Atypical hemolytic uremic syndrome: a nationwide Colombian pediatric series
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Fecha
2023
Autores
Espitaleta, Zilac
Domínguez-Vargas, Alex
Villamizar-Martínez, Johanna
Carrascal-Guzmán, Martha
Guerrero-Tinoco, Gustavo
Silva-Diaz, Diana
Baquero, Richard
Pinto-Bernal, Claudia
González-Chaparro, Luz
Rojas-Rosas, Luisa
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Research Square Company
Resumen
Background
Atypical hemolytic uremic syndrome (aHUS) is a rare complement-mediated kidney disease with genetic
predisposition and represents up to 10% of pediatric hemolytic uremic syndrome (HUS) cases. Few studies have
evaluated aHUS in Latin American population. We studied a Colombian pediatric cohort to delineate disease
presentation and outcomes.
Methods
A multicenter cohort of 27 Colombian children with aHUS were enrolled between 2010 to 2019. Patients were grouped
by age at onset. Clinical features were compared using analysis of variance (ANOVA) and Fisher exact tests. Renal
biopsy was performed on six patients who were suspected of having other renal diseases before aHUS diagnosis.
Results
Most patients were male (70%). The onset of aHUS occurred frequently before age 4 years (60%) and followed
gastroenteritis as the main triggering event (52%). Age groups were comparable in clinical presentation, disease
severity, treatment, and outcomes. Pulmonary involvement (67%) was the main extrarenal manifestation. A higher
frequency was observed in the 1–7 age group (p = 0.01). Renal biopsies were as follows: three had
membranoproliferative glomerulonephritis (MPGN) type I, one MPGN type III, one C3-glomerulonephritis, and one
rapidly progressive glomerulonephritis. Genetic screening was available in five patients and identified 2xCFHR5,
2xMCP, and 1xADAMTS-13/THBD mutations. A total of 15 relapses were seen, of which 8 (72%) occurred in the 1–7
age group. The renal outcome was not significantly different regardless of age group.
Conclusion
In our cohort, we observed a relatively high frequency of extrarenal involvement at first presentation represented by
pulmonary manifestations. The renal prognosis at initial presentation was worse than in previous reports.
Descripción
Palabras clave
Pediatric Atypical hemolytic uremic syndrome, Thrombotic microangiopathy, Extrarenal manifestations