GOVARESH JOURNAL
Year:2006 | Volume:11 | Issue:2|Papers Count:10

Background: We aimed to determine the frequency of liver copper storage diseases in Iranian children with cryptogenic chronic liver disease (CLD).Materials and Methods: One-hundred and twenty children (52 girls, 70 boys; age range: 3 months-14 years) with CLD who attended Children's Medical Center in Tehran, Iran during the years 1997 through 1999, were enrolled. All viral, metabolic and autoimmune causes of CLD were excluded in them, all underwent slit lamp examination for KF ring by an experienced ophthalmologist, and liver biopsy done on all of them. In addition to the routine histopathologic examination, the liver biopsy specimen was checked for its copper content with proton-induced X-ray method (PIX-E).Results: Eight (6.6%, 4 girls, 4 boys, age: 6 to 14 years) of the 122 children with CLD had no identifiable cause despite extensive evaluation.All 8 had cirrhosis on liver biopsy and elevated hepatic copper content (mean 31 times normal). None had KF rings and all had normal serum Ceruloplasmin levels. NICC was diagnosed for all by the absence of KF ring, normal serum ceruloplasmin levels and pathognomic histopathologic findings. Mean hepatic Cu level showed a 7-fold increase in children with CLD other than NICC. Mean serum ceruloplasmin level was 45 mg/dl in NICC and 31 mg/dl in CLD patients. Mean 24-hour urinary Cu excretion was 225 mg and 76 mg in NICC and CLD patients, respectively. All patients with NICC (n=8) had the history of using cooking utensils made of copper. Four of these were from one family.Conclusion: According to our data 6.6% of Iranian children with CLD who diagnosed as cryptogenic cirrhosis suffer from NICC, Therefore early diagnosis and appropriate treatment with D-Penicillamin are important for survival in patients with NICC. Hence it is suggested that all children with cryptogenic cirrhosis be assessed for NICC.

Background: Acute upper GI bleeding (UGIB) is a common medical emergency situation. In the past years, important changes have taken place that might have influenced the incidence, etiology, and outcome for patients with acute UGIB. The aim of this study was to determine current time trends in the incidence, management, and prognosis of patients with UGIB in 14 years period in a region in center of Iran. Materials and Methods: All of the patients with UGIB who hospitalized in all hospitals from 1991 to 2004 in Semnan city were evaluated in a retrospective study. The patients with permanent residency in this area and age more than 15 years enrolled in the study. The patients were divided in two subgroups based on endoscpic diagnosis; peptic ulcer (PU) and non-peptic ulcer (Non-PU). Again, analysis was performed by dividing the patients to acid-pepsin disorders (APDs) and non-acid pepsin disorders (Non-APDs).Results: 873 patients [617 male (70.7%), Mean ± SD (46.9±21.2)] enrolled in the study. The incidence rate of UGIB has decreased from 98.4 to 40.1 in 100.000 person in year in this period (p<0.001). The incidence rates of bleeding due to PU and Non-PU have decreased. However, in the second analysis the rate of APDs has decreased, but the rate of non-APDs has remained unchanged, during the study period. In regard to prognosis, the rates of endoscopic therapy, surgery and mortality were not changed significantly in this period.Conclusion: The overall incidence of UGIB, and bleeding due to APDs was declined in recent years that may be due to better approach to peptic ulcers and eradication of helicobacter pylori and wide spread use of PPIs and H2 blockers in APDs.

Background: An increase in the number of intraepithelial lymphocytes (IEL) in the duodenal mucosa is an important criterion for the histological diagnosis of celiac disease (CD). We determined the normal range for intraepithelial lymphocytes (IEL) in the second part of duodenum in healthy Iranian population.Materials and Methods: Four biopsy samples of the endoscopically normal appearing mucosa at the second part of duodenum were obtained from 50 individuals referred to Shariati hospitals (48 for epigastric pain, 2 for esophageal varices). They had no sign, symptoms and evidence for malabsorption or small intestinal disorders in history, physical examination, Laboratory tests and IgA anti tissue transglutaminase (t.T.G). Four-micrometer thick sections were stained with Hematoxillineosine (H&E) and immunohistochemistry (IHC) for leukocyte common antigen (LCA). At least 500-600 cells from the tip and body of villi were counted separately and the number of IEL was given as mean/ 100 epithelial cells.Results: The mean for IEL count in total villi in IHC method was 21/100 (23/100 in tip, 21/100 in body, p = 0.058) and 19/100 in H&E method (19/100 in tip, 18/100 in body, p =0.035) (p = 0.006). Considering total villi, the normal upper limit (Mean+2SD) was calculated to be 35/100 in IHC and 34/100 in H&E stained sections and normal upper limit of confidence interval (the 95 percentile) was 39/100 in IHC and 37/100 in H&E stained sections. The villus height to crypt depth ratio was 3/94 in average.Conclusion: Respectively in IHC and H&E methods, the amounts equal or less than 35/100 and 34/100 are considered as normal., values between 35-39/100 and 34-37/100 as border line and counts more than 39/100 and 37/100 represent a pathology. Although the difference between two staining methods was statistically significant, considering cost effectiveness, we recommend H&E staining for routine clinical practice.

Background: minimal bright red bleeding per rectum (MBRBPR) is a common complaint in adult patients. Most studies have not been performed in patients with strictly minimal BRBPR, and there are no evidence-based recommendations for this group of patients. Materials and Methods: patients with complaints as small amounts of red blood after wiping or a few drops of blood in the toilet bowl after defecation not intermixed with stool were consecutively enrolled. Those with a history of inflammatory bowel disease, colorectal cancer, adenomatous polyps, significant weight loss, anemia, strong family history of colorectal cancer and not consenting for colonoscopy were excluded. Patients underwent total colonoscopy or rectosigmoidoscopy plus double contrast barium enema (age>40) or a minimum of rectosigmoidoscopy (to the splenic flexure in patients aged < 40). Results: two-hundred twenty patients were eligible in the study period. Normal findings were found in 23 (10.5%). There was at least one distal lesion in the remaining patients. From 11 patients with a proximal lesion, 10 had the same lesion in distal colon and there was only one patient with an adenomatous polyp near the hepatic flexure and hemorrhoids. In patients with anorectal pathologies (n=189), there was a concomitant colonic lesion in 25 (13.2%). All patients with clinically significant lesions (IBD, polyp or cancer) located over 30 cm from anus were aged more than 40 years.Conclusion: the causative lesions for MBRBPR are located in the distal colon. Rectoscopy (40 years or less) or flexible rectosigmoidoscopy (over 40 years) seem to be the appropriate evaluation in these patients.

Background: At present, surgery is the best method for chronic anal fissure, but by acquiring more knowledge about the pathogenesis and function of internal anal sphincter, non surgical treatment such as using of Glycerin trinitrate, Isosorbide, Bethancol, Diltiazem and Butilinum toxin has been suggested. There is controversy about using of L-Arginine. The aim of the present study was to compare the effect of L-Arginine (chemical Sphincterotomy) with lateral internal Sphincterotomy (surgical Sphincterotomy) in treatment of chronic anal fissure.Materials and Methods: This clinical trial study was performed on 60patients referring to our hospital complaining for chronic anal fissure. They were randomly divided in tow groups of equal number: patients undergoing internal Sphinctrotomy (control), and using of topical L-Arginine gel (case). All of the patients under study where followed for three months, data about pain, bleeding, and wound healing were recorded, and analyzed by SPSS 11 software, chi- square and t-test. Results: The results observed in this study showed that in case group, rectal bleeding discontinued in 70% and pain controlled in 70%, while wound healing occurred in only 26.7%. in control group, 100% had no pain and no bleeding after treatment and wound healing occurred in 56.7% . Conclusion: Local application of L-Arginine can not replace internal sphinctrotomy, but can be used when patient is unwilling for surgery and  in cases with underlying disease.

Ampullary tumor is a rare cancer. These patients most commonly present with obstructive jaundice but sometimes disease appear with uncommon signs. This report is an example of such cases.Our case is a 53 year old man who presented with recurrent fever and chills and iron deficiency anemia and has been misdiagnosed for two years. The proper diagnosis occurred when the patient showed abnormal liver function tests and more evaluation with endoscopic ultrasonography detected a mass in distal part of common bile duct then pathology confirmed our diagnosis.Despites that jaundice is most prevalent symptom in patients with an ampullary tumor but sometimes disease appear with rare signs such as iron deficiency anemia.

Endocrine tumors of the pancreas (Islet-cell tumors) are rare with neuroendocrine origin. The patient was a 41- year- old man, admitted with chief complains of epigastric mass feeling, dull abdominal pain, lack of appetite and weight loss up to 2-3 kg since 6 months ago. In physical examination, a mass was detected in epigaster and left upper quadrant (LUQ). In abdominal x-ray, a calcified mass was seen in LUQ. For better evaluation, abdominal CT was performed in which, a large calcified mass in pancreas tail was reported. Finally, by means of immunohistochemical markers, the neuroendocrine carcinoma of the pancreas was confirmed. In spite of the rareness of calcification in these tumors, it is also necessary to consider malignancies when encountering a large calcified mass in pancreas.

Background: Helicobacter pylori (Hp) is a gram negative, spiral shaped bacterium which colonizes the gastric mucosa and induces gastroduodenal complications varying from mild gastritis with no clinical complications to peptic ulcer diseases and even gastric malignancies. The rate of Hp infection is 30-50% in developed countries whereas it has been rated up to 80% of the adult population in developing countries like Iran.Hp infection can be detected by various diagnostic methods. Culturing biopsy specimens and Rapid Urease Test (RUT) are the most common and reliable tests which can manifest Hp infection through proper sampling but these methods are invasive ones due to the need for endoscopy procedure in isolation of biopsy specimen. Application of serological assays are being increasingly used for epidemiological studies and detecting systemic immune responses toward past Hp infection. ELISA assays are the most popular techniques particularly in cases with no previous treatment.Materials and Methods: In this study we tested three imported IgG ELISA kits which are available for clinical diagnostics in detecting host sero-reactivity to Hp infection and compared them with a home made IgG ELISA kit. Histology and RUT were used as the gold standard tests for determination of Hp positive vs. Hp negative subjects using biopsy specimens from antrum. Sensitivity, specificity, accuracy and other required criteria were evaluated for each ELISA kit.Results: According to the results the original criteria (Sensitivity and specificity) for each kit were as follows: BIOHIT (41.6%, 100%), Trinity (100%, 86.6%), Pishtaz (100%, 86.6%), Home made (100%, 92.6%).Conclusions Evaluation of these different IgG ELISA kits originating from different parts of the world and cross comparison of the results indicated that the cut off values should be refined for user country in order to obtain the highest sensitivity and specificity. These differences can be due to the vast geographic heterogeneity among Hp antigens. Furthermore, this study showed that home made ELISA kit can be substituted for imported ELISA kits due to its valid serological criteria.