Luis A. Boerr

Gynaecological and obstetric disorders in coeliac disease: frequent clinical onset during pregnancy or the puerperium.

Background and aim While gynaecological and obstetric disorders have been reported among women with coeliac sprue, their true prevalence and relationship to the coeliac disease process has not been completely elucidated. Our aims were to determine: (1) the prevalence of gynaecological and obstetric problems in patients with coeliac disease and the influence of strict gluten restriction on their occurrence, (2) the effect of pregnancy on the clinical course of coeliac disease and (3) the clinical features of those patients with onset of coeliac disease during pregnancy and the puerperium. Patients and methods The gynaecological and obstetric history of 130 coeliac patients and 130 age-matched healthy female controls were compared in a case-control study. Results In comparison to the controls, untreated coeliac disease patients exhibited significantly later menarche, an earlier menopause, an increased prevalence of secondary amenorrhoea and a greater incidence of spontaneous abortions. Patients who had adhered, in the long term, to a gluten-free diet had gynaecological and obstetric history indistinguishable from controls. Clinical deterioration of coeliac disease was observed in untreated patients during 17% of their pregnancies. In 14% of those untreated patients who were pregnant symptoms related to coeliac disease were manifested for the first time during either pregnancy (n = 7) or the puerperium (n = 4). Nine of these patients had underestimated features suggestive of coeliac disease. Conclusion The early diagnosis and treatment of coeliac disease may avoid significant gynaecological and obstetric complications in affected women. Celiac sprue must always be borne in mind among patients who develop diarrhoea and weight loss during pregnancy and/or the puerperium.

Value of endoscopic markers in celiac disease

Duodenoscopy in celiac disease has identified several markers of the disease. Our aim was to evaluate, in a prospective study, the usefulness of the different endoscopic features in 100 consecutive cases referred to endoscopy for intestinal biopsy. Histological examination of duodenal samples showed severe villous atrophy (grade III/IV) in 36 patients. Of these patients, 34 had endoscopic markers suggestive of celiac disease. These were reduction in number or loss of Kerkrings folds (in 27), mosaic pattern (14), scalloped folds (12), and visibility of the underlying blood vessels (5). Endoscopic visualization of these markers had a sensitivity of 94%, a specificity of 92%, and a positive predictive value of 84%. Reduction in number, or loss of, Kerkrings folds was the most sensitive (76%) and specific (98%) single endoscopic change indicating celiac disease. Duodenoscopy permitted diagnosis in three of four asymptomatic patients in a group of 24 first-degree relatives of celiac disease patients. We conclude that endoscopy of distal duodenum is a sensitive and specific indicator of celiac disease.

Successful treatment of retractile mesenteritis with oral progesterone

Retractile mesenteritis is a rare inflammatory mesenteric disorder that involves the intestine secondarily. The natural history of this process is diverse, but most patients require some empiric therapeutic measures. Up to now, pharmacological therapy has included corticosteroids, colchicine, and immunosuppressive drugs. Although these drugs are successful in most patients, some have been refractory to these therapies and, in others, the beneficial effects were counterbalanced by adverse reactions. Many patients require surgery, but most have poor results. This report describes a 42-year-old man with histologically proven retractile mesenteritis refractory to surgical intervention who had a good response to oral progesterone (10 mg/day for 6 months) with complete disappearance of tumor mass and clinical symptoms. No adverse effects were detected. Current knowledge about the mechanism by which progesterone affects fibrogenesis is scanty. It seems likely that progesterone down-regulates proliferation and metabolism of fibroblasts and fibrogenesis.

High frequencies of telomeric associations, chromosome aberrations, and sister chromatid exchanges in ulcerative colitis

OBJECTIVE:Chromosome instability provides a predisposing background to malignancy, contributing to the crucial genetic changes in multistep carcinogenesis. The aim of this work was to analyze chromosome instability in patients with ulcerative colitis (UC) to achieve a better understanding of the increased risk for colorectal cancer.METHODS:Peripheral blood lymphocyte cultures from 20 untreated UC patients and 24 controls were used to study chromosome instability by assessing telomeric associations (TAS), chromosome aberrations (CA), and sister chromatid exchanges (SCE).RESULTS:Mean frequencies of TAS and CA were significantly increased in UC patients compared to controls (p < 0.001). Chromosomes 10, 11, 21, 16, and 19 were the most frequently involved in TAS. A total of 104 CA clustered in 66 breakpoints could be exactly localized. Seven nonrandom bands significantly affected in UC patients were found (p < 0.004), showing a significant correlation with the location of cancer breakpoints (p < 0.003), particularly with colorectal carcinoma rearrangements. SCE analysis showed higher levels in patients compared to controls (p < 0.006), but no differences were observed in cell cycle kinetics.CONCLUSIONS:Our results demonstrate the presence of an unstable genome in UC patients that could be related to the cancer development observed in this disease.

Superior mesenteric artery blood flow in celiac disease

Measurements of the hemodynamic parameters of the superior mesenteric artery were performed in 18 patients with celiac disease. Ten were studied at the time of diagnosis, when a small bowel biopsy showed a flat mucosa. The remaining eight patients were studied after complete clinical and histological recovery induced by a gluten-free diet. Doppler ultrasound flowmetry was used to measure blood flow in physiological and fasting conditions and after a mixed liquid test meal (Ensure-Plus). The results were compared with those of healthy subjects (N=7). Mean basal flow was 50% higher in untreated celiac disease patients than in healthy controls and patients with chronic pancreatitis (P=NS). Postprandial mesenteric blood flow was significantly increased (P<0.002) and delayed in time (P<0.005) in celiac disease as compared to controls. Successful treatment reduced the mesenteric blood flow in celiac disease to normal values. Our study demonstrates that pathophysiological changes in the small bowel mucosa during the active clinical phase of celiac disease induce an abnormal splanchnic circulation.

Gluten sensitivity in patients with primary biliary cirrhosis.

Objective:Whereas celiac disease and primary biliary cirrhosis have been reported to coexist in the same patient, the frequency of this relationship has not been clarified. Nowadays, the concept of celiac disease has been extended from that of a severe enteropathy to a broader concept of gluten-driven intestinal immunological response. In this study we assessed features of gluten sensitivity in a cohort of patients with primary biliary cirrhosis.Methods:Ten patients with primary biliary cirrhosis were evaluated a mean of 2 yr after diagnosis. The following features of gluten sensitivity were assessed: serum antigliadin and endomysial antibodies, small bowel histology (degree of atrophy and quantitative histological parameters), the presence of the typical celiac HLA genotype (DQ2), and intraepithelial lymphocyte response in the rectal mucosa after local gluten instillation (rectal gluten challenge).Results:Overall, three patients presented evidence of gluten sensitivity. AH three had abnormal liters of antigliadin antibody type IgA and one was positive for endomysial antibody. Two patients had partial villous atrophy. The rectal gluten challenge showed a celiac-like response, evidenced by an increase in intraepithelial lymphocyte infiltration after gluten exposure, in the three patients. The characteristic celiac HLA genotypes (DQA1 0501 and DQB1 0201) were identified in three patients. One of them also exhibited other features of gluten sensitivity. However, despite evidence of gluten intolerance, patients had minimal or no symptoms characteristic of celiac disease.Conclusion:We detected features of gluten sensitivity in a high proportion of patients with primary biliary cirrhosis. Further studies should be performed to elucidate the clinical significance of this association.

Short-term antibiotic treatment in Whipple's disease.

We report the results of short-term antibiotic treatment in 19 patients with Whipples disease (WD). The diagnosis was based on clinical features and on a characteristic small bowel biopsy. Patients received treatment for a mean of 7.9 weeks (range 4-20). Fourteen were treated with de-methyl-chlortetracycline (600 mg/day), and 1 also received chloramphenicol (1 g/day); 1 was treated with ampicillin (2 g/day), and 4 were treated with amoxicillin (1.5 g/day). In all patients, the clinical response was rapid and excellent, body weight increased significantly, diarrhea subsided, and fecal fat values returned to normal. Intestinal biopsies obtained after treatment was completed showed significant improvement based on a decrease in the number of macrophages staining positive with periodic acid-Schiff (PAS), normalization of villous structure, and decreased dilatation of lymphatic channels; free bacilli were absent, as shown both by light and electron microscopy. Seventeen patients have been followed for a mean of 99.4 months (range 6-300). Two died 30 and 72 months after diagnosis of Whipples disease, 1 of laryngeal carcinoma and the other of colonic carcinoma. Fifteen patients are in excellent health. Three patients treated with tetracycline have had clinical and/or histologic relapses. In our experience, short-course antibiotic treatment with tetracycline or ampicillin and derivatives can be effective in WD, with few relapses and excellent outcome. No neurologic symptoms, either initially or during follow-up were observed.

Celiac sprue after surgery of the upper gastrointestinal tract : report of 10 patients with special attention to diagnosis, clinical behavior, and follow up

The clinical onset of celiac sprue (CS) may be precipitated by upper digestive tract surgery. We report a series of 10 patients who developed CS after diverse types of peptic ulcer surgery. Six were male and 4 female. Gastrectomy with Billroth II anastomosis was performed in 5 patients, truncal vagotomy and pyloroplasty in 2, parietal cell vagotomy and pyloroplasty in 1, and vagotomy with gastrojejunal anastomosis in 2. We found that eight patients had had previous symptoms that suggested CS. Symptoms occurred early in the postoperative period. Severe diarrhea and striking weight loss were the most prominent clinical findings. The response to gluten-free diet was independent of the type of surgical procedure performed and was similar to that observed in the general celiac population.

Increased mucosal levels of leukotriene B4 in pouchitis : evidence for a persistent inflammatory state

Design and Methods In order to evaluate its possible role in the pathogenesis of pouchitis we measured the release, into the incubation medium, of leukotriene B4 from mucosal samples from patients with ileal pouch-anal anastomosis and correlated release with clinical, endoscopic and histological features. Results Leukotriene B4 release was significantly elevated in patients with active pouchitis in comparison to those with a normal pouch mucosa (P<0.007). No overlap was observed between leukotriene B4 levels from patients with active pouchitis samples and those obtained from individuals without-pouchitis. Effective treatment of pouchitis was associated with a significant reduction in leukotriene B4 mucosal release to the incubation medium (P<0.03). However, even in remission, levels of leukotriene B4 release remained significantly increased in these patients in comparison to people who never experienced pouchitis (P< 0.003). A modest correlation was observed between pouchitis disease activity index and leukotriene B4 release (r = 0.596; P< 0.01). Conclusion These results suggest that the increased production of leukotriene B4 may be implicated in the pathogenesis of pouchitis. The persistence of an increased mucosal release of leukotriene B4 in pouchitis patients during clinical remission suggests the presence of a chronic, ongoing, underlying inflammatory process.

Steatocrit: a reliable semiquantitative method for detection of steatorrhea.

Steatocrit is a semiquantitative method for determination of fat content in fecal samples. Previous studies, mostly performed in children, reported controversial results. The aim of our study was to compare the determination of fat content in 148 fecal samples by two methods: the conventional van de Kamer and the steatocrit. Seventy-seven fecal samples had steatorrhea (> 7 g/day). The upper normal limit for the steatocrit (determined by the mean +/- 2 SD of samples without steatorrhea) was 2.1%. The steatocrit showed a sensitivity of 87%, specificity of 97%, and positive and negative predictive values of 97 and 87%, respectively. When fecal fat excretion > 20 g/day was evaluated, sensitivity increased to 98%. A significant linear correlation was found between steatocrit and the quantitative chemical method (r = 0.80; p < 0.0001). In conclusion, the steatocrit is satisfactory in the discrimination of patients with and without fat malabsorption. It is a simple, rapid, inexpensive, and reliable semiquantitative test that can be used when other methods are impractical.