Pancreas Pipe Problems
There is a patient with CF in the ED. I tell the ED doc these criteria are needed to diagnose pancreatitis...
What are 1) Epigastric pain, 2) Imaging, 3) Lipase 3x or greater than ULN
2 of the 3 must be present
20% of patients with CF get pancreatitis
A FT baby boy was transferred to a tertiary care NICU at 30 hours of age because of abdominal distention, failure to pass meconium, bilious emesis. The neonatologist sees this on the AXR. (NEJM 3/26/25)
1. Distended small bowel loops +/- air fluid levels due to a meconium ileus
2? Calcifications from peritonitis in utero if perforated
3? Inspissated meconium (granular/sandy)
15% of patients with CF get gallstones. These are 3 presentions of gallstones.
what are 1) biliary colic, 2) cholecystitis/pancreatitis, 3) asymptomatic
Four most common vitamin deficiencies in CF.
A, D, E, K
the fat soluble vitamins!
The month your pediatric GI fellowship application is due.
When is August of 3rd year?
Two reasons that pancreatic insufficiency occurs in CF. Three if you are feeling extra.
What are 1. thick secretions that clog the ducts so less enzymes are released to the lumen, and 2. decreased bicarb content of secretions degrades some of the enzymes, and 3. auto digestion of parenchyma due to recurrent pancreatitis (less impactful reason)
This procedure is used to treat meconium ileus.
What is serial hyperosmolar gastrograffin enemas
*risk of perforation
40% of patients with CF develop liver disease in their lifetime (20% during childhood/adolescence). This includes meeting 2 criteria of transaminitis, hepatomegaly, US abnormality (steatosis) and bx finding). 20% of this subset develop advanced liver disease. This is a leading cause of death in CF (3%). CF may cause liver disease for these reasons.
?Prolonged cholestasis --> constant inflammation
?CFTR mutation is directly pro-inflammatory in the liver
?Disrupted epithelial barriers allow gut microbiota to enter portal circulation
This type of diet is recommended for patients with CF.
high fat! 40% calories
high calories -- 1.5-2x recommendations
The month you interview for your GI fellowship
When is Sept/Oct of your 3rd year?
The pathognomonic clinical sign of pancreatic insufficiency.
What are greasy stools.
Also -- colicky abdominal pain, malabsorption of nutrients particularly A,D,E,K, and FTT
I hear the neonatologist comment that the baby transferred to the NICU with delayed meconium passage as a "complicated MI." I dont know what that means. My senior resident tells me to look for... and to call...
Intestinal perforation, Meconium peritonitis
Volvulus
Atresia
We need a surgeon -- note, short gut can be detrimental to success in CF.
Patients with CF are monitored annually for CFLD including labs (ALT, AST, GGT, Platelets) and assessment of hepatosplenomegaly. How do you know if a liver is enlarged?
- Extends beyond the right costal margin without inhalation in teen; extends >3 cm beyond the margin in children < 2 years old
- Average liver span at MCL is 4-5 cm (neonates), 6-7 cm (12-year-old girls), 7-8 cm (12-year-old boys), and up to 16 cm (adults).
- Ultrasound with age-specific standards
My patient has CF and FTT. They eat double the recommended calories for a child their age and size. They have no stool problems or abdominal pain. This will be my next step.
GT
Start this conversation early
The person you look up and contact to arrange an away rotation before you apply to your pediatric GI fellowship.
Who is the resident rotation coordinator?
Stanford: Meagan Christofferson
UCSF: Catherine Le
...ideally start this communication 6 moths in advance
My patient with CF's growth chart has started to plateau and they have having abdominal pain and bloating after meals. I order this test to check for pancreatic insufficiency and expect it to be (high? low?) because....
Fecal elastase = measures instrinsic function of the pancreas
>200 µg/g - normal
<100 µg/g - severe *be careful about dilution
10 yo with CF has abdominal pain and flatulance. AXR shows large rectal/colonic stool burden. In addition to updating his PERT, I start him on this medication.
Miralax!! (and Senna!)
50% of children with CF have constipation due to fluid composition, dysmotility and PI.
Risk for rectal prolapse (though less common with advances in PERT)
Advanced CF-Related liver disease involves portal hypertension with or without cirrhosis (more likely in children). This illustration explains how PHT can cause variceal bleeding, low platelets and ascites.
Lower esophageal varices from left gastric vein congestion
Congestive splenomegaly via splenic vein
Transudative ascites via SMV
These three enzymes make up PERT
Lipase: Breaks down fats <--dosing is based on lipase
Protease: Breaks down proteins
Amylase: Breaks down carbohydrates
Your top 5 reasons for wanting to become a gastroenterologist/hepatologist!
1. Intellectually stimulating -- food, microbiology, autoimmune disease, holistic
2. Cutting edge treatments -- biologics, transplant, GLP-1RAs?
3. Interdisciplinary -- RD, psychology/psychiatry, rheumatology, ID, surgeons, integrative medicine
4. Procedural -- EGD, colonoscopy, rectal bx, pH probes, IBStim
5. Whole life spectrum -- NICU --> college = continuity of care
6. Health Equity -- many GI disorders disproportionately affect children with food insecurity
7. Spectrum of acuity -- constipation to transplant and intestinal rehab
8. Academic and research opportunity -- highly academic, multiple advanced fellowships
9. Work-life balance -- 33% burnout rate, 85% would choose it again (2023)
10. The people!!
A medical student on my team asks, if the pancreas's sticky secretions causes itself to autodigest over time and reduces its exocrine function, is endocrine function also impaired or spared? I explain the following...
What is CF-related diabetes?
- destruction of the islets of langerhans
- cannot rely on hga1c in CF because RBC turnover is higher! Must use glucose tolerance testing
- Affects 2% of children, 19% of adolescents, and up to 50% 30+ years with CF
- treated with insulin
A 10 yo with CF is in the ED with abdominal pain and distension. AXR shows dilated small bowel loops and an accumulation of stool in the area of the cecal valve. This happened.
What is DIOS -- Distal obstruction syndrome?
More likely with PI and poor PERT adherence -- extra-fatty chyme. Also poor underlying motility, opiate exposure, and poorly controlled constipation.
Tell us the story of the baby in the PICU right now who has CF.
Neonatal cholestasis of CF??
Biliary atresia??
Gilberts??
My patient has been taking a boatload of PERT enzymes for so many years. I am worried about...!?
What is fibrosing colonopathy?
Stricturing fibrosis of the colon that was common in the 1990s. Now, maximum PERT dose is limited to 2,500 lipase units/kg/meal (or 10,000 lipase units/kg/day) to prevent this complication.
The name of the program for 2nd year residents interested in pediatric gastroenterology.
What is "Teaching and Tomorrow" at the NASPGHAN Conference Oct/Nov --- first years, you are next!