Survey data from Australia, Fiji, Malaysia, Philippines, and the Republic of Korea are used to model older workers' choices. The co-existence of a traditional sector along with a modern sector in much of the Asia Pacific region offers a traditional family lifestyle, as well as paid work and retirement choices. Differences are analyzed between countries, by expanding choices to include traditional family support, and within countries by use of ethnic group dummies along with economic factors. Results demonstrate the importance of cultural and developmental factors within and between countries. There is less dependency on family in more developed countries but inverse effects for wealthy persons. Wealthier households in more developed countries depend upon income from their own work while in developing countries they depend on families. Women in the developing countries work whilst those in developed countries tend to retire with their husbands to share retirement leisure.
Cornelia de Lange Syndrome (CdLS) is a rare, dominantly inherited multisystem developmental disorder characterized by highly variable manifestations of growth and developmental delays, upper limb involvement, hypertrichosis, cardiac, gastrointestinal, craniofacial, and other systemic features. Pathogenic variants in genes encoding cohesin complex structural subunits and regulatory proteins (NIPBL, SMC1A, SMC3, HDAC8, and RAD21) are the major pathogenic contributors to CdLS. Heterozygous or hemizygous variants in the genes encoding these five proteins have been found to be contributory to CdLS, with variants in NIPBL accounting for the majority (>60%) of cases, and the only gene identified to date that results in the severe or classic form of CdLS when mutated. Pathogenic variants in cohesin genes other than NIPBL tend to result in a less severe phenotype. Causative variants in additional genes, such as ANKRD11, EP300, AFF4, TAF1, and BRD4, can cause a CdLS-like phenotype. The common role that these genes, and others, play as critical regulators of developmental transcriptional control has led to the conditions they cause being referred to as disorders of transcriptional regulation (or "DTRs"). Here, we report the results of a comprehensive molecular analysis in a cohort of 716 probands with typical and atypical CdLS in order to delineate the genetic contribution of causative variants in cohesin complex genes as well as novel candidate genes, genotype-phenotype correlations, and the utility of genome sequencing in understanding the mutational landscape in this population.