Evaluation of Patients with Hemolytic Uremic Syndrome

Abstract

Introduction: This study investigated the relationship between age, gender, initial laboratory findings, treatment modalities, and prognosis in pediatric patients diagnosed with hemolytic uremic syndrome (HUS) at Necmettin Erbakan University Meram Medical Faculty Pediatric Nephrology Department. Materials and Methods: A retrospective analysis was conducted on 30 patients under 18 years of age who presented to the outpatient clinic between 2009 and 2020. Patient data, including demographics, clinical presentation, prodromal period, laboratory values at presentation, treatment strategies, and follow-up outcomes, were extracted from medical records. Results: The cohort comprised 17 females (56.7%) and 13 males (43.3%). Nineteen patients (63.3%) presented with typical HUS, while 11 (36.7%) had atypical HUS. The mean age at presentation was 3.63 ± 3.69 years. The mean duration between symptom onset and hospital admission was 6.33 ± 3.95 days. The most frequent presenting symptoms were diarrhea (63.4%), bloody diarrhea (26.7%), and gross hematuria (20%). Hypertension was observed in 73.3% of the patients. During the course of the disease, 10% developed chronic renal failure, and 6.6% experienced recurrence. Anuria occurred in 56.6% of the patients. All patients exhibited proteinuria, with 93.3% demonstrating nephrotic-range proteinuria. Hypoalbuminemia was observed in all patients with nephrotic-range proteinuria. Hematuria was universally present, with 20% exhibiting gross hematuria. Eculizumab was administered to 33.3% of the patients, with 13.3% receiving regular treatment. Persistent proteinuria was noted in 13.3% despite treatment, and these patients remain under clinical observation with stable medication. Dialysis was required in 60% of the cases, with peritoneal dialysis employed in 36.6% and hemodialysis in 23.3%. Fresh frozen plasma was administered in 53.3% of the cases, with a higher proportion in atypical HUS (91%) compared to typical HUS (36.8%). Conclusion: HUS is a prevalent thrombotic microangiopathy in children. Initial laboratory parameters can provide valuable insights into disease progression. Prolonged hospitalization was associated with anuria exceeding one day and the need for dialysis. Among the key diagnostic laboratory markers, platelet count and urea levels normalized earliest. Eculizumab demonstrated efficacy in atypical HUS cases. No significant association was found between other treatment modalities and prognosis.

Keywords

• hemolytic uremic syndrome
• acute renal failure
• thrombocytopenia
• eculizumab