P73 | A rare case of atypical hemolytic uremic syndrome

P. Ghiringhelli, F. Macchi, A. Diana, M. Zaza, E. Pagliaro, L. Ferrari | Dipartimento di Scienze Mediche, ASST Valle Olona, Italy

Premises: Emergencies in Internal Medicine sometimes include very rare diseases.
Description of the Case report: A 36-year-old woman presents to the emergency department 15 days after a COVID 19 infection with predominantly abdominal symptoms. Bilateral low back pain had also been present, complicated by episodes of vomiting and hemorrhagic diarrhea. Later, declivous edema and profound asthenia appeared. On blood examinations PLT 131000, Hb 12, total protein 4.3, kalemia at lower limits. In Emergency room: flat and treatable abdomen, not tender or painful. Declivous and periorbital edema with fovea. Toracic RX and ECG were within limits. After 12 hours she was admitted in Internal Medicine. On blood chemistry normal PCR, Hb 10.5. PLT 78.000, total bilirubin 3.06 and direct 0.77, creatinine 2.8 mg/dl. Fragmented and helmeted red cells were present. Her brother had been treated at the age of 8 years for hemolytic uremic syndrome and had died. In suspicion of haemolytic uremic syndrome, steroid boluses were started. The following day she was transferred to the Nefrology (CROFF) of Milano, and was treated with eculizumab (anti C5 factor of complement), which allowed progressive clinical and laboratory improvement. Adams 13 was in the normal range and shiga toxin search negative. Genetic analysis was positive for a related gene: one received from the father and one from the mother.
Conclusions: Investigations allowed the diagnosis of Atypical Hemolytic Uremic Syndrome. The disease has an incidence of 0.5 cases per million population. Until recently, the prognosis was poor.