Longevity and Mortality in Down's Syndrome

Thase, Michael E.

doi:10.1111/j.1365-2788.1982.tb00144.x

Cited by 81 publications

(48 citation statements)

References 74 publications

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“…The incidence has been increasing in recent years [65]. Over the past few decades there has been a substantial increase in the life expectancy of children with Down's Syndrome, from around 12 yrs in the 1940s [66] to 35 yrs in the 1980s [67] to 60 yrs today [68,69]. Down's syndrome is strongly associated with the development of pulmonary hypertension, predominantly associated with upper airway obstruction and CHD.…”

Section: Review: Pah Associated With Chdmentioning

confidence: 99%

Pulmonary arterial hypertension associated with congenital heart disease

2012

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Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD.The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges.This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.

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Section: Review: Pah Associated With Chdmentioning

confidence: 99%

Pulmonary arterial hypertension associated with congenital heart disease

2012

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“…Indeed, some persons with the disorder have already survived into their ninth decade. 32 Respiratory infections have often been reported as a major cause of mortality in Down syndrome, [33][34][35][36] primarily due to the enhanced vulnerability of the patients' immune systems.…”

Section: Epidemiologymentioning

confidence: 99%

Clinical, social, and ethical implications of changing life expectancy in Down syndrome

Bittles

Glasson

2004

Develop Med Child Neuro

325

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Between 1 and 4% of the populations of developed nations are diagnosed with learning disability*. 1,2 In Australia, an estimated 1.9% of the population exhibit learning disability either as a primary disability or as a secondary condition, and approximately half of these people require continuing support in daily living, including mobility, self-care, and socialization. 3 After improvements in basic public health measures, life expectancy in most developed nations increased over the course of the 20th century, and this trend included people with learning disability. Thus, by the end of that century, the survival estimates for people with mild learning disability living in developed countries was 70 years, and nearly 60 years of age for those with severe learning disability. 4,5 The estimates of increased life expectancy mirror improved access to medical treatment for those with learning disability, and a shift in public attitude during the past 20 to 30 years that has resulted in better care within the community. The increased availability of more appropriate medication, surgical intervention, and overall health care management strategies have led to a positive effect on general health and have decreased mortality. More recently, national and international agencies have strongly argued for further improvements in health care opportunities for individuals with disabilities, and for the narrowing of health differentials between people with learning disability and the general population. 6-9 However, this is a complex issue with challenging social, economic, and ethical ramifications, because many people with learning disability are affected by major adverse health issues throughout their lives. As a result, increased survival is not only associated with a longer period of care, but is also related to a longer period of more specialized needs. Here we discuss these issues with specific reference to the circumstances of people with Down syndrome, although it should be emphasized that to a large degree they equally apply to a wide range of inherited disorders resulting in learning disability. EpidemiologyDown syndrome is the most commonly inherited form of learning disability, with an incidence rate of 1 out of every 650 to 1000 live births, 10-12 and in developed countries it accounts for 12 to 15% of the population of those with learning disability. 4,13,14 The actual number of Down syndrome conceptions is unknown, but occurrence of the disorder increases significantly with advancing maternal age, 15 and there is some evidence that advanced paternal age might also be involved. 16 There is a high rate of spontaneous prenatal loss and, for example, in one study 43% of Down syndrome pregnancies detected by chorionic villus sampling at 10 weeks gestation failed to reach term. 17 The trisomy 21 karyotype is present in 93 to 95% of people with Down syndrome; a further 3 to 4% exhibit translocation anomalies, and 1 to 3% are mosaic. 18-20 Down syndrome is usually associated with moderate to severe levels of learning disab...

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“…It is well documented that DS is associated with immune deficiencies and host response impairment (Kusters, et al, 2009;. Infections, in particular respiratory infections are an important cause of death in DS individuals (Thase, 1982). The most likely reason for this increased susceptibility to infection and reduced immunity in DS individuals is an increased dosage of a protein product or products encoded by chromosome 21.…”

Section: Discussionmentioning

confidence: 99%

Down Syndrome and Periodontal Disease

Khocht¹

2011

Genetics and Etiology of Down Syndrome

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Longevity and Mortality in Down's Syndrome

Cited by 81 publications

References 74 publications

Pulmonary arterial hypertension associated with congenital heart disease

Pulmonary arterial hypertension associated with congenital heart disease

Clinical, social, and ethical implications of changing life expectancy in Down syndrome

Down Syndrome and Periodontal Disease

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