No difference in symptomatology was discernible between boys and girls. Podocyturia was detectable in children serving as a possible early marker of kidney injury. LysoGb3 was elevated in all cases, emphasizing the importance for diagnosis especially in female patients with normal αGal A activity. A possible association between lysoGb3 and symptom severity and histological involvement in kidney biopsy should be assessed in prospective studies with enough statistical power to determine if lysoGb3 can be used to predict nephropathy in children with Fabry disease.
The enrolment of Argentinean patients into the Fabry Registry has steadily increased, as has the inclusion of female and paediatric patients with FD. The medical community in Argentina should be aware of FD in these populations, as awareness will facilitate prompt diagnosis and initiation of treatment, thus leading to improved outcomes.
Introduction. Renal involvement among pediatric patients with coronavirus disease 2019 ranges between 1.2% and 44%. Given the limited information available locally, the primary objective of this study was to estimate the prevalence of renal involvement in our setting. Population and methods. Cross-sectional study conducted in 13 Argentine sites between March and December 2020. Patients aged 1 month to 18 years hospitalized due to COVID-19 and with at least one measurement of serum creatinine and/or a urinalysis were included. Those with a known kidney disease were excluded. Renal involvement was defined as the presence of acute kidney injury (AKI), proteinuria, hematuria, leukocyturia and/or arterial hypertension (HTN). Results. Among 528 eligible medical records, 423 patients were included (55.0% were males; median age: 5.3 years). The clinical presentation was asymptomatic in 31%; mild, in 39.7%; moderate, in 23.9%; severe, in 1.2%; critical, in 0.7%; and 3.5% had multisystem inflammatory syndrome in children (MIS-C). Two patients (0.47%) died. The prevalence of renal involvement was 10.8% (95% confidence interval: 8.2-14.2); it was described as leukocyturia (16.9%), proteinuria (16.0%), hematuria (13.2%), HTN (3.7%), and AKI (2.3%). No patient required dialysis. Renal involvement was associated with severe forms of disease (p < 0.0001). Conclusion. The prevalence of renal involvement among pediatric patients hospitalized due to COVID-19 in 13 Argentine sites was 10.8%; severe forms of disease prevailed.
Presentación de casos clínicos RESUMENEl síndrome de lisis tumoral es una complicación potencialmente letal y constituye, junto con las infecciones, la emergencia oncológica más frecuente. En pediatría, este cuadro puede ser secundario a enfermedades neoplásicas, y los corticoides son un factor desencadenante. En este trabajo se presenta el caso de una paciente adolescente, sin neoplasias conocidas o evidentes, que desarrolló un síndrome de lisis tumoral luego de la administración de corticoides por sospecha de una infección respiratoria. Se discute la forma de presentación y los diagnósticos diferenciales del cuadro clínico inicial. Se hace especial foco en la administración de corticoides en cuadros clínicos en los que no existe evidencia científica que respalde fuertemente su indicación. El uso de corticosteroides sistémicos en infecciones respiratorias agudas debe ser evaluado en el contexto clínico y solo debe indicarse en situaciones con probada efectividad. Palabras clave: síndrome de lisis tumoral, corticosteroides, niño, pediatría.
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