Silvana Bonilla

Cow's-milk allergy is a risk factor for the development of FGIDs in children.

Objectives: Functional gastrointestinal disorders (FGIDs) are common in children. Their pathogenesis remains unknown and is most likely multifactorial. We hypothesized that noninfectious causes of inflammation affecting the gastrointestinal (GI) tract early in life, such as cows-milk allergy (CMA), can predispose to the development of FGIDs later in childhood. Patients and Methods: Case-control study. Subjects were patients between 4 and 18 years diagnosed with CMA in the first year of life at Childrens Memorial Hospital in Chicago, IL, between January 2000 and June 2009. Diagnosis of CMA was based on history and clinical findings. Siblings 4 to 18 years of age without a history of CMA were selected as controls. Cases completed the parental form of the Pediatric Gastrointestinal Symptoms Rome III version questionnaire to assess for GI symptoms. Results: Fifty-two subjects (mean age 8.1 ± 4.48 years, 62% girls) and 53 controls (mean age 9.7 ± 4.20 years, 55% girls) participated in the study. Twenty-three of 52 subjects (44.2%) reported GI symptoms that included abdominal pain, constipation, or diarrhea compared with 11 of 53 controls (20.75%) (odds ratio 3.03, P = 0.01). Abdominal pain was significantly more common in cases (16/52, 30.8%) versus controls (5/53, 9.43%) (odds ratio 4.27 [1.43–12.7]) (χ2 = 7.47, P = 0.01). Abnormal stool habits were more common in cases (15/52, 28.8%) versus controls (7/53, 13.2%), but the difference was not statistically significant. Ten of 52 subjects (19.2%) met the Questionnaire on Pediatric Gastrointestinal Symptoms Rome III version criteria for diagnosis of an FGID (7 irritable bowel syndrome, 2 functional dyspepsia, 1 functional abdominal pain), whereas none in the control group did. Conclusions: CMA constitutes a risk factor for the development of FGIDs in children.

Early Life Events: Infants with Pyloric Stenosis Have a Higher Risk of Developing Chronic Abdominal Pain in Childhood

OBJECTIVE We hypothesize that children who had pyloric stenosis are at greater risk for developing chronic abdominal pain because this cohort combines various risk factors: an early stressful event, gastric surgery, and perioperative nasogastric tube placement in most cases. STUDY DESIGN This was a case control study of all children diagnosed with pyloric stenosis during infancy (cases) between January 1, 2000, and June 31, 2005, at Childrens Memorial Hospital, Chicago. Because of their similar genetic and socioeconomic backgrounds, siblings aged 4 to 20 years without a history of pyloric stenosis were selected as controls. Parents of children with symptoms completed the parental form of the Pediatric GI Symptoms Rome III version questionnaire for both cases and controls. The primary outcome was the prevalence of chronic abdominal pain, and the secondary outcome was the presence of pain-associated functional gastrointestinal disorder (FGID), in accordance with Rome III criteria. RESULTS Cases (n = 100; mean age, 7.49 ± 1.43 years; 29 girls) and controls (n = 91; mean age, 9.20 ± 4.19 years; 29 girls) participated in the study. Mean time to follow-up was 7.2 ± 1.6 years. Chronic abdominal pain was significantly more common in cases than in controls (20/80 [25%] vs 5/91 [5.8%]; OR, 4.3; 95% CI, 1.5-12; P = .0045). Seven out of 20 subjects (35%) met the Rome III criteria for diagnosis of a pain-associated FGID (3 with irritable bowel syndrome, 2 with functional dyspepsia, and 2 with functional abdominal pain), and 1 patient in the control group (with irritable bowel syndrome) met these criteria (OR, 6.8; 95% CI, 0.82-56; P = .043). CONCLUSION We have described a new model to study early life events in infants. Our findings suggest that the presence of pyloric stenosis in infancy and factors involved in its perioperative care represent risk factors in the development of chronic abdominal pain in children at long-term follow-up. This study provides important data to sustain the multifactorial theoretical construct of pain-associated FGID and underscores the importance of early life events in the development of chronic abdominal pain in children.

Parental report of abdominal pain and abdominal pain-related functional gastrointestinal disorders from a community survey.

Background and Aims: Functional gastrointestinal disorders (FGIDs) are common in children. Abdominal pain (AP) is the most common gastrointestinal (GI) symptom in children. The severity of AP drives medical consultations and quality of life in adult patients with irritable bowel syndrome (IBS). Thirty-eight percent of 8- to 15-year-old schoolchildren report AP weekly with 24% of those children reporting persistence of AP >8 weeks. Despite the high prevalence of AP, only 2% of school children seek medical attention for AP. Lack of parental knowledge on their childs symptoms may constitute one of the factors affecting the low ratio of consultation in children reporting AP. The aim was to assess parental reports of AP symptoms in a population of healthy community children. Methods: Data of 5 studies with identical methodology to assess GI symptoms in children with celiac disease (CD), cows milk allergy (CMA), pyloric stenosis (PS), Henoch-Schönlein purpura (HSP), and stem cell transplant (SC) and their healthy siblings were reviewed: a phone questionnaire on GI symptoms and Pediatric Gastrointestinal Symptoms Rome III version questionnaire (QPGS-RIII). Inclusion criteria were healthy children 4 to 18 years of age with a sibling previously diagnosed with CD, CMA, PS, HSP, or SC. Results: Data on 246 healthy children, mean age (9.8 years, range 3–24, 112 girls) were obtained. Parents reported presence of AP in the last 8 weeks before the telephone contact in 20 (8.1%) children (age range 4–18 years, 11 girls). There was no significant difference in AP prevalence between boys and girls (P = 0.64). Six children (2.4%) met QPGS-RIII diagnostic criteria for FGIDs: 3 functional abdominal pain (FAP) and 3 IBS. Conclusions: AP was common in community children. FAP was the most common FGID among healthy community children. The prevalence of AP by parental report is lower than the previously published prevalence of AP reported by children. Lack of awareness of childrens symptoms may play a role in the low ratio of consultation for AP in symptomatic children. Future prospective studies should confirm our findings and investigate the factors influencing the medical consultation decision including parental awareness of childrens symptoms.

Relationship of Proximal Renal Tubular Dysgenesis and Fetal Liver Injury in Neonatal Hemochromatosis

Renal tubular dysgenesis has been reported in isolated cases of neonatal hemochromatosis (NH). We hypothesized that fetal liver injury in NH impairs proximal renal tubular development via impaired hepatic angiotensinogen (AGT) elaboration. Morphometric analyses were performed of postmortem liver and kidney sections of cases of proven NH and postconception age-matched controls for renal proximal tubule density, hepatocyte mass, and hepatic AGT expression. Proximal tubule density was markedly reduced in NH cases, although they showed a spectrum from mild to severe paucity. Hepatic AGT expression was markedly reduced in NH cases and correlated closely with reduced hepatocyte mass. A linear relationship was established between hepatic AGT expression and the degree of renal tubular dysgenesis suggesting that there is a relationship between them. Our results demonstrate that there is a spectrum of kidney pathology in patients with NH including a large proportion of cases with severe proximal tubular dysgenesis. Hepatic synthetic failure resulting in insufficient production of AGT to support renal tubular development is the likely mechanism of kidney disease in NH.

The Prognostic Value of Obtaining a Negative Endoscopy in Children With Functional Gastrointestinal Disorders

Objectives: Functional gastrointestinal disorders (FGIDs) are common. Diagnosis is based on the Rome criteria. It has been argued that performing endoscopies could help by providing reassurance and thus improve outcomes. This study was conducted to evaluate the impact of obtaining normal endoscopies in the outcome of children with FGIDs. Methods: Medical records of patients diagnosed with abdominal pain related-FGIDs were reviewed. To assess the child’s symptoms at follow-up, parents completed the Questionnaire on Pediatric Gastrointestinal Symptoms III (QPGS III). Primary outcome was presence of abdominal pain (AP) and secondary outcomes were AP severity. Results: A total of 301 patients were diagnosed with abdominal pain—related FGIDs. Overall, 62.6% reported persistence of AP and 37.4% were asymptomatic at follow-up. Among patients with endoscopies, 61% reported AP. Among patients without endoscopies, 64% were symptomatic (P = .76). AP frequency, AP intensity, and child’s disability were similar in those with and without endoscopies. Conclusion: The study does not suggest that a negative endoscopy improves the outcome of children with FGIDs.

Rapunzel syndrome: a rare cause of biliary obstruction.

522 e is a rare manifestat ands of hair extendin R apunzel syndrom ion of gastric trichobezoars with str g beyond the pylorus into the small intestine, and occasionally up to the colon. So far, only 29 cases of Rapunzel syndrome have been reported in the literature worldwide, with the first case described in 1968 (1). With the exception of 1 male, all of the other reported cases were females. Nausea, vomiting, abdominal pain, and symptoms of obstruction were the common presenting features. Trichobezoars rarely results in biliary obstruction or pancreatitis. We describe a 3-year-old girl with Rapunzel syndrome presenting with a picture of cholestasis because of biliary obstruction from a trichobezoar.

Management of pediatric patients with refractory constipation who fail cecostomy

BACKGROUND Antegrade continence enema (ACE) is a recognized therapeutic option in the management of pediatric refractory constipation. Data on the long-term outcome of patients who fail to improve after an ACE-procedure are lacking. PURPOSE To describe the rate of ACE bowel management failure in pediatric refractory constipation, and the management and long term outcome of these patients. METHODS Retrospective analysis of a cohort of patients that underwent ACE-procedure and had at least 3-year-follow-up. Detailed analysis of subsequent treatment and outcome of those patients with a poor functional outcome was performed. RESULTS 76 patients were included. 12 (16%) failed successful bowel management after ACE requiring additional intervention. Mean follow-up was 66.3 (range 35-95 months) after ACE-procedure. Colonic motility studies demonstrated colonic neuropathy in 7 patients (58%); abnormal motility in 4 patients (33%), and abnormal left-sided colonic motility in 1 patient (9%). All 12 patients were ultimately treated surgically. Nine patients (75%) had marked clinical improvement, whereas 3 patients (25%) continued to have poor function issues at long term follow-up. CONCLUSIONS Colonic resection, either segmental or total, led to improvement or resolution of symptoms in the majority of patients who failed cecostomy. However, this is a complex and heterogeneous group and some patients will have continued issues.

Management of chronic intractable constipation in children.

Background: Chronic intractable constipation (CIC) is a debilitating disease that is challenging to manage. Treatment options in children include medications, enemas, and surgical management in selected cases. Method: We reviewed medical records of pediatric patients diagnosed as having CIC at Tufts Medical Center from 2005 to 2012. Demographic variables, diagnostic procedures, and medical and surgical outcomes were collected. Clinical outcome was defined using the Rome III criteria. Results: A total of 14 patients were included in the study (10 boys). The age range was 10 to 21 years. All of the patients had the diagnosis of CIC. Eleven patients had cecostomy placement. During the follow-up period, 10 patients underwent total abdominal colectomy with ileorectal anastomosis, 1 had total colectomy with ileostomy, and 1 had partial colectomy with colorectal anastomosis. Successful clinical outcome was reported in 7 patients with 3 patients reporting persistent fecal incontinence. Colonic motility studies were performed on 12 patients (colonic neuropathy in 11 patients and normal study in 1 patient). Defecography was consistent with isolated pelvic floor dysfunction in 1 patient, abnormal motility and anatomy in 1 patient, pelvic floor dysfunction and abnormal motility in 2 patients, and found abnormal motility only in 5. Defecography study was normal in 5 patients. All of the patients with abnormal colonic manometry underwent a surgical procedure. Conclusions: Anorectal manometry, colonic manometry, and defecography help in understanding the pathophysiology of defecation disorders in children. The majority of patients with abnormal colonic manometry underwent TAC-IRA. There was no statistical correlation between individual investigations (anorectal manometry, colonic manometry, and defecography) with surgical intervention (P > 0.35). TAC-IRA may be safe and useful intervention in a subset of patients when other treatment options have failed.

Prevalence of Abdominal Pain and Functional Gastrointestinal Disorders in Community Children

ence among countries. In 77.3%, laboratory tests were performed. Etiology was 51.1% viral, 22.7% bacterial, 12.9% parasitological, 1.5% antibiotic-induced or 16.1% other/unknown. Bacterial etiology was higher in Egypt (33.1%), and parasitological cause in Venezuela (23.7%). In American countries, oral rehydration (86.9%), probiotics (80.6%), antipyretics (33.5%), antibiotics (19.5%), antiemetics (16.1%) and antidiarrheals (6.5%) were prescribed at entry. In Egypt, therapeutic pattern differed markedly: oral rehydration (54.2%), probiotics (22.5%), antipyretics (61.8%), antibiotics (39.5%), antiemetics (56.2%) and antidiarrheals (60.5%). Remission of diarrhea was confirmed at longitudinal visit in 92.3% of children, without clear difference among countries (range 89.9% to 96.7%). Prescription of antibiotics at entry was significantly associated with persistence of diarrhea (Odds Ratio 1.64, CI 1.092.49; p=0.018) and incidence of clinical complications (OR 1.60, CI 1.13-2.26; p=0.007) at longitudinal visit, whereas prescription of probiotics was associated with reduced risk of diarrhea persistence (OR 0.56, CI 0.38 to 0.83; p=0.003) and clinical complications (OR 0.64, CI 0.46 to 0.88; p=0.006). Overall, 90% of physicians claimed using recommended guidelines. Conclusions: Therapeutic strategies for acute diarrhea in children differed among countries, but there were no significant differences in achieving remission. Use of antibiotics as initial treatment in this population suffering mainly from viral diarrhea was significantly associated with poor outcome whereas use of probiotics was associated with improved outcome.