Collagenofibrotic glomerulopathy in a kidney transplant recipient: A first report.

Collagenofibrotic glomerulopathy (CG) is a rare disease characterized by the deposition of collagen type 3 fibrils in the glomeruli. Patients may have proteinuria, hematuria, and/or renal dysfunction. CG is considered a progressive disease with variable rates of progression. The definitive diagnosis is made by electron microscopy with the presence of characteristic subendothelial and mesangial curved, comma-like, banded collagen type 3 fibers of 40-65 nm periodicity. We are reporting the first case of CG in a kidney transplant recipient with kidney disease of unknown cause.

Pediatric nephrogenic systemic fibrosis is rarely reported: a RADAR report.

BACKGROUND: Nephrogenic systemic fibrosis is a fibrosing disorder associated with exposure to gadolinium-based contrast agents in people with severely compromised renal function. OBJECTIVE: The purpose of this study was to determine the reported number of cases of nephrogenic systemic fibrosis in children using three distinct publicly available data sources. MATERIALS AND METHODS: We conducted systematic searches of the U.S. Food and Drug Administration Adverse Event Reporting System (FAERS), the International Center for Nephrogenic Systemic Fibrosis Research (ICNSFR) registry and published literature from January 1997 through September 2012. We contacted authors of individual published cases to obtain follow-up data. Data sets were cross-referenced to eliminate duplicate reporting. RESULTS: We identified 23 children with nephrogenic systemic fibrosis. Seventeen had documented exposure to gadolinium-based contrast agents. Six children had been reported in both the FAERS and the literature, four in the FAERS and the ICNSFR registry and five in all three data sources. CONCLUSION: Nephrogenic systemic fibrosis has been rarely reported in children. Although rules related to confidentiality limit the ability to reconcile reports, active pharmaco-vigilance using RADAR (Research on Adverse Drug events And Reports) methodology helped in establishing the number of individual pediatric cases within the three major data sources.

Impact of Sodium-Glucose Cotransporter-2 Inhibitors in the Management of Chronic Kidney Disease: A Middle East and Africa Perspective.

Chronic kidney disease (CKD) is a major public health concern in the Middle East and Africa (MEA) region and a leading cause of death in patients with type 2 diabetes mellitus (T2DM) and hypertension. Early initiation of sodium-glucose cotransporter - 2 inhibitors (SGLT-2i) and proper sequencing with renin-angiotensin-aldosterone system inhibitors (RAASi) in these patients may result in better clinical outcomes due to their cardioprotective properties and complementary mechanisms of action. In this review, we present guideline-based consensus recommendations by experts from the MEA region, as practical algorithms for screening, early detection, nephrology referral, and treatment pathways for CKD management in patients with hypertension and diabetes mellitus. This study will help physicians take timely and appropriate actions to provide better care to patients with CKD or those at high risk of CKD.

Capacity for the management of kidney failure in the International Society of Nephrology Middle East region: report from the 2023 ISN Global Kidney Health Atlas (ISN-GKHA).

The highest financial and symptom burdens and the lowest health-related quality-of-life scores are seen in people with kidney failure. A total of 11 countries in the International Society of Nephrology (ISN) Middle East region responded to the ISN-Global Kidney Health Atlas. The prevalence of chronic kidney disease (CKD) in the region ranged from 4.9% in Yemen to 12.2% in Lebanon, whereas prevalence of kidney failure treated with dialysis or transplantation ranged from 152 per million population (pmp) in the United Arab Emirates to 869 pmp in Kuwait. Overall, the incidence of kidney transplantation was highest in Saudi Arabia (20.2 pmp) and was lowest in Oman (2.2 pmp). Chronic hemodialysis (HD) and peritoneal dialysis (PD) services were available in all countries, whereas kidney transplantation was available in most countries of the region. Public government funding that makes acute dialysis, chronic HD, chronic PD, and kidney transplantation medications free at the point of delivery was available in 54.5%, 72.7%, 54.5%, and 54.5% of countries, respectively. Conservative kidney management was available in 45% of countries. Only Oman had a CKD registry; 7 countries (64%) had dialysis registries, and 8 (73%) had kidney transplantation registries. The ISN Middle East region has a high burden of kidney disease and multiple challenges to overcome. Prevention and detection of kidney disease can be improved by the design of tailored guidelines, allocation of additional resources, improvement of early detection at all levels of care, and implementation of sustainable health information systems.

Effects of weight reduction regimens and bariatric surgery on chronic kidney disease in obese patients.

Increasing evidence nowadays is showing that obesity by itself, independent of other comorbidities like diabetes and hypertension, is associated with renal functional changes and structural damage. Intentional weight loss demonstrates beneficial reduction in proteinuria and albuminuria in patients with mild to moderate chronic kidney disease, particularly those whose renal damage is likely induced by obesity. The safety of some weight loss interventions, particularly the use of high-protein diets and/or medications, is questionable in this population due to the lack of well-designed randomized controlled studies reporting on their efficacy or harm. Bariatric surgery showed the most promising results with regards to ameliorating glomerular hyperfiltration and albuminuria albeit with a modest risk of increased perioperative complications with advanced stages of chronic kidney disease (CKD).

Recommendations for Early and Comprehensive Management of Type 2 Diabetes and Its Related Cardio-Renal Complications.

Type 2 diabetes (T2D) is a global health problem accompanied by an elevated risk of complications, the most common being cardiac and renal diseases. In Lebanon, the prevalence of T2D is estimated at 8-13%. Local medical practice generally suffers from clinical inertia, with gaps in the yearly assessment of clinical manifestations and suboptimal screening for major complications. The joint statement presented here, endorsed by five Lebanese scientific medical societies, aims at providing physicians in Lebanon with a tool for early, effective, and comprehensive care of patients with T2D. Findings from major randomized clinical trials of antidiabetic medications with cardio-renal benefits are presented, together with recommendations from international medical societies. Optimal care should be multidisciplinary and should include a multifactorial risk assessment, lifestyle modifications, and a regular evaluation of risks, including the risks for cardiovascular (CV) and renal complications. With international guidelines supporting a shift in T2D management from glucose-lowering agents to disease-modifying drugs, the present statement recommends treatment initiation with metformin, followed by the addition of sodium-glucose cotransporter 2 inhibitors or glucagon-like peptide-1 receptor agonists due to their CV and renal protection properties, whenever possible. In addition to the selection of the most appropriate pharmacological therapy, efforts should be made to provide continuous education to patients about their disease, with the aim to achieve a patient-centered approach and to foster self-management and adherence to the medical plan. Increasing the level of patient engagement is expected to be associated with favorable health outcomes. Finally, this statement recommends setting an achievable individualized management plan and conducting regular follow-ups to monitor the patients' glycemic status and assess their risks every 3-6 months.

Biospecimen Repositories in Low- and Middle-Income Countries: Insights From an American University of Beirut and Memorial Sloan Kettering Collaboration.

PURPOSE: Biobanking helps source tissue and blood for studying cancer genomics. Access to biorepository resources in low- and middle-income countries is lacking. Memorial Sloan Kettering Cancer Center (MSK) and the American University of Beirut (AUB) established a joint tissue biorepository at AUB in Beirut, Lebanon. The undertaking encountered key challenges that were unanticipated. MATERIALS AND METHODS: Patients age 18 years or older were eligible for enrollment at AUB. After consent, biospecimens were obtained at the time of routine diagnostic and/or therapeutic interventions. Both normal and abnormal tissue and solid and/or liquid specimens were collected from varied body sites. Early on, declining consent was frequently observed, and this was highlighted for investigation to understand potential participants reasoning. RESULTS: Of 850 patients approached, 704 (70.8%) elected to consent and 293 (29.5%) declined participation. The number of declined consents led to an amendment permitting the documentation of reasons for same. Of 100 potential participants who declined to consent and to whom outreach was undertaken, 63% indicated lack of research awareness and 27% deferral to their primary physician or family member. A financial gain for AUB was cited as concern by 5%, cultural boundaries in 4%, and 1% expressed concern about confidentiality. Of the patients who elected to consent, 682 biospecimens were procured. CONCLUSION: The AUB-MSK biospecimen repository has provided a unique resource for interrogation. Patient participation rate was high, and analyses of those who elected not to consent (29%) provide important insights into educational need and the local and cultural awareness and norms.

Renal transplantation for nephrogenic systemic fibrosis: a case report and review of the literature.

Nephrogenic systemic fibrosis (NSF) is a rare fibrosing disorder described among patients with renal disease. Currently, no standard therapy exists, although therapeutic modalities have included plasmapheresis, extracorporeal photopheresis, sodium thiosulphate, imatinib and renal transplantation. We describe a patient with NSF who was physically debilitated and underwent renal transplantation. After transplantation, the patient's lesions improved clinically, and the patient was ambulatory. Despite developing worsening renal function, her lesions remained unchanged. We conclude that renal transplantation improves symptoms of NSF, and believe that in patients with NSF, careful consideration should be made for early renal transplantation.

International Society of Nephrology Global Kidney Health Atlas: structures, organization, and services for the management of kidney failure in the Middle East.

Kidney failure is the permanent impairment of kidney function associated with increased morbidity, hospitalization, and requirement for kidney replacement therapy. A total of 11 countries in the Middle East region (84.6%) responded to the survey. The prevalence of chronic kidney disease in the region ranged from 5.2% to 10.6%, whereas prevalence of treated kidney failure ranged from 152 to 826 per million population. Overall, the incidence of kidney transplantation was highest in Iran (30.9 per million population) and lowest in Oman and the United Arab Emirates (2.2 and 3.0 per million population, respectively). Long-term hemodialysis services were available in all countries, long-term peritoneal dialysis services were available in 9 (69.2%) countries, and transplantation services were available in most countries of the region. Public funding covered the costs of nondialysis chronic kidney disease care in two-thirds of countries, and kidney replacement therapy in nearly all countries. More than half of the countries had dialysis registries; however, national noncommunicable disease strategies were lacking in most countries. The Middle East is a region with high burden of kidney disease and needs cost-effective measures through effective health care funding to be available to improve kidney care in the region. Furthermore, well-designed and sustainable health information systems are needed in the region to address current gaps in kidney care in the region.

Tumor lysis syndrome and acute kidney injury: evaluation, prevention, and management.

Tumor lysis syndrome (TLS) describes a constellation of biochemical and clinical abnormalities resulting from rapid and massive tumor cell death. TLS is frequently associated with hyperuricemia, hyperkalemia, hyperphosphatemia, and secondary hypocalcemia that may lead to serious clinical complications, including acute kidney injury and cardiac arrest. Identification of tumor- and patient-specific risk factors for TLS and early recognition of laboratory and clinical TLS based on established criteria are essential for preventing TLS and forestalling acute kidney injury. Early collaboration between oncologists and nephrologists will help improve assessment of patients' kidney function and risk factors, paving the way for timely and efficacious interventions.

Framework for establishing integrated kidney care programs in low- and middle-income countries.

Secular increases in the burden of kidney failure is a major challenge for health systems worldwide, especially in low- and middle-income countries (LMICs) due to growing demand for expensive kidney replacement therapies. In LMICs with limited resources, the priority of providing kidney replacement therapies must be weighed against the prevention and treatment of chronic kidney disease, other kidney disorders such as acute kidney injury, and other noncommunicable diseases, as well as other urgent public health needs. Kidney failure is potentially preventable-not just through primary prevention of risk factors for kidney disease such as hypertension and diabetes, but also by timely management of established chronic kidney disease. Among people with established or incipient kidney failure, there are 3 key treatment strategies-conservative care, kidney transplantation, and dialysis-each of which has its own benefits. Joining up preventive care for people with or at risk for milder forms of chronic kidney disease with all 3 therapies for kidney failure (and developing synergistic links between the different treatment options) is termed "integrated kidney care" and has potential benefits for patients, families, and providers. In addition, because integrated kidney care implicitly considers resource use, it should facilitate a more sustainable approach to managing kidney failure than providing one or more of its components separately. There is currently no agreed framework that LMIC governments can use to establish and/or scale up programs to prevent and treat kidney failure or join up these programs to provide integrated kidney care. This review presents a suggested framework for establishing integrated kidney care programs, focusing on the anticipated needs of policy makers in LMICs.

Corrigendum to "Tonelli M, Nkunu V, Varghese C, et al. Framework for establishing integrated kidney care programs in low- and middle-income countries" Kidney Int Suppl. 2020;10:e19-e23.

[This corrects the article DOI: 10.1016/j.kisu.2019.11.002.].

Considerations on equity in management of end-stage kidney disease in low- and middle-income countries.

Achievement of equity in health requires development of a health system in which everyone has a fair opportunity to attain their full health potential. The current, large country-level variation in the reported incidence and prevalence of treated end-stage kidney disease indicates the existence of system-level inequities. Equitable implementation of kidney replacement therapy (KRT) programs must address issues of availability, affordability, and acceptability. The major structural factors that impact equity in KRT in different countries are the organization of health systems, overall health care spending, funding and delivery models, and nature of KRT prioritization (transplantation, hemodialysis or peritoneal dialysis, and conservative care). Implementation of KRT programs has the potential to exacerbate inequity unless equity is deliberately addressed. In this review, we summarize discussions on equitable provision of KRT in low- and middle-income countries and suggest areas for future research.

Successful use of intraperitoneal daptomycin in the treatment of vancomycin-resistant enterococcus peritonitis.

Peritoneal dialysis-associated peritonitis from such resistant organisms as vancomycin-resistant enterococci increasingly is occurring and is challenging to treat. We describe 2 cases of vancomycin-resistant entercoccus peritonitis successfully treated with intraperitoneal daptomycin. Both patients were on automated peritoneal dialysis therapy with culture-positive vancomycin-resistant Enterococcus faecium peritonitis and were treated with 10 to 14 days of intraperitoneal daptomycin given every 4 hours through manual peritoneal dialysate exchanges. Despite the known degradation in dextrose solutions, intraperitoneal daptomycin was effective in clearing both infections. Neither patient experienced a relapse or repeated peritonitis. Additional studies of dosing and pharmacokinetics of intraperitoneal daptomycin in the treatment of patients with vancomycin-resistant enterococcus peritonitis are needed.

Gadolinium-based contrast agents and nephrogenic systemic fibrosis: why did it happen and what have we learned?

This article addresses two questions about gadolinium-based contrast agents (GBCAs) and nephrogenic systemic fibrosis (NSF): "Why did it happen" and "What have we learned"? It reviews the events leading to the discovery of an association between NSF and GBCAs. Various factors are elucidated that contributed to the delay between the time when GBCA came into widespread clinical use and a link was made with NSF, including use in renal-compromised patients, high-dose magnetic resonance angiography (MRA), lack of documentation and adequate databases, policy and regulatory changes, and an absence of scientific evidence. The authors conclude that the overriding cause was lack of awareness. J. Magn. Reson. Imaging 2009;30:1236-1239. (c) 2009 Wiley-Liss, Inc.

Gadolinium-containing magnetic resonance image contrast agent promotes fibrocyte differentiation.

Gadolinium-containing magnetic resonance imaging (MRI) contrast agents such as Omniscan are associated with nephrogenic systemic fibrosis (NSF). To determine if Omniscan can affect the differentiation of monocytes into fibroblast-like cells called fibrocytes that are found in the fibrotic lesions of NSF, peripheral blood mononuclear cells (PBMCs) from NSF patients, hemodialysis patients without NSF, and healthy, renally sufficient controls were exposed to Omniscan in a standardized in vitro fibrocyte differentiation protocol. When added to PBMCs, the gadolinium-containing MRI contrast agent Omniscan generally had little effect on fibrocyte differentiation. However, 10(-8) to 10(-3) mg/mL Omniscan reduced the ability of the fibrocyte differentiation inhibitor serum amyloid P (SAP) to decrease fibrocyte differentiation in PBMCs from 15 of 17 healthy controls and one of three NSF patients. Omniscan reduced the ability of SAP to decrease fibrocyte differentiation from purified monocytes, indicating that the Omniscan effect does not require the presence of other cells (such as T cells) in the PBMCs. Omniscan also reduced the ability of a different fibrocyte differentiation inhibitor, interleukin-12, to decrease fibrocyte differentiation. These data suggest that Omniscan interferes with the regulatory action of signals that inhibit the differentiation of monocytes to fibrocytes. J. Magn. Reson. Imaging 2009;30:1284-1288. (c) 2009 Wiley-Liss, Inc.

The association of Hashimoto disease and Congo red negative amyloidosis.

Systemic amyloidosis which is characterized by extracellular deposition of monoclonal immunoglobulin light chains in various organs may be difficult to diagnose at an early stage, especially when the Congo red stain is negative. We describe herein a case of Congo red negative primary amyloidosis associated with Hashimoto thyroiditis. The patient presented with multiple organ involvement suggestive of amyloidosis including heart failure, renal failure, and macroglossia. Serum and urine immunofixation studies were positive for monoclonal chains. Even though a biopsy taken from the enlarged tongue of the patient was negative when stained with Congo red, electron microscopy showed ultrastructural features of amyloid deposition. In conclusion, we are reporting a rare case of primary amyloidosis with a negative Congo red stain associated with Hashimoto thyroiditis.