Light chain deposition disease recurrence in renal allograft after long-term remission
Light chain deposition disease (LCDD) is a rare manifestation of monoclonal gammopathy, which can lead to renal failure. We previously reported a detailed recurrence process in a case of LCDD after renal transplantation. To the best of our knowledge, no report has described the long-term clinical course and renal pathology findings of recurrent LCDD in patients after renal transplantation. In this case report, we describe the long-term clinical presentation and changes in renal pathology of the same patient after early LCDD relapse in a renal allograft. A 54-year-old woman with recurrent immunoglobulin A λ-type LCDD in an...
Source: Nephron - February 21, 2023 Category: Urology & Nephrology Source Type: research
Clinicopathological features of Gitelman syndrome with proteinuria and renal dysfunction
Introduction. Gitelman syndrome (GS) is a rare renal tubular salt-wasting disorder. Besides kidney electrolytes loss, proteinuria and renal dysfunction were also observed. However, their incidence, risk factors, pathological features, and prognosis were unclear.
Methods. We retrospectively reviewed 116 GS patients and analyzed their clinical, genetic, and pathological characteristics. We also systematically reviewed articles on GS with proteinuria and renal dysfunction.
Results. Twenty-three GS patients had proteinuria (69.6%) and renal dysfunction (43.5%) with a mean age of 35.3 ±13.2 years and 65.2% were male. Compare...
Source: Nephron - February 20, 2023 Category: Urology & Nephrology Source Type: research
Serum potassium trajectory during AKI and mortality risk
Background: The association between potassium (sK) level trajectory and mortality or the need for kidney replacement therapy (KRT) during acute kidney injury (AKI) has not been adequately explored.
Methods: In this prospective cohort, AKI patients admitted to the Hospital Civil de Guadalajara were enrolled. Eight groups based on the sK (mEq/L) level trajectories during 10 days of hospitalization were created, (1) normokalemia (normoK), defined as sK between 3.5-5.5; (2) hyperkalemia to normoK; (3) hypokalemia to normoK; (4) fluctuating potassium; (5) persistent hypoK; (6) normoK to hypoK; (7) normoK to hyperK; (8) persis...
Source: Nephron - February 17, 2023 Category: Urology & Nephrology Source Type: research
Co-Occurrence of Nephronophthisis Type 1 and Alstr öm Syndrome: A Case Report
We describe the unique case of a patient in whom two ciliopathies with autosomal recessive transmission were clinically and molecularly diagnosed: nephronophthisis type 1 (NPHP1) and Alstr öm syndrome (AS).NPHP1 is one of the main genetic causes of terminal kidney failure in childhood. AS is an ultra-rare multi-systemic disease, characterized by progressive kidney disease, hepatic failure, dystrophy of the rods and cones to blindness, slowly progressive neuro-sensory deafness, dilated cardiomyopathy, obesity, insulin resistance/type 2 diabetes mellitus. The coexistence in the same patient of two rare syndromes with overla...
Source: Nephron - February 6, 2023 Category: Urology & Nephrology Source Type: research
Co-occurrence of nephronophthisis type 1
and alstr Öm syndrome: a case report.
We describe the unique case of a patient in whom two ciliopathies with autosomal recessive transmission were clinically and molecularly diagnosed: Nephronophthisis type1 (NPHP1) and Alstr öm Syndrome (AS). NPHP1 is one of the main genetic causes of terminal kidney failure in childhood. AS is an ultra-rare multi-systemic disease, characterized by progressive kidney disease, hepatic failure, dystrophy of the rods and cones to blindness, slowly progressive neuro-sensory deafness, dilat ed cardiomyopathy, obesity, insulin resistance / type 2 diabetes mellitus.
The coexistence in the same patient of two rare syndromes with ove...
Source: Nephron - February 6, 2023 Category: Urology & Nephrology Source Type: research
The clinicopathologic spectrum of membranous nephropathy with lupus-like features
Conclusion: LL-MN appears to have a significant association with underlying AD and has a subset showing EXT1/2 positivity, whereas most LL-pMN and idiopathic LL-MN likely represent an atypical pathologic presentation of pMN. (Source: Nephron)
Source: Nephron - February 6, 2023 Category: Urology & Nephrology Source Type: research
The cardinal trial of bardoxolone methyl in alport syndrome: when marketing interests prevail over patients clinical needs
Context. Alport syndrome (AS) is a hereditary chronic kidney disease (CKD) with X-linked, autosomal and digenic patterns of transmission. Sieving dysfunction of the glomerular basement membrane caused by congenitally defective type IV collagen results in persistent proteinuria, hematuria and progressive renal dysfunction. There are no disease-specific medications and treatment is based on conservative interventions in particular with renin-angiotensin-aldosterone system inhibitors.
Subject of Review. Evidence that AS is accompanied by glomerular and tubular inflammatory changes and that bardoxolone methyl exerts anti-infl...
Source: Nephron - February 2, 2023 Category: Urology & Nephrology Source Type: research
Association between hypokalemia and albuminuria in a Japanese general population
Conclusions: Hypokalemia was significantly associated with the high preval ence of albuminuria in general population. Regardless of the presence/absence of renal dysfunction, hypertension, or hyperglycemia, hypokalemia was positively associated with the prevalence of albuminuria, and the associations were significant except for the subjects with renal dysfunction. (Source: Nephron)
Source: Nephron - February 1, 2023 Category: Urology & Nephrology Source Type: research
Piceatannol protects against high glucose-induced injury of renal tubular epithelial cells via regulating carbonic anhydrase 2
Conclusion: PIC protects against HG-induced injury of HK-2 cells via regulating CA2. (Source: Nephron)
Source: Nephron - January 30, 2023 Category: Urology & Nephrology Source Type: research
The Clinical and Mutational Spectrum of 69 Turkish Children with Autosomal Recessive or Autosomal Dominant Polycystic Kidney Disease: A Multicenter Retrospective Cohort Study
Conclusion: Based on renal survival analysis, type of genetic variant, growth retardation, and/or malnutrition at presentation were observed to be factors associated with progression to chronic kidney disease (CKD). Differentiation of ARPKD and ADPKD, and identification of the predictors of the development of CKD are vital for optimal management of patients with ARPKD or ADPKD.Nephron (Source: Nephron)
Source: Nephron - January 19, 2023 Category: Urology & Nephrology Source Type: research
Peripheral Eosinophil Count Associated with Disease Activity and Clinical Outcomes in Hospitalized Patients with Lupus Nephritis
Conclusion: Lower EOS count was independently associated with severe disease activity and kidney progression in LN.Nephron (Source: Nephron)
Source: Nephron - January 19, 2023 Category: Urology & Nephrology Source Type: research
Effect of Orthostatic Hypotension on Kidney Function
Conclusions: Classic OH can negatively affect long-term kidney function in the general population.Nephron (Source: Nephron)
Source: Nephron - January 17, 2023 Category: Urology & Nephrology Source Type: research
Age-Dependent Effects of Acute Kidney Injury on End-Stage Kidney Disease and Mortality in Patients with Moderate to Severe Chronic Kidney Disease
Conclusion: Older adults with CKD exhibited a slower decline rate of eGFR, yet they were more likely to develop ESKD following AKI episodes. These results suggest tackling AKI is needed to prevent accelerated initiation of renal replacement therapy in elderly patients with pre-existing CKD.Nephron (Source: Nephron)
Source: Nephron - January 17, 2023 Category: Urology & Nephrology Source Type: research
Kidney Disease and Viral Infection in COVID-19: Why Are Kidney Organoid and Biopsy Studies Not in Agreement?
Context: The clinical course of coronavirus disease-19 (COVID-19) can be complicated by acute kidney injury and proteinuria. Kidney cells express receptors for SARS-CoV-2, the virus responsible for COVID-19. Direct infection of the kidney parenchyma by SARS-CoV-2 has been proposed as the cause of renal dysfunction in COVID-19.Subject of Review: Kidney organoids derived from human embryonic stem cells or induced pluripotent cells can be reproducibly infected by SARS-CoV-2 in vitro and used to study therapeutics. However, kidney biopsy studies of COVID-19 patients with renal dysfunction have shown no evidence of viral infect...
Source: Nephron - January 17, 2023 Category: Urology & Nephrology Source Type: research
Functional characterization of renal tubular epithelial cells in optimized co-culture systems
Conclusion: Our results demonstrate that co-culture with RPTECs has great potential for use in renal replacement therapy, thereby providing fundamental information for manufacturing a bioartificial kidney. (Source: Nephron)
Source: Nephron - January 17, 2023 Category: Urology & Nephrology Source Type: research
