Multiple endocrine neoplasia 2B presenting with pseudo-Hirschsprung's disease.

Multiple endocrine neoplasia type 2B (MEN 2B) is a rare syndrome characterized by medullary thyroid carcinoma (MTC), pheochromocytoma and typical phenotypic features, such as marfanoid habitus, multiple mucosal ganglioneuromas and thickened corneal nerves. Individuals with MEN 2B may develop megacolon and pseudo-obstruction due to intestinal ganglioneuromatosis simulating Hirschsprung's (HSCR) disease. We hereby describe the clinical and genetic features of a 21-year-old male patient with MEN 2B associated with pseudo-HSCR disease. The patient had MTC, pheochromocytoma, marfanoid habitus, multiple mucosal ganglioneuromas, thickened corneal nerves and severe gastrointestinal involvement. Emergent laparotomy was performed when he was presented with acute bowel obstruction. The myenteric and submucosal nerve plexuses in the small and large intestines were composed of diffusely hyperplasic, disorganized, mature ganglion cells. Genetic testing revealed a de novo ret proto-oncogene germline mutation in codon 918 in exon 16. Megacolon and pseudo-obstruction similar to the HSCR disease may develop in patients with MEN 2B. However, the observed dysmotility is the result of an abnormal proliferation of intramural ganglion cells in contrast to the absence of enteric ganglia which were present in the HSCR disease. Attentiveness about the phenotypic characteristics and unusual findings might lead to early and correct diagnosis of the MEN 2B syndrome. This approach improves the survival rate and quality of life considerably.

The addition of O2 to the CO2 does not prevent the systemic effects of the CO2 pneumoperitoneum in a rabbit model.

CO2 pneumoperitoneum used in endoscopic surgery induces systemic effects by CO2 absorption. It was claimed that a reduction in CO2 pneumoperitoneum-induced metabolic hypoxemia was achieved by the addition of small amounts of O2 to the CO2 in a rabbit ventilated model. We reevaluated the effects of the addition of O2 to the CO2 pneumoperitoneum upon CO2 absorption in a rabbit model. The effects of a pneumoperitoneum using 100% CO2, 90% CO2 + 10% O2, 95% CO2 + 5% O2, or 100% O2 on arterial blood gases, acid base and O2 homeostasis were evaluated in nonintubated rabbits. A pneumoperitoneum pressure of 10 cm H2O (approximately 7.35 mm Hg) was used. CO2 pneumoperitoneum of 120 minutes affected blood gases and acid base homeostasis. Whereas partial pressure of CO2 and HCO3 increased (P < 0.001) during pneumoperitoneum, pH and partial pressure of O2 decreased (P < 0.001). Similar results were obtained in O2-CO2 pneumoperitoneum (P > 0.05). CO2 pneumoperitoneum profoundly affected blood gases and acid base homeostasis, resulting in metabolic hypoxemia. The addition of O2 to the CO2 did not prevent the systemic effects of CO2 pneumoperitoneum in nonintubated animals.

Comparison of hyaluronate/carboxymethylcellulose membrane and melatonin for prevention of adhesion formation in a rat model.

BACKGROUND: To investigate the effectiveness of melatonin in preventing post-operative adhesion formation and to compare it with the efficacy of hyaluronate/carboxymethylcellulose membrane in a rat model. MATERIALS AND METHODS: Following pilot studies, 35 rats were operated on in the full study. In 15 animals (group one), 10 standard lesions were inflicted in each uterine horn (total 30 horns) and melatonin was applied before closure of the abdomen. In the second group, 20 animals were operated on and one of the uterine horns (total 20 horns) with standard lesions was treated with hyaluronate/carboxymethylcellulose membrane and the other uterine horn served as a control. Second-look operations were performed 1 week later and adhesion scores were compared. RESULTS: The adhesion scores of uterine horns treated with melatonin and of uterine horns treated with hyaluronate/carboxymethylcellulose membrane were significantly lower than the scores of the controls (P < 0.001). There was no statistically significant difference between the adhesion scores of uterine horns treated with melatonin and of uterine horns treated with hyaluronate/carboxymethylcellulose membrane (P > 0.05). CONCLUSIONS: Both melatonin and hyaluronate/carboxymethylcellulose membrane were effective in prevention of adhesion formation in a rat uterine horn model.

Effect of mesalazine, metronidazole and gentamicin on bacterial translocation in experimental colitis.

BACKGROUND AND AIM: In inflammatory bowel disease it has been established that enteric microorganisms are present in the final stage of the active inflammatory process. The purpose of the present study was to investigate the effects of mesalazine, and metronidazole-gentamicin combination, on bacterial translocation in an animal colitis model. METHODS: Fifty rats were stratified into five groups. The control group (group NC) was given only 2 mL saline enema and the remaining four groups were given 2 mL acetic acid enema. Group CC was the diseased control group. The treatment regimens started on the fifth day: mesalazine enema in group MesC, metronidazole-gentamicin in group MGC, and mesalazine + metronidazole + gentamicin in group MesMGC. After death on day 10, 2.5-cm colonic segments from all groups were weighed separately. In all rats, histopathological scoring was done, and samples from feces, blood, liver and spleen underwent microbiological analyses. RESULTS: For all diseased rats, both mean weight loss and colonic segment weight/bodyweight ratio was significantly higher than that in the sham group. As compared with other groups, body and colonic segment changes as well as histopathological scoring in rats receiving mesalazine enema either solely or in combination with the antibiotics were lower. No bacterial growth was found in the blood, liver and spleen of the rats in the control group while enteric bacteria, mainly Escherichia coli (35%) were the most common bacteria translocated to that in the latter. Antibiotic combination, alone or in combination with mesalazine was effective in reducing the bacterial translocation while mesalazine administration did not properly influence its regression. CONCLUSIONS: Gram-negative enteric bacteria, predominantly E. coli, was the most common bacteria isolated in bacterial translocation occurring in acetic acid-induced colitis. This trial showed that mesalazine alone did not incorporate the reduction of infectious events, despite its beneficial effect on inflammatory changes in experimental colitis. Metronidazole and gentamicin combination given intraperitoneally was more effective than topical mesalazine in decreasing bacterial translocation.