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Ganglioneuromas are typically of slow growth and benign evolution and may remain clinically silent for a considerable time if favourably situated. Many large examples are discovered incidentally on X-ray examination, routine abdominal palpation or at necropsy. Ganglioneuromas are often encountered in childhood and are found more frequently in the posterior mediastinum than in any other single situation; other sites are the lumbar and pelvic retroperitoneal tissues, the gastrointestinal tract and the mesentery. Diffuse alimentary tract ganglioneuromatosis has been described as port of the multiple endocrine neoplasia syndrome (MEN) type II-B. Sometimes ganglioneuromas are found in the von Recklinghausen Syndrome. The authors report in this paper a rare case of a retropancreatic ganglioneuromas.

n-Hexane neuropathy was studied in 20 workers exposed for prolonged periods to this solvent, and with urinary 2,5-hexanedione concentrations exceeding the biological exposure index recommended by the American Conference of Governmental Industrial Hygienists (5 mg/L) with a mean of 11.02 mg/L (range 5.3-24.2 mg/L). Although neurological examination did not detect significant anomalies in any of the patients, and the conduction velocity and F waves of all the nerves tested were normal, neurographic studies revealed significant differences in the amplitude of sensory nerve action potentials (SNAP) recorded from the sural (mean 14.0 microV), median (mean 17.3 microV), and ulnar (mean 7.9 microV) nerves when compared with normal values from healthy adults of the same age range, examined under identical conditions. The amplitude of the SNAP in sural and median nerves correlated significantly with the number of years worked. The notable decrease in mean amplitude of the SNAP appeared to reflect the primary neurotoxic effects of 2,5-hexanedione.

Transgenic mice harbouring 5' flanking sequences of the human beta 1 integrin gene linked to the Escherichia coli lacZ gene have been generated to examine spatial and temporal distribution of the promoter activity during development. Our previous data showed that this regulatory region is composed by two promoters, called distal and proximal, located closely on the human genome. To determine the role of each promoter region during development we generated transgenic mice using these two sequences linked to the lacZ reporter gene. Their analysis shows that these two sequences, as determined by in vitro studies, have different efficiencies in promoting transcription. Actually mice carrying the proximal promoter region exhibit a weak lacZ expression resulting in an undetectable beta-galactosidase activity in both embryonic and adult tissues. On the other hand, transgenic mice carrying the distal promoter express beta-galactosidase at high efficiency during embryonic development. The pattern of transgene expression is consistent with the localization of beta 1 protein on mouse embryos evidenced by immunohistochemistry. Moreover the distal promoter is subjected to a temporal modulation since in adult transgenic mice lacZ expression decreases to a level detected only by RT-PCR analysis. We have determined a similar down-regulation analysing by Northern blot beta 1 mRNA in adult and embryonic organs such as heart and gut.

The authors present one case of patients with adenocarcinoma of the small bowel. Primary malignant tumors of the small intestine are uncommon neoplasms accounting for 1-2 per cent of all gastrointestinal malignancies. Patients are usually seen late in the course of their illness when curative therapy is unlikely. The rarity of these neoplasms explains in part why the early signs and symptoms frequently go unrecognized and is undoubtedly a major factor contributing to their poor prognosis. Despite a fourfold greater length and a nearly tenfold greater mucosal surface area, the incidence of adenocarcinoma of the small intestine is about a fortieth that of the colon. This relative immunity of the small bowel to the development of the malignant tumors is unexplainable. Several theories have been suggested and include the following: a) the rapid transit time of the small intestine may reduce its exposure to dietary carcinogens; b) the relative sterility of the small intestine compared with the colon may lessen the formation of carcinogenic substances by the action of bacteria on components of bile or other substances within the intestinal lumen; c) certain mucosal enzymes such as benzopyrene hydroxylase that detoxify potential carcinogens are present in higher concentrations in the small intestine than in the colon; d) immunoglobulin A which is found in high concentrations in the small bowel, may protect it against carcinogenic viruses. Interestingly, patients deficient in IgA and those receiving immunosuppressive therapy have been found to have a higher incidence of small intestinal cancer. Adenocarcinoma is the most common primary malignant small bowel neoplasm.(ABSTRACT TRUNCATED AT 250 WORDS)

We report the cloning of two full-length cDNAs coding for the human beta 1-integrin which diverge from each other for their 5'-untranslated sequences. Characterization of a genomic clone containing these two sequences showed that they are contiguous, spaced by 261 nucleotides, and both followed by donor splice sites. Analysis by primer extension and transient transfection in a human osteogenic sarcoma cell line (MG-63) demonstrated the existence of two independent promoters for transcription initiation. The two promoter regions are very G+C-rich, and lack both a TATA box and a CAAT box. Northern blot analysis showed that transcripts starting from the distal promoter (with respect to the first coding exon) are at least 20-fold more abundant than transcripts originating from the proximal one. The levels of both transcripts increase after transforming growth factor-beta 1 induction, however, mRNAs originating from the proximal promoter increase at an higher extent. Reverse transcriptase/polymerase chain reaction analysis performed on different human tissues and cell lines revealed that, while the distal promoter is ubiquitously active, the proximal promoter is not. These findings suggest a possible complex pattern for regulation of the human beta 1-integrin gene expression.