AIM: To study the role of needle knife assisted ampullary biopsy in the diagnosis of periampullary carcinoma.

METHODS: In this study the authors retrospectively analyzed clinical records of patients with periampullary tumors diagnosed by ampullary biopsy taken after needle knife papillotomy in whom surface ampullary biopsies were non contributory.

RESULTS: Between January 2008 and December 2010, 38 patients with periampullary tumors were seen by us and initial side viewing endoscopy with surface biopsy from the papilla was positive for malignancy in 25 patients. Thirteen patients with a negative surface biopsy for malignancy underwent a repeat ampullary biopsy following needle knife papillotomy. There were 8 (61.5%) males and 5 (38.5%) females. The most common presenting symptom was jaundice (100%), followed by fever (46.2%), melena (38.5%), abdominal pain (30.8%) and weight loss (30.8%). All the patients had hyperbilirubinemia with a mean ± SD serum bilirubin of (11.2 ± 1.9) mg/dL (normal value < 1 mg%) and the mean ± SD serum alkaline phosphatase was (288.0 ± 94.3) IU/L (normal value < 129 IU/L). Serum CA 19.9 level estimation was done in 11 patients; it was elevated (cut off value > 70.5 IU/L) in all of them with a median of 1200 IU/L (inter quartile range 274-3500). Side viewing endoscopy showed a bulky papilla in all of them. Adequate tissue was obtained in all of the 13 patients for histological evaluation; 12 of the 13 patients were reported to have adenocarcinoma while one patient had adenoma. There were no complications from the needle knife papillotomy in any of the patients.

CONCLUSION: Needle knife assisted ampullary biopsy appears to be a safe and effective diagnostic modality for periampullary carcinoma.

Endoscopic balloon dilatation (EBD) has important role in the management of benign gastric outlet obstruction. Although there are many reports on the role of EBD in the management of corrosive-induced and peptic benign GOO, there is scanty data on its role in the management of NSAID-induced GOO. We report 10 cases of NSAID-induced pyloroduodenal obstruction and their endoscopic management. The most common site of involvement was duodenum (5/10) followed by both pylorus and duodenum (4/10) and pylorus (1/10). Most of the strictures were short web-like, and the mean (SD) number of stricture was 2.0 (0.94). Endoscopic balloon dilatation was successful in 90% (9/10) cases requiring mean (SD) of 2.0 (1.6) sessions of dilatation to achieve target diameter of 15 mm and mean (SD) of 5.3 (2.7) sessions to maintain it over a treatment period of 4.5 months (IQR 2–15 months). There was no procedure-related complication or mortality.

Background and aims

Acute-on-chronic liver failure (ACLF) is associated with a high mortality rate in the absence of liver transplantation. There is limited data on predictors of survival in ACLF in children. Therefore, we prospectively studied the predictors of outcome of ACLF in children.

Methods

A prospective evaluation of 31 children in the age group of 1–16 years who fulfilled the criteria for ACLF according to Asian Pacific Association for the Study of the Liver (APASL) 2008 consensus was done. All consecutive children were evaluated for etiology, diagnosis and severity of ACLF. For grading of organ dysfunction, the sequential organ failure assessment (SOFA) score was calculated. SOFA constitutes the parameters of respiration, coagulation, cardiovascular system, central nervous system, and renal and liver functions. We evaluated possible correlation between outcomes and different variables.

Results

Of the 31 children who fulfilled the criteria for ACLF, the common underlying chronic liver diseases (CLD) were autoimmune hepatitis (AIH) in 41.9% and Wilson disease in 41.9% of the patients. Superinfection with hepatitis A virus (HAV) (41.9%) was the most common etiology of acute deterioration. To find the best predictor for outcome, linear regression analysis was performed. Multivariate analysis revealed that the SOFA score and the International Normalized Ratio (INR) were predictors of survival. Six (19.4%) patients died. Causes of death were multiorgan failure in four and liver failure in two patients.

Conclusion

The mortality in ACLF is 19.4% and the causes of death were multiorgan failure and liver failure. The SOFA score and INR were predictors of outcome of ACLF in children.

Objective

Celiac disease is an important cause of chronic diarrhea, failure to thrive, and anemia in children. Mode of presentation of celiac disease has changed in last few years. Study was conducted to determine the mode of clinical presentation of a large group of patients with celiac disease and whether there has been a change in the presentation with the time.

Methods

A prospective study was conducted on 134 children diagnosed to be having celiac disease in the Pediatric Gastroenterology, PGIMER, Chandigarh, from July 1st 2006 to December 31st 2007. Their detailed clinical profile was recorded on a pretested proforma and all patients underwent hemogram, liver function tests, IgA anti-tissue transglutaminase (anti tTG), and upper gastro-intestinal endoscopy.

Findings

Major symptoms at presentation were diarrhea (54.5%), failure to thrive (52.2%), abdominal distension (41%), anemia (40%), pain abdomen (19.4%), vomiting (15.7%) and constipation (2.2% of cases). 60.4% of patients had short stature. Anemia was microcytic hypochromic in 79.1% of patients, and dimorphic in 20.9%. Serum transaminases were raised in 38.8% of cases. The mean serum anti tTG level was 164.24U/ml (Range 0-749 U/ml) and levels correlated with the severity of small intestinal damage on biopsy. 15 patients were negative for the serology but 8 out of them had IgA deficiency and all had histopathology suggestive of celiac disease.

Conclusion

Classical presentation of celiac disease is less commonly encountered these days probably related to the more widespread use of serologic testing and early recognition of atypical manifestations of celiac disease.

This communication reviews recent literature and summarizes hepatobiliary abnormalities that may complicate the clinical course of celiac disease. A wide spectrum of hepatobiliary diseases has been described, including asymptomatic elevations of liver enzyme levels, nonspecific hepatitis, nonalcoholic fatty liver disease, and autoimmune and cholestatic liver disease. Moreover, in the majority of patients, liver enzyme levels will normalize on a gluten-free diet. In addition, celiac disease may be associated with rare hepatic complications, such as hepatic T-cell lymphoma. Because many celiac patients do not have overt gastrointestinal symptoms, a high index of suspicion is required. Simple methods of detecting celiac disease such as serum antibody tests help in the early identification of the disease, thus preventing serious complications of the disorder. The IgG DGP antibody test and IgA tTG antibody test used in combination are an excellent screening test for suspected cases of celiac disease.

Background and objective

Portal hypertension may affect intestinal functions, brush border enzymes and absorption parameters. Information about these in extrahepatic portal venous obstruction (EHPVO) in children is limited and poorly reported. We therefore studied the brush border enzymes and absorptive capacity in EHPVO in children.

Methods

The study was conducted on 52 children of EHPVO. The diagnosis of EHPVO was made on the basis of clinical presentation and ultrasound examination. All patients underwent upper gastrointestinal endoscopy. Endoscopic tissue biopsies from duodenum were taken on aluminum foils and kept immediately at −20°C for estimation of brush border enzymes. Tissue biopsies were homogenized in sodium maleate buffer, 0.1 M pH 6.0, by a homogenizer and processed for the enzymes: lactase, maltase, and sucrase. Enzyme levels were compared to normal healthy controls (n = 20). d-Xylose test, stool acid steatocrit for fat excretion in stools and stool alpha-1 antitrypsin were done to know about the absorptive parameters.

Results

Enzyme levels of lactase (6.21 ± 5.67 IU/mg) and sucrase (37.07 ± 21.06 IU/mg) in EHPVO group were significantly lower as compared to lactase (23.32 ± 10.48 IU/mg) and sucrase (95.96 ± 46.55 IU/mg) in normal healthy controls. Maltase levels were lower, but difference was not statistically significant in EHPVO group (56.90 ± 28.65 IU/mg) as compared to normal controls (63.28 ± 22.88 IU/mg). There was no significant difference of urinary d-xylose and stool fat in patients with normal or short stature EHPVO patients.

Conclusion

EHPVO leads to decrease in levels of brush border enzymes in small bowel but their absorption capacity remains normal.

We report a case of embolism of the sclerosant dye with subsequent formation of foreign-body giant cell reaction within the veins of pulmonary and portal circulation in an autopsy case of hepatocellular carcinoma developing over an underlying primary biliary cirrhosis.

Adenosine deaminase activity (ADA) was estimated in cerebrospinal fluid (CSF) of 30 patients of tuberculous meningitis (TBM) and 10 patients each of partially treated pyomeningitis (PTM), aseptic meningitis (AM) and pyogenic meningitis (PM). Mean ADA levels in CSF of TBM patients were higher (18.22 U/L) as compared to 6.28 U/L, 3.43 U/L and 7.98 U/L in PTM, AM and PM respectively. This difference of ADA values in CSF between TBM and other types of meningitis was statistically significant (p<0.01) different. Sensitivity and specificity of ADA levels in CSF of children to diagnose tuberculous meningitis was 66.6% and 90% respectively at 10 U/L cut off of ADA levels in CSF. ADA levels in CSF could also differentiate PTM, AM and PM from TBM with a specificity of 90%, 100% and 80% respectively.

The normal coagulation process is initiated by disruption and exposure of the subendothelial components of blood vessels. Platelets adhere to subendothelium-bound von Willebrand factor via glycoprotein (GP) Ib complex. This initial interaction per se and the release of platelet agonists transduce signals that leads to the rise in intracellular Ca2+ which induces shape change, prostaglandin synthesis, release of granular contents and conformational changes in platelet Gp IIb-IIIa. Gp IIb-IIIa in activated platelets binds fibrinogen and other adhesive proteins and mediates platelet cohesion the primary haemostatic plug. Furthermore, the activated platelets due to aggregation, result in the formation of fibrin (secondary hemostasis). Normally the haemostatic process plays a delicate balance between keeping the blood in the fluid state to maintain flow and rapidly forming an occluding plug following vessel injury. Thrombosis occurs because of alteration in this delicate balance. Arterial thrombosis occurs in the setting of previous vessel wall injury mostly because of atherosclerosis, while venous thrombosis occurs in areas of stasis. The recent advances in understanding of the haemostatic process have led to a better understanding of the mechanism of action of many antithrombotic drugs and identification of new targets for drug development. The molecular target of the ticlopidine has been identified. Large numbers of IIb-IIIa inhibitors have been developed. The mechanism of action of heparin has been defined at the molecular level. As a result, a synthetic pentasaccharide, based on antithrombin-binding domain of heparin, has been developed and tested successfully in clinical trials. New generation direct thrombin inhibitors are being developed. Factor Xa has a critical position at the convergence of intrinsic and extrinsic pathway. The clinical tolerability and the efficacy of low molecular weight heparins has established that inhibition of further thrombin generation, by blocking factor Xa alone can be an effective way of preventing thrombus growth without inactivating thrombin. A large number of specific factor Xa inhibitors are under development. Some of these are in preliminary clinical trials and appear to be promising. Future clinical trials will determine whether these new drugs will provide better risk-benefit ratio in treatment of thrombotic disorders. Similarly role of thrombolytics has been clearly established in many diseases including coronary artery disease.

A case of isolated candidal fungal balls in the common bile duct causing obstructive jaundice and cholangitis is described. There were no predisposing factors. The fungal balls were removed from the common bile duct and a transduodenal sphincteroplasty was performed. Microscopic analysis yielded colonies of candida. Postoperative period was uneventful. At follow-up no evidence of candida infection was evident. He is now 3 years post-surgery and is well.

Nocardia species aromatized 19-hydroxyprogesterone, 3β, 19-dihydroxy-pregn-5-en-20-one 3-acetate and pregn-5-ene-3β, 19, 20β-triol 3-acetate, without cleavage of the side chain, into 3-hydroxy-19-norpregna-1,3,5 (10)-trien-20-one. Septomyxa affinis aromatized the ring A and cleaved the side chain of 19-hydroxyprogesterone to yield estrone. With 19-hydroxypregna-4, 7-diene-3, 20-dione as substrate, the transformation was more complex and many products were formed.

The intense red color produced by a reagent specific for ξ7-estrogens is used to directly detect microorganisms which produce these estrogens from various steroids.

Images

Hydrolysis of phenoxymethyl penicillin into 6-aminopenicillanic acid with spores of fusaria was studied.

Spores of Aspergillus ochraceus and Septomyxa affinis were produced on a large scale by surface sporulation on moist wheat bran and barley. 11α-Hydroxylation of progesterone and Reichstein's compound S by spores of A. ochraceus and 1-dehydrogenation of compound S by spores of S. affinis were carried out in 5-liter fermentors. It was shown that, above a certain minimum, increase in aeration and agitation did not significantly affect steroid conversion. The industrial feasibility of the spore process was further demonstrated by 11α-hydroxylation of 6α-fluoro-16α,17α-dihydroxypregn-4-ene-3,20-dione in a modified 200-gal stainless-steel vessel with spores of A. ochraceus. Strict aseptic conditions are not necessary, either during harvesting of spores or during steroid transformation.

Images

Conidia of Aspergillus ochraceus convert progesterone into 11α-hydroxyprogesterone and 6β, 11α-dihydroxyprogesterone. The conversion ability does not depend on the sporulation medium. Transformation depends on the strain and on the conidia concentration. Adaptation has never been observed. Age and storage of conidia, pH, aeration-agitation, nitrogen source, metal ions, chelating agents, and metabolic activators showed no great influence within wide limits. Mercuric chloride, p-chloromercuribenzoate, NaN3, and KCN inhibit conversion. Glucose is necessary, but can be replaced completely by d(+)-xylose, and partially by some other carbon sources. The ratio mono-/di-hydroxyprogesterone is influenced by progesterone concentration and period of incubation; also, a mutant that accumulates only monohydroxyprogesterone has been produced. Conidia of A. ochraceus also hydroxylate a variety of steroids. Spores of certain streptomycetes, phycomycetes (mucors), ascomycetes, and deuteromycetes are active. Most reactions already observed with vegetative cells have been repeated with spores. In general, spores of a particular organism effect fewer reactions than its mycelium, and fewer products accumulate.