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Abstract Ursodeoxycholic acid is currently the only established drug for the treatment of chronic cholestatic liver diseases. It has cytoprotective, anti-apoptotic, membrane stabilizing, anti-oxidative and immunomodulatory effects. Prolonged administration of ursodeoxycholic acid in patients with primary biliary cirrhosis (PBC) is associated with survival benefit and a delaying of liver transplantation. There is evidence that it might even prevent progression of the histologic stage of PBC. It also has a beneficial effect on primary sclerosing cholangitis, intrahepatic cholestasis of pregnancy, liver disease associated with cystic fibrosis, chronic graft versus host disease, total parenteral nutrition associated cholestasis and various pediatric cholestatic liver diseases. In the present review the current knowledge about the mechanisms of the action and role of ursodeoxycholic acid in the treatment of various liver diseases has been discussed. [ABSTRACT FROM AUTHOR]

Objective: This paper aims to highlight the clinical features, investigations and treatment of retroperitoneal teratomas condition.Methods: 12 patients (8 females and 4 males, age range-2 months to 14 yrs) of retroperitoneal teratoma admitted to the department of Pediatric Surgery, King George Medical University, Lucknow between 1980 and 2004 were studied. Investigations included hematology, plain X-ray of the abdomen, intravenous urography, ultrasound, computerised tomography (CT) of the abdomen (after 1990, 8 patients) and serum alpha-fetoprotein assay (after 1991, 6 patients, preoperatively). All patients underwent surgery. Serum alpha-fetoprotein assay was used during follow-up to detect recurrence.Results: Majority of the tumors were left pararenal in location. In two patients there was bilateral involvement. In all except one, the tumor could be excised easily preserving the kidneys. In one child with a massive cystic tumor with bilateral involvement, the tumor was marsupialised in the first stage and excised subsequently. One child died postoperatively, the other 11 children are well and there has been no tumor recurrence on follow-up.Conclusions: Retroperitoneal teratomas are uncommon lesions in children mostly arising in close relation to the kidneys. The majority are benign but complete excision is necessary for cure. Even large tumors with bilateral involvement of the retroperitoneum can be excised while preserving adjacent organs. Serum alpha-fetoprotein assay is a reliable method of detecting recurrence. [ABSTRACT FROM AUTHOR]

The role of diet and its manipulation in the management of inflammatory bowel disease (IBD) is gradually acquiring central stage. Certain dietary factors have been identified as putative triggers in IBD as some other factors are found to be protective. The dietary manipulation as part of comprehensive IBD care should be done by the clinician in conjunction with a skilled dietitian. Nutritional deficiencies are common in patients with IBD and can have long-term effects on disease course and quality of life in these patients. So, early identification and correction of these deficiencies along with proper nutritional supplementation should be addressed routinely as a part of IBD management. Oral nutritional supplementation is sufficient for most patients, but in some sick patients, tube feeding may be necessary. Diet needs to be individualized based on the nutritional deficiencies and dietary triggers in a specific patient. Multiple specific diets, with elimination of components that trigger inflammation or addition of components that alter gut microbes in a favorable way, are now appearing as a treatment option in IBD, but more evidence is required before their universal recommendation. Though enteral nutrition (EN) (both exclusive enteral nutrition [EEN] and partial enteral nutrition [PEN]) have proven therapeutic role in pediatric IBD, their uses and role are now expanding in adult IBD patients as well.

Background and Aim: The absence of lactase in the intestinal villi due to mucosal injury or genetic factors causes undigested lactose to reach the colon where it is fermented. Lactose intolerance is diagnosed based on clinical symptoms like bloating, abdominal pain and flatulence, lactose hydrogen breath test (HBT), and lactose tolerance test. No Indian studies are available on the use of lactase supplements. The aim was to study the effect of lactase chewable tablets on clinical symptoms and hydrogen breath excretion in patients with lactose intolerance., Methods: This was a randomized, double-blind, crossover placebo-controlled trial to study the effect of lactase tablets on symptoms and hydrogen breath levels in adults with lactose intolerance, confirmed by Lactose HBT. Clinical symptom severity was recorded using a visual analog scale, and HBT was performed every 30 min for 180 min. As it was a crossover design, the same patients were tested with both lactase and placebo, acting as their own controls with a washout period of 1 week between visits., Results: Forty-seven patients (mean age 33.6 years; 30 males) with lactose intolerance formed the study group. Clinical symptoms, mean clinical score ( P  < 0.05), and mean hydrogen breath levels ( P  < 0.05) were improved when the patients were given lactase. Reduction in cumulative hydrogen breath level over 180 min was 55% when patients received lactase compared to placebo., Conclusions: Orally supplemented lactase enzyme significantly reduced the clinical symptoms and hydrogen breath excretion in patients with lactose intolerance., (© 2020 The Authors. JGH Open: An open access journal of gastroenterology and hepatology published by Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)

Objectives: In the largest head-to-head comparison between an oral and an intravenous (IV) iron compound in patients with inflammatory bowel disease (IBD) so far, we strived to determine whether IV iron isomaltoside 1,000 is non-inferior to oral iron sulfate in the treatment of iron deficiency anemia (IDA)., Methods: This prospective, randomized, comparative, open-label, non-inferiority study was conducted at 36 sites in Europe and India. Patients with known intolerance to oral iron were excluded. A total of 338 IBD patients in clinical remission or with mild disease, a hemoglobin (Hb) <12 g/dl, and a transferrin saturation (TSAT) <20% were randomized 2:1 to receive either IV iron isomaltoside 1,000 according to the Ganzoni formula (225 patients) or oral iron sulfate 200 mg daily (equivalent to 200 mg elemental iron; 113 patients). An interactive web response system method was used to randomize the eligible patient to the treatment groups. The primary end point was change in Hb from baseline to week 8. Iron isomaltoside 1,000 and iron sulfate was compared by a non-inferiority assessment with a margin of -0.5 g/dl. The secondary end points, which tested for superiority, included change in Hb from baseline to weeks 2 and 4, change in s-ferritin, and TSAT to week 8, number of patients who discontinued study because of lack of response or intolerance of investigational drugs, change in total quality of life (QoL) score to weeks 4 and 8, and safety. Exploratory analyses included a responder analysis (proportion of patients with an increase in Hb ≥2 g/dl after 8 weeks), the effect of regional differences and total iron dose level, and other potential predictors of the treatment response., Results: Non-inferiority in change of Hb to week 8 could not be demonstrated. There was a trend for oral iron sulfate being more effective in increasing Hb than iron isomaltoside 1,000. The estimated treatment effect was -0.37 (95% confidence interval (CI): -0.80, 0.06) with P=0.09 in the full analysis set (N=327) and -0.45 (95% CI: -0.88, -0.03) with P=0.04 in the per protocol analysis set (N=299). In patients treated with IV iron isomaltoside 1,000, the mean change in s-ferritin concentration was higher with an estimated treatment effect of 48.7 (95% CI: 18.6, 78.8) with P=0.002, whereas the mean change in TSAT was lower with an estimated treatment effect of -4.4 (95% CI: -7.4, -1.4) with P=0.005, compared with patients treated with oral iron. No differences in changes of QoL were observed. The safety profile was similar between the groups. The proportion of responders with Hb ≥2 g/dl (IV group: 67%; oral group: 61%) were comparable between the groups (P=0.32). Iron isomaltoside 1,000 was more efficacious with higher cumulative doses of >1,000 mg IV. Significant predictors of Hb response to IV iron treatment were baseline Hb and C-reactive protein (CRP)., Conclusions: We could not demonstrate non-inferiority of IV iron isomaltoside 1,000 compared with oral iron in this study. Based on the dose-response relationship observed with the IV iron compound, we suggest that the true iron demand of IV iron was underestimated by the Ganzoni formula in our study. Alternative calculations including Hb and CRP should be explored to gauge iron stores in patients with IBD.

Significance: Chronic pancreatitis (CP) is a progressive, inflammatory disease of the pancreas leading to slow destruction of pancreatic parenchyma and progressive fibrosis. The pathophysiological mechanism of CP is not well understood., Recent Advances: A pathophysiologic role of oxidative stress in CP has, however, been suggested in recent years. Pancreatic acinar cells contain phase I cytochrome P450 (CYP 450) biotransforming enzymes and phase II conjugation reactions for the metabolism of xenobiotics. The oxidative stress in the acinar cell may result from generation of free radicals through CYP induction, concurrent exposure to a chemical that undergoes bioactivation, and insufficiency of micronutrients that are required to sustain antioxidant (AO) capacity., Critical Issues: Studies have shown that there is indeed a state of oxidative stress as evidenced by increased levels of products of oxidative stress and reduced AO capacity in patients with CP. A recent randomized, controlled trial has shown beneficial effect of AO therapy in CP; a combination of AOs (0.54 g ascorbic acid, 9000 IU β-carotene, 270 IU α-tocopherol, 600 μg organic selenium, and 2 g methionine per day in divided doses) led to significant reductions in pain and oxidative stress in patients with CP., Future Directions: Similar studies from other centers and multicenter studies should confirm that oxidative stress plays an important role in the pathophysiology of CP and supplementation with AOs leads to significant pain relief in patients with this disease.

The Asia-Pacific region has been marked as an area with a low incidence of inflammatory bowel disease (IBD), although confusion always existed as to whether this low incidence was a result of low diagnostic awareness, a high incidence of infective diarrhoea and its diagnostic overlap or a true low incidence. As epidemiological studies from this region are being made available it is clear that the incidence and prevalence rates of IBD in Asia-Pacific region are low compared with Europe and North America. They are however, increasing rapidly. There are substantial variations in the incidence and prevalence rates of IBD in various ethnic groups in Asia. The highest incidence rates are recorded from India, Japan and the Middle East and there exists a genetic predisposition of South Asians (Indians, Pakistanis and Bangladeshis) to ulcerative colitis (UC). It appears that certain racial groups are more prone than others to develop IBD. For instance, Indians in South-East Asia have higher rates than Chinese and Malays. While there is a host genetic predisposition, environmental factor(s) may be responsible for this difference. The clinical phenotypes and complication rates of Asian IBD resemble those of the Caucasian population in general, but some heterogeneity is observed in different regions of Asia. There is no evidence of a north-south or an east-west divide in the Asia-Pacific region. The available studies suggest an increasing incidence of UC in the Asia-Pacific region and hence it is an appropriate time to launch well-designed epidemiological studies so that etiopathogenetic factors can be identified. There is a male predominance in Crohn's disease in the Asian population. The NOD2/CARD15 gene is not associated with CD in the Japanese, Korean, Chinese and Indian population.

Background & Aims: Probiotics can maintain ulcerative colitis (UC) in remission effectively, but little is known of their ability to induce remission. We conducted a multicenter, randomized, double-blind, placebo-controlled trial of a high-potency probiotic, VSL#3, for the treatment of mild-to-moderately active UC., Methods: Adult patients with mild-to-moderate UC were assigned randomly to groups that were given 3.6 x 10(12) CFU VSL#3 (n = 77) or placebo (n = 70), twice daily for 12 weeks. The primary end point was a 50% decrease in the Ulcerative Colitis Disease Activity Index (UCDAI) at 6 weeks. The secondary end points included remission by 12 weeks and reduction in total individual UCDAI parameters from baseline at 12 weeks. Intention-to-treat analysis was performed., Results: At week 6, the percentage of patients with an improvement in UCDAI score that was greater than 50% was significantly higher in the group given VSL#3 (25; 32.5%) than the group given placebo (7; 10%) (P = .001). At week 12, there were 33 patients given VSL#3 (42.9%) who achieved remission, compared with 11 patients given placebo (15.7%) (P < .001). Furthermore, significantly more patients given VSL#3 (40; 51.9%) achieved a decrease in their UCDAI that was greater than 3 points, compared with those given placebo (13; 18.6%) (P < .001). The VSL#3 group had significantly greater decreases in UCDAI scores and individual symptoms at weeks 6 and 12, compared with the placebo group., Conclusions: VSL#3 is safe and effective in achieving clinical responses and remissions in patients with mild-to-moderately active UC.

Purpose: Vestibular fistula is the commonest anorectal malformation in the female child. This article reports the treatment and long-term follow-up of 1206 patients of vestibular fistula treated by anterior sagittal anorectoplasty (ASARP) in a single center for 38 years., Material and Methods: All patients of vestibular fistula admitted and operated on at the Department of Pediatric Surgery, King George Medical University (Lucknow, UP India), from 1970 were included in the study; the age ranged from 2 days to 40 years. The diagnosis was made by clinical examination. We differentiated between anovestibular fistula (AVF) and rectovestibular fistula (RVF) in that the latter is a longer narrow fistula closely applied to the posterior wall of the vagina. Preoperative investigations included hemogram and blood glucose. Echocardiography was done in those patients showing a physical sign of cardiac anomaly. All patients were operated on in the lithotomy position by ASARP; this was done without colostomy in 1169 patients. In 6 patients, preliminary colostomy was done because of excessive perineal excoriation, and 31 others had colostomy done elsewhere. The striated muscle complex was delineated by electrostimulation, and anoplasty was performed after anchoring the rectum within the muscle complex. Washing of the perineum after passage of stools with application of povidone-iodine ointment constituted the local care. Intravenous antibiotics were administered for 48 hours and oral antibiotics (including metronidazole) for 5 days. The patient was discharged home by the fifth day., Results: Follow-up ranges from 3 months to 19 years; uneventful postoperative recovery was seen in 1147 patients. They had normal growth and development, normal appearance of the perineum, and a normal quality of life. Complications were seen in 60 patients (5%) of which 42 had AVF and 18 had RVF. Eight patients had postoperative wound disruption that was minor in 4 and required colostomy in the other 4. Four patients had recurrence of vestibular fistula thus creating an iatrogenic perineal canal; this could be repaired by a second ASARP in 3 patients and required colostomy and PSARP in one child. Anal stenosis was seen in 11 patients; this was treated by dilatation alone in 6 and required posterior Y-V plasty in 5. The rate of complications in RVF was lower than AVF probably because of lesser number of patients; there was no difference in stooling pattern or continence between uncomplicated patient of RVF and AVF; however, fecal staining was seen in all patients undergoing revision surgery for complications., Conclusions: This experience with ASARP showed a good result in 95% patients in a single-stage procedure. The technical ease and minimal preoperative and postoperative measures make ASARP the procedure of choice for vestibular fistula in females at all ages. Until sufficient experience is gained, it may be safer to operate on patients with RVF under cover of a protective colostomy.

Survival rates for infants who have esophageal atresia (EA) with or without fistula (TEF) have improved dramatically in the past 50 years. Despite excellent long-term survival for patients with esophageal atresia with tracheoesophageal fistula (EA-TEF), many significant complications can occur. Anastomotic leak at the esophagoesophagostomy site is one such problem resulting in considerable morbidity and mortality in these patients. The methods of esophageal anastomosis for long period has remained the simple end to end anastomosis of esophageal ends with various modifications described from time to time. The present study aims to study the effect on the early postoperative complications, following horizontal mattress suture technique on the primary esophageal anastomosis in cases of EA-TEF. A total of 32 patients with EA-TEF, were operated by our technique during a period of 1 year (2007-2008). The results were compared with the patients (n = 66), who were operated by the traditional simple technique during the same period. Among those patients in whom the esophageal anastomosis was done by horizontal mattress suture, only one had major anastomotic leak, while two had minor anastomotic leaks, as compared to six and nine cases correspondingly in other patients in whom anastomosis was done by simple technique. There was single mortality. We propose that, the utilization of our technique of horizontal mattress suture in primary anastomosis of esophagus in cases of EA-TEF significantly reduces the risk of anastomotic leaks and subsequent morbidity and mortality.

Objectives: The purpose of the study is to present the authors' clinical and surgical experience with congenital bladder diverticula in nine pediatric patients at a developing world tertiary care center., Patients and Methods: Records of nine patients diagnosed and treated as congenital bladder diverticula from 2000 to 2007 were retrospectively reviewed for age, sex, chief complaints, associated anomalies, investigative work-up, operative notes, and postoperative follow-up., Results: All were males. Age at presentation ranged from six months to eight years (mean three years). All were diagnosed postnatally by ultrasound and/or voiding cystourethrography (VCUG) and confirmed on urethrocystoscopy. Open surgical excision of diverticulum was done in all the patients. Ureteral reimplantation was simultaneously done only in three patients with VCUG-documented high-grade vesicoureteral reflux (VUR). With an average follow-up of four years, gradual resolution of symptoms was seen in seven of nine patients whose postoperative follow up records were available. There was no diverticulum recurrence at the defined mean follow-up., Conclusion: Pediatric patients with recurrent urinary tract infections and voiding dysfunction should always be evaluated for congenital bladder diverticulum. Although investigations such as intravenous urography (IVU), urodynamic studies, nuclear renal scanning, and, sometimes, CT scan and MRI, form an important part of preoperative diagnostic work-up and postoperative follow up, USG and VCUG may be enough when availability and cost are the constraints. Diverticulectomy, with ureteral reimplantation for high-grade reflux, provides good results without recurrence.

Aims: It was the aim of this study to report a prospective study of 110 spina bifida patients with hydrocephalus treated by simultaneous ventriculoperitoneal (VP) shunt and repair of the meningomyelocele., Methods: Between January 2005 and February 2008, 264 patients with spina bifida were admitted to the author's department. Those patients in whom the preoperative cranial ultrasonography measurement of the bifrontal diameter was >26 mm, the bicaudate diameter >20 mm and the diameter of the body of the lateral ventricle >26 mm were predicted to develop postoperative hydrocephalus (n = 245). Of these, 110 patients underwent simultaneous (group 1) VP shunt with repair of the meningomyelocele, while 135 (group 2) underwent sequential surgery due to the preference of the attending consultant., Results: Twenty-two of the 110 patients in group 1 developed complications (wound problems, cerebrospinal fluid leak, shunt malfunction, death) compared with 38 of 135 patients in group 2. The remaining patients had an uneventful postoperative and follow-up course., Conclusions: Simultaneous VP shunt with surgery for hydrocephalus was feasible in our setup. The rate of complications was not higher than in staged surgery. Single-stage repair of spina bifida with hydrocephalus offers considerable advantage in the form of a reduction in hospital burden, costs and patient morbidity., (2009 S. Karger AG, Basel)

Background & Aims: Oxidative stress has been implicated in the pathophysiology of chronic pancreatitis (CP). We evaluated the effects of antioxidant supplementation on pain relief, oxidative stress, and antioxidant status in patients with CP., Methods: In a placebo-controlled double blind trial, consecutive patients with CP were randomized to groups that were given placebo or antioxidants for 6 months. The primary outcome measure was pain relief, and secondary outcome measures were analgesic requirements, hospitalization, and markers of oxidative stress (thiobarbituric acid-reactive substances [TBARS]) and antioxidant status (ferric-reducing ability of plasma [FRAP])., Results: Patients (age 30.5+/-10.5 years, 86 male, 35 alcoholic, and 92 with idiopathic CP) were assigned to the placebo (n=56) or antioxidant groups (n=71). After 6 months, the reduction in the number of painful days per month was significantly higher in the antioxidant group compared with the placebo group (7.4+/-6.8 vs 3.2+/-4, respectively; P< .001; 95% CI, 2.07, 6.23). The reduction in the number of analgesic tablets per month was also higher in the antioxidant group (10.5+/-11.8 vs 4.4+/-5.8 respectively; P= .001; 95% CI, 2.65, 9.65). Furthermore, 32% and 13% of patients became pain free in the antioxidant and placebo groups, respectively (P= .009). The reduction in the level of TBARS and increase in FRAP were significantly higher in the antioxidant group compared with the placebo group (TBARS: placebo 1.2+/-2.7 vs antioxidant 3.5+/-3.4 nmol/mL; P= .001; 95% CI 0.96, 3.55; FRAP: placebo -5.6+/-154.9 vs antioxidant 97.8+/-134.9 microMFe(+2) liberated, P= .001, 95% CI 44.98, 161.7)., Conclusions: Antioxidant supplementation was effective in relieving pain and reducing levels of oxidative stress in patients with CP.

Pain is the most distressing symptom of chronic pancreatitis. Although the pathogenesis of pain is still poorly understood, an increase in intraductal pressure may be the dominant factor. The management of pain can involve medical, endoscopic, neurolytic, and surgical therapies. Endotherapy includes pancreatic sphincterotomy, extraction of stones, placement of stent, and dilatation of strictures, sometimes preceded or followed by extracorporeal shock-wave lithotripsy. Several studies have now shown that endotherapy provides partial or complete relief of pancreatic pain in a majority of patients with an acceptable frequency of early and late complications. Endotherapy should now graduate from an experimental form of treatment to a realistic treatment option in patients with chronic or relapsing pain, particularly in the setting of calcific chronic pancreatitis.

Purpose: The aim of this paper is to describe the management of 9 patients with anorectal junction stenosis and present the diagnostic features together with a single-stage surgical technique with reproducible results., Materials and Methods: Nine patients with anorectal junction stenosis were seen over a period of 12 years. The children (aged from 2 months to 15 years) presented with constipation . Anorectal junction stenosis was diagnosed by rectal examination during which the tip of the finger was unable to pass beyond the stenosis in all cases. Contrast study showed the dilated rectosigmoid proximal to the stenosis., Results: Six patients (who did not have significant rectal dilation) underwent single-stage surgery by posterior Y-V plasty which was curative. Two patients were operated through the posterior sagittal route with a covering colostomy done during the same session; the oldest child required resection of the megasigmoid with abdominoperineal pull-through. None of the six patients operated with Y-V plasty experienced any complications. One of the patients operated via the posterior sagittal route had a leak from the anorectal anastomosis requiring revision. The follow-up ranged from 6 months to 12 years. All patients are alive and well and there was no recurrence of stenosis in any case., Conclusion: Anorectal junction stenosis is a rare anorectal anomaly easily diagnosed by digital rectal examination. Single-stage surgery by posterior Y-V plasty is effective in curing the majority of these patients if significant rectosigmoid dilation is not present.

Background and Aim: Patients with chronic pancreatitis are often malnourished. The role of malnutrition in the pathogenesis of chronic pancreatitis is unclear. The aim of the present article was to study prospectively the cause and effect relationship of malnutrition with idiopathic chronic pancreatitis in a case-control study., Methods: Consecutive patients with chronic pancreatitis underwent anthropometry, nutritional and dietary assessments. For dietary assessment, food frequency questionnaire and 24-hour dietary recall methods were used. Primary outcome measure was cause and effect relationship of malnutrition with idiopathic chronic pancreatitis., Results: Of 201 patients with chronic pancreatitis, 120 had idiopathic chronic pancreatitis (mean age 29.60 years, 74 males) who formed the study group. None of the patients consumed cassava. The nutritional status and dietary intake of the patients before the onset of chronic pancreatitis were comparable with those of controls with 20.6% of patients and 22.5% of controls being malnourished (body mass index [BMI] < 18.5). After the onset of chronic pancreatitis, 56.5% of patients lost weight and significantly more patients became malnourished compared with controls (45.8% vs 22.5%; P < 0.001). The causes of weight loss were diabetes, higher calories from proteins, and pseudocyst., Conclusion: Malnutrition was not a cause of idiopathic chronic pancreatitis and weight loss occurred as an effect of chronic pancreatitis. Cassava was not found to be a cause of idiopathic chronic pancreatitis.

Geographical differences have been shown in the clinical outcomes of Helicobacter pylori-associated gastritis phenotypes and in gastric cancer risk. This study tested whether the Operative Link on Gastritis Assessment (OLGA) staging correlated with gastric cancer risk in populations from 3 continents. Mapped gastric biopsies were obtained from 316 dyspeptic adults aged less than 41 years from 8 geographic areas that differed in gastric cancer risk. Gastric atrophy was assessed according to internationally validated criteria. Gastritis stage was established according to the OLGA staging system. The most prevalent gastritis stages were 0 to II, which included all subjects entered from Chile, Germany, India, Italy, and Thailand. Gastritis Stages III and IV were limited to the Chinese and Korean populations. Indians had a high prevalence of H pylori infection without high-stage gastritis. In populations at different cancer risk, the gastritis OLGA stage mirrored the gastric cancer incidence. Gastritis staging identifies a subgroup of higher-risk patients.

Aim: The purpose was to evaluate the diagnosis and efficacy of management of congenital diaphragmatic hernia (CDH) in a tertiary health center of a developing country., Methods: Forty-six children aged from 1 day to 7 years were studied. Parameters studied were age, sex, clinical features, and management., Results: Fifty-six percent of patients presented in the neonatal period; however, none of them presented on the first day of life. The majority (91.3%) of patients had left-sided CDH. Respiratory distress was the most common clinical feature observed (91.3%). Chest X-ray confirmed the diagnosis in 82.6% of patients, and contrast study was needed in the remaining 17.4%. The survival rate was 87%. It was better in patients presenting late than those presenting in the early neonatal period. Stabilization in the preoperative period improved survival. Not using a chest tube had no adverse effect on survival., Conclusion: The relatively increased survival rate of CDH in a tertiary health center of a developing country is attributed to delayed arrival to the center. Respiratory infections compound the survival. More studies are needed before it can be safely said that not using a chest tube has no adverse outcome. Late presentation has been associated with varied manifestations, hence proper clinical evaluation, a high index of suspicion and adequate management, which includes imaging and surgery after stabilization, gives excellent results.

Objective: To study the clinical profile of the cases of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and various factors affecting the surgical and early postoperative management and their outcome., Materials and Methods: A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading., Results: EA with TEF was the commonest type in 117 cases (92%). Associated congenital anomalies were present in 52 (41%) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5%) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (P = < 0.001). Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%). Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases). Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C., Conclusion: Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.

Chronic pancreatitis (CP) is characterized by progressive fibrosis, pain and/or loss of exocrine and endocrine functions. Recent in vitro and in vivo experiments have proven objectively the role of activated pancreatic stellate cells (PSC) in fibrogenesis in CP. Molecular mediators shown to regulate the pathogenesis include transforming growth factor beta (TGF-beta), platelet-derived growth factor (PDGF), and pro-inflammatory cytokines such as IL-1, IL-6 and TNF-alpha. Furthermore, molecular pathways involving mitogen-activated protein kinases (MAPK), phosphatidyl inositol 3-kinase (PI3K), Ras superfamily G proteins, serine threonine protein kinase Raf-1 and peroxisome proliferator activated receptor gamma (PPAR-gamma) have been elucidated. Understanding of the pathogenesis has led to identification of novel molecular targets and development of potential newer therapeutic agents. Those found to retard the progression of experimental CP and fibrosis in animal models include interferon (IFN) beta and IFN-gamma; a Japanese herbal medicine called Saiko-keishi-to (TJ-10); curcumin; PPAR-gamma ligand (troglitazone); antioxidants (vitamin A, vitamin E, DA 9601 and epigallocatechin-3-gallate); a protease inhibitor (camostat mesilate) and hydroxymethylglutaryl-CoA inhibitor (lovastatin). This review summarizes the current literature addressing the role of different pharmacological agents aimed at reducing or preventing inflammation and the consequent fibrogenesis in CP.

Urethrocutaneous fistula is a common complication after hypospadias repair. If congenital, it is usually associated with other genitourinary and gastrointestinal anomalies. Isolated congenital urethral fistula is a very rare anomaly. We present a 4-year old circumcised boy with this unusual anomaly. Etiology, embryology, and management are discussed. We emphasize meticulous clinical examination for the diagnosis and to rule out other associated anomalies.

Chronic diarrhea and steatorrhea occur frequently in patients with autoimmune polyglandular syndrome (APS) type I. Intestinal lymphangiectasia has been reported earlier as a cause of steatorrhea in a young girl with APS Type I. We describe 2 patients with APS Type I who were found to have intestinal lymphangiectasia, one of whom had symptomatic protein-losing enteropathy.

Total gut irrigation (TGI) is a safe method of bowel preparation in children. Many solutions are used but none is ideal. This study has been done to compare household common salt solution prepared by dissolving 10 g of pure NaCl salt (Active Reagent Quality) in 1 l of tap water, polyethylene glycol with electrolytes (PEG) and ringers lactate for TGI in children. We prospectively evaluated the three solutions in terms of efficacy, safety, rapidity, tolerability and cost effectiveness in patients undergoing a variety of colorectal procedures. Patients (126) were randomly assigned into one of the three groups; Group I, household common salt solution, 40; Group II, Peglec, 55; Group III, Ringer lactate, 31. TGI with PEG is the most rapid method of bowel preparation but is least tolerable. Household common salt solution is inexpensive and most tolerable of the three preparations. All three are similar in safety and effectiveness in bowel preparation. Household common salt solution is effective, safe, cost effective and the most tolerable method of bowel preparation.

Background and Aim: Cytokines play an important role in the pathogenesis of acute pancreatitis (AP). The aim of the present paper was to study the profile of anti- and proinflammatory cytokines in AP and to determine their predictive value for severity of AP, organ failure and mortality., Methods: Consecutive patients with AP were included in the study. Cytokines were measured in those patients who presented within the first 72 h of the onset of AP. Plasma levels of proinflammatory cytokines tumor necrosis factor (TNF)-alpha, interleukin (IL)-Ibeta, IL-6 and anti-inflammatory cytokine IL-10 were measured on days 1, 3, 7 and 14 of AP., Results: Of 108 patients, 30 presented within 72 h of the onset (mean age 40.27 +/- 13.89 years; 22 males). Of the 30 patients, 13 (43.3%) had severe and 17 (56.7%) had mild pancreatitis. Eleven (36.7%) patients developed organ failure and three died. The level of IL-6 on day 3 was significantly higher in severe pancreatitis than in mild pancreatitis (146.29 +/- 57.53 pg/mL vs 91.42 +/- 71.65 pg/mL; P = 0.04) and was significantly higher in patients who developed organ failure compared with those who did not (161.59 +/- 53.46 pg/mL vs 88.16 +/- 65.50 pg/mL; P = 0.004). At a cut-off value of 122 pg/mL on day 3, IL-6 predicted organ failure and severe pancreatitis with a sensitivity and specificity of 81.8% and 77.7%, respectively. TNF-alpha and IL-10 were detectable only in one-third of patients and were not related to the severity of pancreatitis, while Il-1beta was not detectable., Conclusion: Elevated levels of IL-6 predicted organ failure and severe pancreatitis and suggested its pathophysiological significance in AP.

Hepatic calcification can be seen with various infectious and neoplastic conditions. We report a 32-year- old man who developed massive calcification in the right lobe of liver following recovery from dengue virus-associated fulminant liver failure.

Background & Aims: The cause of recurrent acute pancreatitis (RAP) is not known in 10%-30% of patients. The aim of the present study was to determine the cause of idiopathic RAP in a long-term follow-up study., Methods: All consecutive patients with idiopathic RAP underwent detailed evaluations and investigations to find out the cause. The pancreatitis was considered to be idiopathic when no cause could be found after standard investigations that included serum biochemistry, transabdominal ultrasonography, and computerized tomography scan of the abdomen. The detailed work-up included repeat serum biochemistry and transabdominal ultrasonography, an endoscopic retrograde cholangiopancreatography, duodenal bile microscopy to diagnose biliary microlithiasis, and endoscopic ultrasonography., Results: Seventy-five patients were studied from June 1995 to May 2003. Their mean age was 31.9 years and 80% were male. The mean number of attacks of acute pancreatitis was 4.82 (range, 2-10). The cause of RAP was attributed to biliary microlithiasis in only 10 (13%) of 75 patients. Two additional patients developed gallstones during the follow-up period. Thirty-five (47%) patients developed chronic pancreatitis during the follow-up period. Ten of these 35 patients with chronic pancreatitis had biliary microlithiasis; 8 of these 10 patients had undergone cholecystectomy/endoscopic sphincterotomy yet continued to have recurrent pancreatitis and developed chronic pancreatitis. Miscellaneous causes were found in 10 (13%) patients. No cause was found in the remaining 18 (24%) patients., Conclusions: Microlithiasis was not a significant cause of idiopathic RAP in our patients. About one half of the patients with RAP developed chronic pancreatitis during the follow-up period.

Unlabelled: Aim/Purpuse: To evaluate our experience of 19 patients of lymphangioma who were treated by intralesional Bleomycin., Materials and Methods: Nineteen patients of lymphangioma aged between 16 days to 11 years were managed in the department. The male-female ratio was of 2:1. Commonest sites were in the neck (58%) followed by axilla (21%). The patients were treated by intralesional bleomycin injection. Bleomycin was given at a dose not exceeding 0.5 unit/kg/dose at interval of 2 weeks. Reduction in size of the mass was noted in between 2 weeks to 16 weeks and number of injections required for each patient varied from 1 to 6. Follow up ranged from 1- 7 Year., Result: In injection group, significant reduction of mass was noted in 84% (n=l6) and 57% (n=11) of them showed complete disappearance. No serious complications were noted in any patient., Conclusion: Our experience showed that Bleomycin in aqueous solution is a good sclcrosing agent in the management of lymphangioma.

Background: Acute pancreatitis (AP) is the commonest complication of endoscopic retrograde cholangiopancreatography (ERCP). Data regarding the clinical course and outcome of post-ERCP pancreatitis are sparse, although the available data suggest it to be a severe disease., Objective: To examine the clinical course, disease severity, and outcome of patients with post-ERCP-AP., Methods: All consecutive patients with post-ERCP-AP were included. They were managed according to a standard protocol. Outcome measures were severity of pancreatitis, infectious complications, need for surgery and mortality. The clinical course and outcome of patients with post-ERCP-AP were also compared with those of patients with gallstone pancreatitis (GS-AP)., Results: Of the 1497 de novo ERCP procedures, 57 (3.8%) patients developed AP. Their mean age was 40.2 years (13.1), 16 were males of them, 54 (95%) patients had mild pancreatitis. Only 2 patients developed organ failure. Fifty-four (95%) patients recovered with conservative management. One of the 57 patients died. As compared with patients with GS-AP (n=174), APACHE II scores at admission [3.3 (3.1) vs. 5.8 (4.8); P=0.011], occurrence of pancreatic necrosis (17.5% vs. 39.1%; P=0.020), organ failure (3.5% vs. 19.0%; P=0.015), infectious complications (8.7% vs. 24.7%; P=0.040), and mortality (1.8% vs. 13.2%; P=0.044) were significantly less among patients with post-ERCP-AP., Conclusion: Unlike previous belief, we found that post-ERCP AP was a mild disease with a favorable outcome in most cases.

Background: Necrotizing fascitis (NF) is a severe infection of the subcutaneous tissue and fascia affecting children and adults. Conventional management includes resuscitation, aggressive debridement of necrotic tissue, and sometimes, additional measures such as hyperbaric oxygen and immunoglobulin therapy. This paper reports conservative management of 18 patients with NF with minimal morbidity and mortality., Material and Methods: Patients with NF admitted to our department between January 2000 and February 2004 were included in the study (N = 18). In all cases, the presentation was rapidly progressing cellulitis progressing to cutaneous gangrene between 6 and 18 hours. The patients were managed by aggressive fluid resuscitation, analgesia, broad-spectrum antibiotics, and dressing with liberal quantities of povidone iodine ointment. After separation of the gangrenous skin margins from the surrounding healthy tissue between 24 and 72 hours, dead skin and fascia were removed with forceps on the ward, the wound washed with liberal quantities of water, and the ointment dressing reapplied. This procedure was repeated until all the dead tissue had been removed. Once the wound was granulating, dressings were changed at increasing intervals until healing took place by secondary intention., Results: The patients were aged between 5 days and 11 years. In all, NF began as a small boil progressing to a rapidly spreading cellulitis. None of the patients was operated during the acute stage of the infection. Blackening of the skin and separation of the edges occurred within 8-72 hours, the dead tissue was allowed to separate from the granulating base and could be removed at the bedside with minimal blood loss. Blood transfusion was required only in 2 patients where hemoglobin was < 9 gm/dL. Of the 18 patients, 6 grew group A streptococci and staphylococci in a polymicrobial wound culture, whereas the other 12 had polymicrobial flora without streptococci. The clinical course and outcomes were similar in both types of wounds. There was 1 death in the study group, and 1 patient required skin grafting. All other survivors had healing by secondary intention without disability. The period for complete epithelization varied between 3 and 8 weeks. Patients were discharged home when 70% of the wound had healed. There was extensive scarring in 3 children with NF involving the back. The other children had minimal or no scarring. None of the patients had any restriction in the movement of limbs or joints. These findings were compared with 16 retrospective patients of NF treated before January 2000 by the conventional approach of aggressive early debridement, the results of the conservative approach were superior with shorter hospital stay, lower number of blood transfusions, earlier appearance of granulation tissue, and shorter duration of complete healing., Conclusions: We conclude that the conservative management of NF offers advantages in morbidity without compromising the outcome. In our hospital setup, conservative treatment was less expensive and easily carried out. We would therefore advocate conservative management for the treatment of this condition.

We report a 24-year-old woman and a 58-year-old man who developed short-segment esophageal strictures in the upper and mid esophagus two weeks after ingestion of aluminium phosphide tablets. They responded well to endoscopic dilatation.

A 52 yrs old male presented with multiple, painless, firm nodules over extremities, which were mimicking benign neoplastic lesion. Fine needle aspiration was performed from three such nodules which revealed chalky white aspirate. After staining the smears with H&E and Giemsa stains, smears show amorphous pink material, needle shaped crystalline structures, many macrophages and foreign body type giant cells. A diagnosis of gouty tophi was offered which was confirmed by histopathology and serum uric acid level.

Significant proportions of Indian patients with gallbladder cancer are young. Multiple risk factors for gallbladder cancer are recognized among Indian patients. The effect of these risk factors on the age of development of gallbladder cancer is not known. We conducted a study to determine the influence of risk factors on the age at diagnosis of patients with gallbladder cancer and to assess the interactions between these risk factors. Patients with gallbladder cancer from two tertiary care institutions during the period 1994-2001 were prospectively studied. An ultrasound examination was done to look for the presence of gallstones. The influence of gender, gallstones, socio-economic status, smoking, residence in rural areas and in the Gangetic belt on the age at presentation was analysed using univariate analysis, logistic and linear regression analyses. The mean age of the 121 patients studied was 55+/-11.7 (SD) years. There were 51 (42%) patients aged less than 50 years. The younger patients (age < or =50 years) were more likely to have gallstones (88 versus 66%; P=0.008) and to have come from a lower socio-economic background (88 versus 71%; P=0.02) in comparison with older patients. However, there was no effect of the other risk factors. The independent determinants for younger age of patients with gallbladder cancer on logistic regression analysis were gallstones [odds ratio (OR) 4, 95% confidence interval (CI) 1.5-11; P=0.006] and lower socio-economic status (OR 3.1, 95% CI 1.1-8.6; P=0.03). On linear regression analysis, age at presentation was lowered by 5.6 years if there were associated gallstones. The mean age of patients with these two risk factors was significantly lower than that of those with one risk factor or no risk factor (52+/-12 years versus 57+/-11 years versus 61+/-9 years; P=0.007). In conclusion, the presence of gallstones and lower socio-economic status were both independently associated with a younger age at diagnosis of gallbladder cancer and their effect was additive.

Stress, defined as an acute threat to homeostasis, evokes an adaptive or allostatic response and can have both a short- and long-term influence on the function of the gastrointestinal tract. The enteric nervous system is connected bidirectionally to the brain by parasympathetic and sympathetic pathways forming the brain-gut axis. The neural network of the brain, which generates the stress response, is called the central stress circuitry and includes the paraventricular nucleus of the hypothalamus, amygdala and periaqueductal gray. It receives input from the somatic and visceral afferent pathways and also from the visceral motor cortex including the medial prefrontal, anterior cingulate and insular cortex. The output of this central stress circuit is called the emotional motor system and includes automatic efferents, the hypothalamus-pituitary-adrenal axis and pain modulatory systems. Severe or long-term stress can induce long-term alteration in the stress response (plasticity). Corticotropin releasing factor (CRF) is a key mediator of the central stress response. Two CRF receptor subtypes, R1 and R2, have been described. They mediate increased colonic motor activity and slowed gastric emptying, respectively, in response to stress. Specific CRF receptor antagonists injected into the 0 block these visceral manifestations of stress. Circulating glucocorticoids exert an inhibitory effect on the stress response by receptors located in the medial prefrontal cortex and hippocampus. Many other neurotransmitters and neuroimmunomodulators are being evaluated. Stress increases the intestinal permeability to large antigenic molecules. It can lead to mast cell activation, degranulation and colonic mucin depletion. A reversal of small bowel water and electrolyte absorption occurs in response to stress and is mediated cholinergically. Stress also leads to increased susceptibility to colonic inflammation, which can be adaptively transferred among rats by sensitized CD4(+) lymphocytes. The association between stress and various gastrointestinal diseases, including functional bowel disorders, inflammatory bowel disease, peptic ulcer disease and gastroesophageal reflux disease, is being actively investigated. Attention to the close relation between the brain and gut has opened many therapeutic avenues for the future.

Background: Gallbladder cancer (GBC) is one of the most common gastrointestinal malignancies. The data regarding GBC are, however, limited., Methods: Records of 634 patients with GBC over a 10-year period were examined with regard to the clinical presentation, investigative findings, treatment, operative findings and outcome., Results: The mean age of patients was 51 +/- 11 years and men : women ratio was 0.36:1.00. Pain, jaundice and hepatomegaly were seen in 81.0%, 76.0% and 61.5% patients, respectively. On imaging, a mass replacing the gallbladder was seen in 73% patients. Gallstones were present in 54% patients. Surgery was carried out in 291 (46%) patients and endoscopic treatment in 72 (19%) patients but no intervention was carried out in the remaining patients because of disseminated disease. Among the patients who were operated on, 2.0% had stage I GBC, 3.4% stage II, 17.5% stage III, 47.0% stage IVa and 29.8% stage IVb. Radical resection was possible in 133 (46%) patients. The 30-day mortality was 10% with most (90%) deaths in patients with stage IV disease. The median survival after simple cholecystectomy and radical surgery was 33.5 and 12.0 months, respectively. However, among those who underwent debulking, palliative bypass or exploratory laparotomy alone, the survival ranged between 1 and 3 months. Logistic regression analysis showed that only radical resection improved the long-term survival (P < 0.05)., Conclusions: The majority of patients with GBC in India have advanced unresectable disease. Detection of GBC at an early stage is incidental and rare but is associated with long-term survival. Radical surgery, when feasible, is the only option for achieving long-term survival., ((c) 2004 Blackwell Publishing Asia Pty Ltd.)