CLINICAL SPECTRUM, COMPLICATIONS AND OUTCOME OF ATYPICAL HEMOLYTIC UREMIC SYNDROME

Abstract

Objective: To Determine the clinical spectrum, complications and outcome of atypical Hemolytic uremic Syndrome (aHUS).

Study Design: Case series study.

Place and Duration of Study: The Children’s Hospital & The Institute of Child Health Lahore, from Mar 2017 to Jun 2018.

Methodology: Twenty five Children fulfilled the inclusive and exclusive criteria of atypical hemolytic uremic syndrome. Their demographics, clinical characteristics, investigations and treatment modalities used, were docu-mented on the specially designed proforma.

Results: Out of twenty five cases, 15 (60%) were male and 10 (40%) were female with mean age of presentation being 8.47 ± 3.8 years. Seven children (28%) each with preceding history of respiratory tract infection (RTI) and family history of HUS. Twenty four (96%) children presented with anemia, hematuria in 15 (60%). Respiratory distress in 9 (36%) children and seizure in 7 (28%). Fragmented RBCs were present in all children, with throm-bocytopenia in 22 (88%). Plasma therapy in 23 (92%) patients and plasma exchange was offered to 2 (8%) while peritoneal dialysis was done in 18 (72%) and hemodialysis was continued in 10 (40%) subjects. Hypertension was persistent in both acute and chronic phase of illness.

Conclusion: Although not uncommon, atypical hemolytic uremic syndrome have variable presentation with high mortality rate. Therefore one should be vigilant in prompt diagnosis as early detection can improve outcome of disease.