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1.
Medicine (Baltimore) ; 103(36): e38659, 2024 Sep 06.
Artículo en Inglés | MEDLINE | ID: mdl-39252328

RESUMEN

RATIONALE: Anti-CD19 chimeric antigen receptor T-cell (CAR-T) therapy is a successful treatment for B-cell malignancies associated with cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS). Cardiovascular toxicities have also been reported in this setting. However, there is scarce data regarding development of autonomic disorders after CAR-T cell therapy. PATIENT CONCERNS: We report a case with a patient with non-Hodgkin B-cell lymphoma, refractory to 2 prior lines of immunochemotherapy, treated with CAR-T therapy. DIAGNOSES: Orthostatic hypotension secondary to autonomic dysfunction was diagnosed as manifestation of ICANS. INTERVENTIONS: The patient received metilprednisolone 1000 mg IV daily for 3 days and anakinra 100 mg IV every 6h. OUTCOMES: The vast majority of autonomic symptoms ceased and 4 months after CAR-T therapy, autonomic dysfunction was resolved. LESSONS: New-onset autonomic dysfunction can occur as manifestation of ICANS in patients who experience persistent neurologic and cardiovascular symptoms after resolution of acute neurotoxicity and should be early recognized. Differences in differential diagnosis, mechanisms and treatment approaches are discussed.


Asunto(s)
Enfermedades del Sistema Nervioso Autónomo , Humanos , Enfermedades del Sistema Nervioso Autónomo/etiología , Enfermedades del Sistema Nervioso Autónomo/diagnóstico , Inmunoterapia Adoptiva/efectos adversos , Masculino , Síndrome de Liberación de Citoquinas/etiología , Persona de Mediana Edad , Linfoma de Células B/complicaciones , Linfoma de Células B/tratamiento farmacológico , Síndromes de Neurotoxicidad/etiología , Síndromes de Neurotoxicidad/diagnóstico , Hipotensión Ortostática/etiología , Hipotensión Ortostática/diagnóstico , Metilprednisolona/uso terapéutico
2.
Sci Rep ; 14(1): 20991, 2024 09 09.
Artículo en Inglés | MEDLINE | ID: mdl-39251824

RESUMEN

Although COVID-19 infection is an immunosuppressant disease, many immunosuppressant agents, such as pulse methylprednisolone (PMP), dexamethasone (DXM), and tocilizumab (TCZ), were used during the pandemic. Secondary infections in patients with COVID-19 have been reported recently. This study investigated these agents' effects on secondary infections and outcomes in patients with COVID-19 in intensive care units (ICUs). This study was designed retrospectively, and all data were collected from the tertiary intensive care units of six hospitals between March 2020 and October 2021. All patients were divided into three groups: Group I [GI, PMP (-), DXM (-) and TCZ (-)], Group II [GII, PMP (+), DXM (+)], and Group III [GIII, PMP (+), DXM (+), TCZ (+)]. Demographic data, PaO/FiO2 ratio, laboratory parameters, culture results, and outcomes were recorded. To compare GI-GII and GI-GIII, propensity score matching (PSM) was used by matching 14 parameters. Four hundred twelve patients with COVID-19 in the ICU were included in the study. The number of patients with microorganisms ≥ 2 was 279 (67.7%). After PSM, in GII and GIII, the number of (+) tracheal cultures and (+) bloodstream cultures detected different microorganisms ≥ 2 during the ICU period, neuropathy, tracheotomized patients, duration of IMV, and length of ICU stay were significantly higher than GI. The mortality rate was similar in GI and GII, whereas it was significantly higher in GIII than in GI. The use of immunosuppressant agents in COVID-19 patients may lead to an increase in secondary infections. In addition, increased secondary infections may lead to prolonged ICU stay, prolonged IMV duration, and increased mortality.


Asunto(s)
COVID-19 , Inmunosupresores , Unidades de Cuidados Intensivos , Humanos , Masculino , Femenino , Estudios Retrospectivos , COVID-19/mortalidad , COVID-19/complicaciones , COVID-19/epidemiología , Persona de Mediana Edad , Inmunosupresores/uso terapéutico , Inmunosupresores/efectos adversos , Anciano , Dexametasona/uso terapéutico , Tratamiento Farmacológico de COVID-19 , Metilprednisolona/uso terapéutico , SARS-CoV-2/aislamiento & purificación , Anticuerpos Monoclonales Humanizados/uso terapéutico , Adulto
3.
BMJ Case Rep ; 17(9)2024 Sep 03.
Artículo en Inglés | MEDLINE | ID: mdl-39231562

RESUMEN

We present a case of a woman in her 20s with inadequately treated systemic lupus erythematosus (SLE). She presented with heavy menstrual bleeding, along with nasal and gum bleeding worsening over 3 months. There was no bleeding history in her family, childhood, dental procedures or childbirth. Evaluation ruled out structural causes, revealing prolonged activated partial thromboplastin time (incomplete correction on mixing studies), normal prothrombin time, moderate thrombocytopenia, and lupus anticoagulant and anti-phosphatidylserine/prothrombin antibody positivity twice, 12 weeks apart. Further evaluation showed very low von Willebrand factor (vWF) levels (<5%). She was treated with pulse methylprednisolone for 3 days, resulting in complete symptom resolution and improvement in vWF levels to 130%. The absence of bleeding history, family history, presence of very low vWF and its response to corticosteroids led to a diagnosis of acquired vWF syndrome as the cause of mucosal bleeding in an SLE patient with concomitant positive antiphospholipid antibody. She was discharged on hydroxychloroquine, mycophenolate mofetil and tapering oral corticosteroids.


Asunto(s)
Anticuerpos Antifosfolípidos , Lupus Eritematoso Sistémico , Enfermedades de von Willebrand , Humanos , Femenino , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/diagnóstico , Anticuerpos Antifosfolípidos/sangre , Enfermedades de von Willebrand/complicaciones , Enfermedades de von Willebrand/diagnóstico , Enfermedades de von Willebrand/tratamiento farmacológico , Enfermedades de von Willebrand/etiología , Adulto , Menorragia/etiología , Menorragia/tratamiento farmacológico , Metilprednisolona/uso terapéutico
4.
BMJ Open ; 14(8): e076872, 2024 Aug 29.
Artículo en Inglés | MEDLINE | ID: mdl-39209781

RESUMEN

INTRODUCTION: Intratympanic corticosteroids are commonly used in the treatment of Menière's disease (MD). However, few and small randomised controlled trials (RCT) on the effectiveness of intratympanic corticosteroids have been performed. A recent Cochrane review suggested that a well-conducted placebo-controlled RCT with a large study population is required to evaluate the effectiveness of the use of intratympanic corticosteroids in MD. The following protocol describes a phase-3 multicentre, double-blinded, randomised, placebo-controlled trial to compare the effectiveness of methylprednisolone (62.5 mg/mL) to a placebo (sodium chloride 0.9%). METHODS AND ANALYSIS: We aim to recruit 148 patients with unilateral MD from six hospitals in the Netherlands. Patients will be randomly assigned to either the methylprednisolone or the placebo group. Two injections will be given, one at baseline and one after 2 weeks. Follow-up assessments will be done at 3, 6, 9 and 12 months. The primary outcome will be the frequency of vertigo attacks. Attacks will be evaluated daily with the DizzyQuest app. Secondary outcomes include hearing loss, tinnitus, health-related quality of life, use of co-interventions and escape medication, (serious) adverse events and cost-effectiveness. These will be evaluated with audiometry and multiple commonly used, validated questionnaires. For the primary and secondary outcomes mixed model analysis, generalised estimating equation analysis and logistic regression analysis will be used. ETHICS AND DISSEMINATION: This study was submitted via the Clinical Trials Information System, reviewed and approved by the Medical Research Ethics Committee Leiden The Hague Delft and the local institutional review board of each participating centre. All data will be presented ensuring the integrity and anonymity of patients. Results will be published in scientific journals and presented on (inter)national conferences. TRIAL REGISTRATION NUMBER: This study is registered at ClinicalTrials.gov Protocol Registration and Results System, with the registration ID: NCT05851508.


Asunto(s)
Inyección Intratimpánica , Enfermedad de Meniere , Metilprednisolona , Vértigo , Humanos , Ensayos Clínicos Fase III como Asunto , Método Doble Ciego , Glucocorticoides/administración & dosificación , Glucocorticoides/uso terapéutico , Enfermedad de Meniere/tratamiento farmacológico , Metilprednisolona/administración & dosificación , Metilprednisolona/uso terapéutico , Estudios Multicéntricos como Asunto , Países Bajos , Calidad de Vida , Ensayos Clínicos Controlados Aleatorios como Asunto , Resultado del Tratamiento , Vértigo/tratamiento farmacológico
5.
Medicine (Baltimore) ; 103(32): e39278, 2024 Aug 09.
Artículo en Inglés | MEDLINE | ID: mdl-39121314

RESUMEN

RATIONALE: Myelin oligodendrocyte glycoprotein (MOG) antibody-related disease is a relatively recent entity in inflammatory demyelinating disease. Its clinical presentation varies in severity and the lack of specific imaging features makes it easy to misdiagnose. We now report the case of a MOG antibody-positive patient who presented with diplopia and dizziness, and whose brain magnetic resonance imaging (MRI) showed abnormal signals in the bilateral pontine brachium. PATIENT CONCERNS: A previously healthy 52-year-old woman presented with diplopia and dizziness, and was hospitalized 4 days after onset. DIAGNOSES: Brain MRI demonstrated abnormal hyperintense signals in the bilateral pontine brachium on T2-weighted fluid attenuated inversion recovery imaging. MRI enhancement showed abnormal enhancement foci in bilateral pontine brachium and pons. Cerebrospinal fluid examination showed Oligoclonal IgG bands were negative. The IgG index was normal, and serum aquaporin-4 antibody was negative, while serum MOG-Ab was positive (1:100). In conjunction with a positive serum MOG antibody and exclusion of other diseases, diagnosis of MOG antibody-related disease was made. INTERVENTIONS: Intravenous methylprednisolone followed by oral corticosteroids. OUTCOMES: Symptoms resolved completely. At 4-month follow-up. Follow-up after 4 months showed disappearance of the abnormal signal in the left pontine brachium and diminution of abnormal high signal in the right compared to the previous one, and there was no recurrence 1 year after the onset of the disease. LESSONS: If brain MRI indicating bilateral, multiple, and diffuse abnormal signals in the pontine brachium, and a discrepancy between the clinical symptoms and the imaging severity, a diagnosis of demyelinating disease should be considered highly probable. In such cases, anti-MOG antibody testing is essential for further defining the etiology. The clinical phenotype and imaging manifestations of MOG antibody-positive brainstem encephalitis may lack sufficient specificity to be readily identifiable. Timely diagnosis and early glucocorticoid therapy are beneficial in improving prognosis and preventing recurrence.


Asunto(s)
Autoanticuerpos , Imagen por Resonancia Magnética , Glicoproteína Mielina-Oligodendrócito , Puente , Humanos , Femenino , Persona de Mediana Edad , Glicoproteína Mielina-Oligodendrócito/inmunología , Autoanticuerpos/sangre , Puente/diagnóstico por imagen , Puente/patología , Metilprednisolona/uso terapéutico
7.
Spinal Cord Ser Cases ; 10(1): 55, 2024 Aug 03.
Artículo en Inglés | MEDLINE | ID: mdl-39097598

RESUMEN

INTRODUCTION: Spinal cord infarction (SCI) is a rare disease representing nearly 1% of all strokes with a wide variety of symptoms at presentation. SCI diagnosis is very challenging owing to its low incidence and the variety of symptoms, and could be misdiagnosed with neuromyelitis optica spectrum disorders (NMOSD). CASE PRESENTATION: We describe the case of an 18-year-old girl who presented to the emergency department with acute neck pain and flaccid paralysis of the left upper and lower extremities. Few hours later, she developed apnea and was endotracheally intubated. Brain MRI was normal but spinal cord MRI revealed non-enhancing longitudinal abnormal high T2 signal intensity extending from C1 to C5. The patient underwent steroid therapy with methylprednisolone (1 g daily for 7 consecutive days) combined with physiotherapy. She was extubated after 3 weeks and discharged after 30 days of hospitalization with a muscle force of 4/5 in her left extremities. DISCUSSION: Idiopathic SCI in adolescence is a rare but often devastating disorder with unknown pathophysiology, however, some specific conditions in adolescent such as mechanical stresses on the immature spine can be considered as risk factors for SCI development. Early diagnosis and treatment can improve outcomes in SCI.


Asunto(s)
Médula Cervical , Infarto , Dolor de Cuello , Humanos , Femenino , Adolescente , Infarto/diagnóstico , Infarto/complicaciones , Infarto/diagnóstico por imagen , Médula Cervical/diagnóstico por imagen , Dolor de Cuello/etiología , Parálisis/etiología , Parálisis/diagnóstico , Metilprednisolona/uso terapéutico
8.
Medicine (Baltimore) ; 103(33): e39146, 2024 Aug 16.
Artículo en Inglés | MEDLINE | ID: mdl-39151524

RESUMEN

RATIONALE: Anti-Myelin oligodendrocyte glycoprotein (MOG) and anti-metabotropic glutamate receptor 5 (mGluR5) double antibody positive encephalitis characterized by optic neuritis is extremely rare. We present a case of overlapping syndrome of MOG-IgG-associated disease and anti-mGluR5 encephalitis manifested as optic neuritis. PATIENT CONCERNS: A 60-year-old Chinses woman presented to the hospital with progressive vision loss and headache for 1 week. The cerebrospinal fluid examination was within the normal range. Visual evoked potentials study disclosed prolonged latency of P100 bilaterally. Fundus examination revealed indistinct boundaries of both optic discs. Her brain magnetic resonance imaging showed patchy hyperintensity in the posterior horn of the left ventricle and the left optic nerve. Her serum was positive for anti-MOG and anti-mGluR5 antibodies. DIAGNOSIS: The patient was diagnosed with overlapping syndrome of anti-MOG antibody-associated disease and anti-mGluR5 encephalitis mainly based on the clinical symptoms and further test of the antibody in serum. INTERVENTIONS AND OUTCOMES: She was subsequently subjected to empirical treatment with intravenous methylprednisolone. After discharge, she was given a tapering dose of oral prednisone, alongside mycophenolate mofetil. On outpatient follow-up, her symptoms showed no relapse after 1 month, and her condition remained stable. LESSONS: Early recognition of autoimmune encephalitis is crucial. The detection of cerebrospinal fluid and serum of autoimmune encephalitis and demyelinating diseases of the CNS, including MOG-IgG and mGluR5-IgG, should be strengthened in order to make a precise diagnosis and develop a comprehensive treatment plan in a timely manner.


Asunto(s)
Autoanticuerpos , Glicoproteína Mielina-Oligodendrócito , Neuritis Óptica , Receptor del Glutamato Metabotropico 5 , Humanos , Femenino , Neuritis Óptica/diagnóstico , Neuritis Óptica/inmunología , Neuritis Óptica/tratamiento farmacológico , Persona de Mediana Edad , Glicoproteína Mielina-Oligodendrócito/inmunología , Autoanticuerpos/sangre , Encefalitis/diagnóstico , Encefalitis/inmunología , Encefalitis/tratamiento farmacológico , Metilprednisolona/uso terapéutico , Imagen por Resonancia Magnética , Síndrome
9.
Transpl Immunol ; 86: 102107, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39142540

RESUMEN

The reportedly poor outcome of late-onset idiopathic pneumonia syndrome (IPS) necessitates new approaches to its treatment. A 55-year-old man who had undergone allogeneic hematopoietic cell transplantation (allo-HCT) for myelodysplastic syndrome 1 year ago developed dyspnea with acute skin graft-versus-host disease (GVHD) flare-up while tapering immunosuppressive agents. He presented with acute respiratory distress syndrome with ground-glass opacities in the right upper and left lower lobes. All infectious tests, including multiplex polymerase chain reaction of nasal wash, were negative, and broad-spectrum antibiotic therapy was refractory. The patient was diagnosed with late-onset IPS and was refractory to methylprednisolone pulse therapy. He then showed a favorable response to mesenchymal stem cell (MSC) infusion. After eight infusions of MSCs, he had no IPS recurrence for over one year. Recently, preclinical studies have reported the potential therapeutic utility of MSC infusion for treating IPS, and our case supports its potential for treating late-onset IPS.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Trasplante de Células Madre Mesenquimatosas , Trasplante Homólogo , Humanos , Masculino , Persona de Mediana Edad , Síndromes Mielodisplásicos/terapia , Enfermedad Injerto contra Huésped , Células Madre Mesenquimatosas , Metilprednisolona/uso terapéutico , Neumonía/etiología , Neumonía/terapia , Síndrome
10.
J Pak Med Assoc ; 74(8): 1514-1517, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39160724

RESUMEN

A rare subtype of autoimmune encephalitis consists of antibodies targetting the alpha-amino-3-hydroxy-5- methyl-4-isoxazolepropionic acid receptor in the central nervous system. We describe the clinical presentation and autoimmune profile of the first case of alpha-amino-3- hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with concurrent anti-acetylcholine receptor antibodies in Pakistan. The patient was a 58-year-old male who presented with the characteristic symptoms of limbic encephalitis with memory loss, irritability, agitation, and confusion. Antibodies against the alpha-amino-3-hydroxy- 5-methyl-4-isoxazolepropionic acid receptor were detected in both serum and cerebrospinal fluid by indirect immunofluorescence. Computerised tomography of the chest showed an anterior mediastinal mass. The patient was treated with high dose Methylprednisolone and five sessions of plasma exchange. There was a short period of improvement; however, the patient now continues to exhibit irritability, aphasia, confusion, and memory loss. Video-assisted thoracoscopic surgery for mediastinal mass resection and histological testing was planned, however after review by the interventional radiologist the associated risks were deemed too high to proceed with the procedure and biopsy was not done.


Asunto(s)
Miastenia Gravis , Humanos , Masculino , Persona de Mediana Edad , Miastenia Gravis/diagnóstico , Miastenia Gravis/complicaciones , Receptores AMPA/inmunología , Autoanticuerpos/sangre , Encefalitis/inmunología , Encefalitis/diagnóstico , Metilprednisolona/uso terapéutico , Metilprednisolona/administración & dosificación , Encefalitis Límbica/inmunología
11.
BMJ Case Rep ; 17(8)2024 Aug 24.
Artículo en Inglés | MEDLINE | ID: mdl-39181572

RESUMEN

A woman in her 20s presented with 6 weeks of fever, persistent vomiting and 28% loss of body weight. Symptoms were refractory to treatment with antiemetics and broad spectrum antibiotics.Further investigation via oesophageogastroduedenoscopy revealed a large gastric ulcer and pyloric stricture, causing gastric outlet obstruction (GOO). Biopsies of the stomach and duodenum showed plasma cell infiltration with a large proportion being IgG4 positive.Treatment with methylprednisolone, and later prednisolone, quickly improved inflammatory markers and symptoms. Balloon dilatation of the pyloric stricture also improved vomiting, allowing eventual re-establishment of oral nutrition. The patient made a full recovery with maintenance treatment on mycophenolate mofetil.IgG4-related disease (IgG4-RD) is a multisystem disorder with unpredictable presentation. The case highlights diagnostic challenges in IgG4-RD and identifies it as a rare differential in upper gastrointestinal symptoms. To our knowledge this is the first published case of IgG4-RD in the duodenum causing GOO.


Asunto(s)
Obstrucción de la Salida Gástrica , Enfermedad Relacionada con Inmunoglobulina G4 , Humanos , Femenino , Obstrucción de la Salida Gástrica/etiología , Obstrucción de la Salida Gástrica/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Adulto , Diagnóstico Diferencial , Inmunoglobulina G/sangre , Metilprednisolona/uso terapéutico , Metilprednisolona/administración & dosificación , Prednisolona/uso terapéutico , Úlcera Gástrica/complicaciones , Úlcera Gástrica/diagnóstico , Vómitos/etiología , Estenosis Pilórica/diagnóstico , Estenosis Pilórica/complicaciones , Duodeno/patología
12.
Ann Med ; 56(1): 2381086, 2024 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-39165240

RESUMEN

BACKGROUND: Acute respiratory distress syndrome (ARDS), are respiratory diseases with high morbidity and mortality. Clinical trials investigating the efficacy of corticosteroids in the treatment of ARDS often yield contradictory results. We hereby conducted a systematic review and meta-analysis to investigate the efficacy of corticosteroids in ARDS management. MATERIALS AND METHODS: We conducted a search for randomized clinical trials (RCT) and observational studies that utilized corticosteroids for patients with ARDS in Web of Science, PubMed, and Embase. The primary outcome was mortality. Risk of bias was assessed using Cochrane or NOS scales. Statistical effect size was analyzed using the Mantel-Haenszel method. RESULTS: A total of 20 studies, comprising 11 observational studies and 9 RCTs, were eligible for analysis. In RCTs, corticosteroids were associated with a reduction of mortality in ARDS patients (relative risk [RR] = 0.80, 95%CI: 0.71-0.91, p = 0.001). Further subgroup analysis indicated that specific variables, such as low-dose (RR = 0.81; 95%CI: 0.67-0.98; p = 0.034), methylprednisolone (RR = 0.70; 95%CI: 0.49-0.98; p = 0.035), and dexamethasone (RR = 0.82; 95%CI: 0.69-0.98; p = 0.029) were associated with mortality among patients receiving corticosteroids. However, in observational studies, corticosteroids increased the risk of death (RR = 1.16, 95%CI: 1.04-1.29; p = 0.001). Subgroup analysis showed that the use of high-dose corticosteroids was associated with higher patient mortality (RR = 1.20; 95%CI: 1.04-1.38; p = 0.001). CONCLUSIONS: The efficacy of corticosteroids on the mortality of ARDS differed by the type and dosage of corticosteroids used, as well as the etiologies. Current data do not support routine use of corticosteroids in ARDS since protective effects were observed in RCTs but increased mortality was found in observational studies. More well designed and large clinical trials are needed to specify the favorable subgroups for corticosteroid therapy.


Corticosteroid use may reduce the risk of death in patients with acute respiratory distress syndrome (ARDS) according to randomized controlled trials.Observational studies indicate that corticosteroid use may increase the risk of death in non-COVID-19 ARDS patients but not in COVID-19 ARDS patients.Both regular and low-dose corticosteroids show benefits in reducing mortality in RCTs, but observational studies associate these doses with increased mortality.


Asunto(s)
Corticoesteroides , Dexametasona , Estudios Observacionales como Asunto , Ensayos Clínicos Controlados Aleatorios como Asunto , Síndrome de Dificultad Respiratoria , Síndrome de Dificultad Respiratoria/tratamiento farmacológico , Síndrome de Dificultad Respiratoria/mortalidad , Humanos , Corticoesteroides/uso terapéutico , Dexametasona/uso terapéutico , Resultado del Tratamiento , Metilprednisolona/uso terapéutico , Metilprednisolona/administración & dosificación
13.
BMC Ophthalmol ; 24(1): 361, 2024 Aug 21.
Artículo en Inglés | MEDLINE | ID: mdl-39169342

RESUMEN

PURPOSE: The primary objective of this study was to identify predictive factors linked to the normalization of thyroid-stimulating immunoglobulin (TSI) levels in patients diagnosed with active, moderate-to-severe Graves' orbitopathy (GO). The study also tracked the longitudinal changes in TSI levels over a 36-month period following treatment. METHODS: The study population consisted of individuals who were recently diagnosed with active, moderate-to-severe GO and received a 12-week course of intravenous methylprednisolone (IVMP) treatment. A subgroup of patients who did not respond to the initial treatment received an additional 20 Gy of radiation therapy (RTx). TSI levels were monitored at the time of diagnosis, after treatment, and subsequently every 6 months for 36 months. Normalization was defined as a TSI level below 140%. Patients were divdied into two groups with success and failure group depending on whether TSI became normal or not. RESULTS: Out of 83 patients, 36 (43.4%) achieved normalized TSI levels within two years post-IVMP treatment. Lower initial TSI levels (< 425%), absence of additional RTx, and early treatment initiation were associated with a higher likelihood of TSI normalization (P = 0.035, P = 0.028, P < 0.001, respectively). Notably, significant differences in TSI level reduction were observed from 18 months post-treatment between the two groups (P = 0.031). A TSI cutoff value of 413% was identified as predictive for normalization at 24 months (P = 0.002). CONCLUSION: This study is the first to identify key factors that influence normalization of TSI levels in moderate-to-severe Graves' Orbitopathy. It highlights the importance of early treatment decisions, particularly for patients with initial TSI levels above 425%. Despite the treatment, less than half of the patients achieved TSI normalization within 24 months, underscoring the need for additional research to explore the relationship between TSI levels and the clinical manifestations of chronic GO.


Asunto(s)
Glucocorticoides , Oftalmopatía de Graves , Inmunoglobulinas Estimulantes de la Tiroides , Metilprednisolona , Humanos , Oftalmopatía de Graves/tratamiento farmacológico , Oftalmopatía de Graves/diagnóstico , Oftalmopatía de Graves/sangre , Femenino , Masculino , Persona de Mediana Edad , Pronóstico , Adulto , Metilprednisolona/uso terapéutico , Metilprednisolona/administración & dosificación , Glucocorticoides/uso terapéutico , Inmunoglobulinas Estimulantes de la Tiroides/sangre , Estudios Longitudinales , Estudios de Seguimiento , Anciano , Índice de Severidad de la Enfermedad , Estudios Retrospectivos
14.
J Clin Apher ; 39(4): e22142, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39092798

RESUMEN

BACKGROUND: Acute cerebellitis is a rare complication of pediatric infections. There are many reports that viral infections lead to neurological manifestations, including acute cerebellitis. METHODS: A retrospective chart review was conducted for pediatric patients diagnosed with enterovirus cerebellitis between 2000 and 2024. The methods involved reviewing clinical and radiological records and assessing the treatment methods. RESULTS: Case Report We present the case of a 4-year-old immunocompetent child who initially presented with acute encephalopathy followed by truncal ataxia, and eventually received a diagnosis of postinfectious cerebellitis. Enterovirus real-time polymerase chain reaction were positive in the nasopharyngeal swab. Therapeutic plasma exchange (TPE) was started due to neurological deterioration despite IVIG treatment. She improved significantly with TPE, and methylprednisolone treatment and was discharged in good health status. The patient is being followed up as neurologically normal. CONCLUSION: Acute cerebellitis associated with enterovirus is a rare pediatric disorder. Early diagnosis and treatment with TPE in this severe case is thought to be preventive for the potentially fatal complications.


Asunto(s)
Infecciones por Enterovirus , Intercambio Plasmático , Humanos , Intercambio Plasmático/métodos , Preescolar , Infecciones por Enterovirus/complicaciones , Infecciones por Enterovirus/terapia , Femenino , Enfermedades Cerebelosas/terapia , Enfermedades Cerebelosas/etiología , Metilprednisolona/uso terapéutico , Enfermedad Aguda , Enterovirus/aislamiento & purificación
15.
Medicine (Baltimore) ; 103(31): e39195, 2024 Aug 02.
Artículo en Inglés | MEDLINE | ID: mdl-39093772

RESUMEN

RATIONALE: The interstitial pneumonia (IP) linked to vedolizumab (VDZ) in patients with ulcerative colitis (UC) is rare. Prompt diagnosis and treatment can improve patient outcomes. PATIENT CONCERNS: A 39-year-old man with UC who received VDZ as sole therapy developed symptoms such as chest tightness, cough, and suffocation. DIAGNOSES: IP was confirmed through pulmonary function tests, chest computed tomography, and bronchoscopic biopsy. INTERVENTIONS: The patient was given methylprednisolone and VDZ cessation. OUTCOMES: The patient's symptoms improved and remained symptom-free after nearly 2 years. LESSONS: VDZ-induced IP should be considered when evaluating pulmonary infections in UC patients treated with VDZ.


Asunto(s)
Anticuerpos Monoclonales Humanizados , Colitis Ulcerosa , Fármacos Gastrointestinales , Enfermedades Pulmonares Intersticiales , Humanos , Colitis Ulcerosa/tratamiento farmacológico , Masculino , Adulto , Anticuerpos Monoclonales Humanizados/efectos adversos , Anticuerpos Monoclonales Humanizados/uso terapéutico , Enfermedades Pulmonares Intersticiales/inducido químicamente , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Fármacos Gastrointestinales/efectos adversos , Fármacos Gastrointestinales/uso terapéutico , Metilprednisolona/uso terapéutico
16.
Turk J Haematol ; 41(3): 146-159, 2024 08 28.
Artículo en Inglés | MEDLINE | ID: mdl-38994780

RESUMEN

Objective: The prognostic factors and outcomes of Turkish children with newly diagnosed acute lymphoblastic leukemia (ALL), treated with the Modified St. Jude Total XV Protocol, which was adjusted by adding high-dose methylprednisolone (HDMP) before induction in the original protocol, were assessed in this study. Materials and Methods: The Modified St. Jude Total XV Protocol was administered to 183 newly diagnosed ALL patients, aged 1-18 years, between 1 January 2008 and 30 January 2016. HDMP was applied at doses of either 10 mg/kg/day (Group A) or 20 mg/kg/day (Group B) for 7 days before induction and then tapered over the next 7 days to 5 or 10 mg/kg/day, and continued at 2 mg/kg/day for 2 weeks during the induction phase. Absolute blast count (ABC) in peripheral blood and minimal residual disease (MRD) in bone marrow were assessed at the end of the initial 7-day HDMP treatment. MRD in the bone marrow was evaluated on day 15 and at the end of the induction period. The follow-up for these patients ended on 15 July 2019. Results: The 5-year event-free (EFS) and overall survival (OS) rates for all patients were 85.6±2.6% and 89.2±2.3%, respectively. The rate of good response to steroids (defined as ABC in peripheral blood of less than 1000/mm3 on day 7) was 88% and 97% of children achieved complete remission after induction. The survival rate and infection frequency did not show statistically significant differences between Group A and B. EFS and OS correlated with initial leukocyte count, age of 10-18 years at diagnosis, CD20 positivity at diagnosis, and gram-negative bacterial infection during remission induction. Conclusion: The remarkable response rates on days 7 and 15, along with the promising EFS and OS results in childhood ALL patients treated with the Modified St. Jude Total XV Protocol, highlight the early and substantial response effect of HDMP. At the onset of induction, short-term HDMP can be initiated, preferably at 10 mg/kg/day for the first 7 days, to minimize potential side effects.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica , Metilprednisolona , Leucemia-Linfoma Linfoblástico de Células Precursoras , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidad , Niño , Femenino , Masculino , Preescolar , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Estudios Retrospectivos , Lactante , Turquía/epidemiología , Metilprednisolona/administración & dosificación , Metilprednisolona/uso terapéutico , Resultado del Tratamiento , Neoplasia Residual/diagnóstico , Pronóstico , Inducción de Remisión
17.
J Autoimmun ; 148: 103298, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39067314

RESUMEN

Multiple sclerosis (MS) is an autoimmune neurodegenerative disease of unknown etiology characterized by infiltration of encephalitogenic cells in the central nervous system (CNS) resulting in the presence of multifocal areas of demyelination leading to neurodegeneration. The infiltrated immune cells population is composed mainly of effector CD4+ and CD8+ T lymphocytes, B cells, macrophages, and dendritic cells that secrete pro-inflammatory factors that eventually damage myelin leading to axonal damage. The most common clinical form of MS is relapsing-remitting (RR), characterized by neuroinflammatory episodes followed by partial or total recovery of neurological deficits. The first-line treatment for RRMS relapses is a high dose of glucocorticoids, especially methylprednisolone, for three to five consecutive days. Several studies have reported the beneficial effects of melatonin in the context of neuroinflammation associated with MS or experimental autoimmune encephalomyelitis (EAE), the preclinical model for MS. Therefore, the objective of this study was to evaluate the effect of the combined treatment of melatonin and methylprednisolone on the neuroinflammatory response associated with the EAE development. This study shows for the first time the protective synergistic effect of co-treatment with melatonin and methylprednisolone on reducing the severity of EAE by decreasing CD4 lymphocytes, B cells, macrophages and dendritic cells in the CNS, as well as modulating the population of infiltrated T and B cells toward regulatory phenotypes to the detriment of pro-inflammatory effector functions. In addition to the potentiation of the protective role of methylprednisolone, treatment with melatonin from the clinical onset of EAE improves the natural course of the EAE and the response to a subsequent treatment with methylprednisolone in a later relapse of the disease, pointing melatonin as potential therapeutic tool in combination with methylprednisolone for the treatment of relapses in MS.


Asunto(s)
Modelos Animales de Enfermedad , Sinergismo Farmacológico , Encefalomielitis Autoinmune Experimental , Melatonina , Metilprednisolona , Esclerosis Múltiple , Melatonina/farmacología , Melatonina/uso terapéutico , Melatonina/administración & dosificación , Encefalomielitis Autoinmune Experimental/tratamiento farmacológico , Encefalomielitis Autoinmune Experimental/inmunología , Animales , Metilprednisolona/farmacología , Metilprednisolona/uso terapéutico , Ratones , Esclerosis Múltiple/tratamiento farmacológico , Esclerosis Múltiple/inmunología , Femenino , Enfermedades Neuroinflamatorias/etiología , Enfermedades Neuroinflamatorias/tratamiento farmacológico , Enfermedades Neuroinflamatorias/inmunología , Ratones Endogámicos C57BL , Antiinflamatorios/farmacología , Antiinflamatorios/uso terapéutico , Macrófagos/inmunología , Macrófagos/efectos de los fármacos , Macrófagos/metabolismo
18.
BMJ Case Rep ; 17(7)2024 Jul 04.
Artículo en Inglés | MEDLINE | ID: mdl-38964873

RESUMEN

Multisystem inflammatory syndrome in children (MIS-C) is a known complication of COVID-19. There is still limited knowledge about this condition. Here, we report the case of a previously healthy toddler boy, who presented with acute liver failure and duodenal lesions resulting in severe haematemesis and haemorrhagic shock, requiring intensive care unit care. The patient had persistent transaminitis, a deranged coagulation profile, inflammatory markers were elevated, and laboratory tests were negative for common infectious hepatitis aetiologies as well as COVID-19 Reverse transcription polymerase chain reaction. His COVID-19 antibody was reactive. Upper gastrointestinal endoscopy revealed a Forrest grade III duodenal ulcer. Looking into the constellation of symptoms and laboratory findings a confirmed diagnosis of acute viral hepatitis caused by MIS-C was made. Hence, he was given intravenous methylprednisolone along with intravenous immunoglobulins, after which he improved clinically and transaminitis resolved. The patient was discharged on clinical improvement and was doing fine on follow-up up to 6 months.


Asunto(s)
COVID-19 , Hemorragia Gastrointestinal , Fallo Hepático Agudo , Metilprednisolona , Síndrome de Respuesta Inflamatoria Sistémica , Humanos , Masculino , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , Síndrome de Respuesta Inflamatoria Sistémica/complicaciones , COVID-19/complicaciones , Fallo Hepático Agudo/diagnóstico , Fallo Hepático Agudo/etiología , Fallo Hepático Agudo/complicaciones , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/diagnóstico , Metilprednisolona/uso terapéutico , Metilprednisolona/administración & dosificación , Inmunoglobulinas Intravenosas/uso terapéutico , Hematemesis/etiología , Úlcera Duodenal/complicaciones , Úlcera Duodenal/diagnóstico , SARS-CoV-2 , Preescolar
19.
BMC Pulm Med ; 24(1): 321, 2024 Jul 04.
Artículo en Inglés | MEDLINE | ID: mdl-38965516

RESUMEN

BACKGROUND: Patients with multiple myeloma are immunosuppressed due to both the disease itself and immunosuppressive therapies. Thus, when presenting with respiratory failure and pulmonary opacities, pneumonia must be considered. However, while rare, immunomodulating medications used in the treatment of multiple myeloma can also cause potentially life-threatening respiratory failure, a distinction which has important treatment implications. CASE PRESENTATION: An 80-year-old male with recently diagnosed multiple myeloma undergoing treatment with lenalidomide and daratumumab presented with acute, rapidly progressive hypoxic respiratory failure ultimately requiring intubation and mechanical ventilatory support. Imaging revealed bilateral pulmonary opacities, however infectious workup was negative, and he was ultimately diagnosed with lenalidomide-induced interstitial pneumonitis, a rare but serious adverse effect of this medication. He was treated with drug discontinuation and methylprednisolone, and quickly recovered. CONCLUSION: Lenalidomide is an immunomodulating medication used in the treatment of multiple myeloma, and is associated with rare but serious cases of drug-induced interstitial pneumonitis. Thus, if a patient receiving lenalidomide develops shortness of breath and/or hypoxia, drug-induced pneumonitis must be on the differential. Permanent drug discontinuation with or without corticosteroids is the mainstay of treatment, and patients are often able to fully recover, underscoring the need for early recognition of this condition.


Asunto(s)
Lenalidomida , Enfermedades Pulmonares Intersticiales , Metilprednisolona , Mieloma Múltiple , Insuficiencia Respiratoria , Humanos , Lenalidomida/efectos adversos , Mieloma Múltiple/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/inducido químicamente , Masculino , Anciano de 80 o más Años , Insuficiencia Respiratoria/inducido químicamente , Metilprednisolona/uso terapéutico , Hipoxia/inducido químicamente , Agentes Inmunomoduladores/efectos adversos , Tomografía Computarizada por Rayos X , Anticuerpos Monoclonales
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