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1.
Medicine (Baltimore) ; 99(6): e18535, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32028387

RESUMO

BACKGROUND: In recent years, many studies focused on the association between the neutrophil-to-lymphocyte ratio (NLR) and the risk of intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (rKD), with inconsistent results. Therefore, we aimed to investigate the role of NLR as a biomarker in detecting rKD. METHODS: We searched PubMed, EMBASE, the Cochrane Central Register of Controlled Trials, and China National Knowledge Infrastructure through May 18th, 2019. Meta-disc 1.4 and STATA 15.1 were used to perform this metaanalysis in a fixed/random-effect model. RESULTS: A total of 7 relevant studies were eligible to analyze pooled accuracy. The overall performance of NLR detection was: pooled sensitivity, 0.66 (95% confidence interval [CI], 0.63 - 0.70); pooled specificity, 0.71 (95%CI, 0.69 - 0.73); and area under the summary receiver operating characteristic curves value (SROC), 0.7956. The meta-regression analysis showed that the type of samples was the sources of heterogeneity. The subgroup analysis suggested that NLR detection after the initial treatment of IVIG had the largest area under curve of SROC in all the subgroups: pooled sensitivity, 0.58 (95%CI, 0.53 - 0.63); pooled specificity, 0.77 (95%CI, 0.75 - 0.79); and SROC, 0.8440. CONCLUSIONS: This is the first meta-analysis demonstrated that NLR might be a biomarker for detecting rKD, especially NLR value after the initial treatment of IVIG. More well-designed researches need to be done to launch the application of NLR for predicting rKD in the clinic.


Assuntos
Resistência a Medicamentos , Imunoglobulinas Intravenosas/uso terapêutico , Linfócitos/citologia , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Neutrófilos/citologia , Biomarcadores/sangue , Humanos , Síndrome de Linfonodos Mucocutâneos/sangue
2.
Medicine (Baltimore) ; 99(4): e18805, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31977873

RESUMO

RATIONALE: Anti-GQ1b antibody syndrome refers to a distinct variant of Guillain- Barré syndrome. Involvement of the optic nerve in anti-GQ1b antibody syndrome is extremely rare. PATIENT CONCERNS: Here, we report a case of anti-GQ1b antibody syndrome presenting with visual deterioration as the initial symptom. A 73-year-old man presented with a 5-day history of bilateral blurred vision and ptosis. He had a previous history of diarrhea starting 10 days before admission. Physical examination showed visual deterioration, ophthalmoplegia, and peripheral facial paralysis. Testing of both serum and cerebrospinal fluid was positive for anti-GQ1b immunoglobulin G antibodies and negative for anti-aquaporin 4antibodies. DIAGNOSIS: Anti-GQ1b antibody syndrome. INTERVENTIONS: The patient was treated with intravenous methylprednisolone and human immunoglobulin. OUTCOMES: After a 20-day follow-up, the patient's condition took a favorable turn. LESSONS: This case reminds us that anti-GQ1b antibody syndrome should be suspected in patients with visual deterioration and preceding infection.


Assuntos
Síndrome de Guillain-Barré/diagnóstico , Imunoglobulina G/sangue , Idoso , Autoanticorpos/sangue , Síndrome de Guillain-Barré/sangue , Síndrome de Guillain-Barré/classificação , Síndrome de Guillain-Barré/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Masculino , Oftalmoplegia/diagnóstico , Oftalmoplegia/etiologia , Síndrome , Transtornos da Visão/etiologia
3.
Autoimmun Rev ; 19(1): 102431, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31734403

RESUMO

INTRODUCTION: Little is known about systemic sclerosis (SSc)-associated myopathy (SScAM) treatment. Herein we evaluated the use of intravenous immunoglobulin (IVIg) in SScAM. METHODS: We conducted a retrospective study of patients with SScAM in the Internal medicine department of Cochin University Hospital between 1993 and 2017. RESULTS: Fifty-two patients were included comprising 18 (34.6%) with limited SSc and 34 (65.4%) with diffuse SSc. SScAM occurred at a median [interquartile range (IQR)] time of 1 month [0-15] after SSc diagnosis. Thirty-four patients (65.4%) had muscle weakness, 28 (53.8%) had myalgia and 24 (46.2%) had dysphagia. Fifty patients (96.2%) had increased creatine kinase, 22/26 (84.6%) had myopathic electromyography, 10/12 (83.3%) had a high intensity signal of girdle muscles on MRI and 49/50 (98%) had abnormal muscle biopsy. Eighteen (34.6%) patients received IVIg. Severe adverse events occurred in 3/18 (16.7%) patients. When compared to patients who did not receive IVIg, patients who received IVIg had a significantly higher maximal corticosteroid (CS) dose ever, a greater decrease of CS at 3 months, and a lower CS dose at one year and at the end of follow up. CONCLUSIONS: This study suggests the benefit of IVIg as adjunctive therapy, with an acceptable tolerance profile, and supports its use as a CS-sparing agent, in SScAM.


Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Doenças Musculares/tratamento farmacológico , Escleroderma Sistêmico/complicações , Corticosteroides/uso terapêutico , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento
5.
Medicine (Baltimore) ; 98(49): e18160, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31804328

RESUMO

RATIONALE: stiff limb syndrome (SLS) is a variant of stiff-man syndrome, primarily affecting a specific limb. Its diagnosis has always been challenging due to the lack of a specific confirmation test. We present a rare case of a patient with lower limb myoclonus and rigidity. PATIENT CONCERNS: A 53-year-old male presented with a sudden onset of progressive left lower extremity myoclonus and muscle rigidity for 3 days. He rapidly showed signs of right lower limb involvement with severe joint stiffness and inability to walk. DIAGNOSIS: The symptoms nature, physical examination, careful elimination of differential diagnosis suggested a diagnosis of stiff limb syndrome. INTERVENTIONS: Intravenous infusion of gamma globulin 0.4 mg/kg coupled with baclofen and clonazepam were given after admission. He also received an injection of botulinum toxin A to relieve his muscle stiffness. OUTCOMES: The patients' condition improved after the initial treatment with complete disappearance of muscle twitching. Further improvements were seen later on after the local administration of botulinum toxin A. LESSONS: Stiff limb syndrome shares the same complex symptoms with many other conditions. Its diagnosis relies heavily on clinical presentations and on ruling out other conditions. However, unusual symptoms such as myoclonus can occur in few cases and together with the rarity of the condition, the prevalence of misdiagnosis is high. Therefore, being aware and recognizing the signs and symptoms is crucial for proper management. Additionally, EMG is a very important test if the present condition is suspected. However, a negative EMG result or a negative anti-glutamic acid decarboxylase antibody test should not exclude SLS diagnosis.


Assuntos
Rigidez Muscular/etiologia , Mioclonia/etiologia , Rigidez Muscular Espasmódica/complicações , Rigidez Muscular Espasmódica/fisiopatologia , Baclofeno/uso terapêutico , Toxinas Botulínicas Tipo A/uso terapêutico , Diagnóstico Diferencial , Eletromiografia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Extremidade Inferior , Masculino , Pessoa de Meia-Idade , Rigidez Muscular/fisiopatologia , Mioclonia/fisiopatologia , Rigidez Muscular Espasmódica/diagnóstico , Rigidez Muscular Espasmódica/tratamento farmacológico
6.
Medicine (Baltimore) ; 98(51): e18070, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31860956

RESUMO

INTRODUCTION: In this study, we presented a rare case of Epstein-Barr virus (EBV) meningoencephalitis presented with meningoencephalitis-like symptoms and diffuse edematous hemorrhage. PATIENT CONCERNS: A 77-year-old male patient was admitted to our hospital with fever, headache, confusion, and unconsciousness for 7 days. Physical examination revealed unconsciousness and stiffness of the neck. DIAGNOSIS: The final diagnosis was EBV meningoencephalitis. INTERVENTIONS: Ganciclovir (two times 350 mg/day, 21 days), methylprednisolone sodium succinate (120 mg, 5 days), and IV immunoglobulins (IV Ig) (0.4 g/kg, 5 days) were given to this patient. OUTCOMES: But the patient's clinical symptoms did not improve, and he was still in a coma. His family refused to be further diagnosed and discharged. After discharge for 2 months, the patient was in a coma. Four months later, the patient died of complications of pulmonary infection. CONCLUSION: The patient is an adult, and imaging was dominated by intracranial diffuse microhemorrhage and edema, which was different from the typical imaging characteristics of EBV encephalitis as previously reported. This specific imaging change may provide new clinical value for the diagnosis of EBV encephalitis.


Assuntos
Infecções por Vírus Epstein-Barr/tratamento farmacológico , Ganciclovir/administração & dosagem , Imunoglobulinas Intravenosas/uso terapêutico , Imagem por Ressonância Magnética/métodos , Meningoencefalite/tratamento farmacológico , Metilprednisolona/administração & dosagem , Idoso , Quimioterapia Combinada , Infecções por Vírus Epstein-Barr/diagnóstico por imagem , Seguimentos , Humanos , Masculino , Meningoencefalite/diagnóstico por imagem , Meningoencefalite/virologia , Medição de Risco , Índice de Gravidade de Doença , Resultado do Tratamento
7.
Rev Chilena Infectol ; 36(5): 636-641, 2019 Oct.
Artigo em Espanhol | MEDLINE | ID: mdl-31859805

RESUMO

BACKGROUND: Kawasaki disease (EK) is an acute systemic vasculitis with a risk of developing coronary aneurysms. AIM: To describe the clinical and epidemiological characteristics of children with EK in Argentina and to analyse the risk factors for the development of coronary's complications (CC). METHODS: Multicenter, retrospective, cross-sectional, observational and analytical study. It included patients younger than 18 years of age diagnosed with EK in hospitals in Argentina, between January the 1st, 2010 and December the 31th, 2013. RESULTS: N = 193 subjects. Age: medium: 29 months. Total incidence 5 cases / 10,000 hospital discharges. CC was observed in 15.5% of patients. Increased risk factors for CC: Elevated number of days with fever at the time of treatment placement (p = 0.0033); Increased of: heart frequency (p = 0.0021), erythrosedimentation (ESR) (p = 0.005), C-reactive protein (CRP) (p < 0.0001), leukocytes (p = 0.0006), neutrophils (p = 0.0021); Decreased of hematocrit (p = 0.0007) and hemoglobin (p < 0.0001).Association with CC: non-coronary cardiological alterations (OR = 10,818); PCR greater than 68 mg /L (OR = 11,596); leukocytes greater than 20,000 / mm3 (OR = 4.316); and ESR greater than 64 mm / 1 hour (OR = 4.267). CONCLUSION: The most frequent form of presentation was complete EK, the risk of CC was higher in males, younger than 5 years old, the risk factors (clinical and laboratory) were similar to those described in the literature.


Assuntos
Doença das Coronárias/epidemiologia , Doença das Coronárias/etiologia , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Argentina/epidemiologia , Sedimentação Sanguínea , Proteína C-Reativa/análise , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Incidência , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/terapia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Estações do Ano , Estatísticas não Paramétricas
8.
Presse Med ; 48(11 Pt 2): 354-359, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31679899

RESUMO

Plasma exchange (PEX) is a therapeutic procedure used to treat diseases caused by pathogenic antibodies or immune-complexes through the removal and the replacement of plasma. The frequency of complications and reactions associated with PEX are mild and of limited duration. In systemic autoimmune diseases and in a variety of other conditions, PEX might be use in association with intravenous immunoglobulin (IVIg) or therapeutic monoclonal antibodies for the management of acute or refractory patients to achieve a durable remission.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Doenças Autoimunes/terapia , Imunoglobulinas Intravenosas/uso terapêutico , Troca Plasmática/métodos , Terapia Combinada/métodos , Humanos
9.
Medicine (Baltimore) ; 98(42): e17595, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31626134

RESUMO

RATIONALE: Extremes of age is an important risk factor for the development of coronary arteries aneurysms (CAAs) associated to Kawasaki disease (KD) along with male gender, prolonged fever and a delay in diagnosis or treatment. PATIENT CONCERNS: We report two cases of KD in the extremes of age, a 5-month-old male infant and a 9-year-old child in order to underline the features of this disorder outside the typical age range of 1 to 4 years. The 5-month-old male was admitted in our clinic for generalized polymorphous exanthema and fever for approximately 7 days. The laboratory test pointed out leukocytosis and increased inflammatory biomarkers. The 9-year-old male child was admitted in our clinic for fever and submandibular adenopathy. The onset was approximately 5 days before the admission with a sudden development of unilateral, painless, submandibular lymphadenopathy for which the ENT specialist recommended antibiotics and nonsteroid anti-inflammatory drugs. In the 2nd day of admission, he presented severe desquamation of hands and soles. DIAGNOSIS: Both cases were diagnosed with KD. The echocardiography showed no cardiac impairment in the infant, while in the older patient it revealed mild dilation of the left coronary artery. INTERVENTIONS: Both patients received intravenously immunoglobulin and pulsed methylprednisolone. OUTCOMES: The evolution was favorable in both cases, but in the infant, the C-reactive protein levels persisted mildly elevated for approximately 2 months after the diagnosis. LESSONS: The peculiarities of KD in the extremes of age are related to a higher frequency of incomplete features and an increased incidence of coronary artery lesions resulting in a delay of the diagnosis, and subsequent poorer outcomes.


Assuntos
Aneurisma Coronário/diagnóstico , Imunoglobulinas Intravenosas/uso terapêutico , Metilprednisolona/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Criança , Aneurisma Coronário/tratamento farmacológico , Aneurisma Coronário/etiologia , Vasos Coronários/diagnóstico por imagem , Quimioterapia Combinada , Ecocardiografia , Seguimentos , Glucocorticoides/uso terapêutico , Humanos , Fatores Imunológicos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico
10.
Curr Opin Ophthalmol ; 30(6): 462-466, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31567467

RESUMO

PURPOSE OF REVIEW: This article will update and review the Miller Fisher variants (MFV) of Guillain-Barré syndrome (GBS) including the clinical presentation, diagnostic testing, and treatment. RECENT FINDINGS: Although the diagnosis of GBS and MFV can be made on clinical grounds, cerebrospinal fluid (CSF) analysis, nerve conduction studies, imaging (e.g. ultrasound and MRI), and serologic testing can help to confirm the diagnosis. Some patients may need immunotherapy with either intravenous immunoglobulin (IVIg) or plasma exchange, and recent studies suggest that complement inhibition combined with IVIg could be of benefit, but further studies are needed to prove efficacy. SUMMARY: GBS is characterized by an acute, ascending polyneuropathy, ataxia, areflexia, and CSF albuminocytologic dissociation. The MFV of GBS is associated with ophthalmoplegia. Clinicians should have high index of suspicion for MFV of GBS in patients with acute ophthalmoplegia in order to establish the diagnosis, perform appropriate evaluation, and start treatment. SDC VIDEO LINK:.


Assuntos
Síndrome de Miller Fisher/diagnóstico , Oftalmoplegia/diagnóstico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunoterapia , Síndrome de Miller Fisher/terapia , Oftalmoplegia/terapia
11.
BMJ ; 366: l5021, 2019 09 10.
Artigo em Inglês | MEDLINE | ID: mdl-31506273

RESUMO

Human respiratory syncytial virus (RSV) belongs to the recently defined Pneumoviridae family, Orthopneumovirus genus. It is a negative sense, single stranded RNA virus that results in epidemics of respiratory infections that typically peak in the winter in temperate climates and during the rainy season in tropical climates. Generally, one of the two genotypes (A and B) predominates in a single season, alternating annually, although regional variation occurs. RSV is a cause of disease and death in children, older people, and immunocompromised patients, and its clinical effect on adults admitted to hospital is clarified with expanded use of multiplex molecular assays. Among adults, RSV produces a wide range of clinical symptoms including upper respiratory tract infections, severe lower respiratory tract infections, and exacerbations of underlying disease. Here we discuss the latest evidence on the burden of RSV related disease in adults, especially in those with immunocompromise or other comorbidities. We review current therapeutic and prevention options, as well as those in development.


Assuntos
Carga Global da Doença , Infecções por Vírus Respiratório Sincicial/epidemiologia , Vírus Sinciciais Respiratórios/genética , Infecções Respiratórias/epidemiologia , Adulto , Antivirais/uso terapêutico , Epidemias , Genótipo , Humanos , Hospedeiro Imunocomprometido , Imunoglobulinas Intravenosas/uso terapêutico , Infecções por Vírus Respiratório Sincicial/imunologia , Infecções por Vírus Respiratório Sincicial/terapia , Infecções por Vírus Respiratório Sincicial/virologia , Vírus Sinciciais Respiratórios/patogenicidade , Infecções Respiratórias/imunologia , Infecções Respiratórias/terapia , Infecções Respiratórias/virologia , Ribavirina/uso terapêutico , Estações do Ano
13.
Orv Hetil ; 160(38): 1487-1494, 2019 Sep.
Artigo em Húngaro | MEDLINE | ID: mdl-31537095

RESUMO

Immune status was investigated in 186 patients with chronic lymphoid leukaemia between January 2012 and March 2015. Incidences of infections and mortality were analysed in patients who did not receive prophylactic immunoglobulin therapy. Immunoglobulin G (IgG) levels were normal (7-17.8 g/L) or decreased in 62.37% and 35.48% of patients, respectively. We measured high immunoglobulin levels only in a few cases (2.15%). Immunoglobulin levels became increasingly lower in more advanced disease stages (Rai stages). The number of infections was inversely proportional to that. Hypogammaglobulinaemia proved to be more important than disease progression in terms of the development of infections. The most common infections were upper respiratory tract (33.07%) and sepsis (18.90%). Two months after chemotherapy, initially normal immunoglobulin levels decreased by an average of 21%, and at the same time the incidence of infections increased. The most common cause of death was sepsis: 30% occurred at low immunoglobulin levels, while 20% at normal immunoglobulin levels. According to literature, prophylactic immunoglobulin treatment is indicated in patients with chronic lymphoid leukaemia and immunodeficiency for decreasing both morbidity and mortality. According to recommendations in literature, replacement treatment must be administered in severe or moderately severe recurrent bacterial infections. Immunoglobulin prophylaxis may be provided as low dose (10 g), fix dose (18 g) or individually customized higher dose (300-400 mg/kg body weight) treatment. According to recommendations, higher dose immunoglobulin prophylaxis, administered every three weeks on six occasions, is more efficient when customized. With this dose, infection-free condition may be achieved in 50% of patients. Orv Hetil. 2019; 160(38): 1487-1494.


Assuntos
Agamaglobulinemia/tratamento farmacológico , Agamaglobulinemia/mortalidade , Imunoglobulinas Intravenosas/uso terapêutico , Imunoglobulinas/administração & dosagem , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Sepse/mortalidade , Agamaglobulinemia/complicações , Relação Dose-Resposta a Droga , Feminino , Humanos , Hungria/epidemiologia , Imunoglobulina G/sangue , Imunoglobulinas Intravenosas/administração & dosagem , Controle de Infecções , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/mortalidade , Masculino , Sepse/diagnóstico , Resultado do Tratamento
14.
Ideggyogy Sz ; 72(7-8): 285-288, 2019 Jul 30.
Artigo em Inglês | MEDLINE | ID: mdl-31517463

RESUMO

Morvan syndrome is a rare disease characterized by peripheral nerve hyperexcitability, encephalopathy, dys-autonomia and significant insomnia. The patient, who was included in the present study, was followed-up at our clinics for confusion, myokymia, hyperhidrosis, epileptic seizures, tachycardia, agitation, hypokalemia, and hyponatremia. The cranial MRI of the patient demonstrated hyperintensities at the T2 and FLAIR sections of the medial temporal lobe and insular lobes. Electromyography and neurotransmission examination results were concordant with peripheral nerve hyperreactivity. Contactin-associated protein-like 2 antibodies and leucine-rich glioma inactivated protein 1 antibodies were detected as positive. The patient was diagnosed with Morvan syndrome; intravenous immunoglobulin and corticosteroid treatment was started. Almost full remission was achieved. This very rare syndrome implies challenges in diagnosis and treatment; however, remission can be achieved during the follow-up. In addition, caution is needed in the long-term follow-up of these patients regarding the development of malignancies.


Assuntos
Encefalopatias , Encéfalo/diagnóstico por imagem , Transtornos dos Movimentos/fisiopatologia , Doenças Musculares/fisiopatologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Corticosteroides/uso terapêutico , Autoanticorpos/sangue , Eletromiografia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Encefalite Límbica , Transtornos dos Movimentos/tratamento farmacológico , Doenças Musculares/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Convulsões/etiologia , Resultado do Tratamento
15.
Int Arch Allergy Immunol ; 180(3): 221-232, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31509832

RESUMO

BACKGROUND: Immunoglobulin (Ig) therapy is highly effective in reducing the frequency and severity of infections. However, a subset of patients does not respond adequately. OBJECTIVE: To determine in adult patients with primary hypogammaglobulinemia (a) if failure to reconstitute IgG subclass(es) is associated with inadequate clinical response, (b) whether reconstitution of IgG subclasses differs between routes of Ig administration, (c) which subclasses contribute to low total IgG, and (d) what are the most commonly impaired Streptococcus pneumoniae serotypes. METHODS: A retrospective review of the records of patients with primary hypogammaglobulinemia followed up at the Immunology Clinic between 2010 and 2018 was conducted. Demographic, clinical, and laboratory data were collected. RESULTS: Seventy-one patients with primary hypogammaglobulinemia were included. All subclasses were reconstituted in 85% of the patients. IgG3 and IgG4 were most commonly not reconstituted. Reconstitution occurred in 85% of the patients on intravenous Ig (IVIG), 81% of the patients on conventional subcutaneous Ig (SCIG), and 100% of the patients on enzyme-facilitated subcutaneous Ig (fSCIG). The annual infection rate was 0.87 with IVIG, 0.88 with conventional SCIG, and 0.6 with fSCIG. IgG subclasses contributing to low total IgG included IgG1 (61%), IgG2 (49%), IgG3 (23%), and IgG4 (28%). In patients with concomitant specific antibody deficiency (n = 47), the most commonly impaired antibody responses were against pneumococcal serotypes 3, 4, 6b, 12f, and 23f. CONCLUSIONS: Failure to reconstitute subclasses does not correlate with an inadequate clinical response to immunoglobulin therapy in primary hypogammaglobulinemia. Full reconstitution of IgG subclasses was observed with fSCIG. A smaller panel of pneumococcal antibody responses may be used to define specific antibody deficiency.


Assuntos
Agamaglobulinemia/terapia , Isotipos de Imunoglobulinas/biossíntese , Imunoglobulinas Intravenosas/uso terapêutico , Imunoterapia/métodos , Infecções Pneumocócicas/epidemiologia , Streptococcus pneumoniae/fisiologia , Adolescente , Adulto , Agamaglobulinemia/epidemiologia , Agamaglobulinemia/imunologia , Idoso , Idoso de 80 Anos ou mais , Anticorpos Antibacterianos/sangue , Autorrenovação Celular , Feminino , Homeostase , Humanos , Infusões Subcutâneas , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Estados Unidos , Adulto Jovem
16.
Muscle Nerve ; 60(6): 707-715, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31487038

RESUMO

INTRODUCTION: The Myasthenia Gravis Patient Registry (MGR) is a voluntary, patient-submitted database dedicated to improve understanding of care/burden of myasthenia gravis (MG). METHODS: In this study we present analyses of baseline records through July 2017 (n = 1140) containing data on the MG-Activities of Daily Living (MG-ADL) and the MG 15-item Quality of Life (MG-QOL15) instruments, two validated scales assessing quality of life in MG patients at sign-up into the MGR. RESULTS: Most registrants reported moderate to severe impairment of health-related quality of life, with a median MG-ADL score of 6 and a median MG-QOL15 score of 21. Seventy-one percent of the patients had received pyridostigmine. Corticosteroids, mycophenolate mofetil, and azathioprine were the most common immunomodulators/immunosuppressants, with 85% of participants having ever using one of these agents. Forty-seven registrants reported receiving intravenous immunoglobulin, and 30% received plasma exchange. Twelve percent reported other treatments, and 40% were unsure whether they received less common therapies. Forty percent had undergone thymectomy. DISCUSSION: The MGR data correlate well with other MG cohorts. Many MG patients remain negatively impacted despite treatment.


Assuntos
Atividades Cotidianas , Inibidores da Colinesterase/uso terapêutico , Imunossupressores/uso terapêutico , Miastenia Gravis/fisiopatologia , Miastenia Gravis/terapia , Qualidade de Vida , Corticosteroides/uso terapêutico , Adulto , Idoso , Azatioprina/uso terapêutico , Estudos Transversais , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/uso terapêutico , Troca Plasmática/métodos , Brometo de Piridostigmina/uso terapêutico , Sistema de Registros
18.
Transplant Proc ; 51(7): 2254-2256, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31474291

RESUMO

OBJECTIVE: High panel-reactive antibody (PRA) levels limit patients' access to kidney transplantation from potential living donor candidates and decrease renal graft survival by causing acute antibody-mediated rejection (AAMR). In this article, we report our experiences about the efficiency of plasmapheresis (PP) and intravenous immunoglobulin (IVIG) in reduction of serum PRA levels in candidates for renal transplantation and in patients with AAMR. METHODS: We examined retrospectively 47 patients with high PRA levels (18 for desensitization (DS) and 29 with AAMR) at Ankara University. The reduction in PRA class 1 and PRA class 2 levels before and after the PP, IVIG, and rituximab or eculizumab therapy were evaluated. RESULTS: In the DS group, mean reduction in PRA class I ± SD was 28.0 ± 9.10 to 22.1 ± 8.14 (P <.05), and mean reduction in PRA class II ± SD was 40.3 ± 6.89 to 32.2 ± 5.68 (P < .05). In the AAMR group; mean reduction in PRA class I ± SD was 23.9 ± 9.56 to 17.8 ± 8.64 (P > .05), and mean reduction in PRA class II ± SD was 28.1 ± 8.37 to 26.7 ± 7.96 (P > .05). In total, mean reduction in PRA class I was 25.7 ± 6.66 to 19.7 ± 6.00 (P < .01). Mean reduction in PRA class II was 33.8 ± 5.93 to 29.2 ± 4.96 (P > .05). In the DS group, 3 (16.7%) patients were treated with rituximab. In the AAMR group, 9 (31.0%) patients were treated with rituximab, and 1 (5.5%) patient received eculizimab.In the DS group, the mean follow-up period in years ± SD was 5.06 ± 3.01 and no patient had graft loss. In the AAMR group, the mean follow-up period in years was 5.06 ± 2.74 and 6 (33.3%) patients had graft loss with acute rejection. CONCLUSIONS: PP and IVIG treatment provide significant reduction in PRA levels and can be used in DS protocols.


Assuntos
Dessensibilização Imunológica/métodos , Rejeição de Enxerto/terapia , Imunoglobulinas Intravenosas/uso terapêutico , Transplante de Rim/efeitos adversos , Plasmaferese/métodos , Adulto , Feminino , Rejeição de Enxerto/imunologia , Sobrevivência de Enxerto/imunologia , Humanos , Fatores Imunológicos/uso terapêutico , Masculino , Pessoa de Meia-Idade , Período Pós-Operatório , Estudos Retrospectivos , Resultado do Tratamento
19.
Arerugi ; 68(7): 857-868, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31406082

RESUMO

BACKGROUND AND AIMS: It is difficult to evaluate neurological signs of multiple mononeuritis (MM) in patients with eosinophilic granulomatosis with polyangiitis (EGPA). We created a new questionnaire about motor and sensory disturbances in EGPA and investigated whether the questionnaire would be a useful tool in the management of MM in EGPA patients. METHODS: We classified 40 EGPA patients attending Hiratsuka City Hospital into two groups, namely 30 who were treated with intravenous immunoglobulin (IVIG) and 10 who achieved remission by conventional treatment without IVIG. We created a questionnaire for the evaluation of motor and sensory disturbance in EGPA (ANCA related.com). In patients who received IVIG, we evaluated motor and sensory disturbance scores at disease onset, before IVIG, 1 week and 1 month after the end of IVIG. In patients treated without IVIG, we evaluated these scores at disease onset and at the time of the latest examination. RESULTS: The total motor disturbance score at disease onset was significantly lower in EGPA patients who received IVIG than in those who did not receive it. Disease duration was significantly inversely correlated with the change in the sensory disturbance ratio, but not with the motor disturbance ratio. The motor disturbance ratio was significantly correlated with the manual muscle test improvement ratio. In patients who received IVIG, the total motor disturbance score increased significantly, and the total sensory disturbance score decreased significantly, 1 month after IVIG. CONCLUSION: By using the questionnaire we could evaluate changes in motor and sensory disturbance after IVIG treatment in patients with EGPA. The questionnaire should be useful in the management of MM in EGPA patients.


Assuntos
Síndrome de Churg-Strauss/diagnóstico , Granulomatose com Poliangiite/diagnóstico , Inquéritos e Questionários , Síndrome de Churg-Strauss/complicações , Granulomatose com Poliangiite/complicações , Humanos , Imunoglobulinas Intravenosas/uso terapêutico
20.
J Clin Neurosci ; 69: 114-119, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31416736

RESUMO

We determined the clinical progression, disability and outcome of 11 Multifocal Motor Neuropathy (MMN) patients from Malaysia. Mean patient age was 46.8 (SD 13.3), with mean disease duration of 108.0 months (SD 80.2). All reported unilateral limb weakness at onset. At diagnosis, after mean 49.9 months (SD 73.5) delay, 7 (63.6%) had more than 2 limbs involvement. Nine (90%) of 10 patients received induction IVIg dose of 2.0 gm/kg responded, demonstrated improvement in MRCSS of > 2 points or mRS score of > 1 point. We observed 38.5% drop in IVIg dose to mean 1.12 gm/kg/month after 12 months of treatment, and a further 34.8% drop upon 24th month treatment to mean dose of 0.73 gm/kg/month. This was in parallel with initial improvement in MRCSS and mRS, observed among 88.9% and 77.8% of the patients, and later further improvement (33.3%) or stabilization (66.7%) of mRS score toward 2nd year. During the same period, 50% of patients reported deterioration in ONLS, 33.3% in grip strength and 16.7% in MMN-RODS. Beyond 36th month, average annual IVIg dose increased at 0.12 gm/kg/year (SD 0.09) or 11.2%, up to the 84th months. Despite that, progressive deterioration was observed in term of number of limbs involvement, definite motor conduction blocks on electrophysiology study, and both clinical as well as functional scores. Although IVIg dose reduction for maintenance treatment in MMN is recommended, careful clinical assessment is required to prevent under-treatment. Use of reliable and responsive modern outcome measures is important to quantify clinically relevant change to guide therapy.


Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Doença dos Neurônios Motores/tratamento farmacológico , Adulto , Avaliação da Deficiência , Progressão da Doença , Feminino , Humanos , Malásia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
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