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1.
J Infect Chemother ; 30(8): 812-814, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38316186

RESUMO

Low-density lipoprotein apheresis (LDL-A) is a blood purification therapy used to treat refractory ulcers in patients with arteriosclerosis obliterans. We describe a case of vancomycin treatment in a patient undergoing maintenance hemodialysis and LDL-A therapy and assess its impact on serum vancomycin concentration. The patient underwent LDL-A twice a week (Mondays and Fridays) and maintenance dialysis three times a week (Tuesdays, Thursdays, and Saturdays) for diabetic nephropathy associated with type 1 diabetes mellitus. Following the wound culture results, vancomycin was initiated with a 1.75 g administration post-dialysis. Serum vancomycin levels before and after LDL-A, measured on the subsequent day, exhibited only slight fluctuations within the intermeasurement variability range. Despite continuing vancomycin administration at the standard dose in patients undergoing hemodialysis, the serum concentration remained consistent, suggesting a minimal impact of LDL-A on vancomycin pharmacokinetics.


Assuntos
Antibacterianos , Remoção de Componentes Sanguíneos , Lipoproteínas LDL , Diálise Renal , Vancomicina , Humanos , Vancomicina/sangue , Vancomicina/uso terapêutico , Vancomicina/farmacocinética , Remoção de Componentes Sanguíneos/métodos , Antibacterianos/sangue , Antibacterianos/uso terapêutico , Antibacterianos/farmacocinética , Antibacterianos/administração & dosagem , Lipoproteínas LDL/sangue , Masculino , Nefropatias Diabéticas/terapia , Nefropatias Diabéticas/sangue , Diabetes Mellitus Tipo 1/sangue , Diabetes Mellitus Tipo 1/tratamento farmacológico , Diabetes Mellitus Tipo 1/terapia , Diabetes Mellitus Tipo 1/complicações , Pessoa de Meia-Idade
2.
J Lipid Res ; 61(12): 1776-1783, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33037132

RESUMO

Lipopolysaccharide (LPS) is a key player for innate immunity activation. It is therefore a prime target for sepsis treatment, as antibiotics are not sufficient to improve outcome during septic shock. An extracorporeal removal method by polymyxin (PMX) B direct hemoperfusion (PMX-DHP) is used in Japan, but recent trials failed to show a significant lowering of circulating LPS levels after PMX-DHP therapy. PMX-DHP has a direct effect on LPS molecules. However, LPS is not present in a free form in the circulation, as it is mainly carried by lipoproteins, including LDLs. Lipoproteins are critical for physiological LPS clearance, as LPSs are carried by LDLs to the liver for elimination. We hypothesized that LDL apheresis could be an alternate method for LPS removal. First, we demonstrated in vitro that LDL apheresis microbeads are almost as efficient as PMX beads to reduce LPS concentration in LPS-spiked human plasma, whereas it is not active in PBS. We found that PMX was also adsorbing lipoproteins, although less specifically. Then, we found that endogenous LPS of patients treated by LDL apheresis for familial hypercholesterolemia is also removed during their LDL apheresis sessions, with both electrostatic-based devices and filtration devices. Finally, LPS circulating in the plasma of septic shock and severe sepsis patients with gram-negative bacteremia was also removed in vitro by LDL adsorption. Overall, these results underline the importance of lipoproteins for LPS clearance, making them a prime target to study and treat endotoxemia-related conditions.


Assuntos
Hemoperfusão , Lipopolissacarídeos/sangue , Lipopolissacarídeos/isolamento & purificação , Sepse/sangue , Sepse/terapia , Adulto , Feminino , Voluntários Saudáveis , Humanos , Masculino
3.
Blood Purif ; 49(5): 513-523, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32074606

RESUMO

Focal segmental glomerulosclerosis (FSGS) is one of the most frequent and severe glomerular kidney disease with frequent progression to end-stage renal disease and a high rate of recurrence in renal transplantations. Due to intolerance or resistance to the current immunomodulatory treatments, the management of FSGS is a therapeutic challenge. Over the last few years, development in extracorporeal therapies has shown potential beneficial outcomes in drug-resistant and recurrent FSGS patients. Thus, this study reviews the current literature on the use of extracorporeal therapies, such as plasma exchange therapy, immunoadsorption, and low-density lipoprotein apheresis, for the treatment of FSGS in the pediatric population.


Assuntos
Glomerulosclerose Segmentar e Focal/terapia , Troca Plasmática , Plasmaferese , Humanos
4.
Pediatr Nephrol ; 34(10): 1655-1669, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-30218191

RESUMO

Low-density lipoprotein (LDL) apheresis has been used increasingly in clinical practice for the treatment of renal diseases with nephrotic syndrome (NS), specifically focal segmental glomerulosclerosis (FSGS). Persistent hyperlipidemia for prolonged periods is nephrotoxic and leads to chronic progressive glomerular and tubulointerstitial injury. Effective management of hyperlipidemia with HMG-CoA reductase inhibitors or LDL apheresis in drug-resistant NS patients may prevent the progression of renal disease and, in some patients, resolution of NS symptoms. Available literature reveals beneficial effects of LDL apheresis for NS refractory to drug therapy. Here we update on the current understanding of lipid nephrotoxicity and application of LDL apheresis to prevent progression of renal diseases.


Assuntos
Remoção de Componentes Sanguíneos/métodos , Glomerulosclerose Segmentar e Focal/terapia , Hiperlipidemias/terapia , Lipoproteínas LDL/metabolismo , Síndrome Nefrótica/terapia , Animais , Criança , Modelos Animais de Doenças , Progressão da Doença , Glomerulosclerose Segmentar e Focal/sangue , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/fisiopatologia , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Hiperlipidemias/sangue , Hiperlipidemias/etiologia , Glomérulos Renais/metabolismo , Glomérulos Renais/fisiopatologia , Metabolismo dos Lipídeos , Lipoproteínas LDL/sangue , Síndrome Nefrótica/sangue , Síndrome Nefrótica/etiologia , Eliminação Renal/fisiologia , Resultado do Tratamento
5.
J Clin Apher ; 33(3): 259-264, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28940696

RESUMO

INTRODUCTION: A therapeutic apheresis (TA) database helps to increase knowledge about indications and type of apheresis procedures that are performed in clinical practice. The objective of the present report was to describe the type and number of TA procedures that were performed at our institution in a 10-year period, from 2007 to 2016. MATERIAL AND METHODS: The TA electronic database was created by transferring patient data from electronic medical records and consultation forms into a Microsoft Access database developed exclusively for this purpose. Since 2007, prospective data from every TA procedure were entered in the database. RESULTS: A total of 5940 TA procedures were performed: 3762 (63.3%) plasma exchange (PE) procedures, 1096 (18.5%) hematopoietic progenitor cell (HPC) collections, and 1082 (18.2%) TA procedures other than PEs and HPC collections. The overall trend for the time-period was progressive increase in total number of TA procedures performed each year (from 483 TA procedures in 2007 to 822 in 2016). The tracking trend of each procedure during the 10-year period was different: the number of PE and other type of TA procedures increased 22% and 2818%, respectively, and the number of HPC collections decreased 28%. CONCLUSION: The TA database helped us to increase our knowledge about various indications and type of TA procedures that were performed in our current practice. We also believe that this database could serve as a model that other institutions can use to track service metrics.


Assuntos
Remoção de Componentes Sanguíneos/métodos , Bases de Dados Factuais , Remoção de Componentes Sanguíneos/estatística & dados numéricos , Bases de Dados Factuais/estatística & dados numéricos , Conjuntos de Dados como Assunto , Registros Eletrônicos de Saúde , Células-Tronco Hematopoéticas/citologia , Humanos , Troca Plasmática
6.
Clin Exp Nephrol ; 21(2): 228-235, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27101825

RESUMO

BACKGROUND: Corticosteroids have been widely used in patients with cholesterol crystal embolism (CCE) and low-density lipoprotein apheresis (LDL-A) was reported to reduce the risk of end-stage renal disease in patients with CCE. This study was designed to evaluate the renoprotective effects of LDL-A in combination with corticosteroids in patients with CCE. METHODS: Thirty-five patients with CCE who, between 2008 and 2013, had shown renal deterioration after vascular interventions were retrospectively evaluated. All patients received corticosteroids; of these, 24 also received LDL-A and 11 did not, designated LDL-A and control groups, respectively. Differences in eGFR (ΔeGFR), 3 months and 1 year after CCE diagnosis, were compared in the two groups. RESULTS: The median estimated glomerular filtration rate (eGFR) in all patients was 38.9 [interquartile range (IQR) 31.9-49.4] ml/min/1.73 m2 at baseline (before vascular intervention). At diagnosis, it was 14.4 (IQR 11.3-21.8) ml/min/1.73 m2. The initial corticosteroid dose was 0.34 ± 0.10 mg/kg/day. The mean number of LDL-A treatment sessions in the LDL-A group was 4.3 ± 1.8. eGFR was increased significantly after LDL-A treatments, from 15.0 (IQR 12.3-20.1) to 19.6 (IQR 14.3-23.6) ml/min/1.73 m2 (P < 0.05). ΔeGFR tended to be higher in the LDL-A than in the control group at 3 months [median 6.5 (IQR 5.1-9.3) vs. 2.6 (IQR -0.6 to 6.3) ml/min/1.73 m2, P = 0.095] and was significantly higher at 1 year [median 7.5 (IQR 5.4-8.7) vs. 2.2 (IQR -3.8 to 5.1) ml/min/1.73 m2, P = 0.019]. CONCLUSIONS: LDL-A plus corticosteroids may restore deteriorated renal function better than corticosteroids alone in patients with CCE.


Assuntos
Corticosteroides/uso terapêutico , Remoção de Componentes Sanguíneos/métodos , LDL-Colesterol/sangue , Embolia de Colesterol/terapia , Falência Renal Crônica/prevenção & controle , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Terapia Combinada , Cristalização , Embolia de Colesterol/sangue , Embolia de Colesterol/complicações , Embolia de Colesterol/diagnóstico , Feminino , Taxa de Filtração Glomerular , Humanos , Rim/fisiopatologia , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/etiologia , Falência Renal Crônica/fisiopatologia , Masculino , Pessoa de Meia-Idade , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
7.
J Lipid Res ; 55(10): 2064-72, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25121984

RESUMO

Lipoprotein-apheresis (apheresis) removes LDL-cholesterol in patients with severe dyslipidemia. However, reduction is transient, indicating that the long-term cardiovascular benefits of apheresis may not solely be due to LDL removal. Microparticles (MPs) are submicron vesicles released from the plasma membrane of cells. MPs, particularly platelet-derived MPs, are increasingly being linked to the pathogenesis of many diseases. We aimed to characterize the effect of apheresis on MP size, concentration, cellular origin, and fatty acid concentration in individuals with familial hypercholesterolemia (FH). Plasma and MP samples were collected from 12 individuals with FH undergoing routine apheresis. Tunable resistive pulse sensing (np200) and nanoparticle tracking analysis measured a fall in MP concentration (33 and 15%, respectively; P < 0.05) pre- to post-apheresis. Flow cytometry showed MPs were predominantly annexin V positive and of platelet (CD41) origin both pre- (88.9%) and post-apheresis (88.4%). Fatty acid composition of MPs differed from that of plasma, though apheresis affected a similar profile of fatty acids in both compartments, as measured by GC-flame ionization detection. MP concentration was also shown to positively correlate with thrombin generation potential. In conclusion, we show apheresis nonselectively removes annexin V-positive platelet-derived MPs in individuals with FH. These MPs are potent inducers of coagulation and are elevated in CVD; this reduction in pathological MPs could relate to the long-term benefits of apheresis.


Assuntos
Remoção de Componentes Sanguíneos , Micropartículas Derivadas de Células/metabolismo , LDL-Colesterol/sangue , Hiperlipoproteinemia Tipo II/sangue , Hiperlipoproteinemia Tipo II/terapia , Idoso , Feminino , Citometria de Fluxo , Humanos , Masculino , Pessoa de Meia-Idade
8.
Intern Med ; 63(3): 433-438, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-37258157

RESUMO

We herein report three cases of steroid-resistant nephrotic syndrome successfully treated with low-density lipoprotein apheresis (LDL-A). All patients were treated with a combination of steroids, cyclosporine, and LDL-A. In all cases, the serum concentrations of LDL, total and high-density lipoprotein cholesterol, and triglycerides were significantly lowered following LDL-A administration. Furthermore, the estimated LDL receptor activity increased, while both serum LDL and total cholesterol levels decreased, suggesting that LDL-A increases LDL receptor activity by driving changes in serum cholesterol concentration. This case series suggests that LDL-A increases LDL receptor activity, which may improve the intracellular uptake of cyclosporine.


Assuntos
Remoção de Componentes Sanguíneos , Glomerulosclerose Segmentar e Focal , Síndrome Nefrótica , Humanos , Síndrome Nefrótica/tratamento farmacológico , Lipoproteínas LDL/uso terapêutico , Ciclosporina/uso terapêutico , Apolipoproteínas/uso terapêutico , Receptores de LDL , Progressão da Doença , Colesterol
9.
Front Neurol ; 15: 1342751, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38510381

RESUMO

Objectives: To investigate the safety and efficacy of the delipid extracorporeal lipoprotein filter from plasma (DELP) system, a new low-density lipoprotein cholesterol (LDL-C) adsorption system, in acute ischemic stroke (AIS) patients. Patients and methods: In the present study, a total of 180 AIS patients were enrolled during March 2019 to February 2021. They were divided into DELP group (n1 = 90) and the control group (n2 = 90). The treatment protocol and vascular access of DELP treatment was established and evaluated. For the DELP group, clinical data and laboratory results including plasma lipid and safety parameters before and after the apheresis were collected and analyzed. For all participants, neurological scores were assessed and recorded. Results: For the DELP group, 90 patients including 70 males and 20 females were included. The mean LDL-C was significantly decreased from 3.15 ± 0.80 mmol/L to 2.18 ± 0.63 mmol/L (30.79%, p < 0.001) during a single DELP treatment, and decreased from 3.42 ± 0.87 mmol/L to 1.87 ± 0.48 mmol/L (45.32%, p < 0.001) after two DELP treatments. No clinically relevant changes were observed in hematologic safety parameters and blood pressure levels except for hematocrit and total protein throughout the whole period of DELP treatment. The DELP group showed improvement relative to the control group in National Institute of Health stroke scale scores (NIHSS) on the 14th and 90th day after stroke. Moreover, the DELP group had a significantly higher ratio of mRS 0 to 1 on the 90th day after stroke. Conclusion: The new LDL-C adsorption system, the DELP system, may provide a new option for intensive lipid lowering therapy in AIS patients in view of its safety, efficacy, and operation feasibility.

10.
J Clin Apher ; 28(4): 330-4, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23450792

RESUMO

Peripheral arterial disease (PAD) is very common in dialysis patients, who tend to have diffuse calcification and severe peripheral arterial stenosis that make it difficult to treat limbs using only surgical or endovascular interventions. Better ways to treat this condition are therefore required and also follow-up studies to evaluate the effects of these treatments on the microcirculation. A 59-year-old man who had a cadaveric kidney transplant five years previously after 25 years of regular hemodialysis complained of pain at rest in his right lower limb and subsequently developed an intractable decubitus ulcer on his right fifth toe (Fontaine IV). Digital subtraction angiography revealed a severe obstruction of the right femoral artery and diffuse stenosis of the right superficial femoral artery. The patient underwent percutaneous transluminal angioplasty (PTA) and six sessions of low-density lipoprotein apheresis (LDL apheresis). At the end of these sessions his complaints were almost completely alleviated. The mean elevation in skin temperature after each session was (1.58 ± 0.99)°C [mean ± SD] over the right dorsalis pedis artery and (1.52 ± 0.88)°C at the tip of the right fifth toe. Ultrasound-measured blood flow rates in the right dorsalis pedis artery were 9.2 cm/s before PTA and 20.2 cm/s one month after PTA. Hemodialysis was resumed 3 days after the PTA due to contrast-induced nephropathy. The combination of PTA and LDL apheresis is useful for treating PAD in hemodialysis patients, with the changes in peripheral artery patency are able to be evaluated effectively by measuring skin temperature.


Assuntos
Angioplastia , Remoção de Componentes Sanguíneos , Lipoproteínas LDL/isolamento & purificação , Doença Arterial Periférica/terapia , Diálise Renal , Humanos , Lipoproteínas LDL/sangue , Masculino , Pessoa de Meia-Idade , Doença Arterial Periférica/fisiopatologia , Temperatura Cutânea
11.
Ther Apher Dial ; 27(6): 1000-1009, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37587685

RESUMO

INTRODUCTION: Patients with peripheral arterial disease (PAD) have a poorer prognosis than those without PAD. PAD complications worsen the prognosis of patients with chronic kidney disease (CKD), especially those on maintenance dialysis. Although low-density lipoprotein apheresis (LDL-A) is expected to be effective in treating severe PAD, there are no large-scale reports on the prognosis of patients undergoing LDL-A. METHODS: We obtained a clinical database from April 2008 to August 2021 and selected 924 238 patients with CKD. We selected patients with disease codes of lower limb arteriosclerosis obliterans, arteriosclerosis obliterans, and critical limb ischemia or foot ulcer. Patients who were prescribed antithrombotic medications were included. Patients who used steroids were excluded. Among these patients, those undergoing blood purification considered LDL-A were selected, and their current status was investigated. RESULTS: We included 147 patients (113 males and 34 females). The mean patient age was 70 ± 10 years. Diabetes mellitus was present in 86%, ischemic heart disease in 34%, and stroke in 48%. Maintenance dialysis patients accounted for 86% of the patients. Statins were administered to 40% of the patients, and bypass surgery was performed in 2.7%. The median observation period was 812 days, and the mortality rate was 41%. CONCLUSION: LDL-A was performed in a small population of patients with CKD with the most severe form of PAD. The prognosis for these patients is extremely poor. Therefore, strategies to improve prognosis are important.


Assuntos
Remoção de Componentes Sanguíneos , Doença Arterial Periférica , Insuficiência Renal Crônica , Masculino , Feminino , Humanos , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , População do Leste Asiático , Doença Arterial Periférica/terapia , Insuficiência Renal Crônica/terapia , Lipoproteínas LDL
12.
J Atheroscler Thromb ; 30(5): 531-557, 2023 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-36682777

RESUMO

As atherosclerosis begins in childhood, early diagnosis and treatment of familial hypercholesterolemia (FH) is considered necessary. The basic diagnosis of pediatric FH (under 15 years of age) is based on hyper-low-density lipoprotein (LDL) cholesterolemia and a family history of FH; however, in this guideline, to reduce overlooked cases, "probable FH" was established. Once diagnosed with FH or probable FH, efforts should be made to promptly provide lifestyle guidance, including diet. It is also important to conduct an intrafamilial survey, to identify family members with the same condition. If the level of LDL-C remains above 180 mg/dL, drug therapy should be considered at the age of 10. The first-line drug should be statin. Evaluation of atherosclerosis should be started using non-invasive techniques, such as ultrasound. The management target level is an LDL-C level of less than 140 mg/dL. If a homozygous FH is suspected, consult a specialist and determine the response to pharmacotherapy with evaluating atherosclerosis. If the response is inadequate, initiate lipoprotein apheresis as soon as possible.


Assuntos
Aterosclerose , Inibidores de Hidroximetilglutaril-CoA Redutases , Hiperlipoproteinemia Tipo II , Humanos , Criança , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/tratamento farmacológico , Hiperlipoproteinemia Tipo II/genética , Aterosclerose/diagnóstico , Aterosclerose/tratamento farmacológico , Remoção de Componentes Sanguíneos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Guias como Assunto , LDL-Colesterol
13.
Ther Apher Dial ; 26(6): 1289-1295, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35352478

RESUMO

BACKGROUND: Low-density lipoprotein apheresis is not specific to lipoproteins but removes immunoglobulins as well. It remains elusive, whether protective SARS-CoV-2 antibodies after vaccination from COVID-19 are eliminated as well. METHODS: A cross-sectional case-control study on 55 patients undergoing weekly lipoprotein apheresis and 21 patients with comparable comorbidities and epidemiology not undergoing apheresis. SARS-CoV-2 IgG was assessed in all patients prior to apheresis and in 38 patients both before and after apheresis. RESULTS: SARS-CoV-2 IgG concentrations before a session of lipoprotein apheresis were comparable to control patients not undergoing apheresis(1727 IU/ml, IQR 365-2500) vs. 1652 IU/ml,(IQR408.8-2500), p = 0.78). SARS-CoV-2 IgG concentrations were reduced by lipoprotein apheresis from 1656 IU/ml(IQR 540.5-2500) prior to 1305 IU/ml (IQR 449-2500) afterwards(p < 0.0001). CONCLUSION: Lipoprotein apheresis removes SARS-CoV-2 IgG. The average elimination rate was 21.2%. In the present population of patients undergoing apheresis once weekly, however, the elimination did not lead to inferior concentrations compared to patients not undergoing lipoprotein apheresis.


Assuntos
Remoção de Componentes Sanguíneos , COVID-19 , Humanos , SARS-CoV-2 , Estudos de Casos e Controles , Estudos Transversais , COVID-19/terapia , Lipoproteínas , Lipoproteínas LDL , Imunoglobulina G
14.
Intern Med ; 61(12): 1863-1867, 2022 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-34803098

RESUMO

A 39-year-old woman was hospitalized for nephrotic syndrome. Laboratory test results showed increased serum creatinine levels and urinary excretions of beta-2-microglobulin, and N-acetyl-beta-D-glucosaminidase. A renal biopsy revealed collapsing focal segmental glomerulosclerosis (FSGS) and acute interstitial nephritis. Despite treatment with pulse steroid followed by oral high-dose glucocorticoids and cyclosporines, heavy proteinuria persisted. After low-density lipoprotein apheresis (LDL-A) therapy was initiated, her proteinuria gradually decreased, leading to complete remission. A repeat renal biopsy after treatment revealed no collapsing glomeruli. Immediate LDL-A should be performed to treat cases of collapsing FSGS poorly responding to other treatments.


Assuntos
Glomerulosclerose Segmentar e Focal , Nefrite Intersticial , Síndrome Nefrótica , Adulto , Feminino , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/terapia , Humanos , Glomérulos Renais/patologia , Nefrite Intersticial/complicações , Nefrite Intersticial/terapia , Síndrome Nefrótica/complicações , Síndrome Nefrótica/terapia , Proteinúria/complicações
15.
CEN Case Rep ; 11(1): 43-49, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34287815

RESUMO

Immune thrombocytopenia (ITP) may lead to membranous nephropathy (MN). Here, we report a case of MN complicated by ITP and validate the hypothesis that circulating antiplatelet antibodies cause MN using immunofluorescence analysis for immunoglobulin (Ig) G subclass and anti-phospholipase A2 receptor (PLA2R) antibodies. A 39-year-old Japanese man with ITP, who had been treated with prednisolone for 10 months, achieved a stable disease condition. However, 4 months after tapering the dose down to 10 mg prednisolone, he developed nephrotic syndrome, with a urinary protein-to-creatinine ratio (U-PCR) of 10.6 g/g Cr and was admitted to our hospital. His platelet count, at 89,000/µL, was lower than the normal range, indicating the recurrence of ITP. Renal biopsy revealed the thickening of the glomerular basement membrane with the deposition of IgG and complement component 3. Predominant deposition of IgG1 and negativity for anti-PLA2R staining indicated secondary MN; however, no typical conditions of secondary MN were evident. Although oral prednisolone and cyclosporine A were administered, he was refractory to treatment. A total of 12 sessions of low-density lipoprotein apheresis (LDL-A) decreased his U-PCR to < 3 g/g Cr. Seven months after discharge, his U-PCR further decreased to 0.54 g/g Cr and platelet count recovered to > 200,000/µL. Our literature review reveals that this condition is refractory to steroid therapy. LDL-A can be an effective treatment in drug-resistant MN complicated by ITP.


Assuntos
Remoção de Componentes Sanguíneos , Glomerulonefrite Membranosa , Púrpura Trombocitopênica Idiopática , Trombocitopenia , Adulto , Glomerulonefrite Membranosa/complicações , Glomerulonefrite Membranosa/terapia , Humanos , Imunoglobulina G , Lipoproteínas LDL , Masculino , Prednisolona/uso terapêutico , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Trombocitopenia/terapia
16.
Eur J Prev Cardiol ; 27(2): 157-165, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31403880

RESUMO

AIMS: The aim of this study was to evaluate the effect of microsomal triglyceride transfer protein inhibitor (lomitapide) in patients with homozygous familial hypercholesterolaemia. METHODS AND RESULTS: In 12 homozygous familial hypercholesterolaemia patients treated with lipid-lowering drugs ± biweekly lipoprotein apheresis sessions (nine patients), daily lomitapide was added. The lipid profile (total cholesterol, low-density lipoprotein cholesterol, triglycerides, high-density lipoprotein cholesterol) before and after lomitapide treatment was evaluated. The follow-up period with lomitapide treatment was 3-24 months (13.8 ± 7.9). The median baseline low-density lipoprotein cholesterol level was 900 mg/dl (348-1070), after lipid-lowering drugs therapy was 383.5 mg/dl (214-866) and after lipid-lowering drugs + time-averaged level was 288 mg/dl (183.7-716.6). The addition of lomitapide lowered low-density lipoprotein cholesterol levels further by 56.8% compared to lipid-lowering drugs alone (mean reduction 262, 95% confidence interval (105.5-418.7), p = 0.005) and by 54% (mean reduction 182.9, 95% confidence interval (-342 - -23), p = 0.031) comparing to lipid-lowering drugs + lipoprotein apheresis (time-averaged level). The time-averaged level of low-density lipoprotein cholesterol in lipid-lowering drugs + lipoprotein apheresis patients compared with lipid-lowering drugs + lomitapide was 54% in favour of lomitapide (p = 0.031). CONCLUSIONS: Treatment with lomitapide in homozygous familial hypercholesterolaemia patients has a beneficial effect with a constant decrease of low-density lipoprotein cholesterol by 57% compared with classical lipid-lowering therapy and by 54% compared with classical lipid-lowering therapy and time-averaged level of low-density lipoprotein cholesterol.


Assuntos
Anticolesterolemiantes/uso terapêutico , Benzimidazóis/uso terapêutico , Proteínas de Transporte/antagonistas & inibidores , LDL-Colesterol/sangue , Homozigoto , Hiperlipoproteinemia Tipo II/tratamento farmacológico , Adolescente , Adulto , Anticolesterolemiantes/efeitos adversos , Benzimidazóis/efeitos adversos , Biomarcadores/sangue , Remoção de Componentes Sanguíneos , Criança , HDL-Colesterol/sangue , Feminino , Predisposição Genética para Doença , Humanos , Hiperlipoproteinemia Tipo II/sangue , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/genética , Masculino , Pessoa de Meia-Idade , Fenótipo , Fatores de Tempo , Resultado do Tratamento , Triglicerídeos/sangue , Adulto Jovem
17.
Int J Nephrol Renovasc Dis ; 13: 157-162, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32606890

RESUMO

Low-density lipoprotein apheresis (LDL-A) has been developed as a therapy for familial hypercholesterolemia, but LDL-A has also been used as a general treatment for drug-resistant nephrotic syndrome (NS) due to focal segmental glomerulosclerosis (FSGS). The patients with NS due to minimal change disease (MCD) are often difficult to control effective circulating plasma volume, causes acute kidney injury (AKI), and when diuretics are not effective and the respiratory condition of patients worsens, patients require acute renal replacement therapy (ARRT). The effectiveness of LDL-A is not only reduction of serum low-density lipoprotein but also various other benefits. LDL-A might have improved renal hemodynamics by reducing vasoconstrictive eicosanoids and contributed to the therapeutic effect of antiproteinuric drugs such as corticosteroids. We treated a 49-year-old Japanese woman and a 71-year-old Japanese man with AKI caused by NS due to MCD, who required ARRT. Although these patients received ARRT and corticosteroids, their AKI and MCD did not improve sufficiently. We initiated LDL-A treatment for these patients as an additional treatment modality, because their total serum cholesterol levels were high at the time of admission. After the additional LDL-A treatment, both patients were able to discontinue ARRT, because NS and AKI in both patients were improved sufficiently. It is possible that early additional LDL-A is effective for patients with AKI and NS due to MCD who require ARRT, and may help patients discontinue ARRT because of the effect of LDL-A such as improving hypercoagulability and renal hemodynamics and contributing to the therapeutic effect of corticosteroids.

18.
J Cardiol Cases ; 19(2): 55-58, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31193688

RESUMO

We report here a familial hypercholesterolemia (FH) patient with a rare mutation, exon 2-6 duplication in the low-density lipoprotein (LDL) receptor gene, who had received LDL apheresis with drug treatment for 15 years. We added evolocumab (proprotein convertase subtilisin/kexin type 9 inhibitor) 140 mg bi-weekly to the treatment, and checked lipid profiles [LDL cholesterol, lipoprotein(a), malondialdehyde-modified LDL, etc.] for 34 weeks. Evolocumab enabled the patient to discontinue LDL apheresis and decrease the dose of statin. We demonstrate that evolocumab contributed to the management of atherogenic lipoproteins in an FH patient with exon 2-6 duplication as an alternative to LDL apheresis. .

19.
Ther Apher Dial ; 23(6): 575-583, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30993827

RESUMO

Low-density lipoprotein apheresis (LDL-A) has been used for nephrotic syndrome (NS) caused by focal segmental glomerulosclerosis in Japan. Idiopathic membranous nephropathy (iMN) can also cause treatment-resistant NS. Therefore, we investigated the effect of LDL-A during initial induction for it. This retrospective, observational, and single-center study enrolled consecutive iMN patients who received steroids from March 2000 to May 2015. We compared data between 11 patients treated with LDL-A (LDL-A group) and 27 patients without (non-LDL-A group) at baseline and 4 and 8 weeks later. Reduction rate of proteinuria and increase rate of serum albumin in LDL-A group were significantly higher than the other after 4 weeks (P = 0.036 and 0.030) and 8 weeks (P = 0.030 and <0.001), respectively. There was no adverse event caused by LDL-A and immunosuppressant dose was not significantly different. In conclusion, LDL-A may be an effective choice for initial induction of nephrotic iMN.


Assuntos
Remoção de Componentes Sanguíneos/métodos , Glomerulonefrite Membranosa/terapia , Imunossupressores/administração & dosagem , Lipoproteínas LDL/sangue , Idoso , Feminino , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Proteinúria/etiologia , Proteinúria/terapia , Estudos Retrospectivos , Albumina Sérica Humana/metabolismo , Esteroides/administração & dosagem , Resultado do Tratamento
20.
Intern Med ; 56(12): 1531-1535, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28626179

RESUMO

Two elderly patients (a 76-year-old man and a 75-year-old woman), who had been previously diagnosed with familial hypercholesterolemia (at 58 and 48 years of age, respectively) underwent long-term treatment with oral therapy and low-density lipoprotein (LDL) apheresis. As their LDL cholesterol levels remained high (>150 mg/dL and >120 mg/dL, respectively) and their familial hypercholesterolemia was complicated with angina pectoris, we added evolocumab to their prescription. Thereafter, their LDL cholesterol levels decreased rapidly, and the patients were successfully weaned from LDL apheresis. Evolocumab therapy should thus be considered when LDL apheresis cannot achieve the target LDL cholesterol levels, though the prognosis of such treatment remains unclear.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Anticolesterolemiantes/uso terapêutico , Remoção de Componentes Sanguíneos/métodos , Hiperlipoproteinemia Tipo II/terapia , Idoso , Angina Pectoris/complicações , Anticorpos Monoclonais Humanizados , LDL-Colesterol/sangue , Feminino , Humanos , Hiperlipoproteinemia Tipo II/complicações , Hiperlipoproteinemia Tipo II/tratamento farmacológico , Lipoproteínas LDL/sangue , Masculino
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