UNASSIGNED: To describe ocular findings in individuals with primary hyperoxaluria type 1 (PH1), focusing on the correlations between retinal anatomy and retinal function. To characterize the retinal alterations that occur at different disease stages by evaluating individuals with diverse degrees of renal impairment associated with PH1.
UNASSIGNED: A cross-sectional study.
UNASSIGNED: Patients diagnosed with PH1 based on clinical criteria and genetic testing, treated in the Pediatric Nephrology Unit of the Ruth Children\'s Hospital, Rambam Health Care Campus, Haifa, Israel between 2013 and 2021.
UNASSIGNED: The ophthalmological assessment included a slit-lamp biomicroscopy of the anterior and posterior segment or indirect ophthalmoscopy. Electroretinography was employed for assessment of the retinal function, and retinal imaging included spectral-domain OCT and fundus autofluorescence. A systematic evaluation of the disease stage was based on clinical criteria including physical examination, purposeful imaging (X-ray, echocardiography, and US abdomen), and laboratory tests as needed.
UNASSIGNED: Anatomical and functional assessment of the retina in patients with PH1, and the relationship between retinal dysfunction and kidney impairment.
UNASSIGNED: A total of 16 eyes were examined in the study of 8 children ranging in age from 4 to 19 years. Four eyes (25%) showed normal structural and functional retinal findings, 8 eyes (50%) presented functional impairment in the absence of pathological structural findings, and 4 eyes (25%) had advanced retinal damage that manifested as significant morphological and functional impairment. There was no direct relationship between the severity of the renal disease and the severity of the retinal phenotype.
UNASSIGNED: Subjects with PH1 present varying severity levels of the retinal phenotype, with possible discrepancy between the clinical retinal morphology and the retinal function noted on electroretinography. These findings raise questions about the molecular basis of the retinal manifestations in PH1. The presence of functional impairment in the absence of evident crystal deposition in the retina suggests that, in addition to oxalate crystal accumulation, other biomolecular processes may play a role in the development of retinopathy.

With the increasing threat of metabolic syndromes, a focus on maintaining kidney health from early- to mid-adulthood is necessary. This study elucidates mortality risk and years of life lost (YLLs) due to abnormal renal function. This was a retrospective, matched cohort study from health checkup data from 2000 to 2015. We identified 12,774 participants with abnormal renal function (eGFR < 60 mL/min/1.73 m2) and used propensity score matching to identify 25,548 participants with normal renal function (eGFR ≥ 60). YLLs were estimated using the life expectancy differences between the abnormal and matched normal cohorts. Cox models were used to estimate the adjusted mortality risk. The estimated life expectancy of participants with proteinuria and eGFR < 60 was 26.24 years, with a 95 % confidence interval of (23.96, 29.36), 17.62 (16.37, 18.78), and 11.70 (11.02, 12.46) for age groups of 30 - 54, 55 - 64, and 65 - 79 years, respectively. The estimated YLLs of participants with proteinuria and eGFR < 60, as compared with the matched normal cohort, were 17.86 (13.41, 20.36), 12.55 (11.41, 13.78), and 8.31 (7.47, 9.13) years for the three age groups, respectively. The Cox model estimates of mortality hazard ratios of participants having proteinuria and eGFR < 60 against matched referents were 5.29 (3.97, 7.05), 3.99 (3.34, 4.75), and 3.05 (2.62, 3.55) for the three age groups, respectively. Abnormal renal function shortens life expectancy, particularly in patients with proteinuria and in younger adults. Active health management of renal function can reduce the disease burden.

Gastric pneumatosis (GP) is a rare finding. It can be seen with both gastric emphysema (GE) and emphysematous gastritis (EG); however, both conditions present similarly and differentiating between the 2 is difficult radiographically. Moreover, the treatment is vastly different for both conditions, in which treatment for GE is focused on supportive care while treatment for EG may even involve gastrectomy. Making the distinction between GE and EG is crucial because GE has a benign clinical course, while EG carries significant mortality. Early endoscopy may be a useful tool in differentiating between the 2 conditions and to guide further management. Herein, we present a case series of 2 immunocompromised patients who presented with symptoms and radiographic evidence consistent with gastric pneumatosis. We found that early endoscopy assisted in risk stratification and helped guide our management strategy. We recommend consideration of endoscopic evaluation as part of ritualized evaluation of patients presenting with gastric pneumatosis.

Cirrhosis predisposes to abnormalities in energy, hormonal, and immunological homeostasis. Disturbances in these metabolic processes create susceptibility to sarcopenia or pathological muscle wasting. Sarcopenia is prevalent in cirrhosis and its presence portends significant adverse outcomes including the length of hospital stay, infectious complications, and mortality. This highlights the importance of identification of at-risk individuals with early nutritional, therapeutic and physical therapy intervention. This manuscript summarizes literature relevant to sarcopenia in cirrhosis, describes current knowledge, and elucidates possible future directions.

Carnitine is a medically needful nutrient that contributes in the production of energy and the metabolism of fatty acids. Bioavailability is higher in vegetarians than in people who eat meat. Deficits in carnitine transporters occur as a result of genetic mutations or in combination with other illnesses such like hepatic or renal disease. Carnitine deficit can arise in diseases such endocrine maladies, cardiomyopathy, diabetes, malnutrition, aging, sepsis, and cirrhosis due to abnormalities in carnitine regulation. The exogenously provided molecule is obviously useful in people with primary carnitine deficits, which can be life-threatening, and also some secondary deficiencies, including such organic acidurias: by eradicating hypotonia, muscle weakness, motor skills, and wasting are all improved l-carnitine (LC) have reported to improve myocardial functionality and metabolism in ischemic heart disease patients, as well as athletic performance in individuals with angina pectoris. Furthermore, although some intriguing data indicates that LC could be useful in a variety of conditions, including carnitine deficiency caused by long-term total parenteral supplementation or chronic hemodialysis, hyperlipidemias, and the prevention of anthracyclines and valproate-induced toxicity, such findings must be viewed with caution.

Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia and is associated with substantial increases in the risk for stroke and systemic thromboembolism. With the successful introduction of the first non-vitamin K antagonistdirect oral anticoagulant agent (NOAC) in 2009, the role of vitamin K antagonists has been replaced in most clinical settings except in a few conditions for which NOACs are contraindicated. Data for the use of NOACs in different clinical scenarios have been accumulating in the past decade, and a more sophisticated strategy for patients with AF is now warranted. JACC: Asia recently appointed a working group to summarize the most updated information regarding stroke prevention in AF. The aim of this statement is to provide possible treatment options in daily practice. Local availability, cost, and patient comorbidities should also be considered. Final decisions may still need to be individualized and based on clinicians\' discretion. This is part 2 of the statement.

Chronic kidney disease (CKD) is a global health concern and public health priority. The condition often involves inflammation due to the accumulation of toxins and the reduced clearance of inflammatory cytokines, leading to gradual loss of kidney function. Because of the tremendous burden of CKD, finding effective treatment strategies against inflammation is crucial. Substantial evidence suggests an association between kidney disease and the inflammasome. As a well-known multiprotein signaling complex, the NLR family pyrin domain containing 3 (NLRP3) inflammasome plays an important role in inducing renal inflammation and fibrosis. Small molecule inhibitors targeting the NLRP3 inflammasome are potential agents for the treatment of CKD.The NLRP3 inflammasome activation amplifies the inflammation response, promoting pyroptotic cell death. Thus, it may contribute to the onset and progression of CKD, but the mechanism behind inflammasome activation in CKD remains obscure.In this review, we summarized recent findings on the role of the NLRP3 inflammasome in CKD and new strategies targeting the NLRP3 inflammasome.

UNASSIGNED: The association between chronic kidney disease and adverse outcomes after coronary artery bypass grafting is well established; in contrast, the association between chronic kidney disease and readmission has been less thoroughly investigated. We hypothesized that patients at higher chronic kidney disease stages have greater risk of readmission, poorer operative outcomes, and greater hospitalization cost.
UNASSIGNED: Using the 2016-2018 Nationwide Readmissions Database, we identified 519,387 patients who underwent isolated coronary artery bypass grafting. Patients were stratified by chronic kidney disease stage based on International Classification of Diseases 10th Revision classification. Multivariable logistic regression was used to assess risk factors for in-hospital mortality and 90-day readmission.
UNASSIGNED: Hospital readmission, in-hospital mortality, and cost progressively increased with worsening chronic kidney disease stage; patients with end-stage renal disease had the highest in-hospital mortality rate (7.2%), hospitalization costs ($59,616) (P < .001), and 90-day readmission rate (40%) (P < .001). Chronic kidney disease stage greater than 3 was associated with in-hospital mortality (odds ratio, 1.56, 95% confidence interval, 1.40-1.73; P < .001) and 90-day readmission (odds ratio, 1.66, 95% confidence interval, 1.56-1.76; P < .001). At 30 days after discharge, new-onset dialysis dependence was more frequent in patients readmitted with chronic kidney disease 4 to 5 (8.9%; n = 1495) than in patients with chronic kidney disease 1 to 3 (1.4%; n = 8623) and patients without chronic kidney disease (0.3%; n = 38,885). At 90 days after discharge, dialysis dependence increased to 11.1% (n = 1916) in readmitted patients with chronic kidney disease 4 to 5 but remained stable for patients with chronic kidney disease 1 to 3 (1.4%; n = 10,907) and patients without chronic kidney disease (0.3%; n = 50,200).
UNASSIGNED: Chronic kidney disease stage is strongly associated with mortality, new-onset dialysis dependence, readmission, and higher cost after coronary artery bypass grafting. Patients with chronic kidney disease 4 and 5 and patients with end-stage renal disease are readmitted at the highest rates. Although further research is needed, a targeted approach may reduce costly readmissions and improve outcomes after coronary artery bypass grafting in patients with chronic kidney disease.

Pregnancy after simultaneous pancreas and kidney transplantation (SPKT) carries a high risk of maternal and fetal complications. We report the case of a 39-year-old woman with three consecutive pregnancies with favorable outcomes after SPKT. Within the first year of SPKT, the patient had a spontaneous pregnancy. At 32 weeks of gestation, she underwent an emergency cesarean section (CS) due to severe preeclampsia and HELLP syndrome. The infant was of average birth weight and was transferred to the neonatal intensive care unit for further management. A second unplanned pregnancy occurred almost nine months after the first. The antenatal assessments for fetal growth, blood glucose, and blood pressure were normal throughout follow-up. Early in her pregnancy, the patient developed an uneventful retinopathy of the left eye. At 37 weeks of gestation, she underwent an elective CS due to a short inter-pregnancy interval and delivered a healthy baby with an average birth weight. At the age of 39 years, the patient had a third unplanned pregnancy. She was diagnosed with seronegative antiphospholipid syndrome. She suffered from bilateral vitreous hemorrhage and was managed successfully with a minimally invasive laser treatment combined with an intravitreal injection of anti-vascular endothelial growth factor during her third trimester. At 35 weeks of gestation, the patient presented with labor pain and underwent an emergency CS and delivered a healthy baby with an average birth weight. Pregnancy after SPKT requires a multidisciplinary approach with a careful workup.

Guidelines consider radial access a relative contraindication in patients with end-stage renal disease as part of a vessel preservation strategy. Radial access distal to a hemodialysis fistula, what we term transradial-transfistula access, offers a solution to radially access this population without affecting their vessel preservation plan. (Level of Difficulty: Advanced.).