Unusual eye findings in a patient with atypical hemolytic uremic syndrome: Questions

Excerpt

A 4-year-old girl presented with abdominal pain and diarrhea that started about a week before her application. In her physical examination, there was periorbital edema, her skin color was pale, turgor decreased, and mucous membranes were dry. Liver was palpable. Complete blood count revealed hemoglobin (Hb) level of 7.7 g/dL, white blood cell count (WBC) of 23,300/mm³, and platelet count of 34,000/mm³. Peripheral blood smear showed features of microangiopathic hemolytic anemia with anisocytosis, poikilocytosis, and fragmented erythrocytes. Reticulocyte count was 2.8%. Dipstick urine analysis revealed density of 1014, 3 + protein; on microscopic analysis, microscopic hematuria was detected. Serum creatinine was 1.2 mg/dL, urea was 132 mg/dL, and liver enzymes were within the normal range. Lactate dehydrogenase (LDH) level was 2838 U/L (normal range 105–235), and haptoglobulin was 1 mg/dL. D-dimer test and Coombs test results were negative. Complement C3 was 0.78 g/L (normal range 0.82–1.85), and complement C4 was 0.18 (normal range 0.14–0.44). ADAMTS-13 activity was 112% (normal range 40–130). In her abdominal ultrasonography (USG), both kidney parenchyma echogenicities diffusely increased to grade 1. The patient was diagnosed with hemolytic uremic syndrome (HUS), and plasma exchange was performed, and hemodialysis was started due to anuria. On follow-up, she developed seizures and had heart failure. Together with low C3 levels and negative STEC test, the patient was considered to have atypical HUS (aHUS), so eculizumab was administered. Due to seizures, brain computerized tomography (CT) was performed, and a cortical focal hemorrhage area in the left posterior parietal region, a microangiopathic ischemic gliotic change in the left periventricular white matter, and a millimeter-diameter lesion in the left globe were detected. The family was questioned, and it was learned that the patient had said that she could not see far for the last few days, but she did not have any visual complaints before she was admitted to the hospital. …