Italian journal of gastroenterology and hepatology最新文献

A 44-year-old Caucasian male who had been on long-term steroid treatment for an unspecified collagen disease was referred to our Unit because of fever, severe hypopharyngeal dysphagia, night sweats and evidence of marked superior vena cava compression. Extrapulmonary disseminated tuberculosis also involving bone and liver was eventually diagnosed and proven by means of specific polymerase chain reaction assay. Antimycobacterial treatment, which led to a dramatic improvement within two months, was protracted for 18 months until the complete return to normal of both clinical and laboratory findings.

Background: Neuropathy of the pudendal nerves which may be found in constipated patients has been considered the result of pelvic floor descent due to the repetitive acts of straining at stool. However, the relationship between abdominopelvic dyssynergia, which may lead to repetitive acts of straining and neurophysiopathologic alterations of the pelvic floor has not yet been fully elucidated.

Aim: Of this study was to assess the relationship between neurophysiologic alterations of the external anal sphincter, patterns of altered evacuation and defaecographic pelvic floor physiology in 32 patients with chronic idiopathic constipation.

Results: At electromyography partial muscle denervation, identified as chronic neurogenic lesions of the external anal sphincter, were found in 19% and dyssynergia (co-contraction of external anal sphincter and abdominal muscles) in 34% of the investigated subjects. Patients with different electromyography patterns did not differ as far as concerns symptoms of altered evacuation, bowel frequency, use of digital manoeuvres, age, and duration of symptoms. The presence of neurophysiologic alterations was significantly associated with altered defaecographic findings: reduced ano-rectal angle at rest in chronic neurogenic lesions and abdomino-pelvic dyssynergia (p < 0.01); excessive pelvic floor descent in the presence of chronic neurogenic lesions (p < 0.05).

Conclusions: In chronically constipated patients symptoms of altered defaecation do not appear to be related to abdomino-pelvic dyssynergia and/or chronic neurogenic lesion of the external anal sphincter and do not show any association with defaecographic alterations. These results suggest that straining at evacuation can be induced by additional factors other than abdomino-pelvic dyssynergia and chronic neurogenic lesions and that these two alterations have different pathogenetic mechanisms.

Peptide receptor scintigraphy with [111In-DTPA-D-Phe1]-octreotide is a sensitive and specific technique to show in vivo the presence and abundance of somatostatin receptors on various tumours. With this technique primary tumours and metastases of neuroendocrine cancers as well as of many other cancer types can be localised. This technique is currently used to assess the possibility of peptide receptor radionuclide therapy with repeated administrations of high doses of [111In-DTPA-D-Phe1]-octreotide. 111In emits Auger and conversion electrons having a tissue penetration of 0.02 to 10 microns and 200 to 500 microns, respectively. Twenty end-stage patients, mostly with neuroendocrine progressing tumours, were treated with [111In-DTPA-D-Phe1]-octreotide, up to a maximal cumulative patient dose of about 74 GBq, in a phase I trial. Results showed there were no major clinical side-effects after up to 2 years treatment, except that in a few patients a transient decline in platelet counts and lymphocyte subsets occurred. Promising beneficial effects on clinical symptoms, hormone production and tumour proliferation were found. Of the 16 patients who received a cumulative dose of more than 20 GBq, 5 patients showed stabilisation of disease and 5 other patients a reduction in size of tumours. There is a tendency towards better results in patients whose tumours have a higher accumulation of the radioligand. In conclusion, peptide receptor radionuclide therapy is feasible, also with 111In as radionuclide. Theoretically, depending on the homogeneity of distribution of tumour cells expressing peptide receptors, beta-emitting radionuclides, e.g. 90Y, labelled to DOTA-chelated peptides may be more effective than 111In for peptide receptor radionuclide therapy. The first peptide receptor radionuclide therapy trials with [90Y-DOTA-Tyr3]-octreotide started recently.

Surgery still plays an important role even in advanced endocrine tumours of the pancreas, owing to their biological behaviour. Sometimes it is possible to attempt a radical approach, but more often only cytoreduction is feasible. In fact, when the malignancy is not completely resectable on account of vessel involvement or extensive liver metastases, surgical reduction of the tumour burden (debulking) can be proposed, aimed at improving the clinical conditions and survival of these patients. Forty-one patients suffering from advanced endocrine tumour of the pancreas were observed from 1985 to 1996. In 13 patients, the disease was locally advanced as far as concerns lymph node metastases and/or vessel involvement, while the other 28 patients presented liver metastases. In the former group, we performed 6 radical resections, in the latter we submitted 2 patients to radical resection and 12 patients to cytoreductive surgery, with complete removal of the pancreatic malignancy. The overall survival of the resected patients was 87% (7/8). Three patients (37.5%) are alive and free of disease, while the other 4 have subsequently developed liver metastases. One patient died with hepatic recurrence. Half the patients (6/12) undergoing cytoreductive surgery are alive, 3 with stable and 3 with progressive disease. The other 6 patients have died due to liver progression of the disease. As data in the literature concerning the role of debulking as regards the survival are conflicting, we have modified our surgical approach in patients with advanced disease. We perform cytoreductive surgery whenever complete removal of the pancreatic tumour is feasible. The rationale of this approach is to leave only a liver with residual disease, with a view to giving targeted adjuvant treatment.

Recent studies report that the radiolabelled synthetic somatostatin analogue, [111In-DTPA-DPhe1]octreotide, is useful for imaging carcinoid tumours and pancreatic endocrine tumours. At present, it is unclear whether this method is superior to conventional imaging studies (computed tomography, magnetic resonance imaging, ultrasound, angiography) and what its role should be, if any, in the management of these patients. The aim of this paper is to review five recent studies performed at the National Institutes of Health in patients with Zollinger-Ellison syndrome to define the role of somatostatin receptor scintigraphy. Patients were from a tertiary referral centre, all had Zollinger-Ellison syndrome. In Study n. 1: the sensitivity of somatostatin receptor scintigraphy was assessed compared to conventional studies in 80 patients. Study n. 2: the effect of somatostatin receptor scintigraphy on management was determined in 122 patients. Study n. 3: ability of somatostatin receptor scintigraphy and other conventional methods to distinguish small hepatic metastases (< 2 cm) from hepatic haemangiomas was assessed in 29 patients. Study n. 4: somatostatin receptor scintigraphy, magnetic resonance imaging and bone scanning were compared in 115 consecutive patients to detect bone metastases. Study n. 5: ability of somatostatin receptor scintigraphy to detect gastrinomas found at surgery in 35 patients and its effect on cure rate and determinants of detection of gastrinomas by somatostatin receptor scintigraphy were analysed. Briefly, results showed: Study n. 1: somatostatin receptor scintigraphy is the most sensitive modality for detection of primary or metastatic gastrinomas; Study n. 2: somatostatin receptor scintigraphy changes management in 47% of cases; Study n. 3: somatostatin receptor scintigraphy is the only method to distinguish small liver metastases from small haemangiomas; Study n. 4: somatostatin receptor scintigraphy and magnetic resonance imaging have higher sensitivity and predictive values for bone metastases than bone scanning; Study n. 5: somatostatin receptor scintigraphy misses 33% of gastrinomas found at surgery, primarily small duodenal tumours. Size is the important factor. The use of somatostatin receptor scintigraphy does not increase cure rate. In conclusion, Somatostatin receptor scintigraphy is now the imaging method of choice in patients with Zollinger-Ellison syndrome for preoperative primary tumour localization, detection of bone or liver metastases, and to distinguish small liver metastases from small hepatic haemangiomas. Its specificity appears to be high but has been poorly studied as has the use of it in combination with endoscopic ultrasound. Studies by others suggest these recommendations will apply to carcinoid tumours and other pancreatic endocrine tumours except insulinomas.

Over the last few years, remarkable progress has been made in diagnosis, severity assessment and treatment as well as in our understanding of the pathophysiology of acute pancreatitis. New treatment modalities and new specific drugs have been introduced and this has led to practical changes in the daily bedside management of patients with acute pancreatitis. Treatment is essentially medical, both for mild and severe disease, and is aimed at reducing abdominal pain, restoring electrolyte and fluid losses, removing the aetiological factor(s), attenuating inflammation and autodigestive processes, as well as preventing local and systemic complications. Diagnostic and interventional percutaneous or endoscopic procedures are indicated mainly for patients with severe forms of the disease. Surgery is generally indicated for patients with necrosis infection or other local complications not manageable by percutaneous or endoscopic means.

Historically, carcinoids are a morphologically distinct class of rare intestinal tumours that behave less aggressively than the more common intestinal adenocarcinomas. Some authors restrict the term carcinoid to intestinal endocrine tumours, and others include a large variety of neuroendocrine tumours. Within the gastrointestinal tract, carcinoids are most commonly found in the appendix, followed by the distal small bowel, rectum and stomach. In the vast majority of cases, the carcinoid syndrome is associated with carcinoid tumours of the small intestine that have metastasised to the liver. Episodic flushing and diarrhoea are the most common initial symptoms. Metastatic disease may require no treatment for months or even years in the patient whose symptoms are not seriously interfering with quality of life and if the tumour is not exhibiting a biologically aggressive growth pattern.

Multiple endocrine neoplasia type 1 has intrigued clinicians since its description because of its rarity and complex presentation of tumours in disparate endocrine organs. Its unpredictable course has made the development of strategies for clinical management difficult. The frequently indolent course in some family members, punctuated by lethal, aggressively malignant disease in others, has been frustrating as we strive to develop low-morbidity schemes for the prevention of the lethal manifestations of the syndrome. The recent description of the genetic abnormality responsible for the disease in many, if not all, families, will be of great assistance, even if it only allows the cessation of diagnostic testing for kindred members who are found to not carry the genetic abnormality. In fact, there is great promise in this discovery, as it may shed light on significant aspects of neuroendocrine oncogenesis. In spite of this, the clinical management strategies for these families will require further focused attention, in order to develop rational, prospective studies to answer the many questions that remain.

Background and aims: In the past, the reported prevalence of coeliac disease ranged from 1:1000 to 1:4000, whereas recent studies using serological screening methods have found a significantly higher prevalence. The aim of this study was to investigate the prevalence of coeliac disease in healthy blood donors in a North-eastern region of Italy.

Subjects: A total of 4000 healthy blood donors were studied from two immunotransfusion centres.

Methods: Serum IgA-antiendomysium antibodies were detected by indirect immunofluorescence using human umbilical cord vein sections, and positive sera were tested also on monkey oesophagus tissue. Intestinal biopsy was performed in all antiendomysium-positive subjects.

Results: Ten out of 4000 sera screened were found to be antiendomysium positive on human umbilical cord vein. All positive patients had flat mucosa on intestinal biopsy. Five subjects had coeliac disease-related clinical features (2 had a history of gastrointestinal symptoms, 1 a family history of IDDM, 1 sideropenic anaemia, and 1 IgA deficiency). One of the ten serum, antiendomysium positive on human umbilical cord vein, was found to be negative when tested on monkey oesophagus.

Conclusions: These data confirm the high prevalence of undiagnosed silent coeliac disease in the healthy adult population. This is the first study where umbilical cord was used for screening coeliac disease in a large population. The human umbilical cord vein indirect immunofluorescence test is more specific for villous atrophy than conventional indirect immunofluorescence test on monkey oesophagus and is a reliable screening test for coeliac disease in an apparently healthy population.