2,523 results match your criteria Complement Deficiencies


Recurrent invasive meningococcal infections - quantifying the risk, Germany, 2002 to 2018.

Euro Surveill 2020 Jun;25(25)

Institute for Hygiene and Microbiology, National Reference Laboratory for Meningococci and Haemophilus influenzae, University of Würzburg, Würzburg, Germany.

IntroductionInvasive meningococcal disease (IMD) is a rare condition with a high case fatality rate. While most patients suffer from one single episode in life, there is anecdotal evidence for recurrent infection.AimThe German National Reference Laboratory for Meningococci and (NRZMHi) analysed IMD cases from 2002 to 2018 to retrospectively quantify the risk of recurrent infection. Read More

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http://dx.doi.org/10.2807/1560-7917.ES.2020.25.25.1900565DOI Listing

Integrating metabolomics, genomics and disease pathways in age-related macular degeneration: The EYE-RISK Consortium.

Ophthalmology 2020 Jun 14. Epub 2020 Jun 14.

Department of Ophthalmology, Donders Institute for Brain, Cognition and Behaviour, Radboud university medical center, Nijmegen, the Netherlands. Electronic address:

Objective: In the current study we aimed to identify metabolites associated with age-related macular degeneration (AMD) by performing the largest metabolome association analysis in AMD to date. In addition, we aimed to determine the effect of AMD-associated genetic variants on metabolite levels, and aimed to investigate associations between the identified metabolites and activity of the complement system, one of the main AMD-associated disease pathways.

Design: Case-control assocation analysis of metabolomics data. Read More

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http://dx.doi.org/10.1016/j.ophtha.2020.06.020DOI Listing

Classical and lectin complement pathways and markers of inflammation for investigation of susceptibility to infections among healthy older adults.

Immun Ageing 2020 10;17:18. Epub 2020 Jun 10.

Division of Pulmonary, Allergy, and Critical Care, University of Alabama at Birmingham, Birmingham, AL USA.

Background: There is increasing recognition of the significance of chronic, low-level inflammation in older adults, or "inflammaging." Innate immune responses and host-bacterial interactions are recognized as key factors in inflammaging. Inflammatory cytokine IL-6, and complement protein C1q have been identified as biomarkers for the development of frailty and aging-related diseases. Read More

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http://dx.doi.org/10.1186/s12979-020-00189-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7285792PMC

Training LSTM-neural networks on early warning signals of declining cooperation in simulated repeated public good games.

MethodsX 2020 16;7:100920. Epub 2020 May 16.

Institute of Systems Sciences, Innovation and Sustainability Research, University of Graz, Graz, Austria.

We present results of attempts to expand and enhance the predictive power of Early Warning Signals (EWS) for Critical Transitions (Scheffer et al. 2009) through the deployment of a Long-Short-Term-Memory (LSTM) Neural Network on agent-based simulations of a Repeated Public Good Game, which due to positive feedbacks on experience and social entrainment transits abruptly from majority cooperation to majority defection and back. Our method extension is inspired by several known deficiencies of EWS and by lacking possibilities to consider micro-level interaction in the so far primarily used simulation methods. Read More

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http://dx.doi.org/10.1016/j.mex.2020.100920DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7264060PMC

Effectiveness of Auricular Acupuncture for Insomnia: An Overview of Systematic Reviews.

Evid Based Complement Alternat Med 2020 13;2020:6920902. Epub 2020 May 13.

School of Traditional Chinese Medicine, Southern Medical University, Guangzhou, Guangdong Province 510515, China.

Objectives: The effectiveness of auricular acupuncture (AA) for insomnia is far from uniform. The aim of this overview was to summarize and critically evaluate the evidence from systematic reviews (SRs)/meta-analysis (MAs) and provide an overall verdict about the therapeutic value of AA for insomnia.

Methods: A search of relevant literature for SRs/MAs was performed on major medical databases. Read More

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http://dx.doi.org/10.1155/2020/6920902DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7244956PMC
May 2020
1.880 Impact Factor

Assessment and Interpretation of Vitamin and Trace Element Status in Sick Children: A Position Paper From the European Society for Paediatric Gastroenterology Hepatology, and Nutrition Committee on Nutrition.

J Pediatr Gastroenterol Nutr 2020 Jun;70(6):873-881

Department of Clinical Sciences, Pediatrics, Umeå University, Umeå, Sweden.

Assessment of vitamin and trace element status (VTE) is important in the clinical management of the sick child. In this position paper, we present the various assessment methods available to the clinical practitioner, and critically discuss pitfalls with interpretation of their results. There are 4 main approaches to assess the VTE body status of an individual patient including clinical examination, dietary assessment, and measurement of direct and indirect biomarkers of VTE in biological samples. Read More

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http://dx.doi.org/10.1097/MPG.0000000000002688DOI Listing

Meningococcaemia causing necrotizing cellulitis associated with acquired complement deficiency after gastric bypass surgery: a case report.

BMC Infect Dis 2020 May 20;20(1):361. Epub 2020 May 20.

Department of Intensive Care, Erasme Hospital, Université Libre de Bruxelles, 808 Route de Lennik, Brussels, Belgium.

Background: Neisseria meningitidis has rarely been described as an agent of necrotic soft tissue infection.

Case Presentation: We report a case of a septic shock with necrotizing cellulitis due to Neisseria meningitidis serogroup W, treated by urgent extensive surgical debridement followed by skin grafts. The invasive meningococcal disease occurred together with a complement deficiency, possibly acquired after bypass surgery that took place 1 year before. Read More

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http://dx.doi.org/10.1186/s12879-020-05079-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7240913PMC

Patterns of C1-Inhibitor/Plasma Serine Protease Complexes in Healthy Humans and in Hereditary Angioedema Patients.

Front Immunol 2020 5;11:794. Epub 2020 May 5.

Research Laboratory, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.

C1-inhibitor (C1-INH) is an important regulator of the complement, coagulation, fibrinolytic and contact systems. The quantity of protease/C1-INH complexes in the blood is proportional to the level of the activation of these four cascade-like plasma enzyme systems. Parallel determination of C1-INH-containing activation complexes could be important to understand the regulatory role of C1-INH in diseases such as hereditary angioedema (HAE) due to C1-INH deficiency (C1-INH-HAE). Read More

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http://dx.doi.org/10.3389/fimmu.2020.00794DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7214733PMC

Recurrent macroscopic hematuria in a pediatric patient: is it early to diagnose as having type I hereditary C2 deficiency?

CEN Case Rep 2020 May 5. Epub 2020 May 5.

Department of Pediatric Rheumatology, Faculty of Medicine, Cukurova University, Saricam, Adana, 01331, Turkey.

Hereditary C2 deficiency is the most common early complement deficiency and characterized by recurrent infections and autoimmunity despite most patients are also asymptomatic. Type I hereditary C2 deficiency is caused by a heterozygous deletion in C2 gene resulting in early stop codon and lack of C2 production. Clinical spectrum may vary and pure nephrological involvement without the presence of recurrent infections is scarce in hereditary C2 deficiency. Read More

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http://dx.doi.org/10.1007/s13730-020-00487-5DOI Listing

Evaluating healthcare workers' hand hygiene performance using first-person view video observation in a standardized patient-care scenario.

Am J Infect Control 2020 05;48(5):496-502

Department of Social Psychology, Ulm University, Ulm, Germany.

Background: Monitoring healthcare workers' (HCWs) hand hygiene (HH) performance is recommended for improving compliance. Observer biases challenge data validity, thus supplemental approaches such as video observation are needed to complement monitoring.

Methods: We investigate first-person view (FPV) video observation during simulated standardized patient care handling a catheter in a study with 71 HCWs. Read More

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http://dx.doi.org/10.1016/j.ajic.2019.11.032DOI Listing

[Hereditary angioedema due to C1-esterase inhibitor deficiency : novel approaches].

Rev Med Suisse 2020 Apr;16(689):675-678

Service d'immunologie et allergie, CHUV, 1011 Lausanne.

Hereditary angioedema type 1 and 2 are due to a deficiency in C1--esterase inhibitor. This molecule inhibits the generation of bradykinin, a potent inflammatory mediator that increases vascular permeability. Upon accumulation of bradykinin, patients affected develop painful subcutaneous or submucosal edemas that last for several days. Read More

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Chinese Herbal Medicine Alleviates Thyroidectomy-Induced Cardiopulmonary Exercise Dysfunction in Rats.

Evid Based Complement Alternat Med 2020 20;2020:9415082. Epub 2020 Mar 20.

School of Life Science, National Taiwan Normal University, Taipei, Taiwan.

Hypothyroidism frequently causes cardiopulmonary dysfunction, such as heart failure and respiratory and metabolic deficiencies. This study investigated the effects of Chinese herbal formula B307 on thyroidectomy-induced cardiopulmonary exercise dysfunction in rats. Twenty male rats were equally divided into four groups: negative control with sham treatment, positive control with oral B307 treatment only, thyroidectomy treatment only, and thyroidectomy with B307 posttreatment groups. Read More

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http://dx.doi.org/10.1155/2020/9415082DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7109548PMC

Newborn Screening for Presymptomatic Diagnosis of Complement and Phagocyte Deficiencies.

Front Immunol 2020 17;11:455. Epub 2020 Mar 17.

Division of Clinical Immunology and Transfusion Medicine, Department of Laboratory Medicine, Karolinska University Hospital Huddinge, Stockholm, Sweden.

The clinical outcomes of primary immunodeficiencies (PIDs) are greatly improved by accurate diagnosis early in life. However, it is not common to consider PIDs before the manifestation of severe clinical symptoms. Including PIDs in the nation-wide newborn screening programs will potentially improve survival and provide better disease management and preventive care in PID patients. Read More

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http://dx.doi.org/10.3389/fimmu.2020.00455DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7090021PMC

Complement Deficiencies Result in Surrogate Pathways of Complement Activation in Novel Polygenic Lupus-like Models of Kidney Injury.

J Immunol 2020 May 1;204(10):2627-2640. Epub 2020 Apr 1.

Division of Rheumatology, University of Washington, Seattle, WA 98109;

Lupus nephritis (LN) is a major contributor to morbidity and mortality in lupus patients, but the mechanisms of kidney damage remain unclear. In this study, we introduce, to our knowledge, novel models of LN designed to resemble the polygenic nature of human lupus by embodying three key genetic alterations: the interval leading to anti-chromatin autoantibodies; , leading to defective clearance of apoptotic cells; and either or , leading to low complement levels. We report that proliferative glomerulonephritis arose only in the presence of all three abnormalities (i. Read More

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http://dx.doi.org/10.4049/jimmunol.1901473DOI Listing

Hereditary Angioedema.

N Engl J Med 2020 Mar;382(12):1136-1148

From the Division of Clinical Immunology and Allergy, Icahn School of Medicine at Mount Sinai, New York (P.J.B.); and the Department of Medicine, University of California, San Diego, San Diego (S.C.C.).

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http://dx.doi.org/10.1056/NEJMra1808012DOI Listing

Hereditary Deficiency of the Second Component of Complement: Early Diagnosis and 21-Year Follow-Up of a Family.

Medicina (Kaunas) 2020 Mar 10;56(3). Epub 2020 Mar 10.

Internal Medicine, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Complement deficiencies are rare and often underdiagnosed primary immunodeficiencies that may be associated with invasive bacterial diseases. Serious infections with encapsulated organisms (mainly Streptococcus pneumoniae, but also Neisseria meningitides and Haemophilus influenzae type B) are frequent in patients with a deficiency of the second component of complement (C2), but no data are available on long-term follow-up. This study aimed to evaluate the long-term clinical outcome and the importance of an early diagnosis and subsequent infection prophylaxis in C2 deficiency. Read More

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http://dx.doi.org/10.3390/medicina56030120DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7143546PMC

Short-term prophylaxis in patients with angioedema due to C1-inhibitor deficiency undergoing dental procedures: An observational study.

PLoS One 2020 12;15(3):e0230128. Epub 2020 Mar 12.

Odontoiatric Unit, ASST Fatebenefratelli Sacco, Milan, Italy.

Background: Patients affected by angioedema due to hereditary and acquired C1-inhibitor (C1-INH) deficiency (HAE and AAE, respectively) report trouble accessing dental care, due to the risk of a life-threatening oropharyngeal and laryngeal attack triggered by dental procedures. The aim of this study was to assess the identification of hurdles in receiving dental care, and the effectiveness of short-term prophylaxis (STP) in preventing angioedema attacks. In addition, the study evaluated the impact of dental care in angioedema disease. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0230128PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7067439PMC

Complement Activation in 22q11.2 Deletion Syndrome.

J Clin Immunol 2020 Apr 9;40(3):515-523. Epub 2020 Mar 9.

Center for Rare Diseases, Division of Pediatric and Adolescent Medicine, Oslo University Hospital, Oslo, Norway.

The 22q11.2 deletion syndrome (22q11.2 del), also known as DiGeorge syndrome, is a genetic disorder with an estimated incidence of 1:3000 to 1:6000 births. Read More

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http://dx.doi.org/10.1007/s10875-020-00766-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7142058PMC

Vitamin D and selenium blood levels and acute skin toxicity during radiotherapy for breast cancer.

Complement Ther Med 2020 Mar 31;49:102291. Epub 2019 Dec 31.

Department of Gynaecology and Obstetrics, Ortenau Klinikum Offenburg-Kehl, Ebertplatz 12, 77654 Offenburg, Germany.

Objectives: Vitamin D blood levels have been shown to influence acute chemotherapy toxicities. Therefore, it was investigated whether it is an intrinsic factor influencing acute skin toxicity in patients receiving radiotherapy for breast cancer.

Design/setting: In a total of 107 patients receiving radiotherapy for resected breast cancer, vitamin D and selenium blood levels were determined. Read More

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http://dx.doi.org/10.1016/j.ctim.2019.102291DOI Listing

Site-specific C-terminal dinitrophenylation to reconstitute the antibody Fc functions for nanobodies.

Chem Sci 2019 Oct 28;10(40):9331-9338. Epub 2019 Aug 28.

Key Laboratory of Carbohydrate Chemistry & Biotechnology , Ministry of Education , School of Biotechnology , Jiangnan University , Wuxi , 214122 , China . Email:

Nanobodies are a class of camelid-derived single-domain antibodies that have many potential advantages over conventional antibodies and have been utilized to develop new therapeutic strategies for cancer and other diseases. However, nanobodies lack the Fc region of a conventional antibody, which possesses many functions, , eliciting antibody-dependent cellular cytotoxicity (ADCC) and complement-dependent cytotoxicity (CDC), essential for effective immunotherapy. The small molecular size of nanobodies also leads to poor pharmacokinetics, such as short half-life. Read More

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http://dx.doi.org/10.1039/c9sc03840jDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7006623PMC
October 2019

Oxidative stress in hereditary angioedema caused by C1 inhibitor deficiency: an interesting finding that deserves further studies.

Pol Arch Intern Med 2020 02 27;130(2):73-75. Epub 2020 Feb 27.

Department of Medical Sciences and Public Health, University of Cagliari, Monserrato, Italy

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http://dx.doi.org/10.20452/pamw.15213DOI Listing
February 2020

Data science in neurodegenerative disease: its capabilities, limitations, and perspectives.

Curr Opin Neurol 2020 Apr;33(2):249-254

Department of Bioinformatics, Fraunhofer Institute for Algorithms and Scientific Computing, Sankt Augustin.

Purpose Of Review: With the advancement of computational approaches and abundance of biomedical data, a broad range of neurodegenerative disease models have been developed. In this review, we argue that computational models can be both relevant and useful in neurodegenerative disease research and although the current established models have limitations in clinical practice, artificial intelligence has the potential to overcome deficiencies encountered by these models, which in turn can improve our understanding of disease.

Recent Findings: In recent years, diverse computational approaches have been used to shed light on different aspects of neurodegenerative disease models. Read More

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http://dx.doi.org/10.1097/WCO.0000000000000795DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7077964PMC

Human genetics of meningococcal infections.

Hum Genet 2020 Jun 17;139(6-7):961-980. Epub 2020 Feb 17.

Department of Paediatric Infectious Disease, Faculty of Medicine, Imperial College London, Norfolk Place, London, W2 1PG, UK.

Neisseria meningitidis is a leading cause of bacterial septicaemia and meningitis worldwide. Meningococcal disease is rare but can be life threatening with a tendency to affect children. Many studies have investigated the role of human genetics in predisposition to N. Read More

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http://dx.doi.org/10.1007/s00439-020-02128-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7272491PMC

European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) Complement Guideline: Deficiencies, Diagnosis, and Management.

J Clin Immunol 2020 May 17;40(4):576-591. Epub 2020 Feb 17.

Immunodeficiency Centre for Wales, Cardiff University & University Hospital of Wales, Cardiff, UK.

This guideline aims to describe the complement system and the functions of the constituent pathways, with particular focus on primary immunodeficiencies (PIDs) and their diagnosis and management. The complement system is a crucial part of the innate immune system, with multiple membrane-bound and soluble components. There are three distinct enzymatic cascade pathways within the complement system, the classical, alternative and lectin pathways, which converge with the cleavage of central C3. Read More

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http://dx.doi.org/10.1007/s10875-020-00754-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253377PMC

PARP and PARG inhibitors in cancer treatment.

Authors:
Dea Slade

Genes Dev 2020 03 6;34(5-6):360-394. Epub 2020 Feb 6.

Department of Biochemistry, Max Perutz Labs, Vienna Biocenter (VBC), University of Vienna, 1030 Vienna, Austria.

Oxidative and replication stress underlie genomic instability of cancer cells. Amplifying genomic instability through radiotherapy and chemotherapy has been a powerful but nonselective means of killing cancer cells. Precision medicine has revolutionized cancer therapy by putting forth the concept of selective targeting of cancer cells. Read More

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http://dx.doi.org/10.1101/gad.334516.119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7050487PMC

Contact activation-induced complex formation between complement factor H and coagulation factor XIIa.

J Thromb Haemost 2020 Apr 20;18(4):876-884. Epub 2020 Feb 20.

Unit for Thrombosis Research, Department of Regional Health Research, University of Southern Denmark, Esbjerg, Denmark.

Background: The complement and coagulation systems share an evolutionary origin with many components showing structural homology. Certain components, including complement factor H (FH) and coagulation factor XII (FXII), have separately been shown to have auxiliary activities across the two systems.

Objectives: The interaction between FXII and FH was investigated. Read More

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http://dx.doi.org/10.1111/jth.14742DOI Listing

sgp120 and the contact system in hereditary angioedema: A diagnostic tool in HAE with normal C1 inhibitor.

Mol Immunol 2020 03 16;119:27-34. Epub 2020 Jan 16.

Department of Pediatrics, Duke University Medical Center, Durham, NC, USA.

Mutations in Factor XII, plasminogen gene, angiopoietin-1 gene and kininogen 1 gene have been found in some patients with hereditary angioedema with normal C1 inhibitor (HAE-nl-C1inh), but the underlying disease mechanisms remain unclear. Additionally, there are no accepted biomarkers for this disease. Because the contact system has been implicated in hereditary angioedema with C1 inhibitor deficiency (HAE-C1inh), we studied the fragmentation patterns of serum glycoprotein 120 (sgp120), a protein that is highly susceptible to cleavage by kallikrein, in 31 HAE-C1inh and 13 HAE-nl-C1inh patient plasma samples. Read More

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http://dx.doi.org/10.1016/j.molimm.2020.01.003DOI Listing

[Monoclonal antibodies for monogenic diseases: a 2019 update].

Med Sci (Paris) 2019 Dec 6;35(12):1026-1028. Epub 2020 Jan 6.

CHRU de Tours, Service de Médecine interne;,   - Université de Tours, France.

Monogenic diseases are rare genetic diseases but they are numerous and display a highly variable degree of severity. First uses of monoclonal antibodies to treat monogenic diseases started in the 2000's and many clinical trials are ongoing. Anti-IL-1β therapies have greatly modified the outcome of auto-inflammatory diseases by modulating inflammatory response and reducing the risk of secondary amyloidosis. Read More

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http://dx.doi.org/10.1051/medsci/2019203DOI Listing
December 2019

[Two cases of laryngeal obstruction with hereditary angioedema].

Authors:
J L Li R Hu S Z He

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2019 12;54(12):937-939

Department of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.

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http://dx.doi.org/10.3760/cma.j.issn.1673-0860.2019.12.012DOI Listing
December 2019

Should MASP-2 Deficiency Be Considered a Primary Immunodeficiency? Relevance of the Lectin Pathway.

J Clin Immunol 2020 Jan 11;40(1):203-210. Epub 2019 Dec 11.

Department of Immunology, Hospital Universitario de Gran Canaria Dr. Negrín, Las Palmas de Gran Canaria, Spain.

Mannose-binding lectin (MBL)-associated serine protease-2 (MASP-2) is an indispensable enzyme for the activation of the lectin pathway of complement. Its deficiency is classified as a primary immunodeficiency associated to pyogenic bacterial infections, inflammatory lung disease, and autoimmunity. In Europeans, MASP-2 deficiency, due to homozygosity for c. Read More

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http://dx.doi.org/10.1007/s10875-019-00714-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7223972PMC
January 2020

Anticholinesterase and Antioxidant Potential of Hydromethanolic Extract of (Rhamnaceae) Leaves on Scopolamine-Induced Memory and Cognitive Dysfunctions in Mice.

Evid Based Complement Alternat Med 2019 24;2019:4568401. Epub 2019 Oct 24.

Department of Organic Chemistry, University of Yaoundé I, P.O. Box 812, Yaoundé, Cameroon.

Willd, also known as "buffalo thorn," belongs to the family Rhamnaceae. Its bark and leaves are used in folk medicine for the treatment of various deficiencies related to nociception, inflammation, mood, and depression. Still, there is a lack of scientific data regarding its potential effect on learning and memory process. Read More

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http://dx.doi.org/10.1155/2019/4568401DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6855091PMC
October 2019

Recurrent swelling and pain in the abdomen and joints in a patient with hereditary angioedema and Ehlers-Danlos syndrome.

BMJ Case Rep 2019 Nov 27;12(11). Epub 2019 Nov 27.

College of Osteopathic Medicine, Michigan State University, East Lansing, Michigan, USA.

A 23-year-old woman was referred to the allergy and immunology clinic for recurrent abdominal, cutaneous and joint swelling and pain with a history of mucosal infections since childhood. Her history and clinical findings were suggestive of two rare and complex disorders, hereditary angioedema (HAE) and Ehlers-Danlos syndrome (EDS). Her recurrent episodes of abdominal and joint pain were initially misattributed to more common diagnoses such as esophagitis, depression and chronic pain syndrome. Read More

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http://dx.doi.org/10.1136/bcr-2019-231484DOI Listing
November 2019

Hereditary angioedema with deep vein thrombosis and pulmonary thromboembolism during pregnancy.

Taiwan J Obstet Gynecol 2019 Nov;58(6):895-896

Department of Obstetrics and Gynecology, Juntendo University Faculty of Medicine, Tokyo 113-8421, Japan.

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http://dx.doi.org/10.1016/j.tjog.2019.04.003DOI Listing
November 2019

Qingkailing injection ameliorates cerebral ischemia-reperfusion injury and modulates the AMPK/NLRP3 Inflammasome Signalling pathway.

BMC Complement Altern Med 2019 Nov 20;19(1):320. Epub 2019 Nov 20.

School of Traditional Chinese Medicine Department, Beijing University of Chinese Medicine, 11 Beisanhuandong Road, Chao Yang District, Beijing, 100029, China.

Background: Cerebral ischemia is the second-leading cause of death and the main cause of permanent adult disabilities worldwide. Qingkailing (QKL) injection, a patented Chinese medicine approved by the China Food and Drug Administration, has been widely used in clinical practice to treat cerebral ischemia in China. The NOD-like receptor pyrin 3 (NLRP3) inflammasome is activated in cerebral ischemia and thus, is an effective therapeutic target. Read More

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http://dx.doi.org/10.1186/s12906-019-2703-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6868863PMC
November 2019

Hospitalizations due to Angioedema without Urticaria in a Portuguese Center: Five Year Retrospective Study.

Acta Med Port 2019 Nov 4;32(11):714-720. Epub 2019 Nov 4.

Serviço de Imunoalergologia. Hospital de Santa Maria. Centro Hospitalar Universitário de Lisboa Norte. Lisboa. Clínica Universitária de Imunoalergologia. Faculdade de Medicina. Universidade de Lisboa. Lisboa. Portugal.

Introduction: Hospitalizations due to angioedema are important especially in debilitating or life-threatening situations. The aim of this study was to evaluate the frequency and etiology of angioedema without urticaria in hospital admissions.

Material And Methods: The admissions between 2009 and 2013 in Centro Hospitalar Lisboa Norte with a diagnosis grouped under the ICD9 codes of angioedema were retrospectively analysed. Read More

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http://dx.doi.org/10.20344/amp.11893DOI Listing
November 2019

Lanadelumab Injection Treatment For The Prevention Of Hereditary Angioedema (HAE): Design, Development And Place In Therapy.

Drug Des Devel Ther 2019 22;13:3635-3646. Epub 2019 Oct 22.

Department of Cure Subacute, Istituti Clinici Scientifici Maugeri, IRCCS, Milan, Italy.

Despite the efficacy of the on-demand treatment for the control of acute attacks of Hereditary Angioedema due to C1-Inhibitor Deficiency (C1-INH-HAE), the number and severity of attacks and the impairment in the quality of life of the affected patients have led to the development of a new monoclonal antibody, lanadelumab, directly addressed to the blockage of bradykinin, the principal mediator of vasodilation during angioedema attacks. It is indicated for the prophylactic treatment, it is easy to administer, highly effective and with known limited side effects. The current review summarizes the development of the drug, its clinical background and its perspectives. Read More

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http://dx.doi.org/10.2147/DDDT.S192475DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6815761PMC
March 2020
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Hereditary angioedema in children: a review and update.

Curr Opin Pediatr 2019 12;31(6):863-868

Department of Medicine and Pediatrics at Penn State University, College of Medicine, Hershey, Pennsylvania, USA.

Purpose Of Review: Hereditary angioedema (HAE) most often presents in the first two decades of life. Despite these patients often see multiple doctors and go many years before confirmation of the diagnosis. the impact on quality of life, productivity and risk of anxiety, depression, and posttraumatic stress emphasizes the need for early diagnosis and appropriate treatment. Read More

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http://dx.doi.org/10.1097/MOP.0000000000000832DOI Listing
December 2019
1 Read

Prevention and control of meningococcal disease: Updates from the Global Meningococcal Initiative in Eastern Europe.

J Infect 2019 12 1;79(6):528-541. Epub 2019 Nov 1.

Kazakh National Medical University, Almaty, Kazakhstan. Electronic address:

The Global Meningococcal Initiative (GMI) aims to prevent invasive meningococcal disease (IMD) worldwide through education, research and cooperation. In March 2019, a GMI meeting was held with a multidisciplinary group of experts and representatives from countries within Eastern Europe. Across the countries represented, IMD surveillance is largely in place, with incidence declining in recent decades and now generally at <1 case per 100,000 persons per year. Read More

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http://dx.doi.org/10.1016/j.jinf.2019.10.018DOI Listing
December 2019

Lanadelumab for the treatment of hereditary angioedema.

Expert Opin Biol Ther 2019 12 4;19(12):1233-1245. Epub 2019 Nov 4.

Division of Internal Medicine, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital - Polo Universitario - University of Milan, Milan, Italy.

: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare yet still probably underdiagnosed clinical condition. Recurrent episodes of subcutaneous and sub-mucosal swelling may involve the skin, the gastrointestinal tract or even the upper airways, exposing the patients to the risk of death. With the aim of improving patients' quality of life, the therapeutic scenario has expanded over the years. Read More

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http://dx.doi.org/10.1080/14712598.2019.1685490DOI Listing
December 2019

Hereditary angio-oedema as a rare cause of small-bowel obstruction.

BMJ Case Rep 2019 Oct 25;12(10). Epub 2019 Oct 25.

Surgery, Our Lady of Lourdes Hospital, Drogheda, Ireland.

A 52-year-old man with known hereditary angio-oedema (HAE) presented with a 2-day history of progressive severe abdominal pain, distension, nausea, vomiting and constipation. CT of his abdomen and pelvis showed small-bowel obstruction and ascites. HAE is a rare autosomal dominant disorder caused by a C1 esterase deficiency and involves episodic oedema of subcutaneous and mucosal tissues. Read More

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http://dx.doi.org/10.1136/bcr-2019-231186DOI Listing
October 2019

Oral plasma kallikrein inhibitor BCX7353 for treatment of hereditary angioedema.

Immunotherapy 2019 12 22;11(17):1439-1444. Epub 2019 Oct 22.

Department of Medicine & Pediatrics, College of Medicine, Allergy, Asthma & Immunology, Pennsylvania State University, Hershey, PA 17033, USA.

Hereditary angioedema (HAE) is rare disorder caused by a gene mutation that triggers severe swelling of the skin and upper airway. Treatment options for HAE with deficient and dysfunctional C1-inhibitor are expanding to include small-molecule drugs that inhibit protein interactions in the kallikrein-kinin system. Discovered by BioCryst Pharmaceuticals, BCX7353 is a synthetic, once-daily, small molecule drug that can be taken as an oral capsule to treat HAE attacks and for prophylaxis. Read More

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http://dx.doi.org/10.2217/imt-2019-0128DOI Listing
December 2019
4 Reads

Serial horizontal transfer of vitamin-biosynthetic genes enables the establishment of new nutritional symbionts in aphids' di-symbiotic systems.

ISME J 2020 01 17;14(1):259-273. Epub 2019 Oct 17.

UMR 1062 Centre de Biologie pour la Gestion des Populations, INRA, CIRAD, IRD, Montpellier SupAgro, Univ. Montpellier, Montpellier, France.

Many insects depend on obligate mutualistic bacteria to provide essential nutrients lacking from their diet. Most aphids, whose diet consists of phloem, rely on the bacterial endosymbiont Buchnera aphidicola to supply essential amino acids and B vitamins. However, in some aphid species, provision of these nutrients is partitioned between Buchnera and a younger bacterial partner, whose identity varies across aphid lineages. Read More

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http://dx.doi.org/10.1038/s41396-019-0533-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6908640PMC
January 2020
1 Read

Pediatric hereditary angioedema: what the otolaryngologist should know.

Curr Opin Otolaryngol Head Neck Surg 2019 Dec;27(6):499-503

Department of Otolaryngology-Head and Neck Surgery, West Virginia University School of Medicine, Morgantown, West Virginia, USA.

Purpose Of Review: To review pediatric hereditary angioedema for otolaryngologists, with emphasis on articles within the past 12-18 months.

Recent Findings: Biologic therapies are accepted for adult hereditary angioedema (HAE), but have been studied less for pediatric HAE. Recent literature supports expanded use of biologic agents in pediatrics as acute treatment and prophylaxis. Read More

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http://dx.doi.org/10.1097/MOO.0000000000000589DOI Listing
December 2019
1 Read

Management of patients with hereditary angio-oedema in dental, oral, and maxillofacial surgery: a review.

Authors:
T Uzun

Br J Oral Maxillofac Surg 2019 12 4;57(10):992-997. Epub 2019 Oct 4.

Trabzon Oral and Dental Health Hospital, Department of Oral and Maxillofacial Surgery, DDS, Trabzon, Turkey. Electronic address:

Hereditary angio-oedema (HEA) is an autosomal dominant, life-threatening genetic disorder that is caused by insufficiency or dysfunction of the C1 esterase inhibitor that develops coincidentally with recurrent oedema in the skin, internal organs, and upper respiratory tract. Increased production of bradykinin secondary to increased plasma kallikrein activity is the primary cause of attacks. Dental procedures cause emotional stress and mechanical trauma and may also initiate attacks. Read More

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http://dx.doi.org/10.1016/j.bjoms.2019.09.008DOI Listing
December 2019
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Self-administration of icatibant in acute attacks of Type I hereditary angioedema: A case report and review of hereditary angioedema.

Dermatol Ther 2019 11 28;32(6):e13098. Epub 2019 Oct 28.

Department of Dermatology, University Hospital of Coimbra, Coimbra, Portugal.

Hereditary angioedema (HAE) is a rare group of genetic disease characterized by non-itchy swelling of subcutaneous and submucosal tissues of the extremities, genitalia, gastrointestinal tract, and upper airways, which can be life threatening. Moreover, unpredictability and recurrence of HAE attacks significantly affect patients' quality of life. Short- and long-term prophylaxis is used to decrease the severity and frequency of attacks, but during severe or potentially severe acute episodes, treatment with C1-INH replacement or icatibant is mandatory. Read More

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http://dx.doi.org/10.1111/dth.13098DOI Listing
November 2019
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Lanadelumab: A Review in Hereditary Angioedema.

Authors:
Yahiya Y Syed

Drugs 2019 Nov;79(16):1777-1784

Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Lanadelumab (Takhzyro™), a first-in-class fully human monoclonal antibody against plasma kallikrein, has been approved in several countries, including Australia, Canada, those of the EU, Switzerland and the USA, for the prevention of hereditary angioedema (HAE) attacks in patients aged ≥ 12 years. Subcutaneous lanadelumab significantly reduced HAE attack rates relative to placebo in the pivotal HELP trial. The clinical benefits of lanadelumab were seen regardless of prior long-term prophylaxis use, baseline disease activity, sex or body mass index. Read More

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http://dx.doi.org/10.1007/s40265-019-01206-wDOI Listing
November 2019
3 Reads

[LATEST ADVANCES IN HEREDITARY ANGIOEDEMA (HAE)].

Arerugi 2019;68(8):919-922

Clinical Research Center, National Hospital Organization Kyushu Medical Center.

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http://dx.doi.org/10.15036/arerugi.68.919DOI Listing
October 2019
2 Reads

Novel insights into the treatment of complement-mediated hemolytic anemias.

Ther Adv Hematol 2019 9;10:2040620719873321. Epub 2019 Sep 9.

Department of Medicine, Henri Mondor Hospital, Université Paris-Est, Assistance Publique Hôpitaux de Paris Creteil, Creteil, France.

Complement-mediated hemolytic anemias can either be caused by deficiencies in regulatory complement components or by autoimmune pathogenesis that triggers inappropriate complement activation. In paroxysmal nocturnal hemoglobinuria (PNH) hemolysis is entirely complement-driven. Hemolysis is also thought to be complement-dependent in cold agglutinin disease (CAD) and in paroxysmal cold hemoglobinuria (PCH), whereas warm antibody autoimmune hemolytic anemia (wAIHA) is a partially complement-mediated disorder, depending on the subtype of wAIHA and the extent of complement activation. Read More

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http://journals.sagepub.com/doi/10.1177/2040620719873321
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http://dx.doi.org/10.1177/2040620719873321DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6734604PMC
September 2019
5 Reads

Data mining of reference intervals for coagulation screening tests in adult patients.

Clin Chim Acta 2019 Dec 9;499:108-114. Epub 2019 Sep 9.

Department of Transfusion Medicine and Haemostaseology, University Hospital of Erlangen, Erlangen, Germany.

Background: Appropriate reference intervals are essential when evaluating laboratory test results. However, establishment of reference intervals is challenging, especially for coagulation screening tests, and uncertainty exists regarding age- and sex-dependency of test results. Data mining of laboratory information systems is an emerging approach to reference interval determination, and we evaluated its applicability to coagulation tests. Read More

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http://dx.doi.org/10.1016/j.cca.2019.09.006DOI Listing
December 2019
2 Reads

Structural Basis for Properdin Oligomerization and Convertase Stimulation in the Human Complement System.

Front Immunol 2019 22;10:2007. Epub 2019 Aug 22.

Department of Molecular Biology and Genetics, Center for Structural Biology, Aarhus University, Aarhus, Denmark.

Properdin (FP) is a positive regulator of the immune system stimulating the activity of the proteolytically active C3 convertase C3bBb in the alternative pathway of the complement system. Here we present two crystal structures of FP and two structures of convertase bound FP. A structural core formed by three thrombospondin repeats (TSRs) and a TB domain harbors the convertase binding site in FP that mainly interacts with C3b. Read More

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http://dx.doi.org/10.3389/fimmu.2019.02007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6713926PMC
August 2019
2 Reads