How rare is LHON

How many people have LHON?

LHON is not limited to a particular country or ethnic group.

Cases of LHON are reported from a lot of countries around the world, and in many different populations.

A lot of the LHON cases and LHON research looks at people who are caucasian (white), especially from a Northern European background.  This probably has more to do with the history of scientific research than the actual number of cases in any part of the world.

LHON is officially classed as a “Rare Disease”

The official definition of a “Rare Disease in the EU is one which affects less than 5 in 10,000 of the genral population.  In the USA a “Rare Disease” affects less than 200,000 people.

Most studies of LHON start with a patient affected by LHON and look at their relatives to build a family tree or Pedigree. Doctors look at how many blood relatives are affected to work out the risk of a carrier developoing symptoms.

Some researchers count up the number of affected people diagnosed in a country and use that figure to work out how common LHON is in a country.

Some researchers put the number of LHON cases as around 1 in 31,000.

 

One problem with this approach is that doctors do not know exactly why some people carrying a LHON gene develop LHON and others do not.

Many people could be carrying a LHON gene but never know it.

Researchers in England took a different approach.  They did genetic testing on a large number of people to find out who was carrying a LHON related mutation.

They found that about 1 in 8,500 people carries one of the 3 main LHON causing mutations.

 

Taking these figures for the population of the UK in the 2011 census gives the following numbers for LHON Carriers and LHON Affected people.

  8,50031,00050,000
CountryPopulationLHON CarriersLHON Affected 1 in 31000LHON Affected 1 in 50000
UK64,100,0007,5422,0681,282
England53,900,0006,3421,7391,078
Scotland5,300,000624171106
Wales3,100,00036510062
Northern Ireland1,800,0002125936

Estimates for the number of people affected by LHON vary from 1 in 31,000 to 1 in 50,000.

This table uses 2014 population figures from Worldmetrics.info to estimate the number of LHON carriers and LHON affected in each country. A “high” and “low” figure is given  based on the 1 in 31,000 and 1 in 50,000 estimates.

CountryPopulationLHON CarriersLHON Affected 1 in 31000LHON Affected 1 in 50000
UK63,489,2347,4702,0491,270
China1,393,783,836163,97544,96127,876
India1,267,401,849149,10740,88425,349
USA322,583,00637,95110,4066,452
Indonesia252,812,24529,7438,1565,057
Brazil202,033,67023,7696,5184,041
Pakistan185,132,92621,7815,9733,703
Nigeria178,516,90421,0025,7593,571
Bangladesh158,512,57018,6495,1143,171
Russia142,467,65116,7614,5962,850
Japan126,999,80814,9424,0972,540
Mexico123,799,21514,5653,9942,476
Philippines100,096,49611,7773,2292,002
Ethiopia96,506,03111,3543,1141,931
Vietnam92,547,95910,8882,9861,851
Egypt83,386,7399,8112,6901,668
Germany82,652,2569,7242,6671,654
Iran78,470,2229,2322,5321,570
Turkey75,837,0208,9232,4471,517
Congo69,360,1188,1612,2381,388
Thailand67,222,9727,9092,1691,345
France64,641,2797,6052,0861,293
Italy61,070,2247,1851,9711,222
Burma53,718,9586,3201,7331,075
South Africa53,139,5286,2521,7151,063
Tanzania50,757,4595,9721,6381,016
South Korea49,512,0265,8251,598991
Colombia48,929,7065,7571,579979
Spain47,066,4025,5381,519942
Kenya45,545,9805,3591,470911
Ukraine44,941,3035,2881,450899
Argentina41,803,1254,9191,349837
Algeria39,928,9474,6981,289799
Uganda38,844,6244,5701,254777
Sudan38,764,0904,5611,251776
Poland38,220,5434,4971,233765
Canada35,524,7324,1801,146711
Iraq34,768,7614,0911,122696
Morocco33,492,9093,9411,081670
Afghanistan31,280,5183,6811,010626
Venezuela30,851,3433,630996618
Peru30,769,0773,620993616
Malaysia30,187,8963,552974604
Saudi Arabia29,369,4283,456948588
Uzbekistan29,324,9203,450946587
Nepal28,120,7403,309908563
Mozambique26,472,9773,115854530
Ghana26,442,1783,111853529
North Korea25,026,5882,945808501
Yemen24,968,5082,938806500
Australia23,630,1692,781763473
Madagascar23,571,9622,774761472
Cameroon22,818,6322,685737457
Angola22,137,2612,605715443
Syria21,986,6152,587710440
Romania21,640,1682,546699433
Sri Lanka21,445,7752,524692429
Cote d'Ivorie20,804,7742,448672417
Chile17,772,8712,091574356
Burkina Faso17,419,6152,050562349
Malawi16,829,1441,980543337
Netherlands16,802,4631,977543337
Ecuador15,982,5511,881516320
Guatemala15,859,7141,866512318
Mali15,768,2271,856509316
Cambodia15,408,2701,813498309
Zambia15,021,0021,768485301
Zimbabwe14,599,3251,718471292
Senegal14,548,1711,712470291
Chad13,211,1461,555427265
Rwanda12,100,0491,424391243
Guinea12,043,8981,417389241
South Sudan11,738,7181,382379235
Cuba11,258,5971,325364226
Belgium11,144,4201,312360223
Greece11,128,4041,310359223
Tunisia11,116,8991,308359223
Bolivia10,847,6641,277350217
Somalia10,805,6511,272349217
Czech Republic10,740,4681,264347215
Portugal10,610,3041,249343213
Benin10,599,5101,248342212
Dominican Republic10,528,9541,239340211
Burundi10,482,7521,234339210
haiti10,461,4091,231338210
Hungary9,933,1731,169321199
Sweden9,631,2611,134311193
Azerbaijan9,514,8871,120307191
Serbia9,468,3781,114306190
United Arab Emirates9,445,6241,112305189
Belarus9,307,6091,096301187
Austria8,526,4291,004276171
Tajikistan8,408,947990272169
Honduras8,260,749972267166
Switzerland8,157,896960264164
Israel7,822,107921253157
Jordan7,504,812883243151
Papua New Guinea7,476,108880242150
Hong Kong7,259,569855235146
Bulgaria7,167,998844232144
Togo6,993,244823226140
Paraguay6,917,579814224139
Laos6,894,098812223138
Eritrea6,536,176769211131
El Salvador6,383,752752206128
Libya6,253,452736202126
Sierra Leone6,205,382731201125
Nicuaragua6,169,269726200124
Denmark5,640,184664182113
Kyrgyrstan5,625,015662182113
Singapore5,517,102650178111
Slovakia5,454,154642176110
Finland5,443,497641176109
Turkmenistan5,307,171625172107
Norway5,091,924600165102
Lebanon4,965,914585161100
Costa Rica4,937,75558116099
Central African Republic4,709,20355515295
Ireland4,677,34055115194
Congo4,558,59453714892
New Zealand4,551,34953614792
Palestine4,436,41152214489
Liberia4,396,87351814288
Georgia4,322,84250914087
Croatia4,272,04450313886
Mauritania3,984,45746912980
Oman3,926,49246212779
Panama3,926,01746212779
Bosnia & Herzegovina3,824,74645012477
Puerto Rico3,683,60143411974
Kuwait3,479,37141011370
Moldova3,461,38040811270
Uruguay3,418,69440311169
Albania3,185,41337510364
Lithuania3,008,2873549861
Armenia2,983,9903529760
Mongolia2,881,4153399358
Jamaica2,798,8373309156
Qatar2,267,9162677446
Macedonia2,108,4342496943
Lesotho2,097,5112476842
Slovenia2,075,5922456742
Latvia2,041,1112416641
Botswana2,038,5872406641
Gambia1,908,9542256239
Guinea-Bissau1,745,7982065735
Gabon1,711,2942025635
Trinidad & Tobago1,344,2351594427
Bahrain1,344,1111594427
Estonia1,283,7711524226
Sqaziland1,267,7041504126
Mauritius1,249,1511474125
Cyprus1,153,0581363824
Timor-leste1,152,4391363824
Fiji887,0271052918
Djibouti886,3131052918
Reunion885,3281052918
Guyana803,677952617
Equatorial Guinea778,061922616
Bhutan765,552912516
Comoros752,438892516
Montenegro621,542742113
Western Sahara585,646691912
Macau575,481681912
Solomon Islands572,865681912
Suriname543,925641811
Luxembourg536,761641811
Cape Verde503,637601711
Guadeloupe468,016561610
Malta430,14651149
Brunei423,20550149
Martinique404,70548149
Bahamas382,57146138
Maldives351,57242128
Belize339,75840117
Iceland333,13540117
Barbados286,06634106
French Polynesia279,83533106
New Caledonia259,8243196
Vamuatu258,3013196
French Guiana255,4553196
Mayotte228,0702785
Sao Tome197,8822474
Samoa191,8312374
Saint Lucia183,5982264
Channel Islands162,7782064
Curacao161,8362064
Saint Vincent & the Grenadines109,3711343
US Virgin Islands106,7921343
Grenada106,3031343
Tonga105,7821343
Kiribati103,9421343
Micronesia103,9031343
Aruba103,4311343
Seychelles93,3061142
Antigua and Barbuda90,9031132
World Total7,181,681,855845,006231,773143,733

 

 

Scientific reports of LHON by Ethnicity

Note: The citations are a sample of papers mentioning the ethnicity of patients, to indicate at least one documented case.  This page is not intended to be a complete list of relevant research.

African / African-American [citehttp://www.ncbi.nlm.nih.gov/pubmed/2575667[/cite](21734595)(8659531)(8037217)

Amerindian (24672219)

Asian (25342614)(9012411)(22517755)(21398275)(12436196)(11754915)(8500789)(12436196)(12379308)

Caucasian / European [citehttp://www.ncbi.nlm.nih.gov/pubmed/2575667[/cite](9150158)(16418878)(24319328)(21788663)(24319328)(12749053)(7977345)

Chinese (23665487)(20627642)(20599858)(25863085)(24404670)(23905692)(19555656)(18440284)(25194554)(24846978)(24398099)(23304069)(22917908)(22400981)(21983721)(21742061)(21724059)(21074518)(20053576)(19458970)

Cuban (mixed race) (8037217)

Hispanic (15884420)(8016139)

Indian (20809775)

Middle Eastern (15126312)

Native American (8016139)(8016139)

Scientific reports of LHON by Country

Note: The citations are a sample of papers mentioning the nationality of patients, to indicate at least one documented case.  This page is not intended to be a complete list of relevant research.

Australia (8742999)(25343117)(22266889)(21887510)

Austria (25053773)(15139661)(12031765)

Belgium (23864591)(22260353)(8990216)

Brazil (19098324)(22893673)(20676915)(20565249)(19098324)(18363168)(17471325)(17460303)(16303983)(15692724)(15455797)(14962416)(12545691)

Cambodia (19026397)

Canada [citehttp://www.ncbi.nlm.nih.gov/pubmed/2575667[/cite](15954041)(21788663)

Chile (24672219)

China(24404670)(23905692)(19555656)(18440284)

Croatia (16617593)

Cuba (15337616)

Czech Republic  (8037217)

Denmark (2238193)(26108648)(7770969)(25053773)

Finland [citehttp://www.ncbi.nlm.nih.gov/pubmed/2575667[/cite](22970697)(17406640)(11906302)(11523562)

France (23350551)(18563041)(25917160)(24161764)(24145886)(22879922)

Germany (21788663)(25053773)(22879922)(23263355)(21145289)(17822909)(15573947)(12271374)

Greece [citehttp://www.ncbi.nlm.nih.gov/pubmed/2575667[/cite]

Hungary (15483043)(12227465)

India (16137960)(20809775)(23805034)(23674761)(23170061)(20809775)(20454697)(19753312)

Indonesia (14611946)(12436196)(12827453)(12436196)

 

Iran (15126312)(24158608)(19009343)(17045122)(16751977)(14671420)

Ireland [citehttp://www.ncbi.nlm.nih.gov/pubmed/2575667[/cite](23756086)(20882363)

Israel (11995959)(8240356)(8213820)

 

Italy(23209682)(19098324)(17122117)(25053773)(22879922)(25792533)(25132831)(24369379)

Japan(16380132)(8500789)(26062390)(22183138)

Latvia (24319328)

Netherlands (12736867)(19836344)(19024058)(18664474)(18320530)

New Zealand (8742999)

Norway (18396045)

Poland (24747208)(12136948)(18801464)(23748049)

Portugal (17254817)

Russia (16050984)(16523671)(16050984)

Serbia (24508359)

Slovenia (25690485)

South Korea (25342614)

Spain (17573650)(25053773)

Sweden (20385529)(8448903)(16879580)

Switzerland(22684678)

Thailand (25215595)(16477364)(21398275)(11754915)(16148621)(15272763)

Tibet (22517755)

UK [citehttp://www.ncbi.nlm.nih.gov/pubmed/2575667[/cite](12518276)(11897814)(21788663)(25053773)(19525327)

USA [citehttp://www.ncbi.nlm.nih.gov/pubmed/2575667[/cite](19753312)(23869167)(23433229)(23433437)

 

 

This page was last updated September 14 2015

References

Gene Reviews LHON Article

 

Bibliography

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