Author Archives: gutsandgrowth

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About gutsandgrowth

I am a pediatric gastroenterologist at GI Care for Kids (previously called CCDHC) in Atlanta, Georgia. The goal of my blog is to share some of my reading in my field more broadly. In addition, I wanted to provide my voice to a wide range of topics that often have inaccurate or incomplete information. Before starting this blog in 2011, I would tear out articles from journals and/or keep notes in a palm pilot. This blog helps provide an updated source of information that is easy to access and search, along with links to useful multimedia sources. I was born and raised in Chattanooga. After graduating from the University of Virginia, I attended Baylor College of Medicine. I completed residency and fellowship training at the University of Cincinnati at the Children’s Hospital Medical Center. I received funding from the National Institutes of Health for molecular biology research of the gastrointestinal tract. During my fellowship, I had the opportunity to work with some of the most amazing pediatric gastroenterologists and mentors. Some of these individuals included Mitchell Cohen, William Balistreri, James Heubi, Jorge Bezerra, Colin Rudolph, John Bucuvalas, and Michael Farrell. I am grateful for their teaching and their friendship. During my training with their help, I received a nationwide award for the best research by a GI fellow. I have authored numerous publications/presentations including original research, case reports, review articles, and textbook chapters on various pediatric gastrointestinal problems. In addition, I have been recognized by Atlanta Magazine as a "Top Doctor" in my field multiple times. Currently, I am the vice chair of the section of nutrition for the Georgia Chapter of the American Academy of Pediatrics. In addition, I am an adjunct Associate Clinical Professor of Pediatrics at Emory University School of Medicine. Other society memberships have included the North American Society for Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN), American Academy of Pediatrics, the Food Allergy Network, the American Gastroenterology Association, the American Association for the Study of Liver Diseases, and the Crohn’s and Colitis Foundation. As part of a national pediatric GI organization called NASPGHAN (and its affiliated website GIKids), I have helped develop educational materials on a wide-range of gastrointestinal and liver diseases which are used across the country. Also, I have been an invited speaker for national campaigns to improve the evaluation and treatment of gastroesophageal reflux disease, celiac disease, eosinophilic esophagitis, hepatitis C, and inflammatory bowel disease (IBD). Some information on these topics has been posted at my work website, www.gicareforkids.com, which has links to multiple other useful resources. I am fortunate to work at GI Care For Kids. Our group has 17 terrific physicians with a wide range of subspecialization, including liver diseases, feeding disorders, eosinophilic diseases, inflammatory bowel disease, cystic fibrosis, DiGeorge/22q, celiac disease, and motility disorders. Many of our physicians are recognized nationally for their achievements. Our group of physicians have worked closely together for many years. None of the physicians in our group have ever left to join other groups. I have also worked with the same nurse (Bernadette) since I moved to Atlanta in 1997. For many families, more practical matters about our office include the following: – 14 office/satellite locations – physicians who speak Spanish – cutting edge research – on-site nutritionists – on-site psychology support for abdominal pain and feeding disorders – participation in ImproveCareNow to better the outcomes for children with inflammatory bowel disease – office endoscopy suite (lower costs and easier scheduling) – office infusion center (lower costs and easier for families) – easy access to nursing advice (each physician has at least one nurse) I am married and have two sons (both adults). I like to read, walk/hike, bike, swim, and play tennis with my free time. I do not have any financial relationships with pharmaceutical companies or other financial relationships to disclose. I have helped enroll patients in industry-sponsored research studies.

“This Is A Stick Up — Your Money or Your Life”

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When I read a recent Hepatology editorial (Hepatology 2015; 61: 1106-8), I could not help think of the aforementioned title of this blog.

Here’s the scoop:

The two most commonly used medications for Wilson’s disease are trientine (Syprine) and D-penicillamine (Cupramine). For about 20 years, the original manufacturer of these medications kept the consumer cost at ~$1 per 250 mg tablet.  Currently the cost of Syprine is ~$200 per 250 mg tablet and Cuprimine costs ~$55 per 250 mg tablet.  This 200-fold increase translates into a yearly cost of ~$300,000.

How did this happen?

  • Little competition
  • Profit motive
  • Patients are reluctant to protest (they need this medication to be manufactured)

Why is this outrageous?

This increase in cost was not driven by any new discovery or research innovation.

Are there options?

Zinc is inexpensive and may be an option after initial period of chelation/normalization of liver biochemistries.  Zinc needs to be taken two to three times per day and “well away from meals for best absorption.”

Bottomline: These medication prices are outrageous.

Briefly noted:

  • “Molecular pathophysiology of portal hypertension”  Hepatology 2015; 61: 1406-15. Terrific review with excellent figures.
  • “Ezetimibe for the treatment of Nonacloholic Steatohepatitis” (MOZART trial) Hepatology 2015; 61: 1239-50. This randomized double-blind, placebo-controlled trial with 50 patients (biopsy-proven NASH) showed that Ezetimbe was not significantly different from placebo in histologic response rates, serum aminotransferases, or in magnetic resonance elastography findings.
  • Van Biervliet et al. “Clinical Zinc Deficiency as Early Presentation of Wilson Disease” JPGN 2015; 60: 457-9. Case report.

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Chronic Pancreatitis in Pediatrics -Descriptive Study

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“Genius is one percent inspiration and ninety-nine percent perspiration.” – Thomas A. Edison

I thought about this saying as I was reading an editorial titled: “Understanding Pediatric Chronic Pancreatitis: Inspiration and Hard Work Required” (Pant C, Sferra TJ. J Pediatr 2015; 166: 798-800). The editorial was reviewing the article “Pediatric Chronic Pancreatitis Is Associated with Genetic Risk Factors and Substantial Disease Burden” (Schwarzenberg SJ et al. J Pediatr 2015; 166: 890-6).

The study comes from the International Study Group of Pediatric Pancreatitis: In Search of a Cure (INSPPIRE) consortium.  None of the findings in the study are particularly surprising; nevertheless, a descriptive study of the patients in the registry who had strictly defined chronic pancreatitis (n=76) is still an important early step in improving our understanding of this dreaded problem.

Chronic pancreatitis required either:

  • Abdominal pain consistent with pancreatic pain with imaging findings suggestive of chronic pancreatic damage
  • Evidence of exocrine or endocrine pancreatic insufficiency and imaging findings suggestive of chronic pancreatic damage
  • Histology (surgical biopsy) findings suggestive of chronic pancreatitis

Key points:

  • Two-thirds of patients with genetic testing had identified genetic mutations: PRSS1 (n=33), SPINK1 (n=14), CFTR (n=11), chymotrypsin C (CTRC) (n=2).  Mutations in more than 1 gene were noted in 9 patients, including 6 of the 11 with CFTR mutations.  Several newer mutations, like calcium-sensing receptor and carboxypeptidase A1, were not evaluated in any of the patients.
  • Pancreas divisum was present in 15 patients; however, 8 of 15 of these patients had an identified genetic mutation as well.
  • Radiographic findings of chronic pancreatitis were most commonly ductal abnormalities and pancreatic atrophy. This is in contrast to adults in which pancreatic calcifications are common.
  • The researchers also document severe disease burden with patients reporting a median of 3 emergency dept visits and 2 hospitalizations in the previous year. In addition, 70% (n=47) had missed 1 day of school in the past month and 34% had missed 3 or more days.
  • Medical treatment (eg. pancreatic enzymes) was ineffective in the majority of patients.
  • 43% had undergone ERCP and two-thirds noted improvement from this intervention
  • Surgical procedures were performed in 39% and were helpful in the majority.  Total pancreatectomy with islet autotransplantation was the most common surgery in this cohort and was helpful in 20 of 21 patients.
  • The authors recommend avoidance of CT scans due to concerns of accumulating excess ionizing radiation exposure.

Take home message: For me, this study helps define the problem.  As a practical matter, it would be helpful to have a genetic panel to check for the lesser frequent mutations if PRSS1, SPINK1, and CFTR are normal.

Related blog posts:

Stool Color Cards -Not Flashy but Effective

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A recent report (Gu YH et al J Pediatr 2015; 166: 897-902) shows the cumulative experience of 19 years of using stool color cards to detect biliary atresia.  Cards were distributed to all pregnant women (Tochigi Prefecture, Japan) prior to or during the postnatal one-month health check.

Key findings:

  • 34 patients detected among 313,230 live births. Reported sensitivity: 76.5% and specificity 99.9%.
  • Mean age for performance of Kasai was 59.7 days.
  • Improved long-term retention of native liver: 88% at 5 years, 77% at 10 years, and 49% at 15 years.

Limitation: Children in Western countries have had lower success rates following Kasai procedure, so it is unclear whether stool cards would be as effective in different regions.

Take-home message: Detecting biliary atresia earlier will improve outcomes.  Stool color cards should be an easy low-tech option.  Other options would include stool color apps and checking bloodwork.

Image below from Screenshot from John Pohl’s twitter feed:

Embedded image permalink

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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.

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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)