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Prognosis of patients with hepatocellular carcinoma treated with immunotherapy - development and validation of the CRAFITY score.

J Hepatol 2021 Oct 11. Epub 2021 Oct 11.

Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Laboratory, Medical University of Vienna, Vienna, Austria; Christian-Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria; CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

Background: Immunotherapy with atezolizumab plus bevacizumab represents the new standard of care in systemic front-line treatment of hepatocellular carcinoma (HCC). Prognostic biomarkers are an unmet need.

Methods: Patients with HCC put on PD-(L)1-based immunotherapy in 6 European centers (training set; n=190) and in 8 European centers (validation set; n=102) were included. Read More

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October 2021

Large brain size is associated with low extra-pair paternity across bird species.

Ecol Evol 2021 Oct 14;11(19):13601-13608. Epub 2021 Sep 14.

College of Life Science Yangtze University Jingzhou China.

Background: Gaining extrapair copulations (EPCs) is a complicated behavior process. The interaction between males and females to procure EPCs may be involved in brain function evolution and lead to a larger brain. Thus, we hypothesized that extrapair paternity (EPP) rate can be predicted by relative brain size in birds. Read More

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October 2021

Ancestral Spectrum Analysis With Population-Specific Variants.

Front Genet 2021 27;12:724638. Epub 2021 Sep 27.

State Key Laboratory of Integrated Services Networks, Xidian University, Xi'an, China.

With the advance of sequencing technology, an increasing number of populations have been sequenced to study the histories of worldwide populations, including their divergence, admixtures, migration, and effective sizes. The variants detected in sequencing studies are largely rare and mostly population specific. Population-specific variants are often recent mutations and are informative for revealing substructures and admixtures in populations; however, computational methods and tools to analyze them are still lacking. Read More

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September 2021

Multiple sites of arterial thrombosis in a 35-year old patient after ChAdOx1 (AstraZeneca) vaccination, requiring emergent femoral and carotid surgical thrombectomy.

Ann Vasc Surg 2021 Oct 10. Epub 2021 Oct 10.

Vascular Surgery Unit, Department of Cardiothoracic and Vascular Surgery, Medical School, University of Crete.

Vaccine Induced Thrombotic Thrombocytopenia (VITT) is a rare complication following ChAdOx1 (AstraZeneca) vaccination. Venous thrombosis in unusual sites such as splachnic or intracranial thrombosis, is the commonest manifestation. We report a 35-year-old male patient who presented with acute left leg ischemia and thrombocytopenia 11-days after vaccination requiring emergent thrombectomy. Read More

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October 2021

Giant Idiopathic Lymphocele 18 Years After Kidney Transplantation, Treated Using Lymphatic Embolization With Lipiodol: Report of a Rare Case.

Exp Clin Transplant 2021 Oct;19(10):1099-1102

>From the Servicio de Trasplante Renal, Hospital de Alta Complejidad en Red "El Cruce" Dr. Néstor Carlos Kirchner, Florencio Varela, Buenos Aires, Argentina.

Kidney transplant is the best therapeutic option for patients with end-stage kidney disease. However, kidney transplant is not exempt from postoperative complications. One of the most frequent urological complications is lymphocele, which can appearin up to 20% of patients. Read More

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October 2021

Introduction of probiotic bacterial consortia promotes plant growth via impacts on the resident rhizosphere microbiome.

Proc Biol Sci 2021 Oct 13;288(1960):20211396. Epub 2021 Oct 13.

Jiangsu Provincial Key Lab for Organic Solid Waste Utilization, Key Lab of Plant immunity, National Engineering Research Center for Organic-based Fertilizers, Jiangsu Collaborative Innovation Center for Solid Organic Waste Resource Utilization, Nanjing Agricultural University, Weigang 1, Nanjing 210095, People's Republic of China.

Plant growth depends on a range of functions provided by their associated rhizosphere microbiome, including nutrient mineralization, hormone co-regulation and pathogen suppression. Improving the ability of plant-associated microbiomes to deliver these functions is thus important for developing robust and sustainable crop production. However, it is yet unclear how beneficial effects of probiotic microbial inoculants can be optimized and how their effects are mediated. Read More

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October 2021

Deep Learning-Based Analysis of Face Images as a Screening Tool for Genetic Syndromes.

Sensors (Basel) 2021 Oct 2;21(19). Epub 2021 Oct 2.

Multimedia Department, Polish-Japanese Academy of Information Technology, 02-008 Warsaw, Poland.

Approximately 4% of the world's population suffers from rare diseases. A vast majority of these disorders have a genetic background. The number of genes that have been linked to human diseases is constantly growing, but there are still genetic syndromes that remain to be discovered. Read More

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October 2021

Development of A Surgical Treatment Algorithm for Breast Reconstruction in Poland Syndrome Patients Considering Severity, Sex, and BMI.

J Clin Med 2021 Sep 29;10(19). Epub 2021 Sep 29.

Department of Plastic and Reconstructive and Aesthetic Surgery, Marienhospital Stuttgart, Teaching Hospital of the Eberhard Karls University, 70199 Tuebingen, Germany.

Introduction: Poland syndrome is a rare, challenging combination of chest wall and breast deformities for reconstructive surgeons and selecting the treatment can prove difficult. This study aims to help surgeons in choosing the best viable option for treatment by sharing our institutional experience and proposing a guiding algorithm.

Methods: A retrospective analysis of all patients with Poland syndrome undergoing treatment for breast and chest wall deformities at a single institution between December 2011 and May 2020 was performed. Read More

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September 2021

Epidemiologic, Postmortem Computed Tomography-Morphologic and Biomechanical Analysis of the Effects of Non-Invasive External Pelvic Stabilizers in Genuine Unstable Pelvic Injuries.

J Clin Med 2021 Sep 24;10(19). Epub 2021 Sep 24.

Institute of Legal Medicine and Forensic Sciences, Charité-Universiätsmedizin, 10117 Berlin, Germany.

Unstable pelvic injuries are rare (3-8% of all fractures) but are associated with a mortality of up to 30%. An effective way to treat venous and cancellous sources of bleeding prehospital is to reduce intrapelvic volume with external noninvasive pelvic stabilizers. Scientifically reliable data regarding pelvic volume reduction and applicable pressure are lacking. Read More

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September 2021

Characteristics of Retinitis Pigmentosa Associated with and Comparison with in Patients from a Multicentric Usher Syndrome Study Treatrush.

Int J Mol Sci 2021 Sep 26;22(19). Epub 2021 Sep 26.

Institut de l'audition, Institut Pasteur, Université de Paris, INSERM, 75012 Paris, France.

In contrast to , variants in are a minor cause of Usher syndrome type 2, and the associated phenotype is less known. The purpose of the study was to characterize the retinal phenotype of 18 patients (9 male, 9 female; median age 52 years) and compare it with that of 204 patients (111 male, 93 female; median age 43 years) in terms of nyctalopia onset, best corrected visual acuity (BCVA), fundus autofluorescence (FAF), and optical coherence tomography (OCT) features. There was no statistical difference in the median age at onset (30 and 18 years; Mann-Whitney U test, = 0. Read More

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September 2021

Paucicellular Fibroma of the Ascending Aorta.

Aorta (Stamford) 2021 Oct 12. Epub 2021 Oct 12.

Department of Cardiovascular and Thoracic Surgery, Zucker School of Medicine at Hofstra/Northwell, Manhasset, New York.

Primary tumors of the aorta are extremely rare. To the best of our knowledge, herein, we present the first case in the literature of a paucicellular fibroma originating from the aortic wall. Read More

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October 2021

Jael's syndrome: Case report and literature review.

Int J Surg Case Rep 2021 Oct 7;88:106484. Epub 2021 Oct 7.

Department of Otorhinolaryngology-Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.

Introduction And Importance: Jael's syndrome, an intentional injury caused by a knife in the face or skull is a rare encounter in clinical and forensic practice, rarely involving an impacted knife. Clinical and radiographic diagnosis is essential to identify severity of injury and location of the retained knife. To the best of our knowledge, this is the first reported novel case of Jael's syndrome in Tanzania. Read More

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October 2021

Surgical management of duodenal web in an adult presenting with melaena; Case report and literature review.

Int J Surg Case Rep 2021 Oct 6;88:106488. Epub 2021 Oct 6.

Department of General Surgery, Waikato District Health Board, Hamilton, New Zealand.

Introduction: Duodenal web is a rare pathology presenting infrequently in adults. Diagnosis is challenging and definitive management is commonly delayed. We present a case of a patient with a late diagnosis of duodenal web, who underwent laparotomy, intraoperative gastroscopy and surgical removal of the web performed by two general surgeons. Read More

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October 2021

Transcriptomics Reveals the Putative Mycoparasitic Strategy of the Mushroom on Species of the Mushroom Genus .

mSystems 2021 Oct 12:e0054421. Epub 2021 Oct 12.

Department of Plant Pathology, University of Nebraska, Lincoln, Nebraska, USA.

During mycoparasitism, a fungus-the host-is parasitized by another fungus-the mycoparasite. The genetic underpinnings of these relationships have been best characterized in ascomycete fungi. However, within basidiomycete fungi, there are rare instances of mushroom-forming species parasitizing the reproductive structures, or sporocarps, of other mushroom-forming species, which have been rarely investigated on a genetic level. Read More

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October 2021

Mechanical ileus and mesenteric ischemia as complications of MiniMizer-gastric-ring after laparoscopic banded Roux-en-Y-gastric bypass: A case report.

Int J Surg Case Rep 2021 Oct 7;87:106476. Epub 2021 Oct 7.

Department of Bariatric and Metabolic Surgery, Helios Klinikum Berlin-Buch, Germany.

Introduction: Banded laparoscopic Roux-en-Y-gastric bypass (B-LRYGB) is a surgical technique that involves reinforcing the restriction by placing a silicone ring 2 cm above the gastrojejunostomy to prevent pouch dilation, thereby maintaining the achieved weight loss and preventing weight regain. Gastrojejunostomy stenosis, erosions, and ring-migration (slippage) are well-known complications in patients undergoing banded laparoscopic procedures. We believe that our study makes a significant contribution to the literature because, to the best of our knowledge, cranial slippage (herniation) of the alimentary limb through a non-slipped MiniMizer gastric ring after B-LRYGB as well as mesenteric ischemia because of ventral slippage have not been described before in the published literature. Read More

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October 2021

Behçet's disease: a case report about a rare cause of intra-cardiac mass.

Eur Heart J Case Rep 2021 Oct 8;5(10):ytab299. Epub 2021 Oct 8.

Cardiology Department, Hospital Garcia de Orta EPE, Av. Prof. Torrado da Silva, 2801-951 Almada, Portugal.

Background: Intra-cardiac masses are always a challenging diagnosis, especially when it involves the right side of the heart. There are multiples aetiologies that can be responsible for these masses, namely thrombosis, neoplasm, or vegetations. Occasionally, these may be related to an autoimmune process not yet diagnosed. Read More

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October 2021

An Italian Disease-Based Registry of Axial and Peripheral Spondyloarthritis: The SIRENA Study.

Front Med (Lausanne) 2021 22;8:711875. Epub 2021 Sep 22.

S.C. Reumatologia ASST Centro Specialistico Ortopedico Traumatologico Gaetano Pini - CTO, Milan, Italy.

Data about the clinical presentation and management of early and mild spondyloarthritis (SpA) are limited. The objective of this study was to describe the baseline characteristics of disease-modifying antirheumatic drug (DMARD)-naïve patients with axial or peripheral SpA. The Spondyloarthritis Italian Registry: Evidence from a National Pathway (SIRENA) study is an ongoing, Italian, multicenter, prospective registry of patients with a first or newly confirmed diagnosis of SpA according to the Assessment of SpondyloArthritis International Society (ASAS) criteria. Read More

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September 2021

Features of primary pancreatic lymphoma: A bi-institutional review with an emphasis on typical and atypical imaging features.

World J Clin Oncol 2021 Sep;12(9):823-832

Department of Diagnostic Imaging, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.

Background: Primary pancreatic lymphoma (PPL) is a rare neoplasm. Being able to distinguish it from other pancreatic malignancies such as pancreatic ductal adenocarcinoma (PDAC) is important for appropriate management. Unlike PDAC, PPL is highly sensitive to chemotherapy and usually does not require surgery. Read More

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September 2021

Development of a risk score to guide targeted hepatitis C testing among human immunodeficiency virus patients in Cambodia.

World J Hepatol 2021 Sep;13(9):1167-1180

Department of Clinical Sciences, Institute of Tropical Medicine Antwerp, Antwerp 2000, Belgium.

Background: The World Health Organization recommends testing all human immunodeficiency virus (HIV) patients for hepatitis C virus (HCV). In resource-constrained contexts with low-to-intermediate HCV prevalence among HIV patients, as in Cambodia, targeted testing is, in the short-term, potentially more feasible and cost-effective.

Aim: To develop a clinical prediction score (CPS) to risk-stratify HIV patients for HCV coinfection (HCV RNA detected), and derive a decision rule to guide prioritization of HCV testing in settings where 'testing all' is not feasible or unaffordable in the short term. Read More

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September 2021

Assessment of Imputation Quality: Comparison of Phasing and Imputation Algorithms in Real Data.

Front Genet 2021 22;12:724037. Epub 2021 Sep 22.

Institut für Medizinische Biometrie und Statistik, Universität zu Lübeck, Universitätsklinikum Schleswig-Holstein, Lübeck, Germany.

Despite the widespread use of genotype imputation tools and the availability of different approaches, late developments of currently used programs have not been compared comprehensively. We therefore assessed the performance of 35 combinations of phasing and imputation programs, including versions of SHAPEIT, Eagle, Beagle, minimac, PBWT, and IMPUTE, for genetic imputation of completely missing SNPs with a HRC reference panel regarding quality and speed. We used a data set comprising 1,149 fully sequenced individuals from the German population, subsetting the SNPs to approximate the Illumina Infinium-Omni5 array. Read More

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September 2021

Identification and Antibiotic Profiling of , an Underestimated Human Pathogen.

Front Microbiol 2021 22;12:712775. Epub 2021 Sep 22.

Institute of Medical Microbiology and Virology, University Hospital Carl Gustav Carus, Dresden, Germany.

In the past 12 years, several case reports have clearly demonstrated that is capable of causing sepsis and bacteremia in humans. However, since most clinicians are not familiar with this species, little is known about its pathogenicity and treatment options while it is as rare but underestimated human pathogen. Therefore, a larger strain collection is required so that methods can be identified that are most suitable to obtain rapid and reliable identification. Read More

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September 2021

Efflorescence of acquired epidermodysplasia verruciformis due to immune reconstitution inflammatory syndrome-like mechanisms following anti-HPV vaccination in a HIV-positive patient.

Int J STD AIDS 2021 Oct 11:9564624211048343. Epub 2021 Oct 11.

Department of Medical Sciences, Dermatology Clinic and Surgical Pathology Section, 9314University of Turin, Torino, Italy.

Epidermodysplasia verruciformis (EV) is a rare cutaneous pre-cancerous condition characterized by presence of flat, scaly macules, verruca-like papillomatous papules, seborrheic keratosis-like lesions, and pink-red pityriasis versicolor-like macules. The disease is caused by abnormal susceptibility to certain specific beta-HPV subtypes, most commonly 5 and 8. Classic EV is genetically determined, but in immunocompromised individuals, an acquired form can occur. Read More

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October 2021

A rare emphysematous splenic infection caused by diabetes mellitus: a case report.

Ann Palliat Med 2021 Sep;10(9):10091-10094

Department of General Surgery, The Second Affiliated Hospital of Air Force Medical University, Xi'an, China.

Emphysematous splenic infection is a rare disease. In this case, a 33-year-old woman presented to the emergency department with a 10-day history of left-upper-quadrant abdominal pain and intermittent fever. She positively denied any previous history of illness or trauma. Read More

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September 2021

Considerations for transition from subcutaneous to oral prophylaxis in the treatment of hereditary angioedema.

Allergy Asthma Clin Immunol 2021 Oct 9;17(1):100. Epub 2021 Oct 9.

Marycliff Clinical Research, 820 S McClellan St #414, Spokane, WA, 99204, USA.

Background: Hereditary angioedema (HAE) is a rare genetic disorder characterized by unpredictable localized episodes of edema, which is frequently managed with long-term prophylactic medications. Until recently, long-term prophylaxis has predominantly required regular intravenous or subcutaneous administration, however the recent approval of berotralstat (Orladeyo™) offers an orally administered prophylactic which may be associated with a lower burden of treatment compared to injectable options for some patients.

Case Presentation: This report describes four participants in the APeX-S trial who transitioned from subcutaneously administered lanadelumab (Takhzyro) to daily oral berotralstat for long-term HAE prophylaxis. Read More

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October 2021

Acute Promyelocytic Leukemia: Update on Risk Stratification and Treatment Practices.

Cancer Treat Res 2021 ;181:45-55

City of Hope Medical Center, 1500 E Duarte Rd., Duarte, CA, 91010, USA.

Acute promyelocytic leukemia (APL) is a rare but highly curable form of acute myeloid leukemia (AML) whose genetic hallmark is the balanced reciprocal translocation t(15;17)(q22;q12) which fuses the promyelocytic leukemia (PML) and retinoic acid receptor alpha (RARα) genes (Wang and Chen in Blood 111:2505-2515, 2008; Lallem and-Breitenbach et al. in J Exp Med 189:1043-1052, 1999). It is a rare disease and accounts for 5-10% of adult AML with an estimated incidence of 0. Read More

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January 2021

Efficacy and safety of gemcitabine plus doxorubicin in patients with renal medullary carcinoma.

Clin Genitourin Cancer 2021 Sep 15. Epub 2021 Sep 15.

Department of Genitourinary Medical Oncology, Division of Cancer Medicine, University of Texas MD Anderson Cancer Center, Houston, TX; Department of Translational Molecular Pathology, Division of Pathology and Laboratory Medicine, University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address:

Introduction: Renal medullary carcinoma (RMC) is a rare and lethal renal cell carcinoma characterized by the loss of tumor suppressor SMARCB1. Molecular profiling studies have suggested that RMC cells may be vulnerable to therapies that generate DNA damage, such as the combination of the nucleoside analog gemcitabine, and topoisomerase inhibitor doxorubicin.

Patients And Methods: We retrospectively analyzed the records of patients with RMC treated with gemcitabine plus doxorubicin at our institution between January 2005 and September 2020. Read More

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September 2021

Phase 2 study of anastrozole in rare cohorts of patients with estrogen receptor/progesterone receptor positive leiomyosarcomas and carcinosarcomas of the uterine corpus: The PARAGON trial (ANZGOG 0903).

Gynecol Oncol 2021 Oct 5. Epub 2021 Oct 5.

Royal Women's Hospital, Melbourne, VIC, Australia.

Background: Aromatase inhibitors have been used empirically to treat a subset of patients with hormone receptor positive uterine leiomyosarcomas(LMS) and carcinosarcomas (UCS) mainly supported by retrospective data. We evaluated the activity of anastrozole in two rare cohorts; patients with recurrent/metastatic LMS and UCS enrolled in PARAGON, a basket trial of anastrozole in estrogen receptor (ER+)/progesterone receptor positive (PR+) gynecological cancers.

Method: An investigator-initiated, single-arm, prospective open-label trial of anastrozole 1 mg/day in patients with ER &/or PR + ve LMS or UCS with measurable disease, treated until progression or unacceptable toxicity. Read More

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October 2021

What's old is best again Is anterior plating best for fixation of type-C pelvic fractures? A systematic review and meta-analysis.

Injury 2021 Sep 9. Epub 2021 Sep 9.

The Trauma and Orthopaedic Research Unit, Department of Surgery, The Canberra Hospital, Garran, Australian Capital Territory, Australia; The Australian National University, Canberra, Australian Capital Territory, Australia.

Background: Type-C pelvic fractures are a rare but potentially fatal injury that often leads to poor outcomes, despite surgical fixation. Many fixation methods are used but the optimal method remains contentious, with failure and complications common. This study compared outcomes for each fixation method. Read More

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September 2021

The Discovery and Biosynthesis of Nicotinic Myxochelins from an sp. SDU34.

J Nat Prod 2021 Oct 8. Epub 2021 Oct 8.

State Key Laboratory of Microbial Technology, Institute of Microbial Technology, Shandong University, Qingdao 266237, People's Republic of China.

Myxobacteria are a prolific source of structurally diverse natural products, and one of the best-studied myxobacterial products is the siderophore myxochelin. Herein, we report two new compounds, myxochelins N () and O (), that are nicotinic paralogs of myxochelin A, from the terrestrial myxobacterium sp. SDU34; is functionalized with a rare 2-oxazolidinone. Read More

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October 2021