Word: amish
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...geneticists the fascinating fact about the Old Order Amish, one of the sects of the Pennsylvania Dutch country's "Plain People," is that they all are descended from about 200 immigrants of 200 years ago. A few Amish leave the ancestral acres and simple (no motors, no worldly entertainments) way of life, but virtually no new blood has been introduced to create genetic confusion. For such a group, to survive is to inbreed, and the Amish have more than survived; they now number 44,000. In 1963, to take advantage of this unique opportunity into the land...
...common name and is so uncommon elsewhere in the world that only about 50 cases had been reported until McKusick's Hopkins team moved into Pennsylvania. There they found proof of at least 49 cases since 1860, with 24 still living. Most exciting, genetically at least: the Amish keep such exact genealogical records that McKusick was able to trace all 60 parents to whom the 49 were born. And all were descended from a single immigrant and his wife...
Snipped Fingers. The first known case, said Dr. McKusick at Bar Harbor, was born in 1860. Though the Amish average a couple of inches shorter than the general U.S. population, there is no mistaking the deformity. Ellis-van "Creveld dwarfs range in height from only 40 to 60 inches. They have six fingers on each hand, the extra one being on the outside of the hand beyond the little finger. Sometimes (but not consistently) there is a sixth toe on one foot or both. Although it is not conspicuous at birth, many dwarf babies have an abnormal heart with only...
Most parents have the children's extra fingers amputated when they are a few months old. The children show no mental retardation or IQ loss. And they probably fare better in the closed Amish community than in the less tolerant world .outside...
Fine Hair. Equally bizarre, and also transmitted through a recessive gene, is the new form of dwarfism found by Dr. McKusick among the Amish in more than a dozen communities. It is a new kind of genetic defect. Doctors who earlier noticed cases of this kind of dwarfism among the Amish mistook it for achondroplasia, a form made familiar by Velásquez's paintings of dwarfs as court jesters, with short arms and legs, a large head and a "scooped-out" nose. But Dr. McKusick's team found significant differences. These Amish dwarfs do not have...