This morning’s blog discussed mitochondrial liver disease.
Another useful reference:
The Journal of Pediatrics Volume 163, Issue 4 , Pages 942-948, October 2013 Mitochondrial Hepatopathies: Advances in Genetics, Therapeutic Approaches, and Outcomes Way Seah Lee, MD and Ronald J. Sokol, MD
A recent review provides advice for the evaluation of the child with suspected mitochondrial liver disease (JPGN 2013; 57: 269-79).
Clinical presentations:
Suggested Tiered Diagnostic Evaluation: Table 1 provides extensive suggestions.
Table 2 describes potential evaluations in other organs. For example, for brain, MRI, EEG and CSF.
Table 3 lists ~27 mutation/syndromes and clinical features.
The last three words from the conclusion of the publication are not supported by the review. The authors state that this systematic approach “can aid in making a timely, accurate, and cost-effective diagnosis.” While the authors do not provide estimates of the expense of these tests, they are probably very expensive, though less costly than a failed liver transplantation.
Bottomline: “Available technology to aid in diagnosis has improved substantially. Nonetheless, diagnosis of suspected mitochondrial disease in children is complicated.”
Related blog post:
Proven treatments for mitochondrial disorders | gutsandgrowth
While this topic is not directly related to pediatrics or pediatric gastroenterology, I found a recent article regarding the treatment of back pain with steroids interesting. This study challenges a treatment algorithm of using steroids to relieve inflammation triggering back pain. The investigators showed that steroids per se are not more beneficial then saline in improving back pain. Here is an excerpt from the NY Times, http://t.co/RF5O78odWe:
Injecting steroids into the area around the spinal cord, known as an epidural, is the most commonly used treatment for back pain, but a new review of studies suggests that injecting any liquid, even plain saline solution, works just as well.
Researchers pooled the results of 43 studies involving more than 3,600 patients who got various kinds of injections for back pain. As they expected, they found some evidence that epidural steroid injections provided more relief than steroid injections into the muscles.
But the study, published online in Anesthesiology, also found that there was little difference between the amount of relief provided by steroidal and nonsteroidal epidural injections.
The researchers suggest that any liquid injected epidurally can help reduce inflammation, enhance blood flow to the nerves and clean out scar tissue.
Comment: There are inherently many limitations in pooling 43 studies and trying to reach a definitive conclusion. Nevertheless, this study challenges some long-term treatment approaches.
Summary of study (Circulation 2013; 128: 337-343) from Epocrates (emphasis in blue by blog):
Study Question:
Is eating breakfast or not associated with risk for coronary heart disease (CHD) among men residing in the United States?
Methods:
Data for this analysis were from the Health Professionals Follow-up Study, an ongoing prospective study of male health professionals. Approximately 97% of participants were of white European descent. Eating habits, including breakfast eating, were assessed in 1992 in 26,902 American men, ages 45-82 years, who were free of cardiovascular disease and cancer. Participants were followed through mailed biennial questionnaires that ascertained medical history, lifestyle, and health-related behaviors. Cox proportional hazards models were used to estimate relative risks and 95% confidence intervals for CHD, adjusted for demographic, diet, lifestyle, and other CHD risk factors.
Results:
Participants who did not report eating breakfast were younger than those who did, and were more likely to be smokers, to work full-time, to be unmarried, to be less physically active, and to drink more alcohol. Men who reported that they ate late at night were more likely to smoke, to sleep <7 hours a night, or to have baseline hypertension compared with men who did not eat late at night. The late-night eating abstainers were more likely to be married and to work full-time, and ate on average one time less per day than the late-night eaters. The mean diet quality of the participants was high among participants, regardless of their breakfast or late-night eating status. During 16 years of follow-up, 1,527 incident CHD cases were diagnosed. Men who skipped breakfast had a 27% higher risk of CHD compared with men who did not (relative risk, 1.27; 95% confidence interval, 1.06-1.53). Compared with men who did not eat late at night, those who ate late at night had a 55% higher CHD risk (relative risk, 1.55; 95% confidence interval, 1.05-2.29). These associations were mediated by body mass index, hypertension, hypercholesterolemia, and diabetes mellitus. No association was observed between eating frequency (times per day) and risk of CHD.
Conclusions:
The investigators concluded that eating breakfast was associated with significantly lower CHD risk in this cohort of male health professionals.
Perspective:
These data suggest that time of meals is associated with other lifestyle behaviors. Adjustment for body mass index, hypercholesterolemia, hypertension, and diabetes [resulted in the relationship between breakfast (and late-night meals) and CHD no longer being significant.] Physicians may use this information to assist in the identification of those who may be at risk and need to improve lifestyle habits. However, it is unlikely that eating breakfast by itself would confer significant protection against heart disease.
Full text available at http://circ.ahajournals.org/content/128/4/337
Related blog post:
Skipping breakfast –boomerang effect for obesity | gutsandgrowth
A report on the “Effect of Carbonation on Brain Processing of Sweet Stimuli in Humans” (Gastroenterology 2013; 145: 537-39) highlights how the addition of carbonation could lead to increased consumption of sugar products. The authors examined neural activity in response to carbonated beverage consumption with the aid of functional MRI. An easy-to-read analysis of this study can be found at this link: Carbonation affects brain processing of sweet stimuli : Family …
An excerpt:
Carbonation produces a decrease in the neural processing of sweetness-related signals, particularly those from sucrose, a small functional neuroimaging study shows.
The findings, which suggest that the combination of CO2 and sucrose might increase consumption of sucrose, could have implications for dietary interventions designed to regulate caloric intake, according to Dr. Francesco Di Salle of Salerno (Italy) University and his colleagues.
To assess the interference between CO2 and perception of sweetness, as well as the differential effects of CO2 on sucrose and aspartame-acesulfame, (As-Ac, an artificial sweetener combination commonly used in diet beverages), the investigators performed two functional magnetic resonance imaging (fMRI) experiments to evaluate changes in regional brain activity…
The first experiment, performed in nine volunteers, analyzed the effect of carbonation in four sweet Sprite-based solutions, including one carbonated and sweetened with sucrose, one noncarbonated and sweetened with sucrose, one carbonated and sweetened with As-Ac, and one noncarbonated and sweetened with As-Ac. The second experiment evaluated the spatial location of the strongest neural effects of sour taste and CO2 within the insular cortex of eight subjects.
On fMRI, the presence of carbonation in sweet solutions “independently of the sweetening agent, reduced neural activity in the anterior insula (AI), orbitofrontal cortex (OFC), and posterior pons … the effect of carbonation on sucrose was much higher than on perception of As-Ac,” they noted, explaining that “at the perceptual level … carbonation reduced the perception of sweetness and the differences between the sensory profiles of sucrose and As-Ac.”
This effect may increase sucrose intake, but is also favorable to diet beverage formulations being perceived as similar to regular beverage formulations, the investigators reported…
It may be that taste and CO2-related information influence food choices and intake through integration in the tractus solitarius with input from the gastrointestinal tract, they suggested, explaining that “the reduced discrimination between sucrose and As-Ac induced by CO2 would promote the consumptions of low-calorie beverages and would converge with CO2-induced gastric distention in limiting caloric intake.”
This study was supported in part by the Coca-Cola Company. One author, Dr. Rosario Cuomo, was sponsored by the Coca-Cola Company. The remaining authors reported having no disclosures.
Related blog posts:
From the LA Times, http://t.co/9Tt2C4EOPf, an except:
Sell by, use by, best by. Most consumers use the dates stamped on foods to decide what to toss out — and they are often discarding food that’s good to eat, according to a report…
Those dates are manufacturers’ suggestions for when an item is at its peak, or efforts to help stores manage their inventory, and not indications of food safety, the report from the Natural Resources Defense Council and the Harvard Food Law and Policy Clinic says.
More than 90% of Americans say they use date stamps to decide whether to discard food, the report notes.
“I don’t know of any data that consuming a product beyond the date has caused illness,” said Ted Labuza, a professor of food science and engineering at the University of Minnesota who has studied shelf life for decades.
There are several ways that products can be contaminated and can cause illness, including poor handling on farms or in factories and stores, and improper treatment by consumers.
Wednesday’s report follows one about food waste from the NRDC showing that 40% of our food is discarded, resulting in losses of $165 billion a year….People are throwing away food because they believe it’s not safe, she said. And they also may be eating unsafe food because they put too much trust in those date labels.
While there is no research of the exact role those dates play in the 160 billion pounds of annual food waste in the U.S., estimates based on British studies suggest it could be $275 to $455 worth of food per household per year, the report said.
Business suffers, too, as millions of dollars of food is discarded before it’s sold based on those dates, the report said. There is a “dizzying” array of state laws regarding date stamps on food, including no regulations in nine states, Gunders said….
The NRDC report calls for three major changes:
—Putting sell-by dates — meant for businesses — into code so they are invisible to consumers.
—Establishing a uniform date labeling system that differentiates dates for safety from those for quality.
—Increasing the use of safe-handling instructions.
… Among the possibilities being considered is a two-date system that’s clearly marked for the retailer and the consumer.
U.S. Rep. Nita Lowey (D-N.Y.) said she planned to reintroduce the Freshness Disclosure Act, which she had previously proposed, to establish a consistent food-dating system. She said in a statement Wednesday that consumers now were “left in the lurch, forced to decipher the differences between ‘sell-by’ and ‘best if used by,’ and too often food is either thrown out prematurely, or families wind up consuming dangerous or spoiled food. The status quo is really quite absurd.”
I had a few free minutes so I decided to take a look at a bunch of upcoming lectures from the 2013 NASPGHAN upcoming meeting. With electronic media, it is easy to take a quick glance. Here’s the master link to all of the following talks:
Some of the power point lectures that I’ve seen so far:
At the time of this posting, I haven’t had a chance to look through these talks:
It takes a long time, even when there is no longer biochemical evidence of parenteral nutrition-associated liver disease (PNALD). A recent study provides long-term data from a population-based, cross-sectional study on liver histology from pediatric intestinal failure (IF) patients (Hepatology 2013; 58: 729-38). Patients were followed from 1984-2010. IF was defined as having either >50% small bowel resection or need for PN >30 days.
The 38 IF participants had a median age of 7.2 years. 16 remained on PN after 74 months (range 2.5-204), 22 had weaned off PN 8.8 years (range 0.3-27) earlier after an average of 35 months of PN exposure.
Key findings:
Bottomline: While there have been important clinical advances in the management of IF, the data from this study indicate that many patients who have normal liver biochemistries continue to have abnormal liver histology. Whether this will have an important clinical impact is not known.
Previous related blog entries:
A recent report provides a useful reference for the often difficult care of pediatric perianal Crohn disease (JPGN 2013; 57: 401-412).
The statement reviews the background, etiology, presentation and classification systems. Most helpful are Figures 2 & 3.
Figure 2 provides an algorithm for assessment and treatment of perianal fistula.
Figure 3 provides an algorithm for assessment and treatment of perianal abscess.
After outlining these algorithms, the report details the treatments. Almost all treatments that are effective or questionable for Crohn’s disease are discussed. The report reiterates that ‘corticosteroids must be used with caution in treatment of perianal fistula; studies have shown worse fistula outcomes for steroid-treated patients.
With regard to noncutting setons, the authors note that they do not prevent treatment with biologic agents and help prevent abscess formation. They “usually deteriorate and fall out on their own in about 1 year.” Medical therapy often allows for seton removal.
Ostomy diversion can be helpful in patients with severe perianal disease; however, “the risk of the ostomy becoming permanent is significant.”
Rectal strictures are typically dilated with multiple sessions with general anesthesia. The goal is at least 18 mm in younger patients and at least 24 mm in adolescents.
Related blog post:
Previous references:
A recent review (Ann Intern Med 2013; 158: 365-8) notes that “over the past 12 years, since the publication of the Institute of Medicine’s report, ‘To Err is Human: Building a Safer Health System, ‘ improving patient safety has been the focus of considerable public and professional interest.” The following is a summary of this report (Epocrates docalert summary):
Patient-safety experts in North America and the U.K. systematically reviewed the growing evidence base for 158 patient-safety topics, including 41 strategies designated as most important to practitioners and patients. All reviews are published in the Agency for Healthcare Research and Quality (AHRQ) evidence report entitled “Making Health Care Safer II: An Updated Critical Analysis of the Evidence for Patient Safety Practices” to update the original 2001 publication. After carefully analyzing each patient-safety problem and its related safety strategy, the authors strongly recommend immediate adoption of the following 10 strategies:
The authors also provide a list of 12 “encouraged” (rather than “strongly encouraged”) patient-safety practices, these are listed in Table 2 of the paper.
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