Parenteral fish oil-associated burr cell anemia.

We report the development of burr cell anemia in an infant with short bowel syndrome who received parenteral fish oil (Omegaven, Fresenius-Kabi, Graz, Austria) after development of total parenteral nutrition-associated liver disease. Parenteral fish oil was discontinued, and the burr cell anemia disappeared, suggesting that parenteral fish oil might be associated with hemolytic anemia.

Pediatric gastroenterology fellows, class of 2007: how well are they prepared for the future?

OBJECTIVES: Pediatric gastroenterology fellows are expected to acquire skills as clinicians, researchers, and educators. An e-mail survey was conducted to examine training experiences of individual fellows; to understand how graduating fellows rate their abilities as clinicians, researchers, and teachers; and to answer whether the size of a pediatric gastroenterology training program affects a fellow's training and future position choice. MATERIALS AND METHODS: A survey was e-mailed to 76 third-year pediatric gastroenterology fellows. Respondents were ranked according to the size of their training program. RESULTS: Completed surveys were returned by 50 respondents. Of these, 75% planned to pursue careers in academic medicine and 16% in private practice. In all, 68% of trainees participated in some type of basic research and 64% in clinical research. As attending physicians, 22% of fellows hoped to conduct basic science research and 74% clinical research. The majority thought they were competent or proficient teachers, and rated themselves as advanced beginners or competent when asked to evaluate their research skills. The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition recommends that fellows perform 100 colonoscopies, 20 liver biopsies, and 5 paracenteses to be considered competent. We found that regardless of program size, 48% of fellows performed fewer than 100 colonoscopies, 62% performed fewer than 20 liver biopsies, and 80% performed fewer than 5 paracenteses. CONCLUSIONS: The majority of fellows will pursue academic careers. Trainees may not be performing sufficient numbers of procedures. The number of procedures performed during fellowship was independent of program size.

Long-term partial remission of autoimmune thrombocytopenia in a patient treated with the anti-tumor necrosis factor-alpha antibody infliximab for refractory fistulizing Crohn's disease.

BACKGROUND: Up to the present date, the treatment of recurrent chronic fistulas occurring with Crohn's disease represents a challenging task for both internists and surgeons alike. METHODS: Conservative methods of treatment using steroids, dietotherapy, antibiotics or immunosuppressive agents are not particularly effective in treating fistulas. Treatment with anti-tumor necrosis factor-alpha (TNFalpha) antibodies results in more remissions of fistulas. However, its use requires appropriate medical experience and causes substantial costs. Surgical procedures such as fistula sanitation or protective ileostomy are often obfuscated by the recurrence of the condition. The efficiency of anti-TNFalpha antibodies for the treatment of active Crohn's disease has been evidenced through several multicenter, double-blind, placebo-controlled studies. RESULTS: Here we report the successful anti-TNFalpha treatment of a patient suffering from chronic, fistulizing, therapy-resistant Crohn's disease and a concomitant chronic autoimmune thrombocytopenia. The chimeric anti-TNFalpha antibody infliximab markedly reduced the disease activity of Crohn's disease, and, in addition, substantially increased the platelet counts. After infliximab application, no other treatment of autoimmune thrombocytopenia was required. After infliximab therapy, autoantibodies to dsDNS could be observed. However, there were no signs of a lupus-like disease. CONCLUSION: This report indicates a favorable therapeutic response of autoimmune thrombocytopenia associated to Crohn's disease.

Dermatologic and immunologic findings in the immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome.

BACKGROUND: The immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare genodermatosis associated with dermatitis, enteropathy, type 1 diabetes, thyroiditis, hemolytic anemia, and thrombocytopenia. IPEX results from mutations of FOXP3, a gene located on the X chromosome that encodes a DNA-binding protein required for development of regulatory T cells. If untreated, affected males die early in life from malabsorption and other complications. To our knowledge, this syndrome has never been described in the dermatology literature. OBSERVATIONS: We studied an 11-year-old boy with IPEX. Mutation analysis revealed a G-->A transition (1150G>A) in exon 11, resulting in a putative substitution of Ala-->Thr at residue 384, within the DNA-binding site. Histopathologic examination of an active skin lesion revealed psoriasiform dermatitis. The lesions improved with clobetasol ointment. The patient also displayed alopecia universalis, which had been present since age 18 months, accompanied by longitudinal ridging of the nails. Lymphocyte challenge tests revealed a profound inability to synthesize interferon gamma (INF-gamma) and dysregulated production of other cytokines. CONCLUSIONS: IPEX is an often fatal genodermatosis associated with multiple autoimmune disorders. Cutaneous findings may include dermatitis, bullae, urticaria, alopecia universalis, and trachyonychia. Recognition of this life-threatening disorder is crucial for optimal treatment and genetic counseling.

Serum levels of soluble E-selectin are associated with the clinical course of metastatic disease in patients with liver metastases from breast cancer.

Increasing evidence indicates that the expression of the endothelial adhesion molecule E-selectin is associated with progression and metastasis of breast cancer. Patients with liver metastases also show increased serum levels of the soluble form of E-selectin. It was our aim to compare serum levels of soluble E-selectin (sES) in such patients with the biology of the primary tumor and the course of the metastatic disease under therapy. We examined 69 patients with liver metastases from breast cancer who were selected to receive systemic tumor therapy because of progressive disease (n = 44) or newly detected liver metastases (n = 25). Serum concentrations of sES were measured before each therapy cycle using a specific ELISA. Serum concentrations of sES before the start of therapy were compared to clinical parameters and histopathological findings referring to the primary tumor. Secondly, serum levels of sES were compared to serum concentrations of the corresponding tumor markers. We observed a possible trend for certain unfavorable prognostic parameters (e.g., young women, low-graded tumors, human epidermal growth factor receptor 2 overexpression) to be related to higher serum levels of sES. Serum levels of sES were correlated with tumor marker levels in a logarithmical relation (r = 0.44, P < 0.0005). In some cases it could be demonstrated that serum levels of sES changed similarly to the course of tumor marker levels. We conclude that serum levels of sES are associated with the clinical course of liver metastases from breast cancer. Further investigations are needed to clarify if serum levels of sES may serve as tumor marker in certain clinical situations. E-selectin should be evaluated as a possible target for antimetastatic therapy studies.

Colon capsule endoscopy: detection of colonic polyps compared with conventional colonoscopy and visualization of extracolonic pathologies.

BACKGROUND: Conventional colonoscopy (CC) is the gold standard for diagnostic examination of the colon. However, the overall acceptance of this procedure is low due to patient fears of complications or embarrassment. Colon capsule endoscopy (CCE) represents a minimally invasive, patient-friendly procedure that offers complete visualization of the entire intestine. OBJECTIVE: To assess the PillCam Colon 2 (Given Imaging Ltd, Israel) capsule with regard to feasibility, sensitivity and specificity for the detection of colonic pathologies and additional recorded extracolonic findings. METHODS: CCE was performed before CC in patients indicated for CC for known or suspected colonic disease. The results of both techniques were compared with regard to polyp detection. Additionally, bowel preparation and extracolonic pathologies were analyzed. RESULTS: Twenty-four patients (mean age 51.1 years) were included in the analysis. Visualization of the colon was complete in 23 CCs and 17 CCEs. No adverse events or major technical failures occurred. CC detected 47 polyps and CCE detected 43 polyps of any size (per-finding sensitivity 90.9%, specificity 67.6%). The accuracy of CCE in detecting polyp carriers was 81.5% (per-patient analysis). On average, the colon was adequately cleansed in 90.1% of patients. CCE identified esophageal, gastric and small bowel pathologies in seven (24%), nine (38%) and 14 (58%) patients, respectively. CONCLUSIONS: CCE proved to be technically feasible and safe. Acceptable sensitivity and moderate specificity levels in polyp detection were recorded. Bowel preparation was adequate in most patients. Because extracolonic pathologies were effectively visualized, new indications for the PillCam Colon 2 may be defined.

Use of Polyflex Airway stent in the treatment of perforated esophageal stricture in an infant: a case report.

Anastomotic stricture is a common sequela after primary repair of esophageal atresia with esophagoesophagostomy. Esophageal perforation secondary to dilatation of the stricture, and refractory stricture are not uncommon. We present a case using a Polyflex Airway stent (Boston Scientific, Natick, MA) as an alternative treatment of esophageal stricture and perforation in an infant.

Serum levels of hepatocyte growth factor/scatter factor in patients with liver metastases from breast cancer.

OBJECTIVE: Recent studies have shown that the pleiotropic cytokine hepatocyte growth factor/scatter factor (HGF/SF) and its receptor c-Met play major roles in the malignant progression of numerous tumors. For patients with breast cancer liver metastases, increased serum levels of HGF/SF have been reported. We studied the relationship between the clinical course of the disease and the serum levels of HGF/SF in such patients. METHODS: We examined 51 patients treated for breast cancer liver metastases. Serum concentrations of HGF/SF were measured before each therapy cycle and compared to the corresponding tumor marker levels. RESULTS: Mean serum levels of HGF/SF in patients with liver metastases were increased above the reported reference levels of primary breast cancer patients. Serum levels of HGF/SF were correlated with tumor marker levels in a logarithmic relation (r = 0.47, p < 0.001). In some cases serum concentrations of HGF/SF changed similarly to the course of the corresponding tumor markers. CONCLUSIONS: Serum levels of HGF/SF are associated with the clinical course of metastatic breast cancer patients with liver metastases. Further studies are required to clarify the potential value of the HGF/SF serum concentration as a tumor marker. HGF/SF and its receptor c-Met should be further evaluated as therapeutic targets.

Prognostic factors for patients with liver metastases from breast cancer.

BACKGROUND: The prognosis of patients with liver metastases from breast cancer is commonly poor. After initial diagnosis of hepatic metastases, a median survival time of 1-20 months can be expected. The definition of prognostic factors for such patients may influence therapeutic decisions. In particular, the characterization of patients who can expect long-term survival could assist in optimizing treatment. METHODS: We retrospectively studied n = 350 patients with liver metastases from breast cancer. All patients were stratified following their survival after occurrence of liver metastases. Kaplan-Meier studies were performed, as well as univariate and multivariate analyses of several clinical, histopathological and therapeutic factors. RESULTS: Median survival time was 14 months. N = 66 (18.9%) patients survived longer than 36 months after the primary diagnosis. Multivariate analysis showed prognostic relevance for the time interval between the primary diagnosis of breast cancer and the initial diagnosis of hepatic metastases (p < 0.05). Furthermore, prognostic relevance was found for the pattern of metastasization (p < 0.05) and for signs of hepatic dysfunction (ascites, jaundice, p < 0.005). Univariate analysis showed a prognostic benefit for patients with an expression of Ki-67 < 20%, p53 < 50% and a positive hormonal receptor status. Patients who received a regional therapy survived on average longer than patients who were only treated systemically (33 versus 11 months, p < 0.001). CONCLUSIONS: Consideration of prognostic implications of the described parameters may help to find the most appropriate treatment for patients with liver metastases from breast cancer. The possibility of local therapeutic interventions should be considered in a defined subgroup.