Результаты поиска

[Clinical effect of allogeneic hematopoietic stem cell transplantation in children with hyper-IgM syndrome].

OBJECTIVES: To evaluate the clinical effect of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children with hyper-IgM syn … were positive for Epstein-Barr virus, 7 were positive for cytomegalovirus, and 2 were positive for JC virus; 9 children were found to have invasive fungal disease. There were 6 children with acute graft-versus-host disease and 3 children with chronic graft-versus-host disease. The median follow-up time was about 2 years, and 3 children died in the early s …

Human pegivirus persistence in human blood virome after allogeneic haematopoietic stem-cell transplantation.

OBJECTIVES: Because commensal viruses are defined by the immunologic tolerance afforded to them, any immunomodulation, such as is received during haematopoietic stem-cell transplantation, may shift the demarcation between innocuous viral resident and disease-causing pathogen. METHODS: We analysed by deep-sequencing the plasma virome of 40 allogeneic haematopoietic stem-cell transplantation patients 1 month after transplantation. Because human pegivirus (HPgV) was highly prevalent, we performed a 1-year screening of 122 plasma samples by specific real-time reverse transcription PCR assay. We u

Metabolic profiles by 1H-magnetic resonance spectroscopy in natalizumab-associated post-PML lesions of multiple sclerosis patients who survived progressive multifocal leukoencephalopathy (PML).

PURPOSE: Early diagnosis and treatment of multiple sclerosis-related progressive multifocal leukoencephalopathy (PML) significantly improve clinical outcomes. However, there is a lack of information regarding the restart of immunomodulatory therapy in the post-PML setting, when multiple sclerosis activity reappears. We aimed at the examination of metabolic differences using 1H-magnetic resonance spectroscopy (1H-MRS) in multiple sclerosis patients at various post-PML stages and at the exploration of differences according to their disease and JC virus (JCV) status. METHODS: 1H-MRS of PML lesio…

Case 346.

A 66-year-old man with a history of hypertension presented with left-sided jaw jerking, repetitive movements, aphasia, and dysarthria. He wa … ormal protein and glucose levels. Cerebral spinal fluid studies, including protein electrophoresis, JC virus, herpes simplex virus polymerase chain reaction, MOG antibodies, cytologic examination, and a paraneoplastic panel (Table), were negative. The patient was treated with nicardipine and levetiracetam, with clinical improvement. A presumptive clinical …

Natalizumab treatment perturbs memory- and marginal zone-like B-cell homing in secondary lymphoid organs in multiple sclerosis.

Natalizumab, an antibody against the α4 subunit of α4 integrins, has been approved for multiple sclerosis (MS) therapy based on its high efficacy and safety profile. However, natalizumab has been associated with the development of progressive multifocal leukoencephalopathy (PML), a disorder caused by JC virus (JCV) infection. In order to improve our understanding of the mechanism of action of natalizumab and to identify possible risk factors for PML development, we have characterized in detail the cell blood composition in MS patients treated with natalizumab for more than 30 months. Natalizum…

Pharmacological properties, toxicology and scientific rationale for the use of natalizumab (Tysabri) in inflammatory diseases.

Natalizumab (Tysabri) was the first adhesion molecule antagonist to make it into clinical trial for patients with multiple sclerosis (MS) and other inflammatory disorders. Natalizumab is a humanized recombinant monoclonal antibody (MAb) that binds to the alpha (alpha)(4) chain of the alpha(4) beta (beta)(1) (very late activating antigen 4; VLA-4) and alpha(4)beta(7) integrins. The scientific rationale for natalizumab therapy is the reduction of leukocyte extravasation into peripheral tissues. Natalizumab, like other VLA-4 antagonists, may also interfere with the activation of T lymphocytes in

Anaphylaxis induced by glucocorticoids.

Natalizumab is a highly effective immunomodulator in the treatment of multiple sclerosis (MS). Treatment with natalizumab has been associated with progressive multifocal leukoencephalopathy (PML), an infection of the central nervous system (CNS) caused by a pathogenic form of the normally benign JC virus (JCV). We searched PubMed and used current data from the natalizumab global safety database to assess risk factors and quantify the risk of PML. Natalizumab treatment duration and prior use of immunosuppressive therapies are established risk factors for development of PML in natalizumab-treate…

Генетика прогрессивной многофукальной лейкоэнцефалопатии: обновление отчетов по делам с врожденной ошибкой иммунитета и вариантами риска, обнаруженными в случаях, связанных с наркотиками.

В настоящее время генетическая предрасположенность к PML в значительной степени подкрепляется сообщениями о случаях, когда пациенты молекулярно диагностировали врожденную ошибку иммунитета (IEI) и прогрессивную многофукальную лейкоэнцефалопатию (PML). За последние 10 лет 4 гена IEI, связанные с PML, теперь выросли до 26 в 2025 году. Из этих 26 генов, которые, как считается, являются причиной IEI и PML, 24 (92%) также связаны с гемофагоцитическим лимфогиистическим синдромом активации/макропатическим синдромом (HLH/MAS) - с сильным гипервоспалительным синдромом, связанным с несколькими генами IE

Совпадение в аннотации оригинала
A genetic predisposition to PML is now substantially supported by case reports of patients molecularly diagnosed with an inborn error of imm … enes are associated with life-threatening Epstein-Barr virus infections, which analogously suggests JC virus infection plus presence of a pathogenic variant in an HLH-linked IEI gene also increases risk of PML. PML also occurs as a serious adverse event for a subset of immunosuppressive therapies (e.g., natalizumab and rituximab) used to treat patients wit …

Metagenomic Virome Sequencing in Living Donor and Recipient Kidney Transplant Pairs Revealed JC Polyomavirus Transmission.

BACKGROUND: Before kidney transplantation, donors and recipients are routinely screened for viral pathogens using specific tests. Little is known about unrecognized viruses of the urinary tract that potentially result in transmission. Using an open metagenomic approach, we aimed to comprehensively assess virus transmission in living-donor kidney transplantation. METHODS: Living kidney donors and their corresponding recipients were enrolled at the time of transplantation. Follow-up study visits for recipients were scheduled 4-6 weeks and 1 year thereafter. At each visit, plasma and urine sampl

Natural history of GATA2 deficiency in a survey of 79 French and Belgian patients.

Heterozygous germline GATA2 mutations strongly predispose to leukemia, immunodeficiency, and/or lymphoedema. We describe a series of 79 pati … , juvenile myelomonocytic leukemia, fatal progressive multifocal leukoencephalopathy related to the JC virus, and immune/inflammatory diseases. A revised International Prognostic Scoring System (IPSS) score allowed a distinction to be made between a stable disease and hematologic transformation. Chemotherapy is of limited efficacy, and has a high toxicity …

New-onset seizure in HIV-infected adult Zambians: A search for causes and consequences.

OBJECTIVE: To identify the etiology of new-onset seizure in HIV-infected Zambian adults and identify risk factors for seizure recurrence. M … (26%), hyponatremia in 23 (28%), and other infections in 8 (10%). OIs included Cryptococcus (17%), JC virus (7%) and 5% each for tuberculosis, cytomegalovirus, and varicella-zoster virus. No etiology could be identified in 16 (20%). Thirty (37%) patients died during follow-up and 20 (25%) had recurrent seizures with survival being the only identifiable ri …

Hemorrhagic cytitis after bone marrow transplantation.

OBJECTIVES: Hemorrhagic cystitis (HC) presenting with gross hematuria, bladder pain and urinary frequency develops in 13-38% of patients following bone marrow transplantation (BMT). The objective of the study was to study the characteristics of patients suffering hemorrhagic cystitis after hematopoietic stem cell transplantation in our center. METHODS: We conducted a retrospective chart review of all patients who underwent BMT at our institution between January 1996 and August 2012. We recorded the age, sex, diagnosis, conditioning regimen, interval between BMT and development of symptoms of

Infectious complications in polymyositis and dermatomyositis: a series of 279 patients.

OBJECTIVES: To assess the prevalence and characteristics of severe pyogenic, nonpyogenic, and opportunistic infections in polymyositis and d … osis), Helicobacter heilmanii, cytomegalovirus, herpes simplex and zoster virus, hepatitis B and C, JC virus, Leishmania major, Strongyloides stercoralis. Esophageal dysfunction, ventilatory insufficiency, malignancy, and lymphopenia were significantly more frequent in the group of PM/DM patients with infections. CONCLUSION: Our study underscores the high …

Incidence, clinical presentation, and outcome of progressive multifocal leukoencephalopathy in HIV-infected patients during the highly active antiretroviral therapy era: a nationwide cohort study.

BACKGROUND: Human immunodeficiency virus (HIV) infection predisposes to progressive multifocal leukoencephalopathy (PML). Here, we describe … and 47% of cases were diagnosed by means of brain biopsy or polymerase chain reaction analysis for JC virus. The predominant neurological symptoms at presentation were coordination disturbance, cognitive defects, and limb paresis. Thirty-five patients died; the median survival time was 0.4 years (95% confidence interval [CI], 0.0-0.7) in 1995-1996 and 1.8 …

Вирусная регуляционная последовательность в почечной ткани, получаемая от пациентов с нефропатией BK.

В геноме полиомавируса содержится область некодирования (NCCR), которая, как считается, содержит элементы усиления, которые являются важными инициаторами вирусной транскрипции. Мы предположили, что вариации ДНК в этом регионе играют определенную роль в патогенезе нефропатии вируса BK (BKVAN). МЕТОДЫ: Тканевые секции были получены из 26 биопсий с парафинами и BKVAN, полученных от 15 пациентов. Разделы были лизаны в буфере протеиназы K и подвергнуты ДНК-последовательности с использованием секвенировочных реакций Большого Дая с помощью автоматизированного секвенирования ДНК (ABI 310). Анатомия NC

Совпадение в аннотации оригинала
BACKGROUND: The polyomavirus genome contains a noncoding control region (NCCR), which is believed to contain the enhancer elements that are … oter (3/22 biopsies). Five biopsies showed complex rearrangements reminiscent of those described in JC virus-associated progressive multifocal leukoencephalopathy. CONCLUSION: The regulatory region of BKV shows sequence heterogeneity in biopsy tissue with viral nephropathy. Sequence variations frequently affect putative transcription factor binding sites, …

Successful treatment of HIV-related progressive multifocal leukoencephalopathy and immunological reconstitution inflammatory syndrome with intravenous human immunoglobulin: a case report.

BACKGROUND: Progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome (PML-IRIS) is a high-mortality disease among patients with AIDS. It is caused by infection with the John Cunningham virus (JCV). Currently, there are no specific antiviral treatments targeting JCV. Thus, immune reconstitution remains the primary therapeutic approach. CASE PRESENTATION: A 29-year-old male patient diagnosed with AIDS presented for medical evaluation after two months of antiretroviral therapy (ART), reporting symptoms of dizziness and headache. The detection of JC virus was confirm…

Evaluation of risk management in a natalizumab home infusion procedure.

Natalizumab is a well-established disease-modifying therapy used in active multiple sclerosis (MS). The most serious adverse event is progressive multifocal leukoencephalopathy. For safety reasons, hospital implementation is mandatory. The SARS-CoV-2 pandemic has deeply affected hospital practices leading French authorities to temporarily authorize to administer the treatment at home. The safety of natalizumab home administration should be assessed to allow ongoing home infusion. The aim of the study is to describe the procedure and assess the safety in a home infusion natalizumab model. Patie

Detection of Residual Tumor With 68Ga-Pentixafor PET/CT in a Patient With Waldenström Macroglobulinemia and Concurrent John Cunningham Virus-Related Progressive Multifocal Leukoencephalopathy.

A 75-year-old man diagnosed with Waldenström macroglobulinemia (WM) complained of neurological symptoms. Baseline F-FDG PET/CT showed diffusely increased radioactivity in the bone marrow and decreased FDG uptake in the right cerebellum. After rituximab-containing immunochemotherapy, the patient had clinically partial response of WM, but the cerebellar lesion was enlarged. Repeated F-FDG PET/CT was similar to the baseline. Ga-pentixafor PET/CT detected residual tumor of WM in occipital bone and cervical lymph nodes, but there was no uptake in the cerebellar lesion. Finally, John Cunningham viru

Progressive Multifocal Leukoencephalopathy in the Absence of Typical Radiological Changes: Can We Make a Diagnosis?

BACKGROUND Progressive multifocal leukoencephalopathy (PML) is a serious opportunistic infectious disease with high morbidity and mortality. Its incidence in multiple sclerosis (MS) patients has risen since the introduction of disease modifying drugs. In the absence of a specific treatment, the outcome depends heavily on early diagnosis, which illustrates the importance of the role of characteristic brain magnetic resonance imaging (MRI). However, when relying mainly on MRI, the diagnosis of cases with atypical radiological changes may be missed or delayed. CASE REPORT A 32-year-old female dia

Progressive multifocal leukoencephalopathy in a renal transplant patient.

End-stage renal disease (ESRD) has a major impact on health and affects more than 600,000 people in the USA. The current mainstay treatments include dialysis and kidney transplantation (KT), and patients who have received KT have a higher quality of life and a lower mortality risk than those on chronic dialysis. Therefore, KT is considered the more preferred treatment modality for patients with ESRD. However, even though KT results in a higher long-term survival rate, the use of immunosuppressants is associated with various complications, including opportunistic infections and malignancies, wh