A long time ago in a galaxy far far away, I was taught that children with esophageal atresia would have reflux for life due to dysmotility following repair. Thus, these children presumably should remain on acid blockers indefinitely. It turns out that this was fiction (just like Star Wars).
In this retrospective study with 48 children, the authors had the following key points:
Microscopic esophagitis was found in 33 (69%)
Pathological esophageal acid exposure on MII-pH was detected in 12 (25%)
The presence of long-gap esophageal atresia was associated with abnormal MII-pH.
The authors conclude that “histological esophagitis is highly prevalent at 1 year after esophageal atresia repair, but our results do not support a definitive causative role of acid-induced GERD. Instead, they support the hypothesis that chronic stasis in the dysmotile esophagus might lead to histological changes.”
My take: Along with endoscopy, pH probe testing can be helpful in selecting which children with esophageal atresia should continue with PPI therapy.
It is very difficult to try to understand potential toxic substances in our environments. Some of the reasons for this are that there are always numerous simultaneous exposures and harm from substances can accrue over long periods. Once a substance is identified, it can take a long time to develop convincing evidence and even longer time frames to try to enact policy changes.
Background/Methods: Per- and polyfluoroalkyl substances (PFAS) are widespread and persistent pollutants that have been shown to have hepatotoxic effects in animal models. However, human evidence is scarce. PFAS chemicals have a myriad industrial/household applications which include nonstick cookware and products that confer resistance to stains. According to the editorial (MC Cave, pg 1518-21), some refer to PFAS as “forever chemicals” due to their decades-long half-lives.
The study authors used data from 1105 mothers and their children (median age 8.2 years) from the European Human Early-Life Exposome cohort. Key findings:
High prenatal exposure to PFAS resulted in children who were at higher risk of liver injury (odds ratio, 1.56; 95% confidence interval, 1.21–1.92)
PFAS exposure is associated with alterations in key amino acids and lipid pathways characterizing liver injury risk.
As noted in previous blog posts (see below), allergy testing can lead to unnecessary food restrictions which can in turn lead to numerous subsequent problems. Case in point: YV Virkud et al (NEJM 2020; 383: 2462-2470) report on A 29-Month-Old Boy with Seizure and Hypocalcemia
This boy presented with severe hypocalcemia, rickets, and seizures one year after allergy testing led to additional dietary restrictions. Also, his mother was a vegetarian. At time of allergy testing, IgE testing suggested allergies to milk, cashews, pistachios, egg whites, almonds, soybeans, chickpeas, green peas, lentils, peanuts, and sesame seeds. Many of these foods caused no symptoms with food challenges.
Besides working through the potential reasons for hypocalcemia, the authors make several key points:
Nutritional rickets is NOT a historical relic. Vitamin D deficiency appears to be increasing in high-income countries despite food-fortification strategies.
There are frequent misdiagnosis of food allergies. “Clinical and laboratory testing is severely limited by poor specificity…approximately 20 to 25% of children have positive IgE blood tests to specific food allergens, even though the true prevalence of IgE-mediated food allergy is likely closer to 6 to 8%.”
Avoid indiscriminate use of IgE blood testing. Allergen panels are “particularly problematic, because they often uncover false positives and lead to unnecessary food avoidance.” Individual IgE testing can be used to help confirm a diagnosis after an allergic reaction to a food trigger.
The most accurate diagnostic tool is an oral food challenge.
In children with food allergies, supplements are often needed to avoid micronutrient deficiencies and a low threshold is needed for involvement of dieticians.
Early introduction of foods can reduce incidence of allergies and periodic reassessment is needed to determine if a child has outgrown an allergy.
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Stroumsa et al note that about 1.9 million adults in U.S. identify as transgender. Key points:
This summer’s Supreme Court ruling in the employment-discrimination case Bostock v. Clayton County is likely to influence future court rulings regarding discrimination in health care coverage. “In the majority opinion, Justice Neil Gorsuch wrote, ‘It is impossible to discriminate against a person for being homosexual or transgender without discriminating against that individual based on sex.'”
Despite this favorable ruling for transgender persons, the current administration has tried to perpetuate discrimination on the basis of religious freedom. “The Trump rules stripping transgender rights from ACA protection are most likely invalid under Bostock.”
“The medical profession has an ongoing obligation to act..[to create] health care environments that are as welcoming for transgender and nonbinary patients as they are for cisgender patients.”
Shteyler et al discuss how birth certificate gender assignments can be detrimental. They note that birth certificates have changed many times to collect useful public information. One prominent feature has been a ‘line of demarcation’ in which there is legally identifying fields above the line and deidentified fields (eg. race, marital status) below the line which are reported in aggregate. They argue that sex assignment should be deidentified. Key points:
“Designating sex as male or female on birth certificates suggests that sex is simple and binary when, biologically, it is not.”
~1 in 5000 people have intersex variations
~1 in 100 exhibit chimerism, mosaicism, or micromosaicism, “conditions in which a person’s cells may contain varying sex chromosomes”
~6 in 1000 people identify as transgender. “Others are binary, meaning they don’t exclusively identify as a man or a woman, or gender nonconforming, meaning their behavior or appearance doesn’t align with social expectations for their assigned sex.”
“Only 9% of transgender people who want to update their gender on the documents succeed in doing so.”
“Leaving any sex designation visible on birth certificates sacrifices privacy and exposes people to discrimination.”
Medical providers have a duty to help policymakers understand the science and to make sure that “medical evaluations aren’t being misused in legal contexts.”
My take: When I was a child/adolescent, I barely had any concept regarding the spectrum of sexuality. Though, it was easy to see many individuals who were ostracized due to their differences. As a medical provider, I see children/teens whose sexual identity is homosexual, transgender, or nonbinary. I think it is a sign of progress that there is more acceptance to the variation in sexual identity but much more is needed.
On another hot button topic, David Brooks explains why programs aimed at reducing racial discrimination don’t work: 2020 Taught Us How To Fix This “The superficial way to change minds and behavior doesn’t seem to work, to bridge either racial, partisan or class lines. Real change seems to involve putting bodies from different groups in the same room, on the same team and in the same neighborhood.”
“Rejecting the advice of its scientific advisers, the federal government has released new dietary recommendations that sound a familiar nutritional refrain, advising Americans to “make every bite count” but dismissing experts’ specific recommendations to set new low targets for consumption of sugar and alcoholic beverages...
The dietary guidelines have an impact on Americans’ eating habits, influencing food stamp policies and school lunch menus and indirectly affecting how food manufacturers formulate their products…
The new guidelines do say for the first time that children under 2 should avoid consuming any added sugars, which are found in many cereals and beverages.”
I have a deep admiration and fondness for Jorge. When I first did a gastroenterology rotation during my pediatric residency, he was the first person who handed me an endoscope and showed me how to handle it. During my training as a resident and as a fellow (1991-1997), I had the opportunity to get to know Jorge; for some of that time, he was completing his training as he started his GI fellowship in 1990.
I really enjoyed reading this introduction to learn a lot more about Jorge, because I don’t remember Jorge speaking about himself. Of course, he has been part of some very important advances in pediatric hepatology including the very useful MMP-7 assay, the ‘Jaundice chip’ and the START study.
The article delves into some personal attributes including the description of Jorge being ‘the Pele of pediatric hepatology’ (per Dr. Ronald Sokol). It also describes his family and some characteristics. “He has inspired us with his calm demeanor, decency, humor, positivity, and kindness.”
It is a personal thrill for me to read about one of my heroes in our field.