A 26-year-old female was hospitalized with acute lower motor neuron quadriplegia. Laboratory tests pointed to the presence of distal renal tubular acidosis, which was characterized by hyperchloremic metabolic acidosis, severe hypokalemia, alkaline urine, and a positive urinary anion gap. She also had aminoaciduria, hyperphosphaturia, hypophosphatemia, and normoglycemic glycosuria, all of which are indicative of dysfunction of proximal tubules. Further investigation confirmed Sjogren's syndrome. Strangely, our patient also experienced carpopedal spasms and had low calcium and magnesium levels. As the hypokalemia, hypocalcemia, and acidosis were corrected, the quadriplegia and carpopedal spasm improved. By the time of discharge, proximal tubular abnormalities were rectified (with the exception of albuminuria). One well-known renal symptom of Sjogren's syndrome is distal tubular acidosis. The brief proximal tubular dysfunction and distal tubular acidosis in Sjogren's syndrome is rare. This case report highlights a rare renal complication of Sjogren's syndrome.
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http://dx.doi.org/10.7759/cureus.48268 | DOI Listing |
J Transl Med
January 2025
Department of Rheumatology and Immunology, Peking University Third Hospital, No. 49, North Garden Road, Beijing, 100191, China.
Background: Sjogren syndrome (SS) is a chronic systemic autoimmune disease and its pathogenesis often involves the participation of numerous immune cells and inflammatory factors. Despite increased researches and studies recently focusing on this area, it remains to be fully elucidated. We decide to incorporate genetic insight into investigation of the causal link between various immune cells, inflammatory factors and pathogenesis of Sjogren syndrome (SS).
View Article and Find Full Text PDFPediatr Nephrol
January 2025
Division of Nephrology, Department of Pediatrics, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan.
Distal renal tubular acidosis (dRTA) is a significant clinical expression of Sjögren's syndrome (SS). While SS-related dRTA is traditionally linked to impaired H-ATPase, we report a unique case demonstrating selectively decreased anion exchanger 1 (AE1) expression with preserved H-ATPase expression. A 16-year-old girl with SS presented with muscle weakness, difficulty in ambulation, and severe hypokalemia.
View Article and Find Full Text PDFACS Appl Mater Interfaces
January 2025
Center Laboratory, Peking University School and Hospital of Stomatology & National Center for Stomatology & National Clinical Research Center for Oral Diseases & National Engineering Research Center of Oral Biomaterials and Digital Medical Devices & Beijing Key Laboratory of Digital Stomatology & NHC Key Laboratory of Digital Stomatology & NMPA Key Laboratory for Dental Materials, No. 22, Zhongguancun South Avenue, Haidian District, Beijing 100081, P. R. China.
: Sjögren's syndrome (SS), an autoimmune disease, was characterized by sicca syndrome and systemic manifestations, presenting significant treatment challenges. Exosomes, naturally derived nanoparticles containing bioactive molecules, have garnered interest in regenerative medicine. The present study aimed to elucidate the immunoregulatory properties and mechanism of exosomes obtained from the stem cells derived from human exfoliated deciduous teeth (SHED-exos) in SS-induced sialadenitis.
View Article and Find Full Text PDFCentrioles play central roles in ciliogenesis and mitotic spindle assembly. Once assembled, centrioles exhibit long-term stability, a property essential for maintaining numerical control. How centriole stability is achieved and how it is lost in certain biological contexts are still not completely understood.
View Article and Find Full Text PDFInt J Immunogenet
January 2025
Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India.
The association between heterozygous C4 deficiency and systemic lupus erythematosus (SLE) is unclear. There is a lack of data in South Asian Indians on any possible association of C4A and C4B null alleles with lupus. We aimed to study the prevalence of C4A and C4B null alleles in a cohort of SLE patients with persistently low C4 levels compared to healthy controls (HC).
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