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Future Directions in the Management of Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.
J Clin Endocrinol Metab
January 2025
Departments of Pharmacology and Internal Medicine, Division of Metabolism, Endocrinology and Diabetes, University of Michigan Medical School, Ann Arbor, MI 48109, USA.
Context: The traditional management of classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21OHD) is difficult and often suboptimal.
Objective: To review improvements in the diagnosis and management of 21OHD.
Design: Literature review, synthesis, and authors' experience.
Rapid and Sustained Resolution of Peristomal Pyoderma Gangrenosum With Aerosol Steroid Treatment.
J Wound Ostomy Continence Nurs
January 2025
Kyriaki Stefania Mitsaki, MBBCh, BSc (Hons), MSc, MRCP, Department of Dermatology, Northwick Park Hospital, London North West University Hospital NHS Trust, London, UK.
Background: Peristomal pyoderma gangrenosum (PPG) is a non-infectious neutrophilic dermatosis most commonly seen in the context of ostomies in inflammatory bowel disease. The lack of established treatment guidelines and high-quality evidence in the form of randomized controlled trials present a major challenge in PPG management, owing to the rarity of the condition. Treatment can be further complicated by difficulties in maintaining the stoma pouch seal with conventional topical corticosteroids.
View Article and Find Full Text PDFManagement of chimeric antigen receptor T-cell-related toxicity of a patient affected by cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome, followed by an intestinal perforation: a case report.
J Med Case Rep
January 2025
Director of Hospital Pharmacy, Santa Croce e Carle Hospital, Cuneo, Italy.
Background: Mantle cell lymphoma is a diverse B-cell lymphoma with varying clinical behaviors. Treating relapsed or refractory mantle cell lymphoma is challenging, with Bruton's tyrosine kinase inhibitors proving effective but not curative. Post-Bruton's tyrosine kinase inhibitor failure, the prognosis remains unfavorable.
View Article and Find Full Text PDFAcute macular neuroretinopathy occurrence in a Behçet disease patient: a case report.
J Ophthalmic Inflamm Infect
January 2025
Ophthalmology Department, CHIREC Braine-l'Alleud-Waterloo Hospital, Braine l'Alleud, Belgium.
Purpose: To report the occurrence of AMN (Acute Macular Neuroretinopathy) in a Behçet Disease (BD) patient during an active systemic inflammatory relapse and to describe the SD-OCT features of this entity.
Patients And Methods: Retrospective observational case report of a patient who presented with an AMN during a BD associated ocular inflammation (Saint Pierre Hospital, Brussels, Belgium). Clinical record and imaging, including infrared reflectance image (IR) and spectral domain optical coherence tomography (SD-OCT), were analyzed.
Evaluating Avacopan in the Treatment of ANCA-Associated Vasculitis: Design, Development and Positioning of Therapy.
Drug Des Devel Ther
January 2025
Center of Expertise for Lupus-, Vasculitis- and Complement-Mediated Systemic Diseases (Luvacs), Department of Internal Medicine - Nephrology Section, Leiden University Medical Center, Leiden, the Netherlands.
Recently, avacopan has been approved for the treatment of ANCA-associated vasculitis (AAV). Avacopan is an inhibitor of the C5a-receptor, which plays an important role in chemotaxis and the amplification loop of inflammation in AAV. In the most recent, international guidelines avacopan is recommended as steroid-sparing agents for the management of AAV.
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