Sindromul hemolitic uremic tipic. Abordare diagnostică și terapeutică. Serie de cazuri

Haemolytic uremic syndrome ( HUS) is the most frequent and severe cause of acute kidney injury (AKI) in infants and children. The authors present an update in HUS with prodromal diarrhea, also called typical HUS, caused by Shiga-like toxins produced by enterohemorhagic, invasive, type of E. coli, EHEC- Stx or verotoxins. Typical HUS has a sudden onset, affecting children who were previously healthy. The severity of this syndrome is remarkable, being a life-threatening multisystemic disease. By definition, HUS consists of a triad of symptoms: haemolytic anaemia with fragmented erythrocytes, schizocytes, thrombocytopenia and AKI. All over the world there are outbreaks of STEC-HUS and unfortunately there is no specific therapy for D+ HUS. The mortality rates (between 5-10%) is constantly associated with extrarenal involvement of the disease, with multiple organ failure, and mainly central nervous system involvement (cerebral edema, vascular injuries, intracranial hypertension), followed by seizures and other neurological signs noted early in the course of the disease. Some other HUS major complications are also described: colonic strictures, intestinal perforations, intussuception, billiary lithiasis, pancreatitis, diabetes, cardiac, pulmonary and muscle impairment, hypertension and progressive chronic renal disease (CKD), or end stage renal disease (ESRD) in 4% of the cases who need dialytic therapy. Early diagnosis and supportive care are of major importance. In 10% of cases, CKD is diagnosed even after 15 years or more of follow-up, in 20-60% of the patients who manifested persistent proteinuria and/or hypertension. These problems may appear after several years of apparent recovery. This is why this pathology still remains a major cause of morbidity and mortality in children younger than five years old. The authors also present a series of 32 cases admitted in the Emergency Children’s Hospital ”Maria Sklodowska Curie”, Nephrology - Dialysis department, in the year 2016.