In this case report, a 38 yo was incidentally discovered to have a purple liver while undergoing surgery for cholelithiasis. A diagnosis of Dubin-Johnson syndrome was made. It “is a benign autosomal-recessive liver disease with clinical manifestations of chronic or intermittent conjugated hyperbilirubinemia. It is caused by mutations in the ABCC2 gene leading to MRP2 [multidrug resistance-associated protein 2] dysfunction or deletion.”
This retrospective study dating back to 1988 examined 1007 patients diagnosed with CD who were followed up for a median duration of 8.8 years.
Key findings:
The risk for intestinal resection at 5 years decreased significantly over time (P1, 35%; P2, 31%; and P3, 22%; P = .0003. This decrease in resections coincided with increased use of immunosuppressive (IS) and anti-TNF therapy: IS and anti-TNF exposure rate at 5 years increased from 33.9% (in P1) to 76.5% (in P3) and from 0% (in P1) to 50.5% (in P3).
The risk for progression from inflammatory to stricturing behavior decreased significantly over time (P1, 27%; P2, 28%; and P3, 20%)
LE Targownik et al. Clin Gastroenterol Hepatol 2022; 20: 2607-2618. Earlier Anti-TNF Initiation Leads to Long-term Lower Health Care Utilization in Crohn’s Disease but Not in Ulcerative Colitis
Methods: The authors “used health administrative data from Manitoba, Canada to identify all persons with a new diagnosis of inflammatory bowel disease (IBD) between 2001 and 2018 who received tumor necrosis factor antagonists (anti-TNF) therapy and had at least 1 year of post anti-TNF initiation follow-up.”
Key findings:
Among 742 persons with CD, early anti-TNF initiators had fewer IBD-specific and overall hospitalizations over the 5 years following the start of therapy
Incidence of resective surgery was also lower in earlier anti-TNF initiators with CD if the first year following initiation was excluded from the analysis.
In 318 cases of UC, there was no impact of the timing of anti-TNF therapy on the rates of hospitalization and surgery.
My take: These two studies show that use of biologic therapy is associated with better outcomes in Crohn’s disease including fewer intestinal resections and fewer hospitalizations. It appears that earlier use may alter the natural history in part by reducing the likelihood of stricturing disease. Interestingly, the RISK study showed a reduction in penetrating disease with early use of biologics but not a reduction in stricturing disease (Related blog post: CCFA: Updates in Inflammatory Bowel Disease 2017 (part 3))
This article has 91 authors! Using Delphi surveys, the authors recommend the following:
“EGID” was the preferred umbrella term for disorders of gastrointestinal (GI) tract eosinophilic inflammation in the absence of secondary causes
Involved GI tract segments will be named specifically and use an “Eo” abbreviation convention: eosinophilic gastritis (now abbreviated EoG), eosinophilic enteritis (EoN), and eosinophilic colitis (EoC)
For EoN, “it is desirable, but not required, to name specific locations of small bowel involvement, if these are known…The abbreviation for eosinophilic duodenitis should be “EoD”… for eosinophilic jejunitis should be “EoJ”….eosinophilic ileitis should be “EoI”
The term “eosinophilic gastroenteritis” is no longer preferred as the overall name (but can be used to indicate involvement of both the stomach and small bowel)
When >2 GI tract areas are involved, the name should reflect all of the involved areas
There has been a lot written about ChatGPT (see some NY Times links below). Earlier this week, one of my sons placed the following prompt:
“Write me a funny poem about a doctor from Atlanta who deals with diarrhea all day long”
This is the ChatGPT response:
There once was a doctor from Atlanta Whose patients all suffered from diarrhea They came in by the dozens Leaving behind a stench that was quite humbling But this doctor was quite unflappable
He had a cure for every case And a smile on his face As he worked tirelessly To help each patient feel less queasy
His office was filled with the sounds of relief As patients left feeling much less grief They praised his skill and his bedside manner And promised to spread the word about this Atlanta diarrhea-banner
So if you’re in Atlanta and feeling quite ill And your stomach is acting quite shrill Don’t hesitate, don’t be shy Just make your way to this doctor’s side And say goodbye to that diarrhea for good!
“It can extrapolate and take ideas from different contexts and merge them together.” The new chatbots do this with what seems like complete confidence. But they do not always tell the truth. Sometimes, they even fail at simple arithmetic. They blend fact with fiction. And as they continue to improve, people could use them to generate and spread untruths.
My take: ChatGPT (& similar programs) is a huge technological advance. In educational settings, it could help explain complicated topics. Of course, there is a concern that it could undermine education if students do not go through the process of doing their own work. In healthcare, it is possible that these programs could advance patient education and help in the diagnostic process.
Another example (on 12/16/22) -this took less than 30 seconds:
In this prospective study, pediatric patients with NAFLD received intensive lifestyle therapy which encompassed caloric restriction, physical activity, education on a healthy lifestyle, and psychosocial support. Key findings:
After 6 months, the median body weight loss was 16.0% in the 167 patients evaluated. Fibrosis improved in 75.0% (P < .001).
Fasting serum alanine aminotransferase and homeostasis model assessment of insulin resistance decreased significantly over the 1-year period (P < .001).
Related article: MH Malespin et al. Clin Gastroenterol Hepatol 2022; 20: 2393-2395. In this study, adults who were enrolled in TARGET-NASH (n=2019) were reviewed. Only 32% of overweight/obese adults with NAFLD receiving usual care in the U.S. achieved weight loss of 5% or more. In addition, only 25% who lost 5% or more of their weight maintained weight loss at follow-up at 5 years.
My take: Together these studies show that weight loss can reverse liver damage (steatosis and fibrosis) in NAFLD; however, maintaining weight loss with lifestyle treatment is quite difficult. As such, it is anticipated that newer pharmacologic agents (eg. semaglutide) will be available (after appropriate trials), especially for more severe NAFLD.
This retrospective review examined health care utilization within 3 months before and after patients received a constipation action plan (CAP which was implemented in 2019). There were 336 patients who received a CAP and 2812 patients who did not.
Key findings:
There were fewer patient telephone calls for patients who received the CAP (P = 0.0006). The difference in patient electronic messages was not statistically significant (P = 0.09).
There were no differences in UC/emergency department visits or hospitalizations.
Medications for CAP included the following:
Polyethylene glycol 3350, Senna, Milk of Magnesia, Magnesium Citrate, Lactulose, Bisacodyl (tablets, suppository, enema), Normal saline enema, Glycerin suppository, and Sodium phosphate enema
My take: A CAP likely helps educate and empower families on how to manage their child’s symptoms. In this study, it resulted in fewer phone calls (& trend of less electronic messages). Better education is likely to help with patient outcomes even if this is difficult to prove in a retrospective study. This action plan appears easier to understand than a previous CAP, and uses the Red Zone as the cleanout section. Related blog post:Pictographic Constipation Action Plan (2021)
This is a sample of the institution’s constipation action plan (similar to Figure 1) shared by the author.
FH Bloomfield et al. NEJM 2022; 387: 1661-1672. Early Amino Acids in Extremely Preterm Babies
My take: For preterm infants <1000 gram, this study showed that the usual dosing of amino acids 2.5 gram to 3.5 gram per day (the placebo group) appeared to have better secondary outcomes (though within confidence intervals) than those who received an additional 1 gram per day.
Methods: In this 5-year retrospective study, the authors identified 22 patients with NRCD; they were following a gluten-free diet for at least 12 months but had persistent symptoms and enteropathy (Marsh 3). Treatments for NRCD were either a GCED (n=13), budesonide (n=9) or both (n=4). Four patients were lost to follow-up and did not receive either treatment.
Key findings:
Thirteen were treated with the GCED for 3 months with 46% achieving both histological and symptomatic resolution
Nine patients were treated with budesonide (6–9 mg daily), with 89% achieving both symptomatic and histologic resolution after a median 3-month treatment course
67% of patients who responded to the GCED and 100% of patients who responded to budesonide remained in remission for at least 6 months following treatment transition back to exclusive GFD
My take: This important article shows that many patients thought to be receiving a GFD can respond to a more stringent approach. In addition, it offers an alternative strategy with budesonide which had a high response rate.
In patients believed to have celiac disease who have persistent or recurrent symptoms or signs, the initial diagnosis of celiac disease should be confirmed by review of prior diagnostic testing, including serologies, endoscopies, and histologic findings.
Best Practice Advice 2
In patients with confirmed celiac disease with persistent or recurrent symptoms or signs (nonresponsive celiac disease), ongoing gluten ingestion should be excluded as a cause of these symptoms with serologic testing, dietitian review, and detection of immunogenic peptides in stool or urine. Esophagogastroduodenoscopy with small bowel biopsies should be performed to look for villous atrophy. If villous atrophy persists or the initial diagnosis of celiac disease was not confirmed, consider other causes of villous atrophy, including common variable immunodeficiency, autoimmune enteropathy, tropical sprue, and medication-induced enteropathy.
Best Practice Advice 3
For patients with nonresponsive celiac disease, after exclusion of gluten ingestion, perform a systematic evaluation for other potential causes of symptoms, including functional bowel disorders, microscopic colitis, pancreatic insufficiency, inflammatory bowel disease, lactose or fructose intolerance, and small intestinal bacterial overgrowth.
Best Practice Advice 4
Use flow cytometry, immunohistochemistry, and T-cell receptor rearrangement studies to distinguish between subtypes of refractory celiac disease and to exclude enteropathy-associated T-cell lymphoma. Type 1 refractory celiac disease is characterized by a normal intraepithelial lymphocyte population and type 2 is defined by the presence of an aberrant, clonal intraepithelial lymphocyte population. Consultation with an expert hematopathologist is necessary to interpret these studies.
Best Practice Advice 5
Perform small bowel imaging with capsule endoscopy and computed tomography or magnetic resonance enterography to exclude enteropathy-associated T-cell lymphoma and ulcerative jejunoileitis at initial diagnosis of type 2 refractory celiac disease.
Best Practice Advice 6
Complete a detailed nutritional assessment with investigation of micronutrient and macronutrient deficiencies in patients diagnosed with refractory celiac disease. Check albumin as an independent prognostic factor.
Best Practice Advice 7
Correct deficiencies in macro- and micronutrients using oral supplements and/or enteral support. Consider parenteral nutrition for patients with severe malnutrition due to malabsorption.
Best Practice Advice 8
Corticosteroids, most commonly open-capsule budesonide or, if unavailable, prednisone, are the medication of choice and should be used as first-line therapy in either type 1 or type 2 refractory celiac disease.
Best Practice Advice 9
Patients with refractory celiac disease require regular follow-up by a multidisciplinary team, including gastroenterologists and dietitians, to assess clinical and histologic response to therapy. Identify local experts with expertise in celiac disease to assist with management.
Best Practice Advice 10
Patients with refractory celiac disease without response to steroids may benefit from referral to a center with expertise for management or evaluation for inclusion in clinical trials.
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My take: While the prices are very good, I know some have had trouble working with the online pharmacy to get their prescriptions filled in a timely manner.
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