Authors experienced a case of A3B in a 46-year-old patient with liver cirrhosis and two cases of A3 in her children by family study. A3 subgroups were confirmed by delayed and weak positive with anti-A and anti-A,B, negative in anti-A1 lectin, adsorption-elution test, and family study. We report a family case of A3B and A3 with brief review of literatures.
Child ; Humans ; Liver Cirrhosis ; Middle Aged

BACKGROUND: Observation on the hemodialysis treatment; whether there are any different effects on mortality rate, side effects, or effectiveness of the treatment depending on patients' age. METHODS: Chronic renal failure patients who started hemodialysis treatment between 2002 and 2003 were separated in two groups, 33 patients of 60 and older and 76 patients under 60. We investigated mortality rate difference, A-V shunt re-operation, and total protein and albumin level. Also, comparing and analyzing the urea reduction ratio, we examined whether effectiveness is different depending on the patients' age. RESULTS: The 3 year survivor rate of patients who initially started hemodialysis treatment over 60 was 66.7%, and that of the patients under 60 resulted as 73.1%. The average total protein lefel for over 60 was 6.48g/dL and for under 60 was 6.80g/dL, and albumin for over 60 was 3.15g/dL and under 60 was 3.46g/dL. We identified that the patients who repeated shunt operation in over 60 group was 4(12%) and in under 60 group was 4(5.2%). Effectiveness of the treatment, Urea Reduction Ratio(URR), for both groups was 71%. CONCLUSION: The 3 year survivor rate of patients who initially started hemodialysis treatment over 60 was lower than the control group, but considering the remaining life expectancy, it seems to be no significant difference between the groups; and the treatment effectiveness was same in both groups.
Humans ; Kidney Failure, Chronic ; Life Expectancy ; Prognosis ; Renal Dialysis ; Survivors ; Treatment Outcome ; Urea

Background: Direct immunofluorescence (DIF) is a histochemical technique used to detect tissue-bound autoantibodies and diagnose various immune-mediated skin diseases. Objective: This study aimed to evaluate the sensitivity of DIF for each disorder, and the consistency between clinical, histopathological, and DIF results. Methods: A retrospective study was conducted in 194 patients who underwent skin biopsy and DIF testing at our hospital between January 2011 and December 2021. An antibody panel against immunoglobulin G (IgG), IgA, IgM, C3, C1q, and fibrinogen was used. The concordance rate and κ-coefficient between the clinical, histopathological, and DIF results were evaluated. Results: DIF was observed to be positive in 87 cases; 51 cases of immune-mediated bullous diseases, seven cases of connective tissue diseases (CTDs), 25 cases of vasculitis, and four cases of other diseases. The overall sensitivity of DIF for immune-mediated bullous diseases was 71.8%, which was higher than that of histopathology (64.8%). In CTDs and vasculitis, the overall sensitivities of DIF were 30.4% and 65.8%, respectively, which were lower than those of histopathology (73.9% and 84.2%, respectively). In addition, good concordance among the clinical, histological, and DIF results was observed. Conclusion DIF is a useful diagnostic method, especially for immune-mediated bullous diseases, lupus erythematosus, and Henoch-Schonlein purpura. However, in other CTDs and vasculitis cases, the sensitivity of DIF is relatively low. Therefore, the diagnostic value of DIF along with clinical and histopathological findings will be maximized only when the DIF test is performed for appropriate diseases.

BACKGROUND/OBJECTIVES: Atherosclerosis is associated with increased inflammation in the visceral adipose tissue (VAT). Vitamin D has been reported to modulate the inflammatory responses of stromal vascular cells (SVCs) and adipocytes in adipose tissue, but the role of vitamin D in atherosclerosis biology is unclear. This study examined the effects of in vitro 1,25-dihydroxyvitamin D 3 (1,25[OH] 2 D 3 ) treatment on the inflammatory responses of SVCs and adipocytes from atherosclerotic mice.MATERIALS/METHODS: C57BL/6J (B6) mice were divided randomly into 2 groups and fed a 10% kcal fat control diet (control group, CON) or 41% kcal fat, 0.21% cholesterol (high fat+ cholesterol, HFC) diet (obese group, OB), and B6.129S7-Ldlr tm1Her /J (Ldlr −/− ) mice were fed a HFC diet (obese with atherosclerosis group, OBA) for 16 weeks. SVCs and adipocytes isolated from VAT were pre-incubated with 1,25(OH) 2 D 3 for 24 h and stimulated with lipopolysaccarides for the next 24 h. Proinflammatory cytokine production by adipocytes and SVCs, the immune cell population in SVCs, and the expression of the genes involved in the inflammatory signaling pathway in SVCs were determined. RESULTS: The numbers of total macrophages and SVCs per mouse were higher in OB and OBA groups than the CON group. The in vitro 1,25(OH) 2 D 3 treatment significantly reduced macrophages/SVCs (%) in the OBA group. Consistent with this change, the production of interleukin-6 and monocyte chemoattractant protein 1 (MCP-1) by SVCs from the OBA group was decreased by 1,25(OH) 2 D 3 treatment. The 1,25(OH) 2 D 3 treatment significantly reduced the toll-like receptor 4 and dual-specificity protein phosphatase 1 (also known as mitogenactivated protein kinase phosphatase 1) mRNA levels in SVCs and MCP-1 production by adipocytes from all 3 groups. CONCLUSIONS These findings suggest that vitamin D can attribute to the inhibition of the inflammatory response in VAT from atherosclerotic mice by reducing proinflammatory cytokine production.

Background: Fibroblasts produce collagen molecules that support the structure of the skin.The decrease and hypersynthesis of collagen causes skin problems such as skin atrophy, wrinkles and scars. Objective: The purpose of this study is to investigate the mechanism of mitoxantrone on collagen synthesis in fibroblasts. Methods: Cultured fibroblasts were treated with mitoxantrone, and then collagen synthesis was confirmed by reverse transcription-polymerase chain reaction and Western blot. Results: Mitoxantrone inhibited the expression of type I collagen in fibroblasts at both the mRNA and protein levels. In the collagen gel contraction assay, mitoxantrone significantly inhibited gel contraction compared to the control group. Mitoxantrone inhibited transforming growth factor (TGF)-β-induced phosphorylation of SMAD3. Finally, mitoxantrone inhibited the expression of LARP6, an RNA-binding protein that regulates collagen mRNA stability. Conclusion These results suggest that mitoxantrone reduces collagen synthesis by inhibiting TGF-β/SMAD signaling and LARP6 expression in fibroblasts, which can be developed as a therapeutic agent for diseases caused by collagen hypersynthesis.

BACKGROUND: The research sought to identify the clinical features of pleomorphic carcinoma of the lung generally known as a rare subtype in accordance with the lung cancer classification done in 1999 by WHO. MATERIAL AND METHOD: 256 cases of surgically resected lung cancers were collected in this hospital from January 1992 to December 2001. This study included 42 cases of pleomorphic carcinoma diagnosed through light microscope and immunohistochemistry. RESULT: Out of 42 cases, males represented 31, and females 11, the age ranged from 26 to 77. Main clinical symptoms included coughing, hemoptysis, sputum. Diagnoses disclosed the stage as stage Ia in 3 cases (7%), Ib in 16 (38%), IIa in 1 (2%), IIb in 8 (19%), IIIa in 15 (35%), and IIIb in 1 (2%). Out of these, no lymph node metastasis was represented in 23 cases (54%), while N1 and N2 involving lymph node metastasis was shown 19 cases (46%). A total of 19 patients developed metastasis, comprising the brain in 5 cases (26%), bone in 4 (21%), muscle in 4 (21%), Lymph node in 2 (10%), and 1 liver, ovary, contralateral lung, and adrenal gland, respectively. The size of the tumor ranged from 1 cm to 11 cm, averaging 5.85 cm. Out of the 42 patients, the total two-year and five-year survival rates in accordance with the Kaplan-Meier method represented 26% and 13%, respectively, These figures compared to the corresponding 44% and 34% in cases other than pleomorphic carcinoma from the survey target of 256 cases, proved to be significantly low (p <0.002). No significant difference was found in the survival rates compared between age and tumor size, between stage I and above stage II, and between N0 and above N1. Patients who developed postoperative metastasis all died, and showed significantly low survival rates (p <0.002) compared to those patients without metastasis. CONCLUSION: With the new diagnosis method of 1999 WHO's lung cancer classification applied, pleomorphic carcinoma showed a higher prevalence rate than under previous classifications, their postoperative survival rate was significantly low compared to histologic type of non small cell lung carcinomas.
Adrenal Glands ; Brain ; Carcinoma, Non-Small-Cell Lung ; Classification ; Cough ; Diagnosis ; Female ; Hemoptysis ; Humans ; Immunohistochemistry ; Liver ; Lung ; Lung Neoplasms ; Lymph Nodes ; Male ; Neoplasm Metastasis ; Ovary ; Prevalence ; Sputum ; Survival Rate

BACKGROUND: Alopecia areata (AA) is an autoimmune skin disease difficult to manage and treat. The pathogenesis of AA features a T-cell-associated autoimmune process, and systemic immunosuppressive therapy is prescribed widely for AA. OBJECTIVE: To evaluate the efficacy and tolerance of systemic low-dose methotrexate (LD-MTX) therapy in treatment of recalcitrant AA multiplex. METHODS: In a retrospective, non-controlled study, we evaluated 29 patients with recalcitrant AA treated with LD-MTX and assessed the therapeutic response according to severity of disease, disease duration, cumulative dose of MTX, and drug safety. RESULTS: MTX was administered twice weekly, and the mean maximum weekly dose was 14.48 mg. The response was A5 (regrowth=100.0%) in 14 (48.3%) patients and A4 (regrowth of 75%~90%) in 12 (41.4%) patients. Three patients had poor response to LD-MTX treatment (A2: n=2 [6.9%], A1: n=1 [3.4%]). All three of the patients showing a poor response had disease durations exceeding 24 months. Relapse was observed in 31% of patients with more than 75% regrowth. Common side-effects were elevated liver enzyme levels and gastrointestinal discomfort. CONCLUSION: LD-MTX appears to be an effective and well-tolerated treatment for recalcitrant AA multiplex.
Alopecia Areata* ; Alopecia* ; Humans ; Liver ; Methotrexate* ; Recurrence ; Retrospective Studies ; Skin Diseases

OBJECTIVE: Although three-dimensional time-of-flight magnetic resonance angiography (3D TOF-MRA) is used frequently as a follow-up tool to assess the response of arteriovenous malformations (AVMs) after radiosurgery, the diagnostic accuracy of 3D TOF-MRA is not well known. We evaluated the diagnostic accuracy of contrast-enhanced 3D TOF-MRA at 3.0 Tesla for the detection of residual AVMs. MATERIALS AND METHODS:This study included 32 AVMs from 32 patients who had been treated with radiosurgery (males/females: 21/11; average patient age, 33.1 years). The time interval between radiosurgery and MRA was an average of 35.3 months (range, 12-88 months). Three-dimensional TOF-MRA was obtained at a magnetic field strength of 3.0 Tesla after infusion of contrast media, with a measured voxel size of 0.40 x 0.80 x 1.4 (0.45) mm3 and a reconstructed voxel size of 0.27 x 0.27 x 0.70 (0.05) mm3 after zero-filling. X-ray angiography was performed as the reference of standard within six months after MRA (an average of two months). To determine the presence of a residual AVM, the source images of 3D TOF-MRA were independently reviewed, focusing on the presence of abnormally hyperintense fine tangled or tubular structures with continuity as seen on consecutive slices by two observers blinded to the X-ray angiography results. RESULTS: A residual AVM was identified in 10 patients (10 of 32, 31%) on X-ray angiography. The inter-observer agreement for MRA was excellent (kappa= 0.813). For the detection of a residual AVM after radiosurgery as determined by observer 1 and observer 2, the source images of MRA had an overall sensitivity of 100%/90% (10 of 10, 9 of 10), specificity of 68%/68% (15 of 22, 15 of 22), positive predictive value of 59%/56% (10 of 17, 9 of 16), negative predictive value of 100%/94% (15 of 15, 15 of 16) and diagnostic accuracy of 78%/75% (25 of 32, 24 of 32), respectively. CONCLUSION: The sensitivity of contrast-enhanced 3D TOF-MRA at 3.0 Tesla is high but the specificity is not sufficient for the detection of a residual AVM after radiosurgery.
Adult ; Contrast Media ; Female ; Humans ; Intracranial Arteriovenous Malformations/*diagnosis/*surgery ; Magnetic Resonance Angiography/methods/*standards ; Male ; *Radiosurgery ; Sensitivity and Specificity

BACKGROUND: Protease-activated receptor 2 (PAR-2) participates in various biological activities, including the regulation of epidermal barrier homeostasis, inflammation, pain perception, and melanosome transfer in the skin. OBJECTIVE: To evaluate the basic physiological role of PAR-2 in skin. METHODS: We investigated PAR-2 expression in human epidermis, skin tumors, and cultured epidermal cells using western blot and immunohistochemical analysis. Additionally, we examined the effect of the PAR-2 agonist, SLIGRL-NH2, on cultured keratinocytes. RESULTS: Strong PAR-2 immunoreactivity was observed in the granular layer of normal human skin and the acrosyringium of the eccrine sweat glands. In contrast, weak PAR-2 immunoreactivity was seen in the granular layer of callused skin and in the duct and gland cells of the eccrine sweat glands. Interestingly, PAR-2 immunoreactivity was very weak or absent in the tumor cells of squamous cell carcinoma (SCC) and syringoma. PAR-2 was detected in primary keratinocytes and SV-40T-transformed human epidermal keratinocytes (SV-HEKs), an immortalized keratinocyte cell line, but not in SCC12 cells. SV-HEKs that were fully differentiated following calcium treatment displayed higher PAR-2 expression than undifferentiated SV-HEKs. Treatment of cultured SV-HEKs with PAR-2 agonist increased loricrin and filaggrin expression, a terminal differentiation marker. CONCLUSION: Our data suggest that PAR-2 is associated with terminal differentiation of epidermis and eccrine sweat glands.
Blotting, Western ; Bony Callus ; Calcium ; Carcinoma, Squamous Cell ; Cell Line ; Epidermis* ; Homeostasis ; Humans ; Inflammation ; Keratinocytes ; Melanosomes ; Pain Perception ; Receptor, PAR-2 ; Skin ; Sweat Glands* ; Sweat* ; Syringoma

OBJECTIVES: This study examined the color changes of a resin composite with different shades upon exposure to water with different pH. MATERIALS AND METHODS: Nanohybrid resin composites (Filtek Z350XT, 3M ESPE) with four different shades (A2, A3, B1, and B2) were immersed in water with three different pH (pH 3, 6, and 9) for 14 day. The CIE L*a*b* color coordinates of the specimens were evaluated before and after immersion in the solutions. The color difference (DeltaE*) and the translucency parameter (TP) were calculated using the color coordinates. RESULTS: DeltaE* ranged from 0.33 to 1.58, and the values were affected significantly by the pH. The specimens immersed in a pH 6 solution showed the highest DeltaE* values (0.87 - 1.58). The specimens with a B1 shade showed the lowest DeltaE* change compared to the other shades. TP ranged from 7.01 to 9.46 depending on the pH and resin shade. The TP difference between before and after immersion in the pH solutions was less than 1.0. CONCLUSIONS: The resulting change of color of the tested specimens did not appear to be clinically problematic because the color difference was < 1.6 in the acidic, neutral, and alkaline solutions regardless of the resin shade, i.e., the color change was imperceptible.
Composite Resins* ; Hydrogen-Ion Concentration* ; Immersion* ; Water