Khalil El-Chammas

Gluten-free diet in nonceliac disease.

A gluten-free diet (GFD) is commonly recognized as the treatment for celiac disease. It also has been investigated as a treatment option for other medical conditions, including dermatitis herpetiformis, irritable bowel syndrome, neurologic disorders, rheumatoid arthritis, diabetes mellitus, and HIV-associated enteropathy. The strength of the evidence for the use of a GFD in these nonceliac diseases varies, and future research may better define the benefits of a GFD for those conditions with weak existing evidence.

Subjective Global Nutritional Assessment in Critically Ill Children

BACKGROUND Underweight children admitted to the pediatric intensive care unit (PICU) have a higher risk of mortality than normal-weight children. The authors hypothesized that subjective global nutrition assessment (SGNA) could identify malnutrition in the PICU and predict nutrition-associated morbidities. METHODS The authors prospectively evaluated the nutrition status of 150 children (aged 31 days to 5 years) admitted to the PICU with the use of SGNA and commonly used objective anthropometric and laboratory measurements. Each child was administered the SGNA by a dietitian while anthropometric measurements were performed by an independent assessor. To test interrater reproducibility, 76 children had SGNA performed by another dietitian. Occurrence of nutrition-associated complications was documented for 30 days after admission. RESULTS SGNA ratings of well nourished, moderately malnourished, or severely malnourished demonstrated moderate to strong correlation with several standard anthropometric measurements (P < .05). The laboratory markers did not demonstrate any correlation with SGNA. Interrater agreement showed moderate reliability (κ = 0.671). Length of stay, pediatric logistic organ dysfunction, and Pediatric Risk of Mortality III were not significantly different across the groups and did not correlate with SGNA.

Colon High-Resolution Manometry: Using Pressure Topography Plots to Evaluate Pediatric Colon Motility

Objectives: Colon manometry is usually performed using the 8-pressure sensor water-perfused manometry system. High-resolution manometry (HRM), using closely spaced solid-state pressure recording sensors, provides more detailed information of gut luminal pressure changes, and, by displaying the HRM data as a pressure topography plot (PTP), helps with data interpretation. Our aim was to compare the colon and rectal luminal pressure data obtained using 8 pressure sensors and displayed as conventional line plot (CLP) with data obtained using a custom-made solid state manometry catheter with 36 pressure recording sensors and displayed as PTP. Methods: We evaluated colon manometry patterns during fasting, response to meal, and bisacodyl stimulation in 10 patients with constipation and stool expulsion disorders. The data from 8 pressure sensors were displayed as CLP and data from 36 pressure sensors as PTP. Two gastroenterologists independently interpreted these studies. We calculated variability in interpreting colon, rectal, and anal manometry data. Results: Intermode, interobserver, and intraobserver reliability were good to excellent for recognizing colon contraction patterns when data are displayed as PTP compared with when displayed as CLP, whereas the reliability for recognizing anal contractions were poor to excellent. Conclusions: Colonic and anal manometry patterns are easily recognized when HRM data are expressed as PTP. Obtaining information of colonic luminal pressure changes with rectum and anal pressure changes using HRM can help better understand the pathophysiology of pediatric constipation and stool expulsion disorders.

Challenges in meeting fellowship procedural guidelines in pediatric therapeutic endoscopy and liver biopsy

Objective: The aims of this study were to assess the opportunities for therapeutic endoscopy, liver biopsies, and percutaneous endoscopic gastrostomy (PEG) placements available to fellows during a 3-year pediatric gastroenterology fellowship, and to evaluate access to ancillary procedural-training opportunities. Methods: Data were collected from 12 pediatric gastroenterology fellowship programs in the United States. Procedures completed in the years 2009–2011 were queried using CPT codes and endoscopy databases. The maximal opportunity for procedures was based on the total procedures performed by the institution in 3 years divided by the total number of fellows in the program. The centers completed a questionnaire regarding ancillary opportunities for endoscopic training. Results: There is significant variability in pediatric endoscopic training opportunities in specialized gastrointestinal (GI) procedures. Under the 1999 guidelines, no centers were able to meet the thresholds for polypectomy and control of nonvariceal bleeding. The 2013 guidelines allowed the number of programs reaching polypectomy thresholds to increase by 67% but made no difference for control of bleeding despite a decrease in the threshold. Training in PEG placement was not available in 42% of the surveyed centers. Elective ancillary procedural training is offered by 92% of the surveyed centers. Conclusions: Most training programs do not have the volume of therapeutic endoscopy procedures for all of the fellows to meet the training guidelines. Training in therapeutic endoscopy, PEG placement, and liver biopsy in pediatric GI fellowships should be supplemented using all of the possible options including rotations with adult GI providers and hands-on endoscopy courses. A shift toward evaluating competency via quality measures may be more appropriate.

Calprotectin in Cystic Fibrosis

BackgroundThere is increasing evidence that intestinal inflammation plays a major role in gastrointestinal symptoms in cystic fibrosis (CF). Fecal calprotectin is a marker that is elevated in several gastrointestinal inflammatory diseases, but little is known about its value in CF. We aimed to look for associations of elevated fecal calprotectin among CF patients and whether its level correlates with the clinical manifestations of CF.MethodsA single stool specimen was collected from 62 patients with CF. Fecal calprotectin was measured using the commercially available ELISA kits (PhiCal™ test). Clinical data were collected from patients’ records and CF registry.ResultsThere were no significant differences between CF patients with normal and abnormal fecal calprotectin levels. However, patients who were not receiving inhaled antibiotics had higher fecal calprotectin levels than those who were.ConclusionElevated fecal calprotectin may not accurately predict intestinal inflammation in CF. However, the fact that it was elevated in both pancreatic sufficient and insufficient groups supports the concept of “cystic fibrosis enteropathy” regardless of the pancreatic status.

Rectal prolapse and cystic fibrosis.

ABSTRACT Screening for cystic fibrosis (CF) is suggested in patients with rectal prolapse (RP). Little is known about the association between CF and RP in the era of newborn screening for CF. Our retrospective review showed that 3.6% of patients with RP had CF, and 3.5% of patients with CF had RP. No demographic or clinical factors appear to predict the likelihood of RP in patients with CF. Sweat chloride testing for patients with RP has a low yield in the era of newborn screening but may still need to be considered in children with RP to avoid missing the rare child with CF.

Do oro-anal transit markers predict which children would benefit from colonic manometry studies?

Objectives: The aim of the study was to compare oro-anal transit time (OTT) measured by radio-opaque markers with colon motility (CM) findings in children with chronic constipation and to assess clinical outcomes in children with chronic constipation evaluated by OTT and CM studies. Methods: Twenty-four children with chronic constipation (12 girls; median age 12 years [3–18 years]; median symptoms 87 months [6–186 months]) who underwent OTT and CM studies were reviewed. The OTT was determined using commercially available Sitzmarks. Patients were studied for a median of 23 months (4–60 months) and outcomes reviewed. Results: All 5 children with normal OTT had normal CM; however, only 47% (9/19) of children with slow OTT had an abnormal CM. The abnormal CM findings were total colonic pseudo-obstruction in 3 and left colonic pseudo-obstruction in 6 children. Of the 9 children with abnormal CM, 5 were managed surgically, 1 with medicine escalation, and 3 were lost to follow-up; all 6 children with known follow-up have more bowel movements and less soiling. Of the 15 children with normal CM, 10 were managed with medication escalation, 3 with behavioral intervention, and 2 surgically. Of these 15 children, 8 improved, 1 did not change, 2 worsened, and 4 were lost to follow-up. Conclusions: OTT studies may be helpful to predict which children should be referred for CM studies. Normal OTT studies may predict normal colon manometry; however, abnormal OTT studies may not predict abnormalities in colonic manometry in children with chronic constipation. Therefore, patients with slow transit marker studies should be assessed by colon manometry to evaluate colon neuromuscular integrity.

Pancreatic insufficiency in Toriello-Carey syndrome: report of a second patient.

Toriello–Carey syndrome is characterized by multiple congenital anomalies. Pancreatic insufficiency is suspected when patients present with poor weight gain, diarrhea, or maldigestion. The diagnosis is confirmed by low stool elastase and pancreatic stimulation testing. To our knowledge, only one patient with Toriello–Carey syndrome has been reported to have pancreatic insufficiency. We report on a second patient with Toriello–Carey syndrome and pancreatic insufficiency, and describe the management of pancreatic insufficiency in patients with this syndrome.

Chronic Intestinal Pseudo-obstruction

Chronic intestinal pseudo-obstruction (CIP) is defined by either continuous or intermittent symptoms of bowel obstruction in the absence of fixed lumen excluding lesion. CIP includes a heterogeneous group of disorders which result either from diseases affecting the enteric neurons and smooth muscle lining or those involving the autonomic innervation of the bowel. Symptoms associated with CIP are nonspecific, which can sometimes contribute to the delay in recognizing the condition and making the correct diagnosis. The diagnostic workup should include imaging and manometry studies and, occasionally, full-thickness bowel biopsies for histopathological examination may be required. Multidisciplinary team approach for the management of these patients is recommended, and the team members should include a gastroenterologist, surgeon, chronic pain specialist, clinical nutritionist, and a psychologist. The treatment goals should include optimizing the nutritional status and preventing or delaying the development of intestinal failure. The majority of the patients require enteral or parenteral nutrition support, and chronic pain is a common and distressing symptom. Small bowel transplantation may be required if patients develop liver complications due to parenteral nutrition, have difficult central line access, or have poor quality of life and worsening pain despite aggressive medical management.

Irritable Bowel Syndrome and Functional GI Disorders in Inflammatory Bowel Disease

Irritable bowel syndrome (IBS) is characterized by abdominal pain and altered bowel habits in the absence of an identifiable structural abnormality. Abdominal bloating, loose stools, urgency associated with bowel movements, and feelings of incomplete evacuation are common symptoms. Since there are no diagnostic laboratory tests to confirm the diagnosis, IBS is usually a clinical diagnosis, and symptom-based diagnostic criteria have been proposed. Unlike adults, there are few published studies in pediatric age group, especially in young children, validating these criteria. It is generally thought that IBS symptoms result from the convergence of multiple factors including a genetic predisposition, an infectious or inflammatory injury to the gastrointestinal (GI) tract leading to altered sensory perception by the brain, and an underlying bowel dysmotility. Children with inflammatory bowel disease are prone to developing visceral hypersensitivity due to chronic GI tract inflammation. Abdominal pain and loose stools in the absence of detectable and clinically significant gut mucosal inflammation should prompt one to consider this diagnosis in patients with IBD. Judicious use of endoscopic examination in addition to laboratory test to screen for bowel mucosal inflammation can be helpful in differentiating symptoms due to relapse of IBD for visceral hypersensitivity and IBS. Symptom relief is the primary goal of management in IBS; targeting the most distressing symptom is recommended. Current treatment includes diet modification, antibiotics for small bowel bacterial overgrowth and probiotics, cognitive behavioral therapy (CBT) to empower the patient to cope with chronic illness and learn to control their symptoms, and drugs for neuropathic pain.