Some of these institutions provided a very basic level of education (such as differentiation between colors and basic word recognition and numeracy), but most continued to focus solely on the provision of basic needs of food, clothing, and shelter. Conditions in such institutions varied widely, but the support provided was generally non-individualized, with aberrant behavior and low levels of economic productivity regarded as a burden to society. Individuals of higher wealth were often able to afford higher degrees of care such as home care or private asylums. Heavy tranquilization and assembly-line methods of support were the norm, and the medical model of disability prevailed. Services were provided based on the relative ease to the provider, not based on the needs of the individual. A survey taken in 1891 in Cape Town, South Africa shows the distribution between different facilities. Out of 2046 persons surveyed, 1,281 were in private dwellings, 120 in jails, and 645 in asylums, with men representing nearly two-thirds of the number surveyed. In situations of scarcity of accommodation, preference was given to white men and Black men (whose insanity threatened white society by disrupting employment relations and the taboo sexual contact with white women).In the late 19th century, in response to Charles Darwins On the Origin of Species, Francis Galton proposed selective breeding of humans to reduce intellectual disability. Early in the 20th century, the eugenics movement became popular throughout the world.
Histidinemia is a rare autosomal recessive metabolic disorder caused by a deficiency of the enzyme histidase. Histidase is needed for the metabolism of the amino acid histidine. Although originally thought to be linked to multiple developmental disorders histidinemia is now accepted as a relatively benign disorder, leading to a reduction in the prevalence of neonatal screening procedures. Presentation
Histidinemia is considered benign as most patients remain asymptomatic, early correlational evidence from the first decade of histidinemia research lead to the theory that histidinemia was associated with multiple developmental symptoms including hyperactivity, speech impediment, developmental delay, learning difficulties, and sometimes mental retardation. However, these claims were later deemed coincidental as a large subpopulation of infants that tested positive for histidinemia were found to have normal IQ and speech characteristics; as such histidinemia has since been reclassified as a benign inborn error of metabolism. Molecular mechanism
Histidinemia occurs as the result of an inborn error of metabolism that may result in either an inactive or a severely reduced histidine ammonia-lyase (HAL) enzyme activity. The gene that encodes for HAL spans a roughly 25 kb and consists of 21 exons located at the 12q22-q24.1 position of human chromosome 12. There are eight mutations currently associated with autosomal recessive histidinemia, that include: four missense mutations, two exonic polymorphisms and two intronic polymorphisms. Diagnosis
Histidenemia is characterized by increased levels of histidine, histamine and imidazole in blood, urine and cerebrospinal fluid. This also results in decreased levels of the metabolite urocanic acid in blood, urine, and skin cells.