A new document from NASPGHAN highlights the potential for enteric formulas (oral and nasogastric) as an alternative 1st line therapy for Crohn’s disease remission. The following is a link with specific case examples along with background information and practical advice:
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A lot has changed in the field of pediatric gastroenterology since I completed my training 16 years ago. One technology that is used frequently now is the gastrojejunal (GJ) tube for feeding neurologically-impaired children. Previously, GJ tubes were used as a temporary solution. Part of the rational for short-term usage was that these tubes were often difficult to maintain; they could easily become dislodged or clogged.
A recent study documents the more frequent usage of GJ tubes and their indefinite usage to treat complex feeding issues (JPGN 2013; 56: 523-27).
This retrospective chart review encompassed a 10 year period (1999-2009) at a single academic center. In total 33 patients were treated with GJ tubes with 160 placements. The mean age at initial placement was 6 years and the mean weight 19.4 kg. 76% of the patients had cerebral palsy/neurologic disorder, 21% had congenital heart disease, and 9% had chronic lung disease.
Common indications for replacement: dislodgment, obstruction, coiling into stomach, and broken tubing.
Three techniques were used:
Most procedures (85%) did not require sedation.
Outcomes:
When reading the study, it is hard to ignore Figure 3 which shows more than 30 placements per year after 2007 whereas the number was about two per year before 2001. In the discussion, the authors do not focus on how this technology has been embraced so widely. It is mostly a discussion on the indications, methods, and complications. Indications included high aspiration risk, intractable vomiting, failed Nissen fundoplication, and gastroparesis. “Our study showed that long-term jejunal feeding via GJ tubes is possible and safe.”
My preference is generally to avoid GJ feedings as a primary intervention for long-term feeding problems. That is, when patients need gastrostomy tube feeds but are prone to vomiting, most often a fundoplication is worthwhile. When a patient has had a fundoplication that is no longer effective, a GJ tube should be considered.
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While gastroenterology focuses more on being skinny than being short, understanding the current treatment approach to short stature is important. Certainly, this is a common condition in a pediatric gastroenterology practice. An update is available: NEJM 2013; 368: 1220-8.
This article presents the case of an 11-year-old with probable constitutional delay of growth and puberty (CDGP) who is projected to achieve an adult height of 5 ft 5 in. It asks the question of the optimal management but does not answer this definitively.
Background: Most children with short stature have either CDGP or familial short stature and are generally healthy. The FDA has approved human growth hormone therapy for children with idiopathic short stature and height below the 1st percentile. In the U.S. this amounts to more that 500,000 children (1% of children 4-13 years). However, growth hormone is expensive $10,000-60,000 per year and achieves modest results, 1.2-2.8 inches in added final stature.
Key Recommendations:
Are there risks to growth hormone therapy?
Yes. There have been occurrences of intracranial hypertension, glucose intolerance, and slipped capital femoral epiphysis. The absolute risks are considered low. Long-term risks are not entirely clear. One study suggested a slight increased mortality rate with increased mortality from bone cancer and circulatory disorders. A similar study did not confirm these findings.
What are the costs?
For idiopathic short stature, the cost has been estimated to be $35,000-50,000 per inch of height.
Who benefits the most?
Predictors of growth hormone response in idiopathic short stature include younger age at baseline, delay in skeletal maturation, and taller parents. Also, daily administration achieves superior results to less frequent administration.
Related reference:
More data indicate that copper levels in infants receiving parenteral nutrition are usually not affected by cholestasis (JPEN 2013; 37: 92-96).
A retrospective study reviewed all patients younger than 1 year who had copper levels measured between 1999-2009 at Riley Hospital for Children. Inclusion criteria: parenteral nutrition for at least 50% of caloric needs and cholestasis (direct bilirubin >2 mg/dL).
Key findings:
Bottomline:
Measure copper values periodically in patients requiring parenteral nutrition. Most patients, even cholestatic patients, will require standard dosing but some will need less and some more.
Additional References:
Maybe. According to a recent study (Pediatrics 2013; 131: 14-20), salt intake is associated with consumption of sugar-sweetened beverages (SSB); hence, it might make you fat.
This cross-sectional study used data (4283 participants, ages 2-16 years) from the 2007 Australian National Children’s Nutrition and Physical Activity Survey. Calculation of dietary intake (salt, fluid, sugary beverages) was determined by looking at two 24-hour dietary recalls.
Each gram of salt was associated with a 46 gram intake of fluid. Of those who took SSB (n=2571), salt intake was associated with increased consumption of SSB; each gram of salt was associated with a 17 gram increased intake of SSB. Participants with SSB intake of more than 1 serving (≥250 g), in turn, were 26% more likely to be overweight/obese (odds ratio 1.26).
Study limitations included the following:
Conclusion: Besides lowering blood pressure and lowering the risk of kidney stones, reducing salt intake may help with obesity prevention.
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This is the question that I wonder after reading a recent review (NEJM 2013; 368: 149-60) -especially since effective treatment is readily available.
While vitamin B12 deficiency is most common in individuals 70 to 80 years, it affects all age groups. A particularly vulnerable group are infants of mothers with vitamin B12 deficiency. These infants may be born with deficiency or it may develop if exclusively breast-fed, usually between 4 and 6 months of age. Indications of this deficiency include failure of brain development, poor growth, hypotonia, and feeding difficulties. Some infants develop tremors, lethargy, and hyperirritability. Imaging may show atrophy and delayed myelination.
Mothers who are at most risk:
Other pediatric conditions that cause B12 deficiency: ileal resections, Imerslund-Grasbeck syndrome (Imerslund–Gräsbeck syndrome (selective vitamin B12 malabsorption ..), inflammatory bowel disease, and pernicious anemia.
Other Key Points from this review:
Additional references:
Clinical Sx: FTT, weakness, anorexia, neuro/psych sx, macrocytic anemia, pancytopenia, glossitis, vomit/diarrhea
Dx: low vit B12, incr methylmalonic acid & incr homocysteine. MMA specific for B12; homocysteine incr also if folate deficient.
If Vit B12 deficient, reason for this needs to be determined.
It has been one year and 300 posts since I’ve started this blog. I’ve enjoyed putting together my thoughts about recent articles. In addition, I like being able to search for previous blogs for information that I find helpful.
Currently there are close to ~35 hits per day in addition to the email/wordpress followers (about 30). This number has been increasing and it is fascinating to see that people from all over the world will sometimes stumble across this blog.
If you have suggestions for topics, articles or other ways to improve this pediatric GI blog, please send me an email at jjhochman@gmail.com.
Postgraduate course syllabus: naspghn.informz.net/
So far I’ve looked at the first few talks from the postgraduate course syllabus (see link above), including CVS slides by B Li. Several good pointers are given. For example, for abortive therapy, he recommends use of Zofran 0.3 mg/kg/dose. Page 17 of course book details preventative measures. Also, with regard to amitriptyline, he recommends starting at 0.3 mg/kg and titrating as needed up to 1-1.5 mg/kg/day. Mitochondrial-type support often helpful as well (eg. CoQ10 10 mg/kg/day divided BID).
At the end of this lecture are a couple of questions –see how you do:
3. NASPGHAN recommended evaluation of a child with episodic vomiting:
Answers: 1. d;2.e;3.b;4.a;5.c
Foreign body talk: if object >2cm or longer than 5 cm, may have difficulty passing.
NASPGHAN POSTGRADUATE COURSE Table of Contents
MODULE A: WHAT GOES IN, MUST COME OUT: CLINICAL GASTROINTESTINAL ISSUES
FROM PROPRANOLOL TO INDUCING COMA: CARING FOR A CHILD WITH INTRACTABLE CYCLIC VOMITING SYNDROME (CVS)………………………13 INCONTINENCE WITHOUT FECAL IMPACTION …………………………….23 ELIMINATION DIETS: RISKS AND BENEFITS……………………………………..37
MODULE B: LIVER BEYOND VIRUS, METABOLIC, STORAGE, TUMORS
METABOLIC LIVER DISEASE: WORKING THROUGH THE MAZE ……………….51 UPDATE ON ALPHA‐1‐ANTITRYPSIN DEFICIENCY ……………………………61 THERE IS A LIVER MASS ON THE ULTRASOUND: WHERE DO YOU GO FROM HERE? ….75
MODULE C: THE INFLAMED INTESTINE
GI INFLAMMATION, IMMUNE FUNCTION AND IBD ………………………………87 MY STOMACH IS BUGGING ME!: THE MICROBIOME IN IRRITABLE BOWEL SYNDROME ……………………………………………………………………………………………101 THE SORE BOTTOM: PERIANAL INFLAMMATORY BOWEL DISEASE ……111 RESCUE ME FROM MY IBD: UPDATES ON INFLAMMATORY BOWEL DISEASE THERAPY ………………………………………………………………………………………………125
MODULE D: IMAGING AND ACCESSING THE TUBES
LOOKING DEEPLY INTO THE NOT SO SMALL INTESTINE ……………………………..137 PUTTING TUBES WITHIN TUBES: ENTERAL THERAPEUTIC ACCESS ………….151 IMAGING THE PANCREATO‐BILIARY TREE …………………………………….169 UPDATE ON CRITICAL FOREIGN BODY INGESTIONS ………………………………….189
MODULE E: WHEN ALL ELSE FAILS: LIVER, INTESTINE AND POUCH
THE KID IS ON THE LIST: KEEPING COMPLICATIONS AT BAY FOR THE
NON‐TRANSPLANT HEPATOLOGIST ……………………………………………………201 TRICKS OF THE TRADE FOR INTESTINAL FAILURE ……………………………….213 GASTROINTESTINAL AND LIVER COMPLICATIONS OF BONE MARROW TRANSPLANT …………………………225 POUCH DYSFUNCTION AND SURVEILLANCE: WHAT ARE MY OPTIONS? .235
A nationwide survey of 983 patients with Rett syndrome identifies a high prevalence of GI and nutritional problems (Motil KJ et al. JPGN 2012; 55: 292-98).
Parents from 983 female patients with Rett syndrome responded to the study questionnaire; this was a 59% response rate from the 1666 families in the North American Rett Database. Patients included those who fulfilled clinical criteria for diagnosis or who had MECP2 gene (methyl-CpG-binding-protein).
Prevalence of GI problems were listed in article’s Table 2 and included the following:
Z-scores for height-for-age, weight-for-age, and BMI are presented for ages 0-40 (see Table 1) -these are all significantly lower than age-matched healthy children.
Important findings:
Addtional references:
In a pediatric Boston cohort of 579 patients presenting for celiac evaluation but no previous diagnosis, 7.4% had previous gluten avoidance (J Pediatr 2012; 161: 471-5). In this cohort, the mean age at presentation was 8.7 years.
Independent predictors of gluten avoidance and the odds ratio (OR) included the following:
From my perspective, many families and sometimes referring physicians are convinced that their child has celiac disease before subspecialty evaluation. While a gluten-free diet may reduce some gastrointestinal symptoms even in the absence of celiac disease, it is probably helpful for families to complete diagnostic testing and to obtain dietary counseling prior to implementing a gluten-free diet.
Previous related blog entries:
gutsandgrowth 