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1.
BMC Nephrol ; 22(1): 394, 2021 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-34837968

RESUMO

BACKGROUND: Although muscle mass loss and pneumonia are common and crucial issues in hemodialysis (HD) patients, few reports have focused on their association, which remains unclear. This study assessed the association between skeletal muscle mass and the incidence of pneumonia in HD patients using the psoas muscle index (PMI). METHODS: This retrospective study included 330 patients on HD who were treated at a single center between July 2011 and June 2012. The observation period was between July 2011 and June 2021. Demographic, clinical, and HD data were collected, and the associations between PMI and hospitalization due to bacterial pneumonia were evaluated using Cox proportional hazards models adjusted for patients' background data. Additionally, the correlation between patient characteristics and PMI was evaluated using multivariable linear regression. RESULTS: Among 330 patients (mean age, 67.3 ± 13.3; 56.7% male; median dialysis vintage 58 months, (interquartile range [IQR] 23-124), 79 were hospitalized for pneumonia during the observation period (median observation period was 4.5 years [IQR 2.0-9.1]). The multivariable Cox proportional analysis, which was adjusted for age, sex, dialysis vintage, diabetes mellitus, and stroke history and considered death as a competing risk, indicated that decreased PMI/(standard deviation) was closely associated with the development of pneumonia (hazard ratio: 0.67, 95% confidence interval: 0.47-0.95, p = 0.03). CONCLUSIONS: Skeletal muscle mass was associated with the development of pneumonia in patients on HD and could be a useful marker for the risk of pneumonia.

2.
Sci Rep ; 11(1): 22290, 2021 Nov 16.
Artigo em Inglês | MEDLINE | ID: mdl-34785712

RESUMO

Muscle mass loss and worsening physical function are crucial issues in patients receiving hemodialysis (HD). However, few studies have investigated the association between temporal changes in muscle mass and physical function in a large number of HD patients. We examined 286 patients receiving HD (males, 58%; age, 66.8 ± 13.0 years) at a single center, and calculated the percent changes in psoas muscle mass index (%PMI) using computed tomography over two screenings, once per year (July 2011-June 2013). Physical function was evaluated using the Eastern Cooperative Oncology Group Performance Status (ECOG-PS) (range 0-4). The observation period was from July 2012 to June 2021. The median %PMI was -9.5%, and those with the lowest quartile of %PMI (< -20.5%) showed a significantly poor prognosis compared with other patients (p < 0.001). Multivariable logistic regression analysis revealed that these patients tended to have decreased physical function (ECOG-PS 2-4) [odds ratio (OR): 2.46, p < 0.001] and albumin levels (OR: 0.22, p = 0.007). Multiple-factor-adjusted Cox regression analyses showed that %PMI (hazard ratio: 0.99, p = 0.004) and each ECOG-PS stage (1-4 vs. 0) (p < 0.01) were associated with mortality. Augmenting physical activities in daily life and serum albumin levels should be considered to maintain muscle mass and improve the prognosis of patients receiving HD.

3.
Am J Case Rep ; 22: e933386, 2021 Oct 28.
Artigo em Inglês | MEDLINE | ID: mdl-34707080

RESUMO

BACKGROUND Calcification in arteries is sometimes observed in patients undergoing hemodialysis; however, ectopic calcification in other organs is uncommon. In particular, diffuse liver calcification is very rare. We report a case of rapidly developing diffuse liver calcification in a patient undergoing hemodialysis. CASE REPORT An 82-year-old woman started hemodialysis because of diabetic nephropathy, and her renal function worsened due to acute coronary syndrome. Percutaneous coronary intervention was conducted, and she was referred to our hospital. However, she subsequently contracted various infections, including a urinary tract infection and pneumonia. On day 43 of hospitalization, she developed septic shock and liver dysfunction due to catheter-induced infection. Although she did not have any medical history of liver disease, hypoperfusion of the liver resulted in liver dysfunction, and a computed tomography scan conducted 3 months later showed diffuse calcification in her liver. Despite recovering from septic shock, she ultimately died of multiple organ failure 21 months after admission to our hospital. CONCLUSIONS Diffuse liver calcification is extremely rare; however, it can be observed in patients undergoing hemodialysis who experience liver hypoperfusion. The precise mechanisms underlying this disorder remain unknown, but a critically ill status and specific characteristics of hemodialysis patients may play important roles in liver calcification.


Assuntos
Calcinose , Hepatopatias , Choque Séptico , Idoso de 80 Anos ou mais , Feminino , Humanos , Diálise Renal/efeitos adversos , Choque Séptico/etiologia
4.
J Clin Med ; 10(19)2021 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-34640403

RESUMO

We aimed to investigate the factors associated with the development of aortic stenosis (AS) in patients undergoing hemodialysis (HD), and to elucidate the prognosis of HD patients with AS. Patients on HD that had also undergone echocardiography at Nagasaki Renal Center between July 2011 and June 2012 were included. Patients with AS at the time of inclusion were excluded. The diagnosis of AS was based on an annual routine or additional echocardiography. The patients were followed up until June 2021. The association between patient background and AS was also evaluated. Of the 302 patients (mean age, 67.4 ± 13.3 years; male, 58%; median dialysis history, 4.7 years), 60 developed AS and 10 underwent aortic valve replacement. A Cox proportional hazards model revealed that age (hazard ratio (HR), 1.07; 95% confidential interval (CI), 1.04-1.10; p < 0.001) and serum phosphate levels (HR, 1.40; 95%CI, 1.16-1.67, p < 0.001) were independent risk factors for developing AS. Incidentally, there was no significant mortality difference between patients with AS and those without (p = 0.53). Serum phosphate levels are a risk factor for developing AS and should be controlled. Annual echocardiography may contribute to the early detection of AS and improves the prognosis of patients undergoing HD.

5.
Intern Med ; 2021 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-34565774

RESUMO

Objective The quality of life and activities of daily living (ADL) are generally poor among dialysis patients after intracerebral hemorrhaging, and their precise clinical course remains unclear. In addition, the association between the severity of cerebral hemorrhaging and the long-term prognosis in these patients has not been fully elucidated. This study aimed to evaluate the subsequent prognosis of hemodialysis patients who survived the acute phase of intracerebral hemorrhaging. Methods We included hemodialysis patients who were admitted to Nagasaki University Hospital between 2007 and 2015 for intracerebral hemorrhaging treatment. After excluding cases of in-hospital death, survivors were classified using the 5-point modified Rankin Scale (mRS), which specifically measures the ADL in patients with cerebrovascular diseases. The patients were followed up at the medical facilities to which they were transferred in the same medical zone until 2017. Results Out of 91 patients with cerebral hemorrhaging (65±11 years old, 66% men, hemodialysis duration 108±91 months), 62 survived until discharge. Twenty-one patients died during observation, largely due to infectious diseases, such as sepsis and pneumonia (n=16, 76%). Compared to patients with mRS 0-4 (n=31), those with mRS 5 (n=31) showed a significantly poorer prognosis. The hazard ratio adjusted for age and antiplatelets was 13.7 (95% confidence interval: 3.88-63.7, p<0.001). Conclusion Hemodialysis patients with intracerebral hemorrhaging who were bedridden showed poor outcomes. The major causes of death were infections. Therefore, these patients should be carefully monitored for infections in order to improve their prognosis.

6.
Intern Med ; 2021 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-34393162

RESUMO

A 68-year-old woman developed systemic blisters while receiving treatment for nephrotic syndrome. As she also developed marked liver dysfunction and disseminated intravascular coagulation, she was admitted to our hospital. She was diagnosed with varicella zoster virus (VZV) infection. Treatment was administered in the intensive-care unit, but the patient died on day 24 post-admission after severe VZV infection. A post-mortem examination showed micro-abscesses and necrosis caused by varicella zoster infection in multiple organs, including the liver, kidneys, and gastrointestinal tract. Because VZV infection can become severe in immunocompromised patients, careful consideration is needed for the prevention and treatment of the viral infection.

7.
CEN Case Rep ; 2021 Jul 25.
Artigo em Inglês | MEDLINE | ID: mdl-34304384

RESUMO

Wiskott-Aldrich syndrome (WAS) is an X-chromosome recessive immunodeficiency disease characterized by the triad of thrombocytopenia, eczema, and susceptibility to infection owing to WAS protein gene abnormalities. Kidney transplantation is rarely offered to WAS patients with end-stage renal disease because of concerns that thrombocytopenia and immune disorders may affect the clinical outcome. Here, we report the case of a 20-year-old kidney transplant patient who developed end-stage renal disease owing to immunoglobulin (Ig)A nephropathy caused by WAS. Despite recurrent IgA nephropathy and T-cell-mediated rejection 7 months after transplantation, two rounds of steroid pulse therapy attenuated his renal function and urinary abnormality. His serum creatinine level was maintained at approximately 1.5 mg/dL 1 year after transplantation. No other WAS-related complications were observed throughout the clinical course. Although WAS can cause poor prognosis in kidney transplant patients, careful follow-up may allow kidney transplantation to be performed.

8.
Intern Med ; 60(14): 2255-2260, 2021 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-33612667

RESUMO

A 48-year-old woman presented with a fever, microscopic hematuria, proteinuria, and rapid deterioration of the renal function. Pulmonary alveolar hemorrhaging and a high level of anti-glomerular basement membrane (GBM) antibodies (700 IU/mL) were observed. Based on her medical history and positive findings of serum lupus anticoagulant, anti-phospholipid antibody syndrome (APS) was suspected. A renal biopsy revealed cellular crescentic glomerulonephritis with thrombosis, suggesting anti-GBM disease with catastrophic APS. The patient was treated with pulse steroid therapy, plasma exchange, hemodialysis, and intravenous cyclophosphamide pulse therapy. To our knowledge, this is the first report of a patient with anti-GBM disease and APS.


Assuntos
Doença Antimembrana Basal Glomerular , Síndrome Antifosfolipídica , Glomerulonefrite Membranoproliferativa , Doença Antimembrana Basal Glomerular/complicações , Doença Antimembrana Basal Glomerular/diagnóstico , Doença Antimembrana Basal Glomerular/terapia , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Autoanticorpos , Feminino , Hemorragia/etiologia , Humanos , Pessoa de Meia-Idade , Troca Plasmática
9.
Ann Transplant ; 26: e928858, 2021 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-33558451

RESUMO

BACKGROUND Although the risk factors for chronic kidney disease progression after deceased donor liver transplantation have been widely reported, there are few reports describing the factors associated with kidney function changes in patients after living donor liver transplantation (LDLT). This study aims to further investigate these kidney function change factors. MATERIAL AND METHODS This retrospective study was performed using the data of patients who underwent LDLT at the Nagasaki University Hospital, Japan from August 2000 to November 2017. Factors contributing to post-transplantation estimated glomerular filtration rate (eGFR) changes were analyzed. RESULTS A total of 191 cases were reviewed. The average age was 53.8 years, and 108 (56.5%) patients were male. Compared to pre-transplantation eGFR levels, eGFR 1 year after LDLT improved in 65 patients (34%) and deteriorated in 126 patients (66%). Multivariate regression analysis revealed that pre-transplant diuretics (P=0.04) and tacrolimus trough value 1 year after transplantation (P=0.04) were significantly associated with elevated eGFR changes. eGFR elevation 1 year after LDLT was more pronounced in patients with a low tacrolimus trough level 1 year after LDLT (P=0.01). Therefore, mycophenolate mofetil was added to tacrolimus in patients with poor renal function before LDLT. CONCLUSIONS Tacrolimus trough level was associated with eGFR changes 1 year after LDLT. The adjusted dose of tacrolimus and combined use of other immunosuppressants may be important to maintain renal function after LDLT.


Assuntos
Taxa de Filtração Glomerular , Transplante de Fígado , Tacrolimo/farmacocinética , Doença Hepática Terminal , Feminino , Humanos , Imunossupressores/farmacocinética , Imunossupressores/uso terapêutico , Doadores Vivos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Tacrolimo/uso terapêutico
10.
Rheumatology (Oxford) ; 60(5): 2333-2341, 2021 05 14.
Artigo em Inglês | MEDLINE | ID: mdl-33166998

RESUMO

OBJECTIVE: We aimed to compare life prognosis and renal relapse after induction therapy in proliferative (PLN) and pure membranous LN (MLN). METHODS: We retrospectively analysed the cases of 140 of 172 patients with LN who underwent a renal biopsy at our hospital or community hospitals from 1993 to 2016. We determined the complete response (CR) rate at 12 months after the patients had started induction therapy, and we evaluated the predictive factors for CR, life prognosis and renal relapse in PLN and pure MLN. We defined PLN as International Society of Neurology and the Renal Pathology Society (ISN/RPS) Class III or IV and MLN as ISN/RPS Class V. RESULTS: The renal pathology of 99 (70.7%) patients was classified as PLN, and that of the other 41 (29.3%) patients as MLN. Fifty patients (50.5%) with PLN and 22 patients (53.7%) with MLN achieved a CR at 12 months. A multivariate analysis showed that a lower index of chronicity in PLN and a higher total haemolytic complement (CH50) level in MLN were predictive factors for achieving a CR at 12 months. A Kaplan-Meier analysis showed that the life prognosis (P = 0.93) and renal relapse (P = 0.52) were not significantly different between PLN and MLN. CONCLUSIONS: The predictive factors for a CR at 12 months post-induction therapy were index of chronicity in PLN and CH50 level in MLN. There were no significant differences in life prognosis or renal relapse between PLN and MLN in the achievement of a CR at 12 months post-induction therapy.


Assuntos
Glomerulonefrite Membranosa/tratamento farmacológico , Imunossupressores/uso terapêutico , Quimioterapia de Indução , Nefrite Lúpica/tratamento farmacológico , Adulto , Feminino , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Estudos Retrospectivos
11.
SAGE Open Med ; 8: 2050312120973502, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33282300

RESUMO

Objectives: Although angiotensin II receptor blockers are effective for patients with chronic kidney disease, dose-dependent renoprotective effects of angiotensin II receptor blockers in patients with moderate to severe chronic kidney disease with non-nephrotic proteinuria are not known. Our aim was to elucidate the dose-dependent renoprotective effects of angiotensin II receptor blockers on such patients. Methods: A multicenter, prospective, randomized trial was conducted from 2009 to 2014. Patients with non-nephrotic stage 3-4 chronic kidney disease were randomized for treatment with either 40 or 80 mg telmisartan and were observed for up to 104 weeks. Overall, 32 and 29 patients were allocated to the 40 and 80 mg telmisartan groups, respectively. The composite primary outcome was renal death, doubling of serum creatinine level, transition to stage 5 chronic kidney disease, and death from any cause. Secondary outcomes included the level of urinary proteins and changes in the estimated glomerular filtration rate. Results: There was no difference in the primary outcome (p = 0.78) and eGFR (p = 0.53) between the two groups; however, after 24 weeks, urinary protein level was significantly lower in the 80 mg group than in the 40 mg group (p < 0.05). No severe adverse events occurred in either group, and the occurrence of adverse events did not significantly differ between them (p = 0.56). Conclusion: Our findings do not demonstrate a direct dose-dependent renoprotective effect of telmisartan. The higher telmisartan dose resulted in a decrease in the amount of urinary protein. Even though high-dose angiotensin II receptor blockers may be preferable for patients with stage 3-4 chronic kidney disease, the clinical importance of the study results may be limited. The study was registered in the UMIN-CTR (https://www.umin.ac.jp/ctr) with the registration number UMIN000040875.

12.
Med Sci Monit ; 26: e928236, 2020 12 21.
Artigo em Inglês | MEDLINE | ID: mdl-33347426

RESUMO

BACKGROUND Liver-type fatty acid-binding protein (L-FABP) is a predictive marker for the early detection of acute kidney injury; however, less is known about how useful it is for predicting residual renal function (RRF) decline in patients on peritoneal dialysis (PD). MATERIAL AND METHODS The study subjects were 35 patients on PD who underwent multiple peritoneal equilibration tests (PETs) between October 2011 and October 2019. Urinary L-FABP levels were analyzed with enzyme-linked immunosorbent assay. The relationship between baseline clinical data, including urinary L-FABP levels and the subsequent annual rate of renal Kt/V decline, was investigated. RESULTS The median follow-up duration was 11 months and the rate of renal Kt/V decline was 0.29/y. Compared with outcomes in the group with renal Kt/V preservation, renal Kt/V decline was associated with both high daily levels of urinary protein excretion (0.60 g/d [range, 0.50-0.87] vs. 0.36 g/d [range, 0.19-0.48]; P=0.01) and high daily levels of urinary L-FABP excretion (111.2 mg/d [range, 76.1-188.6] vs. 61.5 mg/d [range, 35.7-96.0]; P=0.002). Multiple logistic regression analysis showed that only high daily levels of urinary L-FABP excretion were independently associated with renal Kt/V decline (odds ratio 1.03, 95% confidence interval 1.00-1.05; P=0.001). Furthermore, higher daily levels of urinary L-FABP excretion were significantly correlated with the higher annual rate of renal Kt/V decline (r=0.71, P<0.001). CONCLUSIONS We demonstrated that daily levels of urinary L-FABP are associated with RRF decline in patients on PD. The results of the present study indicate that assessment of urinary L-FABP levels may help predict RRF decline in patients on PD.


Assuntos
Proteínas de Ligação a Ácido Graxo/urina , Falência Renal Crônica , Diálise Peritoneal/métodos , Biomarcadores/urina , Progressão da Doença , Feminino , Humanos , Rim/fisiopatologia , Falência Renal Crônica/fisiopatologia , Falência Renal Crônica/terapia , Falência Renal Crônica/urina , Testes de Função Renal/métodos , Masculino , Pessoa de Meia-Idade
13.
Nutrients ; 12(9)2020 Sep 04.
Artigo em Inglês | MEDLINE | ID: mdl-32899702

RESUMO

Educational hospitalization of patients with chronic kidney disease (CKD) may slow the progression of renal dysfunction. However, the educational aspect that is more effective has not been identified to date. In this study, patients with CKD were evaluated for gustatory threshold for salty taste and received augmented salt reduction guidance under educational hospitalization at Nagasaki University Hospital from October 2016. In total, 277 eligible patients were enrolled and hospitalized from 2012 to 2019 (mean age of 69.2 years; men comprised 62.1%). We compared 141 patients (Group A) who were educated in the hospital after October 2016 and 136 patients (Group B) who received standard education in the hospital before October 2016. The changes in the estimated glomerular filtration rate (ΔeGFR) after hospitalization and dialysis induction rate within one year after hospitalization were evaluated. The ΔeGFR was significantly improved in Group A compared to Group B (A: 1.05 mL/min/1.73 m2/month, B: 0.55 mL/min/1.73 m2/month; p = 0.02). The dialysis induction rate was significantly lower in Group A than in Group B (A: 8.5%, B: 15.5%; p = 0.001). These trends were also observed by multivariate analyses. In conclusion, educational hospitalization with enhanced salt reduction guidance may reduce the risk of end-stage renal disease.


Assuntos
Dieta Hipossódica/psicologia , Falência Renal Crônica/prevenção & controle , Educação de Pacientes como Assunto/métodos , Insuficiência Renal Crônica/psicologia , Sódio na Dieta/análise , Idoso , Progressão da Doença , Feminino , Taxa de Filtração Glomerular , Hospitalização , Humanos , Falência Renal Crônica/etiologia , Masculino , Análise Multivariada , Diálise Renal/estatística & dados numéricos , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/fisiopatologia , Comportamento de Redução do Risco , Limiar Gustativo
14.
Ann Transplant ; 25: e924805, 2020 Sep 08.
Artigo em Inglês | MEDLINE | ID: mdl-32895363

RESUMO

BACKGROUND Renal function is strongly associated with patient survival after liver transplantation. However, the estimated glomerular filtration rate (eGFR) after liver transplantation changes, especially in patients who receive diuretics or have urinary abnormalities. We aimed to elucidate how adjusting for these factors affecting eGFR predicted liver graft prognosis. MATERIAL AND METHODS This retrospective study included patients who underwent adult-to-adult living donor liver transplantation (LDLT) between 2000 and 2017. The factors affecting eGFR were assessed, and the association between eGFR and prognosis was investigated using Cox regression models after adjusting for factors affecting renal function. RESULTS We enrolled 244 patients. The median observation period was 4.6 years, and 88 patients reached graft loss or death with a functioning graft. One year after transplantation, 193 patients were living, and one-third of these showed improved eGFR; most of the patients with improved eGFR had taken diuretics before transplantation. A Cox regression model adjusted for the classical risk factors showed that donor age (P<0.001) and lower eGFR (P=0.02) were the independent risk factors associated with poor prognosis. After adjusting for diuretics and urinary abnormalities, eGFR was more strongly associated with liver graft prognosis (P=0.003). CONCLUSIONS Pre-transplant eGFR was associated with prognosis following LDLT and had a stronger effect on prognosis after adjusting for factors affecting eGFR.


Assuntos
Doença Hepática Terminal/cirurgia , Taxa de Filtração Glomerular/fisiologia , Sobrevivência de Enxerto/fisiologia , Rim/fisiopatologia , Transplante de Fígado , Doadores Vivos , Adulto , Idoso , Diuréticos/farmacologia , Doença Hepática Terminal/fisiopatologia , Feminino , Taxa de Filtração Glomerular/efeitos dos fármacos , Sobrevivência de Enxerto/efeitos dos fármacos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos
15.
BMC Nephrol ; 21(1): 282, 2020 07 16.
Artigo em Inglês | MEDLINE | ID: mdl-32677916

RESUMO

BACKGROUND: Renal hypouricemia (RHUC) is a genetic disorder caused by mutations in the SLC22A12 gene, which encodes the major uric acid (UA) transporter, URAT1. The clinical course of related, living donor-derived RHUC in patients undergoing kidney transplantation is poorly understood. Here, we report a case of kidney transplantation from a living relative who had an SLC22A12 mutation. After the transplantation, the recipient's fractional excretion of UA (FEUA) decreased, and chimeric tubular epithelium was observed. CASE PRESENTATION: A 40-year-old man underwent kidney transplantation. His sister was the kidney donor. Three weeks after the transplantation, he had low serum-UA, 148.7 µmol/L, and elevated FEUA, 20.8% (normal: < 10%). The patient's sister had low serum-UA (101.1 µmol/L) and high FEUA (15.8%) before transplant. Suspecting RHUC, we performed next-generation sequencing on a gene panel containing RHUC-associated genes. A heterozygous missense mutation in the SLC22A12 gene was detected in the donor, but not in the recipient. The recipient's serum-UA level increased from 148.7 µmol/L to 231.9 µmol/L 3 months after transplantation and was 226.0 µmol/L 1 year after transplantation. His FEUA decreased from 20.8 to 11.7% 3 months after transplantation and was 12.4% 1 year after transplantation. Fluorescence in situ hybridization of allograft biopsies performed 3 months and 1 year after transplantation showed the presence of Y chromosomes in the tubular epithelial cells, suggesting the recipient's elevated serum-UA levels were owing to a chimeric tubular epithelium. CONCLUSIONS: We reported on a kidney transplant recipient that developed RHUC owing to his donor possessing a heterozygous mutation in the SLC22A12 (URAT1) gene. Despite this mutation, the clinical course was not problematic. Thus, the presence of donor-recipient chimerism in the tubular epithelium might positively affect the clinical course, at least in the short-term.


Assuntos
Transplante de Rim , Túbulos Renais/metabolismo , Doadores Vivos , Transportadores de Ânions Orgânicos/genética , Proteínas de Transporte de Cátions Orgânicos/genética , Erros Inatos do Transporte Tubular Renal/genética , Ácido Úrico/metabolismo , Cálculos Urinários/genética , Adulto , Quimerismo , Feminino , Heterozigoto , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Mutação de Sentido Incorreto , Eliminação Renal/genética , Erros Inatos do Transporte Tubular Renal/metabolismo , Irmãos , Cálculos Urinários/metabolismo
16.
Arthritis Res Ther ; 22(1): 175, 2020 07 22.
Artigo em Inglês | MEDLINE | ID: mdl-32698892

RESUMO

BACKGROUND: Most patients with systemic lupus erythematosus (SLE) progress to lupus nephritis (LN) within 5 years of their SLE diagnosis, although it is not uncommon for LN to develop at later time points. Here we evaluated the clinical features of early- and late-onset LN. PATIENTS AND METHODS: We retrospectively analyzed the cases of 184 of the 201 patients who underwent a renal biopsy at Nagasaki University Hospital and associated community hospitals between 1990 and 2016 and were diagnosed as having LN. Early onset was defined as the development of LN within the first 5 years after the patient's SLE diagnosis, and late onset was defined as LN development > 5 years post-diagnosis. We analyzed the complete renal response (CR) at 6 and 12 months after induction therapy, the classification of renal pathology, and the mortality of the early- and late-onset LN groups. RESULTS: The mean follow-up duration after the renal biopsy was 123 ± 85 months. There were 113 (61.4%) early-onset patients and 71 (38.6%) late-onset patients. A multivariate analysis revealed that the following factors were predictive of CR: at 6 months: female sex (odds ratio [OR] 3.93, 95% confidence interval [CI] 1.31-11.77, p = 0.010), proteinuria (OR 0.83, 95% CI 0.71-0.97, p = 0.009), index of activity (0-24) (OR 0.83, 95% CI 0.70-0.99, p = 0.030), and early-onset LN (OR 2.39, 95% CI 1.15-4.98, p = 0.018); at 12 months: female sex (OR 3.60, 95% CI 1.32-9.83, p = 0.013), mixed LN (OR 0.18, 95% CI 0.04-0.80, p = 0.024), index of activity (0-24) (OR 0.80, 95% CI 0.68-0.94, p = 0.007), and early-onset LN (OR 2.10, 95% CI 1.05-4.23, p = 0.035). In a Cox proportional hazards and Fine-Gray regression model, the early-onset LN group had a significantly better mortality rate than the late-onset LN group (p = 0.038 and p = 0.043, respectively). CONCLUSIONS: In our cohort, early-onset LN was a better predictor of CR at 6 and 12 months than late-onset LN. Our results suggest that early-onset LN patients had lower mortality than late-onset LN patients.


Assuntos
Lúpus Eritematoso Sistêmico , Nefrite Lúpica , Estudos de Coortes , Feminino , Humanos , Japão/epidemiologia , Nefrite Lúpica/diagnóstico , Nefrite Lúpica/epidemiologia , Masculino , Estudos Retrospectivos
17.
CEN Case Rep ; 9(4): 375-379, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32506365

RESUMO

Bartter syndrome and Gitelman syndrome (GS) are autosomal recessive disorders usually caused by homozygous or compound heterozygous mutations in causative genes. In some patients, these two syndromes cannot be discriminated based on clinical features or mutation type; thus, a single disease concept, salt-losing tubulopathies (SLTs), has been used instead. Despite the existence of several SLT causative genes, cases of digenic heterozygous mutations in two different genes are extremely rare. Here, we report the case of a 36-year-old woman with renal insufficiency and hypokalemia caused by an SLT. To evaluate the SLT phenotype, we performed next-generation sequencing (NGS) with a gene panel including SLC12A3, SLC12A1, CLCNKB, and CLCNKA as well as laboratory examinations and diuretic loading tests. The results of the diuretic loading tests were consistent with a GS phenotype, while the NGS results showed that the patient had heterozygous mutations in SLC12A1 and CLCNKB. Both genes have been associated with BS, suggesting that the SLT was caused by digenic heterozygous mutations in two different genes. To date, only a few SLT cases caused by digenic heterozygous mutations in two different genes have been reported. The digenic SLT phenotype in the patient was presumably accelerated by moderate renal insufficiency.


Assuntos
Síndrome de Bartter/diagnóstico , Síndrome de Bartter/genética , Síndrome de Gitelman/diagnóstico , Insuficiência Renal/complicações , Adulto , Síndrome de Bartter/tratamento farmacológico , Canais de Cloreto/genética , Diagnóstico Diferencial , Quimioterapia Combinada , Febuxostat/administração & dosagem , Febuxostat/uso terapêutico , Feminino , Síndrome de Gitelman/genética , Supressores da Gota/administração & dosagem , Supressores da Gota/uso terapêutico , Heterozigoto , Humanos , Hiperuricemia/etiologia , Hipopotassemia/etiologia , Mutação , Fenótipo , Cloreto de Potássio/administração & dosagem , Cloreto de Potássio/uso terapêutico , Membro 1 da Família 12 de Carreador de Soluto/genética , Resultado do Tratamento
18.
BMC Nephrol ; 21(1): 195, 2020 05 24.
Artigo em Inglês | MEDLINE | ID: mdl-32448180

RESUMO

BACKGROUND: Fibrillary glomerulonephritis (FGN) is distinguished from amyloidosis by thicker fibrils and the lack of staining with histochemical dyes typically reactive with amyloid. However, congophilic FGN has been proposed recently and adding laser microdissection followed by mass spectrometry (LMD/MS) to conventional pathological methods would be helpful to diagnose FGN. Here, we report a patient initially diagnosed with FGN whose final pathological diagnosis was changed to immunoglobulin heavy-and-light-chain amyloidosis (AHL) after LMD/MS. CASE PRESENTATION: A 75-year-old male developed nephrotic syndrome. Protein electrophoresis showed IgM κ type M proteinemia and he was diagnosed with IgM monoclonal gammopathy. A renal biopsy was performed and pathological examination showed marked periodic acid-Schiff-positive enlargement of the mesangial region and silver stain positivity, but weak direct fast scarlet staining. Immunofluorescence analysis showed monoclonal deposition of IgM-κ chain in the glomerulus. Under electron microscopy, the fibrils were about 20 nm in diameter, which was thicker than typical amyloid fibrils. Based on these findings, the patient was diagnosed with FGN. Although cyclophosphamide and prednisolone were administered, his renal function deteriorated and progressed to end stage renal disease requiring maintenance hemodialysis. As congophilic FGN has been recognized since 2018, Congo red staining and LMD/MS were performed. The Congo red staining was positive and LMD/MS results indicated that this was a case of AHL. CONCLUSIONS: We reported a case of µ and κ chain AHL resembling FGN requiring LMD/MS for definitive diagnosis. Since FGN and amyloidosis exhibit pathological findings, even if Congo red staining is positive, LMD/MS needs to be considered in cases atypical pathological findings, such as silver stain positivity or thicker fibrils.


Assuntos
Glomerulonefrite/diagnóstico , Cadeias Pesadas de Imunoglobulinas/metabolismo , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Imunoglobulina M/metabolismo , Síndrome Nefrótica/etiologia , Idoso , Diagnóstico Diferencial , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/metabolismo , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Masculino , Síndrome Nefrótica/patologia , Espectrometria de Massas em Tandem
19.
Clin Exp Nephrol ; 24(5): 465-473, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31933049

RESUMO

BACKGROUND: Asymptomatic blood pressure (BP) elevation may be associated with cerebral hemorrhage (CH); however, few studies have investigated this association. We aimed to evaluate BP elevation before CH in hemodialysis (HD) patients and elucidate its associated factors. METHODS: We reviewed HD patients treated for CH at our hospital between 2008 and 2019 (CH group). The control group comprised HD patients treated at Nagasaki Renal Center between 2011 and 2012. Data were obtained from medical records and three consecutive HD charts, made immediately before CH. HD1 was the session closest to onset, followed by HD2 and HD3. Systolic and mean BP were evaluated at the beginning of HD, and factors associated with BP elevation were investigated. RESULTS: The CH and control groups included 105 and 339 patients, respectively. Systolic and mean BP at HD1 were significantly higher than those at baseline (HD2 + HD3) in the CH group by 5 and 3 mmHg, respectively (P < 0.001). Multiple linear regression analysis showed that lower calcium levels were significantly associated with BP elevation in the CH group (P < 0.05). The CH group was sub-divided by June 2013; the latter group had lower calcium levels (9.2 mg/dL) and a marked systolic BP difference from baseline (+ 10 mmHg) compared with the former (9.5 mg/dL and - 4 mmHg). CONCLUSION: Asymptomatic BP elevation was observed in HD patients before CH; this elevation was associated with lower serum calcium levels and observed more frequently in the recent era. The precise mechanism underlying this effect remains unknown.


Assuntos
Pressão Sanguínea , Cálcio/sangue , Hemorragia Cerebral/etiologia , Hipocalcemia/fisiopatologia , Diálise Renal/efeitos adversos , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sístole
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