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vol. 2 • no.

4 ICAN: Infant, Child, & Adolescent Nutrition

Evidence-Based Practice Reports

Best Practices in Nutrition


for Children With Myelomeningocele
Wendy Wittenbrook, MA, RD, CSP, LD

Abstract: Children born with myelo- Myelomeningocele is a neural tube development of MMC. A recent study
meningocele (MMC), the most severe defect caused by incomplete closure of found a significant increase in a woman’s
form of spina bifida, face multiple the spinal cord during early pregnancy. risk of having a child with a neural tube
challenges throughout their life span. The neural tube typically closes by day defect with a serum B12 level ≤250
Neurogenic bowel, neurogenic bladder, 28 of pregnancy, usually before a woman mcg/L at 15 weeks of gestation.7
Arnold-Chiari II malformation, and even knows she is pregnant. When the
hydrocephalus are common complica- neural tube fails to close, it leaves a sac Wellness
tions that have implications for or lesion along the midline of the spinal In 2001, the World Health Organization
elimination, feeding, and learning. cord. The level and severity of the lesion released the International Classification of
Nutrition education and intervention determines the level of sensation and Function, Disability and Health (ICF).8
that use the concepts of family-centered motor function in the individual. There This is the first universal method to
care beginning in infancy and are 4 types of spina bifida, as shown in classify body structures, body functions,
continuing throughout childhood, Table 1. Table 2 shows some of the activities and participation, and environ-
adolescence, and adulthood are complex medical issues commonly found mental factors for individuals with
important. These can help maximize in MMC.3 disabilities. There has been a shift in
wellness, fitness, and independence in focus to emphasize a person’s function
children with MMC, as constipation, Folic Acid Supplementation and health instead of his or her disability.
dysphagia, and obesity are common In 1991, the Centers for Disease Control Early emphasis on wellness and fitness at
comorbidities seen in individuals with (CDC) recommended all high-risk women an early age fits into the ICF model and
MMC. This article reviews the literature (ie, a diagnosis of spina bifida or a has been found to promote self-reliance,
on nutrition assessment and interven- previous pregnancy/child with a self-care, and lean body mass in children
tion in this unique population to assist diagnosis of spina bifida, anencephaly, or with MMC.9-11 Interdisciplinary teams can
nutrition professionals in caring for other neural tube defect) take 4 mg of use the ICF to address the multiple needs
children with MMC. folic acid daily (available through of a child with MMC during childhood
prescription) when they start trying to and adolescence8 as well as transition
Keywords: spina bifida; myelomeningocele; conceive and during the first trimester of planning for adult care.12
developmental disabilities; folic acid pregnancy.4 The US Public Health Service Family-centered care is crucial when
issued its recommendation in 1992 for all working with any child who has special
women of childbearing age to consume health care needs, as family strengths and
400 mcg (micrograms) of folic acid daily resources are important contributors to
Background
to reduce the risk of MMC.5 A few years the success of the child’s nutrition care
Myelomeningocele (MMC), also known later, the Food and Drug Administration plan.13 Assessing a child’s nutritional
as spina bifida, is one of the most required folic acid fortification of status and providing nutrition education
common birth defects in the United States, enriched grain products to begin January 1, on a regular basis in the context of
affecting 1 in 2500 babies.1 Advances in 1998.6 In addition to folic acid, genetics, family-centered care support the family in
medicine have significantly increased the maternal obesity, and environmental establishing good eating habits and
survival rate to 90% reaching adulthood.2 factors also play a role in the managing some of the complications

DOI: 10.1177/1941406410375983. From the Texas Scottish Rite Hospital for Children, Dallas, Texas. Address correspondence to Wendy Wittenbrook, MA, RD, CSP, LD,
Texas Scottish Rite Hospital for Children, 2222 Welborn St, Dallas, TX 75219; e-mail: Wendy.Wittenbrook@tsrh.org.
For reprints and permissions queries, please visit SAGE’s Web site at http://www.sagepub.com/journalsPermissions.nav.
Copyright © 2010 The Author(s)
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ICAN: Infant, Child, & Adolescent Nutrition August 2010

related to MMC, such as constipation,


Table 1. osteopenia, and obesity.
Types of Spina Bifida2 The Ellyn Satter “division of responsibil-
ity” for feeding children is a useful tool to
share with parents of children with MMC
Type Definition/Issues
when establishing healthy eating habits.
Spina bifida A small gap or defect in some of the vertebrae that is not In this method, the child is responsible
occulta visible. There are usually no problems with the spinal for how much and whether he or she
cord or nerves. Most people do not know they have this eats, and the parent is responsible for the
type of spina bifida until they have an X-ray or magnetic what, when, and where of feeding.14 This
resonance imaging for other medical reasons. approach can help families as they try to
get their undernourished child diagnosed
Meningocele The meninges are exposed through the spine in a sac with failure to thrive to eat more or their
or cyst containing cerebrospinal fluid. Although there “picky eater” to try more vegetables by
is typically no nerve damage, affected individuals may clearly defining the feeding relationship.
have some medical issues. Parents of a child with special needs can
Occult spinal An infant presents with a dimple, red marks, or tufts of
feel particularly burdened with the
dysraphism hair on the lower back. There may be medical issues,
multiple challenges related to feeding
such as urologic, neurologic, or orthopedic issues, their child.
related to the spinal cord that require further evaluation.
Anthropometrics
Myelomeningocele The most severe and complex type of spina bifida in which Consistently obtaining an accurate
the meninges and spinal nerves are exposed through the length/height measurement in children
spine at the level of the defect. This results in disabilities with MMC may be difficult secondary to
and nerve damage. Surgery to repair and close the scoliosis, contractures, and body structure
defect is typically done within 24 to 72 hours after birth. differences. Measurements of length/
height are typically obtained using length
boards, wall-mounted stadiometers, or
segmental length. The 2000 CDC growth
charts15 are used to plot and assess a
Table 2.
child’s growth trends. Linear growth
Common Medical Issues in Spina Bifida3 usually slows down around 2 years of
age, but weight gain may continue
Endocrine Growth hormone deficiency trending or increase at a faster rate, which
Precocious puberty reiterates the importance of accurate and
consistent measurements beginning in
Neurologic Hydrocephalus infancy. MMC growth charts16,17 may be
Arnold-Chiari II malformation used in conjunction with the CDC growth
Seizures charts to evaluate growth velocity in a
Paralysis child with MMC. Recent charts include
Orthopedic Scoliosis growth data according to lesion level and
Lumbar lordosis ambulatory status.17 Arm span measure-
Contractures of the hips/knees ments are not typically used in children
with MMC secondary to growth issues
Gastrointestinal Dysphagia seen in older children with a higher level
Neurogenic bowel lesion, with the differences most notable
in the lower extremities.18 However, some
Other Neurogenic bladder
studies have shown that the use of arm
Hypertension
span measurements is accurate during
Learning differences
growth hormone treatment.19,20 Children
Insensate skin
with MMC have higher rates of precocious
Pressure sores
puberty and an earlier growth spurt21;
Latex allergy
however, their final adult height is shorter
in relation to their peers,10,21-24 even after

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vol. 2 • no. 4 ICAN: Infant, Child, & Adolescent Nutrition

successful growth hormone treatment to assessing their growth trends over time in For further assessment of a child’s resting
increase linear growth.20,25 addition to growth charts. energy expenditure, indirect calorimetry is
Overweight and obesity have long been considered the “gold standard,” which can
recognized as comorbidity risks related to Energy Needs be helpful when the goal is to prevent
multiple factors, including ambulatory The aforementioned differences in body over/underfeeding. Once resting energy
status, activity level, and hypotonicity in composition of children with MMC are expenditure has been calculated from
children with MMC21,22,26 that continue reflected in their measured resting energy indirect calorimetry measurements, a
through adulthood.27 This can result in expenditure. Each child’s energy needs steady state should be verified to ensure
additional medical conditions associated should be determined on an individual accuracy of the measurement.41 Then the
with obesity, such as metabolic syndrome, basis, as typical predictive equations addition of activity factors results in a
hypertension, and diabetes. Nelson and overestimate energy requirements in 1/5 of value for total energy expenditure. The
colleagues28 reported that one third of children with special needs.26,39 A lower recently published Dietary Reference
MMC patients met the criteria for lean body mass and lower resting energy Intakes (DRI) has physical activity
metabolic syndrome. Contributing to expenditure10,11,29 that results in lower total coefficients42 that have been used as
these medical issues are the higher energy expenditure26,29,35 can make it a activity factors for indirect calorimetry in
percentage of body fat10,22,26,29-32 and lower challenge for children with MMC to clinical practice.
amount of lean body mass11,26,29,32,33 seen achieve and maintain a healthy weight.
in children with MMC. Activity level plays Children with MMC have been shown to Bone Health
a major role in body composition. Studies maintain their weight with an energy Children with MMC have an increased
have shown that ambulatory children with intake meeting 80% of the Recommended risk of osteopenia and osteomalacia, as
MMC who have a higher level of function Dietary Allowance (RDA).23 In addition, shown in Table 3. It is well established
in daily living and physical activities have children with MMC have been shown to that ambulation significantly increases
a higher level of fitness27,31,34-36 that results expend 25% less energy than children of bone mineral density (BMD) in children
in higher lean body mass11 with reduced the same age not affected by MMC.26 The with MMC24,43,44; however, this effect
amounts of body fat.24 However, the RDA for energy is often used to calculate appears to dissipate in adulthood.45
percentage of body fat between children energy needs for infants, with adjustments Children with a neurogenic bladder that
who are ambulatory and those who are made based on growth velocity. Grogan has been augmented have shown lower
nonambulatory is similar.24,34 In addition, and Ekvall11 published formulas in 1999 to BMD in previous studies.46,47 Metabolic
children who have a higher MMC lesion calculate energy needs after infancy in acidosis has not been determined as the
typically have impaired linear growth and children with MMC. Their recommenda- cause of low BMD in children with
develop obesity.18,30 tions are 50% of the RDA/age for weight MMC.46,48 An earlier report by Quan et al49
Body mass index (BMI) is widely maintenance after infancy, 9 to 11 kcal/cm found increased urinary calcium
accepted as a screening tool for obesity in for weight maintenance, and 7 kcal/cm excretion in nonambulatory children
children older than 2 years of age.37 The for weight loss after age 6. Patt et al40 with lower BMD, and Boylu et al47
difficulty in obtaining an accurate height describe an equation that uses a height theorized that the underlying neuro-
measurement in children with MMC, measurement and injury level to determine logic processes related to a neurogenic
along with their differences in body energy needs in patients with spinal cord bladder rather than bladder augmenta-
habitus, can make assessment of BMI injury. This equation has been used in tion surgery resulted in a lower BMD.
inaccurate. Children with MMC have clinical practice to determine energy There is a higher risk of osteomalacia
disproportionate body fat distribution in needs in children with MMC: and osteopenia/osteoporosis in children
the trunk and upper limbs.9,11 Skinfold and adults with MMC related to lower
measurements are useful and easy to use Boys (11.7 × height in cm) BMD,44,45,49 even though 1 study showed
tools in a clinical setting that can be used + (30.7 × level) - 931.23 most children with MMC met the RDA
longitudinally to assess trends in growth for calcium.44 Emerging research
and body composition if used regularly by Girls (12.4 × height in cm) continues to expand the knowledge of
an experienced clinician. Humeral length, + (21.8 × level) - 1036.42 the multiple factors involved in bone
mid-arm circumference (MAC), and triceps health in addition to dietary calcium
skin fold (TSF) can be measured on a where level is an injury number cor- intake and weight-bearing status and
regular basis during clinic visits. Then responding to the level of the affected supports recommendations for a
MAC and TSF can be compared with vertebrae. The lesion level in children with well-balanced diet with a variety of
reference percentiles.38 Because MAC and MMC can be used as the injury number foods.
TSF standards are based on data collected corresponding to the affected vertebrae. A nutrition assessment should include a
on children without disabilities, tracking Assessment of energy needs has to child’s typical dietary intake of calcium to
serial measurements on individual children consider growth velocity, ambulatory ensure the DRI for age is met.50 Clinical
with MMC can serve as a method for status, MMC lesion level, and activity level. practice has suggested that calcium

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ICAN: Infant, Child, & Adolescent Nutrition August 2010

supplementation is often necessary dysphagia. Feeding team members may


Table 3. because of the individual food prefer- include a physician, registered dietitian,
Risk Factors for Osteopenia and ences of children with MMC. speech-language pathologist, psycholo-
Osteomalacia in Myelomeningocele In 2008, the American Academy of gist, occupational therapist, social worker,
Pediatrics recommended increasing and nurse. A clinical feeding evaluation
Ambulatory status vitamin D intake of all infants, children, and a videofluoroscopic swallow study
and adolescents to 400 IU daily.51 Vitamin (VFSS) can provide valuable information
Anticonvulsant medications D status is assessed by checking levels of to the feeding team. Observation of
serum 25(OH)-D, with a deficiency mealtime allows the team to evaluate
Obesity diagnosed at a level <20 ng/mL. There is many variables contributing to feeding,
Lack of sensation no established consensus or change in including the parent-child feeding
the DRI to recommend repletion doses in relationship, the child’s oral-motor skills,
Limited sun exposure children, although maintenance doses in and typical positioning for feedings.
children have been suggested in the Once this evaluation is completed, a
Hypotonia
range of 400 to 1000 IU/d.52 Once vitamin VFSS or modified barium swallow study
Malabsorption D supplementation has been started, can be conducted to identify aspiration as
levels of serum 25(OH)-D should be well as abnormalities in oral-motor skills,
Diet low in calcium and vitamin D rechecked and assessed every 3 months swallow initiation, pharyngeal dysphagia,
to ensure levels return to normal.51 and slow esophageal motility. Silent
aspiration can occur without obvious
external symptoms. An interdisciplinary
Table 4. Common Challenges feeding team is valuable to work with
Risk Factors for Dysphagia in Arnold- families to implement team recommen-
Feeding Problems
Chiari II Malformation53,54 dations.56,57 The National Dysphagia Diet58
Dysphagia provides universal definitions and
Arnold-Chiari II malformation is a standards for thickened liquids and diet
Problems sucking and swallowing
hindbrain herniation of the brainstem and types for texture modifications.
Difficulty positioning for feedings cerebellum present in most children
(98%) with MMC. This malformation Gastroesophageal Reflux Disease
Respiratory symptoms, stridor
affects coordination of muscles involved A child with MMC who has difficulty
Difficulty forming seal on nipple in the suck, swallow, and breathe patterns with feeding and meeting his or her
in infants and can result in neurogenic growth needs should be assessed for
Refusal of cup/sippie cup dysphagia. Identifying dysphagia and gastroesophageal reflux. Common
Loss of food from mouth providing intervention when dysphagia symptoms reported by parents in clinical
symptoms manifest (Table 4) is crucial in experience include difficult feedings, food
Nasal regurgitation children with MMC. Mathisen and refusal, emesis, stridor, grimacing,
Shepherd53 found that hypotonicity along irritability, posturing, reactive airway
Long feeding times
with the Arnold-Chiari II malformation disease, and difficulty gaining weight/
High number of formula changes put infants with MMC at a higher risk for weight loss. Although there is not a
to improve formula tolerance feeding issues. Feeding issues/delays diagnostic test considered to be a “gold
resulted in low iron status, low protein standard” by the North American Society
Perceived lack of satiety levels, and energy intake that met 72% of for Pediatric Gastroenterology,
recommended needs and ultimately Hepatology, and Nutrition (NASPGHAN)
Refusal to advance textures/
impaired growth in this study. Severe and European Society for Pediatric
increase variety
cases of Arnold-Chiari II malformation Gastroenterology, Hepatology, and
Delays in self-feeding can be treated with decompression Nutrition (ESPGHAN), there are several
surgery that may improve dysphagia diagnostic tests commonly used in the
Choking with feedings symptoms; however, recurrence of diagnosis of gastroesophageal reflux
Poor salivary control dysphagia symptoms is common.54,55 disease (GERD). Although an upper
Dysphagia can be evaluated and treated gastrointestinal (GI) series is frequently
Aspiration with a comprehensive, interdisciplinary used to diagnose GERD, it may not
feeding team. Difficulties in the oral always be evident during the short testing
Weight loss/growth failure
phase, initiation of swallow, and/or period. This test is useful to identify
pharyngeal phase of feeding characterize structural abnormalities, such as

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vol. 2 • no. 4 ICAN: Infant, Child, & Adolescent Nutrition

obstruction, malrotation, stenosis, intake, it may be helpful to send a water basis.65 There is a paucity of literature on
esophageal strictures, or hiatal hernia in bottle and physician’s orders to school to wound healing in children. Most literature
children with special needs and/or spinal ensure the child is able to drink during on wound healing in adults cites that
abnormalities. Upper GI endoscopy can the school day. Some families find it adequate energy intake should be ensured
show reflux esophagitis, although helpful to offer their child fluids after to allow protein sparing for wound
additional studies may need to be routine bladder catheterization. healing. Although obese adult patients
performed to rule out other cause of Fiber goals are typically calculated using have demonstrated slower wound
esophagitis. A 24-hour pH probe can the “age + 5 = grams of fiber” rule of healing,66 weight loss is not recommended
measure pH in the stomach over a thumb.64 The DRI for fiber intake in when trying to heal a wound. The focus
24-hour time period and can provide children is currently 19 to 38 grams daily42 should be strictly on providing sufficient
important diagnostic information when and is not realistic for most children with nutrition to promote wound healing.
combined with caregiver or family report MMC because of a noted lack of high- Adequate protein should be provided to
of GERD symptoms.59 fiber foods in their food preferences ensure positive nitrogen balance during
Treatment of GERD in infants typically commonly seen in clinical experience. If wound healing.67,68 Clinical practice in
focuses on nonpharmacologic methods, dietary changes are not sufficient to children with MMC has shown good
such as thickened feedings, positioning increase fiber intake, then a fiber results with increasing protein intake to
changes, hydrolysate formula, and supplement may be needed in the 2 to 3 g/kg/d with careful monitoring of
increasing caloric density of the diet.59 regimen. Fiber intake should not be serum urea nitrogen and creatinine levels.
The latest pediatric GERD Clinical significantly increased unless fluid intake Pompeo69 has suggested using a PUSH
Practice Guidelines published in 2009 is sufficient, as this may worsen constipa- (pressure ulcer scale for healing) score to
suggested insufficient evidence for tion. Medical management of constipation determine the amount of protein to
elimination of GERD-provoking foods in is often necessary to ensure regular provide for adult wound patients, which
children and adolescents with GERD and evacuations and to preserve kidney, may be adapted for pediatric use, as
recommended lifestyle changes, such as bladder, and bowel health. Leibold et al61 shown in Table 5. Jaksic70 has published
weight loss and positioning changes,
along with drug therapy that may assist
in GERD management. In severe cases
of GERD, a fundoplication may be
“Early and frequent contact with the dietitian using
necessary.59 a family-centered approach can help children
Bowel Management with MMC and their families maximize wellness,
Neurogenic bowel, dysmotility, and
medications are common factors in
fitness, and independence.”
children with MMC that predispose them
to constipation that can result in multiple
complications unless proactive steps are have presented an excellent interdisciplin- recommendations for protein intake for
taken for bowel management. ary model for formulating and monitoring critically ill children (included in Table 5)
Constipation can affect gastric emptying, a bowel management program in children that can be used for wound healing in
bladder health, and kidney health. The with MMC that considers multiple variables, children. Ideal body weight should be
hypomotility noted in persons with MMC60 including the child’s diet, learning issues, used if the child is overweight or
increases colonic transit time and often and family routines. This model reiterates underweight, whereas actual weight
necessitates a comprehensive program to the importance of adequate fluid and fiber should be used if the child is 90% to
address bowel continence. Bowel and intake, along with daily bowel movements, 125% ideal body weight.
bladder continence should be a goal for as a first step in achieving continence.61 All children with MMC who have a
every child with MMC to reduce social Starting a bowel continence program at an wound should be on a multivitamin with
stigma and the risk of skin breakdown.61-63 early age helps children with MMC iron; other individual nutrients, such as
A nutritional assessment to evaluate the maximize continence success.61,62 zinc, should be supplemented when
child’s diet, with particular attention to deficiency is evident.66 Zinc has an
fluid and fiber intake, is often the first Wounds established role in wound healing and
step in bowel management.61 Then Children with MMC are at a high risk for immunity as a cofactor for multiple
recommendations can be made to skin breakdown and wounds related to enzymes. Albumin is a zinc carrier
increase fluid and fiber intake as needed. multiple risk factors, primarily insensate through the body; therefore, when
Fluid goals should primarily be based on skin and obesity. Children with MMC and albumin levels are low, zinc absorption is
body weight and adjusted as needed. If their families should be educated on how reduced and zinc deficiency is probable.
children with MMC have inadequate fluid to assess skin for breakdown on a routine Plasma zinc levels should be evaluated as

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ICAN: Infant, Child, & Adolescent Nutrition August 2010

Table 5. Table 6.
Protein Recommendations for Wounds Foods With a High Food Allergy Risk
for Children Who Are Latex Allergic/
Based on PUSH (Pressure Ulcer Scale for Healing) Score69 Sensitive

PUSH Score Protein Needs, g/kg/d Bananas


0-15 1.4-1.6 Kiwi
16-30 1.6-2.0 Figs
>30 2.0-2.4 Plums

Avocados
Critically Injured Pediatric Patients 70
Passion fruit
Age of Child Protein Needs, g/kg/d Peaches
0-2 y 2.0-3.0 Tomatoes
2-13 y 1.5-2.0 Chestnuts
Adolescents 1.5 Papaya
From Jaksic T. Effective and efficient nutritional support for the injured child. Surg Clin North Am. Nectarines
2002;82:279-391. Reprinted with permission from Elsevier.
Celery

part of a nutritional assessment, and exposures to latex.75 Children with


supplementation should be initiated using latex allergy/sensitivity are at risk of
Table 7.
the DRI as a reference.71 When zinc levels the following food allergies: banana,
normalize, supplementation should be avocado, and kiwi (see Table 6 for a Learning Differences in Children With
discontinued, as supplemental zinc can more comprehensive list of high-risk Myelomeningocele78
impair copper and iron absorption.66,72 foods). When solid foods are introduced
Iron also plays an important role in to the diet of an infant with MMC, Perceptual motor problems
collagen synthesis66 and is routinely parents should be reminded to monitor
supplemented during wound healing for signs and symptoms of food Comprehension
when iron deficiency anemia is evident. allergies, particularly if the child is latex Attention
Vitamin A is involved in skin integrity allergic/sensitive. Children with MMC
and immunity. When vitamin A deficiency who are latex allergic/sensitive should Sequencing
is evident, supplementation should be avoid latex in the home and commu-
Hyperactivity/
given according to the DRI for age.73 nity.76 However, they do not need to
impulsivity
Vitamin C, a water-soluble vitamin, acts as avoid foods containing some of the
a cofactor with iron during collagen same proteins as latex unless they Memory
synthesis via proline and lysine hydroxyl- demonstrate allergy symptoms with
ation. Supplementation to correct the these foods. Organization
deficiency should be the DRI for age,74
although 100 to 500 mg/d is typically Learning Differences in
provided for 7 to 10 days with a docu- Children With MMC
mented vitamin C deficiency and then Children with MMC and shunted abstract concepts (such as direction,
reduced to the DRI for age.66,68 hydrocephalus often demonstrate position) and using appropriate
learning differences that should be language in social situations. They
Latex Allergy considered when providing nutrition recommended using visual aids, short
Children with MMC have an increased education, as shown in Table 7.77 Vachha commands and sentences, and multiple
risk of latex allergy, which appears to and Adams78 found that children with opportunities for repetition and practice
be associated with early and repeated MMC have difficulty understanding to enhance understanding in children

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vol. 2 • no. 4 ICAN: Infant, Child, & Adolescent Nutrition

with MMC. For example, providing a and other neural tube defects, 1983-1991. 17. Adams RC, Keefover-Hicks A, Browne R,
handout with a pictorial rendering of the MMWR. 1991;40:513-516. Hardin DS. Growth curves for children
  5. Centers for Disease Control. with myelomeningocele using longitudi-
number of cups of fluid that need to be nal data. Dev Med Child Neurol.
Recommendations for the use of folic acid
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of his or her fluid intake by allowing bifida and other neural tube defects. 18. Duval-Beaupére G, Kaci M, Lougovoy J,
him or her to check off each cup of MMWR. 1992;41(RR-14):1-7. Caponi MF, Touzeau C. Growth of trunk
fluid he or she drinks. Dietitians should   6. Food standards: amendment of standards and legs of children with Myelomeningo-
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  8. World Health Organization. Towards a Does growth hormone (GH) enhance
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