Samnaun dwarfs

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Seven of the eight "Samnaun dwarfs"
Left one of the Samnaun dwarfs
Two of the short women

The phenomenon of the frequent births of small children in the remote Graubünden high valley Samnaun between 1873 and 1892 is called Samnaun dwarfs .

history

In the hamlets of Compatsch, Plan and Ravaisch in Samnaun, eight small children were born within 19 years, five girls and three boys. Four families were affected.

The height of the adult short stature was 80 cm for the smallest and 100 cm for the largest. All siblings of the short stature, however, were of normal size.

The small people were not excluded, but were considered to be well integrated into the villages. They learned professions such as farmer , tailor and singer. With the exception of one affected person, all of the minors reached a normal or even old age. The last “dwarf”, Rudolf Prinz, died in 1959 at the age of 77.

root cause

Both inbreeding and malnutrition have long been suspected as causes of the malformations . In the meantime, however, a hereditary recessive genetic defect has been confirmed as the cause, which only became visible in the phenotype due to the isolated location of the Samnaun.

DNA examination

At the beginning of this century, Primus-Eugen Mullis, Professor of Pediatric Endocrinology at the University of Bern , was able to use blood samples from all potential descendants to prove that Laron's syndrome is not the cause of the short stature in the Samnaun dwarfs. In this disease, growth hormones are formed to a normal or, under certain circumstances, increased levels, but they cannot work because the corresponding receptor is defective.

With the Samnaun dwarfs, however, a defect in the growth hormone gene is the cause, which leads to a reduced production of growth hormones and is therefore responsible for the short stature.

literature

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