A Review of Nutrition in Duchenne Muscular Dystrophy

REVIEW

A review of nutrition in Duchenne muscular dystrophyZ. E. Davidson & H. Truby

The University of Queensland, Children’s Nutrition Research Centre, School of Medicine, Queensland, Australia

Background

Duchenne muscular dystrophy (DMD) is a recessive X

linked genetic disorder affecting approximately one in

3500 live male births. In DMD, the body is unable to

make the protein dystrophin as a result of a large variety

of mutations/deletions of the dystrophin gene, found on

chromosome Xp21 (Strober, 2000). Dystrophin is essen-

tial for muscle contraction because it binds actin to the

membrane bound dystroglycan complex in smooth, car-

diac and skeletal muscle. The absence of dystrophin

expression leads to progressive muscle weakness, with

chronic degeneration of muscle and replacement of

muscle with fat and endomysial fibrosis. DMD follows a

predictable clinical course because the deficiency of

dystrophin and inflammatory response causes muscle

inflammation, necrosis and fibrosis. Characteristic

progressive muscle wasting becomes noticeable in early

childhood and, by their early teens, boys have become

wheelchair bound. Premature death in their early twenties

usually results from failure of the respiratory or cardiac

musculature.

Boys with DMD present in early childhood with

delayed walking, increased falls, decreased activity, diffi-

culty climbing stairs or getting up off the floor. Unique

identifiers of DMD include ‘Gower’s sign’, whereby a boy

Keywords

Duchenne, evidence-based dietary treatment,

muscular dystrophy, nutrition therapy,

paediatric dietetics.

Correspondence

Z. Davidson, Department of Paediatrics and

Child Health, Children’s Nutrition Research

Centre, Royal Children’s Hospital, Herston,

Queensland, Australia.

Tel.: +61 (7) 3636 9294

Fax: +61 (7) 3346 4684

E-mail: [email protected]

doi:10.1111/j.1365-277X.2009.00979.x

Abstract

Duchenne muscular dystrophy (DMD) is a recessive X linked genetic disorder

characterised by progressive muscle weakness and reduced muscle tone. Affect-

ing only boys, it limits life expectancy to approximately 20 years. A literature

review was conducted using MEDLINE and the Cochrane Library, employing

the term ‘Duchenne muscular dystrophy’. A total of 1491 articles in English

were recovered. These papers were searched thematically under the headings:

body composition (n = 10), energy expenditure (n = 10), nutrition (n = 6),

corticosteroid therapy (n = 55) and gene therapy (n = 199). Key dietetic prac-

tice points were identified relevant to nutritional management. Papers support-

ing these key themes were assigned a level of evidence and grade of

recommendation. There is limited high-quality evidence to guide the nutri-

tional management of boys with DMD. Currently, the majority of evidence is

based on expert opinion and clinical expertise. Delayed growth, short stature,

muscle wasting and increased fat mass are characteristics of DMD and impact

on nutritional status and energy requirements. The early introduction of

steroids has altered the natural history of the disease, but can exacerbate weight

gain in a population already susceptible to obesity. Prior to commencing

steroids, anticipatory guidance for weight management should be provided.

Malnutrition is a feature of end stage disease requiring a multidisciplinary

approach, such as texture modification and supplemental feeding. Micronutri-

ent requirements are yet to be determined but, as a result of corticosteroid

treatment, vitamin D and calcium should be supplemented. Some evidence

exists supporting supplementation with creatine monohydrate to improve mus-

cle strength. More research is needed to provide a higher quality of evidence

for dietitians working within this area.

Journal of Human Nutrition and Dietetics

ª 2009 The Authors. Journal compilation.

ª 2009 The British Dietetic Association Ltd 2009 J Hum Nutr Diet, 22, pp. 383–393 383

will use his hands to ‘walk himself up’, and pseudohyper-

trophy of the calf muscles, which is indicative of fibrous

infiltration of the muscle. Diagnosis can be confirmed by

elevated serum creatine kinase, muscle biopsy or genetic

analysis. A range of other complicating features can

occur, such as learning disabilities and gastrointestinal

symptoms (see Supporting Information, Table S1). Medi-

cal management of the muscular dystrophies has included

the use of corticosteroids; however, their precise mode of

action is unclear, as is the optimal time to commence

treatment. The standard initial therapeutic dose is

0.75 mg kg)1day)1 for prednisone and 0.9 mg kg)1 day)1

for deflazacort (Moxley et al., 2005) and this is now

accepted as best practice. Boys treated with steroids have

improved functional capacity (Yilmaz et al., 2004), less

scoliosis (Balaban et al., 2005) and better pulmonary

function than those who are not treated with steroids

(Pradhan et al., 2006; Daftary et al., 2007). Side effects

such as excessive weight gain, cushingoid features, hirtsu-

ism, glucose intolerance, cataracts, behavioural changes

and delayed puberty (Sorva & Turpeinen, 1994) can be of

sufficient concern to cause their withdrawal (Balaban

et al., 2005; Strober, 2006).

Daily physiotherapy combined with multidisciplinary

treatment aims to reduce complications such as the devel-

opment of contractures and scoliosis.

The body composition changes observed in DMD are

unique, and hence nutritional management is complex.

Boys with DMD can move between the spectrum of over

to under nutrition within their shortened lifespan. Nutri-

tional issues have taken a backseat in the past; however,

as the life expectancy slowly inches forward with

improvements in medical management, such concerns

warrant immediate attention. There is no consensus avail-

able on the nutritional management, and so dietitians are

forced to tread lightly through a potential minefield of

issues. Here, a summary of the literature is conducted in

order to provide some much needed clinical guidance for

dietitians.

Methods

A literature search was conducted in MEDLINE (Ovid,

from January 1950 to March 2008) and the Cochrane

Library using the term ‘Duchenne muscular dystrophy’.

Nutritional textbooks or chapters on management of neu-

romuscular disease (NMD) were examined for content

and references. A total of 1690 articles were recovered,

and only articles in English were included in this review

(n = 1491). These papers were searched thematically

under the headings: body composition (n = 10), energy

expenditure (n = 10), nutrition (n = 6), corticosteroid

therapy (n = 55) and gene therapy (n = 199). Reference

Box 1Nutrition requirements

l Monitor weight to guide energy prescription

(Grade D)l Ensure adequate intake of micronutrients as per

dietary reference values (Grade D)l Supplement Vitamin D (1000 IU daily) and Cal-

cium (750 mg daily), especially in those receiving ste-

roid therapy (Grade D)l Monitor serum 25-hydroxyvitamin D (Grade D)

Nutrition assessment and monitoring Shortstature is a characteristic of boys with DMD(Grade B)

l Measure height and weight six monthly and plot

on standard growth charts (Grade D)l Upper arm length, tibial length, or knee height can

be measured in the advanced stage of disease (Grade D)l Body composition is characterised by a decreased

lean body mass and increased intramuscular fat mass

(Grade C)l BMI as a screen for obesity is not accurate in boys

with DMD (Grade C)l Various tools can be used to measure body compo-

sition with DXA and MRI being accurate, appropriate

and noninvasive measurement instruments (Grade C)

Management

l Prevent excess weight gain by providing anticipa-

tory guidance around energy balance prior to com-

mencement of steroid therapy (Grade D)l Advise reduction in energy intake with caution.

Follow up with regular measurement of fat free mass

to monitor progress where available (Grade D)l Dietary texture modifications may be required to

accommodate eating difficulties (Grade D)l Support patients and families in decisions regard-

ing enteral feeding and/or gastrostomy tube placement

(Grade D)l A multidisciplinary team should be involved in the

management of feeding difficulties (Grade D)

Nutriceuticals

l There is a limited evidence base to support the use

of nutriceuticals in boys with DMD (Grade D)l Short- and medium-term creatine monohydrate

treatment improves muscle strength in people with

NMD (Cochrane)

Nutrition in Duchenne muscular dystrophy Z. E. Davidson and H. Truby


384 ª 2009 The British Dietetic Association Ltd 2009 J Hum Nutr Diet, 22, pp. 383–393

lists were examined for papers that were not identified by

the search strategy. Where systematic or Cochrane reviews

were available, these were utilised in preference to indi-

vidual papers.

Key themes were identified from all relevant literature.

Practice points were agreed between the authors as the

most helpful to practitioners, and papers relevant to these

key themes were assigned a level of evidence and grade of

recommendation using the Oxford Centre for Evidence-

based Medicine criteria (Phillips et al., 1998). These

points are summarised in Box 1 with the grade of recom-

mendation, with a scale of A being the strongest through

to D being the lowest quality of evidence (see Supporting

Information, Table S2). A summary of the search process

is provided in Fig 1.

Nutritional requirements

Energy requirements

It is unclear whether decreased energy expenditure, either

from reduced voluntary activity, or a primary defect in

energy metabolism are contributing factors to obesity in

DMD. Studies within this area are limited with contradic-

tory findings. The six studies published (Gonzalez-Berme-

jo et al., 2005; Zanardi et al., 2003; Satomura et al., 2001;

Hankard et al., 1996a,b, Okada et al., 1992, Berardinelli

et al., 2001) have limitations: small sample sizes and a

large age range. Only three of these studies examined

energy requirements in younger children and it is unclear

whether the participants were receiving steroid therapy.

Energy requirements become complicated as the DMD

boys become older. In particular, resting energy expendi-

ture (REE) can be affected when gas exchange is impaired

(Bodamer et al., 1997), secondary to a decrease in respira-

tory muscle strength. The literature is inconclusive with

respect to energy requirements in the advanced stages of

disease. Okada et al. (1992) measured basal metabolic rate

(BMR) in 310 individuals with DMD aged 11–29 years.

DMD subjects had an increased BMR compared to con-

trols, and BMR increased with age. Gonzalez-Bermejo

et al. (2005) measured REE in 20 males aged 20–33 years

on mechanical ventilation. REE was significantly lower in

DMD participants compared to controls. The difference

in REE findings could be the result of the presence of

ventilation support in the latter study. Gonzalez-Bermejo

et al. (2005) also found that measured REE was 22%

lower than the theoretical REE calculated using the

Harris Benedict equation. Dietitians need to make an

individualised ‘best guess’ to establish energy require-

ments using the equation: total energy expenditure = REE

(measured if possible) · physical activity level

(Department of Health, 1991). Individual longitudinal

monitoring of body weight will provide a crude

indication on the accuracy of energy prescription

(Almond et al., 2007).

Micronutrient requirements

There has been little investigation into the micronutrient

status and requirements of boys with DMD. In the advent

of corticosteroid therapy, the micronutrients that require

immediate attention are vitamin D and calcium. Canalis

et al. (2004) describes how pharmacological doses of oral

glucocorticosteriods can suppress bone formation and

increase bone resorption, thereby leading to osteoporosis.

The use of steroid therapy combined with decreased

mobility suggests that boys with DMD are at an increased

risk of fractures and poor bone health (Soderpalm et al.,

Medline and Cochrane library search “duchenne muscular dystrophy” = 1690 articles

English articles = 1491 articles

Body composition (10)

Energy expenditure (10)

Nutrition (6)

Corticosteroid therapy(55)

Gene therapy (199)

Reference lists searched

Themes identified

Supporting papers given level of evidence 1–5

Key practice points graded A–D

Figure 1 Literature search method.

Z. E. Davidson and H. Truby Nutrition in Duchenne muscular dystrophy



2007). Serum 25-hydroxyvitamin D [25(OH) D] was

examined in 24 boys with DMD aged 2.3–19.7 years, with

the majority being treated with prednisolone, and in 24

controls (Soderpalm et al., 2007). Significantly lower

levels of 25(OH) D were found in the DMD

group (21 ± 6 lg L)1) compared to controls (30 ±

8 lg L)1), as well as a lower bone mineral density and

decreased bone turnover. Interestingly, food records

revealed an apparent adequate intake of dietary calcium

and vitamin D, implying either that requirements are

increased or there is another mechanism that predisposes

the boys with DMD to vitamin D insufficiency. Vitamin

D metabolism is an area which would benefit from future

research. Biggar et al. (2006) recommends daily calcium

(750 mg) and vitamin D (1000 IU) supplementation in

conjunction with corticosteroid therapy.

Nutritional assessment and monitoring

Height

Short stature in boys with DMD has been documented

but its aetiology is unclear. Eiholzer et al. (1988), Nagel

et al. (1999) and Willig et al. (1993) determined mean

the z-scores for height for their populations as )1.5, )1.0

and )0.936, respectively. McDonald et al. (1995) demon-

strated that reduced stature remains a feature into adult-

hood. Biggar et al. (2006) demonstrated that boys treated

with deflazacort were significantly shorter than boys who

were not treated at age 10, 15 and 18 years. Interactions

of steroids and growth require long-term follow up.

Although standing height is the preferable measure-

ment, accurate measurements of height become difficult

to obtain on approaching adolescence because of an

inability to stand combined with the development of sco-

liosis and/or contractures. Stewart et al. (2006) suggested

measuring upper arm length, tibial length or knee height

in these circumstances.

Weight

In steroid naıve boys, weight is disproportionately distrib-

uted throughout the stages of DMD. McDonald et al.

(1995) reported that 44% of boys aged 9–13 years were

above the 90th percentile in weight, whereas, after age

17 years, 65% weighed less than the 10th percentile. Simi-

lar findings were presented by Willig et al. (1993). Of the

252 boys studied, the prevalence of obesity, defined as

having a weight above the 90th percentile, reached 54%

by age 13 years. Undernutrition (i.e. a weight below the

10th percentile) occurred after age 14 years, involving

54% of the boys by age 18 years. The interaction between

weight and steroid treatment is significant, and requires

longitudinal follow up.

Body composition

Body composition is characterised by a decreased lean

body mass and increased intramuscular fat mass (Leroy-

Willig et al., 1997). Therefore, standard anthropometric

measures (i.e. skinfold thickness) are not accurate (Leroy-

Willig et al., 1997; Pichiecchio et al., 2002; Mok et al.,

2006a). Various body composition measures have been

documented, including dual energy X-ray absorptiometry

(DXA) (Palmieri et al., 1996; Douvillez et al., 2005;

Soderpalm et al., 2007), bioelectrical impedance (Hankard

et al., 1996a; Gonzalez-Bermejo et al., 2005; McDonald

et al., 2005; Mok et al., 2006a), total body potassium

(Griggs et al., 1983; Edmonds et al., 1985), magnetic reso-

nance imaging (MRI) (Huang et al., 1994; Leroy-Willig

et al., 1997; Gong et al., 2000; Berardinelli et al., 2001;

Pichiecchio et al., 2002; Zanardi et al., 2003) and urinary

creatinine excretion (Griggs et al., 1983; Edmonds et al.,

1985; Hankard et al., 1996a; Franciotta et al., 2003).

Because different methodologies and outcome measures

were utilised in these studies, a meta-analysis of the out-

come data was not possible. However, the studies demon-

strate that DXA and MRI are accurate and noninvasive

measures that would be appropriate for monitoring nutri-

tional status in a paediatric population.

Classifying boys with DMD as being overweight or

underweight by using height and weight or body mass

index (BMI) without accurate body composition mea-

sures is problematic. Pessolano et al. (2003) cautioned

against using BMI for the evaluation of nutritional status

in boys with DMD. The researchers compared the num-

ber of boys classified as being overweight using BMI

(n = 5) with the number of boys classified as being over-

weight using the percentage of expected weight for zero

muscle mass (n = 30).

Stewart et al. (2006) suggest that height and weight

should be measured at a minimum of every 6 months

and plotted on growth charts. Standard growth charts,

such as the ‘UK 90’ or the ‘CDC 2000’, can be used in

clinical practice to monitor longitudinal growth and

development in NMD (Almond et al., 2007). In 1988, a

specific DMD weight chart was created (Griffiths and

Edwards). This chart is a theoretical model that accounts

for weight growth in the presence of muscle wasting.

When comparing this chart with the CDC 2000 chart, the

percentiles sit lower, taking into account the loss of

muscle mass.

Management

Obesity

Excess weight gain occurs in the early stages of the dis-

ease, with a dramatic inflexion in the observed weight




90th percentile line from 7 years of age (Willig et al.,

1993). Obesity contributes to the progression of the dis-

ease by exerting extra force on already weak muscle

groups, essentially decreasing mobility. In an examination

of body composition of 13 males aged 9–13 years with

DMD, Hankard et al. (1996a) split the group into obese

DMD and nonobese DMD, sensu Griffiths & Edwards

(1988). Their analysis showed that there was no difference

in muscle mass between obese and nonobese participants,

suggesting that obesity should be considered only as a

factor increasing the handicap. Obesity also has implica-

tions for increased respiratory involvement and poorer

psychosocial development, and can make activities of

daily living and care at home more difficult.

Excess weight gain is exacerbated in boys treated with

steroids because increased appetite contributes to an over

consumption of energy (Desilva et al., 1987) and the

most frequently reported side effect (Angelini, 2007).

Obesity is a major concern for parents/caregivers. A sys-

tematic review conducted by Moxley et al. (2005) deter-

mined that weight gain occurred over a prednisone dose

range of 0.3–1.5 mg kg)1day)1. In those individuals trea-

ted for 6 months, 75–80% of patients receiving

0.75 mg kg)1day)1 prednisone increased their weight by

‡ 10% compared to 20–24% of those receiving placebo.

Despite this weight gain, patients showed clinical

improvement. Interestingly, the distribution of the weight

gained, in relation to muscle and fat, differed between

patients receiving prednisone compared to placebo. At

the completion of the 18-month prednisone trial, a 36%

increase in muscle mass in the 0.75 mg kg)1day)1 predni-

sone group compared to placebo-treated patients was

demonstrated using urinary creatinine excretion. These

findings challenge health professionals to re-consider the

definition of ‘excessive weight gain’ and to consider the

need for measurement of body composition as a routine

part of nutrition assessment and monitoring.

There is debate about whether the type of steroid

affects the severity of induced side effects. Bonifati et al.

(2000) and Balaban et al. (2005) found that deflazacort

was successful in reducing the amount of weight gained

compared to prednisone.

Standard dietetic practice for weight management is to

manipulate the energy balance equation by decreasing the

intake of food energy, and by increasing activity to

increase total energy expenditure. This theory of ‘eat less,

do more’ should not be applied to boys with DMD.

Although obesity raises considerable parental concern,

caution is needed when inducing a negative energy bal-

ance because this may increase the loss of lean body mass,

which does not have the potential to regenerate. In adults

on various energy restricted diets, it has been shown that

weight loss comprises approximately 70% fat mass and

30% lean mass (Truby et al., 2006). There is only one rel-

atively old case study of successful weight loss in a boy

with DMD and another with Becker’s muscular dystrophy

(Edwards et al., 1984). These case studies demonstrated

that weight loss was possible and led to an increase in

mobility and self esteem without deleterious effects on

total body nitrogen (i.e. a surrogate measure of lean

mass). However, the authors cautioned that weight loss

should be carefully monitored because of the potential

loss of lean tissue that usually results during periods of

negative energy balance. To achieve this weight loss in the

DMD participant, energy was restricted to 2633 kJ day)1

(54 g protein, 30 g fat, 40 g carbohydrate) from January

to December 1981, raising questions about micronutrient

adequacy and patient satisfaction.

Although estimating energy requirements and macro-

nutrient composition are uncharted territory, so too is

the issue of activity prescription. There is a risk of

increased muscle breakdown from overuse versus

increased muscle weakness from disuse (McDonald,

2002). Grange & Call (2007) discuss how muscle weak-

ness may be attenuated by regular, low intensity exercise;

however, there is a critical lack of data to support exercise

prescription. The authors recommend a systematic analy-

sis of muscle function to determine potential exercise

thresholds. In 2005, Carter et al. (2005) reported anec-

dotal evidence of weight loss in two boys with DMD

using the anti-epileptic drug, Topiramate (Topamax;

Janssen-Cilag, Titusville, NJ, USA). The patients lost 26%

and 41% of their initial body weight. The authors

concluded that further study investigating the use of

pharmaceutical weight loss agents is warranted.

The most constructive advice in the management of

overweight boys with DMD is given by Griffiths &

Edwards (1988): prevention of excess weight gain is pref-

erable to severe restriction in the already obese. The mod-

ern manifestation of this advice would be to provide

anticipatory guidance to boys commencing corticoster-

oids, and to follow up with regular monitoring of growth,

in particular with respect to weight gain. However, in the

already obese child, the dietitian does need to consider

the devastating effects of excess weight on mobility versus

the potential for muscle loss associated with inducing

negative energy balance.

Malnutrition

The spectrum of over nutrition to under nutrition occurs

with disease progression. As boys with DMD age, the

likelihood that they will become underweight is increased.

Significant muscle wasting will result in weight loss. This

is accentuated by eating difficulties experienced at meal

times likely leading to a reduced food intake. Iannaccone




et al. (2003) investigated malnutrition after surgical pro-

cedures and found weight loss after surgery to be associ-

ated with loss of the ability to self feed. Some possible

explanations suggested for this were: a decrease in neck

range of motion, or head control; lack of adequate care

giving; and low cognitive function. Intervention to

increase energy intake resulted in weight gain. Pane et al.

(2006) reported on feeding problems experienced by indi-

viduals with DMD. These included difficulty in opening

their mouth and chewing leading to an increase in meal

duration, with episodic choking also reported. In a simi-

lar survey, Willig et al. (1994) found the most prominent

feeding difficulty was getting food to the mouth.

Food texture modification is an important strategy to

manage feeding difficulties. Modifications include cutting

food into smaller pieces and changing the texture to

smooth foods (Pane et al., 2006). A multidisciplinary

team should be involved in the management of feeding

difficulties (Stewart et al., 2006; Almond et al., 2007) with

individual preference being paramount. Gonzalez-Bermejo

et al. (2005) analysed dietary intake in an older group of

boys (aged 22–33 years) reporting that, although they ate

slowly and with difficulty, these inconveniences were lar-

gely compensated for by the pleasure of eating a normal

meal.

In the literature, the incidence of gastrostomy feeding

tubes in older boys with DMD ranges from nil (Gonz-

alez-Bermejo et al., 2005) to 50% (Hilton et al., 1993).

Indications include difficulty in swallowing leading to

aspiration, and undernutrition (Bushby et al., 2005). As

with other conditions, multiple issues should be consid-

ered before gastrostomy placement can occur. At the stage

of disease when alternate feeding options need to be con-

sidered, such patients have severe respiratory insuffi-

ciency, putting them at high risk of major complications

when they are anaesthetised or sedated for such a proce-

dure (Birnkrant et al., 2006). Despite these risks, Birnk-

rant et al. (2006) detailed successful gastrostomy

placements in a 22- and 19-year-old male. The authors

encourage physicians to consider whether providing

enteral nutrition support should be proactively planned

at earlier stages of the disease process, before the risks

associated with anaesthesia in those with compromised

respiratory capacity.

In the past, gastrostomy placement in the DMD popu-

lation was rare (Pane et al., 2006), most likely as a result

of ethical issues surrounding this procedure. Hilton et al.

(1993) presents an excellent discussion on such ethical

considerations associated with advanced stages of DMD.

‘The ‘‘can do’’ versus the ‘‘should do’’ … the ability to

act does not justify the action’ (Hilton et al., 1993). In

the face of such confronting ethical decisions in medicine,

and in this case nutrition and dietetics, the authors sug-

gest four considerations to professionals: medical indica-

tions, patient preference, quality of life, and contextual

features (i.e. social and medical support). No studies have

specifically examined the effect of gastrostomy feeding in

DMD on quality of life, although the nutritional benefits

of enteral feeding are demonstrated by Goldstein

et al.(1989).

Nutriceuticals

There are four randomised controlled trials, summarised

in a recent Cochrane review (Kley et al., 2007), providing

high-quality evidence for the use of dietary supplements

to enhance muscle function. Because many families seek

alternative or naturopathic supplements, dietitians need

to be aware of their potential nutritional effects or inter-

actions. The most promising of these is creatine monohy-

drate. Creatine is an amino acid found in muscle that

stores energy in the form of phosphocreatine for immedi-

ate use (Strober, 2006). Several studies have supported

the use of creatine for increasing athletic performance

and strength in healthy populations (Grande & Graves,

2005). A recent Cochrane review (Kley et al., 2007) con-

cluded that the available evidence from randomised trials

shows that short- and medium-term creatine treatment

improves muscle strength in people with NMD, and is

well tolerated. In 138 participants with NMD treated with

creatine, there was a significant increase in maximum vol-

untary contraction in the creatine group compared to

placebo, with a weighted mean difference of 8.47% (95%

confidence interval = 3.55–13.38). There was also an

increase in lean body mass during creatine treatment

compared to placebo (weighted mean difference 0.63 kg;

95% confidence interval = 0.02–1.25). The supplement

doses of the included trials were 5 g day)1 (Escolar et al.,

2005), 3 g day)1 (Louis et al., 2003), 100 mg kg)1day)1

(Tarnopolsky et al., 2004) and 5 g day)1 for children, and

10 g day)1 for adults (Walter et al., 2000). Another inter-

esting finding from these studies was the effect on bone

health in boys with NMD. Creatine treatment increased

bone mineral density by 3% (Louis et al., 2003), and

reduced levels of N-telopeptides, a marker of bone degra-

dation (Tarnopolsky et al., 2004). Although these results

are statistically significant, the clinical relevance should be

considered.

Of the studies included in the Cochrane review, all bar

one (Tarnopolsky et al., 2004) was conducted in steroid

naıve dystrophic males. The use of complementary thera-

pies could possibly increase the positive effect on muscle

function. Using the mdx mouse model, Payne et al.

(2006) tested several compounds (creatine mono-

hydrate, conjugated linoleic acid, a-lipoic acid and

b-hydroxy-b-methylbutyrate) in combination therapy with




prednisolone, which provided the most consistent evidence

of efficacy.

Glutamine has also been studied. Despite promising

beginnings in nondystrophic humans (Hankard et al.,

1996b, Darmaun et al., 1998) and in dystrophic males

(Hankard et al., 1998, 1999; Mok et al., 2006b), a rando-

mised controlled trial (Escolar et al., 2005) was unable to

demonstrate a statistically significant effect of glutamine

supplementation based on manual and quantitative mea-

surements of muscle strength. The authors did conclude,

however, that a disease-modifying effect of glutamine in

younger dystrophic males cannot be excluded.

l-arginine (Chaubourt et al., 1999; Voisin et al., 2005)

and green tea extract (Buetler et al., 2002; Dorchies et al.,

2006) have also been investigated with some success in

the mdx mouse model; however, these therapies are yet

to move from ‘bench level’.

Future developments

The multidisciplinary management of boys with DMD

has progressed significantly and now involves the use of

steroid therapy, ventilator support, spinal management

and improved therapies such as physiotherapy. As a

result, the life expectancy of boys with DMD is steadily

increasing. Eagle et al. (2002) concluded that the chances

of survival to 25 years have increased from 0% in the

1960s to 53% in the 1990s; and this was before steroid

treatment. Data on the longer-term effects of steroids are

beginning to emerge (Balaban et al., 2005; Biggar et al.,

2006; Angelini, 2007), although it would appear overall

that the body of evidence is still catching up with the

now regular use of steroids in clinical practice. Further

research is required to remap the natural history of DMD

in steroid-treated boys. With an older DMD population

emerging, it is unclear what nutritional issues will emerge

in the adulthood life of DMD males. With increased body

fat mass and an inability to exercise, are these young men

at risk of developing a cluster of cardiovascular risk fac-

tors, such as the metabolic syndrome? Aitkens et al.

(2005) and Kilmer & Zhao (2005) suggest that this might

happen, which will provide a new level of complexity to

dietetic management.

Future research avenues

The dystrophin gene is the largest, spanning approxi-

mately 2.5 Mb of genomic sequence, and is composed of

79 exons (Blake et al., 2002); hence, gene therapy has

been hampered by the complexity, size and sheer number

of mutations. Phase III clinical trials are ongoing with

PTC124, an orally administered drug that promotes ribo-

somal read-through of nonsense mutations. The treat-

ment will have limited reach because nonsense mutations

are one of the least common genotypes. Other potential

therapies include those that increase the uptake of utro-

phin, as well as antisense oligonucleotide-mediated exon

skipping to correct out-of-frame mutations and restore

truncated, yet functional dystrophins. Until a cure is

found, therapeutic and supportive interventions will assist

in preventing or delaying complications and preserving

quality of life.

Conclusion

Reviewing the evidence base for nutrition in DMD reveals

consistent limitations across all areas, with little high-

quality evidence to support the key practice points high-

lighted. Overall, there is little research into nutrition and

DMD. There are often no other completed studies to sup-

port findings in publications; small sample sizes are com-

mon because recruiting from such a sparse patient

population can be difficult, randomised controlled studies

are scarce, and countless projects remain at the bench

stage. For these reasons, many recommendations are

based on extrapolations from non DMD studies and

expert opinions. Such recommendations will inevitably

always be ranked as poor using evidence-based medicine

criteria. However, they are not without merit. Given the

obstacles that potential DMD researchers face, expert

opinion based on clinical expertise and fundamental

nutrition principles may comprise the strongest and most

accurate evidence available to assist this neglected group.

Acknowledgments

Helen Posselt from MontroseAccess, Brisbane, is thanked

for her advice on exercise prescription and future research

avenues.

Conflict of interests, source of funding andauthorship

The authors declare that they have no conflict of

interests.

ZED is in receipt of a University of Queensland Research

Scholarship. HT was a senior research fellow employed by

The University of Queensland at the time of submission.

ZED was responsible for the literature search, thematic

collation of papers, structure and writing of the article,

drafting, and submission and revision. HT was responsi-

ble for the conception of the article, and was heavily

involved in the drafting of the paper, from revising the

structure through to editing the multiple drafts and revi-

sions. Both authors critically reviewed the manuscript and

approved the final version submitted for publication.




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Supporting Information

Additional Supporting Information may be found in the

online version of this article:

Table S1. Other potential issues identified in males with

DMD.

Table S2. Key practice points and supporting evidence.

Please note: Wiley-Blackwell are not responsible for the

content or functionality of any supporting materials sup-

plied by the authors. Any queries (other than missing

material) should be directed to the corresponding author

for the article.




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A Review of Nutrition in Duchenne Muscular Dystrophy