International Journal of Immunopathology and Pharmacology最新文献

{"title":"Treatment of systemic sclerosis complicated with pyoderma gangrenosum with adalimumab: A case report of a rare disease.","authors":"Chengqiang Ren, Cheng Yu, Maoquan Zhang, Ding Li, Yueyue Zhao","doi":"10.1177/03946320241300137","DOIUrl":"10.1177/03946320241300137","url":null,"abstract":"<p><p>Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic dermatosis characterized by recurrent, painful ulcers that commonly affect the lower extremities but can also involve other parts of the body. Over half of patients with PG have concomitant systemic immune diseases, with the association of PG with systemic sclerosis (SSc) being extremely rare. Treatment of PG primarily involves local therapy, steroids, and immunosuppressants, with an increasing emphasis on biologic agents. Among these, tumor necrosis factor-alpha (TNF-α) antagonists are considered effective. The patient in this report was an elderly female with a history of systemic sclerosis for many years and initially presented with gangrenous ulcers on the fingertips. After inconclusive conventional treatment, adalimumab was added for 5 weeks, resulting in disease suppression, a reduction in ulcer size, and re-epithelialization of the skin lesions after 6 months.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241300137"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11587178/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142711646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Coexistence of tubulocystic renal cell carcinoma and polycythemia vera: A rare case report.","authors":"Renjie Wei, Fudong Liu, Xu Luo, Chunyu Gong, Ruitu Ran","doi":"10.1177/03946320241307777","DOIUrl":"10.1177/03946320241307777","url":null,"abstract":"<p><p>Tubulocystic Renal Cell Carcinoma (TC-RCC) and Polycythemia Vera (PV) are both infrequent medical conditions. TC-RCC was recognized as a distinct subtype of kidney cancer by the World Health Organization in 2016, while PV is a rare myeloproliferative neoplasm distinguished by the excessive production of red blood cells. The coexistence of these two conditions is exceptionally uncommon and lacks comprehensive documentation. This study presents a case report of a 35-year-old male patient who has been diagnosed with PV for the past 20 years. The patient underwent a radical nephrectomy to remove the renal tumor, and subsequent histopathological analysis confirmed the presence of TC-RCC. Throughout the 6-month follow-up period, the patient exhibited no signs of abnormalities. The rarity of the coexistence of TC-RCC and PV highlights the intricate nature of managing such instances, necessitating a cautious approach to diagnosis and treatment, particularly in surgical interventions. The present study serves as a valuable resource for diagnosing and treating individuals presenting with concurrent renal neoplasms and PV.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241307777"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11650623/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142839854","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tuberostemonine may alleviates proliferation of lung fibroblasts caused by pulmonary fibrosis.","authors":"Amei Tang, Yang Liu, Qian Ding, Gao Huang, Zongge Sha, Changfu Yang, Feng Cao","doi":"10.1177/03946320241274225","DOIUrl":"10.1177/03946320241274225","url":null,"abstract":"<p><strong>Objectives: </strong>Tuberostemonine has several biological activity, the aim of study examined the impact of tuberostemonine on the proliferation of TGF-β1 induced cell model, and its ability to alleviate pulmonary fibrosis stimulated by bleomycin in mice.</p><p><strong>Methods: </strong><i>In vitro</i>, we assessed the effect of tuberostemonine (350, 550 and 750 µM) on the proliferation of cells stimulated by TGF-β1 (10 μg/L), as well as on parameters such as α-SMA vitality, human fibronectin, collagen, and hydroxyproline levels in cells. <i>In vivo</i>, we analyzed inflammation, hydroxyproline, collagen activity and metabolomics in the lungs of mice. Additionally, a comprehensive investigation into the TGF-β/smad signaling pathway was undertaken, targeting lung tissue as well as HFL cells.</p><p><strong>Results: </strong>Within the confines of an in vitro setup, the tuberostemonine manifested a discerned IC50 of 1.9 mM. Furthermore, a significant reduction of over fifty percent was ascertained in the secretion levels of hydroxyproline, fibronectin, collagen type I, collagen type III and α-SMA. <i>In vivo</i>, tuberostemonine obviously improved the respiratory function percentage over 50% of animal model and decreased the hydroxyproline, lung inflammation and collagen deposition. A prominent decline in TGF-β/smad pathway functioning was identified within both the internal and external cellular contexts.</p><p><strong>Conclusions: </strong>Tuberostemonine is considered as a modulator to alleviate fibrosis and may become a new renovation for pulmonary fibrosis.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241274225"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11325469/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141976995","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Activating transcription factor 3 is a new biomarker correlation with renal clear cell carcinoma progression.","authors":"Zhicong Yang, Yongwang Hou, Jingqi Li, Dandan Xu, Zhichao Yang, Xinsheng Wang","doi":"10.1177/03946320241227320","DOIUrl":"10.1177/03946320241227320","url":null,"abstract":"<p><p><b>Background:</b> Clear cell renal cell carcinoma (ccRCC) is the most invasive type of cancer, with a high risk of metastasis and recurrence. Therefore, there is an urgent need to identify novel prognostic predictors and therapeutic targets of ccRCC. Activating transcription factor 3 (ATF3), a tumor oncogene or repressor, has rarely been examined in ccRCC. In the present study, we comprehensively elucidate the prognostic value and potential functions of ATF3 in ccRCC.<b>Methods:</b> Several TCGA-based online databases were used to analyze ATF3 expression in ccRCC and determine ccRCC prognosis. The upstream-binding micro (mi) RNAs of ATF3 and long non-coding (lnc)RNAs were predicted using the StarBase database.<b>Results:</b> Analysis of several TCGA-based online databases showed that ATF3 expression is decreased in ccRCC, suggesting a significant association with the prognosis of patients with ccRCC. Furthermore, we found hsa-miR-221-3p to be potential regulatory miRNA of ATF3 in ccRCC. Prediction and analysis of the upstream lncRNAs indicated that PAXIP1-AS2 and OIP5-AS1 were the most potent upstream lncRNAs of the hsa-miR-221-3p/ATF3 axis in ccRCC. The results of the GO and KEGG analyses implied that ATF3 is likely involved in the regulation of apoptotic signaling in response to endoplasmic reticulum (ER) stress in ccRCC. Correlation analysis revealed a positive relationship between ATF3 expression and ER stress.<b>Conclusions:</b> Our in silico findings highlighted that ATF3 expression was low in ccRCC and negatively correlated with poor prognosis. Furthermore, PAXIP1-AS2 and the OIP5-AS1/hsa-miR-221-3p/ATF3 axis were identified as significant potential regulators of ER stress-mediated apoptosis in ccRCC.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241227320"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10804930/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139513908","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"CXC ligand 13 orchestrates an immunoactive microenvironment and enhances immunotherapy response in head and neck squamous cell carcinoma.","authors":"Xiaohu Lin, Xiaomei Zhao, Yiming Chen, Rong Yang, Zhenlin Dai, Wei Li, Chengzhong Lin, Wei Cao","doi":"10.1177/03946320241227312","DOIUrl":"10.1177/03946320241227312","url":null,"abstract":"<p><p><i>Objectives</i>: This study aims to systematically explore the role of chemokine CXC ligand 13 (CXCL13) in head and neck squamous cell carcinoma (HNSCC). <i>Methods</i>: The Genotype-Tissue Expression (GTEx) and The Cancer Genome Atlas (TCGA) databases provided the RNA-seq data for cancer and normal tissues, respectively. Gene set enrichment analysis was applied to search the cancer hallmarks associated with CXCL13 expression. TIMER2.0 was the main platform used to investigate the immune cell infiltration related to CXCL13. Immunohistochemistry was applied to explore the relationship between CXCL13 and patients' prognosis and the relationship between CXCL13 and tertiary lymphoid structures (TLSs). <i>Results</i>: The expression of CXCL13 was upregulated in most tumors, including HNSCC. The higher expression of CXCL13 was closely related to the positive prognosis of HNSCC. CXCL13 was mainly expressed in B cells and CD8 + T cells, revealing the relationship between its expression and immune activation in the tumor microenvironment. Furthermore, immunohistochemistry and multiple fluorescence staining analysis of HNSCC samples showed a powerful correlation between CXCL13 expression, TLSs formation, and positive prognosis. Finally, CXCL13 significantly increased the response to cancer immunotherapy. <i>Conclusions</i>: CXCL13 may function as a potential biomarker for predicting prognosis and immunotherapy response and associate with TLSs in HNSCC.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241227312"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10807398/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139522193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The global immune-nutrition-inflammation index (GINI) as a robust prognostic factor in glioblastoma patients treated with the standard stupp protocol.","authors":"Erkan Topkan, Nilufer Kilic Durankus, Sukran Senyurek, Duriye Öztürk, Ali Ayberk Besen, Huseyin Mertsoylu, Berrin Pehlivan, Ugur Selek","doi":"10.1177/03946320241284089","DOIUrl":"10.1177/03946320241284089","url":null,"abstract":"<p><strong>Background: </strong>Systemic inflammation can significantly impact gliomas' onset, progression, and prognosis. Glioblastoma multiforme (GBM) represents the glioma subtype characterized by the most profound inflammatory and immunosuppressive states. Consequently, various blood-borne biomarkers have been scrutinized concerning their prognostic value in GBM patients.</p><p><strong>Objective: </strong>We sought to investigate whether the recently introduced Global Immune-Nutrition-Inflammation Index (GINI) holds prognostic significance for GBM patients treated with the standard Stupp protocol.</p><p><strong>Methods: </strong>We retrospectively analyzed the data from a cohort of newly diagnosed GBM patients receiving the standard Stupp regimen using the propensity score-matching methodology. The GINI was computed using the original formula: GINI = [(C-reactive protein × Monocytes × Platelets × Neutrophils) ÷ (Albumin × Lymphocytes)]. We employed receiver operating characteristic (ROC) curve analysis to identify the optimal cutoff values for GINI, which could help distinguish between different survival outcomes. The primary and secondary objectives were the differences in overall survival (OS) and progression-free survival (PFS) between the GINI groups.</p><p><strong>Results: </strong>The optimal GINI cutoff value was 1350. Out of 294 eligible patients, 211 were PSM-matched: GINI<1350 (<i>N</i> = 95) and GINI≥1350 (<i>N</i> = 116). Comparative Kaplan-Meier estimates indicated that the GINI≥1350 patients had substantially worse median PFS (8.0 vs 16.8 months; <i>p</i> < .001) and OS (14.3 vs 22.9 months; <i>p</i> < .001) durations than their GINI<1350 counterparts.</p><p><strong>Conclusion: </strong>High pretreatment GINI values are robustly and independently associated with inferior PFS and OS outcomes in selected GBM patients who receive standard Stupp protocol. These findings suggest that if further confirmed, the novel GINI could serve as a valuable biological marker for the prognostic stratification of GBM patients.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241284089"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11440555/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142298953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Antidiabetic and antioxidant potential of Crocin in high-fat diet plus streptozotocin-induced type-2 diabetic rats.","authors":"Syed Mohammed Basheeruddin Asdaq, Basheerahmed Abdulaziz Mannasaheb, Raha Orfali, Ibrahim Ahmed Shaikh, Ahmed Alshehri, Adel Alghamdi, Meshal Mohammed Alrashdi, Moneer E Almadani, Faisal Mohammad Ali Abdalla","doi":"10.1177/03946320231220178","DOIUrl":"10.1177/03946320231220178","url":null,"abstract":"<p><strong>Objectives: </strong>Crocin, the principal water-soluble active constituent of saffron, possesses numerous pharmacological activities. The present investigation examined the potential antidiabetic and antioxidant characteristics of Crocin in rats with type-2 diabetes by administering it orally and intraperitoneally (i.p.).</p><p><strong>Methods: </strong>After 2 weeks of a high-fat diet, streptozotocin (STZ) (i.p., 40 mg/kg) was administered to male adult rats to induce type-2 diabetes mellitus. Body weight and fasting blood glucose (FBG) were measured on days zero, weeks 1, and 2. At the end of 2 weeks of drug administration in their respective groups, fasting insulin and glucose levels were estimated, and insulin resistance (HOMA-IR) was determined. Intraperitoneal glucose (IPGTT) and insulin tolerance tests (ITT) were carried out. Histopathological investigation and biochemical parameters were estimated in pancreatic tissues.</p><p><strong>Results: </strong>The Crocin (100 mg/kg) treatment has significantly improved body weight, abatement of FBG, fasting insulin, and HOMA-IR. Likewise, Crocin treatment significantly improved the glucose and insulin challenges. We observed a significantly marked elevation in endogenous antioxidant enzymes in Crocin-treated groups. Similarly, Crocin treatment reversed the histopathological changes and restored the normal integrity and function of the pancreas.</p><p><strong>Conclusion: </strong>The overall finding indicates that intraperitoneal administration of Crocin demonstrated better control of glycemic level and body weight. Further, it has improved insulin levels in the serum and potentiated antioxidant properties.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320231220178"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10798082/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139486396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ondansetron or beta-sitosterol antagonizes inflammatory responses in liver, kidney, lung and heart tissues of irradiated arthritic rats model.","authors":"Rokaya E Maarouf, Mohamed K Abdel-Rafei, Noura M Thabet, Khaled S Azab, Laila Rashed, Nermeen M El Bakary","doi":"10.1177/03946320241260635","DOIUrl":"10.1177/03946320241260635","url":null,"abstract":"<p><strong>Background: </strong>Rheumatoid arthritis (RA) is a chronic inflammatory autoimmune disorder mainly affecting joints, yet the systemic inflammation can influence other organs and tissues. The objective of this study was to unravel the ameliorative capability of Ondansetron (O) or β-sitosterol (BS) against inflammatory reactions and oxidative stress that complicates Extra-articular manifestations (EAM) in liver, kidney, lung, and heart of arthritic and arthritic irradiated rats.</p><p><strong>Methods: </strong>This was accomplished by exposing adjuvant-induced arthritis (AIA) rats to successive weekly fractions of total body γ-irradiation (2 Gray (Gy)/fraction once per week for four weeks, up to a total dose of 8 Gy). Arthritic and/or arthritic irradiated rats were either treated with BS (40 mg/kg b.wt. /day, orally) or O (2 mg/kg) was given ip) or were kept untreated as model groups.</p><p><strong>Results: </strong>Body weight changes, paw circumference, oxidative stress indices, inflammatory response biomarkers, expression of Janus kinase-2 (JAK-2), Signal transducer and activator of transcription 3 (STAT3), high mobility group box1 (HMGB1), and nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB), as well as pro- and anti-inflammatory mediators in the target organs, besides histopathological examination of ankle joints and extra-articular tissues. Treatment of arthritic and/or arthritic irradiated rats with BS or O powerfully alleviated changes in body weight gain, paw swelling, oxidative stress, inflammatory reactions, and histopathological degenerative alterations in articular and non-articular tissues.</p><p><strong>Conclusion: </strong>The obtained data imply that BS or O improved the articular and EAM by regulating oxidative and inflammatory indices in arthritic and arthritic irradiated rats.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241260635"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11149447/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141238475","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pre-pandemic antibodies screening against SARS-CoV-2 and virus detection among children diagnosed with eruptive fevers.","authors":"Nouhaila Najimi, Latifa Tajount, Zakia Regragui, Chaimae Remz, Rokaya Ait-Lhaj-Mhand, Chaimae Kadi, Lamiae Belayachi, Fouad Seghrouchni, Nadia Dakka, Rabii Ameziane El Hassani, Elmir Elharti, Hicham Oumzil, Youssef Bakri","doi":"10.1177/03946320241260633","DOIUrl":"10.1177/03946320241260633","url":null,"abstract":"<p><strong>Objectives: </strong>This study aims to assess the seroprevalence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) IgG antibodies against the spike (S) and nucleocapsid (NP) proteins, as well as neutralizing antibodies against the receptor-binding domain (RBD). Additionally, it aims to detect viral RNA of SARS-CoV-2 in pre-pandemic archival pediatric specimens collected before the announcement of the COVID-19 pandemic spread on March 20^th, 2020, in Morocco. The objective is to investigate the existence of pre-pandemic immunity to SARS-CoV-2.</p><p><strong>Methods: </strong>We conducted a cross-sectional study, to analyze IgG antibody levels in a cohort of 106 pre-pandemic pediatric participants. Using an indirect enzyme-linked immunosorbent assay (ELISA), we measured the IgG levels against the S and NP proteins of SARS-CoV-2. Additionally, we staged a competitive ELISA assay to evaluate the neutralizing capability of these antibodies. We used reverse transcription polymerase chain reaction (rRT-PCR) to detect viral NP and ORF1ab genes of SARS-CoV-2 in oropharyngeal swabs. Moreover, we conducted on the same specimens a multiplexed RT-PCR to detect RNA of the most common 27 pathogens involved in lower respiratory tract infections.</p><p><strong>Results: </strong>Among the 106 serum samples, 13% (<i>n</i>n = =14) tested positive for SARS-CoV-2 IgG antibodies using ELISA. Temporal analysis indicated varying IgG positivity levels across 2019. Neutralizing antibodies were found in 21% of the 28 samples analyzed, including two with high inhibition rates (93%). The SARS-CoV-2 RNA was detected using rRT-PCR in 14 samples. None of the samples tested positive for the other 27 pathogens associated with lower respiratory tract infections, using multiplexed RT-PCR.</p><p><strong>Conclusion: </strong>Our study addresses the possibility, that COVID-19 infections occurred in Morocco before the recognized outbreak. On the other hand, some of the cases might reflect cross-reactivity with other coronaviruses or be influenced by previous viral exposures or vaccinations. Understanding these factors is crucial to comprehending pediatric immune responses to newly emerging infectious diseases.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241260633"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11155355/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141248824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) with acquired hemophilia A: A case report and review of literature.","authors":"Cong Mai Thanh, Khuyen Nguyen Thi, Hiep Nguyen Canh, Thuy Nguyen Thi Dieu","doi":"10.1177/03946320241301734","DOIUrl":"10.1177/03946320241301734","url":null,"abstract":"<p><p>We report a case of IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) with acquired hemophilia A (AHA) in a pediatric patient. An 11-year-old female presented with intermittent swelling of the bilateral upper eyelids and neck areas. Clinical examination revealed bilateral swollen upper eyelids with palpable mass lesions in the lacrimal fossa. The submandibular and sublingual glands were enlarged, tender, and movable. Neck ultrasound showed bilateral gland enlargement with irregular hypoechoic parenchyma. Maxillofacial magnetic resonance imaging (MRI) revealed bilateral lacrimal glands enlargement, homogeneous enlarged bilateral submandibular and parotid glands, measuring 4.7 × 3.9 cm. Laboratory investigation showed increased activated partial prothromboplastin time (80.9 s), markedly decreased FVIII activity (0.6%), a high titer of FVIII inhibitor (480 Bethesda units/mL), and a remarkable increase in serum IgG4 level (1005.68 mg/dL). A left submandibular gland biopsy revealed marked lympho-plasmacytic infiltration with scattered eosinophils. Immunohistochemical staining for IgG4 showed numerous IgG4-positive plasma cells (>100 per high-power field), with a ratio of IgG4-positive to IgG-positive cells >40%. The symptoms were markedly relieved following corticosteroid therapy. IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) with acquired hemophilia A (AHA) can also be seen in the pediatric population and should be considered a differential diagnosis in patients with relevant symptoms.</p>","PeriodicalId":48647,"journal":{"name":"International Journal of Immunopathology and Pharmacology","volume":"38 ","pages":"3946320241301734"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11603467/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142740785","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}