Postgraduate Course Notes -Pancreatitis Module

When and how to assess pancreatic function: an update for clinicians –Sohail Z. Husain, MD (page 31)

Reviewed methods of detecting pancreatic insufficiency

Indirect (non-stimulatory) Methods

Stool:

  • Fecal Fat Analysis: Coefficient of fat absorption (CFA): (fat intake – fat in stool / fat intake) * 100
  • Normal > 93% (> 85% in less than 6 mo.
  • old)
  • 72 hr collection gold standard

ELASTASE-1: Stable, specific for human pancreas

  • Normal > 200 μg elastase/g stool
  • Particularly good for monitoring the development of PI in patients with CF
  • Low levels (false-positive) with diarrhea
  • Only detects severe PI

Other tests

  • Chymotrypsin: less sensitive; requires discontinuation of enzymes
  • Steatocrit: cheap; has low sensitivity
  • Serum
  • Breath
  • Direct (stimulatory)
  • Dreiling tube
  • Endoscopic pancreatic function testing (ePFT)
  • Secretin-enhanced MRCP (sMRCP)

Causes of Pancreatic Insufficiency

-85% of patients with Cystic Fibrosis have pancreatic insufficiency

Shwachman-Diamond Syndrome

  • Mutation in SBDS, found in ~90% of SDS patients
  • PI affects almost all SDS pts

Johanson-Blizzard syndrome (JBS): Key findings

  • PI
  • Severe developmental delay
  • Hypoplasia or aplasia of the nasal wings

Pearson marrow pancreas syndrome Key findings: Severe hypoplastic,macrocytic anemia,  Pancreatic insufficiency (due to pancreatic fibrosis)

Diagnosis: Clinical picture, High serum lactate/pyruvate,  Southern blot for mtDNA rearrangements

Other causes of pancreatic insufficiency

  • Chronic pancreatitis
  • Pancreatic obliteration after severe, acute pancreatitis
  • Pancreatic tumors
  • Celiac disease
  • Diabetes
  • IBD

Managing nutrition in cystic fibrosis: the role of the pediatric gastroenterologist  Sarah Jane Schwarzenberg, M.D. (page 41)

Good nutrition status correlates with better heights, better lung function, and better survival.  (Presentation did not delve into the issue of potential reverse causation.)

  • Patients with a Weight-for-Age percentile >50% at age 4 years reached a much higher height-for-age early in life and maintained this advantage into adulthood
  • Pulmonary function (FEV1%predicted) was much lower in CF patients with WAP<10% at age 4 years. This finding tracked through age 18 years.
  • Small bowel overgrowth is common in CF
  • Small bowel bacterial overgrowth contributes to poor nutritional intake and increased nutrient losses

Options to improve nutrition in CF

  • Review and optimize enzyme dose and adherence
  • Review patient’s diet with an experienced CF dietician
  • Consider adding a PPI to improve intestinal pH
  • Consider confounding disease
  • Evaluate for signs and symptoms of small bowel overgrowth and consider trial of metronidazole or rifaximin
  • Ask patient about abdominal pain
  • Evaluate for gastroparesis
  • Evaluate for DIOS
  • Consider non-CF gastrointestinal disease
  • Consider oral glucose tolerance test

Therapy to improve nutrition

  • Time-limited interventions
  • Behavior therapy to improve intake
  • Offer oral supplements
  • Consider cyproheptadine as an appetite stimulant
  • Consider a G-tube for nocturnal feeds
  • Consider Endocrinology consult

Beyond the Basics in the Management of Pancreatitis  –Aliye Uc, M.D. (page 51)

INSPPIRE To Study Acute Recurrent and Chronic Pancreatitis in Children-180 children from 14 centers enrolled to study the etiologies, epidemiology, natural history and outcome.

Pediatric Acute Recurrent and Chronic Pancreatitis-etiologies

  • Genetic (49%) (61 of 91 tested)
  • PRSS1-30%, CFTR-22%, SPINK1-14%, CTRC-3%
  • Obstructive (34%)
  • Idiopathic (20%)
  • Toxic-Metabolic (17%)
  • Autoimmune (3%)

Genetics of Pancreatitis:

1. PRSS1 (cationic trypsinogen): Autosomal dominant, 80% penetrance, Mutations are due to increased activation or decreased inactivation of trypsin (i.e. R122H, N29I).

2. SPINK1 (trypsin inhibitor): Autosomal recessive/complex inheritance, 2% have mutation, <1% have pancreatitis (i.e. N34S), Pancreatitis is dose-related (homozygous>>>het), Associated with other mutations (CFTR)

3. CFTR (>1700 mutations):

  • 2 Severe mutations = Cystic Fibrosis
  • 1 severe, 1 mild mutation = mild or atypical CF, ARP, CP
  • CF carriers = 3-4 fold increase risk in pancreatitis.
  • 1 any +SPINK1 = CFTR-associated pancreatitis
  • 1 any +divisum = CFTR-associated pancreatitis

4.New Modifier Genes in ARP and CP

  • CTRC (trypsin degrading enzyme)
  • CASR (a calcium-sensing receptor)
  • CLDN2 (tight junction protein on X chromosome)
  • CPA1 (Carboxypeptidase 1)  increased riskf for CP in younger patients

Management:

  • Unclear if antioxidants helpful for pain.
  • The role of pancreatic enzymes in CP is equivocal.

Diet

  • When to start feeds? depends on the severity of AP, OK to start early; correlate with clinical readiness, abd pain
  • What mode of nutrition? prefer enteral over TPN, NG vs. NJ
  • What to feed?  recent studies in adults with mild AP support full diet

(Moraes JM et al. J Clin Gastroenterol 2010 44:517)

No evidence that low-fat diet is helpful

IV Fluids: With acute presentation, Lactated Ringer’s preferred over Normal saline.

NG Suction

  • Not shown to decrease symptoms,mortality or hospital stay.
  • May be useful if: severe gastric distention, refractory nausea and vomiting, or obstruction seen on abdominal x-ray

2 thoughts on “Postgraduate Course Notes -Pancreatitis Module

  1. Pingback: Chronic Pancreatitis in Pediatrics -Descriptive Study | gutsandgrowth

  2. Pingback: Briefly Noted: Shwachman-Diamond | gutsandgrowth

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

This site uses Akismet to reduce spam. Learn how your comment data is processed.