bluuebunny/crossref_metadata_2025_split huggingface dataset:
Malignancy and immune disorders in patients with hereditary angioedema
Authors: Stepaniuk Peter , Kanani Amin 
Published: December 2021

Abstract
Background
Hereditary angioedema (HAE) is an inherited condition manifesting as recurrent angioedema episodes which is caused by deficiency or dysfunction of C1 inhibitor. Although complement dysregulation has historically been shown to be associated with various malignancy and immune disorders, it is currently not known if HAE patients are at an increased risk of developing malignancy or autoimmune conditions.

Case presentation
We reviewed the charts of 49 HAE patients and identified 6 patients who had a co-existing malignancy diagnosis (two with breast cancer, one with melanoma, one with pancreatic cancer, one with renal cancer and one with cervical dysplasia) and 6 patients who had a diagnosis of a co-existing immune disorder (two with rheumatoid arthritis, two with ulcerative colitis, one with chronic urticaria with hypothyroidism and one with Sjogren’s syndrome). Nearly all malignancy cases occurred in older HAE patients (> 50 years) and malignancy was diagnosed before HAE in 3 of the patients.

Conclusions
Our case series identified multiple hereditary angioedema (HAE) patients with co-existing malignancy and immune disorders. Based on these findings, we would advocate that physicians managing HAE patients should maintain a high index of suspicion for these conditions and that in patients with angioedema, C1 inhibitor deficiency and malignancy, a diagnosis of HAE should still be considered in addition to acquired angioedema (AAE).
Acute reversible rhabdomyolysis during direct-acting antiviral hepatitis C virus treatment: a case report
Authors: Qatomah Abdulrahman , Bukhari Majidah , Cupler Edward , Alardati Hosam , Mawardi Mohammad 
Published: December 2021

Abstract
Introduction
Treatment of hepatitis C infection has evolved dramatically since 2011. Previous conventional therapy with interferon and ribavirin used to have a low sustained virological response rate of less than 40%. In the new direct-acting antiviral therapy era, a sustained virological response can be achieved in more than 90% of cases.

Case presentation
We report a rare case of severe reversible acute rhabdomyolysis in a 31-year-old Saudi male patient with very long-chain acyl-coenzyme A dehydrogenase deficiency and chronic hepatitis C infection. The patient was clinically asymptomatic with no signs of decompensated liver disease.
The patient received new direct-acting antiviral agents: sofosbuvir and daclatasvir. Fourteen days after initiation of direct-acting antiviral agents, the patient was found to have asymptomatic rhabdomyolysis. His creatine kinase peaked at 2572 IU/l, and he was treated conservatively; the direct-acting antiviral agents were discontinued and within 7 days, the patient’s creatine kinase levels normalized.

Conclusion
This case highlights possible direct-acting antiviral agent-induced rhabdomyolysis in a patient with very-long-chain acyl-CoA dehydrogenase deficiency, presumably through alteration of mitochondrial membrane potential. Further studies are required to assess the possible impact and associations.
Platypnea–orthodeoxia syndrome in a postoperative patient: a case report
Authors: Pinto Pereira João , Ghaye Benoit , Laterre Pierre-François , Hantson Philippe 
Published: December 2021

Abstract
Background
We report a case of platypnea–orthodeoxia syndrome observed in a complex clinical situation associating a bilateral pleural effusion, lobar pulmonary embolism, and a partial anomalous pulmonary venous return.

Case presentation
A 57-year-old Caucasian woman developed acute dyspnea in the postoperative course of an elective gynecological surgery for advanced stage ovarian cancer. Preoperative evaluation had failed to reveal any respiratory or cardiac problem. After evidence of a low arterial oxygen saturation, blood gas analysis from the central venous line correctly inserted in the right internal jugular vein revealed a higher oxygen saturation than in the arterial compartment. A thoracic computed tomography showed bilateral pleural effusion, lobar pulmonary embolism, and a drainage of a left pulmonary vein into the left innominate vein. This unique combination resulted in an uncommon cause of platypnea–orthodeoxia syndrome.

Conclusion
Often associated with right-to-left shunting, platypnea–orthodeoxia syndrome may be observed in complex clinical conditions with several factors influencing the ventilation/perfusion ratio. The paradoxical finding of a higher oxygen saturation in a central venous line than in an arterial line should prompt the clinician to look at the possibility of partial anomalous pulmonary venous return. No specific treatment is required in asymptomatic adults, except for an echocardiographic follow-up to detect the onset of pulmonary hypertension.
Giant peripheral ossifying fibroma with coincidental squamous cell carcinoma: a case report
Authors: Karube Takeshi , Munakata Kanako , Yamada Yuka , Yasui Yuta , Yajima Shosuke , Horie Nobuyuki , Kawana Hiromasa , Mikami Shuji , Nakagawa Taneaki , Asoda Seiji 
Published: December 2021

Abstract
Background
Peripheral ossifying fibroma is an inflammatory or reactive hyperplasia of the gingiva that is usually small. It is formed by hard tissue in fibrous tissue, and the name “neoplastic lesion” has tended to be used frequently in Europe and America. Clinically, peripheral ossifying fibromas are painless, solitary, exophytic, sessile, or pedunculated and more frequently found in females than in males. To the best of our knowledge, there have been no reports of malignant cases. We herein report the case of giant peripheral ossifying fibroma with squamous cell carcinoma.

Case presentation
The patient was an 83-year-old Japanese woman who visited our hospital with a gingival massive mass. She was referred to us for an examination and treatment because it was difficult to perform tracheal intubation for surgery of sigmoid colon cancer at another hospital. The mass measured 83 × 58 × 35 mm, and it protruded to the extra-oral region from the right maxillary premolar alveolar region. Panoramic X-ray revealed the shadow of the mass in the right maxillary premolar region, which included some hard tissue. Computed tomography showed scattering calcified images in the mass. Magnetic resonance imaging was not performed because she had vertebral artery clips and screws in her forehead. Given the above findings, we performed a biopsy under local anesthesia. However, we were unable to diagnose absolutely whether the dysplastic squamous epithelia were pseudocarcinomatous hyperplasia of the gingiva or well-differentiated squamous cell carcinoma. Therefore, tumor resection was performed under general anesthesia. The histopathological diagnosis was peripheral ossifying fibroma with coincidental squamous cell carcinoma. There have been no signs of recurrence during follow-up as of 2 years after surgery.

Conclusions
The etiology of giant peripheral ossifying fibroma with squamous cell carcinoma is still not definite. Therefore, careful observation is necessary. It needs to be examined by accumulation of more cases in the future. We herein report the case of giant peripheral ossifying fibroma coincidental squamous cell carcinoma.
Recurrent Takotsubo syndrome complicated with ischemic enteritis successfully treated by hydration: a case report
Authors: Todani Shunsuke , Takahashi Mao 
Published: December 2021

Abstract
Background
Takotsubo syndrome is a sudden and an acute form of transient cardiac dysfunction, triggered by mental and physical stress. The treatment for Takotsubo syndrome is not well understood and is incompletely established. Takotsubo syndrome is partly thought to be caused by coronary ischemia under sympathetic nerve activation.

Case presentation
We report the case of an 80-year-old Japanese woman with recurrent Takotsubo syndrome complicated with ischemic enteritis. In this case, abdominal pain and dehydration due to ischemic enteritis is thought to have triggered Takotsubo syndrome. Her life was saved with rapid, adequate intravenous hydration. She was diagnosed with coronary vasospastic angina using coronary angiography on her second admission. This case highlights the potential of adequate intravenous hydration in increasing coronary blood flow. In our case, it should be noted that pulmonary congestion was mild and may have improved Takotsubo syndrome without the use of diuretics.

Conclusion
Adequate hydration must be considered for prompt improvement of cardiac function in Takotsubo syndrome. Replenishment of fluid to increase coronary blood flow, improvement of heart load without exacerbating heart failure, and stabilization of circulation dynamics can help treat patients with Takotsubo syndrome without using diuretics.
Co-occurrence of sickle cell disease and oculocutaneous albinism in a Congolese patient: a case report
Authors: Mbiya Mukinayi Benoît , Mpoyi Kalenda John , Kalombo Kalenda Didier , Disashi Tumba Ghislain , Gulbis Béatrice 
Published: December 2021

AbstractBackgroundSickle cell disease and oculocutaneous albinism are rare autosomal recessive disorders both related to mutations on chromosome 11. The diagnosis of patients suffering from both pathologies is necessary to enable dedicated monitoring of any complications at the ophthalmic and skin level. However, few cases are described in the literature.Case presentationA 14-month-old Congolese male child affected by oculocutaneous albinism, presented with pallor and jaundice. Blood indices revealed severe hemolytic anemia, which led to the diagnosis of sickle cell disease. The patient received a blood transfusion and close follow-up.ConclusionsThe co-inheritance of sickle cell disease and oculocutaneous albinism is a reality in the Democratic Republic of Congo, although it is rarely described. Given the current state of our knowledge, specific surveillance, specifically regarding cutaneous and ophthalmological complications, should be offered in this particular population. To enable this dedicated follow-up, sensitization to screening for sickle cell anemia in albino individuals should be carried out.
Piglet innate immune response to Streptococcus suis colonization is modulated by the virulence of the strain
Authors: Neila-Ibáñez Carlos , Brogaard Louise , Pailler-García Lola , Martínez Jorge , Segalés Joaquim , Segura Mariela , Heegaard Peter M. H. , Aragon Virginia 
Published: December 2021

AbstractStreptococcus suis is a zoonotic pathogen of swine involved in arthritis, polyserositis, and meningitis. Colonization of piglets by S. suis is very common and occurs early in life. The clinical outcome of infection is influenced by the virulence of the S. suis strains and the immunity of the animals. Here, the role of innate immunity was studied in cesarean-derived colostrum-deprived piglets inoculated intranasally with either virulent S. suis strain 10 (S10) or non-virulent S. suis strain T15. Colonization of the inoculated piglets was confirmed at the end of the study by PCR and immunohistochemistry. Fever (≥40.5 °C) was more prevalent in piglets inoculated with S10 compared to T15 at 4 h after inoculation. During the 3 days of monitoring, no other major clinical signs were detected. Accordingly, only small changes in transcription of genes associated with the antibacterial innate immune response were observed at systemic sites, with S10 inducing an earlier response than T15 in blood. Local inflammatory response to the inoculation, evaluated by transcriptional analysis of selected genes in nasal swabs, was more sustained in piglets inoculated with the virulent S10, as demonstrated by transcription of inflammation-related genes, such as IL1B, IL1A, and IRF7. In contrast, most of the gene expression changes in trachea, lungs, and associated lymph nodes were observed in response to the non-virulent T15 strain. Thus, S. suis colonization in the absence of systemic infection induces an innate immune response in piglets that appears to be related to the virulence potential of the colonizing strain.
Delivering the pain: an overview of the type III secretion system with special consideration for aquatic pathogens
Authors: Rahmatelahi Hadis , El-Matbouli Mansour , Menanteau-Ledouble Simon 
Published: December 2021

AbstractGram-negative bacteria are known to subvert eukaryotic cell physiological mechanisms using a wide array of virulence factors, among which the type three-secretion system (T3SS) is often one of the most important. The T3SS constitutes a needle-like apparatus that the bacterium uses to inject a diverse set of effector proteins directly into the cytoplasm of the host cells where they can hamper the host cellular machinery for a variety of purposes. While the structure of the T3SS is somewhat conserved and well described, effector proteins are much more diverse and specific for each pathogen. The T3SS can remodel the cytoskeleton integrity to promote intracellular invasion, as well as silence specific eukaryotic cell signals, notably to hinder or elude the immune response and cause apoptosis. This is also the case in aquatic bacterial pathogens where the T3SS can often play a central role in the establishment of disease, although it remains understudied in several species of important fish pathogens, notably inYersinia ruckeri. In the present review, we summarise what is known of the T3SS, with a special focus on aquatic pathogens and suggest some possible avenues for research including the potential to target the T3SS for the development of new anti-virulence drugs.
Antioxidant supplements promote tumor formation and growth and confer drug resistance in hepatocellular carcinoma by reducing intracellular ROS and induction of TMBIM1
Authors: Zhang Vanilla Xin , Sze Karen Man-Fong , Chan Lo-Kong , Ho Daniel Wai-Hung , Tsui Yu-Man , Chiu Yung-Tuen , Lee Eva , Husain Abdullah , Huang Hongyang , Tian Lu , Wong Carmen Chak-Lui , Ng Irene Oi-Lin 
Published: December 2021

AbstractBackgroundControversy over the benefits of antioxidants supplements in cancers persists for long. Using hepatocellular carcinoma (HCC) as a model, we investigated the effects of exogenous antioxidantsN-acetylcysteine (NAC) and glutathione (GSH) on tumor formation and growth.MethodsMultiple mouse models, including diethylnitrosamine (DEN)-induced and Trp53KO/C-MycOE-induced HCC models, mouse hepatoma cell and human HCC cell xenograft models with subcutaneous or orthotopic injection were used. In vitro assays including ROS assay, colony formation, sphere formation, proliferation, migration and invasion, apoptosis, cell cycle assays were conducted. Western blot was performed for protein expression and RNA-sequencing to identify potential gene targets.ResultsIn these multiple different mouse and cell line models, we observed that NAC and GSH promoted HCC tumor formation and growth, accompanied with significant reduction of intracellular reactive oxygen species (ROS) levels. Moreover, NAC and GSH promoted cancer stemness, and abrogated the tumor-suppressive effects of Sorafenib both in vitro and in vivo. Exogenous supplementation of NAC or GSH reduced the expression of NRF2 and GCLC, suggesting the NRF2/GCLC-related antioxidant production pathway might be desensitized. Using transcriptomic analysis to identify potential gene targets, we found that TMBIM1 was significantly upregulated upon NAC and GSH treatment. Both TCGA and in-house RNA-sequence databases showed that TMBIM1 was overexpressed in HCC tumors. Stable knockdown of TMBIM1 increased the intracellular ROS; it also abolished the promoting effects of the antioxidants in HCC cells. On the other hand, BSO and SSA, inhibitors targeting NAC and GSH metabolism respectively, partially abrogated the pro-oncogenic effects induced by NAC and GSH in vitro and in vivo.ConclusionsOur data implicate that exogenous antioxidants NAC and GSH, by reducing the intracellular ROS levels and inducing TMBIM expression, promoted HCC formation and tumor growth, and counteracted the therapeutic effect of Sorafenib. Our study provides scientific insight regarding the use of exogenous antioxidant supplements in cancers.
Acaricidal and repellent activities of Litsea cubeba (Lour.) oil and 3,7-dimethyl-2,6-octadienal against Haemaphysalis longicornis (Acari: Ixodidae)
Authors: Park Jun-Hwan , Lee Hoi-Seon , Chung Namhyun 
Published: December 2021

AbstractThe bioactivity of the essential oil extracted from Litsea cubeba fruits against tick vectors of severe fever with thrombocytopenia syndrome is unknown. In this study, L. cubeba oil and its main constituents, 3,7-dimethyl-2,6-octadienal and its similar structures, were evaluated for their acaricidal and repellent activities on the unfed nymphs and adults of Haemaphysalis longicornis. L. cubeba oil displayed both acaricidal and repellent activities against both life stages. Among the constituents of L. cubeba oil, only 3,7-dimethyl-2,6-octadienal exhibited both acaricidal and repellent activities against both life stages. In a repellent bioassay, 3,7-dimethyl-2,6-octadienal and L. cubeba oil at dose of 0.08 mg/cm2 provided excellent repellence (100%) against the nymphs and adults for over 60 min post-application. When the acaricidal and repellent activities of 3,7-dimethyl-2,6-octadienal and its similar structures were compared, activities of all tested derivatives were significantly less potent than those of 3,7-dimethyl-2,6-octadienal. The strong acaricidal and repellent activities of 3,7-dimethyl-2,6-octadienal in L. cubeba oil suggests that it is a promising natural candidate for developing new sustainable acaricidal and repellent agents.
Role of the lectin pathway of complement in hematopoietic stem cell transplantation-associated endothelial injury and thrombotic microangiopathy
Authors: Gavriilaki Eleni , Ho Vincent T. , Schwaeble Wilhelm , Dudler Thomas , Daha Mohamed , Fujita Teizo , Jodele Sonata 
Published: December 2021

AbstractHematopoietic stem cell transplantation-associated thrombotic microangiopathy (HSCT-TMA) is a life-threatening syndrome that occurs in adult and pediatric patients after hematopoietic stem cell transplantation. Nonspecific symptoms, heterogeneity within study populations, and variability among current diagnostic criteria contribute to misdiagnosis and underdiagnosis of this syndrome. Hematopoietic stem cell transplantation and associated risk factors precipitate endothelial injury, leading to HSCT-TMA and other endothelial injury syndromes such as hepatic veno-occlusive disease/sinusoidal obstruction syndrome, idiopathic pneumonia syndrome, diffuse alveolar hemorrhage, capillary leak syndrome, and graft-versus-host disease. Endothelial injury can trigger activation of the complement system, promoting inflammation and the development of endothelial injury syndromes, ultimately leading to organ damage and failure. In particular, the lectin pathway of complement is activated by damage-associated molecular patterns (DAMPs) on the surface of injured endothelial cells. Pattern-recognition molecules such as mannose-binding lectin (MBL), collectins, and ficolins—collectively termed lectins—bind to DAMPs on injured host cells, forming activation complexes with MBL-associated serine proteases 1, 2, and 3 (MASP-1, MASP-2, and MASP-3). Activation of the lectin pathway may also trigger the coagulation cascade via MASP-2 cleavage of prothrombin to thrombin. Together, activation of complement and the coagulation cascade lead to a procoagulant state that may result in development of HSCT-TMA. Several complement inhibitors targeting various complement pathways are in clinical trials for the treatment of HSCT-TMA. In this article, we review the role of the complement system in HSCT-TMA pathogenesis, with a focus on the lectin pathway.
Repetitive negative thinking: transdiagnostic correlate and risk factor for mental disorders? A proof-of-concept study in German soldiers before and after deployment to Afghanistan
Authors: Hummel Katrin V. , Trautmann Sebastian , Venz John , Thomas Sarah , Schäfer Judith 
Published: December 2021

Abstract
Background and objectives
Disorder-specific forms of Repetitive Negative Thinking (RNT) are associated with multiple diagnostic categories, indicating a transdiagnostic nature. Few studies examined content-independent RNT processes across groups of diagnosed mental disorders. Moreover, theory describes RNT processes as critically involved in the etiology of mental disorders, empirical evidence however is scarce. We first tested the transdiagnostic nature by examining levels of RNT across groups of internalizing and externalizing mental disorders compared to healthy individuals and explored RNT levels in a comorbid disorder-group. Second, we examined whether RNT predicts incident psychopathology.

Methods
In a sample of German soldiers (n = 425) scheduled for deployment in Afghanistan, we compared RNT levels between diagnosed groups with alcohol use disorders, anxiety disorders and healthy individuals cross-sectionally. Exploratory analyses were conducted comparing a comorbid disorder-group to healthy individuals and to both single-disorder-groups. Longitudinally, we examined the predictive value of pre-deployment RNT levels for incident psychopathology after deployment (n = 167). RNT was measured using the Perseverative Thinking Questionnaire (PTQ), DSM-IV diagnoses were assessed using the standardized Composite International Diagnostic Interview (CIDI).

Results
Cross-sectional comparisons revealed that soldiers with alcohol use disorders and anxiety disorders showed significantly higher degrees of RNT compared to healthy soldiers. RNT levels in those with comorbid disorders were significantly higher compared to healthy soldiers but also compared to both single-disorder-groups. Longitudinal analyses revealed that higher levels of RNT prior to deployment were associated with a higher risk to have any incidental mental disorder after deployment. This however is only attributable to individuals with a PTQ score above a cut-off of 15.

Conclusions
Findings provide evidence for RNT as a transdiagnostic correlate and a vulnerability factor for the development of mental disorders.