ArtsAutosBooksBusinessEducationEntertainmentFamilyFashionFoodGamesGenderHealthHolidaysHomeHubPagesPersonal FinancePetsPoliticsReligionSportsTechnologyTravel

Clinical Significance Of The Abnormalities Of Sex Hormones Caused By Adrenocortical Lesions

Updated on February 11, 2014

Sex Hormones In Action

Source

A General Overview

Anatomy: Adrenals are paired glands each weighing about 4-7g. Section shows an outer cortex and inner medulla. The arterial supply is derived from several branches of the phrenic, renal and ovarian or spermatic arteries and aorta. Venous drainage on the left is into the left renal vein and inferior vena cava on the right. Histologically, three zones are visible- the zona glomerulosa, zona fasciculate and zona reticularis from without inwards.

Embryology: Adrenal cortex in the fetus is formed during the fourth to sixth weeks of gestation from the celomic mesoderm of the posterior abdominal wall. The permanent adrenal cortex is formed from the small basophilic cells which appear five weeks later. The adrenal medulla develops in the seventh week from sympathogonia which migrate from the neural crest into the fetal adrenal cortex. The adrenals are relatively bigger at birth, but with the passage of time, the size reduces since the inner zone of acidophilic cells undergo involution.

Physiology: Several hormones are produced by the adrenal cortex. It synthesizes cholesterol and also takes cholesterol from the circulation for the production of its hormones. Synthesis of hormones take place in several steps.

The adrenal cortical Hormones

 
Secretory rate in mg/24 hours
Cortisol (hydrocortisone)
12- 30
Corticosterone
1- 4
Aldosterone
0.05- 0.15
Deoxycorticosterone
0.05- 0.2
Dehydroepiandrosterone
15- 20

Adrenocorticotropic hormone (ACTH) secreted by the anterior pituitary stimulates secretion of adrenal cortical hormones (glucocroticoids) and sex hormones) by acting on specific receptors present on the adrenal cortex through the mechanism of c-AMP.

Adrenal cortex may secrete excess of androgens if it is the seat of tumour or congenital enzyme defects. The final clinical picture is determined by the sex of the patient, age of onset of the disorder and its severity. The condition may remain unnoticed in adult males. Women develop virilisation. Male children show precocious puberty. Female children develop clitoromegaly and other signs of hyperandrogenism.

Drake Congenital Adrenal Hyperplasia

Source

Adrenogenital syndrome (Congenital Adrenal Hyperplasia)

This rare disorder is characterized by virilisation in female and precocious puberty in male children. The underlying defect is congenital deficiency of enzymes leading to metabolic block in hormone synthesis. This results in the accumulation of intermediary metabolites with androgenic activity. Deficient synthesis of glucocorticoids and mineralocorticoids results in functional adrenocortical deficiency.

Etiology: The enzyme defect is transmitted as an autosomal recessive disorder, with clinical expression in the homozygous state. Females are affected more than males. The severity of enzyme defect determines the age of manifestation. Severe defects manifest earlier in life. At least six different enzyme defects are known, but four of these are more frequent. These are C-21-hydroxylase defect, C-11 hydroxylase defect, 3-beta hydroxydehydrogenase defect and C-18 dehydrogenase defect. In each type, slight differences are recognizable.

Clinical features: In some cases, the newborn female infants show pseudohermaphrodism due to androgen effect on the genitalia. Male infants show precocious sexual development after birth. Manifestations of adrenal insufficiency are failure to thrive, vomiting, hyponatremia and hypotension. Death may occur due to adrenal insufficiency unless the condition is recognized and treated.

Milder cases manifest as virilisation in girls and sexual precocity in boys. Due to the premature fusion of epiphyses, growth is stunted. Hyperandrogenisms causes testicular atrophy and infertility in adult males.

Diagnosis: Adrenogenital syndrome should be considered in the differential diagnosis of all cases of hermaphroditism or ambigious sexual development. Diagnosis is confirmed by estimation of the products of androgens in urine.

Treatment: Deficiency of glucorcoticoids and mineralocorticoids should be corrected by supplementation. Liberal intake of salt helps in mild cases. Fluodrocortisone in a daily dose of 0.05 to 0.1mg orally is given to replace mineralocorticoids. Prednisolone in a dose of 5 mg/day serves to overcome glucocorticoid deficiency. Surgery may be required for cosmetic correction of the genitalia. In those cases in whom adrenal tumours or hyperplastic glands are demonstrable, surgical removal is indicated.

© 2014 Funom Theophilus Makama

Comments

    0 of 8192 characters used
    Post Comment

    No comments yet.

    working

    This website uses cookies

    As a user in the EEA, your approval is needed on a few things. To provide a better website experience, hubpages.com uses cookies (and other similar technologies) and may collect, process, and share personal data. Please choose which areas of our service you consent to our doing so.

    For more information on managing or withdrawing consents and how we handle data, visit our Privacy Policy at: https://hubpages.com/privacy-policy#gdpr

    Show Details
    Necessary
    HubPages Device IDThis is used to identify particular browsers or devices when the access the service, and is used for security reasons.
    LoginThis is necessary to sign in to the HubPages Service.
    Google RecaptchaThis is used to prevent bots and spam. (Privacy Policy)
    AkismetThis is used to detect comment spam. (Privacy Policy)
    HubPages Google AnalyticsThis is used to provide data on traffic to our website, all personally identifyable data is anonymized. (Privacy Policy)
    HubPages Traffic PixelThis is used to collect data on traffic to articles and other pages on our site. Unless you are signed in to a HubPages account, all personally identifiable information is anonymized.
    Amazon Web ServicesThis is a cloud services platform that we used to host our service. (Privacy Policy)
    CloudflareThis is a cloud CDN service that we use to efficiently deliver files required for our service to operate such as javascript, cascading style sheets, images, and videos. (Privacy Policy)
    Google Hosted LibrariesJavascript software libraries such as jQuery are loaded at endpoints on the googleapis.com or gstatic.com domains, for performance and efficiency reasons. (Privacy Policy)
    Features
    Google Custom SearchThis is feature allows you to search the site. (Privacy Policy)
    Google MapsSome articles have Google Maps embedded in them. (Privacy Policy)
    Google ChartsThis is used to display charts and graphs on articles and the author center. (Privacy Policy)
    Google AdSense Host APIThis service allows you to sign up for or associate a Google AdSense account with HubPages, so that you can earn money from ads on your articles. No data is shared unless you engage with this feature. (Privacy Policy)
    Google YouTubeSome articles have YouTube videos embedded in them. (Privacy Policy)
    VimeoSome articles have Vimeo videos embedded in them. (Privacy Policy)
    PaypalThis is used for a registered author who enrolls in the HubPages Earnings program and requests to be paid via PayPal. No data is shared with Paypal unless you engage with this feature. (Privacy Policy)
    Facebook LoginYou can use this to streamline signing up for, or signing in to your Hubpages account. No data is shared with Facebook unless you engage with this feature. (Privacy Policy)
    MavenThis supports the Maven widget and search functionality. (Privacy Policy)
    Marketing
    Google AdSenseThis is an ad network. (Privacy Policy)
    Google DoubleClickGoogle provides ad serving technology and runs an ad network. (Privacy Policy)
    Index ExchangeThis is an ad network. (Privacy Policy)
    SovrnThis is an ad network. (Privacy Policy)
    Facebook AdsThis is an ad network. (Privacy Policy)
    Amazon Unified Ad MarketplaceThis is an ad network. (Privacy Policy)
    AppNexusThis is an ad network. (Privacy Policy)
    OpenxThis is an ad network. (Privacy Policy)
    Rubicon ProjectThis is an ad network. (Privacy Policy)
    TripleLiftThis is an ad network. (Privacy Policy)
    Say MediaWe partner with Say Media to deliver ad campaigns on our sites. (Privacy Policy)
    Remarketing PixelsWe may use remarketing pixels from advertising networks such as Google AdWords, Bing Ads, and Facebook in order to advertise the HubPages Service to people that have visited our sites.
    Conversion Tracking PixelsWe may use conversion tracking pixels from advertising networks such as Google AdWords, Bing Ads, and Facebook in order to identify when an advertisement has successfully resulted in the desired action, such as signing up for the HubPages Service or publishing an article on the HubPages Service.
    Statistics
    Author Google AnalyticsThis is used to provide traffic data and reports to the authors of articles on the HubPages Service. (Privacy Policy)
    ComscoreComScore is a media measurement and analytics company providing marketing data and analytics to enterprises, media and advertising agencies, and publishers. Non-consent will result in ComScore only processing obfuscated personal data. (Privacy Policy)
    Amazon Tracking PixelSome articles display amazon products as part of the Amazon Affiliate program, this pixel provides traffic statistics for those products (Privacy Policy)