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Biomedical subjects

J P Barbier

Publications and source records attributed to J P Barbier.

At least 19 recordsLinked to original sources

Association of a duodenal follicular lymphoma and hereditary nonpolyposis colorectal cancer.

Hereditary nonpolyposis colorectal cancer (HNPCC) is an inherited predisposition to colorectal and endometrial cancers caused by germline mutation of mismatch repair genes, with hMLH1 and hMSH2 underlying the majority of the cases. Although lymphoid tumors are the most common tumors in mouse models for HNPCC, lymphomas are almost never encountered in patients who have HNPCC, except in rare families with germline homozygous deletion of hMLH1. We report the case of a 53-year-old man who had a history of colon cancers related to constitutional hMLH1 mutation and who was diagnosed as having a duodenal follicular lymphoma This diagnosis was supported by IgH-BCL2 rearrangement and BCL2 immunoreactivity in tumor cells. The association of both of these possibly related rare diseases has never been reported. To clarify this relationship, we searched for hMLH1 expression and mismatch repair deficiency in the duodenal lymphoma. hMLH1 immunostaining was positive in lymphoid tumor cells, and no microsatellite instability was detected. In agreement with mouse models for HNPCC, these results suggest the involvement of alternative mechanisms to complete mismatch repair deficiency for lymphomagenesis in HNPCC syndrome.

Adaptor Proteins, Signal Transducing↗

Distinction between coeliac disease and refractory sprue: a simple immunohistochemical method.

AIMS: We recently showed that refractory sprue is distinct from coeliac disease, the former being characterized by abnormal intraepithelial T-lymphocytes expressing a cytoplasmic CD3 chain (CD3c), lacking CD3 and CD8 surface expression, and showing TCRgamma gene rearrangements. To take advantage of the abnormal phenotype of CD3c + CD8 - intraepithelial lymphocytes (IEL) in refractory sprue we developed a simple method to distinguish coeliac disease from refractory sprue. METHODS AND RESULTS: Comparative immunohistochemical studies using anti-CD3 and anti-CD8 antibodies were applied on paraffin-embedded and frozen biopsy specimens in refractory sprue (n = 6), coeliac disease (n = 10), healthy controls (n = 5) and suspected refractory sprue (n = 6). Comparable results were obtained on fixed and frozen biopsy specimens. In four of the six patients with suspected refractory sprue, abnormal CD3c + CD8 - IEL and TCRgamma gene rearrangements were found, as in refractory sprue; the remaining two patients had normal (CD3 + CD8 +) IEL and no TCRgamma gene rearrangements. Both patients had coeliac disease, as one failed to comply with a gluten-free diet, while the other was a slow responder. CONCLUSION: This simplified immunostaining method using anti-CD3 and anti-CD8 antibodies on paraffin sections can distinguish active coeliac disease from refractory sprue and should prove useful in clinical practice.

Adult↗

Biliary varices on endoscopic ultrasonography: clinical presentation and outcome.

BACKGROUND AND STUDY AIMS: Bile duct varices are not a well-recognized feature of portal venous obstruction. The aim of the present study was to describe the clinical and endosonographic features of biliary involvement in patients with extrahepatic portal venous obstruction (EPVO). PATIENTS AND METHODS: A retrospective study was conducted of the clinical features, outcome, and endosonographic findings (using Olympus EUM-3 or EUM-20 probes) in 21 patients with EPVO and endosonographic features of biliary varices. Biliary varices were defined as multiple, large, serpiginous, anechoic vascular channels in and/or surrounding the extrahepatic biliary tracts. RESULTS: Biliary varices have not previously been visible using conventional imaging methods (computed tomography and ultrasonography). They were identified using EUS in the wall of the common bile duct in 16 patients (76%), surrounding the common bile duct (CBD) in 11 patients (52%), and in the gallbladder in nine (43%). The varices were the cause of obstructive jaundice in three of the 21 patients (14%), but only when they were in the wall of the CBD. Two of these patients were treated using portosystemic shunting, and the other received a biliary endoprosthesis. The EUS examination also provided evidence of unrecognized pancreatic or biliary tumors in three other patients with EPVO of undetermined origin. CONCLUSIONS: EUS can serve to diagnose biliary varices in patients with EPVO and jaundice. Although biliary varices are mainly asymptomatic, they may cause obstructive jaundice when they are located in the wall of the CBD. EUS can also detect unrecognized malignant tumors in patients with EPVO of undetermined origin.

Adult↗

Right colonic involvement is associated with severe forms of ischemic colitis and occurs frequently in patients with chronic renal failure requiring hemodialysis.

OBJECTIVE: The aim of this study was to identify factors associated with severe outcome in patients with ischemic colitis. METHODS: The files of 60 consecutive inpatients (34 women, 26 men, mean age 67 yr) with ischemic colitis were reviewed. The following data were analyzed: age, sex, smoking, medications, history of cardiovascular disease, metabolic disease, chronic renal failure and hemodialysis, the time elapsed between the first symptoms and the diagnosis, and the site and extension of their colonic involvement. Patients were divided into two groups according to outcome: those with severe disease, including those who died from ischemic colitis (n = 3) or who required surgical resection (n = 21); and those with mild forms of colitis who were treated successfully without surgery (n = 36). The two groups were compared by means of univariate and multivariate analysis to identify factors associated with unfavorable outcomes. Only patients who had a complete examination of the colon (n = 51) were entered into the statistical analysis. RESULTS: By univariate analysis, chronic renal failure (p = 0.03), hemodialysis (p = 0.01), short delay between symptoms and diagnosis (p = 0.01), and right colonic involvement (p = 0.002) were significantly more common in the patients with severe colitis. By logistic regression, right colonic involvement was the only factor independently associated with severity (p = 0.01). Right-sided lesions were present in 82% of patients on dialysis but in only 26% of patients not on dialysis (p = 0.0005). CONCLUSIONS: Right colonic involvement is associated with severe forms of ischemic colitis and occurs frequently in patients with chronic renal failure requiring hemodialysis.

Adult↗

Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil.

The VIPoma syndrome is rare. It is usually caused by a neuroendocrine tumor located in the pancreas. Somatostatin analogs and interferon-a can be helpful in the symptomatic control of the disease, but the efficacy of chemotherapy in metastatic disease is limited. We report the case of a 32-yr-old patient who had a primary intestinal VIPoma with peritoneal carcinomatosis and hepatic metastases. Somatostatin analogs and conventional chemotherapy regimens were not effective on VIPoma syndrome and tumor progression. The combination of 5- fluorouracil and interferon-alpha was associated with a major clinical improvement and tumor regression. Further investigations should evaluate the place of such a combination as a first line treatment for patients with metastatic neuroendocrine tumors.

Adult↗

Outcome after surgical resection of intraductal papillary and mucinous tumors of the pancreas.

OBJECTIVE: Treatment of intraductal papillary and mucinous tumors of pancreas (IPMT) usually requires surgery. The objective of this study was to evaluate the risk of recurrence in patients after surgery according to the histological nature of the neoplasm and the type of surgery. METHODS: The outcome of 45 patients who underwent partial pancreatectomy (n = 35) or total pancreatectomy (n = 10) for IPMT was studied according to the nature of the neoplasm (invasive carcinoma or noninvasive neoplasm), type of surgery (partial or total pancreatectomy), and lymph nodes status. RESULTS: The overall 3-yr actuarial survival rate was 83%. Death occurred in seven of 20 (35%) patients with invasive carcinoma and in one of 26 (4%) patients with noninvasive tumors (p<0.05). There were two recurrences in the seven patients with noninvasive neoplasm who underwent partial pancreatectomy with involved resection margins, and none in the 13 patients with disease-free margins. In patients with invasive carcinoma, there was one recurrence after total pancreatectomy, six after partial pancreatectomy with disease-free margins and six after partial pancreatectomy with involved margins. In patients with invasive carcinoma, total pancreatectomy and the absence of lymph nodes involvement were independently associated with a low risk of recurrence. CONCLUSIONS: IPMT may be managed as follows: 1) in patients with noninvasive neoplasms, partial pancreatic resection should be guided by frozen section examination until disease-free margins are obtained; and 2) in patients with invasive carcinoma, total pancreatectomy seems most likely to cure the patient, but should be discussed according to the general status and the age.

Actuarial Analysis↗

Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumours.

BACKGROUND/AIM: Some endoscopic ultrasonographic (EUS) features have been reported to be suggestive of malignancy in gastrointestinal stromal cell tumours (SCTs). The aim of this study was to assess the predictive value of these features for malignancy. METHODS: A total of 56 histologically proven cases of SCT studied by EUS between 1989 and 1996 were reviewed. There were 42 gastric tumours, 12 oesophageal tumours, and two rectal tumours. The tumours were divided into two groups: (a) benign SCT, comprising benign leiomyoma (n = 34); (b) malignant or borderline SCT (n = 22), comprising leiomyosarcoma (n = 9), leiomyoblastoma (n = 9), and leiomyoma of uncertain malignant potential (n = 4). The main EUS features recorded were tumour size, ulceration, echo pattern, cystic spaces, extraluminal margins, and lymph nodes with a malignant pattern. The two groups were compared by univariate and multivariate analysis. RESULTS: Irregular extraluminal margins, cystic spaces, and lymph nodes with a malignant pattern were most predictive of malignant or borderline SCT. Pairwise combinations of the three features had a specificity and positive predictive value of 100% for malignant or borderline SCT, but a sensitivity of only 23%. The presence of at least one of these three criteria had 91% sensitivity, 88% specificity, and 83% predictive positive value. In multivariate analysis, cystic spaces and irregular margins were the only two features independently predictive of malignant potential. The features most predictive of benign SCTs were regular margins, tumour size < or = 30 mm, and a homogeneous echo pattern. When the three features were combined, histology confirmed a benign SCT in all cases. CONCLUSIONS: The combined presence of two out of three EUS features (irregular extraluminal margins, cystic spaces, and lymph nodes with a malignant pattern) had a positive predictive value of 100% for malignant or borderline gastrointestinal SCT. Tumours less than 30 mm in diameter with regular margins and a homogeneous echo pattern are usually benign.

Adult↗

Push enteroscopy in celiac sprue and refractory sprue.

BACKGROUND: The aim of this study was to determine in patients with sprue whether jejunal endoscopy improves the diagnostic yield or provides information that may modify management, when compared with evaluation limited to the duodenum. METHODS: From January 1994 to June 1998, a total of 31 patients (6 men, 25 women, mean age 41 years) were prospectively evaluated by push enteroscopy. They were divided into two groups: (1) celiac disease at different stages of activity (n = 23) and (2) refractory sprue (n = 8). The endoscopic and histologic findings in the duodenum and in the jejunum were compared. RESULTS: Celiac disease: In 19 patients, endoscopic and histologic findings in the duodenum and jejunum were similar; in four patients villous atrophy was more severe in the duodenum than in the jejunum. Refractory sprue: In 5 of 8 patients, enteroscopy revealed ulcerative jejunitis, whereas ulcerations were found in the duodenum in only one case. CONCLUSION: In refractory sprue, push enteroscopy with jejunal biopsies was of diagnostic value in 50% of cases demonstrating ulcerative jejunitis, whereas it did not modify the management of patients with responsive celiac disease.

Adult↗

Small bowel metastases from primary carcinoma of the lung: clinical findings and outcome.

OBJECTIVE: Symptomatic small bowel metastases from primary carcinoma of the lung have been rarely reported. The aim of this study was to describe clinical presentation and outcome in a series of patients. METHODS: Between 1984 and 1996, 1544 patients with lung cancer were referred to our institution for surgery and 1399 were operated on. Seven of them developed a symptomatic small bowel metastasis. Clinical, radiological, and pathology records were reviewed. RESULTS: In 6 of 7 patients, the lung cancer was previously operated on from 0.5 to 24 months before the diagnosis of small bowel metastasis. In 1 patient, the primary tumor was diagnosed after small bowel metastasis resection. Clinical symptoms at presentation were acute peritonitis in 2 patients, progressive digestive obstruction in 3, and gastrointestinal bleeding in 2. The diagnosis was suspected on abdominal ultrasonography in 2 cases, and small bowel radiography in 3 cases. It was confirmed either by computed tomographic scan or by push enteroscopy. All patients underwent operation (intestinal resection in 6 and bypass in 1) with no postoperative death. Small bowel metastases were located in the jejunum in 2 patients, in the ileum in 3, and in both sites in 2. Histological features of the metastases were identical to the primary tumor: squamous cell carcinoma (n = 3), undifferentiated large cell carcinoma (n = 2), adenosquamous carcinoma (n = 1), and adenocarcinoma (n = 1). In 6 patients, small bowel metastases were associated with other metastatic sites. Six patients died within 8 months after metastasis resection. One patient was alive 22 months after bowel resection. CONCLUSIONS: Symptomatic small bowel metastases can occur early in the course of lung cancer. Resection should be considered as the best palliative treatment to prevent bowel obstruction or peritonitis.

Adult↗

[Selective segmental colectomy in diverticular sigmoiditis. The surgical risk is not increased after 70 years of age].

UNLABELLED: Segmental colectomy for complicated diverticulitis is often indicated, between attacks, in elderly patients. We report a retrospective study of operated patients over the age of 70. The aim of this retrospective study was to evaluate the age risk of consecutive patients operated between diverticular attacks by segmental colectomy, and whether such a surgical procedure is justified, as the life expectancy of these patients was 74 years for men and 81.9 years for women in 1996. PATIENTS AND METHOD: From January 1990 to December 1996, 117 patients were operated, between attacks, for complicated sigmoid diverticulitis; 31% (n = 37) were over the age of 70 years. They were 16 men and 21 women with an overall mean age of 77 (range: 70-85 years). Indications for surgery were repeated attacks (n = 17), large bowel stenosis (n = 14) and colovesical fistula (n = 6). The operation was performed an average 8 weeks after the last infectious episode (3-10 weeks). RESULTS: Among patients over the age of 70 years, the postoperative morbidity rate was 40% and there was no mortality. One patient was reoperated for an anastomotic abscess and was managed by protective colostomy and pelvic drainage with conservation of the anastomosis. CONCLUSION: This retrospective study shows that in our experience, one third of patients operated for a complication of diverticulitis were over the age of 70 years. In this subgroup, left colectomy, when performed between attacks carries an acceptable specific morbidity and no mortality. A further study could compare the percentage and outcome of operated patients with those who were denied surgery.

Adult↗

Simple clinical assessment of colonic response to food.

The colonic response to food (CRF) is an integrated function of the colon that has been poorly studied in clinical practice. This study describes a new method to measure it, based on the progress of radio-opaque markers, and shows the results in healthy subjects and in patients with irritable bowel syndrome (IBS). Thirty healthy subjects and 43 patients suffering from IBS were studied. Two studies of colonic transit time (CTT), at rest and after eating a standard test meal, were performed. CRF was quantified by calculating the variation in number of markers in each zone of interest of the large bowel using X-ray films of the abdomen taken before and after eating. The results were as follows: (1) CRF is characterised by caudal propulsion of the colonic contents in the two groups. In controls, there is emptying of the cecum-ascending colon region and filling of the distal large bowel. In IBS patients, only emptying of the left transverse colon and the splenic flexure is found. (2) IBS patients have a delayed CTT because of slowing in the right and left colon, and this is both before and after a meal. The determination of the movements of markers after eating is a simple method that is useful in clinical practice to evaluate CRF.

Adult↗

Abnormal intestinal intraepithelial lymphocytes in refractory sprue.

BACKGROUND & AIMS: The etiology of refractory sprue is unclear. To gain insight into its pathogenesis, the phenotype and T-cell receptor (TCR) gene rearrangement status of intestinal lymphocytes were analyzed in a group of patients with clinical or biological features of celiac disease but either initially or subsequently refractory to a gluten-free diet. METHODS: Intestinal biopsy specimens were obtained from 26 adults: 6 patients with refractory sprue, 7 patients with active celiac disease, and 13 normal controls. The phenotype of intestinal lymphocytes was studied by immunohistochemistry and, in 3 patients with refractory sprue, by cytometry of lymphocytes purified from intestinal biopsy specimens. TCR rearrangements were assessed by studying TCRgammaV-J junctional regions from DNA extracted from intestinal biopsy specimens and purified intestinal lymphocytes. RESULTS: In the 6 patients with refractory sprue, but not in normal controls or patients with active celiac disease, the intestinal epithelium was massively infiltrated by small lymphocytes that lacked CD8, CD4, and TCR, contained intracytoplasmic but not surface CD3epsilon chains, and exhibited restricted TCRgamma gene rearrangements. CONCLUSIONS: Refractory sprue is associated with an abnormal subset of intraepithelial lymphocytes containing CD3epsilon and restricted rearrangements of the TCRgamma chain but lacking surface expression of T-cell receptors.

Adult↗

Intraductal papillary and mucinous tumors of the pancreas: accuracy of preoperative computed tomography, endoscopic retrograde pancreatography and endoscopic ultrasonography, and long-term outcome in a large surgical series.

BACKGROUND: Few data are available on the accuracy of preoperative imaging or on long-term outcome after surgery for intraductal papillary and mucinous tumors of the pancreas. The aims of this study were to assess the following: (1) the accuracy of preoperative computed tomography, endoscopic retrograde pancreatography, and endoscopic ultrasonography for determination of tumor invasion and pancreatic extension as compared with surgical findings; (2) the long-term outcome after surgery. METHODS: Forty-seven patients who underwent surgery between 1980 and 1995 for pathologically diagnosed intraductal papillary and mucinous tumors were included in this study. The findings of available computed tomography (n = 25), endoscopic retrograde pancreatography (n = 29), and endoscopic ultrasonography (n = 21) were reviewed by experienced clinicians blinded to pathologic diagnosis to assess tumor invasion and pancreatic extension. Pathologic specimens were reviewed by experienced pathologists. Postoperative follow-up data were analyzed. RESULTS: Histologic features of invasive carcinoma were found in 43% of patients, severe dysplasia in 21%, and mild or moderate dysplasia in 36%. The overall accuracy of computed tomography, endoscopic retrograde pancreatography, and endoscopic ultrasonography in distinguishing between invasive and noninvasive tumors were, respectively, 76%, 79%, and 76%. The overall 3-year disease-free survival rate was 63%, but it was 21% among patients with invasive carcinoma at surgery (p < 0.001). CONCLUSIONS: This study emphasizes the need for early surgical resection in patients with suspected intraductal papillary and mucinous tumors of the pancreas because of the high frequency of invasive carcinoma and the inadequacy of preoperative imaging for assessing malignancy.

Adenocarcinoma, Mucinous↗

Gluten-free diet induces regression of T-cell activation in the rectal mucosa of patients with celiac disease.

OBJECTIVE: An increase in the number of intraepithelial lymphocytes (IEL) in the rectal epithelium of patients with active celiac disease has been described. No data are available about how they vary during a gluten-free diet. The aim of the study was to assess the effect of a gluten-free diet on T-cell activation in the rectal mucosa of adult patients with celiac disease. METHODS: Frozen duodenal and rectal biopsies were available in four celiac patients (one male, three female, mean age 39 yr) both before and after 7 to 24 months on a gluten-free diet. Biopsy samples were stained using monoclonal antibodies directed against CD3, betaF1, TcRdelta1, CD25, and HLADR. Numbers of IEL were estimated by counting the peroxidase-stained cells per 100 epithelial cells. Four patients without histological abnormalities were used as control subjects. RESULTS: In the four patients with active celiac disease but in none of the controls, CD25 was expressed by both duodenal and rectal lamina propria cells and HLADR was expressed by duodenal (4/4) and rectal (2/4) epithelial cells. In addition, two patients with active celiac disease had features of lymphocytic colitis, i.e., >20 IEL per 100 epithelial cells. After a gluten-free diet, the mean number of rectal CD3+ betaF1+ IEL decreased (9% vs 21%) and the expression of CD25 and HLADR was no longer present. These changes mirrored those found in the small intestinal biopsies. CONCLUSION: These results suggest that in celiac disease, gluten-driven T-cell activation is not restricted to the proximal part of the intestine but is present on the whole intestinal length. Assessment of the effectiveness of a gluten-free diet through rectal biopsies warrants investigation, as it could lessen discomfort for patients and prove more cost-effective.

Adult↗