Martina Cacciapuoti, Giovanni Bertoldi, Ilaria Caputo, Giulia Driussi, Gianni Carraro, Lorenzo A Calò
Fabry disease is a rare X-linked disease characterized by deficient expression and activity of alpha-galactosidase A with consequent lysosomal accumulation of glycosphingolipids, particularly globotriaosylceramide in various organs. Currently, enzyme replacement therapy with recombinant human α-galactosidase is the cornerstone of the treatment of Fabry patients, although in the long term enzyme replacement therapy fails to halt disease progression, in particular in case of late diagnosis. This suggests that the adverse outcomes cannot be justified by the lysosomal accumulation of glycosphingolipids alone, and that additional therapies targeted at further pathophysiologic mechanisms might contribute to halting the progression of cardiac, cerebrovascular and kidney disease in Fabry patients...
June 15, 2024: Journal of Nephrology
Haley N Johnson, Lalita Prasad-Reddy
Chronic kidney disease (CKD) affects approximately 14% of adults in the United States and is present in at least 10% of the population worldwide. Blood glucose and blood pressure control are imperative to adequately manage CKD as they are the only primary prevention measures for the condition. Recent changes in CKD evaluation and medication therapies that modify disease progression and aid in managing complications such as anemia of CKD have emerged, including a newly approved mineralocorticoid receptor antagonist and hypoxia-inducible factor-prolyl hydroxylase inhibitor, respectively...
June 14, 2024: Journal of Pharmacy Practice
Seiya Inoue, Atsushi Kondo, Yuta Inoki, Yuta Ichikawa, Yu Tanaka, Chika Ueda, Hideaki Kitakado, Ryota Suzuki, Eri Okada, Nana Sakakibara, Tomoko Horinouchi, Kandai Nozu
BACKGROUND: Wilms tumor 1 (WT1; NM_024426) causes Denys-Drash syndrome, Frasier syndrome, or isolated focal segmental glomerulosclerosis. Several WT1 intron variants are pathogenic; however, the pathogenicity of some variants remains undefined. Whether a candidate variant detected in a patient is pathogenic is very important for determining the therapeutic options for the patient. METHODS: In this study, we evaluated the pathogenicity of WT1 gene intron variants with undetermined pathogenicity by comparing their splicing patterns with those of the wild-type using an in vitro splicing assay using minigenes...
June 14, 2024: Clinical and Experimental Nephrology
Sami Alobaidi
This study aimed to explore chronic kidney disease (CKD)-related knowledge and its predictors among non-dialysis patients with CKD in the Kingdom of Saudi Arabia (KSA). It was a cross-sectional survey conducted at 2 nephrology centers in KSA. Data were gathered using a survey questionnaire that included sociodemographic information and enquiries about CKD. The questionnaire used to explore CKD knowledge consisted of 24 questions with 3 multiple-choice answers for each question: "True," "False," and "I don't know...
June 14, 2024: Medicine (Baltimore)
Daniel A Muruve
No abstract text is available yet for this article.
June 14, 2024: Journal of the American Society of Nephrology: JASN
Raphael Wilson, Neije Mukherjee-Roy, Jyothsna Gattineni
Phosphate is essential for numerous biological processes, and serum levels are tightly regulated to accomplish these functions. The regulation of serum phosphate in a narrow physiological range is a well-orchestrated process and involves the gastrointestinal (GI) tract, bone, kidneys, and several hormones, namely, parathyroid hormone, fibroblast growth factor 23 (FGF23), and 1,25-dihydroxyvitamin D (1,25 Vitamin D). Although primarily synthesized in the bone, FGF23, an endocrine FGF, acts on the kidney to regulate phosphate and Vitamin D homeostasis by causing phosphaturia and reduced levels of 1,25 Vitamin D...
June 14, 2024: Pediatric Nephrology
Cyril Garrouste, Marine Freist, Mathilde Prezelin-Reydit, Antoine Bouquegneau, Thomas Fournier, Betoul Schvartz, Antoine Thierry, Virginie Paumier Sanson, Valentin Mayet, Bruno Pereira, Christophe Mariat
The management of patients with kidney transplant failure (KTF) remains a complex process involving multiple stakeholders. A working group of the Transplantation Commission of the French-speaking Society of Nephrology, Dialysis and Transplantation (SFNDT) conducted a survey on the management of immunosuppressants (IS) after KTF among nephrologists at transplant centres and general nephrologists in France, Switzerland and Belgium between March and June 2023. We analysed 232 replies from 58 nephrologists at transplant centres and 174 general nephrologists, aged 43...
June 14, 2024: Néphrologie & Thérapeutique
Katherine Rizzolo, Nathan Rockey, Lilia Cervantes
PURPOSE OF REVIEW: Inequities in kidney disease are a result of differences in healthcare access and inequitable structural policies that lead to downstream social challenges. An individual with kidney disease sits at the intersection of a variety of governmental and institutional policies that directly affect their access to kidney healthcare and different care delivery models. However, their voice in policy change is often neglected by stakeholders with more structural power. Marginalized individuals with kidney disease are disproportionately affected by kidney disease and inequitable policies can further these health disparities...
June 17, 2024: Current Opinion in Nephrology and Hypertension
Gajapathiraju Chamarthi, Tatiana Orozco, Jennifer Hale-Gallardo, Shobha Subhash, Popy Shell, Kailyn Pearce, Huanguang Jia, Ashutosh M Shukla
RATIONALE & OBJECTIVE: The Advancing Americans Kidney Health Executive order has directed substantial increases in home dialysis use for incident kidney replacement therapy (KRT). Clinical guidelines recommend patients' self-selection of KRT modality through a shared decision-making process, which, at the minimum, requires predialysis nephrology care and KRT-directed comprehensive prekidney failure patient education (CoPE). The current state of these essential services among Americans with advanced (stages 4 and 5) chronic kidney disease (CKD) and their informed preferences for home dialysis are unknown...
June 2024: Kidney medicine
Yasuyuki Mimura, Katsuhito Kojima, Arisa Fujikawa, Shioko Okada, Akira Fujimori, Akihiro Kuma, Takahiro Kuragano
BACKGROUND: TAFRO syndrome is a rare disorder that causes thrombocytopenia, generalized oedema, fever, organ enlargement, and renal impairment. Few reports have suggested an association with vaccines, and few cases have undergone renal biopsy. TAFRO syndrome is often severe and fatal, and its cause is unknown. We report a case of TAFRO syndrome that occurred after vaccination with the coronavirus disease 2019 (COVID-19) vaccine. CASE PRESENTATION: An 82-year-old woman received two doses of the BNT162b2 mRNA vaccine 3 weeks apart...
June 13, 2024: BMC Nephrology
Jiansheng Xiao, Tairong Liu, Qiuhua Zhu, Liling Qiu, Jiaqi Ge, Hua Chen
OBJECTIVE: This study is to investigate the safety and efficacy of ureteroscope-assisted laparoscopic ureteroplasty in treating ureteral stricture after pelvic surgery. METHODS: A retrospective analysis of the clinical data of 95 patients treated for ureteral stricture at Ganzhou People's Hospital from June 2017 to March 2023 after pelvic surgery. In this group, 49 patients underwent ureteroscope and laparoscopic ureteroplasty under lithotomy position. The control group consisted of 46 patients who underwent simple laparoscopic ureteroplasty in a supine position...
June 13, 2024: International Urology and Nephrology
Michiaki Abe, Takuhiro Yamaguchi, Seizo Koshiba, Shin Takayama, Toshiki Nakai, Koichiro Nishioka, Satomi Yamasaki, Kazuhiko Kawaguchi, Masanori Umeyama, Atsuko Masaura, Kota Ishizawa, Ryutaro Arita, Takeshi Kanno, Tetsuya Akaishi, Mariko Miyazaki, Takaaki Abe, Tetsuhiro Tanaka, Tadashi Ishii
BACKGROUND: The beneficial effects of oral supplements with alkalinizing agents in patients with chronic kidney disease (CKD) have been limited to the severe stages. We investigated whether two types of supplements, sodium bicarbonate (SB) and potassium citrate/sodium citrate (PCSC), could maintain renal function in patients with mild-stage CKD. METHODS: This was a single-center, open-labeled, randomized cohort trial. Study participants with CKD stages G2, G3a, and G3b were enrolled between March 2013 and January 2019 and randomly assigned by stratification according to age, sex, estimated glomerular filtration rate (eGFR), and diabetes...
June 13, 2024: Clinical and Experimental Nephrology
Jana L Kröse, Jeroen H F de Baaij
Magnesium (Mg2+) is essential for energy metabolism, muscle contraction, and neurotransmission. As part of the Mg-ATP complex, it is involved in over 600 enzymatic reactions. Serum Mg2+ levels are tightly regulated between 0.7 mmol/L and 1.1 mmol/L by interplay of intestinal absorption and renal excretion. In the small intestine, Mg2+ is absorbed paracellularly via claudin-2, and -12. In the colon, transcellular absorption of Mg2+ is facilitated by TRPM6/7 and CNNM4. In the kidney, the proximal tubule reabsorbs only 20% of the filtered Mg2+...
June 13, 2024: Nephrology, Dialysis, Transplantation
Bruce B Duncan, Fernando S Thomé, Theo Vos
No abstract text is available yet for this article.
June 13, 2024: Nephrology, Dialysis, Transplantation
Rui-Zhi Tan, Jian Jia, Tong Li, Li Wang, Fahsai Kantawong
The intricate crosstalk between long noncoding RNAs (lncRNAs) and epigenetic modifications such as chromatin/histone methylation and acetylation offer new perspectives on the pathogenesis and treatment of kidney diseases. lncRNAs, a class of transcripts longer than 200 nucleotides with no protein-coding potential, are now recognized as key regulatory molecules influencing gene expression through diverse mechanisms. They modulate the epigenetic modifications by recruiting or blocking enzymes responsible for adding or removing methyl or acetyl groups, such as DNA, N6-methyladenosine (m6A) and histone methylation and acetylation, subsequently altering chromatin structure and accessibility...
June 12, 2024: Biomedicine & Pharmacotherapy
Shengting Liu, Saijun Bao, Yan Ma
OBJECTIVE: Hyponatremia is one of the main types of complications of primary nephrotic syndrome in children, which can cause significant damage to the patient's nervous system and can lead to death or shock if not intervened early. In addition, patients are relatively young and often need to pay attention to the nursing cooperation of various key points in order to achieve ideal clinical treatment effects. To analyze the key points of nursing cooperation and the implementation effects of primary nephrotic syndrome in children complicated with hyponatremia...
June 14, 2024: Alternative Therapies in Health and Medicine
Qandeel Soomro, Valeria Mezzano, Navneet Narula, Amy Rapkiewicz, Cynthia Loomis, David M Charytan
No abstract text is available yet for this article.
June 13, 2024: Clinical Journal of the American Society of Nephrology: CJASN
Fabiani P Machado, Alessandra R Vicari, Andrea C Bauer
BACKGROUND: Infection following kidney transplantation is a significant risk factor for adverse outcomes. While the donor may be a source of infection, microbiological assessment of the preservation fluid (PF) can mitigate potential recipient contamination and help curb unnecessary antibiotic use. This scoping review aimed to describe the available literature on the association between culture-positive preservation fluid, its clinically relevant outcomes, and management. METHODS: Following the Joanna Briggs Institute's scoping review recommendations, a comprehensive search in databases (EMBASE, MEDLINE, and gray literature) was conducted, with data independently extracted by two researchers from selected studies...
June 13, 2024: Journal of Nephrology
Orit Erman Helper, Husam Qasim, Shmuel Tiosano, Nabil Abu-Amar, Elad Maor, Pazit Beckerman
BACKGROUND: The cardiovascular and metabolic benefits of physical activity have been studied at length, however, data on the association between physical fitness and progression to kidney disease is lacking. We aimed to identify the association between cardiorespiratory fitness and development of chronic kidney disease (CKD) among the healthy population. METHODS: We retrospectively investigated 11,579 healthy self-referred subjects who underwent annual medical screening...
June 13, 2024: Journal of Nephrology
Ji Yan, Sen Li
PURPOSE: This study aimed to explore the relationship between the lipid accumulation product (LAP) index and kidney stone prevalence, utilizing data from the National Health and Nutrition Examination Survey (NHANES) spanning 2007 to 2018. METHODS: An observational study was executed employing the NHANES dataset from 2007 to 2018. Analytical methods encompassed multivariate logistic regression, restricted cubic splines (RCS), subgroup analysis, and interaction tests...
June 13, 2024: International Urology and Nephrology
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