Faculty Bibliography

Background Abnormal elevation in serum lipids is extremely common. Hypertriglyceridemia may be primary or a secondary disorder related to alcohol abuse, diabetes, and even pregnancy. If severe, hypertriglyceridemia can have serious medical consequences including the potentially life-threatening complication of acute pancreatitis. We present a case of a pregnant woman presenting with severe hypertriglyceridemia and acute pancreatitis eventually suffering fetal distress requiring emergent caesarean section and the use of plasmapheresis when conservative treatments failed to significantly reduce her triglyceride levels. Case Presentation 29-year-old woman G1P0 at 32 weeks gestation with a past medical history of HBV and hyperlipidemia presented with sharp right upper quadrant abdominal pain, nausea and vomiting for one day. She began having contractions, developed vaginal bleeding, and was taken for primary cesarean section due to placenta abruptia. Copious amounts of whitish, malodorous fluid was observed in the abdomen during the procedure. The patient was found to have acute pancreatitis with an initial amylase level of 251 and a lipase level of 736, peaking on the second day of admission to lipase-1625 and amylase-1499. CT scan of the abdomen and pelvis revealed severe pancreatitis with an enlarged, edematous pancreas with extensive peripancreatic inflammatory. Triglyceride levels were not measurable and the blood was noted to be grossly lipemic. The patient was started on fenofibrate, antibiotic therapy, and aggressive calcium replacement. On day 3 of the hospital stay, after no improvement in clinical status or triglyceride levels, the patient received plasmapheresis. On day 4 of the hospital stay the triglyceride level was detectable at 313. Amylase and lipase levels returned to normal levels after 1 week and the triglycerides remained between 313 and 685 during the course of the hospital stay. Conclusion Although experience with plasmapheresis in the above setting is limited, it has been shown to be a rapid and effective intervention for the emergent management of severe hypertriglyceridemia induced pancreatitis reducing triglyceride levels significantly within hours as was shown in our case. Plasmapheresis should be considered early in the course of treatment when severe hypertriglyceridemia is the cause of acute pancreatitis. Multiple sessions may be needed and the patient should be followed closely both clinically and biochemically

Introduction: Mediastinal histoplasmosis present as mediastinal adenitis, granulomas or fibrosing mediastinitis. Whether these are different faces of a continuous spectrum of disease is not yet confirmed. Below is a case of mediastinal granulomas with evolving fibrosis. An extensive literature review from 1951 to 2011, revealing over 200 cases will also be detailed. Case Presentation: A 19 year old man non-smoker presented with dyspnea, night sweats, fever and non-productive cough. Paternal history was significant for lymphomas. His lungs were clear and there were no palpable lymphadenopathies. CXR revealed a right mediastinal mass/adenopathy. Chest CT showed extensive calcified mediastinal adenopathies, soft tissue densities encircling the right main bronchus and air space disease of the RML. His PPD, HIV test and sputum AFB smears and cultures were all negative. Inspection bronchoscopy showed bulging of the main carina and narrowing of the right main bronchus. EBUS lymph node sampling as well as BAL were negative for viral, bacterial, fungal, AFB cultures and malignancies. Mediastinoscopy with biopsy showed fibrotic lymphoid tissue with areas of caseating necrotizing granulomas, fibrosis and scattered calcifications. Therapy was initiated with itraconazole. Discussion: Primary histoplasmosis is usually a self-limited respiratory infection. Coalescence of caseous mediastinal lymph nodes results in mediastinal granulomas. These presents with cough, chest pain, dysphagia, hemoptysis, and dyspnea. Compression of the SVC, esophagus, or airways, and disruption of the capsule creating fistulas may all be seen. Cultures are usually negative, as are special stains for organisms. This was the case with our patient. Imaging reveals heavy calcifications, the presence of which may obviate the need for biopsy for tissue diagnosis as per some authorities. Urinary antigen and blood or bone marrow cultures are most useful in AIDS patients with disseminated diseases. Skin tests and serologies are not useful diagnostically because of high background rates of positivity in endemic areas. Differential diagnosis includes tuberculosis, other fungal infections, lymphomas post-radiation, metastases especially mucinous adenocarcinoma, sarcoidosis, and silicosis. Most authorities believe that neither antifungal nor anti-inflammatory treatment ameliorates the outcome of fibrosing mediastinitis. Our patient had both spectrum of diseases with mediastinal fibrosis revealed by the soft tissue densities encircling the right main bronchus. This explains his presentation with a post-obstructive pneumonia. Our case brings more questions than answers. What are the optimal tests to confirm histoplasmosis? Does treatment with anti-fungals prevent the mediastinal granulomas from evolving into the more devastating late complication of mediastinal fibrosis? (Figure Presented)

Osteoarthritis (OA) is a slowly progressive degenerative joint disease that is associated with joint space narrowing, osteophyte formation and subchondral sclerosis. Despite extensive effort actual breakthroughs in the field of genetic or biochemical biomarkers of OA are limited. As secretory apolipoprotein J/clusterin (sCLU) has been implicated in both inflammatory and apoptotic molecular processes which contribute to the OA phenotype, the sCLU concentration in human serum and synovial fluid during advanced primary knee and hip OA was analysed. Elevated sCLU protein levels were shown in these two biological fluids. sCLU mRNA expression was also studied in normal cartilage and in advanced primary knee and hip OA samples. A significant up-regulation of sCLU mRNA expression (~25-fold) was found in samples collected from the tibial bone that was osteotomized during total knee arthroplasty in patients with primary knee OA, as compared to healthy tissue samples collected from the femoral head of macroscopically normal cartilage during the surgical treatment of subcapital fractures. By studying sCLU mRNA expression levels in samples collected during total hip arthroplasty in patients with advanced primary hip OA, an additional up-regulation of the sCLU mRNA expression (~4-fold), as compared to advanced primary knee OA, was found. Taken together, these observations indicate that the sCLU protein or mRNA expression level may be of a significant diagnostic and/or prognostic value during OA progression.

BACKGROUND/AIMS: The pathogenetic mechanisms of development of non-alcoholic steatohepatitis (NASH) and fibrosis are not clear, although thrombosis of small intrahepatic veins has been suggested to trigger liver tissue remodelling and thrombotic risk factors have been associated with more advanced fibrosis in chronic viral hepatitis (CVH). We evaluated the prevalence of thrombotic risk factors (RFs) in non-alcoholic fatty liver disease (NAFLD) and their possible association with fatty liver or NASH. METHODS: We included 60 patients with histologically documented NAFLD and a historical cohort of 90 patients with chronic hepatitis B (n=39) or C (n=51). Thrombophilic factors were evaluated on the day of the liver biopsy. RESULTS: One or more thrombotic RFs were detected in 37% of NAFLD patients, and >or= 2 RFs were detected in 12% of NAFLD patients, being less frequently present than in CVH patients (37% and 68%, respectively; P

PMID: 19726097

ISSN: 1600-0641

CID: 1893852