scholarly journals Amiloidosis Renal: Reporte de un Caso y Revisión de Tema

2013 ◽  
Author(s):  
Gustavo Aroca Mart ◽  
Ra Garc ◽  
Andr Cadena Bonfanti ◽  
Marco Anaya Taboada ◽  
Lucia Mercedes Ni ◽  
...  
Keyword(s):  
Renal Failure ◽  
Cause Of Death ◽  
De Novo ◽  
Renal Involvement ◽  
Clinical History ◽  
Amyloid Deposit ◽  
Light Chains ◽  
Renal Amyloidosis ◽  
History Of ◽  
Cardiac Disorders

Se designa con el nombre de amiloidosis a un grupo de enfermedades poco frecuentes, que se caracterizan por el depósito extracelular de un material proteico fibrilar denominado amiloide. La afectación renal en la amiloidosis sistémica es frecuente, por lo que se le asocia un pronóstico desfavorable, al ser la falla renal la segunda causa de muerte en los pacientes que la presentan. El objetivo de este trabajo consistió en presentar el caso de un hombre de 55 años, hipertenso de novo, con cuadro clinico de 7 meses de evolución de edema progresivo asociado a disnea, oliguria y hematuria, documentándose síndrome nefrótico en paraclínicos. El reporte histopatológico reveló acumulación de material eosinofílico rojo congo positiva a nivel glomerular, e inmunofluoresencia positiva para cadenas livianas en patrón mesangial, compatible con amiloidosis renal. La amiloidosis sigue siendo un desafío para los clínicos, en cuanto a su diagnóstico y tratamiento, y el pronóstico empeora a medida que se comprometen más órganos. La principal causa de muerte de los pacientes con amiloidosis obedece a trastornos cardiacos, siendo la segunda causa la falla renal.ABSTRACTA group of rare diseases are called amyloidosis. It is characterized by extracellular fibrillar protein material named amyloid deposit. Renal involvement in systemic amyloidosis is often, giving an unfavorable prognostic, being this one the second death cause in these kind of patients. It aims to show a 55 years old man, hypertensive with a clinical history of 7 months with a progressive edema associated with dyspnea, oliguria and hematuria, reporting nephrotic syndrome in paraclinical patients. The histopathological report reveals accumulation of Congo red coloured material positive in glomerular level and positive iimmune-fluorescence for light chains in mesangial pattern associated with renal Amyloidosis. Amyloidosis is still a challenge for clinical patients. based on its diagnosis and treatment. The prognosis is even worst as more organs are committed. It should be considered that the main cause of death of an amyloidosis patient is cardiac disorders, being renal failure the second cause of death.

scholarly journals The rare hemoglobin variant Hb Mizuho: report of a Swiss family and literature review

2021 ◽  
Author(s):  
Linet Njue ◽  
Cesare Medri ◽  
Peter Keller ◽  
Miriam Diepold ◽  
Behrouz Mansouri Taleghani ◽  
...  
Keyword(s):  
Literature Review ◽  
Hemolytic Anemia ◽  
De Novo ◽  
Clinical History ◽  
Molecular Techniques ◽  
De Novo Mutation ◽  
First Case ◽  
History Of ◽  
Transfusion Dependency ◽  
Unstable Hemoglobin

AbstractHb Mizuho is a very rare unstable hemoglobin; here, we describe the clinical history of three Swiss family members with Hb Mizuho together with a systematic review of the previously six published cases. The clinical history of the adult woman we report here is unique since this is the first Hb Mizuho presenting with Moyamoya complications and the first case reported with long-term erythrocyte exchange. The literature review showed that Hb Mizuho was mainly reported as a de novo mutation, with the exception of children descended from known cases. All published patients with this unstable hemoglobin showed severe hemolytic anemia with the exception of one; all were regularly transfused. Patients with higher HbF levels might require fewer transfusions. All patients underwent splenectomy at a median age of 4 years and had variable clinical improvement; some achieved complete resolution of transfusion dependency after splenectomy. Iron overload in Hb Mizuho patients seems to be mainly attributed to transfusions and has less to do with ineffective erythropoiesis. Diagnosis might be challenging; a normal hemoglobin electrophoresis should not rule out the diagnosis of unstable hemoglobin in patients with otherwise unexplained hemolytic anemia. This series shows the enormous utility of using molecular techniques for diagnosis.

Rapid Reduction of Light-Chains and Proteinuria in Multiple Myeloma (MM) Patients with Light Chain Induced Acute Renal Failure Treated with Lenalidomide and Dexamethasone

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5114-5114
Author(s):  
Heinz Ludwig ◽  
Josef Thaler ◽  
Jan Koren ◽  
Ludek Pour ◽  
Ercan Müldür ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Median Time ◽  
Light Chain ◽  
De Novo ◽  
Light Chains ◽  
High Risk Population ◽  
Renal Response ◽  
Increased Risk ◽  
Grade 3

Abstract Abstract 5114 Introduction: Light chain-induced renal failure (LC-ARF) is a severe complication of MM associated with increased risk of infections, dependency on chronic hemodialysis and shortened survival. Reversibility of renal impairment depends on the degree of renal damage, the duration of renal failure and the quality of response to anti-myeloma therapy. In this phase II trial we assess the efficacy of lenalidomide-dexamethasone in reducing pathogenic light chains and restoring renal function. In addition, we analyze the kinetics of treatment response in patients with LC-ARF. Patients and Methods: 24 patients with LC-ARF as formerly defined (JCO 2010) have been enrolled so far. Age (median): 65.5 years (range: 46–78 years), Gender: male/female: 12/12. All patients presented with ISS stage III. 20 (83.3%) had de novo MM and 4 (16.7%) previously treated, but relapsing disease. Median GFR was 19.9 ml/min (range 6.1 – 37.2 ml/min). ECOG performance was 0 in 6, I-II in 14 and III-IV in 4 patients, respectively. One patient died before first study medication, 3 patients died within the first cycle and 2 patients dropped out early (< 2 cycles). Lenalidomide was given from d 1–21 with dose adaptation according to GFR. Dexamethasone 40 mg was administered on d 1–4, 9–12, 17–20 during cycle 1; thereafter 1x/week. Cycles were repeated q 4 weeks. Results: Presently, 17 patients are evaluable for response (completed ≥2 cycles and fully documented). The median number of cycles is 9 (range 2–9). CR was achieved in 5 (31.3%), nCR in 4 (25%), VGPR in 2 (12.5%) and PR in 5 (25%) patients, respectively, yielding an ORR (CR+nCR+VGPR+PR) of 94% for the evaluable and 69.6% for the ITT population. Median time to best tumor response was 132 days. The greatest proportional reduction in 24 hour urinary excretion (86%) in responding patients occurred within the first 4 weeks of therapy, with only little further improvement beyond that time (figure 1). Renal response was assessed as formerly defined (JCO 2010). 3 patients achieved CRrenal, 3 PRrenal and 5 MRrenal, yielding an ORRrenal in 11 patients (64.7% of the evaluable and 47.8% of the ITT population). Median time to best renal response was 83 days. 3 of 10 dialysis dependent patients became dialysis independent. Median GFR of evaluable patients increased from 15.2 (range 6.1 – 35.1 ml/min) at baseline to a median best GFR of 28.3 ml/min (range 11.3 – 101.1 ml/min) (p<0.0075). The greatest increase in median GFR was noted in the 5 patients with CR (26.7 to 60.9 ml/min, p<0.024) while in those with nCR/VGPR/PR a less pronounced improvement in GFR (10.6 to 22.4 ml/min, p<0.025) was observed Tolerance: Full documentation of adverse events is presently available in 23 patients. Four patients died, 1 (4.3%) each due to infection and cardiac arrest and 2 (8.7%) with unknown causes of death (sudden death). Grade 3/4 anemia, thrombopenia and leucopenia, were seen in 11 (47.8%), 7 (30.4%), and 3 (13%) patients, respectively. Other common grade 3/4 toxicities were infection/sepsis in 9 (39.1%), and cardiac dysfunction in 5 (21.7%) patients, respectively. Exanthema and fatigue were seen in 2 patients (8.7%), and pulmonary embolism and macula edema in 1 patient each (4.3%). Conclusions: LD showed significant anti-myeloma activity with an overall response rate of 94% in the evaluable and of 69.9% in the ITT population. The greatest proportional decrease in 24 hour proteinuria (86%) was obtained already within the first 4 weeks of therapy while renal recovery occurred with delay only. Improvement in renal function was obtained in 65% of the evaluable and in 48% of the ITT population. Toxicity of the LD regimen with the lenalidomide dose adjusted to GFR was as expected in this high risk population. Updated results will be presented. Disclosures: Ludwig: Celgene: Research Funding, Speakers Bureau.

scholarly journals Clinical profile and renal complications among cases of malaria in children attending a tertiary care hospital of South India

2019 ◽  
Vol 6 (4) ◽  
pp. 1445
Author(s):  
Tenali Ravi Kumar ◽  
Sai Lakshmi Ananya Tenali
Keyword(s):  
Renal Failure ◽  
Vivax Malaria ◽  
Statistical Significance ◽  
Demographic Data ◽  
Renal Involvement ◽  
Tertiary Care ◽  
Clinical History ◽  
Clinical Profile ◽  
Care Hospital ◽  
Early Recovery

Background: Malaria is one of the major vectors borne disease globally responsible for 1 million deaths a year. Changing trends in the causative species and epidemiological distribution have identified icterus and renal involvement as an emerging complication associated with severe mortality in children. The objectives of this study were aimed to study the clinical profile of malaria cases admitted in a pediatric ward. The study also highlights the involvement of renal manifestations in the cases with regard to species distribution and associated complications in the study group.Methods: A prospective study for 14 months was conducted, and all positive cases of malaria admitted in paediatric unit were enrolled and socio demographic data, clinical history were collected, and biochemical investigations were performed and analyzed. SPSS software version 12 was used for analysis. Statistical significance was set at p ≤0.05.Results: About 278 subjects with 55.4% males, 44.6% females and with 5-10 years was most common age group. 102 cases of vivax malaria, 152 cases were falciparum and 24 were mixed cases. Cerebral malaria, hyperparasitemia was identified in 28 cases, DIC in 5.04% of cases. Renal involvement was observed in 38.16% of falciparum infections and 27.45% of vivax infections. 68 cases developed acute renal failure as a severe complication.Conclusions:  Renal involvement is more in falciparum and mixed infections than vivax malaria. Early diagnosis and prompt treatment help in early recovery of cases and halts to progression to renal failure. An urgent need for a biomarker for early identification of renal involvement in malaria before biochemical involvement is detected.

scholarly journals Renal involvement as the first symptom of multiple myeloma

2019 ◽  
Author(s):  
Yongjing Du ◽  
Ping Zhang ◽  
Xiang Zhong ◽  
Shasha Chen ◽  
Guisen Li ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Nephrotic Syndrome ◽  
Light Chain ◽  
Clinical Laboratory ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Renal Amyloidosis ◽  
Cast Nephropathy ◽  
Immunofixation Electrophoresis

Abstract Background . Renal involvement is a common complication of multiple myeloma (MM). However, most studies have focused on renal failure in MM, and little information is available about the other renal manifestations in MM and their association with immunophenotypes and renal pathology. Methods . We retrospectively analyzed the clinical, laboratory and pathology data of 283 MM patients treated in Sichuan Provincial People’s Hospital, West China, between January 1990 and May 2017. The patients were divided into a renal involvement group (n = 200) and a non-renal involvement group (n = 83). Results. In the renal involvement group, 90 (45.0%) patients were diagnosed with MM in the Nephrology department, and isolated proteinuria, renal failure and nephrotic syndrome were detected in 90(45.0%), 94 (47.0%) and 58 (29.0%) patients, respectively. 135 patients with renal involvement underwent immunofixation electrophoresis, and IgG, IgA, IgD, IgE, pure light chain and nonsecretory MM were detected in 52 (38.5%), 32 (23.7%), 1 (0.7%), 1 (0.7%), 45(33.3%) and 4 (3.0%) patients, respectively. 47 patients without renal involvement also underwent immunofixation electrophoresis, and IgG and IgA MM were found in 24 (51.0%) and 18 (38.3%) patients, respectively. Severe anemia and hypertension, hypercalcemia and pure light chain were more frequent in patients with renal involvement (P < 0.05).9 patients with renal involvement were performed renal biopsy, and cast nephropathy, renal amyloidosis were proved in 5 and 4 patients, respectively. Conclusions. Renal involvement was common at MM diagnosis and had diverse clinical manifestations. The most common clinical manifestations include renal failure, isolated albuminuria and nephrotic syndrome. Nephrologists should rule out MM in patients presenting with renal involvement.

scholarly journals Renal involvement as the first symptom of multiple myeloma

2019 ◽  
Author(s):  
Yongjing Du ◽  
Ping Zhang ◽  
Xiang Zhong ◽  
Shasha Chen ◽  
Guisen Li ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Light Chain ◽  
Clinical Laboratory ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Renal Amyloidosis ◽  
Cast Nephropathy ◽  
Light Chain Disease ◽  
Immunofixation Electrophoresis

Abstract Background Renal involvement is a common complication of multiple myeloma (MM). However, most studies have focused on renal failure in MM, and little information is available about the other renal manifestations in MM and their association with immunophenotypes and renal pathology. Methods We retrospectively analyzed the clinical, laboratory and pathology data of 283 MM patients treated in Sichuan Provincial People’s Hospital, West China, between January 1990 and May 2017. The patients were divided into a renal involvement group (n = 200) and a non-renal involvement group (n = 83). Results In the renal involvement group, 90 (45.0%) patients were diagnosed with MM in the Nephrology department, and isolated proteinuria, renal failure and nephrotic syndrome were detected in 90(45.0%), 94 (47.0%) and 53 (27.0%) patients, respectively. 135 patients with renal involvement underwent immunofixation electrophoresis, and IgG, IgA, IgD, IgE, pure light chain and nonsecretory MM were detected in 52 (38.5%), 32 (23.7%), 1 (0.7%), 1 (0.7%), 45(33.3%) and 4 (3.0%) patients, respectively. 47 patients without renal involvement also underwent immunofixation electrophoresis, and IgG and IgA MM were found in 24 (51.0%) and 18 (38.3%) patients, respectively. Severe anemia and hypertension, hypercalcemia and pure light chain disease were more frequent in patients with renal involvement (P < 0.05). 9 patients with renal involvement were performed renal biopsy, and cast nephropathy and renal amyloidosis were proved in 5(55.6%) and 4(44.4%) patients, respectively. Conclusions Renal involvement was common at MM diagnosis and had diverse clinical manifestations. Nephrologists should rule out MM in patients presenting with renal involvement.

scholarly journals Renal Involvement in Leprosy: A Case Report

1970 ◽  
Vol 19 (2) ◽  
pp. 84-86
Author(s):  
AKMM Islam ◽  
E Alam ◽  
A Rezzak ◽  
N Huda
Keyword(s):  
Renal Failure ◽  
Case Report ◽  
Interstitial Nephritis ◽  
Membranoproliferative Glomerulonephritis ◽  
Renal Involvement ◽  
Renal Amyloidosis ◽  
Renal Lesion ◽  
Multisystem Disorder

Leprosy is a chronic granulomatous multisystem disorder. Renal involvement is one of the dangerous complications of leprosy. Kidneys are usually involved during splanchnic localization of leprosy. The histopathological renal lesion spectrum includes spectrum of glomerulonephritis, renal amyloidosis and interstitial nephritis. Here we report a case of leprosy who presented with diffuse membranoproliferative glomerulonephritis with renal failure.   doi: 10.3329/taj.v19i2.3157 TAJ 2006; 19(2): 84-86

scholarly journals Renal Abscess Caused by Salmonella Typhi

2015 ◽  
Vol 7 (02) ◽  
pp. 121-123 ◽  
Author(s):  
Amarjeet Kaur ◽  
Smita Sarma ◽  
Navin Kumar ◽  
Sharmila Sengupta
Keyword(s):  
Enteric Fever ◽  
Renal Involvement ◽  
Hepatic Abscess ◽  
Clinical History ◽  
Salmonella Typhi ◽  
The Body ◽  
Renal Abscess ◽  
Rare Presentation ◽  
Life Threatening ◽  
History Of

ABSTRACT Salmonella typhi is a true pathogen, which is capable of causing both intestinal and extraintestinal infections. Unusual presentations of Salmonella should always be kept in mind as this organism can cause disease in almost any organ of the body. S. typhi has been reported to cause the life-threatening infections such as meningitis, endocarditis, myocarditis, empyema, and hepatic abscess. Renal involvement by S. typhi is a relatively rare presentation. We report a case of renal abscess caused by S. typhi in an afebrile, 10-year-old child who did not have any clinical history of enteric fever. To our knowledge, this is the first reported case of isolation of S. typhi from the renal abscess, and interestingly this isolate was found to be resistant to quinolones.

Townes–Brocks syndrome

2015 ◽  
Author(s):  
Udo Vester ◽  
Stefanie Weber
Keyword(s):  
Central Nervous System ◽  
Renal Failure ◽  
Renal Function ◽  
Autosomal Dominant ◽  
Renal Agenesis ◽  
De Novo ◽  
Renal Involvement ◽  
Renal Cysts ◽  
Variable Expression ◽  
Dominant Disease

Townes–Brocks syndrome (TBS) is an autosomal dominant disease with variable expression. Classical features are imperforate anus, dysplastic ears with congenital hearing deficit, and triphalangeal thumbs in most cases. A variety of other malformations (renal, genitourinary, heart, central nervous system, eyes) or hypothyroidism has been described. Mutations in SALL1 have been identified in patients with TBS and genetic testing allows confirmation of the diagnosis. Familiar and sporadic forms (caused by de novo mutations) seem to be equally distributed. Renal involvement in TBS is not uncommon and includes renal agenesis, hypo-/dysplasia, and renal cysts and may eventually lead to chronic renal failure. As renal function may not deteriorate before adulthood, renal function should be monitored in all patients. As cases with TBS can be oligosymptomatic, TBS should be suspected in every case with unexplained renal failure, minor abnormalities, or indicative family history. Genetic counselling is mandatory in identified cases.

Complete Response (CR) with Total Therapy 2 (TT2) for Multiple Myeloma (MM): Low Frequency in Case of Prior MGUS without Adverse Consequences for Survival - Yet Critical for Disease Control in Presumed “De Novo” MM.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1172-1172
Author(s):  
Athanasios Fassas ◽  
Erik Rasmussen ◽  
John Shaughnessy ◽  
Guido Tricot ◽  
Maurizio Zangari ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Cox Regression ◽  
De Novo ◽  
Low Frequency ◽  
Clinical History ◽  
Complete Response ◽  
Prior History ◽  
Solitary Plasmacytoma ◽  
Kaplan Meier ◽  
History Of

Abstract Background: It is now widely accepted that stringently defined CR is a prerequisite for durable EFS and OS also in MM. Patients and Methods: The results of TT2 (4 cycles of intensive induction prior to and consolidation chemotherapy after tandem autotransplant) have recently been reported (ASCO 2005), demonstrating a significantly higher CR rate and longer EFS but not OS among those randomized to thalidomide. Baseline laboratory variables and the clinical history off 668 patients enrolled in TT2 were examined for their potential impact on CR and, in turn CR’s impact on EFS and OS. The Kaplan-Meier method was used to estimate EFS and OS. Cox regression was used to evaluate multivariate factors of CR, EFS, and OS; the prognostic value of CR and 1st and 2nd transplants were modeled as time-dependent covariates in order to adjust for the guarantee time associated with reaching these treatment markers. Results: Of 668 patients enrolled, 45 had a documented history of prior MGUS (n=22) or smoldering MM (SMM) (n=23); 20 had a prior solitary plasmacytoma (SPC) of bone. 2-yr estimates of CR (negative immunofixation, normal bone marrow) were 47% for the 588 without history of preceding SPC/MGUS/SMM, 55% for SPC and 22% for the MGUS/SMM groups. EFS and OS at 4 yrs were 57% and 71%, independent of a preceding disorder. Multivariate analysis revealed faster onset and higher frequency of CR with only light chain MM (HR, 2.6; p&lt;.001), in the absence of MGUS/SMM (HR, 1.8; p=.049), and with thalidomide (HR, 1.7: p&lt;0.001). Cytogenetic abnormalities (CA), del13q14 and amp1q21, while adversely affecting EFS/OS, did not influence CR. On multivariate analysis for EFS and OS accounting, in addition to baseline variables, also for T, CR, 1st and 2nd transplant, and pre-MM history of MGUS/SMM, we observed that patients attaining a CR had significantly improved EFS and OS independent of key baseline variables (amp1q21, del13q14, CA, LDH, albumin) and treatment markers (T, 1st and 2nd transplants). Conclusion: We confirm that documented MGUS/SMM pre-MM is associated with lower CR rate without impacting survival negatively, probably implying the re-establishment of the precursor condition. For the remainder, CR was critical for prolonged survival. Gene expression analysis is applied to recognize, in the absence of prior history, those patients likely having evolved from MGUS and thus contribute fundamentally to a distinction of “de novo” MM from “MGUS-evolved MM”. Those results will be presented at the meeting.

Sign in / Sign up

Export Citation Format

Share Document