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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.

Why U.S. Consumers Pay More For Medications

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The NY Times has highlighted the high cost of medical care in the U.S. in a series of articles.  While only affecting about 10% of the total U.S. health cost, the wide variation in the cost of medicines between the U.S. and other countries leaves the U.S. consumer feeling ‘ripped-off.’

Here’s the link:  bit.ly/17p8kKR (from Jay Bookman) and here’s an excerpt:

Pulmicort, a steroid inhaler, generally retails for over $175 in the United States, while pharmacists in Britain buy the identical product for about $20 and dispense it free of charge to asthma patients. Albuterol, one of the oldest asthma medicines, typically costs $50 to $100 per inhaler in the United States, but it was less than $15 a decade ago, before it was repatented.

“The one that really blew my mind was the nasal spray,” said Robin Levi, Hannah and Abby’s mother, referring to her $80 co-payment for Rhinocort Aqua, a prescription drug that was selling for more than $250 a month in Oakland pharmacies last year but costs under $7 in Europe, where it is available over the counter…

Unlike other countries, where the government directly or indirectly sets an allowed national wholesale price for each drug, the United States leaves prices to market competition among pharmaceutical companies, including generic drug makers. But competition is often a mirage in today’s health care arena — a surprising number of lifesaving drugs are made by only one manufacturer — and businesses often successfully blunt market forces….

On the same topic, in Forbes: forbes.com/sites/peterubel/2013/10/15/-expensive-new-drugs/ …

In the U.S., the FDA must deem a drug safe and effective before allowing it on the market.  But at that point, there are no economic barriers to the use of those medications.  By contrast, drugs in the United Kingdom must go through economic analyses by a unit known as NICE—United Kingdom’s National Institute for Health and Clinical Excellence.  Being safe and effective is not enough to pass muster with NICE.  The drug must also be cost effective. …

Should we demand proof of cost effectiveness before allowing drugs on the market?  Or before agreeing to pay for them in Medicare and Medicaid?  Doing so would undoubtedly reduce healthcare expenses.  With medical spending threatening our fiscal future, it makes no sense that Medicare is prevented by law from considering the cost of care when making coverage decisions.  You heard that right—forbidden by law!

Related Blog Post:

Should Physicians Dispense Drug Coupons? | gutsandgrowth

Update on Hepatitis B & C -Postgraduate Course

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Update on Hepatitis B –Jean Pappas Molleston

Hepatitis B: Who to Treat:

  • Immune active Hepatitis B with active disease: HBeAg+ (> 6 months), HBV DNA > 20,000 IU/ml, ALT > 1.5 x normal or > 60 IU/L, &  moderate/severe inflammation/fibrosis
  • Reactivated Hepatitis B with active disease: HBeAg‐ (> 12 months), HBV DNA > 2000 IU/ml, ALT > 1.5 x normal or > 60 IU/L

Hepatitis B: Who to Not Treat:

  • Immunotolerant Hepatitis B: HBeAg+, HBV DNA > 20,000 IU/ml, Normal ALT
  • Inactive carrier: HBe Ag, HBV DNA < 2000

What to Use to Treat Children with Hepatitis B and When:

  • Only children with active disease should be treated
  • Many would suggest IFN as a first line drug, especially for younger children
  • Nucleoside analogues can now be considered in older children: Tenofovir is licensed for over age 12,  Entecavir is licensed for over age 16

What’s Exciting?

  • NCT01519960 Peg‐IFN monotherapy for children with chronic active hepatitis B
  • NCT01368497 Peg‐IFN and Entecavir for treatment of Hepatitis B in immunotolerant children
  • New drugs
  • New ways to predict who will have worse disease and who will respond
  • Direct Acting Antivirals

Treating HCV: 2013 and Beyond… Regino P. González-Peralta, M.D.

Standard of Care HCV Therapy: Children

  1. IFN/PEG-IFN-α-2a (PEG-2a):  ‘‘Branched’’ 40-kDa PEG moiety, Dose: 104 μg/m2 SQ once weekly, Available: prefilled syringes or as vials
  2. PEG-IFN-α-2b (PEG-2b): ‘‘Linear’’ 12-kDa PEG, Dose: 60 μg/m2 SQ once weekly, Available: Measured vials/ready-use pens

Other pointers:

  • Discussed IL-28 B Polymorphism –No pediatric data yet
  • Close monitoring for those who are treated
  • PEG-RBV is standard of care for children though with suboptimal efficacy and significant toxicity
  • Warp-speed evolution of HCV therapies
  • All ORAL’ regimen on horizon
  • Yearly evaluation: CBC, liver tests, HCV RNA and PT/INR (cirrhosis)

HCV Rx in Children: to treat or not:

IN FAVOR…

  • Avoid disease progression
  • Remove social stigma
  • Decrease HCV burden
  • Children ‘better’ candidates

…AGAINST

  • Benign disease
  • Efficacy
  • Toxicity
  • Direct Acting Antivirals (in the pipeline)

Full slides available on postgraduate Course Syllabus (posted with permission): PG Syllabus

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

One More Day Syndrome & Necrotizing Enterocolitis

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In many situations, the advice is to wait one more day and then decide/act; however, sometimes one more day winds up being a week, a month, or longer.  A recent editorial indicates that there is enough evidence now for probiotic usage in neonates to prevent necrotizing enterocolitis (NEC).  The authors state that to continue “with the standard of care, in which no new products are provided…is ethically unacceptable” (JAMA Pediatrics 2013; 167: 885-6).  Thanks to Ben Gold for this reference.

Key arguments:

  • A 2011 Cochrane review identified 16 eligible trials with 2842 premature infants (<2500 g, <37 weeks).  Probiotics reduced the incidence of NEC with a relative risk of 0.35 and mortality with a relative risk of 0.40.  Despite the typically cautious recommendations from Cochrane reviews, the authors state “updated review of available evidence supports a change in practice.”
  • While the American Academy of Pediatrics in 2010 noted there is some evidence to support probiotic usage and called for more studies, there are no studies currently being conducted in the U.S.
  • The authors note that the “FDA Center for Biologic Evaluation and Research is committed to policies that effectively prohibit probiotic efficacy trials.” Under current policies, the authors state these “studies will not be conducted in a US setting for the next 20 to 30 years.”
  • Other countries , like Australia, allow use of probiotic with parental consent.
  • The authors propose that probiotic efficacy be studied in a comparative effectiveness design.

Bottomline: Current regulations have stymied the use of probiotic trials for NEC.  What will it take for regulatory agencies to relent and allow this promising research?

Related blog posts:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

Civet Cat Poop Coffee

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From KT Park’s twitter feed: npr.org/blogs/thesalt/2013/09/02/218232266/is-your-pricey-cup-of-cat-poop-coffee-fake-there-s-a-test-for-that …

Dr. Balistreri briefly referred to civet cat poop coffee in his clinical year in review of studies.  This year’s focus was on the microbiome.  The lecture pulled together a large number of divergent sources to show how the microbiome can affect everything, including inflammatory bowel disease, colic and obesity.  The reference to Civet Cat poop coffee indicates that ingesting stool may not be as disgusting as it sounds.

TPN Drug Shortages -A Useful Reference

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A recent article in Today’s Dietician offers advice on drug shortages with TPN.

The following is a link (from a Kipp Ellsworth retweet) and an excerpt: http://t.co/CBKti0mNep

Lipids. Two US manufacturers produce three lipid concentrations  (10%, 20%, and 30%) of IV fat emulsions. The 30% concentration can be used only  in total nutrient admixtures. ICU patients receiving propofol can forgo IV fat  emulsions, since propofol is in a lipid-based emulsion that provides 1.1  kcal/mL, just like the 10% IV fat emulsion.

Since essential fatty acid deficiency doesn’t develop until  after two weeks of lipid-free PN, IV fat emulsions can be safely withheld for  the first two weeks if lipids are in short supply.1 After two weeks of  lipid-free PN, the minimum dose of IV fat emulsions, which is 100 g/week, can  be provided to prevent essential fatty acid deficiency.

IV fat emulsions should be discontinued in patients  tolerating EN and who don’t have malabsorption concerns.

IV Multiple Vitamins               When there’s a shortage of IV multiple vitamins, dietitians  should evaluate all patients for their ability to absorb enteral multiple  vitamin supplements in capsule, tablet, liquid, or chewable forms. For patients  who can’t absorb enteral vitamin supplements, the IV multiple vitamin dose  should be decreased from 10 mL to 5 mL/day to conserve supplies. If IV multiple  vitamins remain in short supply despite conservation efforts, the standard dose  of 10 mL should be given three times per week.2

If supplies have been exhausted, PN must be supplemented  intravenously with individual parenteral vitamins according to the following  ASPEN recommendations: thiamin: 6 mg; folate: 0.6 mg; ascorbic acid: 200 mg;  pyridoxine: 6 mg; and vitamin K: 0.5 to 1 mg/day or 5 to 10 mg/week.2 In  addition, cyanocobalamin (vitamin B12) must be given intramuscularly at least  once per month.2

Trace Elements               Combination trace elements and individual trace element  products offer alternatives to PN products in short supply.

Combination Multiple Trace Element Products               Dietitians have a choice of two different multiple trace  element combination products: MTE4 and MTE5. MTE4 products contain zinc,  copper, chromium, and manganese and come in a standard 3 mL dose or a 1 mL  concentrated dose. MTE5 products contain the same four trace elements with the  addition of selenium in either the standard 3 mL dose or the 1 mL concentrated  dose.

If there’s a shortage of the concentrated products, RDs can  use the standard 3 mL dose of MTE4 and MTE5. When MTE4 products aren’t  available, RDs can substitute the MTE5 products. If MTE5 products aren’t  available, RDs should substitute MTE4 products and add 60 mcg of selenium  individually to achieve the equivalent composition of MTE5.

…If no MTE products are available, individual trace elements  should be added to PN solutions.

Individual Trace Elements               Individual trace elements are used when combination trace  element products are unavailable…

There’s no need to supplement manganese when there are  shortages of multiple trace element products. Whole blood manganese levels  frequently are elevated in long-term PN patients, and manganese contamination  often occurs in other PN products. No alternative IV forms of chromium are  available but, like manganese, there may be some chromium despite the fact it  isn’t intentionally added because of its contamination potential in other PN  products. RDs can evaluate a patient’s ability to absorb chromium as part of  multivitamin and mineral supplementation through the enteral route and monitor  for signs of deficiencies.

Other than the selenium content of MTE5 products, selenium  is available as a single IV trace element product. When MTE5 products and individual  IV selenium products aren’t available, RDs can consider using oral selenium  supplementation.

Copper is available as a single PN trace element in two  forms: IV copper chloride or IV cupric sulfate. If all supplies of IV copper  have been exhausted, a patient should be evaluated for oral copper supplements.

Zinc is available in either IV zinc sulfate or IV zinc  chloride. It’s important to note that if zinc is given enterally in high doses,  RDs should monitor for a copper deficiency, as zinc and copper both compete for  absorption with the same carrier protein when EN is used….

— Mandy L. Corrigan,  MPH, RD, LD, CNSC, is a nutrition support dietitian with Coram Specialty  Infusion Pharmacy.

 Professional  Resources • American Society of Health-System Pharmacists Drug  Shortages Resource Center: www.ashp.org/menu/DrugShortages

• American Society for Parenteral and Enteral Nutrition Drug  Shortages Update: www.nutritioncare.org/Professional_Resources/Drug_Shortages_Update

• FDA Current Drug Shortages Index: www.fda.gov/Drugs/DrugSafety/DrugShortages/ucm050792.htm

• FDA Fact Sheet: Drug Products in Shortage in the United  States: www.fda.gov/RegulatoryInformation/Legislation/FederalFoodDrugandCosmeticActFDCAct/ SignificantAmendmentstotheFDCAct/FDASIA/ucm313121.htm

• FDA Frequently Asked Questions About Drug Shortages: www.fda.gov/Drugs/DrugSafety/DrugShortages/ucm050796.htm

• Fresenius Kabi Adult Multitrace Element Availability  (product information): www.fda.gov/downloads/Drugs/DrugSafety/DrugShortages/UCM355392.pdf

• Fresenius Kabi Pediatric Multitrace Element Availability  (product information): www.fda.gov/downloads/Drugs/DrugSafety/DrugShortages/UCM354265.pdf

• Fresenius Kabi Phosphate Injection Availability (product  information): www.fda.gov/downloads/Drugs/DrugSafety/DrugShortages/UCM354277.pdf

• National Medication Errors Reporting Program (patients and  clinicians): www.ismp.org/orderforms/reporterrortoISMP.asp

 

For More Information               The following references can serve as viable resources for  dietitians to learn more about parenteral nutrition drug shortages and their  impact on patient safety and patient care:

• Buchman AL, Howard LJ, Guenter P, Nishikawa RA, Compher  CW, Tappenden KA. Micronutrients in parenteral nutrition: too little or too  much? The past, present, and recommendations for the future. Gastroenterology.  2009;137(5 Suppl):S1-S6.

• Corrigan ML, Kirby DF. Impact of a national shortage of  sterile ethanol on a home parenteral nutrition practice: a case series. JPEN  J Parenter Enteral Nutr. 2012;36(4):476-480.

• Holcombe B. Parenteral nutrition product shortages: impact  on safety. JPEN J Parenter Enteral Nutr. 2012;36(2 Suppl):44S-47S.

Related Blog Posts:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

PSC 2013 Review

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A recent review of PSC was published (Gastroenterology 2013; 145: 521-36).  This review is a little more detailed than a previous review noted in this blog less than 6 months ago (Staying current with PSC | gutsandgrowth).

A couple of useful comments from the review:

  • “An increased serum level of alkaline phosphatase is the most common biochemical abnormality detected in patients with PSC.  In some cases, it is the only biochemical alteration observed, such as in patients with intrahepatic involvement.”
  • “Typically, a liver biopsy is not required to diagnose PSC unless small duct PSC is suspected or if there are concerns that a patient also has AIH.”  Cholangiography is the best way to identify PSC.
  • “Patients diagnosed with PSC should undergo colonoscopy… to determine if they have IBD, even when there are no symptoms.”
  • Autoimmune hepatitis-PSC overlap is thought to occur in ≤6% of cases.  AIH-PSC should be suspected if there are biochemical features of AIH (positive serology, increased transaminases), histology suggestive of AIH, or in AIH patients that become refractory to treatment.
  • No controlled trials have identified effective medical treatments.  Studied medications have included corticosteroids, etanercept, tacrolimus, cyclosporine, azathioprine, methotrexate, infliximab, and ursodeoxycholic acid.  The latter may increase disease progression, particularly at higher doses.

Also noted:

Hepatology 2013; 58: 1392-1400. “Primary Sclerosing Cholangitis, Autoimmune Hepatitis, and Overlap in Utah Children: Epidemiology and Natural History”

In this study the authors identified 607 cases of IBD, 29 cases of PSC, 12 cases of ASC (overlap), and 44 cases of AIH.  “Cholangiocarcinoma developed in 2 of the 29 PSC patients (6.9%).  PSC occurred in 9.9% of patients with ulcerative colitis (UC) and 0.6% of patients with Crohn’s disease.”

Related blog posts:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

Top Lecture: Enteral Nutrition for Crohn’s Disease

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In my opinion, the best lecture from this year’s postgraduate course was from Dr. Baldassano.  Enteral nutrition in Crohn disease: Where should this be in our treatment algorithm?  Robert N. Baldassano, MD (page 115)

Dr. Baldassono has personal experience with improving with enteral therapy after failing methotrexate/remicade.  His conclusion:

Enteral Nutritional Therapy: Where should this be in our treatment algorithm?

  • Should be offered to all newly diagnosed Crohn’s patients who can tolerate Nutritional Therapy
  • Special groups (especially a good idea): Malnourished patients, Younger patients, Growth failure, History of Cancer, Family history of Lymphoma, Consider when failing other therapies

This conclusion is supported by his presentation.

Should we be immunosuppressing our Patients?  Hypothesis: IBD arises from inappropriate handling of intestinal bacteria

Elements of Modern Lifestyle Lead to Changes in Gut Microbiota

  1. Improved sanitation
  2. Less crowded living conditions
  3. Decline in parasites
  4. Vaccinations
  5. Increased antibiotic use
  6. Caesarean section
  7. Refrigeration
  8. Food processing
  9. Diet changes
  10. Improved sanitation

Diet is associated with new onset IBD

  • High dietary intakes of total fats, PUFAs, omega‐6 and meat were associated with an increased risk of CD and UC
  • High fiber and fruit intakes were associated with decreased CD risk
  • High vegetable intake was associated with decreased UC risk.  Reference: Hou JK et al. American Journal of Gastro 2011; 106:563-73
  • The Bacteroides enterotype highly associated with animal protein and saturated fats which suggests meat consumption as in a Western diet
  • The Prevotella enterotype, high values for carbohydrates and simple sugars, indicating association with a carbohydrate-based diet, more typical of agrarian societies.  References: Wu G, et al. Science. 2011 Oct 7;334(6052):105‐8

Partial or Complete Enteral Nutrition?

  • 50% vs 100% of total caloric needs for induction with elemental formula (PCDAI < 10 at 6 weeks)
  • 50% of total caloric needs 15% remission
  • 100% of total caloric needs 42% remission
  • Labs improved only in the 100% group
  • Weight gain similar in the 2 groups. References: Akobeng et al Clin Nutr 2007; Ludvigsson et al Acta Paediatr 2004;Johnson et al Gut 2006;Critch et al. JPGN: 2012 

Pediatric Longitudinal Study of Semi‐Elemental Diet and Stool Microbiome (PLEASE)

Prospective cohort study of children with Crohn disease from Philadelphia (used Peptamen), Toronto (used Modulen) and Halifax (used Osmolite); (n=90)

  • Enteral therapy with defined formula diet (n=38) vs. anti‐TNFα therapy (n=52)
  • Similar drop in PCDAI and calprotectin in TNF group and diet group. 

Other points:

  1. Insurance generally will cover nasogastric feeds
  2. Disease location –not clear that this matters with Crohn’s disease
  3. The reason EN works may be not what you are giving the patient but what the patient is not getting
  4. Bacterial populations in pediatric IBD subjects on semi‐elemental diet (16S rDNA sequencing) develop a rapid change in gut bacterial populations upon initiating diet.
  5. Partial (50%) nutrition, as noted above, helped maintain remission compared to normal diet.

Nutrition Therapy: “European” Protocol

• Induction:  Exclusive enteral nutrition with an elemental, semielemental,or polymeric formula

• Duration: 4 – 12 weeks

• Maintenance Therapy: (either)

– Nutritional therapy: Repeat 4 week cycle of exclusive enteral nutrition every 3– 4 months

OR

– Medical therapy: 6‐MP/AZA/MTX after induction with nutritional therapy

CHOP EN Experience: What if >80% of calories is from Enteral Nutrition?

  • Methods: Semi‐elemental formula, 80%‐90% of patient’s caloric needs from formula, Nocturnal NG feeds (outpatient teaching program), Normal diet as tolerated during the day
  • Duration:  7 days per week for 8‐12 weeks (induction), 5 days per week (maintenance) Reference: Gupta et al. Inflamm Bowel Dis. 2013:1374-8.
  • Induction of remission: 65% (at 8 weeks)
  • Response: 87% (at 8 weeks)
  • Significant improvement in weight and linear growth
  • Protocol is well tolerated:  no serious adverse events

Postgraduate Course Syllabus (posted with permission): PG Syllabus

Related blog posts:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

“Gluten-Related Disorders” (Part 2)

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Non-celiac Gluten Sensitivity NCGS -Focused discussion in Section III
  • No biologically measurable response has been found – these are people with normal celiac serology (neg ttg/ema) and normal biopsies.
  • Specific discussions regarding autism and schizophrenia.  On page 44, authors note that a 2008 Cochrane review concluded the evidence for a gluten-free diet for autism was poor.  In 2012, a two-stage RCT (Whiteley et al) of gluten-free casein-free diet reported significant group improvements after 8 and 12 months on diet. Thus, diet may be helpful.
  • Other chapters allude to NCGS as well.  Page 124: “There are no epidemiologic studies assessing the prevalence of NCGS. Bizzaro et al estimated that for every one person with CD, there are at least six to seven with gluten sensitivity.”
Wheat Allergy -Section IV
  • Forms include oral food allergy, “wheat-dependent, exercise-induced anaphylaxis,” and Baker’s asthma (aerosolized exposure).
  • Skin prick tests or RAST’s are notorious for providing a high rate of false-positive results.  Low rate of false negative results, though, are noted.

Treatment -Section VI:

  • This section provides a number of tables to assist with diet and hidden sources of gluten.
  • GFD may lead to specific nutrient deficiencies: fiber, iron, folate, niacin, zinc, vitamins B12, A, D, E, and K; also, GFD may be higher in fat.

Psychological Aspects -Section VII:

 “The family has to buy gluten-free foods and all members have to learn how to avoid contaminating gluten-free foods, dishes, toasters, and so on.” Parents have to read all food labels and prepare special meals while attending social events.
A nice sample letter is included on page 129 –should make a good EPIC smartphrase.
Difficult Cases -Section VIII:
  • Labs to check in sick CD patient (Table 2 -page 133).
  • Causes of Nonresponse to GFD: poor compliance, accidental ingestions, nonceliac disease causing symptoms.
  • While some of the authors state that true refractory disease is “rare in adults,extremely rare in children,” in other parts of the book it is noted that complete histologic response is not seen in all patients (some with apparently good adherence).

IgA deficiency (page 139).

  • 85-90% of IgA deficient patients have no clinical symptoms.  Occurs in about 1 in 300.  For those with symptoms, manifestations could include sinopulmonary disease, allergy/atopy, autoimmune diseases, giardiasis/infections, and transfusion reactions (against IgA) (see Table 8 on page 143).
  • Transiently low IgA is common in children <4 years.
  • For IgA deficient patients, risk of CD is 10-20 times general population.
  • In true deficiency, level is typically <7 mg/dL.  More often, there is a partial deficiency which is ‘almost always asymptomatic.’  In partial IgA deficiency, IgA assays identify about 90% of CD cases.
Also, in the difficult cases section an algorithm for follow-up of newly diagnosed CD is presented and discussed (page 154).  Recommendations include nutritional counseling, resource identification, family screening, and celiac education.  Consider checking iron status, vitamin D, folate, zinc, copper and DEXA.  Recommends followup serology 6 months following diagnosis and if normal, then on a yearly basis.
Related blog posts:
Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

“Gluten-Related Disorders” (Part 1)

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On the way back from our National Meeting (NASPGHAN), I had the opportunity to read “Gluten-Related Disorders” ed. by Alessio Fasano.  The book is a very good summary about the science of celiac disease (CD), wheat allergy, and nonceliac gluten sensitivity (NCGS); at the same time, there is some redundancy due to multiple authors (particularly evident in later chapters). One of the book’s features is clinical vignettes to drive home multiple teaching points.  For example, the ‘refractory’ CD patient who in fact has Crohn’s disease. The book also provides a code to obtain the information online, so it is fully searchable.  In the introduction, there is an in-depth explanation of why gluten can be so difficult for the GI tract.  The discovery that a gluten-free diet can be helpful was a byproduct of wheat shortages during WWII.  Here are some useful insights that were noteworthy:

Definitions (page 9):  reviews the terms “silent CD,” “potential CD,” and “latent CD.”

  • Silent =asymptomatic but with all other features: +antibodies, +HLA type, +abnl histology
  • Potential =+antibodies but lack of histology evidence  (antibodies often precede development of clinical disease)
  • Latent =previous evidence of CD but currently tolerating gluten in diet with normal histology

Epidemiology:

  • While increase in CD is partly due to awareness, there has been a “true increase in prevalence, with rates doubling every 20 years or so.”
  • Early vaccinations are not risk factors for the development of CD
  • Breastfeeding can reduce risk of CD by about 50% though gluten should be introduced between 4-6 months.

Presentation:

  • Table of the main extraintestinal manifestations on page 24.  Most common: anemia (especially iron deficiency), short stature, and pubertal delay.
  • Associated diseases (Table 3, page 29): Down syndrome, Turner syndrome, Type I Diabetes, Williams syndrome, IgA deficiency, and Autoimmune thyroid disease.
  • Eosinophilic esophagitis has been identified in a small number of patients with celiac disease.  The book notes a study with 7 pediatric patients; only one of them improved their esophageal eosinophilia with a GFD.

Tips on diagnosing celiac

  • Bulb abnormalities with a normal 2nd portion of duodenum biopsy can be seen in 10% or more of patients with celiac.  The authors recommend obtaining 4 biopsies from 2nd and 3rd portion and 2 biopsies from bulb (separate containers) (page 144-145).
  • Most celiac experts say there is no celiac without DQ2 or DQ8.  There are several situations in which a negative HLA type could be helpful (page 78)
  1. -negative serology but abnormal histology
  2. -gluten-free diet (GFD) started before diagnosis confirmed
  3. -failure to respond to GFD
  4. -asymptomatic high-risk individuals to help determine if periodic serology is worthwhile
  • In most individuals, obtaining TTG IgA along with serum IgA is recommended for diagnosis (and avoiding older gliadin antibody tests).  If clinical suspicion is high, endoscopy is warranted regardless of result.
  • Under the age of 2, deamidated anti-gliadin antibodies appear months earlier than the TTG in prospective studies, so order the dAGA IgG and dAGA IgA in kids under 2. (Available with both quest and labcorp).  The deamindated anti-gliadin antibodies may be more helpful/sensitive in monitoring dietary adherence than TTG.
  • Infants who have a first degree family member with celiac should be introduced to “small” amounts of gluten between 4 and 6 mos of age – not before and not delayed.  It appears to promote tolerance though it’s not clear if it just delays inevitable onset.  Small amounts can be a serving a day of a mixed, barley baby cereal.
  • Section V is devoted to diagnosis.  Table 1 (page 72) lists the sensitivity/specificity of the available serologies.
  • Screening asymptomatic persons.  The controversy regarding this practice is alluded to on page 75.  Currently NASPGHAN recommends screening at risk groups whereas AGA does not.
  • Endoscopy/Biopsy discussed (pages 78-82).  States a biopsy is not needed in the case of dermatitis herpetiformis due to characteristic deposits of IgA in the dermal papilla.  The authors recommend biopsy in all cases, but review ESPGHAN guidelines which state that biopsy can be omitted if TTG IgA >10 time ULN –if verified by positive EMA, HLA typing, and followed for symptomatic improvement.
  • Antibody tests “become negative in 15% after 1 month on GFD and in 57% after 3 months…diagnosis of CD cannot be made while on GFD.”  Algorithm for diagnosis of CD with a child on GFD presented on page 148.
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Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

Postgraduate Course Notes – Hepatitis Module

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Notes from NASPGHAN’s postgraduate course:

Refractory Autoimmune Hepatitis (AIH):  — Vicky Ng

Dr. Ng’s talk started with an overview of AIH and referred to AASLD guidelines: Diagnosis and Management of Autoimmune Hepatitis – AASLD

Recommendations included the following:

  • Cholangiography for all new cases of AIH
  • Starting azathioprine after seeing some improvement in transaminases with steroids
  • Monitoring for HCC
  • Monitor bone density/bone protection strategies discussed
  • Long term Rx needed in majority, though small number may be able to come off therapy if doing well for 2 years and normal liver biopsy

Refractory mgt:

  • This is applicable in 15-20% of patients
  • Reasons for refractory disease: non response, drug intolerance, non-compliance, overlap syndrome, comorbidities
  • If treatment failure, options could include increasing steroids and azathioprine.  If concerns for decompensation, refer for liver transplant evaluation.
  • NO standard Rx for refractory, but consider MMF (mycophenolate mofetil), cyclosporin (CYA), or tacrolimus (FK)
  • MMF most promising agent for refractory disease.  Small studies of MMF in adults/pediatrics indicates response in about 2/3rds of patients; best for those intolerant to azathioprine & helpful in dropping steroid dosing.  In pediatrics, a starting dose of 20 mg/kg/day is typical and increasing up to 40 mg/kg/day.  Pediatric study: 18/26 (69%) with response and 14/18 with normal AST w/in 2 months.
  • Tacrolimus –small study showed about ~90% response.  Dose was 0.1 mg/kg/day & target trough was 3 ng/mL
  • Briefly discussed budesonide.  More data in pediatrics needed.

Postgraduate Course Syllabus (posted with permission): PG Syllabus

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.