Shaffer Mok

Vanishing bile duct syndrome as a manifestation of Hodgkin's lymphoma: a case report and review of the literature.

Vanishing bile duct syndrome (VBDS) is characterized by cholestasis and progressive destruction of the intrahepatic bile ducts (ductopenia). The current definition of ductopenia is the loss of interlobular bile ducts in more than 50% of portal tracts. Ductopenia is believed, at a molecular level, to result from the misbalance in cell regeneration and apoptosis. In the literature various etiologies have been reported to cause ductopenia, with Hodgkins lymphoma (HL) being listed as a rare example. How HL causes ductopenia remains ambiguous, and seems to be related to a paraneoplastic phenomenon causing cytokine release from lymphoma cells, not tumor infiltration or obstructive lymphadenopathy. VBDS is generally considered irreversible, unlike its histopathological counterpart, idiopathic cholestasis, where ductopenia is not present and liver function improves with therapy. Therefore, a distinction between the two is warranted. There have been only 19 case reports in the English literature associating VBDS with HL. Here we report a 64-year-old female patient who presented with distributive shock and jaundice. Initial laboratory values revealed leukocytosis, mild transaminase elevation with significantly elevated alkaline phosphatase, along with direct hyperbilirubinemia. During hospital stay, the patients liver function progressively worsened. Further workup did not reveal ductal dilation or obstruction and there were unremarkable results for infectious and autoimmune etiologies. Imaging studies with biopsy revealed extensive lymphadenopathy consistent with HL; liver biopsy showed cholestasis and ductopenia. Despite chemotherapy the patient succumbed to progressive liver failure and sepsis.

Chicken Bone Impaction Diagnosed by Computer Tomography Angiography: A Rare Cause of Lower Gastrointestinal Bleeding.

A 71-year-old man presented with hematochezia and paraumbilical abdominal pain. The patient had a history of left hemicolectomy for unresectable large colonic polyps, but he had no prior episodes of gastrointestinal bleeding and was not on anticoagulants, antiplatelet therapy, or non-steroidal anti-inflammatory drugs. Upon arrival, the patient was hemodynamically stable with a normal complete blood count and international normalized ratio (INR). A computerized tomography angiogram (CTA) utilizing a “bleeding scan protocol” was performed. The radiologist identified a foreign body in the proximal sigmoid colon with adjacent inflammatory changes, with structural characteristics suggesting a chicken gracile bone (Figure 1). The radiologist felt that the location of the gracile bone was the likely source of gastrointestinal hemorrhage due to visible extravasation of contrast. The patient underwent colonoscopy, which demonstrated an impacted gracile bone not adjacent to any diverticula at 25 cm from the anal verge, which was removed via snare (Figure 2). No residual bleeding was detected on careful inspection of the colon after extraction of the bone. Although gracile bone impaction has been described in the past as a cause of upper gastrointestinal bleeding, only 1 case report described lower gastrointestinal bleeding from a chicken bone.1-4 Vosskamp et al described a case in which the patient underwent a hemicolectomy for lower gastrointestinal bleeding that showed a submucosal gracile bone adjacent to a bleeding diverticulum.4 Our case report exemplifies a rare etiology of lower gastrointestinal bleeding caused by acute gracile bone impaction in the sigmoid colon.

A genetic database can be utilized to identify potential biomarkers for biphenotypic hepatocellular carcinoma-cholangiocarcinoma

BACKGROUND Biphenotypic hepatocellular carcinoma-cholangiocarcinoma (HCC-CC) is an uncommon primary liver neoplasm. Due to limitations in radiologic imaging for the diagnosis of this condition, biopsy is a common method for diagnosis, which is invasive and holds potential complications. To identify alternative means for obtaining the diagnosis and assessing the prognosis of this condition, we evaluated biomarkers for biphenotypic HCC-CC using a genetic database. METHODS To evaluate the genetic associations with each variable we utilized GeneCards(®), The Human Gene Compendium (http://www.genecards.org). The results of our search were entered into the Pathway Interaction Database from the National Cancer Institute (PID-NCI) (http://pid.nci.nih.gov), to generate a biomolecule interaction map. RESULTS The results of our query yielded 690 genes for HCC, 98 genes for CC and 50 genes for HCC-CC. Genes depicted in this analysis demonstrate the role of hormonal regulation, embryonic development, cell surface adhesion, cytokeratin stability, mucin production, metalloproteinase regulation, Ras signaling, metabolism and apoptosis. Examples of previously described markers included hepatocyte growth factor (HGF), mesenchymal epithelial transition (MET) and Kirsten rat sarcoma viral oncogene homolog (KRAS). Novel markers included phosphatidylinositol-4,5-bisphosphate 3-kinase, catalytic subunit alpha (PIK3CA), GPC3, choline kinase alpha (CHKA), prostaglandin-endoperoxide synthase 2 (PTGS2), telomerase reverse transcriptase (TERT), myeloid cell leukemia 1 (MCL1) and N-acetyltransferase 2 (NAT2). CONCLUSIONS GeneCards is a useful research tool in the genetic analysis of low frequency malignancies. Utilizing this tool we identified several biomarkers are methods for diagnosing HCC-CC. Finally, utilizing these methods, HCC-CC was found to be predominantly a subtype of CC.

The NT-ProBNP Test in Subjects with End-Stage Renal Disease on Hemodialysis Presenting with Acute Dyspnea: Is Knowing Worth the Cost?

Background. The NT-ProBNP/BNP test has been validated as a marker for determining the etiology of acute dyspnea. In the setting of end-stage renal disease on hemodialysis (ESRD on HD), the utility of the NT-ProBNP/BNP test has not been validated. This study examines the clinical utility of the NT-ProBNP test in the setting of ESRD on HD patients presenting with acute dyspnea. Methods. A retrospective case series of 250 subjects were admitted to Cooper University Hospital, 07/2010-03/2011, with ESRD and HD presenting with dyspnea. The incidences of echocardiography, cardiology consultation, and NT-ProBNP elevated and normal were examined. Correlation coefficients were calculated for NT-ProBNP with age (years), estimated dry weight (kg), amount of fluid removed (L), and ejection fraction (EF in %) among other echocardiography parameters. Results. Of the total sample 235 patients had NT-ProBNP levels performed. Cardiology consults were placed in 68.8% and 58% who underwent echocardiography. Of those for whom an echocardiography was performed estimated mean EFs of 54.6%, 50.8%, and 61.7% were observed among the NT-ProBNP elevated group, normal group, and no NT-ProBNP group, respectively. No differences were detected in all other echocardiography measurements. No correlation was observed between NT-ProBNP and age (r = 0.05), baseline EDW (r = −0.09), amount of fluid removed (r = 0.07), or EF (r = 0.02). Conclusion. In the setting of ESRD on HD, the NT-ProBNP test has no clinical utility in determining the etiology of acute dyspnea. This can be demonstrated through echocardiographic and therapeutic parameters measured in this study.