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More STEP Experience

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A retrospective review of all serial transverse enteroplasty (STEP) procedures from Omaha provides more data on long-term outcomes (J Pediatr 2014; 164: 93-8).  In total 51 patients underwent a total of 68 STEP procedures.  Underlying bowel diseases: NEC (n=8), Gastroschisis (n=29), Intestinal Atresia (n=11), and Volvulus (n=4).  Median length prior to STEP: 30 cm.

Results:

  • Median bowel length gain of 54% (median 51 cm) was noted with first STEP.
  • Median age of 1 year at first STEP
  • Parenteral caloire requirement decreased to median <20 kcal/kg/day at 1 year post-op
  • Longer length gains had higher risk of stricture formation.  Six strictures developed which occurred in the first 22 procedures in the series.  Subsequently, the authors state that they have not reduced the luminal diameter below 2 cm at any point.
  • 7 children underwent transplantation; 60% of non-transplanted children were enterally independent.  The authors note that “no child has gone on to transplant following a STEP procedure since August 2009.”
  • 48 of 51 children are alive at a median of 39 months of followup.

Bottomline: The authors philosophy is that the STEP should create uniformity of luminal diameter as the first priority rather than increased length.  Based on their experience, there appears to be a learning curve to optimizing outcomes with STEP procedures.

Also noted: J Pediatr 2013; 163: 1692-6.  Retrospective study of prospectively collected data from 178 children with regard to micronutrient deficiency among intestinal failure patients transitioning to enteral feeds. Figures 1 and 2 along with Table 2 provide the prevalence of micronutrient deficiency while receiving supplemental parenteral nutrition (PN) and while on full enteral nutrition (FEN).  Iron deficiency was most common in both situations with prevalence of 84% and 61% respectively. With the exception of folate (0%), all of the vitamins and micronutrients had fairly high rates of deficiency.  While on FEN defiencies were  the following:

  • Vitamin A 19%
  • Vitamin B12 6.5%
  • Vitamin D 30%
  • Vitamin E 6%
  • Copper 8%
  • Iron 61%
  • Selenium 4%
  • Zinc 23%

A recent post on The Pediatric Nutritionist blog provides a suggested approach to the monitoring of vitamins and micronutrients based on the need for parenteral nutrition and on the need to consider inflammatory markers in the interpretation of these lab values: The Importance of Nutrition Lab Monitoring Protocols Featuring …

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Newest FODMAPs Study for IBS

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From AGA twitter feed: http://t.co/vFwhS5YEF4 -Full text article.

From Abstract:

Methods

In a study of 30 patients with IBS and 8 healthy individuals (controls, matched for demographics and diet), we collected dietary data from subjects for 1 habitual week. Participants then randomly were assigned to groups that received 21 days of either a diet low in FODMAPs or a typical Australian diet, followed by a washout period of at least 21 days, before crossing over to the alternate diet. Daily symptoms were rated using a 0- to 100-mm visual analogue scale. Almost all food was provided during the interventional diet periods, with a goal of less than 0.5 g intake of FODMAPs per meal for the low-FODMAP diet. All stools were collected from days 17–21 and assessed for frequency, weight, water content, and King’s Stool Chart rating.

Results

Subjects with IBS had lower overall gastrointestinal symptom scores (22.8; 95% confidence interval, 16.7–28.8 mm) while on a diet low in FODMAPs, compared with the Australian diet (44.9; 95% confidence interval, 36.6–53.1 mm; P < .001) and the subjects’ habitual diet. Bloating, pain, and passage of wind also were reduced while IBS patients were on the low-FODMAP diet. Symptoms were minimal and unaltered by either diet among controls. Patients of all IBS subtypes had greater satisfaction with stool consistency while on the low-FODMAP diet, but diarrhea-predominant IBS was the only subtype with altered fecal frequency and King’s Stool Chart scores.

Conclusions

In a controlled, cross-over study of patients with IBS, a diet low in FODMAPs effectively reduced functional gastrointestinal symptoms. This high-quality evidence supports its use as a first-line therapy.

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What’s Wrong with Ordering an AXR for Constipation in the ER?

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My understanding is that shortly before my twin and I were born, a nurse used a pencil test to predict our genders.  Though my mother is quite smart, she believed the nurse knew what she was doing.  However, shortly thereafter, it turned out that I had a twin brother not a twin sister.

ER doctors often perform a similar service to the pencil test when they use an abdominal radiograph (AXR) to determine if their patients have constipation.  A new pediatric study from Toronto highlights this phenomenon and current recommendations (J Pediatr 2014; 164: 83-8).

Background:  this retrospective cohort study of children <18 years took place between 2008-2010.  As part of the study, a single pediatric radiologist (blinded to participant classification, assigned Leech scores to all misdiagnosis AXRs along with 20% of the remaining AXRs.  From a total of 112,381 ER visits, the review identified 3987 where constipation was the discharge diagnosis (3.5% of all visits).  In the cohort diagnosed with constipation, the mean age was 6.6 years.

Key findings:

  • Only 9% of children returned within 7 days.  20 of these (0.5%) had a significant misdiagnosis based on the authors definition, including 7 with perforated appendix, 2 with intussception, and 2 with bowel obstruction.  Other misdiagnosis included ovarian torsion, thalamic brain tumor, acute lymphoblastic leukemia, cardiomyopathy, ileal volvulus, and pancreatitis.
  • Children with a misdiagnosis had similar amounts of stool on AXR as those who were not misdiagnosed.
  • AXR was performed more frequently in those with a misdiagnosis (75% vs. 46%).
  • Rectal examination was documented in only 9% of those with a diagnosis of constipation (low frequency rectal examination has been shown in other ED-based studies).
  • Abdominal pain and tenderness were more common in those with a misdiagnosis.

Why I think this study is important:

While the authors point out that 1 in 200 children ultimately required a surgical or radiologic  intervention within 7 days, I do not think that this error rate or diagnostic delay is particularly high.  What is important is that this study reiterates the fact that AXRs are not useful for the diagnosis of constipation.  The authors note “reviews have concluded that there is no evidence of an association between clinical symptoms of constipation and fecal loading on AXR.”  Furthermore, AXRs may lead ER physicians to a cognitive diagnostic error.

Also, the misdiagnosis rate is much greater than reported in the study due to the definition adopted by the authors.  The authors did not include treatable infectious diseases (e.g.. pneumonia, urinary tract infection) as well as a large number of other medical diagnosis. Other “incipient” disease processes may have been missed including inflammatory bowel disease and celiac disease.

The authors imply that using a more standard definition of constipation would be useful, namely the Iowa criteria which requires the presence of 2 of the following:

  • ❤ stools/week,
  • ≥1 episoded of fecal incontinence/week
  • large stool palpable on rectal/abdominal examination
  • passing large stool which obstructs toilet
  • withholding posturing
  • painful defecation

The authors reference a study which indicated that AXRs should be restricted to patients with high-yield clinical features: prior abdominal surgery, foreign body ingestion, abnormal bowel sounds, addominal distention, and peritoneal signs.

Bottomline: AXRs do not establish a diagnosis of constipation.  Yet, after families have been told their child is constipated because of an AXR it is not easy to convince them that an AXR is about as useful as a pencil test for this diagnosis.

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What helps kids poop?

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While there are a number of answers to the above title, the answer that I’m looking for is physical activity (JPGN 2013; 57: 768-74).

With regard to the referenced study, a large prospective birth-cohort study (n=347 participants) in Rotterdam showed that preschool children with increased physical activity had about 1/3rd less frequency of functional constipation in the fourth year of life.  Activity measurements at the age of 2 years were accomplished by wearing ActiGraph accelerometers during 1 weekday and 1 weekend day.  Additionally, children who had physical activity of 60 min/day at age 4 had about 1/2 the likelihood of having functional constipation.  There are several limitations to the study; reduced activity and constipation could both be present in some individuals as a consequence of personality or psychologic attributes rather than physical activity having a causal relationship in causing constipation.

Bottomline: Another good reason to encourage physical activity –it might help with regular bowel habits.

Also, on a separate note, a recent blog post by Kipp Ellsworth is a useful reference for lab monitoring (micronutrients and vitamins) in children with short bowel syndrome:

Blog | The Pediatric Nutritionist | Covering the world of infant, child …

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What does the law require with regard to food allergen labeling? (plus one)

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From APFED twitter feed: What does the Food Allergen Labeling and Consumer Protection Act require?: http://youtu.be/nhBd9iTYkUQ?a (<2 minute video)

Plus one more reference on Achalasia:

A recent review on achalasia highlights the recent advances in our understanding of this disorder (Gastroenterol 2013; 145: 954-65). This post is mainly to note it as a useful reference.

Specific topics covered include the following:

  • High-resolution manometry
  • Criteria for achalasia diagnosis
  • Physiology/pathogenesis/pathophysiology.  Achalasia is “an autoimmune disease targeting esophageal myenteric neurons with both a cell-mediated and antibody-mediated attack directed against an as yet unidentified antigen.”
  • Subtypes (Figure 2 shows, in color, manometric pattens with each subtype)

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IBD Update 2014 (part 2)

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5. Inflamm Bowel Dis 2013; 19: 2927-36.  This reference is another article that tries to help discuss the risks and benefits of biologic therapy for pediatric inflammatory bowel disease.  After reviewing the potential risks, the authors provide their “Option Grid” (Page 2932).  The authors state, “in summary, the adult literature supports the concept of the early use of combination therapy…the risks associated with anti-TNF therapy are really not significantly different as compared with thiopurine therapy and perhaps in some cases safer.  Therefore, we should be moving closer to the idea of using anti-TNF therapy early, with or without an immunomodulator.  In the sickest patients, combination therapy probably adds benefit, and then once in remission, consideration can be given for stopping one of the medications, more likely the thiopurine.

6. Gut 2013; 62: 689-94.  Risk of ischemic heart disease in patients with inflammatory bowel disease: a nationwide Danish cohort study.  From 1997 to 2009, the authors compared 28,833 IBD persons to >4.5 million persons without IBD who were matched for age, gender, socioeconomic status, and calendar year.  With a mean follow-up of 13 years, they identified a 59% higher incidence rate of ischemic heart disease in patients with IBD.  Long-term use of immunosuppressive medications, such as azathioprine and anti-tumor necrosis factor-alpha agents, was not associated with an increased risk of ischemic heart disease.

7.   Gastroenterol 2013; 145: 1459-63.  AGA Guideline for Use of Thiopurines, Methotrexate, and Ant-TNF-alpha Biologic Drugs for the Induction and Maintenance of Remission in Inflammatory Crohn’s Disease. This reference was previously noted in blog (with a link) AGA Guidelines for the Use of Thiopurines and Anti … – gutsandgrowt.  The print version does have a nice algorithm (pg 1463).  The accompanying technical review: Gastroenterol 2013; 145: 1464-78.

8. BMJ 2013;347:f6633. Free full-text BMJ article PDF. (Thanks to Mike Hart for this reference) From the abstract:  During 3 421 972 person years of follow-up, we documented 284 cases of Crohn’s disease and 363 cases of ulcerative colitis. The risk of Crohn’s disease was inversely associated with physical activity (P for trend 0.02). Compared with women in the lowest fifth of physical activity, the multivariate adjusted hazard ratio of Crohn’s disease among women in the highest fifth of physical activity was 0.64 (95% confidence interval 0.44 to 0.94). Active women with at least 27 metabolic equivalent task (MET) hours per week of physical activity had a 44% reduction (hazard ratio 0.56, 95% confidence interval 0.37 to 0.84) in risk of developing Crohn’s disease compared with sedentary women with ❤ MET h/wk. Physical activity was not associated with risk of ulcerative colitis (P for trend 0.46). The absolute risk of ulcerative colitis and Crohn’s disease among women in the highest fifth of physical activity was 8 and 6 events per 100 000 person years compared with 11 and 16 events per 100 000 person years among women in the lowest fifth of physical activity, respectively. Age, smoking, body mass index, and cohort did not significantly modify the association between physical activity and risk of ulcerative colitis or Crohn’s disease (all P for interaction >0.35). Conclusion In two large prospective cohorts of US women, physical activity was inversely associated with risk of Crohn’s disease but not of ulcerative colitis.

Comment: While physical activity may directly reduce the risk of Crohn’s disease, it could also be an epiphenomenon of another unmeasured variable (eg. dietary habits) that modifies this risk.

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Understanding IBD Therapy Risks -A Good Link | gutsandgrowth  Provides link to useful 6-minute internet video for families.

IBD Update 2014 (part 1)

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A number of recent articles that may be helpful for clinicians who help patients with inflammatory bowel disease.

1. Inflamm Bowel Dis 2013; 19: 2778-86.  “The Incidence and Predictors of Lupus-Like Reaction in Patients with IBD treated with Anti-TNF therapies.”  Key result: 20 of 289 (6.9%) developed lupus-like reactions (LLRs).  Female gender and IBD-unclassified were more prevalent in this group.  Clinical features included arthropathy (100%); fatigue and dermatitis were common.  All tested positive for ANA, 16 of 20 also had anti-dsDNA.  LLRs resolved with cessation of culprit agent and steroids.  Only one patient had recurrence who had switched to an alternative anti-TNF.

2. Inflamm Bowel Dis 2013; 19: 2753-62. This phase 3, randomized open-label multicenter study enrolled 60 children and provided data regarding infliximab pharmacokinetics in patients with moderate-to-severe ulcerative colitis.  The findings indicate that infliximab exposure-response is similar to adult patients.  At week 8, those with higher serum infliximab levels (≥41.1 mcg/mL) had higher efficacy (response 92.9%, remission 64.3%) compared with those with a lower levels <18.1 mcg/mL (response 53.9%, remission 30.8%).  Trough levels (at week 30) for q8 week-dosing was 1.9 mcg/mL compared with 0.8 mcg/mL for q12 week-dosing.

3. Inflamm Bowel Dis 2013; 19: 2744-52. A lot of pediatric IBD patients are colonized with Clostridium difficile.  In this prospective study of 85 outpatient IBD pediatric patients and 78 age-matched controls, asymptomatic C difficile carriage was noted in 17% of IBD patients compared with 3% of controls.  Use of proton pump inhibitors was associated with an increased carriage rate.

4. Inflamm Bowel Dis 2013; 19: 2937-48.  Excellent review article regarding fertility and pregnancy for women with IBD.  This review includes a discussion about the timing of pregnancy with regard to remission, effects of surgery and medications, acceptable radiology testing in pregnant patients, and issues regarding delivery.

Updated Pediatric Expert Constipation Guidelines

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Updated guidelines for the diagnosis and treatment of constipation by NASPGHAN and ESPGHAN have undergone formal peer review are likely to be published soon (available online with the following link: bit.ly/1geLxrk).  “Evidence-Based Recommendations from ESPGHAN and NASPGHAN for Evaluation and Treatment of Functional Constipation in Infants and Children” by Merti Tabbers, Carlo DiLorenzo et al. The following are some of their recommendations.

Diagnostic recommendations:

  • The ROME III criteria are recommended for the definition of functional constipation for all age  groups.
  • Diagnosis of functional constipation is based on history and physical examination.
  • There is no role for the routine use of an abdominal X-ray to diagnose functional constipation.
  • A plain abdominal  radiography may be used in a child in whom fecal impaction is  suspected but in whom physical examination is unreliable/not possible.
  • Based on expert opinion, a 2-4 weeks trial of avoidance of cow’s milk protein may be indicated in the child with intractable constipation.
  • Routine laboratory testing to screen for hypothyroidism, celiac disease and hypercalcemia is not recommended in children with constipation in the absence of alarm symptoms. 

Treatment Recommendations:

  • We do not recommend the use of biofeedback as additional treatment in childhood constipation.
  • Polyethylene glycol (PEG) with or without electrolytes orally 1-1.5 gr/kg/day for 3-6 days is recommended as first-line treatment for children presenting with fecal impaction
  • An enema once a day for 3-6 days is recommended for children with fecal impaction if PEG is not available.
  • PEG with or without electrolytes is recommended as first-line maintenance treatment. A starting dose of 0.4 gr/kg/day is recommended and the dose should be adjusted according to the clinical response.
  • Addition of enemas to the chronic use of PEG is not recommended.
  • Based on expert opinion, use of milk of magnesia, mineral oil and stimulant laxatives may be considered as additional or second line treatment.
  • Antegrade enemas are recommended in the treatment of selected children with intractable constipation.

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Less Red Meat, More Anemia

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The title of this entry is not particularly surprising.  A recent study (JPGN 2013; 57: 722-27) showed that among 263 children (1.5-6 years in age) in Jerusalem, that anemia was present in 11.2%, iron deficiency in 22%, and iron-deficiency anemia in 3.7%.

Key finding:

Children with extremely low red meat consumption had 4-fold higher rates of iron deficiency than those who consumed ≥2 servings per week.  Poultry intake was not protective.

Bottomline: As more families choose a ‘health-conscious diet,’ iron deficiency may become more frequent.

Related blog entry: Help with hepcidin | gutsandgrowth

JAMA Thalidomide Study for Crohn’s Disease

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The link (from KT Park’s twitter feed): media.jamanetwork.com/news-item/drug-improves-remission-crohn-disease-among-children-adolescents/ …

An except:

The study included 56 children and was conducted August 2008-September 2012 in 6 pediatric care centers in Italy. Children were randomized to thalidomide or placebo once daily for 8 weeks. The primary measured outcomes were a reduction in the Pediatric Crohn Disease Activity Index (PCDAI) score of ≥ 25 percent or ≥ 75 percent at weeks 4 and 8 (clinical remission). Nonresponders to placebo received thalidomide for an additional 8 weeks. All responders continued to receive thalidomide for an additional minimum 52 weeks.

The researchers found that clinical remission was achieved by more children treated with thalidomide (13/28 [46.4 percent] vs. 3/26 [11.5 percent]). Responses were not different at 4 weeks, but greater improvement was observed at 8 weeks in the thalidomide group. Of the nonresponders to placebo who began receiving thalidomide, 11 of 21 (52.4 percent) subsequently reached remission at week 8. Overall, 31 of 49 children treated with thalidomide (63.3 percent) achieved clinical remission, and 32 of 49 (65.3 percent) achieved 75 percent response.

Average duration of clinical remission in the thalidomide group was 181 weeks vs. 6.3 weeks in the placebo group.

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