#NASPGHAN19 Postgraduate Course (Part 2)

Here are some selected slides and notes from this year’s NASPGHAN’s postrgraduate course. My notes from these lectures may contain errors of omission or transcription.

Link to the full NASPGHAN PG Syllabus 2019 (Borrowed with permission)

9:00 – 10:20 “Potpourri”

46 Alessio Fasano, MD, MassGeneral Hospital for Children  Celiac disease: Beyond diagnosis

  • Reviewed potential non-biopsy option for diagnosis if anti-TG2 >10 x normal. Pediatricians are not following recommendations –>many children placed on gluten-free diet at lower titer antibody-positivity.
  • Recommends checking Hepatitis B antibody because many children with celiac disease do not seroconvert.
  • TTG levels are good for diagnosis but not as helpful for monitoring after diagnosis.
  • Only 10 out of 1000 are true refractory, about 100 out of 1000 are exquisitely sensitive to gluten

56 Meghana Sathe, MD, UT Southwestern Medical Center The role of the gastroenterologist and hepatologist in Cystic Fibrosis (CF) care today

  • Fecal elastase monitoring useful for determining need for PERT.
  • Discussed CF liver involvement.  Multilobular cirrhosis, 7% of individuals, is most important liver disease in CF.
  • Modulator therapy can elevate liver enzymes and may need to hold if ALT >5 ULN or lower elevation if elevated bilirubin (see Stop Rules -Practical Advice on DILI)
  • DIOS -for partial obstruction, polyethylene glycol and/or gastrogastrin enemas could be used.
  • Consider treatment of SBBO as well which is frequent with CF.

67 Sonia Michail, MD, Children’s Hospital Los Angeles Update on C. difficile

The slide I liked the best was showing a change in microbiome after FMT which is not in syllabus.

82 Ed Hoffenberg, MD, Children’s Hospital Colorado  What the pediatric GI provider needs to know about cannabis

Disclaimer: NASPGHAN/gutsandgrowth assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. The discussion, views, and recommendations as to medical procedures, choice of drugs and drug dosages herein are the sole responsibility of the authors. Because of rapid advances in the medical sciences, the Society cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure. Some of the slides reproduced in this syllabus contain animation in the power point version. This cannot be seen in the printed version.

Cystic Fibrosis Expert Update 2017

During one of our recent group clinical meetings, one of my partners (Edith Pilzer, MD) presented an update on cystic fibrosis.

Here is a link to her slides:Link: cf-presentation

Here are a few of my notes:

There has been a great improvement in survival of cystic fibrosis patients..  From the Cystic Fibrosis Foundation:

  • ” Today the median predicted survival age is close to 40. This is a dramatic improvement from the 1950s, when a child with CF rarely lived long enough to attend elementary school.”

cftr2org  —website provide information on specific genotypes, including whether genotype is associated with pancreatic insufficiency

From the website:

  • This website provides information for members of the general public, including cystic fibrosis patients and their family members, about what is currently known about specific genetic variants related to cystic fibrosis.
  • Patients and their family members are encouraged to visit the section, “For patients and family members” first.
  • This website also provides more in-depth research-related information for health care professionals and researchers

Pancreatic enzyme replacement therapy (PERT) (see slides)

  • Creon 3000 beads are small enough to go through Gastrostomy tube
  • Pertzye has bicarbonate; thus, additional acid blocker administration is usually not needed
  • Viokase is hard to obtain
  • Relizorb –external lipase cartiledge.  This allows formula, delivered by NG, to run through column and obviates the need for additional PERT dosing.  One cartridge designed for 500 cc but several cartridges can be ‘piggy-backed.’ Here is website: relizorb.com.  Relizorb intent is to eliminate enzymes for night feeds, though it only has lipase; yet, there still could be a need additional PERT for protease and amylase.  Potentially PERT could be administered before or after and hopefully avoid awakening at night for enzymes..

Cystic Fibrosis Related Diabetes (CFRD)

  • Frequent reason for poor growth
  • Now, with increased survival, ~35% of Cystic Fibrosis patients develop CFRD

Distal Intestinal Obstruction Syndrome (DIOS)

  • If mild, treatment with miralax is reasonable
  • If vomiting, consider surgery consult
  • If more than mild, consider water-soluble enema with 10% mucomyst

My take: Great update.  Edith has been taking care of children with cystic fibrosis for more than 30 years and has witnessed/participated in the improvement in the survival of these patients.

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.

 

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