Rough Skin -Tough Case

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Briefly noted:

“D is Delay” NEJM 2014; 371: 2218-23.  This case presentation described how a 47-year-old homeless man presented with a neuropathy and over the course of FIVE YEARS developed dermatitis, diarrhea, and dementia.  Ultimately, he was diagnosed with niacin deficiency or pellagra. It is almost painful reading the case report knowing how long it took to establish the diagnosis. Take home points from this case report:

  • Pellagra is derived from the Italian pelle agra which means “rough skin”
  • Nutritional deficiencies are difficult to diagnose in this country due to their rarity/lack of pattern recognition

Family Feud with Allergies and Celiac Disease

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A recent article in Allergic Living highlights the common phenomenon of other family members not believing or not willing to make changes in the face of food allergies and celiac disease.

Here’s an excerpt:

Every day, adults and kids are diagnosed with food allergies or celiac disease, and they naturally expect that the people closest to them will take the most care – as they would with any serious health condition. After all, you should be able to trust your mom to keep gluten out of her gravy, and assume that, when your brother babysits your peanut-allergic daughter, he carefully reads the ingredients on that chocolate bar, right?

For too many living with food allergies and celiac disease, sadly the answer is no. In the fall of 2010, Allergic Living sent out a request for anecdotes of family experiences (both good and bad), and within days we were inundated with responses…

In the end, there is no magic cure that will work for every family because complex problems cannot be solved with simple solutions – and, as they say, you don’t choose your family. But clear and calm communication is vital, as is the ability for those living with allergies to put themselves in their relatives’ shoes.

Related blog post:

Save a life with free allergy education | gutsandgrowth

Update on Enteral Nutrition in Pediatric Intestinal Failure

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It is always nice to see how other centers manage clinical problems.  In a recent review (J Pediatr 2014; 165: 1065-90) from Boston Children’s, the authors provide details on how they use enteral nutrition in pediatric intestinal failure (IF) patients.  Prior to reviewing their approach, the authors provide a few definitions:

  • “IF occurs when there is a reduction of functional intestinal mass necessary for adequate digestion and absorption for nutrient, fluid, and growth requirements, resulting in the need for intensive nutritional support.”
  • “IF resulting from extensive intestinal resection is termed SBS” (short bowel syndrome)

The authors also discuss intestinal adaptation and factors that predispose to improvement.  Enteral nutrition (EN) stimulates adaptation and ‘gut rest’ results in atrophy of intestinal mucosa.

Key points:

  • “Prompt initiation of enteral feeding after bowel resection has been shown to decrease the duration of hospitalization”
  • “The optimal choice for EN in infants with IF seems to be human milk…If human milk is unavailable, amino acid-based formulas have been associated with improved outcomes.”
  • If intact colon with ileocecal valve, supplementation with dietary fiber (e.g.. green beans) at 2 g/kg/d may be helpful.
  • In this population, there is a high prevalence of micronutrient deficiencies while on partial PN support, TPN (depending on availability of components), and when on exclusive enteral feedings.
  • “We commonly employ an approach that uses … continuous feeding at night and bolus feeding during the day.
  • Outcomes of IF are reviewed (noted in previous blog entry –see below). Citrulline can be useful predictor of enteral autonomy.

Feeding Advancement Principles -Figure 1:

When feeds are held, usually held for 8 hours and then restarted at 75% of previous rate

Stool output:

  • If <10 mL/kg/d or < 10 stools/d —->advance rate by 10-20 mL/kg/d
  • If 10-20 mL/kg/d or 10-12 stools/d —>no change
  • If >20 mL/kg/d or >12 stools/d  —->reduce or hold feeds

Ostomy output:

  • If 2 mL/kg/h —> advance rate by 10-20 mL/kg/d
  • If 2-3 mL/kg/h –>no change
  • If >3 mL/kg/h  –>reduce or hold feeds

Also suggested to reduce or holding feeds when/if:

  • signs of dehydration
  • stool reducing substances >1%
  • gastric aspirates > four times previous hour’s infusion rate

Oral feeds:

  • When developmentally appropriate, offer one hour’s worth of continuous feeds BID-TID after 5 days of continuous feeds.  Hold tube feeds during oral feeds.
  • More than an hour’s worth of oral feeds once infant has reached full volume of feeds by continuous route and gaining weight.

Take-home message: Outcomes of IF have improved.  This review provides one approach towards optimizing enteral nutrition.

Related blog posts:

 

Taking a History: Man versus Computer

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As noted in a previous blog (“The future of gastrointestinal disease and symptom … – gutsandgrowth), a well-designed computer program (Link: Computer-generated history) allows GI patients to provide a history that is better than a history taken by a physician.

Here’s a link: LA Times Business review of this report

Here’s the abstract of the study:

Computer-Generated Vs. Physician-Documented History of Present Illness (HPI): Results of a Blinded Comparison

Christopher V Almario, William Chey, Aung Kaung, Cynthia Whitman, Garth Fuller, Mark Reid, Ken Nguyen, Roger Bolus, Buddy Dennis, Rey Encarnacion, Bibiana Martinez, Jennifer Talley, Rushaba Modi, Nikhil Agarwal, Aaron Lee, Scott Kubomoto, Gobind Sharma, Sally Bolus, Lin Chang and Brennan M R Spiegel

Objectives:

Healthcare delivery now mandates shorter visits with higher documentation requirements, undermining the patient–provider interaction. To improve clinic visit efficiency, we developed a patient–provider portal that systematically collects patient symptoms using a computer algorithm called Automated Evaluation of Gastrointestinal Symptoms (AEGIS). AEGIS also automatically “translates” the patient report into a full narrative history of present illness (HPI). We aimed to compare the quality of computer-generated vs. physician-documented HPIs.

Methods:

We performed a cross-sectional study with a paired sample design among individuals visiting outpatient adult gastrointestinal (GI) clinics for evaluation of active GI symptoms. Participants first underwent usual care and then subsequently completed AEGIS. Each individual thereby had both a physician-documented and a computer-generated HPI. Forty-eight blinded physicians assessed HPI quality across six domains using 5-point scales: (i) overall impression, (ii) thoroughness, (iii) usefulness, (iv) organization, (v) succinctness, and (vi) comprehensibility. We compared HPI scores within patient using a repeated measures model.

Results:

Seventy-five patients had both computer-generated and physician-documented HPIs. The mean overall impression score for computer-generated HPIs was higher than physician HPIs (3.68 vs. 2.80; P<0.001), even after adjusting for physician and visit type, location, mode of transcription, and demographics. Computer-generated HPIs were also judged more complete (3.70 vs. 2.73; P<0.001), more useful (3.82 vs. 3.04; P<0.001), better organized (3.66 vs. 2.80; P<0.001), more succinct (3.55 vs. 3.17; P<0.001), and more comprehensible (3.66 vs. 2.97; P<0.001).

Conclusions:

Computer-generated HPIs were of higher overall quality, better organized, and more succinct, comprehensible, complete, and useful compared with HPIs written by physicians during usual care in GI clinics.

Amplified Pain Syndromes in Children

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A recent review (Curr Opin Rheumatol 2014; 23: 1-12 -thanks to our pain team for sending reference) makes a number of important points regarding the pathogenesis and management of amplified pain syndromes (APS).

Table 1 lists the diagnosis and pain presentations.  These include complex regional pain syndromes, juvenile fibromyalgia, diffuse idiopathic pain, concomitant conditions (including irritable bowel syndrome, chronic fatigue syndrome, interstitial cystitis, chronic headache, functional abdominal pain, and conversion symptoms/disorder).

Key points:

  • Pediatric APS are widespread and under-recognized
  • Pathophysiology is complex with numerous contributors “including central sensitization, abnormal cytokine production, sympathetic-sensory disorders, autoimmune responses, altered blood flow, genetic predisposition, and psychosocial factors.”
  • “The clinical effectiveness of medication management in pediatric APS remains unclear and controversial.”  It is noted that preoperative gabapentin and pregabalin may reduce the incidence of chronic post surgical pain (in adults); this has not been documented in a pediatric population.
  • “Exercise-based and cognitive-based treatments remain the cornerstone of therapy.” Intensive multidisciplinary pain rehabilitation “restores functioning rapidly, reduces pain in the long run, improves comorbid psychological distress, and reduces medical utilization.”
  • Potential elements of treatment noted in Table 2 (geared more towards rheumatology), including exercise, desensitization, self-regulation (eg. diaphragmatic breathing, guided imagery), and stress management/counseling.

Bottomline: For children with severe pain symptoms, multidisciplinary pain teams can be very helpful.  However, there is not a simple pill that will fix everything.

Related blog posts:

 

Fidaxomicin Effective in Open-Label Pediatric Study

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At combined annual Infectious Diseases Society of America/Pediatric Infectious Diseases Society, the results of an open-label study of fidaxomicin reported the following (from Family Practice News Link):

“Fidaxomicin, an approved treatment for Clostridium difficile-associated diarrhea in adults, appears safe and effective in children, according to findings from an open-label study.

The overall clinical response rate was 92% in 38 children aged 11 months through 17 years who were treated with 32 mg/kg/day for 10 days. Although 74% of patients had at least one adverse event, most of the events were mild (45%) or moderate (21%) in severity and were the type of events that would be expected in children with moderate to severe underlying illness….

Of the children in the study with a clinical response, 29% experienced a relapse, but all but one of these children had a history of recurrent C. difficile-related diarrhea.

Fidaxomicin is a first-in class narrow spectrum macrocyclic antibiotic drug approved in the United States and several other countries for the treatment of C. difficile-associated diarrhea in adults.“

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Race Associated with Outcomes in Intestinal Failure

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“The best preparation for tomorrow is to do today’s work superbly well”  –William Osler (quote cited in NEJM 2014; 371: 1565-66).

The quote above is not directly related to today’s post but I liked it a lot.

While race “encompasses social, economic, and cultural issues,” it is a marker for health outcomes including in children with intestinal failure (JPGN 2014; 59: 537-43). This Pediatric Intestinal Failure Consortium study retrospectively analyzed 272 subjects, though 22 did not have adequate data regarding race.  In this cohort, there were 204 white and 46 nonwhite children.

Key findings:

  • Nonwhite children were more likely to die without an intestinal transplant (P<0.001). At 48 months after entry criteria were met, cumulative probability of death without an intestinal transplant was 0.40 for nonwhite children compared with 0.16 for white children.
  • Nonwhite children were less likely to receive an intestinal transplant (P=0.003). At 48 months after entry criteria were met, cumulative probability of receiving an intestinal transplant was 0.07 for nonwhite children compared with 0.31 for white children.

These findings held up when examined for biological factors like low birth rate and reason for intestinal transplantation; other factors that were accounted for included evidence of liver disease, residual bowel length, and whether child had received care at an intestinal transplantation center.  Even factors like receiving breast milk in the nursery were similar between the two groups.

Bottomline: Nonwhite race appears to be a marker for poor outcomes in children with intestinal failure.  Based on this retrospective data which examined multiple factors, the reasons do not have a biological basis.  As such, issues like barriers to treatment/access to care, social support, parental education, and cultural differences need to be considered.

 

Mobile Health Apps

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Last month I posted some notes from Brennan Spiegel who gave the keynote address at NASPGHAN (“The future of gastrointestinal disease and symptom …).  This past month, he reviewed mobile health apps.  Here’s the link: AGA Perspectives on mHealth Apps

Here’s an excerpt:

The IMS Institute for Healthcare Informatics evaluated each of the apps on the market and concluded that well over 90 percent were of low quality…

IMS points to iTriage as a model for evaluation. iTriage collects signs and symptoms, crunches the input through algorithms, yields a differential diagnosis, and suggests an action plan and list of appropriate local providers. “Virtual visit” apps like HealthTap, Teladoc, American Well and MDLive go a step further by offering patients direct and immediate access to a physician through their smartphone or tablet device. For $49 on average, physicians can conduct a virtual face-to-face interview, make a diagnosis and even send prescriptions. The app “Pager” goes yet a step further. Founded by the team that developed Uber — the wildly successful car service app — Pager allows patients to select among a panel of doctors and obtain rapid house calls for $199. Insurance is now starting to cover some of the virtual visits and app-generated house calls.

10 Years of Intractable Diarrhea

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A recent report (JPGN 2014; 59: 571-76) from Turkey reviewed their experience with intractable diarrhea of infancy from 2000-2010.  This retrospective review included 60 hospitalized patients.

Key findings:

  • Carbohydrate malabsorption, including glucose-galactose malabsorption, and food allergy accounted for 11 patients (18%) each.
  • 16 patients (27%) had no definitive diagnosis.
  • Other etiologies included infections, inflammatory bowel disease, autoimmune enteropathy, microvillus inclusion disease, tufting enteropathy, short bowel syndrome, cystic fibrosis, proprotein convertase 1 deficiency, congenital disorder of glycosylation, abetalipoproteinemia.

The authors provide a useful diagnostic algorithm (Figure 1).

Related blog post:

Four IBD Articles –Four Teaching Points

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  1. Inflamm Bowel Dis 2014; 20: 1891-1901
  2. Inflamm Bowel Dis 2014; 20: 1996-2003
  3. Inflamm Bowel Dis 2014; 20: 2013-21.
  4. Inflamm Bowel Dis 2014; 20: 2056-66.

The first article is listed as a ‘basic science’ article.  However, it has direct relevance to the clinical problem of anti-TNF-induced psoriasiform skin lesions.  The article notes that this problem affects about 5% of patients treated with anti-TNF agents.  The authors found that IL-36γ and IL-17C are increased in anti-TNF-induced psoriasiform skin lesions of patients with Crohn’s disease (n=13 patients).  An important clinical point was that 7 of these patients with “severe anti-TNF induced skin lesions were successfully treated with the IL-12/IL-23 neutralizing antibody ustekinumab.”  This was superior to topical steroids or topical tacrolimus.

The second article is a proof-in-principle type article showing that proactive measurements of infliximab (IFX) levels may improve outcomes.  This retrospective observational study examined 48 patients who had proactive IFX levels. In 12 of 48, IFX dosing was escalated after the first proactive monitoring.  In addition, over the study period, 15% of patients had their dosing lowered. In those with proactive monitoring, the probability of remaining on IFX was >80%.  Those patients who achieved trough levels >5 mcg/mL had >90% probability of remaining on IFX therapy.  The authors hypothesize that better IFX levels may reduce anti-infliximab levels.

The third article examines carbohydrate intake in relation to the development of Crohn’s disease (CD) and ulcerative colitis (UC). The authors utilized the “EPIC” cohort (European Prospective Investigation into Cancer and Nutrition) (Public Health Nutr 2002; 5: 1113-24).  among 401,326 enrollees at recruitment, the dietary intakes of carbohydrates were measured using validated food frequency questionnaires. In this cohort, 110 developed CD and 244 developed UC during followup. Key finding: there was no significant risk for IBD based on total carbohydrate intake.  This study does not exclude the possibility that specific carbohydrates could have an etiological role.

The fourth article, a case-control study (2002-2011),  examines risk factors for endoscopy-associated perforation and perforation-associated complications (PAC) in patients with and without IBD. n=217,334 lower endoscopies (with 9518 in IBD patients).  Perforation rates: 18.91 per 10,000 and 2.50 per 10,000 for IBD and non-IBD endoscopy respectively.  The use os systemic corticosteroids at the time of endoscopy was associated with a 13 times greater risk for PAC.

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