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.

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.

Vascular Changes in Eosinophilic Esophagitis (EOE), Report of an Unusual Case.

Background: Submucosal or lamina propria arteries are not often included in esophageal biopsies. We report an esophageal biopsy with eosinophilic esophagitis (EOE) overlying small arteries with medial hypertrophy to the point of obstruction. Case presentation: A two-year-old boy with a 1-year history of asthma frequently vomited after coughing. Esophageal biopsy showed EOE. Within the lamina propria there were small arteries with markedly thickened media to the point of luminal obstruction next to a hyperplastic lymphoid aggregate. There was no significant inflammatory infiltrate in the arterial walls. Subsequent biopsies did not show these vascular changes. Conclusion: Small artery changes in EOE have not previously been reported, and although the significance is unknown, in this case may be incidental to eosinophilic esophagitis.

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.

Growth Status and Its Relationship with Serum Lipids and Albumin in Children with Cystic Fibrosis.

Cystic fibrosis (CF) is an autosomal recessive disease, which affects many organs as it impairs chloride channel. This study was performed to evaluate growth status and its relationship with some laboratory indices such as Cholesterol (chol), Triglyceride (TG), albumin and total protein in children with CF referred to pediatrics center. This study was designed as a cross-sectional study in one year section. Demographic features were compared with standard percentiles curves. Chol, TG, albumin, total protein, prothrombin time, and hemoglobin were measured. Stool exams were also performed. A questionnaire was designed to obtain a history of the first presentation of disease, birth weight, type of labor and parent relativity. In 52% of patients, failure to thrive (FTT) was the first presentation. Steatorrhea and respiratory infections were the first presentations, which were seen in 13.7% and 33% of the cases, respectively. The weight of 88% of patients was below the 15th percentile while 82% had a height percentile below 15th. Head circumference in 53% of patients was below the 15th percentile. There was a significant association between weight percentile and serum albumin and total protein (P=0.03 and P=0.007, respectively). There was also a significant relationship between height percentile and serum albumin and total protein (P<0.001 and P<0.000, respectively). The relationships between head circumference and serum albumin and total protein were also significant (P=0.006 and P<0.000, respectively). There was also a significant association between height percentile and hemoglobin. The decrease in anthropometric percentiles leads to decreased serum albumin and total protein.

Novel VIPAS39 mutation in a syndromic patient with arthrogryposis, renal tubular dysfunction and intrahepatic cholestasis.

ARC syndrome is a rare autosomal recessive disease, characterized by arthrogryposis, renal tubular dysfunction and cholestasis. Herein a 2.5 month old infant with dysmorphic features, including small anterior fontanel, low set ears, beaked nose and high arched palate is presented who was referred because of icterus. He also suffered from some additional anomalies, including unilateral choanal atresia, club foot, and bilateral developmental dislocation of hip, while further studies showed renal tubular acidosis and hearing impairment in addition to cholestasis. Genetic studies showed a homozygous mutation in the VIPAS39 gene. Making the definite diagnosis of the syndrome is important, while increased risk of mutation in other siblings highlights the importance of prenatal diagnosis.

Comparison of one and two-day bowel preparation with polyethylene glycol in pediatric colonoscopy.

BACKGROUND/AIMS: Proper colon preparation in children has been a challenge for many years. Different regimens have been used for this purpose, but the best regimen is not determined. The aim of this study was to evaluate successful colon preparation before colonoscopy in children who were treated with one- or two-day regimen with polyethylene glycol (PEG) plus bisacodyl and clear liquids. MATERIALS AND METHODS: In this randomized clinical trial, 100 children (2-14 years old) who were candidates for colonoscopy were enrolled and divided into two groups. The children in group one were started on 2 g/kg PEG powder (17 g in 240 mL water or another beverage) and 5 mg bisacodyl suppository (BD) the day before colonoscopy, whereas those in the other group were started on 1.5 g/kg PEG with fruit juices for two days and 5 mg bisacodyl suppository (BD) for two days before colonoscopy. RESULTS: Compliance rates, regimens, adverse effects, and complete colonoscopy were not significantly different between the two groups. The Boston score was excellent and good in 70% of group one and 72% of group two children, respectively. Compliance rate, adverse effects, and need for enema were similar in both groups. The rate of compliance and non-requirement of enema were significantly higher in children with satisfactory colon preparation. CONCLUSION: The one-day PEG plus bisacodyl regimen for bowel preparation is as effective as the two-day regimen in children; furthermore, it is well tolerated and has low adverse effects.

Low incidence of alpha-1-antitrypsin deficiency in Iranian patients with neonatal cholestasis.

BACKGROUND/AIMS: There is little data concerning the incidence of alpha-1-antitrypsin"(AAT) deficiency, the most common genetic cause of liver disease, among children with neonatal cholestasis in Iran. Thus, this study was performed to analyze AAT deficiency in this group of patients. MATERIALS AND METHODS: DNA samples from patients with neonatal cholestasis were investigated for Pi S and Pi Z alleles, using polymerase chain reaction-restriction fragment length polymorphism. RESULTS: Thirty patients with neonatal cholestasis were enrolled. Among those who underwent biopsies, the results revealed neonatal hepatitis in 19, bile duct paucity in 1, steatohepatitis in 1, bile duct proliferation in 1, cirrhosis in 2, fibrosis in 2, and extrahepatic biliary atresia in 1 patient. No mutant allele was found in any patient. CONCLUSION: The incidence of AAT deficiency is very low in Iran; therefore, screening for AAT is not recommended for patients with neonatal cholestasis in Iran.

Bloody Diarrhea as a Presentation Manifestation of Familial Mediterranean Fever in a Patient with Compound Heterozygote Mutations of the MEFV Gene.

Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by episodic fever and inflammatory polyserositis, which could lead to a variety of manifestations, including recurrent abdominal pain. Herein, a 12-year-old boy who has suffered from fever and bloody diarrhea since early childhood is described. All structural and underlying disorders leading to bleeding were excluded. Genetic studies indicated compound heterozygote mutations of M680I/R761H in the MEFV gene, which confirmed the diagnosis of FMF. Therefore, treatment with colchicine was started, which led to symptom relief. As gastrointestinal manifestations appear to be the main features of FMF, bloody diarrhea could also be considered an initial symptom of FMF.

Novel UBR1 gene mutation in a patient with typical phenotype of Johanson-Blizzard syndrome.

Johanson-Blizzard syndrome is a rare autosomal recessive disorder, characterized by exocrine pancreatic deficiency and a wide range of other abnormalities. We present here an infant with failure to thrive, exocrine pancreatic deficiency, short stature and developmental delay, cutis aplasia on the scalp, aplasia of alae nasi, hypospadias, hypothyroidism, myxomatous mitral valve, and patent ductus arteriosus. Molecular studies revealed a novel homozygous nonsense mutation in exon 38 of the UBR1 gene, which confirmed the diagnosis of Johanson-Blizzard syndrome. It should be acknowledged that the combination of exocrine pancreatic insufficiency and nasal wing hypo-aplasia is pathognomonic for this syndrome. Prompt diagnosis and exact monitoring of the patients with JBS are required to avoid further complications.

Screening for celiac disease in diabetic children from Iran.

Celiac disease has been shown to be associated with type 1 diabetes mellitus. We conducted this study to determine the frequency of celiac disease in a group of Iranian diabetic children. Ninety-six patients with type 1 diabetes mellitus were tested for anti-tissue transglutaminase antibodies. Six patients (6.25%) were seropositive, and histopathological changes were compatible with celiac disease in intestinal biopsy.

Clinical characteristics of Iranian pediatric patients with inflammatory bowel disease.

BACKGROUND: Inflammatory bowel disease (IBD) is a group of disorders, including Crohn's disease (CD), ulcerative colitis (UC) and indeterminate colitis (IC). Small intestine and colon are primarily affected in this group of disorders. PURPOSE: This single center study was performed to define demographic features and clinical characteristics of Iranian pediatric patients with IBD. METHOD: Fifty nine patients with IBD, who have been referred to Children's Medical Center Hospital during a 10-year period, were investigated in this study. The data was gathered by interviewing the patients and their families, as well as reviewing their medical records. RESULTS: Among 59 patients with IBD, 23 cases had UC and 19 cases had CD, while the remaining 17 cases had IC. Patients with UC were significantly younger at the time of diagnosis in comparison with patients with CD. The most common symptoms were abdominal pain, diarrhea, fever and growth failure. Hepatobiliary abnormalities and arthritis were common extra intestinal manifestations. The median Pediatric Crohn's Disease Activity Index was 42.5 (range 20-60), whereas the median Pediatric Ulcerative Colitis Activity Index was 40 (range 20-70). Seven of 23 UC (30.4%) had proctatis, while 16 (69.6%) had extensive colitis. In CD, 11 of 19 (57.9%) had involvement of terminal ileum and colon, while inflammation was limited to the colon in 8 cases (42.1%). The colonoscopic findings in the IC group were heterogeneous. CONCLUSION: This study provides epidemiological data on pediatric patients with IBD, which could be useful for health care workers in prompt diagnosis and appropriate treatment of early onset IBD.

Congenital choledochal cyst in an infant with cystic fibrosis.

Congenital choledochal cyst is malformation of the biliary ductal system, which rarely occur. We describe here a 4-month old boy, who was referred to our center with respiratory distress and low level consciousness. In physical examination, a mass was detected in right upper quadrant of abdomen. Sonographic examination indicated a cystic structure representing the choledochal cyst. Further evaluation confirmed the diagnosis of cystic fibrosis in this patient. Although choledochal cyst is considered as a rare disease, it is the most frequent malformation of the extrahepatic biliary ducts, which easily could be misdiagnosed.

Colorectal polyps: a clinical, endoscopic and pathologic study in Iranian children.

OBJECTIVE: To study the clinical presentation, histology and colonoscopic features of lower gastrointestinal polyps in Iranian children. MATERIAL AND METHODS: Medical reports of children with colorectal polyps were retrospectively reviewed from 1996 to 2005 at the Children's Medical Center Hospital, Iran. A total of 563 cases were studied. Data related to age, sex, family history, signs and symptoms, the size, location, polyp types and associated lesions were collected and analyzed. RESULTS: The mean age of children was 5.66 +/- 2.88 years (range 2 months to 17 years), with a male-to-female ratio of 1.61:1.0. The highest incidence was between ages 2 and 10 years (85.1%). Rectal bleeding was the presenting symptom in 78.5% cases. The polyps were solitary in 94% of cases. A majority of polyps (86.3%) were juvenile and 86.7% located in the rectosigmoid area. Three percent of cases had a positive family history. One case of Turcot syndrome was also identified. CONCLUSION: Juvenile polyps remain the most common polyps in Iranian children. Although the presence of a solitary polyp in the rectosigmoid colon is more prevalent, in a significant number of cases they are multiple and located in proximal parts. Polyps must be removed even when asymptomatic because of their probable neoplastic potential.

The effect of omeprazole on asthmatic adolescents with gastroesophageal reflux disease.

The prevalence of gastroesophageal reflux disease (GERD) is increasing in patients with asthma and the effect of proton pump inhibitor therapy on asthma outcome has shown variable results. The aim of this study was to determine the efficacy of omeprazole in the treatment of asthma and improvement of pulmonary function in adolescents with GERD. Thirty-six consecutive patients (range, 13-20 years old) with moderate persistent asthma and GERD were recruited on regular follow-up in Mashhad City. The case group included 18 patients who received oral omeprazole (20 mg twice a day for 6 weeks) and the control group included 18 patients who received placebo. A pulmonary function test was examined in two groups immediately before and 6 weeks after medication. The symptoms of GERD were significantly improved with omeprazole in the case group. After 6 weeks of study, the mean values of forced vital capacity, forced expiratory volume in 1 second, and peak expiratory flow rate were higher in patients treated with omeprazole (p<0.0001). Treatment by omeprazole is effective for treatment of asthmatic patients with GERD.

Cyclic vomiting syndrome in children: experience with 181 cases from southern Iran.

AIM: To evaluate the clinical presentation, response to prophylactic therapy and outcome of children with cyclic vomiting syndrome (CVS) in Shiraz, Iran. METHODS: During a period of 11 years (March 1994 to March 2005), 181 consecutive children with a final diagnosis of CVS were evaluated, treated and followed in our center. Patients were randomized to receive either amitriptyline or propranolol as prophylactic treatments. RESULTS: There were 88 boys and 93 girls with mean age of onset of symptoms of 4.9 +/- 3.3 years (range, neonatal period to 14 years), the mean age at final diagnosis was 6.9 years (range, 1.5 to 14), and the mean duration between the onset of the first attack and the final diagnosis of CVS was 2 +/- 1.81 years (range, 1/6 to 8). The mean duration of each attack was 4.26 days (range, from few hours to 10 d) and the mean interval between the attacks was 1.8 mo (range, 1 wk to 12 mo). The time of onset of the attacks was midnight to early morning in about 70% of cases. Amitriptyline was effective in 46 out of 81 (56%) patients (P < 0.001). Propranolol appeared to have a superior action and was effective in 74 out of 83 (92%) patients (P < 0.0001). CONCLUSION: There is a significant lag time between the onset of clinical symptoms and the final diagnosis of CVS in our area. In patients with typical clinical presentations of CVS, who are examined by an experienced physician, invasive workup is not necessary. Propranolol appears more effective than amitriptyline for prophylactic use in children with CVS.

Helicobacter pylori culture and antimicrobial resistance in Iran.

OBJECTIVE: Helicobacter pylori is considered as an important etiologic factor in pathogenesis of peptic ulcer disease, chronic gastritis and gastric cancer. To eradicate this micro-organism, numerous regimens containing various antimicrobial agents have been suggested. However, H pylori antimicrobial resistance is a leading factor to treatment failure and recurrence of the disease. The aim of the study was to evaluate the prevalence of H pylori resistance to metronidazole, clarithromycin, tetracycline, amoxicillin, erythromycin and furazolidone in authors pediatric patients. METHODS: Antral biopsy of all pediatric patients with negative history of receiving anti-H pylori regimen and endoscopic findings of nodular gastritis or peptic ulcer without previous history of NSAID consumption, burning and trauma were performed for H pylori histology, urease test and culture. All positive cultures were tested for antimicrobial susceptibility. RESULTS: Twenty four patients (14 male and 10 female) between 3.5 and 14 years of age were culture positive. 54.16% of the isolates were resistant to metronidazole, 8.33% to amoxicillin, 4.16% to erythromycin and 4.16% to clarithromycin. None of authors patients were resistant to tetracycline and furazolidone. CONCLUSION: H. pylori antimicrobial resistance could be a major contributor to failure of H pylori eradication. Continuous prospective surveillance of H. Pylori is essential. Moreover, culture and antimicrobial susceptibility test is recommended for resistant cases after the first failure to therapy.

Antimicrobial resistance of Helicobacter pylori isolates from Iranian adults and children.

BACKGROUND: Helicobacter pylori isolates from 84 adults and 51 children were assessed during the period of 2001 through 2004 to find out whether their susceptibilities to metronidazole, clarithromycin, amoxicillin, tetracycline, and furazolidone differ between the two groups or have changed compared with the results from our previous study done between 1997 - 2000. METHODS: Biopsies from 135 dyspeptic patients were cultured on Brucella blood agar. Pure cultures of H. pylori isolates were used for antibiotic susceptibility tests. Disk diffusion method was recruited to assess the susceptibility of H. pylori isolates to different dilutions of the above mentioned antimicrobials. Results obtained from the two groups were compared and minimum inhibitory concentrations determined. RESULTS: Thirty-five percent of H. pylori isolates from adults and 37% from children were resistant to metronidazole. Clarithromycin resistance rate was 2.4% and 5.9% in H. pylori isolates from adults and children, respectively. Amoxicillin resistance was 2.4% in isolates from adults and 5.9% in isolates from children. Tetracycline resistance rate was 0% in H. pylori isolates from adults and 2.0% in isolates from children. Resistance to furazolidone was not observed. CONCLUSION: Resistance rates of H. pylori isolates from adults and children to metronidazole, clarithromycin, amoxicillin, tetracycline, and furazolidone are similar and not significantly affected by age and time.