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Brittle bone disease

Osteogenesis imperfecta (OI), commonly known as brittle bone disease, is a group of genetic bone disorders. People with OI either have less collagen than normal or the quality is poorer than normal. As collagen is an important protein in bone structure this impairment causes those with the condition to have weak or fragile bones. more...

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As a genetic disorder, OI is a autosomal dominant defect. Most people with OI receive it from a parent but it can also be an individual (de novo or "sporadic") mutation.

Types

There are four types of OI, though the symptoms range from person to person. Type I is the most common and mildest form, followed by Type IV, Type III and Type II. A Type V has been proposed based on studies of Type IV.

Type I

  • OMIM 166200 - Type I
  • OMIM 166240 - Type IA

Collagen is normal but not of a high enough quantity:

  • Bones fracture easily, especially before puberty
  • Slight spinal curvature
  • Loose joints
  • Poor muscle tone
  • Discolouration of the sclera (whites of the eyes)
  • Early loss of hearing

Type II

  • OMIM 166210 - Type II

Collagen is not of a sufficient quality or quantity

  • Most cases die before adulthood
  • Severe respiratory problems due to underdeveloped lungs
  • Severe bone deformity and small stature

Type III

  • OMIM 259420 - Type III

Collagen quantity is sufficient but is not of a high enough quality

  • Bones fracture easily, sometimes even before birth
  • Bone deformity, often severe
  • Respiratory problems possible
  • Short stature, spinal curvature and barrel-shaped rib cage
  • Loose joints
  • Poor muscle tone in arms and legs
  • Discolouration of the sclera (whites of the eyes)
  • Early loss of hearing

Type IV

  • OMIM 166220 - Type IV

Collagen quantity is sufficient but is not of a high enough quality

  • Bones fracture easily, especially before puberty
  • Short stature, spinal curvature and barrel-shaped rib cage
  • Bone deformity is mild to moderate
  • Discolouration of the sclera (whites of the eyes)
  • Early loss of hearing

Treatment

At present there is no cure for OI so treatment is aimed at maintaining mobility and strengthening bones as much as possible.

Physiotherapy is used to strengthen muscles and improve motility in a gentle manner which minimises bone breakages. This often involves hydrotherapy and the use of support cushions to improve posture. Individuals are encouraged to change positions regularly throughout the day in order to balance the muscles which are being used and the bones which are under pressure. One of the biggest problems is that children often develop a fear of trying new ways of moving due to movement being associated with pain. This can make physiotherapy difficult to administer to young children.

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A recipe for strong bones: diet strategy there's more to dietary calcium than just bone health: this vital mineral also may play an important role in weight
From Shape, 5/1/04 by Robin Vitetta-Miller

Osteoporosis, the loss of bone density, is responsible for 1.5 million fractures of the spine, hip and wrist every year in the United States. While these brittle-bone injuries may seem like a danger that threatens only older women, the sooner you act to fend off this preventable disease, the safer you'll be.

One of the best defenses against osteoporosis is a diet high in calcium. Nonfat and lowfat dairy products are the most effective sources of the mineral, but you can find moderate amounts in dark leafy green vegetables (such as spinach, kale and collard greens), canned salmon (if you eat the bones), almonds and calcium-fortified juices and cereals.

It's best to get your calcium from food rather than supplements because food provides you with other critical nutrients as well: vitamin D from dairy products (which works with calcium to maintain strong bones), omega-3 fatty acids from fish, vitamin C from fortified juices, and fiber from dark-green vegetables and fortified cereals. And getting calcium from food sources also may help you lose weight (see "Calcium & Weight Loss," page 218).

The recommended daily allowance for calcium for women ages 19-50 is 1,000 milligrams per day; for women 51 and older, it's 1,200 mg per day. Because May is Osteoporosis Awareness and Prevention Month, we've created three delicious recipes that boast flavor, nutrients and loads of calcium-rich ingredients to help you meet your daily requirement. (To learn some surprising myths about osteoporosis and how to keep the disease at bay, turn to "Bad to the Bone," on page 256.)

Corn-Potato Chowder

Serves 4

Prep time: 5-10 minutes

Cook time: 10 minutes

Note: If desired, add 1/2 pound of diced lean ham (252 calories and 8 grams of fat, 2 grams saturated) when you simmer the vegetables in milk.

Combine first 9 ingredients in a large stockpot; set over high heat and bring to a boil. Reduce heat to medium and simmer 10 minutes, until potatoes are fork-tender. Remove from heat and stir in buttermilk.

Transfer 3 cups only of the corn-potato mixture to a blender or food processor and puree until smooth. Return puree to remaining corn-potato mixture in stockpot and set pan over medium heat. Simmer 1 minute to heat through. Remove from heat and stir in fresh cilantro.

Ladle corn-potato chowder into bowls and top with chives.

Nutrition Score per serving (2 cups): 245 calories, 5% fat (1 g; <1 g saturated), 76% carbs (47 g), 19% protein (12 g), 5 g fiber, 312 mg calcium, 1 mg iron, 227 mg sodium.

Salmon Sandwich With Yogurt Sauce

Serves 4

Prep time: 6 minutes

Cook time: 12-14 minutes

Preheat oven to 425[degrees]F. In a large bowl, gently combine salmon, mayonnaise, bread crumbs, oregano and black pepper until blended. Shape mixture into 4 cakes, each about 1 inch thick.

Heat oil in a large, oven-proof skillet over medium-high heat. Add salmon cakes and saute 1-2 minutes per side, until browned. Transfer pan to oven and bake 10 minutes, until salmon cakes are golden.

Meanwhile, to make the yogurt sauce, stir together yogurt, chives, Dijon mustard and tarragon.

Arrange each salmon cake on a slice of bread. Spoon yogurt sauce over top and add second slice of bread.

Nutrition Score per serving (1 salmon cake, 1/4 cup sauce, 2 slices bread): 469 calories, 26% fat (14 g; 3 g saturated), 32% carbs (38 g), 42% protein (50 g), 5 g fiber, 346 mg calcium, 3 mg iron, 640 mg sodium.

Almond-Crusted Chicken With Creamy Blue Cheese

Serves 4

Prep time: 5 minutes

Cook time: 10-15 minutes

Salt and pepper both sides of chicken. Mix flour and garlic powder with a fork in a shallow dish; add chicken and turn to coat both sides well.

Place egg whites in a separate shallow dish, add flour-coated chicken and turn to coat both sides. Press almonds into both sides of chicken.

Heat olive oil in a skillet over medium-high heat. Add chicken and saute 1 minute per side, until almonds are golden. Add milk and bring mixture to a boil. Reduce heat to medium-low and simmer 8 minutes, until chicken is cooked through.

Meanwhile, to make the couscous, bring 1 1/4 cups of water to a boil in a medium saucepan. Add couscous, cover and remove from heat. Let stand 5 minutes, until liquid is absorbed.

To steam broccoli, place florets in a microwave-safe dish and add 1/2 inch of water. Cover with lid and microwave on high for 3 minutes. Let stand, covered, until ready to serve.

Using tongs, remove chicken breasts from skillet and transfer to individual plates. Add crumbled blue cheese and nutmeg to simmering milk in skillet and cook 1 minute, until blue cheese melts, stirring constantly. Remove from heat and stir in fresh parsley. Spoon bluecheese sauce over chicken breasts and serve with couscous and broccoli on the side.

Nutrition Score per serving (1 chicken breast half, 1/4 cup sauce, 1/2 cup couscous, 1/2 cup broccoli): 591 calories, 29% fat (19 g; 4.7 g saturated), 39% carbs (58 g), 32% protein (47 g), 12 g fiber, 309 mg calcium, 4 mg iron, 353 mg sodium.

RELATED ARTICLE: Calcium & Weight Loss

There are more health benefits to calcium than just strong bones. Some studies show that getting the mineral from milk, yogurt and other dairy products may help you manage your weight too. How? Calcium may aid in the breakdown of body fat: It seems that the more calcium a fat cell has, the more fat the cell will burn. Additional research needs to be conducted to find out whether calcium from other foods--such as leafy greens--has the same effect. So if you're trying to lose weight, don't drop dairy products; just make sure you choose the non- and lowfat varieties.

RELATED ARTICLE: Calcium on the Go

* Total cereal 1 1/3 cups: 333 mg calcium, 147 cal, 1 g fat; with 8 ounces nonfat milk: 613 mg calcium, 226 cal, 1 g fat; with 8 ounces 1% lowfat milk: 612 mg calcium, 242 cal, 3 g fat

* Viactiv Soft Calcium Chews 1 chew: 500 mg calcium, 20 cal, 0.5 g fat

* Lowfat fruit-flavored yogurt 1 cup: 330-400 mg calcium, 210-240 cal, 1-3 g fat

* Orange juice fortified with calcium 8 ounces: 330 mg calcium, 110 cal, 0 g fat

* 1% lowfat chocolate milk 8 ounces: 260 mg calcium, 143 cal, 2 g fat

* Cheddar cheese 1 ounce: 203 mg calcium, 111 cal, 9 g fat; lowfat Cheddar cheese 1 ounce: 203 mg calcium, 71 cal, 4 g fat

* Part-skim mozzarella string cheese 1 ounce: 183 mg calcium, 81 cal, 6 g fat

Robin Vitetta-Miller, M.S., is a food writer in Yardley, Pa., who appears regularly as a nutrition expert on CNN Headline News.

By Robin Vitetta-Miller, M.S.

Photography by Lisa Hubbard

COPYRIGHT 2004 Weider Publications
COPYRIGHT 2004 Gale Group

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