Results 41 to 50 of about 131,340 (225)

Clinical, Histologic, and Serological Predictors of Renal Function Loss in Lupus Nephritis

Arthritis Care &Research, EarlyView.
Objective Kidney survival is the ultimate goal in lupus nephritis (LN) management, but long‐term predictors remain inadequately studied, requiring long‐term follow‐up. This study aimed to identify baseline and early longitudinal predictors of kidney survival in the Accelerating Medicines Partnership LN longitudinal cohort.
Shangzhu Zhang, Laurence Magder, Daniel Goldman, Judith A. James, Joel M. Guthridge, Carla Guthridge, Peter Izmirly, Jill P. Buyon, H. Michael Belmont, Richard A. Furie, Noa Schwartz, Chaim Putterman, Jennifer L. Barnas, Jennifer H. Anolik, Sarah French, Maria Dall'Era, Avi Z. Rosenberg, Jeffrey Hodgin, Dawit S. Demeke, the Accelerating Medicines Partnership Rheumatoid Arthritis and Systemic Lupus Erythematosus (RA/SLE) Network, Michelle Petri, Andrea Fava   +21 more
wiley   +1 more source

R116C mutation of cationic trypsinogen in a Turkish family with recurrent pancreatitis illustrates genetic microheterogeneity of hereditary pancreatitis [PDF]

, 2001
Hereditary pancreatitis is due to heterozygosity for gain-of-function mutations in the cationic trypsinogen gene which result in increased levels of active trypsin within pancreatic acinar cells and autodigestion of the pancreas.
Dertinger, S., Ruebsamen, H., Lohse, P., Beck, M., Tautermann, G., Drexel, H.   +5 more
core   +1 more source

Phacolytic uveitis associated with spontaneous rupture of anterior capsule in a patient with Alport syndrome

Iatreia, 2019
Alport syndrome is a rare genetic disorder due to mutations involving the coding genes for type IV collagen characterized by renal failure, sensorineural hearing loss and ocular abnormalities.
López Torres, Vanessa, Ramírez-Cheyne, Julián Andrés , Lozano Cruz, Edgar, Salamanca Libreros, Omar Fernando   +3 more
doaj   +1 more source

Progressive and nonprogressive hereditary chronic nephritis [PDF]

, 1973
Progressive and nonprogressive hereditary chronic nephritis. Two groups of patients had hereditary chronic nephritis (HCN). The first group included six patients: microscopic hematuria was found in all six; the nephrotic syndrome developed in 3 patients ...
Grünfeld, Jean-Pierre, Hinglais, Nicole, Bois, Etienne P.   +2 more
core   +1 more source

Development of a Disease Activity Index for the Assessment of VEXAS Syndrome (VEXAS‐DAI)

Arthritis Care &Research, EarlyView.
Objective Vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic syndrome (VEXAS) syndrome is characterized by a complex spectrum of inflammatory and hematologic manifestations. Clinical research to identify effective therapies is urgently needed but is hindered by the lack of validated outcome measures.
Kevin Byram, Heřman Mann, Danielle Hammond, Onima Chowdhury, Sinisa Savic, Yohei Kirino, Carmelo Gurnari, Maël Heiblig, Thibault Comont, Arsène Mekinian, Mrinal M. Patnaik, Lachelle D. Weeks, Gary Ho, Adam Al‐Hakim, Scott Goldberg, Marcela A. Ferrada, Sophie Georgin‐Lavialle, Peter C. Grayson, Emma M. Groarke, Bhavisha Patel, Megan Sullivan, Sarah A. Buckley, Bryan G. Harder, Raman Garcha, David B. Beck, Matthew J. Koster   +25 more
wiley   +1 more source

Analysis of the Treatment Efficacy in Late Diagnosis of Alport Syndrome in a Child: Clinical Case

Вопросы современной педиатрии, 2023
Background. Alport syndrome is a systemic, hereditary, progressive disease characterized by ultrastructural changes in the glomerular basement membrane caused by pathogenic variants of type IV collagen genes.
Svetlana Ya. Volgina, Nailya A. Solovyeva, Galina A. Kulakova, Elena A. Kurmayeva, Liliya I. Mukhametdinova, Elina L. Rashitova   +5 more
doaj   +1 more source

Engineering Approaches to Modify Immunomodulatory Functions of Mesenchymal Stromal Cells (MSCs): Tissue Regeneration and Clinical Application

Advanced Science, EarlyView.
Mesenchymal stromal cells (MSCs) show promise for treating immune‐related disorders through immunomodulation and tissue regeneration. This review gives a brief overview of current clinical approval of MSC therapies. It also discussed how bioengineering, including genetic modification, biomaterial delivery, extracellular vesicles, and iPSC‐derived MSCs,
Sichen Yang, Kejia Li, Ning Wang, Zhen Li, Zhiyong Zhang, Rocky S. Tuan, Yangzi Jiang   +6 more
wiley   +1 more source

Apoptosis in renal disease: a brief review of the literature and report of preliminary findings in childhood lupus nephritis

The Turkish Journal of Pediatrics, 2001
Apoptosis, a programmed form of cell death, is an important mechanism that maintains cellular homeostasis. The cellular content of tissues is regulated by a balance between cell proliferation and cell loss.
K Tinaztepe, S Ozen, S Güçer, S Ozdamar   +3 more
doaj  

Epidemiology of biopsy‐proven glomerular diseases in Chinese children: A scoping review

Chronic Diseases and Translational Medicine, 2022
Background Glomerular disease is the leading cause of chronic kidney disease globally. No scoping review reports have focused on China's spectrum of glomerular diseases in children.
Yetong Li, Yue Yang, Li Zhuo, Dan Wu, Wenge Li, Xiaorong Liu   +5 more
doaj   +1 more source

Severity of disease and risk of malignant change in hereditary multiple exostoses. A genotype-phenotype study [PDF]

, 2004
We performed a prospective genotype-phenotype study using molecular screening and clinical assessment to compare the severity of disease and the risk of sarcoma in 172 individuals (78 families) with hereditary multiple exostoses.
Monaco, AP, Porter, JR, Simpson, AHRW, Lonie, L, Porter, D E, Simpson, Hamish, Porter, DE, Porter, J R, Monaco, Anthony, Simpson, AH, Monaco, A P, Fraser, M, Dobson-Stone, C   +12 more
core   +1 more source