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1.
Medicine (Baltimore) ; 100(6): e24627, 2021 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-33578576

RESUMO

RATIONALE: Steroid-resistant nephrotic syndrome (SRNS) is a special kidney disease. SRNS is characterized by steroid-resistant, clinical variability, and genetic heterogeneity. Patients with SRNS often may eventually need renal transplantation. PATIENT CONCERNS: A 10-month-old Chinese male infant presented with oliguria, renal dysfunction, hypertension, and anemia. DIAGNOSES: Combined with clinical manifestations, laboratory testing and sequencing results, the patient was diagnosed as SRNS. INTERVENTIONS: Combined intravenous methylprednisolone and cefoperazone sulbactam did not improve the patient's condition. Thus, SRNS associated with hereditary nephrotic syndrome was strongly suspected. Genetic testing for hereditary renal disease of the patient revealed 2 novel heterozygous mutations in the Nucleoporin 93 (NUP93) gene, which were predicted pathogenic and harmful by bioinformatic softwares of SIFT, PolyPhen_2 and REVEL. OUTCOMES: As general physical health deterioration and renal dysfunction, the patient died of a severe infection. LESSONS: The novel NUP93 heterozygous mutations identified in the current study broadened the genetic spectrum of SRNS and further deepened our insight into pathogenic mutations of NUP93 to improve disease diagnosis.


Assuntos
Síndrome Nefrótica/diagnóstico , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Cefoperazona/administração & dosagem , Cefoperazona/uso terapêutico , Evolução Fatal , Aconselhamento Genético , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Lactente , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/genética
2.
Sci Rep ; 11(1): 2169, 2021 01 26.
Artigo em Inglês | MEDLINE | ID: mdl-33500507

RESUMO

To evaluate the predictive effect of T-lymphoid subsets on the conversion of common covid-19 to severe. The laboratory data were collected retrospectively from common covid-19 patients in the First People's Hospital of Zaoyang, Hubei Province, China and the Third People's Hospital of Kunming, Yunnan Province, China, between January 20, 2020 and March 15, 2020 and divided into training set and validation set. Univariate and multivariate logistic regression was performed to investigate the risk factors for the conversion of common covid-19 to severe in the training set, the prediction model was established and verified externally in the validation set. 60 (14.71%) of 408 patients with common covid-19 became severe in 6-10 days after diagnosis. Univariate and multiple logistic regression analysis revealed that lactate (P = 0.042, OR = 1097.983, 95% CI 1.303, 924,798.262) and CD8+ T cells (P = 0.010, OR = 0.903, 95% CI 0.835, 0.975) were independent risk factors for general type patients to turn to severe type. The area under ROC curve of lactate and CD8+ T cells was 0.754 (0.581, 0.928) and 0.842 (0.713, 0.970), respectively. The actual observation value was highly consistent with the prediction model value in curve fitting. The established prediction model was verified in 78 COVID-19 patients in the verification set, the area under the ROC curve was 0.906 (0.861, 0.981), and the calibration curve was consistent. CD8+ T cells, as an independent risk factor, could predict the transition from common covid-19 to severe.


Assuntos
Linfócitos T CD8-Positivos/virologia , Progressão da Doença , Corticosteroides/administração & dosagem , Adulto , Algoritmos , China , Feminino , Humanos , Hipóxia/metabolismo , Lopinavir/administração & dosagem , Masculino , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Análise Multivariada , Oxigênio/química , Valor Preditivo dos Testes , Prognóstico , Curva ROC , Reação em Cadeia da Polimerase em Tempo Real , Análise de Regressão , Estudos Retrospectivos , Fatores de Risco , Ritonavir/administração & dosagem
3.
BMJ Case Rep ; 14(1)2021 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-33431450

RESUMO

An 83-year-old woman was referred to hospital with a 2-week history of short-lived episodic unpleasant sensations in her head and running down her body. This was accompanied by new short-term memory impairment and arm spasms. Initial investigations including blood tests and brain imaging did not reveal the diagnosis. The patient developed an increasing frequency of abnormal movements of her face and arm. These were clinically recognised as faciobrachial dystonic seizures (FBDS). FBDS are pathognomonic of an autoimmune encephalitis caused by an antibody directed against leucine-rich glioma-inactivated 1 (LGI1). The clinical diagnosis resulted in treatment with immunotherapy, leading to cessation of seizures and rapid cognitive recovery. Later, the predicted serology was confirmed. This reversible and under-recognised cause of cognitive impairment, typically affecting elderly patients, can be diagnosed clinically to enable early and effective treatment.


Assuntos
Autoanticorpos/sangue , Disfunção Cognitiva/imunologia , Imunoterapia/métodos , Encefalite Límbica/diagnóstico , Convulsões/imunologia , Administração Intravenosa , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Disfunção Cognitiva/terapia , Feminino , Glucocorticoides/administração & dosagem , Humanos , Peptídeos e Proteínas de Sinalização Intracelular/imunologia , Encefalite Límbica/complicações , Encefalite Límbica/imunologia , Encefalite Límbica/terapia , Sistema Límbico/diagnóstico por imagem , Sistema Límbico/imunologia , Metilprednisolona/administração & dosagem , Troca Plasmática , Tomografia por Emissão de Pósitrons , Convulsões/terapia , Resultado do Tratamento
4.
BMJ Case Rep ; 14(1)2021 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-33431530

RESUMO

Granulomatosis with polyangiitis (GPA) is a necrotising vasculitis of unknown cause that has several systemic manifestations. The disease is characterised by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can involve the central nervous system. This case report presents a case of GPA with facial nerve palsy as the first manifestation of the disease, which has been rarely reported in the medical literature.


Assuntos
Paralisia Facial/etiologia , Granulomatose com Poliangiite/diagnóstico , Perfuração do Septo Nasal/etiologia , Convulsões/etiologia , Adolescente , Anticorpos Anticitoplasma de Neutrófilos/sangue , Encéfalo/diagnóstico por imagem , Ciclofosfamida/administração & dosagem , Paralisia Facial/sangue , Paralisia Facial/diagnóstico , Paralisia Facial/terapia , Feminino , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/terapia , Humanos , Imagem por Ressonância Magnética , Metilprednisolona/administração & dosagem , Perfuração do Septo Nasal/diagnóstico , Septo Nasal/diagnóstico por imagem , Seios Paranasais/diagnóstico por imagem , Plasmaferese , Pulsoterapia , Convulsões/sangue , Convulsões/diagnóstico , Convulsões/terapia , Tomografia Computadorizada por Raios X
5.
Med. clín (Ed. impr.) ; 156(1): 7-12, ene. 2021. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-196134

RESUMO

OBJETIVO: Analizar si existe asociación entre el uso de glucocorticoides a dosis altas y la evolución de la SAFI (saturación/fracción inspirada de oxígeno) o el tiempo hasta el alta, en pacientes hospitalizados por COVID-19. MÉTODOS: Estudio observacional sobre una cohorte de 418 pacientes ingresados en 3 hospitales comarcales de Cataluña (España). Como resultados primarios se estudiaron la evolución de la SAFI en las primeras 48h de tratamiento y el tiempo hasta el alta. Los resultados se compararon entre pacientes tratados y no tratados con glucocorticoides (metilprednisolona 1-2mg/kg/día o dexametasona 20-40mg/día), mediante el análisis de subcohortes emparejadas por múltiples factores clínicos y pronósticos, así como mediante modelos multivariantes de Cox, ajustados por diversos factores pronósticos. El uso simultáneo de diferentes tratamientos para la COVID-19 fue tenido en cuenta, tanto en el emparejamiento de subcohortes como en la regresión de Cox. RESULTADOS: Hubo 187 pacientes con glucocorticoides; de ellos, 25 pacientes pudieron ser emparejados con un número equivalente de pacientes control. En las subcohortes emparejadas, no se apreció diferencia en el tiempo hasta el alta (log-rank: p = 0,291), ni en el cambio en la SAFI a las 48h desde la basal (glucocorticoides: −0,04; controles: +0,37; p = 0,095). Los modelos multivariantes mediante regresión de Cox mostraron un tiempo hasta el alta significativamente más largo en pacientes tratados con glucocorticoides (hazard ratio: 7,26; IC 95%: 3,30-15,95). CONCLUSIONES: No hemos encontrado mejoría en la función respiratoria o tiempo hasta el alta, asociado al uso de glucocorticoides a dosis altas


OBJECTIVE: To analyze whether there is an association between the use glucocorticoids at high doses, and the evolution of saturation/fraction of inspired oxygen (SAFI) or time to discharge, in patients hospitalized with COVID-19. METHODS: This was an observational study on a cohort of 418 patients admitted to three regional hospitals in Catalonia, Spain. As primary outcomes, we studied the evolution of SAFI in the first 48hours of treatment and the time to discharge. The results were compared between patients treated and untreated with glucocorticoids (methylprednisolone 1-2mg/kg/day o dexamethasone 20-40mg/day) through sub-cohort analyses matched for multiple clinical and prognostic factors, as well as through Cox multivariate models adjusted for prognostic factors. The simultaneous use of different treatments for COVID-19 was taken into account, both in sub-cohorts matching and in Cox regression. RESULTS: There were 187 patients treated with glucocorticoids; of these, 25 patients could be matched with an equivalent number of control patients. In the analysis of these matched sub-cohorts, no significant difference was observed in time to discharge (log-rank: p = 0.291) or the increment in SAFI at 48hours of treatment (glucocorticoides: −0.04; controls: +0.37; p = 0.095). Multivariate models using Cox regression showed a significantly longer time to discharge in patients treated with glucocorticoids (hazard ratio: 7.26; 95% IC: 3.30-15.95). CONCLUSIONS: We have not found improvement in respiratory function or time until discharge, associated with the use of glucocorticoids at high doses


Assuntos
Humanos , Masculino , Feminino , Idoso , Glucocorticoides/administração & dosagem , Alta do Paciente , Estudos de Coortes , Infecções por Coronavirus/tratamento farmacológico , Pneumonia Viral/tratamento farmacológico , Betacoronavirus/efeitos dos fármacos , Oxigênio/administração & dosagem , Metilprednisolona/administração & dosagem , Dexametasona/administração & dosagem , Síndrome Respiratória Aguda Grave/tratamento farmacológico
6.
Ned Tijdschr Geneeskd ; 1642020 10 22.
Artigo em Holandês | MEDLINE | ID: mdl-33331715

RESUMO

Paediatric Multisystem Inflammatory Syndrome Temporally Related to SARS-CoV-2 (PIMS-TS) is a rare novel clinical entity observed in children and adolescents with evidence of a recent COVID-19 infection, and is characterized by a marked hyperinflammatory state with involvement of multiple organ systems.We report a case of a previously healthy 15-year-old female patient, who was admitted to paediatric intensive care with cardiac failure and was subsequently shown to have positive COVID-19 serology. The presenting symptoms were fever, cough, chest pain and gastro-intestinal symptoms. She was supported with milrinone and a low dose of vasopressors. Her hyperinflammatory state was treated with intravenous immunoglobulins, high dose aspirin and high-dose methylprednisolone. PIMS-TS is a rare, potentially life threatening novel clinical entity in children and adolescents with evidence of a COVID-19 infection. Clinicians need to be aware of the possibility of this new disease, to ensure prompt recognition and treatment.


Assuntos
Aspirina/administração & dosagem , Imunoglobulinas Intravenosas/administração & dosagem , Metilprednisolona/administração & dosagem , Milrinona/administração & dosagem , Síndrome de Resposta Inflamatória Sistêmica , Vasoconstritores/administração & dosagem , Adolescente , Anti-Inflamatórios/administração & dosagem , /fisiopatologia , Cardiotônicos/administração & dosagem , Relação Dose-Resposta a Droga , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Humanos , Síndrome de Resposta Inflamatória Sistêmica/imunologia , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Resultado do Tratamento
7.
PLoS One ; 15(12): e0244128, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33382734

RESUMO

PURPOSE: To evaluate the efficacy and safety of methylprednisolone in treating the coronavirus disease 2019 (COVID-19) patients. METHODS: A retrospective cohort study was conducted, and all COVID-19 patients were recruited who were admitted to the Yichang Third People's Hospital from February 1st to March 31st, 2020. One-to-one propensity score matching (PSM) was used for minimizing confounding effects. The primary outcome was hospital mortality, with the secondary outcomes being the time needed for a positive SARS-CoV-2 nucleic acid test to turn negative and the length of hospital stay. RESULTS: Totaling 367 patients with COVID-19 hospitalized at the Yichang Third People's Hospital were identified, of whom 276 were mild or stable COVID-19, and 67 were serious or critically ill. Among them, 255 patients were treated using methylprednisolone, and 188 did not receive any corticosteroid-related treatment. After PSM, no statistically significant difference was found in the baseline characteristics between the two groups. Regarding the outcomes, there also were no statistically significant difference between the two groups. Patients without the use of methylprednisolone were more quickly to obtain negative results of their nasopharyngeal swab tests of SARS-CoV-2 nucleic acid after treatment, compared to those receiving methylprednisolone. CONCLUSION: Methylprednisolone could not improve the prognosis of patients with COVID-19, and the efficacy and safety of the use of methylprednisolone in patients with COVID-19 still remain uncertain, thus the use of corticosteroids clinically in patients with COVID-19 should be with cautions.


Assuntos
Mortalidade Hospitalar , Tempo de Internação , Metilprednisolona/administração & dosagem , Adulto , Idoso , /tratamento farmacológico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Pontuação de Propensão , Estudos Retrospectivos
8.
BMJ Case Rep ; 13(12)2020 Dec 22.
Artigo em Inglês | MEDLINE | ID: mdl-33370994

RESUMO

Traumatic optic neuropathy is sinister sequelae of craniofacial trauma leading to vision loss. The decision between early medical or surgical intervention is usually individualised. Visual evoked potentials may guide the treatment plan. We describe a young male presenting 5 days after a road traffic accident with no perception of light vision in the right eye. He was managed medically with high dose of intravenous steroids. At the 3-month follow-up, he reported a reversal of vision loss with return of visual acuity to 3/60, which improved to 6/36 at 5 months and remained stable at 8 months.


Assuntos
Acidentes de Trânsito , Cegueira/tratamento farmacológico , Metilprednisolona/administração & dosagem , Traumatismos do Nervo Óptico/tratamento farmacológico , Fraturas Orbitárias/complicações , Administração Intravenosa , Adolescente , Cegueira/diagnóstico , Cegueira/etiologia , Potenciais Evocados Visuais/efeitos dos fármacos , Humanos , Masculino , Nervo Óptico/diagnóstico por imagem , Nervo Óptico/efeitos dos fármacos , Traumatismos do Nervo Óptico/diagnóstico , Traumatismos do Nervo Óptico/etiologia , Órbita/diagnóstico por imagem , Órbita/inervação , Fraturas Orbitárias/diagnóstico , Tomografia de Coerência Óptica , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Acuidade Visual/efeitos dos fármacos
9.
Crit Care ; 24(1): 643, 2020 11 10.
Artigo em Inglês | MEDLINE | ID: mdl-33172477

RESUMO

BACKGROUND: The impact of corticosteroid therapy on outcomes of patients with coronavirus disease 2019 (COVID-19) is highly controversial. We aimed to compare the risk of death between COVID-19-related ARDS patients with corticosteroid treatment and those without. METHODS: In this single-center retrospective observational study, patients with ARDS caused by COVID-19 between January 20, 2020, and February 24, 2020, were enrolled. The primary outcome was 60-day in-hospital death. The exposure was prescribed systemic corticosteroids or not. Time-dependent Cox regression models were used to calculate hazard ratios (HRs) and 95% confidence intervals (CIs) for 60-day in-hospital mortality. RESULTS: A total of 382 patients [60.7 ± 14.1 years old (mean ± SD), 61.3% males] were analyzed. The median of sequential organ failure assessment (SOFA) score was 2.0 (IQR 2.0-3.0). Of these cases, 94 (24.6%) patients had invasive mechanical ventilation. The number of patients received systemic corticosteroids was 226 (59.2%), and 156 (40.8%) received standard treatment. The maximum dose of corticosteroids was 80.0 (IQR 40.0-80.0) mg equivalent methylprednisolone per day, and duration of corticosteroid treatment was 7.0 (4.0-12.0) days in total. In Cox regression analysis using corticosteroid treatment as a time-varying variable, corticosteroid treatment was associated with a significant reduction in risk of in-hospital death within 60 days after adjusting for age, sex, SOFA score at hospital admission, propensity score of corticosteroid treatment, comorbidities, antiviral treatment, and respiratory supports (HR 0.42; 95% CI 0.21, 0.85; p = 0.0160). Corticosteroids were not associated with delayed viral RNA clearance in our cohort. CONCLUSION: In this clinical practice setting, low-dose corticosteroid treatment was associated with reduced risk of in-hospital death within 60 days in COVID-19 patients who developed ARDS.


Assuntos
Corticosteroides/administração & dosagem , Betacoronavirus , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/mortalidade , Pneumonia Viral/tratamento farmacológico , Pneumonia Viral/mortalidade , Pontuação de Propensão , /mortalidade , Idoso , Estudos de Coortes , Dexametasona/administração & dosagem , Feminino , Hospitalização/tendências , Humanos , Masculino , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Pandemias , Estudos Retrospectivos , Taxa de Sobrevida/tendências
10.
J Med Case Rep ; 14(1): 210, 2020 Nov 03.
Artigo em Inglês | MEDLINE | ID: mdl-33138853

RESUMO

BACKGROUND: Since the World Health Organization declared a global pandemic due to the novel coronavirus disease2019, there have been targeted efforts to establish management modalities. Hydroxychloroquine has been suggested as a possible treatment; however, it is associated with multiple adverse reactions. We report a rare case of a patient with acute generalized exanthematous pustulosis with Stevens-Johnson syndrome due to hydroxychloroquine. Acute generalized exanthematous pustulosis is characterized by acute onset of a generalized rash that is pustular and erosive in nature, affecting limbs; trunk; face; and, less often, mucosal membranes. Although rare, it is important to be mindful of this side effect because the diagnosis is often delayed, and the disease has the potential to be life-threatening. CASE PRESENTATION: A 68-year-old American woman presented to our hospital with a painful, rapidly spreading rash. Its morphologic features included erythema multiforme-like lesions with extensive skin sloughing in various regions of the head, neck, and trunk and mucosal involvement. Her Nikolsky sign was negative, and she had no evidence of lesions on areas of skin trauma. Four weeks prior, she had been initiated on hydroxychloroquine for a presumed diagnosis of cutaneous sarcoidosis. Three punch biopsies of the head and neck area revealed subcorneal pustules consistent with acute generalized exanthematous pustulosis. Treatment began with high doses of methylprednisolone, leading to only minimal improvement of existing areas and ongoing spread to new areas. Treatment with intravenous immunoglobulin was initiated, at which point disease stability was achieved. The patient's rash ultimately resolved, as did her cutaneous pain and pruritus. CONCLUSIONS: Among many potential adverse reactions involving hydroxychloroquine, cutaneous side effects are varied and can lead to significant morbidity or even death. The drug is currently being investigated in a multitude of trials for coronavirus disease2019 treatment, prevention, and prophylaxis after exposure to severe acute respiratory syndrome coronavirus 2. Acute generalized exanthematous pustulosis is a rare side effect of hydroxychloroquine, and even fewer cases demonstrate histologic evidence of acute generalized exanthematous pustulosis while clinically presenting with Stevens-Johnson syndrome. Patients who develop Stevens-Johnson syndrome/toxic epidermal necrolysis require best supportive care with aggressive fluid and electrolyte replacement and prevention of further breakdown of the skin barrier. With the potential of widespread hydroxychloroquine use, it is important that providers be aware of its potential severe adverse drug reactions.


Assuntos
Pustulose Exantematosa Aguda Generalizada , Infecções por Coronavirus/epidemiologia , Hidroxicloroquina , Imunoglobulinas Intravenosas/administração & dosagem , Metilprednisolona/administração & dosagem , Pneumonia Viral/epidemiologia , Sarcoidose/tratamento farmacológico , Síndrome de Stevens-Johnson , Pustulose Exantematosa Aguda Generalizada/diagnóstico , Pustulose Exantematosa Aguda Generalizada/etiologia , Pustulose Exantematosa Aguda Generalizada/fisiopatologia , Pustulose Exantematosa Aguda Generalizada/terapia , Idoso , Antimaláricos/administração & dosagem , Antimaláricos/efeitos adversos , Biópsia/métodos , Feminino , Humanos , Hidroxicloroquina/administração & dosagem , Hidroxicloroquina/efeitos adversos , Fatores Imunológicos , Pandemias , Pele/patologia , Dermatopatias/tratamento farmacológico , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/etiologia , Síndrome de Stevens-Johnson/fisiopatologia , Síndrome de Stevens-Johnson/terapia , Resultado do Tratamento
13.
Medicine (Baltimore) ; 99(43): e22926, 2020 Oct 23.
Artigo em Inglês | MEDLINE | ID: mdl-33120848

RESUMO

RATIONALE: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies directed against the activity of factor VIII (FVIII) and presents with prolonged bleeding. 5.7% of systemic lupus erythematosus (SLE) patients are affected by AHA. PATIENT CONCERNS: A 51-year-old female patient with SLE presenting with the fatigue and spontaneous clinical bleeding symptoms such as hematuria and ecchymoses for 1 week. DIAGNOSIS: Laboratory examinations revealed prolongation of the activated partial thromboplastin time (APTT) (65.7 s), decreased FVIII activity (1.4%), and a titer of FVIII inhibitors of 8.5 Bethesda units/mL. INTERVENTIONS: Transfusion of recombinant human FVIII (ADVATE) in combination with intravenous methylprednisolone, cyclophosphamide, plasmapheresis, and fresh frozen plasma successfully stopped the bleeding and reduced the level of FVIII inhibitor. OUTCOMES: The size of the hematoma slowly decreased. The skin ecchymosis was gradually absorbed, the hemoglobin count increased, and the coagulation index gradually improved. There was no new bleeding or bleeding site. The patient was discharged and transferred to a local hospital for hospice care. LESSONS: AHA in a patient with SLE is rare. Once it occurs, it can be life-threatening. Clinicians should remain aware that because some cases of AHA may have features of SLE, appropriate distinction and diagnosis of these different but associated diseases is necessary.


Assuntos
Coagulantes/uso terapêutico , Fator VIII/uso terapêutico , Hemofilia A/etiologia , Lúpus Eritematoso Sistêmico/complicações , Administração Intravenosa , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/uso terapêutico , Testes de Coagulação Sanguínea/métodos , Coagulantes/administração & dosagem , Terapia Combinada , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Equimose/diagnóstico , Equimose/etiologia , Fator VIII/administração & dosagem , Feminino , Hematúria/diagnóstico , Hematúria/etiologia , Hemofilia A/terapia , Hemorragia/diagnóstico , Hemorragia/etiologia , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Tempo de Tromboplastina Parcial/métodos , Tempo de Tromboplastina Parcial/estatística & dados numéricos , Plasma , Plasmaferese/métodos , Resultado do Tratamento
14.
Medicine (Baltimore) ; 99(40): e22616, 2020 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-33019482

RESUMO

RATIONALE: Neuromyelitis optica spectrum disorders (NMOSDs) are inflammatory demyelinating disorders of the central nervous system; they are characterized by severe optic neuritis and transverse myelitis. Intravenous methylprednisolone pulse (IVMP) therapy is an effective treatment that is administered to patients in the acute phase of NMOSD; this therapy has achieved remarkable results in clinical practice. However, there are no reports on NMOSD patients who have experienced an acute bilateral cerebral infarction while undergoing IVMP treatment. PATIENT CONCERNS: We report on a 62-yr-old woman who was undergoing IVMP therapy for the primary diagnosis of NMOSD. Unexpectedly, the patient's existing limb weakness worsened, and she developed motor aphasia on the second day of IVMP treatment. Additionally, brain magnetic resonance imaging revealed acute bilateral cerebral infarction. DIAGNOSIS: The patient's clinical manifestations, medical imaging results, and laboratory test results were taken into consideration; the final diagnosis was acute bilateral cerebral infarction in the presence of NMOSD. INTERVENTIONS: Subsequent to the onset of acute cerebral infarction, the patient was immediately treated with oral aspirin, atorvastatin, and intravenous butylphthalide. The hormone dose was adjusted to an oral 60-mg/d dose for maintenance; this was followed by immunoadsorption plasmapheresis for 3 days, and double-filtration plasmapheresis for 2 days. OUTCOMES: Following treatment onset, the patient's ocular symptoms significantly improved, and her limb muscle strength gradually recovered. Two months after discharge, the patient's husband reported that she was able to walk with the help of others and take care of herself, and that there was no recurrence. LESSONS: Medical professionals must be aware of the possibility of NMOSD patients with cerebrovascular risk factors suffering an acute cerebral infarction while undergoing high-dose IVMP therapy, as this therapy can exacerbate existing problems.


Assuntos
Infarto Cerebral/diagnóstico por imagem , Infarto Cerebral/etiologia , Neuromielite Óptica/complicações , Neuromielite Óptica/tratamento farmacológico , Doença Aguda , Administração Intravenosa , Administração Oral , Anticolesterolemiantes/uso terapêutico , Afasia de Broca/induzido quimicamente , Aspirina/administração & dosagem , Aspirina/uso terapêutico , Atorvastatina/uso terapêutico , Benzofuranos/administração & dosagem , Benzofuranos/uso terapêutico , Infarto Cerebral/tratamento farmacológico , Feminino , Humanos , Imagem por Ressonância Magnética , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Fármacos Neuroprotetores/administração & dosagem , Fármacos Neuroprotetores/uso terapêutico , Plasmaferese/métodos , Inibidores da Agregação de Plaquetas/administração & dosagem , Inibidores da Agregação de Plaquetas/uso terapêutico , Resultado do Tratamento
15.
Biomed Res Int ; 2020: 2854186, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33015160

RESUMO

Objectives: To evaluate the role of short-term low-dose glucocorticoids in mild COVID-19 patients. Methods: We conducted a retrospective, cross-sectional, single-center study in Kunming, China. A total of 33 mild COVID-19 cases were divided into two treatment groups (with and without glucocorticoids, methylprednisolone, were used in this setting), and the absolute value of peripheral blood lymphocyte count; CD3+, CD4+, and CD8+ T cell counts; and the time to achieve negative transformation of a nucleic acid pharyngeal swab were recorded. Peripheral blood lymphocyte and T cell counts were compared between the treatment group and 25 healthy individuals. At the point of time when there was a 50% accumulation conversion rate (positive to negative nucleic acid on pharyngeal swab), and the nucleic acid turned negative in half of the patients in two groups, the peripheral blood lymphocyte and T cell counts were compared between treatment groups. Results: The mean cumulative time for the 50% negative conversion rate of the nucleic acid in the pharyngeal swab was 17.7 ± 5.1 days and 13.9 ± 5.4 days in the glucocorticoid group and the nonglucocorticoid group, respectively. The absolute peripheral blood lymphocyte count and the T cell subset count in the glucocorticoid group were lower than those in the nonglucocorticoid group. When the nucleic acid turned negative in half of the patients, the absolute value of peripheral blood lymphocyte count and CD4+ T cells of the glucocorticoid group and the nonglucocorticoid group was not significantly different; the CD3+ and CD8+ T cells in the glucocorticoid group were lower than those in the nonglucocorticoid group. The absolute peripheral blood lymphocyte count, CD3+ T cells, and CD4+ T cells in the glucocorticoid group were lower than those of the healthy group during the whole disease period, and CD8+ T cells returned to normal at 19-21 days of the disease period. There was no significant difference between the nonglucocorticoid group and the healthy group for absolute peripheral blood lymphocyte and CD8+ T cells; moreover, CD3+ T cells and CD4+ T cells were lower in the nonglucocorticoid group than those in the healthy group from the day of admission to the 18th day and returned to normal at the period of 19-21 days. The absolute peripheral lymphocyte count (P = 0.048, effect size d = 0.727) and T cell subset count (CD3: P = 0.042, effect size d = 0.655; CD4: P < 0.01, effect size d = 0.599; and CD8: P = 0.034, effect size d = 0.550) in the nonglucocorticoid group were higher than those in the glucocorticoid group, and the difference between the groups was statistically significant. Conclusions: This study found that the use of short-term, low-dose glucocorticoids does not negatively influence the clinical outcome, without affecting the final clearance of viral nucleic acid in mild COVID-19 patients.


Assuntos
Betacoronavirus , Infecções por Coronavirus/tratamento farmacológico , Glucocorticoides/administração & dosagem , Pneumonia Viral/tratamento farmacológico , Adolescente , Adulto , Betacoronavirus/isolamento & purificação , Criança , China/epidemiologia , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/virologia , Estudos Transversais , Feminino , Humanos , Contagem de Linfócitos , Masculino , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/imunologia , Pneumonia Viral/virologia , Estudos Retrospectivos , Subpopulações de Linfócitos T/imunologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
16.
Rev. neurol. (Ed. impr.) ; 71(7): 257-269, 1 oct., 2020.
Artigo em Espanhol | IBECS | ID: ibc-195711

RESUMO

INTRODUCCIÓN: La enfermedad asociada a anticuerpos contra la glucoproteína del oligodendrocito asociado a la mielina (MOG) es una entidad infrecuente y prácticamente nueva en la medicina. En países en desarrollo, aún hay importantes limitaciones para la detección de los anticuerpos anti-MOG mediante ensayo basado en células, por lo que conocer las características clínicas de los diferentes fenotipos y sus diferencias con otras patologías desmielinizantes del sistema nervioso es fundamental, y con ello realizar un abordaje diagnóstico y terapéutico adecuado de los pacientes. OBJETIVO: Presentar una actualización en cuanto a las características clínicas del espectro de la enfermedad. Éste es el primer artículo en castellano que reúne los fenotipos más frecuentes y brinda una descripción clara de lo que se debe tener en cuenta en cada uno de ellos. DESARROLLO: Esta entidad se caracteriza por tener un curso monofásico o recurrente. La neuritis óptica es el fenotipo de presentación más frecuente en la población general, y la encefalomielitis aguda diseminada, la más frecuente en los niños. Otros fenotipos que se describen en la presente revisión son la mielitis transversa, la encefalitis cortical y los síndromes de tallo cerebral, así como los criterios propuestos para el diagnóstico de la enfermedad asociada a anticuerpos anti-MOG. CONCLUSIONES: En la actualidad no existen estudios que busquen caracterizar a la población hispanoparlante con esta enfermedad ni artículos de revisión en lengua castellana, por lo que es importante difundir conocimiento y desarrollar investigación en esta área


INTRODUCTION: Myelin oligodendrocyte glycoprotein (MOG) antibody disease is a rare and practically new entity in medicine. In developing countries, there are still important limitations for the detection of anti-MOG antibodies by cell-based assay, so knowing the clinical characteristics of the different phenotypes and their differences with other demyelinating pathologies of the central nervous system is essential in order to make a proper diagnostic and therapeutic approach of the patients. AIM: To present an update regarding the clinical characteristics of the disease spectrum, being the first article in Spanish that gathers the most frequent phenotypes and provides a clear description of what should be considered to identify each of these phenotypes. DEVELOPMENT: This disease is characterized by having a monophasic or recurrent course, with optic neuritis being the most frequent presentation phenotype in general population and disseminated acute encephalomyelitis the most frequent in children. Other phenotypes described in this review are transverse myelitis, focal cortical encephalitis and cerebral stem syndromes, as well as the proposed criteria for the diagnosis of the disease associated with MOG antibody disease. CONCLUSION: Currently there are no studies that seek to characterize the Spanish-speaking population with this disease, or review articles in Spanish, so it is important to disseminate knowledge and develop research in this area


Assuntos
Humanos , Glicoproteína Oligodendrócito-Mielina/análise , Encefalite/epidemiologia , Neurite Óptica/epidemiologia , Mielite Transversa/diagnóstico , Encefalite/diagnóstico , Glicoproteína Oligodendrócito-Mielina/imunologia , Mielite Transversa/imunologia , Encefalomielite Aguda Disseminada/tratamento farmacológico , Metilprednisolona/administração & dosagem
17.
Rinsho Shinkeigaku ; 60(10): 682-687, 2020 Oct 24.
Artigo em Japonês | MEDLINE | ID: mdl-32893240

RESUMO

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a brainstem predominant lymphocytic inflammatory disease, which often relapses without oral immunosuppressants. This report describes a 37-year-old male case of CLIPPERS without relapse for 1 year after early steroid treatment. He was admitted to our hospital because of sensory disturbance in the left side of his body and ataxic gait. Gadolinium-enhanced T1-weighted MRI revealed multiple punctate and curvilinear enhancements in the pons and right middle cerebellar peduncle. We started treatment with high-dose intravenous methylprednisolone (IVMP) therapy on the 20th day of the illness. His neurological symptoms dramatically improved. Follow-up MRI showed that the enhancing lesions disappeared. We diagnosed him with CLIPPERS based on the clinical course, radiological findings, and steroid response. He did not take any oral immunosuppressant after discharge. However, there was no clinical and radiological relapse for 1 year after the IVMP therapy. Although this case requires careful follow-up because of recurrence risk, early steroid treatment was possibly related to 1-year remission.


Assuntos
Encefalite/tratamento farmacológico , Doenças Linfáticas/tratamento farmacológico , Metilprednisolona/administração & dosagem , Adulto , Tronco Encefálico , Doença Crônica , Imagem de Difusão por Ressonância Magnética , Encefalite/diagnóstico por imagem , Seguimentos , Humanos , Inflamação , Infusões Intravenosas , Doenças Linfáticas/diagnóstico por imagem , Masculino , Pulsoterapia , Resultado do Tratamento
18.
PLoS One ; 15(9): e0239401, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32960899

RESUMO

OBJECTIVE: To analyze the effects of a short course of methyl-prednisolone pulses (MP) during the second week of disease (week-2) in patients with severe coronavirus disease 2019 (COVID-19) pneumonia. METHODS: Comparative observational study using data collected from routine care at Hospital Universitario Cruces, Barakaldo, Bizkaia, Spain in patients with COVID-19 pneumonia. We compared patients who received week-2-MP (125-250 mg/d x3) with those who did not, with the end-points time to death and time to death or endotracheal intubation. RESULTS: We included 242 patients with COVID-19 pneumonia and elevated inflammatory markers at admission. Sixty-one patients (25%) received week-2-MP. Twenty-two patients (9%) died and 31 (12.8%) suffered death or intubation. The adjusted HRs for death and death or intubation for patients in the week-2-MP group were 0.35 (95%CI 0.11 to 1.06, p = 0.064) and 0.33 (95%CI 0.13 to 0.84, p = 0.020), respectively. These differences were specifically seen in the subcohort of patients with a SpO2/FiO2 at day 7 lower than 353 (adjusted HR 0.31, 95% CI 0.08 to 1.12, p = 0.073 and HR 0.34, 95%CI 0.12 to 0.94, p = 0.038, respectively) but not in patients with higher SpO2/FiO2. Patients receiving out-of-week-2-MP, non-pulse glucocorticoids or no glucocorticoids had an increased adjusted risk for both outcomes compared with week-2-MP group: HR 5.04 (95% CI 0.91-27.86), HR 10.09 (95% CI 2.14-47.50), HR 4.14 (95% CI 0.81-21.23), respectively, for death; HR 7.38 (95% CI 1.86-29.29), HR 13.71 (95% CI 3.76-50.07), HR 3.58 (95% CI 0.89-14.32), respectively, for death or intubation. These differences were significant only in the subgroup with low SpO2/FiO2. CONCLUSIONS: Week-2-MP are effective in improving the prognosis of patients with COVID-19 pneumonia with features of inflammatory activity and respiratory deterioration entering the second week of disease. The recognition of this high-risk population should prompt early use of MP at this point.


Assuntos
Infecções por Coronavirus/diagnóstico , Metilprednisolona/administração & dosagem , Pneumonia Viral/diagnóstico , Idoso , Infecções por Coronavirus/patologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Inflamação , Intubação Intratraqueal , Masculino , Metilprednisolona/farmacologia , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Oxigênio/sangue , Pandemias , Pneumonia Viral/patologia , Prognóstico , Estudos Retrospectivos , Risco , Fatores de Risco , Espanha , Fatores de Tempo
19.
Medicine (Baltimore) ; 99(39): e22299, 2020 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-32991435

RESUMO

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a condition characterized by a hyperinflammatory state and persistent macrophage activation, resulting in reactive phagocytosis of the hematopoietic elements. In children, it is usually a hereditary disorder, while in adults it is usually acquired secondary to viral infections, collagenoses, or tumors. Although accounting for 10% of hematologic malignancies, HLH is rarely associated with multiple myeloma (MM) and other plasmacytic dyscrasias. PATIENT CONCERNS: A 64-year-old Brazilian man seeked medical care with a 3-month history of intermittent fever, weight loss, night sweats, and progressive anemic symptoms. DIAGNOSIS: Total blood count showed severe bicytopenia (normocytic-normochromic anemia and thrombocytopenia), biochemical exams showed elevation of creatinine, as well as monoclonal peak in serum protein electrophoresis, high IgA dosage, and serum immunofixation with IgA kappa paraprotein. Bone marrow biopsy showed 30% of monoclonal and phenotypically anomalous plasmocytes, confirming the diagnosis of MM. Diagnosis of HLH was established by the presence of clinical and laboratory criteria: fever, splenomegaly, cytopenias, hypofibrinogenemia, hyperferritinemia, elevation of triglycerides, and several figures of erythrophagocytosis in bone marrow aspirate. INTERVENTIONS: The patient experienced pulse therapy with methylprednisolone for hemophagocytic lymphohistiocytosis, followed by initial therapy for multiple myeloma with cyclophosphamide and dexamethasone. OUTCOMES: Once the diagnosis of MM and secondary hemophagocytic syndrome was established, the patient had a rapid clinical deterioration despite the established therapeutic measures, evolving with cardiovascular failure, acute liver failure, acute disseminated intravascular coagulation, worsening renal dysfunction requiring dialysis support, respiratory dysfunction, and lowering of consciousness, characterizing rapid multiple organ dysfunction, ultimately leading to the death of the patient. INNOVATION: Here, we aimed to describe the sixth reported case of HLH associated with MM, according to cases cataloged in the PubMed database, and the first case evaluated by 18-fluordeoxyglucose positron emission tomography (18-FDG-PETCT). CONCLUSION: Our case report seeks to provide support for a better clinical and laboratory characterization of this rare paraneoplastic entity associated with MM, and aims to call the attention of hematologists and intensivists to this condition that falls within the scope of the differential diagnosis of rapid onset multiple organ failure in patients with plasmacytic neoplasms.


Assuntos
Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/etiologia , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Anemia/sangue , Anemia/etiologia , Antineoplásicos Alquilantes/administração & dosagem , Antineoplásicos Alquilantes/uso terapêutico , Medula Óssea/patologia , Brasil/epidemiologia , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Dexametasona/administração & dosagem , Dexametasona/uso terapêutico , Quimioterapia Combinada , Evolução Fatal , Febre/diagnóstico , Febre/etiologia , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Linfo-Histiocitose Hemofagocítica/sangue , Linfo-Histiocitose Hemofagocítica/diagnóstico , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Mieloma Múltiplo/metabolismo , Insuficiência de Múltiplos Órgãos/complicações , Paraproteinemias/sangue , Plasmócitos/patologia , Esplenomegalia/diagnóstico , Esplenomegalia/etiologia , Trombocitopenia/sangue , Trombocitopenia/etiologia , Perda de Peso
20.
Medicine (Baltimore) ; 99(39): e22341, 2020 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-32991448

RESUMO

RATIONALE: The Goodpasture syndrome is an extremely rare disease, with renal and pulmonary manifestations, and is mediated by anti-glomerular basement membrane (anti-GBM) antibodies. Renal pathological changes are mainly characterized by glomerular crescent formation and linear immunofluorescent staining for immunoglobulin G on the GBM. There are few reports on the atypical course of the syndrome involving serum-negative anti-GBM antibodies. Therefore, we present a case of Goodpasture syndrome that presented with nephrotic-range proteinuria and was seronegative for anti-GBM antibodies. PATIENT CONCERNS: A 38-year-old Chinese man presented with a lung lesion that was discovered by physical examination a month prior to presentation. The chief concern was occasional hemoptysis without fever, cough, chest pain, and edema. DIAGNOSES: Laboratory testing revealed that the urinary protein level and urine erythrocyte count were 7.4 g/24 hours and 144/high-power field (HPF), respectively. Serological testing for anti-GBM antibodies was negative. Chest computed tomography revealed multiple exudative lesions in both lungs, indicating alveolar infiltration and hemorrhage. Electronic bronchoscopy and pathological examination of the alveolar lavage fluid indicated no abnormalities. However, kidney biopsy suggested cellular crescent formation and segmental necrosis of the globuli, with linear IgG and complement C3 deposition on the GBM. These findings were consistent with the diagnosis of anti-GBM antibody nephritis. INTERVENTIONS: The patient underwent 7 sessions of double filtration plasmapheresis. He was also administered with intravenous methylprednisolone and cyclophosphamide. After renal function stabilization, he was discharged under an immunosuppressive regimen comprising of glucocorticoids and cyclophosphamides. OUTCOMES: Three months later, follow-up examination revealed that the 24-hour urine protein had increased to 13 g. Furthermore, the urine erythrocyte count was 243/HPF. After a 6-month follow-up, the patient achieved partial remission, with a proteinuria level of 3.9 g/24 hours and a urine erythrocyte count of 187/HPF. LESSONS: This extremely rare case of Goodpasture syndrome manifested with seronegativity for anti-GBM antibodies and nephrotic-range proteinuria. Our findings emphasize the importance of renal biopsy for the clinical diagnosis of atypical cases. Furthermore, because renal involvement achieved only partial remission despite therapy, early detection and active treatment of the Goodpasture syndrome is necessary to improve the prognosis of patients.


Assuntos
Doença Antimembrana Basal Glomerular/complicações , Doença Antimembrana Basal Glomerular/imunologia , Autoanticorpos/sangue , Proteinúria/etiologia , Administração Intravenosa , Adulto , Assistência ao Convalescente , Doença Antimembrana Basal Glomerular/diagnóstico , Doença Antimembrana Basal Glomerular/terapia , Grupo com Ancestrais do Continente Asiático/etnologia , Complemento C3/metabolismo , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Quimioterapia Combinada , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Hemoptise/diagnóstico , Hemorragia/etiologia , Hemorragia/patologia , Humanos , Imunoglobulina G/metabolismo , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Rim/patologia , Glomérulos Renais/metabolismo , Glomérulos Renais/patologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Nefrite/diagnóstico , Nefrite/imunologia , Plasmaferese/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
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