Briefly Noted: Bleeding in Noonan Syndrome, Pets Help Children’s Emotional Health

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B Briggs et al. J Pediatr 2020; 220: 154-8.  This study confirmed a high rate of bleeding problems in infants with Noonan syndrome.  9/70 (12.8%) had bleeding complications in those without comprehensive preoperative testing and undergoing major or dental surgery. Hematology evaluation is indicated in these children.

H Christian et al. J Pediatr 2020; 220: 200-6. Using data from the Longitudinal Study of Australian Children with data points at ages 5 (n=4242) and at ages 7 (n=4431).  Key finding: Owning pets was associate with improved emotional health for children: odds of abnormal emotional scores on SDQ questionnaire was OR 0.81, peer problems OR 0.71, and prosocial behavior OR 0.70 compared with non-pet owners. Prosocial behavior was most improved for children without any siblings with OR 0.21.

Updated Outcome Data for Necrotizing Enterocolitis

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A recent systematic review (IH Jones, NJ Hall. J Pediatr 2020; 220: 86-92) provides contemporary outcomes for infants with necrotizing enterocolitis (NEC). The authors analyzed from 38 articles (from 1375 abstracts); the authors excluded data from developing countries. This review included 21,349 infants with any stage of NEC and 7540 with Bell stage 2a+.

Key findings:

  • Overall mortality was 23.5% in all neonates with confirmed NEC (Bell stage 2a+), 34.5% for infants who underwent surgery
  • Mortality rates were higher for extremely low birthweight infants (<1000 g) at 40.5%; the rate was 50.9% for surgical NEC in this cohort
  • Neurodevelopmental disability was reported in only 4 studies and ranged between 24.8% and 61.1% (n=1209)
  • Intestinal failure was reported with an incidence of 15.2% to 35.0% (n=1370)

A limitation with this study is the lack of agreement on definitions/diagnosis for necrotizing enterocolitis and intestinal failure.

My take: This study shows that NEC still carries a high mortality.

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Sandy Springs

COVID-19 Retractions

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Two recent retractions indicate why multiple studies and careful analysis are often necessary to draw definitive conclusions, even from the most respected journals.

  1. NEJM: Retraction: Cardiovascular Disease, Drug Therapy, and Mortality in Covid-19. N Engl J Med. DOI: 10.1056/NEJMoa2007621.  Link to original article: Cardiovascular Disease, Drug Therapy, and Mortality in Covid-19
  2. Lancet: Retraction—Hydroxychloroquine or chloroquine with or without a macrolide for treatment of COVID-19: a multinational registry analysis Original study: Hydroxychloroquine or chloroquine with or without a macrolide for treatment of COVID-19: a multinational registry analysis

Surprised This Was Published: Liver Transplantation in Undocumented Immigrants

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I was keenly interested in a recent study: BP Lee, NA. Terrault. Liver Transplantation in Unauthorized Immigrants in the United States. Hepatology 2020; 71: 1802-12.  Given the potential for causing a political firestorm, I was surprised it was published.

Definitions: “Unauthorized immigrants, also termed illegal aliens in US federal statures are…all foreign-born non-citizens who are not legal residents.”  Since March 2012, UNOS has required transplant centers to record citizenship…”primarily to better understand transplant tourism.” The authors excluded international transplant tourists in their cohort.

Key findings: 

  • 116 of 43,192 (0.4%) liver transplant (LT) recipients were unauthorized immigrants
  • The majority were from Mexico (52%).  Others came from Guatemala (7%), China (6%), El Salvador (5%) and India (5%).
  • Unauthorized immigrant recipients had a similar risk of graft failure (sHR 0.74) and death (sHR 0.68), though at time of LT, there was higher disease severity (higher MELD scores and increased need for renal replacement therapy).
  • Most LTs for unauthorized immigrants took place in California (47%) and New York (18%).  Texas (3%) and Florida (4%) had a lower proportion of LTs for unauthorized immigrants based on population distribution.
  • The authors note that unauthorized immigrants are different that transplant tourists  –they pay social security tax/other taxes and contribute to organ donation (~3% of donated organs) whereas transplant tourists do not.
  • The authors note that unauthorized immigrant LTs were less than half the number of transplant tourist LTs; the later LT recipients are commonly individuals from Persian Gulf countries.
  • Current federal law mandates that LT be distributed based on “established medical criteria” which does not suggest a “tiered allocation system by citizenship.”  Almost half of the unauthorized immigrant LTs were covered by Medicaid.

My take: Unauthorized immigrants are underrepresented as LT recipients compared to their total population distribution in the U.S.  This likely is due to a number of barriers.  Interestingly, this population is not underrepresented when it comes to organ donation.

 

One More Problem with HIDA Scans

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A recent case study (A Adeyemi et al. J Pediatr 2020; 220: 245-8) provides information on 6 infants with a subsequent diagnosis of biliary atresia who had HIDA scans which reported excretion.

Methods: HIDA scans from 1992-2012 were reviewed from CHOP, this included 223 infants up to 4 months of age.

Key findings:

  • While there were six cases with HIDA scans that showed excretion into the bowel, none of these infants had truly normal HIDA scans.
  • 4 of the 6 patients had excretion qualified as slight, mild, or subtle and faint.
  • 5 of the 6 patients did not have the gallbladder visualized on HIDA.

Commentary:

  • HIDA scans are well-known to have a high sensitivity but a low specificity for biliary atresia (even with pretreatment choleretic agents). Liver biopsies have a higher diagnostic accuracy.
  • Since biliary atresia is a progressive disease, some excretion on HIDA does not exclude the diagnosis.  Though, age at HIDA was not a significant variable in this small series.

My takes:

  • Don’t rely too much on any test, including HIDA scans.  Equivocal findings need to be reported as such.
  • Fortunately, MMP-7 has emerged as another quick way with good (not perfect) specificity for biliary atresia.
  • Another related caveat is to look carefully at ultrasounds in this age group.  Often a small or retracted gallbladder is overlooked and could be an important clue to the diagnosis of biliary atresia.

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Rhododendron Flowers (Spring 2020)

High Risk Workers Need N95 Masks

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NY Times article that summarized recent Lancet study: Medical Workers Should Use Respirator Masks, Not Surgical Masks

Original Lancet Study (DK Chu et al. June 1, 2020
https://doi.org/10.1016/S0140-6736(20)31142-9): Physical distancing, face masks, and eye protection to prevent person-to-person transmission of SARS-CoV-2 and
COVID-19: a systematic review and meta-analysis

An excerpt from NY Times article:

The surgical masks used in risky settings like hospitals offer much less protection against the coronavirus, an analysis found…

The results, published on Monday in The Lancet, make it clear that the W.H.O. and the Centers for Disease Control and Prevention should recommend that essential workers like nurses and emergency responders wear N95 masks, not just surgical masks

N95 masks offered 96 percent protection, the analysis found, while the figure for surgical masks was 77 percent. The findings are particularly important as the United States moves to reopen the economy, Dr. Michaels said…

Workers in health care settings are not the only ones at high risk of coronavirus infection: employees in meatpacking plants and some farms are all also at high risk of coronavirus infection and could benefit from N95 masks..

The new analysis also suggests that covering the eyes with face shields, goggles and glasses may provide additional safeguards for health care workers and people in the community.

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Does Stopping Cannabis Improve Cyclic Vomiting Syndrome?

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Cannabis use has been linked to hyperemesis. However, a recent cross-sectional study (T Venkatesan et al. Clin Gastroenterol Hepatol 2020; 18: 1082-90) that stopping cannabis rarely results in improvement in cyclic vomiting syndrome (CVS).

This study enrolled 140 patients who had CVS with a mean age of 37 years, all seen at a specialized clinic; 41% were current cannabis users and were classified as regular users (≥4/wk, n=30) or occasional users (<4/wk, n=26).

Key findings:

  • Only 1 of 56 (2%) reported that cannabis abstinence (for a month) resolved their CVS symptoms and 1 of 56 (2%) noted improvement with cannabis abstinence.
  • 27 of 56 (56%) reported that cannabis abstinence worsened their CVS symptoms; 19 (40%) reported no change with cannabis abstinence
  • Only 1 patient taking cannabis met Rome IV criteria for cannabinoid hyperemesis syndrome (CHS). This patient subsequently resumed cannabis with a higher proportion of CBD (less THC) without recurrence of CVS symptoms.  This provides some support to the idea that THC in cannabis is responsible for CHS.

My take: (borrowed from authors) “If a patient with CVS and chronic regular cannabis use is refractory to standard therapy, we recommend a period of abstinence of at least 6 months or a duration of time that exceeds at least 3 consecutive cycles.”

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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

 

Nutritional Risks in Adolescents After Bariatric Surgery; Prevention of Childhood Obesity; Convalescent Serum for COVID-19

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S Xanthakos et al. Clin Gastroenterol Hepatol 2020; 18: 1070-81. Full Text: Nutritional Risks in Adolescents After Bariatric Surgery

This was a multicenter prospective cohort study with 226 adolescents (mean age 16.5 years, mean BMI of 52.7) who had either Roux-en-Y bypass (RYGB, n=161) or vertical sleeve gastrectomy (VSG, n=67).

Key findings:

  • At 5 years, 59% of RYGB and 27% of VSG had ≥2 nutritional deficiencies
  • The most prevalent abnormality we observed was hypoferritinemia, which affected nearly twice as many RYGB recipients by Year 5 compared with VSG.
  • Vitamin B12 status likewise worsened disproportionately after RYGB, despite similar trajectories of weight loss after VSG
  • Image below shows the prevalence of abnormal values for vitamins over time

My take: This study shows that adolescents undergoing VSG had fewer nutritional deficiencies than RYGB and provides data supporting nutritional monitoring after bariatric surgery.

B Koletzko et al. JPGN 2020 70: 702-10. Full Text: Prevention of Childhood Obesity: A Position Paper of the Global Federation of International Societies of Paediatric Gastroenterology, Hepatology, and Nutrition (FISPGHAN)

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Relative Efficacy of Medications for Irritable Bowel Syndrome with Constipation

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A recent systematic review and network meta-analysis (CJ Black, et al. Clin Gastroenterol Hepatol 2020; 18: 1238-39) reviewed the relative efficacy of medications for irritable bowel syndrome with constipation. In total, the 14 trials randomized 9113 patients.

Key points:

  • All treatments were significantly more effective than placebo
  • Linaclotide was ranked most effective; however, indirect comparison of active treatments revealed no significant differences between the individual drugs

Liver Shorts -May 2020 & CDC Recommendations for Office (NY Times Summary)

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NY Times:  C.D.C. Recommends Sweeping Changes to American Offices

FDA Approves Hepatitis C Pangenomic Treatment for Children (Mar 19, 2020):

The U.S. Food and Drug Administration today approved a supplemental application for Epclusa (sofosbuvir and velpatasvir) to treat hepatitis C virus (HCV) in children ages 6 years and older or weighing at least 37 pounds (17 kilograms) with any of the six HCV genotypes—or strains—without cirrhosis (liver disease) or with mild cirrhosis.

Review: NAFLD in China 1999-2018 J Zhou et al. Hepatology 2020; 71: 1851-4.

  • NALFD increased by 8-9% in prevalence, to 29.1%.  This means there are more than 230 million individuals with NAFLD in China.

Use of HCV-positive donors for liver transplantation to HCV-negative recipients. N Anwar et al. Liver Transplantation 2020; 26: 673-80. Key finding: HCV-positive organs had similar outcomes regarding graft function, patient survival and post-LT complications.

Recent Decline in Hepatocellular Carcinoma Rates in U.S. MS Shiels, TR O’Brien. Gastroenterol 2020; 158: 1503-5. Using SEER-21 population based cancer registries covering 37% of U.S. population, the authors found a recent decline in rates of HCC:

  • 2000-2016: 119,078 cases of HCC in SEER-21 registries, 5.84/100,000
  • Rates increased b 5.6% per year from 2000-2007, then by 2.7% per year from 2007 to 2013, subsequent rate reached a plateau and declined with drop of 1.4% per year (P=.12)
  • Improvement could have been due in part to improvement in viral hepatitis treatment; a less favorable explanation could be that the drop occured due to a death from another cause (eg. non-HCC death due to cirrhosis, opioid-related death

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Potential Treatment for Nonalcoholic Steatohepatitis N Chalasani et al. Gastroenterol 2020; 158: 1334-45. The study explored the use of Belapectin, an inhibitor of Galectin-3, in patients with nonalcoholic steatohepatitis and cirrhosis. n=162, phase 2 randomized, double-blind study. Key finding: 1 year of every 2 week infusions were safe but not associated with significant reductions in hepatic venous pressure gradient (HVPG) or fibrosis. However, in a subgroup without varices, there was lowered HVPG and lowered risk of new varices.

Treatment Options for Minimal Hepatic Encephalopathy:  RK Dhiman et al. Clin Gastroenterol Hepato 2020; 18: 800-12.  This meta-analysis which included 25 trials (n=1563) found the following:

  • For reversing minimal hepatic encephalopathy (MHE), rifaximin (OR 7.53) and lactulose  (OR 5.39) were effective with moderate quality evidence.  Probiotics had OR 3.89 and L-ornithine L-aspartate had OR 4.45 —both with low quality evidence.
  • For prevention of HE, L-ornithine L-aspartate had OR 0.19 (‘high moderate’ quality), and lactulose had OR 0.22 (moderate quality) were effective. Probiotics had OR 0.27 with low quality evidence.
  • The authors conlude that lactulose is the most effective agent for prevention and reversal of MHE.

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Curbside Humor