A case of Crohn＇s disease involving the gallbladder

Crohn's disease is well known to affect any part of thegastrointestinal tract including the oral cavity and anus.Various extraintestinal complications have been reportedin Crohn's disease,but extraintestinal involvementcharacterized by granulomatous lesions is uncommon.Here,we have reported a case about the involvement ofthe gallbladder in Crohn's disease.A 33-year-old womanwas diagnosed having panperitonitis due to intestinalperforation and cholecystitis.The patient was moved tothe surgical service for an emergency operation.On theresected specimen,there was a broad longitudinal ulcerat the mesenteric side.The mucosa of the gallbladderwas nodular and granular,and the wall was thickened.The surface epithelium of the gallbladder was partiallyeroded and pyloric gland metaplasia was observedfocally.Rokitansky-Aschoff sinuses were also present.From the lamina propria to the subserosal layer,therewere several well-formed epithelioid cell granulomas,which were the non-caseating sarcoidal type differentfrom the foreign-body and xanthomatous granulomas.Periodic-acid Schiff and acid fast stains revealed noorganism within the granulomas.Lymphoid aggregateswere present throughout the gallbladder wall.Sectionsfrom the resected ileum showed typical features of theCrohn's disease.When cholecystectomy is performedin a patient with Crohn's disease,the possibility ofgallbladder involvement should be carefully examined byhistopathological tests.     

Kirsner＇s inflammatory bowel disease

Very few medical textbooks have so thoroughly dominated,and even defined a field, as has Inflammatory Bowel Diseases by Joe Kirsner. Originally co-edited with Roy Shorter of Mayo Clinic, this book, beginning with its first edition in 1975, encapsulated the science and art of caring for patients with Crohn‘s disease and ulcerative colitis. Thus it is with considerable respect, and indeed some awe and trepidation,that we eagerly embraced the opportunity to assume the editorship of this preeminent textbook and the obligation to transition it to reflect the changing, increasingly complex pathophysiology and treatment of these diseases.     

Prior appendectomy and the phenotype and course of Crohn＇s disease

AIM: To determine whether prior appendectomy modifies the phenotype and severity of Crohn's disease. METHODS: Appendectomy status and smoking habits were specified by direct interview in 2838 patients consecutively seen between 1995 and 2004. Occurrence of complications and therapeutic needs were reviewed retrospectively. Additionally, annual disease activity was assessed prospectively between 1995 and 2004 in patients who had not had ileocecal resection and of a matched control group. RESULTS: Compared to 1770 non-appendectomized patients, appendectomized patients more than 5 years before Crohn's disease diagnosis (n=716) were more often females, smokers, with ileal disease. Cox regression showed that prior appendectomy was positively related to the risk of intestinal stricture (adjusted hazard ratio, 1.24; 95% confidence interval, 1.13 to 1.36; P=0.02) and inversely related to the risk of perianal fistulization (adjusted hazard ratio, 0.75; 95% confidence interval, 0.68 to 0.83; P=0.002). No difference was observed between the two groups regarding the therapeutic needs, except for an increased risk of surgery in appendectomized patients, attributable to the increased prevalence of ileal disease. Between 1995 and 2004, Crohn's disease was active during 50% of years in appendectomized patients (1318 out of 2637 patient-years) and 51% in non-appendectomized patients (1454 out of 2841 patient-years; NS). CONCLUSION: Prior appendectomy is associated with a more proximal disease and has an increased risk of stricture and a lesser risk of anal fistulization. However, the severity of the disease is unaffected.     

Gallbladder bile composition in patients with Crohn＇s disease

AIM:To further elucidate the pathogenesis andmechanisms of the high risk of gallstone formation inCrohn's disease.METHODS:Gallbladder bile was obtained from patientswith Crohn's disease who were admitted for electivesurgery (17 with ileal/ileocolonic disease and 7 withCrohn's colitis).Fourteen gallstone patients servedas controls.Duodenal bile was obtained from tenhealthy subjects before and after the treatment withursodeoxycholic acid.Bile was analyzed for biliary lipids,bile acids,bilirubin,crystals,and crystal detection time(CDT).Cholesterol saturation index was calculated.RESULTS:The biliary concentration of bilirubin wasabout 50% higher in patients with Crohn's disease thanin patients with cholesterol gallstones.Ten of the patientswith Crohn's disease involving ileum and three of thosewith Crohn's colitis had cholesterol saturated bile.Fourpatients with ileal disease and one of those with colonicdisease displayed cholesterol crystals in their bile.About1/3 of the patients with Crohn's disease had a shortCDT.Treatment of healthy subjects with ursodeoxycholicacid did not increase the concentration of bilirubin induodenal bile.Several patients with Crohn's disease,with or without ileal resection/disease had gallbladderbile supersaturated with cholesterol and short CDT andcontained cholesterol crystals.The biliary concentrationof bilirubin was also increased in patients with Crohn'scolitis probably not due to bile acid malabsorption.CONCLUSION:Several factors may be of importance forthe high risk of developing gallstones of both cholesteroland pigment types in patients with Crohn's disease.     

Characteristics of patients with columnar-lined Barrett＇s esophagus and risk factors for progression to esophageal adenocarcinoma

AIM: To determine the risk factors for the development of esophageal adenocarcinoma in these patients with columnar-lined esophagus (CLE). METHODS: Data collected retrospectively on 597 consecutive patients diagnosed at endoscopy and histology to have CLE at Leeds General Infirmary between 1984 and 1995 were analyzed. Factors evaluated included age, sex, length of columnar segment, smoking, and drinking habits, history of non-steroidal ingestion, presence of endoscopic esophagitis, ulceration or benign strictures and presence of Helicobacter pylori in esophageal biopsies. Univariate and multivariate analyses were performed to identify risk factors for the development of adenocarcinoma. RESULTS: Forty-four patients presented or developed esophageal adenocarcinoma during follow-up. Independent risk factors for the development of adenocarcinoma in patients with CLE were males (OR 5.12, 95%CI 2.04-12.84, P = 0.0005), and benign esophageal stricture (OR 4.37, 95%CI 2.02-9.45, P = 0.0002). Male subjects and patients who developed benign esophageal stricture constituted 86% (n = 38) of all patients who presented or developed esophageal adenocarcinoma. The presence of esophagitis was associated with a significant reduction in the development of esophageal carcinoma (OR 0.28, 95%CI 0.13-0.57, P = 0.0006). No other clinical characteristics differentiate between the non-malignant and malignant group. CONCLUSION: In patients with CLE, endoscopic surveillance for the early detection of adenocarcinoma may be restricted to male subjects, as well as patients who develop benign esophageal strictures.     

Intestinal Behcet＇s disease with pyoderma gangrenosum： A case report

We report here a very rare case of intestinal Behcet'sdisease with pyoderma gangrenosum.A 16-year-oldwoman who was diagnosed with intestinal Behcet'sdisease by the presence of cutaneous pathergy togetherwith two major criteria(oral and genital aphthoses)andone minor criterion(gastrointestinal manifestations),wasreferred to our hospital with a left lower leg ulcer andabdominal pain in September 1989.Colonoscopy dem-onstrated flare-up colitis involving the entire colon.Herlower leg lesion was a painful destructive ulcer with anirregular margin and a ragged overhanging edge.Basedon these clinical and laboratory findings,we diagnosedher cutaneous ulcer as pyoderma gangrenosumdeveloping with exacerbated intestinal Behcet's disease.Her cutaneous and intestinal lesions were poorlycontrolled though she received oral prednisolone treat-ment for a month.Because of aggravated abdominalsymptoms with peritoneal irritation,we performed totalcolectomy in November 1989.The resected specimenwas histologically compatible with intestinal Behcet's disease showing severe inflammation with deepulcerations and neutrophil accumulation.Subsequently,pyoderma gangrenosum rapidly improved.This clinicalcourse may suggest the close relationship betweenpyoderma gangrenosum and intestinal Behcet's disease.     

Microproteinuria in patients with inflammatory bowel disease： Is it associated with the disease activity or the treatment with 5-aminosalicylic acid？

AIM: To investigate whether microproteinuria in patients with inflammatory bowel disease (IBD) is associated with the disease activity or the treatment with 5-aminosalicylic acid (5-ASA). METHODS: We prospectively studied microproteinuria in 86 consecutive patients with IBD, 61 with ulcerative colitis (UC) and 25 with Crohn's disease (CD), before as well as 2 and 6 months after their inclusion in the study. Forty-six patients received 5-ASA for a period of 28.8 months (range 1-168 mo). Microalbuminuria (mALB) and urine levels of the renal tubular proteins beta2-microglobulin (beta2mGLB) and beta-N-acetyl-D-glucosamidase (beta-NAG) as well as the creatinine clearance were determined in a 12-h overnight urine collection. Tumor necrosis factor-alpha (TNF-alpha) serum levels were also measured. RESULTS: A total of 277 measurements (194 in UC patients and 83 in CD patients) were performed. The prevalence of abnormal microproteinuria in UC and CD patients was 12.9% and 6.0% for mALB, 22.7% and 27.7% for beta2mGLB, and 11.3% and 8.4% for beta-NAG, respectively. mALB was not associated with IBD activity. Beta2mGLB and beta-NAG urine levels were correlated to UC activity (UCAI: P<0.01; UCEI: P<0.005). mALB in UC patients and beta-NAG urine levels in CD patients were related to TNF-alpha serum levels. An association was noticed between microproteinuria and smoking habit. Treatment with 5-ASA was not correlated to the severity of microproteinuria or to the changes of creatinine clearance. CONCLUSION: Microproteinuria is mainly associated with UC and its activity but not affected by 5-ASA.     

Acute duodenal Crohn＇s disease successfully managed with low-speed elemental diet infusion via nasogastric tube： A case report

Duodenal Crohn's disease is rare,and patients withoutobstruction are treated medically.We herein report onecase whose duodenal Crohn's disease was successfullymanaged with low-speed elemental diet infusion througha nasogastric tube.A 28-year-old female developedacute duodenal Crohn's disease.Upper GI radiologicand endoscopic examinations showed a stricture in theduodenal bulb.Using the duodenal biopsy specimens,mucosal cytokine levels were measured;interleukin(IL)-1β,IL-6,IL-8,and tumor necrosis factor-α levelswere remarkably elevated.For initial 2 wk,powderedmesalazine was orally given but it was not effective.Forthe next 2 wk,she was treated with low-speed elementaldiet therapy using a commercially available Elental~(TM),which was infused continuously through a nasogastrictube using an infusion pump.The tip of the nasogastrictube was placed at an immediate oral side of the pylorus.The infusion speed was 10 mL/h(usual speed,100 mL/h).After the 2-wk treatment,her symptoms were very muchimproved,and endoscopically,the duodenal stricture andinflammation improved.The duodenal mucosal cytokinelevels remarkably decreased compared with those beforethe treatment.Although our experience was limited,low-speed elemental diet infusion through a nasogastric tubemay be a useful treatment for acute duodenal Crohn'sdisease.     

Heart-touching Chilaiditi＇s syndrome

Symptomatic hepato-diaphragmatic interposition of a bowel loop or Chilaiditi's syndrome is a peculiar anatomical condition most often found by chance. Its described symptoms range from intermittent, mild abdominal pain and dyspepsia to acute intestinal obstruction. We report a case of hepato-diaphragmatic migration of the hepatic flexure of the colon associated to an unusual, heretofore unreported, angina-like pain exclusively evoked by the left lateral decubitus. To maximize the chance of observing anatomical changes in different postures, computed tomography of the chest and abdomen was performed after air insufflation into the colon. While frank herniation into the chest was excluded, the scan showed that the hepatic flexure-with the interposition of the diaphragm-came in contact with the right side of the heart in the left lateral, but not in the supine, decubitus. This finding was reproduced by echocardiography which also showed virtually unaltered hemodynamics after the change of posture. ECG, left and right ventricular global and regional function as well as cardiac injury markers also remained unchanged during the maneuver, indicating that the pain evoked by the latter was unlikely due to myocardial ischemia. This case suggests that Chilaiditi's syndrome should be included among the possible, although rare, causes of unexplained angina-like symptoms.     

Endoscopic treatment and follow-up of gastrointestinal Dieulafoy＇s lesions

AIM: To investigate retrospectively the clinical and endoscopic features of bleeding Dieulafoy's lesions and to assess the short- and long-term effectiveness of endoscopic treatment. METHODS: Twenty-three patients who had gastrointestinal bleeding from Dieulafoy's lesions underwent endoscopic therapy. Demographic data, mode of presentation, risk factors for gastrointestinal bleeding, blood transfusion requirements, endoscopic findings, details of endoscopic therapy, recurrence of bleeding, and mortality rates were collected and analyzed retrospectively. RESULTS: Hemostasis was attempted by dextrose 50% plus epinephrine in 10 patients, hemoclipping in 8 patients, heater probe in 2 patients and ethanolamine oleate in 2 patients. Comorbid conditions were present in 17 patients (74%). Overall permanent hemostasis was achieved in 18 patients (78%). Initial hemostasis was successful with no recurrent bleeding in patients treated with hemoclipping, heater probe or ethanolamine injection. In the group of patients who received dextrose 50% plus epinephrine injection treatment, four (40%) had recurrent bleeding and one (10%) had unsuccessful initial hemostasis. Of the four patients who had rebleeding, three had unsuccessful hemostasis with similar treatment. Surgical treatment was required in five patients (22%) owing to uncontrolled bleeding, recurrent bleeding with unsuccessful retreatment and inability to approach the lesion. One patient (4.3%) died of sepsis after operation during hospitalization. There were no side-effects related to endoscopic therapy. None of the patients in whom permanent hemostasis was achieved presented with rebleeding from Dieulafoy's lesion over a mean long-term follow-up of 29.8 mo. CONCLUSION: Bleeding from Dieulafoy's lesions can be managed successfully by endoscopic methods, which should be regarded as the first choice. Endoscopic hemoclipping therapy is recommended for bleeding Dieulafoy's lesions.     

Castleman's disease with coagulation defect

Summary The case of a young man with the plasmacell type of Castleman's disease is described. Beyond the well known systemic manifestations the coagulation tests showed a decrease in the activity of factors V, VIII and X due to the presence of lupus-like circulating anticoagulants. After surgical excision of the mediastinal mass both hematochemical pathological data and the coagulation defect desappeared. It is suggested that the lupus-like anticoagulant was secreted by the tumor.Supported in part by Consiglio Nazionale delle Ricerche, Grant CT 82.02224.04     

Castleman's病合并糖尿病Graves病1例

1病历资料患者女,53岁。主因Castleman s病6年,糖尿病5年,烦热伴消瘦明显6个月,咳嗽伴发热2 d入院。患者自     

Endoscopic ultrasonography-guided trucut biopsy for the preoperative diagnosis of peripancreatic castleman＇s disease： A case report

Castleman＇s disease （CD） of the pancreas/peripancreas is extremely rare. The recently introduced, endoscopic ultrasonography （EUS）-guided trucut biopsy （TCB） is a useful diagnostic rnodality for obtaining tissue samples from peripancreatic lesions. However, its role in diagnosing CD remains unknown. We report a case of localized, peripancreatic, hyaline-vascular CD biopsied using EUS. The pathology results were initially interpreted as an extranodal, marginal-zone B-cell lyrnphoma. However, polyrnerase chain reaction （PCR） study for the IgH gene rearrangement revealed a polyclonal pattern. We also reviewed the relevant literature. To our knowledge, this is the first illustrated report on EUS-TCB findings of CD with its pathology results of EUS-TCB mimicked a B-cell lymphoma.     

多中心性Castleman病的诊断标准探讨

目的:多中心性巨大淋巴结增生症(multicentric Castleman's disease,MCD)发病机制不明,诊断标准不统一.结合我院诊治的10例MCD病例,就本病的诊治标准进行探讨.方法:全部10例均为初次诊断的MCD患者,淋巴结病理符合CD改变,详细记录并分析患者的临床表现、实验室检查结果及治疗反应.结果...     

Unicentric Castleman disease presenting as a retroperitoneal peripancreatic mass: A report of two cases and review of literature

Castleman disease(CD) is a rare disorder of lymph nodes and related tissues. CD generally occurs in the mediastinum, as well as in cervical, retroperitoneal and axillary regions. The disease is classified into two major types: unicentric CD(UCD) and multicentric CD. The occurrence of UCD in the retroperitoneal peripancreatic region is quite rare. We encountered two cases of retroperitoneal peripancreatic UCD in our hospital during the past three years. Following a series of medical examinations, including magnetic resonance imaging, computed tomography, ultrasonography and postoperative histopathological examination, these two patients were diagnosed with UCD,which presented as a retroperitoneal peripancreatic mass.The mass in each patient was completely excised,and no postoperative radiochemotherapy was administered.Both patients recovered well without recurrence during a follow-up period of 30 mo and 8 mo.     

Hodgkin's and Castleman's disease in a patient with systemic mastocytosis

 Systemic mastocytosis is a rare condition characterized clinically by the local consequences of vasoactive peptides released from infiltrating mast cells in the reticuloendothelial tissues. Mast cells originate from the pluripotent bone marrow stem cells; it is therefore not surprising that myeloproliferative and myelodysplastic disorders commonly coexist or terminate the clinical phase of mastocytosis. We report here, to our knowledge, the first case of Hodgkin's and Castleman's disease occurring in a patient with co-existent systemic mastocytosis, which remained unchanged after combination chemotherapy for Hodgkin's disease. Received: December 18, 1997 / Accepted: September 8, 1998     

Autoimmune haemolytic anaemia presenting 9 years prior to Castleman's disease

Summary Castleman's disease (CD) is a rare disease of unknown aetiology and pathogenesis. We present an unusual case of abdominal CD in whom the first manifestation was an autoimmune haemolytic anaemia presenting 9 years before the diagnosis. The Coombs test became negative 2 months after surgical resection of the mass, suggesting that the mass was the source of the autoantibody. CD may be present in autoimmune haemolytic anaemia in patients with no evidence of any other disease.     

A case of Castleman's disease associated with diffuse idiopathic skeletal hyperostosis and ossification of the posterior longitudinal ligament of the spine

Castleman's disease (CD), diffuse idiopathic skeletal hyperostosis (DISH), and ossification of the posterior longitudinal ligament of the spine (OPLL) are three different entities. Castleman's disease displaying a variety of calcifications in the abdomen and/or pelvis has been reported in some papers. However, there were no reports suggesting an association between CD and ossification/calcification in spine and joints. So far, there has been no case report regarding the coexistence of these diseases in the literature. Herein, we detail a 75-year-old man suffering from CD who demonstrated the features of DISH with coexisting features of OPLL. The cardinal symptoms such as fatigue, high fever, and swollen glands in this case were reduced by corticosteroid therapy. However, it is possible to produce actual symptoms of ossifying/calcified diathesis of entheses and ligaments as a consequence, like the pathology of calcification found in the region of the spleen. In this paper, we describe this patient in order to discuss the association of these diseases.     

Long-term remission in HIV-negative patients with multicentric Castleman's disease using rituximab

Multicentric Castleman's disease (MCD) is an indolent lymphoproliferative disorder. The pathogenesis of MCD has not been established, and its treatment remains uncertain. Several authors have described the relationship of human herpes virus type 8 (HHV-8) to MCD in human immunodeficiency virus (HIV)-positive patients. Recently, anti-CD20 monoclonal antibody (rituximab) is increasingly being used to treat HIV-positive MCD; although it is uncertain whether rituximab is effective for HIV-negative patients with MCD. To explore the benefit of rituximab for HIV-negative patients with MCD, we describe the clinical and biologic course in three HIV-negative patients with MCD, and examined the relationship of HHV-8 infection to HIV-negative MCD. Their polymerase chain reaction analyses for the HHV-8 sequence in peripheral blood were negative, and there was no relationship between HHV-8 infection and symptoms of HIV-negative MCD. Two of three patients (66%) achieved a near complete remission with no clinical symptoms due to MCD with a follow-up of 16-40 months after rituximab administration. One of the three patients presented no clinical remission of MCD after rituximab administration, although a significant decrease of inflammatory parameters was observed. These findings suggest that rituximab treatment may be an appropriate first-line therapy for HIV-negative MCD.