No abstract available.
Child* ; Humans ; Peritoneal Dialysis, Continuous Ambulatory* ; Peritonitis*

Congenital hepatic fibrosis is an uncommon disease of children and young adults with two major risks: gastrointestinal hemorrhage caused by portal hypertension, and cholangitis related to bacterial infection of dilated intrahepatic bile ducts. It is characterizeed by stony hard hepatomegaly and portal hypertension with rather well preserved hepatic function and architecture, and frequent association of the renal lesions. We have recently experienced a case of congenital hepatic fibrosis in a 24 year-old Korean male. The chief complaint was hematemesis from esophageal varices. There were marked hepatosplenomegaly, mild pancytopenia and the liver function test was within normal engorgement and dilatation of portal and splenic veins and multiple cysts of both kidneys.
Child ; Adult ; Male ; Female ; Humans ; Cysts

No abstract available.
Arthroplasty, Replacement, Hip*

PURPOSE: To compare chest radiographic findings of pulmonary tuberculosis in non-AIDS immunocom- promised adult patients with those in immunocompetent patients. MATERIAL AND METHOD: Eighty six patients who had pulmonary tuberculosis were included in the study. Of these, 41 were non-AIDS immunocompromised adult patients and 45 were immunocompetent adult patients. Chest radiographs obtained from 86 patients were retrospectively evaluated with regard to the followings ;the anatomic distribution and extent of tuberculous lesions, typical or atypical patterns of radiographic findings. We then compared the results in non-AIDS immunocompromised adult patients with those in immunocompetent adult patients. RESULTS: The characteristic manifestation of pulmonary tuberculosis was a tendency of pulmonary lesions to localize in the apico-posterior segments of the upper lobe and the superior segment of the lower lobe in both groups but more wide distribution such as the anterior segment and the lingular segment of the upper lobe and the basal segments of the lower lobe was frequently identified in non-AIDS immunocompromised adult patients, and also bilateral, multisegmental and multilobular extents were common findings. in immunocompetent adult patients, more common findings were in local exudative and productive lesions and several cavities in preferential sites. Atypical plain radiographic findings were more common in non-AIDS immunocompromised adult patients, and which were multiple cavitary lesions, wide extent of bronchogenic spread and tuberculous pneumonia, and .miliary disseminations and mass like lesions. CONCLUSIONS: Pulmonary tuberculosis in non-AIDS immunocompromised adult patients is characterized by frequent bilateral distribution, wide pulmonary extent, and atypical radiographic findings.
Adult* ; Humans ; Pneumonia ; Radiography, Thoracic ; Retrospective Studies ; Tuberculosis, Pulmonary*

No abstract available.
Child ; Humans ; Kidney Failure, Chronic ; Peritoneal Dialysis, Continuous Ambulatory*

No abstract available.
Child ; Humans ; Kidney Failure, Chronic ; Peritoneal Dialysis, Continuous Ambulatory*

No abstract available.
Animals ; Horns* ; Kidney*

PURPOSE: To compare, with the use of chest radiographic findings, improvement and complications in newborns treated with exogenous surfactant for hyaline membrane disease(HMD), and an untreated control group. MATERIALS AND METHODS: Thirty-six patients with HMD were randomly assigned to a control group (n=18) or surfactant treated group (n=18). As part of an initial evaluation of their pulmonary status, we then performed a retrospective statistical analysis of chest radiographic findings obtained in exogenous surfactant treated and untreated infants within the first 90 minutes of life. Subsequent examinations were performed at less than 24 hours of age. RESULTS: Chest radiograph before treatment showed no significant differences between the two groups, but significant improvement was noted in the surfactant treated group, in contrast to the control group. The most common chest radiographic finding after surfactant administration was uniform (n=15) or disproportionate (n=2) improvement of pulmonary aeration. Patent ductus arteriosus developed in three treated neonates and in four cases in the control group. Air leak occurred in three cases in the treated group and in five cases in the control group. In one treated patient pulmonary hemorrhage developed and intracranial hemorrhage occurred in three treated neonates and in four cases in the control group. Bronchopulmonary dysplasia was developed in 6 cases of treated group and 3 cases of control group. CONCLUSION: A chest radiograph is considered to be helpful in the evaluation of improvement and complications of HMD in infants treated with surfactant.
Bronchopulmonary Dysplasia ; Ductus Arteriosus, Patent ; Hemorrhage ; Humans ; Hyalin* ; Hyaline Membrane Disease* ; Infant ; Infant, Newborn ; Intracranial Hemorrhages ; Membranes ; Radiography, Thoracic ; Retrospective Studies

BACKGROUND/AIMS: This study was performed to determine whether adding coenzyme Q10 (CoQ(10)) to metformin (MET) has a beneficial effect as a treatment for sirolimus (SRL)-induced diabetes mellitus (DM). METHODS: DM was induced in rats by daily treatment with SRL (0.3 mg/kg, subcutaneous) for 28 days, and animals were treated with CoQ(10) (20 mg/kg, oral) and MET (250 mg/kg, oral) alone or in combination for the latter 14 days of SRL treatment. The effects of CoQ(10) and MET on SRL-induced DM were assessed with the intraperitoneal glucose tolerance test (IPGTT) and by determining plasma insulin concentration and the homeostatic model assessment of insulin resistance (HOMA-R) index. We also evaluated the effect of CoQ(10) on pancreatic islet size, apoptosis, oxidative stress, and mitochondria morphology. RESULTS: IPGTT revealed overt DM in SRL-treated rats. The addition of CoQ(10) to MET further improved hyperglycemia, decreased HOMA-R index, and increased plasma insulin concentration compared with the SRL group than MET alone therapy. While SRL treatment induced smaller islets with decreased insulin staining intensity, the combination of CoQ(10) and MET significantly improved insulin staining intensity, which was accompanied by a reduction in oxidative stress and apoptosis. In addition, co-treatment of CoQ(10) and MET significantly increased the levels of antiperoxidative enzymes in the pancreas islet cells compared with MET. At the subcellular level, addition of CoQ(10) to MET improved the average mitochondrial area and insulin granule number. CONCLUSIONS Addition of CoQ(10) to MET has a beneficial effect on SRL-induced DM compared to MET alone.

Objective: We investigated the proportions of and reclassified BRCA1/2 variants of unknown significance (VUS) in Korean patients with epithelial ovarian, tubal, and primary peritoneal cancers. Methods: Data from 805 patients who underwent genetic testing for BRCA1/2 from January 1, 2006 to August 31, 2018 were included. The VUS in BRCA1/2 were reclassified using the 2015 American College of Medical Genetics and Genomics and the Association for Molecular Pathology standards and guidelines. Results: A BRCA1 pathogenic variant was found in 17.0% (137/805) of the patients, and BRCA1 VUS were found in 15.9% (128/805) of the patients. Further, 8.7% (69/805) of the patients possessed a BRCA2 pathogenic variant and 18.4% (148/805) of the patients possessed BRCA2 VUS. Fifty-three specific BRCA1 VUS were found and 20 were further reclassified as benign (n=11), likely benign (n=5), likely pathogenic (n=3), and pathogenic (n=1). The remaining 33 remained classified as VUS. For BRCA2, 55 specific VUS were detected; among these, 14 were reclassified as benign or likely benign, and 2 were reclassified as likely pathogenic. Among the 805 patients, 195 were found to have only VUS and no pathogenic variants (PV), and 41.5% (81/195) were reclassified as benign or likely benign, and 10.3% (20/195) as pathogenic or likely pathogenic variants. Conclusions Approximately 33.3% (36/108) of the specific BRCA1/2 variants analyzed in this study that were initially classified as VUS over a 13-year period were reclassified. Among these, 5.6% (6/108) were reclassified as pathogenic or likely pathogenic variants.