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1.
J Assoc Physicians India ; 69(10): 11-12, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34781663

RESUMO

Thrombotic thrombocytopenic purpura (TTP) is characterized by disseminated thrombotic occlusions in the microcirculation and a syndrome of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fever, renal and neurologic abnormalities. Several factors such as viral and bacterial pathogens, pancreatitis, drugs, collagen-vascular diseases, cancers, and pregnancy have been reported to cause TTP, Brucellosis is an exceptional cause of this disorder. We present a case of a 33 year old male who was found to have Brucella antigen (IgG) positivity who responded well to antibiotic therapy directed to Brucella infection. He subsequently reported back with B/L diminution of vision, fever and was found to have severe thrombocytopenia. Ophthalmology opinion revealed retinal hemorrhages. In view of severe thrombocytopenia with a normal coagulogram, raised LDH, renal azotemia and peripheral blood smear showing fragmented RBCs he was diagnosed to have Thrombotic Thrombocytopenic Purpura (TTP) secondary to Brucellosis. He was immediately treated with Plasma exchange; however, he relapsed after initial cycles. He underwent further plasma exchanges with unsatisfactory response, thus was eventually started on Rituximab to which he responded well.


Assuntos
Brucelose , Púrpura Trombocitopênica Trombótica , Adulto , Antibacterianos/uso terapêutico , Brucelose/complicações , Brucelose/diagnóstico , Brucelose/tratamento farmacológico , Feminino , Humanos , Masculino , Plasmaferese , Gravidez , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia
2.
Artigo em Russo | MEDLINE | ID: mdl-34693696

RESUMO

The article presents a clinical example of Guillain-Barre syndrome with a predominant involvement of cranial nerves, which developed after COVID-19. Comprehensive clinical and laboratory diagnostics, including examination of cerebrospinal fluid, electromyography, examination for possible etiological infectious agents, was carried out. A course of pathogenetic therapy was used in the form of plasmapheresis sessions, supportive therapy. A good clinical effect was obtained. To this moment, only a few cases of the development of Guillain-Barré syndrome after a new coronavirus infection have been described. The peculiarity of our case is the development of a clinical picture of insufficiency of predominantly cranial nerves with subclinical involvement of the nerves of the extremities.


Assuntos
COVID-19 , Síndrome de Guillain-Barré , Nervos Cranianos , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/etiologia , Síndrome de Guillain-Barré/terapia , Humanos , Plasmaferese , SARS-CoV-2
3.
Transplant Proc ; 53(9): 2675-2677, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34610865

RESUMO

BACKGROUND: A decrease in the isoagglutinin titer <1:8 is usually required for ABO-incompatible (ABOi) transplantation and the presence of high predesensitization titers may condition future transplantation. The aim of the study was to analyze the prognosis of ABOi patients undergoing desensitization and to compare the results according to the baseline isoagglutinin titer. METHODS: ABOi patients transplanted in our center after desensitization with rituximab, apheresis (plasmapheresis, immunoadsorption with Glycosorb, or both) and immunoglobulins were studied. Survival, renal function, and complications were analyzed and the results were compared according to the presence of a baseline isoagglutinin titer higher or lower than 1:128. We analyzed 48 patients (34 male) with a mean age of 50.9 ± 11 years and a mean follow-up of 44.6 ± 30 months. Thirty-eight patients had a basal isoagglutinin titer ≤1:128 and 10 had a titer >1:128. We did not observe differences in patient survival: 96% vs 100% at 5 years (P = .64) and renal survival: 91% vs 100% at 5 years (P = .39), incidence of acute rejection: 13.2% vs 0% (P = .22), infectious complications (cytomegalovirus; 16% vs 30%, P = 0.30; Polyomavirus BK virus: 13% vs 0%, P  =  .22), or surgical (hematoma): 47% vs 60% (P = .47) between the 2 groups. A higher number of apheresis sessions was observed (4.8 ± 1.9 vs 10.9 ± 3.9; P = .001); use of both techniques (0% vs 100%, P < .001) and higher processed volume (1 ± 0.1 vs 1.4 ± 0.5; P = .049) in patients with titer >128 was observed. Creatinine and proteinuria were similar and not significant. CONCLUSIONS: Baseline isoagglutinin titer does not influence the prognosis of ABOi patients after desensitization. The number of sessions required to achieve baseline titer <1:8 is higher but does not influence the number of days of hospital admission.


Assuntos
Vírus BK , Transplante de Rim , Sistema ABO de Grupos Sanguíneos , Adulto , Incompatibilidade de Grupos Sanguíneos , Rejeição de Enxerto , Sobrevivência de Enxerto , Humanos , Transplante de Rim/efeitos adversos , Masculino , Pessoa de Meia-Idade , Plasmaferese
4.
Am J Trop Med Hyg ; 105(4): 942-945, 2021 08 23.
Artigo em Inglês | MEDLINE | ID: mdl-34614478

RESUMO

Chikungunya virus (CHIKV) is an arbovirus endemic to South Asia with frequent outbreaks. A wide spectrum of neurological complications has been described in Chikungunya infections. Myeloneuropathy is a rare complication seen in Chikungunya and is proposed to have an underlying immune mediated pathogenesis. We report a case of a 45-year-old man presenting to the emergency services with acute onset of quadriparesis, breathlessness, urinary retention, profound pain, and sensory disturbances 6 weeks after the onset of high-grade fever and arthralgia. On examination, the patient had Medical Research Council grade 1 flaccid quadriparesis with prominent wasting and areflexia with distinct sensory level at T4. Immunoglobulin M CHIKV antibodies were positive, tested twice at a 1-week interval. He had notable magnetic resonance imaging (MRI) findings in the form of patchy T2 hyperintensities involving the entire length of the cervical and thoracic cord with normal brain imaging and extensive short tau inversion recovery hyperintense signal changes on muscle MRI. He was treated with five cycles of plasmapheresis and intravenous methylprednisolone followed by oral steroids for 8 weeks. At 20-week follow-up, the patient had improvement in upper limb weakness, but paraparesis persisted. The case highlights the presence of unusual MRI findings and also the importance of early recognition of after infective neurological complications, and prompt treatment with immunomodulation may be beneficial.


Assuntos
Febre de Chikungunya/complicações , Doenças da Medula Espinal/diagnóstico por imagem , Doenças da Medula Espinal/virologia , Artralgia/diagnóstico por imagem , Artralgia/virologia , Febre de Chikungunya/virologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Plasmaferese , Doenças da Medula Espinal/classificação , Doenças da Medula Espinal/tratamento farmacológico
5.
Rinsho Ketsueki ; 62(8): 1222-1228, 2021.
Artigo em Japonês | MEDLINE | ID: mdl-34497210

RESUMO

Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially life-threatening disease that is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and ischemic organ damage resulting from the formation of platelet-rich thrombi in the microvasculature. It is especially related to a severe deficiency of ADAMTS13, the specific von Willebrand factor-cleaving protease. Thus, <10% of the normal activity of ADAMTS13 is an essential diagnostic marker for establishing a diagnosis of TTP. TTP can be of congenital form that is termed the Upshaw-Schulman syndrome (USS) that is caused by genetic abnormality of ADAMTS13 and acquired form that is caused by autoantibodies against ADAMTS13. The congenital form is treated with the infusion of fresh frozen plasma, and the treatment of the acquired form involves plasma exchange combined with immunosuppressive therapy that uses corticosteroids and rituximab. Recently, the efficacy and safety of new drugs caplacizumab, single-domain antibody against ADAMTS13, and recombinant ADAMTS13 products have been reported in large-scale clinical trials conducted in other countries. These results suggested the better outcome in the treatment for the patients with TTP in the near future.


Assuntos
Anemia Hemolítica , Púrpura Trombocitopênica Trombótica , Proteína ADAMTS13 , Autoanticorpos , Humanos , Troca Plasmática , Plasmaferese , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia
6.
Neurologist ; 26(5): 196-224, 2021 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-34491938

RESUMO

BACKGROUND: Central nervous system complications are reported in an increasing number of patients with Coronavirus Disease 2019 (COVID-19). COVID-19-related Guillain-Barré syndrome (GBS) is of particular importance given its association with higher mortality rates and prolonged respiratory failure. REVIEW SUMMARY: We conducted a systematic review of published cases for COVID-19-related GBS, and provide a summary of clinical management strategies for these cases. Sixty-three studies, including 86 patients, were included. Seventy-six cases with reported outcome data were eligible for the outcome analysis. Ninety-nine percent of patients were diagnosed with COVID-19 before diagnosis of GBS (median: 14 d prior, interquartile range: 7 to 20). Intravenous immunotherapy (intravenous immunoglobulin: 0.4 g/kg/d for 5 d) was the most frequently used treatment approach. The review indicated that the outcome was not favorable in 26% of cases (persistent neurological deficits). A mortality rate of 3.5% was observed in patients with COVID-19-related GBS. CONCLUSIONS: Although evidence to support specific treatments is lacking, clinicians should consider the benefits of immunotherapy and plasma exchange in addition to the standard antimicrobial and supportive therapies for patients who meet the diagnostic criteria for acute sensory and motor polyradiculoneuritis. Intravenous immunoglobulin treatment alone is not shown to result in improved outcomes or mortality. More extensive studies aimed at exploring the neurological manifestations and complications of COVID-19 and distinctive treatment options for COVID-19-related GBS are warranted.


Assuntos
COVID-19/tratamento farmacológico , Síndrome de Guillain-Barré/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , SARS-CoV-2/efeitos dos fármacos , Neoplasias da Glândula Tireoide/tratamento farmacológico , Síndrome de Guillain-Barré/diagnóstico , Humanos , Troca Plasmática/métodos , Plasmaferese/efeitos adversos , Plasmaferese/métodos
7.
Medicine (Baltimore) ; 100(35): e27139, 2021 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-34477166

RESUMO

INTRODUCTION: Acute liver failure (ALF) is a life-threatening condition that remains challenging for physicians despite several advances in supportive care. Etiologies vary worldwide, with herpes simplex virus (HSV) hepatitis representing less than 1% of cases. Despite its low incidence, ALF is a lethal cause of acute necrotizing hepatitis and has a high mortality. Early antiviral treatment is beneficial for survival and decreased liver transplantation necessity. However, plasmapheresis, despite its theoretical potential benefit, is scarcely reported. PATIENT CONCERNS: A 25-year-old woman with no known disease presented with painful pharynx ulcers, increased transaminases and impaired liver function. DIAGNOSIS: ALF due to a disseminated HSV-2 primary infection was diagnosed with a positive polymerase chain reaction for HSV-2 in the biopsied liver tissue and blood. INTERVENTIONS: Empiric antiviral treatment was initiated. After clinical deterioration, plasmapheresis was also initiated. OUTCOMES: After 6 cycles of plasmapheresis and supportive care, the patient's condition improved without undergoing liver transplantation. CONCLUSIONS: ALF is a life-threatening condition, and HSV as an etiology must be suspected based on background, clinical manifestation, and laboratory information. The potential role of plasmapheresis in HSV hepatitis should be considered.


Assuntos
Aciclovir/uso terapêutico , Antivirais/uso terapêutico , Herpes Simples/complicações , Falência Hepática Aguda/virologia , Plasmaferese , Adulto , Feminino , Herpesvirus Humano 2/isolamento & purificação , Humanos , Falência Hepática Aguda/diagnóstico por imagem , Falência Hepática Aguda/terapia , Tomografia Computadorizada por Raios X
9.
Rev Colomb Obstet Ginecol ; 72(2): 210-218, 2021 Jun 30.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-34506707

RESUMO

Objective: To report the case of pregnant woman with Guillain-Barré syndrome (GBS) presenting as the Miller Fisher variant, and to review the literature on the diagnosis, treatment and prognosis of this GBS variant during gestation. Materials and Methods: Pregnant woman presenting at 27 weeks of gestation with Miller Fisher syndrome (MFS), treated in a military referral hospital with a satisfactory course after 15 days, continuation of normal pregnancy and delivery of a healthy neonate at 38 weeks. A search of the literature was conducted in the Medline via PubMed, Lilacs, SciELO, ScienceDirect and Ovid databases using the terms "Pregnancy," "Miller Fisher syndrome," "Guillain-Barré syndrome". Cohorts, case series and case reports of pregnant women with MFS were included. Data on diagnostic methods, treatment and maternal and perinatal prognosis were extracted. The search was made on June 2020, with no restriction by date, but restriction by language (Spanish and English). Results: Overall, 423 titles were identified, three studies met the inclusion criteria, the three of them corresponding to case reports. All cases were found to be seropositive for anti-GQ1b ganglioside antibodies. No imaging abnormalities were found in any of the cases. Two patients received IV immunoglobulin and the third patient was kept under observation. No obstetric complications have be documented so far. Conclusion: There are few cases of MFS reported during pregnancy. Intravenous immunoglobulin is the most frequently used treatment option. Plasmapheresis was used in the case presented here. The impact of the Miller Fisher variant on the normal course of gestation and on long-term perinatal outcomes is unknown. Further studies that look into the diagnosis, treatment and prognosis of this condition are required.


Assuntos
Síndrome de Guillain-Barré , Síndrome de Miller Fisher , Anticorpos , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Recém-Nascido , Síndrome de Miller Fisher/diagnóstico , Síndrome de Miller Fisher/terapia , Plasmaferese , Gravidez
11.
Tidsskr Nor Laegeforen ; 141(2021-12)2021 09 07.
Artigo em Norueguês | MEDLINE | ID: mdl-34505493

RESUMO

BACKGROUND: Immunotherapy is used to treat several cancer types. As it is a relatively new type of treatment, knowledge of optimal handling of rare side effects is sparse. CASE PRESENTATION: A woman in her early eighties with inoperable metastases from malignant melanoma was given immunotherapy. Five weeks after the first dose of nivolumab 480 mg she was admitted to the hospital with symptoms of coronary artery disease. On suspicion of side effects from nivolumab, she was given prednisolone 80 mg daily. MRI of the heart showed findings highly suggestive of myocarditis. Her condition initially improved, but 9 days after leaving the hospital she developed muscular weakness and ptosis. Neurography and electromyography revealed acute axonal polyneuropathy and she was treated with mycophenolate mofetil. In the days that followed she had increasing paresis, apnoea and pain in the right shoulder. Echocardiography showed reduced systolic function. The patient died 11 days after the second hospitalisation. INTERPRETATION: The patient developed hepatitis, myocarditis and neuropathy after only one course of immunotherapy. Those are known to be rare side effects with a high mortality rate. The patient was given high dose prednisolone and mycophenolate mofetil. Intravenous immunoglobulins and/or plasmapheresis could have been alternative treatments.


Assuntos
Melanoma , Nivolumabe , Dor no Peito , Feminino , Humanos , Imunoterapia , Melanoma/tratamento farmacológico , Plasmaferese
12.
J Neurovirol ; 27(5): 797-801, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34550544

RESUMO

Guillain-Barré syndrome (GBS) is an ascending demyelinating polyneuropathy often associated with recent infection. Miller Fisher syndrome represents a variant with predominant facial and cranial nerve involvement, although Miller Fisher and Guillain-Barré overlap syndromes can occur. Guillain-Barré spectrum syndromes have been thought to be rare among solid organ transplant recipients. We describe an immunocompromised patient with a liver transplant who presented with ophthalmoplegia and bulbar deficits. His symptoms rapidly progressed to a state of descending paralysis involving the diaphragm; he then developed acute respiratory failure and eventually developed quadriparesis. Electromyography and a nerve conduction study demonstrated a severe sensorimotor axonal polyneuropathy consistent with Miller Fisher variant Guillain-Barré syndrome. Despite several negative nasopharyngeal swabs for COVID-19 polymerase chain reaction, a serology for SARS-CoV-2 IgG was positive. He was diagnosed with Miller Fisher-Guillain-Barré overlap syndrome with rapid recovery following treatment with plasma exchange. Although Guillain-Barré is a rare complication in solid organ transplant recipients, this case highlights the importance of rapid diagnosis and treatment of neurologic complications in transplant patients. Furthermore, it demonstrates a possible case of neurological complications from COVID-19 infection.


Assuntos
COVID-19/complicações , Síndrome de Guillain-Barré/imunologia , Síndrome de Guillain-Barré/virologia , Síndrome de Miller Fisher/imunologia , Síndrome de Miller Fisher/virologia , Síndrome de Guillain-Barré/terapia , Humanos , Hospedeiro Imunocomprometido , Transplante de Fígado , Masculino , Pessoa de Meia-Idade , Síndrome de Miller Fisher/terapia , Plasmaferese , SARS-CoV-2 , Transplantados
13.
Artigo em Inglês | MEDLINE | ID: mdl-34452974

RESUMO

BACKGROUND AND OBJECTIVES: Since the onset of the COVID-19 pandemic, a growing number of reports have described cases of acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic leukoencephalitis (AHLE) following infection with COVID-19. Given their relatively rare occurrence, the primary objective of this systematic review was to synthesize their clinical features, response to treatments, and clinical outcomes to better understand the nature of this neurologic consequence of COVID-19 infection. METHODS: Patients with a history of COVID-19 infection were included if their reports provided adequate detail to confirm a diagnosis of ADEM or AHLE by virtue of clinical features, radiographic abnormalities, and histopathologic findings. Cases purported to be secondary to vaccination against COVID-19 or occurring in the context of a preexisting relapsing CNS demyelinating disease were excluded. Case reports and series were identified via PubMed on May 17, 2021, and 4 additional cases from the authors' hospital files supplemented the systematic review of the literature. Summary statistics were used to describe variables using a complete case analysis approach. RESULTS: Forty-six patients (28 men, median age 49.5 years, 1/3 >50 years old) were analyzed, derived from 26 case reports or series originating from 8 countries alongside 4 patient cases from the authors' hospital files. COVID-19 infection was laboratory confirmed in 91% of cases, and infection severity necessitated intensive care in 67%. ADEM occurred in 31 cases, whereas AHLE occurred in 15, with a median presenting nadir modified Rankin Scale score of 5 (bedridden). Anti-MOG seropositivity was rare (1/15 patients tested). Noninflammatory CSF was present in 30%. Hemorrhage on brain MRI was identified in 42%. Seventy percent received immunomodulatory treatments, most commonly steroids, IV immunoglobulins, or plasmapheresis. The final mRS score was ≥4 in 64% of patients with adequate follow-up information, including 32% who died. DISCUSSION: In contrast to ADEM cases from the prepandemic era, reported post-COVID-19 ADEM and AHLE cases were often advanced in age at onset, experienced severe antecedent infection, displayed an unusually high rate of hemorrhage on neuroimaging, and routinely had poor neurologic outcomes, including a high mortality rate. Findings are limited by nonstandardized reporting of cases, truncated follow-up information, and presumed publication bias.


Assuntos
COVID-19/complicações , Encefalomielite Aguda Disseminada/etiologia , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Encefalomielite Aguda Disseminada/mortalidade , Encefalomielite Aguda Disseminada/fisiopatologia , Encefalomielite Aguda Disseminada/terapia , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Unidades de Terapia Intensiva , Leucoencefalite Hemorrágica Aguda/etiologia , Leucoencefalite Hemorrágica Aguda/mortalidade , Leucoencefalite Hemorrágica Aguda/fisiopatologia , Leucoencefalite Hemorrágica Aguda/terapia , Imageamento por Ressonância Magnética , Plasmaferese , SARS-CoV-2 , Índice de Gravidade de Doença
14.
Front Immunol ; 12: 650782, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34367127

RESUMO

The changes in the serum levels of aquaporin-4-IgG (AQP4-IgG), immunoglobulins, and inflammatory mediators in neuromyelitis optica spectrum disorder (NMOSD) cases treated with immunoadsorption have been rarely described in detail. Here we report a 29-year-old steroid-resistant NMOSD female with a severe disability (bilateral blindness and paraplegia) who received protein-A immunoadsorption as a rescue treatment. During the total 5 sessions, the circulating level of AQP4-IgG, immunoglobulins, and complement proteins (C3 and C4) showed a rapid and sawtooth-like decrease, and the serum AQP4-IgG titer declined from 1:320 to below the detectable limit at the end of the 3rd procedure. Of all the antibodies, IgG had the biggest removal rate (>96.1%), followed by IgM (>66.7%) and IgA (53%), while complement C3 and C4 also dropped by 73% and 65%, respectively. The reduced pro-inflammatory cytokines (interleukin-8 and tumor necrosis factor-α) and marked increased lymphocyte (T and B cell) counts were also observed. The improvement of symptoms initiated after the last session, with a low AQP4-IgG titer (1:32) persisting thereafter. Accordingly, protein-A immunoadsorption treatment could be one of the potential rescue therapies for steroid-resistant NMOSD patients with a severe disability.


Assuntos
Aquaporina 4/imunologia , Biomarcadores/sangue , Imunoglobulina G/imunologia , Neuromielite Óptica/terapia , Plasmaferese/métodos , Proteína Estafilocócica A/imunologia , Adulto , Complemento C3/imunologia , Complemento C4/imunologia , Feminino , Humanos , Imunoglobulina G/sangue , Interleucina-8 , Contagem de Linfócitos , Neuromielite Óptica/sangue , Neuromielite Óptica/imunologia , Fator de Necrose Tumoral alfa/sangue , Fator de Necrose Tumoral alfa/imunologia
15.
Adv Chronic Kidney Dis ; 28(1): 59-73, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-34389138

RESUMO

Therapeutic plasma exchange (TPE) is frequently the most common Apheresis Medicine technique used for extracorporeal therapy of a wide variety of renal, neurological, hematological, and other clinical indications. Many of these clinical indications require intensive care during critical illness. Conventional TPE uses one of two main technical methods to achieve the goal of removing known disease mediators from the plasma: using centrifugal forces to separate and remove components of blood, or a membrane filtration method that separates plasma from the cellular components of blood. The following review discusses the basic principles of TPE, the technological aspects, and relevant clinical scenarios encountered in the intensive care unit, including relevant guidelines and recommendations from the American Society for Apheresis.


Assuntos
Estado Terminal , Troca Plasmática , Humanos , Unidades de Terapia Intensiva , Plasmaferese , Tecnologia
16.
Anesth Analg ; 133(5): 1180-1186, 2021 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-34415867

RESUMO

BACKGROUND: Heparin-induced thrombocytopenia (HIT) is an immune-mediated adverse reaction to heparin. Patients undergoing cardiac surgery with cardiopulmonary bypass (CPB) are routinely anticoagulated with heparin before the initiation of bypass. Heparin is contraindicated, however, in patients with acute HIT, and alternatives to routine practice are often used. While guidelines have recently been published addressing this topic 10, there remains variance between institutions in how these cases are treated. Our goal was to better delineate practice trends in the diagnosis and management of HIT patients requiring CPB. METHODS: We surveyed members of the Society of Cardiovascular Anesthesiologists (SCA) and the American Society for Extracorporeal Technology (AmSECT) using an online survey tool. RESULTS: We received 304 completed surveys (5.8% response rate), 75% completed by an anesthesiologist, and 24% by a perfusionist. The majority of respondents used clinical history and/or antibody testing (71% and 63%, respectively) to diagnose HIT. Seventy-five percent of respondents reported using an institutional protocol for HIT-CPB cases. Most respondents (89%) reported having at least 1 case in the last 3 years, with a total case experience of at least 785 cases (785 = the minimum number of cases in each case volume category × the number of respondents choosing that category). The strategy recommended in published guidelines, bivalirudin, was the most commonly reported alternative anticoagulation strategy (75%) used by respondents in HIT cases, with most (83%) using the activated clotting time (ACT) to monitor anticoagulation. CONCLUSIONS: Most responding SCA and AmSECT members reported that their institution used a protocol or guideline for HIT/CPB cases, and most guidelines directed the use of bivalirudin as an alternative anticoagulant. Various other methods such as plasmapheresis are also being used with success in this patient population. Further research, including comparison studies of alternative anticoagulant strategies, is required to elucidate the best approach to these difficult cases.


Assuntos
Anticoagulantes/administração & dosagem , Procedimentos Cirúrgicos Cardíacos , Ponte Cardiopulmonar , Heparina/efeitos adversos , Padrões de Prática Médica/tendências , Trombocitopenia/terapia , Anticoagulantes/imunologia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Ponte Cardiopulmonar/efeitos adversos , Contraindicações de Procedimentos , Monitoramento de Medicamentos/tendências , Substituição de Medicamentos/tendências , Fidelidade a Diretrizes/tendências , Pesquisas sobre Serviços de Saúde , Heparina/imunologia , Hirudinas , Humanos , Fragmentos de Peptídeos/uso terapêutico , Plasmaferese/tendências , Guias de Prática Clínica como Assunto , Proteínas Recombinantes/uso terapêutico , Medição de Risco , Fatores de Risco , Trombocitopenia/induzido quimicamente , Trombocitopenia/diagnóstico , Trombocitopenia/imunologia , Tempo de Coagulação do Sangue Total/tendências
17.
BMC Neurol ; 21(1): 275, 2021 Jul 12.
Artigo em Inglês | MEDLINE | ID: mdl-34253174

RESUMO

BACKGROUND: Guillain-Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy characterized by rapidly evolving weakness and areflexia, reaching nadir within 4 weeks. Data on the characteristic of GBS in Saudi Arabia are limited. This study aimed to describe the clinical, electrophysiological, and laboratory characteristics and outcome of a multicenter cohort of patients with GBS. METHODS: This is a retrospective multicenter nationwide study. Patients who had GBS, identified through Brighton Criteria, between January 2015 and December 2019 were included. Data collected included demographics, clinical features, cerebrospinal fluid profile, reported electrophysiological patterns, treatment, and outcome. Reported GBS subtypes were compared using chi-square, Fisher's exact, or Mann-Whitney U tests, as appropriate. RESULTS: A total of 156 patients with GBS were included (men, 61.5%), with a median age of 38 (interquartile range, 26.25-53.5) years. The most commonly reported antecedent illnesses were upper respiratory tract infection (39.1%) and diarrhea (27.8%). All but two patients (98.7%) had weakness, 64.1% had sensory symptoms, 43.1% had facial diplegia, 33.8% had oropharyngeal weakness, 12.4% had ophthalmoplegia, and 26.3% needed mechanical ventilation. Cytoalbuminological dissociation was observed in 69.1% of the patients. GBS-specific therapy was administered in 96.8% of the patients, of whom 88.1% had intravenous immunoglobulin, and 11.9% had plasmapheresis. Approximately half of the patients were able to walk independently within 9 months after discharge, and a third regained the ability to walk independently thereafter. Death of one patient was caused by septicemia. Acute inflammatory demyelinating polyradiculoneuropathy was the most commonly reported GBS subtype (37.7%), followed by acute motor axonal neuropathy (29.5%), and acute motor-sensory axonal neuropathy (19.2%). CONCLUSION: The clinical and laboratory characteristics and outcome of GBS in the Arab population of Saudi Arabia are similar to the international cohorts. The overall prognosis is favorable.


Assuntos
Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/terapia , Adolescente , Adulto , Idoso , Feminino , Síndrome de Guillain-Barré/sangue , Síndrome de Guillain-Barré/epidemiologia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Pessoa de Meia-Idade , Plasmaferese/métodos , Prognóstico , Respiração Artificial/métodos , Estudos Retrospectivos , Arábia Saudita/epidemiologia , Resultado do Tratamento , Adulto Jovem
18.
Front Immunol ; 12: 711915, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34276706

RESUMO

Passive antibody therapy has been used to treat outbreaks of viral disease, including the ongoing pandemic of severe respiratory acute respiratory syndrome (SARS) coronavirus 2 (SARS-CoV-2) or COVID-19. However, the real benefits of the procedure are unclear. We infused a concentrated solution of neutralizing anti-SARS-CoV-2 antibodies obtained from a convalescent donor with a single session of double filtration plasmapheresis (DFPP) into a 56-year-old woman with long history of unremitting, severe COVID-19. She was unable to establish an adequate antiviral immune response because of previous chemotherapy, including the infusion of the anti-CD20 monoclonal antibody rituximab, administered to treat a diffuse large B-cell lymphoma. The disease promptly recovered despite evidence of no endogenous anti-SARS-CoV-2 antibody production. The observation that passive antibody therapy might prove particularly effective in immunodepressed COVID-19 patients requires evaluation in prospective randomized controlled trial.


Assuntos
Anticorpos Neutralizantes/uso terapêutico , Anticorpos Antivirais/uso terapêutico , COVID-19/terapia , Imunização Passiva/métodos , Hospedeiro Imunocomprometido , Imunoglobulina G/uso terapêutico , Plasmaferese/métodos , SARS-CoV-2/genética , Antineoplásicos Imunológicos/efeitos adversos , Antivirais/uso terapêutico , COVID-19/imunologia , COVID-19/virologia , Feminino , Humanos , Imunidade/efeitos dos fármacos , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Pessoa de Meia-Idade , RNA Viral/genética , Rituximab/efeitos adversos , Resultado do Tratamento
19.
Brain Nerve ; 73(7): 819-828, 2021 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-34234040

RESUMO

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune peripheral neuropathy with chronic progression over 2 months or more. In this review, we provide an overview of key clinical studies involved in the development of CIDP therapy, as well as a discussion of changes in the concept of treatment. Although a definitive therapy has not yet been established, international and Japanese clinical guidelines recommend three first-line treatment options for symptom improvement, namely corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis, based on the results of clinical studies conducted before the early 2000s. Since 2010, several treatments for the prevention of CIDP relapse (maintenance therapy) have been developed and more recently, studies have focused on the optimization of each treatment. On the other hand, CIDP treatment is associated with several limitations, including a lack of biomarkers for prediction of disease progression, differences in response to treatment between CIDP subtypes, and difficulties in the selection of appropriate maintenance therapy. Several studies aimed at resolving these issues are currently being conducted. (Received 22 May, 2020; Accepted 12 January, 2021; Published 1 July, 2021).


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Corticosteroides , Humanos , Imunoglobulinas Intravenosas , Plasmaferese , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/tratamento farmacológico , Recidiva , Resultado do Tratamento
20.
Rinsho Ketsueki ; 62(5): 480-485, 2021.
Artigo em Japonês | MEDLINE | ID: mdl-34248125

RESUMO

The key clinical symptoms of thrombotic thrombocytopenic purpura (TTP) are severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia/infarction due to microthrombi. Hemolytic anemia in TTP is characterized by mechanical damage to red blood cells. TTP is caused by a severe deficiency of ADAMTS13 activity, which is caused by mutations in the ADAMTS13 gene (congenital TTP) or by autoantibodies affecting the function or clearance of ADAMTS13 (immune TTP). Patients with congenital TTP receive fresh frozen plasma (FFP) transfusion for the supplementation of ADAMTS13. Meanwhile, those with immune TTP receive plasma exchange therapy using FFP for the supplementation of ADAMTS13 and the removal of anti-ADAMTS13 autoantibodies. Corticosteroid therapy is concurrently administered to suppress autoantibody production. In terms of novel treatment, the use of rituximab, a humanized anti-CD20 monoclonal antibody, in patients with immune TTP was approved by the Japanese health insurance in 2020. Novel and promising drugs are currently developed. A first-in-human study of recombinant ADAMTS13 for congenital TTP was reported in 2017. Caplacizumab is a humanized nanobody that inhibits the interaction between von Willebrand factor and platelets. This drug can prevent early thrombus formation and organ damage in patients with immune TTP. Therefore, these novel drugs can improve mortality in patients with TTP.


Assuntos
Anemia Hemolítica , Púrpura Trombocitopênica Trombótica , Proteína ADAMTS13/genética , Humanos , Troca Plasmática , Plasmaferese , Púrpura Trombocitopênica Trombótica/terapia , Fator de von Willebrand
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