Gitelman syndrome is an autosomal recessive, chronically salt-losing tubulopathy depicted by renal potassium wasting, hypokalemia, hypocalciuric, hypomagnesemia, metabolic alkalosis, and hyperreninemic hyperaldosteronism with average or low blood pressure. This case report describes a 10-year-old boy who presented with acute respiratory tract infection with respiratory distress, myalgia, generalized muscle weakness, and significant biochemical changes like hypokalemia, hypomagnesemia, and metabolic alkalosis associated with failure to thrive. Further investigations, like genetic testing, showed a SLC12A3 gene mutation, a pathogenic homozygosity variant, proving the diagnosis of Gitelman syndrome. The child needed Intensive Care Unit (ICU) admission for life-threatening electrolyte imbalances with Electrocardiogram (ECG) changes for the acute care and later, requiring a multidisciplinary team approach for the management. The early presentation of Gitelman syndrome in young children must be kept in mind, as it could be missed. The persistent metabolic alkalosis, hypokalemia and hypomagnesemia should raise the concern about the possibility of chronic salt-losing nephropathic conditions.
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http://dx.doi.org/10.11604/pamj.2024.49.59.45186 | DOI Listing |
Front Endocrinol (Lausanne)
March 2025
Department of Endocrinology, Central Hospital of Dalian University of Technology, Dalian, China.
Introduction: Familial hypocalciuric hypercalcemia (FHH) is an autosomal dominant disorder caused by an inactivating mutation in the gene, while Gitelman syndrome (GS) is an autosomal recessive renal tubular disorder resulting from a pathogenic mutation in the gene. Both genetic disorders are relatively rare. This report presents a patient with both FHH and GS, exhibiting unique clinical and genetic complexities.
View Article and Find Full Text PDFAging (Albany NY)
March 2025
Division of Nephrology, Department of Internal Medicine, National Defense Medical Center, Taipei 104, Taiwan.
Gitelman syndrome (GS) is the most common hereditary renal tubular disorder, with a higher carrier frequency among Asians often overlooked in older adults. Electrolyte imbalances, such as those seen in GS, are crucial considerations for older adults experiencing recurrent falls. We described an 83-year-old diabetic female on metformin, who was admitted due to recurrent falls with the preceding dizziness and palpitations when standing.
View Article and Find Full Text PDFJ Med Case Rep
February 2025
Department of Pathological Sciences, College of Medicine, Ajman University, P.O. Box 346, Ajman, United Arab Emirates.
Background: Gitelman syndrome is a rare autosomal recessive disorder that affects the distal convoluted tubules of the kidneys. It often manifests through various symptoms, including muscle weakness, paresthesia, fatigue, or paralysis. Owing to the scarcity of case reports regarding Gitelman syndrome in the Middle East and North Africa region, it is imperative to spread awareness about this syndrome for prompt diagnosis.
View Article and Find Full Text PDFOrphanet J Rare Dis
February 2025
Department of Clinical Laboratory, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, 150 Ximen Street, Linhai, China.
Background: Gitelman syndrome (GS) is an inherited renal tubular disorder characterized by hypokalemic alkalosis and hypomagnesemia, due to biallelic pathogenic variants in the solute carrier family 12 member 3 (SLC12A3) gene encoding a sodium-chloride (Na-Cl) cotransporter (NCC). This work aimed at identifying SLC12A3 variants in the GS pedigree and reveal the effect of the mutations on protein structure and function.
Methods: Whole-exome sequencing (WES) and Sanger sequencing were performed in the pedigree.
Pan Afr Med J
February 2025
Department of Pediatrics and Pediatric Intensive Care Unit, King Hamad University Hospital, Al Sayh, Bahrain.
Gitelman syndrome is an autosomal recessive, chronically salt-losing tubulopathy depicted by renal potassium wasting, hypokalemia, hypocalciuric, hypomagnesemia, metabolic alkalosis, and hyperreninemic hyperaldosteronism with average or low blood pressure. This case report describes a 10-year-old boy who presented with acute respiratory tract infection with respiratory distress, myalgia, generalized muscle weakness, and significant biochemical changes like hypokalemia, hypomagnesemia, and metabolic alkalosis associated with failure to thrive. Further investigations, like genetic testing, showed a SLC12A3 gene mutation, a pathogenic homozygosity variant, proving the diagnosis of Gitelman syndrome.
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