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Metabolics Questions

I am looking for nutritional guidelines for cystinuria. I have a newly dx patient with this disorder as well as chronic pancreatitis.
Category: Metabolics
Thank you for submitting your question to the Ask the Expert Panel of Nutricia Learning Center.

A literature search on this subject points to the following recent publications:
1. Saravakos P et al. Cystinuria: current diagnosis and management. Urology. 2014 Apr;83(4):693-9.
2. Mattoo A et al. Cystinuria. Semin Nephrol. 2008 Mar;28(2):181-91.
3. Knoll T et al. Cystinuria in childhood and adolescence: recommendations for diagnosis, treatment, and follow-up. Pediatr Nephrol. 2005 Jan;20(1):19-24.
Currently, there is no consensus on nutrition management in cystinuria. Basis of the medical management is prevention of kidney stone formation through hyperhydration, urinary alkalization, and pharmacologic therapy. Some clinicians have used a low-methionine diet to reduce production of cysteine/cystine. In addition, increased fluid intake and reduction of salt has been used.

Thank you again for your question. We hope you find this answer helpful for your clinical practice.

Steven Yannicelli, PhD, RD

Disclaimer: Please note that this answer is not meant to diagnose individual patients, and that any treatment or disorder management decisions have to be taken with your own professional experience.
Tags: Cystinuria, DX

I understand that there are new recommendations for the dietary management of phenylalanine hydroxylase deficiency. Where can I find them?
Category: Metabolics
Thank you for submitting your question to the Ask the Expert Panel of Nutricia Learning Center.

The new guidelines were published in early 2014. Please also see the concurrently published American College of Medical Genetics and Genomics guideline for the medical treatment of phenylalanine hydroxylase deficiency.
1. Singh RH et al. Recommendations for the nutrition management of phenylalanine hydroxylase deficiency. Genet Med. 2014 Feb;16(2):121-31
  Here is the link to the abstract. You can access the full article through this link as well. Reference
2. Vockley J et al. Phenylalanine hydroxylase deficiency: diagnosis and management guideline. Genet Med. 2014 Feb;16(2):188-200
  Here is a link to the abstract: Reference
Please also note the publication on the 2012 NIH Conference on PKU.
1. Camp KM et al. Phenylketonuria Scientific Review Conference: state of the science and future research needs. Mol Genet Metab. 2014 Jun;112(2):87-122
  Here is the link to the abstract: Reference
Thank you again for your question. We hope you find this answer helpful for your clinical practice.

Ulrike Reichert, MS

Disclaimer: Please note that this answer is not meant to diagnose individual patients, and that any treatment or disorder management decisions have to be taken with your own professional experience.
Tags: phenylalanine hydroxylase deficiency

Should I be concerned about my PKU patients’ carnitine levels and should I monitor them on a regular basis?
Category: Metabolics
Thank you for submitting your question to the Ask the Expert Panel of Nutricia Learning Center.

We don't think there is enough evidence to be concerned about carnitine levels in PKU patients unless your patients are not taking formula. From what we understand from the literature, low carnitine levels have mainly been found in patients who did not adhere to the diet recommendations or consumed medical food that did not contain any carnitine.
1. Vileseca MA et al. Controled diet in phenylketonuria may cause serum carnitine deficiency. J Inherit Metab Dis. 1993; 16(1): 101-4.
2. Sitta A et al. L-Carnitine blood levels and oxidative stress in treated phenylketonuric patients. Cell Mol Neurobiol 2009; 29(2):211-8.
Weigel et al (2008) found suboptimal carnitine status in patients consuming both medical food with and without carnitine compared to healthy control and recommends giving carnitine levels careful consideration.
1. Weigel C et al. Carnitine staus in early-treated children, adolescents and young adults with phenylketonuria on low phenylalanine diets. Ann Nutr Metab 2008;53(2):91-5.
The NIH Fact Sheet on Carnitine notes that carnitine intake in vegans is 10-12 mg/d and is typically of no concern in respect to carnitine deficiency as the liver and kidney produce sufficient amounts.
1. NIH Fact Sheet
The new PKU Periflex Junior Plus and TYR Anamix Next contain 24 mg/100 g product (=28 g PE), which would provide more carnitine (in many cases double) than the documented intake by vegans, so as long as patients are consuming formula with carnitine, they should be fine. The amount is lower compared to the previous Periflex Junior formulation, but the same as the current Periflex Advance product (per 10 g PE). We have no reports of carnitine deficiency in any PKU patient using our products.

Thank you again for your question. We hope you find this answer helpful for your clinical practice.
Ulrike Reichert, MS

Disclaimer: Please note that this answer is not meant to diagnose individual patients, and that any treatment or disorder management decisions have to be taken with your own professional experience.
Tags: Carnitine in PKU, PKU

Specialized Adult Nutrition Questions

I have many patients with Urinary tract infections and chronic history of the same. Is there a particular strain of bacteria I should make sure is in the probiotic I order that works better for bladder health?
Category: Specialized-Adult-Nutrition

Thank you for submitting your question to the Ask the Expert Panel of Nutricia Learning Center. Your experience with patients with frequent Urinary Tract Infections (UTIs) is not unique. In fact, UTIs are among the most common Healthcare-Associated Infections (HAI) in both acute care and long term care facilities^1,2.

Dietary management of UTIs can be multi-faceted. Probiotics assist with the dietary management of infections in two ways. First, by strengthening the immune system and, second, by promoting the growth of beneficial bacteria in the GI tract. Frequent antibiotic use often reduces the number of beneficial bacteria present. The use of probiotics to restore the balance of beneficial bacteria may help to reduce colonization of pathogens in the GI tract, such as E. Coli. It is bacteria, such as E. Coli, from fecal or vaginal sources that is a primary cause of UTIs. Common probiotics are lactobacillus, bifidobacterium and saccharomyces boullardi^3,4 . There is emerging evidence that Lactobacillus specifically may have a role to play in the management of urinary tract health in adult women⁵. However, more studies are required.

Prebiotic fiber can also assist with improved intestinal health by selectively promoting the growth of beneficial bacteria in the GI tract³. Common prebiotics are fructo-oligosaccharides (FOS), inulin and lactulose. A synbiotic product contains both probiotics and prebiotics; they work together to support intestinal health.

Additional considerations for dietary management of UTIs include the use of cranberry-containing products. The proanthocyanidins present in cranberry may help to inhibit E.coli from attaching to the bladder wall . Supplemental Vitamin C and dextrose-mannose may also contribute to an environment that further supports urinary tract health. Combining these items with adequate hydration to flush harmful bacteria may assist with dietary management of UTIs.

In my experience, dietary management is an important component in an overall approach to reducing UTIs. I have participated in quality assurance programs designed to reduce these infections in skilled nursing facilities. Utilizing the Quality Indicator Reports and a protocol that included the use of cranberry, prebiotics and adequate hydration, we were able to better maintain the urinary tract health of our residents

Thank you again for your question. We hope you find this helpful for your clinical practice.
Lynn Spalding, RDN, CSG

Disclaimer: Please note that this answer is not meant to diagnose individual patients, and that any treatment or disorder management decisions have to be taken with your own professional experience.

¹Nicholle L. Urinary tract infection in long-term care facilities. Infect. Control Hosp. Epidemiology. 1993;14:220-225.
²Richard CL. Urinary tract infections in the frail elderly; Issues for Diagnosis, treatment and Prevention. Int. Urol Nephrol. 2004;36: 457-463
³Vyas,U et al. Probiotics, Prebiotics and Synbiotics: Gut and Beyond. Gastroenterol Res Practice 2012. 2012:872716. Abstract: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3459241
⁴http://kidney.niddk.nih.gov/Kudiseases/pubs/utiadult/#causes, Accessed 10/2/14
⁵Grin PM et al. Lactobacillus for preventing recurrent urinary tract infections in women: meta-analysis. Can J Urol. 2013 Feb;20(1):6607-14. Abstract: http://www.ncbi.nlm.nih.gov/pubmed/23433130
⁶From http://www.cranberryinstitute.org/doclib/doclib.cgi/1/: Nicolosi D et al. Anti-adhesion activity of A2-type proanthocyanidins (a cranberry major component) on uropathogenic E. coli and P. mirabilis strains. Antibiotics 3(2):143-54. Posted: July 25, 2014. Website accessed 10/2/14.

Tags: Chronic UTI

GI/Allergy Questions

How effective is dietary restriction in the management of eosinophilic esophagitis (EoE)?
Category: GI/Allergy
Thank you for submitting your question to the Ask the Expert Panel of Nutricia Learning Center.

Recent articles on this subject point to the following publications:
1. 1. Henderson CJ et al. Comparative dietary therapy effectiveness in remission of pediatric eosinophilic esophagitis. J Allergy Clin Immunol Jun 2012:129(6): 1570-78.
  
  Here is the link to this paper:Reference
2. 2. Gonsalves N and Kagalwalla AF. Dietary treatment of eosinophilic esophagitis. Gastroenterol Clin North Am. 2014 Jun;43(2):375-83.
  
  Here is the link to this paper:Reference
3. 3. Dellon ES and Liacouras CA. Advances in clinical management of eosinophilic esophagitis. Gastroenterol. 2014 Aug 7.
  
  Here is the link to this paper: Reference
EoE is characterized as a chronic immune/antigen-mediated esophageal disease exemplified by esophageal dysfunction and esophageal mucosal eosinophilia. Three distinct types of dietary restriction have demonstrated highly effective histologic remission ranging from 65 to 95% (remission defined as <15 eosinophils per high power field) in both children and adults. These 3 types of restrictive diets include 1) an elemental diet-exclusion of all dietary antigens, thus dietary intake is attained via the use of amino acid-based formula; 2) empiric elimination diet-removal of the most frequent food allergens in the U.S. diet: milk, wheat, egg, soy, peanut/tree nuts, fish/shellfish); and 3) allergy-test directed diet-removal of food antigens based on skin testing results. Typically, following the attainment of histologic remission, food antigen trials are conducted. Over time, this permits identification of specific food antigen triggers that can be avoided and the diet may be expanded, allowing a return to consumption of most table foods. A fundamental advantage of managing esophageal mucosal eosinophilia using dietary restriction and subsequent food antigen reintroduction vs pharmacologic management is the identification of specific food antigen triggers which can be avoided and non-antigen offenders may be used to build a diet that does not promote esophageal dysfunction.

Thank you again for your question. We hope you find this answer helpful for your clinical practice.

Carol Henderson, PhD, RD, LD

Disclaimer: Please note that this answer is not meant to diagnose individual patients, and that any treatment or disorder management decisions have to be taken with your own professional experience.
Tags: Eosinophilic Esophagitis, EoE

Do children diagnosed with autism spectrum disorder (ASD) experience gastrointestinal (GI) symtpoms more frequently than their non-affected peers?
Category: GI/Allergy
Thank you for submitting your question to the Ask the Expert Panel of Nutricia Learning Center.

Recent articles on this subject point to the following publications:
1. 1. Gorrindo P, Williams KC, Lee EB, Walker LS, McGrew SG, and Levitt P. Gastrointestinal dysfunction in autism: Parental report, clinical evaluation, and associated factors. Autism Res. 2012 Apr; 5(2):1-1-108.
  
  a. Here is the link to this paper:Reference
2. 2. McElhanon BO, McCracken C, Karpen S, and Sharp WG. Gastrointestinal symptoms in autism spectrum disorder: A meta-analysis. Pediatrics. 2014 May; 133(5):872-883.
  
  a. Here is the link to this paper:Reference
Parents commonly report concerns for GI issues in their child diagnosed with ASD. In a study designed to compare parental report of the presence of GI issues to an assessment by pediatric gastroenterologists, results were highly concordant. Constipation was the most prevalent type of GI issue and was associated with language and social impairments. The authors suggest healthcare providers of children with ASD be vigilant for the presence of GI issues particularly in children who lack the ability to communicate verbally.

Meta-analysis of the relatively few highly robust publications that investigated GI status indicated a greater prevalence of GI symptoms among children with ASD compared with control children. Assessment of GI status involved caregiver report or medical chart review. Diarrhea was the most common symptom assessed followed by general GI concerns (a composite category denoted as the percentage of children experiencing ≥1 GI symptom: constipation, diarrhea, abdominal pain). Findings demonstrated a greater than 3-fold elevated risk of general GI concerns, constipation or diarrhea and a greater than 2-fold elevated risk of abdominal pain among children with and without ASD. These results align with parental concerns about the possible occurrence of GI symptoms in ASD.

Thank you again for your question. We hope you find this answer helpful for your clinical practice.

Carol Henderson, PhD, RD, LD

Disclaimer: Please note that this answer is not meant to diagnose individual patients, and that any treatment or disorder management decisions have to be taken with your own professional experience.
Tags: Autism

Neurology Questions

New to Ketogenic Diet I am new to the ketogenic diet and would like to find a resource and/or manual to help me learn about the diet(s) and how to create the diet for patients. Any suggestions are appreciated!
Category: Neurology
Thank you for submitting your question to the Ask the Expert Panel of Nutricia Learning Center.

The resource that helped me the most in understanding the rules of the ketogenic diet and how to calculate the diet parameters was the following book:
1. Ketogenic Diets: Treatments for epilepsy and other disorders by EH Kossoff, JM Freeman, Z Turner and JE Rubenstein. The book is now in its 5th edition and available through bookstores or Amazon.
2. As a follow up to reading the book a few times, I made myself familiar with KetoCalculator (https://www.ketocalculator.org), an online tool that is free to healthcare professionals who have a working knowledge of the ketogenic diet. KetoCalculator makes calculating the ketogenic diet much easier. This resource also helped me to tie the information I learned from the book into practical terms of patient care.
3. In addition, Nutricia offers a series of recorded Webinars on many aspects of the Ketogenic Diet. Please check out the Webinar section of this site (NutriciaLearningCenter.com). Please also visit MyKetoCal for additional resources.
Thank you again for your question. We hope you find this answer helpful for your clinical practice.

Robyn Blackford, RDN LD, Ellen Sviland-Avery MS, RD, LD, CNSC

Disclaimer: Please note that this answer is not meant to diagnose individual patients, and that any treatment or disorder management decisions have to be taken with your own professional experience.
Tags: New to Ketogenic, Ketogenic Resources

Ketogenic Diet Is there protocol for admission and discharge of patients for inpatient initiation?
Category: Neurology
Thank you for submitting your question to the Ask the Expert Panel of Nutricia Learning Center.

At my facility, we do not have a set protocol for admission or discharge. However, our general rule is:
1. • First day: 24 hour fast with zero calories and zero carbohydrate fluids
2. • Second day: 1/3 kcal strength at goal ratio for the next 24 hours with either eggnog or formula
3. • Third day: 2/3 kcal strength at goal ratio for the next 24 hours with either eggnog or formula
4. • Forth day: Full strength kcal
We also educate our families and patients daily to familiarize them with the ketogenic diet, cooking, recipes and expectations. It may also be helpful to get them in touch with families who are already successfully using the ketogenic diet.

Thank you again for your question. We hope you find this answer helpful for your clinical practice.

Zahava Turner, RD CSP LDN

Disclaimer: Please note that this answer is not meant to diagnose individual patients, and that any treatment or disorder management decisions have to be taken with your own professional experience.
Tags: New to Ketogenic, Ketogenic Resources