Neuro-Stim for Refractory Cyclic Vomiting?

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K Karrento et al. JPGN 2023; 77: 347-353. Percutaneous Electrical Nerve Field Stimulation for Drug-Refractory Pediatric Cyclic Vomiting Syndrome

In this prospective study with 30 children with drug-refractory CVS, response was classified as ≥50% improvement in either frequency or duration of attacks at extended follow-up.

Key findings:

  • At follow-up, 80% met criteria for treatment response with a median (IQR) response duration of 113 (61–182) days.
  • At end of therapy, 66% and 55% patients reported global response of at least “moderately better” and “a good deal better,” respectively.
  • There were no serious side effects.

Limitations: episodic nature of CVS, no control group, hx/o strong placebo control group and lack of validated assessment tool

My take: 1. As with the drug therapies with CVS, it is difficult to know how effective PENFS is for CVS. At the same time, its good safety profile makes this therapy an intriguing option in those not responding to more typical treatments. 2. The visual abstract is funny -mainly due to the two laughing kids holding hands. Though it would be even better if the person on the left was puking a lot instead of having a PENFS device.

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FLIP Patterns for Adults with Eosinophilic Esophagitis

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DA Carlson et al. Gastroenterol 2023; 165: 552-563. Open Access! A PhysioMechanical Model of Esophageal Function in Eosinophilic Esophagitis

215 adults with EoE who completed FLIP during endoscopy were included in a cross-sectional study. FLIP helped separate the physiomechanical properties of esophageal function in this cohort. The criteria used to define the PhysioMechanical classification in EoE with a representative FLIP panometry image for each classification. Normal compliance was defined as a DP >17 mm and body compliance >450 mm3/mm Hg; reduced compliance (fibrostenosis) was defined by DP ≤17 mm or compliance ≤450 mm3/mm Hg. Normal EGJ opening was defined as a maximum EGJ diameter ≥16 mm; reduced as maximum EGJ diameter <16 mm. ∗Spastic-reactive contractile response (SRCR) with normal body distensibility and normal EGJ opening was assigned as “achalasia pattern” (n = 1 in this cohort).

Key findings:

  • FLIP was normal in 50 (23%), weak pattern in 7 (3%), IsoEGJOO stricture pattern in 27 (13%), IsoEGJOO achalasia pattern in 26 (12%), Fibrostenosis with normal reactivity in 61 (28%), spastic reactive fibrostenosis with normal reactivity in 30 (14%), and noreactive fibrostenosis in 14 (7%)

My take: FLIP testing helps define the mechanism of esophageal dysfunction in patients with EoE. Longer duration of symptoms was associated with more severe esophageal dysfunction.

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How to Provide More Cost-Effective Celiac Care

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PF Farmer et al. J Pediatr 2023; 259: 113487. Single-Center Analysis of Essential Laboratory Testing in Patients with Newly Diagnosed Celiac Disease

In this study, the authors analyzed laboratory testing results from pediatric patients newly diagnosed with celiac disease (2018-2021) to determine the usefulness of each test derived from recommended guidelines (J Snyder et al. Pediatrics 2016; 138: e20153147). Screening protocols in their center resulted in an estimated cost of approximately $320,000 during the study. Tests at diagnosis included hemoglobin, alanine/aspartate aminotransferase, ferritin, iron, TSH, Free T4, and vitamin D screening. These screening tests were done in ~80% of 468 patients.

Key findings:

  • Ferritin was abnormal in 29%, hemoglobin was abnormal in 12%, and iron was abnormal in 22%. Abnormal ferritin captured all patients in this cohort with an abnormal iron. If ferritin was used as an isolated screen with reflective iron testing, this would have reduced costs by about $12,000
  • AST and ALT were abnormal in 2% and 11% respectively
  • 25-OH Vitamin D was abnormal in 14%. Recent data indicated that low Vit D levels are similar among patients with and without celiac disease (R Ahlawat et al. JPGN 2019; 69: 449-454)
  • TSH and Free T4 were abnormal in 7% and 0.3% respectively. For thyroid disease, TSH and free T4 testing did not lead to any new diagnosis of thyroid disease (7 carried a preexisting diagnosis). There were 19 additional patients with abnormal lab values who had more testing due to initial abnormalities. If TSH alone were used for screening, costs savings would be about $29,000. If no thyroid testing were done, this would have reduced costs by about $40,000.
  • Hepatitis B immunity was NOT present in 69%. However, recent studies have shown similar levels of immunity in those with and without celiac disease. In addition, it is not clear that a low level hepatitis B surface antibody always indicates a lack of immunity. Eliminating hepatitis B screening would have reduced costs by about $63,000.
  • The authors note that the cost savings by adopting their recommendations would have saved about $104,000 (out of $320,000).

My take: This is a very useful study and indicates that curtailing initial testing for celiac disease could reduce costs substantially and without compromising care. This would include not checking a serum iron, a free T4, or hepatitis B studies. The authors note that the value of Vit D testing is also questionable but may be worthwhile due to increased risk of bone disease in individuals with celiac disease.

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Blackwater Falls near Davis, WV
Seneca Rocks Trail, WV

Nonanaphylactic Alpha-Gal and Chronic Gastrointestinal Symptoms

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D Glynn et al. J Pediatr 2023; 259: 113486. Nonanaphylactic Variant of Alpha-Gal Syndrome as an Etiology for Chronic Gastrointestinal Symptoms in Children

Background: A CDC report showed that between 2010 and 2022, more than 110,000 suspected cases of alpha-gal syndrome were identified. The majority of cases are linked to bites from the lone star tick which affects much of the U.S. (map below) as well as Central and South America, Asia, Africa, Australia and parts of Europe.

Findings: This study reports 3 pediatric patients who presented with only nonanaphylactic symptoms of alpha-gal syndrome. These patients with recurrent gastrointestinal distress and emesis after consuming mammalian meat, even in the absence of an anaphylactic reaction.

The diagnosis in these three patients was established by history, serum alpha-gal immunoglobulin E elevation and response to avoidance of red meat.

My take: Checking a serum Alpha-gal IgE seems like a good idea in some children with unexplained abdominal pain with episodic exacerbation with vomiting, especially if tick exposure. Anecdotally, I have checked this a few times and so far I have not I identified a case. Most cases of Alpha-gal will be associated with urticaria.

Related blog post: Tick Bites Can Lead to Allergy to Red Meat

CDC: Alpha-gal syndrome “Symptoms commonly appear 2-6 hours after eating meat or dairy products, or after exposure to products containing alpha-gal (for example, gelatin-coated medications).”

CDC: Food products that may contain alpha-gal: Mammalian meat (such as beef, pork, lamb, venison, rabbit, etc.) can contain high amounts of alpha-gal. Food products that contain milk and milk products typically contain alpha-gal (though many patients tolerate dairy products)

Foods that do NOT contain alpha-gal (unless cross contamination):

  • Poultry, such as chicken, turkey, duck, or quail
  • Eggs
  • Fish and seafood, such as shrimp
  • Fruits and vegetables
From CDC website: https://www.cdc.gov/ticks/maps/lone_star_tick.html

Case Report: 20 month old with Abrupt Vomiting and Multisystem Disease

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Case history: A well-nourished previously healthy 20 month old was admitted to the hospital with a one week history of frequent vomiting. He had a history of frequent lint ingestion. He had been seen by three different health care providers during the week prior to his admission.

At the time of admission, he had numerous electrolyte derangements (Na 124, K 3.2, CL 76) and acute kidney dysfunction with a BUN of 118 and Creatinine of 3.06. He had severe multisystem disease including severe ventricular dysfunction (BNP 2196). He needed an oscillator ventilator, dialysis and cardiac medications (including epinephrine, and milrinone).

He had an extensive evaluation. After he had stabilized and then markedly improved (12 days after admission), an UGI study demonstrated an obstruction near the 2nd-3rd portion of the duodenum with a dilated proximal duodenum.

UGI study:

Due to the obstruction which was thought to be anatomic, the GI service deferred management to pediatric surgery. The surgical service requested GI inspection with endoscopy immediately prior to surgery. If a bezoar was identified, the surgical plan was for a mini-laparotomy. If not, the surgical plan was for laparoscopy repair.

Endoscopy findings: There was a narrowed opening (different orientation of same narrowing in both pictures) with a string-like material.

Surgical findings: A duodenal web with a piece of lint was identified. The patient had a laparoscopic wedge excision with a transverse closure of the duodenotomy. The lint may have occluded the tiny opening of the web that he had been living with since he was born.

My take: This is the first time I have seen the endoscopic appearance of a duodenal web.

In my view, this was a ‘great case.’ In a commentary by Jerome Groopman (N Engl J Med 2004; 351:2043-2045), his wife noted that a case is “a great case because you not only make the diagnosis — you do something fundamental about it. You can really help.” However, Dr. Groopman stopped using the words, ‘a great case’ after his personal experience when his son was severely ill as an infant with a bowel obstruction due to intussusception: “For me and for Pam, the experience had no resonance of “a great case.” There was no intellectual pleasure in solving a clinical puzzle, no charge of exhilaration from the drama of the operation. Instead, there was terror, raw and palpable, as we realized how close we had come to burying our first son….I still find myself unable, except in retrospect, to retrieve the language of my youth and speak about “a great case.” It is as if medicine at this stage of my life has split into two streams — a current of marvelous biology and an undertow that pulls at the soul.”

Hepatitis C is Undertreated in the U.S.

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C Wester et al. MMWR 2023; 72 (26): 716-720. Open Access! Hepatitis C Virus Clearance Cascade — United States, 2013–2022 (starts on page 16 of PDF)

Key findings:

  • Among the approximately 1.0 million persons in this analysis with initial infection, only 34% had laboratory evidence of viral clearance
  • Overall, viral clearance was lowest among persons aged 20–39 years (24%). Patients 0-19 were not included in this analysis
  • To overcome the low cure rate, some have recommended a subscription model for HCV treatment; this was piloted in Louisiana. In this pilot, the state paid a lump sum to make the drug available for free to all patients on Medicaid and federal prisoners. Francis Collins has indicated that a national program, while expensive, would save the government $13 billion in 10 years (Source: Infectious Disease Special Edition, 6/30/23: Most Americans With HCV Not Receiving DAAs)

My take: Improving access to HCV treatment has the potential to save livers, save lives and save money.

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Good Review on Newest Medications for Obesity

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For those wanting an in-depth review:

Eric Topol: “The GLP-1 and now triple G-Agonists are exceeding expectations as weight loss drugs. Benefits for cardiovascular outcomes, possibly Type 1 diabetes, addiction, and more are reviewed in the latest Ground Truths.” His newsletter also describes the early results of these medications with MASH/NASH with improvement in ~90%.

To get the full review, go to Ground Truths: http://erictopol.substack.com (can sign up to get regular emails on many current medical topics)

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Subcutaneous Vedolizumab Now FDA-Approved for Adults with Ulcerative Colitis

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9/27/23 Takeda: U.S. FDA Approves Subcutaneous Administration of Takeda’s ENTYVIO® (vedolizumab) for Maintenance Therapy in Moderately to Severely Active Ulcerative Colitis

” Takeda (TSE:4502/NYSE:TAK) today announced that the U.S. Food and Drug Administration (FDA) has approved a subcutaneous (SC) administration of ENTYVIO® (vedolizumab) for maintenance therapy in adults with moderately to severely active ulcerative colitis (UC) after induction therapy with ENTYVIO intravenous (IV).1 ENTYVIO SC is expected to be available in the U.S. as a single-dose pre-filled pen (ENTYVIO Pen) by the end of October. Additionally, a Biologics License Application for an investigational SC administration of ENTYVIO for the treatment of adults with moderately to severely active Crohn’s disease is currently under review by the FDA.”

From WJ Sandborn et al. Gastroenterol 2020; 158: 562-572. Open Access! Efficacy and Safety of Vedolizumab Subcutaneous Formulation in a Randomized Trial of Patients With Ulcerative Colitis

In the VISIBLE1 Trial, dosing was IV for week 0 and 2, then every other week SC for maintenance.

Out of Pocket Maximum (Sad Humor)

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Dr. Glaucomflecken Twitter Link (w/o login): Out of Pocket Maximum (1:13 min)

There are a bunch of new videos (one for everyday in September) similar to this on related topics -search 30 Days of US Healthcare. Here’s another one: 30 Days of US Healthcare: Surprise Billing (YouTube)

My take: As usual, Dr. Glaucomflecken humor sheds light on the faults in our nation’s health care insurance coverage.