Differences in Durability, Dislodgement, and Other Complications With Use of Low-Profile Nonballoon Gastrostomy Tubes in Children.

BACKGROUND: Nonballoon low-profile gastrostomy tubes (GTs) are used for enteral nutrition support in a subset of pediatric patients with feeding difficulties when use of balloon GTs is problematic. Different nonballoon low-profile tube types are available, but comparative studies are lacking. MATERIALS AND METHODS: This was a retrospective cohort study comparing complications and outcomes between different low-profile nonballoon GTs at a pediatric tertiary care center over 10 years. RESULTS: We identified 43 patients with 160 tube placement procedures, including 93 (58%) BARD tubes (type A) and 67 (42%) Mini-ONE tubes (type B). Accidental tube dislodgment occurred exclusively with type B (33% vs 0%, P < .0001) with dislodgment occurring at a median of 54 days after placement. Type A GTs were more likely to be changed due to leakage (47% vs 8%, P < .0001). Minor gastrostomy site bleeding was more likely to be seen with type A tube changes (46% vs 7%, P < .0001). Patient sedation or site dilation was rarely needed in either group. Time to tube change was longer in the type B GTs (BARD) ( P = .016) with a median tube survival in the type A and type B groups at 432 and 284 days, respectively, with a hazard ratio of 1.89 (95% confidence interval, 1.2-2.99), but once confounders were accounted for, the effect of tube type was no longer statistically significant. CONCLUSION: Our study shows that differences exist with use of various low-profile nonballoon GTs. This should be taken into consideration when counseling families about the most appropriate tube type for their children.

Difficulty Achieving Vitamin D Sufficiency With High-Dose Oral Repletion Therapy in Infants With Cholestasis.

OBJECTIVES: Oral high-dose repletion vitamin D therapy, also known as stoss therapy, can be effective in the treatment of nutritional vitamin D deficiency rickets in infants and young children without liver disease and in patients with cystic fibrosis. There is no literature about this approach in infants with new-onset cholestasis. METHODS: This was a retrospective chart review of infants with cholestasis from March 2010 to March 2012 at a pediatric tertiary care center. Four cases satisfied the inclusion criteria, and were described in detail. RESULTS: All of the patients received oral high-dose repletion therapy with ergocalciferol (vitamin D2) 300,000 IU daily for 2 to 3 days. Follow-up vitamin D levels approximately 4 weeks later showed failure to achieve sufficiency levels (>20 ng/dL) in any patient. CONCLUSIONS: Unlike infants without liver disease, use of oral high-dose repletion therapy may not be adequate as treatment of vitamin D deficiency in the setting of cholestasis.

Urologic and gastrointestinal symptoms in the dystroglycanopathies.

OBJECTIVE: To determine the frequency of urologic and gastrointestinal (GI) symptoms in a cohort of individuals with dystroglycanopathy compared with healthy household controls. METHODS: Participants in a North American dystroglycanopathy natural history study (NCT00313677) and other members of their households completed a questionnaire modified from validated instruments and clinical criteria. Urologic and GI symptom frequency, effect on patient life, and medications taken for these symptoms were assessed. Those younger than 4 years or not toilet trained were excluded. Healthy human bladder, esophagus, and duodenum from surgical specimens were immunostained for glycosylated α-dystroglycan. RESULTS: Thirty of 58 potential participants with dystroglycanopathy (51.7%) and 16 household controls participated. Subjects were aged 6 to 51 years (mean 26.7); 60.0% were female. Controls were aged 7 to 55 years (mean 34.6); 56.3% were female. The dystroglycanopathy cohort had higher frequency of urinary voiding symptoms (p = 0.02), higher urologic symptom scores (p = 0.05), and higher dysphagia symptom scores (p = 0.04). A correlation existed between urologic symptom score and effect on life (r = 0.71; 95% confidence interval 0.46, 0.85; p < 0.0001) and between dysphagia symptom score and effect on life (r = 0.72; 95% confidence interval 0.48, 0.86; p < 0.0001). Glycosylated α-dystroglycan was present in visceral smooth muscle of all normal tissues analyzed. CONCLUSIONS: Urologic symptoms and dysphagia are reported more frequently by individuals with dystroglycanopathies than by household controls. These symptoms can cause a perceived negative effect on patient life. Our results suggest urologic and GI dysfunction may be part of the dystroglycanopathy phenotype, and that questions about these symptoms should be incorporated into routine care because they may influence medical management.

Effects of 70% ethanol locks on rates of central line infection, thrombosis, breakage, and replacement in pediatric intestinal failure.

OBJECTIVES: Parenteral nutrition is essential for the growth and nutrition of patients with intestinal failure (IF). Catheter-related bloodstream infections (CRBSI) are a major complication of parenteral nutrition use. Few retrospective studies have shown that 70% ethanol lock (ETL) therapy for central lines can reduce the infection rate. Studies assessing line breakage, thrombosis, and replacement with the use of ETL are lacking in the pediatric literature. METHODS: The present study is a retrospective chart review, with a primary outcome of CRBSI rate per 1000 catheter days, and secondary outcomes of line thrombosis, line breakage requiring repair, and line replacement rates with use of ETL compared with heparin locks. Rates were compared using the Wilcoxon signed-rank test for paired nonparametric data. RESULTS: Seven patients satisfied the inclusion criteria that included having a single-lumen silicone central line exposed to both heparin and ETL therapy during the study period. There was a statistically significant decrease in overall and Gram-negative CRBSI rates per 1000 catheter days with a use of ETL therapy (10.3-1.4 per 1000 catheter days for overall CRBSIs, P = 0.02; 6.7-0 per 1000 catheter days for Gram-negative CRBSI, P = 0.03). There was an increasing trend in line thrombosis and repair rates with ETL therapy. Logistic regression analysis evaluating the impact of line luminal size on line repair rate showed a reduced risk of line repair when using larger-diameter central lines. CONCLUSIONS: ETL therapy is an effective method for decreasing CRBSI in patients with IF; however, it may have a negative impact on line integrity. Patients should be carefully selected when deciding on ETL therapy use for central line care. Studies are needed on the effect of different ethanol concentrations on infection rate and line integrity to optimize the outcome in this high-risk population.

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.

Gastrojejunal feeding tube use by gastroenterologists in a pediatric academic center.

BACKGROUND AND OBJECTIVES: Enteral feeding through gastrojejunal (GJ) tubes is an established method of nutrition for patients with feeding difficulty who do not tolerate intragastric feedings. The pediatric literature about the long-term outcome, safety, and complications of different GJ tubes and placement methods is lacking. Our study aims to provide information about indications, techniques, and long-term outcome of GJ tube use in children. METHODS: Retrospective chart review for GJ tube placement procedures was used at our center for 10 years (1999-2009). Data collected included demographics, placement indications, underlying diagnosis, tube type, placement methods, complications, tube survival, and patient outcome. RESULTS: Thirty-three patients using GJ tubes were identified, with a total of 160 successful procedures documented (overall success rate of 97.6%). At initial placement, the mean age was 6 years (range 0.6-21.6) and the mean weight was 19.4 kg (range 6.6-72.2). Patients had a mean of 4.9 tubes placed per patient (range 1-20) during a follow-up of 26.8 months (range 0.4-115.3). The most common indications for replacement included accidental dislodgement, tube obstruction, coiling back into the stomach, and broken tube component. At the end of the study, 39% continued using GJ tubes, 30% were transitioned back to gastrostomy or oral feeds, and 15% underwent a surgical intervention. CONCLUSIONS: Long-term GJ tube use is possible and safe in children. Various feeding tubes and placement methods can be used by pediatric gastroenterologists to provide long-term jejunal feeds in children.

Prospective randomized comparative study of low-profile balloon gastrostomy tubes in children.

BACKGROUND: Supplemental enteral nutrition through gastrostomy tubes is well established in children, but prospective studies comparing different tubes remain lacking in this population. The study aimed at comparing different aspects related to the use of low-profile balloon gastrostomy tubes in children. MATERIALS AND METHODS: The authors prospectively studied the use of 2 tube types--tube A (MIC-Key gastrostomy tube; Ballard Medical Products, Draper, UT) and tube B (MINI One gastrostomy tube; Applied Medical Technology, Brecksville, OH)--in a cohort of children in a crossover study design. Children were randomly assigned to 1 tube type for 4 months, followed by the other tube type for the next 4 months. Patients were evaluated at enrollment and at 4 and 8 months, with monitoring phone calls at 2 and 6 months. Variables measured included caregiver satisfaction, tube-related complications, and device durability. RESULTS: Twenty-one patients were included in the study. Infection rate (range, 4.8%-5.0%) and overall leakage rate (range, 42.9%-50.0%) were similar in both groups. Trends were noted with other variables measured but without statistical significance. These included lower rates of formula leakage and granulation tissue growth and higher satisfaction scores with tube B and more favorable device durability with tube A. CONCLUSION: Both low-profile balloon gastrostomy tubes performed well and had comparable caregiver satisfaction, complications, and overall device durability.

Safety techniques for percutaneous endoscopic gastrostomy tube placement in Pierre Robin Sequence.

Pierre Robin Sequence (PRS) is a craniofacial anomaly characterized by a triad of micrognathia, glossoptosis, and cleft palate. Infants with PRS frequently have feeding problems that may require supplemental nutrition through a nasogastric or gastrostomy tube. Very few published studies have illustrated the most appropriate method for securing an enteral feeding route in this patient population. One case report described a major complication leading to death from airway compromise following percutaneous endoscopic gastrostomy (PEG) tube placement. The authors describe a case of an infant with PRS who underwent successful PEG tube placement without complications, and they highlight certain techniques to improve procedure success and patient safety.

Identifying small bowel intussusception related to a gastroenteric feeding tube.

Enteral feedings through gastroenteric (GE) tubes are commonly utilised in patients with feeding difficulties and intolerance to gastric feeding. A variety of complications related to GE tubes have been described which rarely include small bowel intussusception. The case of a 23-year-old man with proximal small bowel intussusception related to an endoscopically placed low profile GE tube is described. The patient presented with abdominal pain and bilious vomiting. Symptoms resolved with tube shortening. This report includes a short review aimed at raising awareness about this complication with a discussion on diagnosis and management.

Initial clinical experience with allopurinol-thiopurine combination therapy in pediatric inflammatory bowel disease.

BACKGROUND: Thiopurines are a mainstay of immunomodulator therapy in inflammatory bowel disease (IBD). Despite their efficacy, some patients may have a poor response due to inability to achieve adequate levels of the active metabolite, 6-thioguanine (6-TGN). Others experience hepatotoxicity, which correlates with excessive 6-methylmercaptopurine (6-MMP) levels. Two adult studies have demonstrated successful manipulation of thiopurine metabolism with allopurinol, a xanthine oxidase inhibitor, to achieve more optimal thiopurine levels. The aim was to retrospectively characterize the utility of allopurinol to optimize thiopurine metabolite levels in pediatric IBD patients. METHODS: Thirteen patients received allopurinol daily (100 mg in patients >or=30 kg and 50 mg <30 kg), and their thiopurine dose was simultaneously reduced to 25%-50% of the previous maintenance dose. Metabolite levels and other screening labs were checked 2-4 weeks later. RESULTS: The mean azathioprine dose was decreased from 148.1 to 59.6 mg daily (60% of the mean original dose). The mean 6-TGN level increased from 173 to 303 pmol/8 x 10(8) red blood cell count (RBC) (P = 0.03), and the mean 6-MMP level decreased from 7888 to 2315 pmol/8 x 10(8) RBC (P < 0.001). Elevated transaminase levels improved or resolved in all patients. Two patients experienced reversible neutropenia. At the conclusion of the study 9 patients (69%) remained on combination therapy with a mean duration of follow-up of 162.8 +/- 119.2 days. CONCLUSIONS: Combination therapy successfully shunted thiopurine metabolites to a more favorable pattern. Reversible neutropenia was the most common side effect (2 patients). Long-term prospective studies are needed in this patient population.

Differential effects of Francisella tularensis lipopolysaccharide on B lymphocytes.

Francisella tularensis, a designated Category A biological agent, can cause severe infection in humans. Previous studies have demonstrated a significant immunoprotective role for B lymphocytes in animal models, but the responses of human B lymphocytes to F. tularensis components are largely unknown. The LPS of F. tularensis is atypical and has been reported to lack biological activity on myeloid cells and mouse B cells. Our study characterized the immunological effects of highly purified LPS from different stains of F. tularensis on human B lymphocytes and compared these effects with those on mouse B cells and human monocyte-derived macrophages. Results indicate that marked differences exist between cell type and species in specific responses to this interesting bacterial component. In sharp contrast to responses of mouse splenic B cells or human macrophages, human peripheral B cells showed reproducibly elevated IL-6, TNF-alpha, and antibody production in response to F. tularensis LPS. Data also indicated that these activated human B lymphocytes may subsequently promote the activation of other immune cell types by direct cell-cell interaction. Further investigation into the potential usefulness of F. tularensis LPS as an adjuvant component of a more optimal subunit vaccine is warranted, as it is now clear that it is not biologically inactive, as assumed previously.