Nutrition Guidelines for Cystic Fibrosis

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Wilschanski et al (JPGN 2016; 63: 671-5) provide a summary (“highlights”) of a full report (Turck D et al. Clin Nutr 2016; 35: 557-77) on nutritional recommendations for infant and children with cystic fibrosis.

What’s in here:

Table 1: criteria for adequate nutritional status including

  • Age <2 yrs: 50% for weight & height compared to healthy-age peers
  • Age 2-18 yrs: 50% BMI compared to healthy peers

Table 2: nutritional assessment and followup

  • Assess elastase-1 annually if pancreatic sufficient
  • Assess pancreatic enzyme supplementation
  • Annual blood tests: CBC/d, iron status, fat-soluble vitamins, LFTs.  Possibly: fatty acids
  • If older than 10 years, annual glucose tolerance
  • Dietary review every 3 months
  • Bone density assessment between 8-10 yrs and then every 1-5 yrs

Table 3: Energy requirements

  • Anticipate need for 110-200% compared with healthy peers

Table 4: Pancreatic enzyme replacement therapy (PERT)

  • 0-1 yr: 2000-4000 units lipase/120 mL of formula/breast milk & 2000 units lipase/gram of dietary fat
  • 1-4 yrs: 2000-4000 units of lipase/gram of dietary fat (max 10,000 units lipase/kg/day)
  • >4 yrs: starting dose; 500 units lipase/kg/meal -titrate up to 1000-2500 units lipase/kg/meal (max 10,000 units lipase/kg/day)

Table 5: Fat-soluble vitamin/vitamin guidelines

Table 6: Sodium supplementation

  • 0-6 months: 1-2 mmol/kg/day –give salt in small portions throughout the day, “diluted in water or fruit juice”.  In some infants, up to 4 mmol/kg/day if increased losses (eg. due to heat, gastrointestinal losses)
  • Older children: anticipate need for additional salty foods or use sodium chloride capsules, especially when excessive sweating (eg. fever, sports, hot weather)

Related blog posts:

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.

Maine Coast, near Acadia

Maine Coast, near Acadia

 

IBD Updates -January 2017

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L Beswick et al. Inflamm Bowel Dis 2016; 22: 2966-2976. The authors provide an algorithm for diagnosis and management of CMV in the setting of inflammatory bowel disease which primarily in the setting of steroid-refractory colitis.  Despite conferring a worse prognosis, the authors note that in most cases the virus is nonpathogenic and thus antiviral is usually ineffective.  Figure 1 outlines their algorithm, in those with high density inclusions on tissue (≥5 per biopsy) and/or high blood CMV PCR, the authors recommend treatment (including ganciclovir).  The details of this figure are too complex to easily summarize and if faced with this clinical scenario, I recommend reviewing source article.

KA Dunn et al.  Inflamm Bowel Dis 2016; 22: 2853-62. Fecal samples from 10 patients & 5 controls (age 10-16) showed that microbial diversity was lower in Crohn’s and lowest in patients who did not achieve SR.

EL Barnes, R Burakoff. Inflamm Bowel Dis 2016; 22: 2956-65. New biomarkers for diagnosing inflammatory bowel disease: biomarker signatures, gene expression analysis, protein profiling and microRNA

Does a Healthy Lifestyle Result in Better Outcomes?

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It’s easy to become discouraged that sensible actions may not be effective due to general pessimism and sometimes conflicting medical reports.  On the positive side of the ledger, a recent study (AV Khera et al. NEJM 2016; 375: 2349-58) provides compelling data that a combination of healthy lifestyle changes make a BIG difference.

The study focused on 4 healthy lifestyle factors: no smoking, no obesity, regular physical activity, and a healthy diet.  The study examined three large prospective cohorts with a total of more than 55,000 patients.

Key finding:

  • Among participants with high genetic risk, a favorable lifestyle was associated with a 46% lower relative risk of coronary events compared to those with an unfavorable lifestyle over the 10-year study period.

In the same issue, a review of the human intestinal microbiome (pages 2369-79) notes that “dietary intake appears to be a major short-term and long-term regulator of the structure and function of gut microbiota.  Still, only a relatively small number of randomized, clinically controlled dietary interventions targeting the gut microbiota have been reported in humans and these show that energy restriction and diets rich in fiber and vegetables are associated with gut microbial changes that, in turn, are associated with a health benefit.”

My take: To enhance your odds of good health, avoid smoking, stay fit, and eat your fruits/veggies.

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Care Coordination and Magical Thinking

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One myth that has been promulgated has been that care coordination will lead to cost containment. A commentary on this topic (JM McWilliams. NEJM 2016; 375: 2218-20) explains the fallacy of this thinking.  While care coordination can improve medical care, “conflating cost containment and care coordination poses many potential dangers.”  Good care is worthy goal even in the absence of saving money.

Key points:

  • Care coordination often improves outcomes but typically involve interventions to correct underuse of care
  • For every costly complicated prevent, “a care coordination program must manage care for multiple patients…[which] is costly.”
  • Early evaluation of care coordination in accountable care organizations (ACOs) have shown the efforts “have meaningfully improved patient experiences but not rates of hospitalizations for ambulatory care-sensitive conditions.” There has not been evidence of fewer readmissions or fewer preventable hospitalizations with care coordination.
  • Other strategies to reduce cost are now being targeted, like steering patients to lower-priced providers

My take (from author): “We should coordinate care not to save money but because coordinated care is better care.”

Yosemite

Yellowstone (I took this picture!)

Primary Sclerosing Cholangitis (PSC) –Natural History Study

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Last week, I posted an blog referencing new guidelines for peanut introduction.  A more detailed explanation of these guidelines: New Guidelines: Early Introduction of Peanut to Prevent Peanut Allergy from David Stukus (Thanks to Kipp Ellsworth for sharing this information)

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A recent study (PL Valentino et al. JPGN 2016; 63: 603-09) follows “the largest reported pediatric PSC cohort” to determine the natural history.

Study characteristics:

This retrospective study followed 120 children (1-21 yrs) with a median age of 14 years.  27% had autoimmune sclerosing cholangitis (ASC), 63% had PSC; 24% (n=29) of entire cohort had exclusive small duct PSC. Median followup was 3.7 years.

Key findings:

  • 81% of PSC patients had inflammatory bowel disease; most (72/97) had ulcerative/indeterminant coliits. 40/72 had pancolitis.
  • PSC-IBD was more common than ASC-IBD (85% vs 68%).
  • 10-year transplant-free survival in this cohort was 89%; there were 6 liver transplants.
  • The rate of cirrhosis was lower in the group who had IBD preceding PSC (15% vs 31%,P=0.05).
  • PSC is clinically silent in the majority of patients; 64% presented with abnormal chemistries and no other symptoms.
  • ERCP therapeutic intervention was low, 3% for stenting and 7% for balloon dilatation.

The authors speculate that one reason for milder PSC-IBD disease could relate to the fact that IBD patients undergo frequent chemistries.  In those without IBD, subacute PSC could be present for a much longer period before detection.  The authors note that PSC in children presents as a milder disease with only 10% having cirrhossi compared with 30% in studies with adult patients.

My take: We have a lot to learn about PSC including which patients are likely to develop clinically significant liver disease and whether most patients benefit from treatment.

Related blog post: Should we care about subclinical PSC? (This post has links to others related to PSC)

Thunder Hole, Acadia Nat'l Park

Thunder Hole, Acadia Nat’l Park

Can the FDA stop snake oil salesmen?

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A recent commentary (C Robertson, AS Kesselheim. NEJM 2016; 375: 2313-5) examines how the issue of “free speech” may undermine the FDA’s ability to regulate ineffective or dangerous medications. This has been discussed in a previous blog:

Can the FDA prohibit free speech?

In a previous case, Caronia had promoted sodium oxybate for a wide range of nonapproved uses; some of these uses “were likely to cause patients substantial harm.”

Yet, the 2nd Circuit court reversed a lower court in ruling that Caronia’s sale pitches were protected free speech.  This decision “subverted decades of presumptions about how the government could oversee the behavior of the pharmaceutical and medical device industries.”

The authors hope that an upcoming case to the 1st circuit will uphold the FDAs ability to assure that patients are protected and that the use of drugs is driven by science and not marketing.  If manufacturers are allowed to promote a wide range of uses for drugs with narrow indications, there will not be an incentive to determine if these medications are safe and effective.

My take: If the principles of free speech are extended to promoting bogus claims about pharmaceuticals and medical devices, this would be a huge blow to medical science.

Acadia Natl Park

Acadia Natl Park

Better Hydration May Lead to Better Outcomes For Hemolytic Uremic Syndrome due to E coli

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According to a recent meta-analysis of 8 studies (1511 children), better hydration may reduce the risk of bad outcomes for hemolytic uremic syndrome (HUS): From JAMA Pediatrics: Shiga Toxin-Producing E coli, Hydration Status and Outcomes of Patients Infected With Shiga Toxin–Producing Escherichia coliA Systematic Review and Meta-analysis

Reference: JAMA Pediatr. Published online November 28, 2016. doi:10.1001/jamapediatrics.2016.2952

Results: A hematocrit value greater than 23% as a measure of hydration status at presentation with HUS was associated with the development of oligoanuric HUS (OR, 2.38 [95% CI, 1.30-4.35]; I2 = 2%), renal replacement therapy (OR, 1.90 [95% CI, 1.25-2.90]; I2 = 17%), and death (OR, 5.13 [95% CI, 1.50-17.57]; I2 = 55%). Compared with putatively hydrated patients, clinically dehydrated patients had an OR of death of 3.71 (95% CI, 1.25-11.03; I2 = 0%). Intravenous fluid administration up to the day of HUS diagnosis was associated with a decreased risk of renal replacement therapy (OR, 0.26 [95% CI, 0.11-0.60]).

Conclusion from abstract: Two predictors of poor outcomes for STEC-infected children were identified: (1) the lack of intravenous fluid administration prior to establishment of HUS and (2) a higher hematocrit value at presentation. These findings point to an association between dehydration and adverse outcomes for children with HUS.

This study is in agreement with a prior study referenced on this blog: Changing Paradigm in Hemolytic Uremic Syndrome

acadia5

 

Peanut Allergy Prevention Guidelines

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From USA Today: Peanut allergy: Everything they told you was wrong

LINK::

An excerpt:

Research suggests the method to stopping a lifelong peanut allergy is to, well, feed your baby peanut foods.

The National Institute of Allergy and Infectious Diseases, part of the federal government’s National Institute of Health, issued new guidelines to health care providers and parents Thursday…

The guidelines are based on whether a child has eczema or an egg allergy, good indicators of peanut allergies. Fauci suggests parents check with their doctor before moving forward with peanut foods.

The guidelines are as follows:

– For infants deemed a high risk for developing a peanut allergy, based on eczema or egg allergies, experts suggest feeding them food with peanuts as early as four to six months old.

– Infants with mild to moderate eczema should be introduced to peanuts at six months old.

– For babies without eczema or egg allergies, researchers say parents can start giving them peanut foods when they see fit.

 

From NBC News: Peanut Allergy Prevention (includes video)

High-risk infants

Babies with with severe eczema or an egg allergy should be tested at a specialist’s office when they’re 4 to 6 months old and have started taking solid food.

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Related blog posts:

 

Improved Understanding 18 Years Later

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A deeply painful experience for me occurred 18 years ago when a child that I cared for had a complication following an endoscopy.  Now, a recent publication (A Sierra et al. JPGN 2016; 63: 627-32) provides relevant information.  To be clear, this article would not have averted the complication but may help explain why it happened.

This retrospective study from 2010-2014 identified 7 cases of biopsy-induced intraduodenal hematoma (IDH) from a total of 2705 nontherapeutic upper endoscopies and 1163 duodenal biopsies.

Key findings:

  • 6 of 7 children had undergone a bone marrow transplantation and were at risk for graft-versus-host disease (GVHD)
  • 1 had Noonan syndrome
  • Thrombocytopenia was NOT correlated with IDH
  • No early perforations were associated with IDH

As part of this study, the authors reviewed the entirety of published IDH in children, 47 cases.  One prior author, Sahn et al (JPGN 2015; 60: 69-74) suggested that any organ transplant could increase the risk of IDH.  In this series, 29% of their patients had undergone transplantation (2 liver, 1 heart, 1 BMT).  Interestingly, among the entire 47 cases, there had been another report of a child with Noonan syndrome, suggesting some underlying susceptibility in the coagulation or platelet function pathways.

Clinical features of IDH:

  • Following endoscopy, particularly the first 3 days, signs/symptoms included epigastric pain, abdominal tenderness, and vomiting
  • Imaging including U/S, CT and MRI can confirm diagnosis
  • Resolution can take 2-3 weeks, during which parenteral nutrition is needed
  • IDH can cause acute pancreatitis or obstructive cholestasis
  • In trauma-induced IDH, surgery is much more likely than with endoscopic/biopsy-induced IDH

My take: BMT (and other types of transplantation) markedly increase the risk of biospy-induced duodenal hematoma. In this series, 7% of BMT patients had IDH compared with 0.1% of all others.

Related blog posts:

Jones Bridge & Chattahoochee River

Jones Bridge & Chattahoochee River

What’s Wrong with “I Want My Kid Tested for Food Allergies” (Part 2)

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In a previous blog entry, What’s Wrong with “I Want My Kid Tested for Food Allergies,” the pitfalls of allergy testing are detailed.

A recent study (DR Stutkus et al.Pediatrics December 2016, VOLUME 138 / ISSUE 6) suggests that primary care providers could used more education on utilizing allergy testing more effectively.  The main problem with food allergy testing is its very low positive predictive value. In a previous study of food allergy testing, the positive predictive value of food allergy testing was 2.2%!

Thanks to Kipp Ellsworth for this reference.

Abstract:

BACKGROUND AND OBJECTIVE: Immunoglobullin E (IgE)-mediated food allergies affect 5% to 8% of children. Serum IgE levels assist in diagnosing food allergies but have low positive predictive value. This can lead to misinterpretation, overdiagnosis, and unnecessary dietary elimination. Use of IgE food allergen panels has been associated with increased cost and burden. The scale of use of these panels has not been reported in the medical literature.

METHODS: We conducted a retrospective review of a commercial laboratory database associated with a tertiary care pediatric academic medical center for food IgE tests ordered by all provider types during 2013.

RESULTS: A total of 10 794 single-food IgE tests and 3065 allergen panels were ordered. Allergists ordered the majority of single-food IgE tests (58.2%) whereas 78.8% of food allergen panels were ordered by primary care providers (PCPs) (P < .001). Of all IgE tests ordered by PCPs, 45.1% were panels compared with 1.2% of orders placed by allergists (P < .001). PCPs in practice for >15 years ordered a higher number of food allergen panels (P < .05) compared with PCPs with less experience. Compared with allergists, PCPs ordered more tests for unlikely causes of food allergies (P < .001). Total cost of IgE testing and cost per patient were higher for PCPs compared with allergists.

CONCLUSIONS: Review of food allergen IgE testing through a high volume outpatient laboratory revealed PCPs order significantly more food allergen panels, tests for uncommon causes of food allergy, and generate higher cost per patient compared with allergists. These results suggest a need for increased education of PCPs regarding proper use of food IgE tests.

From Cadillac Mountain, Acadia Natl Park

From Cadillac Mountain, Acadia Natl Park