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Article
Forging future organizational leaders for sustainability science
Leaders of sustainability research organizations need to provide an environment where interdisciplinary and transdisciplinary science flourish. Develo** the necessary leadership skills and attributes require...
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Article
Changing access to ice, land and water in Arctic communities
Arctic climate change has the potential to affect access to semi-permanent trails on land, water and sea ice, which are the main forms of transport for communities in many circumpolar regions. Focusing on Inui...
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Article
Sensitivity of systematic biases in South Asian summer monsoon simulations to regional climate model domain size and implications for downscaled regional process studies
Global climate models (GCMs) have good skill in simulating climate at the global scale yet they show significant systematic errors at regional scale. For example, many GCMs exhibit significant biases in South ...
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Article
A regulatory circuit that involves HR23B and HDAC6 governs the biological response to HDAC inhibitors
Histone deacetylase (HDAC) is an emergent anticancer target, and HR23B is a biomarker for response to HDAC inhibitors. We show here that HR23B has impacts on two documented effects of HDAC inhibitors; HDAC inh...
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Article
21st Century Drought Scenarios for the UK
A 6-month drought severity index (DSI6) is applied to each of the 11-member perturbed-physics ensemble (HadRM3-PPE-UK) monthly precipitation dataset from 1950 to 2100 to investigate projected 21st century drou...
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Article
MM5 simulations of interannual change and the diurnal cycle of southern African regional climate
Two cumulus convection and two planetary boundary layer schemes are used to investigate the climate of southern Africa using the MM5 regional climate model. Both a wet (1988/89) and a dry (1991/92) summer (Dec...
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Chapter
Abnormal Bone Metabolism in Thalassemia
Historically, homozygous beta-thalassemia major has been associated with marked osseous changes, originally described by Cooley et al. in 1927 [1]. The pathogenesis of the abnormal bony changes has been ascrib...
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Article
Bone marrow transplantation for Sanfilippo disease type B
Allogeneic bone marrow transplantation was performed on twins with Sanfilippo B disease. They were the first two patients with this disorder to undergo the procedure. There was definite evidence of engraftment...
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Article
102 PROGRESSIVE ENCEPHALOPATHY IN PATIENT TREATED WITH PITUITARY HUMAN GROWTH HORMONE
We report the case of a hypopituitary patient treated with human pituitary-derived growth hormone with a neurological disorder initially thought to be Creutzfeld-Jacob disease (CJD). Between 1978. and 1981. he...
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Article
HLA-DR 3 is associated with a more slowly progressive form of Type 1 (insulin-dependent) diabetes
The presence of HLA-DR 3 was analysed in 745 patients with Type 1 (insulin-dependent) diabetes with age at diagnosis between 1–19 years. HLA-DR 3 and/or 4 was found in 678/745 (91%) of the patients. Presence o...
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Article
Progressive adrenal failure in polyglandular autoimmune disease
We describe here the clinical course of a boy who developed isolated failure of the zona glomerulosa as part of polyglandular autoimmune disease(PAD)and in whom function of the zona fasciculata remains intact....
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Article
Close Genetic Linkage Between Congenital Adrenal Hyperplasia (21-hydroxylase deficiency) and HLA
We report on a combined study of 32 families from NY and Zurich with a total of 46 patients with CAH.It was found that all patients were HLA genotypically different from the healthy sibs and that 2 or more aff...
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Article
Deficiency of Cortisol 11β-Ketoreductase — A New Metabolic Defect
A deficiency of cortisol 11β-ketoreductase was observed in 2 patients with the syndrome of apparent mineralocorticoid excess and no evidence of oversecretion of any known steroid hormone. The syndrome is chara...
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Article
Deoxycorticosterone(DOC) and 18Hydroxy DOC (18OHDOC) in Childhood
Plasma DOC and 18OHDOC and urinary tetrahydroDOC (THDOC),free DOC, and 18OHDOC (per m2/24h) were measured by radioimmunoassay in children with no endocrinopathies (age 6m-18yr). No hormonal differences were seen ...
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Article
ABSENCE OF LINKAGE BETWEEN C-11 OH DEFICIENT CONGENITAL ADRENAL HYPERPLASIA AND HLA
A collaborative study revealed an unusually high frequency of congenital adrenal hyperplasia (CAH) due to C-11 OH deficiency in Israel. Seventeen families with 24 affected individuals were identified, mostly o...
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Article
Aldosterone metabolism after prolonged ACTH administration in juvenile hypertension
The effect of a 5-day continuous ACTH infusion (Acthar, 40 U/24h) on aldosterone metabolism was evaluated in 3 hypertensive children with hyperaldosteronism, 2 children with essential hypertension, and 1 normo...
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Article
Hyperaldosteronism with non-suppressible ACTH
In an 11 yr old male with the syndrome of primary hyperaldosteronism and hypertension due to adrenal hyperplasia, we have found evidence for abnormal ACTH regulation. Since renin is completely suppressed, it i...
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Article
Psychosocial dwarfism: Normal Somatomedin (SM)
Psychosocial Dwarfism (PSD) is a syndrome of severe growth failure in an abnormal home environment with a hostile or a depriving parent. We studied an 8 yr. old boy with PSD. While in the hospital after an ini...
