BioEnterics intragastric balloon for treatment of morbid obesity in Prader-Willi syndrome: specific risks and benefits.

BACKGROUND: Obesity in Prader-Willi Syndrome (PWS) is progressive, severe, and resistant to dietary, pharmacological, and behavioral treatment. A body weight reduction is mandatory to reduce the risk of cardio-respiratory and metabolic complications. The aim of the study was to assess risks and benefits of BioEnterics Intragastric Balloon (BIB) for treatment of morbid obesity in PWS patients. METHODS: Twenty-one BIB were positioned in 12 PWS patients (4 M, 8 F), aged from 8.1 to 30.1 years, and removed after 8 +/- 1.4 months (range: 5-10 months). Auxological, clinical, and nutritional evaluations were performed every 2 months. Variations in body composition were analysed by dual energy X-ray absorbiometry (DXA). RESULTS: One patient (28.5 years, BMI: 59.3 kg/m(2)) died 22 days after BIB positioning because of gastric perforation. In another case (26.2 years, BMI: 57.6 kg/m(2)), BIB was surgically removed after 25 days because of symptoms suggesting gastric perforation (not confirmed). The remaining ten patients showed a significant decrease of BMI (p = 0.005) and of fat tissue as measured by DXA (p = 0.012). No significant modifications in bone mineral density (BMD) occurred, but a slight loss in lean body mass (p = 0.036) was documented. In five patients, BIB treatment was repeated more than once. CONCLUSION: This study shows that when noninvasive pharmacological therapies fail, BIB may be effective to control body weight in PWS patients with morbid obesity, particularly when treatment is started in early childhood. However, careful clinical follow-up and close collaboration with parents are crucial to avoid severe complications, which can be caused by persisting unrestrained food intake.

Role of cyclosporin in preventing or delaying colectomy in children with severe ulcerative colitis.

BACKGROUND: Oral cyclosporin (CyA) has been widely and successfully used in adult patients with severe ulcerative colitis (UC) to delay or avoid colectomy. AIM: To determine if treatment with oral CyA is similarly effective in pediatric patients MATERIALS AND METHODS: Data on all patients with severe UC treated with oral CyA in our unit were collected retrospectively. Patients were treated with CyA if dependent on or resistant to steroids, and therefore, candidates for colectomy. RESULTS: Thirty-two patients with severe UC were treated with CyA administered orally at a dose needed to obtain therapeutic blood levels (150-250 ng/ml). Twenty-eight of 32 patients (87%) had an immediate response within 11 days. Four (13%) did not respond and underwent colectomy. One patient had two cycles of treatment and is in remission. Two patients underwent three cycles of treatment because of relapse, but both eventually underwent elective colectomy. Three other patients underwent elective colectomy. A total of nine colectomies were performed. CONCLUSIONS: Treatment with oral CyA altered the course of UC in 28/32 (87%) of patients; 4/32 (13%) did not respond to oral CyA and underwent colectomy. Of the 28 patients that responded to CyA, five underwent later elective colectomy. Overall, in 72% of patients, colectomy was avoided. We, therefore, suggest a trial of oral CyA in all children with severe UC who are dependent or resistant to corticosteroids.

The role of transanal endorectal pull-through in the treatment of Hirschsprung's disease - a multicenter experience.

The transanal approach (TAA) is a new technique for surgery of Hirschsprung's disease (HD) that was introduced by de la Torre in 1998. The purpose of this multicenter study, including experience from three Austrian and one Italian departments of peadiatric surgery, was to evaluate the role of this approach in HD in 18 children aged 1-72 months. In 14 children the TAA only was performed; in 3 an additional laparoscopy was performed and in 1 conversion to a laparotomy was necessary. One complication (abscess) occurred after laparoscopic-assisted pull-through. The postoperative recovery was rapid, no severe long-term problems were observed. The transanal pull-through technique is generally possible in most classic cases of HD with extension of the disease to the sigmoid colon. If necessary, it can be combined with laparoscopy. Our preliminary results show that the technique is safe, less invasive, and gives excellent cosmetic results, and allows rapid recovery. Long-term results are still pending.

The effect of mucin on bacterial translocation in I-407 fetal and Caco-2 adult enterocyte cultured cell lines.

Although the intestinal mucosa forms a crucial barrier between the host and the environment, bacterial translocation (BT) occurs frequently in neonates and may be a source of sepsis. The intestinal mucous gel layer is thought to be a vital component of the gut barrier and is composed, in part, of a family of glycoproteins known as mucins. Our aim was to study the effects of mucin on BT in an enterocyte cell-culture model using a fetal (I-407) and an adult (Caco-2) intestinal cell line. I-407 and Caco-2 cells were grown to confluence on porous filters in a two-chamber Transwell system. The integrity of the monolayers was confirmed by transepithelial electrical resistance (TEER) and permeability using the macromolecule dextran blue. Cells were treated with mucin (40 mg/ml) prior to inoculation of 1 x 10(6) Escherichia coli C25. The magnitude of BT was determined quantitatively by culturing the samples from the basal chamber of the wells and was expressed as log 10 [Colony Forming Units (CFU)/ml]. Statistical analysis was performed by the Mann-Whitney U test with statistical significance at P < 0.05. Mucin inhibited BT across both fetal and adult cultured enterocyte monolayers; however, the inhibitory effect was less on the fetal cells compared to the adult cells. Dextran-blue studies showed that monolayers were intact throughout the experiments. Despite 98% inhibition of BT, mucin had a statistically significant effect on post-bacterial inoculation TEER in Caco-2 cells and no effect in I-407 cells. The ability of mucin, a mucous-barrier glycoprotein, to inhibit BT across immature intestinal enterocytes, as in the neonate, may be diminished compared to mature adult enterocytes.

Gastroesophageal reflux in neurologically impaired children: partial or total fundoplication?

BACKGROUND: It is difficult to give guidelines when approaching gastroesophageal disease in neurologically impaired children. Indication for surgery has been increasing over recent years, but there is no consensus on the surgical technique of choice. Nothing has been written specifically comparing the results of different procedures in these patients, so far. STUDY DESIGN: We retrospectively compare the short- and long-term results of two different types of fundoplication in a series of children operated on for documented gastroesophageal reflux disease at our institution. RESULTS: One group (group A) of 27 patients, operated on between 1977 and 1993, underwent Nissen fundoplication, the other (group B), formed of 20 patients all of whom were operated on between 1993 and 1995, underwent Thai fundoplication. We compared the results in terms of positive outcome (recovery) and negative outcome (minor and major complication), computing the relative odds of group A versus group B in terms of risk of complication, and we compared the mean operative time and the length of hospital stay by means of a student's t-test analysis. CONCLUSIONS: Our results show that there is no statistical difference between the two procedures in terms of relative risk of complication and success rate. The duration of surgery and hospital stay were significantly shorter in group B. The Thal procedure can, therefore, be proposed as first choice in the management of these patients.

[Wolfram syndrome. Peculiar urologic aspects]. / La sindrome di Wolfram. Aspetti urologici peculiari.

The 6-year follow-up of a patient affected by Wolfram's syndrome, a rare disease characterized by diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), neurosensory deafness (D), atony of the urinary tract and other abnormalities (DIDMOAD or Wolfram's syndrome), is described. Our patient has diabetes insipidus, diabetes mellitus, abnormal audiograms, without subjective evidence of hearing loss, and dilatation of the urinary tract. Diagnosis was suspected at the age of 8 years. Diabetes mellitus was the first manifestation and treatment with insulin was necessary. Desmopressin therapy decreased dramatically the daily urinary output. In view of the significant morbidity and mortality from renal failure associated with recurrent urinary infections, we have drawn special attention to the urological manifestations of the syndrome. During the follow-up, the patients underwent some investigations, such as renal ultrasound and echotomography and cystourethroscopy. Outstanding results of these studies are severe bilateral hydronephrosis with dilatated ureters and loss of renal tissue. The particular finding is the presence of posterior urethral valves with obstructed bladder. The anatomical outlet obstruction are variable and may be disastrous. There may be failure to thrive, sepsis, anemia be disanal failure. In such instances corrective surgery could improve bladder and ureteral functions.

The use of cyanoacrylate tissue adhesive in high-risk intestinal anastomoses.

The success of every intestinal surgical procedure primarily depends on correct technical execution of the intestinal sutures. Despite the continuing improvements in intestinal synthesis techniques and the introduction of mechanical staplers, the risk of anastomotic dehiscence remains a major concern. For high-risk anastomoses, defined as those performed under critical conditions, n-butyl-2-cyanoacrylate tissue adhesive allows for quick sealing of the two stumps and supports the physiological wound-healing process. Furthermore, no experimental or clinical studies have shown that this glue has any carcinogenic or mutagenic properties. Thus, we believe that n-butyl-2-cyanoacrylate will be extremely useful for intestinal anastomoses with a high risk of dehiscence.

Polymeric glues in intestinal surgery.

Authors reports the results of an experimental study on the usefulness of n-butyl-2-cyanoacrylate in abdominal surgery. The research, performed on Wistar rats and Landrace pigs, is constituted by an early phase, to verify the tissue reaction to the n-butyl-2-cyano-acrylate and its adhesive properties, and a main phase, in which it was evaluated the efficacy of n-butyl-2-cyano-acrylate as the only support or as an adjunct to the usual methods in intestinal synthesis. histological and angiographic examination of the surgical specimens demonstrated the tissue autotoxicity and the good adhesive effect of the tissue glue. Because of these characteristic, authors propose its employment to reinforce intestinal sutures performed with the usual methods in high risk condition.