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[Association of carcinoid tumor of the appendix and Crohn disease (case report and review of the literature)].

The authors describe the coexistence of the carcinoid of the appendix and Crohn's disease. In the case of their woman patient the carcinoid was identified with the examination of the resected ileoascendent part of the bowel resulting of the complication of the Crohn's disease. 10 similar association is known in the literature but none of the patients had the signs of the carcinoid syndrome. Subsequent adrenerg syndrome after an alimentary hypoglycemia (increased evacuation of the cathecolamines and their metabolites in the urine), food allergy (increased IgE type antibody to milk protein) or gastroenteropancreatic (GEP) neuroendocrine tumor (based on the result of the 111In-octreoscan and the increased 5-hydroxyindolaceticaciduria) equally suspected of the symptoms (palpitation, flush) manifested after the operation. They look for the connection between the genesis of the intestinal carcinoid and the Crohn's disease with working up the bibliography. Summing up the references with a view of the latest it can be stated that the carcinoid of the appendix occurs more than orders of magnitude in the samples from inflamed surrounding than the samples from autopsy (0.24%--820/338,000 inflamed appendix and 0.03%--19/53,430 appendix from autopsy). Consequently the inflammation create favourable condition for the development of the carcinoid of the appendix.

Adult↗

Mucocele of the appendix with hematuria.

Mucocele of the appendix, a nonspecific and descriptive term for an abnormal mucous accumulation within the appendiceal lumen, regardless of the underlying cause, is a rare clinical disease that is not usual as a consideration in the differential diagnosis of right lower quadrant lesions. The reported prevalence of mucocele of the appendix in appendectomy specimens is 0.2-0.3 percent. Hematuria due to mucocele of the appendix is extremely rare. Up to now, only few cases of such condition have been recorded in the literature. We describe a woman who experienced intermittent episodes of right lower quadrant pain and hematuria. Abdominal exploration incidentally displayed mucocele of the appendix. No evidence of other lesions was found. The patient was still in good health after operation. This case highlights mucocele of the appendix as a consideration in the differential diagnosis of right lower quadrant pain with hematuria.

Aged↗

Carcinoid tumors of the appendix. Our experience in a university hospital.

OBJECTIVE: To present our experience of carcinoid tumors of the appendix managed at a university teaching hospital. Complex symptomatology, varied biochemical affections and different surgical therapeutic modalities are discussed. METHODS: The medical records of all the patients who underwent consecutive appendectomies at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia from 1994 to 1999 were retrospectively analyzed. The data of patients identified to have histological evidence of carcinoid tumors of the appendix were further reviewed for the demographic details, indications for surgery, surgical procedure, tumor localization in the appendix and size; concomitant appendicitis and further surgical procedures were considered. RESULTS: During the study period, 1547 appendectomies were performed and, out of these, 9 (0.6%) cases were reported to have carcinoid tumors of the appendix. There were 4 male and 5 female patients, age range 17-51 years (median 29.8 years). Seven subjects had a clinical evidence of appendicitis while 2 presented with chronic abdominal pain. There were 6 open and 3 laparoscopic appendectomies. Six carcinoid tumors were encountered at the appendiceal apex, 2 at the midportion, and one at the base with a mean diameter of 9.5 mm (range, 4-19 mm). One patient had histologically confirmed residual tumor, which necessitated a right hemicolectomy 3 weeks later. All patients remained disease-free during a mean follow up of 7 years (range, 4-10 years). CONCLUSION: Carcinoid tumors of the appendix are extremely rare and invariably remain asymptomatic. Simple appendectomy offers adequate relief while the need for further extensive surgery depends on tumor characteristics and dissemination. Despite an excellent prognosis, all reported patients should be followed up with urinary 5-hydroxyindoleacetic acid and abdominal ultrasonography.

Abdominal Pain↗

Effect of cecum and appendix on 7 alpha-dehydroxylation and 7 beta-epimerization of chenodeoxycholic acid in the rabbit.

The effect of surgical removal of cecum and appendix on bile acid metabolism was studied in the rabbit. Bile acid composition of bile and intestinal contents were analyzed by gas-liquid chromatography and individual bile acids were further identified by mass spectrometry. In normal, intact rabbits (group I), the biliary bile acids consisted largely of deoxycholic acid (DA) 95.3 +/- 1.0 (mean +/- SE) %; cholic acid (CA) 2.3 +/- 1.1% and lithocholic acid (LA) 1.0 +/- 0.3% were also detected. Removal of cecum and appendix (group II) produced significant changes in biliary bile acid composition: DA fell to 58.3 +/- 31.8%, CA rose to 37.7 +/- 10.4%, and LA was barely detectable (0.3 +/- 0.1%). Administration of chenodeoxycholic acid (CDA), 125 mg/day, produced severe hepatotoxicity, reduced food intake, and produced weight loss (group III). Biliary LA rose to 15.0 +/- 1.3%, while DA was 61.3 +/- 14.0% and CDA 21.8 +/- 14.6% of total biliary bile acids. Feeding CDA to animals without cecum and appendix (group IV) resulted in a slight increase of LA (3.2 +/- 2.2%) compared to group III, and appreciable amounts of ursodeoxycholic acid (UDA) 32.0 +/- 9.8% and of 7-ketolithocholic acid (7-KLA) 3.0 +/- 0.6% appeared in bile. The animals of group IV exhibited no hepatotoxicity and ate and gained weight normally. These results indicate that the microbial population of cecum and appendix is active in 7alpha-dehydroxylation of primary bile acids and that removal of these organs results in an increased formation of UDA by an unknown mechanism.-Yahiro, K., T. Setoguchi, and T. Katsuki. Effect of cecum and appendix on 7alpha-dehydroxylation and 7beta-epimerization of chenodeoxycholic acid in the rabbit.

Animals↗

The vermiform appendix: not a useless organ.

The appendix has often been seen more as a nuisance rather than an important part of the human anatomy. Early misconceptions have led to the indiscriminate removal of the appendix from the body. Long thought to be an evolutionary remnant of little significance to normal physiology, the appendix has more recently been identified as an important component of mammalian mucosal immune function, particularly B-lymphocyte-mediated immune responses and extrathymically derived T-lymphocytes. This structure helps in the proper movement and removal of waste matter in the digestive system, contains lymphatic vessels that regulate pathogens, and lastly, might even produce early defences that prevent deadly diseases. The appendix is one of the guardians of the internal environment of the body from the hostile external environment.

Appendix↗

Gas in the appendix: a sometimes significant but nonspecific diagnostic sign.

The gas-filled appendix is an uncommon roentgenographic sign that has been considered to be virtually diagnostic of acute appendicitis when the appendix is positioned caudad to the cecum in the right lower quadrant of the abdomen. Three patients had gas in the appendix in the absence of acute appendicitis. This diagnostic sign is therefore not specific for appendicitis. Regardless of its location, the appendix may contain gas despite the absence of intrinsic disease.

Acute Disease↗

Experimental model for the study of the human appendix.

A simple and reproducible model for the experimental study of the normally functioning human appendix is described. The appendix removed from a human is transplanted into a nude mouse and is kept vital for at least 4 hours. This model is an improvement on previous models. It corresponds well with the working of the normal human appendix as regards tissue type and characteristics. Results are easily quantified and a paired control is possible in the experimental animal. This simplifies the experimental design and statistical calculations. The model can be used to gain additional information about the pathophysiology of the human appendix and the possible influence of drugs on this organ.

Animals↗

Germinal centers and the B-cell system. VI. Migration pattern of germinal-center cells of the rabbit appendix.

The migration pattern of germinal center cells of the rabbit appendix was studied and compared with that of appendix dome cells, spleen cells, thymus cells and thoracic duct lymphocytes. To discriminate T- and B-cell migration pathways, normal or T-cell-depleted rabbits were used as donors. Cell suspensions were labeled in vitro with 3H-leucine followed by intravenous transfer. The migration of labeled cells in lymphoid organs was studied using autoradiography, particular attention being paid to the spleen of the recipient. B-cells from the appendix dome, spleen and thoracic-duct lymph migrate to primary follicles or the corona of secondary follicles via thymus-dependent areas of peripheral lymphoid organs. In contrast, a B-cell subpopulation from the germinal centers of the appendix migrates to the center of splenic primary follicles and into germinal centers. The migration of germinal center cells to splenic follicle centers is not enhanced by specific antigens. The migration properties of B-cells, possibly changing during differentiation, may be instrumental in the two types of immune reactions, i.e., plasma-cell reaction and germinal-center reaction.

Animals↗

A case of cecocolic intussusception with complete invagination and intussusception of the appendix with villous adenoma.

Villous adenoma of the appendix is a rare neoplasm and intussusception of the appendix is a rare pathologic condition. A very rare case seen in a 35-year-old male with pain in the right lateral abdomen is reported. In this patient, the appendix along with the villous adenoma intussuscepted and invaginated into the cecal lumen, and presented as cecocolic intussusception. A polypoid lesion was diagnosed in the cecum by fiberoptic colonoscopy. Unlike polypoid lesions at other sites in the large intestine, polypoid lesions of the cecum may accompany intussusception and invagination of the appendix. Consequently, caution is required in performing endoscopic polypectomy in cases of polypoid lesions of the cecum.

Adenoma↗

Germinal centres and the B cell system. III. Germinal centre forming capacity of rabbit appendix germinal centre (-derived) cells.

Following appendectomy and sublethal (450 rads) whole body X-irradiation, reconstitution with autologous appendix germinal centre cells led to excellent germinal centre formation in the recipient's spleen. Heat killing of the cells to be injected completely abolished this phenomenon. Apparently a suspension of appendix germinal centre cells contains a (sub)populations of cells capable of germinal centre formation (Germinal Centre Precursor Cells). Following whole body X-irradiation while shielding the appendix, germinal centre activity could only be detected after antigenic stimulation in otherwise normally regenerated splenic follicular structures. This suggests that the cell population recently derived from appendix germinal centres is able to function once again as a population of germinal centre precursors.

Animals↗

[Perforated diverticulum of the vermiform appendix].

Diverticulitis of the vermiform appendix is an often disregarded disease. Diagnosis follows after appendectomy due to inflammatory complications in the form of diverticulitis and appendicitis. Diverticula can also be found as a reason for complaints in histologically unaltered appendix or as an incidental finding. Diverticula of the vermiform appendix are classified as false (acquired) or true (congenital). We report on the case of a 57-year-old man with a perforated appendix diverticulum as reason for peritonitis and a paralytic ileus of the colon and small intestine.

Appendectomy↗

Macroscopic assessment of the appendix at diagnostic laparoscopy is reliable.

BACKGROUND: Healthy-looking appendixes are often removed at laparoscopy for suspected appendicitis. This practice may have adverse secondary effects. METHODS: We reviewed the literature for the years 1978 to 1998 to analyze the negative appendectomy rates, complication rates, the accuracy of laparoscopic appendix assessment, and the incidence of false negative diagnosis of appendicitis at surgical and gynecological laparoscopy. RESULTS: The respective negative appendectomy rates were 22% and 15% in studies that compared laparoscopic with open appendectomy. The appendix was left in situ in 37% of 4,281 surgical diagnostic laparoscopies. There were instances of missed appendicitis among the 3,367 gynecological diagnostic laparoscopies performed on women for lower abdominal pain, and there were 188 appendectomies in this group. Studies comparing the macroscopic appearance of the appendix at operation with microscopic findings from the excised specimen had a false negative error rate of 3%. CONCLUSIONS: Contrary to general opinion, there is no substantial evidence to support the assumption that the macroscopic diagnosis of appendicitis is unreliable. High rates of conflicting diagnoses of excision specimens suggest that endoappendicitis has little clinical significance. At present, negative appendectomy rates are considerably higher for laparoscopic appendectomy than for the open approach. The role of diagnostic laparoscopy in suspected appendicitis should be reconsidered. It may be useful in particular subgroups of patients, but it is no substitute for good clinical judgment. Furthermore, it is not always necessary to perform an incidental appendectomy.

Appendectomy↗

CD5+ B cells are preferentially expanded in rabbit appendix: the role of CD5 in B cell development and selection.

Although only a small proportion of mouse and human B cells are CD5(+), most adult rabbit B cells express CD5. However, CD5 was not detectable on the majority of B cells in neonatal appendix 1 and 3days after birth. Cell trafficking studies demonstrated that CD5(+) and CD5(-) CD62L(+) B cells from bone marrow migrated into appendix. There, CD5(+) B cells were preferentially expanded and predominated by approximately 2weeks of age. In mutant ali/ali rabbits, VHa2(+) B cells develop through gene conversion-like alteration of rearranged VH genes upstream of deleted VH1a2. Correlated appearance of individual CD5(+) germinal centers and VHa2(+) B-cells in mutant appendix suggests that CD5 binding positively selects cells with a2(+) framework regions that bind CD5. Following negative and positive selection, cells with diversified rearranged heavy- and light-chain sequences exit appendix, migrate to peripheral tissues and constitute the preimmune repertoire of CD5(+) B cells that encounter foreign antigens.

Amino Acid Sequence↗

Sonographic detection of normal and abnormal appendix.

AIM: The aim of the study was to assess the value of sonography in detecting the normal appendix and in identifying abnormality. METHODS: The appendiceal wall thickness (normal: <3 mm) and ultraluminal contents (abnormal: large appendicolith, non-expressible fluid) were used as the primary criteria to determine the appendiceal status in 716 appendices. In patients who underwent appendicectomy (n = 166), surgical and histopathological findings were correlated with the ultrasound (US) findings; in patients who did not have surgery the reference standard was the clinical consensus based on follow-up. RESULTS: Thirty-four patients out of 179 with abnormal sonographic findings did not undergo appendicectomy and recovered spontaneously; in 22 of these, the US changes were confined to the appendiceal tip. A normal appendix was identified in 537 patients (45.9% of all patients without appendicitis), with histologic verification subsequently obtained in 21. In 76 normal appendices (14.2% out of all normal appendices), luminal dilatation due to non-expressible inspissated faeces resulted in appendiceal outer diameter >6 mm (range, 6.2-12 mm); a histopathologic proof of non-inflamed appendix was obtained in seven of these. CONCLUSIONS: A normal appendix can be visualized in a high percentage of cases and it may present with an outer diameter >6 mm (the widely-accepted upper limit of normal) due to the inspissated faecal material within the lumen. A significant percentage of early appendicitis can resolve spontaneously, especially when confined to the appendiceal tip.

Acute Disease↗

Characteristics and use of the in situ appendix as a continent catheterization stoma for continent urinary diversion in adults.

Use of the in situ appendix to provide continence was evaluated in 21 adults undergoing continent urinary diversion. The appendix with minimal or no manipulation was used in 20 of the 21 adults to provide the continence mechanism. Our findings indicate that in the majority of adults with no prior appendectomy the unaltered or minimally altered appendix is suitable to provide continence, and that dilating a narrow appendix is safe, effective and compatible with providing an adequate continence mechanism.

Adult↗

Goblet cell carcinoid of the appendix: Investigation of the expression of beta-catenin and E-cadherin.

Goblet cell carcinoids are rare neoplasms that predominantly occur in the appendix. In this report we present a case of goblet cell carcinoid of the appendix. A 58-year-old male patient complaining of pain in the right lower quadrant was diagnosed with acute appendicitis and underwent an appendectomy. Histological examination of the resected appendix revealed goblet cell carcinoid. Infiltration of tumor cells beyond the appendix was observed and the surgically resected margin was positive for tumor cells. Carcinoembryonic antigen (CEA) was diffusely detected by immunohistochemistry, and cytokeratin 20, neuron-specific enolase (NSE), chromogranin A and serotonin were focally observed in the tumor cells. The expression of beta-catenin and E-cadherin was investigated to compare with that of typical rectal carcinoids (n = 3) and colon adenocarcinomas (n = 3). In normal colonic and rectal mucosae, beta-catenin and E-cadherin stained positive on the plasma membrane. In the case reported here, beta-catenin showed a preserved expression on the plasma membrane of goblet cell carcinoid; a pattern similar to typical carcinoids rather than to adenocarcinomas. However, E-cadherin demonstrated a reduced expression on the plasma membrane of the tumor cells. This staining pattern was identical to those both of carcinoids and of adenocarcinomas. These findings suggest the possibility that, in some cases, the adherens junctions of goblet cell carcinoids are similar to those of typical carcinoids rather than to those of adenocarcinomas.

Appendix↗

Appendicitis and infections of the appendix.

The pathologic spectrum of the acutely inflamed appendix encompasses a wide range of infectious and noninfectious entities. The appendix suffers alone in some of these disorders, and in others may be involved through extension from other areas of the gastrointestinal tract. Although the appendix is the most commonly resected and examined intraabdominal organ, the pathogenesis and etiology of acute nonspecific appendicitis (the most common diagnosis made in this organ) remains enigmatic. This review encompasses the pathology, pathogenesis, and bacteriology of acute appendicitis, as well as controversial issues such as the diagnosis of chronic appendicitis and the significance of a morphologically unremarkable appendectomy specimen in the clinical context of appendicitis. In addition, the pathologic features, pertinent diagnostic techniques, and clinical significance of several specific bacterial, viral, fungal, and parasitic infections affecting the appendix are presented, including adenovirus, cytomegalovirus, Yersinia species, actinomycosis, Mycobacteria species, histoplasmosis, pinworms, schistosomiasis, and Strongyloides stercoralis.

Appendicitis↗

The use of pedicled appendix graft for substitution of urethra in recurrent urethral stricture.

BACKGROUND: Recurrent posterior urethral strictures after failed urethroplasty may need urethral substitution. Skin or mucosal grafts, currently used for this purpose, have a high complication rate. The authors describe the use of pedicled appendix for posterior urethral substitution. METHODS: Two boys with pelvic fracture urethral distraction injuries were treated for recurrent posterior urethral strictures after a failed perineal anastomotic urethroplasty. Through a perineal-transpubic approach the stricture tissue was excised, which resulted in a gap of 5 to 7 cm between the healthy ends. The vermiform appendix was mobilised on its own pedicle and transposed to the perineum; the proximal end of appendix was anastomosed to the prostatic urethra and the distal end (tip discarded) to the bulbar/penile urethra. Omentum was transposed to wrap the anastomosis and fill the dead space. RESULTS: Normal micturition was restored in both patients. No further treatment was required after 1 dilatation in the first case. Both patients are continent. Potency status remains unchanged from the preoperative period with normal erections in 1 case. Follow-up (1 to 3 years) has been satisfactory with no complications. CONCLUSIONS: The appendix is a promising organ for posterior urethral replacement. It can be brought to the perineum on its own vascular pedicle.

Adolescent↗