Conflicting Cholesterol Guidelines –Massive Undertreatment or Massive Overtreatment?

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A fascinating article (Gooding HC et al. JAMA Pediatr doi:10.1001/jamapediatrics.2015.0168) studies a cross-sectional analysis of the National Health and Nutrition Examination Survery (NHANES) population and determines the frequency of the need for statin therapy for hyperlipidemia based on two separate guidelines.

  • 2011 Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents of the National Heart, Lung, and Blood Institute (Pediatrics 2011; 128 (sup 5): S213-S256) PEDS RECS
  • 2013 Guidelines on the Treatment of Blood Cholesterol to Reduce Atherosclerotic Cardiovascular Risk in Adults from the American College of Cardiology and American Heart Association (Circulation 2014: 129 (25) (supl 2) S1-S45) ADULT GUIDELINES

Specifically, the design of the study focused on 17-21 year olds in which the guidelines had overlapping recommendations. While the NHANES population involved only 6338 patients, this representative sample was used to calculate the likelihood of statin therapy more broadly among the US population of 20.4 million in this age group.

Key Findings:

  • Among the cohort of 6338, 2.5% would qualify for statin treatment using PEDS RECS compared with 0.4% under ADULT GUIDELINES.
  • This finding extrapolates to 483,500 patients nationwide compared with 78,200, respectively.  This is a difference of more than 400,000 and reflects a 6-fold difference.

Why the discrepancy?

  • ADULT GUIDELINES recommend use of statins only if LDL-C is >190. PEDS RECS extend to as low as 130 or 160 if additional risk factors (highly prevalent) are present, including hypertension, obesity, and smoking.
  • ADULT GUIDELINES are based on randomized clinical trials, though “they advocate for physician’s judgement in areas where the evidence base is insufficient.” PEDS RECS use extrapolated evidence for lifetime risk of coronary vascular disease.

Bottomline: While these guidelines highlight differences among 17-21 year olds, the decision regarding statin therapy extends across the age spectrum in terms of whether a low or high threshold should be in place.  Also, it is unfortunate that the additional modifiable risk factors (smoking, hypertension, and obesity) are so prevalent as to create this divergence in approach.

Related references:

  • NEJM 2015; 372: 1489-99. Alirocumab, a monoclonal antibody that inhibits PCSK9, lowered LDL 62% in patients receiving maximal statin therapy. Randomized, placebo-controlled study with 2341 patients.
  • NEJM 205; 372: 1500-09. Evolocumab, a monoclonal antibody that inhibits PCSK9, lowered LDL 61% in two open-label randomized trials (n=4465).

Related blog posts:

Sandy Springs

Sandy Springs

Inequality in Pediatric Health Care

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“Of all the forms of inequality, injustice in health care is the most shocking and inhuman.”

-Martin Luther King, Jr

This quote is part of an editorial (Flores G, “Dead Wrong: The Growing List of Racial/Ethnic Disparities in Childhood Mortality” J Pediatr 2015; 166: 790-3). The author discusses the disparities among African-American (AA) and Latino children in comparison to white children.

Key points:

  • AA children and young adults had ~6 times the death rate for drowning in swimming pools, 4 times more likely of dying after liver transplant, and about twice the likelihood of dying due to acute lymphoblastic leukemia.
  • Latino children have higher cancer death rates with about twice the likelihood of dying due to acute lymphoblastic leukemia and increased drowning death rate as well.
  • One new study (pages 812-8) shows that black children have increased in-hospital mortality (OR 1.66) after complications following congenital heart surgery and that hispanic children have an increased complication rate following surgery (OR 1.13). This was a retrospective study using the Kids’ Inpatient Database with approximately 3 million discharge abstracts for three separate years.
  • A second study (pages 819-26) with a data set of 98,833 children shows that birth defects resulted in higher 8-year adjusted hazards of death for black, latino, and Asian/Pacific Islander children.

Recognizing these disparities inevitable leads to the question of why. Dr. Flores postulates several factors.

  • Genetic differences.  For example, some ethnicities have more difficult to treat cancers, either due to genetic mutations or due to metabolism of medications.
  • Delays in diagnosis and treatment.  Patients who present at a later stage of diagnosis often have lower cure/response rates. The author notes that black children receive a diagnosis of autism a mean of 1.4 years later than white children.
  • Barriers to specialty care.  Specialty care can result in improved outcomes.
  • Bias in healthcare delivery, both conscious and unintentional.

Bottomline: The problems of racial inequality is not just a matter of relationships between the police and the community.  It is clear that more needs to be done to improve outcomes in healthcare as well.

Related blog posts:

Unrelated Link: Surgeon General Tells Elmo to Get His Vaccines

What is the Connection between Psychological Stress and Ulcers?

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Briefly noted: “Psychological Stress Increases Risk for Peptic Ulcer, Regardless of Helicobacter pylori Infection, or Use of Nonsteroidal Anti-Inflammatory Drugs” Clin Gastroeenterol Hepatol 2015; 498-506. From an initial sample of 3379 Danish adults with medical data/blood  in 1982-83, 76 subjects developed ulcers in this prospective study. Life stress at baseline increased the risk of subsequent ulcer and was not fully explained by confounding variables, by socioeconomic status or by association with NSAIDs or smoking. Ulcer incidence wa 3.5% in the highest tertile of stress compared with 1.6% in the lowest tertile.

Bottomline: Since the discovery of Helicobacter pylori, the role, if any, of psychological stress in contributing to ulcers has been questioned. This study indicates that stress may play a role in ulcer development or at the very least is a marker of individuals at increased risk.

Hyperbilirubinemia and Central Apnea

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Briefly noted: “Unbound Unconjugated Hyperbilirubinemia is Associated with Central Apnea in Premature Infants” J Pediatr 2015; 166: 571-5.  This was a prospective observational study with 100 27-33 gestational infants.  The group with central apnea had higher unconjugated hyperbilirubinemia (UB).  The authors speculate that UB could cause neurotoxicity via central chemoreceptors and more aggressive treatment of UB with phototherapy could be needed.

Wheat Intolerance Syndrome?

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Even though we’ve lived in our house for many years, some of our neighbors refer to our house as the ‘Walden’ house; the Waldens lived here for a long time before we did. Probably when we move, our neighbors will call our present home the “Hochman” house, regardless of who resides there.

I think nomenclature in medicine has a similar reluctance to adopt new terms.  A recent medical progress report (Guandalini S, Polanco I. J Pediatr 2015; 166: 805-10) suggests dropping the term “Nonceliac gluten sensitivity” (NCGS) in favor of “Wheat Intolerance Syndrome.”

It’s probably a good idea and their arguments are sound. Two key points:

  • “There is no proof that gluten is causing NCGS.”
  • It is likely that the majority of patients considered NCGS have not even eliminated celiac disease before instituting a gluten-free diet.

With regard to the first point, the authors note that recent studies have suggested that a “FODMAP” (fermentable oligosaccharides, disaccharides, monosaccharides, and polyols) diet is likely the culprit in many cases of so-called NCGS.  They review a pivotal double-blind study (see related blog post: An Unexpected Twist for “Gluten Sensitivity” | gutsandgrowth) there was no role for gluten “at least in these patients with IBS-like NCGS.”  In addition, other studies have demonstrated a strong role for a placebo/nocebo effect of dietary elimination.  “It is quite conceivable that a portion of patients with NCGS, and arguably a substantial one, fall in this category.”

With regard to the second point, it is not a good idea to initiate a gluten-free diet before excluding the diagnosis of celiac disease (hence the prior term: “nonceliac” gluten sensitivity).  A related comment from the authors is that a “Grade 1 [Marsh] intestinal lesion has traditionally been considered of a very low specificity for celiac disease.”  More testing in this circumstance can help determine if celiac disease is the reason, including checking the levels of ϒδ T-cell receptors in intraepithelial lymphocytes (very specific for celiac disease) and/or detection of IgA anti-tissue transglutaminase antibody deposits in intestinal mucosa.

Other pointers:

  • Genetic testing for HLA-DQ2 and/or HLA-DQ8 genotypes (which are nearly 100% in celiac disease) are present in about 40% of NCGS which does not differ from the general population
  • “Estimating the prevalence of NCGS is impossible.”  Estimates have ranged from 0.6% of the U.S. population to as high as 50% according to some websites.

Bottomline: While “Wheat Intolerance Syndrome” works fine for me, I think the term nonceliac gluten sensitivity is going to be around for a while.  Hopefully, more families and care providers will exclude celiac disease before contemplating this label and consider other foods as potential contributors to the symptomatology.

Related Reference: “Coeliac Disease and Noncoeliac Gluten Sensitivity” Meijer CR, Shamir R, Mearin ML. JPGN 2015; 60: 429-32.  This reference covers much of the same territory.  The Table 1 in this article nicely summarizes the relevant literature/studies from 2008-2014.

Related blog posts:

 

Foreign Bodies in Children -Expert Guidance

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The NASPGHAN Endoscopy Committee has published a very helpful “Management of Ingested Foreign Bodies” Report (Kramer RE et al. JPGN 2015; 60: 562-74).  At the current time, one way to access a PDF of the report is the following CME link on the NASPGHAN website (no login required): Management of Ingested Foreign Bodies -Clinical Report

Some key definitiions from the report.

  • Emergent removal <2 hours from presentation regardless of NPO status
  • Urgent removal <24 hours from presentation following usual NPO guidelines

As a general rule, all symptomatic ingestions in the esophagus require emergent removal if feasible.  Most gastric ingestions do not require emergent removal, exceptions include multiple magnets, sharp objects in stomach (possibly with surgery), and possibly absorptive objects (due to obstruction).

1. Button Batteries:

  • Even with “spent” batteries, there is enough residual charge to cause injury and all ingestions (even if asymptomatic) into the esophagus require emergent removal. If these batteries are in the stomach & asymptomatic, urgent removal is recommended if age < 5 years and BB ≥20 mm.

What is different in the proposed algorithm (Figure 1) compared with the Poison Center Guidelines (see:Button Battery Algorithm Link | gutsandgrowth) is more detail regarding concerns about aortoenteric fistula & what to do after endoscopy. Key points:

  • If active bleeding or unstable, endoscopic removal in OR with surgery/cardiovascular surgery is recommended.
  • If any esophageal injury, recommendations include admission, NPO, and IV antibiotics.  Chest imaging (CT angiography &/or MRI of chest) can help decide length of stay.  In those with injury close to aorta, continuation of NPO/antibiotics are recommended along with followup imaging every 5-7 days.  In those with clinical deterioration (eg. hematemesis w/in 21 days of injury), ‘assume aortoenteric fistula and emergently prepare for cardiovascular surgery.’

2. Magnets.  Figure 3 provides algorithm for single and multiple magnets (adapted from Hussain SZ et al. JPGN 2012; 55: 239-42).

  • For single magnets, emergent removal from esophagus is recommended (like all other foreign bodies) if difficulty managing secretions, otherwise urgent esophageal removal is suggested.
  • If there are multiple magnets within reach of endoscope, then if symptomatic, emergent removal is recommended, otherwise, urgent removal is suggested.
  • For asymptomatic magnets beyond the reach of an upper endoscopy, potential for colonoscopy, or enteroscopy for removal &/or serial x-rays to follow progression. If there is no progression on X-rays (every 8-12 hrs) &/or development of symptoms, then surgical removal/endoscopic removal is recommended.

3. Sharp objects. Figure 4 provides algorithm.

  • Emergent removal from esophagus/stomach is recommended (like all other foreign bodies) if difficulty managing secretions, otherwise urgent esophageal removal is suggested. For radiolucent objects, if the ingestion was witnessed, urgent removal is suggested; if not witnessed, then further imaging (CT, esophagram, MRI) could be considered.
  • With regard to sharp foreign bodies beyond the reach of an endoscope, “follow clinically with serial x-ray.  Enteroscopy or surgical removal considered if symptoms develop or >3 days without passage.”
  • Despite the low risk of severe morbidity/mortality from sharp objects (beyond esophagus), report recommends urgent “removal of all of the sharp objects within the reach of the endoscope..if possible.”

4. Food impaction in esophagus.  If symptomatic, emergent removal; if asymptomatic, then urgent removal.  Biopsies of the esophagus are recommended with endoscopy (Figure 5).

5. Coin ingestions/Blunt objects. Figure 6. “>250,000 ingestions and 20 deaths reported in the United States during a 10-year period.”

  • For esophagus: If symptomatic, emergent removal; if asymptomatic, then urgent removal.  Report recommends check X-ray immediately before sedation. While the report does not address this, a possible alternative to x-ray would be the use of a metal detector.  “Consider glucagon if distal esophageal coin or if endoscopy not readily available.”
  • For stomach: No endoscopy needed. Repeat X-ray at 2 weeks.  Remove if not passed w/in 2-4 weeks. Report recommends check X-ray immediately before sedation. While the report does not address this, a possible alternative to x-ray would be the use of a metal detector.
  • For small bowel: removal (enteroscopy/surgery) if symptomatic.
  • For objects >25 mm width or >6 cm in length –> should be removed from stomach urgently.

6. Superabsorbent objects.  The authors describe ingestions from materials from toys and diapers with polymers that can retain ‘up to 100 times their weight in water.”

  • For esophagus: If symptomatic, emergent removal; if asymptomatic, then urgent removal.
  • Stomach/small intestine: urgent removal is recommended

The authors state these recommendations are based on consensus rather than strong evidence and are “no substitute for clinical judgement.”

Take-home message: These guidelines are a good starting point to improve the management of children with foreign bodies.

Related blog posts:

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.

University of Chicago

University of Chicago

N2U Part 5 -Biliary Atresia and Kwashiokor

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2015 N2U Syllabus & Presentations

Growth Failure, Macro- and Micronutrients, and Biliary Atresia  James Heubi   (Syllabus pg 62 –68)

Case in Point:  AK is a 5-month-old Hispanic male with biliary atresia s/p hepatoportoenterostomy at age 6 weeks who was seen in clinic with a 2 month history of poor nutritional intake (full details on syllabus pg 62).

Initial Focus/Management:

  • Increasing caloric density 24 cal with MCT-containing formula, then up to 30 cal (avoid monotherapy with portagen due to EFA deficiency); if not effective, then nocturnal tube feedings (NG/NJ) are likely to be needed. Parenteral supplementation –not being used at Cincinnati in pretransplant patients.
  • Ascites, when present, limits fluid volume intakes. Aldactone (often at about 3 mg/kg/day) can be helpful; check to make sure urine sodium indicates some natriuresis.
  • Fat soluble vitamin supplementation/micronutrient supplementation (when needed). Check levels at about 3 months of age. If Vitamin D (25-OH) is greater than 20 (in pediatrics), this is probably reasonable in this population. With Alagille/hyperlipidemic patients, need to correct vitamin E for cholesterol (or total lipid) (Sokol RJ, Heubi JE, et al. NEJM 1984; 310: 1209-12).
  • For biliary atresia, direct bilirubin >2 indicates need for fat soluble monitoring; in other cholestatic conditions (eg. Alagille, PFIC), don’t rely on direct bilirubin as fat soluble deficiency can develop with lower direct bilirubins.
  • Vitamin D supplementation: 1000 units/kg/day Drisdol D3 (expensive). D3 preferred but D2 usually OK. Monitor levels and increase dosing if needed. Check monthly until adequate level. Alternatives: Bio-D-Mulsion Forte (D3) http://www.bioticsresearch.com/node/1570, Nature’s Blend Ultra Strength (D3). http://www.nationalvitamin.com
  • Vitamin E supplementation: Liqui-E (w TPGS) or Nutr-E-sol 15-25 IU/kg/day. Alternative: Aqua-E
  • Vitamin A supplementation/monitoring: AquaADEKs is reasonable supplement. Harder to monitor vitamin A levels.
  • If failed Kasai, likely headed to transplantation fairly quickly.

Kwashiokor –Rob Shulman (Syllabus pgs 21-33)

Case in point: 15 mo –Fed a diet of coconut and rice milks managed by pediatrician and chiropractor.  (This can occur with BRAT diets as well.)

Key points:

  • Terminology: from language spoken in a region of Ghana. Term developed to describe the sickness a baby gets when the new baby comes. This is a result of child who gets displaced from breastfeeding as the result of a sibling being born. Willaims CD. Lancet 1935; 226: 1151-52. Original description
  • Etiology: protein deficiency, protein quality, infection (‘pushes them over the edge’)
  • Microbiome in Kwashiokor References: Tilg et al. Nature Rev. 2013;10:261-262.  Smith et al. Science. 2013;339:548-554. Initial study showed discrepant microbiome in identical twins with and without Kwashiokor. Followup study by placing stool (from Kwashiokor and from healthy children) in mice. Stool from twin with Kwashiokor resulted in mice malnutrition (Garrett W. NEJM 2013; 368: 1746-47). (Related blogs:Gut microbiomes of Malawian twin pairs discordant for … and Microbiome and the risk of Kwashiokor | gutsandgrowth )

Feeding plan/ Prevention of Refeeding Syndrome:

  • Check labs –including protime, zinc, phosphorus, potassium, magnesium
  • Oral feedings with standard formulas/diet (usually). Limit feeding volumes initially for 1st week Kwashiokor (page 37 in syllabus) –about ½ full caloric intake (consider ½ strength formula).
  • ‘Advancing diet slowly is not needed with other forms of malnutrition.’
  • Give multivitamin. In 3rd world, it is recommended to add additional vitamin A (200,000 units once).
  • Hold off on iron (even in multivitamin) until improved for a few weeks.
  • Albumin infusions are not recommended àassociated with worse outcomes
  • Refeeding syndrome is an iatrogenic disease! This is associated with Kwashiokor and not with other malnutrition diseases.
  • Add Kphos to feedings (eg Neutraphos, NeutraphosK). Usually drop in phosphorus drop most likely in first 48 hours –monitor carefully in first few days and again during increments in feeding.
  • In 3rd World countries, addition of antibiotics (amoxicillin or cefdinir for 7 d) to therapeutic regimens for uncomplicated severe acute malnutrition associated with a significant improvement in recovery and mortality rates. In U.S. this translates to low threshold for using antibiotics but not required in every case.

Disclaimer: This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

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.

Subway Art (Chicago)

Subway Mosaic Art (Chicago)

N2U -Part 4: Obesity and Micronutrients

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2015 N2U Syllabus & Presentations

Critically-ill Obese Patient (Syllabus 51-61) Ann Scheimann

Case: 14 year old with obesity –admitted to PICU with respiratory distress, BM1 51, recent hx/o 30 lb weight loss and declining school performance.

  • Nutritional assessment (syllabus pg 55) References: Port et al. Curr Opin Clin Nutr Metab Care. 2010;13:184-191.McClave et al. JPEN J Parenter Enteral Nutr . 2011: 35: 88s.
  • Components of nutritional support (syllabus pg 56) References: Hurt et al. JPEN J Parenter Enteral Nutr . 2011;35: 60S.
  • Presentation provides good summary of vitamin deficiencies and toxicities (syllabus 57-61), which are more common after bariatric procedures.

 

IMG_1681 Nutrient Location

 

B12 Slide From Syllabus

B12 Slide From Syllabus

Key points:

  • For obese/overweight, for calorie calculation, can use adjusted weight to estimate energy expenditure (but don’t give more than 2000 calories per day). For teens/adults, usually need at least 1200 calories per day.
  • Harris-Benedict Equation (HBE) is often used but often overestimates needs (compared with indirect calorimetry) by about 600 cal/day. Common Equations (see below)
  • Protein –provide about 2 gm/kg for weight-for-length at 75-90% for age
  • Formulas with relatively higher protein content (eg. Jevity or Promote) can be helpful to provide adequate protein/nutrients/fluid without excessive calories
  • BUN can help with monitoring adequate nitrogen balance (in the abscess of diuretics). Goal: BUN 7-12 range
  • Goal initially (first 2 weeks) is weight stability or no more than 2-3 lbs of weight loss per week

Bottomline:  N2U served as a good review on a broad range of nutrition topics.  While this talk discussed obesity in critically-ill patients, given the prevalence of obesity and impact on long-term outcomes, I would suggest more attention to this topic at future meetings.

 

Energy Calculations

Energy Calculations

 

Disclaimer: This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

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.

 

Downtown Chicago

Downtown Chicago

 

N2U -Part 3: EoE, IBD, and Cystic Fibrosis

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2015 N2U Syllabus & Presentations

EoE Dietary Pointers (Syllabus pg 83-94): Sally Schwartz, Valeria Cohran

  • Even with SFED, elemental supplements helpful
  • Drink elemental beverages from covered glass with straw (improves palatability)
  • Cross-contamination –big issue
  • Label reading critical

Related posts:

IBD EEN Pointers (Syllabus pg 95-102): Rebecca Pipkorn, Justine Turner

  • Polymeric formulas –most palatable and least expensive. Oral EEN is used costly/not covered
  • EEN particularly helpful with microperforation/flare-up presentation and with infections (eg. TB)
  • EEN induces mucosal healing and improved symptoms

References:

  • Levin et al. Inflamm Bowel Dis. 2014;20:278-285.
  • Johnson et al. Gut 2006;55:356-361.
  • Sigall-Boneh et al. Inflamm Bowel Dis. 2014;20:1353-1360.
  • Wilschanski et al. Gut 1996;38543–548.
  • Critich et al. J Pediatr Gastroenterol Nutr. 2012;54: 298–305. NASPGHAN Guidelines

Conclusions:

  • Enteral therapy offers an alternative to steroids in patients with CD
  • Has potential to improve growth and IBD symptoms
  • Avoids the side effects of steroids
  • Need further research:
  1. – Unclear of the mechanism
  2. – Unclear of the best protocol
  3. – No standard protocol for reintroduction of food

Related posts:

Cystic Fibrosis (Syllabus pg 34-50) Justine Turner

Case in point: 10 yo with CF and poor growth, hx/o DIOS, poor intake, and distention.  Family had refused tube feedings previously.

Key point: Long-term survival is linked to nutritional status

  • Zemel et al. J Pediatr. 2000; 137(3):374-380.
  • Stallings et al. J Am Diet Assoc. 2008; 108(5):832-839.
  • McPhail et al. J Pediatr. 2008; 153(6):752-757.
  • Sharma et al. Thorax 2001; 56:746-750.

Other Caveats:

  • Intervene early
  • Breast milk (often with supplements) is optimal for infants
  • Poor oral intake àcould need periactin and/or supplemental feeds
  • Discussion re: pros/cons of Gtubes (pg 47 in syllabus)
  • Psychology support

Nutrition Goals

  • – Normal growth and optimal nutritional status
  • – Ages 0-2 year: Weight for length >50th percentile
  • – Ages 2-20 year: BMI percentile at or above 50th percentile
  • – BMI for males:23
  • – BMI for females: 22

Nutritional assessment at every visit & review:

  • – Weight, length/height, weight for length, BMI, head circumference in infants
  • – Nutritional education & dietary counseling
  • – Review PERT
  • – Review need for micronutrient supplementation: fat soluble vitamins (A, D, E, K), Ca, Fe, Zn, Na (salt), essential fatty acids

PERT (Pancreatic enzyme replacement therapy):

  • Infants 2000-4000 U lipase with 120 mL breast milk or formula– Mouth care for infants (and breast feeding mother)
  • Children 500-2500 U lipase/kg per meal (≤10000 U/kg/day or ≤ 4000 U/g fat/day); half meal dose with snacks
  • Ideally taken with meals and orally
  • Microspheres preferred formulation
  • Acid blockade (used to optimize enzyme activity)
  • Gold standard to assess adequacy is 72h fecal fat collection

Cystic Fibrosis Related Diabetes

  • Rare before 10 years of age
  • Increases mortality risk 6-fold
  • Weight loss and pulmonary decline begin 2-4 years prior to
  • diagnosis of CFRD

Related posts:

 

Robie House (at Univ Chicago)

Robie House (at Univ Chicago)

 

 

N2U -Part 2: Poor Growth and Short Bowel Syndrome

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Chicago -from Lincoln Park

Chicago -from Lincoln Park

2015 N2U Syllabus & Presentations

Failure to Thrive –Praveen Goday

These sessions were case-based learning.

Case 1

2 mo birth weight 4.5 kg, taking 80 cal/kg/day –20 cal, formula-fed.  Taking 8 gm/day

What to do?

Point –If infant has a high birth weight (relative to height), there is a tendency to drop significant percentiles.  Often, careful observation is best approach. (Taal et al. Obesity. 2013;21:1261-8.)

Case 2: 14 mo birth weight 2.2 kg (at term), weight and length below the 3rd percentile but tracking. Weight-for-length is at the 25th percentile.

What to do?

For SGA babies, ensure adequate calories, avoid juice, ensure no GI symptoms, follow their growth

Case 3: Patient born at 36 weeks gestation, birth weight 3 lb. 14 oz. lbs., birth length 17 in.; Growth was a consistent problem throughout pregnancy; Dysmorphic; genetic workup – negative (Growth curves on pg 72-73 of syllabus).

More data: Taking 27 cal/oz, high-calorie baby foods, no GI symptoms, screening labs negative.  What are your options?  Make sure the length is accurate.  If the weight-for-length is really decreasing, then probably a trial of nasogastric feedings.  In Milwaukee, AMT bridle is often used to prevent dislodgement –youtube video (7:37 min), uses magnets.  Still, tubes need to be changed month.  The AMT bridle can work for tubes as small as 5 Fr.

Practical definition of Failure to Thrive:

  • Weight-for-length <2nd percentile (WHO growth chart for kids <2 yrs) or BMI ❤rd percentile. BMI more problematic in infants because of accuracy of length. If any inaccurate measurement, BMI value squares the length value; thus exponentially inflating any discrepancy.
  • Poor or no weight gain over a period of time that varies according to the age of the child
  • Significant downward trend in weight percentiles; however, 30% of full-term infants cross one percentile and 23% cross two percentiles between birth and 2 years of age
  • Keep in mind parental heights and correction for prematurity (where applicable).

Key points:

  • Large for gestational infants often have “catch-down” growth. Avoid overly aggressive nutritional intervention
  • In small infants who are growing steady and with good wt-for-ht, avoid overly aggressive nutritional intervention.
  • Older kids with poor growth –screening labs: TTG IgA, IgA, CBC, ESR, CMP, TSH, Urinalysis, and possibly fecal elastase.
  • Older kids with poor growth—1st steps: avoid juices, avoid grazing (no feeding outside mealtimes except water)/scheduled meals & snacks, and probably cyprohepatadine. Management: Have child sit at table for 20 minutes, feedings every 3 hours, and avoid force feeding.
  • In children with history of prematurity under 32 weeks gestation who do not catch up by ~6 months of age — usually never catch up.
  • In infants/children with highly selective diets, may be presentation of autism. Often, an approach in those with food selectivity is to start by offering only foods the child used to eat (for a day) and see if this will work (should be safe for at least one day).

Short Bowel Syndrome –Valeria Cohran (pages 9-20 in syllabus)

Case:  3 ½-year-old AAF who presents for a second option. She is a former 26-week infant who had NEC. She has approximately 45 cm of residual bowel anastomosed to the transverse colon.

  • TPN-dependent
  • Minimal oral intake
  • Diarrhea up to 60 ml/kg with Enfacare

 

GI Fluid losses –see page 15 of syllabus (Wessel et al Semin Perinat 2007; 31: 104-11).  Sodium losses ~140 mEq/L from stomach, 80-140 mEq/L from ileostomy –in comparison, normal stool with sodium of ~5 mEq/L.

Key points:

  • Normal intestinal length varies greatly by gestational age; so residual 45 cm length in a 26 week infant suggests much greater potential for improvement than 45 cm length in a full term infants (page 14 in syllabus).
  • Avoid probiotics in patients with central lines.
  • Sodium depletion (urine sodium <10) associated with poor growth. Probably urine sodium >20 is adequate. Though, if high urine potassium (more than double urine sodium), this could indicate that urine sodium is retained at the expense of spilling potassium (ie. May need more sodium) Related post: Don’t Forget to Check Urine Sodium | gutsandgrowth
  • Pectin (liquid) can be helpful: 1% of volume intake. Benefiber can be helpful –expensive. Related blog post: Green beans for short gut syndrome | gutsandgrowth
  • Bacterial overgrowth –treatment can help diarrhea. Try to minimize PPIs –6 months after resection (period of gastric hypersecretion). Cholestyramine is not a popular option due to trouble with usage. Related post: Rehabilitation for Short Bowel Syndrome | gutsandgrowth
  • Micronutrient/vitamin monitoring. Page 16 in syllabus lists the micronutrient concentration of parenteral products and RDAs of micronutrients. “Don’t take copper out of TPN” –unless high level. ‘Worry some about micronutrient deficiency while on TPN but perhaps worry even more when transitioning off.’ Ubesie et al J Pediatr 2013 162: 1692-96. 93% anemic in this study of transitioning off TPN (iron,copper, other causes –pg 18 in syllabus). Related blog posts:Missing ingredients in TPN -Case Report | gutsandgrowth and TPN Drug Shortages -A Useful Reference | gutsandgrowth
  • B12 deficiency. If high MMA (likely due to B12 deficiency), then B12 shots recommended. B12 important for cognition. Related posts: Are we missing Vitamin B12? | gutsandgrowth and What I Didn’t Know About Vitamin B12 and Crohn’s Disease …
  • Iron deficiency. Consider anastomotic ulcers/ulceration of STEP procedure.
  • Lipid minimization/fish oil lipid formulations
  • Follow kids even after coming off TPN –at least annually. These kids can develop problems many years later.

More related posts:

Disclaimer: This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

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.