1/6. The role of primary prophylactic factor replacement therapy in children with severe factor x deficiency.Severe factor X (FX) deficiency is one of the severest inherited coagulation disorders. Clinical manifestations include umbilical cord, mucosal, joint and central nervous system bleeding. Four Irish children with severe FX deficiency presented with umbilical cord bleeding. One developed an intraperitoneal haemorrhage and another an intracranial bleed. Prophylaxis, using intermediate purity factor ix concentrate, was commenced within the first month of life, necessitating the insertion of central venous access devices in two of the children. All children have normal joint function, suggesting that prophylaxis commenced early in life reduces the incidence of arthropathy and improves quality of life.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
2/6. Spontaneous major bleeding in acquired factor x deficiency secondary to AL-amyloidosis.Abnormal bleeding may be seen in up to a third of patients with amyloidosis. factor x deficiency is the commonest acquired coagulopathy in amyloidosis. While mild intracutaneous bleeding is common, spontaneous life-threatening haemorrhage is rare. We report a case of acquired FX deficiency due to amyloid light chain (AL)-amyloidosis presenting with spontaneous retroperitoneal bleeding. The diagnostic and management issues in this patient are discussed.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
3/6. Hereditary deficiency of all vitamin k-dependent procoagulants and anticoagulants.Hereditary combined deficiency of vitamin k-dependent factors is a rare entity. We report a 7-year-old girl of Arab origin with hereditary deficiency of the procoagulants factors II, VII, IX and X and the natural anticoagulants proteins C and S. The patient is the tenth offspring of a consanguinous marriage and presented at 6 weeks with spontaneous intracerebral haemorrhage. Symptoms improved following plasma infusion. A sibling died at 5 d from uncontrollable umbilical bleeding. blood coagulation work-up at 6 years showed: factor II:C (activity) 12 U/dl, factor II:Ag (antigen) 40 U/dl; factor vii:C 12 U/dl; factor IX:C 36 U/dl, factor ix:Ag 57 U/dl; factor X:C 17 U/dl, factor X:Ag 54 U/dl; protein c activity 43 U/dl; protein c:Ag 45 U/dl; protein s:Ag 34 U/dl; levels of factors V:C and VIII:C were normal. Assays of coagulation factors in the parents and five of the siblings were within the normal range. Following acute infection and dilantin therapy procoagulant activity levels were reduced further and were partially increased after vitamin k infusion. Crossed immunoelectrophoresis of prothrombin in the presence of calcium lactate revealed a population of des-carboxyprothrombin. serum vitamin k epoxide levels were undetectable. The data suggest that the defect in our patient stems from abnormal carboxylation of the vitamin k-dependent proteins and that the mode of inheritance is autosomal recessive.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
4/6. Antenatally diagnosed subdural haemorrhage in congenital factor x deficiency.The presence of a subdural haemorrhage was observed in a fetus during antenatal ultrasound examination. The infant was found to be a homozygote for factor x deficiency. Prompt recognition permitted replacement treatment from an early stage. Inherited coagulation disorders should be suspected when intracranial haemorrhage is detected antenatally.- - - - - - - - - - ranking = 13.478160248447keywords = intracranial haemorrhage, haemorrhage (Clic here for more details about this article) |
5/6. Severe congenital factor x deficiency with intracranial haemorrhage.A Saudi Arabian infant with severe factor x deficiency who had had two intracranial haemorrhages is described. Attempts to raise his factor X level and improve his prothrombin time (PT) and partial thromboplastin time (PTT) by using vitamin k, oestradiol and danazol have failed. New therapeutic trials are necessary for patients with severe forms of this rare disorder.- - - - - - - - - - ranking = 42.390801242236keywords = intracranial haemorrhage, haemorrhage (Clic here for more details about this article) |
6/6. factor x deficiency in the neonatal period.An infant with a severe deficiency of factor X presened in the neonatal period with uncontrollable bleeding from heel prick sites, spontaneous bruising, and haematoma. The deficiency was controlled by infusions of dried human factors II, IX, and X concentrate; the half-life of the infused factor X material is only 18 hours. Despite prophylactic weekly infusions of factor X concentrate, the child developed a fatal intracerebral haemorrhage when only 4 months old. Coagulation studies on both parents and the elder sister showed no obvious coagulation abnormality.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |