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Biomedical subjects

P H R Green

Publications and source records attributed to P H R Green.

10 recordsLinked to original sources

Lack of correlation of degree of villous atrophy with severity of clinical presentation of coeliac disease.

BACKGROUND AND AIM: Both the clinical presentation and the degree of mucosal damage in coeliac disease vary greatly. In view of conflicting information as to whether the mode of presentation correlates with the degree of villous atrophy, we reviewed a large cohort of patients with coeliac disease. PATIENTS AND METHODS: We correlated mode of presentation (classical, diarrhoea predominant or atypical/silent) with histology of duodenal biopsies and examined their trends over time. RESULTS: The cohort consisted of 499 adults, mean age 44.1 years, 68% females. The majority had silent coeliac disease (56%) and total villous atrophy (65%). There was no correlation of mode of presentation with the degree of villous atrophy (p=0.25). Sixty-eight percent of females and 58% of males had a severe villous atrophy (p=0.052). There was a significant trend over time for a greater proportion of patients presenting as atypical/silent coeliac disease and having partial villous atrophy, though the majority still had total villous atrophy. CONCLUSIONS: Among our patients the degree of villous atrophy in duodenal biopsies did not correlate with the mode of presentation, indicating that factors other than the degree of villous atrophy must account for diarrhoea in coeliac disease.

Adult↗

Multifocal axonal polyneuropathy in celiac disease.

The authors report six patients with multifocal axonal polyneuropathy and the subsequent diagnosis of celiac disease (CD). Five patients did not improve or had only modest improvement following dietary intervention or immune therapies; one patient with marked weakness and mild electrodiagnostic findings had complete resolution of the neuropathy following immunomodulatory therapy. CD may be a cause of multifocal axonal polyneuropathy.

Adult↗

Mechanisms underlying celiac disease and its neurologic manifestations.

The extra-intestinal manifestations of celiac disease (CD), including ataxia and peripheral neuropathy, are increasingly being recognized as the presenting symptoms of this autoimmune disease. Although there is a greater understanding of the pathogenesis of the intestinal lesions in CD the mechanisms behind the neurologic manifestations of CD have not been elucidated. In this article, the authors review the cellular and molecular mechanisms behind the histopathologic changes in the intestine, discuss the presentation and characteristics of neurologic manifestations of CD, review the data on the mechanisms behind these manifestations, and discuss the diagnosis and treatment of CD. Molecular mimicry and intermolecular help may play a role in the development of neurologic complications.

Autoimmune Diseases↗

Celiac neuropathy.

BACKGROUND: Celiac disease (CD) is a chronic inflammatory enteropathy resulting from sensitivity to ingested gluten. Neurologic complications are estimated to occur in 10% of affected patients, with ataxia and peripheral neuropathy being the most common problems. The incidence and clinical presentation of patients with CD-associated peripheral neuropathy have not previously been investigated. OBJECTIVE: To determine the incidence of CD in patients with neuropathy and to characterize the clinical presentation. METHODS: The records of 20 patients with neuropathy and biopsy-confirmed CD were reviewed. RESULTS: Six of the 20 patients had neuropathic symptoms alone without gastrointestinal involvement, and neuropathic symptoms preceded other CD symptoms in another 3 patients. All patients had burning, tingling, and numbness in their hands and feet, with distal sensory loss, and nine had diffuse paresthesias involving the face, trunk, or lumbosacral region. Only two had weakness. Results of electrophysiologic studies were normal or mildly abnormal in 18 (90%) of the patients. Sural nerve biopsies, obtained from three patients, revealed mild to severe axonopathy. Using the agglutination assay, 13 (65%) of the patients were positive for ganglioside antibodies. Excluding patients who were referred with the diagnosis of celiac neuropathy, CD was seen in approximately 2.5% of all neuropathy patients and in 8% of patients with neuropathy and normal electrophysiologic studies seen at our center. CONCLUSION: CD is commonly associated with sensory neuropathy and should be considered even in the absence of gastrointestinal symptoms.

Adult↗

Risk of duodenal adenoma in celiac disease.

BACKGROUND: There is a 60- to 80-fold increased risk of small-bowel adenocarcinoma in patients with celiac disease. While the adenoma-carcinoma sequence appears to operate in the small bowel as in the large bowel, the risk of duodenal adenomas in celiac patients is unknown. METHODS: The records of 381 patients (245 F, 136 M) with biopsy-proven celiac disease were reviewed to determine the prevalence of duodenal adenoma found during esophagogastroduodenoscopy (EGD). We conducted an extensive literature review to find data for estimates of the prevalence of duodenal adenoma in a comparable general population; we used data from a study at another New York City medical center of 7346 EGDs conducted between 1976 and 1982 (Ghazi et al., 1984). We estimated the relative risk, expressed as a standard morbidity ratio (SMR), by calculating the observed to expected (O/E) ratio. RESULTS: Duodenal adenomas were found in 3 celiac patients (0.78%), with 24 adenomas (0.33%) in the reference population, giving an SMR of 2.39 (95% CI 0.67-8.48). CONCLUSION: We did not find a significantly increased risk of duodenal adenoma in celiac patients compared to a non-celiac endoscoped population. Thus, despite the previously described elevated risk of small-bowel adenocarcinoma in these patients, routine endoscopic examination of the duodenum may not be adequate for screening.

Adenoma↗

Small bowel neoplasia in coeliac disease.

There is an increased risk of small bowel adenocarcinoma in patients with coeliac disease compared with the normal population. It has been suggested that adenocarcinoma of the small intestine in coeliac disease arises through an adenoma-carcinoma sequence but there has been only one reported case of a small bowel adenoma in a patient with coeliac disease. We report three additional cases of a small bowel adenoma in the setting of coeliac disease. In addition, four cases of small bowel adenocarcinoma are also reported, one of which was found adjacent to a jejunal villous adenoma. These cases emphasise the risk of the development of small bowel neoplasia for patients with coeliac disease and support the concept that small bowel adenocarcinoma in coeliac disease arises from adenomas.

Adenocarcinoma↗