Primary Glomerulonephritis with Unique C4d Deposition and Concurrent Non-infectious Intermediate Uveitis: a Case Report and Literature Review

C4 glomerulopathy is a recently introduced entity that presents with bright C4d staining and minimal or absent immunoglobulin and C3 staining. We report a case of a 62-year-old man with C4 glomerulonephritis (GN) and uveitis. He presented to the nephrology department with proteinuria and hematuria. The patient also had intermediate uveitis along with proteinuria and hematuria. A kidney biopsy that was performed in light of continuing proteinuria and hematuria showed a focal proliferative, focal sclerotic glomerulopathy pattern on light microscopy, absent staining for immunoglobulin or C3 by immunofluorescence microscopy, with bright staining for C4d on immunohistochemistry, and electron-dense deposits on electron microscopy. Consequently, C4 GN was suggested as the pathologic diagnosis. Although laser microdissection and mass spectrometry for glomerular deposit and pathologic evaluation of the retinal tissue were not performed, this is the first report of C4 GN in Korea and the first case of coexisting C4 GN and uveitis in the English literature.

Unmasked chronic renal function deterioration after unilateral adrenalectomy in patients with primary aldosteronism

We report 2 cases of chronic estimated glomerular filtration rate (eGFR) decline after unilateral adrenalectomy due to primary aldosteronism. The patients were diagnosed with unilateral adrenal cortical adenoma releasing aldosterone. Two patients were examined for hypertension and hypokalemia. Unilateral laparoscopic adrenalectomy was performed in both cases, and pathology confirmed adrenal cortical adenoma. After adrenalectomy, hypertension and hypokalemia improved to within normal range. However, the eGFR decreased postoperatively, and abdominal computed tomography scan showed decreased kidney size compared to previous images. Kidney biopsy was performed to delineate the exact cause of renal function deterioration and revealed hypertensive changes with chronic interstitial changes, indicating that glomerular hyperfiltration with aldosterone excess masked renal function damage. Physicians have to consider the probability of postadrenalectomy eGFR decline related to chronic hypertensive change.

Severe Hypernatremia Caused by Acute Exogenous Salt Intake Combined with Primary Hypothyroidism

This report describes a case of severe hypernatremia with a serum sodium concentration of 188.1mmol/L caused by exogenous salt intake. A 26-year-old man diagnosed with Crohn's disease 5 years previously visited our clinic due to generalized edema and personality changes, with aggressive behavior. He had compulsively consumed salts, ingesting approximately 154 g of salt over the last 4 days. Despite careful fluid management that included not only hypotonic fluid therapy for 8 hours but also hypertonic saline administration, his serum sodium level decreased sharply at 40.6 mmol/L; however, it returned to normal within 72-hour of treatment without any neurological deficits. Primary hypothyroidism was also diagnosed. He was discharged after 9 days from admission, with a stable serum sodium level. We have described the possibility of successful treatment in a patient with hypernatremia caused by acute salt intoxication without sustained hypotonic fluid therapy.

Association between Vitamin D Deficiency and Carotid Intima-media Thickness in Patients with Rheumatoid Arthritis

OBJECTIVE: The present study determined if vitamin D deficiency is a potential risk factor for increased carotid intima-media thickness (CIMT) in patients with rheumatoid arthritis (RA). METHODS: This cross-sectional study analyzed 50 consecutive female RA patients without cardiovascular disease history at the Pusan National University Hospital between September and December of 2013. CIMT was measured using a high-resolution ultrasonography. Serum 25-hydroxy vitamin D (25-OHD) levels were assessed by radioimmunoassay, and vitamin D deficiency was defined as serum 25-OHD levels <20 ng/mL. Stepwise multivariable linear regression analyses were performed to evaluate the association between vitamin D deficiency and increased CIMT. RESULTS: The median 25-OHD level (inter-quartile range) was 14.0 (11.0~20.7) ng/mL, and 74% of patients had vitamin D deficiency. The mean+/-standard deviation of CIMT was 0.58+/-0.08 mm. RA patients with vitamin D deficiency had significantly higher CIMT than those without this feature (0.59+/-0.07 vs 0.54+/-0.05, p=0.028). In univariable linear regression models, vitamin D deficiency (beta(SE)=0.047 (0.021), p=0.028), older age (beta(SE)=0.003 (7.2(-4)), p<0.001) and higher disease activity score 28-erythrocyte sedimentation rate (beta(SE)=0.021 (0.010), p=0.034) and Korean version of health assessment questionnaire score (beta(SE)=0.051 (0.015), p=0.002) were significantly associated with increased CIMT. Vitamin D deficiency remained statistically significant in multivariable regression models after adjusting for confounders. CONCLUSION: Vitamin D deficiency was associated with increased CIMT in female RA patients. Our finding suggests that hypovitaminosis D can be a risk factor for atherosclerosis in RA patients.

Pancreatoblastoma in an Adult / 대한췌담도학회지

A blastoma is a type of cancer, which is common in children; it is caused by malignancies derived from in the precursor cells, often called blasts. Examples are nephroblastomas, retinoblastomas, pleuropulmonary blastomas, and pancreatoblastomas. Pancreatoblastomas are extremely rarely in adults. It is difficult preoperatively to distinguish this tumor from other pancreatic tumors including solid and papillary epithelial neoplasm of the pancreas (SPEN), acinar cell carcinoma, islet cell tumor, and ductal adenocarcinoma with cystic degeneration. To our knowledge, this case may be the second report of a pancreatoblastoma occurring in an adult in Korea. We report a case of a pancreatoblastoma that was confirmed by pathology, despite the radiologic finding that assumed it was a SPEN.

A Common Bile Duct Web in Association with Bile Duct Stone Treated with Balloon Dilatation / 대한췌담도학회지

Bile duct web is very rare disease and it's etiology is controversial. Some webs are occurred in the presence of chronic inflammation, frequently associated with bile duct stone, but others are thought to be congenital. Many patients with bile duct web are asymptomatic, but they sometimes present symptom of biliary obstruction and cholangitis. It can be diagnosed by endoscopic retrograde cholangiopancreatogram, typically appearing as thin and shelf like radiolucent ring. We report a case of the common bile duct web with bile duct stones diagnosed by Endoscopic retrograde cholangiopancreatography (ERCP) in a 65-year-old man. The patient was treated by balloon dilatation successfully.

A Case of Intrahepatic Bile Duct Sarcoma; Misdiagnosed with Liver Abscess Combined with Intrahepatic Ductal Stone / 대한췌담도학회지

Sarcoma is rare malignant tumor originated from mesenchymal stem cells that can differentiate to soft tissue and bone. Therefore sarcoma can be arised from any regions in human body. However, the incidence of bile duct sarcoma is extremely rare in adults. Obstructive jaundice is the most common presentation in the patients, but there're no specific symptoms or signs. Also it can be misdiagnosed with other tumors or benign lesions in computed tomography or ultrasonography. For these reasons, it is hard to diagnose and manage. We present a case of intrahepatic bile duct sarcoma misdiagnosed with liver abscess in 70 year-old female with literature review.

New-Onset Malignant Pleural Effusion after Abscess Formation of a Subcarinal Lymph Node Associated with Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration / 결핵및호흡기질환

We present a case of an unusual infectious complication of a ruptured mediastinal abscess after endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), which led to malignant pleural effusion in a patient with stage IIIA non-small-cell lung cancer. EBUS-TBNA was performed in a 48-year-old previously healthy male, and a mediastinal abscess developed at 4 days post-procedure. Video-assisted thoracoscopic surgery was performed for debridement and drainage, and the intraoperative findings revealed a large volume pleural effusion that was not detected on the initial radiographic evaluation. Malignant cells were unexpectedly detected in the aspirated pleural fluid, which was possibly due to increased pleural permeability and transport of malignant cells originating in a ruptured subcarinal lymph node from the mediastinum to the pleural space. Hence, the patient was confirmed to have squamous cell lung carcinoma with malignant pleural effusion and his TNM staging was changed from stage IIIA to IV.

Study on Perceived Occupational Psychosocial Stress and Work-related Musculoskeletal Disorders among VDT Works / 대한산업의학회지

It has been hypothesized that jobs that have both high psychological demands and low decision latitude ('job strain") can lead to musculoskeletal disorder. The objective of this study was to test whether job strain was correlated with the presence of work-related musculoskeletal disorder, especially myofascial pain syndrome (MPS). Information on demographic factors, confounders such as household load and taking care of children or not, and scores for decision latitude, job demand, and social support was obtained by self-administered questionnaire, which had been developed in Korean language, by adopting NIOSH instrument and Extended Karasek Model (16 items). All subjects were also examined by rehabilitation medicine specialists for musculoskeletal disorders. Subjects (n=370) could be categorized into 4 groups, these were, housewives(n=89), shipyard CAD workers(n=89), general female workers(n=79; nurses, insurance counselors, public officials, clerks, etc), and telephone directory assistance operators(n=113). Results from univariate analyses indicated that all demographic factors, all confounders, scores for decision latitude and social support were not associated with the risk of musculoskeletal disorder. However, score for job demand was higher in musculoskeletal disorder cases than others. In subgroup analysis, this association was convinced again, in telephone directory assistance operators. Job strain model showed that the group of telephone directory assistance operators was high-strain group, and OR of musculoskeletal disorder was 2.446 (95% C.I. : 1.174, 5.096), when comparing this with the low strain group. In conclusion, job strain is a risk factor for work-related musculoskeletal disorder.

Changes of Anti-H. influenzae Type b IgG in the Sera of Korean Infants and Children after Birth

PURPOSE: Most of invasive Haemophilus influenzae diseases occur in children under 5 years of age, and are due almost exclusively to type b strain. Although antibodies to several surface antigens of H. influenzae play a role in conferring immunity, antibody to the type b capsular polysaccharide appeared to have the most important protective functions. However, the antibody response to vaccines or natural infections are quite differ according to the ages and ethnic groups. This study was performed to investigate the need of Hib vaccination and its appropriate time in Korean infants. METHODS: Three hundred and forty-five Korean infants and children who were relatively well without history of Hib vaccination or infection were enrolled in the study. All subjects did not receive blood transfusion or blood products and also had no any immunological abnormalities. Anti-PRP IgG was measured in the sera of subjects using ELISA. PRP-albumin was used as a coating antigen. RESULTS: Geometric mean titer (GMT) of anti-PRP IgG in the sera of neonates was 0.594 g/ml and was gradually decreased to 0.186 g/ml and 0.111 g/ml at 2 and 3 months of age, respectively. Ant-PRP IgG was significantly low after 3 months of age, and was gradually increased after 10 months of age. Anti-PRP IgG level p> or = 0.15 g/ml was observed in 70.8% (17/24) in neonatal group, 41.6% (10/24) and 18.7% (3/16) in 2 and 3 months of age. Only 8.7% (10/115) showed anti-Hib IgG levels of p> or = 1.0 g/ml, which has been considered as a level of longterm protection, was observed in 37.5% (9/24) in neonates, 12.5% (3/24) in 2 months of age and less then 10% thereafter. CONCLUSIONS: These results suggest that congenital passive immunity can be obtained enough in Korean infants and was rapidly decreased during the period of 3 months after birth. Hib vaccination will be recommended at early infancy (2 months of age) to provide appropriate antibodies in Korean children.

Analysis of Platelet Membrane Glycoprotein Iib-IIIa Complex in Whole Blood of Glanzmann's Thrombasthenia by Flow Cytometry

Glanzmann's thrombasthenia is a rare autosomal recessive hemorrhagic disorder characterized by prolonged bleeding time, ad deficient or absent clot retraction in the presence of normal platelet count. The major underlying abnormality in this disease is grossly defective first-phase aggregation of platelet, which are unresponsive to ADP or other platelet agonists such as epinephrine, collagen, thrombin in any concentration. This disability is caused by a decrease or absence of the platelet membrans glycoprotein IIb-IIIa complex, a member of the integrin family of adhesive receptors involved in cell-cell and cell-matrix fibronectin, and vitronectin On the development of surface labeling technique, a variety of biochemical techniques such as radioimmunoassay, crossed immunoelectrophoresis and SDS-PAGE have been used to study the structure and the function of platelet membrane glycoproteins, and to detect the platelet functional defect. But all of these techniques demand a relatively large amount of homogeneous paletelet population that requires manipulation through isolation and washing procedures before analysis. In order to eliminaste such an intricate procedure, we have applied method for analyzing platelet surface components in whole blood using monoclonal antibody and flow cytometry to recognize the absence of severe reduction of platelet membrane glycoprotien llb-llla complex. Platelet analysis by flow cytometry is a successful alternative rapid diagnostic technique for Glanzmann's thrombasthenia patients as well as well as for carriers of this disease. Fow cytometry technique provides a sensitive tool for investigating platelet functional defects caused by altered expression or deficiency of platelet surface proteins.

A study on the effect of pulsed Nd: YAG laser impacts to the enamel surface and bond strength with composite resin / 대한치과보철학회지

No abstract available.