Type II membranoproliferative glomerulonephritis (MPGN) is characterized by thickening of the glomerular basement membrane owing to electron-dense deposits on electron microscopy. We experienced a case of type II MPGN in a child presenting with proteinuria, hematuria on school urinary screening tests. He had been treated with losartan and enalapril. This is the first case report of type II MPGN detected by school urinary screening tests in Korea. Thus we report a case of 10-years-old male with type II MPGN with a review of brief literature.
Child ; Enalapril ; Glomerular Basement Membrane ; Glomerulonephritis, Membranoproliferative ; Hematuria ; Humans ; Korea ; Losartan ; Male ; Mass Screening ; Microscopy, Electron ; Proteinuria

BACKGROUND/AIMS: When hepatocellular carcinoma (HCC) is exposed to hypoxic condition, HIF-1α is activated and results in angiogenesis and increased tumor burden. Although inhibition of HIF-1α may reduce tumor growth, there are some limitations to control tumor growth completely. For a more effective therapy for HCC, we investigated HIF-1α independent pathway related tumor growth with angiogenesis. METHODS: We cultured HepG2 cells (HCC cell line) in both normoxia and hypoxia conditions. These cells were divided into three groups: a echinomycin treated group, a echinomycin and quinazoline treated group and a control group without any treatments. Growth morphologies of cells were observed with a microscope after 24 hours. Immunocytochemistry assay was done to detect the angiogenesis during inhibition of HIF-1α and/or NF-κB in hypoxia condition, and compared with results in normoxia condition. RESULTS: In normoxia, the expression of HIF-1α on tumor growth was not found. In hypoxia, inhibition of HIF-1α reduced the tumor growth compared to the control group. But, inhibition of both HIF-1α and NF-κB did not show apparent reduction of tumor growth as shown in HIF-1α only group. CONCLUSIONS: Signaling pathways related to cancer cell growth exist through a vast network. Inhibition of one target molecule may result in over-expression of other molecules related to the tumor growth. For an effective therapy in blocking of the tumor growth, more comprehensive understanding of the network related to signaling pathways on tumor growth is necessary.
Angiogenesis Inducing Agents ; Anoxia ; Carcinoma, Hepatocellular ; Echinomycin ; Hep G2 Cells* ; Immunohistochemistry ; Tumor Burden

Bronchiolitis obliterans (BO), which is associated with graft-versus-host disease after allogenic hematopoietic stem cell transplantation, is a major obstacle to survival after bone marrow transplantation due to its gradual progress, eventually leading to respiratory failure. Pumpless extracorporeal interventional lung assist (iLA) is effective in treatment of reversible hypercapnic respiratory failure. In this paper, we present a 23-year-old female patient who underwent allogeneic peripheral blood stem cell transplantation (PBSCT) for acute lymphocytic leukemia. After 6 months, she complained of shortness of breath and was diagnosed with BO. Five months later, she developed an upper respiratory tract infection that worsened her BO and caused life-threatening hypercapnia. Since mechanical ventilation failed to eliminate CO2 effectively, iLA was applied as rescue therapy. Her hypercapnia and respiratory acidosis showed significant improvement within a few hours, and she was successfully weaned off iLA after 12 days. This is the first case report of iLA application for temporarily aggravated hypercapnia of PBSCT-associated BO followed by successful weaning. This rescue therapy should be considered in ventilator-refractory reversible hypercapnia in BO patients.
Acidosis, Respiratory ; Bone Marrow Transplantation ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Dyspnea ; Female ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Hypercapnia ; Lung* ; Peripheral Blood Stem Cell Transplantation* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Respiration, Artificial ; Respiratory Insufficiency ; Respiratory Tract Infections ; Weaning ; Young Adult

Fabry disease, angiokeratoma corporis diffusum, is a rare X-linked inborn error of glycosphingolipid metabolism due to the lack of the lysosomal enzyme, alpha-galactosidase A, resulting in a progressive deposition of specific neutral glycosphingolipids within the lysosomes of endothelial and smooth muscle cells of the cardiovascular and renal systems predominantly. We reported a case of Fabry disease, following renal biopsy for the investigation of proteinuria(Creatinine clearance 87.28 mL/min/1.73, serum creatinine 1.1 mg/dL, 24-hour urine protein 1,125 mg, 24-hour urine creatinine 1,382 mg). The patient was 46 year old male. He had experienced anterior chest pain regarded as angina pectoris for a few years. A 12- lead electrocardiogram was abnormal(T-wave inversion in II, III, AVF, and V3-V6), but echocardiography and coronary angiography revealed no abnormal. Kidney biopsy findings showed lamella inclusion bodies on electron microscopy, which are typical finding of Fabry disease. The patient is followed at O.P.D without any significant complaints for 18 months after diagnosis of Fabry disease.
alpha-Galactosidase ; Angina Pectoris ; Biopsy* ; Chest Pain ; Coronary Angiography ; Creatinine ; Diagnosis ; Echocardiography ; Electrocardiography ; Fabry Disease* ; Humans ; Inclusion Bodies ; Kidney ; Lysosomes ; Male ; Metabolism ; Microscopy, Electron ; Middle Aged ; Myocytes, Smooth Muscle ; Neutral Glycosphingolipids ; Proteinuria*

PURPOSE: This is study of whether 3-D conformal radiotherapy for carcinomas of the ethmoid sinus were better than those treated with conventional 2-D plan. MATERIALS AND METHODS: The 3-D conformal treatment plans were compared with conventional 2-D plans in 4 patients with malignancy of the ethmoid sinus. Isodose distribution, dose statistics, and dose volume histogram of the planning target volume were used to evaluate differences between 2-D and 3-D plans. In addition, the risk of radiation exposure of surrounding normal critical organs are evaluated by means of point dose calculation and dose volume histogram. RESULTS : 3-D conformal treatment plans for each patient that the better tumor coverages by the planning target volume with improved dose homogeneity, compared to 2-D conventional treatment plans in the same patient. On the other hand, the radiation dose distributions to the surrounding normal tissue organs, such as the orbit and optic nerves are not significantly reduced with our technique, but a substantial sparing in the brain stem and optic chiasm for each patient. CONCLUSION : Our findings represented the potential advantage of 3-D treatment planning for dose homogeniety as well as sparing of the normal tissue surrounding the tumor. However, further investigational studies are required to define the clinical benefit.
Brain Stem ; Ethmoid Sinus* ; Hand ; Humans ; Optic Chiasm ; Optic Nerve ; Orbit ; Radiotherapy, Conformal*

Microinvasive carcinoma of the uterine cervix(FIGO stage IA) has been reported as highly curable disease even with conservative surgery such as conization and simple hysterectomy. Nevertheless, the surgical management for microinvasive carcinomas has been proposed varying from conservative surgery to radical hysterectomy with pelvic nodes dissection according to different diagnostic criterias for microinvasive carcinoma. We reviewed 512 patients who had been diagnosed as microinvasive carcinoma of the uterine cervix at the Department of Obstetrics and Gynecology, Samsung Cheil Hospital and Women's Healthcare Center from Jan. 1988 to Dec. 1995. Among them, 376 patients were included in this study satisfying guided criterias such as proper management and follow up more than at least one year, and they were analyzed retrospectively based on the clinicopathologic characteristics, pattern of surgical management and postoperative status. (continue)
Cervix Uteri* ; Conization ; Delivery of Health Care ; Diagnosis* ; Female ; Follow-Up Studies ; Gynecology ; Humans ; Hysterectomy ; Obstetrics ; Retrospective Studies

Air can be introduced into the epidural space during the loss of resistance technique used to identify needle entry into the epidural space. Complications resulting from the injection of air into the epidural space include pneumocephalus, spinal cord and nerve root compression, retroperitoneal air collection, subcutaneous emphysema, venous air embolism, and possibly, incomplete analgesia and anesthesia. We experienced a case of a large epidural collection of air following epidural anesthesia attempted one month before. The CT scan revealed epidural air extending from L1-S1 with moderate compression of nerve root at L4-5.
Anesthesia and Analgesia ; Anesthesia, Epidural ; Embolism, Air ; Epidural Space ; Needles ; Pneumocephalus ; Radiculopathy ; Spinal Cord ; Subcutaneous Emphysema ; Tomography, X-Ray Computed

PURPOSE: We retrospectively investigated to find out the equation of calculating the probability of minimal change nephrotic syndrome (MCNS) using clinical parameters. We prospectively investigated to determine the usefulness of the mathematical model. METHODS: We retrospectively examined 56 patients with nephrotic syndrome (NS) (30 MCNS and 26 non-MCNS) diagnosed by kidney biopsy. A mathematical model for calculating the probability of MCNS was obtained through multiple logistic analysis in SAS statistics package. In addition, we prospectively studied 28 patients with NS. Clinical MCNS and non-MCNS were classified according to the probability of 85% in the mathematical model. Kidney biopsy was performed, and serum albumin and urinalysis were measured after 2 weeks of steroid treatment. RESULTS: In the retrospective study, the mathematical model was P=ea/(1+ea), a=17.2507 - 5.5777xON - 4.2256xALB-0.000579x24PROT - 1.2569xUBL+2.1703xUAL. The mode of onset (ON), 24 hours urine protein (24PROT), serum albumin concentration (ALB), the grade of hematuria (UBL) and proteinuria (UAL) were included as clinical parameters. At the probability of 85%, the sensitivity and specificity for predicting MCNS was 73.3% and 100% respectively. In the prospective study, the result of kidney biopsy was consistent with clinical MCNS and non-MCNS according to a mathematical model. All clinical MCNS showed negative proteinuria on urinalysis and a significant increase in serum albumin after 2 weeks treatment (1.85+/-0.30 g/dL to 2.88+/-0.26 g/dL, p<0.05). CONCLUSION: We conclude that the mathematical model for predicting the probability of MCNS may be useful in diagnosis of the MCNS.
Biopsy ; Diagnosis ; Hematuria ; Humans ; Kidney ; Models, Theoretical* ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Prospective Studies ; Proteinuria ; Retrospective Studies ; Sensitivity and Specificity ; Serum Albumin ; Urinalysis

BACKGROUND: Bronchial anthracofibrosis usually manifest as a form of obstructive airway disease, and can be accompanied by parenchymal diseases such as pneumonia, and pulmonary tuberculosis. This study investigated the ventilatory dynamics according to the severity of bronchial stenosis in patients with bronchial anthracofibrosis. Method : One hundred and thirteen patients with bronchial anthracofibrosis that was confirmed by bronchoscopy and who had undergone a pulmonary function test were enrolled in this study group. The correlation coefficients between the pulmonary functional parameters and the number of lobes with bronchial stenosis were investigated. RESULTS: The incidence of ventilatory dysfunction was 56(49.6%) for obstructive, 8(7.1%) for restrictive, 2(1.8%) for mixed, and 47(41.6%) for a normal pattern. The FEV1/FVC, FEF25~75%, FEF25%, FEF50%, FEF75%, and PEF showed a significant negative correlation (p<0.05) and the Raw had a significant positive correlation with the number of lobes with bronchial stenosis(p<0.001). CONCLUSION: These findings suggest that the most common abnormality of the ventilatory function in bronchial anthracofibrosis is an obstructive pattern with a small airway dysfunction according to the severity of bronchial stenosis.
Airway Resistance ; Bronchoscopy ; Constriction, Pathologic* ; Humans ; Incidence ; Pneumonia ; Respiratory Function Tests ; Tuberculosis, Pulmonary

Language disorganization, an objective component of formal thought process abnormality, has been regarded as a core symptom of schizophrenia from an evolutionary psychopathology perspective. However, to the best of our knowledge, the network structure of language disorganization has rarely been examined in patients with schizophrenia. Thus, our preliminary study aimed to evaluate the network structure using the Clinical Language Disorder Rating Scale (CLANG) in 167 inpatients with schizophrenia. All 17 of the CLANG items were considered to be ordered categorical variables ranging from 0 to 3. Our results indicated that disclosure failure, excess syntactic constraints, abnormal prosody, and aprosodic speech rank among the top five central domains within the network structure. We deemed that disclosure failure and prosody problems are the most important symptoms of language disorder in schizophrenia. Thus, reduced top-down processing of linguistic information may be a core neurobiological underpinning of language disorganization in schizophrenia. Further studies controlling for the potential effects of confounding factors (i.e., duration of illness) on network analyses of language disorder and formal thought disorder are warranted in patients with schizophrenia.