Osteopetrosis is a genetic disorder of bone remodeling caused by osteoclast dysfunction. Clinical features include short stature, frequent fractures, and recurrent infections. Abnormal bone obliterates the marrow cavity, resulting pancytopenia and extramedullary hematopoiesis in the liver and spleen. The splenomegaly can lead to left-sided portal hypertension. We report the second case of osteopetrosis-induced portal hypertension and the first case of upper gastrointestinal bleeding in a 52-year-old woman with osteopetrosis.

Introduction: Blue rubber bleb nevus (BRBN) syndrome is a rare vascular malformation syndrome of mostly cutaneous and gastrointestinal (GI) mucosal venous malformations. The clinical literature for BRBN syndrome is limited almost exclusively to case reports, and about 220 cases have been reported as of 2014. Occult GI bleeding (GIB) and iron deficiency anemia are the most common presentations; melena and hematochezia have also been reported. BRBN syndrome usually develops in childhood, but there are accounts with delayed onset. Lesions typically consist of discrete mucosal nodules with a central bluish nipple. An 80-year-old man presented for evaluation of GIB. He was diagnosed with T-cell lymphoma 20 years ago, which responded to CHOP chemotherapy. His course was complicated by transformation to diffuse large B-cell lymphoma and multiple disease recurrences. His treatment consisted of rituximab and CEPP regimen, gemcitabine with oxaliplatin plus rituximab, radiation therapy, and then maintenance rituximab every 2 months. He also developed non-ischemic cardiomyopathy attributed to doxorubicin. Two months prior to presentation, he was diagnosed with nonvalvular atrial fibrillation, and rivaroxaban was initiated for CHADS2 score of 2. One month after anticoagulation was started, the patient presented with a 2-week history of dyspnea and melena. Physical exam was absent of cutaneous BRBN, and hemoglobin was 8.7 g/dL (baseline 14 g/dL). Rivaroxaban was held and blood transfusions were given. EGD revealed non-bleeding BRBN in the second part of the duodenum, and colonoscopy (Figure 1A) revealed melena and non-bleeding BRBN throughout the colon and terminal ileum. Capsule endoscopy (Figure 1B) visualized red blood and BRBN in the small bowel. Within 2 days, the hemoglobin stabilized and melena resolved. Rivaroxaban was discontinued after multidisciplinary discussion. He is asymptomatic at 1 month’s follow-up. To our knowledge, this is the first reported case of initial GIB from BRBN syndrome occurring at an advanced age (greater than 70). Most reported cases are of pediatric patients and adults less than 45 years of age. Association between malignancy and BRBN syndrome is reported with 1 case of osteoid osteoma and 1 of leukemia. This is the first reported association of BRBN syndrome with non-Hodgkin lymphoma. BRBN syndrome should be considered even in older patients presenting with GIB.Figure 1: (A) BRBN on colonoscopy. (B) Red blood and BRBN on capsule endoscopy.

Introduction: Osteopetrosis (OP) is a rare genetic disorder of abnormal bony architecture that obliterates the marrow cavity, resulting in bone marrow failure. Extramedullary hematopoiesis occurs in the spleen and can lead to left-sided portal hypertension. We present a rare case of OP induced portal hypertension manifesting as an upper GI bleed. Case Report: A 52-year-old woman with OP complicated by recurrent fractures and transfusion dependent pancytopenia was admitted for coffee ground emesis. She had no history of PUD, NSAID or alcohol use. Her exam revealed hypotension, tachycardia and a palpable spleen tip in the left lower quadrant with a normal liver span. Hemoglobin was 5.0 g/dL and platelet count was 39,000/uL. Liver enzymes and INR were normal. EGD revealed grade 3 non-bleeding esophageal varices and severe portal gastropathy. Workup was negative for chronic hepatitis, iron overload and portal system thrombi. CT abdomen revealed marked splenomegaly measuring 23cm longitudinally extending into left iliac fossa, abdominal ascites and normal sized liver. The GI bleed resolved with octreotide and pantoprazole. A diuretic was started for ascites along with nadolol for esophageal varices. Discussion: Our case highlights the rare occurrence of left-sided portal hypertension in a patient with OP. Left sided portal hypertension occurs when splenic venous outflow increases to a level greater than hepatic capacity. Elevated pressures in the splenic vasculature causes dilatation of collateral vessels leading to variceal formation. We hypothesize that our patient had left-sided portal hypertension from extramedullary hematopoiesis in the spleen. Her spleen was the major reservoir for erythropoiesis due to bone marrow failure from OP, but the excessive blood flow through the spleen overwhelmed the liver’s capacity, resulting in varices and eventual rupture. To our knowledge, there has only been one case report of OP manifesting as portal hypertension in 1971. Our case is the second reported case of portal hypertension from OP and the first presenting as GI bleeding.Figure 1: CT abdomen/pelvis with marked splenomegaly with the spleen measuring 23 cm longitudinally.

Gemcitabine induces apoptosis during DNA replication and has been used as a chemotherapeutic agent in various types of cancer. This drug is generally considered to be well-tolerated with mild common side effects including gastrointestinal disturbance, flu-like symptoms and rash. Myelosuppression and abnormalities of liver enzymes, usually mild, transient and reversible, are frequently reported. Gemcitabine has rarely been identified as a cause of acute liver failure (only four cases of cholestatic hepatotoxicity have been reported). This case involves a patient with acute cholestatic liver failure after adjuvant treatment with gemcitabine for pancreatic adenocarcinoma.

(2006). Acquired tracheoesophageal fistula after prolonged mechanical ventilation. Clinical Intensive Care: Vol. 17, No. 1-2, pp. 51-54.

s Submitted for the 68th Annual Scientific Meeting of the American College of Gastroenterology October 10-15, 2003, Baltimore, Maryland: PANCREATIC/BILIARY: PDF Only

Purpose: Although maintenance PPI's are empirically advocated in Barrett's esophagus (BE), there is only one study, from this institution, showing a trend in reduction of CA and HGD after 5–11 yrs of maintenance PPI therapy. The aim of this study is to examine whether this trend has been sustained after 6–13 yrs.

s Submitted for the 68th Annual Scientific Meeting of the American College of Gastroenterology October 10-15, 2003, Baltimore, Maryland: CLINICAL VIGNETTES: PDF Only

BACKGROUND:The mortality associated with acute pancreatitis varies markedly in different studies, with most frequently reported mortality rates of 10% to 15% for all cases and 15% to 90% for attacks regarded as "severe." More recently, various centers have recorded lower mortality rates of 4% to 7% for all attacks of acute pancreatitis and 20% to 50% for those regarded as severe. GOALS:To investigate whether there has been a reduction in mortality associated with acute pancreatitis over the past 20 years and the reasons for this reduction. STUDY:Intended as a review, this study included the authors' 20-year prospective assessment of mortality as it relates to the severity of the disease, complications, and current therapy. For the mortality results, the study was divided into four 4-year periods from 1977 to 1998 and the past 3 years (i.e., 1998-2001). For comparison, the mortality figures from some other large studies are presented. RESULTS:This study showed that the initial reduction in mortality related to acute pancreatitis coincided with the recognition and application of the signs of severity, either Ranson's prognostic signs or Bank's clinical criteria. These signs dictated admission to intensive care unit (ICU) therapy, the intensity of ICU monitoring, and the importance of organ-specific emergent therapy. Further mortality reduction in the 1990s could be attributed to either a more select study sample or earlier and more selective endoscopic or surgical debridement of infected tissue, endoscopic cyst drainage, and angiographic control of gastrointestinal bleeding. Improved nutritional support by jejunal feeding, earlier use of antibiotic therapy, gut sterilization, early endoscopic retrograde cholangiopancreatography for common bile duct stones and necrosectomy for noninfected necrosis have reduced the overall mortality associated with acute pancreatitis to a mean of 5% (range, 3.8-7%) for all cases and 20% (range, 15-25%) for severe cases. However, it is clear that the greater the number of signs denoting severity of organ failure, the higher the mortality. CONCLUSIONS:There has been considerable reduction in the mortality associated with acute pancreatitis over the past 20 years. The reasons are multifactorial, but recognition of severity signs, early implementation of organ-specific therapy, and newer endoscopic, surgical, and angiographic therapy for infection cyst and bleeding appear to have been the major factors in reducing mortality.

Background: Percutaneous endoscopic gastrostomy (PEG) is a well accepted mean of achieving enteral nutrition in patients who have lost the ability to take oral feeds.In the literature there have been case reports noting technical difficulty and high risk of complication of PEG in patients who have had a gastrectomy.It has been suggested that radiological assisted gastrostomy or a formal surgical gastrostomy is the preferred approach in this group of patients.Objective: To study the safety and efficacy of PEG placement in patients with gastrectomy.Method: Non-concurrent, cohort study was carried out comprising of 45 patients admitted during 1995 to 1999.Study group comprised of 15 patients with gastrectomy (Billroth II-13; Billroth I-2).Control group comprised of 30 patients matched for age and underlying medical conditions.Mean duration of follow up was 8 weeks (range 1 week to 2 years).Outcome comprised of technical difficulty in doing the procedure, procedure related complications and tolerance to PEG feeds.Technical difficulty was defined on the basis of time taken to complete the procedure.Result: Eight of the gastrectomy patients had a PEG placed successfully and seven patients had a percutaneous endoscopic jejunostomy.No technical complication was noticed in both the study group or the control group.Incidence of pneumonia was higher in patients with gastrectomy as compared to intact stomach, however it did not reach statistical significance.Feed tolerance was similar in both the groups.One patient was lost to follow up.Conclusion: PEG placement in patients with partial gastrectomy is a technically simple, safe and effective procedure.High risk of pneumonia in this group of patients may be related to relative proximal peg tube placement and small gastric remnant.

Background: Percutaneous endoscopic gastrostomy (PEG) is a well accepted mean of achieving enteral nutrition in patients who have lost the ability to take oral feeds.In the literature there have been case reports noting technical difficulty and high risk of complication of PEG in patients who have had a gastrectomy.It has been suggested that radiological assisted gastrostomy or a formal surgical gastrostomy is the preferred approach in this group of patients.Objective: To study the safety and efficacy of PEG placement in patients with gastrectomy.Method: Non-concurrent, cohort study was carried out comprising of 45 patients admitted during 1995 to 1999.Study group comprised of 15 patients with gastrectomy (Billroth II-13; Billroth I-2).Control group comprised of 30 patients matched for age and underlying medical conditions.Mean duration of follow up was 8 weeks (range 1 week to 2 years).Outcome comprised of technical difficulty in doing the procedure, procedure related complications and tolerance to PEG feeds.Technical difficulty was defined on the basis of time taken to complete the procedure.Result: Eight of the gastrectomy patients had a PEG placed successfully and seven patients had a percutaneous endoscopic jejunostomy.No technical complication was noticed in both the study group or the control group.Incidence of pneumonia was higher in patients with gastrectomy as compared to intact stomach, however it did not reach statistical significance.Feed tolerance was similar in both the groups.One patient was lost to follow up.Conclusion: PEG placement in patients with partial gastrectomy is a technically simple, safe and effective procedure.High risk of pneumonia in this group of patients may be related to relative proximal peg tube placement and small gastric remnant.