OpenMed/multicare-articles huggingface dataset:
Influence of pCP1NetB ancillary genes on the virulence of Clostridium perfringens poultry necrotic enteritis strain CP1
Authors: Hongzhuan Zhou, Dion Lepp, Yanlong Pei, Mei Liu, Xianhua Yin, Rongcai Ma, John F Prescott, Joshua Gong
Journal: Gut Pathog (2017)
Keywords: clostridium perfringens, neloc-1, necrotic enteritis, netb, plasmid-deficient mutant
MeSH terms: Case Reports

Background:
Necrotic enteritis (NE) is an economically important disease of poultry caused by certain Clostridium perfringens type A strains. The NetB toxin plays a critical role in the pathogenesis of NE. We previously demonstrated that netB is located within a 42 kb plasmid-encoded pathogenicity locus (NELoc-1), which also encodes 36 additional genes. Although NetB clearly plays a role in pathogenesis, the involvement of the other NELoc-1 genes has not yet been established. The current study was to provide experimental evidence to confirm the involvement of these genes in NE pathogenesis.
Results:
The present study has characterized a virulent C. perfringens strain (CP1) that has spontaneously lost the NELoc-1-encoding plasmid, pCP1netB. When assessed for cytotoxicity on Leghorn Male Hepatoma (LMH) cells, the culture supernatant of the pCP1netB-deficient CP1 variant (CP1ΔpCP1netB) demonstrated significantly reduced cytotoxicity compared to the wild-type. In addition, CP1ΔpCP1netB was unable to cause intestinal lesions in chickens in a NE disease model. When netB alone was introduced into CP1ΔpCP1netB, in vitro cytotoxicity was restored to the wild-type level; however, it did not completely restore virulence when used to challenge broiler chickens [mean lesion score of 0.71 compared to 3.23 in the wild type control group (n = 14)].
Conclusions:
The results of this study suggest that other genes present in NELoc-1, in addition to netB, are required for full virulence in the chicken challenge model.
Two rare diseases, acute calcific retropharyngeal tendinitis, and crowned dens syndrome, mimicking meningitis: A case report
Authors: Eriko Igami, Jiro Fukae, Kazo Kanazawa, Atsuhito Fuse, Asuka Nakajima, Hiroto Eguchi, Nobutaka Hattori, Yasushi Shimo
Journal: Front Neurol (2022)
Keywords: acute calcific retropharyngeal tendinitis, cervical computed tomography, crowned dens syndrome, fever, neck pain
MeSH terms: Case Reports

We report two rare cases. One involved acute calcific retropharyngeal tendinitis, an inflammatory condition of the longus colli tendon triggered by the deposition of calcium hydroxyapatite crystals. The other involved crowned dens syndrome, caused by pseudogout of the atlantoaxial junction following deposition of calcium pyrophosphate dehydrate or calcium hydroxyapatite. Although these two diseases involve different mechanisms, the common symptoms of neck pain and fever resemble those of meningitis. Accurate diagnosis can thus be difficult without background knowledge of these conditions. Cerebrospinal fluid examination and cervical computed tomography are useful for distinguishing these pathologies from meningitis.
Using R in Regulatory Toxicology
Authors: Felix M Kluxen, Signe M Jensen
Journal: EXCLI J (2022)
Keywords: r, continued education, regulatory toxicology, software tutorial, statistics

Statistical analyses are an essential part of regulatory toxicological evaluations. While projects would be ideally monitored by both toxicologists and statisticians, this is often not possible in practice. Hence, toxicologists should be trained in some common statistical approaches but also need a tool for statistical evaluations. Due to transparency needed in regulatory processes and standard tests that can be evaluated with template approaches, the freely available open-source statistical software R may be suitable. R is a well-established software in the statistical community. The principal input method is via software code, which is both benefit and weakness of the tool. It is increasingly used by regulating authorities globally and can be easily extended by software packages, e.g., for new statistical functions and features. This manuscript outlines how R can be used in regulatory toxicology, allowing toxicologists to perform all regulatory required data evaluations in a single software solution. Practical applications are shown in case studies on simulated and experimental data. The examples cover a) Dunnett testing of treatment groups against a common control and in relation to a biological relevance threshold, assessing the test's assumptions and plotting the results; b) dose-response analysis and benchmark dose derivation for chronic kidney inflammation as a function of Pyridine; and c) graphical/exploratory data analysis of previously published developmental neurotoxicity data for Chlorpyrifos.
A Rare Case of Extensive Cerebral Venous Sinus Thrombosis Complicated by Heparin-Induced Thrombocytopenia
Authors: Noman Ahmed Jang Khan, Ashar Farooqi, Mohamed Alsharedi
Journal: Case Rep Hematol (2022)
MeSH terms: Case Reports

Cerebral venous sinus thrombosis (CVST) is a rare but potentially life-threatening cause of stroke. Several risk factors have been identified including hypercoagulable state, malignancy, use of oral contraceptives, pregnancy, head injury, infection, and prothrombotic states such as heparin-induced thrombocytopenia (HIT). HIT is a prothrombotic state leading to thrombosis in several distinct locations including CVST requiring prompt discontinuation of heparin and initiation of nonheparin anticoagulation to prevent catastrophic consequences. Very rarely, HIT can complicate the ongoing CVST leading to worsening thrombosis and clinical deterioration. We here report an exceedingly rare case of CVST complicated by HIT in a 22-year-old female patient who showed remarkable clinical improvement after discontinuation of heparin and initiation of argatroban.
Bleeding complication of triple therapy of rivaroxaban, prasugrel, and aspirin: a case report and general discussion
Authors: Dane D Gruenebaum, Ahmad Alsarah, Osama Alsara, Heather Laird-Fick
Journal: Case Rep Cardiol (2014)

Hemorrhagic side effects are the bane of oral anticoagulation. Despite careful selection of medications and close monitoring, some adverse events are unavoidable. The available literature about the risks of triple oral anticoagulation therapy versus dual antiplatelet therapy does not address all of the medication combinations currently available. This report describes a patient with atrial fibrillation and recent stent placement who developed severe, recurrent epistaxis on aspirin, prosugrel, and rivaroxaban. We believe this is the first case report of severe bleeding with this combination, and it may help provide insights into the risk for other patients.
Inpatient treatment of community-acquired pneumonias with integrative medicine
Authors: Ulrich Geyer, Klas Diederich, Maria Kusserow, Andreas Laubersheimer, Klaus Kramer
Journal: Evid Based Complement Alternat Med (2013)

Introduction. The aim of the presented observational case series was to evaluate the experience in treating patients with community-acquired pneumonia (CAP) within integrative medicine, particularly anthroposophic medicine in a well-experienced and specialized unit. Patients and Methods. Patients with proven CAP were evaluated (CAP-study group) based on a retrospective chart review. To estimate the severity of pneumonia, the pneumonia severity index (PSI) was applied. Treatment efficacy was evaluated regarding body temperature, CRP level, leukocytes blood count, the need to be treated on ICU, and mortality. Results were compared with the inpatient data of the Pneumonia PORT Validation Cohort. Results. 15/18 patients of the CAP-study group belonged to risk class groups I-III (low and moderate risk), 2 patients to risk class IV, and one patient to risk class V (severe pneumonia). 16/18 patients were treated with anthroposophic medicine only and 2/18 got additionally antibiotic therapy (both of risk class IV). A significant reduction of body temperature, CRP level, and leukocytes blood count has been obtained by applying anthroposophic medicine, while neither complications nor pneumonia-related death occurred. Compared with the control group there was no significant difference in mortality rate, whereby no patient had to be treated on the ICU, but the duration of hospital stay was significantly longer in the presented series. Conclusion. Inpatient treatment of CAP with anthroposophic medicine without the use of antibiotics may achieve reasonable results in selected cases. Additional larger sized prospective controlled trials should further clarify the role of AM in the treatment of CAP.
Case Report: Streptococcus alactolyticus as a Rare Pathogen of Mitral Endocarditis
Authors: Mattia Vinciguerra, Valeria Santamaria, Silvia Romiti, Mizar D'Abramo, Gianmarco Toto, Antonio De Bellis, Gloria Taliani, Giuseppe Sangiorgi, Ernesto Greco
Journal: Front Cardiovasc Med (2021)
Keywords: streptococcus alactolyticus, streptococcus bovis, infective endocarditis, mitral valve, mitral valve regurgitation
MeSH terms: Case Reports

Streptococcus bovis/Streptococcus equinus complex (SBSEC) is a group of non-enterococcal group D Streptococci that colonizes both humans and animals. Due to gastrointestinal disease, they can switch in opportunistic pathogens passing through intestinal mucosal barrier and may cause bacteremia and distant organs damage. Despite infective endocarditis (IE), extra-cardiac manifestations of organs damage include osteoarticular infections, meningitis, and biliary infections among others; moreover, the association with colonic pathological lesions has been largely described. Streptococcus alactolyticus as a species included in SBSEC may share pathophysiological similarities, although it represents an extremely rare cause of distant organ infections, being reported in literature as causative agent of IE in only two other cases. We describe a case of 69-year-old male admitted to our institution due to mild-moderate dyspnea and fever, affected by cervico-brachialgia for 3 weeks. Streptococcus alactolyticus was identified as causative agent of IE on the mitral valve, causing severe regurgitation.
Jejunoileal GIST: A Rare Case of Transient Intussusception and Gastrointestinal Bleeding
Authors: Sara Catarino Santos, Cláudia Leite, Helena Pinho, Carlos Casimiro
Journal: Case Rep Surg (2019)
MeSH terms: Case Reports

Gastrointestinal stromal tumors (GIST) comprised 0,2% of all GI tumors. They are typically asymptomatic, but can manifest with nonspecific GI symptoms, GI bleeding, or intussusception. The authors report a case of a 55-year-old female patient with hematochezia and a palpable mass on the left lower quadrant. Ultrasound revealed possible intussusception. However, CT scan did not show any signs of lesions or intussusception. On reevaluation, the mass was no longer palpable. The patient had recurrent episodes of hematochezia with need of transfusional support. CT enterography revealed a 20-24 mm jejunoileal lesion. A laparotomy was undertaken with small bowel resection containing the lesion. Histological examination confirmed GIST. GIST presentation as transient intussusception and intermittent GI bleeding is rare. This case report emphasizes the rarity of jejunoileal GIST, its clinical details, diagnostic study, and treatment.
Using an experiment among clinical experts to determine the cost and clinical impact of rapid whole exome sequencing in acute pediatric settings
Authors: Nattiya Kapol, Wuttichart Kamolvisit, Lalida Kongkiattikul, Evan Huang-Ku, Namfon Sribundit, Surasit Lochid-Amnuay, Nathapol Samprasit, Thamonwan Dulsamphan, Parntip Juntama, Chotika Suwanpanich, Ponghathai Boonsimma, Vorasuk Shotelersuk, Yot Teerawattananon
Journal: Front Pediatr (2023)
Keywords: acute pediatrics, cost-effectiveness, low- and middle-income countries, next-generation sequencing, rapid whole-exome sequencing

Objective:
Evaluate the cost and clinical impacts of rapid whole-exome sequencing (rWES) for managing pediatric patients with unknown etiologies of critical illnesses through an expert elicitation experiment.
Method:
Physicians in the intervention group (n = 10) could order rWES to complete three real-world case studies, while physicians in the control group (n = 8) could not. Costs and health outcomes between and within groups were compared.
Results:
The cost incurred in the intervention group was consistently higher than the control by 60,000-70,000 THB. Fewer other investigation costs were incurred when rWES could provide a diagnosis. Less cost was incurred when an rWES that could lead to a change in management was ordered earlier. Diagnostic accuracy and the quality of non-pharmaceutical interventions were superior when rWES was available.
Conclusion:
In acute pediatric settings, rWES offered clinical benefits at the average cost of 60,000-70,000 THB. Whether this test is cost-effective warrants further investigations. Several challenges, including cost and ethical concerns for assessing high-cost technology for rare diseases in resource-limited settings, were potentially overcome by our study design using expert elicitation methods.
A review of the clinical spectrum of BRAT1 disorders and case of developmental and epileptic encephalopathy surviving into adulthood
Authors: Ross Fowkes, Menatalla Elwan, Ela Akay, Clinton J Mitchell, Rhys H Thomas, David Lewis-Smith
Journal: Epilepsy Behav Rep (2022)
Keywords: adult, brat1, epilepsy, epileptic encephalopathy, lethal neonatal rigidity and multifocal seizure syndrome
MeSH terms: Case Reports

Pathogenic variants in BRAT1 are associated with a spectrum of clinical syndromes ranging from Lethal Neonatal Rigidity and Multifocal Seizure syndrome (RMFSL) to Neurodevelopmental Disorder with Cerebellar Atrophy and with or without Seizures (NEDCAS). RMFSL is characterized by early-onset multifocal seizures with microcephaly. Death occurs during infancy although a less severe course with later onset seizures and longer survival into childhood has been described. Here, we summarize published cases of BRAT1 disorders and present the case of a 20-year-old man with two heterozygous BRAT1 variants and a relatively later age of seizure onset with survival into adulthood. This case expands the spectrum of disease associated with BRAT1 variants and highlights the utility of genetic testing to identify the cause of developmental and epileptic encephalopathies where clinical heterogeneity within a spectrum of disease exists.
Multiple Faces of Chronic Lymphocytic Leukaemia: A Patient with Renal, Cardiac, and Skeletal Complications
Authors: Zay Myo Htet, Cesar Gomez, Ahsan Ali, Sunil Nair, Mahzuz Karim
Journal: Case Rep Nephrol (2019)
MeSH terms: Case Reports

We describe a patient who had chronic lymphocytic leukaemia (CLL) Binet stage A at presentation with further evidence of disease at multiple sites but who initially required no treatment. However, several years later, her peripheral blood lymphocyte count started to increase, and soon after that she suffered an acute myocardial infarct (in the absence of coronary atheroma) together with proteinuric renal failure due to membranoproliferative glomerulonephritis. Her renal function improved markedly following anti-CLL chemotherapy. We postulate that her cardiac and renal disease were both complications of her CLL. In patients with CLL who develop new clinical signs or symptoms (even if apparently unrelated), consideration should be given as to whether these may be disease complications as this may serve as an indication to commence anti-CLL therapy; close liaison between different specialties is vital.
Reactivation of cutaneous leishmaniasis after renal transplantation: a case report
Authors: Hossein Mortazavi, Mehrnaz Salehi, Kambiz Kamyab
Journal: Case Rep Dermatol Med (2014)

A 45-year-old man with reactivation of previously existing and subsiding cutaneous leishmaniasis on his wrist and lower leg (shin) after renal transplantation was admitted to our dermatology service on March 2008. He presented to us with two huge tumoral and cauliflower-like lesions. Skin smear and histopathology of skin showed leishman bodies and confirmed the diagnosis. After renal transplantation, he received cyclosporine plus prednisolone to induce immunosuppression and reduce the probability of transplant rejection. After immunosuppressive therapy, reactivation of cutaneous leishmaniasis with the above presentation took place. The patient responded to 800 mg/day intravenous sodium stibogluconate for 3 weeks plus local cryotherapy. Systemic plus local therapy along with reducing the doses of immunosuppressive drugs led to improvement of lesions. Reactivation of leishmaniasis after immunosuppression has been rarely reported.
Ogilvie's Syndrome after Cesarean Section: Case Report in Saudi Arabia and Management Approach
Authors: Lamiaa Elsebay, Mariam Ahmed Galal
Journal: Case Rep Obstet Gynecol (2017)
MeSH terms: Case Reports

Background:
Acute colonic pseudoobstruction or Ogilvie's syndrome is a rare entity that is characterized by acute dilatation of the colon without any mechanical obstruction. It is usually associated with medical disease or surgery and rarely occurs spontaneously. If not diagnosed early, Ogilvie's syndrome may cause bowel ischemia and perforation.
Case:
A G7P4+2, 40-year-old woman, who is a known case of gestational diabetes mellitus during her current pregnancy, four previous cesarean sections, two early pregnancy losses at six-week gestation, and hypothyroidism, underwent uncomplicated elective cesarean section, after which she complained of abdominal distention.
Conclusion:
Ogilvie's syndrome is a rare condition yet of interest to obstetricians, midwifery staff, and general surgeons because its early diagnosis and prompt treatment are the keystones to avoid any subsequent fatal complications. This case report reviews the clinical characteristics, diagnostic methods, and management of Ogilvie's syndrome. Moreover, we suggest a management approach to help in early diagnosis and prompt management to improve the outcome of this potentially serious condition.
Galactose-Deficient IgA1 Deposits in Clear Cell Renal Cell Carcinoma-Related Henoch-Schönlein Purpura Nephritis
Authors: Yuhong Zhao, Youngki Kim, Milind Junghare, Viral Vakil, Xuesong Su, Faqian Li, Lihong Bu
Journal: Case Rep Nephrol (2020)
MeSH terms: Case Reports

Recent studies suggest that galactose-deficient IgA1 (Gd-IgA1) plays a role in the pathogenesis of primary IgA nephropathy (IgAN) and Henoch-Schönlein purpura nephritis (HSPN). Furthermore, immunostaining of KM55, an antibody that identifies Gd-IgA1, may be helpful to differentiate primary IgAN and HSPN from secondary causes of glomerular IgA deposition. We report sequential kidney biopsies of a malignancy-associated HSPN, showing intense glomerular mesangial IgA deposition at the initial kidney biopsy and dramatic decrease in disappearance of glomerular deposits after tumor removal. We demonstrate that the glomerular IgA deposition contains Gd-IgA1, detected by immunostaining of KM55, with similar distribution and intensity to IgA. This suggests that renal Gd-IgA1 deposition may play a role in the pathogenesis of malignancy-associated HSPN.
Case report: Cyclosporine A-induced extrapyramidal syndrome following hematopoietic stem cell transplantation
Authors: Fatema Al-Amrani, Abdulhakeem Al Rawas, Eiman Al-Ajmi, Amna Al Futaisi
Journal: Front Neurol (2023)
Keywords: chorea, cyclosporine neurotoxicity, extrapyramidal signs, liver transplantation, neurological adverse events
MeSH terms: Case Reports

Introduction:
Cyclosporine A-associated neurotoxicity has been reported in up to 40% of patients and its wide range of neurological adverse effects have been reported, ranging from mild tremors to fatal leukoencephalopathy. Extrapyramidal (EP) neurotoxicity is a rare manifestation of cyclosporine. Cyclosporine-induced extrapyramidal syndrome remains a rare adverse reaction.
Design/methods:
A database search was performed for studies in patients from all age groups. We found a total of 10 articles reporting EP as an adverse effect of cyclosporine A. A total of 16 patients were found, and a thorough review of these patients was performed. A comparison of patients was performed to highlight common clinical presentations, investigations during the symptomatic phase, and prognosis. In addition, we describe an 8-year-old boy who developed cyclosporine-related extrapyramidal signs on day 60 post-hematopoietic stem cell transplantation for beta-thalassemia.
Conclusion:
Cyclosporine A can induce neurotoxicity resulting in diverse symptoms. Signs of EP are rare manifestations of cyclosporine neurotoxicity and should be considered when evaluating post-transplant recipients of cyclosporine when they are present with any EP symptoms. Discontinuation of cyclosporine results in good recovery in most patients.
Pediatric Pulmonary Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma: A Case Report and Review of the Literature
Authors: Eric X Wei, Roberto F Silva, James D Cotelingam, Rodney E Shackelford
Journal: Case Rep Pathol (2017)
MeSH terms: Case Reports

Non-Hodgkin's lymphoma (NHL) is a common malignancy of childhood; however, a lung primary presentation is an uncommon finding, as is finding an association with the Epstein-Barr virus (EBV). We report the case of a 23-month-old female who developed EBV-associated diffuse large B-cell lymphoma (DLBCL) that was initially thought to be pneumonia. Extensive tissue necrosis, focal angioinvasion, and angiodestruction were observed. She was refractory to various therapy regimens, subsequently developed DLBCL in the central nervous system, and eventually expired. Although EBV+ DLBCL was initially considered to occur predominantly in elderly patients over 50 years of age, it is now increasingly recognized to occur in younger patients with primarily nodal involvement who have overall better prognoses. To our knowledge, this case is the first reported EBV+ DLBCL occurring in a patient below two years of age with lung involvement as the initial clinical presentation.
Vasculitis in a Child With the Hyper-IgM Variant of Ataxia-Telangiectasia
Authors: Anna K Meyer, Mindy Banks, Tibor Nadasdy, Jennifer J Clark, Rui Zheng, Erwin W Gelfand, Jordan K Abbott
Journal: Front Pediatr (2019)
Keywords: cd21lo, ataxia-telangiectasia, granuloma, hyper igm, primary immunodeficiency, tumor necrosis factor, vasculitis
MeSH terms: Case Reports

A subset of patients with Ataxia-Telangiectasia (A-T) have dramatically reduced levels of IgG, IgA, and IgE with retained or elevated IgM levels. Several reports suggest that these A-T patients with a "hyper-IgM phenotype" (HIgM) suffer more clinical immunologic consequences than other A-T patients. The immunopathologic mechanism driving this phenomenon is unknown, making it difficult to predict response to immunomodulatory therapy. We describe an A-T patient with HIgM who underwent tumor necrosis factor (TNF) receptor blockade for cutaneous granuloma and after several months of successful therapy developed non-malignant lymphoproliferation, cytopenia, and increased serum immunoglobulin levels. This process was subsequently followed by an immune-complex-mediated intrarenal small vessel vasculitis that led to renal failure. The vasculitis was successfully treated with rituximab and corticosteroids. This case underscores the importance of HIgM as an unfavorable prognostic indicator in A-T and highlights the complexity of immunomodulatory treatment in this population, and the potential for a successful approach tailored to the immune defect.