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Management of Steroid-Refractory Serositis and Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis.
Indian J Pediatr
December 2024
Department of Pediatrics, Dr. D.Y. Patil Medical College, Hospital and Research Center, Pune, Maharashtra, India.
Childhood idiopathic nephrotic syndrome: recent advancements shaping future guidelines.
Pediatr Nephrol
December 2024
Néphrologie Pédiatrique, Centre de Référence du Syndrome Néphrotique Idiopathique de L'enfant Et L'adulte, Hôpital Necker - Enfants Malades, APHP, Inserm U1163, Institut Imagine, Université Paris Cité, Paris, France.
Childhood idiopathic nephrotic syndrome is an important pediatric kidney disease associated with significant morbidities and even mortality. Several guidelines have been developed to standardize the terminology and patient care among the pediatric nephrology community. Since the publication of these guidelines, there have been major breakthroughs in the disease management and the understanding of underlying pathogenesis through multi-omics investigations, including the identification of anti-nephrin autoantibodies, genetic susceptibility loci, and the pathogenic role of B cell subsets.
View Article and Find Full Text PDFAdult minimal change disease: Clinicopathologic characteristics, treatment response and outcome at a single center in Pakistan.
World J Nephrol
December 2024
Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan.
Background: Minimal change disease (MCD) is a significant cause of idiopathic nephrotic syndrome (INS) in adults, representing approximately 10%-15% of INS cases. The data is scanty on clinicopathological features, treatment responses, and long-term outcomes of MCD in adults.
Aim: To determine the clinicopathologic characteristics, treatment responses, and medium-term outcomes of adult patients with MCD in Pakistan.
Corticosteroid therapy for treating acute exacerbation of interstitial lung diseases: a systematic review.
Thorax
December 2024
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Introduction: Acute exacerbation of interstitial lung disease (AE-ILD) often results in death and poses significant challenges in clinical management. While corticosteroids are frequently employed, the optimal regimen and their clinical efficacy remain uncertain. To address this knowledge gap, we undertook a systematic review to evaluate the impact of steroid therapy on clinical outcomes in patients experiencing AE-ILD.
View Article and Find Full Text PDFIdiopathic Vanishing Bile Duct Syndrome in a Young Female: A Case Report.
Korean J Gastroenterol
December 2024
Department of Internal Medicine, Pusan National University College of Medicine, Yangsan, Korea.
Vanishing bile duct syndrome (VBDS) is characterized by the progressive loss and destruction of the intrahepatic bile ducts, leading to bile stasis and associated symptoms such as jaundice. This condition is commonly associated with drug side effects, infections, neoplasms, and autoimmune diseases, but the precise mechanism of its development is unclear. Although VBDS can be diagnosed based on the patient's symptoms and disease progression, a liver biopsy is essential for confirmation, and the prognosis can vary significantly.
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