Cyclic Vomiting ED Protocol

Posted on by

Recently, I was reading information from the Cyclic Vomiting Syndrome Association (Winter 2020 issue). On page 12, there is a pretty good protocol for emergency department treatment with the caveat that this “represents a sample template and should be tailored based on individual needs.” In addition, this protocol was derived from an article by Venkatesan et al (Neurogastroenterology and Motility 2019; S2.https://doi.org/10.1111/nmo.13604 Full text: Guidelines on management of cyclic vomiting syndrome in adults by the American Neurogastroenterology and Motility Society and the Cyclic Vomiting Syndrome Association)

Key points from guidelines article:

  • “The committee strongly recommends that adults with moderate‐to‐severe CVS receive a tricyclic antidepressant (TCA) such as amitriptyline, as a first‐line prophylactic medication. “
  • Topiramate, Aprepitant, “Zonisamide or levetiracetam and mitochondrial supplements (Coenzyme Q10, L‐carnitine, and riboflavin) are conditionally recommended as alternate prophylactic medications, either alone or concurrently with other prophylactic medications.”
  • “For acute attacks, the committee conditionally recommends using serotonin antagonists such as ondansetron, and/or triptans such as sumatriptan or newer agents such as aprepitant (NK1 receptor antagonist) to abort symptoms.”
  • Evidence, dosing regimens, and algorithms are detailed in article

Sample ED CVS Protocol (for Adults):

____[name]____________ has an established diagnosis of Cyclic Vomiting Syndrome
Operational definition
* A recurring pattern of discrete episodes of severe vomiting, accompanied by profound nausea and/or severe abdominal pain
* Patient returns to usual health status between episodes (may have inter‐episodic nausea and or dyspepsia)
* In some patients, CVS episodes resemble a migraine attack
* Patients may be restless, anxious, and distressed
* Patients are not customarily dehydrated until late in the episode
Therapeutic goal
Rapid recognition and intervention may decrease severity of the attack and promote prompt resolution of symptoms
ED management
1. Clinical assessment: Pulse/Temp/BP/Weight, consciousness, and hydration
2. Laboratories/evaluation:
CBC, urea, creatinine, LFT’s, lipase, glucose, and electrolytes
EKG
Urine analysis
Diagnostic imaging at discretion of attending physician
Treatment
1. Intravenous fluids
a. IV saline bolus if clinically dehydrated
b. IV D5NS at 100%‐150% maintenance (suggested rate is 200 cc/h for a 70 kg adult.)
2. For vomiting and nausea
a. IV ondansetron 8 mg IV × 1—may repeat q 4‐6 h if ondansetron is ineffective
b. Consider diphenhydramine 50 mg IV and metoclopramide 10 mg IV
c. Consider IV fosaprepitant 150 mg if available
3. For sedation
a. IV lorazepam 1‐2 mg and b. IV diphenhydramine 50 mg for additional sedation
4. For migraine‐like presentation
a. Sumatriptan nasal 20 mg (head forward technique) or
b. Sumatriptan subcutaneous injection 6 mg/0.5 mL
5. For pain
a. IV ketorolac 30 mg if > 60 minutes from onset; may repeat 15 mg q 6 h x 2 (maximum 60 mg/d)
b. Opioids may be considered as part of an ongoing treatment plan in refractory patientsa
Reassess
1. Treatment failure—intensify treatment as indicated above or admit patient
2. Positive treatment response—discharge
a. Continue ondansetron (soluble tablets) q 6‐8 h × 24‐48 h if initially effective
b. Continue lorazepam × 24‐48 h if initially effective
c. Continue NSAIDs for pain as needed

My take: This reference is very useful. The pediatric NASPGHAN guidelines (BUK Li et al. JPGN 2008; 47:379–393. Full text: North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Consensus Statement on the Diagnosis and Management of Cyclic Vomiting Syndrome) probably need to be updated, especially as there are newer agents available (eg. aprepitant, fosaprepitant).

Related blog posts:

Resources:

Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition

Genetic Testing for Fatty Liver Disease Is Not Ready For Routine Use

Posted on by

A recent study (H Gellert-Kristensen et al. Hepatology 2020; 72: 845-856. Combined Effect of PNPLA3, TM6SF2, and HSD17B13 Variants on Risk of Cirrhosis and Hepatocellular Carcinoma in the General Population) describes genetic risk score (GRS) which can stratify the risk of developing cirrhosis and hepatocellular carcinoma.

The study utilized data and plasma markers from 110,761 individuals from Copenhagen, Denmark, and 334,691 individuals from the UK Biobank. GRS scores were from 0 to 6 based on three common genetic variants: PNPLA3, TM6SF2, and HSD17B13.

Key finding:

  • A GRS of 5 or 6 (compared to GRS of 0) for fatty liver disease confers up to a 12‐fold higher risk of cirrhosis and up to a 29‐fold higher risk of HCC in individuals from the general population

The editorial by RM Pfeiffer et al (Hepatology 2020; 72: 794-795. Genetic Determinants of Cirrhosis and Hepatocellular Carcinoma Due to Fatty Liver Disease: What’s the Score?) is very helpful in placing the findings in context.

  • Only 0.5% of individuals had a GRS of 5 or 6. “A GRS of 4 [or more] which still conveyed large risks (cirrhosis, OR =5.2; HCC, OR =3.3) was found in approximately 5% of this population.”
  • Using a GRS of 4 or more, the positive predictive value of GRS-based test in the Danish population is “0.008 for cirrhosis and 0.003 for HCC. In other words, among 1000 persons with GRS greater than or equal to 4, only 8 will develop cirrhosis and 3 will develop HCC.”

My take: This study confirms that specific genetic variants increase the risk of complications from fatty liver disease. However, poor predictive value will likely preclude routine application.

Shush -Let’s Not Talk About Firearm Safety

Posted on by

A Connor et al. Annals Int Med 2020; https://doi.org/10.7326/M20-6314 Firearm Safety Discussions Between Clinicians and U.S. Adults Living in Households With Firearms: Results From a 2019 National Survey

Methods: Data were obtained from the second National Firearms Survey, conducted online 30 July 2019 to 11 August 2019. Respondents (n=4030) were asked, “Has a physician or other health care practitioner ever spoken to you about firearm safety?”

Key finding:

  • Of all respondents, 7.5% (95% CI, 6.6% to 8.6%) had ever discussed firearm safety with a provider (12.0% [CI, 9.9% to 14.6%] of those living with children vs. 5.3% [CI, 4.4% to 6.3%] in homes without children)

In the comments to this brief study, several useful points were made.

  • #1: Train physicians on this topic: “1. This is not taking a position regarding gun ownership; 2. Access to firearms is associated with suicide, accidents, and firearm-related violence; 3. State laws regarding safe firearm storage; 4. Principles of safe storage; 5. Principle of separate ammunition storage; 5. Concept of removing firearms to another location (e.g., a relative) when children are small.”
  • #2 We can do better. “It’s not that hard to ask the question or put into your intake questionnaire; Is there a gun in the house? and if yes is the answer, follow-up”

Related blog posts:

High Calorie Infant Formula

Posted on by

Our office has participated in research for a 30 cal infant formula that is heading to the market in 2021. Nutricia is calling the formula Fortini. Link to website: Fortini (I have no financial ties/interest to this product or company).

I think having a commercial high calorie infant formula is advantageous and overcomes some of the limitations of concentrating infant formulas. Advantages:

  • This formula will eliminate problems with incorrect mixing and contamination. Despite careful oral and written instructions, many parents incorrectly prepare high calorie formulas
  • This formula, compared with concentrating a standard formula, is likely to have improved tolerability (less hyperosmolar) and better nutrient balance (eg. proper protein content)

The main potential disadvantage is going to be cost. I do not know the cost of the new formula but would be surprised if it is not significantly higher than concentrating a standard formula. At the same time, if the formula is able to improve tolerance and improve poor growth, there could be ‘downstream’ savings with less medical intervention/hospitalizations.

Related blog post: Rarely Seen and “Do Not Miss” Explanation for Failure to Thrive

Chattahoochee River. Sandy Springs, GA

Deluge of Liver Disease Due to COVID-19?

Posted on by

Two articles in a recent issue of Hepatology describe both direct and indirect effects of COVID-19 on the liver.

The first study with 2273 patients (MM Phipps et al Hepatology 2020; 72: 807-817. Full Text: Acute Liver Injury in COVID‐19: Prevalence and Association with Clinical Outcomes in a Large U.S. Cohort), with retrospective data, describes how most cases of COVID-19 are mild. Severe cases of liver disease are generally a marker for elevated inflammatory markers and severe systemic disease. Key findings:

  • 45% had mild (ALT <2 x ULN), 21% moderate (ALT 2-5 x ULN), and 6.4% severe liver injury (SLI) (ALT >5 x ULN).
  • Patients with SLI had a more severe clinical course, including higher rates of intensive care unit admission (69%), intubation (65%), renal replacement therapy (RRT; 33%), and mortality (42%).
  • In multivariable analysis, peak ALT was significantly associated with death or discharge to hospice (OR, 1.14; P = 0.044), controlling for age, body mass index, diabetes, hypertension, intubation, and RRT

Going into this new year, the more concerning effects of COVID-19 pandemic for the liver is likely to be the increase is severe chronic liver disease related to alcohol (and perhaps fatty liver disease too). The second article (BL Da et al. Hepatology 2020; 72: 1102-1108. Coronavirus Disease 2019 Hangover: A Rising Tide of Alcohol Use Disorder and Alcohol‐Associated Liver Disease) discusses the expectation of increased liver disease due to alcohol use disorder (AUD) and alcohol-associated liver disease (ALD). Key points:

  • In China, reports indicate a “>2-fold increase in harmful drinking after COVID-19, an effect likely repeated in the United States where an estimated 12.7% of the population has AUD and ALD is responsible for the highest hospitalization cost burden among all chronic liver diseases (CLDs).”
  • Increased alcohol use is likely to worsen other chronic liver diseases in addition to ALD
  • In addition, all of these effects are compounded by avoidance of health care facilities and delays in care

My take: COVID-19 infections have direct effects on the liver. However, the increased use of alcohol as well as weight gain are likely to be more important in terms of liver-related morbidity and mortality.

More Often Than Not Esophagitis in Children with Esophageal Atresia is NOT due to Reflux

Posted on by

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).

R Tambucci et al J Pediatrics 2021; 228: 155-165. Full text: Evaluation of Gastroesophageal Reflux Disease 1 Year after Esophageal Atresia Repair: Paradigms Lost from a Single Snapshot?

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.

Related blog posts:

Prenatal Liver Pollutants: Perfluoroalkyl Substances

Posted on by

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.

Despite these challenges, fortunately researchers continue to try to tease out these dangerous agents. A recent study (N Stratakis et al. Hepatology 2020; 72: 1758-1770. Free Full text: Prenatal Exposure to Perfluoroalkyl Substances Associated With Increased Susceptibility to Liver Injury in Children)

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.

Related blog posts:

Poorly-Conceived Allergy Testing Can Lead to Unnecessary Diet Restrictions and Complications

Posted on by

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.
Xrays show generalized demineralization. The metaphyses show flaring (dashed arrow) and cupping (arrowbead). The physes are radiolucent and widened (asterisks).

Related blog posts:

Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition

What Doctors Should Know About Discrimination Based on Sexuality

Posted on by

Two recent commentaries help advance the understanding of sexuality and transgender people with regard to discrimination and potential implications for health care.

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.”

NY Times: “U.S. Diet Guidelines Sidestep Scientific Advice”

Posted on by

NY Times (12/29/20): U.S. Diet Guidelines Sidestep Scientific Advice

An excerpt:

“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.”

USDA Website: Dietary Guidelines for Americans

Related article: NY Times (Print edition 12/27/20) Obesity Rates Soar in China and Officials Take Action. Online (12/24/20): Influencers May Face Fines as China Tackles Obesity and Food Waste An excerpt:

“34.3 percent of adults were overweight and 16.4 percent were obese. It looked at a group of 600,000 Chinese residents between 2015 and 2019. By comparison, 30 percent of Chinese adults were overweight and 11.9 obese in 2012…obesity among American adults has increased 12.4 percent over the past 18 years, with 42.4 percent of adults in the United States now living with the condition.“

Related blog posts: