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About gutsandgrowth

I am a pediatric gastroenterologist at GI Care for Kids (previously called CCDHC) in Atlanta, Georgia. The goal of my blog is to share some of my reading in my field more broadly. In addition, I wanted to provide my voice to a wide range of topics that often have inaccurate or incomplete information. Before starting this blog in 2011, I would tear out articles from journals and/or keep notes in a palm pilot. This blog helps provide an updated source of information that is easy to access and search, along with links to useful multimedia sources. I was born and raised in Chattanooga. After graduating from the University of Virginia, I attended Baylor College of Medicine. I completed residency and fellowship training at the University of Cincinnati at the Children’s Hospital Medical Center. I received funding from the National Institutes of Health for molecular biology research of the gastrointestinal tract. During my fellowship, I had the opportunity to work with some of the most amazing pediatric gastroenterologists and mentors. Some of these individuals included Mitchell Cohen, William Balistreri, James Heubi, Jorge Bezerra, Colin Rudolph, John Bucuvalas, and Michael Farrell. I am grateful for their teaching and their friendship. During my training with their help, I received a nationwide award for the best research by a GI fellow. I have authored numerous publications/presentations including original research, case reports, review articles, and textbook chapters on various pediatric gastrointestinal problems. In addition, I have been recognized by Atlanta Magazine as a "Top Doctor" in my field multiple times. Currently, I am the vice chair of the section of nutrition for the Georgia Chapter of the American Academy of Pediatrics. In addition, I am an adjunct Associate Clinical Professor of Pediatrics at Emory University School of Medicine. Other society memberships have included the North American Society for Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN), American Academy of Pediatrics, the Food Allergy Network, the American Gastroenterology Association, the American Association for the Study of Liver Diseases, and the Crohn’s and Colitis Foundation. As part of a national pediatric GI organization called NASPGHAN (and its affiliated website GIKids), I have helped develop educational materials on a wide-range of gastrointestinal and liver diseases which are used across the country. Also, I have been an invited speaker for national campaigns to improve the evaluation and treatment of gastroesophageal reflux disease, celiac disease, eosinophilic esophagitis, hepatitis C, and inflammatory bowel disease (IBD). Some information on these topics has been posted at my work website, www.gicareforkids.com, which has links to multiple other useful resources. I am fortunate to work at GI Care For Kids. Our group has 17 terrific physicians with a wide range of subspecialization, including liver diseases, feeding disorders, eosinophilic diseases, inflammatory bowel disease, cystic fibrosis, DiGeorge/22q, celiac disease, and motility disorders. Many of our physicians are recognized nationally for their achievements. Our group of physicians have worked closely together for many years. None of the physicians in our group have ever left to join other groups. I have also worked with the same nurse (Bernadette) since I moved to Atlanta in 1997. For many families, more practical matters about our office include the following: – 14 office/satellite locations – physicians who speak Spanish – cutting edge research – on-site nutritionists – on-site psychology support for abdominal pain and feeding disorders – participation in ImproveCareNow to better the outcomes for children with inflammatory bowel disease – office endoscopy suite (lower costs and easier scheduling) – office infusion center (lower costs and easier for families) – easy access to nursing advice (each physician has at least one nurse) I am married and have two sons (both adults). I like to read, walk/hike, bike, swim, and play tennis with my free time. I do not have any financial relationships with pharmaceutical companies or other financial relationships to disclose. I have helped enroll patients in industry-sponsored research studies.

For Pediatric GI MDs: Imaging and Anecdote in Cockayne Syndrome

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A brief article (J Pediatr 2014; 165: 416) discusses “susceptibility-weighted imaging” (SWI) for calcification in Cockayne syndrome.

SWI is a gradient-echo MRI sequence with a high sensitivity for blood, blood products, nonheme iron, and calcifications.

The reason why I highlighted this reference relates to a personal experience.  Several years ago I had a patient (about 8 years old at the time) who had mild GI symptoms along with very poor growth.  He had some mild neurologic features but extensive testing by several neurologists and a few well-qualified geneticists did not yield an answer.  Due to my concerns about his poor growth, I convinced the family to have their son admitted to our hospital before considering another trip out-of-town for further testing. As part of his evaluation, I reordered an MRI of his brain.  One of our radiologists (who is brilliant) called me up asking for clinical information and stated specifically that she was concerned about Cockayne syndrome.  After she mentioned the diagnosis, I questioned the family regarding some more specific features of Cockayne (e.g. photosensitivity).  Subsequently, genetic testing proved this child had Cockayne syndrome.

Cockayne syndrome is a rare autosomal recessive disorder that belongs to the family of damaged DNA repair disorders.  Besides photosensitivity and cachectic dwarfism, other features include neurosensory hearing loss, and progressive pigmentary retinopathy.  The physical features are quite characteristic –if you have seen a previous case!  The disease is rare enough that many experienced geneticists may not have seen a case.

Take-home message from this article: Careful MRI study of the brain, potentially with SWI, can help pinpoint the diagnosis of Cockayne syndrome.

On an unrelated matter, I wanted to thank Janet R for her note and let her know that she will be missed.

How Accurate is Serology at Predicting Mucosal Healing in Pediatric Celiac Disease?

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A recent study (Am J Gastroenterol doi: 10.1038/ajg.2014.200) shows that in children, unlike adults, that normalization of celiac disease (CD) serology correlates well with mucosal healing.

In this study, 150 children with biopsy-proven CD were prospectively evaluated with duodenal biopsies, tissue transglutaminase (tTG) IgA serology, and deamidated gliadin peptide (DPG) IgG serology.  After maintaining a gluten-free diet (GFD) for at least 1 year, participants underwent followup evaluation.

  • Of the 97 with normalization of their serology, 91 had normal biopsies (Marsh 0) and 6 had slight abnormalities (Marsh 1).
  • Of the 27 with positive serology, only 6 had Marsh 3 changes.
  • Overall, 124 (82.7%) had normalization of duodenal mucosal biopsies irregardless of serology.

Higher rates of mucosal healing are possible with longer duration of GFD.

On the other hand, a recent retrospective study (JPGN 2014; 59: 229-31) notes that among 40 children who underwent followup endoscopic evaluation (>4 months after GFD, median 24 months), most of whom were symptomatic, only 25 had complete healing. Though among those with adherence to a GFD, only five had persistent villous atrophy (Marsh 3 lesion) . Serological correlation was not provided.

Take-home message: Followup biopsies are not needed in children with normalization of their serology (tTG IgA and DGP IgG).  That is, serology correlates well with mucosal healing in children with celiac disease on a GFD >12 months. However, it is certainly reasonable to consider followup endoscopy in those who are symptomatic, especially if serology is abnormal.
In a related matter, the new requirements regarding gluten-free labeling have been implemented -here’s a link from the LA Times: Celiac Regulations or First federal gluten–free regulation takes effect

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Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

Nutrient Deficiencies with Celiac Disease

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A recent study (JPGN 2014; 59: 225-28) examined fat-soluble vitamin deficiencies in pediatric patients with newly diagonosed Celiac disease (CD).

Of the 83 patients analyzed between 1995-2012 at the Mayo clinic, the key findings:

  • No patients had vitamin A deficiency
  • Two patients had vitamin E deficiency.  Both of these patients had complete villous atrophy along with a malabsorptive presentation.
  • Nine patients had mild-to-moderate vitamin D deficiency (less than the reported frequency in the general pediatric population)
  • All of these vitamin deficiencies corrected with gluten-free diet and vitamin supplements.

A limitation of the study was a selection bias as not all children underwent vitamin level measurements.

Take-home message (from authors): “fat-soluble deficiencies are uncommon in children with a new diagnosis of CD.  Routine measuring of fat-soluble vitamin levels may not be necessary.”

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One More Way to Prevent CVL Infections

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While a recent study (JPGN 2014; 59: 177-81) discusses the results of several strategies for limiting CVL infections, I found one approach in particular of interest.

This single center study (2009-2013, n=48 children) from Birmingham, UK examined a multidisciplinary enhanced care pathway regarding CVL care.  Implementation of this pathway lowered the risk of all-cause line infections from 1.98 per 1000 parenteral nutrition days to 0.45.  The pathway included training care providers, careful discharge planning, having those with skin conditions see dermatologists, and monitoring compliance.

One important observation was that methicillin-sensitive Staphylococcus aureus (MSSA) infections were often preceded by local signs of infection for a short period prior to systemic infection.  “We devised a pathway for exit site infections in which a swab is taken and empiric topical mupirocin commenced immediately. A decision on any further management is made after 24 to 48 hours.”

Take-home message: Implementing a CVL care pathway lowers CVL infections.  Implementing topical therapy at the first signs of a localized infection can be an important part of this effort.

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Magnetic Foreign Bodies –Still a Problem

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After reading a recent article on swallowed magnetic foreign bodies (J Pediatr 2014; 165: 332-35), I was thinking quite tangentially about an article from The Onion a few years ago: Falling Down Laundry Chute And Breaking Neck Remains America .‘s No. 548,221 Killer.  In this Onion article, the author sarcastically bemoans the attitude that many people adopt that “this could never happen to me.”

What this Journal of Pediatrics study adds to the literature is that swallowed magnetic foreign bodies remain a significant health threat and are likely to remain that way despite recent recalls.

Key finding: 94 pediatric patients were identified at this single center as having infested magnetic foreign bodies between 2002-2012, with increasing incidence in the last three years of the study period.  The median age was 4.5 years. Six patients required surgical removal.

Bottomline: Magnet ingestions are a much more serious health threat than falling down a laundry chute.

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HCV Guidelines Updated

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For those trying to stay up-to-date, a headline from The Onion: “Nation’s Third-Graders Now Eating At A Ninth-Grade Level”   .

And, from American Association for the Study of Liver Diseases (AASLD):

AASLD and the Infectious Diseases Society of America (IDSA), in collaboration with the International Antiviral Society-USA (IAS-USA), released the latest section of www.hcvguidelines.org.

The new section, “When and in Whom to Initiate HCV Therapy,” offers clinicians information on:

  • How to prioritize patients who will derive the most benefit, or will have the greatest impact on limiting further HCV transmission.
  • When to treat patients with complications such as advanced fibrosis, compensated cirrhosis, liver transplant, or severe extra-hepatic complications.
  • Additional conditions that warrant prioritization of treatment.

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Bionic Pancreas -It Works!

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From 1974-1978, I watched “The Six Million Dollar Man.”  The premise of the show was that astronaut Steve Austin played by Lee Majors was rebuilt with bionic parts after a severe crash.  He worked for the government to stop the bad guys.

So, when I read about a “bionic” pancreas in a recent publication (NEJM 2014; 371: 313-25), I was definitely interested.  In this study, the bionic pancreas was a “fully automated, bihormonal” (administered both insulin and glucagon) device.  It consisted of an iPhone 4S and a G4 Platinum continuous glucose monitor which were connected by a custom hardware interface.  This study was feasible due to the availability of accurate continuous glucose monitoring allowing the development of a device to more precisely regulate glycemic control.

In this study, both adults (n=20) and adolescents (n=32) with type 1 diabetes were followed closely with either the bionic pancreas or a insulin pump.

Key finding: “as compared with an insulin pump, a wearable, automated, bihormonal, bionic pancreas improved mean glycemic levels.”

Bottomline: A bionic pancreas has the potential to be a significant upgrade from a standard insulin pump –patients with type 1 diabetes may no longer have to think about how to adjust their blood glucose.  While this bionic pancreas does not reach the promise of the 1970s show of ‘we can build him better than he was before,’ it does show that bionic parts and prosthetics are improving.  For those who want a video explanation –here’s a link to 3-minute video explanation from the inventor from Boston University.

Costs versus Quality

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So I can return to our radiology department without cowering, I wanted to elaborate on this morning’s post.  My focus for this morning’s blog was on cost transparency.  What our pediatric radiologists emphasized to me is that quality is another key issue for patients.  Everyday at our institution there are numerous CT scans, MRIs and xrays that are reviewed from outside institutions.  Many of these studies were performed poorly and may not be interpretable = “total crap.”  And, many of these studies were unnecessary to begin with.

A couple pointers:

  • Open MRIs usually produce lousy images.  They are mainly suitable for individuals too large to fit in conventional MRIs.
  • MRI quality is dependent on high strength magnets.  A minimal of 1.5 Tesla magnet, but preferably a 3 Tesla magnet, will produce better images.
  • It is not just the radiologist that matters.  If radiology techs are not certified/accomplished, then imaging results suffer.  Techs may not focus CT scanners properly and produce a much wider field of radiation then is necessary.
  • CT scans need to minimize radiation while producing good images.  Most children’s hospitals have adopted protocols to assure that this takes place.

Radiological tests are often a ‘black box’ for families –they have no idea what is really going on.  A CT scan/MRI at low quality place may be akin to a cold hamburger at a fast food restaurant whereas a study at a high quality institution may be more similar to the best steak in town.  In addition, the quality of the images can truly be life altering.  This is likely more of an issue in pediatrics than in adult medicine.

Bottomline: In our quest for price transparency, we also need to make sure we are truly comparing apples to apples.  Highlighting value of a particular study and quality are equally important.

If any radiologists reading this post have more pointers, please comment.

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What Happens with Cost Transparency in Medicine?

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While it is true that some tests, like MRI and CT scans, may be performed better (better images, better contrast administration, etc) at some locations than others, many times the test is similar but the costs to the patient may be widely divergent.  Yet, for most patients the exact costs are not known until the bill arrives in the mail.  A recent study shows that many patients will consider the costs of these expensive tests if they are provided beforehand.  Here’s the NY Times link, MRI study,  and an excerpt:

A study released Monday in the journal Health Affairs suggests we are smarter than that.

The insurer WellPoint provided members who had scheduled an appointment for an elective magnetic resonance imaging test with a list of other scanners in their area that could do the test at a lower price. The alternative providers had been vetted for quality, and patients were asked if they wanted help rescheduling the test somewhere that delivered “better value.”

Fifteen percent of patients agreed to change their test to a cheaper center. “We shined a light on costs,” said Dr. Sam Nussbaum, WellPoint’s chief medical officer. “We acted as a concierge and engaged consumers giving them information about cost and quality.”

The program resulted in a $220 cost reduction (18.7 percent) per test over the course of two years, said Andrea DeVries, the director of payer and provider research at HealthCore, a subsidiary of WellPoint, which conducted the study. It compared the costs of scanning people in the WellPoint program with those of people in plans that did not offer such services.

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Why Adding Vitamin D May Not Help IBD

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Despite all of the accolades that vitamin D has received, the fact that low vitamin D is associated with worse outcomes, in a number of disease states, does not prove causality. A recent article indicates that vitamin D is likely more of a marker of disease activity than a mediator of disease activity in inflammatory bowel disease (IBD), and specifically Crohn’s disease (CD) (Inflamm Bowel Dis 2014; 20: 856-60).

Background: Binding sites for the vitamin D receptor (VDR) have been “identified in genes associated with CD, and vitamin D has been shown to enhance the production of interleukin-10 (IL-10) and induction of regulatory T-cells.”

Design:The authors prospectively collected samples of 37 CD patients; the mean age in those with active disease (n=20) was 34 years and it was 30 years in those with inactive disease. In 8 patients with active disease, vitamin D levels were measured at the time of active inflammation (day 0) and at 14 days after receiving infliximab (day 14).

  • Key finding in these 8 patients: Vitamin D (25-OH) was 23 ng/mL on day 0 and 40 ng/mL 2 weeks later.  Only 1 of these 8 patients was taking a vitamin D supplement.
  • Key finding in the entire cohort: in the active disease group mean vitamin D level was 27 ng/mL compared with 38 ng/mL in those in remission (P=0.02).

Take-home point: There is an inverse relationship between vitamin D levels and disease activity.  However, the early increases in vitamin D levels with clinical response to anti-TNF therapy suggests that a major mechanism of vitamin D deficiency is related to the burden of systemic inflammation.  Hence, repeat testing when patients are in remission may obviate the need for vitamin D supplementation in many patients.

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