Clinical Pediatrics 1–3 © The Author(s) 2015 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922815580406 cpj.sagepub.com Brief Report Introduction Streptococcus pneumoniae (Sp)–associated hemolytic and uremic syndrome (HUS) accounts for 5% to 15% of all HUS with an incidence of 0.4% to 0.6% 1 following Sp infections. Epidemiology of invasive pneumococcal dis- ease changed since the introduction of antipneumococcal conjugated vaccine and SpHUS appeared to follow the serotype shift. 2 Presently, SpHUS seems to be mostly due to the serotype 3, 6B, 7, 8, 9V, 14, 19, and 23F. 2,3 Renal complication of SpHUS is well known, and 10% to 16% of cases will develop an end-stage renal failure (ESRF). 1 However, hepatic complications are rare and uncommon. Here, we describe a child with SpHUS associated with severe cholestatic jaundice secondary to acute liver injury. Case Report A 2½-year-old female child with fever and cough was admitted to our institution for bilateral pneumonia. Her medical history revealed 2 pyelonephritis (at 10 and 15 months) and correct vaccination, including complete anti- pneumococcal vaccination with Prevenar 13 (3 doses). At admission, blood tests revealed anemia (6.9 g/dL) with schistocytes (25‰), thrombocytopenia (68 g/L), renal insufficiency (serum creatinine [SCreat] 2.9 mg/dL), blood urea nitrogen (BUN) 23.5 mmol/L), indicating HUS. A day later, she developed jaundice and anuria and blood tests revealed the presence of elevated liver enzymes (alanine transaminase [ALT] 128 U/L, aspartate transami- nase [AST] 292 U/L) associated with conjugated hyper- bilirubinemia (21.4 mg/dL, normal value 0-0.6 mg/dL). There was no neurological involvement (Glasgow Coma Scale 15/15). Intravenous antibiotic (cephalosporin third generation) and peritoneal dialysis was initiated and the child was transferred to the pediatric intensive care unit for mechanical ventilation. Thoracic and abdominal com- puted tomography showed bilateral pneumonia, normal liver appearance except for some periportal edema and stoneless gallbladder. Blood cultures were positive for serotype 3 Streptococcus pneumoniae and T-Ag activation was positive in the urine. In the pediatric intensive care unit, SCreat peaked at 3 mg/dL together with an important hemolysis (Hb 5.9 g/dL, schistocytes 30 ‰, direct Coomb’s test was negative, haptoglobin less than 10 mg/L and lactate dehydrogenase (LDH) increased to 2537 U/L. The child showed a favorable evolution recovering progressively her diuresis after 2 days, and peritoneal catheter was removed after 6 days. After a peak at 333 and 128 U/L of AST and ALT, respectively, values were quickly normalized. Total serum bilirubin was maximal at 26.3 mg/dL (normal value 0-1.2 mg/dL) and conju- gated bilirubin was maximal at 21.5 mg/dL, which prompted us to introduce ursodeoxycholic acid. She also normalized her complete blood count, cholestasis, and renal function tests within 2 weeks, and was discharged home after 3 weeks with oral antibiotic and angiotensin- converting enzyme inhibitor treatment for residual arte- rial hypertension, which was stopped after 6 months. After follow-up of 12 months, the child showed a nor- mal physical examination without arterial hypertension, no proteinuria, normal renal function, normal complete blood count, and normal liver function tests. Discussion Hemolytic and uremic syndrome is a rare but increasing complication of Sp infection with higher morbidity than usual shiga-like toxin–producing Escherichia coli (STEC)-HUS. Increased unconjugated bilirubin and serum transaminase levels were described and attributed 580406CPJ XX X 10.1177/0009922815580406Clinical PediatricsAnastaze Stelle et al research-article 2015 1 Lausanne University Hospital, Lausanne, Switzerland Corresponding Author: Hassib Chehade, Department of Pediatrics; Division of Pediatric Nephrology, Lausanne University Hospital, Rue Bugnon 46, 1011 Lausanne, Switzerland. Email: hassib.chehade@chuv.ch Streptococcus pneumoniae–Associated Hemolytic and Uremic Syndrome With Cholestasis: A Case Report and Brief Literature Review Karine Anastaze Stelle, MD 1 , Francois Cachat, MD 1 , Marie-Helene Perez, MD 1 , and Hassib Chehade, MD 1 at Bibliothèques CHUV - Unil FBM on April 20, 2015 cpj.sagepub.com Downloaded from