Development and validation of the Australian Aboriginal racial identity and self-esteem survey for 8-12 year old children (IRISE-C)

Introduction: In Australia, there is little empirical research of the racial identity of Indigenous children and youth as the majority of the current literature focuses on adults. Furthermore, there are no instruments developed with cultural appropriateness when exploring the identity and self-esteem of the Australian Aboriginal population, especially children. The IRISE-C (Racial Identity and Self-Esteem of children) inventory was developed to explore the elements of racial identity and self-esteem of urban, rural and regional Aboriginal children. This paper describes the development and validation of the IRISE-C instrument with over 250 Aboriginal children aged 8 to 12 years. Methods: A pilot of the IRISE C instrument was combined with individual interviews and was undertaken with 35 urban Aboriginal children aged 8-12 years. An exploratory factor analysis was performed to refine the survey and reduce redundant items in readiness for the main study. In the main study, the IRISE C was employed to 229 Aboriginal children aged 6-13 years across three sites (rural, regional and urban) in Western Australia. An exploratory factor analysis using Principal axis factoring was used to assess the fit of items and survey structure. A confirmatory factor analysis was then employed using LISREL (diagonally weighted least squares) to assess factor structures across domains. Internal consistency and reliability of subscales were assessed using Cronbach's co-efficient alpha. Results: The pilot testing identified two key concepts - children's knowledge of issues related to their racial identity, and the importance, or salience, that they attach to these issues. In the main study, factor analyses showed two clear factors relating to: Aboriginal culture and traditions; and a sense of belonging to an Aboriginal community. Principal Axis Factoring of the Knowledge items supported a 2-factor solution, which explained 38.7 % of variance. Factor One (Aboriginal culture) had a Cronbach's alpha of 0.835; Factor 2 (racial identity) had a Cronbach's alpha of 0.800, thus demonstrating high internal reliability of the scales. Conclusion: The IRISE-C has been shown to be a valid instrument useful of exploring the development of racial identity of Australian Aboriginal children across the 8-12 year old age range and across urban, rural and regional geographical locations

Effect of dopamine agonist medication on prolactin producing pituitary adenomas

Conventional light microscopy, immunocyto-chemistry, electron microscopy and in situ hybridization were used to evaluate the effect of dopamine agonists (bromocriptine-LAR and bromocriptine) on the morphology of surgically removed prolactin (PRL)-producing pituitary adenomas. Dopamine agonist therapy resulted in decrease of serum PRL, clinical improvement and tumour shrinkage. Using light and electron microscopy cellular atrophy, interstitial and perivascular fibrosis were noted; in several tumours connective tissue accumulation was pronounced. The cellular response was not uniform. In some adenomas populations of large cells and small cells were distinguished. The large cells contained immunoreactive PRL and expressed the PRL gene indicating resistance to dopamine agonists. It appears that these cells retained the potential to secrete PRL and proliferate despite exposure to dopamine agonists. In the small cells, PRL immunoreactivity and PRL gene expression decreased providing evidence that both PRL release and synthesis were blocked. Small cells can persist in tumours after discontinuation of dopamine agonist medication suggesting these small cells are irreversibly suppressed and are not capable of regaining their endocrine function and proliferative capability. The formation of irreversibly suppressed PRL cells may explain why some PRL-producing adenomas do not recur after withdrawal of dopamine agonists.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47517/1/428_2005_Article_BF01605931.pd

Endocrine and Growth Abnormalities in 4H Leukodystrophy Caused by Variants in POLR3A, POLR3B, and POLR1C.

CONTEXT: 4H or POLR3-related leukodystrophy is an autosomal recessive disorder typically characterized by hypomyelination, hypodontia, and hypogonadotropic hypogonadism, caused by biallelic pathogenic variants in POLR3A, POLR3B, POLR1C, and POLR3K. The endocrine and growth abnormalities associated with this disorder have not been thoroughly investigated to date. OBJECTIVE: To systematically characterize endocrine abnormalities of patients with 4H leukodystrophy. DESIGN: An international cross-sectional study was performed on 150 patients with genetically confirmed 4H leukodystrophy between 2015 and 2016. Endocrine and growth abnormalities were evaluated, and neurological and other non-neurological features were reviewed. Potential genotype/phenotype associations were also investigated. SETTING: This was a multicenter retrospective study using information collected from 3 predominant centers. PATIENTS: A total of 150 patients with 4H leukodystrophy and pathogenic variants in POLR3A, POLR3B, or POLR1C were included. MAIN OUTCOME MEASURES: Variables used to evaluate endocrine and growth abnormalities included pubertal history, hormone levels (estradiol, testosterone, stimulated LH and FSH, stimulated GH, IGF-I, prolactin, ACTH, cortisol, TSH, and T4), and height and head circumference charts. RESULTS: The most common endocrine abnormalities were delayed puberty (57/74; 77% overall, 64% in males, 89% in females) and short stature (57/93; 61%), when evaluated according to physician assessment. Abnormal thyroid function was reported in 22% (13/59) of patients. CONCLUSIONS: Our results confirm pubertal abnormalities and short stature are the most common endocrine features seen in 4H leukodystrophy. However, we noted that endocrine abnormalities are typically underinvestigated in this patient population. A prospective study is required to formulate evidence-based recommendations for management of the endocrine manifestations of this disorder