27 April 2020 : Case report
Challenging differential diagnosis, Rare diseaseRaman Sohal1ABCDEF*, Tanya George1ABCDEF
Am J Case Rep 2020; 21:e919926
BACKGROUND: Calcific uremic arteriolopathy (CUA) is a rare and incredibly painful cutaneous disorder secondary to microvascular involvement in which calcium dysregulation leads to stenosis of medium sized arterial blood vessels along with endothelial dysregulation and thrombosis. Ultimately, these patients are at high risk for non-healing wounds with risk of death from sepsis and multi-organ failure. It is a poorly understood condition with limited therapies that do not offer mortality benefit. Prevalence is about 4% in hemodialysis patients. Sodium thiosulfate (STS) can be used in hemodialysis patients but therapy is often limited by the development of high anion gap metabolic acidosis.
CASE REPORT: A 53-year-old male who had end stage renal disease and who was on hemodialysis and taking warfarin for bio-prosthetic mitral valve replacement and atrial fibrillation presented with non-healing right lower extremity cellulitis which had failed outpatient treatment. A skin biopsy of the lesion was consistent with CUA. The patient failed to improve on calcitriol and cinacalcet and was started on intravenous STS. Subsequently, he developed life threatening metabolic acidosis requiring a bicarbonate drip. He died 12 weeks after his initial diagnosis of CUA.
CONCLUSIONS: This article seeks to describe how the treatment of CUA; a rare disease with high mortality, is limited by the development of metabolic acidosis when using STS therapy. There is an 80% mortality rate within 6 months from CUA with major adverse effect of a high anion gap metabolic acidosis. Further research is needed in the field of establishing optimal dosing and frequency.
Keywords: Acidosis, calciphylaxis, Gold Sodium Thiosulfate, Kidney Failure, Chronic, Warfarin, Chelating Agents, Comorbidity, Fatal Outcome, Middle Aged, Thiosulfates
27 Jan 2023 : Case reportA 37-Year-Old Man with Familial Hereditary Hemorrhagic Telangiectasia and Pulmonary Arteriovenous Malformat...
Am J Case Rep In Press; DOI: 10.12659/AJCR.938548
26 Jan 2023 : Case reportA 39-Year-Old Woman with Endometriosis Who Developed a Subcapsular Liver Hematoma Following Laparotomic Sur...
Am J Case Rep In Press; DOI: 10.12659/AJCR.938169
26 Jan 2023 : Case reportOptic Neuritis as a Consequence of Long-Term Medical Treatment Using Anastrozole: A Case Report
Am J Case Rep In Press; DOI: 10.12659/AJCR.939026
Most Viewed Current Articles
13 Jul 2022 : Case reportWhistling Scrotum: An Unusual Presentation of Pneumomediastinum in the Setting of an Open Scrotal Wound
Am J Case Rep 2022; 23:e936441
06 Dec 2021 : Case reportLipedema Can Be Treated Non-Surgically: A Report of 5 Cases
Am J Case Rep 2021; 22:e934406
23 Feb 2022 : Case reportPenile Necrosis Associated with Local Intravenous Injection of Cocaine
Am J Case Rep 2022; 23:e935250