A tale of two cities: typical celiac sprue presenting symptoms are significantly more common in Turkish than in US Patients.

GOAL: Recent studies suggest an increasing prevalence of atypical initial symptoms in patients with celiac disease (CD). The aim of this study was to compare the presenting symptoms of CD at 2 comparable referral institutions in South Florida, and in Ankara, Turkey. We retrospectively reviewed the records of patients with an initial diagnosis of CD by small bowel biopsy during the same (1991 to 2001) 10-year period at Cleveland Clinic Florida and University of Ankara, Turkey. A numerical score was assigned to presenting symptom and signs. RESULTS: There were 28 patients in the South Florida group (51% men) and 40 patients in the Turkish group (35% men) (P=NS). The Turkish patients were significantly younger at diagnosis (36+/-2 y vs. US patients 66+/-3 y) (P<0.0001). Typical celiac symptom scores were similar (Turkey 1.1+/-0.2 vs. South Florida 1.3+/-0.3) (P=NS). Atypical symptom scores were significantly higher in the US patients (1.9+/-0.2) versus those from Turkey (1.2+/-0.1) (P<0.01). The Turkish patients were significantly more likely (P<0.01) to present with chronic diarrhea, hypoalbuminemia, and mactocytosis at the time of diagnosis. CONCLUSIONS: Although retrospective, this study points to striking differences in the clinical presentation of CD in individuals in South Florida, United States, and Ankara, Turkey, diagnosed during the same time period at both institutions. Turkish patients were younger, and more likely to present with chronic diarrhea, hypoalbuminemia, and microcytosis-as was more commonly seen in the United States in the 1960s and 70s. These findings raise the question of an interplay of demographics with diet and genetics in the presenting symptoms of CD in these 2 distant geographic areas.

Squamous cell carcinoma of the rectum in ulcerative colitis: case report and review of the literature.

The majority of colorectal carcinomas diagnosed are adenocarcinomas. Squamous cell carcinomas (SCC) of the rectum are rare tumors, and were reported as rare complication of inflammatory bowel disease. Surgery is the most effective therapy; and adjuvant chemotherapy and radiotherapy should also be considered. We report two cases of ulcerative colitis-associated SCC of the rectum. The lesions were treated with chemoradiotherapy with complete response.

Not all inflammation in the right lower quadrant is appendicitis: a case report of Escherichia coli O157:H7 with a review of the literature.

Although significant work has been presented on this subject in pediatric, infectious disease, and epidemiologic literature, there is a noteworthy lack of information on Escherichia coli O157:H7 in any surgical journals. As this disease can present with signs and symptoms often ascribed to the acute abdomen, it is imperative that the general surgeon, pediatric surgeon, and colorectal surgeon are all familiar with this infection and its clinical ramifications. A case report followed by a review of the literature is presented.

Urinary retention caused by adenocarcinoma arising in recurrent urethral diverticulum.

We present a case of adenocarcinoma arising in a urethral diverticulum. A 71-year-old woman presented with urinary retention and a mass in the anterior vaginal wall. A biopsy was performed and revealed adenocarcinoma. Anterior pelvic exenteration with continent urinary diversion was performed followed by adjuvant radiation therapy as the tumor was large and poorly differentiated. She developed stomal stenosis and underwent stomal revision. The patient has done well since, with no evidence of recurrence at 1-year follow-up at the time of this report.

To err is human: improving patient safety through failure mode and effect analysis.

Patient care errors occur in the laboratory. Traditionally, most errors have been thought to occur because of individual human failure. The assumption is that with adequate training, education; and orientation, technologists will perform flawlessly. Laboratory processes are designed on the premise that nothing will go wrong. Health-care professionals are looking at new methods of error prevention including Failure Mode and Effect Analysis (FMEA). Based on long experience in the engineering field, FMEA assumes everything will fail, humans err frequently, and the cause of an error often is beyond the individual's control. FMEA is a proactive, systematic, multidisciplinary team-based approach to error prevention. Patient safety is now a high priority with the Joint Commission on Accreditation of Healthcare Organizations, and this article introduces FMEA, a new method for improving our processes to enhance patient safety.

Abnormality of peptide YY and pancreatic polypeptide immunoreactive cells in colonic mucosa of patients with colonic inertia.

The etiopathology of colonic inertia remains unclear. Current studies show that pancreatic polypeptide-fold family members can serve as regulators of colonic motility and transit. Thus, the cells containing these peptides on colonic mucosa could be abnormal in patients with colonic inertia. We aimed to evaluate the immunocytochemical staining of peptide YY (PYY) and pancreatic polypeptide (PP) immunoreactive cells, and detect if alteration of these cells relates to an increase in enterochromaffin cells (EC) demonstrated by chromogranin A (CgA), in the colonic mucosa of patients with colonic inertia. Nineteen consecutive patients (18 female, 1 male; age, 43.7+/-11.5 years) who underwent subtotal colectomy for colonic inertia were assessed. The control group consisted of 15 patients (all female; age, 50.7+/-12.5 years) who underwent colonoscopic biopsies from the right and left colon for indications other than constipation, inflammatory bowel diseases, diarrhea, or neoplasm. Hollande's-fixed, paraffin-embedded tissues of both right and left colons were collected. Immunocytochemical staining of PYY, PP, or CgA was performed on 4-microm tissue sections with the respective primary rabbit antibody, the biotinylated secondary antibody, and enzyme-labeled streptavidin. The average number of positive cells per microscopic field (200x) was calculated. Positive cells were classified as strongly, moderately, and weakly staining. The proportion of the variously stained cells is expressed as the percentage of the entire positive cell population. On both sides of the colon, the percentages of strongly and moderately stained PYY positive cells were higher in the patient group compared to the controls (right side, 10.6 and 27.3 vs. 6.1 and 18.7%, respectively; left side, 9.4 and 23.9 vs. 6.2 and 23.1%, respectively) (P < 0.01). Furthermore, in the patients with colonic inertia, the percentages of strongly and moderately stained PYY-positive cells were higher in the right-side colon than in the left (P < 0.01). There was no difference in the number of PYY-positive cells between the patients and the controls. PP-positive cells were very rare in all specimens and were found in 7 of 19 cases (36.84%) in the right-side colon and 16 of 19 (84.21%) in the left-side colon in the patient group (P < 0.01, left vs. right). In contrast, the number of EC in the left colon of patients (16.8+/-10.2) was significantly higher than that in the right side (9.4+/-6.0) (P < 0.01) or that in the left side in the control group (10.4+/-6.0) (P < 0.05). We conclude that in the colonic mucosa of patients with colonic inertia, PYY-positive cells present with higher immunoreactivity, indicating that they may contain more hormones, especially on the right side of the colon. However, the PPY- and PP-positive cells did not relate to the increased EC. and It is therefore suggested that the altered PYY in the colonic mucosa may partially contribute to the etiopathology of colonic inertia.

Juxta-articular myxoma: a rare cause of painful restricted motion of the knee.

A 68-year-old athletic woman presented to our institution in January 2002 with a several-month history of progressing complaints of pain, swelling, and loss of motion in the right knee. These manifestations had begun the previous July during a game of tennis. She experienced persisting pain and recurring effusions. Because the patient had been residing in another state between July and January, rheumatologic and orthopaedic evaluations of the knee, including a magnetic resonance imaging (MRI), had been performed at a geographically distant (but affiliated) institution. The resulting presumptive diagnosis was a "wear and tear" degenerative articular disorder of the knee. A program of anti-inflammatory medication and physical therapy was begun for several months but produced no therapeutic benefit by the time the patient presented at our institution. After examination confirmed marked losses of both flexion and extension of the knee, effusion, and exquisite medial joint tenderness, an MRI was repeated, using intra-articular gadolinium as a contrast agent. It revealed an intra-articular mass encircling the medial and posterior extents of the medial femoral condyle. An arthroscopic multiportal excisional biopsy was performed. It revealed the existence of a juxta-articular myxoma. The patient recovered most of the range of motion during the next several months, and the effusion and severe pain gradually dissipated. The patient was subsequently followed by sequential physical examinations and MRIs, performed at increasing intervals of time, without recurrence of a mass or of her flagrant symptoms in the first year post surgery. Though the patient's diagnosis was established and treatment outcome was satisfactory, many issues were brought up in this case regarding most appropriate selection of diagnostic tests and treatment approaches.

Interobserver and intraobserver bias exists in the interpretation of anal dysplasia.

INTRODUCTION: Natural history of progression from anal intraepithelial neoplasia to invasive carcinoma remains unproven. The risk of progression may be linked to the severity of dysplasia. Important therapeutic decisions are thus based on the severity of anal intraepithelial neoplasia. Consistency and reliability in the interpretation of anal intraepithelial neoplasia are unproven. METHODS: One hundred ninety anal biopsy specimens were identified for review of dysplasia with a six-point grade system from normal to invasive cancer, evidence of human papillomavirus infection, and quality of histology by three pathologists. RESULTS: Results revealed poor to moderate agreement on grading of quality of histology (weighted kappa score, 0.07-0.22), human papillomavirus status (weighted kappa score, 0.24-0.53), and dysplasia (weighted kappa score, 0.38-0.7). Complete agreement between the original pathology and the three pathologists was observed in only 32 percent of cases. Analysis of 86 slides previously read by one of the pathologists revealed only moderate agreement, with a weighted kappa score of 0.64. CONCLUSION: Significant interobserver and intraobserver bias exists in the interpretation of anal intraepithelial neoplasia. These inconsistencies may explain the uncertainty about the natural progression of anal intraepithelial neoplasia and the varied results of surgery reported for anal intraepithelial neoplasia in the literature.

Are women with urogenital atrophy symptomatic?

OBJECTIVE: The purpose of this study was to determine the degree of correlation between physical signs of genital atrophy and symptoms that are suggestive of atrophic vaginitis. STUDY DESIGN: Female volunteers (n = 135; mean age, 69 years) rated the presence and severity (rating, 0-3) of vaginal atrophy symptoms. The presence and severity of vaginal mucosal changes, which included vaginal pH (0-3), were recorded during a pelvic examination. A vaginal cytologic maturation value was performed. Symptoms, signs, pH, and maturation value were correlated by the Spearman rank test. RESULTS: Symptom scores were low (mean, 0.41; range, 0-2.6). Symptoms were only weakly correlated with physical findings (r = 0.14) and not with maturation value (r = 0.06) or age (r = -0.004). There was a moderate correlation between physical examination score and maturation value (r = -0.48). In women > or =65 years old, symptom score and physical examination score were correlated weakly (r = 0.25). Low pH correlated well with high maturation value (r = -0.52). Women who were undergoing estrogen therapy had higher symptoms scores (P =.0007) and maturation values (P =.0002) than women who were not undergoing therapy. CONCLUSION: Although urogenital atrophy occurs universally after menopause, most elderly women are minimally symptomatic. Those women on estrogen replacement therapy may be more symptomatic. Symptoms alone should not be used as a guide for the initiation of estrogen therapy.

Reduced expression of serotonin receptor(s) in the left colon of patients with colonic inertia.

PURPOSE: Serotonin regulates colonic motility receptors expressed on neural fibers and smooth muscle. Colonic inertia is characterized by delayed colonic transit. Abnormalities in serotonin receptor protein, as judged by immunoreactivity levels, could contribute to the origin of colonic inertia. The aim of this study was to investigate the expression of serotonin receptor(s) immunoreactivity in the left colon of patients with colonic inertia compared with controls. METHODS: Sixteen patients who underwent subtotal colectomy for colonic inertia were assessed. Colonic transit time was measured with the radiopaque marker technique and presented as the number of retained markers in the colon on Day 5. The control group consisted of 18 patients who underwent left hemicolectomy for colonic carcinoma; histologically normal tissues from the left colon were used. Immunohistochemical staining for serotonin receptor was performed with a rabbit anti-idiotypic antibody. The average positive area (square pixels) in the mucosa, muscularis mucosa, submucosa, and circular and longitudinal muscles per microscopic field (63x) was calculated based on measurement of the positively stained area in 20 randomly chosen microscopic fields in each related structure. The Scion Image computer analysis system was used. RESULTS: Serotonin receptor(s) immunoreactivity was mainly detected in the muscular mucosa, circular muscles, and longitudinal muscles and rarely in the mucosa and submucosa. In muscularis mucosa and circular muscle, the positive areas were significantly less in the colonic inertia group than in controls (muscularis mucosa: 29.1 +/- 10.8 vs 109.7 +/- 28.2, P < 0.05; circular muscle: 25.6 +/- 6.2 vs 90.2 +/- 19.1, P < 0.01). There were significantly positive correlations in the control group in serotonin receptor(s) immunoreactivity levels between circular muscle and longitudinal muscle (r = 0.54, P < 0.05) and between muscular mucosa and longitudinal muscle (r = 0.57, P < 0.05) but not in colonic inertia patients. In addition, the positive areas in the circular muscle were positively correlated to the colonic transit time (Spearman's rank correlation, 0.83; P < 0.01). CONCLUSION: In colonic inertia patients, the serotonin receptor(s) immunoreactivity level is lower in muscular mucosa and circular muscle. The absence of a correlation of serotonin receptor(s) immunoreactivity in the muscular mucosa and muscularis propria in the patient group implies that an uncoordinated expression of serotonin receptors may also contribute to colonic inertia. However, the positive correlation between serotonin receptor(s) immunoreactivity levels in the circular muscle and the transit time observed in colonic inertia patients suggests a decrease in stimulatory subtypes and at the same time an increase in inhibitory subtypes of serotonin receptors in this tissue.

Moving your laboratory? 10 keys to a successful transition.

We all have been there. Moving, whether it is from one state or town, or just across the street, can be chaotic and exhausting. Our possessions are placed in a box, loaded with our furniture into a big truck, and transported from one site to another. Most of us use this opportunity to sort through old papers, drawers, and cabinets, cleaning and throwing out items we no longer will use. If we are well organized, things can go smoothly. However, even for the most organized person, items get lost or misplaced, tempers get short, and we are disoriented for a few days or weeks until everything gets into place and we can reestablish our routines. In the summer of 2001, Cleveland Clinic Florida moved their laboratory. This article outlines the clinic's experience, offering suggestions for when you face your own moving day.

Collision of a syncytial meningioma and malignant astrocytoma.

The coexistence of two or more primary brain tumors is a relatively rare occurrence. We report an 87-year-old woman with a 5-cm cystic-solid, peripherally enhancing, right frontal lobe mass. The woman presented with progressive hemiparesis, difficulty ambulating, and urinary incontinence. Histologic examination of the surgically excised lesion showed geographically distinct areas of syncytial meningioma (World Health Organization grade I) and malignant astrocytoma (World Health Organization grade III). Focally, infiltration of the meningioma by astrocytoma was observed. Differential diagnostic issues are considered and the literature reviewed.