Determining the frequency of Helicobacter pylori in children with chronic kidney failure and its relationship to gastrointestinal symptoms.

Key Clinical Message: As there is no significant mutual relationship between Helicobacter pylori infection and chronic kidney disease in children, its routine study is not justified and is recommended only in symptomatic cases. Abstract: Children suffering from chronic kidney disease (CKD) often complain of indigestion but, if it is accompanied by abdominal pain, it is necessary to investigate and rule out Helicobacter pylori infection to confirm functional dyspepsia. Epidemiological studies in adults have conflicting results regarding the association between Helicobacter pylori infection and CKD. In this study, we determined the prevalence of H. pylori in children with kidney failure and its relationship to their gastrointestinal symptoms. In this retrospective study, 54 children with chronic kidney failure admitted to the hemodialysis ward of the Children's Medical Center, Tehran, Iran between 2012 and 2020 were studied. The mean age of our patients was 11.89 ± 3.99 years and their sex distribution was equal. H. pylori infection was reported in only three patients with 5.6%. Based on our findings, epigastric pain in children was the most common gastrointestinal symptom (70.4%). Among all patients, three patients (5.6%) died, all of them were male (P = 0.075). The most prevalent underlying cause of kidney failure in our patients was neurogenic bladder. We did not find any significant relationship between the increased risk of chronic kidney failure and co-infection with H. pylori. Investigating the cause of epigastric pain and looking for H. pylori is very important in CKD children under hemodialysis because if they receive a transplant the possibility of gastrointestinal complications will be increased with the use of steroid and immunosuppressive drugs.

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.

Extraintestinal manifestation of inflammatory bowel disease and associated factors in pediatric patients.

OBJECTIVE: Extraintestinal manifestations in irritable bowel disease (IBD), Crohn's disease and ulcerative colitis are reported in different system of the body. We aimed to investigate the factors determining disease severity and evaluate extraintestinal manifestations in patients with irritable bowel disease based on disease activity scoring system. METHODS: This cross-sectional study was conducted on all patients with irritable bowel disease referred to clinic and gastroenterology ward of (XXX). Medical history, gastrointestinal complications and incidence of extraintestinal manifestations were recorded. Disease severity in ulcerative colitis was rated based on PUCAI (Pediatric Ulcerative Colitis Activity Index) score and in Crohn's disease was calculated based on PCDAI (Pediatric Crohn's Disease Activity Index) score. RESULTS: Of 73 children included in our study, 54.80% were diagnosed with ulcerative colitis and 45.20% were diagnosed with Crohn's disease. The most prevalent symptom was weight loss (81.81%), diarrhea (72.72%) and abdominal pain (69.69%) in Crohn's diseases and bloody diarrhea (80%), abdominal pain (77.5%) and weight loss (57.5%) in ulcerative colitis. The average of PCDAI score was 43.44 and PUCAI score was 35.62. The most prevalent extraintestinal manifestation of the disease was arthritis in 7 Crohn's disease patients (21.21%) and 5 patients (12.5%) with ulcerative colitis, oral plague in 5 patients (15.15%) with Crohn's disease and erythema nodosum in 2 patients (6.06%) with Crohn's disease. CONCLUSION: The severity of oral plaque was positively associated with the severity of the disease. There was no significant association among other parameters. Scoring systems, to determine the extraintestinal manifestation, should be standardized based on the scoring outcomes.

Jordans' Anomaly as a Red Flag for Neutral Lipid Storage Diseases.

Background: Jordans' anomaly is characterized by lipid vacuoles in granulocytes which are observed in neutral lipid storage diseases like Chanarin-Dorfman syndrome. Case report: This six-year-old boy had skin ichthyosis, elevated liver enzymes, and prominent vacuoles in neutrophils, eosinophils, monocytes, and basophils (Jordans' anomaly), leading to the diagnosis of Chanarin-Dorfman Syndrome, which was successfully treated with medium-chain triglyceride oil. Conclusion: Jordans' anomaly is a red flag for the diagnosis of neutral lipid storage diseases especially in patients with ichthyosis and elevated liver enzymes.

Comparative Efficacy of Transcutaneous Functional Electrical Stimulation With or Without Biofeedback Therapy on Functional Non-retentive Fecal Incontinence in Children: A Randomized Clinical Trial.

BACKGROUND: We compared the effects of transcutaneous functional electrical stimulation (TFES) and biofeedback therapy with TFES alone in a cohort of children with functional non-retentive fecal incontinence (FNRFI). METHODS: This prospective, single-center randomized clinical trial was performed on 40 children with FNRFI. Patients were randomly allocated into two equal treatment groups. Group A (n = 20) underwent TFES + biofeedback therapy, and group B (n = 20) received TFES alone. All patients were assessed with a pediatric fecal incontinence (FI) score questionnaire, a visual pain score, and a bowel habit diary both before and at the end of treatment sessions and also at 6 months of follow-up. In addition, a FI quality-of-life (QoL) questionnaire was recorded for all patients before and 6 months after the treatment. RESULTS: FI significantly improved in 13/20 (65%) patients in group A and 11/20 (55%) patients in group B (P < 0.05). A significant reduction in FI score was seen in each group at the end of treatment sessions and maintained at 6 months of follow-up (P < 0.05). A significant improvement in FI-QoL scores was seen in both groups at 6 months of follow-up in which there was no significant difference in terms of FI-QoL scores improvement between both groups after treatment. CONCLUSIONS: The use of electrical stimulation in combination with other treatment methods improves symptoms in patients with FNRFI who are refractory to conventional treatment.

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.

Feeding-based treatment of allergic proctocolitis and associated clinical outcomes.

BACKGROUND: Allergic colitis (AC) is one of the most common etiologies of rectal bleeding in infants aged one to six months. AIM: The aim of this study is to apply step-by-step dietary restrictions in the mother's diet or change of infant formula fed thereby, to evaluate the subsequent clinical response. METHODS: Sixty healthy infants whose clinical and evaluation results indicated proctocolitis in our outpatient gastroenterology clinic were included in this. They were divided into three groups according to the type of feeding; group 1 were exclusively breast fed, group 2 were exclusively formula fed and group 3 were fed with combination of both. In breast feeding women, discontinuation was allergenic food was studied in four stages; cow-related dairy products, soy, sesame and fast food (stage A), egg (stage B), corn, nuts and fish (stage C) and wheat (stage D). RESULTS: Sixty newborns with age at symptom onset 3 days-20 days participated in the study. Up to the time of our initial evaluation, the mean age and weight of infants was 73.34 ± 1.00 day and 3292.71 ± 367.93 g, respectively. There was no significant difference in sex and the type of labor between the groups. Thirty-three infants had a history of eczema and the parents of 47 infants had a history of allergy, with the greatest prevalence in group one. Rectal bleeding in 50% of infants was halted after the elimination of allergenic feed in mother (15 in stage A, 8 in stage B and 7 in stage C). Ten infants needed extensive hydrolyzed formula and 20 needed amino acid-based formulas. CONCLUSIONS: There is no need for immediate use of amino acid or extensive hydrolyzed formulas in the first stage of blood in stool, perhaps discontinuing allergenic food in mothers could be the primary measure.

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.

Evaluating the effects of probiotics in pediatrics with recurrent abdominal pain.

BACKGROUND: Recurrent abdominal pain (RAP) is one of the frequent complaints in general practice, particularly in pediatrics and is among the common cause of referral to gastroenterology clinics. PURPOSE: This study is designed to investigate the effects of probiotics for the treatment of RAP and desired therapeutic outcomes. METHODS: One hundred twenty-five children with the diagnosis of RAP according to Rome III criteria for irritable bowel syndrome (IBS), functional abdominal pain (FAP), functional dyspepsia (FD), and abdominal migraine (AM), were enrolled in this double-blind randomized controlled trial. RESULTS: Sixty-five subjects received probiotics, and others received placebo treatment for 4 weeks. Lactobacillus reuteri was therapeutically effective in 32 patients compared to 8 patients, responding to the placebo treatment. Compared to baseline, all pain-related variables showed a significant reduction for the IBS and FD at the end of the 4th week. However, it did not respond well in FAP and AM groups. Pain-related outcomes such as, frequency of the pain, severity, and duration of the pain were decreased following the probiotic treatment. No therapeutic response was seen in AM group after the administration of probiotics. L. reuteri significantly led to pain relief in the overall population, and also in FAP, FD, and IBS subgroups. CONCLUSION: L. reuteri probiotics are likely to lead to RAP relief and can be recommended for the treatment of functional gastrointestinal disorders.

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.

Unusual presentation of Crohn's disease in a boy with penile swelling.

Crohn's disease is one of the three subtypes of inflammatory bowel disease, with regional, transmural, and granulomatous inflammation. The present study is a case report on an 11-year-old boy who was referred to the hospital due to penis swelling and pain since a year ago, the patient sometimes complained of pain when urinating, he was having a severe painful swelling in the penis shaft; however, his scrotum was completely normal. He was diagnosed with crohn's disease, thus was placed on azathioprine therapy which eradicated his pain after 3 months, and thus, his skin lesion became much smaller. Crohn's disease, especially in children, should be considered in patient with unknown skin complications, and, if clinically suspected, biopsy examination should be performed. The diagnosis of crohn's disease may be delayed in extradigestive cases. This disease occurs when associated with skin manifestations, which is uncommon. We reported a case with penile crohn's disease, of which few cases have been reported in children to date.

Association of Interleukin-6 and Interleukin-1 Family Gene Polymorphisms in Autoimmune Hepatitis.

INTRODUCTION AND AIM: Autoimmune hepatitis (AIH) is an immune-mediated destruction of liver cells, in recognition of interface hepatitis, seropositivity for autoantibodies, and interface hepatitis in histology sections. Hepatocyte destruction in AIH is the direct result of CD4+ T-cell destruction. Yet, Th17 mediated immune attach and a diversity of cytokine networks, including pro-inflammatory cytokines such as Interleukin 1 (IL-1) and Interleukin 6 (IL-6), set the stage for the destructive liver damage. MATERIAL AND METHOD: Peripheral blood samples from 57 patients, with AIH, recruited from referrals to the main pediatric hospital in Tehran. Single nucleotide polymorphisms for the following cytokines genes, were evaluated through, polymerase chain reaction with sequencespecific primers (PCR-SSP) assay: IL-1a (C/T -889), IL-1α (C/T -511), IL-1ß (C/T +3962), IL-1 receptor (IL-1R; C/T Pst-I 1970), IL-1RA (C/T Mspa-I 11100), and IL-6 (C/G -174 and A/G nt565). RESULTS: Significant higher frequency of genotype AA was detected in patients in IL-6 at position nt565 (15.8% in AIH patients vs. 2.9% in controls, p = 0.003). The haplotype GA of IL-6 at -174 and nt565, was significantly overrepresented in the AIH group, compared to (20.9% of AIH vs. 1.4% in controls p < 0.0001). CONCLUSION: Results of our study, indicate significant deviation toward high yield IL-6 polymorphisms, in AIH patients. These data could bring new insights in pathophysiology of disease, which could contribute to developing novel treatments for AIH.

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.

Effects of Interferential Electrical Stimulation Plus Pelvic Floor Muscles Exercises on Functional Constipation in Children: A Randomized Clinical Trial.

OBJECTIVES: Functional constipation is a common condition in children. We assessed the effectiveness of combined interferential (IF) electrical stimulation and pelvic floor muscle (PFM) exercises on functional constipation in children. METHODS: We conducted a single-center, double-blind randomized clinical trial study during 2014-2017 in Tehran, Iran. Ninety children, aged 5-13 years, who fulfilled Rome III criteria were enrolled and randomly assigned into two treatment groups. Case group (n=45) underwent IF electrical stimulation and PFM exercises, whereas the control group (n=45) received PFM exercises plus sham stimulation. A complete bowel habit diary (with concerning 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, after the treatment and 6 months later for all participants. In addition, children in both groups were assessed with a constipation-related quality-of-life questionnaire before, after the end of treatment sessions, and 6 months after the treatment. RESULTS: Treatment success was achieved for 88.4% of children in the case group compared with 43.2% of children in the control group after the treatment (P<0.003). The median constipation score was reduced in both groups, with the cases having significantly lower scores after the treatment (4 vs. 8, P<0.000). Stool form normalized in 75.6% of the cases and 45.5% of the controls after the treatment (P<0.01). CONCLUSIONS: Our results showed that using IF electrical stimulation as an adjuvant therapy to the medical and rehabilitation programs significantly boosts the effects of treatment among these patients.

Systemic Amyloidosis in a Teenage Boy With Inflammatory Bowel Disease.

Systemic amyloidosis is a very rare complication of inflammatory bowel disease (IBD). The reported cases of secondary amyloidosis in children with IBD are much fewer than those reported in adults. Herein, a teenage boy with Crohn's disease is presented who developed nephrotic syndrome due to renal involvement secondary to amyloidosis, whereas the patient was under treatment with corticosteroid and 6-mercaptopurine. To our best knowledge, this is the first reported case of secondary amyloidosis in a teenage Iranian boy with Crohn's disease.

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.

Comparison of Oral Midazolam With Intravenous Midazolam for Sedation Children During Upper Gastrointestinal Endoscopy.

Upper endoscopy is a common procedure for the diagnosis and treatment of upper digestive tract diseases. The increasing number of pediatric gastrointestinal procedures has led to increasing attention on the safety and efficacy of medications used for sedation during the procedure. This randomized blinded interventional study was designed to compare the effect of oral midazolam with intravenous (IV) midazolam as a sedative medication in 119 children undergoing endoscopy. The mean time to sedation was 2.2±0.7 in IV midazolam group and 30.9±0 in oral midazolam group which was statistically significant difference between two groups. Separation from parents in oral midazolam group was as follow: 2 patients were high resistant (3.5%), 2 patients were resisted first and then relaxed (3.5%) and 55 patients were separated from their parents without any resistance (93%); whereas in IV midazolam group, 8 patients were high resistant (13.3%), 29 patients were relatively resistant (48.3%) and 23 patients were separated from their parents without any resistance (38.3%) that shows significant differences between the two groups. In terms of patient comfort during endoscopy, there was also a significant difference between the two groups. In oral midazolam, group parents were more consent, compared with the other group. The present study showed that oral midazolam is a safe and effective sedation during upper endoscopy in pediatrics. Oral midazolam reducing patients' anxiety during separation from parents leads the easy use of endoscopy and comfort of patients during endoscopy as compared with IV midazolam. Oral or IV midazolam were not able to put most patients in deep sedation level.