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Introduction
Atypical hemolytic uremic syndrome (aHUS) is a rare and genetically mediated systemic disease most often caused by uncontrolled and chronic complement activation that leads to systemic thrombotic microangiopathy, renal and extra-renal damage.
Materials and methods
This is descriptive, retrospective and multicenter study, which reports demographic, clinical, laboratory, and genetic characteristics, as well as their treatment response and outcome of 20 aHUS patients diagnosed between 2014 and 2018.
Results
Most patients were female adults (75%) and 30% were associated to pregnancy/postpartum, 15% to autoimmune disease, and 65% to infections. Gastrointestinal involvement (75%) was the most frequent extra-renal organ damage. Antenatal mortality and mortality rate were 5% and 10%, respectively. 25% of the patients progressed to end-stage renal disease. In 4/8 of patients treated within 1 week of presentation, eculizumab treatment restored multi-organ function after 4 weeks of treatment. CFH (37%) and CFI (25%) mutations were the most frequent.
Conclusion
This is the first series of aHUS cases of Colombian Caribbean region which reports the clinical and epidemiological characteristics of this condition in this region.
Chronic liver disease (CLD) refers to a structural and functional change of the liver, which modifies the pharmacokinetics of multiple drugs, including hypoglycemic agents. This alteration depends on the severity degree of the liver disease, clinical characteristics of the patient, and comorbidities presence such as kidney disease and drug biochemistry. Insulin is considered a safe therapeutic strategy in patients with CLD, however, for many oral hypoglycemic agents, its use and dose adjustment will depend on the Child-Pugh score, based on the risk of hypoglycemia in this type of patient.
La vitamina D activa (1,25-dihidroxicolecalciferol) es una hormona esteroidea que posee múltiples funciones, entre ellas el metabolismo del calcio-fósforo y la mineralización ósea; empero, también tiene un papel fundamental en el funcionamiento del sistema inmunológico y es una herramienta imprescindible en la respuesta contra las infecciones bacterianas (incluidas las micobacterias), micóticas y virales. Se ha demostrado una sólida relación entre la hipovitaminosis D y el desarrollo de infecciones de las vías respiratorias, como neumonía, tuberculosis, infección por virus de la influenza, y aun la pandemia actual por COVID-19. Allí radica la importancia de identificar en fase temprana a los pacientes con deficiencia de vitamina D; el objetivo es corregir sus concentraciones y prevenir procesos infecciosos que eleven la morbimortalidad.
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