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Safety and efficacy of intra-erythrocyte dexamethasone sodium phosphate in children with ataxia telangiectasia (ATTeST): a multicentre, randomised, double-blind, placebo-controlled phase 3 trial.
Lancet Neurol
September 2024
Division of Pediatric Allergy and Immunology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Background: Ataxia telangiectasia is a multisystem disorder with progressive neurodegeneration. Corticosteroids can improve neurological functioning in patients with the disorder but adrenal suppression and symptom recurrence on treatment discontinuation has limited their use, prompting the development of novel steroid delivery systems. The aim of the ATTeST study was to evaluate the efficacy and safety of intra-erythrocyte delivery of dexamethasone sodium phosphate compared with placebo in children with ataxia telangiectasia.
View Article and Find Full Text PDFClinical presentations and diagnostic application of proposed biomarkers in psychiatric-onset prodromal dementia with Lewy bodies.
Psychogeriatrics
July 2024
Department of Psychiatry, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Research criteria for the diagnosis of prodromal dementia with Lewy bodies (DLB) include three clinical subtypes: mild cognitive impairment with Lewy bodies (MCI-LB), delirium-onset prodromal DLB, and psychiatric-onset prodromal DLB. Late-onset psychiatric manifestations are at a higher risk of developing dementia, but its relation to prodromal DLB remains unclear. In addition to the risk of severe antipsychotic hypersensitivity reactions, accurate discrimination from non-DLB cases is important due to the potential differences in management and prognosis.
View Article and Find Full Text PDFA narrative review of the literature relevant to obstetric anesthesiologists: the 2023 Gerard W. Ostheimer lecture.
Int J Obstet Anesth
May 2024
Stanford University School of Medicine, Stanford, CA, USA. Electronic address:
Unlabelled: This narrative review of the 2023 Gerard W. Ostheimer lecture presented at the Society for Obstetric Anesthesia and Perinatology 2023 annual meeting summarizes 2022 literature relevant to obstetric anesthesiologists.
Antenatal Studies: Neonatal morbidity is reduced with antenatal maternal buprenorphine compared with methadone for treatment of opioid use disorder.
Dermatographism in popular culture.
Clin Dermatol
December 2022
Private Practice, Pembroke Pines, Florida, USA. Electronic address:
Dermatographism was first described by William Heberden (1710-1801) more than 250 years ago as a type of urticaria brought on by rubbing or scratching the skin. In 1859, William Gull (1816-1890) gave it the name factitious urticaria, distinguishing dermatographism from chronic urticaria, in which the skin lesions appear spontaneously. During the 1870s French physicians at the Salpetriere Hospital in Paris became impressed by their ability to write words on the bodies of patients admitted with hysteria and other psychiatric disorders, who also exhibited dermatographism.
View Article and Find Full Text PDFNovel mutations in unrelated Vietnamese patients with chronic granulomatous disease.
Clin Chim Acta
August 2022
Institute of Genome Research, Vietnam Academy of Science and Technology, 18 - Hoang Quoc Viet str., Caugiay, Hanoi, Viet Nam; Graduate University of Science and Technology, Vietnam Academy of Science and Technology, 18 - Hoang Quoc Viet str., Caugiay, Hanoi, Viet Nam. Electronic address:
Background: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder (PID) due to genetic defects in the NADPH oxidase of phagocytes. Affected patients become susceptible to infections such as pneumonia, diarrhea, and skin ulcer types. The patients require life-long treatment with prophylactic antibiotics, antifungals, or hematopoietic stem cell transplantation (HSCT) therapy.
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