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Norrie disease

Norrie disease is an inherited eye disorder that leads to blindness in male infants at birth or soon after birth. It causes abnormal development of the retina, with masses of immature retinal cells accumulating at the back of the eye. As a result, the pupils appear white when light is shone on them, a sign called leukocoria. The irises or the entire eyeballs may shrink and deteriorate during the first months of life, and cataracts may eventually develop. more...

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About one third of individuals with Norrie disease develop progressive hearing loss, and more than half experience developmental delays in motor skills. Other problems may include mild to moderate mental retardation, often with psychosis, and abnormalities that can affect circulation, breathing, digestion, excretion, or reproduction.

Mutations in the NDP gene cause Norrie disease. The NDP gene produces a protein called norrin, which is believed to be crucial to normal development of the eye and other body systems. In particular, it seems to play a critical role in the specialization of retinal cells for their unique sensory capabilities. It is also involved in the establishment of a blood supply to tissues of the retina and the inner ear. This condition is inherited in an X-linked recessive pattern.

This article incorporates public domain text from The U.S. National Library of Medicine

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Behind Bars
From Better Nutrition, 8/1/00 by Lisa Turner

Instant energy that's nutritious and delicious

No longer relegated to the realm of body builders and distance runners, a hot new crop of energy bars promise instant, rounded nutrition for anyone from working moms to exam-weary students. One thing's for sure, today's energy bars bear little resemblance to the standard high-carbohydrate, low-fat gobs of grain and sweeteners of yesteryear. They may have higher-than-expected amounts of protein and, surprisingly, fat, and in some cases, they may contain fewer carbohydrates. They contain supplements like ginkgo, ginseng and antioxidants. They're marketed to us average folk who may not be in the business of winning marathons or body-building contests, but still want extra energy, protein and heart-healthy supplements. And they actually taste good -- the biggest difference of all.

"What we're seeing is a merger between candy and nutrition," says Lizanne Falsetto, senior vice president of Personal Health Development, Inc., manufacturers of Think Bar, in Ventura, California. "Energy bars weren't very edible 10 years ago. Over time, and through research and polls, we've learned that flavor is the #1 consideration for buying a nutrition bar. What we're finding are essentially nutraceutical candy bars."

The hard part is choosing one. So we've done some of the footwork for you. Here, Better Nutrition goes behind bars to look at the main attributes of 13 of the top-selling energy bars today.

Balance Bar

Loosely based on the Zone's 40-30-30 principle, the original Balance Bar promises complete and balanced nutrition, and is marketed primarily as a nutritious snack, rather than a preparation for pumping iron. "It has a lot of uses," says Charlotte Norrie, spokesperson for Balance Bar in Carpenteria, California. "It can be used as a snack, for pre- and post-workout, and it's good for people with diabetes and hypoglycemia. Rather than a high-carb or high-protein profile, it's a balanced makeup." The basic bar contains 40 percent carbs, 30 percent protein and 30 percent fat, in a dense chewy, candy bar-like format. It comes in seven flavors, including Almond Brownie, Chocolate Raspberry and other varieties. The Balance Outdoor Bar, designed to be edible (read: not frozen or melted) in temperature extremes, is sweetened with organic brown rice syrup and agave nectar. The Balance Gold bar is a triple-layer energy bar that is nearly identical to a Snickers bar.

* Bar basics: Balance Bar

* Calories: 200

* Calories from fat: 50

* Saturated fat: 35 grams

* Protein: 14 grams

* Carbohydrates: 22 grams

Clif Bar

Designed as a more "homemade" version of a classic energy bar, the Clif Bar is targeted primarily to the basic outdoor enthusiast or generally busy person. "Clif Bars contain premium, minimally processed ingredients, and they are free from wheat and dairy," says Molly Lori, RD, consumer service and marketing representative at Clif Bars. "They have been shown to keep blood sugar levels constant, without causing a sugar crash." The bars have a pretty standard nutrient profile, with 8 to 12 grams of protein from a blend of non-GMO (genetically modified) soy and a range of vitamins and minerals. A big selling point for this bar is its blend of soluble and insoluble fibers (apple fiber, oat fiber, milled flaxseed, soy fiber, chicory extract, lemon fiber and psyllium), which helps slow the absorption of sugar and create more sustained energy. Clif Bars are built like dense granola cookies, with chunks of dried fruit and nuts, and come in a variety of flavors including Chocolate Brownie and Ginger Snap. Clif Bar's Luna line is targeted specifically to women, and comes in creative flavors like Chai Tea, S'mores and Chocolate Pecan Pie.

* Bar basics: Clif Bar

* Saturated fat: 1 gram

* Calories: 240

* Protein: 12 grams

* Calories from fat: 45

* Carbohydrates: 41 grams

GeniSoy

Not just an energy bar, GeniSoy emphasizes its soy protein content, and is marketed less to athletes and dieters, and more as a general health supplement. The GeniSoy bar uses non-GMO soy protein processed to maintain the naturally occurring levels of the isoflavones genistein and daidzein, the compounds considered to be most responsible for the health benefits of soy. "Research has demonstrated isoflavones' antioxidant properties, and numerous studies have suggested that soy products have the potential to reduce the risk of heart disease, cancer, osteoporosis and menopause symptoms," says Ed Cabel era, vice president of marketing at GeniSoy Products Company in Fairfield, California. "The majority of these health benefit research studies used the same isolated soy protein with naturally occurring isoflavones that is found in GeniSoy products, not extracted isoflavones used as supplements."

The bars are also boosted with antioxidant vitamin E and selenium. The bar has a dense, taffy-like texture and comes in a variety of flavors, including Apple Spice Yogurt, Chocolate Mint and Creamy Caramel. The Nature Grains Bar, made with whole grains and dried fruit, has a similar nutrition profile but a baked, cookie-like texture, and comes in four flavors, including Oatmeal Raisin and Banana Nut.

* Bar basics: GeniSoy

* Calories from fat: 30

* Protein 14 grams

* Calories: 220

* Saturated fat 2.5 grams

* Carbohydrates: 33 grams

PowerBar

Billed as the "#1 nutrition bar," PowerBar has been the industry standard since 1983, especially for hard-core athletes. "They're designed for athletes, especially endurance athletes like runners and bikers, who are working out for 45 minutes or longer," says Alissa Berman, public relations manager at PowerBar in Berkeley, California. "They're easy to digest, which is essential before intense exercise." The bar is high in carbohydrates, moderate in protein and low in fat, and is designed to be eaten 30 minutes before exercise, with plenty of water. "It maybe not the kind of bar you say `Mmmm, this is an indulgent treat,'" Berman says, "but they can help serious athletes make or break a record."

The original PowerBar has a taffy-like consistency, comes in 10 different flavors, including Malt Nut, Mocha and Vanilla Crisp. It's sweetened with organic brown rice syrup and contains antioxidant vitamins. Other varieties include the PowerBar Harvest Bar, with a moist and crunchy texture designed as a breakfast bar or low-fat snack and the PowerBar Protein Bar, designed to boost protein in any diet. The yummiest: PowerBar Essentials, a chewy, chocolate candy bar-like line with boosted calcium and antioxidant vitamins.

* Bar basics: Power Bar

* Saturated fat: 0.5 gram

* Calories: 230

* Protein: 9 grams

* Calories from fat: 25

* Carbohydrates: 45 grams

Twinlab

Twinlab's Ironman Triathlon Bar (named after the grueling triathlon event, that consists of a 2.4 mile swim, 112 mile bikeride and 26.2 mile run) features more protein and fat, and less carbohydrates, than traditional nutrition bars. Twinlab says that these ingredients are provided in "scientifically designed proportions" to help athletes to burn their own stored body fat during exercise. Flavors include: Creamy Peanut, Chocolate Chunk, Chocolate Praline, Cookie Dough, Cookies & Creme, Granola, Kona Crunch and Yogurt Berry.

* Bar basics: Ironman Triathlon Bar

* Saturated fat: 1.5 grams

* Calories: 230

* Protein: 16 grams

* Calories from fat: 70

* Carbohydrates: 23 grams

Tiger's Milk

In a market where most nutrition bars sell for nearly two bucks, Tiger's Milk bar has differentiated itself as a high-nutrition, no-nonsense bar that costs less. "The original bar was targeted toward a younger market, as a healthy alternative snack, instead of a candy bar," says Chris Lockwood, a manager for Weider Nutrition International in Salt Lake City.

The original Tiger's Milk bar has a carob coating and a dense, chewy texture, and comes in three flavors: Peanut Butter, Peanut Butter and Honey and Peanut Butter Crunch. Tiger's Milk Protein Rich so closely resembles a Snickers bar that it won the American Taste Award of Excellence in 1999. Tiger Sport, designed for athletes, has a high-carb, moderate-protein profile with a taffy-like texture. The company is introducing a new line with a stepped-up vitamin and mineral content, less fat and fresh flavors like raspberry and lemon.

* Bar basics: Tiger's Milk

* Calories from fat: 60

* Protein: 6 grams

* Calories: 150

* Saturated fat: 1 gram

* Carbohydrates: 18 grams

Low Glycemic Technologies

Low Glycemic Technologies says that its Protein Revolution Bars are based on a "revolutionary new concept" -- they're good for you and they taste good! While the bars can certainly be a part of a low-carb diet plan, the company says that the bars can also be used as a lifelong source of healthy, low-carbohydrate nutrition. There are five flavors in all: Creamy Chocolate Peanut Butter Cup, Double Chocolate Fudge Almond, Apple Cinnamon Spice Cake, Chocolate Hazelnut Toffee Crunch and Classic Peanut Butter & Jelly.

* Bar basics: Protein Revolution Low Carb Bar

* Calories from fat: 70

* Protein: 22 grams

* Calories: 230

* Saturated fat: 1 gram

* Carbohydrates 2.5 grams

Atkins

The Atkins Advantage Bar is designed for "on-the-go" Atkins followers, to be used as a meal-replacement or a snack. In addition to being low-carb, it features 40 to 70 percent of the RDA of vitamin C, calcium, zinc and folic acid, as well as omega-3 and omega-6 fatty acids. Flavors include: Chocolate Coconut, Chocolate Peanut Butter, Chocolate Raspberry, Almond Brownie, Chocolate Macadamia Nut, Praline Crunch and Chunky Peanut Butter.

* Bar basics: Advantage Bar

* Calories: 218

* Calories from fat: 117

* Saturated fat: 6 grams

* Protein: 19 grams

* Carbohydrates: 2.6 grams

Met-Rx

Met-Rx says its food bars are designed to "enhance endurance and recovery" and emphasizes that its food bars are not energy bars or snacks, but "a convenient way to ensure a steady supply of protein, vitamins and minerals throughout the day." The bars provide 100 percent the RDI in calcium. Flavors include Chocolate Chip, Peanut Butter, Fudge Brownie and Extreme Vanilla. Met-Rx also has a Natural Krunch line, featuring fruit and protein rice bars; flavors include Apple Cranberry, Cinnamon Oatmeal Raisin and White Chocolate Raspberry. It also has a line called Source One.

* Bar basics: Met-Rx (food bars)

* Calories: 340

* Calories from fat: 35

* Saturated fat: 0.5 gram

* Protein: 27 grams

* Carbohydrates: 50 grams

BioChem Sports and Fitness Systems

BioChem's Ultimate Low-Carb Bar is a high-protein, low-carb snack for athletes and non-athletes alike, featuring 20 grams of protein vs. 2 grams of carbohydrates. It comes in four flavors: Cool Cappuccino, Creamy Peanut Butter, Chocolate Brownie Nut and Honey Almond.

* Bar basics: Ultimate Low-Carb Bar

* Calories: 230

* Calories from fat: 50

* Saturated fat: 1 gram

* Protein: 20 grams

* Carbohydrates: 2 grams

Think!

Far more than a typical bar, the Think! bar is positioned as a healthy version of a candy bar designed to boost mind power, with a blend of such brain-healthy supplements as ginkgo, ginseng, choline and phosphatidylserine. "They help with mental clarity," says Falsetto. "Our slogan is `Good for the mind, body and spirit.' Ultimately, mental performance influences how you end up functioning as a whole." The bars are low in fat, with a moderate amount of protein and carbs and make a handy meal replacement, or can be eaten before sustained and intense mental activity.

The Think! Divine bar, the company's newest offering, is a triple-layer, caramel nougat and chocolate bar, with a dense, creamy consistency reminiscent of a Snickers bar, and soy nuts instead of peanuts to cut fat and boost nutrition. It comes in three flavors: Peanut Butter Chocolate, Chocolate Truffle and Caramel Creme, and, like the original Think! bar, contains a blend of nutrients for mental function.

* Bar basics: Think! Divine

* Saturated fat: 5 grams

* Calories: 200

* Protein: 6 grams

* Calories from fat: 60

* Carbohydrates: 32 grams

Zone Perfect

Based on Dr. Barry Sears' celebrated Zone diet, the ZonePerfect bar uses the now-ubiquitous 40-30-30 profile (carbs, protein, fat, in that order) and was designed for mass, universal appeal to Zone enthusiasts. "The diet has become very well-known, mostly because it's so effective," says Paul Pruett, vice president of ZonePerfect Nutrition Company in Beverly, Massachusetts. "If you walk down the street and ask 10 people `Have you heard of the Zone?' I'll bet at least five would say yes." The bars are designed to do everything the diet does: boost energy, maintain optimum weight and provide for overall enhanced health. They contain omega-3 fatty acids, an essential component of the Zone diet, and come in two varieties: a chocolate-coated, candy-bar style, and a more granola-bar format, in seven flavors.

* Bar basics: ZonePerfect

* Calories: 200

* Calories from fat: 60

* Saturated fat: 3.5 grams

* Protein: 14 grams

* Carbohydrates: 24 grams

Life Services Keto

Life Services says its Low Carb Keto-Bar is the perfect snack for anyone who is reducing carbohydrates and making the switch from fat storage to fat burning because it will not cause high blood sugar and excess insulin release. The bar, which comes in Chocolate Peanut Butter Cup, can be eaten anytime, but is specifically designed to fight between-meal-hunger, lethargy and sweet cravings.

* Bar basics: Low Carb Keto-Bar

* Calories: 248

* Calories from fat: 108

* Saturated fat: 3.5 grams

* Protein: 19 grams

* Carbohydrates 4 grams

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