Patient: Male, 31Final Diagnosis: Sickle cell intrahepatic cholestasisSymptoms: Abdominal pain • fever • jaundiceMedication: —Clinical Procedure: Exchange transfusionSpecialty: HematologyObjective:Rare diseaseBackground:Sickle cell disease can affect the liver by way of the disease process, including sickling in hepatic sinusoids, as well as its treatment, including repeated blood transfusions leading to hemosiderosis and hepatitis. Sickle cell intrahepatic cholestasis (SCIC) is an extreme variant of sickle cell hepatopathy, and is associated with high fatality.Case Report:We present the case of a 31-year-old man with past medical history of sickle cell disease and cholecystectomy who was admitted with uncomplicated vaso occlusive crisis and during the hospital stay developed fever, upper abdominal pain, and jaundice. There was an accelerated rise in total bilirubin to 50 mg/dL, direct bilirubin 38 mg/dL, and Cr 3.0 mg/dL. Hb was 6.4 g/dL, reticulocyte count 16%, ALT 40 IU/L, AST 155 IU/L, ALP 320 IU/L, and LDH 475 IU/L. Hepatitis panel was negative and MRCP showed normal caliber of the common bile duct, with no obstruction. Exchange transfusion of 9 units of packed red blood cells led to great improvement in his condition.Conclusions:SCIC, unlike the other sickle cell hepatopathies, requires urgent and vigorous exchange transfusion. Renal impairment in SCIC has not been well studied but usually is reversible with the hepatic impairment, as in this case. Unresolved renal impairment requires dialysis and is associated with poor outcome. There is limited data on use of hydroxyurea to prevent SCIC, and liver transplant is associated with high mortality. A timely diagnosis of SCIC and appropriate management is life-saving.

Patient: Female, 81Final Diagnosis: Liver abscesSymptoms: Diarrhea • jaundice • vomiting • weaknessMedication: —Clinical Procedure: CT scan guided drainageSpecialty: Gastroenterology and HepatologyObjective:Rare diseaseBackground:Clostridium perfringens is an unusual pathogen responsible for the development of a gas-forming pyogenic liver abscess. Progression to septicemia with this infection has amplified case fatality rates.Case Report:We report a case of an 81-year-old lady with pyogenic liver abscess with gas formation that was preceded by an acute gastroenteritis. The most common precipitating factors are invasive procedures and immunosuppression. Clostridium perfringens was unexpectedly isolated in the drained abscess, as well as blood. It is a normal inhabitant of the human bowel and a common cause of food poisoning, notoriously leading to tissue necrosis and gas gangrene.Conclusions:We report a case of gas-forming pyogenic liver abscess and bacteremia progressing to fatal septic shock, caused by an uncommon Clostridium perfringens isolate.

Staphylococcus schleiferi subsp. schleiferi is a coagulase-negative Staphylococcus which has been described as a pathogen responsible for various nosocomial infections including bacteremia, brain abscess, and infection of intravenous pacemakers. Recently, such bacteria have been described to be found typically on skin and mucosal surfaces. It is also believed to be a part of the preaxillary human flora and more frequently found in men. It is very similar in its pathogenicity with Staphylococcus aureus group and expresses a fibronectin binding protein. Literature on this pathogen reveals that it commonly causes otitis among dogs because of its location in the auditory meatus of canines. Also, it has strong association with pyoderma in dogs. The prime concern with this organism is the antibiotic resistance and relapse even after appropriate treatment. Very rarely, if any, cases have been reported about prostatic abscess (PA) with this microbe. Our patient had a history of recurrent UTIs and subsequent PA resulting in S. schleiferi bacteremia in contrast to gram negative bacteremia commonly associated with UTI. This organism was found to be resistant to methicillin, in spite of being negative for PBP2, which is a rare phenomenon and needs further studies.

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