Clinicopathological characteristics of patients with paraproteinemia and renal damage

Abstract Background This study aimed to analyze the clinicopathological characteristics of patients with paraproteinemia and renal damage. Methods Ninety-six patients from 2014 to 2018 with paraproteinemia and renal damage were enrolled and the clinical data, renal pathology, treatment and prognosis data were collected. Results A total of 96 patients (54 male and 42 female), accounting for 2.7% of all renal biopsies, were enrolled in this study. Among them, 42 were monoclonal gammopathy of renal significance (MGRS), 21 were renal monotypic immunoglobulin alone (renal monoIg), and 19 were monoclonal gammopathy of undetermined significance (MGUS). Individuals with multiple myeloma (MM) accounted for the fewest number of patients (n = 14). In the MGRS group, the main diseases were amyloidosis (n = 25) and cryoglobulinemic glomerulonephritis (n = 7), while in the MM group, the main diseases were cast nephropathy (n = 9) and light chain deposit disease (n = 3). In the MGUS group, it was mainly IgA nephropathy (IgAN, n = 10) and idiopathic membranous nephropathy (n = 5); while in the renal monoIg group, most of the cases were IgAN (n = 19). Chemotherapy was mainly administered to patients in the MM group, while immunosuppression therapy was mostly administered to patients in the renal monoIg group. Most patients with renal monoIg exhibited a major response, followed by the patients with MGUS and MGRS, while most of the patients with MM had a partial response but none had a major response. Approximately more than half (57.1%) of the patients with MM progressed to end-stage renal disease (ESRD), followed by MGRS (33.3%); however, the mortality rate was low in both the MGRS and MM groups. The survival analysis reviewed that serum creatinine, hemoglobin levels, and the serum κ/λ ratio were independent risk factors for ESRD in patients with MGRS. Conclusions The clinicopathological changes in patients with MGRS were between those in patients with MM and MGUS. The treatment for MGRS and MM was more intensive, and the overall mortality rate was low. Both MGUS and renal monoIg alone exhibited slighter clinicopathological features than MGRS and MM, and the treatment was focused mostly on primary renal diseases.

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Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant

Blood ◽

10.1182/blood-2012-07-445304 ◽

2012 ◽

Vol 120 (22) ◽

pp. 4292-4295 ◽

Cited By ~ 289

Author(s):

Nelson Leung ◽

Frank Bridoux ◽

Colin A. Hutchison ◽

Samih H. Nasr ◽

Paul Cockwell ◽

...

Keyword(s):

Multiple Myeloma ◽

Kidney Transplantation ◽

Monoclonal Gammopathy ◽

Renal Damage ◽

Kidney Diseases ◽

Complete Response ◽

Hematologic Disorder ◽

Appropriate Therapy ◽

Stage Renal Disease ◽

End Stage

Abstract Multiple myeloma is the most frequent monoclonal gammopathy to involve the kidney; however, a growing number of kidney diseases associated with other monoclonal gammopathies are being recognized. Although many histopathologic patterns exist, they are all distinguished by the monoclonal immunoglobulin (or component) deposits. The hematologic disorder in these patients is more consistent with monoclonal gammopathy of undetermined significance (MGUS) than with multiple myeloma. Unfortunately, due to the limitations of the current diagnostic schema, they are frequently diagnosed as MGUS. Because treatment is not recommended for MGUS, appropriate therapy is commonly withheld. In addition to end-stage renal disease, the persistence of the monoclonal gammopathy is associated with high rates of recurrence after kidney transplantation. Preservation and restoration of kidney function are possible with successful treatment targeting the responsible clone. Achievement of hematologic complete response has been shown to prevent recurrence after kidney transplantation. There is a need for a term that properly conveys the pathologic nature of these diseases. We think the term monoclonal gammopathy of renal significance is most helpful to indicate a causal relationship between the monoclonal gammopathy and the renal damage and because the significance of the monoclonal gammopathy is no longer undetermined.

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DETERMINING OF MONOCLONAL GAMMOPATHY IN NEPHROLOGICAL PATIENTS

Nephrology (Saint-Petersburg) ◽

10.24884/1561-6274-2019-23-2-82-90 ◽

2019 ◽

Vol 23 (2) ◽

pp. 82-90

Author(s):

L. B. Lysenko ◽

N. V. Chebotareva ◽

N. N. Mrykhin ◽

V. V. Rameev ◽

T. V. Androsova ◽

...

Keyword(s):

Renal Diseases ◽

Chronic Glomerulonephritis ◽

Al Amyloidosis ◽

Hematological Neoplasms ◽

Cast Nephropathy ◽

Number Of Patients ◽

Light Chain Disease ◽

Kidney Involvement ◽

Cryoglobulinemic Glomerulonephritis ◽

Electrophoresis Of Proteins

BACKGROUND. Мonoclonal gammopathy (MG) is not only the state preceding of hematological neoplasms, but also associated with non- hematological diseases, in particular damage of kidneys. Earlier diagnosis of MG represents an important area in treating patients with renal diseases associated with MG. THE AIM: To determine the frequency of MG among therapeutic and nephrological patients for optimization of methods of their diagnosis and treatment. PATIENTS AND METHODS: In common, 11392 patients were analyzed within 4 years (2013-2016). The standard clinical examination was conducted. Method of an electrophoresis of proteins of serum of blood and the 24-hour urine, method of immunofixation of proteins of serum and urine, and method of free light chains definition in serum (Freelite) were used for MG identification. RESULTS: MG is diagnosed in 174 of 11392 patients: 49 % of men and 51 % of women aged from 18 up to 85 years. MG was found 2.1 times more often in nephrological patient than in patients of therapeutic departments. Among patients of this group, AL-amyloidosis with kidney involvement was diagnosed in 41 %, cryoglobulinemic glomerulonephritis – in 18 %, chronic glomerulonephritis – in 35 %, also there was small number of patients with light chain disease and cast-nephropathy. 86 % of nephrological patients had less than 5 g/l of monoclonal protein that corresponds oligo secretory MG, and at 46 % from them – less than 1 g/l, other 10 % had MG of 5-10 g/l, and only in 4.42 % of patients MG more 10g/l was defined. CONCLUSION: We conclude that MG, especially oligo secretory form, play a significant role in pathogenesis of renal damage. It is important to apply sensitive methods – immunofixation of proteins and method «Freelite» for nephrological patients.

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Sustainability of the Peritoneal Dialysis-First Policy in Hong Kong

Blood Purification ◽

10.1159/000441580 ◽

2015 ◽

Vol 40 (4) ◽

pp. 320-325 ◽

Cited By ~ 16

Author(s):

Agnes Shin-Man Choy ◽

Philip Kam-Tao Li

Keyword(s):

Hong Kong ◽

Peritoneal Dialysis ◽

Financial Burden ◽

Training Programme ◽

Number Of Patients ◽

Ultrafiltration Failure ◽

Average Annual Cost ◽

The Government ◽

Stage Renal Disease ◽

End Stage

In Hong Kong, the average annual cost of haemodialysis (HD) per patient is more than double of that of peritoneal dialysis (PD). As the number of patients with end-stage renal disease (ESRD) has surged, it has posed a great financial burden to the government and society. A PD-first policy has been implemented in Hong Kong for three decades based on its cost-effectiveness, and has achieved successful outcomes throughout the years. A successful PD-first policy requires medical expertise in PD, the support of dedicated staff and a well-designed patient training programme. Addressing patients' PD problems is the key to sustainability of the PD-first policy. In this article, we highlight three important groups of patients: those with frequent peritonitis, ultrafiltration failure or inadequate dialysis. Potential strategies to improve the outcomes of these groups will be discussed. Moreover, enhancing HD as back-up support and promoting organ transplantation are needed in order to maintain sustainability of the PD-first policy.

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Medical Adjuvant Therapy in Reducing Thrombosis With Arteriovenous Grafts and Fistulae Use: A Meta-Analysis of Randomized Controlled Trials

Clinical and Applied Thrombosis/Hemostasis ◽

10.1177/10760296211063882 ◽

2021 ◽

Vol 27 ◽

pp. 107602962110638

Author(s):

Kaleem Ullah ◽

Maham Bashir ◽

Noor Ul Ain ◽

Azza Sarfraz ◽

Zouina Sarfraz ◽

...

Keyword(s):

Adjuvant Therapy ◽

Adjuvant Treatment ◽

Meta Analysis ◽

Controlled Trials ◽

Graft Thrombosis ◽

Av Fistula ◽

Number Of Patients ◽

Long Term Follow Up ◽

Stage Renal Disease ◽

End Stage

Hemodialysis is required for patients with end-stage renal disease (ESRD) that require arteriovenous (AV) grafts or fistulas for vascular access. These access points are prone to thrombosis. To determine the effect of medical adjuvant therapy on AV graft/fistula patency among patients with ESRD on hemodialysis. Adhering to the PRISMA 2020 statement, a systematic search was conducted until August 20, 2021, with keywords including arteriovenous graft, fistula, patency, thrombosis, hemodialysis, adjuvant treatment. The following databases were searched: PubMed, Scopus, Web of Science, CINAHL Plus, and Cochrane. A random-effects model was employed using Review Manager 5.4 for data analysis. The meta-analysis pooled in 1985 participants with 1000 (50.4%) in the medical adjuvant treatment group. At a snapshot, medical adjuvant therapy reduced the risk for graft thrombosis (RR = 0.64, P = .02). Notable medications included aspirin for graft thrombosis (RR = 0.36, P = .006) and ticlopidine for fistula thrombosis (RR = 0.53, P = .01). Certain antiplatelet therapies (aspirin and ticlopidine) reduced the number of patients with AV fistula/graft thrombosis among patients with high heterogeneity among the trials. Other therapies (fish oil, sulfinpyrazone, clopidogrel, and aspirin/dipyridamole) did not demonstrate significant improvement but may be promising once concrete evidence is available. Potential benefits of anti-platelet therapies may be explored to maintain the potency of AV grafts/fistulas through well-designed placebo-controlled trials and long-term follow-up.

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Cardiac Arrest Secondary to Bilateral Pulmonary Emboli following Arteriovenous Fistula Thrombectomy: A Case Report with Review of the Literature

Case Reports in Nephrology ◽

10.1155/2012/831726 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 2

Author(s):

Avni Shah ◽

Naheed Ansari ◽

Zaher Hamadeh

Keyword(s):

Cardiac Arrest ◽

Arteriovenous Fistula ◽

Pulmonary Emboli ◽

Hemodialysis Access ◽

Number Of Patients ◽

Life Threatening ◽

Timely Fashion ◽

Pulmonary Embolization ◽

Stage Renal Disease ◽

End Stage

Number of patients with End Stage Renal Disease (ESRD) is growing worldwide. Hemodialysis remains the main modality of renal replacement therapy for ESRD patients. A patent hemodialysis access (arteriovenous fistula or arteriovenous graft) plays a key role in successful delivery of hemodialysis. Common vascular access issues encountered by patients and nephrologists are thrombosis and infection. The thrombosed access is declotted by various percutaneous techniques these days by multiple outpatient access centers in a timely fashion. Thrombolysis can give rise to various complications, a few of which can be life threatening. A young hemodialysis patient underwent percutaneous thrombolysis of his clotted arteriovenous fistula. Outpatient access thrombectomy was complicated immediately afterwards with cardiac arrest requiring cardiac resuscitation in the recovery room. The patient was admitted to intensive care unit after life sustaining care. Work up revealed multiple pulmonary emboli to both lung fields on CT scan of the chest. Patient was anticoagulated and discharged from the hospital. Thrombolysis of clotted hemodialysis access is associated commonly with occurrences of pulmonary embolic which are usually asymptomatic. Massive pulmonary embolization due to access thrombolysis is rare. Nephrologists and radiologists should be aware of this dangerous complication particularly in patients with preexisting cardiopulmonary disease.

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Causes of Increased Renal Echogenicity in Pediatric Patients

PEDIATRICS ◽

10.1542/peds.72.6.840 ◽

1983 ◽

Vol 72 (6) ◽

pp. 840-846

Author(s):

Alan M. Krensky ◽

Joseph M. Reddish ◽

Rita Littlewood Teele

Keyword(s):

Kidney Disease ◽

Renal Disease ◽

End Stage Renal Disease ◽

Pediatric Patients ◽

Renal Diseases ◽

Polycystic Kidney ◽

Renal Size ◽

Stage Renal Disease ◽

End Stage ◽

Echogenic Kidneys

Review of 2,700 abdominal ultrasonic examinations revealed 56 patients whose kidneys showed increased echogenicity. Echogenic kidneys were associated with medical renal disease in 94% of cases (30% glomerular, 48% tubulointerstitial, 16% end-stage) and with no detectable renal disease in 6% (three patients). Patterns of increased echogenicity and renal size were evaluated. Specific patterns occurred in end-stage renal disease and polycystic kidney disease. Other medical renal diseases had overlapping ultrasonographic features. Some generalizations could be made although increased echogenicity was often nonspecific.

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Renal medicine

10.1093/med/9780198810858.003.0008 ◽

2021 ◽

pp. 353-382

Author(s):

Gopesh K. Modi ◽

Vivekanand Jha

Keyword(s):

Kidney Disease ◽

Renal Disease ◽

Rapidly Progressive Glomerulonephritis ◽

Kidney Injury ◽

Renal Stones ◽

Renal Diseases ◽

Acute Glomerulonephritis ◽

Glomerular Diseases ◽

Stage Renal Disease ◽

End Stage

Assessing renal function, Urinalysis, Proteinuria, Hematuria, Chyluria, Imaging in renal disease, Kidney biopsy, Acute Kidney Injury (AKI), Chronic Kidney Disease (CKD), Diabetic Nephropathy, End Stage Renal Disease and Dialysis, Kidney Transplantation, Glomerular diseases, Acute glomerulonephritis, Urinary schistosomiasis (bilharzia), Infections and Kidney Disease, Rapidly Progressive glomerulonephritis, Tubulointerstitial Disease, Urinary Tract Infection, Vesico-ureteric reflux, Renal Stones, Renal Disease in Pregnancy, Renal Artery Stenosis, Renal Mass, Inherited Renal Diseases

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Experience with Continuous Ambulatory Peritoneal Dialysis in Belgium

Peritoneal Dialysis International ◽

10.1177/089686088000100504 ◽

1980 ◽

Vol 1 (5) ◽

pp. 54-58 ◽

Cited By ~ 10

Author(s):

Norbert H. Lameire ◽

Marc De Paepe ◽

Raymond Vanholder ◽

Johan Verbanck ◽

Severin Ringoir

Keyword(s):

Peritoneal Dialysis ◽

Continuous Ambulatory Peritoneal Dialysis ◽

Average Rate ◽

Sodium Concentration ◽

Risk Category ◽

Diabetic Patients ◽

Serum Triglycerides ◽

Number Of Patients ◽

Stage Renal Disease ◽

End Stage

This paper has reviewed experience in Belgium with 99 patients on CAPD. They represent 6-7% of all dialysis patients in this country. The principle reasons for selecting CAPD were old age, problems with vascular access and major cardiovas cular complications. Hemoglobin and hematrocrit values increased in all patients but preliminary measurements of red cell volume in some of them showed no change. Most patients showed moderate increases in serum triglycerides. In three non-diabetic patients with marked elevation in triglyceride levels, insulin, given intraperitoneally, prevented further increases. The frequency of peritonitis was still high; the average rate was one episode every 7.6 patient months. Other major complications included hypotension, which improved after the substitution of dialysate with a higher sodium concentration, severe respiratory disease and gangrene of the legs. After a mean follow-up of seven months, the death rate was 18% and the rate of technical success was 70%. The fact that most of our patients were in the high-risk category should be kept in mind when comparing these results with those obtained with other modes of treatment. At the end of 1978, a total of 1195 patients with end-stage renal disease (ESRD) were treated on either home or hospital dialysis in Belgium. There were 50 dialysis centers for a total population of 9.8 million. Of these 1195 patients, only seven were treated with either continuous ambulatory peritoneal dialysis (2-4) or intermittent peritoneal dialysis. Since then and until July 1, 1980 the number of patients treated with CAPD in Belgium has increased to 99 and this paper describes our experience with these patients.

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Monoclonal gammopathy of undetermined significance coexisting in patients undergoing kidney transplantation does not adversely influence post-graft clinical outcome

Clinical Kidney Journal ◽

10.1093/ckj/sfaa105 ◽

2020 ◽

Author(s):

Roberta Clari ◽

Corrado Tarella ◽

Roberta Giraudi ◽

Maria Cristina Torazza ◽

Ester Gallo ◽

...

Keyword(s):

Graft Survival ◽

Monoclonal Gammopathy ◽

Patient Survival ◽

Diagnostic Procedures ◽

Control Group ◽

Patient Death ◽

Post Transplant ◽

Stage Renal Disease ◽

End Stage ◽

Undetermined Significance

Abstract Background Management of patients with oncohaematological disorders such as monoclonal gammopathy of undetermined significance (MGUS) is a frequent problem in pre-transplant work-up. Insights on disease progression and long-term functional outcomes are still lacking in this setting. Methods This was a retrospective analysis on all patients with MGUS who underwent kidney transplant (KT) at our centre between 1 January 2000 and 31 December 2017 (cases, n = 65). Patients were matched with a control group (KTs with similar characteristics but without history of haematological disease, controls, n = 1079). Primary endpoints were graft and patient survival; secondary endpoints were causes of graft failure, patient death, occurrence of allograft rejection, post-transplant neoplasia (not correlated to previous disorder) and/or infectious episodes. Results The MGUS and control groups had a similar mean age [60 (29–79) versus 55.2 (19.3–79.5) years, respectively] and percentage of males (69.2% versus 64.6%, respectively). Median follow-up time since KT was 3.5 years (0–14) in cases and 8.3 years (0–14.9) in controls. All MGUS patients underwent KT following extensive multidiscliplinary investigations. No differences were found between cases and controls regarding patient and graft survival or post-transplant complications except for lower incidence of infections (58.7% versus 69.8%, P = 0.019) and increased use of mTOR inhbitors (30.3% versus 14.7%, P = 0.001) in MGUS. MGUS isotype did not influence graft and patient survival. The absence of difference in patients and graft survival was also confirmed in an adjunctive analysis where MGUS were compared with controls (ratio 1:2) matched for recipient age, gender, number of transplantations and transplant period. Conclusion Patients with MGUS may undergo KT without significantly increased risks of complications, provided that appropriate diagnostic procedures are carefully followed. Multidiscipline-based studies are crucial for establishing well designed pre- and post-transplant protocols for the best management of patients with coexisting MGUS and end-stage renal disease.

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The Key Role of Epithelial to Mesenchymal Transition (EMT) in Hypertensive Kidney Disease

International Journal of Molecular Sciences ◽

10.3390/ijms20143567 ◽

2019 ◽

Vol 20 (14) ◽

pp. 3567 ◽

Cited By ~ 3

Author(s):

Teresa Seccia ◽

Brasilina Caroccia ◽

Maria Piazza ◽

Gian Paolo Rossi

Keyword(s):

Kidney Disease ◽

Molecular Mechanisms ◽

Epithelial To Mesenchymal Transition ◽

Renal Diseases ◽

Hypertensive Nephropathy ◽

Mesenchymal Transition ◽

Afferent Arterioles ◽

Stage Renal Disease ◽

End Stage

Accumulating evidence indicates that epithelial-to-mesenchymal transition (EMT), originally described as a key process for organ development and metastasis budding in cancer, plays a key role in the development of renal fibrosis in several diseases, including hypertensive nephroangiosclerosis. We herein reviewed the concept of EMT and its role in renal diseases, with particular focus on hypertensive kidney disease, the second leading cause of end-stage renal disease after diabetes mellitus. After discussing the pathophysiology of hypertensive nephropathy, the ‘classic’ view of hypertensive nephrosclerosis entailing hyalinization, and sclerosis of interlobular and afferent arterioles, we examined the changes occurring in the glomerulus and tubulo-interstitium and the studies that investigated the role of EMT and its molecular mechanisms in hypertensive kidney disease. Finally, we examined the reasons why some studies failed to provide solid evidence for renal EMT in hypertension.

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