Tuesday, May 29, 2007

Werewolf Syndrome aka Congenital Generalized Hypertrichosis

Werewolf Syndrome, or, Congenital Generalized Hypertichosis is an extremely rare genetic disorder, causing hair follicles to work overtime!

In earlier eras, people afflicted with CGH would stay indoors during the day, going out only at night, to avoid being ostracized. Because of this behavior, people began to associate these unfortunate folks with werewolves, thus the vulgar name of Werewolf Syndrome. CGH is characterized, in its severest form, by thick hair covering the entire body, sparing the palms of the hands, and soles of the feet! Researchers believe the cause to be a genetic mutation, or an “awakening” of a very old, dormant gene-harkening back in evolution to hairier times!

CGH is extremely rare, with only about 50 documented cases since the Middle Ages. One of the first documented cases was found in French King Henry II’s court! King Henry was highly interested in human oddities and quirks of nature, and in 1547 was given, as a gift, a 10 year old boy who appeared to be half human, half animal. Four inch, thick blond fur covered his entire body, except for lips and eyes. The boy’s name was Pedro Gonzales, born in the Canary Islands. Pedro married a lovely French woman, and fathered many children, five of which inherited their father's genetic defect. Many portraits were painted of this unusual family-some still hang in the Ambras Castle, near Innsbruck, Austria. Since then, known cases have appeared in China, Poland, Germany, Russia, and Mexico.


Researchers are familiar with other atavistic genetic behavior. Some rare examples include additional nipples, and small tail-like extrusions at the end of the spine. The defective gene in CGH is passed by both sexes, to 50% of their offspring. Typically, fetuses lose their fine body hair, called lanugo, by the end of the seventh and eight month of gestation. Babies afflicted with CGH are born with this body hair intact, which occasionally fades in adulthood, but typically lastis a lifetime.


Currently, the best known cases of CGH occurs in a family living in Mexico. Sadly, much of this family has resorted to working in circuses and “freak” shows to earn a living. This Mexican family grows an even thicker, darker body hair than their Asian, and European counterparts. Men have thicker and denser hair than the women in this family, suggesting an X-linked dominant pattern of inheritance.


“"This is probably a mutation of a gene that was a sleeping beauty," said Dr. Jose M. Cantu, head of genetics at the Mexican Institute for Social Security in Guadalajara, an author of the new report. "The mutation awakened a gene that had been put aside during evolution."

But Dr. Cantu and his colleagues emphasized that the idea of generalized hypertrichosis as an atavistic mutation was only a theory. "At this point it's strictly speculation, though the idea is a very interesting one," said Dr. Pragna I. Patel of the Human Genome Center at Baylor College of Medicine in Houston, another author of the report, which appears in the June issue of Nature Genetics.

Biologists have observed many other mutations that they suggest fall into this class of atavisms, the reappearance of normally dormant traits. Some people are born with multiple sets of nipples, for example, just as most nonprimate mammals have a double ridge of mammary tissue down the length of the underside of the torso. In very rare cases, girls develop entire extra breasts at puberty.

Other examples of atavistic mutations include the extension of the human coccyx into a small tail, the appearance of hind limbs in whales and the growth of extra toes on horses and cats.

"Atavistic mutations tell us that a lot of information is kept around for a very long time," said Dr. Brian K. Hall, a developmental biologist at Dalhousie University in Halifax, Nova Scotia. "Just because an animal isn't using a gene anymore doesn't mean the information just disappears." Dr. Hall wrote a commentary about atavistic mutations that appears with the report on hypertrichosis.”

http://query.nytimes.com/gst/fullpage.html?res=990CE5D91130F932A05756C0A963958260&sec=health&spon=&pagewanted=print


Read an interview with Chuy, a Mexican man with hypertrichosis.

http://www.listentome.net/stuff97.php


Five TV’s article on Chuy:

http://www.five.tv/programmes/hiddenlives/wolfboy/


Friday, May 18, 2007

Monday, May 14, 2007

Ipods and Pacemaker Malfunction?




The results of an Ipod's effects on cardiac pacemakers was presented recently at the Heart Rhythm Society, in Denver, Colorado. (Reported by the Denver Post). The senior author of the study was Dr. Jongnarangsin.

A study at
the Thoracic and Cardiovascular Institute at Michigan State University found that an Ipod can cause pacemakers to malfunction by causing electro-magnetic interference.

What's interesting about this study is that it was prompted by a Jay Thaker, a high school student from Okemos, Michigan. Since Jay's mom is a rheumatologist, and his dad is an
electrophysiologist, his interest in physiology is not not surprising. Jay had been pondering the idea of whether an Ipod could cause interference with pacemakers. When he and his dad searched online, they found no information. (Too bad Google Answers is among the dearly departed!)

When asked by one of his patients if there would be a problem using an Ipod with his pacemaker, Jay's father suggested he contact Dr. Krit Jongnarangsin, an assistant professor in the Division of Cardiovascular Medicine at the University of Michigan.

Dr. Jongnarangsin said that most patients with pacemakers are not Ipod users, but they could have children or grandchildren who do.

The study consisted of a cohort of 83 volunteers with pacemakersand found that Ipods interfered with pacemakers about 30 per cent of the time. In one case, a pacemaker stopped working completely!

Read more :
Reuters
http://www.pcworld.com/article/id,131736/article.html&tk=nl_wbxnws#

MSNBC
http://www.msnbc.msn.com/id/18598054/

Monday, May 07, 2007

Cubital Tunnel


Everyone has heard about carpal tunnel these days, but few have heard about cubital tunnel! Cubital tunnel, like carpal tunnel syndrome, is a compressive neuropathy, meaning nerve pain from a stretched or compressed nerve.


Cubital tunnel is caused by pressure on the ulnar nerve, a primary nerve, supplying the hand. CT can cause numbness and tingling of the pinkie and ring fingers. Pain on the side of the arm closest to the chest can also occur in cubital tunnel.

The ulnar nerve reaches from the spinal cord to the hand and forearm and hand, including the skin on the on the outer portion of the hand. (The pinkie side). The ulnar nerve passes over a bony portion of the elbow (medial epicondyle) and when stretched, can cause tingling, even an electrical shock feeling, in the ring finger and pinkie!

How is cubital tunnel treated? The first step is to try and avoid bending the elbow for long periods of time. Don't lean on your elbows! Constant phone use can aggravate CT as the arm is bent while holding a phone-land line or cell! Surgery is the treatment of last resort... taking ibuprofen can help, and some claim that vitamin B6 (never more than 100mg per day) helps alleviate pain and tingling.

Another home remedy is to roll the elbow in a towel, like a pig in a blanket. Wrap the towel with surgical tape or pin with safety pins. This will keep the elbow extended, not bent. Since many people are afflicted by CT due to bending their elbows while sleeping, this will prevent them from folding their arms, and alleviating pressure on the ulnar nerve.


For more in-depth information about cubital tunnel, visit these links:
e-Medicine
http://www.emedicine.com/orthoped/topic479.htm


Hand University
http://www.handuniversity.com/topics.asp?Topic_ID=8