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1.
Clin Immunol ; 219: 108555, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32771488

RESUMO

Respiratory failure and acute kidney injury (AKI) are associated with high mortality in SARS-CoV-2-associated Coronavirus disease 2019 (COVID-19). These manifestations are linked to a hypercoaguable, pro-inflammatory state with persistent, systemic complement activation. Three critical COVID-19 patients recalcitrant to multiple interventions had skin biopsies documenting deposition of the terminal complement component C5b-9, the lectin complement pathway enzyme MASP2, and C4d in microvascular endothelium. Administration of anti-C5 monoclonal antibody eculizumab led to a marked decline in D-dimers and neutrophil counts in all three cases, and normalization of liver functions and creatinine in two. One patient with severe heart failure and AKI had a complete remission. The other two individuals had partial remissions, one with resolution of his AKI but ultimately succumbing to respiratory failure, and another with a significant decline in FiO2 requirements, but persistent renal failure. In conclusion, anti-complement therapy may be beneficial in at least some patients with critical COVID-19.


Assuntos
Lesão Renal Aguda/imunologia , Anticorpos Monoclonais Humanizados/uso terapêutico , Betacoronavirus/patogenicidade , Inativadores do Complemento/uso terapêutico , Infecções por Coronavirus/imunologia , Síndrome da Liberação de Citocina/imunologia , Pneumonia Viral/imunologia , Síndrome Respiratória Aguda Grave/imunologia , Lesão Renal Aguda/complicações , Lesão Renal Aguda/tratamento farmacológico , Lesão Renal Aguda/virologia , Adulto , Betacoronavirus/imunologia , Biomarcadores/metabolismo , Ativação do Complemento/efeitos dos fármacos , Complemento C4b/antagonistas & inibidores , Complemento C5/antagonistas & inibidores , Complexo de Ataque à Membrana do Sistema Complemento/antagonistas & inibidores , Infecções por Coronavirus/complicações , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/virologia , Síndrome da Liberação de Citocina/complicações , Síndrome da Liberação de Citocina/tratamento farmacológico , Síndrome da Liberação de Citocina/virologia , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/metabolismo , Humanos , Imunidade Humoral/efeitos dos fármacos , Masculino , Serina Proteases Associadas a Proteína de Ligação a Manose/genética , Serina Proteases Associadas a Proteína de Ligação a Manose/imunologia , Pessoa de Meia-Idade , Neutrófilos/imunologia , Neutrófilos/patologia , Pandemias , Fragmentos de Peptídeos/antagonistas & inibidores , Pneumonia Viral/complicações , Pneumonia Viral/tratamento farmacológico , Pneumonia Viral/virologia , Síndrome Respiratória Aguda Grave/complicações , Síndrome Respiratória Aguda Grave/tratamento farmacológico , Síndrome Respiratória Aguda Grave/virologia
3.
Anticancer Res ; 40(7): 3995-4000, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32620643

RESUMO

We present here the case of a 39-year-old man with metastatic pancreatic carcinoma receiving chemotherapy with the combination of gemcitabine and nab-paclitaxel as part of a clinical trial. Despite an impressive response to therapy, he ultimately developed profound anasarca, renal insufficiency, progressive cytopenias, and malignant hypertension 6 months into his treatment course. The diagnosis of gemcitabine-associated thrombotic microangiopathy (G-TMA) was made based on renal biopsy, and receipt of the anti-C5 monoclonal antibody eculizumab proved successful at reversing his deteriorating clinical course and improving his laboratory parameters. This case illustrates the importance of recognizing this rare but serious complication, and highlights one potential therapeutic option that can be used in the appropriate clinical context.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Antimetabólitos Antineoplásicos/efeitos adversos , Inativadores do Complemento/uso terapêutico , Desoxicitidina/análogos & derivados , Microangiopatias Trombóticas/tratamento farmacológico , Adulto , Albuminas/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Desoxicitidina/efeitos adversos , Evolução Fatal , Humanos , Masculino , Paclitaxel/uso terapêutico , Neoplasias Pancreáticas/tratamento farmacológico , Microangiopatias Trombóticas/induzido quimicamente
4.
Clin Immunol ; 217: 108493, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32526273

Assuntos
Complexo Antígeno-Anticorpo/biossíntese , Betacoronavirus/patogenicidade , Infecções por Coronavirus/imunologia , Síndrome da Liberação de Citocina/imunologia , Doenças do Complexo Imune/imunologia , Pneumonia Viral/imunologia , Síndrome Respiratória Aguda Grave/imunologia , Vasculite/imunologia , Anticorpos Antivirais/biossíntese , Complexo Antígeno-Anticorpo/efeitos dos fármacos , Betacoronavirus/imunologia , Vasos Sanguíneos/efeitos dos fármacos , Vasos Sanguíneos/imunologia , Vasos Sanguíneos/patologia , Vasos Sanguíneos/virologia , Complemento C3/antagonistas & inibidores , Complemento C3/biossíntese , Inativadores do Complemento/uso terapêutico , Infecções por Coronavirus/complicações , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/virologia , Síndrome da Liberação de Citocina/complicações , Síndrome da Liberação de Citocina/tratamento farmacológico , Síndrome da Liberação de Citocina/virologia , Humanos , Doenças do Complexo Imune/complicações , Doenças do Complexo Imune/tratamento farmacológico , Doenças do Complexo Imune/virologia , Imunidade Humoral/efeitos dos fármacos , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Interleucina-6/antagonistas & inibidores , Interleucina-6/biossíntese , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/tratamento farmacológico , Pneumonia Viral/virologia , Receptores de Interleucina-1/antagonistas & inibidores , Receptores de Interleucina-1/biossíntese , Síndrome Respiratória Aguda Grave/complicações , Síndrome Respiratória Aguda Grave/tratamento farmacológico , Síndrome Respiratória Aguda Grave/virologia , Índice de Gravidade de Doença , Vasculite/complicações , Vasculite/tratamento farmacológico , Vasculite/virologia
5.
Medicine (Baltimore) ; 99(22): e19731, 2020 May 29.
Artigo em Inglês | MEDLINE | ID: mdl-32481360

RESUMO

INTRODUCTION: Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by the sudden onset of hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). HUS is categorized as either typical, caused by Shiga toxin-producing Escherichia coli infection, or atypical HUS (aHUS), usually complement mediated or secondary to systemic disease. We describe a rare case of aHUS in an adult male patient with recurrent acute pancreatitis. PATIENT CLINICAL FINDINGS: A 32-year-old Caucasian male presented to our institution for his third episode of alcohol-induced pancreatitis. He presented with abdominal pain, elevated lipase and pancreatic inflammation on computed tomography consistent with acute pancreatitis. While admitted, he developed sudden onset severe thrombocytopenia, AKI and hemolytic anemia. DIAGNOSIS, THERAPEUTIC INTERVENTIONS, OUTCOMES: Peripheral blood smear, haptoglobin and hemoglobin level confirmed microangiopathic hemolytic anemia. Worsening anemia, thrombocytopenia and AKI were consistent with the diagnosis of aHUS. The patient's pancreatitis resolved with supportive measures, but resolution of significant thrombocytopenia and AKI was not achieved until administration of eculizumab, a complement inhibiting therapy. Eculizumab therapy provided dramatic improvement in this patient, with platelet count increasing from a low of 11,000 to >100,000 within 48 hours of therapy. Creatinine and hemoglobin levels returned to baseline within 3 weeks. CONCLUSION: Recurrent pancreatitis is suggested as the etiology of atypical HUS in this patient and this condition should be recognized and treated in a timely manner for optimal clinical outcomes.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Inativadores do Complemento/uso terapêutico , Síndrome Hemolítico-Urêmica/etiologia , Pancreatite/complicações , Adulto , Consumo de Bebidas Alcoólicas/efeitos adversos , Síndrome Hemolítico-Urêmica/tratamento farmacológico , Humanos , Masculino , Recidiva
6.
EMBO Mol Med ; 12(8): e12642, 2020 08 07.
Artigo em Inglês | MEDLINE | ID: mdl-32559343

RESUMO

A novel coronavirus, SARS-CoV-2, has recently emerged in China and spread internationally, posing a health emergency to the global community. COVID-19 caused by SARS-CoV-2 is associated with an acute respiratory illness that varies from mild to the life-threatening acute respiratory distress syndrome (ARDS). The complement system is part of the innate immune arsenal against pathogens, in which many viruses can evade or employ to mediate cell entry. The immunopathology and acute lung injury orchestrated through the influx of pro-inflammatory macrophages and neutrophils can be directly activated by complement components to prime an overzealous cytokine storm. The manifestations of severe COVID-19 such as the ARDS, sepsis and multiorgan failure have an established relationship with activation of the complement cascade. We have collected evidence from all the current studies we are aware of on SARS-CoV-2 immunopathogenesis and the preceding literature on SARS-CoV-1 and MERS-CoV infection linking severe COVID-19 disease directly with dysfunction of the complement pathways. This information lends support for a therapeutic anti-inflammatory strategy against complement, where a number of clinically ready potential therapeutic agents are available.


Assuntos
Betacoronavirus , Ativação do Complemento/efeitos dos fármacos , Inativadores do Complemento/uso terapêutico , Infecções por Coronavirus/tratamento farmacológico , Pandemias , Pneumonia Viral/tratamento farmacológico , Adulto , Células Epiteliais Alveolares/imunologia , Células Epiteliais Alveolares/metabolismo , Células Epiteliais Alveolares/virologia , Animais , Betacoronavirus/fisiologia , Criança , Complemento C3b/antagonistas & inibidores , Complemento C3b/fisiologia , Inativadores do Complemento/farmacologia , Infecções por Coronavirus/imunologia , Síndrome da Liberação de Citocina/tratamento farmacológico , Síndrome da Liberação de Citocina/etiologia , Síndrome da Liberação de Citocina/imunologia , Glicosilação , Humanos , Imunidade Inata , Ligantes , Camundongos , Modelos Animais , Modelos Moleculares , Reconhecimento Automatizado de Padrão , Peptidil Dipeptidase A/metabolismo , Pneumonia Viral/imunologia , Conformação Proteica , Processamento de Proteína Pós-Traducional , Receptores Virais/metabolismo , Síndrome do Desconforto Respiratório do Adulto/etiologia , Síndrome do Desconforto Respiratório do Adulto/imunologia , Glicoproteína da Espícula de Coronavírus/química , Glicoproteína da Espícula de Coronavírus/metabolismo
8.
Clin Immunol ; 215: 108450, 2020 06.
Artigo em Inglês | MEDLINE | ID: covidwho-172295

RESUMO

Acute respiratory distress syndrome (ARDS) is a devastating clinical manifestation of COVID-19 pneumonia and is mainly based on an immune-driven pathology. Mounting evidence suggests that COVID-19 is fueled by a maladaptive host inflammatory response that involves excessive activation of innate immune pathways. While a "cytokine storm" involving IL-6 and other cytokines has been documented, complement C3 activation has been implicated as an initial effector mechanism that exacerbates lung injury in preclinical models of SARS-CoV infection. C3-targeted intervention may provide broader therapeutic control of complement-mediated inflammatory damage in COVID-19 patients. Herein, we report the clinical course of a patient with severe ARDS due to COVID-19 pneumonia who was safely and successfully treated with the compstatin-based complement C3 inhibitor AMY-101.


Assuntos
Betacoronavirus/efeitos dos fármacos , Ativação do Complemento/efeitos dos fármacos , Complemento C3/antagonistas & inibidores , Inativadores do Complemento/uso terapêutico , Infecções por Coronavirus/tratamento farmacológico , Peptídeos Cíclicos/uso terapêutico , Pneumonia Viral/tratamento farmacológico , Idoso , Antivirais/uso terapêutico , Fibrilação Atrial/tratamento farmacológico , Fibrilação Atrial/imunologia , Fibrilação Atrial/patologia , Fibrilação Atrial/virologia , Betacoronavirus/imunologia , Betacoronavirus/patogenicidade , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/patologia , Infecções por Coronavirus/virologia , Humanos , Hipercolesterolemia/tratamento farmacológico , Hipercolesterolemia/imunologia , Hipercolesterolemia/patologia , Hipercolesterolemia/virologia , Hipertensão/tratamento farmacológico , Hipertensão/imunologia , Hipertensão/patologia , Hipertensão/virologia , Pulmão/efeitos dos fármacos , Pulmão/imunologia , Pulmão/patologia , Pulmão/virologia , Masculino , Pandemias , Pneumonia Viral/imunologia , Pneumonia Viral/patologia , Pneumonia Viral/virologia , Resultado do Tratamento
9.
Clin Immunol ; 215: 108450, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32360516

RESUMO

Acute respiratory distress syndrome (ARDS) is a devastating clinical manifestation of COVID-19 pneumonia and is mainly based on an immune-driven pathology. Mounting evidence suggests that COVID-19 is fueled by a maladaptive host inflammatory response that involves excessive activation of innate immune pathways. While a "cytokine storm" involving IL-6 and other cytokines has been documented, complement C3 activation has been implicated as an initial effector mechanism that exacerbates lung injury in preclinical models of SARS-CoV infection. C3-targeted intervention may provide broader therapeutic control of complement-mediated inflammatory damage in COVID-19 patients. Herein, we report the clinical course of a patient with severe ARDS due to COVID-19 pneumonia who was safely and successfully treated with the compstatin-based complement C3 inhibitor AMY-101.


Assuntos
Betacoronavirus/efeitos dos fármacos , Ativação do Complemento/efeitos dos fármacos , Complemento C3/antagonistas & inibidores , Inativadores do Complemento/uso terapêutico , Infecções por Coronavirus/tratamento farmacológico , Peptídeos Cíclicos/uso terapêutico , Pneumonia Viral/tratamento farmacológico , Idoso , Antivirais/uso terapêutico , Fibrilação Atrial/tratamento farmacológico , Fibrilação Atrial/imunologia , Fibrilação Atrial/patologia , Fibrilação Atrial/virologia , Betacoronavirus/imunologia , Betacoronavirus/patogenicidade , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/patologia , Infecções por Coronavirus/virologia , Humanos , Hipercolesterolemia/tratamento farmacológico , Hipercolesterolemia/imunologia , Hipercolesterolemia/patologia , Hipercolesterolemia/virologia , Hipertensão/tratamento farmacológico , Hipertensão/imunologia , Hipertensão/patologia , Hipertensão/virologia , Pulmão/efeitos dos fármacos , Pulmão/imunologia , Pulmão/patologia , Pulmão/virologia , Masculino , Pandemias , Pneumonia Viral/imunologia , Pneumonia Viral/patologia , Pneumonia Viral/virologia , Resultado do Tratamento
11.
Rev Med Suisse ; 16(689): 675-678, 2020 Apr 08.
Artigo em Francês | MEDLINE | ID: mdl-32270933

RESUMO

Hereditary angioedema type 1 and 2 are due to a deficiency in C1--esterase inhibitor. This molecule inhibits the generation of bradykinin, a potent inflammatory mediator that increases vascular permeability. Upon accumulation of bradykinin, patients affected develop painful subcutaneous or submucosal edemas that last for several days. In case the upper airways are affected, there is risk of suffocation. This type of angioedema does not respond to antihistamines, cortico-steroids or epinephrine. Management of angioedema attacks consists in injecting C1-esterase inhibitor concentrate or icatibant, a bradykinin receptor B2 antagonist. Preventive measures aim at reducing the frequency and the severity of angioedema attacks. Inhibition of -plasma kallikrein by lanadelumab, a monoclonal antibody adminis-tered subcutaneously, is effective and well tolerated.


Assuntos
Angioedema , Angioedemas Hereditários/enzimologia , Proteína Inibidora do Complemento C1/uso terapêutico , Esterases/antagonistas & inibidores , Angioedemas Hereditários/tratamento farmacológico , Anticorpos Monoclonais , Bradicinina/análogos & derivados , Bradicinina/uso terapêutico , Inativadores do Complemento/uso terapêutico , Humanos
13.
Curr Opin Ophthalmol ; 31(3): 215-221, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32205470

RESUMO

PURPOSE OF REVIEW: The purpose of this review is to describe the current clinical landscape of potential future therapies for both nonexudative (dry) and exudative (wet) age-related macular degeneration (AMD). We highlight some of the more promising treatments that are furthest along in development. RECENT FINDINGS: Patients with dry AMD have long been hoping for a highly efficacious treatment that may slow disease progression or even help regain vision. Patients with wet AMD have many effective treatment options but still there are those who have suboptimal responses or are burdened by the high frequency of treatment. We detail exciting new concepts and targets for novel medications. Specifically, for dry AMD we discuss research looking at complement inhibition, neuroprotection, visual cycle modulators, cell-based therapies, and anti-inflammatory agents. For wet AMD we summarize new, potentially more durable anti-vascular endothelial growth factor agents, extended release options, and gene therapy. SUMMARY: There are promising new strategies for AMD. Many of the potential new treatments are in or have recently completed phase 2 or phase 3 clinical trials with promising results thus far, including some that have received US Food and Drug Administration approval. Additional therapeutic breakthroughs will likely continue to occur thanks to the number of clinical trials that are nearing the finish line.


Assuntos
Atrofia Geográfica/tratamento farmacológico , Degeneração Macular Exsudativa/tratamento farmacológico , Inibidores da Angiogênese/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Terapia Baseada em Transplante de Células e Tecidos/métodos , Ensaios Clínicos como Assunto , Inativadores do Complemento/uso terapêutico , Terapia Genética , Humanos , Fármacos Neuroprotetores/uso terapêutico
14.
Intern Med ; 59(1): 93-99, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31902910

RESUMO

Atypical hemolytic uremic syndrome (aHUS) is an extremely rare condition caused by an excessive activation of the complement pathway based on genetic or acquired dysfunctions in complement regulation, leading to thrombotic microangiopathy (TMA). A complement-amplifying condition (CAC) can trigger aHUS occurrence along with complement abnormality. We herein report a case of severe TMA after laparoscopic myomectomy in a healthy woman. This case was eventually diagnosed as complement-mediated TMA secondary to surgical invasive stress as a CAC, with no definitive diagnosis of aHUS despite a genetic test. The patient fully recovered after several eculizumab administrations.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Laparoscopia/efeitos adversos , Hemorragia Pós-Operatória/complicações , Microangiopatias Trombóticas/tratamento farmacológico , Miomectomia Uterina/efeitos adversos , Adulto , Inativadores do Complemento/uso terapêutico , Feminino , Humanos , Doenças Raras , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/etiologia
15.
Transplant Proc ; 52(1): 146-152, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31924403

RESUMO

Atypical hemolytic uremic syndrome (aHUS) after kidney transplantation is rare and carries a grave outcome. We present a single-center experience of all aHUS cases since the program's inception. Six patients were diagnosed with aHUS, all after kidney transplants, except for 1 patient. All had nonreactive crossmatches. Delayed graft function (DGF) occurred in 2 patients. Five patients developed aHUS after transplant; 4 (80%) of these patients manifested aHUS ≤ 14 days. All were confirmed by allograft biopsy. Genetic testing was abnormal in all patients except for 1 patient. Actual patient and graft survival during the first year was 100% and 83.3%, respectively. A single graft was lost early in the study secondary to aHUS (eculizumab was not used in the treatment process). Prophylactic and therapeutic use of eculizumab salvaged all other cases. At 1 year, mean creatinine level was 1.9 mg/dL (range, 1.3-2.5). After 6 months of eculizumab treatment (halted in 2 cases) 1 patient had recurrence 2 months later and eculizumab was restarted. However, graft function continued to worsen, and the graft was ultimately lost at 20 months after kidney transplantation. High index of suspicion, prompt diagnosis, and utilization of eculizumab are key to successful salvage of allografts in cases of aHUS after kidney transplantation. aHUS can be prevented by prophylactic use of eculizumab. It still needs to be determined when and if eculizumab therapy can be safely discontinued.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome Hemolítico-Urêmica Atípica/tratamento farmacológico , Inativadores do Complemento/uso terapêutico , Transplante de Rim/efeitos adversos , Complicações Pós-Operatórias/tratamento farmacológico , Adulto , Síndrome Hemolítico-Urêmica Atípica/sangue , Síndrome Hemolítico-Urêmica Atípica/etiologia , Biópsia , Creatinina/sangue , Feminino , Sobrevivência de Enxerto , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/sangue , Complicações Pós-Operatórias/etiologia , Recidiva
16.
Obstet Gynecol ; 135(1): 46-58, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31809447

RESUMO

OBJECTIVE: To evaluate disease presentation, diagnosis, treatment, and clinical outcomes in pregnancy-associated atypical hemolytic uremic syndrome (aHUS). DATA SOURCES: We searched PubMed, MEDLINE, Cochrane Library, ClinicalTrials.gov, Web of Science, EMBASE and Google Scholar, from inception until March 2018. METHODS OF STUDY SELECTION: We included English-language articles describing aHUS in pregnancy or postpartum. The diagnosis of aHUS was characterized by hemolysis, thrombocytopenia, and renal failure and was distinguished from typical diarrhea-associated hemolytic uremic syndrome. Patients were excluded if individual data could not be obtained, the diagnosis was unclear, or an alternative etiology was more likely, such as thrombotic thrombocytopenic purpura or Shiga toxin-producing Escherichia coli. Reports were appraised by two reviewers, with disagreements adjudicated by a third reviewer. TABULATION, INTEGRATION, AND RESULTS: The search identified 796 articles. After review of titles, abstracts, and full text, we identified 48 reports describing 60 unique cases of pregnancy-associated aHUS, with 66 pregnancies. Twelve cases involved pregnancy in women with known aHUS, and 54 cases involved first-episode pregnancy-associated aHUS. Women with known aHUS, particularly those with baseline creatinine at or above 1.5 mg/dL, had a high rate of adverse pregnancy outcomes. For first-episode pregnancy-associated aHUS, diagnosis most often occurred postpartum (94%), after a cesarean delivery (70%), in nulliparous women (58%). Preceding obstetric complications were common and included fetal death, preeclampsia, and hemorrhage. Diagnosis was usually made clinically, based on the triad of microangiopathic hemolysis, thrombocytopenia, and renal failure. Additional testing included renal biopsy, complement genetic testing, and ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) testing. Treatment modalities included corticosteroids, plasma exchange, dialysis, and eculizumab. More women with first-episode pregnancy-associated aHUS achieved disease remission when treated with eculizumab, compared with those not treated with eculizumab (88% vs 57%, P=.02). CONCLUSION: Pregnancy-associated aHUS usually presents in the postpartum period, often after a pregnancy complication, and eculizumab is effective for achieving disease remission. SYSTEMATIC REVIEW REGISTRATION: PROSPERO, CRD42019129266.


Assuntos
Síndrome Hemolítico-Urêmica Atípica/diagnóstico , Síndrome Hemolítico-Urêmica Atípica/terapia , Complicações Hematológicas na Gravidez/diagnóstico , Complicações Hematológicas na Gravidez/terapia , Anticorpos Monoclonais Humanizados/uso terapêutico , Inativadores do Complemento/uso terapêutico , Feminino , Humanos , Troca Plasmática , Período Pós-Parto , Gravidez , Diálise Renal
17.
Eur J Ophthalmol ; 30(3): NP14-NP17, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-30841747

RESUMO

PURPOSE: To report a case of Purtscher-like retinopathy due to atypical hemolytic uremic syndrome and the changes seen in the optical coherence tomography angiography before and after treatment with eculizumab. CASE DESCRIPTION: A 22-year-old man with an unremarkable medical history presented with acute, bilateral blurred vision and headache of 1-week duration. Best corrected visual acuity of 20/50 and 20/40, respectively, in the patient's right eye and left eye. Funduscopy revealed multiple cotton-wool spots associated with intrarretinal fluid. Swept source optical coherence tomography revealed multifocal retinal detachments with increased choroidal thickness. Optical coherence tomography angiography showed areas of ischemia in both capillary plexus. Due to concurrent symptoms and laboratory analysis, he was diagnosed with atypical hemolytic uremic syndrome and secondary Purtscher-like retinopathy; therefore, treatment with eculizumab was initiated. After 2 months revascularization of the previous ischemic areas was seen in the optical coherence tomography angiography that were correlated with best corrected visual acuity improvement. CONCLUSION: Our findings suggest that evaluation of the macular capillary plexus revascularization by optical coherence tomography angiography during the disease could help to predict an improvement of best corrected visual acuity in these patients and the measurement of choroidal thickness could give us information about the resolution of the pathologic process.


Assuntos
Síndrome Hemolítico-Urêmica Atípica/complicações , Doenças Retinianas/diagnóstico , Doenças Retinianas/etiologia , Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome Hemolítico-Urêmica Atípica/diagnóstico , Síndrome Hemolítico-Urêmica Atípica/tratamento farmacológico , Inativadores do Complemento/uso terapêutico , Angiografia por Tomografia Computadorizada , Angiofluoresceinografia/métodos , Seguimentos , Humanos , Masculino , Doenças Retinianas/tratamento farmacológico , Tomografia de Coerência Óptica/métodos , Acuidade Visual , Adulto Jovem
18.
Clin Nephrol ; 93(1): 51-56, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31661064

RESUMO

C3 glomerulonephritis (C3GN) is a disorder of excess alternative complement activation leading to glomerular injury. Following kidney transplantation, C3GN has a high recurrence rate, and the overall prognosis is poor without treatment. However, treatment efficacy is highly variable. Eculizumab, a humanized monoclonal antibody that targets complement C5 to inhibit terminal complement activity, has emerged as a potential treatment option for C3G, although data regarding its clinical utility remains limited. In this report, we describe the successful use of eculizumab to treat a patient with recurrent post-transplant C3GN caused by a C3 gene gain-of-function mutation, and also review the published literature regarding the use of eculizumab for the treatment of recurrent C3 glomerulopathy.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Complemento C3/genética , Inativadores do Complemento/uso terapêutico , Glomerulonefrite/tratamento farmacológico , Glomerulonefrite/genética , Adulto , Ativação do Complemento/efeitos dos fármacos , Feminino , Mutação com Ganho de Função , Glomerulonefrite/cirurgia , Humanos , Transplante de Rim , Período Pós-Operatório , Recidiva
20.
BMJ Case Rep ; 12(12)2019 Dec 08.
Artigo em Inglês | MEDLINE | ID: mdl-31818897

RESUMO

This case demonstrates chronic fibrosing pleuritis, as a rare pulmonary aetiology for mortality in patients with Degos disease or malignant atrophic papulosis (MAP). Knowledge of this unusual complication will help physicians identify this entity early and provide appropriate treatment.Patients with MAP die from gastrointestinal and brain involvement within 2-3 years of diagnosis. This case is unique as the patient survived for 9 years and died secondary to respiratory failure, which had not been reported before. Our patient was a young man, diagnosed with MAP at the age of 17. His skin and gastrointestinal disease were controlled with eculizumab and parenteral treprostinil. The patient developed severe restrictive pulmonary disease, required ventilatory support, and died from respiratory failure. An autopsy revealed chronic fibrosis pleuritis. Longer surviving patients with MAP might suffer from significant respiratory disease. Pulmonary function test should be obtained to identify subclinical respiratory limitation.


Assuntos
Papulose Atrófica Maligna/complicações , Pleurisia/complicações , Pleurisia/diagnóstico , Insuficiência Respiratória/etiologia , Anticorpos Monoclonais Humanizados/uso terapêutico , Doença Crônica , Inativadores do Complemento/uso terapêutico , Tratamento Conservador , Diagnóstico Diferencial , Quimioterapia Combinada , Epoprostenol/análogos & derivados , Epoprostenol/uso terapêutico , Evolução Fatal , Humanos , Masculino , Papulose Atrófica Maligna/tratamento farmacológico , Pleurisia/etiologia , Adulto Jovem
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