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GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

Chapter 5
Genetic Diseases in Bahrain
Shaikha Salim Al Arrayed, MBChB, DHCG, PhD.
Genetics Department, Salmaniya Medical Complex, Ministry of Health, Bahrain

Historical Background commercial centre on the major trade


routes between the East and West. With
The name Bahrain means the land of life geographic explorations and acquisition
or the sacred land, as mentioned in the of overseas colonies by the Europeans,
Sumerian Legends, and it was so named Bahrain became a battlefield and was
for the presence of water and agriculture, occupied by the Portuguese in 1907. At
a rarity in the Arabian Gulf. The state the time when India became an important
of Bahrain is made up of an archipelago part of the British Empire, Bahrain was
of 40 Islands in the Arabian Gulf. The strategically located on the route to India.
Kingdom of Saudi Arabia is on the west With the passage of time, its location
and Qatar on the East. The total land area contributed to an increased exchange
of the kingdom is 720.14 km2. The largest with various parts of the world. Rapid
island among these 40 islands is the economic progress with the advent of
island of Bahrain, which represents 85% Independence in 1971 made Bahrain a
of the total land area (Central Statistics diversified industrial base and a major
Organization, 2006). banking centre with a number of reputable
international and offshore banks. Bahrain
The geographical, religious and socio- is now (1) an important communication
cultural aspects of Bahrain play an centre between the East and West, and has
important part in the influence of genetic been (2) an important military base from
association with environmental factors the 2nd World War till today. All these
on the people of Bahrain. History clearly factors highlight an infiltration of foreign
shows that from the Stone Age until the cultures and civilization through time,
advent of Islam, Bahrain was influenced giving a cosmopolitan blend of genes
by the Babylonian, Assyrian and Greek mingling with the local population.
civilizations amongst other Ancient
civilizations. The eastern coast of the The foundation of the modern health
Arabian Peninsula was the one most services dates back to 1900 when the
influenced by these cultures; the Greek first hospital was established. The first
influence extended to the southern-most Government doctor was appointed in
part of the Arabian Gulf, profoundly 1925 and a full- fledged government
affecting Bahrain. The Sumerian Legends clinic was set up 11 years later. In
gave a clear mention of the Bahrainis as 1968, a comprehensive health services
the people of Dilmun. However, around development plan was drawn up by the
500 B.C., Greek and Roman geographers government in co-operation with the
called Bahrain as 'Tylos". It is maintained W.H.O, and this was put into operation in
that it was basically the pearls and precious 1970. The present Government provides
stones of Bahrain that gave the Island its free comprehensive health care to all the
name and fame. residents of Bahrain, citizens and non
citizens in a two-tier system. Primary
The Arabian Gulf was responsible for Health Care Centers are drained into
carrying out sea trade and from the dawn various clinical set ups such as Salmaniya
of time Bahrain became an important Medical Complex (SMC).

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Genetic Diseases in Bahrain

A Public Health Directorate is responsible on the sands of time (Central Statistics


for the prevention and control of diseases. Organization, 2006; Bahrain Health
Comprehensive programs for general Statistic Reports, 1985-2005).
sanitation, water purification, hygiene,
health, and education, have led to a
decreasing trend in various contagious Demographic Data
diseases.
According to the demographic data of
Presently, the Health system delivery 2005, the population of Bahrain was
maintains a partnership between both the estimated to be 724,645; close to the data
government and private sectors. In line of 586,110 in 1995. The proportion of
with this, the Ministry of Health (MOH) Bahrainis to Non Bahrainis has remained
has concomitantly played a major relatively constant over the last 10 years.
role in the provision, improvement, In 2005, Bahrainis made up 61.9% of the
and sustenance of quality health care population, whereas 38.1% were non-
services. Health facilities have improved Bahrainis. The crude birth rate in 2005
rapidly during the past five years. This was 20.9/1000. The infant mortality
can be witnessed clearly through the rate of live births was 8.9/1000. The
remarkable evolution with regard to the percentage of people under 15 years was
type and quality of the services offered at estimated as 40.3% while in the 15-64 age
Salmaniya Medical Complex (the major groups it was 56%. However, in the age
hospital in Bahrain). group of above 65, it was 3.4%.

The construction work on the new King Non-communicable diseases, especially


Hamad General Hospital in Al Muharraq genetic diseases, are assumed to be a
Governorate was initiated in February major cause of morbidity and mortality.
2006. This hospital project is anticipated Genetic disorders are a significant
to be a huge training medical centre for burden on health care delivery systems.
the adjacent Medical University, which Their chronic nature requires life-long
will be built later in the future. The medical attention, expensive supportive
expansion of the services in Bahrain was and symptomatic therapy and specialized
not limited to the Secondary Health Care, care. Effective control of these diseases
but it even catered to the Primary Health requires that their natural history,
Care in order to maximize the capacities frequency and distribution be studied
and accessibility to the services in Primary (Bahrain Health Statistics Reports,
Health Care. 2005).

In brief, Bahrain has been subjected to In 1983, a genetic unit was established at
various influences from a number of the Salmaniya Medical Complex (SMC).
different civilizations due to its geographic A national committee for the control of
location. Mingling of populations over hereditary diseases was established in
generations has led to various social and 1993. The aim of the committee was
cultural customs in the Bahraini people. to conduct population studies on the
An inherent desire of this small country to prevalence of genetic diseases within the
preserve its identity as well as its religious country, and to improve management and
and social culture may have planted treatment standards of patients suffering
the seeds of consanguineous marriages from these diseases. During these years,
leaving behind its genetic footprints many studies have been carried out.

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GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

Consanguinity in Bahrain including 26,723 participants. The overall


consanguinity rate was estimated at 32%,
Consanguineous marriage has been a including 24% for first cousin marriages,
historically long-standing practice among hence marking a significant gradual
the different social classes of Bahrain. decline in the rate of marriages between
Several studies were performed to relatives (BNFHS, 1995).
determine the rate and to study the trend
to see if the frequency of cousin marriages The third study was a neonatal screening for
has changed over time. The following are hemoglobinopathies performed in 2002,
the observations of some of these studies including a questionnaire with the intent
conducted. to gather information about consanguinity.
Marriage between relatives occurred at the
In 1990, the first study was performed on rate of 20%, while the rate of first-cousin
a group of 500 young married Bahraini marriages was 12.5%. Marriages between
women in which each participant was distant relatives comprised a total of 7%
asked to complete a standard questionnaire, (Al-Arrayed, 2005).
which included questions about the family
relationship between the husband and the The fourth figure came from the
wife, and the relationship between their premarital counseling study conducted
parents. The questionnaire thus provided in 2006, where 500 couples participated
information about 1000 couples back in the study, which revealed the
to the grand parents’ generation. The consanguineous marriage rate to be 20%,
rate of cousin marriage was estimated with 11.4% being marriages among first
to be 39.4% in the present generation cousins, and 8.6% among distant relatives
(fathers and mothers) and 45.5% in the (Table 5.1). About 80% of couples were
previous generation (grand fathers and non-related; hence, a gradual decline
grand mothers), indicating a high rate of in the consanguinity rate was observed
consanguinity, with a significant decrease (Al-Arrayed and Al-Hajry, unpublished
over time. The rate of first-cousin observations).
marriage was evaluated as 21% (Al-
Arrayed, 1995).
Genetic Blood Disorders
In 1998, through a National Family
Health Survey (BNFHS ‘95), a total Bahrain has been one of the first countries
of 4,166 households were interviewed, in the region to tackle the issue of genetic

Table 5.1. Prevalence of consanguineous marriage in Bahrain (adapted from Al Arrayed, 1995; BFHS, 1995;
Al Arrayed, 2005; Al Arrayed and Al Hajry, unpublished observations).

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Genetic Diseases in Bahrain

blood disorders since 1986. Genetic blood be 2:1, with 60% of the group in the age
diseases are frequent in Bahrain as in all range 15-30 years. Extremity pain was the
the Middle Eastern countries. A history most common presenting feature (86% of
of malaria characterized the gulf region patients), followed by pain in the abdomen/
where it was endemic until eradicated in generalized body ache (71%). Most
1970, resulting in frequent manifestation patients (83%) responded to treatment
of malaria related diseases. with hydration, narcotic analgesics or
non-steroidal anti-inflammatory drugs,
and were discharged from hospital.
Sickle Cell Disease The manifestations suggested that the
Bahrainis have a mild form of SCD (Al-
Previous neonatal screening in 1984- Arrayed and Hamza, 1995).
1985 showed that the birth prevalence
of SCD was estimated to be 2.1%, with Molecular genetic studies were
SCT being 11% and glucose-6-phosphate undertaken to determine the haplotypes
dehydrogenase (G6PD) deficiency being of chromosomes carrying the sickle cell
25% (Mohammed et al., 1992; Nadkarni allele in Bahraini patients, and hence
et al., 1991). In 2004, (SCD) was found to consider the possible origin of these
to be the first cause of admission to SMC. alleles (Al-Arrayed, 1995). The beta S
The number of hospital admissions due gene was found to be linked to the Asian
to SCD gradually increased with 2600 haplotype in 90% and to the S2 haplotype
patients admitted during 2005, which in 5% of the cases. The Bantu haplotype
formed 6.5% of the total admissions to was found in few patients (only 2.5%),
SMC (Bahrain Health Statistics Reports, and the haplotype was found in association
2004-2005). with β-thalassemia chromosomes in 2.5%
of the cases only. The study showed
Several studies have been conducted that the Asian haplotype which is linked
on genetic blood disorders in Bahrain. to a benign form of sickle cell disease is
One such study depicted the nature of predominant in Bahrain (Al-Arrayed et
SCD among the population. The finding al., 1995).
revealed exposure to cold to be the
principal precipitating factor of painful Jassim and Al-Arrayed (2006) reported
crisis (45% of cases), followed by fever on the molecular basis of the benign form
(35%). and exhaustion (35%) being the of sickle cell/beta-thalassemia syndrome
next most common. Moreover, the most in two healthy Bahraini patients. The
common symptoms were pain and fever. aim was to study the different molecular
The main signs were anemia (92%), determinants that might cause an extremely
hepatomegaly (64%), jaundice (64%), and mild form of sickle cell/beta-thalassemia
urinary tract infection (30%; Al-Arrayed syndrome among the population. Two
et al., 1994; Al-Arrayed et al., 1995). healthy Bahraini students belonging to two
unrelated families with normal clinical
Another study sought to evaluate the pictures were noticed to have the sickle
clinical presentations, and management of cell/beta-thalassemia syndrome through
200 Bahraini SCD patients who attended hemoglobin electrophoresis. Different
the SMC Accident and Emergency molecular genetic techniques were
Department for Vaso-occlusive crisis employed to study blood samples from
during the period January-March 1994. these girls. Three different molecular
The male: female ratio was estimated to determinants were found in these students:

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GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

compound heterozygosity for the sickle A), IVS-I-110 (G-to-A)] and the Indian
cell mutation and beta thalassemia subcontinent [CD8/9 (+G), CD 15 (G-
mutation. Their corresponding haplotypes to-A), CD 41/42 (-TCTT)] were also
were the Saudi-Indian haplotype for the reported.
sickle cell mutation and–88 (C-to-A)
beta-thalassemia mutation. Alpha-globin
gene mapping revealed homozygosity for Alpha Thalassemia
the rightward deletion (-α3.7/ -α3.7/) for
both the students. The study concluded Newborn screening in 1985 showed a
that different molecular determinants high frequency of alpha-thalassemia in
were found in association with the mild Bahrain at about 24% (Mohammed et al.
form of sickle cell/beta-thalassemia 1992; Nadkarni et al., 1991), but only a
disease, namely inheritance of a mild few patients presented with manifestation
β+-thalassemia mutation, an HbS of Hemoglobin H (HBH) disease. There
haplotype-associated high HbF expression, were no reported cases of hydrops fetalis.
and coinheritance of alpha-thalassemia.
All of these modulators were responsible Jassim et al. (1999 and 2001) reported
for such a mild state of sickle cell disease on the molecular basis of α-thalassemia
in these patients. in Bahrain. Various polymerase chain
reaction (PCR)-based methodologies
were involved, namely differential PCR
Beta Thalassemia amplification, PCR-restriction fragment
length polymorphism (PCR-RFLP), and
Studies have indicated that the incidence direct PCR-amplified genomic DNA
of beta-thalassemia among Bahraini sequencing. Five alpha-thalassemia
students is 0.02% while the trait rate determinates were identified, which
remains between 2.5 and 3.5% (Al- included three deletional types, the
Arrayed et al., 2003). The number rightward 3.7 kilobase (kb) deletion,
of Bahraini babies born with beta the leftward 4.2 kb deletion, and
thalassemia has shown a decline from the penta nucleotide deletion in 5’
four babies annually to two during the splice donor side of intron I in the
same period of 20 years. alpha 2-globin gene (GGTGAGG
GG…), and two non-deletional alpha-
Jassim and colleagues (2000) studied a thalassemia determinants, the Saudi type
total of 80 Bahraini individuals of whom polyadenylation (polyA) signal mutation
35 were transfusion-dependent beta- (AATAAA AATAAG) and the Turkish
thalassemia major patients, 37 presented type polyA signal mutation (AATAAA
with the trait and eight were sickle cell/ AATGAA) in the alpha 2-globin gene.
beta-thalassemia patients. The application The study concluded that the three
of different molecular techniques on 67 alpha-thalassemia mutations, the Saudi
beta-thalassemia alleles identified 12 type polyA signal mutation, the penta-
different mutations. However, IVS-I-3’ nucleotide deletion and the rightwards
end (-25bp), CD 39 (C-to-T), IVS-II-5 3.7 kb deletion accounted for 97% of
(G-to-C), IVS-II-2 (G-to-C), and IVS-II-2 all alpha-thalassemia determinants in
(G-to-A) accounted for more than 80% of Bahrain. Clearly, the interaction between
the total studied alleles. Other mutations the deletion alleles and the non-deletion
common both to the Mediterranean alleles provides a complex picture in the
basin [IVS-I-1 (G-to-A), IVS-II-1 (G-to- phenotype.

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Genetic Diseases in Bahrain

Al-Mukharraq (1999) studied hemoglobin subjects with deficient and normal G6PD
H (HBH) disease in 26 Bahraini patients, activity. The study showed that nearly
followed by an assessment of the severity 90% (93/102) of the X chromosomes from
of anemia. The mean age at diagnosis G6PD deficient subjects had nt 563 (C-to-
was estimated to be 6.7 years. The mean T; G6PD Med) mutation, whereas ~9% of
hemoglobin was 8.1g/dl, and the mean the X chromosomes from G6PD deficient
HBH was 17.4%. Fifty percent of these subjects might have had other G6PD
patients required blood transfusion, variant(s) or normal X chromosomes in
seventeen children had average growth, heterozygote females. Ninety-six percent
while nine were below average. There (89/93) of the G6PD Med-bearing X
were no significant thalassemic bone chromosomes showed thymine (T) at
changes or splenomegaly observed; thus nucleotide position 1311. In contrast, 70%
depicting a mild to moderate clinical (82/117) of the normal X chromosomes
picture. showed cytosine (C) at nucleotide position
1311, while it was thymine (T) in 30%
(35/117) of the normal X chromosomes.
Glucose-6-Phospate The study concluded that the vast
Dehydrogenase Deficiency majority (91%) of X chromosomes from
G6PD-deficient subjects in Bahrain are
Glucose-6-phosphate dehydrogenase harboring the nt 563 (C-to-T) mutation.
(G6PD) deficiency, a genetic defect The G6PD Med variant in Bahrain is in
underlying a biochemical red cell tight linkage disequilibrium with thymine
abnormality, occurs worldwide with (T) at nt 1311. These data revealed a
variable incidence and clinical presentation high molecular homogeneity of G6PD-
among different ethnic groups. The deficiency in Bahrain.
disease may express itself as a drug-
induced hemolytic anemia with neonatal
jaundice, hemolytic anemia following Hemophilia
infection, chronic non-spherocytic
hemolytic anemia, favism or may be Hemophilia is an X- linked condition
asymptomatic. G6PD-deficiency is a sex- common in some Arab countries, but not
linked disorder. The incidence of G6PD- common in Bahrain. Currently, only 10
deficiency has been observed to be higher patients are under treatment at SMC.
in the Arab World, and is in the range of
8% to 50% in different populations. The
frequency in Bahrain is between 20-23%, Frequencies of ABO Blood Groups
but with little clinical problems (Bhagwat and Rh Types
and Bapat, 1987; Mohammed et al., 1992;
Al-Arrayed et al., 2003). Al-Arrayed and colleagues (2001)
estimated the frequencies of ABO and
Al Momen et al. (2004) reported on Rh phenotypes along with the respective
the molecular homogeneity of G6PD gene frequencies in two study groups: (1)
deficiency in Bahrain. DNA extraction 5675 Bahraini school students in the age
was done for 83 G6PD-deficient subjects group of 16-20 years and (2) 7362 adult
and 80 normal controls. Combinations of Bahraini blood donors. The frequencies of
PCR-RFLP and PCR-DGGE procedures ABO groups in both study groups showed
were employed to uncover the sequence Group O>Group B>GroupA>Group AB;
variations at nt 563 and nt 1311 in both where group O contributed to nearly

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GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

50% of the total. Both study groups also marry to undergo screening by history
showed >90% frequency of the RhD taking, physical examination and
positive phenotype. In conclusion, the further laboratory investigations. In
frequencies of ABO and Rh phenotypes 1993, a voluntary premarital counseling
in Bahrain were similar to those reported (PMC) service was established, which
from other countries in the Arabian Gulf successfully contributed towards reducing
region. These frequencies appear to be the number of affected newborns, thereby
intermediate between the frequencies seen reducing morbidity and mortality rates.
in Europe and Southeast Asia. More recently a law (Government Gazette,
2004) has been passed by the Bahraini
Government which requires all Bahraini
Campaign to Control Hereditary couples planning to marry to undergo a
Blood Diseases mandatory premarital counseling. Any
citizen about to get married, even if the
Bahrain successfully implemented a spouse is non-Bahraini, must now undergo
campaign to control hereditary blood a premarital check up, which includes
diseases. This campaign included hereditary, infectious and other diseases
education and public awareness events, according to the regulation issued by H.E.
as well as antenatal, carriers, students, the Minister of Health. According to the
premarital, newborn, and prenatal results of the check-up, couples are given
screening methods followed by genetic advice to undergo further investigation
counseling. if needed, including treatment, health
education, and counseling. Couples at risk
Information booklets were prepared and of hemoglobinopathies are referred to the
distributed widely in schools and to the genetic department of SMC.
public in an attempt to increase awareness.
In 1991, the Bahrain Hereditary Anemia In addition, couples are subjected to
Society was established to further strengthen counseling regarding high-risk behaviors,
these efforts. In 1993, a premarital including those related to HIV, Hepatitis
counseling (PMC) service was started, and B, and other infectious diseases, which
in 1998, a student-screening project was facilitate early detection and treatment of
initiated (Al-Arrayed, 1997). Additionally, some sexually transmitted diseases, and
attempts aiming at screening Bahraini help providing immunizations, as required.
newborns for these genetic diseases will It also promotes awareness regarding
soon be put into practice. Also, a molecular reproductive health, family planning, and
genetic laboratory has been established to healthy lifestyles, and provides couples
study the genotype of difficult cases. As with medical, social, and psychological
a consequence of all these efforts, which support, when needed.
continued for about 20 years, evidence has
shown a decline in the incidence of sickle After successful counseling of both
cell disease by 50-60% from 2.1%-0.9% partners, certificates are issued, therefore
leaving the decision of marriage to the
couple even if they are not inclined to take
Premarital Counseling Services the advice with respect to the genetics
aspect. At-risk couples are followed
Premarital counseling is intended upon, if they decide to marry and all sorts
to identify couples at high risk, by of medical care are extended to their
subjecting all the couples intending to children.

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Genetic Diseases in Bahrain

The development of this law included Student Screening


wide consultation with all stakeholders
to ensure that socio-cultural mores, The student screening for genetic blood
theological issues, and aspects of diseases started in 1999. Covering about
human rights had been considered. The 50,000 students until now, the aim of the
possibility of issuing a law making the program is to raise awareness among the
premarital counseling obligatory was younger generation about these diseases
raised by the Ministry of Health with through a comprehensive educational
the Shura Council (a consultative body) campaign, followed by screening. It also
after a thorough discussion, with the identifies carriers, thereby empowering
subsequent approval of the ministerial them to make informed reproductive
cabinet and was then forwarded to the decisions in the future. The project
Parliament, to pass into law in 2004. In operates for about six months on an
view of its high degree of sensitivity, average every year for planning, education
the draft law was subjected to extensive sessions, blood collection, laboratory
consultation involving a wide range testing, and data processing, distribution
of stakeholders, and subsequently it of report cards, data analysis, and delivery
received positive support and backing of results.
from the Islamic clergy. The WHO
guidelines and recommendations The public was initially resistant to the
(WHO, 1983) were followed at all screening program, because they feared
stages of the project. In addition, the that girls identified as carriers would not
recommendations of the Genomics and be able to find a husband. The education
World Health Report (WHO, 2002), campaigns, therefore, stressed that (a)
providing guidance on the ethical, all people carry some defective genes;
legal and social implications (ELSI) in (b) carriers could avoid the risk of
genetic screening, while laying stress giving birth to an affected child if they
on the key points of the informed married a non-carrier; and (c) no one,
consent, confidentiality, stigmatization including carrier–carrier couples, would
and discrimination, were carefully be prevented from marriage. Broad based
considered prior to implementation of public campaigns were also required to
the new law. encourage parents and students to agree to
undergo screening.
During 2005, which was the first year
of the implementation of the law, 9107 The screening is equally applicable for
clients underwent observation. Out of both male and female students. Testing
these, 97 (1%) cases presented with is stated to be voluntary and permission
sickle cell disease and 1176 (12.9%) were of the parents is sought before screening,
carriers of the disease. A beta-thalassemia or directly from the students in case
major status was reported in 19 patients they are eighteen years or older. So far,
(0,002%) and 239 individuals (0.03%) approximately 80–85% of parents and
were carriers for beta-thalassemia. In students have voluntarily agreed to be
addition, G6PD deficiency was reported tested each year.
in 2037 patients (22%). These frequency
figures were similar to those obtained In order to maintain privacy, test results
from a student screening program are returned to students on a standardized
(Bahrain Health Statistics Reports, 2005; official medical report card and are
Al-Arrayed, 2006). delivered via their school in a sealed

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GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

envelope. In order to further protect their et al., 2003; Figure 5.1). The study showed
privacy and confidentiality, students are that the frequencies of HbD A= 0.66%,
encouraged to open their envelopes at HbD D= 0.02%, HbE E= very rare, and
home with their families. Certainly, this HbE A= 0.13%. Surely, the benefits and
system also helped them in choosing their effects of these measures will be seen in
appropriate future partners. the improved genetic health of present and
future generations (Al Arrayed, 2005).
Each school receives reports on the
prevalence of the three diseases in their
student body. At the same time, the program Congenital Abnormalities
also ensures that the Ministry of Health
obtains accurate and updated statistics, The incidence of congenital abnormali-
which assists them in planning for future ties in Bahrain was observed in two dif-
services. The Ministry of Education also ferent periods. The first study was car-
observes the prevalence of these diseases ried out to determine the incidence of
among students, enabling them to take these anomalies in Bahrain by referring
necessary steps to ensure improved health to the Ministry of Health statistics for
of the students. The study also yields the 11-year period, 1980 to 1990, while
important information about the frequencies the second study looked at the same ab-
of different types of abnormal hemoglobin normalities in the period 1991 to 2000
levels among the population (Al-Arrayed (Tables 5.2 and 5.3).

Figure 5.1. Prevalence of sickle cell disease (SCD) and sickle cell trait (SCT). Data obtained from the
student screening program for genetic blood disease (1999-2004).

Table 5.2. Average annual incidence rates of congenital anomalies (per 1000 births) in Bahrain for the periods
between 1980-1990 and 1991-2000.

ANOMALY

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Genetic Diseases in Bahrain

The incidence of congenital anomalies in neonates to be affected by metabolic


the first study was found to be 2.7% of diseases such as maple syrup urine disease,
live births, while it was found to be 1.7 medium-chain acyl-Co-A dehydrogenase
% in the second study. A study of each deficiency, primary carnitine deficiency,
anomaly separately showed that anomalies methylmalonic acidemia, and methylene
of the musculoskeletal system had the tetrahydrofolate reductase deficiency.
highest incidence (2.28 per 1000 and Many of these affected babies died within
3/1000), followed by the genitourinary the first or second week, even before the
system (2.13 per 1000 births and 2/1000). diagnosis can be established.
The incidence of chromosomal disorders
in the first study was 0.9 per 1000 births Al Jishi (unpublished observations)
while in the second it was 1.2 /1000, (Al- found an approximate prevalence of
Arrayed, 1995). 1/10,000 for PKU, propionic acidemia,
and fatty acid oxidation defect (CPTII);
2/10,000 for MSUD, isovaleric acidemia,
Metabolic Diseases 3-methylglutaric acidemia, and glutaric
aciduria 11; and 6/10,000 for methyl
Al-Arrayed (1995) highlighted the malonic acidemia, glyceric acidemia , and
frequency of metabolic diseases. In line arginosuccinic aciduria.
with this, a neonatal metabolic screening
pilot study was performed on 1000 Some other metabolic diseases that
neonates, in which electro spray tandem are frequently observed in Bahrain are
mass spectrometry was applied. Blood mucopolysacharidosis (MPS), Neimann-
samples were collected on Guthrie cards Pick disease, GM2 gangliosidosis,
from the heels of infants on the 3rd to 5th Sanfilippo disease, I cell disease,
days of their birth. The dried samples glycogen storage disease, biotinidase
were mailed to the mass spectrometry deficiency, and mitochondrial disorders,
laboratory at King Faisal Specialist but further studies are needed to
Hospital, where they were analyzed determine their prevalence (Al-Arrayed
for more than 20 metabolic diseases, et al., 1999).
such as amino acids, organic acids and
carnitine esters. The results showed a These diseases have also been reported
high incidence of metabolic diseases. from other Arab countries. Ozmand et
Annually, we expect approximately 100 al. (1990) reported that certain lysosomal

Table 5.3. Annual


Annualincidence ratesrates
incidence (per (per
1000 1000
births)births)
of different anomaliesanomalies
of different observed between 1991between
observed and 1991 and 2000
2000 (adapted from the Bahrain Health Statistics Reports, 1991-2000).
(adapted from the Bahrain Health Statistics Reports, 1991-2000).

ANOMALY 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 INCIDENCE AV.
Neural tube defects 3.3 3.2 2.2 2.2 2. 2.6 2.1 3.6 3 2.6 2.68
Congenital heart 2.7 2.2 5 3.7 3.1 1.8 4.2 3.6 4.8 2.4 3.35
Respiratory 0.4 0.2 0.4 0.16 0.15 0.23 0.46 0.23 0.06 0.6 0.29
Cleft palate 1.3 1.5 0.98 0.8 1 0.7 0.6 1.3 0.9 0.2 0.9
Gastroitestinal 1.7 1.1 1.4 0.9 0.7 0.4 0.8 0.7 1.1 1.6 1
Genitourinary 2.2 3.1 2.3 2.24 3.3 1.6 1.7 2.4 1.3 0.96 2
Chromosomal 1.3 1.4 1.2 1.9 1.1 0.86 1.7 1.3 0.9 1.2 1.2
Musculoskeletal 5 4.3 3.2 2 3.5 1.9 2.3 4 3.7 0.73 3
Skin 0.5 0.1 0.2 0.5 1.1 0.4 0.3 0.2 0.2 0.14 0.35
Others 1.7 0.1 0.7 1.3 1.5 3 1.9 5.2 0.3 0.2 0.16
Total 16.4 per 1000

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GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

storage diseases including. MPS was 5 years, with 60% of patients under
type 1VA, Morquio disease, Multiple 1 year of age; the oldest patient was 31
sulphatase deficiency, Niemann-Pick years old. The most common complica-
disease type B, GM1 gangliosidosis tions were chest infections, congenital
type “0” (Sandhoff disease), and ceroid heart disease, increased susceptibility to
lipofuscinosis (Jansky Bielschowsky and all types of infection, anemia, and ear,
Batten Spielmeyer Vogt syndrome) are nose and throat and eye complications.
more frequent in Saudi Arabia. Another Karyotype analysis showed that 97% of
study (Benson, 1988) found that MPS those studied had free trisomy, 2% had
type II (I cell disease) was found to translocation and one patient had a mosaic
occur more frequently in Arabs than in karyotype.
any other ethnic group. Circumstances
demand that frequencies of these A slight increase in trisomy 13 and
diseases is kept track of, since most of trisomy 18 births has been noticed during
these diseases may be diagnosed in the the last few years. Fragile X syndrome
prenatal period, and affected individuals was reported in five Bahraini families (Al-
could receive potentially treatment by Arrayed, 1990).
bone marrow transplantation (Whitley et
al., 1980; Schaison et al., 1989; Teebi et Al Arrayed (1996) also reported on
al., 1987; Wraith et al., 1987). cytogenetic studies on 500 Bahraini
patients suspected of having chromosomal
abnormalities on the basis of physical
Chromosomal Abnormalities and/or developmental clinical features.
Almost 27% of these patients were found
The incidence of chromosomal to have abnormal karyotypes. Numerical
abnormalities was found to be relatively abnormalities (including trisomy 21) were
low in Bahrain. The incidence of Down’s found in 19% of patients, whereas structural
syndrome among Bahrainis in 1993 was abnormalities manifested in 7%.
found to be 0.9 per 1000, compared
with 1.4 per 1000 internationally while Al-Arrayed (2003) studied inheritable
in 1991-2000 it was found to be 1.2 per chromosomal abnormality causing
1000 (Al-Arrayed, 1996). The number recurrent fetal wastage and abnormalities
of confirmed trisomy 21 cases in the in children. Cytogenetic studies
Cytogenetic laboratory during 2004 and were performed on Bahraini patients
2005 among 15000 newborns was 12 and suspected of having chromosomal
17 respectively, indicating a prevalence of abnormalities. Ten families inheriting
0.8/1000, and 1.1/1000, respectively. different chromosomal abnormalities
were reported.
Al-Arrayed and Rajab (1995) studied
the data collected retrospectively of 104 The first family studied had one child
Bahraini patients presenting with Down’s and had six abortions. The chromosomal
syndrome from hospital records during analysis showed interesting results. Both
the period 1989-1993, which included all wife and husband presented with different
patients suffering from Down’s syndrome chromosomal abnormalities. The wife had
admitted to the main hospital in Bahrain 45, XX, der (13; 14) (q10; q10), whereas
during the 5-year period. Cytogenetic the husband had 46, XY, inv (3) (p25q21).
analysis had been performed on 89 pa- However, their child was phenotypically
tients. The mean age of patients admitted normal, though he inherited abnormal

82
Genetic Diseases in Bahrain

chromosomes from both parents. The stillbirth(s), or live-birth(s) with multiple


second family had three mentally retarded congenital anomalies. One couple
children with Trisomy 21. The third was found to be carriers for a balanced
family had a male child with 46, XY, rec reciprocal translocation. A woman was
(8), dup p, inv (8) (P23. 1 q 24. 2). He found to have Robertsonian Translocation,
inherited the abnormal chromosome from while another woman was found to be a
his mother. In the fourth family, the patient mosaic (46, XX, 47, XXX). Moreover,
was married to her first cousin. She had one husband was found to have pericentric
4 p 3 D 2 A1L1. Both the wife and the inversion of Y chromosome. These
husband had the same balanced reciprocal findings reveal that parental chromosome
translocation, t (6; 10) (q 15; q 21.2). abnormalities account for fetal wastage in
Their retarded baby girl presented with the 8% of couples having such a history.
same chromosomal abnormality. The fifth
family had an encephalic stillbirth and an
abortion. The husband had a pericentric Cystic Fibrosis
inversion of the Y chromosome. The sixth
family had two children with trisomy 21; Cystic fibrosis is a hereditary multi-
the mother presented with mild Down’s system disease transmitted as an
syndrome features. The seventh family autosomal recessive disorder. It leads to
had four children, all mentally retarded chronic pulmonary diseases, pancreatic
with fragile X syndrome. The father was enzyme deficiency and abnormally high
declared as the transmitting parent. The concentrations of electrolytes in sweat
eight family had a mentally retarded child (Khan, 1985).
with fragile X syndrome. The mother and
five of her sisters were carriers for fragile Al-Arrayed and Abdullah (1996)
X and the grandfather was observed to performed an intensive retrospective
be the transmitting parent. In the ninth search for patients with cystic fibrosis
family, the father’s karyotype was 46, XY, from clinical data and hospital records
inv. per. (2) (p12:q14). He had a balanced from SMC, with the aim of determining the
pericentric inversion of chromosome 2. prevalence of cystic fibrosis in Bahrain.
His first child was abnormal with trisomy The survey included 27 patients confirmed
13. In the tenth family, the father had 46, as having cystic fibrosis, born during the
XY/46,XY,rea (2). The family had three period 1978—1994. Almost 200,000
abnormal mentally retarded children and children were born in Bahrain during this
had two abortions. This indicates that period. Diagnosis was established by the
inherited chromosomal abnormality is presence of high sodium and chloride
not a rare condition, and that cytogenetic (≥ 70 mmol/L) concentrations in sweat.
studies should be conducted for any The mean incidence during this period
family with abnormal children and with was found to be 1 in 7700. All cases
recurrent fetal wastage. were diagnosed during the first year of
life, and 60% were diagnosed in the first
Al-Arrayed (1990 and 2003) reported 3 months of life. The male:female ratio
on a consecutive series of fifty couples was 14:13. The incidence of meconium
with history of fetal wastage. They were ileus was 16%. Mortality in the neonatal
cytogenetically studied with current period was 60%. The first–cousin
banding techniques. Fetal wastage was marriage rate among theses families was
defined to be occurring in the couples who 63%. Another study in 1998 reported
had had more than two early abortions, the incidence of cf as 1/5800. The gene

83
GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

frequency seems to be low in Bahrain, Ahmed and Selvyn (1988) found that
while it is high in Caucasian population consanguinity rate was positive in 42%
where the carrier frequency is about 1:20 of families with children with visual
to 1:25 (Warwick, 1978). disability, but the consanguinity rate in
the general population at that period was
Eskandarani (2002) undertook a genotyp- estimated to be 40%.
ic study to characterize the cystic fibrosis
transmembrane regulator gene mutations
(CFTR) in the Bahraini cystic fibrosis Hereditary Deafness
(CF) population using a polymerase chain
reaction-based direct gene test to search Jamal examined 196 children presenting
for 15 common CF mutations amongst with deafness and speech delay, who were
Arabs. During the period October 2000 referred to hearing impaired children clin-
to May 2001, 19 patients from 13 families ic at SMC for over a period of 10 years
were recruited in the study. Patients were from 1989-1996. The incidence of pro-
diagnosed as having CF, based on a typi- foundly deaf children was estimated as
cal clinical picture and sweat chloride lev- 1.1 per 1,000 live births. Children with
els (> 60 mmol/l). Eight mutations were positive history of deafness in the family
detected in 21 of the 26 alleles examined due to genetic causes constituted 37.8%.
of the eight mutations detected, four were Reviewing the consanguinity rate among
common among Bahrainis (2043delG > parents illustrated that 48.5% were first
548A-to-T > 4041C-to-G = F508, in order cousins, whereas 12.2% were far related
of decreasing frequency), accounting for which indicated high rate of consanguin-
66 % of the Bahraini CF alleles. Howev- ity among parents. In one family, Con-
er, four different heterozygous mutations nexin 26 was found to be the responsible
were also detected, namely: 1161delC, gene. However, further molecular studies
1756G-to-T, 3120+1G-to-A, and 3661A- are needed in this field (Jamal, 2000).
to-T, accounting for 16 % of the Bahraini
CF alleles.
Review of the Salmaniya Medical
Complex Records (1988-2005)
Congenital Blindness
The Salmaniya Medical Complex (SMC)
According to Al-Arrayed, 1992; Ahmed is the main hospital for secondary and
and Selvyn, 1988; and Al-Alawi et al., tertiary care in Bahrain. All rare, chronic
1998, the most common eye condition and difficult cases are referred to this hos-
leading to low vision and blindness in pital. The records of patients admitted to
Bahrain are cataract, followed by glau- SMC and diagnosed with genetic diseases
coma and corneal diseases. The follow- and were studied. The record system was
ing causes of congenital blindness were based on the WHO International Classifica-
reported in Bahrain: Retinal dystrophy, tion of Disease (ICD) 9 until 2002, when it
congenital glaucoma, congenital cata- was upgraded to ICD 10. The results high-
ract, retinopathy, macular degeneration, light the magnitude of morbidity caused by
mesodermal dysgenesis, optic nerve dys- each of these genetic diseases.
trophy, retinitis pigmentosa, Leber’s op-
tic dystrophy, while oculocutaneous albi- The number of patients admitted into
nism was observed to causes poor vision SCD during this period was estimated
among some families. to be around 7000. On the contrary,

84
Genetic Diseases in Bahrain

the number of patients presenting with Spinomuscular Atrophy


thalassemia did not exceed hundred,
whereas other genetic diseases occurred A significantly increasing number of
at lower frequencies, and were not families were found to present with spi-
recorded in more than dozens or less. nomuscular atrophies. Four families were
reported with spino-muscular atrophy.
During this 17-year period, 14 patients of which three had acute infantile type
admitted with congenital hypothyroid, (Werdnig- Hoffmann or SMA1), while
and the estimated prevalence for this one presented with chronic childhood
disease was 1/2000. Patients with SMA. Apparently, there was an increased
hemolytic anemia due to ABO were frequency of SMA among Bahrainis as
evaluated to be 21 in number. About 19 three to four deaths occurred every year in
patients with leukodystrophy, 511 with the pediatric wards due to SMA1. One of
disorders of lipoprotein metabolism, 138 the mothers had neuromuscular disorder
with congenital hydrocephaly, 106 with manifested in diplopia and hoarseness of
anomalies of spine, 10 with Alzheimer’s, voice. Her younger brother and three of
15 with cerebral lipidodsis, 65 with her male children had died in infancy.
Huntington’s disease, and 103 patients
with multiple sclerosis were reported. SMA1 carrier frequency in the Caucasian
population was shown to be 1:80, and
The numbers of cases presented does not that of chronic childhood SMA was 1:90
accurately reflect the epidemiology of (Pearn, 1973). However, the frequency of
these disorders in Bahrain, as there is no carriers is very high in some communities
referral basis that ensures the investigation (Schaap, 1985; Moosa and Dawood,
of all possible genetic diseases. It does, 1990; Czeizel, 1989; Czeizel and Hamula,
however, give an idea of the common 1991). In the Occupied Territories,
genetic disorders among Bahrainis. Fried and Mundel (1977) have found
high incidence of SMA1 in the Egyptian
Some other disorders reported include Karaite community where the frequency
neurofibromatosis, epidermolysis bullosa, of heterozygotes was about 1:20. Chronic
ichthyosis, G syndrome, polycystic childhood SMA has now been mapped to
kidney, polycystic ovaries, Parkinsonism, chromosome 5q11.2-13.3 with the gene
osteogenesis imperfecta, porphyria, of SMA1 located in the same general area
hereditary 1,25 dihydroxyvitamin (Brzustowicz, 1990; Melki et al., 1990;
D-resistant rickets, and malignant Melki, 1991).
hyperthermia (Khan et al., 1996; 1987;
Al-Jishi, 2004; Al-Arrayed, 1999; Aldeen
et al., 1999; Kooheji, 1988; Zayyani, Noonan Syndrome
1987; Mohammad and Ebrahim,1985; Al-
Ansari, 1984). Noonan syndrome (NS) is an autosomal
dominant dysmorphic syndrome
characterized by hypertelorism, a
Frequently Diagnosed Syndromes downward eye slant, and low-set
in the Genetic Department posteriorly rotated ears. Other features
include short stature, a short neck with
The following diseases were frequently webbing or redundancy of skin, cardiac
seen among patients attending the genetics anomalies, epicanthic folds, deafness,
clinic: motor delay, and a bleeding diathesis.

85
GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

Ten cases of NS have been reported of this condition in an Arab boy. Other
from Bahrain. Four of them were cases were reported by Abdel Aziz in
Bahraini boys, and six were Pakistani 1972, and Temtamy and Shalash in
girls (3 alive and 3 deceased). One of 1975. In Jerusalem, Ehrenfeld et al.
the Bahraini boys was a sporadic case, (1970) reported 18 cases of BBS. These
while the other three were siblings of cases were descended from 12 families,
one family. It was conjectured that with 3 of them being Arabs and 9 being
the father could be the transmitting Jewish. Farag and Teebi (1988 and 1989)
parent as he had ptosis, but was normal reported high incidence of BBS among
mentally. A changing phenotype with Bedouins in Kuwait. They estimated the
age was found to manifest in this family incidence in a mixed Arab population of
(Allanson et al., 1985). The 2- year old Kuwait to be as high as 1:36,000, which
boy had more severe edema, ptosis and was much higher than the prevalence of
a round face whereas the eldest boy had this disease in Switzerland (Buyse, 1979;
EEG abnormality and seizures. Lemmi Klien and Amman, 1969). Amongst 36
(1983) reported that 71% of NS patients cases, 24 were Bedouins. They estimated
had electroencephalogram-graphic the prevalence among Bedouins to be
abnormalities, and that 82% had some 1:13,000. The rest of the cases were from
type of neurological anomalies. In the Saudi Arabia, Iraq, Syria, and Palestine
cases of NS reported in the Pakistani (Temtamy and McKusick, 1978; Pagon
family, all the affected girls had et al., 1982; Bauman and Hogan, 1973;
pulmonary stenosis. The mother had Hurley et al., 1975; Massof et al., 1979).
short stature of about 142 cm, but she
was normal mentally, and could be the
transmitting parent. Carpenter Syndrome

Two of the families had Carpenter


Lawrence–Moon–Bardet–Beidl syndrome, with one family from Egypt
Syndrome and the other one from Iran. In both the
families, the affected individuals were the
Two consanguineous Bahraini families products of consanguineous marriages.
with Lawrence Moon Biedl syndrome The affected presented with acrocephaly,
(LMBBS) were also reported from peculiar facies, brachydactyly, and
Bahrain. Out of these two families, one syndactyly in the hands, and preaxial
presented with Bardet Biedl syndrome polydactyly and syndactyly of the toes.
(BBS) with two siblings manifesting the
typical clinical picture of this syndrome
(Al-Arrayed and Al-Arrayed, 1991). On Goldenhar Syndrome
the contrary, the other family had Laurence
Moon syndrome (LMS). Two other families had babies with
Goldenhar syndrome. One of them was
In 1977, Toledo et al. suggested the Pakistani. The mother in the other family
splitting of LMBBS into LMS and BBS was Bahraini and the father Palestinian.
as different but interrelated autosomal The etiology of this syndrome is unknown,
recessive disorders. Apparently, there is it is usually sporadic but some familial
an increased frequency of this autosomal cases have been reported. The risk for
recessive syndrome among Arabs. first degree relatives is about 2 percent
Kalabian in 1956 reported the first case (Burck, 1983; Wilson, 1983).

86
Genetic Diseases in Bahrain

Other Observed Syndromes NFHS also reported that: the most


frequently reported chronic condition for
§ Brachmann-de Lange syndrome both men and women were high blood
§ Smith-Lemli-Opitz syndrome pressure, joint disease and cardiac disease.
§ Seckel syndrome Prevalence of these conditions rose steadily
§ Retinoblastoma with age, among those aged 50 and over.
§ Achondroplasia Hypertension was the most common
§ Wolman disease (15% for men and 27% for women),
followed by diabetes (14% for men
and 19% for women) and joint disease
Non-Communicable Diseases (7% for men and 18% for women), while
heart disease prevalence was 7% for both
Health problems in Bahrain are men and women.
those generally experienced by
countries passing through a stage The Ministry of Health statistics for
of transition from developing to 2005 reported also on Mental Health.
developed nations. Over the years, It showed that mental illnesses are
communicable diseases have shown increasingly becoming a universal
a declining trend as the major causes problem in their distribution due to the
of mortality and morbidity. They are rapid changes in the country’s socio-
being replaced by non-communicable economic status, education and life style.
ones such as cardiovascular diseases, Although Bahrain is a considerably
cancer, metabolic diseases, congenital small country, it has its own share
anomalies and accidents, as compared to of problems in terms of frequency
spontaneous abortion/miscarriages that distributions of socio-demographic
were the most common complications characteristics, patterns trends and their
in pregnancies throughout the word. relationship. A study was conducted on
the data of inpatients at the Psychiatric
The National Family Health Survey Hospital over the past five years. The
(NFHS) in 1995 reported a dramatic Psychiatric hospital is the only hospital
increase in non-communicable diseases in the country, which provides mental
(NCD), such as cardiovascular disease health care. In 2005, the distribution
(CVD), diabetes and cancer in Bahrain. of discharges (1,159) according to the
Cardiovascular diseases and cancer principal diagnosis showed that 24.2%
were the leading causes of death. of patients admitted were diagnosed as
Diabetes prevalence was estimated to schizophrenics, 23% were depressed,
be between 20 and 30% depending on 22% were drug dependent, and 30.8%
the study. This represented a common suffered from other mental disorders.
global trend, which affected the Arabian About 67% of all the discharges were
Gulf and the Eastern Mediterranean among male patients. It was clear that
Region exponentially. The share of non throughout the selected four age groups,
communicable diseases of the region’s males of age group 15-44 years took the
disease burden is expected to rise to bulk of the total discharges at 44.2%.
60% by the year 2020. The incidence These statistics indicate that the Non
of hypertension in the EMR area is 26% communicable diseases (NCD’s) such
and diabetes ranges between 7-25%. as cardiovascular diseases, cancer, and
The burden in terms of suffering and diabetes are leading causes of morbidity
health costs is also immense. and mortality in Bahrain.

87
GENETIC DISORDERS IN THE ARAB WORLD - BAHRAIN

Diabetes This trend however, did not change


dramatically since the seventies. Coronary
Studies on the epidemiology of diabetes heart disease (CHD; ICD: I20-25) seems
in Bahrain reported a prevalence of to be the predominant type of cardiopathy
about 25% in those aged 20 years and encountered in Bahrain. CHD was also
over in a primary care-based study found to be the fourth leading cause of
(Zorba, 1996) and 30% in people aged discharges from SMC in 2003. Coronary
40-69 years in a population-based study heart disease (CHD) and stroke are the
(Al-Mahrous, 1998). On the other hand, predominant type of CVD encountered in
the study of Bahrain Family Health clinical practice.
Survey, showed a total prevalence of
5.5% of diabetes mellitus and 6.3% of Known risk factors for CVD such as
hypertension. smoking, and raised blood cholesterol,
and risk factors, such as lack of
physical activity, obesity, and alcohol
Cardiovascular Diseases consumption are expected to have
increased in Bahrain over the last
Cardiovascular disease (CVD) is two decades. In addition to that, the
considered the leading identifiable cause continuing rises in the incidence of
of death in Bahrain. They were reported the cardiovascular diseases is also
to be responsible for 28.24% of deaths association with the rise in the size
in 2003. The Health statistics Report of the over-sixty-five-years-of-age-
for 2005 report states that cardiovascular population, who represent nearly 2.5%
diseases constitute the highest single of the total population.
cause of mortality in Bahrain, representing
69.9 per 100,000 of the population,
accounting for more than 22% of total Cancer
deaths at Salmaniya Medical Complex.
The rate of deaths from circulatory Cancer is the third leading cause of death
disease distributed by gender showed in Bahrain, accounting for 11.9% of all
60.6 mortalities per 100,000 males and deaths (Annual Health Statistic Reports,
59.1 mortalities per 100,000 females. 2004; Table 5.4). The age-standardized

Table 5.4. Frequency of different malignancies among the male and female groups in Bahrain (adapted from
Ministry of Health Cancer Statistics, 2004).

CANCER MALES FEMALES

88
Genetic Diseases in Bahrain

rate for incidence of cancer in Bahrain Al-Alawi E, Ahmed A.Fateha B. Causes of visual
is 167 and 141 per 100.000 males disability at Al-Noor Institute for Blind. Saudi J
and females, respectively. However, Ophthalmol. 1998; 2(2):101-5.
these rates are considerably lower than Al-Ansari A. Gilles de la Tourette’s syndrome.
Bahrain Med Bull. 1984; 6(3):109-10.
the corresponding figures reported in
Al-Arrayed H. Cataract blindness: A world Health
industrialized countries. More than 300
problem for 20 million people. Bahrain Med
individuals are diagnosed with cancer
Bull. 1992; 14(1):3-4.
annually with an estimated incidence rate Al-Arrayed S, Hamza A. A survey of patients with
of 66/100,000 population. sickle cell presentation to accident and emergency
department of SMC in Bahrain. J Bahrain Med
Lung cancer is the leading type of causes Soc. 1995; 7(2):105-12.
among males accounting for 14.5% of Al-Arrayed S. Campaign to control genetic blood
registered cases in 1998, and 17.25% of diseases in Bahrain. Comm Genet. 2005; 8:52-5.
Bahraini patients. The crude incidence Al-Arrayed S. Cytogenetics of fetal wastage in
rate was 6/100,000 of the population, Bahrain. Bahrain Med Bull. 1990; 12(1):21-4.
and among Bahrainis it was 11.6/100,000 Al-Arrayed SS , Shome DK, Hafadh N, Amin S,
Al-Mukhareq H, Al-Mulla M, Sanad A. Darwish
(Table 5.4). Breast cancer is the leading
FA. ABO blood group and Rhd Phenotypes:
cancer among females, accounting for
Results of screening school children and blood
30% of all new cases diagnosed in 1998
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Recommendation Al-Arrayed SS, Al-Hajry A. Premarital counselling
services. 2006; in press.
In general, health care in Bahrain has Al-Arrayed SS, Hafadh N, Al-Serafi S. Premarital
improved during the last two decades, counseling: an experience from Bahrain. East
Mediterr Health J. 1997; 13(3):415-9.
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Al-Arrayed SS, Hafadh N. Amin S, Al-Mukhareq
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H, Sanad H. Student screening for inherited
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blood disorders in Bahrain. East Mediterr Health
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