Online Aspen Webinar (Part 1)

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Online Webinar –Annual Aspen Conference  —July 14, 2000

Below I’ve included a few slides and some notes; my notes may have errors of omission or transcription.

Can We Skip Liver Biopsies in Infants with Cholestasis? Jim Squires

Key points:

  • Data suggest that cholestasis in infants needs to be defined as direct bilirubin/conjugated bilirubin >0.3 (if TB <5) or 10% if TB >5.
  • Identifying cholestasis is challenging as cholestasis occurs in ~1 in 2500 whereas jaundice occurs in 15% of all infants
  • Genetic testing (eg. Cholestasis Panel, or exome) needs to be moved up earlier in diagnostic algorithm, after ultrasound completed and after A1AT & biliary atresia considered

Related blog posts:

Not part of webinar:

More School Advice for Organ Transplant Recipients, Plus Another Benefit of the Influenza Vaccine

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Link to PDF (from Pediatric Infectious Disease Society:

FAQs Regarding Return to School for Children after Solid Organ Transplant in the United States During the COVID-19 Pandemic

Some excerpts:

Are pediatric SOT recipients at higher risk for getting COVID-19 compared with other children?
Children of any age can get COVID-19, but they seem to have milder disease than adults. Pediatric SOT recipients do not seem to get COVID-19 more often than other children.

If infected with COVID-19, are pediatric SOT recipients at higher risk for developing severe disease or complications?

Based on experience with other viruses, and from reports of COVID-19 in adult SOT patients, there are a few things that may increase the risk of severe COVID-19. These include:
1) Having undergone transplantation in the last 3-6 months
2) Receiving high doses of immunosuppression (such as for treatment of rejection)
3) Having other medical problems such as diabetes, obesity, or certain lung conditions (refer to CDC website under Helpful Resources for more details)
It is not known if the above factors also put children with SOT at risk. In fact, of all the reports among pediatric SOT recipients with COVID-19 published so far, the majority have had mild symptoms and recovered.

Related blog posts:

Why Physician Burnout Is Happening & How to Fix It

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A recent commentary (P Hartzband, J Groopman. NEJM 2020; 382: 2485-87) provides critical insights into the issue of physician burnout.

Full Text: Physician Burnout, Interrupted

Some excerpts (bold =my highlights):

Initially, the prevailing attitude was that burnout is a physician problem and that those who can’t adapt to the new environment need to get with the program or leave….The unintended consequences of radical alterations in the health care system that were supposed to make physicians more efficient and productive, and thus more satisfied, have made them profoundly alienated and disillusioned…

Solutions have largely targeted the doctor, proposing exercise classes and relaxation techniques, snacks and social hours for decompressing, greater access to child care, hobbies to enrich free time, and ways to increase efficiency and maximize productivity. There is scant evidence that any of these measures have had a meaningful impact…

Medicine is in many ways unique. Doctors, nurses, and other health care professionals have traditionally viewed their work as a calling. They tend to enter their field with a high level of altruism coupled with a strong interest in human biology, focused on caring for the ill. These traits and goals lead to considerable intrinsic motivation. In a misguided attempt to improve the medical system, health care reformers put into place various positive and negative extrinsic motivators, without realizing that they would actually erode and destroy intrinsic motivation, eventually leading to “amotivation” — in other words, burnout...

Gagné and Deci posit that there are three pillars that support professionals’ intrinsic motivation and psychological well-being: autonomy, competence, and relatedness.3 All three have been stripped away as a direct result of the restructuring of the health care system.1  …

Evidence from the meta-analysis of controlled interventions supports the restoration of autonomy; giving doctors flexibility in their schedule to allow for individual styles of practice … The EHR … must be reconfigured to work for physicians rather than forcing physicians to work for it….

Competency can be restored by purging the system of meaningless metric…Relatedness should be authentic, aligning the system’s values with those of physicians, nurses, and other health care professionals

My take: Flexibility in scheduling is a crucial element for satisfaction.  Competency, which in my view equates to high quality care, is the other crucial element.

Audio Interview Link (11 minutes):  Audio Interview with Dr. Pamela Hartzband

Related blog posts:

Lingering Histologic Changes with Eosinophilic Esophagitis in Remission, Plus One

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A recent study (KA Whelan et al. Clin Gastroenterol Hepatol 2020; 18: 1475-82) examined esophageal histology in 243 patients (mean age 16.9 years) in 3 groups: active eosinophilic esophagitis (EoE), inactive EoE (<15 eos/hpf), and a control non-EoE group.

Key findings:

Basal cell hyperplasia and spongiosis were present in 43 (29%) and 109 (74%) respectively of patients with inactive EoE. In comparison, these findings were present in 98% and 100% respectively of those with active EoE and in 6% and 33% of non-EoE patients

My take: This study provides some insight into the idea that esophageal damage may be ongoing in the absence of eosinophils.  These histologic findings could provide part of the reasons for symptoms in those who have had resolution of esophageal eosinophilia.

Related study: ES Dellon et al. Clin Gastroenterol Hepatol 2020; 18: 1483-92. This study showed rapid recurrence of eosinophilic esophagitis after discontinuation of topical steroids.  33/58  (57%) had symptom recurrence before 1 year (median time 244 for symptoms). At time of symptom recurrence, 78% had histologic relapse (≥15 eos/hpf).

Related blog posts:

Current Thinking with Laryngopharyngeal Reflux Symptoms

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A recent study (H-C Lien et al. Clin Gastroenterol Hepatol 2020; 18: 14-66-74) adds a bit more insight into the topic of larygnpharyngeal symptoms (related blog post:  Gastroestophageal Reflux Phenotypes and Where ‘Rome, Lyon, and Montreal Meet’ provides more information on treatment outcomes).

Methods: In this prospective multi-center observational study with adults aged 20-70 years, n=142 completed study), enrollment required chronic laryngitis symptoms >3 months and “laryngoscopic” signs suggestive of reflux.  Subsequently, patients were examined with multiple modalities, including 24-pH testing, manometry, and Bernstein test followed by treatment with omeprazole 40 mg twice a day.

Key Findings:

  • Pathologic reflux was identified in 146/252 (58%) of those meeting inclusion criteria.  Thus, approximately 40% did NOT have objective findings of reflux despite suspicion of laryngopharyngeal reflux (LPR); this is similar to other studies.
  • In those with documented reflux, those with and without typical reflux symptoms had improvement in LPR with omeprazole therapy: 57% and 63% respectively; whereas, omeprazole therapy was effective in 32% in those without objective (pH probe) findings of reflux. In previous studies, reflux laryngitis response to PPIs has been similar to placebo.

My take: Typical reflux symptoms are not needed for patients with LPR to respond to PPIs.  However, more than 40% of individuals with LPR do NOT have objective evidence of reflux; in this subset, response to PPI therapy is low.

Related blog posts:

Briefly Noted: Esophageal Stricture Dilatations

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SJ Clark et al. JPGN 2020; 71: e1-e5. Examining the “Rule of 3” for Esophageal Dilations in Pediatric Stricture Patients.  In this retrospective study with 284 patients and 1384 balloon dilatations, the authors examined the perforation rate compared to the delta dilation diameter (ΔDD).  They note that the “rule of 3” is a 40-year-old expert opinion that suggests dilating an esophageal stricture more than 3 mm is unsafe. In this cohort, the perforation rate was 1.66% overall; there were 8 perforations in 1075 dilations with ΔDD ≤5 mm (0.7%) and 15 perforations in 309 dilations with ΔDD >5 mm (4.9%). The authors indicate that dilatations ≤5 mm may be acceptable.

Solitary Rectal Ulcer Syndrome: How Often is It Solitary? How Often is There an Ulcer?

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A recent restrospective review of 140 pediatric cases (median age 12 years) of solitary rectal ulcer syndrome (SRUS) (U Poddar et al. JPGN 2020; 71: 29-33) highlights the fact that in many, there are multiple ulcerations and in some there are none.

Key findings:

  • Most had dsynergic defecation with prolonged sitting on the toilet (94%), excessive straining (98%), feeling of incomplete evacuation (93%) or “rectal digitation” (51%)
  • Rectal bleeding was presenting feature in 94%
  • Colonoscopy showed in 72% (n=101); a single ulcer was noted in (60%) (n=84)  -thus in those with an ulcer, 83% were solitary.
  • Of the 113 with adequate followup, 63% had clinical improvement and healing of ulcer was documented in 36/82 (44%)
  • The most common treatment was hydrocortisone enema with bulk laxative (n=73) with “improvement” in 52, “better” in 16, and no response in 5 (8.2%).  Other frequent treatments: sulfasalazine enema with bulk laxative (n=12), and bulk laxative alone (n=22)
  • Most children (95/140) were older than 10 years; only 2 were ≤5 years

My take: Asking carefully about dysnergic bowel habits will make this diagnosis much easier.  Many children with SRUS have erythema and not a solitary ulcer; in addition, lesions can be ulcerative or polypoid.

Related blog post: One more cause of rectal bleeding

Does The Degree of Villous Atrophy Affect Long-Term Outcomes with Celiac Disease? Plus One

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S Kroger et al. JPGN 2020; 71: 71-7.  This study, conducted from 1966-2014, examined long-term outcomes of individuals diagnosed with celiac disease in childhood. This study examined 906 children and sent questionnaires to 503 adults (212 responded) who were diagnosed in childhood.

Key findings:

  • More recent diagnosis (after 2006) has been associated with children having milder lesions, more often diagnosis due to screening (rather than symptoms) 30% vs. 25%, less anemia (16% vs 21%), less growth disturbances (22% vs. 36%), and lower TTG-2 titers (mean 64 U/L vs 120 U/L.
  • Among adults completing questionnaire, severity of villous atrophy at childhood diagnosis did not predict complications, persistent symptoms, quality of life, or adherence with glute-free diet

RV Lopez et al. JPGN 2020; 71: 59-63. Influence of the 2012 European Guidelines in Diagnosis and Follow-up of Coeliac Children With Selective IgA Deficiency. This study showed that the guidelines were used incorrectly for individuals with selective IgA deficiency, using a 10-fold elevation of TTG IgG instead of TTG IgA. The associated editorial (pg 2 by P Gillett) recommends “we should perform endoscopy in all sIgAD patients.”  In addition, this population may merit followup endoscopy at much lower threshold due to difficulty using serology for follow-up.

My take: Children with severe villous atrophy due to celiac disease can respond fully to a gluten-free diet.  In the small subset of individuals with selective IgA deficiency, a no-biopsy diagnosis is not recommended.

Related blog posts:

“Health Insurance Is Broken”

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From The Onion:

Almost every Sunday, I spend hours reading the local AJC and the NY Times — a great way to start the week.

On July 5th, the NY Times review section discussed the topic of ‘The Economy We Need.’ The article discussed rising problems with wealth inequity and associated problems including higher mortality rates.  The first article states that “if income had kept pace with overall economic growth since 1970, Americans in the bottom 90 percent of the income distribution would be making an extra $12,000 per year, on average.”

The greater inequities are the direct result of policy changes and can be changed. The subsequent articles detail myriad issues from tax policy that bolster wealth inequality (eg. lower inheritance taxes), minimum wage which requires many to rely on government assistance, legacy admissions to elite colleges, corporation stock buybacks (which come at the expense of lower benefits/wages), policies that discourage unions, and corporations that have stopped profit sharing (except for the highest-paid executives).

The article with the most relevance for health care providers: NY Times: “Health Insurance Is Broken” by Jeneen Interlandi. Link to online version: Employer-Based Health Care, Meet Massive Unemployment

Key points:

  • “The nation spends an average of $3.5 trillion on health care –more than Japan, Germany, France, China, the United Kingdom, Italy, Canada, Brazil, Spain, and Australia combined — ant still loses more people to preventable and treatable medical conditions than any of those countries do…America has created the most expensive, least effective health care system in the modern world.”
  • The problem has deep roots.  One of the issues with the cost, at least historically, has been “price insensitivity.”  “If the insurer is paying, nobody looks at the bill.”…Hospitals ramped up equipment purchases, workforces, specialty clinics and “passed [the price hikes] from hospitals to insurers to customers.”‘
  • “Employer-based insurance is heavily subsidized by the federal government …[and] are not much different than the ones granted to low-income Americans through Medicaid and the Affordable Care Act.”
  • “The country will have to stop making employers the sole source of health care for so many people.”  Alternatives could include single-payer system or ‘public option.’

My take: The U.S. health care system costs too much and has gaping problems with lack of coverage and inequities. These problems have worsened with the current pandemic.

Related article: NY Times:  The U.S. Is Lagging Behind Many Rich Countries. These Charts Show Why.

Related blog posts:

Renal Disease Associated With Fatty Liver Disease & Dexamethasone-COVID-19 Data

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Looking for and managing hypertension has been an important component of care in children and adults with nonalcoholic fatty liver disease (NAFLD)/metabolic syndrome.  In addition, hypertension is frequently associated with renal impairment.

As such, it is perhaps not surprising that in both adults and children, there is a high rate of renal impairment.   The data in children is much more sparse than in adults.  A recent retrospective pediatric cohort study (T Yodoshi et al. J Pediatr 2020; 222: 127-33) adds more information to this problem.

More background information:

  • Chronic kidney disease is highly prevalent in adults with NAFLD: 20-55% (J Hepatol 2020; 72: 785-801; Am J Kidney Dis 2014; 64: 638-52)
  • NAFLD is currently the leading indication for concurrent liver and kidney transplantation
  • In adults, the severity of NAFLD histology is associated with renal impairment
  • The first stage of renal impairment is glomerular hyperfiltration. This is hypothesized to be a precursor of intraglomerular hypertension which leads to albuminuria and glomerular filtration rate (GFR) decline/progressive renal dysfunction
  • Early intervention in high risk patients with angiotensin receptor inhibitors may prevent or delay progressive renal disease

Key findings in 179 patients with biopsy-confirmed NAFLD:

  • 82% non-Hispanic, median age 14 yrs
  • 36 (20%) had glomerular hyperfiltration and 26 (15%) had low GFR (w/in 3 months of liver biopsy) based on Schwartz equation
  • Hyperfiltration was independently associated with higher NAFLD activity score (aOR 2.96)

Discussion:

  • Mechanism: The authors speculate that “it is possible that they [renal and liver disease] are both the end result of the same ‘hit.’ The renin-angiotensin system may play a key role….Notably, there is an ongoing…clinical trial investigating an ATI receptor blocker, losartan, for the treatment of NAFLD in children.” Other potential contributors include fructose and insulin resistance.
  • Limitations: This single center biopsy-confirmed population may not be representative of most children with NAFLD.  Also, as this was a retrospective study, more precise measures of renal function were not available.

My take: This study confirms a high rate of renal dysfunction (35%) in children with NAFLD. As such:

  • Children with NAFLD need to have their blood pressure monitored
  • Clinicians should have a low threshold for nephrology referral if suspected renal impairment.

NEJM Recovery Collaborative Group: July 17, 2020
DOI: 10.1056/NEJMoa2021436: Full Link: Dexamethasone in Hospitalized Patients with Covid-19 — Preliminary Report

Form NEJM Journal blog:

In the open-label RECOVERY trial, some 2100 U.K. patients hospitalized with COVID-19 were randomized to usual care plus oral or intravenous dexamethasone (6 mg once daily for up to 10 days), and 4300 were randomized to usual care alone.

Among patients on invasive mechanical support at the time of randomization, the mortality rate within 28 days was significantly lower with dexamethasone than with usual care alone (29% vs. 41%). A benefit was also seen among those on oxygen without invasive ventilation (23% vs. 26%). However, among patients not receiving respiratory support, mortality rates did not differ significantly between treatment groups.