CONGENITAL DISEASES IN BULGARIA

1
CONGENITAL DISEASES IN BULGARIA

• E. Terlemesian, S. Stoyanov
• University of Chemical Technology and Metallurgy
  Centre of EcologySofia, Bulgaria

2
CONGENITAL DISEASES IN BULGARIA

• Introduction
• Congenital diseases in Bulgaria
• Infant mortality rates
• Discussion
• Conclusion

3
OBJECTIVES

• To present statistical data on
• - the incidence rates,
• - types and
• - trends in the congenital diseases and
• - infant mortality rates
• in Bulgaria during the last years and to discuss
  the causal factors.

4
Sofia Register of Congenital Anomalies (SORCA)

• emerged in 1996
• in response to a growing need for current
  scientific information about the effects of drugs
  and other environmental agents on developing
  human embryo and fetus.
• 40 isolated congenital anomalies (CA) and
  congenital diseases, detectable at birth or to
  the end of the first year of live have been
  detected out of 34124 pregnancies, registered in
  the period 1996 1999
• almost one third of the registered families
  have contacted personally

5
EUROCAT

• has 41 members in 20 countries
• more than one million births per year are
  surveyed
• in EU member states and non-EU countries
• the central database helds a total of more than
  250 000 cases of congenital anomalies since 1980
  including live births, stillbirths and
  termination of pregnancy following prenatal
  diagnosis

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Sofia register database, 1996 1999
Type of registrations Total number Percentage Percentage of CA
Pregnancies 39124 100 -
Congenital anomalies, incl. 778 1,9 100
Live births 668 - 89,3
Fetal deaths 38 - 5,1
Induced abortions 42 - 5,6

Source Euroworkshop on reproductive and
developmental toxicity of pesticides, Sofia, 2000
7
Sofia register data base for congenital
anomalies, 1996 - 1999

Source Euroworkshop on reproductive and
developmental toxicity of pesticides, Sofia, 2000
8
Registered congenital anomalies by type, 1996 -
1999
Type of congenital anomalies Live births, (no) Fetal deaths (No) Induced abortions (No) Total (No) Incidents rate per 10000 pregnancies EUROCAT Register (Rate)
Total number 668 38 42 748 191,49 215,47
Nervous system 83 35 31 148 37,89 21,75
Neutral tube defects 35 18 23 76 19,46 10,03
Spina bifida 34 5 8 47 12,03 5,11
Hearth diseases 245 3 2 250 64,00 60,84
Cleft lip with or without cleft palate 32 2 1 35 8,96 9,11
Cleft palate 9 0 0 9 2,30 5,84
Digestive system anomalies, excluding pyloric stenosis 65 2 2 69 17,66 16,06
Intern.urogenital system 27 0 2 29 7,42 27,86
External genital organs 39 0 0 39 9,98 13,11
Limb anomalies 54 5 2 61 15,62 36,62
Muskoskeletal and connective tissue anomalies 62 4 4 70 17,72 22,17
Down syndrome 44 0 3 47 12,03 18,85
Other chromosomal anomalies, incl. 16 1 8 19 4,86 13,29
Trisomy 13 2 1 0 3 0,77 1,53
Trisomy 18 1 0 1 2 0,51 3,61
Eye anomalies (total) 40 1 1 44 11,26 4,44
Ear anomalies (total) 59 2 3 64 16,38 3,89

Source Sofia Register Database, 1996 1999
EUROCAT, 1996 - 2001
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Registered congenital anomalies in Sofia and
EUROCAT by type, 1996 1999

10
Registered congenital anomalies in Sofia and
EUROCAT by type, 1996 - 1999

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SORCA WORLDWIDE
Spina bifida 0.89 babies per 1000 live births about one in every 1000 - 2000 births (USA)
Neural tube defects 0.92 babies per 1000 newborns 2.6 babies per 1000 newborns (worldwide) about 2 children per 1000 newborns (in W. Australia). until 1996 1.3 children per 1000 births (in W. Australia) after 1996
Congenital facial clefting 1.12 per 1000 live births 1.71 per 1000 live births (in the US) 1.49 per 1000 live births (EUROCAT)

Source http//encyclopedia.thefreedictionary.com
12
Total number of live births in Bulgaria

13
Percentage of total live births in Bulgaria
according to the mothers age

14
Mortality rates all ages, per 1000

15
Infant mortality rate per 1000 live births

16

Delivery at home

• During the last years an increased trend of
  delivery at home is registered in Bulgaria.
• This is a reason for the high mother mortality
• In the rural areas mother mortality is 25,5 per
  100 000 births
• In Kazakhstan 40 per 100 000 births

Source UN Population Fund Report, 2004
17
Infant mortality rates per 100 000 live births
(2003)

18
Trends in the mortality rates in the period 1990
2003 according to different reasons

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Infant mortality rates
Reasons for death Death rate per 100 000 live births Death rate per 100 000 live births Death rate per 100 000 live births Death rate per 100 000 live births Death rate per 100 000 live births
  1990 1995 2000 2002 2003
Congenital anomalies (total) including 401,2 418,2 320,3 282,7 259,8
Spina bifida 20,9 22,2 13,6 16,5 10,4
Other nervous system anomalies 38,0 66,7 43,4 51,1 20,8
Anomalies of bulbus cordis 30,4 26,4 19,0 33,1 38,6
Other congenital heart anomalies 136,9 105,6 103,2 66,2 75,7
Congenital respiratory system anomalies 37,1 59,7 16,3 12,0 13,4
Congenital digestive system anomalies 37,1 37,5 28,5 25,6 32,7
Certain conditions, originating in the prenatal period, including 393,6 464,1 438,4 398,5 386,0
Disorders related to a preterm delivery and low birth weight 130,3 84,8 62,4 120,3 154,4
Difficult breathing syndrome (sudden infant death syndrome) 42,8 62,5 69,2 49,6 44,5
Source National Statistical Institute Yearbook
Health, 2002, 2003
20
Infant mortality rate due to congenital anomalies

21
Infant mortality rates due to congenital anomalies

22
Infant mortality rates due to certain conditions,
originating in the prenatal period
23
Territorial distribution of infants mortality in
2003 caused by congenital anomalies and due to
reasons originating in the prenatal period
Live births rate per Still births rate per Infant mortality per 1000 live birth Infant mortality per 1000 live birth Infant mortality rate per 1000 live births due to Infant mortality rate per 1000 live births due to
District 1000 people 100000 people In the towns In the country-side Congenital anomalies Reasons from the prenatal period
Average for the country 8,6 7,0 10,7 16,5 2,6 3,9
Vratzsa 8,1 6,4 12,3 15,6 2,8 4,0
Montana 7,7 8,0 12,3 22,4 1,5 6,0
Dobrich 8,8 8,6 12,5 13,9 3,8 4,9
Varna 9,9 6,7 9,4 19,2 3,9 3,7
Silistra 8,7 9,4 12,3 18,1 5,8 4,7
Targovishte 9,4 6,4 14,3 23,6 5,3 6,6
Shumen 9,5 20,9 13,3 23,9 3,1 4,7
Burgas 9,6 8,6 12,7 16,6 1,2 1,2
Sliven 11,9 15,9 26,2 38,3 3,1 13,7
Yambol 8,2 4,6 12 0 44,9 4,9 4,1
Kardjali 9,6 4,9 13,2 17,9 1,9 5,2
Plovdiv 8,8 6,5 14,3 18,4 2,9 5,9
Kyustendil 7,2 5,1 15,5 17,9 3,5 0,9
Sofia-capital./district. 8,9/7,5 4,7/7,3 6,9 20,1 1,6/4,0 2,0/3,5
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SORCA data

• About 60 per cent of the registered
  fetus/children cases have been identified as
  isolated, probably multifactorial congenital
  anomalies
• 40 per cent with multiple congenital anomalies
  distributed as follows
• chromosomal aberrations 8 per cent
• single gene disorders 10 per cent,
• purely environmental 2 per cent and
• multiple congenital anomalies of unknown
  etiology 20 per cent
• The experience of the TIS in Jerusalem - reported
  38000 calls for twelve years.
• Of them
• 75 per cent due to exposure to drugs,
• 9 per cent to exposure to diagnostic
  irradiation,
• 6 per cent suspected to have intrauterine
  infection,
• 5 per cent due to vaccination and
• only 2 per cent following exposure to
  environmental pollutants

25
OTIS fact sheets of the effects of environmental
agents

• Summarize six categories of exposure
• medications
• infections
• illicit substances
• herbal products
• maternal medical conditions
• others

26
Risk assessment of prenatal exposures requires
knowledge about

• chemical structure
• dosage
• developmental stage at exposure
• duration of exposure

27

Types of exposures
Type of exposure Distribution,
Mental illness 4
Environmental agent 8
Radiation 3
Occupational agents 17
Herbal products 4
Drugs of abuse 6
Medications 58
Source www.otispregnancy.org
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Distribution of the reproductive status
Reproductive status Distribution,
Pregnancy 74
Paternal 3
Preconception 9
Breast feeding 8
Not specified 6
Source www.otispregnancy.org
29
Epidemiological work in cities with high level of
environmental pollution

• with dioxins (Chapaevsk) higher frequency of
  newborn with congenital hydrocephaly
• with PCBs (Serpukov) 4.5 newborn with CA per
  100 newborn
• 2.2 newborn with CA per 100 newborn in the
  majority of other Russian cities

Source Revich at al, 2000
30
Pesticides used in Bulgaria (in tons) in the
period 2002 - 2004

Source Annual Report, National office for Plant
Defence, 2004, p. 45
31
The area of wine yards treated with herbicides
(thousands ha)

Source Annual Report, National office for Plant
Defence, 2004, p. 44
32
Expenses spent for permanent disposal of the
pesticides unfit for use

Source Annual Report, National office for Plant
Defence, 2004, p. 45
33
Strategy for reduction the hazardous impact of
pesticides in Bulgaria

• Systematic control of the appearance,
  dissemination, density and degree of invasion of
  the plant diseases, insects and weeds and their
  natural regulators.
• Localization of pests in the unplantet areas
  (locusts)
• Determination the exact time of the pesticides
  application
• Use of more effective chemicals
• Control of their content in soil, water, plants
  and food

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Executive Body National Office for Plant Defense

• Responsibilities
• Biological testing of the Plant Defense Products
  (PDP) according Directive 91/414/EEC and Annex I
  for PDP and their active substances
• Official registration of PDP and their active
  substances
• 54 of the included in Annex I 91 active
  substances are components of PDP, permitted in
  Bulgaria
• Control of the import of PDP
• Control of PDP in the market network

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Shortages

• Problems with the pesticides unfit for use
• Deposited in ruined, unprotected stores
• Illegal use of forbidden pesticides
• Trans-boundary pollution with pesticides
• Control is increasing but not regular and not
  enough
• It does not cover all regions of the country

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Measures

• Inventory in correspondence with Directive
  67/548/EEC and Annex 5 for the recent available
  chemicals
• 35 tons DDT from 6 stores are exterminated in the
  Netherlands
• Permanent disposal of the pesticides forbidden
  for use

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Conclusion

• Sofia Register of Congenital Anomalies (SORCA)
  emerged in 1996 in response to a growing need for
  current scientific information about the effects
  of drugs and other environmental agents on
  developing human embryo and fetus.
• 40 isolated congenital anomalies (CA) and
  congenital diseases have been detected out of
  34124 pregnancies, registered in the period 1996
  1999 in Sofia.
• In general, the incidence rates of CA in Sofia do
  not differ significantly from the average figures
  of EUROCAT (1996 2001). Prevalence of
  non-chromosomal congenital anomalies has been
  registered.

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Conclusion

• Total chromosomal anomalies, registered in Sofia
  per 10000 pregnancies, are almost twice less than
  the registered by EUROCAT diseases. At the same
  time the age-specific fertility of Bulgarian
  women in 1998 2002 shows that the average age
  of mothers has increased.
• In 2003 certain conditions, originating in the
  prenatal period (386/1000 live births) and
  congenital anomalies (259,8/1000 live births)
  could be ranked at the first two places between
  the causes of the infant deaths. In the period
  1990 2003 trends of reduction are noticed for
  the both leading reasons for the infant mortality.

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Conclusion

• About 2 per cent of the registered fetus/children
  cases have been identified to be with purely
  environmental etiology.
• In the well developed agricultural areas and
  highly industrialized regions in Bulgaria with
  production of big amount of energy the proportion
  of congenital anomalies and conditions,
  originating in the prenatal period as reasons for
  infant mortality exceed the averages for the
  country.
• Strategy for reduction the amount of the
  pesticides used for plant defence in Bulgaria is
  elaborated, which includes systematic control of
  the appearance, dissemination, density and the
  degree of invasion of the plant diseases, insects
  and weeds and their natural regulators and
  control on the PDP.