PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Factor XI Deficiency”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 379 records · Page 21Linked to original sources
PubMed2004

Is there a 'Basque' profile regarding autosomal recessive deficiencies of coagulation factors?

When excluding haemophilia and von Willebrand disease, coagulation factors deficiencies constitute rare autosomal recessive disorders (<1 in 500,000) of less precisely defined epidemiology. We have reported herein the distribution of these entities in the French Basque Country, a genetic isolate of very old individualization with peculiar biological specificities. The prevalence of these disorders was markedly high, especially, as already shown, factor XI deficiency. This unusual profile needs to be discussed in the view of population genetics.

Adolescent↗
PubMed2003

Efficacy of irradiation and external hyperthermia in locally advanced, hormone-refractory or radiation recurrent prostate cancer: a preliminary report.

PURPOSE: To present a preliminary report on the feasibility, efficacy, and toxicity of irradiation (RT) and hyperthermia (HT) in patients with locally advanced, hormone-refractory prostate cancer (LAHRPC) who may or may not have received prior RT. METHODS AND MATERIALS: Between 1997 and 2002, 13 consecutive patients with LAHRPC or RT-recurrent prostate cancer were treated with RT and HT on a Phase I-II protocol. Eight patients had RT-recurrent LAHRPC (Group A) and 5 had LAHRPC without prior RT (Group B). All patients had large and clinically symptomatic tumors. The median RT dose was 39.6 Gy and 66.6 Gy in Groups A and B, respectively. External deep HT was delivered using a BSD-2000 Sigma-60 applicator. The median number of HT treatments was 8 in group A and 10 in group B. RESULTS: The median follow-up was 14 and 13 months for Groups A and B, respectively. All patients achieved a complete or partial response (CR/PR) and complete palliation of symptoms. Eleven patients had follow-up CT scans that demonstrated a CR in six and a PR in five. Two patients, who died of metastasis, did not have CT scans and had a PR on digital rectal examination. Two patients demonstrated a biochemical CR. The median duration of the CR/PR among Group A patients was 12 months after therapy. Three patients in Group A developed tumor recurrence at 9, 17, and 27 months after repeat RT to doses of 39.6, 36, and 50 Gy, respectively. At last follow-up, no Group B patient developed local recurrence. Grade 1-2 rectal bleeding was noted in 3 patients. RT and HT were generally well tolerated by all patients who had not previously undergone RT. Of the 8 patients who had, 6 (75%) tolerated retreatment well with minimal or no complications. Two patients in the repeat RT group had severe complications. One patient with lymphoma and factor XI deficiency developed Grade 4 hemorrhagic cystitis. Another previously irradiated patient developed a rectovesical fistula 4 months after retreatment, after disappearance of a large, invasive, and necrotic tumor. CONCLUSION: This preliminary report demonstrates the feasibility and efficacy of RT and HT in patients with LAHRPC, who may or may not have received prior RT. Presently, such patients who have undergone previous RT have no effective treatment options. RT and HT were generally well tolerated by patients who were not previously undergone RT. Of those who had been, most (6 of 8) tolerated retreatment well with minimal or no complications. The high-risk factors for treatment- and tumor regression-related side effects include the presence of large necrotic tumors, previous RT with a large dose/fraction, and the presence of bleeding disorders. Despite the size of these large tumors, RT and HT resulted in significant tumor shrinkage, rapid serum prostate-specific antigen decline, durable treatment responses, and durable palliation of symptoms. Additional clinical studies are warranted.

Aged↗
PubMed2006

Are hemostasis and thrombosis two sides of the same coin?

Factor XII (FXII), a clotting enzyme that can initiate coagulation in vitro, has long been considered dispensable for normal blood clotting in vivo because hereditary deficiencies in FXII are not associated with spontaneous or excessive bleeding. However, new studies show that mice lacking FXII are protected against arterial thrombosis (obstructive clot formation) and stroke. Thus, FXII could be a unique drug target that could be blocked to prevent thrombosis without the side effect of increased bleeding.

Animals↗
PubMed1980

The first cases of Fitzgerald factor deficiency in the Orient: three cases in one family.

Asymptomatic, female, 56-year-old identical Japanese twins were found to have a severe abnormality in the surface-mediated intrinsic coagulation, fibrinolysis and esterolytic activity. These defects were thought to be due to the lack of Fitzgerald factor, because of the prolongations of kaolin-activated partial thromboplastin time and kaolin-activated euglobulin lysis time that were not corrected by the addition of Fitzgerald trait-plasma but were corrected to normal levels by the addition of isolated bovine high-molecular-weight kininogen, Fletcher trait-plasma or Hageman trait-plasma.

Animals↗
PubMed1985

Noonan syndrome: a review.

After an introduction dealing with the "historical evolution" of the Noonan syndrome (NS), we try to define the NS phenotype based on clinical descriptions published since 1883. The theories concerning the cause of the NS are discussed fully. The peculiar cardiac involvement deserves special attention and raises the question of whether the Watson and LEOPARD syndromes are indistinguishable from NS. Finally, the recent contributions to the variability of the NS phenotype (reports on lymphatic dysplasia, partial deficiency of factor XI, malignant hyperthermia, perceptual-motor disabilities, and endocrine evaluation) are also described.

Factor XI Deficiency↗
PubMed1998

Anaesthesia in a parturient with Noonan's syndrome.

PURPOSE: To report the management of labour analgesia and subsequent anaesthesia for postpartum bleeding in a 19-yr-old parturient with Noonan's syndrome. CLINICAL FEATURES: The patient presented in active labour at 36-wk gestation. She was known to have Noonan's syndrome and had been assessed regularly throughout pregnancy. Features of the syndrome exhibited by the patient included typical facies, chest skeletal abnormalities, pulmonary valve dysplasia, mental retardation and lymphoedema. In addition, she had Factor XI deficiency (0.46 mg.L-1) and thrombocytopenia (92 x 10(9).L-1), previously unreported in a parturient with this syndrome. Although epidural analgesia may have been considered the labour analgesic technique of choice, the risk of epidural haematoma caused by her bleeding diathesis made this unacceptable. This risk was balanced against the possibility of a potentially difficult intubation due to facial abnormalities, should emergency operative delivery become necessary. Labour analgesia was provided with intravenous patient controlled opioid analgesia (fentanyl 25 micrograms bolus, five minute lockout) despite her mental retardation. Dilatation and curettage required general anaesthesia after intubation with awake direct laryngoscopy using cautious sedation. CONCLUSION: Noonan's syndrome is characterised by multi-system involvement, requiring thorough preoperative assessment of cardiovascular, skeletal, haematological and central nervous systems. Clotting and platelet defects considerably restrict the possible analgesic and anaesthetic options for labouring patients with this syndrome.

Adult↗
PubMed1982

Tissue factor-like activity of the human monocytic tumor cell line U937.

Undisrupted cells of the human monocytic tumor cell line U937 have procoagulant activity that is Ca2+ dependent and is not demonstrable in Factor VII or Factor X deficient plasma. Furthermore, U937 cells when incubated with purified human Factor VII in the presence of Ca2+ and then repeatedly washed promoted coagulation of Factor VII deficient plasma in the absence of added tissue factor. Culture with endotoxin increased the procoagulant activity of U937 cells approximately 5-fold. In separate experiments, exposure to lymphokines obtained from phytohemagglutinin-stimulated lymphocytes enhanced the procoagulant activity of U937 cells 4 to 110-fold. Other cell lines (of myeloid and lymphoid origin) tested lacked the procoagulant activity found in U937 cells. These results indicate that the constitutive tissue factor-like activity of U937 cells resembles that of normal activated human monocytes.

Binding Sites↗