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There are no reported case series of common variable immunodeficiency (CVID) from southern Taiwan. A 20-year review was performed in adult CVID from a southern Taiwan medical center. Patients with ages of 18 years or older were enrolled from May, 2003 to April, 2023. Twelve patients were identified, 8 females/4 males aged 23 to 68 (38.9 ± 13.4) with one to 11 years (5.0 ± 3.3) delay of diagnosis after disease onset. There were concomitant autoimmune disorders in 7 (58 %), splenomegaly in 10 (83 %), lymphadenopathy in 4 (25 %) and B-cell lymphoma in 2 (17 %). All received intravenous immunoglobulin (IVIg) infusion with improved autoimmune-mediated arthritis in 2. Patients with higher IgG trough levels (above 500 mg/dL) had a better survival than those with lower IgG trough levels. Adult CVID in southern Taiwan has a high occurrence of autoimmune and lymphoproliferative manifestations. Early diagnosis with IVIg infusion might improve the presentations., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2023 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Liver dysfunction in systemic lupus erythematosus (SLE) is caused by disease activity or secondary conditions like coexistent autoimmune liver diseases. In Taiwan, despite sporadically reported cases of SLE-autoimmune hepatitis (AIH) overlap disease, larger-scale monocentric investigations for such overlapping patients are not available. Retrospective analyses were performed in a hospitalized SLE cohort with 805 patients for identifying co-existent AIH from 2014 to 2023, focusing on distinct therapeutic modalities and differential diagnosis between SLE-AIH overlap and lupus hepatitis (LH). There were 5 cases (a 0.6% occurrence), all females aged 25-58 years (44 ± 13). Ages for the SLE diagnosis were 19-51 years (30 ± 13), while ages for the AIH diagnosis were 22-57 years (36 ± 14). Contradictory to interface hepatitis in SLE-AIH overlap, liver biopsy only demonstrated non-specific abnormalities in LH. Liver cirrhosis was identified in SLE-AIH overlap but not in LH. After corticosteroids/azathioprine therapy, there were normalized liver function in all LH. In 2 SLE-AIH overlap cases refractory to such therapy, one received B-cell depletion therapy (annual rituximab infusion, 375 mg/m 2 weekly × 4) and another accepted living-donor liver transplantation from sibling due to advanced liver cirrhosis, leading to improved hepatic dysfunction in both., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Objective: Although sporadic case reports have demonstrated successful management of eosinophilic granulomatosis with polyangiitis (EGPA) by anti-IL-5 therapy, larger-scale monocentric studies for the efficacy of mepolizumab (MEP), an IL-5 monoclonal antibody, are still lacking in Taiwan., Methods: Hospitalized EGPA patients aged at least 18 years were enrolled from November 1998 to October 2023, and analyzed for demographic, clinical, laboratory, medication and outcome data, focusing on the efficacy and safety of biologics use, particularly induction therapy with MEP., Results: Twenty-seven EGPA patients aged 10-70 years (43 ± 15) at disease diagnosis were recruited with 21 under combined corticosteroids/cyclophosphamide induction therapy. Seventeen patients received biologics with 13 under MEP therapy. Ten patients aged 19-71 years (48 ± 15) completed 12-month induction therapy with a 100 mg quadri-weekly subcutaneous injection regimen indicated for active or relapse disease. There were reduced BVAS with complete remission in 6 and partial remission in 4 patients, lower CRP levels, decreased eosinophil counts with an inhibition of 92∼96 %, and tapered prednisolone dosages from 5 to 25 (13.0 ± 6.3) to 0-10 (3.3 ± 3.1) mg/day. Only one patient had an adverse event of injection site reactions. Nine patients received the same regimen for annual maintenance therapy. All had a persistent clinical remission. In these patients, 13-56 injections (41 ± 15) were prescribed with a follow-up period of 12∼52 months (38 ± 14)., Conclusion: In this retrospective study, induction therapy with a 12-month 100 mg MEP quadri-weekly subcutaneous injection regimen demonstrates the efficacy and safety for active and relapsing EGPA patients., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: Periodical replacement of venous Hickman catheters is required for the nutritional care of patients with intestinal failure. The conventional de novo operation (DN-OP) involves inserting the catheter into a new venous tract in each replacement; however, this could result in fast consumption of functional central vessels in patients with intestinal failure. Recently, same-route operation (SR-OP) has been adopted as an alternative approach for retaining venous access., Methods: We conducted a retrospective study to compare the efficacy of Hickman catheters and the survival of venous vessels using two different operative strategies., Results: Overall, 181 catheters were inserted, 109 using DN-OP and 72 using SR-OP. The mean catheter duration was 11.9 ± 8.8 months in the DN-OP group and 10.5 ± 5.6 months in the SR-OP group; the infection rate was 0.74 in the DN-OP group and 0.44 in the SR-OP group. The vein accesses used in these insertions (n = 113) were classified: the DN-vein group for veins accessed only by DN-OP (n = 75) and the SR-vein group for veins accessed by an initial DN-OP and subsequent SR-OPs (n = 38). Mean working duration per vein access was 12.3 ± 10.1 months in the DN-vein group and 28.2 ± 14.8 months in the SR-vein group (p < 0.001); mean infection-free duration was 11.4 ± 10.1 months in the DN-vein group and 27.7 ± 15.3 months in the SR-vein group (p < 0.001)., Conclusion: Application of SR-OP in Hickman catheter replacement significantly extended the working duration of venous access by re-using the same venous route without compromising catheter efficacy in patients with IF having poor venous access., Competing Interests: Conflict of interest The authors have no conflicts of interest to declare., (Copyright © 2022. Published by Elsevier B.V.)

Virus-associated human cancers may exhibit two characteristic histopathologic features: (1) the inflammation-rich background as observed in Epstein-Barr virus-associated Hodgkin lymphoma (HL) and nasopharyngeal carcinoma (NPC); and (2) the characteristic nuclear morphology such as the Reed-Sternberg cells in HL. Besides, the hepatocytes of chronic hepatitis B virus (HBV) infection frequently exhibit characteristic ground glass hepatocytes, a phenomenon associated with endoplasmic reticulum stress response induced by the overloaded or malfolded HBV surface antigens. In this review, we explore specifically the pathogenesis of Epstein-Barr virus-associated HL and NPC, and HBV-associated hepatocellular carcinoma based on the observed histopathologic features. We propose that the retention of viral proteins induces inflammation, endoplasmic reticulum stress, and genomic instability in HL, NPC, and hepatocellular carcinoma, whereby the viral oncoproteins may play additional transactivational roles to induce host genes for transformation, invasion, and metastasis. Therapeutic implications based on the pathogenesis of virus-associated cancers are discussed., (Copyright © 2013. Published by Elsevier B.V.)

Background and Purpose: Long-term results of coronary artery bypass are limited by progressive atherosclerosis in venous conduits. Arterial conduits are believed to have a better patency rate. A radial artery in composite graft with the left internal thoracic artery makes total arterial revascularization possible in almost all patients. We sought to evaluate the feasibility and short-term results of this grafting strategy., Methods: A total of 381 consecutive patients received elective coronary artery bypass grafting between 1 May 2000 and 31 August 2002. Patients with associated procedures were also included. There were 333 patients without venous conduits. Patients with left ventricular dysfunction were not excluded from total arterial revascularization. Follow-up time was 13.7 +/- 7.6 months., Results: Total arterial revascularization was achieved in 88% of the patients. Arterial harvest was easy and simple and complete revascularization could be achieved. The 30-day overall mortality rate was 3.29%. In the patients with left ventricle ejection fraction greater than 0.4, the mortality rate was 2.32%. There were 6 late deaths: heart failure in 2, sepsis in 2, respiratory failure in 1, and pneumonia in 1., Conclusions: It is feasible to use total arterial grafts for coronary artery bypass graft in patients with coronary artery disease. Myocardial revascularization can be complete and the early mortality rate is acceptable.

Background and Purpose: Acute type A aortic dissection presents a formidable challenge for the cardiac surgeon, although remarkable improvements have been achieved in diagnosis, surgical techniques and perioperative management. The aim of this study was to identify the most important variables associated with in-hospital mortality in patients undergoing surgery for this condition., Methods: Between July 1998 and June 2002, 80 patients underwent surgery for acute type A aortic dissection. Univariate and multivariate analyses were performed to identify the variables independently correlated with in-hospital mortality., Results: The overall in-hospital mortality rate was 20% (16/80 patients). Univariate analysis revealed 24 preoperative and operative variables, including type of surgery, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, diabetes mellitus, and postoperative (postoperative 24 hours) bleeding > or = 1500 mL, as factors associated with in-hospital death. Stepwise logistic regression analysis showed the factors independently associated with in-hospital death were CPB time, diabetes mellitus, and postoperative bleeding > or = 1500 mL (p <0.05)., Conclusions: Multiple factors affect in-hospital mortality after surgery for acute type A aortic dissection. This study suggests that CPB time, diabetes mellitus and postoperative bleeding > or = 1500 mL are the main determinants of in-hospital death.