Cool picture:
Link (from NEJM twitter feed) to contrast study showing tracheoesophageal fistula: NEJM Image of TEF in newborn. Video available at website.
Author Archives: gutsandgrowth
NASPGHAN Postgraduate Course 2014 -Intestinal Inflammation
This blog entry has abbreviated/summarized the presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.
Link: PG Course Syllabus – FINAL (entire syllabus)
The speakers reviewed a lot of IBD material (both at the postgraduate course and at the meeting); much of it has been has been covered in previous blog posts:
- CCFA Conference Notes 2014 (part 1) | gutsandgrowth
- CCFA Conference Notes 2014 (part 2) | gutsandgrowth
- Coming Soon to a Pharmacy Near You (part 1 …
- Podcast on Vedolizumab Efficacy for Crohn’s Disease …
- Just the Beginning: Mutations in Very Early Onset …
Early Onset Inflammatory Bowel Disease –Scott Snapper (Boston Children’s Hospital) pg 170 in Syllabus
- If one has a 1st degree relative with Crohn’s disease: 26-fold increased risk for IBD compared with 8-fold increased risk if 1st degree relative has ulcerative colitis
- 30% of children have one or more family members with IBD
- Concordance rate much greater in monozygotic vs dizygotic twins: 10-15% in UC and 25-30% in Crohn’s with monozygotic
Infantile IBD (age <2 years)
- Often isolated colonic disease
- Severe course – refractory to multiple immunosuppressant medications, often requiring surgery, occasionally fatal
- > 40 % with one or more family members with IBD
- 25% with infantile IBD have this as their first manifestation of underlying immunodeficiency (pg 174): IPEX, CGD, NEMO, Wiscott-Aldrich, XIAP, common variable immunodeficiency
- NEOPICS: interNational Early Onset Pediatric IBD Cohort Study. Expanded to 80 Centers (250 scientists) on 5 continents with access to over 1000 VEO-IBD patients
- IL10 Receptor defect results in infantile onset IBD. Hematopoietic stem cell therapy can be curative. Increased risk of B-cell lymphomas.
- NCF2 variant (NADPH Oxidase Gene) found in 4% of (n=11/268)
- TTC7A mutations (identified by whole exome sequencing) cause apoptotic enterocolitis, intestinal atresias, and SCID (severe combined immunodeficiency) –may not benefit by stem cell transplantation
- Immune workup for VEO-IBD: immunoglobulins, DHR for CGD, lymphocyte subsets. If negative, further genetic testing (candidate gene testing &/or exome sequencing)
Surgery in Crohn’s Disease –Jason Frischer (Cincinnati Children’s)
- 28% of CD patients need surgery within 10 years of CD diagnosis; 5.7% within one year.
- Reviewed principles: conserve bowel, reserved for complications/does not cure Crohn’s disease, strictures can be treated without resection.
Perioperative care
- Preop-“no answer with regard to biologics,” steroids are detrimental (goal <20 mg of prednisone). Biologics may increase risk of infections (could be related to specific level) but this is unclear.
- Postop: thromboprophylaxis
Surgical problems (JPGN 2013; 57: 394 NASPGHAN Guidelines): Abscess, Fistula, Stricture
- Abscess: percutaneously drain abscess if >2 cm and can remove drain when having less than 10 mL/day. Surgery reserved if refractory to conservative treatment –?timing
- Strictures: steroids to minimize acute inflammation. Stricturolplasty rare in pediatrics –used only in those without fistulas. Most common stricturolplasty: Heineke-Mikulicz.
- In Crohn’s patients at Cincinnati children’s who have undergone ileostomy, long-term only 46% able to have intestinal continuity
Crohn’s and UC ‐ What to do when anti‐TNF isn’t working? –Athos Bousvaros (Boston Children’s) pg 190 in Syllabus
Off-label IBD drugs in children for medically-refractory disease.
Potential Rescue treatments
- Calcineurin inhibitors for UC (eg. tacrolimus, cyclosporine)
- Thalidomide for Crohn disease
- Natalizumab for Crohn disease –>not being used anymore. PML risk
- Vedolizumab for Crohn disease and UC
- Ustekinumab for Crohn disease
- Tofacitinib for UC
Before off-label drugs:
- Optimize TNF: Make sure the diagnosis is right (eg. exclude CGD), Minimize risk of loss of response (combination therapy, optimize dose, scheduled infusions)
- Consider surgery -strictures, ulcerative colitis, limited disease
Data for tacrolimus from Boston. n=46. (Watson et al, IBD Journal 2011). Used most frequently with severe UC.
Data for thalidomide –31 of 49 achieved remission. Lazzerini et al, JAMA. 2013;310(20):2164‐2173. Side effects -birth defects, neuropathy. STEPS program.
Data for vedolizumab. Feagan et al NEJM 2013; 369:699. Remission (in the responders) for ulcerative colitis at 52 weeks:
- 45% of patients getting vedolizumab monthly
- 42% of patients getting it every other month
- 16% of patients randomized to placebo
For Crohns’ disease , Vedolizumab also works in Crohn’s disease, but it takes time (Sands et al: Gastroenterology 2014 147:618‐627)
Off-label does not equate to experimental! pg 199:
FDA Statement: The FD&C Act does not, however, limit the manner in which a physician may use an approved drug. Once a product has been approved for marketing, a physician may prescribe it for uses or in treatment regimens or patient populations that are not included in approved labeling. Such “unapproved” or, more precisely, “unlabeled” uses may be appropriate and rational in certain circumstances, and may, in fact, reflect approaches to drug therapy that have been extensively reported in medical literature.
“Luminitis:” When Inflammation is Not IBD (Microscopic Colitides) –Robbyn Sockolow (Weill Cornell Medical School) pg 180 in Syllabus
Microscopic Colitis -pediatric prevalence unknown (JPGN 2013;57:557-561). Nonbloody diarrhea with normal-appearance grossly.
- Lymphocytic Colitis (>20 intraepithelial lymphocytes/100 colonocytes) -Normal crypt architecture
- Collagenous Colitis -Thick layer (up to 30 micrometers) of collagen in the tissue and increased lymphocytes in colon
Eosinophilic colitis
- At-risk groups? Infants & post-transplant patients (tacrolimus trigger?) (Saeed et al Pediatr Transplantation 2006: 10: 730–735)
- Associated with food allergy, IBD, autoimmune diseases
- Elevated serum IgE.
Microbial Signature in Crohn’s
This recent study (summarized in earlier post today/Dr. Barnard’s talk) provides more information on the microbiome in patients with pediatric Crohn’s. Here’s a link to full article: Specific transcriptome and microbiome signature in pediatric Crohn’s Here’s the abstract:
Interactions between the host and gut microbial community likely contribute to Crohn disease (CD) pathogenesis; however, direct evidence for these interactions at the onset of disease is lacking. Here, we characterized the global pattern of ileal gene expression and the ileal microbial community in 359 treatment-naive pediatric patients with CD, patients with ulcerative colitis (UC), and control individuals. We identified core gene expression profiles and microbial communities in the affected CD ilea that are preserved in the unaffected ilea of patients with colon-only CD but not present in those with UC or control individuals; therefore, this signature is specific to CD and independent of clinical inflammation. An abnormal increase of antimicrobial dual oxidase (DUOX2) expression was detected in association with an expansion of Proteobacteria in both UC and CD, while expression of lipoprotein APOA1 gene was downregulated and associated with CD-specific alterations in Firmicutes. The increased DUOX2 and decreased APOA1 gene expression signature favored oxidative stress and Th1 polarization and was maximally altered in patients with more severe mucosal injury. A regression model that included APOA1 gene expression and microbial abundance more accurately predicted month 6 steroid-free remission than a model using clinical factors alone. These CD-specific host and microbe profiles identify the ileum as the primary inductive site for all forms of CD and may direct prognostic and therapeutic approaches.
From Cincinnati Children’s Pediatric Insights (summary of findings):
“The discovery of specific bacterial populations and a core gene signature associated with Crohn’s disease could lead to new diagnostic testing and improved treatment for inflammatory bowel disease (IBD), according to a study led by researchers at Cincinnati Children’s.
‘This study identifies a set of bacteria that are associated with symptoms, and a group of anti-inflammatory genes that are associated with intestinal damage in children with Crohn’s disease,’ says Lee (Ted) Denson, MD, Medical Director of the Inflammatory Bowel Disease Center, senior investigator for the study, published online July 8 in the Journal of Clinical Investigation. Yael Haberman Ziv, MD, was the study’s first author.
Denson’s team studied tissue samples from the ileum, the lowermost portion of the small intestine, in a large number of children with Crohn’s disease. They found specific types of bacteria and a “core” gene expression signature, both of which appear to affect inflammatory changes in the gut. Certain genes in the core signature appeared to be specifically associated with intestinal damage from deep ulcers.”
Basic Science Year in Review –#NASPGHAN 2014
John Barnard –Basic Science Year in Review
“Emerging Trends and Provocative Findings in Basic Science”
This blog entry has abbreviated/summarized this terrific presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well. To minimize these issues, I have placed a link to most of Dr. Barnard’s slides which he shared:
“Big Data” –big increase in “big data” cited in pubmed over past year.
- Good read on this subject: Foreign Affairs: The Rise of Big Data Kenneth Cukier
Scientific fraud –more attention to this issue this past year. Two papers in Nature were retracted. One researcher committed suicide and one arrested. Scientific fraud undermines important messages & ruins credibility of other important advances.
CRISPR-Cas9: Gene editing. CRISPRs –“RNA guides” Cas9: “molecular scissors” (endonucleases)
“Genome editing has never been easier.” Examples:
- Cell Stem Cell 2013: 13: 653-58. “Functional repair of CFTR by CRISPR-Cas9”
- Also, genome editing has been used in mouse model of tyrosinemia.
Liver regeneration in zebrafish. Implication: Liver cells will be regenerated in humans. Gastroenterol 2014; 146: 789.
Microbiome Big Data:
- J Clin Invest 2014; 124: 3617. This was a very important and complex study. The slides explaining this study start at slide 35.
- Pediatric Crohn disease exhibit specific ileal transcriptome and microbiome signature.
- RISK study (CCFA). Treatment-naïve, 28 sites.
- 1281 ileal host genes in ileocolonic Crohn’s, 1055 in Colonic Crohn’s had ileal host genes =82% similar, 232 host genes in ulcerative colitis –18% similar to ileocolonic Crohn’s.
- Tissue microbiomes are where changes are noted; changes are not evident in luminal microbiome.
- Antibiotics worsen dysbiosis.
- Microbiome at diagnosis strongly correlates with Crohn’s disease.
Microbiome –affects the entire body:
- J Clin Invest 2014; 124: 3391. Incorporation of microbes with genetically-engineered E coli can prevent obesity in mice
Recommended Reading by Dr. Barnard: “Missing Microbes” How the overuse of antibiotics is fueling our modern plagues. Martin Blaser
NASPGHAN Postgraduate Course 2014 -Endoscopy Module
This blog entry has abbreviated/summarized the presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well. Link to full syllabus:
The Dreaded Wake-Up Call (Part A) –Maercedes Martinez (NY Presbyterian Hospital) (pg 55 syllabus)
Variceal Bleeding – “When RED is not attractive”
Discussed presentation of varices (gastric/esophageal), etiologies, association with portal hypertension. Reviewed variceal grading.
Medical management:
- PICU admit
- Avoid over-transfuse (goal ~ 8 g/dL)
- Correct coagulopathy
- Role of platelets is controversial/if trouble with endoscopy, may be helpful
- Suggested dosing for octreotide/somatostatin: 2 mcg/kg bolus then 1-2 mcg/kg/hr (typically max 100 mcg/hr), antibiotics
- Most patients do not require emergency overnight endoscopy.
- Sclerotherapy and banding reviewed -including complications.
- Transjugular intrahepatic portosystemic shunts (TIPS) and Surgical options briefly discussed
The Dreaded Wake-Up Call (Part B) –Lee Bass (Children’s Hospital of Chicago) (pg 67 in syllabus)
Nonvariceal GI Bleeding Management
- Start with ABCs -airway, breathing, cardiovascular –fluid resuscitation/blood products
- Restrictive transfusion strategy (Hgb <7 as threshold) (Villanueva et al NEJM 2013) helpful for survival in adults
- Treatment with PPI improves rates of high risk stigmata on endoscopy
- Prokinetics can improve identication of bleeding lesions
- Preparation for endoscopy is most important (slide on page 70 of syllabus)
- Also on page 70, pictures of typical findings with GI bleeding: nonbleeding vessel, adherent clot, spurting blood, oozing blood, and flat pigmented spot and clean base
- Endoscopic management -combination of two techniques appears to be more effective than single method. injection, thermal probe, hemoclips, hemospray (not available in U.S.
Endoscopic Interventions for Biliary Tract Disease — Victor Fox (pg 75 in Syllabus)
Choledocholithiasis is most common need for interventional biliary endoscopy and increasing related to increase risk with increase in obesity.(Buxbaum J. Gastrointest Clin N Am 2013;23:251‐75)
Requires advanced training to achieve high level of skill and experience
- >200 cases needed to achieve selective cannulation required for interventions
- Acquisition and maintenance of skills by pediatricians is controversial
Other points:
- No equipment is favorably designed for young or small children
- Success and complication rates are similar as in adults (Varadarajulu S, et al. Gastrointest Endosc 2004;60:367)
- Discussed biliary strictures (etiologies, management/stents), choledochocele, papillotomy, bile leak (Soukup ES et al. J Pediatr Surg 2014;49:345‐8)
-
“Most strictures and leaks can be successfully managed endoscopically without need for surgical intervention”
Take-home message: Endoscopic biliary interventions are increasingly employed in children with similar safety and technical success as adult patients
Related blog posts:
- All bleeding stops | gutsandgrowth
- Transfusion strategy in acute GI bleeding | gutsandgrowth
- Is a Continuous IV Proton Pump Inhibitor Really Necessary …
- Why an ERCP Study Matters to Pediatric Care | gutsandgrowth
- What and When for ERCP with Gallstone Pancreatitis …
- Indomethacin to prevent post-ERCP pancreatitis …
- What is the role for preventing variceal bleeding in Biliary …
- Expert advice on portal hypertension | gutsandgrowth
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.
NASPGHAN Postgraduate Course 2014 -Liver Module
This blog entry has abbreviated/summarized the presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well. I’ve attached the course syllabus as well:
Primary Sclerosing Cholangitis –Dennis Black (Le Bonheur Children’s Hospital)
- Up-to-date review provided
- GWAS (genome-wide association study) identified 16 significant risk loci which account for only 7.3% of overall risk; environmental influences need to be worked out
- Pediatric studies –total of 328 patients reported to date
Is pediatric disease the same disease as in adults?
- Incidence in pediatrics: 0.23/100,000 incidence vs 1.1/100,000 in adults
- Mean age at diagnosis 13 years in pediatrics.
- 30% of pediatric patients have overlap with autoimmune hepatitis which is higher than in adult patients.
Other pointers:
- Discussed “Autoimmune cholangitis.” Imaging needed in autoimmune hepatitis to look for primary sclerosing cholangitis.
- IBD Association with PSC: IBD occurs in about 55% of PSC patients. If PSC diagnosed first, usually with right-sided colitis. If IBD diagnosed first, than pancolitis is more commonly noted.
- Add IgG4 as part of workup to rule out IgG4 cholangiopathy (sensitive to immunosuppression).
Treatment:
- Supportive care for cholestasis (vitamins, pruritus management, etc
- Monitoring for complications (rare cases of cholangiocarcinoma in pediatric population). 14 drugs tested to date –mainly in adults. “All without proven positive impact on long-term outcome.”
- Ursodeoxycholic acid –widely used but controversial because higher doses associated with worsened outcomes in adult study (Lindor et al). Ongoing study in pediatric population with ursodeoxycholic acid.
- Vancomycin (Aliment Pharm 37: 2013; 604. Adults n=35). Both Flagyl and Vanc seemed to be helpful. Uncontrolled pediatric studies with vancomycin reviewed. Vancomycin study in the works for pediatric/adults.
- No prospective randomized controlled trials in children and very little data in adults. Hard endpoints –very difficult in children/not practical in children (eg. portal hypertension, transplant, death).
PSC and Transplantation: PSC 2.6% of total transplants –long-term outcome is similar.
Related Blog Posts:
- PSC 2013 Review | gutsandgrowth
- Staying current with PSC | gutsandgrowth
- Population–Based Outcomes for Primary Sclerosing … – gutsandgrowth
The Jaundiced Infant –Saul Karpen (Emory)
- “We don’t estimate jaundice very well… Our eyes do an awful job.”
- Breastmilk Jaundice: Archives of Disease in Childhood 1978; 53: 506-16. Only 12 of 853 had jaundice beyond 3 weeks of life.
- Cholestasis. One of the best studies looking at etiology was recently published: Hoerning A, et al Front Pediatr. 2014; 2: 65. N=82. Only 1 patient had CMV. 41% had biliary atresia.
Biliary atresia (BA):
- Reviewed study indicating that liver biopsy was most accurate means of making diagnosis of biliary atresia (blog comment: this study result may not be accurate in all settings as the interpretation relies on the ability/reliability of pathologist). High utility of stool pigment & ultrasound (including flow).
- In retrospective study (Pediatrics 2011; 128 e1428-33), all the BA patients had elevated direct bilirubin by 24-48 hrs of life.
- Genetic panels and whole exome sequencing (~$4-7K) are happening now. Cost-effective.
Take-home message: Molecular understanding possible for conjugated/unconjugated hyperbilirubinemias. Direct bilirubin >1 is abnormal
Related blog posts:
- “Genetic Testing and the Future of Pediatric Gastroenterology …
- Biliary Atresia More Common in Preterm Infants | gutsandgrowth
- Outcome of “Successful” Biliary Atresia Patients | gutsandgrowth
- Study: Steroids Not Effective For Biliary Atresia … – gutsandgrowth
Acute Liver Failure –Estella Alonso (Children’s Hospital of Chicago) (pg 43)
Points:
- Few patients receive a full diagnostic workup (J Pediatr 2009;155:801‐6)–especially with regard to metabolic and autoimmune disorders.
- Reviewed etiologies –most frequently “indeterminant” especially in younger patients. Acetaminophen is most frequent etiology in teenagers and adults.
- Systemic inflammation is common in acute liver failure (Bucuvalas, J JPGN 2013;56: 311–315). Soluble IL2 receptor alpha –significantly higher in patients that died. Immune regulation important aspect regarding survival. Should steroids be used in cases with high inflammation?
Prognosis: Squires et al. J Pediatr 2006;148:652-8, Lee et al. JPGN 2005;40:575-81, Baliga et al. Liver Transpl 2004;10:1364-71
- 33% ‐53% survival with native liver
- 61% survival including LT
- 70%‐80% after LT
- Multiorgan failure is most common etiology of death. Bleeding is “a rare cause of mortality.”
Management:
- Reviewed including coagulopathy/bleeding, cardiovascular collapse, hepatic encephalopathy/cerebral edema
- Pediatric N-acetylcysteine Trial Squires, et al Hepatology 2013;57:1542‐9 N=182. Patients with NAC seemed to do worse, but not statistically proven. This study has stopped the widespread use of NAC in acute liver failure.
- Discussed approach to neurological complications in ALF. Hussain et al, JPGN 2014;58:449‐56. Retrospective study (n=18). Early EEGs obtained. Hypertonic saline may be more effective than mannitol. Hypothermia may be helpful adjunct.
- Timing of Transplantation discussed (pg 54 in syllabus). Difficult to predict spontaneous survival.
Related blog posts:
- Acute Liver Failure –Is There a Role for Steroids? | gutsandgrowth
- N-acetylcysteine for Acute Liver Failure | gutsandgrowth
- OTCD –Another Reason for Acute Liver Failure | gutsandgrowth
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.
This Year’s Pumpkin
NASPGHAN Postgraduate Course 2014 -3rd Module
This blog entry has abbreviated/summarized the presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well. All of the speakers had terrific presentations. The course syllabus is attached:
The 3rd Module had a “potpourri” of GI problems.
Extraesophageal Manifestations of Gastroesophageal Reflux –Ben Gold, MD (GI Care For Kids, Atlanta) (pg 86)
“Is reflux really the scurge of the earth and the cause of every malady known to human-kind in the head, neck, and lungs…?“
Key points:
Airway protection: “Aerodigestive disease reflexes are intact by 38 weeks gestation.”
Central deglutition apnea: a normal protective mechanism to prevent aspiration during swallowing. (Hasenstab KA, Jadcherla, S. J Pediatr 2014; 165:250-255). No proof at present that central apnea is caused by reflux though there is a biologic plausibility.
“Although reflux causes physiologic apnea, it causes pathologic apneic episodes in only a very small number of newborns and infants.” “When reflux causes pathological apnea, the infant is more likely to be awake and the apnea is more likely to be obstructive in nature.”
Laryngeal Reflux:
- Chronic cough, chronic laryngitis, hoarseness, and asthma may be associated with GERD BUT the data showing a relation between reflux and upper airway disease are weak
- Airway symptoms attributed to reflux in adults include hoarseness, chronic cough, and globus sensation
- Affected adults rarely have typical reflux symptoms
- The sensitivity of laryngoscopic findings to identify reflux disease are poor. Sherman et al. Am J Gastroenterol. 2009;104:1278-95. Vandenplas et al. J Pediatr Gastroenter Nutr. 2009;49:498-547.
Asthma:
“Chronic cough, chronic laryngitis, hoarseness and asthma are multifactorial disease processes and acid reflux can be an aggravating cofactor.” GER is an unlikely contributor to asthma if reflux testing is negative.
“Two NIH-funded blinded, randomized placebo-controlled trials (RCT), one in adults (using esomeprazole), one in children (using lansoprazole) showed NO difference in asthma outcomes comparing placebo and acid suppression therapy”
Multi-Channel Intraluminal Impedance/pH probe studies: Pediatric studies are critically needed to determine if knowing the amount of nonacid reflux changes treatment or outcome
Proton Pump Inhibitors can cause gastric bacterial overgrowth (Rosen R et al JAMA Pediatr 2014; JAMA Pediatr. doi:10.1001/jamapediatrics.2014.696)
Ben Gold (speaker) and Jay Hochman prior to 5K Run
Related blog posts:
- Accuracy of ENT diagnosis of Reflux Changes | gutsandgrowth
- Treating reflux does not help asthma | gutsandgrowth
- The Medical Pendulum and Gastroesophageal Reflux | gutsandgrowth
EoE: PPI, PPI-REE, TCS, OVB, SFED, 4FED….…Alphabet Distress — Sandeep K Gupta, MD (Indiana University) pg 105 in Syllabus
Treatment endpoints discussed -histologic, symptomatic, fibrosis, etc.
- Proton pump inhibitor responsive eosinophilic esophagitis (PPI-REE) may work by blocking STAT6 binding to Eotaxin-3 promoter rather than by acid suppression (PLos ONE 2012;7:e50037). PPIs work (eos <6/hpf) in in 30-40%. May need high dose to work long-term (Dr Molina-Infante – DDW 2014)
- Topical corticosteroids (TCS) -higher dose = better response. (Budesonide. Gupta SK, Vitanza J, Collins, MH Clin Gastro Hepatol 2014 [ePub], Fluticasone. Butz BK. Gastroenterology 2014). Clinical symptoms do not correlate with histologic response. Discussed long term safety concerns.
- Reviewed diets -elemental, targeted, 4-food elimination and 6-food elimination.
Related blog posts:
- Higher Doses of Topical Steroids for Eosinophilic … – gutsandgrowth
- What is the Role for Allergy Testing in Eosinophilic Esophagitis …
- The Latest on EoE and PPI-REE | gutsandgrowth
- EoE: Drugs, Diets, Dilatation and PPI-REE | gutsandgrowth
“Gotta keep on movin”: New tricks and treatments for motility disorders –Carlo DiLorenzo (Nationwide Children’s Hospital) pg 116 in Syllabus
Key points:
- Most important motility study is a normal study. If normal study, then there is more concern for sensory dysfunction. Look for significant findings on motility studies not minor changes.
- Key to confirm if motility disorder is present. Hx/o small intestinal transplant in medical child abuse/Munchausen syndrome by proxy (Trans Proc 1996; 28: 2790)
- New tool: wireless motility capsule (J Pediatr. 2013;162:1181-7)
- Easier to obtain full thickness biopsies (Gastrointest Endosc 2011;73:949-54)
Treatments reviewed -“try everything”
- prucalopride (JPGN 2014; 57: 197-203
- cisapride -still available
- lubiprostone (JPGN 2014;58:283–291)
- linaclotide boxed warning not for <17 years of age –though has been used by motility specialists
- cyproheptadine (J Pediatr 2013; 163: 261-7) –use in dyspepsia
- fludrocortisone -used in orthostatic intolerance
- augmentin -for small bowel motility (JPGN 2012;54: 780–784)
- octreotide -for bowel motility
- pyridostigmine (Colorectal Disease 2010 12, 540–548)
- iberogast
- botulinum toxin (Gastrointest Endosc. 2012 ;75:302-9)
- treat bacterial overgrowth
- surgery: Jube, GJ tube, ileostomy. “Every child with pseudoobstruction on TPN needs a gastrostomy and an ileostomy –(me, now)”
- gastric electrical stimulation
- emerging treatment: Elobixibat (for constipation) Expert Opin. Investig. Drugs
Related blog posts:
What’s New in the Diagnosis and Management of Constipation –Manu Sood (Children’s Hospital of Wisconsin) -page 130 in Syllabus
Reviewed recent guidelines from NASPGHAN
“Miralax is considered a 1st line agent”
Outcomes in children with constipation:
- Almost 50% of patients experienced at least one relapse in first 5 yrs.
- Almost 20% of children were symptomatic at 10 yrs. follow up (Bongers ME, et al. Pediatrics. 2010)
Pointers:
- Slow transit is common
- Rectal compliance does not predict success with treatment. Van den Berg MM, et al. Gastroenterology 2009. Patients with mega-rectum may have motility disturbance as well.
- Success rates for antegrade continence enemas (ACE) 65% to 89%. Colon manometry can help predict ACE success. Up to 40% may be able to stop ACE w/in 2 years
Related blog posts:
- Updated Pediatric Expert Constipation Guidelines | gutsandgrowth
- Miralax Safety | gutsandgrowth
- AGA Constipation Guidelines | gutsandgrowth
- ACE report -10 year effectiveness | gutsandgrowth
- Stimulants for constipation | gutsandgrowth
N4K –Nutrition4Kids
I wanted to let followers of this blog know of a website which was developed under the guidance of one of my partners, Stan Cohen. The website Nutrition4kids.com has a wealth of information and resources on nutrition topics. In addition to Dr. Cohen, the group of medical advisors includes Mel Heyman, Bailey Koch, Kylia Crane, David Cohen, W Allan Walker, and Kathleen Zelman. The official rollout for the website was this past week.
About N4K (from website): N4K is a personal, trustworthy, and expert guide to learning about and making the right food choices for your individual child regardless of age, cultural norms, medical conditions or food sources. Parents trying to feed their kids well are faced with inconsistent and misleading labelling of food products. “All Natural,” “Reduced Fat,” “Reduced Sugar,” “Made with Real Fruit,” “Green” and other terms are all but useless when making good food choices.
“Decision Fatigue” & 1000 Posts
This post is number 1000! I don’t think this fact is related to “decision fatigue.”
From NY Times –here’s the link: Decision Fatigue for Physicians
Here’s an excerpt:
The phenomenon of “decision fatigue” has been found in judges, who are more likely to deny bail at the end of the day than at the beginning. Now researchers have found…As the day wears on, doctors become increasingly more likely to prescribe antibiotics even when they are not indicated.
For the study, published in JAMA Internal Medicine, scientists analyzed diagnoses of acute respiratory infections in 21,867 cases over 18 months in primary care practices in and near Boston.
In two-thirds of the cases, antibiotics were prescribed even though they were not indicated… Over all, compared to the first hour, the probability of a prescription for antibiotics increased by 1 percent in the second hour, 14 percent in the third hour and 26 percent in the fourth.
For pediatric gastroenterologists, what do you think happens more often (or less often) due to decision fatigue? excessive/less testing? excessive/less use of medications (like proton pump inhibitors)?
For Halloween yesterday –some people had some great outfits.
That’s Dinesh under that mask
Malpractice Reform, Defensive Medicine, and Real World Costs
A recent study (NEJM 2014; 371: 1518-25) showed that tort reform has little effect on medical costs, imaging rates, or admission by emergency room physicians.
One frequently heard argument in controlling medical costs is that fear of malpractice lawsuits drive physicians to order unnecessary care (“defensive medicine”); as such, if tort reform is broadly enacted this would presumably be an “easy” way to lower medical costs. To examine this issue more closely, the authors examined three states which changed the malpractice standard for emergency care from “ordinary negligence” to “gross negligence.”
- Texas (in 2003)
- Georgia (in 2005)
- South Carolina (in 2005)
Using a 5% random sample of Medicare fee-for-service beneficiaries, the authors examined all ER visits to hospitals in the three reform states along with neighboring (control) states from 1997-2011 (total of 9 states examined).
Key finding: “We found no reduction in the rates of CT or MRI utilization or hospital admission in any of the three reform states and no reduction in charges in Texas or South Carolina. In Georgia, reform was associated with a 3.6% reduction (95% confidence interval, 0.9 to 6.2%).”
While these reforms caused little changes in practice intensity and charges, the authors note that the Texas reforms, which affected other specialties as well, were associated with a 60% overall reduction in malpractice claims and 70% reduction in malpractice payments.
While the goal of tort reform may have been driven by costs, there could be other potential effects:
- Physician job satisfaction
- Improved physician regional supply
- Reduction in claims
- Less compensation for injured patients
Overall, the “effect of malpractice reform on the quality of care has been mixed.”
Take-away points (from authors)
- “Physicians are less motivated by legal risk than they believe themselves to be”
- “When legal risk decreases, the ‘path of least resistance’ may still favor resource-intensive care.”
- “Malpractice reform may have less effect on costs than has been projected.”
Related blog post: 200 years of Health Law | gutsandgrowth
gutsandgrowth 