Hereditary factor X deficiency( stuart-parasite disease)
Jun 20, 2018
Stewart-Prower's disease is a rare disease that is transmitted through an incomplete autosomal recessive type of inheritance.
Both very heavy forms and light and hidden varieties are possible. With very severe form of the disease, bleeding occurs in early childhood, often at birth or in the first months of life, and usually quickly lead to death. The immediate cause of death is hemorrhage under the periosteum of the bones of the skull, repeated hemorrhages in the brain and massive gastrointestinal bleeding. In severe forms of the disease, bleeding occurs somewhat later and does not lead to death. Often there are hemorrhages in the skin, subcutaneous hematomas, abundant and prolonged nasal and gingival hemorrhages and especially severe uterine and menstrual bleeding. Surgical interventions( from tooth extraction to abdominal operations), childbirth and abortion are accompanied by massive, life-threatening bleeding. Intramuscular hemorrhages and hemorrhages in the joints are extremely rare. In case of a moderate disease, the most common uterine, nasal and gingival hemorrhages, hemorrhages in the skin, bleeding during injuries and operations. The mild form does not give such pronounced manifestations, but still there are frequent uterine bleeding, bleeding during labor and surgical interventions. Periodically, there are "causeless" nosebleeds. The mild illness has long light intervals without any signs of increased bleeding.
Treatment of Stewart-Prower's disease is carried out with the same blood products as replacement therapy for factor VI deficiency of blood clotting.
When uterine bleeding is sometimes used, synthetic hormonal contraceptives( infekundin, mestranol, etc.) for 3-4 tablets in the first 2-3 days of the menstrual cycle, followed by a decrease in dose to 1 tablet a day. When using these drugs, blood control is necessary.
Locally with nosebleeds, bleeding from the dental holes, use thrombin, a hemostatic sponge, a chilled 5-10% solution of e-aminocaproic acid, and other topical agents that promote blood clotting.