Размещаю статью, которую мне прислал Ханс Койпер про диагноз фон Виллебранда касательно дратхааров. Очень жаль, но времени переводить нет. Так что с "переводчиком" дорогу осилит идущий
Von Willebrand’s Disease
in the Deutsch-Drahthaar
Contributions to Clarification in the Matter of von Willebrand’s Disease ( vWD ) in the Deutsch-Drahthaar
Source: DD-Blätter February 2003
Pages 42 - 43
(This manuscript was edited and brought up to date by Mr. J. W. Keuper ( examined by the state and sworn as a translator/interpreter of the High-German language ), Member of Honor of VVDD ( Vereniging Vrienden Duits Draadhaar ( VVDD, DD-Club of the Netherlands, founding member of the Deutsch-Drahthaar World Association ), Member of VDD e. V. since 1990, former Chairman of the Progeny Working Group of VVDD, former Business Manager as well as former Representative of VVDD in Deutsch-Drahthaar World Association ( DD-WV ).
Hans Keuper ( 52 ) is Founder and Administrator of the DNA-vWD-Databank. He is hunter as well as DD trainer and handler since 1980. He trained and handled about 26 DDs and trained many, many more. February 2010
Blood Disorders in the Deutsch-Drahthaar
Coagulation of the blood is the protective mechanism of the body that keeps the loss of blood in check after an injury. It arises from a complex interaction by factors in the injured blood vessels, the thrombozytes and the protein (albumen) in the fluid of the blood. The thrombozytes are disk-like particles of blood that after the occurrence of damage immediately clot together and by means of the von Willebrand factor adhere to the walls of the injured blood vessel. Multiple factors are then necessary in order to stabilize the primary coagulation of blood with a mesh of fibrin and thereby to form the secondary coagulation of blood.
The required factor is lacking in the von Willebrand’s Disease (von Willebrand deficiency / vWD) or is not sufficiently present. This disease appears in the various breeds in different subtypes. Type II of this disease is found in the Deutsch-Drahthaar. Only very little of the von Willebrand factor is thereby present in the blood. In addition, it is formed abnormally, resulting in a loss of effectiveness. The Type II disease is found only in the Deutsch-Drahthaar and the Deutsch-Kurzhaar. This form then exhibits a particularly difficult course. Excessive bleeding occurs already during the docking of the tail, the tattooing and the changing of the teeth. The disease is often not quite so evident with very young puppies because during the gestation the von Willebrand factor is transferred from the female to the puppies and remains effective during the first days of their lives.
Prolonged bleeding may arise later from minor injuries, spontaneously from the nose or the mouth or from under the skin, respectively. Also, blood may be found in the urine or the stool.
Although the Type II is counted amongst the difficult forms of appearance, it is not lethal in most cases. Afflicted puppies as a rule will grow to be adults. They may even survive serious operations with the aid of appropriate transfusions of blood. Heavy bleeding may be treated only with transfusions of blood or by application of plasma. Local measures, such as a dressing, stitching or blood clotting medication are indicated for minor bleeding. Other medications or substances that have an effect on coagulation, such as Aspirin, Sulfonamides, fish oil and Heparin are to be avoided to the utmost.
The von Willebrand Disease (vWD) is autosomal ( in pairs of chromosomes ) passed on recessively with the Deutsch-Drahthaar. The formation of the von Willebrand factor is controlled by two genes for both males and females, one deriving from the sire and the other from the dam. Only dogs that receive two sick genes will tend to bleeding. Dogs with only one bad gene are carriers; for them the disease does not appear. They will, however, pass on the carrying gene for von Willebrand’s Disease to about half of their offspring.
• von Willebrand Disease-DNA-free, no bleeding> genetically healthy / free of characteristics
• von Willebrand Disease-DNA-carrier, no bleeding> passing on the bad gene to half of the progeny
• von Willebrand /Disease-DNA- ill> more or less strong trend to bleeding
Possibilities of passing on:
DNA-vWD-free x DNA-vWD-free all offspring are DNA-vWD-free
DNA-vWD-free x DNA-vWD-carrier 1/2 of the offspring are DNA-vWD-free
1/2 of the offspring are DNA-vWD-carriers
DNA-vWD-carrier x DNA-vWD-carrier 1/4 of the offspring are DNA-vWD-free
1/2 of the offspring are DNA-vWD-carriers
1/4 of the offspring are DNA-vWD-affected
The Coagulation-Factor Test ( Elisa )
The amount of the von Willebrand factor in the blood may be determined in order to learn about the state of the genetic situation. For a long time only one test was available for this, which measures the quantity via an antigen/antibody reaction, the so-called Blood-Coagulation Test (Elisa). The average total amount prevailing in a healthy animal was set at 100%. The normal range extended from 70 to 180 %. A carrier had less than 50 %. Between 50 and 69 % was an unclear range, in which healthy as well as carriers could be found. Animals for which the factor amounted to less than 2 % were considered affected. This test was imprecise. In addition it was found that it was unreliable; investigations in Germany with a number of DD’s – they were tested by the coagulation test and by the DNA test - have confirmed this.
The DNA-vWD-Test
In 1997 geneticists of the Animal Medicine Faculty of the University of Utrecht ( the Netherlands ), specializing in disturbances of coagulation of the blood, developed a DNA test for von Willebrand Disease via two DD’s afflicted with von Willebrand Disease, both of which came from combinations of different, but related and after-the-fact DNA-tested von Willebrand carriers. There are no longer any “gray zones”; i.e., the test differentiates precisely into DNA-vWD genetically healthy, DNA-vWD-carrier and DNA-vWD-affected.
Deutsch-Drahthaar out of a genetically healthy combination “are genetically clear and not carriers of the mutation that causes vWD ( Type II ). They are genetically healthy with respect to vWD II and will pass on to their offspring only the normal gene.” ( Quote from a DNA-vWD certificate of a DNA-vWD healthy dog. )
This DNA-vWD-Test is available in the Netherlands since mid 1998 and is successfully employed by the Dutch DD-Club VVDD in an effort to prevent the spread of vWD – the Dutch DD-breeding stock is DNA-vWD genetically healthy since.
The firm Laboklin in Bad Kissingen, Germany, and the foundation Tierärztliche Hochschule Hannover [Hannover Veterinary Medicine University], Germany, have a license for this test method on Deutsch-Drahthaar since the beginning of 2005. Since approximately the beginning of 2005 both test institutes test Deutsch-Drahthaar for vWD type II with the Leegwater Method, Utrecht, NL. Almost 1800 DD’s of for example German, Finnish, Norwegian, Danish, Swedish, English or Italian origin have since been examined by these institutes. In 2006 researchers of VetGen, Michigan / USA developed a similar, reliable DNA-method to test DD’s on vWD Typ II.
For carrying out the DNA-vWD test 4 ml of blood are taken from your DD by your own veterinarian; it is then well mixed inside a small EDTA vial. The plastic container is marked with the official name of the dog, the breed and the date of extraction. Shipping ought to be inside a reinforced envelope. Bloodcards ( dried blood ) and well as cheek swabs also do!
The four named institutes, the only ones able to test reliably via DNA for vWD, all have their own form for examination that may be completed by your veterinarian and yourself. The time required for analysis runs at about a week. Results can be sent by e-mail!
The forms for examination for the firm Laboklin may be downloaded to your PC from their website (
www.laboklin.de, Tiergenetik>Erbkrankheiten>von Willebrand Erkrankung or
www.laboklin.de, Tiergenetik>Kontakt>Untersuchungsanforderung Erbkrankheiten).
The addresses are: Laboklin, Labor für klinische Diagnostik, GmbH
Prinzregentenstrasse 3
97668 Bad Kissingen, Germany
Tel: 0049 971 72020
Contact person: Mrs. Dr. Ines Langbein-Detsch
E-mail:
labogen@laboklin.de
www.laboklin.de
VetGen
Veterinary Genetic Services
3728 Plaza Drive, Suite 1
Ann Arbor, Michigan, 48108 USA
Contact person: Mrs. Ann Arnold
E-Mail:
vetgen@vetgen.com
www.vetgen.com
Laboklin, Laboratory for clinical diagnostics, UK office
61 Mouldsworth Avenue
Manchester / M20 1GG
United Kingdom
Contact: Mr. Mansour Makki
E-Mail:
info@laboklin.co.uk www.laboklin.co.uk
Animal Medicine Faculty of the University of Utrecht
Take care! Since January 2010 no DNA-vWD-tests are
done anymore by this Faculty!
Institut für Tierzucht und Vererbungsforschung
Hannover Veterinary Medicine University
Bünteweg 17p
30559 Hannover, Germany
The start of DNA-vWD-Databank
As Chairman of the Progeny Working Group of the Dutch DD Club ( VVDD ), Hans Keuper started collecting many information on von Willebrand’s Disease and DD. In the midst of 2009 DNA-vWD-Databank had to cut the ties with the unreliable EB of VVDD. Since then DNA-vWD-Databank is privately run by Hans Keuper in the way he started it 1997.
The goals of DNA-vWD-Databank – acting without any benefit to itself, without remuneration and independently – are:
a. collecting as much factual data as possible
b. open, honest, independent fact-and research-based work of public education and activity concerning von Willebrand’s Disease in the Deutsch-Drahthaar in the interest of the health of this breed worldwide.
Since 1997 Hans Keuper is supported by the University of Utrecht, NL. In addition, by the firm Laboklin, Bavaria and Mrs. Dr. Marjory Brooks of Animal Health Laboratory of Cornell University, Ithaca (State of New York, USA), who successfully guides VDD-Group North America in matters of vWD and since June 2006 maintains contact with the University of Utrecht.
The mentioned laboratories put a great deal of informative material, current scientific reports and/or vWD-DNA results at the disposal of DNA-vWD-Databank, within the bounds of relevant laws protecting data. The Institut für Tierzucht und Vererbungsforschung of Veterinary Universtity of Hannover for some time made available vWD-DNA data.
VetGen Michigan is very cooperative since 2007!
DNA-vWD-Databank is very pleased with the active, honest and knowledge-conveying support by many serious DD breeders, owners of DD stud dogs and DD-handlers in – amongst others – Germany, Denmark, Norway, Finland, Sweden, the Netherlands, Italy, England, Sweden, Spain, North America, Canada and Argentina as well as by the great interest of many DD-people in the subject von Willebrand’s Disease in the DD. Many findings of DNA-vWD were already made available to us.
The DNA-vWD-Databank
The collaboration with and support by the above-mentioned laboratories, DD-clubs and many serious DD-people worldwide has resulted in a significant private , but public, international collection of DNA-vWD data, the DNA-VWD DATABANK D-bases.
These D-bases contain amongst others the official DNA-vWD results of many DD’s with ZBDD-, DKK-, SKK-, NKK-, LOI-, LOSH- and NHSB-pedigrees. Amongst them, fortunately, are many results of DNA-vWD healthy DD’s, but also a percentage of DNA-vWD carriers and some DNA-vWD affected DD’s. Amongst the DNA-vWD carriers are a few stud dogs and bitches of German origin, being used for breeding. The D-bases also contain huge lists of litters out of DNA-vWD-clear parents, DNA-vWD-carrying or vWD-suspicious parents.
The DNA-vWD Databank D-bases as well as the Pedigree Analysis show very clearly that the carrying gene for vWD still is passed on.
This Databank was founded and is administered by the former president of the Progeny Working Group of Dutch VVDD, Mr. Hans Keuper, involved in many DD issues since 1980.
His address: DNA-vWD-Databank
Founder and Administrator: Hans Keuper (member of VDD and JGHV)
Member of Honor of VVDD – DD-club of the Netherlands
Ettenlandseweg 26a
8316 RP Marknesse
The Netherlands
Tel: 0031 527 243556
E-mail:
hanskeuper00@hetnet.nl
DNA-vWD-Databank is interested in a Deutsch-Drahthaar that is healthy, capable of performing and optimally meeting the standard of the breed as much as you are and continues its investigations.
In case you have or will have your DD tested by DNA for von Willebrand’s Disease we recommend ourselves for a copy of the fully completed result(s). All results are stored in the DNA-vWD Databank ánd published.
This Databank will be placed at your disposal - if desired - with great pleasure!
( Only clearly, fully completed copies including the name of the owner of the examined DD’s can be processed and stored. Of course, we will also need your e-mail address in order to be able to reach you - in case you want that. The DNA-vWD Databank ( in Excel-Spreadsheet formats ) can be forwarded only by e-mail ).
Marknesse, the Netherlands, February 2010
Although this manuscript has been reviewed and brought up to date with utmost care, no liability is assumed for the contents.
Source: DD-Blätter, April 1997
Pages 92-93
Coagulation of the Blood and Blood Coagulation Disorder
From the Presentation by Dr. Gutbrod during the Meeting of Breed Wardens of the
VDD e.V. on 1 February 1997 in Grünberg, Hessen, Germany
Some Basics
Many red blood particles circulate in the blood. Their major task is to supply the body with oxygen. They carry it from the lung to the various organs. In addition, we also find the white particles in the blood. They are responsible for the defense of the body. Beyond these there are small cells present. They are called Thrombozytes. They are an important link in the coagulation of the blood.
This coagulation of the blood is a very complicated process. The blood must not coagulate in the veins; therefore all vessels are covered with a neutral layer of cells. One finds collagen in the walls of the vessels. Collagen is also in many other tissues, for example in muscles and tendons. It is a stable and flexible tissue that gives the veins their elasticity and the necessary strength. Fluid in the blood comes into contact with the collagen when a vessel is injured. This is the moment that initiates the coagulation of the blood.
The Fibrin, formed from its preliminary stage – Fibrinogen – by the involvement of numerous factors, causes the small Thrombozytes to clot together. The resulting coagulate seals the vessel again. Scientists are engaged in finding how the process evolves precisely. Even today they come upon new factors that round off the system.
Blood coagulation disorders interest us as breeders, especially if they are passed on genetically.
Blood disease is well known for mankind. Blood diseases occur in the dog as well, where the most common ones have been explored only during recent years. The basis for these investigations was an animal, for which one was able to demonstrate single factors for coagulation. These tests have been available in America for a number of years, while they could be obtained in Germany only lately. Hence all our knowledge originates in America.
I can only report a little of these investigations:
The most common blood disease for mankind and animals is the so-called “von Willebrand’s Disease”. It is caused by an insufficient formation of the “von Willebrand’s” coagulation factor. Because the tests used in human medical processes did not work for the dog, investigation of this disease was possible only during recent years. Furthermore, all the parameters commonly used in the laboratory, that may be determined from the blood, are normal. Only measurement of the time of bleeding in the mucous skin of the cheek shows a clear prolongation. An exact diagnosis would only be possible, however, after the development of special tests by which one might be able to check the "von Willebrand Factor" in the blood.
The disease was divided into three groups for both humans and dogs:
1. There is clearly less “von Willebrand Factor” to be found in the blood than normal.
2. A false, ineffective “von Willebrand Factor” is formed.
3. There is no “von Willebrand Factor” present whatsoever.
1. This is the most commonly occurring form and appears especially in the Dobermann and Airedale.
2. This was described for the Deutsch-Kurzhaar. Both types are passed on in the dominant manner.
Group 3 is a rare, but difficult form of occurrence. It is known for the Scottish Terrier and the Retriever. In America it was also found in the Deutsch-Drahthaar. Type 3 is passed on recessively, which means that both sire and dam must be carriers.
A second inheritable blood coagulation disorder was also found in the DD, namely the Hemophilia B. This is a sex-bound disease - only the males fall ill, the females are healthy carriers of the gene. These insights arise from a study that was initiated by our North American Group after in 1989 dogs with heavy, spontaneous bleedings had come to attention.
From May 1989 to January 1993 335 dogs were investigated. Thereby 8 dogs (males and females) were found with the “von Willebrand” disease. Most of the affected animals came from three litters that were very closely related. Investigations on these three litters led to the conclusion that this disease is passed on by a recessive gene.
As is well known puppies receive half of their genes from the sire and half from the dam. A disease-causing gene must come from both the sire and the dam in case of a recessively passed on disease. If a dog has only one bad gene the dog itself will be healthy, but passes on a disease-causing gene to about half of the offspring.
Hence it may happen that two healthy appearing parents may have an affected offspring.
That the healthy appearing animals may be identified by means of an examination of the blood is of favorable impact on the effect in breeding.
Therefore 467 dogs were examined in another study. 81 % were completely free, 12 % were healthy appearing carriers, 1 % was affected, 6 % could not be classified precisely into the category “free” or “carrier”.
The probability of obtaining an affected puppy was calculated at 1:120 on the basis of these numbers. This probability, however, rises if one does line breeding.
How does this disease evidence itself in the dog?
Which signs cause us to suspect that a dog is a "bleeder”?
Clinical Symptoms:
-prolonged after-bleeding from tattooing
-heavy bleeding when docking the tail
-excessive bleeding during change of teeth
-bleeding of the nose
-under skin bleeding
-bleeding in a joint
-continued bleeding after operations
The symptoms may show in varying intensity.
The Americans have further examined the lines in which bleeders occurred and have kept them under further observation. They were fairly often able to trace the defects to dogs that originated from German breeding and that had been whelped in Germany decades ago.
Now the question arises, whether the Breed Wardens observe similar cases. Dr. Gutbrod is able to report on two cases within the veterinarian sphere.
A Deutsch-Drahthaar - about a year old - was presented because a thigh had suddenly swollen significantly. The skin was tautly under tension. The dog did not have a fever; however, the mucous skins were pale. Examination of the blood did not point towards poisoning (rat poison, for example, hinders the coagulation of the blood so that bleeding in all tissues may hereby occur). It was possible to massage the blood out of the muscles of this dog by means of dressings; the leg became thinner. But just half a year later a bleeding in the same leg was so heavy that the toes had died. The dog had to be put to sleep.
Another dog had broken its thigh. He had been taken into the clinic for an osteosynthesis operation. Already during this procedure a heavy hemorrhage at the break was noted, but this was attributed to the accident. When after six months the break had healed, the implants had to be removed. With that, massive after-bleeding occurred so that a transfusion of blood became necessary.
Another case is to be described:
Fall 1992: heavy bleeding during change of teeth that could only be ended after three days.
January 1993: general lethargy. The veterinarian determines bleeding inside the abdomen and thinks of tearing of the liver or the spleen. The dog is restored by means of transfusions of blood in an animal clinic.
1993 to 1995 always long lasting after-bleeding after minor injuries.
December 1996: nose bleeding lasting days impossible to stop; short ceasing of bleeding after transfusions. When there is repeated torrent-like bleeding from the nose the dog is put to sleep.
These examples demonstrate that this illness is not impossible for us. Unfortunately the symptom is often - also by veterinarians - mistaken for poisoning.
Breed wardens ought to report observations of similar kind.
Although this manuscript was reworked with utmost care, no liability is assumed for the content.
Also of great interest:
Mit hartem Haar ins neue Jahr [With Hardy Hair into the New Year]
Report on the Stud Dog Show and Meeting of Breed Wardens in the area of Baden.
DD-Blätter March 2005, pages 59 – 61,
by the editor of the DD-Blätter, Mr. Henning Bergmann
Concluding Position to the Theme “von Willebrand”
Concluding statement of the EB of VDD e. V. on the subject von Willebrand Disease
DD-Blätter April 2005, page 110,
by the Business Managing Executive of VDD e. V.
Minutes Breedwarden Meeting February 3rd 2007 in Osterburg
DD-Blätter April 2007, Page 99,
by Mrs. Andrea Heutger, VDD-Group Sachsen-Anhalt
Von Willebrand Typ 2: Testresults within Deutsch-Drahthaar
DD-Blätter April 2008, page 103
by Mr. Prof. Dr. Ottmar Distl, TiHo Hannover
Minutes Breedwarden Meeting on February 9th 2008 in Ankum
DD-Blätter April 2008, pages 118 – 119, TOP 10
by Mr. Hans-Jürgen Kenkel, VDD-Group Artland-Südoldenburg
Thoughts on modern animal breeding
DD-Blätter July/August 2008, pages 203 – 206
by Mrs. Dr. Helga Eichelberg ( amongst others VDH, Germany )
From the Breeding Commission – Situation report of Head of Breedwardens
DD-Blätter July/August 2009, page 199
by VDD Head of Breedwardens Mr. Sigurd Croneiß
Acting concerning heriditary diseases
DD-Blätter July/August 2009, page 199
by Mr. Dr. Dr. Peter Schunk, HD-central VDD e.V.
Changes in VDD breeding law
DD-Blätter January2010, page 5/6
by VDD Head of Breedwardens Mr. Sigurd Croneiß
