Genetic prognosis in severe mental handicap. 1975

E Angeli, and B Kirman

698 children below age sixteen years with severe mental handicap (below IQ50) who were admitted to hospital between 1956 and 1959 were surveyed for genetic prognosis. They were divided into 23.7% ascribed primarily to environmental factors, 50.9% unclassified aetiologically, 16.2% of Down's syndrome, 5.3% other genetic syndromes and 3.9% with congenital hydrocephalus. The incidence of a similar degree of mental retardation among the sibs of 660 whose families were traced, was 1.1% in the "environmental" group; 4.7% for the unclassified; 1.7% for Down's syndrome, 11.5% for other genetic syndromes and 4.3% for hydrocephalus with spina bifida. There were no affected sibs of the uncomplicated cases of congenital hydrocephalus. This survey underlines the fact that a high proportion of cases of mental handicap remain without aetiological diagnosis. It emphasises the value of such a diagnosis for genetic counselling. Advice given depends on the circumstances of the particular case which requires a detailed social history and sympathetic rapport by members of the team as well as appropriate clinical expertise (Kirman, 1971, 1972). When families with similarly affected new relative or previous severely mentally handicapped sibs are set aside, the recurrent risk for unclassified cases is reduced to three per cent.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007360 Intelligence The ability to learn and to deal with new situations and to deal effectively with tasks involving abstractions.
D007744 Obstetric Labor Complications Medical problems associated with OBSTETRIC LABOR, such as BREECH PRESENTATION; PREMATURE OBSTETRIC LABOR; HEMORRHAGE; or others. These complications can affect the well-being of the mother, the FETUS, or both. Complications, Labor,Labor Complications,Complication, Labor,Complication, Obstetric Labor,Complications, Obstetric Labor,Labor Complication,Labor Complication, Obstetric,Labor Complications, Obstetric,Obstetric Labor Complication
D008297 Male Males
D008607 Intellectual Disability Subnormal intellectual functioning which originates during the developmental period. This has multiple potential etiologies, including genetic defects and perinatal insults. Intelligence quotient (IQ) scores are commonly used to determine whether an individual has an intellectual disability. IQ scores between 70 and 79 are in the borderline range. Scores below 67 are in the disabled range. (from Joynt, Clinical Neurology, 1992, Ch55, p28) Disability, Intellectual,Idiocy,Mental Retardation,Retardation, Mental,Deficiency, Mental,Intellectual Development Disorder,Mental Deficiency,Mental Retardation, Psychosocial,Deficiencies, Mental,Development Disorder, Intellectual,Development Disorders, Intellectual,Disabilities, Intellectual,Disorder, Intellectual Development,Disorders, Intellectual Development,Intellectual Development Disorders,Intellectual Disabilities,Mental Deficiencies,Mental Retardations, Psychosocial,Psychosocial Mental Retardation,Psychosocial Mental Retardations,Retardation, Psychosocial Mental,Retardations, Psychosocial Mental
D009083 Mucopolysaccharidoses Group of lysosomal storage diseases each caused by an inherited deficiency of an enzyme involved in the degradation of glycosaminoglycans (mucopolysaccharides). The diseases are progressive and often display a wide spectrum of clinical severity within one enzyme deficiency. Mucopolysaccharidosis
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011248 Pregnancy Complications Conditions or pathological processes associated with pregnancy. They can occur during or after pregnancy, and range from minor discomforts to serious diseases that require medical interventions. They include diseases in pregnant females, and pregnancies in females with diseases. Adverse Birth Outcomes,Complications, Pregnancy,Adverse Birth Outcome,Birth Outcome, Adverse,Complication, Pregnancy,Outcome, Adverse Birth,Pregnancy Complication
D001787 Blood Group Incompatibility An antigenic mismatch between donor and recipient blood. Antibodies present in the recipient's serum may be directed against antigens in the donor product. Such a mismatch may result in a transfusion reaction in which, for example, donor blood is hemolyzed. (From Saunders Dictionary & Encyclopedia of Laboratory Medicine and Technology, 1984). ABO Compatibility,ABO Incompatibility,Blood Group ABO Incompatibility,Blood Type Incompatibility,Rh Compatibility,Rh Incompatibility,ABO Compatibilities,ABO Incompatibilities,Blood Group Incompatibilities,Blood Type Incompatibilities,Compatibility, ABO,Compatibility, Rh,Incompatibilities, Blood Group,Incompatibility, ABO,Incompatibility, Blood Group,Incompatibility, Blood Type,Incompatibility, Rh,Rh Compatibilities,Rh Incompatibilities

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