Category Archives: Pediatric Gastroenterology Intestinal Disorder

A cure for satiety

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A safe cure for excessive appetite is a holy grail in pharmacology.  Although gluttony is a much more pervasive problem that poor appetite, an effective cure for satiety is also needed.  In the clinical setting, poor appetite remains a common problem with and without underlying problems.  Treatments to this point, such as cyproheptadine or megestrol, may be useful in some patients.  Better treatments are needed.  One drug of interest is ghrelin (Cancer 2012; DOI: 10.1002/cncr.27430).

The introduction summarizes ghrelin’s biologic activity: “Ghrelin is an endogenous ligand for the growth hormone (GH) secretagogue receptor and is secreted predominantly by gastric endocrine cells.  It induces dose-dependent, GH-releasing activity; stimulates appetite and food intake; and triggers a positive energy balance through a central mechanism involving hypothalamic neuropeptides.”

Plasma ghrelin levels decrease after gastric resection, helping to maintain weight loss. Therefore, the stomach may act as endocrine organ to maintain appropriate weight. Exogenous administration may increase appetite.

This cited article reports a prospective randomized placebo-controlled phase 2 study in which ghrelin was administered to 21 patients with esophageal cancer, receiving cisplatin-based chemotherapy; the dosage was 3 μcg/kg twice daily for 1 week.  A placebo group  (n=20) received saline.  The ghrelin group consumed 18.2 kcal/kg/day compared with 12.7 kcal/kg/day.  A measure of appetite, an appetite visual analog score, was also higher in the ghrelin-treated patients, 6.2 vs 4.1.  Patients in the ghrelin group had fewer adverse effects of chemotherapy related to anorexia and nausea than patients in the control group.  One patient receiving ghrelin stopped therapy because of excessive diaphoresis.

Although this medication is being studied in adult chemotherapy patients, down the road ghrelin and potential analogs may have a role in pediatric patients with a variety of disorders which inhibit their appetite.

Additional references:

  • -Adachi S, Takiguchi S, Okada K, et al. Effects of ghrelin administration after total gastrectomy: a prospective, randomized, placebo- controlled phase II study. Gastroenterology. 2010;138:1312-1320.
  • -NEJM 2002; 346: 1623-30, 1662. Plasma ghrelin levels decrease after gastric resection.   Therefore, stomach may act as endocrine organ to maintain appropriate weight. However, ghrelin levels are reduced in obesity; so it is not clear that further reduction of these levels is clinically important.
  • -Gastroenterology 2003; 125: 1492. Review of ghrelin.
  • -Yamamoto K, Takiguchi S, Miyata H, et al. Randomized phase II study of clinical effects of ghrelin after esophagectomy with gastric tube reconstruction. Surgery. 2010;141:31-38.
  • -Nakazato M, Murakami N, Date Y, et al. A role for ghrelin in the central regulation of feeding. Nature. 2001;409:194-198.
  • -Kojima M, Hosoda H, Date Y, et al. Ghrelin is a growth-hormone- releasing acylated peptide from stomach. Nature. 1999; 402:656- 660.
  • http://www.surgjournal.com/article/S0039-6060(09)00784-3/abstract
  • http://pennmedicine.adam.com/content.aspx?productId=16&pid=16&gid=50495 Review of cited article.

The undiscovered country

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The title of this blog is derived from a Star Trek movie.  I think that when we see patients with eosinophilic esophagitis that we are often seeing something new and poorly characterized.

Despite so many unanswered questions, particularly on an individual basis, this topic has seen a lot of interest and there are many advances in both bedside and basic research.  The review article  (Allergy 2012; DOI: 10: 10.1111/j.1398-9995.2012.02787.x) focuses on many of the similarities and differences between pediatric and adult patients.  Is it the same disease? (Probably yes)

With regard to medical history, the article reminds clinicians to ask about coping strategies:

  • do you wash food down with liquid?
  • are you the last one to finish your food?
  • do you chew your food a long time?
  • do you avoid foods like meats or breads?

Clinical features –main difference is greater presentation variety in children.  Adults almost always have long-standing dysphagia.  In pediatrics, painful symptoms, reflux symptoms, and feeding refusal are often seen in early stages.  In both populations, other atopic diseases are very common.

Immunopathogenesis (same in pediatrics and adults):  Th2-type inflammatory response; not just eosinophils but also IL-5-expressing T-cells, B cells, and IgE-bearing mast cells.  A break-down of all the types of quantified cells from a large number of studies is detailed (Table 2).

Allergic profile –main difference is much higher aeroallergen sensitization in adolescent & adult patients than in pediatric patients.  In children, top four allergens: milk, wheat, egg, and soy.  In older patients/adults, nuts are frequent food allergens.

Treatment strategies –basic question of whether to treat for symptomatic relief or histologic response is still debated.  Three goals of treatment are the same:

  • improve quality of life
  • reduce the risk of severe esophageal injury
  • prevent esophageal damage

3 D’s of treatment drugs, diet, dilatation:

Drugs: topical steroids (fluticasone, budesonide) are effective in ~50% of children & these agents may reverse subepithelial fibrosis, PPIs -small percentage have EoE PPI-responsive disease, & systemic steroids.  Lower doses of budesonide may be effective as maintenance treatment (0.25mg BID).  Interestingly, infliximab has not been effective clinically or histologically despite the high amounts of TNF.  Azathioprine (or 6-MP) was effective in three steroid-dependent patients in a pilot study.

Diet –review does not cover new territory (see previous blog: Eosinophilic Esophagitis -Six Food Group Diet).  States that elemental diets are not practical in adults.  Discusses the fact that food allergy identification is difficult & remains a pressing research need.

Dilatation –can provide long-lasting symptom relief.  Dilatation is infrequently utilized in pediatrics and virtually never in absence of other therapies.

On a side note, in my training I was taught that there were 3D’s to treating every patient: diet, drugs, and demeanor — a good attitude goes a long way, particularly in an uncertain world.

Additional references:

  • -Gastroenterology 2011; 141: 1593.  anti-IL-5.  partially effective for EoE.
  • -JPGN 2010; 51: 723. n=91.  Incidental gastric eosinophils does not predict a worse response to fluticasone then isolated EoE.
  • -Clin Gastro & Hepatology 2011; 9: 400 (editorial 370). Budesonide at dose of 0.25mg BID was partially effective in adult cohort of n=28.
  • -Aceves SS et al. Allergy 2010; 65: 109-116. 3 month course of budesonide can lead to resolution of esophageal remodeling. Lamina propria fibrosis resolution correlates with response to topical steroids. Examined effect on lamina propria after 3 months of Rx.
  • -Gastroenterology 2010; 139: 1526. n=36. (summary pg 1429) 15 day course of budesonide (1mg BID). 13/18 in Rx group had improved dysphagia, 72% wiht histologic remission, 92% reduction in eosinophil count. Did not seem to matter if “allergic” or not. 3 pts developed mild candida.
  • -Gastroenterology 2010; 139: 418. Randomized placebo study showed effectiveness.n=15 Rx (n=9 placebo). 87% of Rx group responded.  2ml of water with 0.5gm pulmicort and mixed it with 4-5 packets of splenda.
  • -JPGN 2007; 45: 281/370/319. Review/research symposium/subepithelial fibrosis associated with EoE & dysphagia.
  • -JPGN 2007; 45: 22-31. Th2 Immunity w Eotaxin-3/ C-C chemokine receptor in EoE.
  • -Gastroenterology 2006; 131: 1381-1391. Randomized double-blind, placebo-controlled trial of fluticasone for EoE: 880mcg divided bid; n=21 Rx, n=15 placebo. 50% (vs 9% controls) achieved histologic remission; Rx more effective in those w/o detectable food allergies. 67% (vs. 27% controls) resolution of vomiting.
  • -Clin Gastro & Hep 2007; 5: xxiv. EoE causing Boerhaave’s syndrome (spontaneous rupture)
  • INCREASED FRAGILITY: -Gastrointest Endosc 2003; 57: 407-12. -Clin Gastro Hepatolo 2003; 1: 433-37.
  • -Clin Gastro & Hep 2006; 4: 1328. absolute eosinophilia (AEC 440 vs 140 controls), eosinophil-derived neurotoxin, and eotaxin-3 act as biomarkers of EE activity.
  • -Gastroenterology2006; 131: 2018 (-J Clin Invest 2006; 116: 536-547. ) Eostaxin-3/EcE transcript signature.
  • -J Pediatr 2005; 147: 540 Picture of ringed esophagitis.
  • -JPGN 2004; 39: S8 [abstract 0005]. CHOP experience in 250 pts. NG elemental diet was most effective. ~6% of pts presenting with GER. Strict avoidance of allergens needed.

One more cause of rectal bleeding

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Two articles in this month’s JPGN, along with the editorials, make it clear that when patients are having rectal bleeding, we should ask if they are spending an inordinate amount of time straining on the toilet.  (JPGN 2012: 54: 263-65, 266-70, 167-68, 169-70).  This question may help uncover Solitary Rectal Ulcer Syndrome (SRUS).

This syndrome which is often a misnomer because many cases have erythema rather than ulcers; and, lesions can be ulcerative or polypoid.  They can be single or multiple involving the distal rectum.  SRUS often has a delayed diagnosis, 1.7 years and 3.2 years respectively in the two studies.  The differential diagnosis includes polyps, infections, IBD, abuse, and rectal manipulation.  Histology features include muscularinization of lamina propia, mixed inflammatory infiltrate, thickened muscularis mucosa, and epithelial hyperplastic changes.

Treatment includes avoidance of straining/behavior modification, use of laxatives, and perhaps topical mesalamine.

Additional references:

  • -Gastroenterol Clin North Am 2008; 37: 645-68. Rectal disorders/SRUS -review.
  • -Gastrointest Endosc 2005; 5: 755-62,
  • -Eur J Gastroenterol Hepatol 2008; 2: 89-92. SRUS in children.
  • -Gut 2004; 53: 368-70.  Biofeedback for SRUS.
  • -Pediatrics 2002; 110: e79.
  • -Clinical Perspectives in Gastroenterology 1999; 2: 190.

Think twice about checking thyroid

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Hypothyroidism is not a frequent cause of constipation in children (JPGN 2012; 54: 285-87).  According to this study, the yield on checking for hypothyroidism in pediatric patients with constipation is no greater than in the general population.

This retrospective study examined the records of 873 patients who underwent thyroid testing ordered by pediatric gastroenterology; 443 (51%) had isolated constipation.  In all, 56 had abnormal thyroid function testing though 40 were normal on repeat testing. Of these 16 remaining cases, 7 were known to have hypothyroidism.  Of the nine remaining “new” cases, 3 had slow growth (2 without constipation), 3 had trisomy 21, and 2 were taking medications which affect thyroid function.  This leaves only 1 patient who presented with constipation without a clear risk factor or other indication for testing.

The authors state that 1/443 (0.2%) is quite similar to background incidence of hypothyroidism (0.3%).  The costs of identifying this case would exceed $18,000 but could be considered much higher (~$200,000) when all of the costs of false-positives are considered (eg. endocrinology consultations, followup testing).  In patients with slow growth (with or without constipation), about 2.5% had hypothyroidism.

In short, think twice about checking thyroid status in patients with isolated constipation.

Additional references:

  • -JPGN 2011; 53: S2. 49, 55. ‘Rarely do children with constipation need w/u’–only if alarming features: Delayed meconium, bilious vomiting, bloody stools, fever, bladder disorders, poor growth, NO RESPONSE to conventional treatment, abnormal exam. 25% of functional constipation problems may persist into adulthood.
  • -JPGN 2010; 51: 155. Lack of utility of AXR -suggested only in uncooperative pts, concerns about abuse/concerns about psychologic impact of exam
  • -J Pediatr 2010; 156: 461-5. MRI in children with chronic constipation or not-retentive soiling (n=130, n=28) -MRI showed lumbar sacral abnormalities in 3% –none required neurosurgery (1 w spina bifida occulta, 3 w terminal filum lipomas). Prospective study.
  • JPGN 2006; 43: e1Ye13.  http://www.naspghan.org/user-assets/Documents/pdf/PositionPapers/constipation.guideline.2006.pdf  Guidelines suggest checking thyroid, calcium, celiac and lead if not responding to therapy.

Unexplained chest pain

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Not surprisingly -unexplained pediatric chest pain has a high association with anxiety/psychiatric disorders (J Pediatr 2012; 160: 320).  In this study, the authors compared patients (8-17 years) with chest pain (n=100) to a cohort referred with innocent heart murmur (n=80).  In addition to cardiology evaluation, patients had a structured interview and a child health questionnaire to assess for psychiatric disorders; also, the investigators interviewed the parent(s).

Based on DSM-IV criteria, 70% of chest pain patients had an anxiety disorder and 9% had depression.  In contrast, 33% of heart murmur patients had an anxiety disorder and none were depressed.  Among the chest pain subjects, 26% had abdominal pain and 26% had headaches -both higher than the control group, 9% and 10% respectively.  Also, 90% of patients with chest pain had psychiatric disorders which preceded the chest pain.

For pediatric gastroenterologists, a take home message from this article is that chest pain is quite similar to abdominal pain (see references below); it might be interesting to discuss with cardiologists.

  • Do cardiologists experience the same reluctance from families to seek help from mental health?
  • How much testing is required before a functional diagnosis is accepted?
  • Do they follow patients with functional chest pain or send back promptly to primary care physician?

This article does not examine parental mental health issues.  This would be interesting.  In functional abdominal pain, maternal anxiety has been ascribed as the most consistent predictor of outcome (Acta Paediatr 2007; 96: 697-701).  Another factor that would be of interest would be level of activity; exercise helps reduce symptoms of irritable bowel/abdominal pain.

At the same time, the issue of reflux is not addressed by this article and not infrequently the issue of whether reflux is causing chest pain needs to be considered.  An article (Gut 2011; 60: 1473-78) regarding chest pain in adults indicates that patients with pH-probe (or endoscopic) proven GERD often respond partially (>50% reduction in symptoms) to PPI use.  This study reviewed RCTs involving chest pain and PPIs -six met inclusion criteria.  The RR of therapeutic gain for PPI usage was 4.3 for those with proven reflux and 0.4 for those with pH-probe (or endoscopic) negative chest pain.  Interestingly, in this study, heartburn was not predictive of whether chest pain was due to GERD on pH study.

Additional references:

  • -Pediatr Emerg Care 2010; 26: 830-6.  Psychopathology among children presenting to ER with unexplained chest pain.
  • -Clin Gastro 2008; 6: 329-32.  Depressive symptoms common in RAP -45%
  • -Pediatrics 2004; 113: 817.  Anxiety & depression commonly associated with RAP.  anxiety in ~79%, depression ~43%; anxiety often precedes RAP.
  • -JPGN 2011; 53: 200. n=98. 79% of FAP responded to low dose tricyclics
  • -Gastroenterology 2009; 137: 1261, 1207– Editorial.  Amitriptyline helped in 66% vs 58% with placebo. n=90. dose 10mg <35kg, 20mg >35kg. 89% had failed Rx prior to study. ‘Inability to use placebo.. in practice may justify amitriptyline’ Rx. Consider hypnotherapy/CBT first.
  • -Gut 2011; 60: 1473-78. PPI use in unexplained chest pain.
  • -Pain 2006; 122: 43-52. (Walker LS et al), J Pain 2006; 7: 319-26.  Distraction/ignoring important.
  • -J Pediatr 2009; 154: 313 (editorial), 322. Prospective school study. n=237. Weekly prevalence of abd pain was 38%. 18% with persistence for >12 weeks.
  • -Clin Gastro Hepatol 2008; 6: 329-32.  FAP persists into adulthood in 1/3 to 1/2 of cases.
  • -Gastroenterol 2006; 130: 1459-1465.  Functional esophageal d/o.
  • -Clin Gastro & Hep 2006; 4: 558. Review.
  • -Ann Heart J 2000; 40: 367-372.  Sertraline decreased chest pain independent of mood alteration/psychological scores.

Can I go?

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How safe is it for IBD patients to travel?  A retrospective study from adult patients with IBD (n=222) compared with controls (n=224) indicates that the risk of travel for IBD patients is only a little more than the general population (Clin Gastroenterol Hepatol 2012; 10: 160-65).

IBD patients with an average age of 37 years had infections during 15% of trips compared with 11% for controls.  92% of infections were due to enteric disease. However, this increased risk was identified in travel to industrialized countries not to developing countries.  This likely indicates that much of the increase is due to IBD flares rather than increased susceptibility to infections.  Nearly half of patients in this study were receiving immunosuppression: immunomodulators 29%, biologics 5%, dual therapy 6%, or corticosteroids 4%.  Not surprisingly, patients with increased IBD flares (OR 1.9) and IBD-related hospitalizations (OR 3.5) represent a group with higher risk for illness.  Most illnesses were mild & self-resolving in a few days.  Only five IBD patients required hospitalization for the following: dehydration, perianal abscess, malaria, flare, & small bowel obstruction).

Additional references:

  • -Clin Gastro & Hep 2010; 8: 490. Review of traveler’s diarrhea. Recs pepto if emesis, rifaximin or cipro or azithromycin if watery diarrhea, azithromycin if bloody diarrhea
  • -Clin Gastro & Hep 2007; 5: 451. Use of rifaximin (200mg tid)-loperamide provided rapid improvement.
  • -NEJM 2006; 354; 119. Traveler’s diarrhea.
  • -NEJM 2002; 347: 505.  Illness after travel.
  • -www.cdc.gov/travel/index.htm

It’s worth the cost

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Recently the cost of Sitzmarks® increased to $175 (for 12)–it’s worth the cost.  According to one study, the use of a transit study helps determine which patients will benefit from colonic manometry (JPGN 2012; 54: 258-62).  A retrospective review of 24 children showed that all five children with normal oral-anal transit (OTT) studies had normal colonic manometry.  In contrast, 9/19 (47%) with abnormal (slow OTT) had abnormal colonic manometry.

The authors define their approach to OTT which is helpful.

  • In patients with a fecal impaction, this was cleared prior to starting study
  • If patients had difficulty with capsule ingestion, markers were administered by embedding in part of a banana or mixed with applesauce
  • Stimulant laxatives withheld for 72hrs prior to study
  • AXR obtained on days 3 and 5
  • Slow OTT (abnormal) defined as >6 markers proximal to rectum on day 5

Of those with abnormal colonic manometry, two-thirds (6) were referred for surgical intervention; one patient with normal OTT had surgery.  Surgeries:  3 cecostomy, 4 subtotal colectomy.

Additional references:

  • -JPGN 2004; 38: 75. Colostomy in 10 children with intractable constipation.
  • -Arch Dis Child. 2004 Jan;89(1):13-6. Benninga MA, Voskuijl WP, Akkerhuis GW, Taminiau JA, Buller HA. Related Articles,  Colonic transit times and behaviour profiles in children with defecation disorders.
  • -J Pediatr Surg. 2004 Jan;39(1):73-7. Youssef NN, Pensabene L, Barksdale E Jr, Di Lorenzo C.  Is there a role for surgery beyond colonic aganglionosis and anorectal malformations in children with intractable constipation?
  • -Am J Gastroenterol. 2003 May;98(5):1052-7.  Pensabene L, Youssef NN, Griffiths JM, Di Lorenzo C. Related Articles, Colonic manometry in children with defecatory disorders. role in diagnosis and management.
  • -JPGN 2002 Jul;35(1):31-8. Gutierrez C, Marco A, Nogales A, Tebar R. Total and segmental colonic transit time and anorectal manometry in children with chronic idiopathic constipation.
  • -JPGN 2001 Nov;33(5):588-91.  Villarreal J, Sood M, Zangen T, Flores A, Michel R, Reddy N, Di Lorenzo C, Hyman PE.  Colonic diversion for intractable constipation in children: colonic manometry helps guide clinical decisions.
  • -http://www.sitzmarks.com/buyonline.aspx

More on ethanol locks

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In a post earlier this week (Four advances for intestinal failure), ethanol locks were discussed.  More on information this subject (Pediatrics 2012: 129: 318-29) comes in a meta-analysis.  These authors identified 4 retrospective studies in pediatric IF populations.  Ethanol locks reduced infections by 81% and line replacements by 72%.  The studies in this meta-analysis overlapped with the six studies cited in the previous post.  Adverse events were rare and included thrombotic events.

Additional reference:

  • -JPEN 2012; 36: 36S-42S

What is calretinin?

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Calretinin is a ‘calcium-binding protein that is normally expressed in cholinergic nerves.’ (NEJM 2012:366: 361-72)  The absence of calretinin-positive mucosal neurites helps establish a diagnosis of Hirschsprung’s disease, even when suction-biopsy specimens may have previously been considered inadequate.  It is not known why calretinin neurites are absent in Hirschsprung’s disease nor why abnormal acetylcholinesterase-positive neurites are present.

More information on the technical aspects of diagnosing Hirschsprung’s disease and illustrations of these stains is available in this case record.

Additional references:

  • -Mod Pathol 2009; 22: 1379-84. Calretinin in Hirschsprung’s.
  • -Pediatr Dev Pathol 2009; 12: 6-15. Calretinin & acetylcholinesterase in Hirschsprung’s.
  • -JPGN 2011; 53: 75.  Problems post-op.
  • -JPGN 2008; 46:13.  Nice review.
  • -Pediatrics 2002; 109: 914-918.  Review of surgical treatments.
  • http://www.ccdhc.org/diseases/Hirschsprung.html

Four advances for intestinal failure

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Several advances in the management of intestinal failure have the potential to improve the outlook for our intestinal failure (IF) (aka Short Bowel Syndrome) patients (JPEN 2012; 36: 36S-42S).

Although IF patients already have improving survival with rates of 80-95% over followup ranging from 1-5 years, many still do not survive, primarily due to bacterial infections or chronic liver disease.  Ongoing research has made some promising steps in the management of these pediatric patients.  This article focuses on four of these steps.

1. Citrulline monitoring

  • Major source of citrulline is enterocyte production.  Citrulline is an amino acid not encoded in human genetic code; it is present in some proteins as a product of posttranslational modification.
  • Watermelon is one of few dietary sources.
  • Useful biomarker for bowel length/absorption –independent of inflammatory markers
  • Levels >15-20 μmol/L indicate good likelihood of achieving enteral autonomy
  • Levels <12μmol/L indicate a very low likelihood of achieving enteral autonomy

2. Teduglutide therapy

  • Analog of glucagon-like peptide 2 (GLP-2) but harder to degrade (longer half-life)
  • Preliminary studies in adults indicate improvement in absorption and villous histology after subcutaneous administration for three weeks.  Improvements reverse when drug is discontinued.
  • Since GLP-2 is produced by colon & increased in IF (if colon present), unclear whether exogenous administration will be as beneficial in patients with residual colon

3. Lipid minimization &/or fish oil lipids

  • Cholestasis increases in patients receiving more than 1 g/kg/day of intralipids (soy based).
  • Fish oil (Omegaven) has shown benefit in lowering cholestasis in numerous case reports.  This may be due the high content of anti-inflammatory ω-3 fatty acids.
  • Another preparation SMOFLipid is a mixed formulation and may be safer than pure fish oil; randomized controlled studies of both of these lipid formulations are underway.
  • Fish oil has not been shown to improve histology
  • Parenteral nutrition associated liver disease (PNALD) may improve with lowering lipids & may not need omegaven

4. Ethanol locks

  • May be beneficial in treating and preventing central line infections.  In both situations, in small studies, ethanol locks lowered incidence of recurrent infections.
  • Six studies involving 75 patients (66 pediatric patients) lowered infection rates from approximately 10 per 1000 catheter days to 2 per 1000 catheter days.
  • Ethanol concentrations were mostly 70% in these studies, though 25% has been used.
  • Dwell times ranged from 2-14 hours.
  • Randomized studies are in progress.
  • Fewer infections should reduce the likelihood of death from sepsis and death due to loss of venous access.

Additional references:

  • -NEJM 2010; 362: 181.  Letter to editor describes use of fish oil in (n=125) Boston pediatric patients.
  • -JPGN 2009; 48: 209. n=12. SBS.9/12 improved with omegaven. 3 had transplant (L-ITx). No controls.
  • -NEJM 2009; 361: 998. Intestinal Rx.  Review claims ~90% 1yr survival. 47% 5yr, 61% 3yr (expecting to go higher)
  • -JPGN 2009; 48: 334. Isolated liver w SBS feasible IF 50cm small bowel remaining or 30cm w ICV, 50% enteral nutrition >4 weeks with good growth, no dysmotility.
  • -Pediatrics 2008; 121: e678. n=18. use of fish oil improved cholestasis compared to historical controls.
  • -Gastroenterology 2008; 135: 61, 303. Survival of ITx (vs. HAL).  In many conditions, better off from survival standpoint without Tx. Tx if failure of TPN (severe liver dz/thrombosis of >/= 2 central veins, multiple bouts of sepsis/frequent dehydration), high risk of death, severe short bowel (<10cm in infants and <20cm in adults), pseudoobstruction, unwillingness to accept long-term tpn. 93% of TPN patients who did not have TPN-complications had 93% survival rate.  Thus, TPN is first line Rx as survival and quality of life often better.
  • -Pediatrics 2006; 118: e197-e201.  Reversal of TPN-AC c IV omega-3 fatty acids (fish oil-derived) instead of intralipids
  • -Liver Transplantation 2006; 12: 1062, 1040. Liver transplant alone reasonable to consider in some SBS patients who tolerate >50% enteral therapy and are less than 2 years old.
  • -Gastroenterology 2006; 130. Supp 1. Summary of NIH workshop on intestinal failure. TX Indications: Liver disease, thrombosis of major veins, recurrent catheter-related sepsis, frequent severe dehydration/electrolyte imbalance.
  • -JPGN 2005; 41: 47A (pg507). Poor prognosis: <40cm, needing >40kcal/kg PN, increased bili (>150 μmol/L)
  • -J Pediatr 2005; 146: 542. Serum citrulline > 19 μ/L associated with bowel adaptation/weaning off HAL.
  • -J Pediatr 2004; 145: 157-163. Survival of SBS with as little as 15
  • -Arch Pediatr Adolesc Med 2006; 160: 104953.  Use of ethanol lock (70%, 08-1.4mL for 12-24hrs, then withdraw). n=51.  High success rate in salvaging line
  • -J Pediatr 2001; 139: 27-33. Review of 30 pts. 3 of 30 pts with bowel length 40cm or less able to wean PN.
  • -Gastroenterology 2001; 120: 806-815. Glucagon-like peptide 2 improves nutrient absorption marginally.