Anal polypoid tumor formation as the form of presentation of rectal gastric heterotopia]

Heterotopic gastric epithelium occurs in all portions of the alimentary tract, but it is extremely rare in the rectum. The authors report the finding of a pedunculated anal polyp in a 9-year-old girl with a six-month history of rectal bleeding. Microscopically, the polyp consisted of epithelium of the fundic type. Both the age of the patient and the finding of heterotopic epithelium of the fundic type alone supports a congenital malformation and not an inflammatory condition.     

Contribution of molecular analyses to the estimation of the risk of congenital myotonic dystrophy.

A molecular analysis of the maternal and child CTG repeat size and intergenerational amplification was performed in order to estimate the risk of having a child with congenital myotonic dystrophy (CMD). In a study of 124 affected mother-child pairs (42 mother-CMD and 82 mother-non-CMD) the mean maternal CTG allele in CMD cases was three times higher (700 repeats) than in non-CMD cases (236 repeats). When the maternal allele was in the 50-300 repeats range, 90% of children were non-CMD. In contrast, when the maternal allele was greater than 300 repeats, 59% inherited the congenital form. Furthermore, the risk of having a CMD child is also related to the intergenerational amplification, which was significantly greater in the mother-CMD pairs than in the mother-non-CMD pairs. Although the risk of giving birth to a CMD child always exists for affected mothers, our data show that such a risk is considerably higher if the maternal allele is greater than 300 repeats.     

Outcome of simultaneous liver-kidney transplantation in highly sensitized,crossmatch-positive patients

BACKGROUND: In simultaneous liver-kidney transplantation (SLKT), the liver has been described to protect the kidney from rejection, and acceptable results are possible despite a pretransplant positive crossmatch. At our center, 21 SLKT have been performed since 1993, 2 of them against a positive crossmatch. OBJECTIVES: In this study we retrospectively analyzed two cases of SLKT after positive pretransplant crossmatch. METHODS: Two highly sensitized women (30 and 52 years) with hepatic cirrhosis VHC on hemodialysis after a first KT failure were assessed. Pretransplant panel reactive antibodies (PRA) by complement dependent cytotoxicity NIH (CDC) were 81% and 99% respectively. Both patients received a SLKT. CM was performed at pretransplant and 24 and 48 hours posttransplant by CDC and by flow cytometry with double labeling with CD3-PE and antihuman IgG-FITC. Patients received ATG, cyclosporine, and prednisone therapy. RESULTS: CM was positive pretransplant by CDC and flow cytometry. At 48 hours, CDC became almost negative (10%-20% mortality) and flow cytometry became negative. One of the patients experienced an episode of acute rejection at 10 days posttransplant that resolved with steroid pulses. Both patients presently have working grafts 26 and 24 months posttransplant (Cr, 1.1 and 1.5 mg/dL; GOT, 34 and 14 IU/L; GTP, 29 and 12 IU/L; GGT, 9 and 66 IU/L). CONCLUSIONS: Our experience suggests that a positive crossmatch is not an absolute contraindication for SLKT. Good graft and patient survival rates are possible even among highly sensitized patients.     

Obesity Surgery in the Philippines: Experience in a Private Tertiary Care Hospital for Years 2002 to 2004

Background Information on experience in bariatric surgery in the Asia-Pacific region is minimal: hence the need for more reports from this area. Methods The procedures of bariatric surgery and outcome as part of a weight management program in a tertiary care private hospital in the Philippines is reported from years 2002 to 2004. 50 patients were included, of which 60% underwent laparoscopic adjustable gastric banding (LAGB) and 40% Roux-en- Y gastric bypass (RYGBP). Results There were more females than males (64% vs 36%) with the mean age 38 ± 13.1 years. Initial mean BMI was 46.2 kg/m2, which decreased to 27.0 kg/m² in 1 year. Initial mean weight was 126.7 ± 25.4 kg, of which the 1 year weight loss was 32.3 kg for the morbidly obese and 58.0 kg for the super obese. %EWL at 1 year was 30.2%. There was greater weight loss with RYGBP compared to LAGB at 1 year (43.5 kg vs 30.2 kg). There was no mortality, and early complications were: wound infection (2/50 or 4%), and 1/50 or 2% each for pneumonia, dehydration, gastritis, and leakage. Late complications were: band slippage (2/20 or 10%), stomal stenosis (1/20 or 5%), and ventral hernia (1/5 or 20%). Conclusion Bariatric surgery is safe with a low complication rate and the outcome was similar to the reported data from Asia and the western world.     

Lymphocytotoxic antibodies in primary biliary cirrhosis

Sera of 30 patients with primary biliary cirrhosis (PBC) and 72 normal subjects matched for age and sex were examined for the presence of lymphocytotoxic antibodies (LCAs) against B and T cells at 4° C and 37° C. Patients were prospectively screened for: Sjögren's syndrome, scleroderma, Hashimoto's thyroiditis, and rheumatoid arthritis, in which LCAs have been described. Seventeen patients with PBC (56.6%) had LCAs against B cells as compared with only 11 of the 72 normal subjects (P<0.001). Five PBC patients (16.6%) also reacted against T cells as compared with none in control group (P<0.01). Clinical and biochemical features and the histological stage of PBC were similar in patients with and without LCA. Sjögren's syndrome was present in 13 patients—two with scleroderma and another with Hashimoto's thyroiditis. No patient had rheumatoid arthritis. The prevalence of LCAs was similar in PBC patients with or without autoimmune associated disease (54% vs 59%). We conclude that in PBC a high incidence of LCA is unrelated to the presence of an associated disease. LCA in PBC might be a nonspecific marker of an immune disorder.     

Growth and development considerations for craniomaxillofacial surgery

The purpose of craniomaxillofacial surgery is to improve function, occlusion, craniofacial balance, and aesthetics. Accurate diagnosis, assessment, and careful treatment planning are essential in achieving a successful outcome, and an understanding of the pattern of facial growth is integral in this process. Patients with craniofacial congenital dysmorphologies, posttraumatic asymmetries, or disturbances of facial balance from radiation may have functional and/or aesthetic issues that require treatment. Understanding the complexities of growth in the skull and face is a key component to appropriate treatment planning for these disorders. This article reviews growth and development in the craniofacial skeleton.