Clinical Phenotype and Disease Course of Inflammatory Bowel Disease in Iran: Results of the Iranian Registry of Crohn's and Colitis (IRCC).

BACKGROUND: Data on the epidemiology of inflammatory bowel disease (IBD) in the Middle East are scarce. We aimed to describe the clinical phenotype, disease course, and medication usage of IBD cases from Iran in the Middle East. METHODS: We conducted a cross-sectional study of registered IBD patients in the Iranian Registry of Crohn's and Colitis (IRCC) from 2017 until 2022. We collected information on demographic characteristics, past medical history, family history, disease extent and location, extra-intestinal manifestations, IBD medications, and activity using the IBD-control-8 questionnaire and the Manitoba IBD index, admissions history, history of colon cancer, and IBD-related surgeries. RESULTS: In total, 9746 patients with ulcerative colitis (UC) (n=7793), and Crohn's disease (CD) (n=1953) were reported. The UC to CD ratio was 3.99. The median age at diagnosis was 29.2 (IQR: 22.6,37.6) and 27.6 (IQR: 20.6,37.6) for patients with UC and CD, respectively. The male-to-female ratio was 1.28 in CD patients. A positive family history was observed in 17.9% of UC patients. The majority of UC patients had pancolitis (47%). Ileocolonic involvement was the most common type of involvement in CD patients (43.7%), and the prevalence of stricturing behavior was 4.6%. A prevalence of 0.3% was observed for colorectal cancer among patients with UC. Moreover,15.2% of UC patients and 38.4% of CD patients had been treated with anti-tumor necrosis factor (anti-TNF). CONCLUSION: In this national registry-based study, there are significant differences in some clinical phenotypes such as the prevalence of extra-intestinal manifestations and treatment strategies such as biological use in different geographical locations.

Effectiveness of sucralfate in preventing esophageal stricture in children after ingestion of caustic agents.

Ingestion of caustic agents by children is a serious health issue that can affect the patient for the rest of his life. The role of sucralfate in preventing stricture caused by caustic agents is controversial, and limited studies have been conducted in this field. We aimed to investigate the effect of sucralfate on preventing esophageal stricture in children. Sixty children with mean age of 36.69 ± 20.50 months and grade II B esophageal burns due to ingestion of caustic agents were enrolled in the study. In the intervention group, in addition to the usual treatment, sucralfate was administered orally at a dose of 80 mg/kg every 2 h for 3 days. For the control group, only the usual treatment was prescribed. Stricture development was compared between groups based on endoscopic and radiologic findings. Of the 60 patients enrolled in the study, 53 were examined. The incidence of esophageal stricture in the intervention group was significantly lower than in the control group (37% versus 67%, P-value = 0.042). In addition, the odds of esophageal stricture after sucralfate intervention was significantly reduced after adjustment for potential confounders (OR = 0.198, P-value = 0.031).  Conclusions: The results of this study showed that sucralfate may reduce the development of esophageal stricture in children when used to manage IIB esophageal burns due to ingestion of caustic agents. What is Known: • Ingestion of caustic agents by children is a serious health issue that can affect the patient for the rest of his life. • The role of sucralfate in preventing stricture caused by caustic agents is controversial and limited studies have been conducted in this field. What is New: • It seems that sucralfate significantly reduces the incidence of esophageal stricture following the ingestion of caustic agents in children compared to the control group. • We believe that the prognosis may be improved and the risk of stricture formation may be reduced with high doses of sucralfate therapy in grade IIB esophageal injury.

Management of Intractable Functional Constipation in Children by Interferential Therapy: Transabdominal or Pelvic Floor.

BACKGROUND: To compare the effectiveness of pelvic floor interferential (IF) therapy with trans-abdominal IF therapy in children with intractable functional constipation. METHODS: Information of 64 children (38 boys, 26 girls; mean age: 6.6 ± 2.2) with functional constipation who had been treated with IF therapy were considered for this retrospective study. Group A (n = 32) underwent pelvic IF electrical stimulation + pelvic floor muscle (PFM) exercises while the group B (n = 32) received transabdominal IF therapy + PFM exercises. A complete bowel habit diary, a constipation score questionnaire and a seeable pain scores had been recorded before, after the ending of treatment courses and six months later for children in both groups. Additionally, children had been assessed with a constipation-related quality of life questionnaire before and six months after treatment. RESULTS: The number of patients who compiled the diagnostic criteria for constipation significantly decreased in both groups after treatment, in which 27/32 (84.3%) of patients in each group had no constipation after the ending of therapy courses (P = 1). Fecal soiling episodes reduced in both groups after treatment, this finding was significantly higher in group A than in group B after the ending of therapy courses (P = 0.05) and six months later (P = 0.01). CONCLUSION: Results of this study showed that using IF therapy both pelvic floor and transabdominal significantly boosts the effects of treatment among patients with functional constipation. Pelvic floor IF therapy is more effective in patients who had concomitant fecal soiling.

Vitamin D therapy in pediatric patients with inflammatory bowel disease: a systematic review and meta-analysis.

BACKGROUND: There is some evidence for the role of vitamin D deficiency in the pathogenesis of inflammatory bowel disease (IBD) in the pediatric population. However, the results are contradictory. Therefore, we have conducted a systematic review and meta-analysis to evaluated the effect of vitamin D on pediatric patients with IBD. METHODS: We carried out a systematic search in databases from inception until 20 January 2022. We included all relevant articles that evaluate the efficacy and safety of vitamin D on disease activity, inflammatory factors, and vitamin D and calcium levels in pediatric patients with IBD. Random effects models were used to combine the data. The main outcomes were then analyzed using weight mean difference (WMD) and respective 95% confidence interval (CI). RESULTS: Fifteen treatment arms met the eligibility criteria and were included. Pooled estimates indicated that intervention with vitamin D has a significantly beneficial effect on 25-hydroxyvitamin D3 [25(OH) D3] (pooled WMD of 17.662 ng/mL; CI 9.77-25.46; P < 0.001), calcium (pooled WMD of 0.17 mg/dL; CI 0.04-0.30; P = 0.009), and inflammatory factors including C-reactive protein (CRP) (pooled WMD of -6.57 mg/L; CI -11.47 to -1.67; P = 0.009) and erythrocyte sedimentation rate (ESR) (pooled WMD of -7.94 mm/h; CI -12.65 to -3.22; P = 0.001) levels. In addition, this effect was greater for vitamin D levels at doses greater than 2000 IU, and when follow-up duration was more than 12 weeks. CONCLUSION: This study showed that vitamin D therapy can have a significant and beneficial effect on 25(OH) D3, calcium, and inflammatory factors in children and adolescents with IBD.

Meta-analysis: efficacy of exclusive enteral nutrition as induction therapy on disease activity index, inflammation and growth factors in paediatric Crohn's disease.

BACKGROUND: There is evidence of inconsistency in sequelae of exclusive enteral nutrition (EEN) as induction therapy in paediatric patients with Crohn's disease (CD). AIM: To investigate the potential effects of EEN on paediatric Crohn's disease activity index (PCDAI), inflammation and biochemical parameters in paediatric patients with CD. METHODS: We performed a comprehensive systematic search of PubMed/MEDLINE, Web of Science, SCOPUS and Embase until 8 January 2022 regardless of the time of publication or language. Random-effects model was applied to combine the datasets. The main outcomes were analysed through mean difference (MD) and its 95% confidence interval (CI). RESULTS: Forty six studies met eligibility criteria and were included in the meta-analysis. Pooled findings indicated that PCDAI score (MD of -27.24; 95% CI -31.84 to -22.64), calprotectin (MD of -842.83 mg/kg; CI -1018.24 to -667.42), CRP (pooled MD of -2.36 mg/dl; CI -2.68 to -2.03), and ESR (MD of -21.09 mm/h; CI -23.79 to -18.38), albumin (MD of 0.65 g/dl; CI 0.58 to 0.72), haemoglobin (MD of 1.12 g/dl; CI 0.87 to 1.37), weight (MD of 4.30 kg; CI 3.39 to 5.22), and height (MD of 0.98 cm; CI 0.35 to 1.62) improved significantly with EEN. CONCLUSIONS: Adherence to EEN can have significant, beneficial effects as induction therapy in paediatric patients with CD.

Relationship of citrulline and tissue transglutaminase antibody with duodenal histopathology among children with celiac disease.

Objectives: Non-invasive biomarkers, for the diagnosis of celiac disease, can reduce the need for biopsy, particularly in pediatric patients. The aim of this study was to investigate the levels tissue transglutaminase antibody (tTG) and plasma citrulline and its correlation with intestinal biopsy. Methods: In this cross-sectional descriptive study, Pediatric patients with celiac disease referred to (XXX)were included. The patients underwent tTG antibody test along with plasma citrulline measurements using HPLC ((high performance liquid chromatography). Biopsy was performed in all the patients and clinical and demographic findings were recorded in a patient form. The data were statistically analyzed using SPSSv22. Results: Of 118 patients with celiac disease, the mean level of citrulline in patients was 17.48 ± 6.92 and the mean tTG titer was 183.17 ± 41.25. The two variables were inversely correlated with each other, p < 0.01. With an increase in Marsh levels, a significant reduction in citrulline levels and an increase in plasma tTG levels were seen, p < 0.01, respectively. The mean citrulline and tTG titer was not associated with gender and the age of the patients. Conclusion: Our findings indicate that citrulline and tTG antibody titer are significant biomarkers for the diagnosis of celiac disease and the severity of intestinal atrophy among pediatric patients.

Co-occurrence of childhood functional constipation and gastroesophageal reflux disease (GERD).

OBJECTIVE: Functional constipation (FC) and Gastroesophageal Reflux Disease (GERD) are common gastrointestinal disorders in children. The aim of this study is to investigate the correlation between functional constipation and GERD in children. METHODS: In this cross-sectional study, a total of 82 children aged <16 years who referred to the pediatric gastroenterology clinic of (XXX) and were diagnosed with functional constipation according to ROME III criteria, and gastroesophageal reflux disease according to clinical history and DeMeester Score. Questionnaire was used to obtain the data regarding age, sex, cause of the visit, presence or absence of any symptom was noted, clinical examinations and difficulty in defecation. RESULTS: Of the 82 children with FC and GERD, 45 were boys and 37 were girls. Among children with FC and GERD, FC was reported in 50 (61%) cases prior to the onset of GERD, whereas 32 (39%) of the children had reflux before FC. The mean age of participants presented with FC was 5.66 ± 3.52 and that of GERD was 5.24 ± 2.83. The difference in mean age was not statistically significant. Of the children with gastroesophageal reflux disease, there were 13 (40.6%) males and 19 females while 32 (64%) males and 18 females had FC and the gender-based difference was statistically significant between the 2 groups. CONCLUSION: The prevalence of functional constipation in children was higher than gastroesophageal reflux disease. Gastroesophageal reflux disease and functional constipation should be simultaneously considered for therapeutic interventions and patients with functional digestive diseases should be monitored and followed-up.

Noninvasive markers for esophageal varices in children with cirrhosis.

BACKGROUND: The diagnosis of esophageal varices (EV) is based on the findings of esophagogastroduodenoscopy (EGD), biopsy, and serum markers. Thus, noninvasive cost-effective tests through which high-risk EV children can be diagnosed are needed. PURPOSE: This cross-sectional study aimed to identify the noninvasive markers for EV in children with liver cirrhosis. METHODS: A total of 98 children with liver cirrhosis were evaluated in this study. The spleen size, platelet count, serum albumin, liver function test results, and risk scores were evaluated prior to endoscopy. The endoscopic investigations aimed to identify the presence of EV and red signs, and determine varices sizes. RESULTS: Endoscopy revealed varices in 43 subjects (43.9%). The spleen size, platelet count, international normalized ratio, aspartate aminotransferase to platelet ratio index (APRI), platelet count to spleen size ratio, and risk score differed significantly between patients with and without EV on univariate analysis; however, the logistic regression analysis showed no differences, indicating that none of these parameters were independently associated with the presence of EV. CONCLUSION: Platelet count, risk score, platelet count to spleen size, and APRI can be useful tools for the identification of highrisk patients with EV and might reduce the need for invasive methods like EGD.

Clinical and Mutation Description of the First Iranian Cohort of Infantile Inflammatory Bowel Disease: The Iranian Primary Immunodeficiency Registry (IPIDR).

We describe a cohort of 25 Iranian patients with infantile inflammatory bowel disease (IBD), 14 (56%) of whom had monogenic defects. After proper screening, patients were referred for whole exome sequencing (WES). Four patients had missense mutations in the IL10 RA, and one had a large deletion in the IL10 RB. Four patients had mutations in genes implicated in host:microbiome homeostasis, including TTC7A deficiency, and two patients with novel mutations in the TTC37 and NOX1. We found a novel homozygous mutation in the SRP54 in a deceased patient and the heterozygous variant in his sibling with a milder phenotype. Three patients had combined immunodeficiency: one with ZAP-70 deficiency (T+B+NK-), and two with atypical SCID due to mutations in RAG1 and LIG4. One patient had a G6PC3 mutation without neutropenia. Eleven of the 14 patients with monogenic defects were results of consanguinity and only 4 of them were alive to this date.

Intrarectal Electromotive Botulinum Toxin Type A Administration in Children With Intractable Constipation: A Randomized Clinical Trial.

INTRODUCTION: Children with refractory constipation might not respond to conventional medical treatments. In this study, we assessed the effectiveness of intrarectal botulinum toxin type A/electromotive drug administration (BoNTA/EMDA) on constipation in these children and compared its efficacy with routine intrasphincteric BoNTA injection. METHODS: From 2017 to 2019, 60 children aged 5-13 years who fulfilled Rome III criteria for intractable constipation were enrolled and randomly assigned into 2 treatment groups. EMDA group (n = 30) underwent BoNTA/EMDA, whereas the control group (n = 30) received injection of BoNTA into the internal anal sphincter. A complete bowel habit diary (with data on the frequency of defecation per week, stool form, and the number of fecal soiling episodes), a constipation score questionnaire, and a visual pain score were recorded before treatment and at 1 month and 6 months after treatment. In addition, children in both groups were assessed with a constipation-related quality of life questionnaire. RESULTS: After 1-month follow-up, treatment reduced the number of patients fulfilling the diagnostic criteria in both EMDA (24/30, 80%) and injection (25/30, 83.3%) groups (P < 0.001). The stool form was normalized in 73.3% (22/30) in EMDA group and 80% (24/30) in injection group after treatment. The median of constipation score and pain score decreased significantly in both groups after treatment (P < 0.001 and P < 0.001, respectively). DISCUSSION: BoNTA/EMDA seems to be as effective as intrasphincteric BoNTA injection for treatment of intractable constipation. In addition, this technique is associated with less comorbidity, is less costly, and can be performed in an office-based setting without general anesthesia.

Effectiveness of a self-care education program on hypertension management in older adults discharged from cardiac-internal wards.

BACKGROUND: The aim of the present study was to determine the effectiveness of a self-care education (SCE) discharge program with telephone follow-ups in managing hypertension (HTN) in older patients. METHODS: The study was conducted on 56 older patients with HTN who had recently been discharged from the cardiac wards of hospitals in Isfahan, Iran, in 2017. Participants were randomly allocated to the intervention and control groups. The intervention was a 60-minute SCE discharge program with 4 re-educative telephone follow-ups every 2 weeks based on 4 chapters of the designed SCE program and booklet. After coding the data and entering them into SPSS software, data were analyzed for the comparison of mean systolic blood pressure (SBP) and diastolic blood pressure (DBP) as well as frequency of managed HTN in the intervention and control groups at baseline (before discharge), and 2 and 3 months after discharge. RESULTS: Statistical tests showed no significant difference in any of the demographic and confounding variables as well as baseline BPs (P > 0.050), but at post-intervention follow-ups, after Mauchly's sphericity test, repeated measurements ANOVA showed that the effect of time (P < 0.001) and group (P = 0.043) on SBP was significant. The effect of time (P = 0.036) and group (P = 0.047) on DBP was also significant. McNemar's test showed that the frequency of managed HTN (normal BP), 3 months after discharge, was significantly higher in the intervention group compared to the control group [87.5% (n = 21) vs. 23.1% (n = 6), respectively] (P < 0.001). CONCLUSION: SCE discharge program with telephone re-educative follow-ups was effective in reducing mean BP. The use of this program as a discharged plan for older adults with HTN and comparison of readmission rates for a longer period are recommended.

Association of IL-10 and TGF-beta cytokine gene polymorphisms with autoimmune hepatitis.

BACKGROUND AND AIMS: Autoimmune hepatitis is a chronic immune-mediated liver injury caused by dysregulated immune response to liver antigens. Genetic susceptibility is affected by multiple single nucleotide polymorphisms in immune-related genes. There are few reports on the association of TGF-ß and IL-10 genetic variants with autoimmune hepatitis. METHODS: Allele frequency and genotype status of IL-10 -1082, -819, -592 and TGF-ß +869 and +915 polymorphisms were investigated in 57 unrelated patients with autoimmune hepatitis and 140 healthy controls by polymerase chain reaction with sequence-specific primers. RESULTS: IL-10 -592 and -819 allele frequencies and genotypes were not associated with autoimmune hepatitis in our population, while IL-10 -1082 genotypes were. IL-10 -1082/-819/-592 "high-producing" haplotype GCC was significantly less frequent in patients. TGF-ß +869 "high-producing" allele C and genotype CC were significantly more in autoimmune hepatitis, compared to controls; whereas, TGF-ß +915 "low-producing" allele C and genotype CC were significantly more in autoimmune hepatitis compared to control. TGF-ß +869/+915 haplotype TG was significantly less frequent in patients while CC haplotype was significantly more frequently observed in patients. CONCLUSION: We identified a significant association between IL-10 -1082/-819 and TGF-ß +869/+915 genotypes and haplotypes with autoimmune hepatitis in Iranians.

IgG4 subclass and gamma-glutamyl transferase in children with ulcerative colitis with primary sclerosing cholangitis and without sclerosing cholangitis.

AIM OF THE STUDY: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease which could be associated with inflammatory bowel disease (IBD), particularly ulcerative colitis (UC). The aim of this study was to compare GGT and IgG4 levels among children with UC with PSC and without PSC. MATERIAL AND METHODS: In this cross sectional study children with UC with PSC and UC without PSC were included. Serum immunoglobulin G4 (IgG4) and gamma-glutamyl transpeptidase (GGT) levels of the 90 UC patients with and without concomitant PSC were measured. Children with serum IgG4 concentration > 175 mg/dl were considered to have elevated IgG4. RESULTS: Elevated serum IgG4 was found in 8 of 30 (26.6%) patients with PSC vs. 3 of 60 (5.0%) patients without PSC. Compared with the group without symptoms of PSC, the group with PSC showed significantly higher levels of aspartate aminotransferases (AST; 22.5 U/l vs. 70.0 U/l, p < 0.001), alkaline phosphatase (ALP; 359.0 U/l vs. 602.0 U/l, p < 0.001), and IgG4 (56.0 vs. 73.0, p = 0.02). The odd ratio of the elevated IgG4 and GGT in predicting PSC was 6.9 (95% CI: 1.6-28.4) and 18 (95% CI: 5.7-55.9), respectively. CONCLUSIONS: AST, alanine aminotransferase (ALT), GGT, ALP, and serum IgG4 were significantly higher in UC patients with sclerosing cholangitis (SC) compared to UC patients without SC. GGT and IgG-4 measurements are recommended for evaluation of UC.

Autoimmune hepatitis association with single nucleotide polymorphism of interleukin-2, but not interferon-gamma.

BACKGROUND: Autoimmune hepatitis (AIH) is a chronic inflammation in hepatocellular tissues associated with circulating autoantibodies. Imbalance in T-cells population and dysregulation in several cytokine profiles has been implicated in pathogenesis of AIH. This study was performed to assess potential association of AIH with interleukin-2 (IL-2) and interferon-gamma (IFN-γ) genes single nucleotide polymorphisms (SNPs). METHODS: Fifty-six patients with AIH and 139 healthy individuals were enrolled in this study. IL-2 and IFN-γ typing was performed, using polymerase chain reaction with sequence-specific primers (PCR-SSP) assay. The frequencies of alleles, genotypes and haplotypes in AIH patients were compared to healthy controls. RESULTS: IL-2 T allele at position +166 (rs2069763) showed significant higher frequency in AIH group (36%), compared to the controls (21%) (OR=2.06; 95% CI, 1.24-3.43, P-value<0.01). The frequency of IL-2 TT genotype at +166 position was also associated with AIH (OR=18.68, 95% CI 3.74-126.04, P-value<0.01). G/T alleles of IL-2 at -330 (rs2069762) and A/T alleles on UTR +5644 position at IFN-γ and their subsequent haplotypes, did not show significant association with AIH. CONCLUSIONS: This study identified IL-2T allele at +166 position and TT genotype as susceptibility gene in AIH which would provide better understandings into the mechanisms of AIH and potential immune modulation therapies.

Antibiotic susceptibility of Helicobacter pylori strains isolated from Iranian children: High frequency of A2143G point mutation associated with clarithromycin resistance.

OBJECTIVE: Increasing antibiotic resistance in Helicobacter pylori is a global concern and is associated with treatment failure. The aim of this study was to evaluate antibiotic resistance of H. pylori to different antibiotics including amoxicillin, erythromycin, ciprofloxacin, furazolidone, tetracycline, metronidazole and clarithromycin. In addition, ​detection of A2143G mutation in clarithromycin resistant isolates was performed using real-time PCR technique. METHODS: Ninety patients with upper gastrointestinal symptoms were enrolled in this study. H. pylori were isolated from 32 specimens and the resistance rate of these strains to amoxicillin, erythromycin, ciprofloxacin, furazolidone, and tetracycline was tested by disc agar diffusion method. The resistance level to metronidazole and clarithromycin was determined by agar dilution method. The presence of A2143G point mutation in clarithromycin resistant isolates was determined using real-time PCR technique. RESULTS: The resistance rates to amoxicillin, erythromycin, ciprofloxacin, furazolidone, tetracycline, metronidazole and clarithromycin were 53%, 50%, 37.5%, 62.5%, 25%, 62.5% and 22%, respectively. The A2143G point mutation was detected in 71% of clarithromycin resistant strains (5 out of 7). CONCLUSIONS: The prevalence of H. pylori resistance to metronidazole, ciprofloxacin, erythromycin, amoxicillin and furazolidone in Iran is high. Determination of antibiotic susceptibility plays an important role in selecting of the appropriate anti H. pylori regimen.

Association of PTPN22 Single Nucleotide Polymorphisms with Celiac Disease.

OBJECTIVES: Celiac disease is a chronic autoimmune disease in which gene-environment interactions cause the immune system to unfavorably react to naturally gluten-containing foods. PTPN22 plays a crucial role in regulating the function of various cells of the immune system, particularly T cells. Polymorphisms of the PTPN22 gene have been associated with many autoimmune diseases. The present genetic association study was conducted to investigate the possible associations between PTPNTT single nucleotide polymorphisms (SNPs) and celiac disease in an Iranian population. MATERIALS AND METHODS: The study population consisted of 45 patients with celiac disease and 93 healthy controls. The study genotyped five SNPs of the PTPN22 gene: rs12760457, rs1310182, rs1217414, rs33996649, and rs2476601. RESULTS AND CONCLUSIONS: Control and patient groups did not differ on the genotype distribution of four of five investigated SNPs in the PTPN22 gene, for example, rs12760457, rs2476601, rs1217414, and rs33996649. The only investigated PTPN22 variant, which could be associated with CD, was rs1310182. A significant increase in the carriage of the T allele of rs1310182 in CD patients was observed (OR (95% CI) = 11.42 (5.41, 24.1), p value < 0.0001). The TT genotype of this SNP was significantly associated with celiac disease. Our study suggests that the rs1310182 SNP of PTPN22 gene may be a predisposing factor of celiac disease in the Iranian population. Further studies are required to investigate the issue in other racial and ethnic subgroups.

Hyperammonemic Induced Coma by Bacterial Overgrowth in a Child With Hirschsprung's Disease.

Cases with Hirschsprung's disease show the functional intestinal obstruction. Obstruction in these patients may lead to bacterial overgrowth with stasis and inflammation of the colon. Bacterial overgrowth can cause hyperammonemia that makes lethargy and loss of conscious and finally admitting in ICU. The purpose of this case report is to present a case that had Hirschsprung's disease and referred to Children's Medical Center with serum hyper-ammonium caused by bacterial overgrowth that induced coma and altered level of consciousness then made her to admit to PICU. A 15-year-old female referred to Children's Medical Center with lethargy and low grade diarrhea. She had hypocalcemia and hypoalbuminemia with high PT and INR. Because of loss of conscious, she admitted at PICU. Laboratory findings showed hyperammonemia in this case, but other criteria were normal. Administration of antibiotic and lactulose therapy was started that lead to a reduction in serum ammonium level and discharging of the case. Thirteen days later she referred again with mentioned symptoms, and clinical evaluations showed high serum ammonium level. This time because of loss of conscious she had to admit at PICU and used NG tube. Administration of lactulose syrup and sodium benzoate make her in a better condition. Narrowing rectum toward the sigmoid and highly enlarged intestinal lopes was on behalf of Hirschsprung's disease. Finally, the patient with the acceptable situation and oral periodic metronidazole discharged. It is essential to check serum ammonium level in the cases with loss of conscious. The choice for controlling hyperammonemia is lactulose therapy.

Evaluation of Serum Adenosine Deaminase in Cystic Fibrosis Patients in an Iranian Referral Hospital.

BACKGROUND: Adenosine, a signaling nucleoside, is controlled in part by the enzyme adenosine deaminase (ADA). There are rare reports on the role of adenosine levels and ADA in cystic fibrosis (CF) patients. OBJECTIVES: The aim of this study was to assess serum ADA in CF patients in order to find whether the severity of lung disease in CF is related to significant changes of ADA or not. PATIENTS AND METHODS: Venous blood serum ADA was measured in CF patients (3-15 years) and 49 healthy children (3-15 years) referred to Children's Medical Center. Classification of respiratory and gastrointestinal disease severity in CF patients as well as Body Mass Index (BMI) was performed. The results were compared with values obtained from healthy children matched for age and gender. RESULTS: This study included 49 children of both genders (20 females and 29 males) with CF (mean age: 6.36 ± 2.22 years). Mean serum ADA in CF patients group and control group was 9.38 ± 2.72 and 16.04 ± 1.27, respectively (P value = 0.001). Mean serum ADA in CF patients with normal BMI was higher than in patients with low BMI (P value = 0.002). CONCLUSIONS: In this study the lower serum level of ADA was seen in CF patients compared to control group. The clinical symptoms, especially respiratory symptoms, in CF patients might be associated with reduction of serum ADA and rising serum adenosine; therefore, further studies on the use of ADA enzyme therapy in CF patients are highly recommended.

Comparison of Ciprofloxacin-Based Triple Therapy with Conventional Triple Regimen for Helicobacter pylori Eradication in Children.

Helicobacter pylori infection is a prevalent disease among Iranian children. The purpose of this study was to compare the effect of ciprofloxacin and furazolidone on eradicating helicobacter pylori in Iranian children in combination with amoxicillin and omeprazole. In this cohort study, helicobacter pylori infection was confirmed by gastroscopy, rapid urease test or pathologic assessments. A total of 66 children were randomly enrolled; based on the random number table, and were divided into two groups; first, a combination regimen consisting of ciprofloxacin, amoxicillin, and omeprazole; second, a three-medication regimen consisting of amoxicillin, furazolidone, and omeprazole. The effect of both medical regimens on the successful eradication of helicobacter pylori infection was assessed and compared. Chi-square test was used for evaluating the association between quantitative variables. All comparisons were made at the significance of P<0.05. Endoscopic tests prior to initiating treatments showed that 66.7% of the patients had a degree of nodularity while peptic ulcer was only observed in one patient. One month after the end of the treatments, eradication of the helicobacter pylori infection was reported 87.9% (29/33) in the first group (CAO) and 60.6% (20.33) in the second group (FAO) (P=0.011). It appears that a major advantage of our proposed regimen over others is a lack of wide use of fluoroquinolones for treating children's diseases. Given FDA's recommendation about the possibility of prescribing ciprofloxacin for infected patients with multidrug resistance, we can use the regimen proposed in this study in patients with resistance to standard treatments.