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A case of Sjögren's syndrome with selective anion exchanger 1 defect causing distal renal tubular acidosis.
Pediatr 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 PDFA Case of Euglycemic Ketoacidosis Secondary to Continuous Renal Replacement Therapy.
Case Rep Crit Care
January 2025
Division of Pulmonary, Critical Care & Sleep Medicine, Keck Hospital of USC, Los Angeles, California, USA.
Euglycemic ketoacidosis (EKA) has been reported as a rare but life-threatening complication of continuous renal replacement therapy (CRRT). EKA should be suspected in the setting of persistent high anion gap metabolic acidosis despite renal replacement therapy. Critically ill patients, especially those with diabetes mellitus, are at risk of EKA due to deficient caloric intake, the presence of excess counterregulatory stress hormones, and nutritional losses from CRRT.
View Article and Find Full Text PDFLactic acidosis with metformin accumulation in the intensive care units of the Nord Pas de Calais region: A known serious adverse event that can be better prevented.
Therapie
December 2024
CHU Lille, University Lille, Intensive Médecine Reanimation, 59000 Lille, France.
Article Synopsis
- The study focused on identifying situations that lead to metformin-associated lactic acidosis (MALA) in ICU patients and evaluating its preventability.
- A total of 198 MALA cases were assessed, with 19.2% resulting in death; the majority of patients had acute events like dehydration or severe infections that contributed to the condition.
- The findings indicated that MALA is often preventable, highlighting the need for better physician and patient education regarding metformin use during high-risk medical situations.
Therapeutic Potential of Ketogenic Interventions for Autosomal-Dominant Polycystic Kidney Disease: A Systematic Review.
Nutrients
December 2024
Centre for Diabetes, Obesity and Endocrinology Research (CDOER), Westmead Institute for Medical Research, Westmead, Sydney, NSW 2145, Australia.
Background: Recent findings have highlighted that abnormal energy metabolism is a key feature of autosomal-dominant polycystic kidney disease (ADPKD). Emerging evidence suggests that nutritional ketosis could offer therapeutic benefits, including potentially slowing or even reversing disease progression. This systematic review aims to synthesise the literature on ketogenic interventions to evaluate the impact in ADPKD.
View Article and Find Full Text PDFAtypical Presentation of Distal Renal Tubular Acidosis in a Child: Correspondence.
Indian J Pediatr
January 2025
Department of Internal Medicine, Yanbian University Hospital, Yanji, Jilin, China.
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