Pain in Children with Severe Neurologic Impairment

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A recent commentary (JM Hauer JAMA Pediatrics; 2018. doi: 10.1001/jamapediatrics.2018.1531) addresses a common misconception regarding children with severe neurologic impairment (SNI):

“we don’t think she experiences pain”

She notes that literature since 2002 has challenged this assumption and that this is addressed in a new AAP clinical report as well (Hauer J, Houtrow AJ. Pediatrics 2017; 139: e20171002).

Key points:

  • Children with SNI may have moaning, grimacing, changes in tone/body position in reaction to pain and treatment can make them comfortable.
  • “We can never prove that such a child does not feel pain…When parents of children with hydranencephaly were asked whether their child felt pain, 96% indicated yes.”
  • Pain can trigger changes in catecholamines, cortisol and stress hormones.  “These considerations suggest that untreated chronic pain is more harmful to the well-being of children with SNI than is treatment used for pain.”
  • Sometimes no source for pain is identified.  This may be related to a CNS etiology (alteration of CNS) and may benefit from treatment.
  • “It is time to do away with the question of whether these children feel pain and focus on how we as individuals” identify/consider pain

My take: Reframing this issue is important; pain can occur in children with SNI.  At the same time, we have to be careful that some “palliative” measures could paradoxically prolong suffering in some children.

Related blog post: Suffering

 

Low Adherence Rate with Polyethylene Glycol

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The sentiment of wanting to get their kid off laxatives/stool softeners is frequently expressed at GI visits.  I certainly understand this.  Though, if a child is not stooling adequately when these medicines are withheld, this is usually detrimental for the child.

Given the frequency of this sentiment, it is not surprising that a recent study (IJN Koppen et al. J Pediatr 2018; 199: 132-9) reports low adherence with polyethylene glycol treatment in children with functional constipation.

In this cross-sectional survey using the Medication Adherence Report Scale (MARS-5), with scores of ≥23 indicating better adherence, the authors found that only 43 of 115 (37%) children were adherent.  The authors note that one of the determinants of adherence was treatment convenience.

The MARS-5 does not objectively measure the exact intake of medication; thus, the exact rate of adherence is unclear.  In addition, there is likely to wide variation in adherence among different populations.

My take: this study shows, at least in some populations, a low adherence with constipation therapy.  Sticking with treatment, for constipation and every other condition, usually results in better outcomes.

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If someone is not taking their medication, perhaps this cereal would help. (I am not officially endorsing this product, but think the name is funny.)

Transmural Disease, Biomarkers, and Correlation between MRI and Endoscopy

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A recent study (I Weinstein-Nakar et al. Clin Gastroenterol Hepatol 2018; 16: 1089-97, editorial 1037-39)) provide data from 151 children who underwent multiple modalities to assess their Crohn’s disease (CD) (ImageKids Study group).

Key findings:

  • MRE and ileocolonoscopy had concordance in 69% of cases.  55% had neither transmural nor mucosal healing, 14% had both transmural and mucosal healing.
  • MRE did not show features of active disease in 25% that was identified on ileocolonoscopy.  This is an expected finding given the ability of endoscopy (& capsule endoscopy) to identify milder mucosal lesions more precisely.
  • MRE did show evidence of disease in 6% who had unremarkable ileocolonoscopy (mucosal healing)
  • Calprotectin at a cut-off of 100 mcg/mL had 71% sensitivity and 92% specificity for diagnosing mucosal and transmural healing whereas a level of 300 mcg/mL had a sensitivity of 80% and specificity of 81%.

My take: This study confirms the complementary nature of cross-sectional imaging with endoscopy to determine healing.  In addition, in children with CD, calprotectin levels of more than 100 mcg/mL could indicate the need for further assessment (if this would affect management).

This is in agreement with another recent post: IBD Reviews: Antibiotics and Biomarkers:  “a calprotectin has a high level of excluding active inflammation/IBD. In populations with IBD, levels more than 250 mcg/g indicate a high likelihood of active inflammation whereas levels between 100-250 are indeterminate.”

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Sunshine Meadows, Banff Nat’l Parke

Better Diet -Less Fatty Liver

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A recent study (J Ma et al. Gastroenterol 2018; 155: 107-17) shows that a “better diet” was associated with less liver fat.

Among the 1521 participants form a Framingham Heart Study cohort (Mean age 51 years at start of study), the authors assessed diet with a 125-item Harvard food frequency questionnaire and liver fat using liver-phantom ratio (LPR) on CT images between 2002-2005 and then again 2008-2011.  They specifically looked at 2 diet scores:

  • Mediterranean-style diet score (MDS)
  • Alternative Healthy Eating Index (AHEI)

Key findings:

  • For each 1 standard deviation increase in MDS, the LPR increased (less liver fat) by 0.57 and the odds for incident fatty liver decreased by 26% (P=.002)
  • Similarly, for each 1 standard deviation increase in AHEI, LPR increased by 0.56 and the odds for incident fatty liver decreased by 21% (P=.02)

My take: This study shows that Improved diet quality over 6 years was associated with reduced liver fat accumulation

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DGAT1 Deficiency

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Briefly noted: JM van Rijn et al. Gastroenterol 2018; 155: 130-43.  This study of 10 patients from 6 families (5 had consanguinity) was the largest cohort to date describing the clinical features in this disorder and used patient-derived fibroblasts and organoids to understand the pathophysiology.

Key clinical features:

  • Early-onset vomiting and/or diarrhea
  • Protein-losing enteropathy/hypoalbuminemia

Key finding in study:

  • These patients had altered lipid metabolism and were susceptible to lipid induced cell death.  Thus DGAT1 mutations cause congenital diarrhea and this is linked to fat intolerance.

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Elastography –Accuracy in Children

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Background: Transient elastography may help identify patients at greater risk for advanced fibrosis but there is a significant overlap between fibrosis stages and values with transient elastography. In some conditions, like fatty liver, elastography may be less helpful than in others (eg. chronic viral hepatitis).

A recent study (J Pediatr 2018; 198: 84-9) examined the results of transient elastography in 267 children (97 for calibration, 170 for validation) between 2006-2016.  The median age was 13 years.  Liver diseases included autoimmune (21%), viral (19%), fatty liver (11%), cholestatic (9%), primary sclerosing cholangitis (9%) and post-transplantation (12%).

Key findings:

  • Cut points to discriminate F3-F4 and F4 were >8.6 kPa and >11.5 with 81% and 84% accuracy, respectively in calibration cohort
  • To discriminate F3-F4 and F4 in validation cohort, these cut points, >8.6 kPa and >11.5, had accuracy of 67% and 75% respectively

Figure 2 provides much greater detail in the typical values for each Metavir stage. For example, in the calibration cohort, the median values and range were the following:

  • F0   6.0 (3.2-12.4)
  • F1   6.2 (3.2-75)
  • F2   5.8 (2.5-52.7)
  • F3  10.3 (4.9-32.6)
  • F4  20.5 (5.9-68.1)

In the validation cohort, values were similar:

  • F0   6.2 (3.0-75.0)
  • F1   7.1 (3.3-31.6)
  • F2   7.1 (2.5-52.7)
  • F3  9.6 (4.4-75.0)
  • F4  20.8 (6.8-75.0)

My take: Elastography in measuring liver stiffness is a lot like checking a CRP for inflammatory bowel disease.  That is, it can be helpful but cannot be relied on the same way as many radiographic tests (eg. CT scans for appendicitis).

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Moraine Lake, Banff

Expert Advice on Bloating

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A recent article (AK Kamboj, AS Oxentenko. Clin Gastroenterol Hepatol 2018; 16; 1030-33) provides some useful guidance on bloating.

They describe bloating as an acronym:

  • Bowel disturbance (constipation, SIBO, celiac, IBD)
  • Liquid (ascites)
  • Obstruction
  • Adiposity
  • Thoracic (overexpansion, diaphragm contraction)
  • Increased sensitivity (functional bloating, IBS, dyspepsia)
  • Neuromuscular (gastroparesis, impaired accommodation, medications)
  • Gas (aerophagia, dietary sources, post-Nissen)

The diagnostic approach they recommend:

  • If bloating with diarrhea, evaluate diet, SIBO, celiac, IBD, IBS-D, and medications
  • If bloating with constipation, evaluate for constipation, pelvic floor dysfunction, IBS-C, and medications
  • If bloating and suspected mechanical disturbance, evaluate for gastric outlet obstruction/small bowel obstruction
  • If bloating without bowel disturbance, consider aerophagia, gastroparesis, and functional dyspepsia

Treatment:

  • Treat any underlying disorder
  • For mild symptoms, reassurance may be sufficient
  • Dietary modifications to avoid food triggers & reduce fermentable food products
  • Treating constipation when present
  • A large number of other treatments can be considered as well including antispasmotics, agents to help with visceral hyperalgesia, cognitive behavioral therapy

My take: I like BLOATING acronym, though the 5 Fs I learned a long time ago is a little easier for me to remember — which include flatus (gas), feces (constipation), fluid, fat, and fetus/masses. Flatus can be caused by swallowing air (aerophagia), malabsorption (celiac, lactose intolerance, parasites), muscular discoordination (abdominal phrenic dyssynergia), and motility problems.”

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Active Colitis More Likely in Children in Clinical Remission Who Have PSC and IBD

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A recent study (A Ricciuto et al. Clin Gastroenterol Hepatol 2018; 16: 1098-1105) provides more data regarding the lack of symptom correlation and inflammatory bowel disease (IBD) activity in children with primary sclerosing cholangitis (PSC).

In a prospective study of children with colonic IBD with and without PSC, the authors followed clinical features (eg. PUCAI), fecal calprotectin and endoscopy severity.

Key findings:

  • Patients with PSC-IBD (n=37) in clinical remission had higher endoscopic scores and greater odd of active endoscopic disease than IBD-only controls (n=50) (odds ratio 5.9, with CI 1.6-21.5)
  • Fecal calprotectin level <93 mcg/g were identified mucosal healing with 100% sensitivity and 92% specificity when compared with UC Endoscopic Index of Severity (UCEIS)

Overall, this study is in agreement with a prior adult study showing higher levels of active disease in those with PSC-IBD compared to those with IBD alone, despite clinical remission (Why does PSC increase the risk of colorectal cancer in UC?).

My take: Particularly in individuals with the combination of IBD-PSC, objective biomarkers (eg. Calprotectin) are needed to identify the accuracy of clinical remission; though, even in patients with IBD without PSC, objective biomarkers are needed as well due to the limitations of clinical symptom indices.

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Clinical Practice Update: Extraesophageal Symptoms Attribute to Gastroesophageal Reflux Disease

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A recent practice update (MF Vaezi, D Katzka, F Zerbib. Clin Gastroenterol Hepatol 2018; 16: 1018-29) reviews the data and provides up-to-date recommendations for extraesophageal symptoms attributed to gastroesophageal reflux disease (GERD) in adults.

Extraesophageal symptoms attributed to GERD could include cough, asthma, hoarseness, sore throat, sinusitis, dental erosions, and ear pain.

Key recommendations:

  • Non-GI evaluations by ENT, pulmonary and/or allergy are essential and often should be performed initially in most patients.
  • “Abnormalities seen on endoscopy have poor predictive value for determination of GERD as the cause of extraesophageal symptoms”
  • “Ambulatory pH/impedance monitoring…have limited ability to establish GERD as the cause of an extraesophageal symptom.  The main role of testing is to document the absence of GERD.”
  • Empiric therapy with ‘aggressive’ acid suppression for 6-8 weeks can help in assessing association between reflux and extraesophageal symptoms.
  • Testing for reflux on therapy should be considered mainly in those with high probability of baseline reflux (eg. previous esophagitis, Barrett’s esophagus, or prior abnormal pH study).
  • Surgical treatment is discouraged in those with extraesophageal reflux symptoms unresponsive to aggressive PPI therapy

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Management of Pediatric Ulcerative Colitis -ESGHAN/ECCO Recommendations

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Two complementary articles provide extensive guidance on the management of ulcerative colitis and acute severe colitis:

  • D Turner et al. JPGN 2018; 67: 257-91
  • D Turner et al. JPGN 2018; 67: 292-310

Between the two articles there are more than 60 practice recommendations, more than 120 practice points, and more than 700 references.  As such, these articles are probably better for a journal review meeting rather than a brief blog post.

Figure 1 (2nd article, page 299) provides a handy algorithm for management of acute severe colitis:

  • On day 1-2, the algorithm recommends stool studies, starting methylprednisolone, and withholding 5-ASA.
  • On day 3, if PUCAI <45, suggests continuing steroid and transitioning to oral therapy when PUCAI <35.  On day 3, if PUCAI ≥45, the authors suggest screening for second line therapy, involve surgery (to discuss colectomy if there is nonresponse to medical treatment), and looking for CMV infection (eg. sigmoidoscopy).
  • On day 5, if PUCAI >65, recommendation is to start 2nd line Rx (eg. infliximab, tacrolimus, or cyclosporine). If PUCAI 35-65, continue corticosteroids for additional 2-5 days. The authors note that infliximab is preferred 2nd line Rx unless planning to transition to vedolizumab.
  • The authors recommend weaning corticosteroids when 2nd line Rx is started
  • The authors recommend addition of an immunomodulator for at least 6 months in responders to infliximab in effort to lower the risk of colectomy.
  • The authors state “urgent colectomy is recommended following failure of 1 second-line therapy.”

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.

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