ABSTRACT
INTRODUCTION: Spontaneous skin necrosis revealed acquired protein S deficiency due to isotype G autoantibodies. CASE: This 31-year-old male renal transplant recipient, receiving immunosuppressive treatment, was hospitalized for necrotic purpural lesions. We were not able to detect any triggering factor. Sustained anticoagulant therapy remained essential to prevent new skin lesions and perhaps more thrombotic events. COMMENTS: This condition is rare in adulthood, but is well described in children's purpura fulminans, especially the post-varicella form. Its mechanism remains unclear.
Subject(s)
Kidney Transplantation , Protein S Deficiency/diagnosis , Skin/pathology , Adult , Anticoagulants/therapeutic use , Autoantibodies/blood , Heparin/therapeutic use , Humans , Immunoglobulin G/immunology , Kidney Transplantation/immunology , Male , Necrosis , Protein S Deficiency/immunologyABSTRACT
BACKGROUND AND OBJECTIVES: A single dose of recombinant factor VIIa (rFVIIa) has been shown to be effective and safe in correcting the prothrombin time (PT) in cirrhotic patients, but no clinical data exists demonstrating its efficacy in arresting active bleeding. MATERIALS AND METHODS: rFVIIa was used in two cirrhotic patients for persistent bleeding following dental extractions despite repeated treatment at the wound site and, in one case, repeated administrations of fresh-frozen plasma (FFP). RESULTS: Bleeding stopped promptly in both patients after administration of rFVIIa. However, bleeding recurred in the patient who had not received concomitant treatment at the extraction sites. No recurrence of bleeding was observed in the second patient, who underwent local treatment 15 min after rFVIIa. CONCLUSIONS: Recombinant factor VIIa arrested bleeding after dental extractions in two cirrhotic patients who had been unsuccessfully treated with FFP. However, additional local treatment is needed to limit the risk of recurrence as a result of the short half-life of rFVIIa.
Subject(s)
Factor VIIa/pharmacology , Hemorrhage/drug therapy , Liver Cirrhosis/complications , Tooth Extraction/adverse effects , Adult , Factor VIIa/therapeutic use , Female , Humans , Male , Middle Aged , Prothrombin Time , Recombinant ProteinsABSTRACT
UNLABELLED: Suicide attempts are frequent during adolescence. Intentional ingestion of rat poison is not well known in France. The complications of this are prolonged and may be serious. CASE REPORT: An adolescent, 15 years old, with clinical hemorrhagic syndrome, had coagulation deficiency. Rat poison had been found in serum. The young girl recognized later that the ingestion of these toxins was intentional. CONCLUSION: Suicide attempt with rat poison is exceptional, but we have to mention it when vitamin K-dependent factors failed without any other explication.
Subject(s)
Poisoning , Rodenticides/poisoning , Suicide, Attempted , Warfarin/poisoning , Adolescent , Animals , Factor VII/analysis , Factor X/analysis , Female , Humans , Poisoning/blood , Poisoning/therapy , Prothrombin/analysis , Rats , Vitamin K 1/therapeutic useABSTRACT
The interferon (IFN) system, both serum IFN levels and the in vitro IFN production, was investigated in 38 clinically asymptomatic multitransfused hemophiliacs, half positive and half negative for HIV antibodies. In most patients, no circulating IFN was detected; similar levels of IFN-alpha were obtained after peripheral blood mononuclear cell (PBMC) stimulation with Sendai virus both in hemophiliacs and controls, while production of IFN-gamma following stimulation with phytohemagglutin (PHA) was diminished in a large number of patients irrespective of their HIV serology. These data indicate that the deficiency in IFN-gamma generation is not only related to HIV contamination but may be a direct consequence of the chronic antigenic stimulation through Factor VIII concentrates.
Subject(s)
HIV Seropositivity/immunology , Hemophilia A/immunology , Interferons/biosynthesis , Transfusion Reaction , HIV Seropositivity/complications , Hemophilia A/complications , Humans , Interferon-gamma/biosynthesis , Interferons/chemistry , Leukocytes, Mononuclear/metabolism , Reference ValuesABSTRACT
The interferon (IFN) system, both serum IFN levels and the in vitro IFN production, was investigated in 38 clinically asymptomatic multitransfused hemophiliacs, half positive and half negative for HIV antibodies. In most patients, no circulating IFN was detected; similar levels of IFN-alpha were obtained after peripheral blood mononuclear cell (PBMC) stimulation with Sendai virus both in hemophiliacs and controls, while production of IFN-gamma following stimulation with phytohemagglutin (PHA) was diminished in a large number of patients irrespective of their HIV serology. These data indicate that the deficiency in IFN-gamma generation is not only related to HIV contamination but may be a direct consequence of the chronic antigenic stimulation through Factor VIII concentrates.
Subject(s)
HIV Seropositivity/physiopathology , Hemophilia A/physiopathology , Interferons/biosynthesis , Humans , In Vitro Techniques , Interferon Type I/biosynthesis , Interferon-gamma/biosynthesis , Parainfluenza Virus 1, HumanABSTRACT
In a study of the transmissibility of human immunodeficiency virus (HIV) in haemophilic and non-haemophilic children living together in a boarding school in France, half of the haemophilic children had seroconverted by the end of a 3-year study period. By contrast none of the non-haemophilic children seroconverted. All children had had close casual contact, some of them for several years. Hepatitis B virus (HBV) markers detected in all polytransfused haemophiliacs were found in 4 of 20 control children in the school, whereas all healthy youngsters living with their families were HBV negative. This study adds support to the theory that transmissibility of HIV among casual contacts is low and that there is no reason to exclude HIV-antibody carriers from communities.
Subject(s)
Acquired Immunodeficiency Syndrome/transmission , Hemophilia A/complications , Acquired Immunodeficiency Syndrome/immunology , Acute Disease , Adolescent , Adult , Antibodies, Viral/analysis , Child , Child, Preschool , Drug Contamination , Factor IX/therapeutic use , Factor VIII/therapeutic use , France , HIV Antibodies , Hemophilia A/immunology , Hemophilia A/therapy , Hepatitis B virus/immunology , Humans , Immunoglobulin G/analysis , Infant , Infant, Newborn , Lymphocytes/classification , Male , Residence Characteristics , Retrospective StudiesABSTRACT
From 7 cases of abnormalities involving chromosome 13, the structural gene(s) coding for coagulation factors VII and X were located in the region 13q34-13qter. Gene-dosage effects for these coagulation factors seem to act in both directions, causing a decrease when there is monosomy of segment 13q34, but also, as has not been demonstrated before, an increase when there is trisomy of this same segment.
Subject(s)
Chromosome Aberrations/blood , Chromosomes, Human, 13-15 , Factor VII/genetics , Factor X/genetics , Genes , Adolescent , Child , Child, Preschool , Chromosome Aberrations/genetics , Chromosome Deletion , Chromosome Disorders , Factor VII/metabolism , Factor X/metabolism , Female , Humans , Infant , Male , Translocation, Genetic , TrisomyABSTRACT
About one homozygote female for haemophilia, authors report circumstances where we can see an haemophilic female: lyonisation, chromosomal abnormalities of X chromosome, and homozygosis. They report the cases of literature.
Subject(s)
Hemophilia A/genetics , Adult , Dosage Compensation, Genetic , Female , Homozygote , Humans , Pedigree , Sex Chromosome Aberrations/genetics , X ChromosomeABSTRACT
For laboratory control of oral anticoagulation, amidolytic factor X (F X) determination may offer an alternative to standardization difficulties of prothrombin time (PT). In order to validate this amidolytic assay on a large scale, a multicenter study was undertaken in 6 French laboratories using the same chromogenic substrate (Stachrom X Stago) and different automated instruments. Intra and between laboratory reproducibility of factor X was estimated on fresh and lyophilized patients plasmas and was found to be highly satisfactory. Standardization of the method did not seem to depend on the chromogenic substrate used, as investigated in two different centers. Results of PT and factor X were compared in over 500 patients on a long-term stabilized oral anticoagulant treatment: there was a strong positive correlation between the 2 tests in each center. The therapeutic range for factor X was evaluated from therapeutic PT values reported by Duckert and Marbet for the different thromboplastin reagents: the estimated mean range was 21 to 32%. Pooling the results of the six different centers a concordant information for prothrombin time and factor X amidolytic assay was found in 76% of patients and a fully discordant response was present in 0.6%. The results suggest that amidolytic factor X may be suitable for monitoring long-term anticoagulation. However, prospective trials are needed to evaluate its usefulness as compared to conventional methods.