Atypical haemolytic uremic syndrome with posterior reversible encephalopathy syndrome in an adolescent: a rare case report

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a group of disorders that affect kidneys which is rare type of HUS that differs from classical hemolytic uremic syndrome (HUS) by absence of prodromal phase consisting of episodes of diarrhoea due to preceding shiga toxin E. coli (STEC-HUS) infection and is 5% of all HUS cases. Approximately 50% cases present with clinical triad of hemolytic anemia, thrombocytopenia and renal insufficiency. However, it can have unusual clinical features in form of central nervous system involvement. This case, of a 15-year-old Indian boy, is one such rare presentation of atypical haemolytic uremic syndrome associated with posterior reversible encephalopathy syndrome (PRES), or reversible posterior leukoencephalopathy syndrome (RPLS) who presented with anaemia, anasarca, papilledema, hypertension, episodic seizures and significant magnetic resonance imaging (MRI) brain findings. We report this uncommon combination of two syndromes to provide useful insight for clinicians to approach and diagnose such presentation in paediatric patients.

Hemolytic Uremic Syndrome: A Case Report

“…Laboratory tests showed: hemoglobin (Hb) 8.5 g/dL, platelet count 55x10 3 /uL, white blood cell (WBC) count 7700/cumm, blood urea 195 mg/dL, blood creatinine 5.9 mg/dl lactate dehydrogenase (LDH) 3289 U/L, peripheral blood smear showed predominantly normocytic normochromic cell admixed with few polychromatophils and schistocytes. Urinalysis revealed haematuria (plenty of RBC) and proteinuria (albumin +++).…”

“… 2 A triad of renal insufficiency, thrombocytopenia, and hemolytic anemia affects about 50% of cases. 3 E. coli 0157:H7-related acute gastroenteritis is regularly linked to HUS, which typically manifests after prodromal bloody diarrhea. 4 We present a case of a 12-year-old female with a history of microangiopathic hemolytic crisis, abdominal pain, and diarrhea.…”