Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 86
Filtrar
1.
Vox Sang ; 119(3): 193-202, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38018260

RESUMEN

BACKGROUND AND OBJECTIVES: Deficiencies of protein C (PC) or protein S (PS) are rare diseases, characterized by mutations in the PC or PS genes, which encode plasma serine proteases with anti-coagulant activity. Severe PC or PS deficiencies manifest in early life as neonatal purpura fulminans, a life-threatening heamorrhagic condition requiring immediate treatment. First-line treatment involves replacement therapy, followed by maintenance with anti-coagulants. Replacement therapy with specific protein concentrates is currently only limited to PC, and therefore, a PC + PS concentrate represents a useful addition to therapeutic options, particularly for severe PS deficiency. Further, the production of a PC + PS concentrate from unused plasma fractionation intermediates would impact favourably on manufacturing costs, and consequently therapy prices for patients and health systems. MATERIALS AND METHODS: Several chromatographic runs were performed on the same unused plasma fractionation intermediates using different supports to obtain a PC/PS concentrate. The best chromatographic mediums were chosen, in terms of specific activity and recovery. A full process of purification including virus inactivation/removal and lyophilization steps was set up. RESULTS: The final freeze-dried product had a mean PC concentration of 47.75 IU/mL with 11% of PS, and a mean specific activity of 202.5 IU/mg protein, corresponding to over 12,000-fold purification from plasma. CONCLUSION: The development of a novel concentrated PC/PS mixture obtained from a waste fraction of other commercial products could be used for its potential therapeutic role in the management of neonatal purpura fulminans pathology.


Asunto(s)
Deficiencia de Proteína C , Púrpura Fulminante , Recién Nacido , Humanos , Púrpura Fulminante/tratamiento farmacológico , Púrpura Fulminante/genética , Deficiencia de Proteína C/tratamiento farmacológico , Proteína C/análisis , Proteína C/uso terapéutico , Proteína S , Plasma/química
2.
Genes (Basel) ; 13(5)2022 04 22.
Artículo en Inglés | MEDLINE | ID: mdl-35627118

RESUMEN

Objectives: Protein C (PC) deficiency is an inherited thrombophilia with a prevalence of 0.5% in the general population and 3% in subjects with a first-time deep vein thrombosis (DVT). Here we report a series of 14 PC-deficient Polish patients with comprehensive clinical and molecular characteristics, including long-term follow-up data and a deep mutational analysis of the PROC gene. Patients and Methods: Fourteen unrelated probands (mean ± SD age 43.8 ± 13.0 years) with suspicion of PC deficiency, who experienced thromboembolic events and a majority of whom received anticoagulants (92.8%), were screened for PROC mutations by sequencing the nine PROC exons and their flanking intron regions. Results: Ten probands (71.4%) had missense mutations, two patients (14.3%) carried nonsense variants, and the other two subjects (14.3%) had splice-site mutations, the latter including the c.401-1G>A variant, reported here for the very first time. The proband carrying the c.401-1A allele had a hepatic artery aneurysm with a highly positive family history of aneurysms and the absence of any mutations known to predispose to this vascular anomaly. Conclusion: A novel detrimental PROC mutation was identified in a family with aneurysms, which might suggest yet unclear links of thrombophilia to vascular anomalies, including aneurysms at atypical locations in women. The present case series also supports data indicating that novel oral anticoagulants (NOACs) are effective in PC deficient patients.


Asunto(s)
Aneurisma , Deficiencia de Proteína C , Trombofilia , Trombosis , Administración Oral , Adulto , Aneurisma/tratamiento farmacológico , Anticoagulantes/uso terapéutico , Femenino , Humanos , Persona de Mediana Edad , Mutación , Polonia , Proteína C/genética , Proteína C/metabolismo , Proteína C/uso terapéutico , Deficiencia de Proteína C/tratamiento farmacológico , Deficiencia de Proteína C/genética , Trombosis/genética
3.
Pediatr Blood Cancer ; 69(1): e29380, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34665512

RESUMEN

Perioperative management of severe congenital protein C deficiency remains unestablished. This deficiency is often treated with anticoagulants, such as warfarin. Although anticoagulants need to be perioperatively discontinued, there are few methods for the management of such patients. We adopted a method for administering prothrombin complex concentrates (PCC), which includes intermittent administration of inactive protein C (PPSB-HT), and examined its outcome as a perioperative management approach for severe congenital protein C deficiency. Three patients underwent our perioperative management six times. We monitored activity levels of protein C, factor IX, and so forth. These patients could be perioperatively managed with PCC treatment.


Asunto(s)
Deficiencia de Proteína C , Anticoagulantes , Factores de Coagulación Sanguínea , Humanos , Proteína C , Deficiencia de Proteína C/tratamiento farmacológico , Protrombina
4.
J Stroke Cerebrovasc Dis ; 30(1): 105320, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33131982

RESUMEN

We herein report a case involving a 32-year-old Japanese man with recurrent cerebral venous thrombosis due to hereditary protein C deficiency. He was admitted to our hospital with impaired consciousness. Brain magnetic resonance imaging demonstrated high intensities diffusely along the bilateral sulci and magnetic resonance venography revealed left transverse sinus and superior sagittal sinus stenoses. His father had a history of cerebral infarction and venous thrombosis. The protein C activity level examined by chromogenic synthetic substrate assay was markedly reduced. He was diagnosed with protein C deficiency, and a genetic analysis revealed a heterozygous mutation at exon 3 c.199G>A,p.Glu67Lys on the protein C gene. Four months later, at his second admission, he had transient aphasia, and his protein C activity was under 10%. We switched warfarin to the direct oral anticoagulants edoxaban. He remains fully recovered with no adverse events after the administration of edoxaban for a year. Direct oral anticoagulants may be a new tool for treating cerebral venous thrombosis due to hereditary protein C deficiency.


Asunto(s)
Inhibidores del Factor Xa/administración & dosificación , Trombosis Intracraneal/prevención & control , Mutación , Proteína C/genética , Piridinas/administración & dosificación , Tiazoles/administración & dosificación , Trombosis de la Vena/prevención & control , Administración Oral , Adulto , Sustitución de Medicamentos , Heterocigoto , Humanos , Trombosis Intracraneal/diagnóstico por imagen , Trombosis Intracraneal/etiología , Masculino , Deficiencia de Proteína C/complicaciones , Deficiencia de Proteína C/diagnóstico , Deficiencia de Proteína C/tratamiento farmacológico , Deficiencia de Proteína C/genética , Recurrencia , Resultado del Tratamiento , Trombosis de la Vena/diagnóstico por imagen , Trombosis de la Vena/etiología
5.
Int J Hematol ; 109(6): 650-656, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30963470

RESUMEN

Patients with severe congenital protein (P)C deficiency require long-term anticoagulant management. Recombinant PC concentrates for prophylactic use are not available in Japan; prothrombin complex concentrates (PCC), containing factors (F)II, VII, IX, X, and PC (PPSB-HT®), have been used 'off-label' in a few patients. We investigated the combined use of prophylactic PCC and Warfarin (VKA; PT-INR 2.0-2.5) in a severely PC-deficient patient in whom VKA alone did not prevent recurrent purpura. Plasma VKA-dependent factor levels and global PC function (Thrombopath®) were assessed. Plasma activity levels of FII/FVII/FIX/FX post-infusion of PCC (6.3 unit/kg) increased 35/27/27/35 (initial level) to 59/60/38/83 IU/dl, respectively. FVII:C and FIX:C rapidly returned to baseline levels 12-24 h post-infusion, but FII:C and FX:C returned more slowly. PC antigen (< 5%) increased to ~ 15%, followed by return to baseline levels 24 h post-infusion. Global PC function was very low (%PiCi 24%), but improved post-PCC infusion. This potential was slightly detectable even at an undetectable PC level. At day 3, high levels of D-dimer and FDP were observed without thrombotic event, but these improved post-infusion. Although PCC restored VKA-dependent coagulation factors, PC contained in PCC significantly improved global anticoagulation, and was clinically beneficial in this severely deficient patient.


Asunto(s)
Anticoagulantes/administración & dosificación , Factores de Coagulación Sanguínea/administración & dosificación , Hemostasis , Deficiencia de Proteína C/sangre , Deficiencia de Proteína C/tratamiento farmacológico , Warfarina/administración & dosificación , Adulto , Quimioterapia Combinada , Humanos , Masculino , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Vitamina K Epóxido Reductasas/sangre , Adulto Joven
6.
Pediatr Blood Cancer ; 66(6): e27686, 2019 06.
Artículo en Inglés | MEDLINE | ID: mdl-30835920

RESUMEN

A male patient diagnosed with severe congenital protein C (PC) deficiency during the neonatal period was treated with long-term warfarin but frequently developed purpura fulminans and bleeding. At four years of age, edoxaban was initiated (direct oral anticoagulant [DOAC]). His d-dimer and fibrin/fibrinogen degradation product levels were closely monitored. His PC activity increased from below the sensitivity range to 17%; this increase was thought to be due to a reduction in PC consumption during edoxaban therapy. After edoxaban introduction, he experienced just one episode of purpura fulminans over two years without any adverse events. Thus, DOAC may be a promising alternative for the management of congenital PC deficiency.


Asunto(s)
Inhibidores del Factor Xa/uso terapéutico , Hemorragia/prevención & control , Deficiencia de Proteína C/tratamiento farmacológico , Púrpura Fulminante/prevención & control , Piridinas/uso terapéutico , Tiazoles/uso terapéutico , Preescolar , Manejo de la Enfermedad , Humanos , Masculino , Pronóstico , Deficiencia de Proteína C/patología
9.
Arch Pediatr ; 24(4): 363-366, 2017 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-28259509

RESUMEN

Neonatal severe protein C deficiency is a serious disease. There is no uniform approach for long-term preventive treatment of thrombotic events. We report the case of neonatal severe protein C deficiency treated with warfarin oral suspension. An international normalized ratio (INR) from 2.5 to 3.5 was expected. The INR was measured by home monitoring using the Coaguchek XS® (Roche Diagnostics, Mannheim, Germany) monitor. During 2years of warfarin treatment, there were only two minor episodes of purpuric access and no bleeding was reported. This case suggests that the early introduction of warfarin oral suspension, home-care monitoring, and parental education programs may be a beneficial treatment option for children with protein C deficiency.


Asunto(s)
Intervención Médica Temprana , Fibrinolíticos/uso terapéutico , Deficiencia de Proteína C/tratamiento farmacológico , Warfarina/uso terapéutico , Administración Oral , Cateterismo Venoso Central , Preescolar , Consanguinidad , Diagnóstico Precoz , Femenino , Estudios de Seguimiento , Servicios de Atención a Domicilio Provisto por Hospital , Humanos , Lactante , Recién Nacido , Relación Normalizada Internacional , Proteína C/administración & dosificación , Deficiencia de Proteína C/genética
10.
Adv Exp Med Biol ; 923: 15-21, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27526119

RESUMEN

It is imperative to maintain normal blood flow to provide adequate oxygen supply to specific organs and cells, as well as for the removal of metabolic byproducts. Therefore, any situation that results in blood clotting can injure or kill living tissues. In this paper, we describe a case where a protein C deficient subject who would, by all medical indicators, be at 100 % risk of experiencing thrombophlebitis, deep vein thrombosis, and or lung emboli, is able to escape all pathologies by using perioperative zymogen protein C (ZPC). This protein C deficient patient has a long history of blood clotting, particularly from surgical procedures. The patient is 81 years old and first experienced clotting due to hernia surgery in 1964, when he was hospitalized for 16 days post-surgery with life threatening complications. It was later determined in 1980, after many episodes, that the patient had hereditary protein C deficiency at the 38 % level. In his hernia surgery, perioperative ZPC was used along with accepted anticoagulation procedures with no blood clots or other related side effects occurring. This procedure can greatly benefit protein C deficient patients, and could potentially find use for non-PC deficient patients in surgeries and a variety of other medical treatments. This particular case helps to validate the importance of ZPC in effecting safer surgery in high-risk patients. It also supports the mechanism of ZPC acting as an anticoagulant without causing bleeding. Most importantly, each clinical case study represents a unique combination of surgeon, hematologist, medical staff, and patient functioning as a coordinated team. In this case, smaller amounts of very expensive ZPC achieved safe and efficacious results, which is hugely important for future clinical applications when considering the production cost of ZPC. More studies must be done to establish minimum dosing while achieving safe and efficacious outcomes.


Asunto(s)
Anticoagulantes/administración & dosificación , Coagulación Sanguínea/efectos de los fármacos , Precursores Enzimáticos/administración & dosificación , Hernia Inguinal/cirugía , Herniorrafia , Deficiencia de Proteína C/tratamiento farmacológico , Proteína C/administración & dosificación , Trombosis de la Vena/prevención & control , Warfarina/administración & dosificación , Anciano de 80 o más Años , Anticoagulantes/efectos adversos , Anticoagulantes/economía , Pruebas de Coagulación Sanguínea , Análisis Costo-Beneficio , Costos de los Medicamentos , Sustitución de Medicamentos , Precursores Enzimáticos/efectos adversos , Precursores Enzimáticos/economía , Herniorrafia/efectos adversos , Humanos , Masculino , Seguridad del Paciente , Proteína C/efectos adversos , Proteína C/economía , Deficiencia de Proteína C/sangre , Deficiencia de Proteína C/diagnóstico , Deficiencia de Proteína C/economía , Recurrencia , Medición de Riesgo , Factores de Riesgo , Resultado del Tratamiento , Trombosis de la Vena/sangre , Trombosis de la Vena/economía , Trombosis de la Vena/etiología , Warfarina/efectos adversos
11.
Pediatr Blood Cancer ; 63(8): 1488-90, 2016 08.
Artículo en Inglés | MEDLINE | ID: mdl-27138381

RESUMEN

Subcutaneous (SC) protein C (PC) was used in a child with purpura fulminans secondary to severe congenital PC deficiency. For maintenance, PC 80-120 IU/kg, given over 60-90 min SC Q48hr, has been successful as a home therapy for more than 3 years. The treatment was monitored by measuring trough PC chromogenic activity (target ≥15%) and D-dimer levels. No change in clinical course was appreciated after discontinuing enoxaparin (and leaving the patient on prophylactic PC replacement alone). A significant discrepancy between clotting-based and chromogenic-based PC activity is shown.


Asunto(s)
Deficiencia de Proteína C/tratamiento farmacológico , Deficiencia de Proteína C/patología , Proteína C/genética , Proteína C/uso terapéutico , Anticoagulantes/uso terapéutico , Enoxaparina/uso terapéutico , Femenino , Humanos , Recién Nacido , Trasplante de Hígado , Proteína C/administración & dosificación
12.
Thromb Haemost ; 116(1): 58-68, 2016 07 04.
Artículo en Inglés | MEDLINE | ID: mdl-27052576

RESUMEN

Severe congenital protein C (PC) deficiency (SCPCD) is associated with disseminated intravascular coagulation (DIC), purpura fulminans (PF), and vascular thromboembolic events (TE), often leading to organ failure and death. PC replacement therapy offers a safe, effective treatment for thromboembolic complications of SCPCD and secondary prophylaxis for recurrent DIC, PF, and TEs. A prospective, multi-centre, open-label, phase 2/3 study was conducted to demonstrate the safety and efficacy of protein C concentrate for treatment of PF and acute TEs. Fifteen enrolled patients with SCPCD received protein C concentrate; 11 received treatment for acute TEs (PF, 18 events; PF and other coumarin-related vascular thromboembolic events [coumarin-induced skin necrosis; CISN], 1 event; venous thrombosis, 5 events). Pre-defined efficacy criteria for treatment of acute TEs were compared with a historical control arm (i. e. patients receiving conventional therapy without protein C replacement). PF/CISN was demonstrated by pre-defined primary and secondary efficacy ratings. Primary ratings of protein C concentrate-treated episodes were significantly higher (p=0.0032) than in the historical control. For 19 PF/CISN episodes in 11 patients, 94.7 % of treatments were rated effective and 5.3 % effective with complications (not related to protein C concentrate). In a secondary efficacy rating, all treatments were rated effective (68.4 % excellent; 21.1 % good; 10.5 % fair). For 5/24 vascular thrombosis episodes, 80 % of treatments were rated excellent and 20 % were rated good. No treatment-related adverse events or serious adverse events occurred. In conclusion, protein C concentrate provides an efficacious, safe treatment for PF, CISN, and other TEs in SCPCD patients.


Asunto(s)
Deficiencia de Proteína C/tratamiento farmacológico , Proteína C/uso terapéutico , Púrpura Fulminante/tratamiento farmacológico , Tromboembolia/tratamiento farmacológico , Adolescente , Adulto , Niño , Preescolar , Coagulación Intravascular Diseminada/tratamiento farmacológico , Coagulación Intravascular Diseminada/etiología , Coagulación Intravascular Diseminada/prevención & control , Femenino , Humanos , Recién Nacido , Masculino , Estudios Prospectivos , Proteína C/efectos adversos , Proteína C/farmacocinética , Deficiencia de Proteína C/complicaciones , Deficiencia de Proteína C/congénito , Púrpura Fulminante/etiología , Púrpura Fulminante/prevención & control , Prevención Secundaria , Tromboembolia/etiología , Tromboembolia/prevención & control , Resultado del Tratamiento , Adulto Joven
13.
World J Gastroenterol ; 21(3): 1024-7, 2015 Jan 21.
Artículo en Inglés | MEDLINE | ID: mdl-25624741

RESUMEN

Obscure gastrointestinal bleeding is an uncommonly encountered and difficult-to-treat clinical problem in gastroenterology, but advancements in endoscopic and radiologic imaging modalities allow for greater accuracy in diagnosing obscure gastrointestinal bleeding. Ectopic varices account for less than 5% of all variceal bleeding cases, and jejunal variceal bleeding due to extrahepatic portal hypertension is rare. We present a 47-year-old man suffering from obscure gastrointestinal bleeding. Computed tomography of the abdomen revealed multiple vascular tufts around the proximal jejunum but no evidence of cirrhosis, and a visible hypodense filling defect suggestive of thrombus was visible in the superior mesenteric vein. Enteroscopy revealed several serpiginous varices in the proximal jejunum. Serologic data disclosed protein C deficiency (33.6%). The patient was successfully treated by therapeutic balloon-assisted enteroscopy and long-term anticoagulant therapy, which is normally contraindicated in patients with gastrointestinal bleeding. Diagnostic modalities for obscure gastrointestinal bleeding, such as capsule endoscopy, computed tomography enterography, magnetic resonance enterography, and enteroscopy, were also reviewed in this article.


Asunto(s)
Anticoagulantes/uso terapéutico , Hemorragia Gastrointestinal/cirugía , Hemostasis Endoscópica , Yeyuno/irrigación sanguínea , Deficiencia de Proteína C/tratamiento farmacológico , Várices/cirugía , Hemorragia Gastrointestinal/sangre , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiología , Humanos , Masculino , Oclusión Vascular Mesentérica/tratamiento farmacológico , Oclusión Vascular Mesentérica/etiología , Venas Mesentéricas/diagnóstico por imagen , Persona de Mediana Edad , Flebografía/métodos , Deficiencia de Proteína C/sangre , Deficiencia de Proteína C/complicaciones , Deficiencia de Proteína C/diagnóstico , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Várices/sangre , Várices/diagnóstico , Várices/etiología , Trombosis de la Vena/tratamiento farmacológico , Trombosis de la Vena/etiología
14.
Dtsch Med Wochenschr ; 139(50): 2597-601, 2014 Dec.
Artículo en Alemán | MEDLINE | ID: mdl-25469695

RESUMEN

HISTORY AND CLINICAL FINDINGS: A 51-year-old female patient with history of longterm drug abuse, was admitted to our hospital with large, stocking-shaped areas of painful, non-displaceable confluent bruising reaching up to the groin. INVESTIGATIONS: The emergency laboratory tests showed leucopenia, thrombocytopenia and anemia as well as a distinct protein C deficiency. DIAGNOSIS, TREATMENT AND COURSE: Purpura fulminans was diagnosed and treated with an initial dose of protein C. The patient survived and the skin necrosis can be treated. CONCLUSION: Purpura fulminans is an internistic and dermatological emergency situation which can lead to shock through consumptive coagulopathy. The serious course of disease can be prevented by rapid treatment with protein C.


Asunto(s)
Urgencias Médicas , Deficiencia de Proteína C/diagnóstico , Púrpura Fulminante/diagnóstico , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana Edad , Pronóstico , Proteína C/administración & dosificación , Deficiencia de Proteína C/sangre , Deficiencia de Proteína C/tratamiento farmacológico , Púrpura Fulminante/sangre , Púrpura Fulminante/tratamiento farmacológico , Trastornos Relacionados con Sustancias/complicaciones
15.
Blood Coagul Fibrinolysis ; 25(5): 522-6, 2014 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-24509341

RESUMEN

We describe the case of a newborn presenting with multicystic encephalomalacy, hydrocephalus and bilateral hemovitreous. An underlying coagulation disorder was suspected and laboratory tests revealed severe protein C deficiency. At 25 days of life, after the appearance of purpura fulminans, replacement therapy with intravenous protein C concentrate (Ceprotin; Baxter, Vienna, Austria) was started.Due to difficulties in getting peripheral venous access and to repeated loss of the venous access, continuous subcutaneous infusion of protein C was started with an insulin pump (VEO 754; Medtronic, Minneapolis, Minnesota, USA), normally adopted in patients with type 1 diabetes mellitus. Protein C values increased into the normal range and the resolution of the purpuric skin lesion was achieved. Chronic prophylaxis with low-molecular-weight heparin failed and, due to cutaneous and cerebral recrudescence, replacement therapy with the pump was started again. The insulin pump allowed us to reduce the number of injections per day and to deal with the difficulties in getting peripheral venous access, permitting medical and paramedical staff an easier management of the therapy. The dosing schedule could be easily adapted with the insulin pump and the continuous subcutaneous administration of small amounts of protein C concentrate prevented fluctuation in trough levels of protein C. This is the first reported case of a novel, successful use of an insulin pump in an extremely rare disease, to administer a drug different from insulin, which needs to be further analyzed, underlining the importance of a multidisciplinary team approach in order to provide effective and efficient care in high-complexity diseases.


Asunto(s)
Deficiencia de Proteína C/tratamiento farmacológico , Proteína C/uso terapéutico , Femenino , Humanos , Recién Nacido , Infusiones Subcutáneas , Sistemas de Infusión de Insulina , Proteína C/administración & dosificación
16.
Br J Haematol ; 164(3): 414-21, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24422725

RESUMEN

Since the first description of subcutaneous protein C concentrate as treatment for severe protein C deficiency in 1996, further cases have been reported but there is no uniform approach to this form of treatment. In order to assess the safety and effectiveness of subcutaneous protein C concentrate and suggest recommendations for future use, patients who had received subcutaneous protein C concentrate were identified from the literature, by contacting the manufacturers and by personal communication. Treatment details were available from 14 cases. Apart from one case where the infusion interval was inadvertently increased, no thrombotic events occurred even when doses were subsequently reduced. Initially, a trough protein C level of >0·25 iu/ml should be aimed for. Subsequently, a smaller dose of subcutaneous protein C concentrate, especially if taken with an oral anticoagulant, may be protective maintenance treatment. The treatment was well tolerated with few side effects. Subcutaneous protein C concentrate on its own or combined with an oral anticoagulant appears to be safe and effective as maintenance treatment of severe protein C deficiency. A major advantage is the avoidance of central venous access devices. The incidence of neurodevelopmental handicap was high with blindness affecting the majority of patients.


Asunto(s)
Deficiencia de Proteína C/tratamiento farmacológico , Proteína C/administración & dosificación , Adolescente , Adulto , Anciano , Niño , Preescolar , Femenino , Humanos , Lactante , Infusiones Subcutáneas , Masculino , Persona de Mediana Edad , Proteína C/efectos adversos , Proteína C/metabolismo , Deficiencia de Proteína C/sangre , Resultado del Tratamiento , Adulto Joven
17.
J Mal Vasc ; 39(3): 203-6, 2014 May.
Artículo en Francés | MEDLINE | ID: mdl-24412009

RESUMEN

Thrombotic events occurring in the course of celiac disease are frequently reported in the literature. The localization is often unusual, mainly affecting the hepatic veins. To our knowledge, this is the first report of intracardiac thrombosis occurring in a patient with celiac disease. A 32-year-old patient with celiac disease adhered poorly to his gluten-free diet. He suffered an ischemic stroke revealing an intracardiac thrombus, which, on radiological imaging, simulated a multiple myxoma. Histological examination of the resected tumor enabled the correct diagnosis. Biological findings revealed severe protein C and S deficiency. The patient improved with anticoagulant therapy and gluten-free diet.


Asunto(s)
Enfermedad Celíaca/complicaciones , Cardiopatías/etiología , Neoplasias Cardíacas/complicaciones , Mixoma/complicaciones , Neoplasias Primarias Múltiples/complicaciones , Deficiencia de Proteína C/complicaciones , Deficiencia de Proteína S/complicaciones , Trombosis/etiología , Adulto , Anticoagulantes/uso terapéutico , Enfermedad Celíaca/dietoterapia , Diabetes Mellitus Tipo 1/complicaciones , Dieta Sin Gluten , Cardiopatías/diagnóstico , Cardiopatías/tratamiento farmacológico , Neoplasias Cardíacas/diagnóstico , Hemangioma Cavernoso/complicaciones , Humanos , Hallazgos Incidentales , Neoplasias Hepáticas/complicaciones , Imagen por Resonancia Cinemagnética , Masculino , Mixoma/diagnóstico , Neoplasias Primarias Múltiples/diagnóstico , Deficiencia de Proteína C/diagnóstico , Deficiencia de Proteína C/tratamiento farmacológico , Deficiencia de Proteína S/diagnóstico , Deficiencia de Proteína S/tratamiento farmacológico , Infarto del Bazo/etiología , Trombosis/diagnóstico , Trombosis/tratamiento farmacológico , Trombosis de la Vena/etiología
19.
Ann Thorac Cardiovasc Surg ; 20 Suppl: 885-9, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-23903711

RESUMEN

The patient was a 41-year-old female with chronic thromboembolism. She was admitted to an affiliated hospital with exertional dyspnea, leg swelling, and hemoptysis, and she was treated medically with tissue plasminogen activator and warfarin therapy. When transferred to our hospital, she was oxygen-dependent with severe dyspnea. A pulmonary arteriogram showed occlusion and stenosis of the pulmonary arteries. Cardiac catheterization revealed marked pulmonary hypertension. The lung perfusion scintigram showedmultiple defects in the right and left lungs. Preoperative laboratory data showed a markedly decreased protein C antigen level. Magnetic resonance angiography showed that a myoma uteri compressed the pelvic vein and that she had deep vein occlusion of the left leg. After the administration of an epoprostenol infusion and the insertion of an inferior vena cava filter, she underwent an operation. Under deep hypothermia, the bilateral pulmonary artery was opened and an endarterectomy was performed during intermittent circulatory arrest. After surgery, her pulmonary vascular resistance was in the normal range. Her New York Heart Association functional classification changed from class IV to class I. She has been in good condition for 7 years since the surgery.


Asunto(s)
Endarterectomía , Deficiencia de Proteína C/complicaciones , Arteria Pulmonar/cirugía , Embolia Pulmonar/cirugía , Adulto , Anticoagulantes/uso terapéutico , Enfermedad Crónica , Femenino , Humanos , Angiografía por Resonancia Magnética , Imagen de Perfusión , Deficiencia de Proteína C/sangre , Deficiencia de Proteína C/diagnóstico , Deficiencia de Proteína C/tratamiento farmacológico , Arteria Pulmonar/diagnóstico por imagen , Embolia Pulmonar/sangre , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/etiología , Recurrencia , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Filtros de Vena Cava
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA
...