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2.
J Hematol Oncol ; 13(1): 131, 2020 10 02.
Artigo em Inglês | MEDLINE | ID: mdl-33008453

RESUMO

SARS-CoV-2 has infected millions of people worldwide, but little is known at this time about second infections or reactivation. Here, we report a case of a 55-year-old female undergoing treatment for CD20+ B cell acute lymphoblastic leukemia who experienced a viral reactivation after receiving rituximab, cytarabine, and dasatinib. She was initially hospitalized with COVID-19 in April and developed a high antibody titer with two negative nasal polymerase chain reaction (PCR) swabs for SARS-CoV-2 on discharge. After recovery, she resumed treatment in June for her leukemia, which included rituximab, cytarabine, and dasatinib. She promptly lost her COVID-19 antibodies, and her nasal PCR turned positive in June. She developed a severe COVID-19 pneumonia with lymphopenia, high inflammatory markers, and characteristic bilateral ground-glass opacities on chest CT, requiring high-flow nasal cannula and transfer to the intensive care unit. She received steroids, anticoagulation, and convalescent plasma, and within 48 h she was off oxygen. She was discharged home in stable condition several days later. Given the short time frame from leukemia treatment to PCR positivity and the low case rate in mid-June in New York City, reinfection appears to have been unlikely and SARS-CoV-2 reactivation is a possible explanation. This case illustrates the risks of treating recently recovered COVID-19 patients with immunosuppressive therapy, particularly lymphocyte- and antibody-depleting therapy, and raises new questions about the potential of SARS-CoV-2 reactivation.


Assuntos
Betacoronavirus/genética , Infecções por Coronavirus/imunologia , Citarabina/uso terapêutico , Imunossupressores/uso terapêutico , Pneumonia Viral/imunologia , Leucemia-Linfoma Linfoblástico de Células Precursoras B/tratamento farmacológico , Rituximab/uso terapêutico , Doença Aguda , Anticorpos Antivirais/sangue , Anticorpos Antivirais/imunologia , Anticoagulantes/uso terapêutico , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/terapia , Infecções por Coronavirus/virologia , Citarabina/efeitos adversos , Feminino , Humanos , Imunização Passiva , Imunossupressores/efeitos adversos , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/tratamento farmacológico , Pneumonia Viral/virologia , Reação em Cadeia da Polimerase , Recidiva , Rituximab/efeitos adversos , Esteroides/uso terapêutico , Resultado do Tratamento
3.
Rev. esp. patol ; 53(3): 182-187, jul.-sept. 2020. ilus, tab
Artigo em Inglês | IBECS | ID: ibc-192407

RESUMO

We describe the implementation of a COVID-19 Autopsy Programme in our Hospital, report the main findings from the first autopsy of the programme and briefly review the reports of lung pathology of these patients


En este artículo presentamos el proceso de implementación de un Programa de Autopsias COVID-19 en nuestro hospital, presentamos los principales hallagos de la primera autopsia realizada y revisamos brevemente la patología pulmonar publicada previamente en estos pacientes


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Embolia Pulmonar/complicações , Autopsia/estatística & dados numéricos , Causas de Morte , Infecções por Coronavirus/patologia , Alvéolos Pulmonares/patologia , Síndrome Respiratória Aguda Grave/complicações , Vírus da SARS/isolamento & purificação , Apneia Obstrutiva do Sono/complicações , Hipertensão/complicações , Reação em Cadeia da Polimerase/métodos , Esteroides/uso terapêutico , Pandemias , Técnicas Histológicas/métodos , Espanha/epidemiologia
4.
Pediatrics ; 146(4)2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32943536

RESUMO

OBJECTIVES: To evaluate the survival and neurodevelopmental impairment (NDI) in extremely low birth weight (ELBW) infants at 18 to 26 months with early hypoxemic respiratory failure (HRF). We also assessed whether African American infants with early HRF had improved outcomes after exposure to inhaled nitric oxide (iNO). METHODS: ELBW infants ≤1000 g and gestational age ≤26 weeks with maximal oxygen ≥60% on either day 1 or day 3 were labeled as "early HRF" and born between 2007 and 2015 in the Neonatal Research Network were included. Using a propensity score regression model, we analyzed outcomes and effects of exposure to iNO overall and separately by race. RESULTS: Among 7639 ELBW infants born ≤26 weeks, 22.7% had early HRF. Early HRF was associated with a mortality of 51.3%. The incidence of moderate-severe NDI among survivors was 41.2% at 18 to 26 months. Mortality among infants treated with iNO was 59.4%. Female sex (adjusted odds ratio [aOR]: 2.4, 95% confidence interval [CI]: 1.8-3.3), birth weight ≥720 g (aOR: 2.3, 95% CI: 1.7-3.1) and complete course of antenatal steroids (aOR: 1.6, 95% CI: 1.1-2.2) were associated with intact survival. African American infants had a similar incidence of early HRF (21.7% vs 23.3%) but lower exposure to iNO (16.4% vs 21.6%). Among infants with HRF exposed to iNO, intact survival (no death or NDI) was not significantly different between African American and other races (aOR: 1.5, 95% CI: 0.6-3.6). CONCLUSIONS: Early HRF in infants ≤26 weeks' gestation is associated with high mortality and NDI at 18 to 26 months. Use of iNO did not decrease mortality or NDI. Outcomes following iNO exposure were not different in African American infants.


Assuntos
Broncodilatadores/uso terapêutico , Hipóxia/complicações , Lactente Extremamente Prematuro , Transtornos do Neurodesenvolvimento/epidemiologia , Óxido Nítrico Sintase Tipo II/uso terapêutico , Insuficiência Respiratória/mortalidade , Administração por Inalação , Afro-Americanos , Índice de Apgar , Peso ao Nascer , Broncodilatadores/administração & dosagem , Feminino , Ruptura Prematura de Membranas Fetais , Mortalidade Hospitalar , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Incidência , Lactente , Recém-Nascido de Peso Extremamente Baixo ao Nascer , Recém-Nascido , Masculino , Transtornos do Neurodesenvolvimento/diagnóstico , Transtornos do Neurodesenvolvimento/tratamento farmacológico , Transtornos do Neurodesenvolvimento/etnologia , Óxido Nítrico Sintase Tipo II/administração & dosagem , Alta do Paciente , Gravidez , Pontuação de Propensão , Insuficiência Respiratória/tratamento farmacológico , Insuficiência Respiratória/etnologia , Insuficiência Respiratória/etiologia , Fatores de Risco , Fatores Sexuais , Esteroides/uso terapêutico
5.
PLoS One ; 15(9): e0237523, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32870918

RESUMO

BACKGROUND: Meniere's disease (MD) is a chronic condition of the inner ear consisting of symptoms that include vertigo attacks, fluctuating sensorineural hearing loss, tinnitus and aural fullness. Despite availability of various interventions, there is uncertainty surrounding their relative efficacy, thus making it difficult to select the appropriate treatments for MD. The objective of this systematic review was to assess the relative effects of the available pharmacologic and surgical interventions in patients with MD with regard to vertigo and other key patient outcomes based on data from randomized clinical trials (RCTs). METHODS: Our published protocol registered with PROSPERO (CRD42019119129) provides details on eligibility criteria and methods. We searched various databases including MEDLINE, Embase and the Cochrane Library from inception to December 10th, 2018. Screening at citation and full-text levels and risk of bias assessment were performed by two independent reviewers in duplicate, with discrepancies resolved by consensus or third-party adjudication. Bayesian network meta-analyses (NMA) were performed for hearing change and vertigo control outcomes, along with pairwise meta-analyses for these and additional outcomes. RESULTS: We identified 2,889 unique citations, that yielded 23 relevant publications describing 18 unique RCTs (n = 1,231 patients). Overall, risk-of bias appraisal suggested the evidence base to be at unclear or high risk of bias. Amongst pharmacologics, we constructed treatment networks of five intervention groups that included placebo, intratympanic (IT) gentamicin, oral high-dose betahistine, IT steroid and IT steroid plus high-dose betahistine for NMAs of hearing change (improvement or deterioration) and complete vertigo control. IT steroid plus high-dose betahistine was associated with the largest difference in hearing improvement compared to placebo, followed by high-dose betahistine and IT steroid (though 95% credible intervals failed to rule out the possibility of no difference), while IT gentamicin was worse than IT steroid. The NMA of complete vertigo control suggested IT gentamicin was associated with the highest probability of achieving better complete vertigo control compared to placebo, followed by IT steroid plus high-dose betahistine. Only two studies related to surgical interventions were found, and data suggested no statistically significant difference in hearing changes between endolymphatic duct blockage (EDB) versus endolymphatic sac decompression (ESD), and ESD with or without steroid injection. One trial reported that 96.5% of patients in EDB group compared to 37.5% of the patients in ESD group achieved complete vertigo control 24 months after surgery (p = 0.002). CONCLUSION: To achieve both hearing preservation and vertigo control, the best treatment option among the pharmacologic interventions compared may be IT steroid plus high-dose betahistine, considering that IT gentamicin may have good performance to control vertigo but may be detrimental to hearing preservation with high cumulative dosage and short interval between injections. However, IT steroid plus high-dose betahistine has not been compared in head-to-head trials against other interventions except for IT steroid alone in one trial, thus future trials that compare it with other interventions will help establish comparative effectiveness with direct evidence.


Assuntos
Doença de Meniere/tratamento farmacológico , Doença de Meniere/cirurgia , Antibacterianos/uso terapêutico , beta-Histina/uso terapêutico , Gentamicinas/uso terapêutico , Audição/efeitos dos fármacos , Humanos , Esteroides/uso terapêutico , Resultado do Tratamento , Vasodilatadores/uso terapêutico
6.
BMC Infect Dis ; 20(1): 708, 2020 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-32993546

RESUMO

BACKGROUND: Intravesical administration of Bacillus Calmette-Guérin (BCG) has proven useful for treatment and prevention of recurrence of superficial bladder cancer and in situ carcinoma. However, fatal side effects such as disseminated infections may occur. Early diagnosis and accurate therapy for interstitial pneumonitis (IP) are important because exacerbation of IP triggered by infections is the major cause of death. Although some fatality reports have suggested newly appeared IP after intravesical BCG treatment, to our knowledge, there are no reports which have demonstrated acute exacerbation of existing IP. Moreover, autopsy is lacking in previous reports. We report the case of a patient with fatal IP exacerbation after BCG instillation and the pathological findings of the autopsy. CASE PRESENTATION: A 77-year-old man with a medical history of IP was referred to our hospital because of fever and malaise. He had received an intravesical injection of BCG 1 day before the admission. His fever reduced after the use of antituberculosis drugs, so he was discharged home. He was referred to our hospital again because of a high fever 7 days after discharge. On hospitalisation, he showed high fever and systemic exanthema. Hepatosplenomegaly and myelosuppression were also observed. Biopsies revealed multiple epithelioid cell granulomas with Langhans giant cells of the liver and bone marrow. Biopsy DNA analyses of Mycobacterium bovis in the bone marrow, sputum, and blood were negative. His oxygen demand worsened drastically, and the ground-glass shadow expanded on the computed tomography scan. He was diagnosed with acute exacerbation of existing IP. We recommenced the antituberculosis drugs with steroid pulse therapy, but he died on day 35 because of respiratory failure. The autopsy revealed a diffuse appearance of multiple epithelioid cell granulomas with Langhans giant cells in multiple organs, although BCG was not evident. CONCLUSIONS: We report the first case of acute exacerbation of chronic IP by BCG infection. This is also the first case of autopsy of a patient with acute exacerbation of existing IP induced by intravesical BCG treatment. Whether the trigger of acute IP exacerbation is infection or hypersensitivity to BCG is still controversial, because pathological evidence confirming BCG infection is lacking. Physicians who administer BCG against bladder cancer should be vigilant for acute exacerbation of IP.


Assuntos
Antituberculosos/uso terapêutico , Vacina BCG/efeitos adversos , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico , Infecções por Mycobacterium não Tuberculosas/etiologia , Esteroides/uso terapêutico , Exacerbação dos Sintomas , Administração Intravesical , Idoso , Autopsia , Vacina BCG/administração & dosagem , Vacina BCG/uso terapêutico , Carcinoma in Situ/tratamento farmacológico , Carcinoma in Situ/prevenção & controle , Evolução Fatal , Humanos , Doenças Pulmonares Intersticiais/microbiologia , Masculino , Infecções por Mycobacterium não Tuberculosas/diagnóstico , Mycobacterium bovis/genética , Resultados Negativos , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/prevenção & controle , Pulsoterapia , Resultado do Tratamento , Neoplasias da Bexiga Urinária/tratamento farmacológico , Neoplasias da Bexiga Urinária/prevenção & controle
7.
Drug Discov Ther ; 14(4): 171-176, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32908072

RESUMO

The healthcare sector has been overwhelmed by the global rise in the number of COVID-19 cases. The primary care physicians at the forefront of this pandemic are being provided with multiple guidelines (state, national, international). The aim of this review was to examine the existing guidelines for congruence and critically analyze them in light of current evidence. A discordance was noted between the national and state guidelines with respect to indication, duration and dosage of antivirals, steroids/immunomodulators, anticoagulation and convalescent plasma. The lack of concordance between various guidelines mandates the need for a unified national guideline that is regularly updated.


Assuntos
Antivirais/uso terapêutico , Betacoronavirus/efeitos dos fármacos , Infecções por Coronavirus/terapia , Pneumonia Viral/terapia , Guias de Prática Clínica como Assunto/normas , Padrões de Prática Médica/normas , Anticoagulantes/uso terapêutico , Antivirais/efeitos adversos , Betacoronavirus/patogenicidade , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/virologia , Interações entre Hospedeiro e Microrganismos , Humanos , Imunização Passiva , Fatores Imunológicos/uso terapêutico , Índia/epidemiologia , Pandemias , Pneumonia Viral/epidemiologia , Pneumonia Viral/imunologia , Pneumonia Viral/virologia , Esteroides/uso terapêutico
8.
Medicine (Baltimore) ; 99(34): e21936, 2020 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-32846862

RESUMO

RATIONALE: IgG4-related disease (IgG4-RD) is a systemic disease that can involve various organs and is characterized by the infiltrations of IgG4-positive plasma cells and lymphocytes, fibrosis, and elevated serum IgG4 levels. IgG4-related sclerosing cholangitis (IgG4-RSC) is a subtype of IgG4-RD. No certain relationship between IgG4-RSC and cholangiocarcinoma has been established as yet, and there have been few reports of the simultaneous diagnosis of IgG4-RSC and cholangiocarcinoma. PATIENT CONCERNS: A 76-year-old male visited our gastroenterology department due to the recent occurrence of pruritus and jaundice. DIAGNOSIS: Computed tomography (CT) scan showed ductal wall swelling and enhancement from both intrahepatic duct confluence to the common bile duct, upper biliary dilatation, and accompanying autoimmune pancreatitis (a sub type of IgG4-RD). Biopsy of the distal common bile duct by endoscopic retrograde cholangiopancreatography (ERCP) resulted in a diagnosis of IgG4-RSC. Subsequently, adenocarcinoma was identified by repeated cytology of bile juice. Finally, Klatskin tumor type IIIA and IgG4-RSC were concurrently diagnosed. INTERVENTIONS: IgG4-RSC was treated with steroid and Klatskin tumors by gemcitabine + cisplatin chemotherapy. OUTCOMES: The jaundice had improved and CT showed substantial improvement of the intrahepatic duct dilatation. LESSONS: IgG4-RSC and cholangiocarcinoma are easily confused, but their treatments are quite different, and thus, care must be taken during diagnosis. Furthermore, these 2 diseases may co-exist. Therefore, even if IgG4-RSC is diagnosed first, the possibility of accompanying cholangiocarcinoma should be thoroughly investigated.


Assuntos
Colangiocarcinoma/complicações , Colangite Esclerosante/patologia , Imunoglobulina G/imunologia , Tumor de Klatskin/complicações , Tumor de Klatskin/patologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Colangiocarcinoma/patologia , Colangiopancreatografia Retrógrada Endoscópica , Colangite Esclerosante/diagnóstico por imagem , Colangite Esclerosante/tratamento farmacológico , Cisplatino/uso terapêutico , Ducto Colédoco/patologia , Desoxicitidina/análogos & derivados , Desoxicitidina/uso terapêutico , Diagnóstico Diferencial , Humanos , Doença Relacionada a Imunoglobulina G4/patologia , Icterícia/diagnóstico , Icterícia/etiologia , Tumor de Klatskin/classificação , Tumor de Klatskin/tratamento farmacológico , Masculino , Prurido/diagnóstico , Prurido/etiologia , Esteroides/uso terapêutico , Tomografia Computadorizada por Raios X , Resultado do Tratamento
9.
Brasília; S.N; 23 jul. 2020.
Não convencional em Português | LILACS, BRISA/RedTESA, PIE | ID: biblio-1117682

RESUMO

O Informe Diário de Evidências é uma produção do Ministério da Saúde que tem como objetivo acompanhar diariamente as publicações científicas sobre tratamento farmacológico e vacinas para a COVID-19. Dessa forma, são realizadas buscas estruturadas em bases de dados biomédicas, referentes ao dia anterior desse informe. Não são incluídos estudos pré-clínicos (in vitro, in vivo, in silico). A frequência dos estudos é demonstrada de acordo com a sua classificação metodológica (revisões sistemáticas, ensaios clínicos randomizados, coortes, entre outros). Para cada estudo é apresentado um resumo com avaliação da qualidade metodológica. Essa avaliação tem por finalidade identificar o grau de certeza/confiança ou o risco de viés de cada estudo. Para tal, são utilizadas ferramentas já validadas e consagradas na literatura científica, na área de saúde baseada em evidências. Cabe ressaltar que o documento tem caráter informativo e não representa uma recomendação oficial do Ministério da Saúde sobre a temática. Foram encontrados 21 artigos e 8 protocolos.


Assuntos
Pneumonia Viral/tratamento farmacológico , Infecções por Coronavirus/tratamento farmacológico , Esteroides/uso terapêutico , Avaliação da Tecnologia Biomédica , Vacina BCG/uso terapêutico , Heparina/uso terapêutico , Almitrina/uso terapêutico , Estudos de Coortes , Corticosteroides/uso terapêutico , Enoxaparina/uso terapêutico , Azitromicina/uso terapêutico , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Darunavir/uso terapêutico , Betacoronavirus/efeitos dos fármacos , Ipilimumab/uso terapêutico , Fondaparinux/uso terapêutico , Nivolumabe/uso terapêutico , Antagonistas dos Receptores Histamínicos/uso terapêutico , Hidroxicloroquina/uso terapêutico , Anticoagulantes/uso terapêutico
10.
Brasília; s.n; 7 jul. 2020.
Não convencional em Português | LILACS, BRISA/RedTESA, PIE | ID: biblio-1117630

RESUMO

O Informe Diário de Evidências é uma produção do Ministério da Saúde que tem como objetivo acompanhar diariamente as publicações científicas sobre tratamento farmacológico e vacinas para a COVID-19. Dessa forma, são realizadas buscas estruturadas em bases de dados biomédicas, referente ao dia anterior desse informe. Não são incluídos estudos pré-clínicos (in vitro, in vivo, in silico). A frequência dos estudos é demonstrada de acordo com a sua classificação metodológica (revisões sistemáticas, ensaios clínicos randomizados, coortes, entre outros). Para cada estudo é apresentado um resumo com avaliação da qualidade metodológica. Essa avaliação tem por finalidade identificar o grau de certeza/confiança ou o risco de viés de cada estudo. Para tal, são utilizadas ferramentas já validadas e consagradas na literatura científica, na área de saúde baseada em evidências. Cabe ressaltar que o documento tem caráter informativo e não representa uma recomendação oficial do Ministério da Saúde sobre a temática. Foram encontrados 17 artigos.


Assuntos
Humanos , Pneumonia Viral/tratamento farmacológico , Infecções por Coronavirus/tratamento farmacológico , Betacoronavirus/efeitos dos fármacos , Esteroides/uso terapêutico , Avaliação da Tecnologia Biomédica , Vitamina D/uso terapêutico , Varfarina/uso terapêutico , Ivermectina/uso terapêutico , Ceftriaxona/uso terapêutico , Cloroquina/uso terapêutico , Metotrexato/uso terapêutico , Corticosteroides/uso terapêutico , Azitromicina/uso terapêutico , Ritonavir/uso terapêutico , Oseltamivir/uso terapêutico , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Lopinavir/uso terapêutico , Infliximab/uso terapêutico , Leflunomida/uso terapêutico , Amoxicilina/uso terapêutico , Hidroxicloroquina/uso terapêutico , Ácido Micofenólico/uso terapêutico
11.
BMC Infect Dis ; 20(1): 538, 2020 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-32703300

RESUMO

BACKGROUND: The risk of life-threatening complications, such as visceral disseminated varicella zoster virus (VZV) infection, is greater in immunosuppressed individuals, such as systemic lupus erythematosus (SLE) patients. CASE PRESENTATION: Here, a case is reported of a Caucasian woman diagnosed with lupus nephritis and anti-phospholipid syndrome, who was subjected to mycophenolate mofetil and high-dose steroid remission-induction therapy. Two months later she developed abdominal pain followed by a fatal rapid multi-organ failure. As no typical skin rashes were evident, death was initially attributed to catastrophic anti-phospholipid syndrome. However, autopsy and virological examinations on archival material revealed a disseminated VZV infection. CONCLUSIONS: Overall, this case highlights the importance of having a high clinical suspicion of fatal VZV infections in heavily immunosuppressed SLE patients even when typical signs and symptoms are lacking.


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Herpes Zoster/complicações , Herpes Zoster/diagnóstico , Herpesvirus Humano 3/genética , Nefrite Lúpica/complicações , Nefrite Lúpica/diagnóstico , Dor Abdominal , Evolução Fatal , Feminino , Herpes Zoster/patologia , Herpes Zoster/virologia , Herpesvirus Humano 3/isolamento & purificação , Humanos , Hospedeiro Imunocomprometido , Nefrite Lúpica/tratamento farmacológico , Pessoa de Meia-Idade , Ácido Micofenólico/uso terapêutico , Reação em Cadeia da Polimerase em Tempo Real , Esteroides/uso terapêutico
12.
Ann Hematol ; 99(9): 2085-2093, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32710167

RESUMO

Clinical research has resulted in an improvement of treatment options for patients with immune thrombocytopenia (ITP) over the last years. However, only few data exist on the real-life management of patients with ITP. To expand the knowledge, a multicenter, national survey was undertaken in 26 hematology practices distributed all over Germany. All patients with a diagnosis of ITP were documented using questionnaires, irrespective of the diagnosis date over a period of 2 years. Overall, data of 1023 patients were evaluated with 56% of patients being older than 60 years. Seventy-nine percent of the patients had chronic (> 12 months), 16% persistent (> 3-12 months), and 5% newly diagnosed (0-3 months) ITP. In 61% of cases, the disease lasted 3 or more years before survey documentation started. Main strategies applied as first-line therapy consisted of steroids in 45% and a "watch and wait" approach in 41% of patients. During second- and third-line strategies, treatment with steroids decreased (36% and 28%, respectively), while treatment modalities such as TPO-RAs increased (19% and 26%, respectively). As expected, patients with a low platelet count and thus a higher risk for bleeding and mortality received treatment (esp. steroids) more frequently during first line than those with a higher platelet count. Up to a third of patients were treated with steroids for more than a year. Overall, our study provides a cross-section overview about the current therapeutic treatment landscape in German ITP patients. The results will help to improve therapeutic management of ITP patients.


Assuntos
Gerenciamento Clínico , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/terapia , Inquéritos e Questionários , Adolescente , Adulto , Criança , Feminino , Alemanha/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/sangue , Estudos Retrospectivos , Esplenectomia/tendências , Esteroides/uso terapêutico , Adulto Jovem
17.
Hautarzt ; 71(10): 802-804, 2020 Oct.
Artigo em Alemão | MEDLINE | ID: mdl-32533203

RESUMO

We present a rare case of a 4-year-old girl suffering from a sclerotic lesion on the forehead as well as uveitis and epileptic seizures. The patient was diagnosed with linear scleroderma "en coup de sabre" (LSECDS). Faced with no gold standard for treatment of LSECDS with severe extracutaneous manifestations, the case was intensively discussed within an interdisciplinary team setting and successfully treated.


Assuntos
Esclerodermia Localizada/diagnóstico , Dermatopatias/complicações , Pré-Escolar , Eletroencefalografia , Feminino , Testa , Humanos , Esclerodermia Localizada/tratamento farmacológico , Esteroides/uso terapêutico , Resultado do Tratamento
18.
Neurology ; 95(2): e111-e120, 2020 07 14.
Artigo em Inglês | MEDLINE | ID: mdl-32554760

RESUMO

OBJECTIVE: Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments. METHODS: We determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy. RESULTS: Seventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3-61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5). CONCLUSION: This large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest ARR being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.


Assuntos
Imunoterapia/métodos , Glicoproteína Mielina-Oligodendrócito/imunologia , Esteroides/uso terapêutico , Adolescente , Adulto , Idade de Início , Antirreumáticos/uso terapêutico , Criança , Pré-Escolar , Doenças Desmielinizantes/imunologia , Doenças Desmielinizantes/prevenção & controle , Doenças Desmielinizantes/terapia , Feminino , Humanos , Imunização Passiva , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/complicações , Esclerose Múltipla/terapia , Recidiva , Estudos Retrospectivos , Esteroides/administração & dosagem , Adulto Jovem
19.
Presse Med ; 49(2): 103909, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32563946

RESUMO

Interstitial lung disease (ILD) in children (chILD) is a heterogeneous group of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. The pathogenesis of the various chILD is complex and the diseases share common features of inflammatory and fibrotic changes of the lung parenchyma that impair gas exchanges. The etiologies of chILD are numerous. In this review, we chose to classify them as ILD related to exposure/environment insults, ILD related to systemic and immunological diseases, ILD related to primary lung parenchyma dysfunctions and ILD specific to infancy. A growing part of the etiologic spectrum of chILD is being attributed to molecular defects. Currently, the main genetic mutations associated with chILD are identified in the surfactant genes SFTPA1, SFTPA2, SFTPB, SFTPC, ABCA3 and NKX2-1. Other genetic contributors include mutations in MARS, CSF2RA and CSF2RB in pulmonary alveolar proteinosis, and mutations in TMEM173 and COPA in specific auto-inflammatory forms of chILD. However, only few genotype-phenotype correlations could be identified so far. Herein, information is provided about the clinical presentation and the diagnosis approach of chILD. Despite improvements in patient management, the therapeutic strategies are still relying mostly on corticosteroids although specific therapies are emerging. Larger longitudinal cohorts of patients are being gathered through ongoing international collaborations to improve disease knowledge and targeted therapies. Thus, it is expected that children with ILD will be able to reach the adulthood transition in a better condition.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Fatores Etários , Criança , Doença Crônica , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico , Exposição Ambiental/efeitos adversos , Interação Gene-Ambiente , Genótipo , Humanos , Doenças do Sistema Imunitário/complicações , Lactente , Recém-Nascido , Doenças Pulmonares Intersticiais/classificação , Doenças Pulmonares Intersticiais/tratamento farmacológico , Fenótipo , Proteinose Alveolar Pulmonar/genética , Transtornos Respiratórios/complicações , Sistema Respiratório/patologia , Esteroides/uso terapêutico
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