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Assessment of Nutritional Status and

Nutrient Consumption of Thalassemia


Patients

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List of Figures

Figure 1: Classification of alpha thalassemia ................................................................... 17

Figure 2: Classification of beta thalassemia ..................................................................... 17

Figure 3: Distribution of thalassemia globally ................................................................. 21

Figure 4: Distribution of thalassemia according to the world .......................................... 22

Figure 5: Average Anthropometric (height & weight) data of study subjects ................. 41

Figure 6: Consumption of cereals in gram by study subjects .......................................... 43

Figure 7: Average intake of nine food groups in gram by study subjects ........................ 46

Figure 8: Distribution of gender among study populations.............................................. 48

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List of Tables
Page
Table No. Name of Tables
no.
Table 1 Anthropometric parameters of the study subjects (n=600) 35

Table 2 Monthly income parameter of the study subjects (n=600) 36


Table 3 Clinical parameters of the study subjects (n=600) 36
Table 4 Cereals consumption by the study subjects (n=600) 37
Table 5 Meat consumption among study subjects (n=600) 38
Table 6 Fish consumption among study subjects (n=600) 38
Table 7 Milk products consumption among study subjects (n=600) 38
Table 8 Vegetables consumption among study subjects (n=600) 38
Table 9 Pulse consumption among study subjects (n=600) 39
Table 10 Beverages consumption among study subjects (n=600) 39
Table 11 Fruits consumption among study subjects (n=600) 39
Table 12 Eggs consumption among study subjects (n=600) 39
Average intake of energy, macronutrients & fiber by study
Table 13 40
subjects (n=600)
Table 14 Average intake of micronutrients by study respondents (n=600) 41
Table 15 Gender among the study subjects (n=600) 42
Table 16 Disease types among the study participants (n=600) 42
Table 17 Districts of study populations (n=600) 43
Table 18 Father’s educational level among the study participants (n=600) 44
Mother’s educational level among the study participants
Table 19 45
(n=600)
Table 20 Father’s occupation among the study participants (n=600) 45
Table 21 Mother’s occupation among the study participants (n=600) 46
Table 22 Number of child of the participants parents (n=600) 46
Table 23 Number of parity of the participants parents (n=600) 47
Table 24 Order of siblings among the respondents (n=600) 47
Table 25 Father’s blood group of the respondents (n=600) 48
Table 26 Mother’s blood group of the respondents (n=600) 48
Table 27 Blood group of the respondents (n=600) 49
Table 28 Carrier of the respondents (n=600) 49

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ABSTRACT

Malnutrition affects growth pattern, learning, treatment and quality of life in

patients with thalassemia. This study aimed to assess nutritional status & focus on the

nutrient intake in patients with thalassemia. The main motive of this study was to see if

thalassemia patients were able to meet their daily body requirement. Methods: Six

hundred patients with (317 males and 283 females) were enrolled in this study. Data of

food intake, anthropometry and biochemical parameters were collected by a pre-designed

questionnaire. Nutritional status was evaluated by anthropometric measurements. Dietary

intake was assessed by 24-hour dietary recall. Dietary recall for three days was counted.

Hemoglobin and Ferritin levels were also determined. Results: The results showed that

these patients had normal nutrition evaluated by BMI and their Hemoglobin & ferritin

levels were not normal. These levels keep fluctuating. However, the anthropometric

measurements including height, weight and body mass index demonstrated that these

patients were stunted. It was also found that they had average total energy intake lower

than the dietary reference intake Conclusion: The results of anthropometric measurements

in this study indicated that patients with thalassemia had growth impairment. Although

BMI appeared normal in these patients, their dietary intakes were apparently inappropriate.

Therefore, the nutritional assessment and appropriate nutritional interventions should be

incorporated into therapeutic plans for these patients to improve growth status and clinical

outcomes.

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Chapter-1
Introduction

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1. INTRODUCTION

1.1. Definition of Thalassemia

Thalassemia is one of the major autosomal recessive hereditary

hemoglobinopathies prevalent in the world populations, particularly in Mediterranean belt,

Far-eastern and South East Asian countries (El-Harth et al., Weatherall, & l., 2000).

Thalassemias are inherited blood disorders characterized by

abnormal hemoglobin production, it is genetic disorders inherited from a person's parents.

(NHLBI, 2012) .

1.2. Prevalence of thalassemia

World Health Organization (WHO) estimates that at least 6.5% of the world

populations are carries of different inherited disorders of hemoglobin (Modell B.,

1995;5:247-58). Another WHO report estimates that 3% are carriers of beta-thalassemia

and 4% are carriers of Hb E in Bangladesh. In Bangladesh, more than 7000 children are

born with thalassemia each year (Khan WA., 2006 ) . As of 2013, thalassemia occurs in

about 280 million people, with about 439,000 having severe disease (Lancet. 386 (9995):

743–80)

1.3. Types of thalassemia

There are two main types, alpha thalassemia and beta thalassemia. The α-

thalassemias involve the genes HBA1 (Alpha locus 1; HBA1 -

141800) and HBA2, inherited in a Mendelian recessive fashion. Beta thalassemias are

due to mutations in the HBB gene on chromosome 11, also inherited in an autosomal,

recessive fashion (Beta Locus; HBB -141900) .

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1.4. Causes of thalassemia

Both α- and β-thalassemias are often inherited in an autosomal recessive manner.

If both parents carry a hemoglobinopathy trait, the risk is 25% for each pregnancy for an

affected child.Estimates suggest that approximately 1.5% of the global population (80 - 90

million people) are β-thalassemia carriers ( Galanello, Renzo, Origa, Raffaella, 2010) .

1.5. Diagnosis of thalassemia

1.5.1. Clinical Diagnosis

Thalassemia major is usually suspected in an infant younger than two years of age

with severe microcytic anemia, mild jaundice and hepatosplenomegaly. (Galanello, Melis,

Ruggeri, 1979).

1.5.2. Hematologic Diagnosis

RBC indices show microcytic anemia. Thalassemia major is characterized by

reduced Hb level (<7 g/dl), mean corpuscolar volume (MCV) > 50 < 70 fl and mean

corpuscolar Hb (MCH) > 12< 20 pg. Thalassemia intermedia is characterized by Hb level

between 7 and 10 g/dl, MCV between 50 and 80 fl and MCH between 16 and 24 pg.

Thalassemia minor is characterized by reduced MCV and MCH, with increased Hb

A2 level (Galanello, Melis, Ruggeri, 1979).

1.6. Management of thalassemia

1.6.1. Nutritional management

Patients with thalassemia have low circulating levels of many nutrients, dietary

intake of vitamin D is insufficient to maintain adequate vitamin D status and dietary intake

of iron is unrelated to total body iron stores, particularly in chronically transfused patients

(J Acad Nutr Diet. 2012 Jul; 112(7): 980–990.2012). Non-transfused patients are

encouraged to consume a moderately low-iron diet—that is, avoiding iron-fortified foods

and excessive consumption of red meat. For transfused patients on chelation therapy, a

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low-iron diet is unnecessary and may decrease quality of life for some patients (UCSF

Benioff Children’s Hospital Oakland, 2016). In addition to iron-related issues, patients

with thalassemia commonly exhibit morbidities linked with poor nutritional status:

inadequate growth, poor immune function, and decreased bone mineralization. As part of

the Thalassemia Clinical Research Network’s Longitudinal Cohort Study, research

investigating dietary intake of thalassemia patients was published in the Journal in 2012

(Fung EB, 2012).

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Chapter-2
Review of Literature

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2. REVIEW OF LITERATURE

2.1. History of thalassemia:

Thalassemia is derived from the Greek word “thalassa” meaning “the sea” because

the condition was first described in populations living near the Mediterranean Sea )(

Whipple and Bradford, 1932).

Thalassemia was first recognized in 1925 by a Detroit physician, Cooley and Lee,

who described a series of infants who became profoundly anemic and developed

splenomegaly and bone change over the first year of life (Cooley and Lee, 1925). George

and William (1932), described the pathological changes of the condition for the first time,

recognized that many of their patients came from the Mediterranean region. It was only

after 1940 that the true genetic character of this disorder was fully appreciated (Weatherall,

2001). Sturgeon et al. (1955) pointed out the chronic hemolytic anemia associated with

thalassemia and sickling trait. Sturgeon, et al (1955a) Searched and described intermediate

types of thalassemia clinically, genetically and biochemical studies of intermediate type

of Cooley’s anemia. Parfrey et al. (1981) he observed the Iron overload in beta-thalassemia

minor. Fuchs et al. (1996) finds the nutritional factors and thalassemia major.

2.2. Definition of Thalassemia:

Thalassemia are a heterogeneous group of genetic disorder of hemoglobin

synthesis characterized by a reduction in the synthesis of one or more of the globins chains

leads to imbalanced globin- chain synthesis, defective hemoglobin production causing

anemia (Victor et al., 1999).

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2.3. Classification of thalassemia:

The two main types are called Alpha and Beta thalassemia, depending on which

part of globin chain is produced in reduced amounts (Victor et al., 1999).

2.3.1. Alpha Thalassemia:

Normally, alpha globin chain is made by four genes (two from each parent), two

on each strand of chromosome 16. The alpha thalassemia are caused by a decrease in

production of alpha globins chains due to deletion or mutation of one or more of the four

alpha globins genes located on chromosome 16 (Hillman and Ault, 2002).

A-Molecular Pathology

Two α thalassemia phenotypes are recognized; one is characterized by thalassemia

minor in the heterozygous state and the other is marked by no clinical or hematologic

abnormality in the heterozygous state. The former phenotype has been referred to as α -

thalassemia 1 and the latter has been labeled α -thalassemia 2. It is now recognized that

the α- thalassemia 1 determinants are associated with complete absence of α-globin

synthesis and the α thalassemia 2 phenotypes with only a reduction in α-globin synthesis.

Accordingly, these two major α thalassemia variants Thalassemia (Lee et al., 1999). + are

now designated thalassemia α° and α.

Alpha (0) thalassemia –

More than 20 different genetic mutations that result in the functional deletion of

both pair of α-globin genes have been identified. Individuals with this disorder are not able

to produce any functional α -globin and thus are unable to make any functional hemoglobin

A, F, or A2. This leads to the development of hydrops fetalis, also known as hemoglobin

Bart, a condition that is incompatible with extra uterine life.

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Alpha (+) thalassemia –

There are more than 15 different genetic mutations that result in decreased

production of α -globin usually due to the functional deletion of 1 of the 4 alpha globin

genes. Based on the number of inherited alpha genes, alpha (+) thalassemia is sub-

classified into 4 general forms:

 A-Thalassemia (-α/α α) is characterized by inheritance of 3 normal α-genes. These

patients are referred to clinically as silent carrier of alpha thalassemia. Other names

for this condition are alpha thalassemia minima, alpha thalassemia-2 trait, and

heterozygosity for alpha (+) thalassemia minor. The affected individuals exhibit no

abnormality clinically and may be hematologically normal or have mild

reductions.

 B- Inheritance of 2 normal alpha genes due to either heterozygosity for alpha (+)

thalassemia (-α /- α) (one from each of two chromosomes) called a "trans deletion"

 Or homozygosity for alpha (+) thalassemia (α α/--) (two on the same chromosome)

called a "cis deletion" results in the development of alpha thalassemia minor or

alpha thalassemia-1 trait. When parents are carriers of the cis deletion, there is a

one in four, or 25 percent, chance with each pregnancy, to have a baby with alpha

thalassemia major.

 C- Inheritance of one normal alpha gene (-α/--) results in abundant formation of

hemoglobin H composed of tetramers of excess beta chains. This condition is

known as Hb H disease.

 D- The loss of all four alpha genes produces a condition that is incompatible with

life. The gamma chains produced during fetal life associate in groups of four to

form an abnormal hemoglobin called "hemoglobin Bart's" (Forget, 2000).

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B-Pathophysiology:

The pathophysiology of alpha thalassemia is different to that of beta thalassemia

.A deficiency of α chain leads to the production of excess chains or β chains , which form

Hb Bart's and Hb H respectively . These soluble tetramers do not precipitate in the bone

marrow and hence erythropoiesis is more effective than in β thalassemia. However, Hb H

is unstable and precipitates in red cells as they age. The inclusion bodies produced in this

way are trapped in the spleen and other parts of the microcirculation leading to shortened

red cell survival. Furthermore, both Hb Barts and Hb H have a very high oxygen affinity;

because they have no α chains, there is no haem-haem interaction and their oxygen

dissociation curves resemble myoglobin (Victor et al., 1999). There are four subtypes of

alpha thalassemia that range from mild to sever in their effect on the body (Cohen et al.,

2004).

(1) Silent carrier state:

This is the one-gene deletion alpha thalassemia condition. This condition

generally causes no symptoms or signs of anemia and will not need treatment

because the lack of alpha protein is so small that the hemoglobin functions

normally (Hillman and Ault, 2002). It is called "silent carrier" because it is difficult

to identify α thalassemia silent carrier state by standard haematological studies.

They are detected only by DNA Studies (Forget, 2000).

(2) Alpha Thalassemia Trait:

Also known as mild alpha-thalassemia. These patients have lost two alpha

globin genes. Patients with this condition have small red cells and a mild anemia

but they do not have clear symptoms. They look and feel normal but may be

discovered upon routine testing (Hillman and Ault, 2002).

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(3) Alpha Thalassemia Intermedia:

Also known as hemoglobin H disease. These patients have lost three alpha

globin genes. Patients with this condition have a severe anemia, and often require

blood transfusions to survive. Infants born with alpha thalassemia intermedia

appear normal at birth but often develop anemia and splenomegaly by the end of

their first year. Hepatomegaly is not a common finding and there may be some

association with mental retardation. Due to the hemolytic nature of this anemia,

there may be an increase in respiratory infections, leg ulcers and gallstones.

Skeletal changes are not commonly seen in hemoglobin H disease (Cohen et al.,

2004).

The severe imbalance between the alpha chain production (now powered

by one gene, instead of four) and beta chain production (which is normal) causes

an accumulation of beta chains inside the red blood cells. Normally, beta chains

pair only with alpha chains. With three-gene deletion alpha thalassemia, however,

beta chains begin to associate in groups of four, producing abnormal hemoglobin,

called "hemoglobin H". The condition is called "hemoglobin H disease".

Hemoglobin H has two problems. First it does not carry oxygen properly,

making it functionally useless to the cell. Second, hemoglobin H protein damages

the membrane that surrounds the red cell, accelerating cell destruction. The

combination of the very low production of alpha chains and destruction of red cells

in hemoglobin H disease produces a severe, life-threatening anemia. Untreated,

most patients die in childhood or early adolescence (Forget, 2000).

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(4) Alpha Thalassemia Major:

Also known as hydrops fetalis. In this condition, there are no alpha genes

in the individual's DNA, which causes four gamma globins produced by the fetus

to form abnormal hemoglobin called hemoglobin Bart's. Most individuals with this

condition die before or shortly after birth (Cohen et al., 2004).

2.3.2. Beta Thalassemia:

There are more than 200 of mutation within the beta globin gene found worldwide

to produce beta thalassemia .Unlike the deletion that constitute most of the alpha

thalassemia syndromes ,beta thalassemia are caused by mutation on chromosome 11 that

affect all aspect of beta globin production : transcription ,translation , and the stability of

the beta globin production (Howard et al.,1996).

A-Molecular Pathology

There are two types of β thalassemia, ß+ and ß° thalassemia, in which there is

respectively a reduction in and total absence of beta chain production. Beta thalassemia

major usually results from the homozygous state for either ß+ or ß° thalassemia, or

occasionally from the compound heterozygous state for both ß+ and ß° thalassemia.

Homozygous ß° thalassemia is associated with a predominance of Hb F, no Hb A ,and

variable amounts of Hb A2. In individuals with homozygous ß+ thalassemia, the amounts

of Hb A are variable, Hb F is increased and is distributed heterogeneously among red cells,

and Hb A2 is normal, decreased , or elevated (Lee et al.,1999).

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B-Pathophysiology:

The molecular defects in β thalassemia result in absent or reduced β chain

production .Alpha chain synthesis is unaffected and hence there is imbalanced globin chain

production leading to an excess of α chains. In the absence of their partners, they are

unstable and precipitate in the red cell precursors, giving rise to large intracellular

inclusions, which interfere with red cell maturation. Hence, there is a variable degree of

intramedullary destruction of red cell precursors (i.e. ineffective erythropoiesis). Those red

cells that mature and enter the circulation contain α chain inclusion, which interfere with

their passage through the microcirculation, particularly in the spleen. These cells, which

show a variety of abnormalities of membrane structure and permeability, are prematurely

destroyed and thus the anemia of β thalassemia results from both ineffective erythropoiesis

and a shortened cell survival. The anemia acts as a stimulus to erythropoietin production

and this causes expansion of the bone marrow, which may lead to serious deformities of

the skull and long bones. Because the spleen is being constantly bombarded with abnormal

red cells, it hypertrophies (Victor et al., 1999).

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Figure 1: Classification of alpha thalassemia

Figure 2: Classification of beta thalassemia

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There are three general categories of beta thalassemia that also range from mild to

severe in their effect on the body (Rund and Rachmilewitz, 1995).

(1) Beta thalassemia Minor:

Also known as thalassemia Trait. In this condition, one of the two beta globin genes

is abnormal but the lack of beta protein is not great enough to cause problems in the normal

functioning of the hemoglobin (Rund and Rachmilewitz, 1995).

Alpha chain production continues at a near normal rate. The alpha chains combine

with the available beta chains resulting in decreased levels of hemoglobin A here still

remains excess alpha chains and this stimulates the increased production of delta chains.

The alpha and delta chains combine to form increased amounts of hemoglobin A2.

This if there is still an excess of alpha chains the normal mechanism which switches off

gamma chain production does not function correctly and the rate of gamma chain

Production is greater than in a normal adult. results in the formation of increased amounts

of hemoglobin F (Weatherall, 2001).

A person with this condition simply carries the genetic trait for thalassemia and

have a 50/50 chance to pass the gene to their offspring, who would also have thalassemia

minor and will usually experience no health problems other than possible mild anemia

(Lee et al.,1999).

(2) Beta thalassemia Intermedia:

In this condition, an affected person has two abnormal genes but is still producing

some beta globin. In this condition the lack of beta protein in the hemoglobin is great

enough to cause a moderately severe anemia and significant health problems, including

fatigue or shortness of breath, bone deformities, mild jaundice and enlargement of the

spleen.( Forget, 2000) There is a wide range in the clinical severity of this condition, and

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the borderline between thalassemia intermedia and the most severe form, thalassemia

major, can be confusing. The deciding factor seems to be the degree of anemia and the

amount of blood transfusions required by the patient. The more dependent the patient is

on blood transfusions, the more likely he or she is to be classified as thalassemia

major(Rund and Rachmilewitz, 1995).

(3) Beta thalassemia Major:

It is also called Cooley's anemia , named after the doctor who first described it in

1925 (Cooley and Lee, 1925). Beta thalassemia Major is the most severe form of beta

thalassemia in which the complete lack of beta globin production, preventing the

production of significant amounts of Hb A. The severe imbalance of globin chain synthesis

(alpha >> beta) results in ineffective erythropoiesis and severe microcytic hypochromic

anemia. The excess unpaired alpha-globin chains aggregate to form precipitates that

damage red cell membranes, resulting in intravascular hemolysis. Premature destruction

of erythroid precursors results in intramedullary death and ineffective erythropoiesis.

The profound anemia typically is associated with erythroid hyperplasia and

extramedullary hematopoiesis (Cunningham et al., 2004).

At birth the baby with thalassemia major seems entirely normal. This is because

the predominant hemoglobin at birth is still fetal hemoglobin (Hb F). Hb F has two alpha

chains (like Hb A) and two gamma chains (unlike Hb A). It has no beta chains so the baby

is protected at birth from the effects of thalassemia major.

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2.4. Prevalence of Thalassemia in the world:

Thalassemia is considered the most common genetic disorder world wide

(Illeyman et al ., 2000). Thalassemia is found in some 60 countries with the highest

prevalence in the Mediterranean region, parts of North and West Africa, the Middle East,

the Indian subcontinent, southern Far East and southeastern Asia, especially Thailand and

southern China together composing the so-called thalassemia belt (Hoffman et al., 2005).

In Asia, the highest incidence of thalassemia found in Maldives with a carrier rate of 18%

of the population (Furuumi et al., 2006). The estimated prevalence is 16% in people from

Cyprus(Yaish, 2007) , in Thailand 1% are affected and more than 20 million were

thalassemia carriers (Chonnanit et al. ,2005) and 3-8% in populations from Bangladesh ,

China , India , Malaysia and Pakistan (Hoffman et al., 2005).

In Europe, the highest concentrations of the disease are found in Greece, including

the Greek islands; in parts of Italy, lower Po valley (Peres et al., 1996); in southern Italy;

and in the Italian islands Sicily, Sardinia (Guiso et al., 1996) , and Malta Corsica (French

island) and Crete (Greek islands)( Hoffman et al., 2005). A very low prevalence has been

reported from people in northern Europe (0.1%) and Africa (0.9%) (Ballas et al., 1997).

The highest frequency of the alpha thalassemia genes is found in Southeast Asia

(Ko and Xu , 1998),Africa (Ballas et al., 1997) and in Mediterranean region including

Portugal with incidence of α -thalassemia carriers is (10%)( Peres et al., 1996), (18%) in

Sardinians(Guiso et al., 1996), and 7% in Greece(Hoffman et al., 2005).

The population of northern Thailand, with a prevalence of about 5% to 10%,

Harbors one of the highest incidences of α-thalassemia in the world (Figure1) (Chonnanit

et al. ,2005). About 150 million people worldwide carry ß-thalassemia genes. Beta

thalassemias are distributed widely in Many Mediterranean islands, including Cyprus

(Yaish, 2007), Sardinia (11-34%)( Hoffman et al., 2005).), and Sicily (10%), have a

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significantly high incidence of severe beta thalassemia, constituting a major public health

problem ( Hoffman et al., 2005). For instance, in Cyprus, 1 in 7 individuals carries the

gene, which translates into 1 in 49 marriages between carriers and 1 in 158 newborns

expected to have β thalassemia major (Yaish, 2007).

These diseases are also common in the other Arab countries such as Libya (5% in

α thalassemia and in β Thalassemia 4%), Tunisia (4,8 in α thalassemia and in β

Thalassemia 4,4%), Algeria (9 in α thalassemia and in β Thalassemia 3%) and Jordan (3,3

in α thalassemia and in β Thalassemia 3,5%) (Zahed, 2001).

Figure 3: Distribution of thalassemia globally

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The prevalence of globin variant in the world population. is 5%. However, the

prevalence of α- or β-thalassemia trait in the world population is 1.7%. Globally, an

estimated 15 million people have thalassemia. Thalassemia trait is more prevalent

affecting 5-30% of people from the following ethnic groups:

Figure 4: Distribution of thalassemia according to the world

1. Alpha thalassemia is more common in: African, Middle Eastern, East Indian, South-east

Asian (Vietnamese, Laotian, Thai, Singaporean, Filipino, Cambodian, Malaysian,

Burmese and Indonesian), Chinese and Occasionally Mediterranean (Italian and Greek).

The most severe form of alpha thalassemia causes fetal or newborn death.

2. Beta thalassemia is more common in: Mediterranean (Italian and Greek), Iranian,

African, Southeast Asian and Chinese.

3. E Beta thalassemia is more common in Southeast Asian (Cambodian, Vietnamese and

Thai).

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2.4.1. Prevalence of alpha Thalassemias in Northern Thyland:

The study was conducted by M. Lemmens-zygulska to observe the highest

frequencies of alpha- thalassemias in the world. According to the study, The population of

northern Thailand has one of the highest frequencies of α-thalassemia in the world. It has

been examined a sample of 215 healthy subjects from four rural districts of Chiang Mai

province. Out of these, 77 exhibited anomalies of the α-globin genes (αα/-α3.7 in 36; -

α3.7/-α3.7 in 3; ––SEA in 30; αα/αCSα in 5; αααanti3.7 in 3). Therefore, no fewer than

2% of the children in northern Thailand are expected to be born with HbH disease or

thalassemic hydrops fetalis. The considerable public health problem of hemoglobinopaties

and the increasing acceptance of family planning necessitates facilities for the pre- and

postnatal diagnosis of these disorders at the DNA level (Lemmens-Zygulska, 1996).

2.4.2. Prevalence of thalassemia in the Mediterranean area and Iran:

The primary aim of this study was to determine the prevalence of thromboembolic

events in patients with β-thalassaemia. The study demonstrated that thromboembolic

events occurred ina clinically relevant proportion (1.65%) of 8,860 thalassemia patients

(TI – 24.7% or TM – 75.3%) from the Mediterranean and Iran. Thromboembolism

occurred 4.38 times more frequently in TI than TM (p<0.001), with more venous events

occurring inTI and more arterial events occurring in TM.Thrombosis in thalassemia was

also more common in females, splenectomized patients and those with profound anemia

(haemoglobin <9 g/dl). Due to the increased risk of thromboembolic events, the rationale

for splenectomy should perhaps be re-assessed and the role of transfusion therapy for the

prophylaxis of thrombosis, among other complications, be evaluated prospectively (Taher,

2006).

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2.4.3. Prevalence of thalassemia among the UAE adolescent populations:

This study examined the prevalence of high blood pressure, heart disease, and

medical diagnoses in relation to blood disorders, among 6,329 adolescent students (age 15

to 18 years) who reside in the United Arab Emirates (UAE). Findings indicated that the

overall prevalence of high blood pressure and heart disease was 1.8% and 1.3%,

respectively. Overall, the prevalence for thalassemia, sickle-cell anemia, and iron-

deficiency anemia was 0.9%, 1.6%, and 5%, respectively. (Barakat-Haddad, 2013).

Another study was carried out by Khawla M. Belhoul in UAE to determine the prevalence

of hemoglobinopathy carriers in United Arab Emirates (UAE) nationals subjected to mandatory

premarital screening in Dubai over a 4-year period. Data from UAE nationals who underwent

premarital screening by the Dubai Health Authority between January 2007 and December 2010

were collected and analyzed. Premarital screening in Dubai is based on complete blood counts

(CBC) and hemoglobin (Hb) high performance liquid chromatography (HPLC). Among the 6,420

UAE nationals screened, 8.5% (n = 545) were suspected to be carriers. The following carrier

frequencies were observed: β-thalassemia (β-thalassemia), 4.56% (n = 293); 2.9% (n = 186);

0.78% (n = 50); 0.17% (n = 11); 0.03% (n = 2); Hb E-Hb S and Hb E-β- thalassemia also occurred

at a rate of 0.016% (n = 1) each; and 0.87% (n = 56) subjects were suspected of carrying silent β-

thalassemia. The prevalence of Hb S trait observed in this study was lower than that in other reports

for the region. Moreover, some couples choose not to have prenatal diagnosis (PND) or pre

implantation genetic diagnosis (PGD), even if they are aware of their risk status. The prevalence

of β-thalassemia trait in the UAE is high (Belhoul, 2013).

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2.5. Prevalence of Thalassemia in the South Asian countries:

Thalassemias are emerging as a global public health concern. Due to remarkable

success in the reduction of childhood mortality by controlling infectious diseases in

developing countries, thalassemias are likely to be a major public health concern in the

coming decades in South Asia (Hossain, 2017).

An estimated 320,000 babies are born each year with a clinically significant

hemoglobin disorder (Modell, B, 2008). Most conservative estimates suggest that at least

5.2% of the world population (over 360 million) carry a significant hemoglobin variant

(Modell, B, 2008). And in excess of 100 million beta thalassemia carriers with a global

frequency of 1.5% (Colah, R., Gorakshakar, A., & Nadkarni, A., 2010). Homozygous or

compound heterozygous states between certain variants can lead to clinical manifestations

of hemoglobinopathies. Hemoglobinopathies are most prevalent in certain malaria prone

parts of the world including Africa, all Mediterranean countries, the Middle East, the

Indian subcontinent and Southeast Asia (Weatherall, D. J., 2010). In each year, over

50,000 new patients are born with a severe form of thalassemia (beta-thalassemia major

and HbE beta thalassemia) worldwide (Colah, R., 2010). In many Asian countries, the

most common form of thalassemia results from the coinheritance of beta thalassemia and

HbE. In the eastern parts of Indian subcontinent, Bangladesh and other Southeast Asian

countries, HbE is the most prevalent hemoglobin variant (Olivieri, N., 2011).

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2.5.1. Prevalence of Thalassemia in the Chittagong district of Bangladesh:

The study was conducted by Robiul H. Bhuiyan in 2012 to observe the prevalence

of thalassemia & it’s correlation with liver function test in different age & sex group.

World Health Organization (WHO) estimates that at least 6.5% of the world populations

are carries of different inherited disorders of hemoglobin (Modell B.1995). Another WHO

report estimates that 3% are carriers of beta-thalassemia and 4% are carriers of Hb E in

Bangladesh. In Bangladesh, more than 7000 children are born with thalassemia each

year(Khan WA.2006). Majority are born in countries with limited resources where priority

tends to be given to tackling high rates of infant and child mortality from infection diseases

and malnutrition ( Palit, S., 2012).

A study carried out by the Dhaka Shishu Hospital Thalassemia Center in 2004 in

school children of Bangladesh, showed that carrier status is higher and there is also

regional variation. This study revealed that the overall prevalence of beta-thalassemia trait

in Bangladesh was 4.1% and Hb E trait 6.3%(Clegg JB.2001). A recent study showed that

carrier status of Hb-E is 6.1% and as high as 40% in tribal children in Bangladesh (Banu

B, Amin SK,2005).

2.5.2. Prevalence of β-thalassemia and other haemoglobinopathies in six cities in

India:

The population of India is extremely diverse comprising of more than 3,000 ethnic

groups who still follow endogamy. Haemoglobinopathies are the commonest hereditary

disorders in India and pose a major health problem. The data on the prevalence of β-

thalassemias and other haemoglobinopathies in different caste/ethnic groups of India is

scarce. Therefore the present multicentre study was undertaken in six cities of six states of

India (Maharashtra, Gujarat, West Bengal, Assam, Karnataka and Punjab) to determine

the prevalence of haemoglobinopathies in different caste/ethnic groups using uniform

26
methodology. Fifty-six thousand seven hundred eighty individuals (college students and

pregnant women) from different caste/ethnic groups were screened. RBC indices were

measured on an automated haematology counter while the percentage of HbA2, HbF and

other abnormal Hb variants were estimated by HPLC on the Variant Hemoglobin Testing

System. The overall prevalence of β-thalassemia trait was 2.78 % and varied from 1.48 to

3.64 % in different states, while the prevalence of β-thalassemia trait in 59 ethnic groups

varied from 0 to 9.3 %. HbE trait was mainly seen in Dibrugarh in Assam (23.9 %) and

Kolkata in West Bengal (3.92 %). In six ethnic groups from Assam, the prevalence of HbE

trait varied from 41.1 to 66.7 %. (Mohanty, 2013).

2.5.3. Prevalence of heterozygous β-thalassemia in Northern areas of Pakistan:

This study was conducted by Mohammad Farooq Khattak & Mohammad Saleem

(1992) to observe the prevalence rate of heterozygous B-thalassemia in northern areas of

Pakistan. According to the study, Five hundred apparently healthy adults from northern

parts of Punjab and NWFP were screened for the prevalence of heterozygous fi-

thalassemia. The trait was detected in all ethnic groups with an overall prevalence rate of

5.4% (27/500). Pathans had significantly (P<0.02) higher prevalence rate (7.96%) than

Punjabis (3.26%) (JPMA 42: 32, 1992). Present study was undertaken to find out the

overall frequency of healthy carriers of B-thalassemia in northern Pakistani population.

Five hundred healthy adult studentvolunteers aged seventeen to twenty-four years (mean

19 years) from educational institutions of Rawalpindi and Peshawar were studied (males

326, females 174). Ml were thoroughly interviewed and clinically examined specially for

jaundice, hepatosplenomegaly and lymphadenopathy. Heterozygous B-thalassemia trait

was detected in 5.4% (27/500) cases in this survey of apparently healthy adults. Sixteen

out of 27 (59.3%) subjects with heterozygous B- thalassemia were Pathans and 8 (30%)

were Punjabis. Hemoglobin A2 level ranged between 4.0-6.6%. MCV of 77 fi or less was

27
found in 92.6% and MCH of 26 pg or less in all subjects of B-thalassemia trait.

heterozygous B- thalassemia was found to be more prevalent in Pathans than Punjabis;

Pathans: 7.96% (16/201); Punjabis: 3.26% (8/245) (P<0.02) (Khattak,1992).

2.6. Nutritional status of thalassemia patients:

2.6.1. Zinc status in patients with major β-thalassemia:

Mahshid Mehdizadeh (2008) described this study to determine the zinc status in

patients with major β-thalassemia and its effect on their growth. He studied 64 thalassemic

patients in comparison with 64 healthy matched individuals. Demographic and

anthropometric data and history of the therapies were collected. Serum zinc level in both

groups and ferritin in the thalassemic group were assigned. Interestingly, mean serum zinc

level was significantly higher in the thalassemic group. No significant correlation between

serum zinc level and short stature, serum ferritin level, desferrioxamine dose, initiating

time of blood transfusion, and chelation therapy was found. The study indicates zinc

deficiency in thalassemic patients who are on regular blood transfusion is rare and it seems

that routine zinc supplementation is not necessary (Mehdizadeh, 2008).

2.6.2. Vitamin D Status in Thalassemia Major:

The survival of patients with thalassemia major has progressively improved with

advances in therapy; however, osteoporosis and cardiac dysfunction remain frequent

complications. Adequate circulating levels of vitamin D are essential for optimal skeletal

health and reducing fracture risk. Vitamin D deficiency and insufficiency is reported to be

high in thalassemic patients in many countries despite the presence of good sunshine and

routine prescription of 400–1,000 IU vitamin D per day. The risk of vitamin D deficiency

in thalassemia and its relation to bone disease; including osteoporosis, rickets, scoliosis,

spinal deformities and fractures as well as to cardiac dysfunction is discussed in this mini-

review. Monitoring and maintaining normal serum level of 25-OH vitamin D through oral

28
intake of vitamin D and early correction of VDD by oral or parental use of vitamin D may

significantly improve bone mineral accretion and ameliorate cardiac function. The survival

of patients with thalassemia major has progressively improved with advances in therapy;

however, osteoporosis and cardiac dysfunction remain frequent complications. Adequate

circulating levels of vitamin D are essential for optimal skeletal health and reducing

fracture risk. Vitamin D deficiency and insufficiency is reported to be high in thalassemic

patients in many countries despite the presence of good sunshine and routine prescription

of 400–1,000 IU vitamin D per day. The risk of vitamin D deficiency in thalassemia and

its relation to bone disease; including osteoporosis, rickets, scoliosis, spinal deformities

and fractures as well as to cardiac dysfunction is discussed in this mini-review. Monitoring

and maintaining normal serum level of 25-OH vitamin D through oral intake of vitamin D

and early correction of VDD by oral or parental use of vitamin D may significantly

improve bone mineral accretion and ameliorate cardiac function (Soliman, 2013).

2.7. Nutrient Deficiency in Thalassemia patients:

2.7.1. Iron deficiency among Thalassemia patients:

Mujahida Rahman stated about iron deficiency in thalassemia trait in 2016 to find

out the pattern of iron status & determine co-existing iron deficiency in them. This cross

sectional study was carried out in the Department of Hematology, BSMMU,

Dhaka from January 2007 to December 2007. Patients having iron deficiency detected

by serum iron profile were compared with those without iron deficiency. Among the study

population, highest frequency of co-existent iron deficiency was found among the age of

21-30 years. Prevalence of iron deficiency was 30.2% among b-thalassemia trait.

(Rahman, M., 2015).

29
2.7.2. Zinc deficiency among thalassemia patients:

AKM Amirul Morshed stated about Growth Status and Serum Zinc Level in

Patients with Hemoglobin –E-β Thalassemia in 2012 To investigate and compare the

serum zinc of Hb E β thalassemia patients and normal children and to see the relationship

between serum zinc and growth status. There was no significant difference between two

groups. Mean percent of 50th centile of weight achieved was 75.9% in cases and 81% in

control. Similarly mean percent of 50th centile of height achieved was 89% in cases and

93% in control. There was significant difference in height for age between both the groups

(p=.05). Serum zinc level did not significantly changed in thalassemic group and control

group children. But there were significant stunting in Hb –E β thalassemia patients and no

significant difference was found between these children in terms of weight for age

(Morshed, A., 2012).

2.7.3. Vitamin B12 & Folic acid deficiency among thalassemia patients:

According to LUHBY & COOPERMAN (1961), folio acid deficiency was

demonstrated by examination of marrow, estimation of for miminoglutamic acid

in urine after histidine, and reticulocytosis and rise of Hb value after treatment with folic

acid In 5 of 8 patients with thalassaemia major(Cooley's anaemia). Values for folic acid in

serum after the vitamin had been given by mouth were lower in the patients than in normal

subjects (Luhby & Cooperman, 1961).

30
2.8. Management of thalassemia patients according to their micro-nutrient

deficiency:

2.8.1. Management of thalassemia patients according to Iron deficiency:

The cause of anemia in thalassemia patients is different from those who suffer from

iron-deficiency anemia. Therefore, eating iron-rich foods or taking iron supplements will

not treat thalassemia. On the contrary, as described above, those who go through blood

transfusion as a treatment for severe thalassemia can have excess iron level that is harmful

to the body because of the excess iron in the body (Chines community health resource

center, 2007).

There is a higher risk of oxidative damage. As a result, thalassemia patients are

advised to avoid iron-rich foods, such as spinach, beef, pork, lamb, liver, and dried beans

(Chines community health resource center, 2007).

2.8.2. Management of thalassemia patients according to Zinc supplementation:

This study is considered as a new approach which was studied by A. Arcasoy MD

(1987). Linear growth was evaluated in 32 patients with beta-thalassemia major. At the

beginning of the study of 40.6% of the patients were below the 10th percentile with

biochemical evidence of zinc deficiency. Effects of zinc supplementation on growth

velocity (height) were assessed in a controlled manner. Twenty-one children received oral

zinc sulphate for a period of 1 to 7 years (15 early- and 6 late-supplemented cases), while

the remaining 11 thalassemics were maintained only on conventional transfusion therapy.

The mean height velocity of early-zinc supplemented children was significantly greater

than that of normal children (P < 0.01). An increase in height was also observed in the

patients who received delayed zinc retardation. The present study demonstrated that zinc

deficiency is one of the factors responsible for retarded linear growth in beta-thalassemia

major. Only the patients who received zinc supplementation showed an acceleration of

31
growth in height. Administration of zinc could, therefore, be considered as an effective

adjuvant therapy in homozygous beta-thalassemia (Arcasoy, 1987).

32
Chapter-3
Hypothesis & Objectives

33
3.1. HYPOTHESIS

Macro & micro nutrient intake from 24 hours dietary recalls for three day lower than

requirements following current RNI according to respective age.

34
3.2. OBJECTIVES

3.2.1. General Objective

The aim of the current study was to determine the nutritional status and food

consumption level of thalassemia patients.

3.2.2. Specific Objectives

 To assess clinical and anthropometric measurements such as height, weight, BMI.

 To assess the food intake and nutrient intake by 24-hour dietary recall.

35
Chapter-4
Subject & Methods

36
4. SUBJECT & METHODS

4.1. Subject: Nutritional status & food consumption level of thalassemia patients

4.2. Study Design: It was a cross-sectional study.

4.3. Number of Sample: In this study, 600 patients were studied

4.4. Study Period: April, 2017 to December, 2017

4.5. Place of Work:

The study was conducted at Bangladesh Thalassemia Foundation which is located

at Green Road, Panthapath Signal. The study was also conducted at Bangladesh Institute

of Research and Rehabilitation in Diabetes, Endocrine and Metabolic Disorders

(BIRDEM) under the supervision of Senior Research Officer, Quamrun Nahar.

4.6. Variable of the study:

 Sex of the respondent

 Age of the respondent

 Anthropometric measurements

 Height (cm)

 Weight (kg)

 Body Mass Index (BMI)

 Socio- Economic information:

 Education Level of the parents

 No. of child

 Monthly family expenses

 Religion

37
 Biochemical Parameters:

 Hemoglobin (g/dl)

 Ferritin (ng/ml)

 Macro & micronutrient intake by the study subjects.

4.7. Data Collection System:

Data were collected by a predesigned questionnaire that included Anthropometric,

Biochemical and 24 hours dietary recalls.

4.8. Data Collection Method:

4.8.1. Development of questionnaire:

A standard questionnaire was developed to obtain relevant information regarding

dietary intake, anthropometric indices, socio-economic status, occupation of parents,

monthly income, educational level of parents, number of child, biochemical parameters

etc. In this study, the questionnaire was used for collecting data. The questionnaire is

included in the appendix.

4.9. Data Collection Process:

For collecting data, Bangladesh Thalassemia Foundation hospital was visited. After

having permission through written consent paper, the data were collected. The participants

(patients) were interviewed by the interviewer and asked to recall the 24-hour dietary

information.

4.10. Collection of anthropometric data:

To assess the nutritional status, the anthropometric measures such as height, weight etc

were collected by trained nutritionist with well-equipped machines.

38
4.10.1. BMI calculation:

Body Mass Index (BMI) of the subjects was calculated as the weight in kg divided by the

square of height in meters. The widely used BMI classification was followed in this study

to assess the nutritional status. Values for BMI of <18.5 were regarded as indicative of

underweight, 18.5-23 were regarded as indicative of healthy range, >23 were regarded as

indicative of overweight.

4.11. Data management:

Data editing was carried out by checking and verifying complete questionnaire at the end

of the interview and also at the end of the whole survey before analysis.

4.12. Statistical analysis:

Statistical analysis was performed using SPSS (Statistical Package for Social Science) for

windows version 16. All the data were expressed as M±SD (mean and standard deviation).

Besides some data were expressed as percent.

39
Chapter-5
Results & Observations

40
5. RESULTS & OBSERVATIONS
Table 1: Anthropometric parameters of the study subjects (n=600)

Variables M±SD

Height (cm) 127±29

Weight (Kg) 34±13

BMI (kg/m2) 21

The results were expressed as M±SD

M±SD height, weight, BMI among the study subjects were 127±29, 34±13 and 21

respectively.

140
127
120
Anthropometric value

100
80
60
40 34
20
0
Height (cm) Weight (kg)
Anthropometric variable

Figure 5: Average Anthropometric (height & weight) data of study subjects

41
Table 2: Monthly income parameter of the study subjects (n=600)

Variables M±SD

Income (TK) 12816±19568

The results were expressed as M±SD

M±SD income of the respondents’ parents was 12816 TK.

Table 3: Clinical parameters of the study subjects (n=600)

Variables M±SD

Hemoglobin (g/dl) 7.0±1.5

Ferritin (ng/ml) 3495±2086

The results were expressed as M±SD.

M±SD Hemoglobin and Ferritin level of the respondents were 7.0 g/dl and 3495 ng/ml

respectively.

42
Table 4: Cereals consumption by study subjects (n=600)

Variable Group M±SD

Rice 289±130

Ruti 34±43

Porota 32±77

Cereals (g) Khichuri 19±61

Potato 19±41

Noodles 11±26

Others(Puffed Rice, Puri, Chop, Singara, 25±95

Biscuits, Semolina)

The results were expressed as M±SD

M±SD rice consumed by study subjects was 289±130, ruti was 34±43, porota was 32±77,

khichuri was 19±61, potato was 19±41, noodles was 11±26 and others were 25±95.

350
300 289

250
Amount (g)

200
150
100
50 34 32 25 19 19 11
0
Rice Ruti Porota Others Khichuri Potato Noodles
Cereals (g)
Consumption of cereals

Figure 6: Consumption of cereals in gram by study subjects

43
Table 5: Meat consumption among study subjects (n=600)
Variable Group M±SD

Meat Chicken 30±35

(g) Beef 1.0±13

The results were expressed as M±SD

M±SD chicken consumed by study subjects was 30±35, and beef was 1.0±13.

Table 6: Fish consumption among study subjects (n=600)


Variable M±SD

Fish (g) 26±31

The results were expressed as M±SD

M±SD fish consumed by study subjects was 26±31.

Table 7: Milk products consumption among study subjects (n=600)


Variable Group M±SD

Milk & milk Milk 86±108

products (g) Curd 1.0±11

The results were expressed as M±SD

M±SD curd consumed by study subjects was 1.0±11 and milk was 86±108.

Table 8: Vegetables consumption among study subjects (n=600)


Variable M±SD

Vegetables (g) 87±70

Spinach (g) 30±57

The results were expressed as M±SD

M±SD vegetables consumed by study subjects was 87±70 and spinach was 30±57.

44
Table 9: Pulse consumption among study subjects (n=600)

Variable Group M±SD

Legumes (g) Pulse 15±14

The results were expressed as M±SD

M±SD pulse consumed by study subjects was 15± 14.

Table 10: Beverages consumption among study subjects (n=600)

Variable Group M±SD

Milk tea 283±577


Beverages (g)
Juice 4.0±21

The results were expressed as M±SD

M±SD juice and Milk tea consumed by study subjects were 283±577 and 4.0±21

respectively.

Table11: Fruits consumption among study subjects (n=600)

Variable M±SD

Fruits (g) 34±58

The results were expressed as M±SD

M±SD fruits consumed by study subjects were 34±58.

Table 12: Eggs consumption among study subjects (n=600)

Variable M±SD

Eggs (g) 24±30

The results were expressed as M±SD

M±SD eggs consumed by study subjects were 24±30 on average.

45
450 428
400
350 288
300
250
AMOUNT (G)

200
150 117
100
87
50 34 31 26 24 15
0

AVERAGE INTAKE OF MAJOR FOOD GROUP

Figure 7: Average intake of nine food groups in gram by study subjects

Table 13: Average intake of Energy, Macronutrients & Fiber by study subjects
(n=600)

Variable M±SD

Energy (Kcal) 751±251

Carbohydrate (g) 144 ± 49

Protein (g) 28± 13

Fiber (g) 11± 6

Fat (g) 5.0± 3

The result was expressed as M±SD

M±SD average intake of energy was 751±251. M±SD average intake of

carbohydrate, protein and fat were 144 ± 49, 28± 13 and 5.0± 3 respectively. M±SD

average intake of fiber was 11± 6.

46
Table 14: Average intake of Micronutrients by study respondents (n=600)

Variable M±SD

Thiamine (mg/day) 1.0±1.0

Riboflavin(mg/day) 0.3±0.1

Niacin (mg/day) 11±4.0

Vitamin C (mg/day) 18±23

Retinol (mcg/day) 85±106

Calcium (mg/day) 121±81

Iron (mg/day) 5.0±3.0

Folic acid (mcg/day) 72±48

Zinc (mg/day) 4.0±2.0

Magnesium(mg/day) 136±58

Sodium(mg/day) 86±51

Potassium(mg/day) 766±385

Phosphorus(mg/day) 408±158

The result was expressed as M±SD

M±SD average intake of thiamine, riboflavin, zinc and niacin were 1±1, 0.3±0.1,

4±2 and 11±4 respectively. M±SD vitamin c intake by study subjects was 18±23, iron was

5±3, sodium was 86±51 and magnesium was 136±58. M±SD value of average intake of

calcium, phosphorus, potassium were 121±81, 408±158, 766±385 respectively. M±SD

folic acid consumed by study subjects was 72±48 and retinol was 85±106.

47
15. Gender among the study subjects (n=600)

Variable Group Frequency %

Male 317 52.8


Gender (%)
Female 283 47.2

The results were expressed as number & percent.

Among the study respondents, about 52.8% participants were male & 47.2% participants.

were female.

47%
52.8, 53% Gender (%) Male
Gender (%) Female

Figure 8: Distribution of gender among study populations

Table 16: Disease types among the study participants (n=600)

Variable Group Frequency %

Disease types beta 566 94.7

(%) e-beta 34 5.7

The results were expressed as number & percent.

Among the study populations, about 94.7% participants has beta thalassemia & 5.7%

participants has e-beta thalassemia.

48
Table 17: District of study populations (n=600)

Variable Group Frequency %

Dhaka 469 78.2

Chittagong 53 8.8

Rajshahi 19 3.2

District (%) Khulna 23 3.8

Barisal 22 3.7

Sylhet 3 .5

Rangpur 11 1.8

The results were expressed as number & percent.

Among the study populations, Most of the study subjects (78.2%) were from Dhaka.

49
Table 18: Father’s educational level among the study participants (n=600)

Variable Group Frequency Percent

MSC/MBA/MA 104 17.4

BSC/BBA/LLB 88 14.7

Degree/BA 58 9.7

Father’s Diploma 7 1.2

Education (%) HSC 93 15.5

SSC 88 14.7

Under 10 88 14.7

Uneducated 73 12.2

The results were expressed as number & percent.

Among the study populations, the education level of 17.4% respondents fathers

were about MSC/MBA/MA, 14.7% respondents fathers were about BSC/BBA/LLB, 9.7%

respondent’s fathers were about Degree/BA, 1.2% participants fathers were about

Diploma, 15.5% respondents was about HSC, 14.7% .

50
Table 19: Mother’s educational level among the study participants (n=600)

Variable Group Frequency %

MSC/MBA/MA 50 8.3

BSC/BBA/LLB 30 5.0

Degree/BA 20 3.3
Mother’s
HSC 56 9.3
educational level
SSC 165 27.5

Under 10 117 19.5

Uneducated 162 27.0

The results were expressed as number & percent.

Among the study populations, 27.5% respondents’ mothers had obtained S.S.C.

Table 20: Father’s occupation among the study participants (n=600)

Variable Group Frequency %

Service 331 55.6

Business 156 26.2


Father’s
Farmer 34 5.7
Occupation (%)
Unemployed 44 7.4

Retired 20 3.4

The results were expressed as number & percent.

Among the study populations, Most of the (55.6%) respondents’ fathers were service

holder.

51
Table 21: Mother’s occupation among the study participants (n=600)

Variable Group Frequency %

Service 100 17.0

Mother’s Business 15 2.5

Occupation (%) Housewife 467 79.3

Retired 6.0 1.0

The results were expressed as number & percent.

Among the study populations, Most of the (79.3%) respondents’ mothers were housewife.

Table 22: Number of child of the participants parents (n=600)

Variable Group Frequency %

1 145 24.3

2 265 44.4

3 108 18.1

Number 4 44 7.4

of Child (%) 5 23 3.9

6 10 1.7

7 1 .2

9 1 .2

The results were expressed as number & percent.

Among the study populations, 44.4% participants’ parents had two children.

52
Table 23: Number of Parity of the participants parents (n=600)

Variable Group %

0 65.1

1 15.9

Number 2 9.2

of Parity (%) 3 4.5

4 1.9

5 2.1

6 1.4

The results were expressed as number & percent.

Among the study populations, 12.6% respondents’ parents had first children before

the respondents who were dead due to unwanted abortion & their parents were unaware

about the thalassemia.

Table 24: Order of Siblings among the respondents (n=600)

Variable Group Frequency %

1 254 43.3

2 246 41.9
Order
3 51 8.7
of Siblings (%)
4 33 5.6

6 03 0.5

The results were expressed as number & percent.

Among the study participants, 43.3% respondents were first child among their

siblings & 41.9% respondents were second child among their siblings.

53
Table 25: Father’s blood group of the respondents (n=600)

Variable Group Frequency %

A+ 152 25.7

A- 5 0.8

B+ 214 36.1

Father’s B- 4 0.7

blood group (%) AB+ 73 12.3

AB- 4 0.7

O+ 138 23.3

O- 2 0.3

The results were expressed as number & percent.

Among the study populations, 36.1% fathers’ blood group was B+.

Table 26: Mother’s blood group of the respondents (n=600)

Variable Group Frequency Percent

A+ 179 30.2

A- 6 1.0

B+ 222 37.5

B- 5 0.8
Blood group
AB+ 80 13.5

AB- 4 0.7

O+ 85 14.4

O- 9 1.8

The results were expressed as number & percent.

Among the study populations, 37.5% mothers’ blood group was B+.

54
Table 27: Blood group of the respondents (n=600)

Variable Group Frequency %

A+ 157 26.2

A- 4 0.7

B+ 240 40.1

B- 9 1.5
Blood Group
AB+ 78 13.0

AB- 3 0.5

O+ 101 16.9

O- 5 0.0

The results were expressed as number & percent.

Among the study populations, 40.1% respondents’ blood group was B+ & 26.2%

respondents’ blood group was A+.

Table 28: Carrier of the respondents (n=600)

Variable Group Frequency Percent

Others (Outside of 442 73.7

family)
Carrier (%)
Father 55 9.2

Mother 38 6.3

Both(Father & Mother) 65 10.8

The results were expressed as number & percent.

Among the study populations, 73.7% of others(Outside of family) work as a carrier

for the respondents, 9.2% fathers work as a carrier for the respondents , 6.3% mother work

as a carrier for the respondents & Both(Father & Mother) work as a carrier for the

respondents.

55
Chapter-6
Discussion & Limitation

56
6. DISCUSSION

Malnutrition has considerable health impacts on growth pattern, learning,

treatment and quality of life in thalassemic patients (Thavorncharoensap, 2010). The result

of the study showed that the nutritional status of the thalassemic patients was poor & their

average consumption of the macronutrients & micronutrients wasn’t sufficient. In this

study, anthropometry, biochemical parameters and 24-hour dietary intake were used to

evaluate nutritional status.

6.1.1. Nutritional status of the thalassemia patient:

A total of 600 patients were included in this study in where 52.8% participants

were male & 47.2% participants were female (figure 8). Among them, about 94.7%

participants has beta thalassemia & 5.7% participants has e-beta thalassemia (Table 16).

This data stated that in this study male patients were more in number than female patients.

Similar data was also found earlier by Wasi. Et al. (1985), Yagnik (1997) and Balgir (1996)

and reported 65.5, 56 and 62.1% of male patients respectively.

The data showed that mean weight of the patients was 34±13 (kg) which was lower

according to the age of them and mean height of the patients was 127±29 (cm). This

indicates that the patients had growth impairment.

This data stated that BMI level of thalassemic patients was 21 kg/m2. The ideal

BMI range was (18- 24.99) kg/m2 (According to WHO). This range indicates that the

nutritional status is normal. Though nutritional status of thalassemia patients was normal,

but the consumption of nutrients wasn’t sufficient according to the ideal requirements.

57
6.1.2. Food consumption level of Thalassemia patients:

This study indicates that M±SD consumption of energy, carbohydrate, protein, fat

& fiber were 751±251, 144 ± 49, 28± 13, 11± 6 & 5.0± 3 respectively. According to WHO,

the required amount of protein, fat, fiber & carbohydrate were 33.66gm, 20-35 gm, 30gm

& 400gm respectively.

The study also found that M±SD intake of thiamin, riboflavin, niacin, vitamin-c ,

retinol, calcium, iron, folic acid, zinc, magnesium, sodium, potassium5.0±3.0m &

phosphorus were 1.0±1.0, 0.3±0.1, 11±4.0, 18±23 ,85±106, 121±81, 5.0±3. 72±48,

4.0±2.0, 136±58, 86±51, 766±385 & 408±158.

According to FAO the requirement of thiamin, riboflavin & niacin is 1.2mg, 1.3mg

& 16mg. The requirement of retinol is 600mcg. The ideal body requirement for vitamin c

, calcium, iron, folic acid & zinc is 45mg, 1000mg, 19.3-20.5mg, 400mcg & 7mg. The

requirement of magnesium, sodium , potassium & phosphorus is 260mg, 2092mg,

3750mg & 700mg respectively.

The study clearly indicates that average requirements of macronutrients &

micronutrients is not sufficient for the thalassemia patients though there BMI level was

found normal in range.

58
6.2. LIMITATIONS:

 The online resources were not available enough for the topic specially in case of

Bangladesh.

 Some of the patients & their parents were not co-operative at all. Due to this it was

nearly difficult to collect the data.

59
Chapter-7
Conclusion & Recommendation

60
7.1. CONCLUSION

This study demonstrated that patients with thalassemia had growth impairment assessed

by anthropometric measurements. The biochemical parameters appeared lower than ideal

range. The dietary intakes are apparently inappropriate and lower than daily

recommendation.

61
7.2. RECOMMENDATION

 The nutrition improvement in both calorie and composition requirement should be

incorporated in to patient care to improve growth status in these patients.

62
Chapter-8
References

63
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69
ANNEXURE-1

List of Abbreviation

BMI Body Mass Index

Cm Centimeter

FAO Food and Agricultural Organization

WHO World Health Organization

Kcal Kilo Calorie

Kg Kilogram

M Mean

Mg Milligram

SD Standard Deviation

Mcg Microgram

Ng Nano gram

SPSS Statistical Package for Social Science

Hb Hemoglobin

70
ANNEXURE-2

TIME LINE

September

November

December
October
April
Works to be

May
accomplished

Selection of
the topic

Development
of the protocol

Methodology
of study

Data collection
& entry

Data editing &


analysis

Discussion,
writing &
conclusion

 Working week:

71
Questionnaires for assessment of nutrition status and nutrient
consumption of thallasemia patients

Case Study: Date:

1. Patient’s information:
a. Name:
b. Age (yrs):
c. Height
d. Weight
e. Zscore value
f. Gender: M/F
g. Admission Date:
h. Diagnostic Date:
i. Types of Thallasemia:
j. Duration of the disease:
k. Living Area:

2. Social status
a. Education Level of father
b. Education Level of Mother
c. Occupation of father
d. Occupation of mother
e. Age of father
f. Age of mother
g. Monthly Income
h. No of children :
i. No of parity :
j. Order of sibling :

72
3. Blood group :
Father :
Mother :
Patient :
Siblings :

4. Age of diagnose :
5. History of Thalassemia :
1st degree relatives
2nd degree relatives

6. Religious :
7. Disease complication :
8. History of chronic disease :
9. 24 hours dietary recall :
 Morning
 Snack
 Lunch
 Mid afternoon snack
 Dinner
 Before bed
10. Assessment of nutrient consumption both macro and micro nutrient
11. KAP regarding restricted food
List of restricted food
12. List of drug

Signature and Date

73