Cretinism: Early diagnosis its relation to mental prognosis

Raiti, Salvatore; Newns, G. H.

doi:10.1136/adc.46.249.692

Cited by 139 publications

(37 citation statements)

References 9 publications

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“…This allowed us to detect and treat most of the CH infants before irreversible damage has occurred. It was shown that starting treatment before the age of 3 months may improve the eventual IQ and prevent mental retardation [1, 14, 15]. Despite the fact that the rate of home deliveries in Bethlehem and Jenin is similar to that of the other regions, 82% of the infants were screened before 7 days of age.…”

Section: Discussionmentioning

confidence: 99%

Congenital Hypothyroidism Screening in the West Bank: A Test Case for Screening in Developing Regions

1998

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Screening for congenital hypothyroidism (CH) among the Arab population of the West Bank began in May 1987 as part of the neonatal screening program in Israel. In the West Bank many infants are born at home or are released from the hospital on the 1st day after birth and thus cannot be screened. However, we tried to reach the infants before the age of 1 month at the maternal and child health centers, where they receive immunization. In this screening program, 64% of the infants were sampled by the 1st week and 93% by 3 weeks of life. In contrast to the screening in Israel, where thyroxine determination is followed by thyroid-stimulating hormone measurement, in the West Bank thyroid-stimulating hormone was tested first in order to decrease the recall rates. From June 1990 to February 1994, 49,694 infants were screened in the West Bank, of whom 24 with CH were detected (an incidence of 1:2,070). From January 1987 to February 1994, 28,938 infants were screened in East Jerusalem, of whom 20 with CH were detected (incidence 1:1,447). There were differences between the incidence rates in the various districts. The incidence rates were higher than those reported from industrialized countries, but similar to those found in Saudi Arabia. This may be due to the high degree of consanguineous marriages among Arab populations and to environmental factors. In conclusion, in spite of the many difficulties, both practical and political, CH screening in the West Bank is feasible. Although screening all newborns shortly after birth is not possible, this study shows that a high percentage of them can be screened at a time when they can still be effectively treated. Our results could be used in due time as a baseline for a future independent screening program.

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Section: Discussionmentioning

confidence: 99%

Congenital Hypothyroidism Screening in the West Bank: A Test Case for Screening in Developing Regions

1998

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“…While this window of opportunity has not been clearly delineated in humans, there are reports of normal intellectual function in patients with hypothyroidism who underwent thyroid replacement therapy (TRT) prior to 3 or even 7 months of age. [10][11][12] Despite these findings, the lack of a comprehensive evaluation of a wide variety of cognitive domains other than general intelligence (eg, attention, language, learning and memory skills) necessitates restraint in inferring that early intervention with TRT results in normal neurocognitive functioning. Evoked potential studies of auditory brainstem responses in children with congenital hypothyroidism who received postnatal TRT as early as 3 weeks of age showed significant audiometric deficits well into midchildhood.…”

Section: Congenital Hypothyroidismmentioning

confidence: 99%

Neurocognitive Aspects of Hypothyroidism

1998

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Hypothyroidism is associated with significant neurocognitive deficits that develop across the life span. This article discusses the patterns of cognitive deficits associated with congenital and adult-onset hypothyroidism. A review of the extant literature shows that the successful treatment of clinically evident thyroid gland hypofunction, resulting in a return to euthyroidism in both infants and adults, may be associated with only partial and typically inconsistent patterns of recovery of overall neurocognitive function. In addition to demonstrating different patterns of cognitive impairments, patients with congenital and acquired adult-onset hypothyroidism have variable responses to thyroid replacement therapy, which increases the risk of higher neurocognitive morbidity associated with congenital hypothyroidism. An evaluation of the commonly held view that hypothyroid dementia is an imminently reversible condition is only partially supported by the medical literature, which is fraught with methodological and conceptual shortcomings. I offer some recommendations for addressing the cognitive and behavioral management concerns of individuals with clinical hypothyroidism.

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“…Crucial to the management of congenital hypothyroidism is the institution of treatment as soon after birth as possible because the later treatment begins, the greater the degree of irreversible mental impairment. 2 3 This underlines the potential benefits of an early neonatal screening programme. The advent of radioimmunoassay for thyroxine and later of thyroid stimulating hormone (TSH) in small samples of whole blood enabled routine screening to become a reality and programmes were introduced throughout Europe and North America with the aim of preventing mental impairment due to hypothyroidism.…”

mentioning

confidence: 99%

Audit of screening for congenital hypothyroidism.

Pharoah

Madden

1992

Archives of Disease in Childhood

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The effectiveness and efficiency of the screening programme for congenital hypothyroidism in the Mersey region from 1983 to 1989 was examined by an analysis oflaboratory, manual and computerised district records, case notes, and Hospital Activity Analysis data.Births from 1 January 1983 to 31 December 1989 formed the study cohort and the proportion of live births screened, age when treatment was started, validity of the screening test, and the outcome of treatment were determined. In nine out of 10 districts coverage of the neonatal population by the screening programme was incomplete and there was failure to follow up infants who had not been screened. The sensitivity of the test was 97% and the specificity was 99 90/o. In 10% of the positive cases treatment was delayed beyond 21 days, the latest was 26 days.It is concluded that administrative deficiencies were predominantly responsible for the inefficiencies of the screening programme. The long term follow up of diagnosed cases should become part ofthe monitoring process.

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Cretinism: Early diagnosis its relation to mental prognosis

Cited by 139 publications

References 9 publications

Congenital Hypothyroidism Screening in the West Bank: A Test Case for Screening in Developing Regions

Congenital Hypothyroidism Screening in the West Bank: A Test Case for Screening in Developing Regions

Neurocognitive Aspects of Hypothyroidism

Audit of screening for congenital hypothyroidism.

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