نتایج جستجو برای: hydroxylase deficiency

تعداد نتایج: 152075  

2013
HIROYUKI KAWASHIMA

tinct 25-hydroxyvitamin D3-la-hydroxylase (la-hydroxylase) systems was studied in the kidneys of vitamin D-deficient rats; one is localized in the proximal convoluted tubule (PCT), is activated in vitamin D deficiency, and is regulated primarily by parathyroid hormone (PTH) via cyclic AMP; the other is localized in the proximal straight tubule (PST), is latent in vitamin D deficiency, and is se...

Journal: :Oman medical journal 2014
Waad-Allah S Mula-Abed Fathima B Pambinezhuth Manal K Al-Kindi Noor B Al-Busaidi Hilal N Al-Muslahi Mohammad A Al-Lamki

This is the first report of congenital adrenal hyperplasia (CAH) due to combined 17α-hydroxylase/17,20 lyase deficiency in an Omani patient who was initially treated for many years as a case of hypertension. CAH is an uncommon disorder that results from a defect in steroid hormones biosynthesis in the adrenal cortex. The clinical presentation depends on the site of enzymatic mutations and the t...

Journal: :The Journal of clinical endocrinology and metabolism 2001
I F Lau F C Soardi S H Lemos-Marini G Guerra M T Baptista M P De Mello

In the classical form of 21-hydroxylase deficiency, CYP21- affected genes either carry mutations present in the CYP21P pseudogene (microconversions) or bear a chimeric gene that replaces the active gene as a result of large conversion or deletion mutational events. Previous genotyping of 41 Brazilian patients revealed 64% microconversion, whereas deletions and large gene conversions accounted f...

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