TY - JOUR AU - Ahmed, Junaid AU - Hafeez, Farkhanda AU - Khan, Fauzia Shafi AU - Akhtar, Naureen PY - 2020/01/27 Y2 - 2024/03/29 TI - CLINICAL SPECTRUM, COMPLICATIONS AND OUTCOME OF ATYPICAL HEMOLYTIC UREMIC SYNDROME JF - Pakistan Armed Forces Medical Journal JA - PAFMJ VL - 70 IS - Suppl-1 SE - Original Articles DO - UR - https://www.pafmj.org/PAFMJ/article/view/3818 SP - S176-81 AB - <p><strong>Objective:</strong> To Determine the clinical spectrum, complications and outcome of atypical Hemolytic uremic Syndrome (aHUS).</p><p><strong>Study Design:</strong> Case series study.</p><p><strong> Place and Duration of Study:</strong> The Children’s Hospital &amp; The Institute of Child Health Lahore, from Mar 2017 to Jun 2018.</p><p><strong>Methodology:</strong> 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.</p><p><strong>Results:</strong> 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.</p><p><strong>Conclusion:</strong> 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.</p> ER -