergens ; Humans ; Korea ; Poaceae ; Pollen ; Prevalence ; Quality of Life ; Rhinitis ; Rhinitis, Allergic, Perennial ; Rhinitis, Allergic, Seasonal ; Seasons ; Sensitivity and Specificity* ; Surveys and Questionnaires

A 41-year-old man sought evaluation at the emergency department for pain in the anterior chest that had been ongoing for approximately 35 hours. The electrocardiogram showed marked ST segment elevation in the precordial leads. Cardiac biomarker levels were elevated. He subsequently underwent coronary angioplasty and stenting of the left anterior descending artery using two sirolimus-eluting stents. The following day, the patient complained of severe pain in his chest and shoulders. Computed tomography (CT) of the chest showed small gas bubbles around the aortic wall and mild pericardial thickening with subtle air densities, suggesting acute mediastinitis. With an impression of postcardiac injury syndrome and acute mediastinitis, he was treated with intravenous antibiotics and oral ibuprofen. Two days later, the patient had subjective improvement and the friction rub was no longer heard.
Adult ; Angioplasty ; Anti-Bacterial Agents ; Arteries ; Electrocardiography ; Emergencies ; Friction ; Humans ; Ibuprofen ; Mediastinitis ; Myocardial Infarction ; Shoulder ; Stents ; Thorax

OBJECTIVE: This study explored the development of metabolic syndrome, changes in body weight and metabolic syndrome parameters (waist circumference, serum glucose and lipids, blood pressure), and effects of psychotropic agents in psychiatric inpatients being treated with psychotropic agents. METHODS: In all, 146 patients who had been admitted to a psychiatric isolated ward for more than 1 month between August 2012 and May 2014 were included in this study. During hospitalization, levels of triglyceride, high density lipoprotein-cholesterol (HDL), low density lipoprotein-cholesterol, and serum glucose, and blood pressure, height, body weight, and waist circumference were regularly measured. For obtaining data on laboratory tests, physical examination and demographic and clinical characteristics, we reviewed patients' medical records. RESULTS: After using psychotropic agents for 3 months, body mass index increased significantly and HDL levels decreased significantly. Of 119 patients without metabolic syndrome at baseline, 15 (12.61%) patients developed a this syndrome after 3 months. Among psychotropic agents, quetiapine most largely increased the number of patients who meet the criteria for metabolic syndrome (17.9%), and this change was significantly larger than that of aripiprazole (p=0.031). Carbamazepine significantly increased waist circumference. Duloxetine and lamotrigine significantly increased triglyceride levels. Olanzapine, aripiprazole, mirtazapine, duloxetine and valproic acid significantly decreased HDL levels. Futher, olanzapine and valproic acid significantly increased body mass index. Fluoxetine significantly decreased body mass index. CONCLUSION: The results of this study indicate that at least 1 in 10 patients using psychotropic agents develop metabolic syndrome within a relatively short time; this finding emphasizes the importance of early diagnosis and treatment. Because abnormality of lipid parameters was prominent in early phase of treatment, clinicians should monitor these levels carefully. In addition, some psychotropic agents could affect body weight and metabolic syndrome parameters and thus clinicians should be aware of this changes in patients using psychotropic agents. Main limitation of this study is high drop-out rate (74%), and this could make the result underestimate.
Blood Glucose ; Blood Pressure ; Body Height ; Body Mass Index ; Body Weight* ; Carbamazepine ; Early Diagnosis ; Fluoxetine ; Hospitalization ; Humans ; Inpatients* ; Medical Records ; Physical Examination ; Triglycerides ; Valproic Acid ; Waist Circumference ; Aripiprazole ; Duloxetine Hydrochloride ; Quetiapine Fumarate

V. fluvialis is a gram-negative, oxidase-producing, halophilic bacterium. It is normally found in coastal waters and seafoods. There have been a few reports on Vibrio fluvialis gastroenteritis in other countries, whereas there has been no previous report of V. fluvialis infections in Korea. Reports from other countries showed that V. fluvialis was isolated mostly from infants and children. We experienced a rare case of gastroenteritis due to V. fluvialis in a 55-year-old man with liver cirrhosis who ate an ark shell. He was admitted due to mild abdominal pain and severe watery diarrhea followed by rapidly progressive dehydration, electrolyte imbalance, and hepatorenal syndrome for a week. This patient was not improved by intensive care and antibiotic therapy.
Abdominal Pain ; Arcidae ; Child ; Dehydration ; Diarrhea ; Gastroenteritis* ; Hepatorenal Syndrome ; Humans ; Infant ; Critical Care ; Korea ; Liver Cirrhosis ; Middle Aged ; Seafood ; Vibrio*

PURPOSE: It is difficult to differentiate between viral and bacterial pneumonia in children and to decide antibiotic therapy. Study was conducted to investigate the clinical usefulness of antibiotic therapy based on procalcitonin (PCT) in children diagnosed with viral pneumonia. METHODS: This study included 108 patients diagnosed with viral pneumonia. Patient's age, fever duration, hospital stay, and treatment progress were noted, and laboratory study including PCT levels were tested. In addition, Polymerase chain reaction was done to test for viruses. Patients were divided into PCT and non-PCT groups according to PCT level. And their clinical patterns, treatment outcome, antibiotic use, severity of complications were compared. RESULTS: The number of patients with respiratory syncytial virus (RSV) was 35 and 50, rhinovirus was 5 and 10 in PCT and non-PCT groups, respectively. Fever duration was longer by 2.5 days in PCT group than in the non-PCT group (P<0.001), but there was no difference in the duration of hospital stay (P=0.191). White blood cell and absolute neutrophil count levels were higher in the PCT group (P=0.013 and P<0.001, respectively). Use of antibiotic therapy was performed in group was on 22% and 90% of patients in the PCT and non-PCT groups, respectively showing a significant reduction in the frequency of antibiotic therapy in PCT group, without a significant difference in treatment outcome, despite more severe clinical signs (P<0.001). CONCLUSION: Antibiotic therapy based on serum PCT levels in children admitted for pneumonia can reduce the frequency of antibiotic therapy in viral pneumonia, without causing significantly different treatment outcome or complications.
Anti-Bacterial Agents ; Child* ; Fever ; Humans ; Length of Stay ; Leukocytes ; Neutrophils ; Pneumonia* ; Pneumonia, Bacterial ; Pneumonia, Viral ; Polymerase Chain Reaction ; Respiratory Syncytial Viruses ; Rhinovirus ; Treatment Outcome

PURPOSE: There are numerous opinions about the methods and usefulness of minimizing leg length discrepancies after hip arthroplasty. In this study, we tried to evaluate the usefulness of an intraoperative x-ray in addition to preoperative templating for minimizing leg length discrepancy. MATERIALS AND METHODS: We reviewed pre- and post-operative pelvis AP x-rays of 46 patients who underwent hip arthroplasty due to a traumatic femoral neck fracture or intertrochanteric fracture between May 2008 and February 2009. A leg length discrepancy is the difference in vertical distance between a horizontal line drawn along the bottom of the ischial tuberosities and the most inferior points of the lesser trochanter. It was measured in a pelvis AP x-ray. In each case, pre-operative templating was performed and an intraoperative pelvis AP x-ray was taken again to assess the accuracy of preoperative planning. Implant positions were readjusted when necessary. RESULTS: The mean post-operative leg-length discrepancy was 0.3 mm (SD, 3.1 mm). The range was from -5.8 mm to +5.9 mm. CONCLUSION: Combining preoperative templating and intraoperative x-rays is a useful method of minimizing leg length discrepancy during hip arthroplasty.
Arthroplasty ; Femoral Neck Fractures ; Femur ; Hip ; Humans ; Leg ; Pelvis