Skip to Main Content

++

Inherited syndrome with mild psychomotor retardation, hypogonadism, short stature, brittle hair.

++

Amish Brittle Hair Syndrome; BIDS; Brittle Hair, Intellectual Deficit, Decreased Fertility, and Short Stature Syndrome.

++

Initially described in 25 children of an Amish kindred with autosomal recessive transmission.

++

The most striking signs are brittle hair and short stature. The hair defect is secondary to a markedly reduced cystine content resulting in lack of sulfur-rich matrix proteins. The microscopic appearance of the hair shows an irregular and grooved surface. The same underlying defect seems to be responsible for nail dystrophy (brittle, short nails).

++

The intellectual deficit usually is mild. Reduced fertility is secondary to hypogonadism.

++

Obtain a full history of the patient's neurologic status. Depending on the severity of mental retardation, the patient may benefit from anxiolytic premedication. No specific anesthetic considerations.

++

Brittle Hair and Mental Deficit Syndrome: Genetic disorder characterized by abnormal hair fibers and mental deficiency.

++

Pollitt Syndrome: Inherited syndrome with developmental delay and trichorrhexis nodosa.

++

Netherton Syndrome: Most likely autosomal recessive transmitted inborn error of metabolism that manifests with bamboo hair, atopic diathesis, congenital ichthyosiform erythroderma, and hypogammaglobulinemia.

Allen RJ: Neurocutaneous syndromes in children. Postgrad Med 50:83, 1971.  [PubMed: 5566208]
Jackson CE, Weiss L, Watson JH: “Brittle” hair with short stature, intellectual impairment and decreased fertility: An autosomal recessive syndrome in an Amish kindred. Pediatrics 54:201, 1974.  [PubMed: 4847854]

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessAnesthesiology Full Site: One-Year Subscription

Connect to the full suite of AccessAnesthesiology content and resources including procedural videos, interactive self-assessment, real-life cases, 20+ textbooks, and more

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessAnesthesiology

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.