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.

Non-retentive faecal incontinence in children: Conventional therapy alone or combined electrical stimulation.

AIMS: To compare the effects of functional electrical stimulation (FES) with conventional therapy and conventional therapy alone on improvement of faecal incontinence (FI) symptoms in a number of children with functional non-retentive FI. METHODS: Data of 28 children with FI were accepted for this retrospective study. The case group (n = 14) underwent FES + conventional therapy and the control group (n = 14) received conventional therapy only. Data of children with faecal retention, inflammatory, anatomic, metabolic and neurological disorders were excluded. Children were assessed with a paediatric FI score questionnaire, and a bowel habit diary both before treatment sessions, after they ended, and after 6 months. A FI quality of life questionnaire was completed before and after treatment for all children. RESULTS: Full response to the treatment (100% reduction in FI episodes) was significantly observed in 8/14 (57.1%) of children in the case group compared to 2/14 (14.2%) of children in the control group after the ending of treatment sessions (P = 0.005). The baseline mean ± SD of FI episodes per week was 3.7 ± 2.1 among both groups which significantly decreased after the ending of treatment sessions in the case group compared to the control group (1.4 ± 2.1 vs 3 ± 2.7; P = 0.05). Mean ± SD of FI score was significantly reduced in the case group compared to the controls after the ending of treatment sessions (3.9 ± 4.5 vs 8 ± 4.7; P = 0.02) and maintained after 6 months (P = 0.05). CONCLUSIONS: Functional electrical stimulation is a safe, effective, non-invasive, inexpensive, reproducible and easy-to-use modality for treatment of functional non-retentive faecal incintinence in children.

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.

Association of autoimmune pancreatitis with Raghib syndrome.

Key Clinical Message: Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis scarcely found in children. Raghib syndrome is a rare congenital heart defect known as persistent left superior vena cava (LSVC) draining into the left atrium. Total signs of Raghib syndrome in AIP case accompanied by an IgG4-related disease were described. Abstract: Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis scarcely found in children. Raghib syndrome is a rare congenital heart defect known as persistent left superior vena cava (LSVC) draining into the left atrium. Here, we describe Raghib syndrome in AIP case accompanied by an IgG4-related disease (AIP/IgG4RD). A 13-year-old boy presented with a 3-month history of fever and abdominal pain. The laboratory findings showed SGOT and SGPT, ALP was increased, while amylase and γ-GT were normal. Immunoglobulins were normal, except for IgG. Endosonography, spiral CT of the abdomen, and cholangiopancreatography showed an enlargement of the pancreas. Contrast echocardiography discovered opacification of the coronary sinus and left atrium. Transesophageal echocardiography for LSVC revealed a dilatation in the coronary sinus, indicating persistent LSVC. Following the injection of agitated saline into the left antecubital vein, bubbles entered both left and right atria in LSVC. It is reasonable to exclude some of these rare disorders as Raghib syndrome, in cases that will be started on medications like corticosteroids, which increases the susceptibility to thromboembolic events.

Previously Unreported Pseudomembranous Duodenitis: A Case Report With Histopathology.

The pseudomembranous inflammatory process is a process characterized by the formation of a white membrane-like exudate over colonic mucosa and is mainly caused by Clostridium difficile toxin. The stool culture is considered to be the gold standard and is technically challenging and is not performed routinely. There are some reports of duodenitis and proximal jejunitis in horses attributed to Clostridium difficile infection. Hereby, we report a case of pseudomembranous duodenitis in a seven-year-old boy with a complaint of severe abdominal pain. Upper endoscopy revealed patchy ulceration and a white membrane in the duodenum. A biopsy was taken with the impression of a fungal infection. The histological study revealed crater-like ulceration with upward exudation of mucus consistent with the pseudomembranous inflammatory process. To the best of our knowledge, pseudomembranous duodenitis is not reported in the human as yet.

Atopy Patch Test in the Diagnosis of Food Allergens in Infants with Allergic Proctocolitis Compared with Elimination/Introduction C.

Allergic proctocolitis is a cell-dependent food allergy that is present in both breast and formula-fed infants. The presence of blood with different amounts in the stool is the main manifestation of the disease. Different results have been published on the accuracy and specificity of the atopic patch test (APT). The purpose of this study was to evaluate the results of the APT and compare them with those obtained in the food elimination/introduction (E/I) challenge, as the gold standard of confirming the allergy. Twenty-eight patients (18 boys, 10 girls, <1 year) with allergic proctocolitis were recruited in this study. The mean age of the disease onset and enrolling the study were 2.23±1.7 and 5.25±2.19 months, respectively. After performing APT with fresh foods, an E/I challenge was done in a patient with positive tests, and results were analyzed. APT was positive in 14/28 (50%) individuals. The most common foods detected by APT in all of the individuals were: milk (10/28), rice (5/28), soy (4/28), and egg white (4/28), while in E/I challenge in the APT-positive individuals were: milk (8/10), rice (3/5), egg white (1/4), and soy (0/4). APT was positive in half of the infants<1 year with allergic proctocolitis and there was no significant correlation between the APT results and the E/I challenge test for all foods. Comparing the results of APT and E/I challenge methods showed a convergence between the milk and rice sensitivity, thus we suppose APT to be a useful tool in identifying these two allergens in cell-mediated food allergies like allergic proctocolitis.

The Resistance Rate of Helicobacter Pylori to Clarithromycin and Main Mutations on Bacterial Genomic Responsible for Bacterial Resistance: A Comparative Study in Children and Adults, Tehran and Iran.

BACKGROUND: High resistance to common antibiotics has become a huge global dilemma in eradicating Helicobacter Pylori infection in both children and adults. The great concern is about the resistance to different classes of antibiotics especially Clarithromycin because of its widespread use. OBJECTIVES: The present survey aimed to assess the resistance rate to Clarithromycin in Helicobacter Pylori isolated in patients aged less than 15 years as compared to patients older than 15 years of age. METHODS: In this cross-sectional study, total 72 patients with upper gastrointestinal symptoms requiring diagnostic endoscopy referred to Rasoul-e-Akram Hospital in Tehran during one year (August 2015 to August 2016). Helicobacter Pylori infection was diagnosed in patients using the Rapid Urease Test. The antibiotics resistance was detected in genomes using the real-time polymerase chain reaction (PCR) on 23S rRNA gene. RESULTS: In total 72 patients, 36 cases aged less than or equal to 15 years and 36 patients were older than 15 years. Of all patients in this study, 17 cases were detected with gene mutations or polymorphisms related to resistance to Clarithromycin. Overall prevalence rate of resistance was reported 23.61%. Three polymorphisms on 23S rRNA gene including A2142G, A2142C, and A2143G were revealed in 47.1%, 5.9%, and 47.1% of patients, respectively. The bacterial resistance to Clarithromycin was observed more prevalent in patients that aged older than 15 years compared to patients younger than 15 years of age. Also, frequent consumption of any type of antibiotics was significantly associated with the higher resistance of bacterium to Clarithromycin. CONCLUSION: The results of our study regarding the resistance of Helicobacter Pylori to Clarithromycin were similar to findings of other studies around the world. But, the Clarithromycin resistance rate was reported higher in patients older than 15 years of age and those patients who repeatedly received different types of antibiotics regardless of their age. Of all mutations in bacterial genome, the prominent mutations responsible for bacterial resistance to Clarithromycin included A2142C, A2142G, and A2143G nucleotide polymorphism on 23S rRNA gene.

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.

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.

IL4 gene polymorphisms in Iranian patients with autoimmune hepatitis.

BACKGROUND: Autoimmune hepatitis (AIH) is a chronic long-lasting hepatocellular inflammation associated with circulating auto antibodies. In addition to the genetic component, several cytokines have been implicated to be involved in AIH. This study was performed to investigate potential associations of AIH with IL4 gene variants. METHOD: The studied alleles and genotypes included: IL4G/T allele polymorphisms at position -1098 and C/T allele polymorphisms at two positions (-33 and -590) on the IL4 gene, in addition to the A/G allele polymorphisms at position +1902 on the IL4RA gene. RESULT: The IL4 C allele and CC genotype at position -590 and TT genotype at position -33 had a significantly higher frequency in AIH patients. CONCLUSION: This study identified the IL4 C allele and CC genotype susceptibility gene in AIH, which will provide better insights into the mechanisms of AIH and potential therapeutic interventions.

Familial congenital hepatic fibrosis: report of a family with three affected children.

Congenital hepatic fibrosis (CHF) is a developmental disorder of the biliary system, characterized by defective remodeling of the ductal plate. Herein a family of three children, from consanguineous parents, with minor thalassemia is presented who suffered from congenital hepatic fibrosis (CHF). Prompt diagnosis and appropriate treatment are necessary to avoid further complications in the affected patients.

The effect of probiotics on fecal calprotectin in patients with cystic fibrosis.

Cystic fibrosis (CF) is a common autosomal recessive disorder with different clinical manifestations, mainly in the gastrointestinal and respiratory tracts. This study was performed to access the effect of probiotics in the status of intestinal inflammation in a group of children with CF by measuring the calprotectin level in the fecal samples. Forty-seven patients with CF were enrolled in this study. The fecal calprotectin levels were measured by enzyme linked immunosorbent assay. In a randomized systematic method, the children were divided into two groups - one group received probiotic powder and another received placebo for four weeks. After this period, fecal calprotectin was re-measured. Thirty-one of 47 enrolled patients (65.9%) had abnormal fecal calprotectin levels (>50 g/g). After the intervention, the fecal calprotectin levels decreased in 29 patients (21 patients in the drug group, and only 8 patients in the placebo group; p<0.001). This study showed that about two-thirds of patients with CF had intestinal inflammation based on fecal calprotectin levels. Probiotic administration was shown to decrease calprotectin concentrations and subsequently intestinal inflammation in CF patients.

Erythropoietic protoporphyria and early onset of cholestasis.

Erythropoietic protoporphyria (EPP) is an inherited defect of mitochondrial ferrochelatase. This defect results in accumulation of protoporphyrin in erythrocytes, plasma, liver, and skin, which causes severe photosensitivity. Liver disease can occur in 1-4% of the patients with EPP, usually after at least a decade of photosensitivity. Herein, we describe a 1.5-year-old child with EPP with severe photosensitivity, heart abnormalities and early onset of cholestatic liver disease, whose clinical condition improved gradually after using ursodeoxycholic acid. It seems that liver disease in EPP patients is not limited to the late phases of the disease and could develop in childhood and early phases of EPP. Awareness among physicians has a major role in the early detection and prevention of mistreatment of EPP in case of its combination with other abnormalities.

The prevalence of exercise-induced bronchospasm in soccer player children, ages 7 to 16 years.

This study represents an attempt to determine the prevalence of exercise-induced bronchospasm among soccer player children. A total of 234 soccer player boys of all soccer schools from Shahr-Rey enrolled in this study. They did not have any history of a recent or chronic respiratory tract disease, a history of allergic diseases, and history of bronchodilator drugs consumption during the 24 hours prior to the study. Pulmonary function test (PFT) was performed for each participant before exercise and 6 and 15 minutes after playing soccer. The diagnosis of EIB was by a decrease in forced expiratory volume in 1 second (FEV1) by at least 10% and in peak expiratory flow rate (PEFR) by at least 15% with exercise challenge. If there was reduction in one parameter alone, the participants were considered as prone to EIB. Considering both FEV1 and PEFR the prevalence of EIB was 2.1% and 18.4% were prone to EIB. If FEV1 or PEFR tests were used as criteria for diagnosis of airway obstruction, the prevalence of EIB would be 6% and 15.8%, respectively. There was no significant difference between the post of players, family history of allergic disease and EIB in soccer players. This study suggests that at least 2.1% of soccer players will develop bronchospasm even if they do not have any history of asthma and allergy.