Samnaun dwarfs
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
- Fadrina Hofmann: On the (DNA) traces of the Samnaun “Zweargla” , in: Die Südostschweiz , June 15, 2012, p. 7.
- Seraina Jenal: The Samnauner Zwerge , Swiss Youth Research Winner 2002.
- Amélie Besson, Souzan Salemi, Sabina Gallati, Arthur Jenal, Rudolf Horn, Pia S. Mullis, Primus E. Mullis: Reduced Longevity in Untreated Patients with Isolated Growth Hormone Deficiency. In: The Journal of Clinical Endocrinology & Metabolism . 88, 2003, pp. 3664-3667, doi : 10.1210 / jc.2002-021938 .
Web links
- The Samnaun dwarfs as part of Samnaun history. Website of the Samnaun school
- The riddle about the Samnaun dwarfs is solved. Neue Zürcher Zeitung, October 10, 2003.