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