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Hereditary angioedema in children: Review and practical perspective for clinical management.
Pediatr Allergy Immunol
December 2024
French National Reference Center for Angioedema (CREAK), Center of Excellence and Reference (ACARE), Internal Medicine Department, Grenoble Alpes University Hospital, Grenoble, France.
Background: Hereditary angioedema (HAE) in children has specific features and requires multidisciplinary management.
Methods: We performed a literature search and underwent in-depth discussions to provide practical tools for physicians.
Results: HAE is a rare, life-threatening genetic disorder.
Is a Susceptibility Locus for Clarkson Disease (Monoclonal Gammopathy-Associated Systemic Capillary Leak Syndrome).
Arterioscler Thromb Vasc Biol
December 2024
Lung and Vascular Inflammation Section, Laboratory of Allergic Diseases (E.C.C., A.J.A., A.D., K.M.D.), National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health, Bethesda, MD.
Article Synopsis
- Vascular leakage is a serious complication of severe infections like septicemia and malaria, leading to shock and multi-organ dysfunction, with a specific syndrome (MG-CLS) causing recurrent severe episodes triggered by minor infections.
- Researchers identified loss-of-function mutations in the PARP15 gene present in MG-CLS patients, linked to reduced enzyme function and increased vascular leakage.
- The PARP15 enzyme plays a crucial role in protecting blood vessel integrity during inflammation by inhibiting certain pathways, indicating a genetic factor influencing susceptibility to MG-CLS-related vascular issues.
Trends in Dermatological Diagnoses During Covid-19 Pandemic in Nigeria: A Retrospective Study.
West Afr J Med
June 2024
Department of Medicine, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria Phone number: 08037756969; Email:
Article Synopsis
- The study investigates the impact of the COVID-19 pandemic on dermatology outpatient services in Sub-Saharan Africa, particularly at a hospital in Nigeria.
- It compares diagnostic patterns of dermatological conditions before and during the pandemic, revealing a decrease in certain conditions and an increase in others.
- The findings suggest that while weekly clinic visits remained stable, the types of skin disorders diagnosed shifted, highlighting the need for better preparedness for future health crises.
Reevaluating Biota Alteration: Reframing Environmental Influences on Chronic Immune Disorders and Exploring Novel Therapeutic Opportunities.
Yale J Biol Med
June 2024
Department of Pathology & Medical Biology, University Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Environmental mismatches are defined as changes in the environment that induce public health crises. Well known mismatches leading to chronic disease include the availability of technologies that facilitate unhealthy diets and sedentary lifestyles, both factors that adversely affect cardiovascular health. This commentary puts these mismatches in context with biota alteration, an environmental mismatch involving hygiene-related technologies necessary for avoidance of infectious disease.
View Article and Find Full Text PDFExpert consensus on the management of infusion-related reactions (IRRs) in patients with sickle cell disease (SCD) receiving crizanlizumab: a RAND/UCLA modified Delphi panel.
Ann Hematol
June 2024
PHAR (Partnership for Health Analytic Research), 280 S Beverly Dr, Suite 404, Beverly Hills, CA, 90212, USA.
Crizanlizumab, a monoclonal antibody against P-selectin, has been shown to reduce vaso-occlusive crises (VOCs) compared to placebo in patients ≥ 16 years with sickle cell disease (SCD). However, there have been rare reports of patients experiencing severe pain and subsequent complications within 24 hours of crizanlizumab infusions. These events are defined as infusion-related reactions (IRRs).
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