Hemophilia- is the name of several genetic diseases inherited that disable the body to control bleeding
Hemophilia- is the name of several genetic diseases inherited that disable the body to control bleeding , an inability known technically as bleeding diathesis .
Deficiencies and a genetic disorder autoimmune rarely can cause decreased activity of coagulation factors in blood plasma , so that compromise blood clotting , therefore, when a blood vessel is damaged, a blood clot does not form and the vessel continues to bleed for a excessive period of Bankruptcy time. Bleeding can be external , if the skin is damaged by a cut or abrasion, or can be built in muscles , joints or organs.
It is the lack of factors of clotting – the Hemophilia A lack of clotting factor VIII to hemophilia B lack of clotting factor IX and Hemophilia C is lack of coagulation factor XI . Haemophilia A is the most common, occurring in 90% of cases.
Index [ hide ]
4 Servings home
7 In Brazil
8 Entities support
9 See also
11 External links
There is record of hemophilia in Jewish text, the Talmud (about 50 – 130 AD ). According to the Talmud a child should not be circumcised if two brothers had already died in this procedure. In the twelfth century , the physician Arab Abulcasis described a case of a family whose men had died of bleeding after minor injuries. In 1803 , the American physician John Conrad stated that it was ” a tendency to bleeding in some families. ” He came to the conclusion that the disease was hereditary and assailed more men than women.
The term Hemophilia first appeared in 1828 Hopff by the University of Zurich . In 1937 , Patek and Taylor, two doctors from Harvard discovered globulin anti-hemophiliac. Pavlosky, a doctor from Buenos Aires , separated Hemophilia A and Hemophilia B laboratory. This test was done by transferring the blood of one hemophiliac to another hemophiliac. That corrected the bleeding, proving there was more than one type of hemophilia.
Haemophilia is often associated with the history of monarchy in Europe . The Queen Victoria passed the disease to her son Leopold , and through several of his daughters, to various European royal families, including the royal families of Spain , Germany , and Russia . Alexei Romanov , son of Czar Nicholas II of Russia , was a the descendants of Queen Victoria who inherited the disease.
During the decades of 1970 and 1980 the lack of other treatment modalities and the lack of technology to diagnose some viruses like the AIDS resulted in contamination of large-scale hemophiliac population that depended on constant transfusions of blood . But this is a scenario that is not entirely in the past, the majority, around 75% of the world’s hemophiliacs do not have the basic medicines to treat this disease , which is still, these are subject to blood transfusions that can save their life but also can take it.
The Queen Victoria passed hemophilia to many of their descendants.
Alexei Nikolaevich Romanov ( 1904 – one thousand nine hundred and eighteen ) son of Nicholas II the last Emperor of Russia was a hemophiliac.
Dosages of the respective coagulation factors.
In the case of factor VIII deficiency should be sought to differentiate the disease of von Willebrand disease . In von Willebrand’s disease can also occur from a decrease of factor VIII.
There is no cure for hemophilia because there are several studies that seek to improve treatment. The disease is controlled with regular injections of clotting factor deficient. Some hemophiliacs develop antibodies (called inhibitors) against the factors which are given by the treatment.
Severe: The patient should receive producer of plasma to prevent or control bleeding episodes throughout your life. The level of factors have to be increased to + / – 30%
Preparations pro therapeutic factor VIII: cryoprecipitate, concentrated lyophilized commercial factor VIII. The factor concentrate IX exists in the form of protein isolated (Concentrate Factor IX lyophilisate) or in the form of “prothrombin complex concentrated” lyophilisate which is a mixture containing the factors (II, V, VII and IX) that presents itself also under form of lyophilisate.
Lightweight: only replacement therapy after a trauma or to prevent postoperative bleeding.
More recently came the commercial concentrates of Factor VIII and IX arising from genetic engineering , these products are not obtained from the purification of human plasma, but the manipulation of organisms genetically modified to carry the gene that expresses these proteins.
The dose domiciliary is to provide, for hemophilia patients, one or more doses for use in case bleeding. These doses are held by the patient and are applied by itself (or a family member previously trained), after undergoing an evaluation for the program dose at home.
This program is adopted in many countries, including Brazil .
It makes all the difference in hemophilia, thereby preventing the bleeding complications due to the strengthening of muscles , as well as speeding recovery and eliminating or drastically reducing the possibility of sequels.
Protect from injury ;
Immobilize the joints in cases of joint bleeding;
Observe and record bleeding episodes;
Adopt special care in realization of trichotomies , enemas , application of heat;
Assist in oral hygiene , paying attention not to hurt the gums and oral mucosa ;
Provide identification card hemophiliac, which should include: blood group , Rh factor , person to be notified in case of emergency, name of doctor and address of the hospital in which it treats.
In Brazil , the cartoonist Henfil and his brother, the social scientist Betinho , anonymous among others, were victims of this type of occurrence. The day April 17 is considered the day of the Hemophiliac . 
AHRJ – Brazil (Association of Hemophiliacs of Rio de Janeiro)
IHTC – Brazil (International Training Centre for Hemophilia)
AHCFM – Association of Hemophiliacs of Governador Valadares and Region
Concentrate Factor VIII .
Classification and external resources
ICD-10 D 66 – D 68
MedlinePlus Health information in MedlinePlus Encyclopedia
The Hemophilia is a recessive genetic disease related to the X chromosome that consists of: the haemophilia A , when there is a deficiency of factor VIII clotting, hemophilia B , when there is a deficit of coagulation factor IX, and C, which is the factor XI deficiency.
Content [ hide ]
1 History of discovery
1.2 Contemporary Age
2.1 Laboratory Tests
7 Guidelines for action in hemophiliacs
8 When to go to hospital
10 Genetic Counseling
12 External links
History of discovery
Earlier studies dating from the second century AD, when Jewish rabbis realized that some boys, when they practiced circumcision bled a lot. The rabbis found that these problems only occurred in certain families, so they made new regulations to help these children bled. Rabbi Judah said that a child who had older siblings with bleeding problems was not to be circumcised and Rabbi Simon Ben Gamaliel prevented a child was circumcised because the children of the three sisters of the mother had bled to death.
Among the written references should be highlighted after the description of the disease was in the eleventh century an Arab physician of Córdoba , Spain, called Albucasín.
In the twelfth century , another rabbi named Maimonides found that if children had hemophilia, were the mothers who transmitted it. He then made a new law: if a mother had children with this problem of bleeding and she remarried, none of its new male offspring should be circumcised.
The first reference occurs in Central Europe in Italy , in 1525 , by Alexander Benedict.
In 1800 , an American doctor named John C. Otto made his first study of families with hemophilia, and in the year 1803 discovered the gene for hemophilia “A”. Found that mothers bleeding problem could transmit hemophilia to his sons and their daughters could pass on to their grandchildren and great grandchildren.
In 1928 , Dr. Hopff describes the disease for the first time the word hemophilia .
The most famous hemophilia was the last Tsarevich of Russia : Alexis Nikolayevich Romanov , after transmitting his mother, Alexandra Feodorovna Romanov , granddaughter of Queen Victoria of England , also transmitting the disease. Also, the cases of hemophilia in the Spanish royalty in the children of Alfonso XIII and Queen Victoria Eugenia , also granddaughter of Queen Victoria of England , and deeply affected the Spanish monarchy.
The diagnosis of the type of hemophilia and its severity is made by history and a blood test to measure in the laboratory, through special tests of coagulation, the degrees of the different factors. The aim is to establish the severity of the disease and decide the best course of treatment by the patient.
Condition Prothrombin time Thromboplastin time Bleeding time Platelet count
Hemophilia without alteration prolonged without alteration without alteration
The main feature of haemophilia A and B is the hemarthrosis spontaneous and prolonged bleeding. The most serious bleeding is occurring in joints, brain, eye, tongue, throat, kidney, gastrointestinal bleeding, genital, etc..
The most common clinical manifestation in hemophilia is hemarthrosis, intraarticular bleeding, particularly affecting the single-axis joints like the knee, elbow or ankle. If a hemarthrosis repeatedly in a joint, originates deformity and muscle atrophy called hemophilic arthropathy. 1
In each cell there are 46 chromosomes : half the we inherited from the mother and half from the father. The chromosomes contain the instructions necessary to direct how cells make proteins that the body requires to function. These instructions are contained in small formations are called genes, consisting of DNA, which are the basic structure of life.
Chromosomes come in pairs, so we have two copies of all our genes, if there is any damage to a gene or a chromosome, there is a backup copy of that gene or chromosome that may fulfill the functions normally. But there is one exception, the sex chromosomes: X and Y.
The female sex is determined by two X chromosomes (XX) and males have one X and one Y chromosome (XY). The X chromosome contains many genes that are common to both sexes, as the genes for the production of factor VIII and factor IX, related to blood clotting.
Women have two copies of these specific genes while males only one. If a male inherits a chromosome with a damaged gene factor VIII is the only gene that receives and has no background information, so it may not produce the clotting factor.
This inherited abnormality occurs in women, but in very low percentage, since women usually carry the gene, also are exposed to its consequences, as to manifest the disease need two defective copies, which is very unlikely. Currently, in Spain, the incidence of people born with hemophilia is one in 15,000.
There is currently no curative treatment available and the only thing you can do is correct the bleeding tendency given intravenously the missing clotting factor, factor VIII or IX.
Replacement therapy was a major breakthrough for both quality of life and the survival of patients. Obtaining clotting factors from human plasma resulted in many cases, transmission of viruses, especially HIV (AIDS) in the 80′s, which meant a serious setback in the life of hemophiliacs.
In the middle of that decade were first introduced viral inactivation methods lyophilized concentrates, transforming them into products much safer.
Currently, lyophilized concentrates double virus inactivated plasma derived are safer and are constantly evaluating and introducing technology in products that will inactivate these new viruses and other infectious agents such as prions, which could pose a threat to those who use products derived from human plasma.
In recent years, development of genetic engineering has made possible start a new era in the treatment of disease. Since few years ago, have developed preparations purest clotting factors, without human plasma. Recombinant factor VIII, the most massified, occurs from cells cultured in laboratory. The World Federation of Hemophilia recommends the use of recombinant products, as it ensures safer with the same efficacy as plasma products.
There are enormous expectations of treatment by gene therapy, which consists in the introduction of genes in certain cells of the patient that are capable of combining with the existing genetic material, providing the missing information to manufacture the protein deficit causing the disease.
The goal of physical therapy in the treatment of hemophilia prophylaxis, is to advise and plan physical activities and sports with minimum risks to prevent the onset of muscle – skeletal injuries consecutive to poor physical condition.
Moreover, regarding the treatment of injuries, physical therapy shows its cooperation and resolution of the bleeding episode, acting on inflammation, decreases pain and recover lost function, avoiding or reducing the consequences.
There are two types of treatment: Prophylaxis (the patient is injected intravenously for the medicine several times a week) or demand (the patient is injected medicine whenever bleeding occurs).
Today the survival of a hemophiliac is high, thanks to the intravenous delivery of AHF. People with asthma can live a normal life with proper treatment.
Guidelines for action in hemophiliacs
Never give Aspirin to a patient with hemophilia.
It is totally contraindicated intramuscular injection.
Coverage with hemostatic factor.
When to go to hospital
It is important to know when to go to the hospital and who they can contact to inquire about a bleeding problem before it occurs. Certain personal circumstances or local conditions, proximity to a specialized center, can modify the possibility of hospital care, but these situations are those that require a quick reference and immediate treatment:
Pain in joints or muscles. Do not wait until the swelling is visible.
External bleeding can not be stopped or recurs after first aid treatment.
Blood in the urine or stool.
After a fall with head injury or other head injury, or if there is headache or nausea and vomiting of time without cause.
Bleeding or swelling in the area around the neck.
Unexplained abdominal pain.
It is important in arriving at a hospital, saying the condition of hemophilia and ask for the hematologist at the center
Transmission table hemophilia
The hemophilia A and hemophilia B are inherited gonosómica (sexual, X-linked), the altered gene in hemophilia A locus is located on the X q 28 , and hemophilia B, X q 27.1 – q 27.2 .
It thus has a much higher prevalence in males, where it acts as holándrico character :
With a hemophiliac father and mother healthy non-carrier: 100% of their daughters will be healthy carriers (inherited the mutated allele from the father), and 100% of the children will be healthy non-carriers (who do not receive the mutant X).
With a hemophiliac father and mother healthy carrier (heterozygous): 50% of daughters will be healthy carriers and 50% of daughters are hemophilic. As for boys, 50% are hemophiliacs (they receive a single maternal X, which in this case is mutated) and 50% will be healthy non-carriers (have received without default X).
With a healthy father and mother healthy carrier *: 50% of daughters will be healthy non-carriers, and 50% will be healthy carriers. As for male children, as in the previous case, 50% will be hemophiliacs and 50% will be healthy noncarriers.
Hemophilia C is autosomal recessive, with equal frequency in both sexes.
* Note that there is no possibility of a male carrier
Hemophilia is a condition suffered by almost exclusively men, and almost all hemophiliacs are children of healthy mothers, who carry the gene, meaning that their ancestors there was a hemophiliac, but nearly one third of patients have occurred without any a family history. In these cases, hemophilia is caused by a mutation in the gene from the mother or child.
The disease has been particularly studied because it affects many of the European royal families, at least those whose members are descendants of Queen Victoria of England who transmitted hemophilia to one of his sons (who died of internal bleeding after a fall) and at least two of his daughters who were the ones that spread the disease by the European royal families. No known cases of hemophilia among the ancestors of Queen Victoria and this was normal, it is assumed that the disease appeared by mutation of one allele of this standard.
In families in which one member is affected is important to identify women at risk of being carriers and perform genetic counseling. Ideally, this advice before making any woman at risk of family arises having children.
Counseling should consider two aspects: the data indicating the severity of hemorrhagic manifestations and the knowledge that women of a family with hemophilia patients are carriers of the disease.
The family should know the implications of the disease, how it is inherited, the probability that happen again and that there are alternatives. Genetic counseling should be an educational and informative, but by no means of taxation.
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Classification and external resources
ICD – 10 D66 – D68
ICD – 9 286
306.9 thousand 264900
DiseasesDB Five thousand five hundred fifty-five 5561 29376
The Hemophilia is an anomaly Constitutional of blood clotting related to a deficiency of coagulation factors . These defects are due to a deficiency of one of the following factors: XII, XI, IX or VIII, or the presence of anticoagulants against one of these factors.
The clinical manifestations of disease are proportional to the deficit of clotting factor. Clinical manifestations correspond to hemorrhages that can reach each organ, especially the joints ( hemarthrosis ) and muscles ( hematoma ). The disease can be severe with manifestations in the first year of life and light with very few events.
There are several types of hemophilia-related coagulation factor deficiency:
the hemophilia A ( classical hemophilia ) is a mutation of factor VIII
the hemophilia B (or Christmas disease ) is a mutation of factor IX
the hemophilia C (or Rosenthal’s Disease ) is a mutation of factor XI .
Summary [ hide ]
1 Different hemophiliacs
1.1 Hemophilia A
1.2 Hemophilia B
1.3 Hemophilia C
1.4 Hemophilia rare
2.1 Hemophilia minor
2.2 Moderate Hemophilia
2.3 Severe hemophilia
4 Transmission of the disease
7 Other syndromes hemophilia
7.1 Other hereditary deficiencies in coagulation factor
7.2 Deficits acquired coagulation factor
8 Notes and references
9.2 External Links
Mutation of the gene F8 the locus q28 of chromosome X encoding coagulation factor VIII. This mutation is often an inversion of intron 22 either deletions and insertions .
The incidence of this disease is 1 in 5,000 births of boys with a prevalence of 1 in 10 000 in countries with high sanitary equipment.
It can be confused with von Willebrand disease because it has also decreased factor VIII.0
Mutation of the gene F9 the locus q27 of chromosome X encoding the coagulation factor IX.
There are over 2100 mutations of this gene may carry. The incidence of this disease is 1 in 20,000 male births with a prevalence of 1 in 25 000 in countries with high sanitary equipment. It can also be called Christmas disease.
Hemophilia C is deficient factor XI . It reaches about one in 100 000 1 .
This is a mild form of hemophilia not related to gender. Most often it does not require treatment. In very rare cases, women are affected.
In very rare cases of hemophiliacs more specific can be observed, for example haemophilia B Leyden, which is progressively eliminated from puberty to end on a rate of 30 to 70%.
The blood of a person with hemophilia does not clot normally. Bleeding are not more abundant, or faster than normal, but last longer. Many people believe that hemophiliacs bleed a lot from minor cuts. The wounds are usually not serious, they do not bleed more than a non-hemophiliac otherwise a simple bandage to stop the bleeding. Internal bleeding, by cons, have a much more serious consequences, especially if they involve the joints, especially knees, ankles and elbows, as well as soft tissue and muscles. The severity of hemophilia is based on the percentage of activity of coagulation factor deficiency. The normal activity of a human is located between 50 and 150% rate defense.
Hemophilia is classified as “minor” to “severe” based primarily on the rate of defense, but also the potential to make spontaneous hemarthrosis (without any movement, an “outpouring” of blood occurs at the joint, attacking the cartilage):
between 5 and 30% activity of clotting factor .
Can never be of bleeding disorder
No bleeding unless an injury
Risk of prolonged bleeding after surgery or serious injury
between 1 and 5% activity of coagulation factor.
Risk of prolonged bleeding after surgery, serious injury or dental procedures.
May have bleeding episodes about once a month.
Bleeding rarely, if ever without obvious reason.
less than 1% of activity of coagulation factor.
Frequent bleeding into muscles or joints (mainly knees, elbows and ankles).
May have bleeding episodes once or twice a week.
Can bleed for no apparent reason.
Based on routine tests performed in the basic exploration of hemostasis :Hemophilia
Classification according to ICD-10
D66 Hereditary factor VIII deficiency
D67 Hereditary factor IX deficiency
D68. - Other coagulopathies
ICD-10 online (WHO version 2011)
Hemophilia ( . ancient Greek αἷμα haima “blood”, gr φίλος philos “friend”, also hemophilia ) is a hereditary disease in which the coagulation is disturbed. The blood clots from wounds or only slowly. Often there is also spontaneous bleeding, which occur without any visible wounds. Hemophilia occurs mainly in men. Affected persons are referred to colloquially as a hemophiliac.
Contents [ Hide ]
2.1 joint bleeds and their effects
2.2 muscle hemorrhage and its consequences
1.3 Treatment with the lack of factor VIII (hemophilia A) or factor IX (hemophilia B)
6 See also
8 Notes and references
9 External links
There are two known forms in the narrow sense of hemophilia and other diseases that are occasionally blurred subsumed under this term:
Hemophilia A (X-linked recessive hereditary clotting defect): Of Plastic Bins these, almost without exception (except for the globe, two documented cases of women) affected men, because they have only one X sex chromosome, while females have two of them. Here it comes to a deficiency of factor VIII (antihemophilic globulin).
Hemophilia B (X-linked recessive inheritance): lack of factor IX (Christmas factor) of the coagulation cascade with different histories of birth (heavy, medium, light). Due to this lack of blood clotting can proceed only very slowly.
The very rare autosomal recessive hereditary clotting defect (eg Stuart-Prower factor deficiency, factor X of the coagulation cascade) can are rather marked in both sexes equally, as in both sexes an equal number of autosomes occur (non-sex-linked chromosomes).
Para hemophilia (Hypoproakzelerinämie, Owren’s disease): autosomal recessive hereditary disease caused by deficiency of coagulation factor V ( factor V ).
Angiohämophilie ( von Willebrand’s disease ): It is the most common autosomal dominant inherited coagulation disorder. It is caused by a structural defect or a deficiency of von Willebrand factor varying degrees. The von Willebrand factor is a protein carrier of clotting factor VIII .
Hemophilia C (Rosenthal Syndrome): Here is lacking factor XI ( PTA ) of the coagulation cascade, so that may occur especially in children or easy bleeding into joints with minimal injuries. As with hemophilia A and B is the Quick value typically normal, while the PTT is prolonged depending on the severity of the defect.
Hemophilia bleed longer than normal people. Depending on the severity of bleeding may occur spontaneously, ie without corresponding obligations. This spontaneous bleeding occurs in healthy people as well and just as often to heal, however, quickly and silently. The bleeding can occur anywhere, but certain sites are typical in people with hemophilia, for example, joint bleeds below
An accident caused by a severe bleeding or may be kept in check only by the administration of clotting factors. Does this help is not possible in time, this may mean (in less severe injuries) death by bleeding.
Cutting, tearing and abrasions result in the most common subtypes of hemophilia initially not to greater blood loss than in healthy people, because the crustal formation caused by the intact blood platelets ( thrombocytes ) works first. Only the delayed blood clotting leads to the build-up may break up again and the bleeding is depending on the severity of hemophilia only very slowly or not at all satisfied. Even without external influence, it can therefore be subcutaneous or intramuscular hematomas occur.
The risk of internal bleeding in hemophilia patients is also increased (eg, renal hemorrhage with severe colic (closure of the urinary tract by thrombus )).
In carriers of the genetic defect (so-called female carriers) may be a bleeding occur, resulting in increased menstrual bleeding , bruising (hematoma) in Bagatelleingriffen such as tooth extraction or during or after childbirth may show. In rare cases, bleeding may also occur, similar to those of male victims (eg, joint bleeding).
Joint bleeding and its consequences
A common site of bleeding, the joints ( hemarthrosis ). The first bleeding in a joint (also referred to as initial bleeding) is often by an accident / trauma caused. Particularly affected are the large joints. Through the joint lining ( synovium ) are enzymes released that break down the blood located in the joint. For large volume effusions increases the synovial fluid (“joint lubricant”) and it will be more attracted to blood vessels. This leads to a higher probability of subsequent bleeding or inflammation. A cycle of inflammation and bleeding is set in motion, creating a so-called hemarthrosis , particularly unguided movements and twists and over stretching (at night), not “buckle”, stumble, etc., other joint bleeding (mostly ankle, knee, elbow, shoulder or Hüftblutungen rarely) result in what is usually associated with severe pain. The consequences of frequent bleeding events in elderly patients (since effective prophylactic therapy has only been about 30 years) are joint stiffness in some cases of severe type, early osteoarthritis – (maybe the surgery such as knee arthroscopy , synovectomy to towards the endoprosthesis (joint replacement) but also orthopedic aids (orthopedic), crutches , etc.) make it necessary – as well as abnormalities of the muscles and bone structure , the mobility of the joints by constant physiotherapy at a certain stress level maintained, or may be, but also improves .
Muscle hemorrhage and its consequences
Muscle bleeding occurred less frequently than spontaneous bleeding into joints and have mostly trauma as the cause. Depending on the location and size of the muscle they can be extremely time consuming and cause irreversible damage to muscle deformities. Muscle bleeding can also occur after intramuscular vaccination, which are given for instance in the buttock, upper arm muscle. Hemophiliac patients should receive only vaccine under the skin (“subcutaneously”). Typical dangerous muscle hemorrhages are to be found:
in the psoas muscle (running from the stomach through the pelvis to the leg, so that “keeping quiet” is almost impossible during the healing process and often recurs after apparent cure the bleeding). The resulting pain have been before the introduction of ultrasound scans mistaken for appendicitis (so-called “pseudoappendicitis”)
in the calf muscle (calf bleeding leads to a shortening of the muscle, making it the equinus, which in turn leads to increased stress on the calf when walking and can lead to further bleeding)
in the forearms (bleeding of the forearm can press on nerves and cause the hand next to extreme pain and immobility, and deformity of the hands)
Treatment with the lack of factor VIII (hemophilia A) or factor IX (hemophilia B)
The common until about 1970 therapy in hemophilia to stop bleeding was, in general, is to direct blood donation, blood or blood plasma to be administered in stronger and more acute bleeding to cool bruises and bleeding wounds derived from bovine blood fibrin bring to clot, which relatively rarely succeeded.
The current therapy is generally is a prophylactic or as needed to replace the missing or defective factor, where bleeding can be largely eliminated, and the patient can lead a relatively normal life, but for example in sports like athletics, boxing, winter sports and must refrain extreme physical stress. The therapy takes place for example in cases hemophilia A, B or von Willebrand’s disease by self-treatment (intravenous) with the missing factors. These factors were up around 2002 mostly derived from human blood plasma, which in the past, including many hemophiliacs with HIV , hepatitis C and B and other viruses have been infected. This was a so-called ” blood scandal known. ” The possibility of contagion can (has the hepatitis C virus discovered in the late 80′s) since 1988 are excluded as good as, if the existing methods of blood purification and virus inactivation are used as intended. Since about 1989, the factor VIII (hemophilia A) is also produced by genetic engineering. (See also gene technology in this paragraph), an intrinsic safety from contamination of factor VIII to offer such as a virus and to always ensure an adequate supply of patients. These genetically engineered concentrates are today considered the safest. The factor IX concentrate must be prepared from human blood. The genetically engineered drug is still under development.
The main complication in hemophilia A therapy today is the formation of neutralizing antibodies against factor VIII (FVIII), the so-called inhibitory antibodies or inhibitors . The antibodies reduce the effect of the added FVIII very strong, so that the necessary increase in the factor mirror is not reached, and there in the episode comes back to bleeding. This complication is known as haemophilia or Immunhemmkörperhämophilie known. Worldwide studies show that develop about 30% of patients treated hemophiliacs or inhibitory antibodies. It is still debated whether the inhibition occurs only by blocking the FVIII activity, or whether there is a higher elimination (English: clearance ) of the FVIII is the recognition by the antibody.
Haemophilia A may also be in substitution of factor IX, which is in the treatment of hemophilia B occur. Yet it is still considerably less – in 2 to 5 percent of cases. 
The disease is X-linked recessive trait. Women can makers for the inheritance of his hemophilia A or B, without actually suffering from the disease. For example, a wearer ( female carrier ) of the defective gene for hemophilia, in which the feature is not pronounced, gets sons, where the probability is 50%, hemophiliacs to be (see also genetic information ). Winner gets the daughters can pass on 50% of these statistically the gene to the next generation, without being personally affected by this disease. Once again, these girls have male offspring, then it is also possible that these are hemophiliacs. Because of this probability, but the disease can also skip several generations, if ever again daughters were present as winners. When hemophiliacs get children, they pass the disease on this any further, since it is X-chromosomally inherited. Male hemophiliacs may transmit the disease so only to their daughters.
In isolated cases of hemophilia A or B is possible in women. If the father and the mother of hemophiliacs is a carrier and passed on from mother to daughter, the feature-bearing X chromosome gets (50 percent probability), the daughter will be a hemophiliac. Furthermore, it is possible in connection with Turner syndrome than women to suffer from hemophilia, as in this case, only one X chromosome is present. However, there are only a few, usually very poorly documented cases of hemophilia in women. The occasional mention of women in literature and fiction haemophiliac is explained by misattribution of other clotting disorders.
Probably the earliest mention of the disease is found in the 5th Century in the Talmud , which exempts from the ritual circumcision to those boys, whose two brothers had died during the circumcision.
In the past, on average, suffered from many members of the European aristocracy and the ruling families of hemophilia, which is why they got the name “disease of kings”. Well-known examples are the British king – and the Russian Imperial family . The starting point here was the bearer of disease Queen Victoria of Great Britain , whose granddaughter Alix of Hesse-Darmstadt, the Tsar Nicholas II and married the disease to their son Alexei was over.
Mario by Depka Prondzinski, Karin Kurnik: hemophilia. A Guide for Patients . Triassic-Verlag, Stuttgart, 2008, ISBN 978-3-8304-3432-0
Mario Depka of: blood clotting. Current aspects of physiology, pathophysiology, clinical manifestations, diagnosis, prophylaxis and therapy . UNI-MED, Bremen / London / Boston 2002, ISBN 3-89599-554-1
Stephen Pemberton: The Bleeding Disease: Hemophilia and the Unintended Consequences of Medical Progress . The Johns Hopkins University Press, 2011, ISBN 1-42140-115-0 .
Victoria (United Kingdom)
Queen Victoria on her Golden Jubilee in 1887
Signature of Queen Victoria
Coat of arms of Queen Victoria
Victoria (German also: Victoria , eigtl Alexandrina Victoria ; * May 24 1819 in Kensington Palace , London , † January 22 1901 at Osborne House , Isle of Wight ) was from 1837 to 1901 Queen of the United Kingdom of Great Britain and Ireland and 1876 was the first British monarch and the second man ever the title Emperor of India . She was the daughter of Edward Augustus, Duke of Kent and Strathearn and Victoria of Saxe-Coburg-Saalfeld .
With its accession to the throne ended because of the Kingdom of Hanover applicable Salic law , which excludes women from the succession, the personal union between Hanover and the United Kingdom. With Victoria’s death ended the reign of the House of Hanover on the British throne, and her son King Edward VII began the reign of the House of Saxe-Coburg and Gotha .
As a constitutional monarch , she was a pro forma ruler of more than one-fifth of the Earth and a third of world population. During her reign saw the upper and middle classes of Great Britain an unprecedented economic boom, and the British Empire was at the height of his power. Characteristic of Denver Divorce Attorney her reign was the influence of her cousin and her husband Prince Albert of Saxe-Coburg and Gotha and her near-total withdrawal from the public after his death, 1861st They handled the constitutional monarchy very headstrong and confident, even though they already at the opening of parliament had to read the mandatory speech of each prime minister – a “theater state” in which they are mostly from the Lord Chancellor was represented. Victoria reigned 63 years and 7 months, longer than any other British monarch before her. Through their numerous offspring, she received the nickname “grandmother of Europe”. It was because of her long reign, named after the Victorian era .
Contents [ Hide ]
A family background
2.1 Early Childhood
2.2 In Kensington Palace
2.4 Victoria’s accession to the throne
2.5 The first years of reign
6.2 The first years of marriage
7.2 Revolution of 1848
8.2 Famine in Ireland
9.2 Conflict with Lord Palmerston
2:10 The Crimean War (1853-1856)
2:12 John Brown
2:13 Gladstone and Disraeli
2:14 The Golden Jubilee
2:15 The Diamond Jubilee
188.8.131.52 Single-digit name
184.108.40.206 Double-digit name
220.127.116.11 Three-digit name
18.104.22.168 Four-digit name
5.3 Europe’s grandmother
Six letters and diaries
7 Title and Title
10 Web Links
11 Notes and references
Victoria of Saxe-Coburg-Saalfeld , painting by an unknown artist
After the prince with Leopold of Saxe-Coburg-Saalfeld, married presumptive heiress British Princess Charlotte Augusta in 1817 after a stillbirth had died, there was the British royal family to legitimate offspring. Of the seven surviving sons of George III. at this time only the Prince Regent were George and two of his brothers married in style. The marriages of the Duke of York and the Duke of Cumberland had been childless, and the Prince Regent with Caroline of Brunswick was as shattered. The death of Charlotte Augusta was, therefore, for the previously unmarried sons of George III. the offense, under the Protestant princesses of Europe to keep on the lookout for suitable wives.
The widow of Prince Leopold made 1814 his sister Victoire of Saxe-Coburg-Saalfeld , the widowed Princess of Leiningen , with Edward Augustus, Duke of Kent and Strathearn known. On 13 July 1818 married in a double wedding of the Duke of Kent with the Princess of Leiningen and his brother William, Duke of Clarence with Adelaide of Saxe-Meiningen .  Securing the legitimate successor to the House of Hanover was not, however, for the Duke of Kent Marriage is the only reason. Highly in debt and because of its pedantic and often sadistic official duties since 1803, he was relieved of his military office. With the marriage, he combined the hope that his appanage would be increased significantly.
Louise, Duchess of Argyll : Statue of Queen Victoria at Kensington Palace
Princess Alexandrina Victoria of Kent was born on 24 May 1819 in Kensington Palace , and was born at that time in fifth place in the British throne . It initially spoke little evidence that they would succeed to the British throne. More likely it was that made the marriage of her father’s older brothers would still emerge offspring. The enthroned before entitled to follow their Prince William had not less than ten illegitimate children. On 24 Alexandrina Victoria was in June 1819 in the Dome Room of the Kensington Palace baptized. For this festive occasion we had the royal baptismal font from the Tower brought.  Previously, there was an uproar with the Prince Regent George arrived. Victoria’s parents had suggested a number of common names back then in the royal family (including Charlotte and Georgina). George did not accept this, leaving only two, rather unusual name. The godfather of the princess were the Prince Regent George, the Russian Tsar Alexander I (his honor, she received her first name), Victoria’s paternal aunt, Queen Charlotte Auguste of Württemberg and the maternal grandmother Augusta of Saxe-Coburg-Saalfeld . The small Victoria was called Drina within the family, later she was named Victoria, the supposedly “englischeren” of the two first names. 
At Kensington Palace
Princess Victoria at the age of 4 years, 1823 (Stephen Poyntz Denning painting)
Eight months after the birth of Princess Victoria’s father died Edward Augustus, Duke of Kent. The Duchess of Kent had given the high debt mountain reject the legacy of her late martial arts denver husband. With her husband’s family, she found little support. King George IV , who had a few days after the death of his brother, Edward Augustus, ascended the British throne, had been skeptical of the marriage between his brother and the Princess of Leiningen over. He ignored his sister and her daughter, and would have preferred it if the Duchess had returned with her family to Germany. On the advice and with the financial support of her brother Prince Leopold continued to live at Kensington Palace, the Duchess continued. 
King Ernst August of Hanover, Duke of Cumberland
The Duchess of Kent came under the influence of John Conroy , the Duke of Kent had used as an executor. John Conroy was betting that Princess Victoria would ascend the throne still a minor, under the regency of the Duchess of Kent. John Conroy, this would have to go through this ruler to influence and power. To secure his influence, he made sure that the Duchess and her daughter largely isolated from the British court circles remained. John Conroy was able, for example, the Duchess of Kent to persuade the Duke of Cumberland – to Victoria the next in line of succession -. the princess strive for life and therefore a secluded and isolated life is necessary  Victoria grew by a environment in which they had little contact with other people. best credit cards Her few playmates counted John Conroy’s daughter Victoire and Victoria’s twelve years older half-sister Feodora .  John Conroy scattered among other rumors that the princess was not mentally stable, and denver car accident lawyer therefore could not exercise their reign. This occasionally in the history books as the Kensington system called power protection continued even after switching to the throne of William IV (1830) continues, which would, unlike his older brother and his niece saw presumptive heir to the throne like to frequent in his court circles. So it was not even allowed Victoria to attend the coronation of her uncle.
When it was increasingly apparent that Crown Princess Victoria at the time of the throne change would already be of age, tried to wrest her Conroy 1835, the signature of appointing him to the throne after moving to their private secretaries. Princess Victoria, despite the enormous pressure exerted by her mother, and just recovering from a serious illness (probably typhus) that force and did not sign.  This led to a complete break with her mother. By the time of accession to the throne, the two hardly exchanged a word with each other. These attempts to manipulate the princess also led the British royal court to a head. During the birthday dinner at the 18th Birthday of the Princess – the day when they came of age – King William IV declared that he was grateful to experience this moment, because he prevented in this way a regency completely unsuitable persons.
As an educator of the young princess was the Baroness Louise Lehzen determined to Victoria had a very close relationship, but it was not very suitable to prepare the princess on her future role as monarch. In 1829 still came to an official tutor: Reverend George Davys, a liberal Anglican priest and later Bishop of Peterborough .  Victoria received a superficial education, which corresponded to the young ladies of high society of her time. The emphasis on biblical teaching, studying ancient and modern languages, dance, singing and drawing lessons and history (Victoria’s favorite subject). The willingness of the student should have kept it in check.  Victoria later spoke fluent German and French. In daily contact with their German mother Victoria spoke English and not German, as the Duchess, this is politically wiser thought. 
Franz Xaver Winterhalter: Leopold I, King of the Belgians
Probably in March 1829 Victoria learned from her place in line of succession. She found a book on English history a page which had been previously removed, presented to the throne. Victoria is said to have told Lehzen: . “I will do my best” . Some authors refer to such statements, but also in the realm of legends 
Among the few people they prepared for their role as monarch, was one of her uncle Leopold. Prince Leopold was crowned in 1830 in Belgium and has since reigned as Leopold I in Brussels. He advised the princess in writing and advised her books and manuscripts that they should prepare for her role as Queen.  In the weeks in which William was IV in the dying and the change of dynasty was imminent, he presented her as a consultant also Christian Friedrich von Stockmar to the side. With the help of the princess was able to fend off John Conroy’s previous attempts to secure power. 
Victoria’s accession to the throne
Franz Xaver Winterhalter : Queen Victoria in Coronation Robes (1837)
Victoria took on 20 June 1837 at the age of 18 years after the death of her uncle William IV to the throne. On 21 June was the proclamation in the St. James’s Palace . Victoria was seen in the following years at numerous ceremonial occasions in order to present the people as the new ruler. The young queen was taken after two very unpopular predecessors in their empire with enthusiasm.  A month after her accession the Queen moved from Kensington Palace to Buckingham Palace to that served for the first time as the official residence of a monarch.
Buckingham Palace, in 1837, before the establishment of the youngest leaf to mall and with Marble Arch as the gateway
Your new independence she used to be the dominating influence of her mother and to liberate this John Conroy. Her mother was granted only the British role in court life, the protocol envisaged for them, their relationship improved again after Victoria’s marriage. John Conroy was no place in the British court, he still remained a member of the household of the Duchess of Kent. On 28 June 1838 took place in a five-hour ceremony, the royal coronation in Westminster Abbey instead. Parliament granted her 200,000 pounds for this event, four times what it had granted William IV. The “Coronation June” allegedly four hundred thousand visitors came to London. For the first time was also attended by the members of the House of Commons at the ceremony, which highlighted the increasing democratization of the country. 
First years of the reign
William Lamb, 2nd Viscount Melbourne
The first year of the reign of the young queen, who was hardly prepared for their role was, through the support of Prime Minister Lord Melbourne successfully. Lord Melbourne was next to her uncle Leopold, the second father’s friend and teacher in Victoria’s life.  to the 58-year-old prime minister gave her full confidence. Melbourne never let on how much he was surprised by her naivete and ignorance, and tried to fill the gaps in their education. He was in the early years of her mentor, who advised them in political, familial, and even fashionable things, and so strengthened their self-confidence. This intimacy was often interpreted as love. Victoria had a lot to learn, to serve as constitutional monarch to be not only the representative of the state that could not express any opinion other than their ministers. 
J. Linell: Portrait of Sir Robert Peel , 1838
From Melbourne, Victoria met to assess the political leaders to find out their strengths and weaknesses,. These were skills that were valuable to them in the following years  These good offices of Melbourne lasted only as long as its government has remained stable.
After the Whigs had lost the majority in the House, introduced Lord Melbourne in May 1839 resigned as prime minister is available. Since neither the Tories nor the Whigs possessed a sufficient majority in parliament, Melbourne was hoping for a failure of the new government and subsequent elections, which should strengthen the Whigs. This was the politically untrained queen hidden for them of the idea was intolerable that the Tories and thus Sir Robert Peel should take over the government. Sir Robert Peel, who was willing to form a government expected by the Queen, the royal household consisted exclusively of Whigs to take some ladies from the Tory circles as the ladies. He held these human adaptation to the future balance of power as inevitable. Victoria refused this categorically because it found that the choice of court ladies as their private affair. Then peel back the government had commissioned, and Lord Melbourne was Prime Minister continued. The Queen celebrated this as a political victory and was convinced that they have defended their dignity.
In this so-called ” Hofdamenaffäre “moving Victoria, with their strict refusal in a constitutional gray area.  The Hofdamenaffäre and her imprudent behavior in the Flora Hastings affair , in the Flora Hastings, one fell ill with a liver tumor -waiting , wrongly an illegitimate pregnancy was suspected, Victoria cost to the public prestige and sympathy. Lord Melbourne was not in the Hofdamenaffäre still in the Flora Hastings affair reacted so strongly, as one would have of him as an adviser and confidant to expect an inexperienced monarch.  Victoria herself judging her performance in her first political campaign 60 years later with the sentence. “It was a mistake”  The British public now increasingly demanded that the British Queen should marry. It was hoped that a husband had a moderating influence on the often very emotional acting queen.
The first years of marriage
Marriage of Albert and Victoria in 1840, painting by Sir George Hayter
Franz Xaver Winterhalter: Albert of Saxe-Coburg and Gotha (1842)
Queen Victoria, 1845 (painting by Alexander Melville)
Victoria married on 10 February 1840 her cousin, mother, Albert of Saxe-Coburg and Gotha , the second son of Duke Ernst I and Louise of Saxe-Coburg-Altenburg . Albert and Victoria, were even though it was the combination of both uncle, King Leopold I of Belgium, arranged, before the marriage fell in love. Had first met the couple in 1836 during a visit to Albert’s in the UK. The then 17-year-old Victoria was, as their diaries, “charmed” and for the first time in her life she had “the prospect of great happiness”.  On 10 October 1839 there was a return visit by Albert, and four days later held at Victoria to Albert’s hand. The subsequent engagement period was marred by some inconsistencies. The public was less enthusiastic about the connection of the British queen with a minor German prince, another “happy Coburg”.  The lack of majority vote of Lord Melbourne in Parliament could not Victoria assert that Albert the Prince Consort (English ” Prince Consort “was nominated). Thus Albert was the first wedding, a simple Prince of Saxe-Coburg and Gotha, and was raised later to the Prince Consort. Albert was also a comparatively low appanage 30,000 pounds per year set – not much compared to Prince Leopold of Saxe-Coburg-Saalfeld , who 23 years earlier, after his marriage to the British heir to the throne Princess Charlotte , had received 50,000 pounds a year. Victoria wore at her wedding, a white dress, white wedding dresses by wearing became popular. 
Victoria and Albert’s nearly twenty-one connection was considered to be very happy. Victoria was during her marriage in all decisions, even politically, much have been under the influence of the Prince Consort, which has been rumored, especially in his later years, both king and prime minister.  expressed Queen Victoria herself in a letter of 9 June 1858 at her oldest daughter like this:
“I can never believe or admit that any other man has been so blessed by fate as me, with such a man, such a perfect man dad was everything to me, it still is. [...] He was everything to me, my father, my protector, my guide, my guide in all things, I would almost say it was my mother and husband at the same time. [...] I am stunned when he is not with me.  ”
Gilbert Munger: Windsor Castle
In the first five years of their marriage the couple had five children. This meant that Albert is increasingly supported in their roles as Queen Victoria. With the birth of their first child was, according to Anson Albert, Albert’s secretary, “in fact if not in title, the private secretary to Her Majesty.” The influence was felt on the Albert’s royal household and the finances. These duties previously covered by the Baroness Lehzen who was in Albert’s eyes but do not necessarily suitable for printing and finally take their leave of the prince had in 1842. As the end of the era, more and more predictable in Melbourne and a Prime Minister Peel was inevitable, Albert prevented by his timely diplomatic intervention and a renewed “Hofdamenaffäre”. Peel himself was later to be very much appreciated by Victoria. Melbourne, Victoria advised in his farewell in 1841, to seek advice from her husband in political affairs, a council, which should follow the queen. The queen grew up in these first years of marriage, more and more into her role, and Prince Albert had helped significantly. 
Osborne House, 1910
Soon after the wedding of Victoria and Albert moved their center of life after Windsor Castle , especially since Albert preferred the country life of the capital. To obtain more privacy, may 1845, the couple bought Osborne House , a 400-acre country estate on the Isle of Wight . The building was given to Albert’s plans, an Italian version, the garden was also designed to his specifications.  For the kids, a Swiss house was imported, in which the prince should learn carpentry and gardening, and the princesses housekeeping and cooking. The purchase was through considerable private expenditure limitations of the queen, who were indebted to Albert’s Council, and through the sale of the Royal Pavilion in Brighton will be funded.
Revolution of 1848
Chartist uprising . (Engraving, from a publication from 1886)
After the first year of the reign of Victoria was without any major political unrest, should the European revolution of 1848 does not pass well to the UK without a trace. Although Prime Minister John Russell discouraged her about it, the deposed king granted Victoria Louis Philippe of France , with whom she was distantly related, asylum and brought him in Claremont House under. In Britain, even a financial crisis broke out: Speculators had caused huge losses due to inflationary railway shares, the price of wheat had fallen to its lowest level. The prevailing unemployment and poverty were the Chartists , a reform movement that is the beginning of the 19th Century, had formed a new impetus. After a mass meeting scheduled for 10 April in London was announced, the royal family was a precaution taken to Osborne House, Victoria had just their sixth child was born. The demonstration was unexpectedly violent and spectacular. Only twenty thousand demonstrators gathered at Kennington Common, and the leader Feargus O’Connor brought a petition that demanded a liberalized citizenship and has been signed by more than one million people had. Victoria felt, despite the revolution in Britain was relatively unscathed expired, for the first time, fear for their existence, and saw the Chartists in the wrong:
“I am of the opinion that revolution are always bad for a country and cause untold misery for the people. Obedience to the law and to the ruler’s obedience to a higher power “. 
The Queen should continue but no significant influence on social policy to take the country. First, because they knew this area is in good hands – Prince Albert were the social conflicts even from a Christian religious conviction does not matter – and, secondly, because this was a world in which it was found difficult to cope. Where misery they personally paying attention to it was found helpful, especially the ordinary people of the highlands made her the burden of poverty quite understandable. The underprivileged classes beyond the middle class remained alien to her. As a widow, should Victoria reminder for several times in the eighties, social policies, but should rather be understood that commitment as a commitment to one’s spouse, rather than personal choice of inner conviction. 
The harsh climate of social policy 1840-1850 was certainly responsible for five of the seven attacks , all the Victoria should survive without any major injuries, fell into this decade, the last two in 1872 and 1882. There was certainly no coincidence that all the defendants, the courts attested mental disorder and were anxious to exclude political motives. It was not in the interest of the State to confirm the urgency of the social conflicts of conspiracies against the queen. Of course it was in the public impression with which self-control – rather unusual for Victoria -. They endured these attacks on their lives 
Famine in Ireland
Henry Doyle : Etching emigrants leaving Ireland , 1868
Ireland was a special case in the United Kingdom: the law after it was part of the Kingdom with representation in parliament, but has been treated in many ways like a colony. The policy of the British landowner in Ireland developed along with the blight of famine of 1845-1849 . Up to 1.5 million Irishmen should thereby starving and many more emigrated to America. The 1845 ruling Prime Minister Robert Peel could not prevail with his demands for repeal of the Corn Laws in order to introduce cheaper grain to Ireland can. Prince Albert also wrote on behalf of Victoria a memorandum in which it expressed its dismay expressed and suggesting suitable measures to alleviate the greatest need. These measures, as they had been taken in other countries affected by the blight successfully contained, for example, the opening of the ports were, however, initially on deaf ears. As could be foreseen for the year 1846 an even poorer potato crop reached Peel the abolition of the Corn Laws, but it lost the support of his party. Victoria, who was allowed to express their sympathy with the Irishman only privately donated, the “British Society for the relief of distress in the most remote communities of Ireland and Scotland,” 2000 pounds (not just 5 pounds, as has been alleged at times). 
In order e-cigarette to support the people of Ireland, Victoria initially planned to buy an Irish country house. She distanced herself but cancel the plan because it “would have been interpreted Irish Landlord conduct” as likely.  In 1849 she decided to become a royal tour of Ireland. The Irishman brought to meet her during her visit enthusiasm and affection. The departure and re-embarkation took place in “every conceivable sign of affection and respect”  instead of so many contemporaries saw in Victoria itself this visit an opportunity for reconciliation, but it was not used by Victoria’s ministers. There were further visits in the years 1853, 1861 and 1901, the responses did not have the opportunities that would have been possible for the first time. On the contrary, they reinforced the feeling of Irishmen to have been abandoned. This should lead to further difficulties. Eventually it came to sharing 
Balmoral Castle, photo from 2004
1852 bought the pair, fascinated by the Scottish landscape, the Balmoral Castle in the County of Aberdeenshire . Like Osborne, it was Albert’s plans for a Victorian baronial rebuilt. This acquisition was made possible by an unexpected inheritance. John Camden Neild (called “miser Neild”) had all his possessions – his own land was worth about 250,000 pounds – the Queen bequeathed. As Osborne House Balmoral Castle was private property of the couple.  Despite the initially very tight conditions in Victoria was far better off in Osborne and Balmoral, as in the “dark beauty” Windsors and the urban atmosphere of Buckingham Palace. In its 40 years, widow of Osborne and Balmoral, much to the chagrin of their politicians who were there cited, fully integrated into the annual process. Even in times of crisis government, she was hardly moving to get to London, to enable efficient contact with their ministers. 
Conflicts with Lord Palmerston
John Henry Palmerston, 3rd Viscount Palmerston
Ireland should also be the trigger for a further tightening of an already long simmering conflict with Lord Palmerston . This was almost continuously since 1830, foreign minister and very popular among the population. The queen, however, he challenged a policy of fait accompli: instructions to the ambassadors were released without authorization Victoria’s open letter to the monarch in the Ministry of Foreign Affairs and Minister informed decisions about the newspaper. Finally, there was a confrontation, which is about the British constitutionalism was. Palmerston thus gave to understand that the Crown did not interfere in foreign policy. Victoria and Albert looked at just this but as an essential right. As explained in Palmerston revolution in 1848, the United Kingdom for any liberation movement allies on the continent, it brought with it the people as a political power factor comes into play. With this liberal foreign policy, he shocked the queen, if only for domestic political reasons. You asked the question what impact this would have with his emancipation efforts in Ireland. All attempts by the court to get rid of the unpopular Minister of Foreign Affairs – Victoria called him her “pilgrims stone” – failed.
As on 2 December 1851 Napoleon III. in Paris, achieved a coup expected, the Queen of Great Britain a strict neutrality. But Foreign Minister Palmerston congratulated the French Ambassador to the successful coup. Palmerston’s dismissal was therefore inevitable. It should be the only time that Victoria obtained the dismissal of a minister, and it should prove to be just as apparent victory. Their subsequent demands to the government, a program with definite guidelines submitted to the foreign policy have to have been part of Prime Minister Lord Russell refused, and even the end of 1852 Palmerston was again a member of the government, now as a Minister of the Interior .  is expected for Palmerston this still painful have been since just now joined the British foreign policy into the public consciousness.
The Crimean War (1853-1856)
→ Main article: Crimean War
The Crimean War began as a conflict between Russia and Turkey, entered at the side of Great Britain and France, Sardinia and later in the war. Cause that was the rule of protection over the Orthodox Christians of the Ottoman Empire, Russia’s expansionist ambitions cloaked in the Balkans, would allow neither Britain nor France. Through this war, numerous challenges came in the British military and hospital system to day, including the 1855 resignation of the Government Aberdeen and led to numerous reforms.
Neither Victoria nor Albert could take the direct influence corresponding to their understanding of sovereignty. But the authority of the crown was large enough that their advice has been taken by the Cabinet and to some extent taken over. Victoria discovered her maternal duty of care for the country’s army. She was the impetus for military reform and supported the renewal of the hospital system. In the future, they should argue that the troops remain beyond the influence of politicians as much as possible would have to stand by the commander in chief, but with the sovereign would have a direct link.
Robert Gibb: The Thin Red Line , 1881. The painting shows the 93rd Regiment of Foot (Sutherland Highlanders) in the fight against Russian cavalry at the Battle of Balaklava
War as a policy was contrary to the monarch, where they could not prevent him, they urged immediate peace, and especially in one respect they differed from other contemporary rulers: they showed compassion and personal concern for their soldiers. As an outward sign of this support Victoria in March 1856 took part in the event of a maneuver.  On 26 June 1856 gave the Queen, during a military parade in Hyde Park , Crimean veterans, the first Victoria Crosses . These were a product of this war, as Victoria said, you need a medal for extraordinary bravery in battle, which could be awarded regardless of rank.  After the victorious conclusion of peace in 1856 thanked Lord Palmerston, who had since 1855 Prime Minister, the Queen, saying that the task had to meet he and his colleagues had been made easy by the relatively “enlightened ideas that had your majesty in all great affairs” .  The relationship between the Court and Lord Palmerston had eased considerably. Palmerston’s vigorous drive for the end of the Crimean War, and Albert’s tireless work as a counselor and organizer led to a rapprochement and appreciation. 
At the beginning of the Crimean War had led to the resignation of Palmerston as Home Secretary at a press campaign against Prince Albert, who was perhaps even initiated by Palmerston himself. Among others, the radical Daily News rumors were circulated, cheap paint ball by which the prince – and even the Queen were even arrested for high treason in the Tower – which is still reviled as “German” was.  was a result of this press campaign but Albert’s constitutionally ambiguous position became clear. His services to the UK were indisputable, even if it only during the first he launched the Great Universal Exhibition of 1851 was really popular. His supervision of the official business of the crown was expressly requested by his wife, but it was in the Constitution is no precedent for his position. Even after he was finally appointed in 1857 to the Prince Consort (Prince Consort) – Victoria had always wanted the title of King Consort (King Consort) for him – there was no description of the powers of this position. The government has merely stated officially that the prince had the right support to advise the Queen, the extent of the consulting work that was not defined, however. 
Queen Victoria. Photography, in 1861)
When Albert in December 1861 only 42 years old to typhoid fever or cancer , died in Victoria began an almost unceasing mourning period. She was dressed up in her life, only her widow’s weeds , expressing their deep grief and appreciation for her husband who died young. The funeral of the Queen took – even for its time – strange forms: Albert’s bedroom at Windsor remained unchanged, his bed sheets and towels were changed regularly, every evening was provided warm water in his room.  For the early death of her husband they made her eldest son Edward in charge, who in Cambridge had been involved in the so-called Nellie Clifden affair. This had prompted Albert, in already diseased condition to travel with him to speak with him.  Victoria withdrew completely from the first public and went back to the solitude of Balmoral. She was far more than a year completely disappeared from public view, her return to London had to be begged by the Government members really.
As for the official duties, Victoria hesitated not to comply with their obligations. She refused, however, consistently to fulfill their public duties as a representative of the United Kingdom.  She appeared on the annual opening of parliament in 40 years, widow of only seven times in person. Only the dedication of monuments Albert, she was willing to make public appearances, in 1865 she traveled to even to Coburg . Through these years of absence, the queen was increasingly coming under fire and was unpopular among the people. Newspaper editor Walter Bagehot put it this way: “From difficult to be named for reasons the Queen has done through its long retreat from public life, the popularity of the monarchy nearly as much damage as the most unworthy of its predecessors has done through his profligacy and recklessness.”  your oldest son, Albert Edward, Prince of Wales, to make her male support and to let him grow into the role that his father refused, however, from Victoria. After apprenticeship with Lord Melbourne, the journeyman years in Prince Albert and a multi-year transition period, they now had the confidence to govern as an independent constitutional monarch. 
Whenever it in the following decades, their will politically against the respective prime minister wanted to put it bluntly threatened her abdication, with the implication that its so easy to fall, because this crown a ” crown of thorns “for she was. So she sat down by almost forever. In the four decades of her widowhood, she was always so politically recorded an emotional advantage for many Britons and was a bit strange recluse in widow’s weeds, a rapt figure, awesome and an empire ruling of global dimensions.
Victoria’s sketches of her servant John Brown when he was 23 years old
A significant proportion of the mental relaxation of the widow Victoria was John Brown . This was first employed as a Scottish hunting servant of the Prince Consort at Balmoral in 1865 and became known as “the Highland servant of the Queen.” He was considered a tough, shrewd Scot who likes zusprach the alcohol and to settle outstanding Notes without regard to rank and status tended, with whom he frequently aroused the resentment in Victoria’s environment. The Queen liked him as a reliable, discreet servants, and made it her constant companions. 
Brown’s position was very controversial during his lifetime. There were rumors that Brown had been Victoria’s lover, or even secretly married to her behind closed doors they were called Mrs Brown . Victoria herself had always protested her affection for Brown and had no criticism of him. After his death in 1883, Victoria said her oldest daughter, Brown did it “does not leave 18 ½ years, a day” . Victoria dedicated the second volume of her diary entries. Victoria’s family and the court saw the influence of Brown’s very reluctant. 
Gladstone and Disraeli
Queen Victoria and Benjamin Disraeli
Ten Prime Minister initiated under Victoria’s reign, the Cabinet. Your relationship to these statesmen was very different.  was known above all their affection for the Conservative Benjamin Disraeli , later Earl of Beaconsfield, and their dislike of his political opponents, the Liberal William Gladstone . Both were on their way mark on British politics in the second half of the 19th Century, Gladstone and Disraeli in domestic politics in foreign policy especially. They were from 1868 until Disraeli’s death in 1881 alternately at the head of government. Victoria’s reign to the chagrin Gladstone and Disraeli be twice as long as these survived for 17 years. Disraeli succeeded during his first term, the “Fairy Queen” as he called Victoria, determined to retain and became her “the Melbourne Age.” He took advantage of sending their weaknesses, created an atmosphere of intimacy, showed exaggerated respect for the Queen and gave it a sense of self-affirmation. Victoria looked at last understood by a politician, and she was thrilled by his imperial policy. These and the prevailing at that time imperial self-confidence in the UK it was Disraeli possible to enforce the personal wish of the queen to another title parliament.  After the proclamation of the German Empire in 1871, her daughter Vicky has become the future Empress, the wife of her son Alfred was the daughter of the Czar, so both had priority over the other children of the queen. After Vicky’s accession to the throne would this even take precedence over her mother had. Hardly anyone looked at the UK’s title as being equal, but the queen feared for their rank.  A change in the British titles had not been enforced, but as Victoria in India was considered as empress, she wanted to at least the title officially. This idea was not new: Disraeli had been the Indian uprising of 1857 stressed the importance of all strata of the Indian people more closely to the crown.  On 1 May 1876 she was with Disraeli in Parliament for the Empress of India appointed. Victoria signed after that date with “VR & I.” (Victoria Regina et Imperatrix), which became a symbol of the heyday of British imperialism was.  The appointment of the Empress was the main motivator for Victoria’s return to public life. [ 53] In India, she met never personally developed, the queen a special affection: She tried Hindustani learn, employed Indian servants who had at Osborne an Durbar wing grow and succeed John Brown was an Indian Munshi as a private secretary.  The numerous wars – for example, the Zulu War 1879 – which took place in their rapidly expanding empire legitimized, Victoria now, unlike before, when it comes to British interests, went with civilizing sense of mission, and held it while regrettable, but necessary, while wars in Europe in civilizational reasons still perceived as objectionable. 
William Ewart Gladstone
In sharp contrast to Disraeli, Gladstone, the “People’s William” was sober and factual, CrossFit Denver pedantic, and he showed no interest in flattering the Queen. Gladstone endured the apparent reluctance of the queen, and was her same result so as faithfully – without ever having to get recognition for it -. That he committed against monarchy opponents in the subject, adopted or controversial Apanageforderungen for their children through continued classroom  Particularly unpopular he made himself at Victoria in that it is the colonial imperialism hardly supported and it even on moral values declined.  His priorities were in domestic politics, especially in the deactivation of Ireland conflict: He solved the Anglican to the state church in Ireland, improved the position of Irish tenants and saw mainly the Irish self-government as inevitable. For Victoria, who saw in Ireland, like most Brits actually only a colony, this approach was shocking, and they complained that their government does not possess the power to subjugate the country.  The queen pointed to the Prime Minister Gladstone behavior that they would not as a constitutional monarch may allow. So they held subordinate officials to to abandon their loyal attitude towards the government, and conspiring against Gladstone, together with the opposition and his own colleagues.  When Gladstone in 1894 at the age of 85 years, his farewell was as prime minister, was Victoria’s rejection of him compared with still so great that they did not even thank him for his service to the country.
The Golden Jubilee
In the spring of 1887 began the celebration of Victoria’s golden jubilee. The people saw in it no longer the sad ones, a reclusive widow who neglected their public duties as a monarch, but for Victoria, she was the mother of the country, which they showed their respect and affection. She embodied a whole country with his achievements. Victoria symbolized the Empire.  The Hauptfestlichkeiten who were scheduled for May and June, expects nearly all the princes of Europe, with the exception of the Russian Tsars, and many delegations from overseas. There were many family dinners and official banquets and pageants, the highlight was a celebration service at Westminster Abbey on 20 July dar.  for Victoria herself were the celebrations overshadowed by the worry that they are fatal to her sick son, the German Crown Prince Frederick , husband of her daughter, Vicky, made.  The marriage had both Victoria and Albert once hoped to export to the British constitutionalism in Prussia and bring about an Anglo-Prussian alliance.  is particularly troubled Victoria the view that Vicky’s eldest son Prince William (the future Wilhelm II ), which in their opinion, all the unfortunate features of Hohenzollern had inherited, an early assumption of office and a long reign seems certain. were  They doubted William’s maturity and experience sufficient for the office of emperor. Even the thought that this hated by Bismarck , they should be a private family visit in April 1888 to get to know personally, would be supported, reassured them in any way. 
The Diamond Jubilee
Queen Victoria on her 60th Kronjubiläum
In 1897 Queen Victoria celebrated her Diamond Jubilee. As 1887 was a Conservative government led by Robert Gascoyne-Cecil, 3rd Marquess of Salisbury , in power, which was determined to use the anniversary to demonstrate the greatness of the Empire.  At this time there was no man in the whole Empire, it would have been with Victoria’s popularity can get. She was at the height of their popularity. All over the world were celebrating their sixtieth instead of Jubilee. For weeks there have been countless fireworks, celebrations, parades and religious services. She was a figure on which the whole Empire paid homage. On 22 June, she went on, cheered by their subjects, by the coach in London . The road leading from Westminster to St Paul’s Cathedral and from there to the poorer districts south of the Thames . At this time the British Empire was the largest empire that had been in the world ever, the expanded and is still regarded as unassailable. But the festivities could not disguise the social problems. In London alone, two million people lived in abject poverty 
Victoria died in the arms of her grandson , Wilhelm II , German Emperor on 22 January 1901 in Osborne House on the Isle of Wight . All surviving children, except daughter Victoria, who was himself dying, were present. Victoria’s personal physician, saw to it that a collection of favorite pieces was placed in the coffin, as she had ordered in a secret directive. These included an alabaster copy of Albert’s hand, photographs and a lock of John Brown’s hair. Her desire to be buried with her bridal veil, was met. 
The funeral two weeks later, as Victoria has had it, all white. It was not like other British monarch in the St. George’s Chapel at Windsor Castle , but in the Mausoleum of Frogmore in Windsor laid to rest, she had for herself and her husband died in 1861 in the Italian Romanesque style can be built.
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Sir Francis Grant (1803-1878): Portrait of Queen Victoria , 1843. Overoptimistic picture of a youth court painter, which reflects not their physical condition
The diary entry of 16-year-old Victoria “I like to concern myself with some things. I hate doing nothing ” can be seen as programmatic for their later life. She is one of their own tightly controlled “free time” rumored. 
As talkative, lively and happy Victoria was only the immediate family, resulting in her awe-inspiring appearance increased with increasing age.  attest to your numerous letters but also her good sense of humor. So she was amused that Disraeli as “mistress” responded that both “ruler”, and “Beloved” means. 
Victoria is said to have caught most of their lives the advice of men. This may be one reason that she’s growing propaganda for “the rights of women” during her reign as “dangerous, unchristian and unnatural”  assessed that the dependence of the wife of the man based on the fact that but the man no one else would protect them. Their own position as the Queen saw her as “anomaly”. 
She was as sincere to indiscretion. With age, this was an air of honesty and openness and unpredictability of nonchalance, which may be because after Albert’s death, no more criticism of her practicing.  increased over the years, their stubbornness, that unwillingness to change their plans and to bear their inability or failure of contradiction. This was reflected both in dealing with politicians as well as in private, where she was guided mostly by their moods and feelings. In many situations it seemed insupportable, because it lacked any understanding or any vision, and she apparently was not possible to put yourself in other people’s feelings.
In political questions it will be a growing complacency rumored  apply their knowledge of history to be incomplete, but they judged most situations as sharp as their minister. 
Foreign nations judged them so often stereotyped as individuals, the Russians were in their eyes a barbarian, and the French people corrupt until the charm of Napoleon III. the low opinion of his country outweighed. Prussia and Germany over, she was a long time positive set, and later – even by the influence of her oldest daughter Vicky – she refused and Bismarck criticized her grandson, Kaiser Wilhelm II  sharp.
As it is estimated to be queen, formulated by the Swedish historian Herbert Tingsten as follows:
” vitality, sincerity, ambition, and impulsiveness were their strong points. Victoria’s intelligence is also lightning fast and lively in their opinions. Almost everything she has written, we read with great interest. One is fascinated by the way he expresses himself. But to lead astute, logical and well thought out discussions, they could not. Victoria’s obstinacy, their prejudice clouded their perspicacity. They would certainly have been a valued and respected figure, where life would have held up well, but they certainly had no history to have played if they had not come as Queen of England to the world.  ”
When Queen Victoria died, the news hit the public but not unprepared, but after she had worn for three generations, the crown was, for many of their subjects without them the UK is difficult to imagine. “Victorian” was and is a seal of approval for a “good old days”. The image of the old Victoria superimposed their early dominance and middle years, when the Queen was unpopular and sometimes threatening the continuity of the monarchy seemed threatened. 
The 19th European Century was characterized by nation-state thinking, democratic emancipation and colonization, a time of change. The industrial revolution led to radical changes in economic and social structures. Great Britain survived this process of change better than most European countries, because the British aristocracy had realized that a revolution avoidable only through fundamental reform was ready. For example, the position of the Prime Minister got a new quality. From the first servant of the crown, could dismiss the rulers at will, initially, a leader of the parliamentary majority and, finally, at least since the 1870s, he was only on the confidence of the House-dependent. Through the electoral reforms of 1832, 1867 and 1884 changed the electoral potential and hence the political spectrum. It set up a process by which the House has developed into a picture of the overall will of the people and the monarchy had to adapt to this development.  Britain became a constitutional democracy, which Queen Victoria but did not want to admit it, it was this development but not in the way. 
The framework for these policy changes were in the Victorian era traditions. These traditions, in the form of monarchy, gave the people security in an increasingly complicated world. For this purpose, and self-presentation of the Empire, the courtly rituals were always decked pompous, without this having had a modest impact on the life of the queen. The political power of the Crown Victoria’s reign was indeed shrunk considerably, but their prestige was increased significantly. The prestige of the monarchy was bound person to Victoria and she beamed again from a political potency that was not to be underestimated.  tried her life through family links to the courts of Europe, conflicts between states to be limited, according to their Death did not have this regulatory authority. Her successor, King Edward VII caused a radical departure from the Victorian Splendid isolation , the alliance of political refusal to freedom’s sake, and it came to the Entente Cordiale between Britain, France and Russia 1907th 
Queen Victoria so persistently on the politics of their country, especially on foreign policy, trying to influence, the social change they had largely unaffected. Whether Britain would have found in these six decades, this kingdom without a fundamentally different phenotype, may be doubted. Victoria has given the name of its era, but it has not significantly influenced. 
Victoria and Albert had nine children:
Franz Xaver Winterhalter: Portrait of Victoria , Crown Princess Prussia, 1867. She was the mother of Kaiser Wilhelm II
Franz Xaver Winterhalter: Prince Albert Edward in a sailor suit , 1846, Royal Collection , St James’s Palace
Victoria (“Vicky”) (born 21 November 1840; † 5 August 1901), Princess Royal
∞ 1858 Frederick III., German Emperor and King of Prussia
Albert Edward (“Bertie”) (born 9 November 1841; † 6 May 1910), Prince of Wales , Edward VII as King of Great Britain and Ireland and Emperor of India
∞ 1863 Princess Alexandra of Denmark
Alice (born 25 April 1843 † December 14, 1878)
∞ 1862 Grand Duke Louis IV of Hesse
Alfred (“Affie”  ) (born 6 August 1844 † July 31, 1900), Duke of Edinburgh and the reigning Duke of Saxe-Coburg-Gotha
∞ 1874 Grand Duchess Maria of Russia
Helena (“Lina”) (born May 25, 1846; † 6 June 1923)
∞ 1866 Prince Christian of Schleswig-Holstein-Sonderburg-Augustenburg
Louise (born March 18, 1848; † 3 December 1939)
∞ 1871 John Campbell, 9th Duke of Argyll
William Arthur (born May 1, 1850; † 16 January 1942), Duke of Connaught and Strathearn
∞ 1879 Princess Louise Margaret of Prussia
Leopold George (born 7 April 1853, † March 28, 1884), Duke of Albany
∞ 1882 Princess Helene of Waldeck and Pyrmont
Beatrice (born 14 April 1857; † 16 October 1944)
∞ 1885 Prince Henry of Battenberg
At the birth of her two youngest children to the doctor permitted Victoria John Snow , it was still very controversial with chloroform to stun, not to feel the contractions. By example, the common anesthesia in obstetrics. 
Victoria was so closely connected with her husband, that her children were quite indifferent in his lifetime. After Albert’s death meant the children certainly more for them, but an intimacy in daily contact with them, nothing could be felt.  Her relationship with Crown Prince Albert Edward was his life difficult and an ongoing disappointment. She even threw his appearance (not unlike her) before. Many sources claim that the strict upbringing of the heir, whose development is highly hindered and caused many of his later behavior. Have  The ratio of the daughters was car insurance comparison better, especially in the later years, a lot. Victoria made sure that there is always a daughter as a secretary and companion in its vicinity. Helena, Louise, and Beatrice took over this task in turn. The marriage of Beatrice, she agreed only on condition that they should also continue to live after the wedding with her. 
Her grandchildren and great grandchildren she behaved towards much more loving and forgiving, they cared intensely for example to the children of their daughter Alice died young. However, they often felt by the large number of their offspring and the personal financial burden that many of them represented, overwhelmed, as Parliament saw no reason to offspring, were far the line of succession to entertain the public. 
are children of Queen Victoria
Example: Victoria (1) and Edward VII (2)
grandchildren of Queen Victoria
identical with the first digit are siblings
Example: George V (2.2) and Princess Maud (2.5)
all others are cousins
Example 1: George V (2.2) and Wilhelm II (1.1)
Example 2: Henry of Prussia (1.3) and his wife Irene of Hesse-Darmstadt (3.3)
are great-grandson of Queen Victoria
same for first two digits are siblings
Example: Edward VIII (2.2.1) and George VI. (2.2.2)
identical with the first digit are cousins
Example: George VI. (2.2.2) and Olaf V. (Norway) (2.5.1)
all others are cousins 2.Grades
Example: Gustav Adolf of Sweden (7.1.1) and his wife Sibylla (8.2.2)
are great-great grandson of Queen Victoria
identical first three digits are siblings
Example: Elizabeth II (22.214.171.124) and Margaret (126.96.36.199)
identical for the first two digits are cousins
Example: Elizabeth II (188.8.131.52) and Michael, Prince of Kent (184.108.40.206)
identical with the first digit are cousins and second cousins
Example: Elizabeth II (220.127.116.11) and Harald V (Norway) (18.104.22.168)
all others are third-degree cousins
Example: Elizabeth II (22.214.171.124) and her husband, Philip Mountbatten (126.96.36.199)
The activated partial thromboplastin time was increased
Determination of coagulation factors VIII, IX and XI
Contrary to popular belief the bleeding time is usually normal
The prothrombin time and the rate of platelets are normal
Transmission of the disease
The hemophilia A and B are recessive and X-linked but one third of hemophiliacs correspond to a de novo mutation.
We observe that a man who bears the Xh is always affected by the disease while the woman is the bearer (but can pass it on to his descendants). It must be considered for genetic counseling .
Rare cases of female haemophilia (Xh / Xh) were identified. This could include women from the union of a hemophiliac father (Xh Y) and a surrogate mother (X Xh), but the probability of such an ancestry remains low. Cases have also been identified where the woman has only one affected X (X Xh) and manifests a severe hemophilia, however, this is due to a severe disorder of the inactivation of the X chromosome healthy 2 , 3 . Moreover women carriers can also exhibit symptoms of varying severity, and are called “carriers at low rates.”
Current treatments do not cure hemophilia but they consist of the intravenous administration of Factor VIII or Factor IX to obtain coagulant activity sufficient to stop or even prevent, bleeding.
Current therapies are either from blood plasma (plasma products, eg FACTANE (factor VIII), BETAFACT (factor IX)), or synthetic products derived from biotechnology (recombinant products) which partly (eg Kogenate, Helixate, Refacto ) or completely (eg Advate, Benefix) removed traces of blood products for their manufacturing processes and in the final product. The elimination of blood products eliminates the risk of transmission of certain diseases of the donor (AIDS, viral diseases).
The French laboratory fractionation and biotechnology develops treatments against hemophilia.
It is a disease known for thousands of years. The Hebrews of the ancient world knew already and never practiced circumcision of boys whose mother was from a family who lost a child due to haemorrhage during a circumcision.
The epidemic of AIDS has been particularly deadly for hemophiliacs. Those, who need regular transfusions, were likely to contract the virus. In France, it resulted in a political scandal, the tainted blood affair .
Other syndromes hemophilia
Other hereditary deficiencies in coagulation factor
Deficiency Factor VII . Autosomal recessive , prevalence: 1:500,000 4 .
Deficiency Factor II . Autosomal recessive.
Deficiency Factor V . Autosomal recessive prevalence: 1/1000.000.
Deficit in X Factor . Autosomal recessive prevalence: 1/1000.000 5 .
Afibrinogenemia (deficiency of fibrinogen or f actor I ). Autosomal recessive prevalence: 5/10.000.000.
Combined deficiency of Factor V and Factor VIII . Autosomal recessive and very rare, located on chromosome 18 6 .
Deficiency Factor XIII . Autosomal recessive, extremely rare 7 .
Congenital deficiency of vitamin K- 8 .
(Deficiencies of factor XII in prekallikrein or high molecular weight kininogen do not cause bleeding but a prolonged aPTT 9 .)
Acquired deficiencies in coagulation factor
Deficiency in vitamin K , vitamin K
Antibodies anti-coagulation factors 10 , 11 .
Although not considered as auto insurance quotes hemophilia , disorders of hemostasis and excessive fibrinolysis are also leading to a risk of bleeding.