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Familial Aggregation of Endemic Congenital Hypothyroidism Syndrome in Congo (DR): Historical Data.
- Source :
-
Nutrients [Nutrients] 2020 Oct 02; Vol. 12 (10). Date of Electronic Publication: 2020 Oct 02. - Publication Year :
- 2020
-
Abstract
- Familial aggregation of endemic congenital hypothyroidism (CH) in an iodine-deficient population from northern Congo (Democratic Republic (DR)) was analysed on data collected four decades ago (1979-1980). During a systematic survey of 62 families, 46 endemic CH subjects (44 myxedematous and 2 neurological) were identified based on clinical evidence within a village cohort of 468 subjects. A distribution analysis showed that two families presented significant excess of cases versus a random background distribution. Both families were characterised by two healthy parents having all of their five offspring affected by some form of endemic CH. Goitre prevalence in endemic CH was lower than that in the general population, while goitre prevalence in the unaffected part of the cohort (parents and siblings) was similar to that of the general population. Some unidentified genetic/epigenetic factor(s) could contribute to the evolution of some iodine-deficient hypothyroid neonates through irreversible and progressive loss of thyroid functional capacity during early childhood (<5 years old). Besides severe iodine deficiency, environmental exposure to thiocyanate overload and selenium deficiency, factors not randomly distributed within families and population, intervened in the full expression of endemic CH. Further exploration in the field will remain open, as iodine deficiency in Congo (DR) was eliminated in the 1990s.
- Subjects :
- Adolescent
Adult
Child
Child, Preschool
Cohort Studies
Congenital Hypothyroidism genetics
Democratic Republic of the Congo epidemiology
Environmental Exposure adverse effects
Female
Goiter, Endemic genetics
Humans
Infant
Infant, Newborn
Male
Pedigree
Phenotype
Prevalence
Thiocyanates toxicity
Young Adult
Congenital Hypothyroidism epidemiology
Goiter, Endemic epidemiology
Iodine deficiency
Selenium deficiency
Subjects
Details
- Language :
- English
- ISSN :
- 2072-6643
- Volume :
- 12
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- Nutrients
- Publication Type :
- Academic Journal
- Accession number :
- 33023116
- Full Text :
- https://doi.org/10.3390/nu12103021