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DR. WEIL: Bone disorder often leads to fractures

Published February 16, 2009 at 6 p.m.

My 2-year-old niece has been diagnosed with osteogenesis imperfecta. It is apparently a mild form. What can you tell me about it, and what can we do for her?

The main problem in osteogenesis imperfecta (OI) is bones that break easily. The disorder results from a genetic mutation that affects the body's production of collagen, a critical protein used to build and maintain bones. Mild cases may result in just a few fractures, while severe ones can lead to hundreds of bone breaks over the course of a lifetime. These fractures can occur for no apparent reason, or as a result of such ordinary events as coughing or rolling over during sleep.

All told, there are eight recognized types of OI. The most common form is the mildest. Most patients with it have a normal or near-normal stature with little or no noticeable bone deformity, even though their bones may break easily. Other characteristics include loose joints, muscle weakness and a tendency toward spinal curvature.

Some patients develop hearing loss relatively early in life, during their 20s or 30s. As yet there's no cure for OI. Instead, the focus is on preventing or controlling fractures. Exercise throughout life is important to building both muscle and bone strength. Swimming and water therapy are especially helpful because moving in water presents little risk of fractures. Walking is also recommended (with or without such mobility aids as leg braces).

Maintaining a healthy weight throughout life is also important. Extra weight can endanger fragile bones and compromise mobility. As children grow, it's a good idea to consult a nutritionist or a dietitian to learn how to limit portion size and make healthy food choices. I recommend a good multivitamin/multimineral supplement for insurance.

You can find information on the disease itself as well as on ongoing research through the OI Foundation at oif.org. Learn as much as you can about it, and keep up with medical advances.

What do you think of lipo-dissolve, the fat-burning injections that have become so popular?

This technique is an update of mesotherapy, a method I've crit- icized. Like mesotherapy, lipo-dissolve is used for "spot reduction" of fat de- posits and cellulite. It's promoted as an alternative to liposuction to re- duce sagging jaw lines, "love handles" and upper-back fat. My objections to mesotherapy center on the fact that there doesn't seem to be any standardization of the "fat-burning" substances injected.

Different doctors use different combinations of drugs, usually a melange of plant extracts, vitamins, Novocain and aminophylline (a stim- ulant drug from tea formerly used to treat the symptoms of asthma). Another substance that's often used is phosphatidylcholine, a constituent of cell membranes that can also be isolated from egg yolks and soybeans. When injected into the skin, it can cause extreme swelling and inflammation at the injection site.

Based on what I've read, lipo- dissolve contains a mixture of phosphatidylcholine and sodium deoxycholate, a bile salt that aids waste removal. This concoction, called PCDC, theoretically produces an inflammatory response that breaks down fat so it can be eliminated from the body. Side effects include redness, swelling, burning and pain, the hallmarks of inflammation.

I have my doubts about the effectiveness and safety of lipo-dissolve, and so do the American Society of Plastic Surgeons, the American Society of Dermatologic Surgery and the American Society of Aesthetic Plastic Surgery, all groups made up of physicians who spend much of their working lives dealing with cosmetic issues. The three organizations maintain that there's no evidence to demonstrate that the procedure is worthwhile.

Worse are reports of complications, including bacterial infections, granulomas (disfiguring masses of chronically inflamed tissue) and localized necrosis (tissue death).

drweil.com

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