Briefly Noted: Shwachman-Diamond

S Cesaro et al. J Pediatr 2020; 219: 196-201.  This prospective study with 121 patients provides long-term survival information regarding Shwachman-Diamond syndrome which is characterized by exocrine pancreatic insufficiency, hematologic alterations, skeletal abnormalities and sometimes liver disease. Key findings:

  • Initial hematologic parameters included severe neutropenia in 25.8%  , thrombocytopenia in 25.5%, and anemia in 4.6%; cumulative incidence of these abnormalities at 30 years of age were 59.9%, 66.8%, and 20.2% respectively
  • 20-year cumulative incidence of myelodysplasia/leukemia was 9.8% and of bone marrow failure/severe cytopenia was 9.9%.
  • 15 (12.4%) underwent stem cell transplantation
  • 15 (12.4%) died with probability of survival at 10 yrs: 95.7% and at 20 yrs 87.4%

My take: This study shows the hematologic morbidities associated with Shwachman-Diamond –important information for the pediatric gastroenterologist following these children for pancreatic insufficiency or liver-related abnormalities.

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Also, a previous post (Do Button Battery Guidelines Need to be Revised?) reviewed an abstract suggesting that gastric button batteries could result in mucosal injury.  This has now been published: (Gastrointestinal Endoscopy, DOI: https://doi.org/10.1016/j.gie.2020.04.037 In press) Gastric injury secondary to button battery ingestions: a retrospective multicenter review

Curbside humor: How do you make a tissue dance?  Put a little boogie in it.

Island Ford National Recreation Area/Chattahoochee River

 

Do Button Battery Guidelines Need To Be Revised?

A recent abstract presented at DDW (R Khalaf et al. abstract Sa2046) with 68 patients identified mucosal findings in the stomach and questioned whether the current guidelines are sufficient.  Generally, guidelines call for the immediate removal of button batteries in the esophagus but in asymptomatic children older than 5 years, most gastric batteries can be observed (see links to previous blog posts below which highlight expert recommendations).

Link: Sa2046 GASTRIC INJURY SECONDARY TO BUTTON BATTERY INGESTIONS IN CHILDREN: A RETROSPECTIVE MULTICENTER REVIEW

This study was reviewed in Gastroenterology & Endoscopy News: Retrieving Swallowed Batteries in Children: Don’t Watch and Wait  This link also highlights an abstract from the Emory pediatric GI group, NASPGHAN 2019 (#24), which found that only 5% of esophageal button batteries were removed within two hours.

An excerpt:

According to the National Poison Data System, between 1985 and 2017, roughly 3,500 button batteries were swallowed in the United States each year (www.poison.org/ battery/ stats). ..

The researchers reviewed 68 cases of children who underwent endoscopy after having swallowed button batteries, which are used in a variety of devices, such as cameras and watches. Eighteen of the patients (26%) were asymptomatic, but 41 (60%) had visible mucosal damage…

Some injuries were more severe. A 9-year-old child with a battery lodged in the antrum experienced a gastric perforation that led to pneumoperitoneum, Dr. Khalaf reported. Although only one other injury was as serious, the researchers identified no risk factors that predicted significant complications.

My take: There are a lot of button battery ingestions.  More data is needed to determine whether more button batteries from the stomach should be retrieved.

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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.

New Button Battery Guidelines –with Honey and Vinegar

Button batteries –definitely more scary than Halloween.  Here is a link to new guidelines from Poison Control: New Button Battery Guidelines 2018

A recent report from Nationwide Childrens, How Sweet It Is: Honey Attenuates Button Battery-Induced Esophageal Damage, highlights two important advances in button battery management that are now incorporated into the new button battery guidelines:

  1. At time of endoscopy,  “a weak acetic acid rinse (sterile vinegar) can help neutralize tissue pH and protect the esophagus from continued tissue breakdown after battery removal. This irrigation concept has now been successfully used in children around the world with good clinical outcomes.”
  2. Prior to endoscopy, “both honey and sucralfate (Carafate®) were able to effectively neutralize the tissue pH and reduce visible injury.”

“More than 3,000 cases occur per year, mostly among children younger than age 6, and severe cases are on the rise. Lodged button batteries can cause rapid injury, including permanent bilateral vocal cord paralysis and even death.”

Guideline recommendations with regard to acetic acid:

After a battery is removed from the esophagus, inspect the area endoscopically for evidence of perforation. If none is evident, irrigate the injured areas with 50 mL to 150 mL of 0.25% sterile acetic acid (obtained from the hospital pharmacy). Irrigate in increments and suction away excess fluid and debris through the endoscope. For decades toxicologists have advised against neutralization for fear of causing a thermal injury. However, a recent study (Jatana, 2016) using piglet esophagus preparations after button battery removal, showed only a minimal increase in temperature (0-3 oC), effective tissue surface pH neutralization, and decrease in the visible injury using this neutralization strategy. The tissue surface pH neutralization may reduce the development of progressive, delayed-onset esophageal injury after battery removal.

From guideline with regard to honey:

Administer honey immediately and while en route to the ER, if:

  1. A lithium coin cell may have been ingested (if you don’t know what kind of button battery was swallowed, assume it is a lithium coin cell unless it is a hearing aid battery);
  2. The child is 12 months of age or older (because honey is not safe in children younger than one year);
  3. The battery was swallowed within the prior 12 hours (because the risk that esophageal perforation is already present increases after 12 hours);
  4. The child is able to swallow; and
  5. Honey is immediately available.

How to dose honey:

      1. Give 10 mL (2 teaspoons) of honey by mouth every 10 minutes for up to 6 doses. Do not worry about the exact dose or timing.
      2. Use commercial honey if available, rather than specialized or artisanal honey (to avoid inadvertent use of large amounts of honey produced from potentially toxic flowers).
      3. Honey is NOT a substitute for immediate removal of a battery lodged in the esophagus. Honey slows the development of battery injury but won’t stop it from occurring. Do not delay going to an ER.

Why give honey?

Honey is administered to coat the battery and prevent local generation of hydroxide, thereby delaying alkaline burns to adjacent tissue. Efficacy is based on a 2018 study (Anfang et al) assessing the in vitro protective effects of various liquids in the cadaveric porcine esophagus and in vivoprotective effects of honey and sucralfate (Carafate®) compared to saline irrigations of batteries placed in the esophagus of live piglets. Both honey and sucralfate (Carafate®) effectively prevented the expected battery-induced pH increase and decreased the depth of the resulting esophageal injury.

References:

  1. Anfang RR, Jatana KR, Linn RL, Rhoades K, Fry J, Jacobs IN. pH-neutralizing esophageal irrigations as a novel mitigation strategy for button battery injury. The Laryngoscope. 2018 Jun 11. [Epub ahead of print]
  2. Jatana KR, Rhoades K, Milkovich S, Jacobs IN. Basic mechanism of button battery ingestion injuries and novel mitigation strategies after diagnosis and removalThe Laryngoscope. 2017 Jun;127(6):1276-1282.

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Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  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.

 

Button Battery -Update For Families

Children’s Healthcare of Atlanta Hope & Will blog: Common Batteries Pose Danger For Kids

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