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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Will This Change ALTE-GERD Practice?

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This blog has highlighted several publications which have shown the lack of benefit and potential harm of pharmaceutical agents for gastroesophageal reflux “disease” (GERD) in infancy (see links below). However, in current practice, proton pump inhibitors and histamine receptor antagonists are used frequently.  Now, another influential study (J Pediatr 2014; 165: 250-5) has shown the lack of GERD as a causal mechanism in acute life-threatening events (ALTEs) and demonstrated other pathophysiologic mechanisms. However, changing physicians’ practice in this regard may prove to be as difficult as avoiding overprescribing antibiotics, or convincing reluctant parents to vaccinate their children.

So what did this study show?

This study of 20 infants (10 with proven ALTE and 10 healthy controls) had pharyngoesophageal manometry.  Key findings:

  • Infants with ALTE (vs controls) had delays in restoring aerodigestive normalcy (P=.03).  This was indicated by more frequent and prolonged spontaneous respiratory events (SREs)
  • Infants with ALTE had a lower magnitude of protective upper esophageal sphincter contractile reflexes (P=.01)
  • Infants with ALTE had swallowing as the most frequent esophageal event associated with SREs (84%), a higher proportion of failed esophageal propagation (10% vs 0%, P=.02), an more frequent mixed apnea mechanisms (P=<.01) along with gasping breaths (P=.04)

The associated editorial (pg 225-26) explains some of the limitations of the study, including the fact that the patients had a mean gestational age of 28 weeks.

The authors conclusion: “In infants with ALTE, prolonged SREs are associated with ineffective esophageal motility ,,,suggestive of dysfunctional regulation of swallow-respiratory junction interactions.  Hence, treatment should not target gastroesophageal reflux, but rather the proximal aerodigestive tract.”

Take-home message: (from the editorial): “Far too many low birth weight (and term) infants are being unnecessarily treated with a variety of antireflux medications that have serious side effects and few, if any, demonstrable benefits.”

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Enteral Fish Oil and Intestinal Adaptation in Premature Infants

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A provocative article (J Pediatr 2014; 165: 274-9) examines supplementation of enteral fat/fish oil in premature infants as a mechanism to reduce parenteral nutrition associated cholestasis (PNAC).  While the study’s limitations will prevent any dramatic conclusions, the article and associated editorial (pgs 226-27) do make several useful points.

Before discussing the limitations, the design of the study:

Infants were block randomized (block size of 8) into either a control group or treatment group.  While both groups received conventional PN, the treatment group received supplemental enteral fat as microlipid and fish oil after tolerating enteral feeds at 20 mL/kg/d.  Microlipid was started at 1 g/kg/d and advanced up to 2.5 g/kg/d; coinciding with microlipid increases, parenteral intralipid was decreased.  Fish oil was started at 0.2 g every 12 hours and was advanced to a maximum of 0.5 g every 6 hours.  The two fish oil products were Major Fish Oil 500 (Major Pharmaceuticals) and Rugby Sea Omega 50 (Rugby Laboratories).

The limitations include the following:

  • Small cohort of 18 patients in each arm
  • Due to the smell of fish oil, the study could not truly be blinded which introduces potential bias
  • Only 7 of the 36 patients could be considered to have short bowel syndrome as most of the infants had small amounts of intestine resected
  • Advancement of enteral feedings were halted if stoma output reached 20 mL/kg/d.  The editorialists note that 40 mL/kg/d would be more typical.  Thus, in both the treatment group and the control group, there was significant opportunity to reduce PN by more aggressive enteral nutrition advancement.

With these limitations in mind, there authors were able to show that supplemental fat (with fish oil) was associated with less parenteral intravenous lipid, and reduced conjugated bilirubin prior to anastomosis.  However, there was no significant difference in PN duration.  Growth parameters were similar prior to anastomosis, but improved in the treatment group after anastomosis.

In the editorial, it is noted that “enteral feeding with a high-fat diet has been demonstrated to enhance structural features of resection-associated adaptation, the underlying mechanisms for this phenomenon are still presently unknown.”

Take-home message: Enteral fat/fish oil supplementation helped decrease parenteral intravenous lipids in this study.  More broadly, advancing enteral nutrition by accepting higher ostomy outputs is likely the best strategy to avoid PNAC and other PN-associated complications.

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