Acquired and genetic drivers of C3 and C5 convertase dysregulation in C3 glomerulopathy and immunoglobulin-associated MPGN.

Dysregulation of the alternative pathway of complement plays a central role in the pathophysiology of C3 Glomerulopathy (C3G). Various autoimmune and genetic factors targeting the alternative pathway have been associated to both C3G and primary Immunoglobulin-associated Membranoproliferative Glomerulonephritis (Ig-MPGN), suggesting shared pathophysiological mechanisms. This review highlights the wide range of disease drivers identified that mainly target components or protein complexes of the alternative pathway, both in C3G and Ig-MPGN.

Plant-Based Films for Food Packaging as a Plastic Waste Management Alternative: Potato and Cassava Starch Case.

The escalating environmental impact of plastic packaging waste necessitates sustainable alternatives in food packaging. This study explores starch-based films derived from cassava and potato as viable substitutes, aiming to mitigate plastic pollution and enhance environmental sustainability. Utilizing a casting method, formulations optimized by CCRD were characterized for their physical, physicochemical, and morphological properties.

Benefits of Incorporating Lignin into Starch-Based Films: A Brief Review.

Polysaccharides are an excellent renewable source for developing food-packing materials. It is expected that these packages can be an efficient barrier against oxygen; can reduce lipid peroxidation, and can retain the natural aroma of a food commodity. Starch has tremendous potential to be explored in the preparation of food packaging; however, due to their high hydrophilic nature, packaging films produced from starch possess poor protective moisture barriers and low mechanical properties.

Thrombotic Microangiopathy as an Emerging Complication of Viral Vector-Based Gene Therapy.

Gene therapy has brought tremendous hope for patients with severe life-threatening monogenic diseases. Although studies have shown the efficacy of gene therapy, serious adverse events have also emerged, including thrombotic microangiopathy (TMA) following viral vector-based gene therapy. In this review, we briefly summarize the concept of gene therapy, and the immune response triggered by viral vectors.

Effect of Adding Red Propolis to Edible Biodegradable Protein Films for Coating Grapes: Shelf Life and Sensory Analysis.

Red propolis is an active ingredient of great nutritional interest which offers numerous benefits as an antioxidant and antimicrobial agent. Thus, the objective of this research was to evaluate the application of an edible and antimicrobial gelatine coating containing red propolis to increase the shelf life of grapes. Gelatine films with an addition of 5, 10, 15, 20 and 25% of red propolis extract were produced to evaluate their antimicrobial activity using the disk diffusion test in solid media.

Magnetic Resonance Elastography and Computational Modeling Identify Heterogeneous Lung Biomechanical Properties during Cystic Fibrosis.

The lung is a dynamic mechanical organ and several pulmonary disorders are characterized by heterogeneous changes in the lung's local mechanical properties (i.e. stiffness).

Multipurpose arrowroot starch films with anthocyanin-rich grape pomace extract: Color migration for food simulants and monitoring the freshness of fish meat.

Extraction of anthocyanins from grape pomace, is a way of valuing these abundant by-products with low added value. Its integration into films may allow it to be used in bioactive packaging, which creates new color and solubility properties for food and smart food packaging which tracks the freshness of fish. Films of arrowroot starch added with different concentrations of grape pomace extract (GPE) were flexible to handle, reddish and presented a high content of anthocyanins.

Pregnancy as a susceptible state for thrombotic microangiopathies.

Pregnancy and the postpartum period represent phases of heightened vulnerability to thrombotic microangiopathies (TMAs), as evidenced by distinct patterns of pregnancy-specific TMAs (e.g., preeclampsia, HELLP syndrome), as well as a higher incidence of nonspecific TMAs, such as thrombotic thrombocytopenic purpura or hemolytic uremic syndrome, during pregnancy.

On the gamma radiation response of commercially available 3D printing materials for medical dosimetry.

3D printing technology has rapidly spread for decades, allowing the fabrication of medical implants and human phantoms and revolutionizing healthcare. The objective of this study is to evaluate some radiological properties of commercially available 3D printing materials as potential tissue mimicking materials. Among fifteen materials, we compared their properties with nine human tissues.

Assessment of epidemiology and outcomes of adult patients with kidney-limited thrombotic microangiopathies.

Thrombotic microangiopathies (TMA) are usually associated with hematological features (RH-TMA). The epidemiology of TMA limited to kidneys (RL-TMA) is unclear Therefore, patients with TMA and native kidney biopsies were identified during 2009-2022 in 20 French hospitals and results evaluated. RL-TMA was present in 341/757 (45%) patients and associated with lower creatinine levels (median 184 vs 346 μmol/L) than RH-TMA.

Hot Spot of Complement Factor I Rare Variant p.Ile357Met in Patients With Hemolytic Uremic Syndrome.

Atypical hemolytic uremic syndrome (aHUS) is a rare kidney disease due to a dysregulation of the complement alternative pathway. Complement factor I (CFI) negatively regulates the alternative pathway and CFI gene rare variants have been associated to aHUS with a low disease penetrance. We report 10 unrelated cases of HUS associated to a rare CFI variant, p.

Thrombotic microangiopathies after kidney transplantation in modern era: nosology based on chronology.

Background: Thrombotic microangiopathies (TMAs) are rare but can be severe in kidney transplant. recipients (KTR).

Methods: We analysed the epidemiology of adjudicated TMA in consecutive KTR during the.

Blood pressure and vascular determinants of glomerular filtration rate decline in diabetic kidney disease.

Objective: In patients with type 2 diabetes and diabetic kidney disease (DKD), explore the relationship between estimated glomerular filtration rate decline (eGFR-d) and simultaneously assessed vascular risk markers including office, ambulatory or central blood pressure, pulse pressure, carotid-femoral pulse wave velocity (PWV), carotid intima-media thickness (IMT) and renal resistive indexes (RRI).

Research Design And Methods: At baseline, vascular risk markers were measured in addition to the routine clinical workup. The eGFR-d was based on 2000-2019 creatinine values.

Targeting the Complement Pathway in Kidney Transplantation.

The complement system is paramount in the clearance of pathogens and cell debris, yet is increasingly recognized as a key component in several pathways leading to allograft injury. There is thus a growing interest in new biomarkers to assess complement activation and guide tailored therapies after kidney transplantation (KTx). C5 blockade has revolutionized post-transplant management of atypical hemolytic uremic syndrome, a paradigm of complement-driven disease.

[Management of genetic renal disorders: local experience and importance of the network].

In nephrology, rare disorders are frequently encountered. In children, about 60% of the renal disorders are rare, with congenital abnormalities of the kidney and urinary tract disorders (CAKUT), being highly prevalent. In adults, about 22% of the disorders leading to renal replacement therapies are rare and include glomerulonephritis and genetic disorders.

A Policy Call to Address Rare Kidney Disease in Health Care Plans.

Despite a large number of people globally being affected by rare kidney diseases, research support and health care policy programs usually focus on the management of the broad spectrum of CKD without particular attention to rare causes that would require a targeted approach for proper cure. Hence, specific curative approaches for rare kidney diseases are scarce, and these diseases are not treated optimally, with implications on the patients' health and quality of life, on the cost for the health care system, and society. There is therefore a need for rare kidney diseases and their mechanisms to receive the appropriate scientific, political, and policy attention to develop specific corrective approaches.