Beruflich Dokumente
Kultur Dokumente
Multiracial
Multiracial population
Indigenous
West Malaysia
Proto-Malay, Negrito, Senoi
East Malaysia
Sabah
Kadazandusun, Bajau, Murut
Sarawak
Dayaks, Iban, Bidayuh, Orang Ulu
Pakistanis Ceylonese
Statistics 2010
Ethnic groups
Statistics 2010
Origins
Indigenous people
50 000 years ago- Paleolithic (early stone age) Out of Africa theory Orang asli
Cham- Malay
Banyak
Bintang Manis Anjing Sedikit Ikan Orang
Banyak
Bintang Manis Anjing Sedikit Ikan Orang
More
Star Sweet Dog Less Fish People
Gunung
Gunung
Mountain
Bronze Tin Bitter Eye Here Water
Tembaga Tembaga Timah Pahit Mata Sini Ayer Tima Pahit Mata Ni Aya
Buat
Buat
Do
Bapa
Pak
Father
Malaysian-Chinese
15th century: Puteri Hang Li Po married Sultan Mansur Shah of Malacca 18th 19th century:
Hokkien from Fujian district > Penang and Malacca > rubber plantation Teochew from Chaoshan district > JB, Selangor > pepper plantation Hainanese from Hainan > Kemaman, Pulau Ketam > cooks Hakka from Guangdong and Fujian > Perak, Selangor > miners Cantonese from Guangdong and Guangxi > KL, Ipoh, Seremban, Sandakan > miners/ developers
Malaysian- Chinese
Malaysian-Indians
Early 20th century Brought in by the British as labourers
Tamils- plantation
Malaysian-Indians
Prevalence
Worldwide
Approx. 270 million people (4.5% of world population) carry abnormal Hb genes 400 million people in South East Asia (SEA) 50% of carriers are in SEA
Prevalence in Malaysia
Cross-sectional study in Tanjong Karang n= 111 screened 1 in 4 (25%) are carriers
Ainoon O, Malaysian J Pathol 1994
*About 4.5% are carriers for thalassaemia 4.5% of Malaysian-Chinese are carriers for 0 thalassaemia
Thalassemia Carrier Diagnosis in Malaysia, E. George, Ed., pp. 16, SP-Muda Printing Sdn Bhd, Kuala Lumpur, Malaysia, 1998
thalassaemias
thalassaemias
Present throughout SEA Higher in northern parts
30% in Chiengmai Province of Thailand vs. 0.5% in Indonesia
thal-1
Deletion of both duplicated globin genes 17.5- 20kb deleted gene is not deleted Known as SEA 0 molecular defect (- - SEA)
thal-2
2 types
Deletion of 4.2 kb DNA (leftward type, -4.2) Deletion of 3.7 kb DNA (rightward type, -3.7)
Hb Constant Spring
1st isolated in a Chinese family from Constant Spring district of Jamaica in 1971 Mutation (TAA>CAA) of termination codon at position 142 of the 2-globin gene 172 amino acids (normal 141)
Unstable mRNA-CS
Milner PF, Lancet 1971
Hb Constant Spring
Most common in SEA In Malaysia, most common in Malays
Malays- 2.24% Chinese- 0.66% Indians- 0.16%
In Bangkok- 1.48%
Fuchaeron S, Hemoglobin 1997 Wee YC, J Obstet Gynaecol Res 2005
Hb H disease, n= 24
Type of thal - - SEA/- 4.2 - -SEA/- 3.7 - -SEA/CS TT Ethnic No. origin cases Chinese Chinese (5) Malay (2) Chinese (5) Malay (8) Chinese 3 7 13 1 Age (yr) Hb (g/dL) Av Av (range) (range) 28 8.0 (21- 40) (7.1- 9.9) 31.5 9.2 (12- 63) (8.2- 10.0) 22.5 8.5 (6- 35) (3.4- 10.7) 57 10.6 HbH (%) Av (range) 4.0 (2.0- 6.1) 6.7 (3.9- 12.1) 15.1 (4.0- 25.6) n.d.
thalassaemias
%
19.4
Chinese
IVS 2-654 (C>T)
%
49.8
23.4
*Codon 17 (A>T)
*Codon 35 (-C)
13.9
8.3
-28 (A>G)
*Codon 17 (A>T)
13.3
10.1
5.6
2.8
2.1
1.3
Our neighbours
Kadazandusun
Deletional -thalassaemia Nondeletional thal -thal
*22 B-thal mutations
-3.7
Filipino deletion 0 16
12.8%
Jin-Ai MAT, J Biomed Biotech 2010
Hb E
Mutation at position 26 on the globin gene Replaces glutamic acid by lysine Limited to mainland SEA Borders joining Thailand, Laos and Cambodia: Hb E triangle
Hb E triangle
Hb Malay
Codon 19 (Asn to Ser) (A>G) Common in Malays and Southern Thailand More severe than Hb E
No.
Hb type
39
EE
16
13
EF 6.6 0.9 57.5 11.6 39.2 13.7
5
EF 7.9 1.0 52.2 8.0 47.7 8.0
EFA EF+Malay 9.5 1.5 9.2 2.8 58.2 3.4 3.4 1.1
GA()0
Both reported in Malaysia G(A)0 found in Cantonese subjects GA()0 found in Thais Hb F between 9.9- 16.6%
Patient numbers
540
500
1341
x 101
Summary
Thalassaemia is the most common inherited single gene disorder in the world 50% of thalassaemia carriers are in South East Asia Thalassaemia syndromes were originally confined to the tropics
Changing epidemiology
SEAsians are now scattered all over
Summary
In Malaysia, the Chinese, Malays and indigenous people are commonly affected Hb E, Hb Malay and Hb Constant Spring more common in Malays 0-thal more common in Chinese
Summary
The common hemoglobinopathies/ thalassaemias in Malaysia are:
thalassaemia major thalassaemia intermedia Hb E thalassaemia Hb H disease Hb H- Constant Spring
Summary
With a high prevalence of about 5%, screening for thalassaemia should be undertaken Screen for -thal, 0- thal, Hb E
The end