Surgical treatment of chronic inflammatory bowel disease in children.

Surgery for chronic inflammatory bowel disease (IBD) is increasingly often necessary in children. This study aimed at assessing the results of these operations in order to facilitate adequate preoperative counseling. We reviewed patients treated from 1992 to 2009. The operations, complications and functional outcome were recorded. For those with preserved rectal defecation, continence (Koivusalo score) and quality of life (standardized questionnaire) were assessed in the long term. Eighty five of 192 patients had Crohn disease (CD), 107 of 192 had ulcerative colitis (UC), and 3 of 192 had indeterminate colitis (IC). 12 of 85 CD patients (15%) aged 14 (12-19) years required 13 resections, 1 stricturoplasty, 1 transplantation and 6 other operations including 3 permanent enterostomies for anorectal involvement. Removal of the involved bowel led to significant improvement of nutritional status, growth and quality of life. The transplanted patient had a striking recovery but eventually died 1 year later of unrelated complications. 29 of 107 UC patients (26%) aged 11 (2-15) years required 87 operations. Nine had emergency colectomy for toxic megacolon (3, one death) or severe hemorrhage (6). 28 had restorative proctocolectomy and ileoanostomy (RPCIA) without (16) or with (12) J-pouch under protective ileostomy. Complications were frequent (40%). Permanent ileostomy was required in five children (17%). Twelve months postoperatively, RPCIA patients had 6.5 (2-13) stools/day; all were continent during daytime, and 25% have nocturnal leaks. Mean Koivusalo score (5-12) was 8.8 ± 2. Quality of life was good in all. All attended normal school and 7 the university, 4 work and 60% of those older than 18 years have sexual partners. Three of 107 children treated as UC with RPCIA had ultimately IC (3%) and were permanently diverted. The nature of IBD involves frustrating surgery. However, it may change life for CD patients and provide a reasonably good quality of life for UC after the first year. Pediatric surgeons should be able to provide adequate preoperative counseling to patients and families.

[Gastric adenocarcinoma in young adults; comparative study with older patients]. / Adenocarcinoma gástrico en adultos jóvenes; estudio comparativo con pacientes mayores.

BACKGROUND: Gastric adenocarcinoma (GA) has been considered a disease of elderly age and has been rarely reported in patients younger than 35 years of age. The aim of thisΩ demographic, clinicopathological and prognosis of gastric cancer in young patients and to compare their features with the behavior in elder adults. METHODS: Between 1993 and 2008, 1536 patients with GA were enrolled in a retrospective database. Clinical and pathologic features of thirty patients aged 35 years or less (young group) were compared with those of 458 aged 75 years or more (elder group). RESULTS: Mean patient age was 31 and 80-years old in the young and elder groups, respectively, with a predominance of females in the last group (61%). Lauren diffuse type carcinoma was more frequent in people younger than 35 years (70%) than in older patients (17.4%). Main symptoms were dyspepsia (40%) and hemorrhage (20%). The most common T stage in young and elder patients was T3 (52.9% and 56.7% respectively). Surgical resection was performed in 68% of cases and the rest received only systemic chemotherapy. CONCLUSION: Gastric adenocarcinoma is rare in young patients and most cases presented at advanced clinical stage similar to elderly patients, so the prognosis in both age groups is poor. For this reason is important to be aware of alarm symptoms and risk factors in order to perform an early endoscopic diagnosis and a treatment with curative intent.

[Long-term results in ulcerative colitis treated with proctocolectomy and ileoanostomy in children]. / Resultados a largo plazo en la colitis ulcerosa tratada con proctocolectomía e ileoanostomía en niños.

AIM: The treatment of Ulcerative Colitis (UC) unresponsive to current medical treatment involves total proctocolectomy and ileanostomy. The aim of this study was to assess the long term results in such patients. MATERIAL AND METHODS: We reviewed children treated from 1992 to 2008. The operations, complications and functional outcome were recorded. For those with preserved rectal defecation, continence (Holschneider score) and quality of life (standardized phone questionnaire) were assessed in the long term. RESULTS: 104 patients had Ulcerative Colitis (UC) and 3 undetermined colitis (excluded from the study). 26/104 UC patients (26%) aged 10.7 +/- 4.1 years required 82 operations. Eight had emergency colectomy for toxic megacolon (3, one death) or severe hemorrhage (5). 25 had restorative proctocolectomy and ileoanostomy (RPCIA) without (16) or with (9) J-pouch under protective ileostomy. Complications were frequent (40%). Permanent ileostomy was required in 5 children (20%). Twelve months postoperatively, RPCIA patients had 6.3 +/- 3 stools/day, all were continent during daytime and 25% have nocturnal leaks. Mean Holschneider score (0-12) was 8.8 +/- 2. Quality of life was good in all with normal school (all) or university attendance (7), 4 work and 60% of those older than 18 have sexual partners. CONCLUSIONS: The nature of UC involves risky and frustrating surgery. However, it may provide a reasonably good quality of life for UC patients after the first year. Pediatric surgeons should be able to provide adequate preoperative counseling to patients and families.

Prognostic value of carcinoembryonic antigen level in rectal cancer treated with neoadjuvant chemoradiotherapy.

BACKGROUND: The purpose of this study was to identify clinical and pathological parameters to improve prediction of disease-free survival (DFS) and overall survival (OS) in patients treated with neoadjuvant chemoradiotherapy for rectal cancer. METHODS: Between July 1995 and May 2007, 148 patients with primary rectal adenocarcinoma received neoadjuvant chemoradiotherapy followed by mesorectal excision. Preoperative treatment included various protocols, UFT and leucovorin (28%) and oxaliplatin-based chemotherapy (72%). Clinical and pathological variables were evaluated in relation to patient outcomes. RESULTS: Thirteen percent of patients achieved a complete pathologic response. No response or minimal response as defined by Dworak (Tumor Regression Grade 0/1) was observed in 30 patients (20%). At a median follow-up of 37 months, the 3-year DFS and OS were 64% and 83%, respectively. Pre-treatment serum carcinoembryonic antigen (CEA) level

Endoscopic, bioptic, and manometric findings in eosinophilic esophagitis before and after steroid therapy: a case series.

BACKGROUND AND AIM: Eosinophilic esophagitis can be associated with a wide range of endoscopic patterns. The aim of the present case series report is to describe and classify endoscopic appearances before and after corticoid therapy in relation to histopathology and manometry. PATIENTS AND METHODS: In 30 patients (m : f, 27 : 3; mean age 36.2 years) with eosinophilic esophagitis, endoscopic findings were prospectively classified according to luminal diameter and mucosal pattern. Manometric and bioptic histopathologic findings were also recorded. Endoscopy was repeated following a 3-month course of steroid therapy. RESULTS: In total, 20 % of patients showed a concentric esophageal stricture, and in 57 % simultaneous contraction rings were visible. Mucosal alterations consisted of granular mucosa (20 %), longitudinal furrows (33 %) and transversal undulations (3 %). Lower esophageal sphincter dysfunction and distal esophageal dysfunctional manometry were seen in 73 % and 57 % of cases, respectively. Following steroids, the esophagus showed a normal caliber in 97 % of patients, and 63 % of patients had normal mucosa. CONCLUSIONS: The most frequent findings were narrowing of the esophageal lumen, which returned to normal following steroid treatment to a larger extent than mucosal alterations.

Startle and prepulse inhibition as a function of background noise: a computational and experimental analysis.

Schmajuk and Larrauri [Schmajuk NA, Larrauri JA. Neural network model of prepulse inhibition. Behav Neurosci 2005;119:1546-62.] introduced a real-time model of acoustic startle, prepulse inhibition (PPI) and facilitation (PPF) in animals and humans. The model assumes that (1) positive values of changes in noise level activate an excitatory and a facilitatory pathway, and (2) absolute values of changes in noise level activate an inhibitory pathway. The model describes many known properties of the phenomena and the effect of brain lesions on startle, PPI, and PPF. The purpose of the present study is to (a) establish the magnitude of startle and PPI as a function of pulse, prepulse, and background intensity, and (b) test the model predictions regarding an inverted-U function that relates startle to the intensity of the background noise.

Immunohistochemical expression of p53, Bcl-2, COX-2, C-erb-B2, EPO-R, beta-catenin, and E-cadherin in non tumoral gastric mucous membrane.

Different authors have investigated the immunohistochemical expression of some proteins in the adenocarcinoma of the stomach, including cell cycle regulators proteins like p53 and Bcl-2; growth factors (c-erb-B2 and EPO-R); angiogenesis-related markers such as COX-2 and cellular adhesion molecules (beta-catenin and E-cadherin). While these proteins have been studied in gastric adenocarcinoma, their immunophenotyping in non tumoral gastric mucous membrane remains unexplored. In the present study, we investigated the expression, function and behavior of these proteins in normal gastric mucous membrane to contribute to gain further knowledge on the significance of their loss or overexpression in malignant gastric tumors.

A pre-clinical study showing how dopaminergic drugs administered during pre-exposure can impair or facilitate latent inhibition.

RATIONALE: It has been suggested that, in classical conditioning, dopamine (DA) codes for (a) attention to the conditioned stimulus (CS) or (b) the intensity of the unconditioned stimulus. OBJECTIVES: To clarify the role of DA in pre-clinical classical conditioning studies. METHODS: An existing model of classical conditioning presented by Schmajuk, Lam, and Gray (J Exp Psychol Anim Behav Process 22:321-349, 1996) suggests that DA cells in the ventral midbrain area code for the attentionally modulated internal representation of the CS. It is assumed that this representation is increased by dopaminergic agonists and decreased by dopaminergic antagonists. Computer simulations with the model describe the effect of nicotine and haloperidol on latent inhibition. RESULTS: Simulations replicate experimental results demonstrating that both nicotine and haloperidol affect latent inhibition when administered during the pre-exposure phase. In addition, the model reproduces data showing that administration of nicotine or haloperidol results in the impairment or facilitation of latent inhibition depending on the duration of CS or the number of CSs. CONCLUSIONS: The model demonstrates that pre-clinical experimental results, including cell activity and pharmacological data, are consistent with an attentional role for DA in classical conditioning.

[Nonalcoholic steatohepatitis, the enigma of bad progression]. / Esteatohepatitis no alcohólica, el enigma de una mala evolucion.

Still, very little is known about the precise pathogenetic mechanisms, the triggering events and in particular, the evolution and treatment of nonalcoholic steatohepatitis (NASH). It is part of the broad spectrum of nonalcoholic fatty liver diseases (NAFLDs). Mainly, it has been reported as a benign disease, associated with metabolic disorders commonly occurrence en the general population. Nevertheless, the syndrome can lead to cirrhosis, liver failure or hepatocellular carcinoma, requiring liver transplantation. We present one patient with diagnosis of NASH, who was treated initially for overweight, HTA and hyperlipaemia with incompleted response and who showed a quickly progress to cirrhosis but no cause of liver decompensated disease could be identified. Currently she is at end-stage waiting a liver transplantation. Controlled and multicentric studies with the same definition of NASH and the study end-points are needed, and will provide information about diagnosis features and novel therapies to early management of the disease.

Free radical scavenging capacity in the aging of selected red Spanish wines.

Free radical scavenging capacity by the DPPH(*) method and main physicochemical properties, polyphenols content by HPLC, color by a tristimulus colorimeter, and UV-vis spectra in the aging of selected red Spanish wines, were studied. As the wines age, they become darker (lower lightness, L) and increase their hue angle (lower red color) as well as the ratio of absorbance at 420 nm to that at 520 nm. Main polyphenolics identified in the samples were tannic acid, oenin, and gallic acid. The antiradical efficiency of the samples increased during aging, which could be related to an increase in the tannic acid concentration shown by the following correlationship: EC(50) = 1/(0.18 + 0.0011[tannic acid](mg/L)) with a correlation coefficient of 0.744.

Gastric heterotopy of the biliary tract.

The case of a 12-year-old girl having cholestatic syndrome, due to the presence of gastric tissue in the gallbladder and extrahepatic biliary tract, is reported here.

Biliary atresia and the polysplenia syndrome: its impact on final outcome.

Up to 25% of babies with biliary atresia (BA) bear associated malformations that most often cluster in the polysplenia syndrome (PS). This article examines the impact of associated PS on the final outcome of the authors' BA patients and the necessary modifications of surgical technique during orthotopic liver transplantation (OLT) in these patients. The authors studied 88 consecutive infants with biliary atresia operated upon during the 10-year period between 1984 and 1993. Biliary drainage was provided by a Roux-en-Y hepaticojejunostomy. OLT was performed in 38. Eleven infants (12%) had three or more components of PS: malrotation (11), preduodenal portal vein (11), polysplenia (10), situs inversus (4), absent inferior vena cava (3), cardiac defects (2), and anomalous hepatic artery supply (2). Bile drainage was achieved in 7/11 infants (63% versus 48% for regular BA patients) 5 of whom (45% versus 31%) are jaundice-free with a mean follow-up of 105 months. Three patients received transplants (2 from living-related donor) and are alive and well with normal liver function after a mean follow-up of 35 months. Overall survival was 72% in the BA plus PS and 66% in the BA group (P = not significant [NS]). Results show that long-term bile drainage in these PS patients can be achieved at least as often as in other BA patients (45% versus 31%) (P = NS) and indicate that Kasai operation remains the best initial choice. When OLT becomes necessary, attention to vascular anatomy is required to determine the feasibility of reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

[Arteriovenous fistula of the cecum diagnosed by digital angiography]. / Fístula arteriovenosa de ciego diagnosticada por angiografía digital.

We report the case of a 69 year-old female, with cardiac disease, hypertension, and chronic renal failure treated by hemodialysis; she had several episodes of gastrointestinal hemorrhage, which could not be diagnosed by conventional methods. Digital angiographic study demonstrated the exact cause: it allowed to localize an arterio-venous malformation. A right hemicolectomy was performed and the pathologic study showed the presence of an arteriovenous fistula. Three years later the patient is free of symptoms. We recommend digital angiography in cases of gastrointestinal hemorrhage of unknown or unclear etiology, particularly in patients in chronic renal failure, on the basis of the association of hemodialysis and arteriovenous malformation.

[Acute severe hypersensitivity reaction to salazopirin]. / Reacción de hipersensibilidad aguda grave a la salazopirina.

Sulphasalazine is the drug most widely used in intestinal inflammatory disease given its efficacy and low cost. Nonetheless, its administration is not lacking adverse effects. Two cases of severe hypersensitivity are herein described. Moreover, the diagnostic difficulties observed and their implication in the management of the underlying disease are discussed.

[Non-immunoallergic hepatotoxicity due to mesalazine]. / Hepatotoxicidad no inmunoalérgica por mesalazina.

Mesalazine is an aminosalicillic derivative considered as a safe alternative to the relative frequency (5-55%) of adverse effects observed with sulfasalazine. The well known hepatoxicity associated with sulfasalazine and attributed to its sulfamidic fraction is limited to few cases described in the treatment with mesalazine. We herein present a new case of hepatoxicity by mesalazine in a patient with lymphocytic colitis. The possible pathogenic mechanism is also commented upon.

[Histopathology of biliary atresia: correlation with biliary flow]. / Histopatología de la atresia biliar: correlación con el flujo biliar.

The objective of KASAI's operation is to obtain a postoperative bile excretion and to maintain an internal biliary fistula. The purpose of this report is to study the relationship between the liver histopathology and the postoperative bile flow and survival. From july 1976 to january 1990, 66 patients underwent a corrective operation for biliary atresia. In 24 patients we performed a KASAI's operation with internalization of the biliary conduit, 36 with externalization, four gall-bladder KASAI and two liver biopsy. The mean age at operation was 66 days and postoperative bile excretion was obtained in 43 of them (66 per 100). The survival rate was 70 per 100. The liver histopathology shown: minimum portal fibrosis (7.8 per 100), moderate (49 per 100) and severe (43 per 100). We found bile ducts in 30 patients (46 per 100), in three bile ducts were less than 50 mu, in 15 between 50 and 100 mu and more than 100 mu in 12. We did not found statistic correlation between grade of liver fibrosis size of bile duct and postoperative flow. We found a statistic correlation between the presence of bile ducts and postoperative flow (p less than 0.05).

[The multifocal hepatic hemangioendothelioma. Is always a benign tumor?]. / Hemangioendotelioma multifocal hepático infantil. Es siempre un tumor benigno?

UNLABELLED: The hepatic multicentric haemangioma is defined by its extension, affecting all the mass of the liver. The high mortality associated with it is mostly related with the complications produced by its enormous size (haemodynamic, platelet trapping, spontaneous rupture and bleeding). There is a general belief that is a benign tumor with possibility of spontaneous regression and cure. AIM: Retrospective analysis of our recent cases of MHH with the purpose of: 1 degrees) To show the evolution and results. 2 degrees) To realize if the "benign character" of the tumor is real or if some cases may be considered as malignant tumors. MATERIAL AND METHODS: 10 cases of MHH treated in the last 10 years. In 9 the age of presentation was less than 6 months and one patient was diagnosed at 3 and half years. The diagnosis was confirmed by image techniques in 7 cases and by biopsy in 3. In 7 patients extrahepatic vascular lesions were associated prior to the treatment. Methylprednisolone was given to all the cases and alpha-2-interferon was administered to the patients that not responded to the steroids. Vincristine was added to 2 patients. In two cases the hepatic artery embolization was tried and one patient had a liver transplant. RESULTS: Four children had at least one episode of congestive cardiac insufficiency, two patients suffered a consumption coagulopathy (Kasabach Merrit syndrome), and one presented acute hepatic failure. In six children it has been complete regression of the tumor, one more is still under treatment and three died. The dead were produced by the malignant behavior of the tumor in one case (tumoral rupture of a MHH recurrence in the transplanted liver), and possibly in other (intracranial haemorrhage and hepatic failure in a liver transplantation candidate without demonstrated extrahepatic extension in the previous studies, but with multiorgan dissemination at autopsy. In both cases it was impossible to discover signs of histologic or biologic malignancy neither in the primitive lesion nor in the metastasis. CONCLUSIONS: 1a) The regression of the MHH, spontaneous or induced by the treatment is frequent. 2a) Some cases of MHH are aggressive and develop local recurrences and distant metastasis. 3a) The discrimination between MHH of "benign" or "malignant" behaviour is not possible. 4a) Despite of the unpredictable biological conduct of the tumor, the liver transplantation must be considered as an option in the symptomatic cases that not respond to the conventional treatment.

[Intestinal transplantation. First experience in Spain]. / Trasplante intestinal. Primera experiencia en España.

UNLABELLED: We present the first case of intestinal transplant (IT) performed in Spacin. CASE REPORT: 28 months old boy with secretory diarrhea since the first month of life, diagnosed of microvillous inclusion disease (MID). He is on total parenteral nutrition (TPN) and had suffered of multiple episodes of catheter related sepsis with lost of standard venous access. An isolated small bowel transplant from a cadaveric donor was performed at the age of 3 years. The native ileocaecal valve and colon were not removed. RESULTS: Enteral feeding was started in the 2nd. week after the IT. On the 25th day he was off TPN. Since the 77th day, he eats regular foods by mouth. At the 6th month post IT the ileostomy was closed. Among the complications, he suffered a rotavirus infection on the 38th post IT day and an episode of mild rejection responsive to methil-prednisolone bolus. CONCLUSIONS: The IT is a therapeutic option that can be already offered with possibilities of success in our country. Although the colonic enterocytes express MVD, the recipient ileocaecal valve and colon can be preserved.

[Activity of a pediatric intestinal transplantation program in Spain]. / Actividad de un programa de trasplante intestinal pediátrico en España.

AIM: To analyze the outcome of children with intestinal failure (IF) included as candidates for intestinal transplantation (IT). Patients, Eight out of 23 children with IF assessed since July 1997 met criteria for IT and were included on the waiting list. The causes of IT were Short Bowel Syndrome (SBS) in 6 and Microvillus Inclusion Disease (MID) in 2. The indication of IT were end stage liver disease (ESLD) in 5 (related to total parenteral nutrition administration, TPN), progressive hepatic fibrosis in 2 and loss of venous access in 1. The patients with ESLD were included for combined liver-small bowel transplantation (LSBT) and the remaining for isolated intestinal transplantation (IIT). RESULTS: Two children died waiting for LSBT, 4 patients are on the waiting list, 2 for LSBT and 2 for IIT (length of stay: 4-11 months). Two children were transplanted, one with IIT in a 2.5 years old boy with MID, and one with LSBT in a 22 months girl with SBS and ESLD. Both patients recovered intestinal function after transplantation and are a live (follow-up of 19 and 10 months respectively). The LSBT's patient is under treatment for postransplant lymphoproliferative disease (PTLD). CONCLUSIONS: The lack of suitable donors for the small children candidate to IT explains the long period of stay on the waiting list and the high pretransplant mortality. Two strategies are possible; early referral of children with IF to a transplant center and surgical techniques like ex vivi-hepatic reductions of the LSB graft.

[Cost-benefit analysis of the anatomo-pathological study of tonsillectomy specimens in the pediatric population]. / Análisis costo-beneficio del estudio anatomopatológico de las piezas de amigdalectomía en la población pediátrica.

To study the cost-benefit of the histological examination of tonsilar samples, we evaluated 567 cases (547 routine and 20 nonroutine cases) of patients under 14 years of age, operated between 1st January 1996 and 30th November 2000. There were 2 routine cases (0.3%), and 6 nonroutine cases (30%) with a diagnosis different to follicular hyperplasia. In this way, our clinical preoperative sensitivity was 75% and specificity 97%. The average cost per case at our Centro to study the tonsilar samples was 30$. We conclude that the histological examination of tonsilar specimens is economically worth only in nonroutine cases, although additional factors (e.g. training of residents of Pathology and the quality control of the institution must be taken into consideration.