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Dilated Cardiomyopathy Induced by Chronic Starvation and Selenium Deficiency

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Protein energy malnutrition (PEM) has been rarely documented as a cause of cardiovascular abnormalities, including dilated cardiomyopathy. Selenium is responsible for antioxidant defense mechanisms in cardiomyocytes, and its deficiency in the setting of PEM and disease related malnutrition (DRM) may lead to exacerbation of the dilated cardiomyopathy. We report a rare case of a fourteen-year-old boy who presented with symptoms of congestive heart failure due to DRM and PEM (secondary to chronic starvation) along with severe selenium deficiency. An initial echocardiogram showed severely depressed systolic function consistent with dilated cardiomyopathy. Aggressive nutritional support and replacement of selenium and congestive heart failure medications that included diuretics and ACE inhibitors with the addition of carvedilol led to normalization of the cardiac function within four weeks. He continues to have significant weight gain and is currently completely asymptomatic from a cardiovascular standpoint.
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Case Report
Dilated Cardiomyopathy Induced by Chronic Starvation and
Selenium Deficiency
Soham Dasgupta1and Ashraf M. Aly2
1Department of Pediatrics, University of Texas Medical Branch, Galveston, TX 77555, USA
2Department of Pediatric Cardiology, University of Texas Medical Branch, Galveston, TX 77555, USA
Correspondence should be addressed to Ashraf M. Aly; amaly@utmb.edu
Received  July ; Accepted  November 
Academic Editor: Bibhuti Das
Copyright ©  S. Dasgupta and A. M. Aly. is is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
Protein energy malnutrition (PEM) has been rarely documented as a cause of cardiovascular abnormalities, including dilated
cardiomyopathy. Selenium is responsible for antioxidant defense mechanisms in cardiomyocytes, and its deciency in the setting
of PEM and disease related malnutrition (DRM) may lead to exacerbation of the dilated cardiomyopathy. We report a rare case
of a fourteen-year-old boy who presented with symptoms of congestive heart failure due to DRM and PEM (secondary to chronic
starvation) along with severe selenium deciency.An initial echocardiogram showed severely depressed systolic function consistent
with dilated cardiomyopathy. Aggressive nutritional support and replacement of selenium and congestive heart failure medications
that included diuretics and ACE inhibitors with the addition of carvedilol led to normalization of the cardiac function within four
weeks. He continues to have signicant weight gain and is currently completely asymptomatic from a cardiovascular standpoint.
1. Introduction
e World Health Organization (WHO) denes malnutrition
as “the cellular imbalance between the supply of nutrients and
energy and the body’s demand for them to ensure growth,
maintenance, and specic functions [].” e term protein
energy malnutrition (PEM) applies to a group of related dis-
orders that include marasmus, kwashiorkor, and intermediate
states of marasmus-kwashiorkor. Marasmus involves inade-
quateintakeofproteinandcaloriesandischaracterizedby
emaciation while kwashiorkor involves normal caloric intake
but inadequate protein intake. We report a rare case of dilated
cardiomyopathy caused by severe malnutrition combined
with selenium deciency in a teenager. e malnutrition seen
in this patient can be more accurately dened as “disease
or injury related malnutrition” and is commonly seen in the
setting of major infection, burns, or trauma [].
2. Case Report
A fourteen-year-old boy from South America was transferred
to the burn unit at our institution for severe malnutrition.
e patient had sustained second-degree burns (>% BSA)
two years prior to admission. He did not receive adequate
nutrition and medical care because of poverty and limited
resources in his home country. He was noted to be severely
cachectic and malnourished at a local hospital and was
transferred to the United States by a charity organization for
higher level of care.
Initial assessment was signicant for a weight of
 kg (<rd percentile on WHO growth chart), height of
 cm (BMI . kg/m2,𝑧score .), blood pressure of
/ mmHg, and a heart rate of  bpm. He experienced
shortness of breath with minimal physical activity. Physical
examination revealed severe cachexia with atrophy of all
muscles without peripheral edema. He was also noted
to be moderately dehydrated. Initial investigations were
signicant for mild hyponatremia [ mmol/L (NL –
 mmol/L)], severe hypoalbuminemia [. g/dL (NL
.–. g/dL)], hypocalcemia [ionized calcium . mg/dL
(NL .–. mg/dL)], severe iron deciency anemia
[hemoglobin/hematocrit . gm/dL/.%, MCV . FL],
and selenium deciency [ ug/L]. e reported normal
serum selenium levels in the United States ranges from 
Hindawi Publishing Corporation
Case Reports in Pediatrics
Volume 2016, Article ID 8305895, 4 pages
http://dx.doi.org/10.1155/2016/8305895
Case Reports in Pediatrics
0
5
10
15
20
25
30
35
1 4 7 10 15 90 120
Number of days
Weight (kg)
F : A diagram showing the increase in body weight with time.
toug/L[]whilethenormalserumlevelsofseleniumin
his country of origin is reported to be .– ug/L []. e
carnitine level was normal.
Immediate intravenous uid resuscitation was initiated.
Since he was severely malnourished and had limited ability
for oral intake, high calorie enteral nutrition was admin-
istered via a nasogastric tube. An initial echocardiogram
(echo) showed a globular dilated le ventricle with a severely
depressed systolic function [ejection fraction (EF) <%]
consistent with dilated cardiomyopathy. He was initially
treated with furosemide and enalapril, and carvedilol was
added a week later since there was minimal improvement
in cardiac function in addition to selenium replacement
[ mcg (. umol) twice daily intravenously]. He received
two blood transfusions and repeated albumin infusions
within the rst ve days. One week later, his cardiac function
dramatically improved [EF %]. His selenium level, as well
as other labs, normalized within two weeks. e cardiac
function normalized within four weeks. His weight continued
to increase and reached  kg [BMI . kg/m2,𝑧score
.] within four months (Figure ). All cardiac medications
were then discontinued. e patient’s stamina continues to
improve,andheisabletotoleratefulloralintake.
3. Discussion
Malnutrition is a signicant cause of morbidity and mortality
in developing countries. It is the direct cause of over ,
deaths annually and is indirectly responsible for about half of
all deaths in children []. e median prevalence of under-
weight children worldwide in the year  was % with
% of them having severe muscle wasting []. Malnutrition
may be secondary to a variety of reasons including poverty,
pathologic states, political situations in certain countries, and
even acts of rebellion in order to achieve certain goals. Speci-
cally, “disease related malnutrition” is dened as malnutrition
in the setting of severe inammation and is commonly seen
in the setting of major infection, burns, or trauma. Critical
illness or injury promotes an acute inammatory response
that has a rapid catabolic eect on lean body mass []. e
inammatory condition in most diseases is chronic in nature
and loss of muscle mass and function may occur over months
to years. is form of malnutrition is partially attributable
to a decrease in nutrient intake and to the eect of the
inammatory state on metabolism. e malnutrition seen in
this case was both disease related and secondary to starvation
asaresultofhisdiseaseandpoverty.
Children suering from severe malnutrition may exhibit
cardiovascular abnormalities including hypotension, cardiac
arrhythmias, cardiomyopathy, cardiac failure, and even sud-
den death []. e cardiac myocytes atrophy during star-
vation similar to other muscles in the body []. Kerpel-
FroniusandVargashoweda%decreaseintheweightofthe
heart in an autopsy study []. Ultrastructural features of rats
with PEM showed hyalinization and vacuolization of cardiac
muscle bers, loss of cross striations and myobrils, small
foci of necrosis, interstitial brosis, and mononuclear cell
inltration []. Histological studies of human myocardium
in patients with PEM show atrophy of the muscle bers with
interstitial edema. e heart has also been reported to be
thin-walled, pale, and abby on gross examination in other
autopsy studies of children with malnutrition [].
Echocardiographic studies have shown decreased le
ventricular (LV) mass in patients with PEM. Singh et al.
reported that LV systolic functions were reduced especially
in children with a loss in bodyweight of more than % of
expected weight []. A recent study showed that parameters
of LV systolic function were signicantly reduced in patients
with PEM as compared to controls []. PEM has been
established as an independent risk factor for mortality in
patients with heart failure [].
Chinese investigators rst showed that selenium de-
ciency is one of the principal factors responsible for Keshan
disease, a dilated cardiomyopathy that aects people living
in rural areas where selenium is decient []. Selenium
mediates its eects via incorporation into selenoproteins
(Figure ). Selenium-dependent enzymes mediate a wide
range of biological functions such as regulation of the
inammatory response and proliferation/dierentiation of
several immune cells.
Selenium is also important in the body’s antioxidant
defense mechanism []. When incorporated into the various
selenoenzymes, selenium increases antioxidant capacity and
suppresses the production of interleukins and tumor necrosis
factor alpha (TNF-𝛼). Lu et al. provided the rst evidence
that selenoproteins contribute to the antioxidant defense
mechanisms in cardiomyocytes. Venardos and colleagues
demonstrated that selenium deciency leads to myocardial
injury secondary to increased protein and lipid peroxidation
in a rat model. ese data were supported by the experiments
of Tanguy et al., who showed that selenium deciency in rats
lead to myocardial damage and altered recovery of cardiac
function.
Given these ndings, various experimental studies have
aimed to limit myocardial injury through selenium supple-
mentation. e Venardos group showed signicantly more
myocardial injury in rats fed with a low selenium diet.
Furthermore, Tanguy and colleagues conrmed selenium’s
protective characteristics by demonstrating (a) an improved
cardiac function recovery, (b) a signicantly reduced infarct
Case Reports in Pediatrics
Heart
GP-3
Prevents LDL oxidation, vascular inammation,
and atherogenesis
GP-4
Protects cellular lipids from oxidation
Trx-1
Reduces hypertrophy
and oxidative stress
SelK
Decreases intracellular ROS
F : A diagram showing the actions of various selenoproteins on the cardiac myocytes. SelK: selenoprotein K. GP-: glutathione
peroxidase-. Trx-: thioredoxin reductase-. GP-: glutathione peroxidase-. ROS: reactive oxygen species. LDL: low density lipoprotein.
T : A comparison between marasmus and kwashiorkor.
Marasmus (PEM) Kwashiorkor
Severe deciency of all
nutrients and inadequate
caloric intake
Severe protein deciency
but normal caloric intake
Peripheral edema is absent Peripheral edema is present
Hair changes absent
Hair changes common
(sparse and easily pulled
out)
Skin is dry and wrinkled
but no dermatosis
Dermatosis, aky paint
appearance of skin
Voracious appetite Poor appetite
Absent subcutaneous fat Reduced subcutaneous fat
Fatty liver uncommon Fatty liver common
Better prognosis Worse prognosis
size, and (c) a decreased incidence of postischemic ventric-
ular arrhythmias in rats that received the highest selenium
intake.
e patient we described had some features consistent
with marasmus (Table ). He also had evidence of dilated
cardiomyopathybasedoncongestiveheartfailuresymptoms
and echocardiographic ndings. He responded well to stan-
dard therapy for dilated cardiomyopathy with furosemide
(preload reduction), enalapril (aerload reduction), and
carvedilol (antioxidant eects). Carvedilol is a nonselective
beta blocker which suppresses reactive oxygen species and
has antioxidant and anti-inammatory eects []. Selenium
supplementation was added for its documented deciency
in addition to providing adequate nutritional support. ere
was a remarkable weight gain from  kg to  kg in just
 weeks and he continues to gain more weight. He is
currently asymptomatic from a cardiovascular standpoint
and is tolerating complete oral intake.
4. Conclusion
Cardiac injury leading to dilated cardiomyopathy in cases of
severe malnutrition is rare but documented in the literature.
During the treatment of patients with malnutrition, levels
of trace elements (specially selenium) should be checked.
Adequate nutrition and replacement of decient elements
shouldbeinitiatedassoonaspossible.Congestiveheart
failure and dilated cardiomyopathy in these patients seem
torespondwelltopreloadandaerloadreductionwiththe
addition of carvedilol as an antioxidant.
Abbreviations
WHO: World Health Organization
PEM: Protein energy malnutrition
BSA: Body surface area
BMI: Body mass index
LV: Le ve ntricular
TNF-𝛼: Tumor necrosis factor-𝛼.
Competing Interests
e authors have no conict of interests relevant to this article
to disclose.
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... Several micronutrients such as carnitine, thiamine, selenium, taurine and niacin are essential co-factors of metabolic reactions in the myocardium and contribute to efficient and appropriate energy utilization. Thus, genetic or acquired deficits in any one of these micronutrients has the potential to lead to specific forms of cardiomyopathies (CMs) [4][5][6]. ...
... Keshan disease is the classical clinical presentation of cardiac disease due to selenium deficiency, often presenting with clinical features characteristic of DCM: cardiogenic shock, cardiac arrhythmias, ECG abnormalities, enlarged heart, and/or heart failure [117]. There is also echocardiographic evidence of biventricular enlargement and histologically defined oedema, mitochondrial swelling, hypercontraction bands, widespread myocytolysis and extensive fibrosis [6]. The possibility of selenium deficiency should be considered in all malnourished patients who receive parenteral nutrition. ...
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