Monthly Archives: November 2017

#NASPGHAN17 Why Rome IV Criteria are important

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More information from this year’s annual NASPGHAN meeting.

This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

The following slides highlight a terrific lecture by Carlo DiLorenzo (Nationwide Children’s Hospital).  Subsequently, I’ve included slides from Miranda van Tilburg (UNC); I was unable to attend her lecture and found some of the slides via twitter.

Key points:

  • Rome IV criteria are helpful, particularly with less common presentations like rumination
  • There has been an increase in nausea.  Morning nausea can be equated as a marker of anxiety until proven otherwise.
  • There is improved wording. “After appropriate medical evaluation, the symptoms cannot be attributed to another condition” may help facilitate the diagnosis of irritable bowel syndrome, for example, in patients with IBD who are in remission.

From Miranda Tilburg:

#NASPGHAN17 Psychosocial Problems in Adolescents with IBD

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This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

Slides from syllabus: APGNN Syllabus 2017

Key points:

  • ~30% of pediatric IBD patients have anxiety or depression.  This has not been shown to be related to disease activity.
  • Advice for parents: “Listen more and talk less.”
  • Antidepressants, when indicated, are about 6 times more likely to be helpful than detrimental

In the following slide, the term “normalize” indicates that checking on emotional health is part of a routine (eg. ‘we ask all our patients to complete this screening’)

#NASPGHAN17 Presentation: Reducing Hospitalization in Intestinal Failure Patients

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This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

Risk Factors for Hospitalization Among Pediatric Intestinal Failure Patients

Tatyana Hofmekler, Janet Figueroa, Hilina Kassa, Rene Romero, Andi Shane.

Dr Hofmekler is now part of GI Care for Kids (my group) and provided a terrific presentation.

NASPGHAN Annual Mtg 2017

Key points:

  • In this study, there were no social or demographic factors which were identified which were associated with increased hospitalization
  • Having a colon and an ileocecal valve lowered the risk of hospitalization
  • The use of SBBO treatment was associated with increased hospitalization though this may have been a marker of more severe disease
  • Vascular catheter infections were reduced compared to study at same institution previously but remained an important risk factor for hospitalization

My take: this study illustrates the challenges in reducing hospitalization.  While the authors did not identify social/demographic factors, my experience is that there are some families who are much more capable than others in taking care of children with complex problems.  If all children had the best parents, that would truly allow the hospitalization rate to be reduced much lower.

 

#NASPGHAN17 Presentations at Annual Meeting: GGT in PSC, Nutrition for Intestinal Failure

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This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

Improvement in GGT Predicts Event-free Survival in Primary Sclerosing Cholangitis Regardless of Ursodeoxycholic Acic Treatment. 

Mark Deneau et al. (Grand Watkins Prize).

Key points:

  • PSC is difficult to study due to its rarity and due to its slow progression; thus surrogate biomarkers are needed.
  • Alkaline phosphatase is not a good biomarker in children
  • GGT level at one year after diagnosis was predictive of prognosis
  • Ursodeoxycholic acid does not appear to be effective

Optimizing Nutrition in Intestinal Failure

Justine Turner, University of Alberta

Key points:

  • Human milk is an ideal “formula” for infants, including those with intestinal failure
  • Oral feedings are important
  • Combination of bolus feeds and continuous feeds is reasonable
  • SMOFlipid allows higher lipid dose administration without hepatoxicity; this may improve cognitive outcomes
  • Amino acid based formulas have higher osmolality which can contribute to diarrhea

Patients with >50% of small bowel and >50% of colon were most likely to achieve enteral autonomy (GIFT registry)

 

 

#NASPGHAN17 Annual Meeting Notes (Part 2): Year in Review

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This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

This first slide shows the growth in NASPGHAN membership:

Year in Review

Melvin Heyman  Editor, JPGN

This lecture reviewed a number of influential studies that have been published in the past year.  After brief review of the study, Dr. Heyman summarized the key take-home point.

 

Consequences of Individual Mandate Repeal

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Link: NY Times: Obamacare’s Insurance Mandate Is Unpopular. So Why Not Just Get Rid of It?

An excerpt: Without the mandate, the C.B.O. has said for years, premiums would spike, and millions fewer Americans would have health insurance. The budget office’s most recent estimate, published last week, said that the ranks of the uninsured would rise by 13 million over 10 years, and that average premiums would be 10 percent higher than under current law.

 

#NASPGHAN17 Annual Meeting Notes (Part 1): Neurostimulation for RAP, PSC-IBD, Organoids

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This blog entry has abbreviated/summarized these presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

William Balistreri Prize: Katja Kovaic et al. Neurostimulation for functional abdominal pain disorders in children –a randomized, double-blind, sham-controlled trial. This study enrolled 104 patients.  Lancet Gastroenterol Hepatol 2017; 2: 727-37.

Summary slide:

Fellow Research Award: Symptoms Underestimate Endoscopic Activity in PSC-IBD. Amanda Ricciuto et al. Hospital for Sick Children.

Key points:

  • In patients with IBD-PSC, clinical remission based on clinical symptoms is not reliable indicator of histologic remission.
  • Patients with PSC-IBD are more likely to have active endoscopic disease even when in “clinical remission”
  • Calprotectin levels (not PUCAIs) are helpful in confirming clinical remission.  A calprotectin <93 mcg/g was optimal level in determining clinical remission
  • Better control of disease could improve clinical outcomes (eg. colon cancer, liver progression)

Keynote Address: Organoids: Current and future promise for changing treatment of gastrointestinal and liver disorders.  James Wells Cincinnati Children’s Hospital Medical Center.

This was a terrific lecture.

  • Example of use of pluripotent stem cell usage: Diabetes. Phase 1 study has been started.
  • Organoids are in essence miniature versions of organs in a dish and with complex combination of cell types.
  • Organoids allow easier testing on these tissues for treatment and diagnosis of diseases
  • Organoids will allow for personalized testing of medications.  Some patients will respond differently.  This technology could be used to grow a specific organoid for a specific person and determine response on the organoid before giving to the patient.
  • Can engraft organoids into mice which can provide blood supply and allow larger organoids.
  • Clinical projects for organoids: Hirschsprung’s,  H pylori, Clostridium difficile, Short bowel syndrome, Fatty liver disease

#NASPGHAN17 IBD Treat to Target and Tight Control

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More information from this year’s annual NASPGHAN meeting.

This blog entry has abbreviated/summarized these presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

IBD Treat to Target: Treat the Patient or Treat the Disease

Robert Baldassano  Children’s Hospital of Philadelphia

I missed the first few minutes of this presentation, even though I had highlighted this as one of my top priorities.  So, if anyone reading this post has some additional comments, they are certainly welcome.

Key points:

  • Do not rely on symptoms alone to assess patient improvement.
  • Best surrogate marker: calprotectin.  Frequent calprotectin levels can help determine objective improvement; it is much more helpful than CRP as ~25% of patients do not elevate their CRP levels
  • Therapeutic drug monitoring is important in improving outcomes. Dose optimization improves response rate and durability of infliximab response.
  • Evolving targets in ulcerative colitis.  Even histologic activity, in the absence of endoscopic activity, is associated with relapsing disease
  • Dr. Baldassano indicated that he no longer is starting patients on thiopurine therapy. There are “36 phase 3 trials underway.” Thus, many promising options for those who may burn through current treatments
  • This lecture reviewed data from the RISK study showing that early (1st 90 days w/in diagnosis) TNF therapy helps prevent penetrating disease (related post: CCFA Update 2017/RISK study)

Another presentation by Philip Minar et al (Cincinnati Children’s Hospital Medical Center) shows that CD64 suppression is an early biomarker of response to infliximab therapy.

#NASPGHAN17 Is it time to stop using thiopurine therapy?

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This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

Safety in Pediatric IBD Therapy: Is it time to stop using thiopurines?

Jeffrey Hyams  Connecticut Children’s Medical Center

Key points from this lecture:

  • Dr. Hyams:  “There are better options than thiopurines in 2017 due to infrequent but serious risks”
  • The DEVEVOP study showed that anti-TNF agents did NOT increase the risk of lymphoma or hemophagocytic lymphohistiocytosis (HLH).  In contrast, these risks do occur with thiopurines –this is infrequent but remains significant.
  • Therapeutic drug monitoring may obviate the need for combination/dual therapy which has been shown to improve response rates to anti-TNF agents; methotrexate may work for combination therapy and may be safer than thiopurines
  • If a thiopurine is used as part of combination therapy, short duration (~6 months) is likely to have low risks
  • In addition to Dr. Hyams, Dr. Baldassano, in his discussion of treat to target (discussed in subsequent post), echoed the sentiment that he no longer recommends thiopurine therapy

Dr. Hyams slides list some of the relative risks of thiopurine therapy.  To understand these risks, the absolute risk is probably more helpful.

My take: This lecture did not focus on the main benefit of thiopurines which is its use in combination therapy. Many experts consider combination therapy to be the standard of care for adults with Crohn’s disease.  The advantages of combination therapy are mainly due to improved durability of anti-TNF therapy and lower antidrug antibodies.  How this benefit stacks up against the risks discussed in this lecture and whether this benefit can be supplanted by the use of therapeutic drug monitoring is uncertain.

 

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.

NASPGHAN Postgraduate Course 2017 (Part 5): Refractory constipation, Extraesophageal GERD, POTS, Recurrent Abdominal Pain

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This blog entry has abbreviated/summarized these presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

Here is a link to postgraduate course syllabus: NASPGHAN PG Syllabus – 2017

The child with refractory constipation

Jose Garza   GI Care for Kids & Children’s Healthcare of Atlanta

Key points:

  • Polyethylene glycol is a first-line agent and many patients require cleanout at start of therapy
  • Adequate dose of laxative is needed for sure regular painless stools
  • Don’t stop medicines before toilet training and until pattern of regular stooling established. “All symptoms of constipation should resolve for at least one month before discontinuation of treatment”
  • Gradually reduce laxatives when improved
  • An abdominal xray is NOT recommended to make the diagnosis of constipation
  • Do you have the right diagnosis? Irritable bowel is often confused with constipation.  With constipation, the pain is relieved after resolution of constipation.
  • Outlet dysfunction. Stimulant laxatives (eg. Senna) are probably underutilized. Biofeedback may help in older children.
  • Slow transit constipation. Newer prosecretory agents may be helpful –lubiprostone and linaclotide.
  • Organic constipation. Hirschsprung’s, Spina bifida, anorectal malformations etc. Testing: anorectal manometry, rectal biopsy (for Hirschsprung’s)
  • For refractory disease, consider rectal therapy –suppositories, transanal irritagations/enemas (~78% success for fecal incontinence/constipation). These treatments should be used prior to surgical therapy (eg. Malone antegrade continence enema/cecostomy)

 

 

The quest for the holy grail: Accurately diagnosing and treating extraesophageal reflux

Rachel Rosen   Boston Children’s Hospital

Key points:

  • It is frequent that EGD or impedance study will be abnormal, though this may not be causally-related.
  • No correlation with ENT exams/red airways and reflux parameters
  • No correlation with lipid laden macrophages and reflux parameters
  • No correlation with salivary pepsin and reflux parameters

Treatment:

  • Lansoprazole was not effective for colic or extraesophageal symptoms (Orenstein et al J Pediatrics 2009)
  • PPIs can increase risk of pneumonia/respiratory infections
  • Macrolides have been associated with increased risk of asthma but may be helpful for pulmonary symptoms
  • Fundoplication has not been shown to be effective for reducing aspiration pneumonia.  Fundoplication could increase risk due to worsened esophageal drainage.
  • ALTEs (BRUEs -brief resolved undefined events) need swallow study NOT PPIs

POTS and Joint Hypermobility: what do they have to do with functional abdominal pain?

Miguel Saps  University of Miami

Key points:

  • Patients with POTS and joint hypermobility have frequent functional abdominal pain as well as other comorbidities
  • Beighton Score can determine if joint hypermobility is present
  • Brighton Score determines if hypermobile Ehlers-Danlos syndrome is present
  • Patients with frequent fatigue.  Gradual progressive and regular exercise is important part of therapy.  Can start with recombant exercise – training bicycle exercise, swimming
  • Need to push salt intake and fluds

Do I need to test that C.R.A.P.?

Rina Sanghavi   Children’s Medical Center Dallas

This basic talk reviewed a broad range of issues related to functional abdominal pain.

Key points:

  • Carnett’s sign can help establish abdominal pain as due to abdominal wall pain rather than visceral pain
  • What is an appropriate evaluation?  Limited diagnostic testing for most patients.
  • Alarm symptoms include: Fevers, Nocturnal diarrhea, Dysphagia, Significant vomiting, Weight Loss/poor growth, Delayed Puberty, and Family history of IBD