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4.
Medicine (Baltimore) ; 98(35): e16984, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31464946

RESUMO

INTRODUCTION: Chronic myelogenous leukemia (CML), a clonal disorder of pluripotent stem cell, rarely presents with bleeding in chronic phase due to the function preservation of the platelets. Factor (F) XIII deficiency, an extremely rare hemorrhagic disease, can cause fatal bleeding, which has been previously described in autoimmune disorders and leukemias. PATIENT CONCERNS: A 38-year-old woman with a 20-day history of spontaneous subcutaneous hemorrhage visited our hospital, who presented with intracranial hemorrhage, hematuria, and delayed hematoma after a bone marrow puncture. Initial management included cytogenetics analysis, molecular analysis, and coagulation evaluation. DIAGNOSIS: Bone marrow puncture, cytogenetics, and molecular analysis indicated the diagnosis of CML. With the normal results of clotting screening tests and platelet counting, as well as the relief of bleeding after infusion of fresh frozen plasma (FFP), acquired rare bleeding disorder probably associated with factor XIII (FXIII) deficiency. INTERVENTIONS: Management with anti-hyperleukocytosis and chemotherapy, hydration, alkalization, diuresis, uric acid-lowering, molecular targeted drugs, and freshly frozen plasma transfusion therapy resolved the bleeding diathesis. OUTCOMES: The patient survived from the initial bleeding, however, she died. Twenty six months later due to the progression of CML. LESSONS: CML can initially present as unusual bleeding, possibly related to FXIII defect. It is essential to screen coagulopathy including FXIII activity and to supplement plasma for CML patients who present initially as bleeding, which cannot be deciphered by the routine clotting screening test.


Assuntos
Deficiência do Fator XIII/complicações , Hemorragia/complicações , Hemorragia/terapia , Leucemia Mielogênica Crônica BCR-ABL Positiva/complicações , Leucemia Mielogênica Crônica BCR-ABL Positiva/diagnóstico , Adulto , Antineoplásicos/uso terapêutico , Terapia Combinada , Feminino , Humanos , Plasma
5.
Medicine (Baltimore) ; 98(34): e16752, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31441848

RESUMO

RATIONALE: Tumor rupture and bleeding at initial presentation of infants with neuroblastoma (NBL) is a rare, but life threatening condition and challenge in pediatric oncology. Here, we report successful multidisciplinary management of an abdominal compartment syndrome as a result of tumor rupture and bleeding in an infant with bilateral high risk stage 4 NBL. PATIENT CONCERNS: The patient was admitted to a cooperating hospital with vomiting, failure to thrive and a large mass in the abdomen and was then referred to our center. DIAGNOSES: Stage 4 NBL with MYC-N amplification and 1p36 deletion was diagnosed in an 11 months old girl. Due to rapid and massive tumor growth she developed abdominal compression with renal failure, severe bleeding, and tumor lysis syndrome (TLS). INTERVENTIONS: Surgical decompression by enterostomy, local, and systemic bleeding control with platelets and coagulation factors, antiinfective and TLS therapy were effective in stabilizing the patient's condition. This allowed initiation of the multimodal antineoplastic treatment according to protocol NB 2004. OUTCOMES: Mechanical ventilation was stopped after 11 days, the abdominal wall was closed 3 months after the start of therapy, and treatment according to the protocol be started and successfully completed. LESSONS: Only the immediate, coordinated multidisciplinary intervention managed to overcome the life-threatening abdominal compartment syndrome and its associated problems, eventually enabling successful curative treatment.


Assuntos
Hemorragia/complicações , Hipertensão Intra-Abdominal/etiologia , Hipertensão Intra-Abdominal/terapia , Neuroblastoma/complicações , Anti-Infecciosos/uso terapêutico , Fatores de Coagulação Sanguínea , Enterostomia/métodos , Feminino , Hemorragia/terapia , Humanos , Lactente , Estadiamento de Neoplasias , Neuroblastoma/patologia
6.
Medicine (Baltimore) ; 98(29): e16544, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31335736

RESUMO

To investigate the status of percutaneous coronary intervention (PCI) in elderly patients with acute myocardial infarction (AMI) and analyze the reasons for not receiving PCI.A cohort of 387 consecutive hospitalized AMI patients aged ≥80 years were recruited from 2005 to 2014. Their clinical data were collected and analyzed.Among 387 elderly patients with AMI (190 men and 197 women, mean age 84.1 ±â€Š3.9 years), there were 171 patients with ST-elevation myocardial infarction (STEMI) and 216 patients with non-ST-elevation myocardial infarction (NSTEMI). The emergency and elective PCI treatment rate was 40.6% and 12.1%, respectively, in patients with STEMI; and 1% and 18%, respectively, in patients with NSTEMI. PCI treatment rate of elderly AMI patients enrolled after 2009 showed no significant difference compared to that before 2009 (P > .05). The in-hospital mortality decreased significantly in PCI treatment group. After adjustment for age, sex, and other factors, PCI treatment was identified as the independent protective factors for in-hospital mortality (odds ratio = 0.323, 95% confidence interval 0.147-0.710, P = .005). The main influence factors for not receiving PCI treatment were hemorrhage, severe renal dysfunction, infection, or severe anemia-associated complications, whereas delayed treatment was the important reason for patients not undergoing emergency PCI.PCI treatment is the independent protective factor for in-hospital mortality of elderly patients with AMI. Due to various complications, PCI treatment rate is still low in elderly patients with AMI and has not been improved recently. Paying attention to performing PCI treatment for elderly patients with AMI has positive significance.


Assuntos
Infarto do Miocárdio sem Supradesnível do Segmento ST/cirurgia , Intervenção Coronária Percutânea , Infarto do Miocárdio com Supradesnível do Segmento ST/cirurgia , Idoso de 80 Anos ou mais , Anemia/complicações , China , Procedimentos Cirúrgicos Eletivos , Serviço Hospitalar de Emergência , Feminino , Hemorragia/complicações , Mortalidade Hospitalar , Humanos , Infecção/complicações , Nefropatias/complicações , Masculino , Infarto do Miocárdio sem Supradesnível do Segmento ST/complicações , Infarto do Miocárdio sem Supradesnível do Segmento ST/mortalidade , Intervenção Coronária Percutânea/efeitos adversos , Complicações Pós-Operatórias , Infarto do Miocárdio com Supradesnível do Segmento ST/complicações , Infarto do Miocárdio com Supradesnível do Segmento ST/mortalidade , Centros de Atenção Terciária , Tempo para o Tratamento
7.
Artigo em Chinês | MEDLINE | ID: mdl-31315355

RESUMO

Objective: To analyze the clinical features and possible pathogenesis of sudden deafenss and vertigo induced by inner ear hemorrhage. Methods: Clinical data of 30 patients with inner ear hemorrhage, from the first affiliated hospital of Sun Yat-sen university during Jan 2016 to May 2017, were retrospectively analyzed. Results: Vergito and profound deafness were seen in all patients. The duration of vertigo ranged from 24 hours to three days in 11 cases, three to 14 days in the remaining 19 cases. Simultaneous occurrence of vergito and deafenss were seen in 24 patients. Semicircular canal hypofunction and abnormal cervical vestibular evoked myogenic potentials(C-VEMP)/ocular vestibular evoked myogenic potentials(O-VEMP) were detected in all cases. Ten patients had benign paroxysmal positional vertigo(BPPV) simultaneously. Hearing recovered in 20% of the cohort posttreatment. Dizziness and balance disturbance disappeared 1 to 2 months after therapy in 16 cases. Long term (6 months) follow up revealed poor hearing outcome and vestibular rehabilitation. Conclusion: Vestibular vertigo and profound sensorineural hearing loss, with unsatisfactory clinical prognosis, constituted the characters of inner ear hemorrhage-associated sudden deafness.


Assuntos
Vertigem Posicional Paroxística Benigna/etiologia , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Súbita/etiologia , Hemorragia/complicações , Doenças do Labirinto/complicações , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Súbita/diagnóstico , Humanos , Prognóstico , Estudos Retrospectivos , Potenciais Evocados Miogênicos Vestibulares
8.
Med. intensiva (Madr., Ed. impr.) ; 43(5): 281-289, jun.-jul. 2019. graf, tab
Artigo em Espanhol | IBECS | ID: ibc-183240

RESUMO

Objetivos: Analizar las complicaciones hematológicas y las necesidades transfusionales en niños tratados con oxigenación por membrana extracorpórea (ECMO). Diseño: Estudio retrospectivo. Ámbito: Unidad de cuidados intensivos pediátricos. Pacientes: Niños menores de 18 años tratados con ECMO entre septiembre de 2006 y noviembre de 2015. Intervenciones: Ninguna. Variables de interés: Características clínicas, de la ECMO, anticoagulación, parámetros hematológicos y de coagulación, transfusiones y evolución clínica. Resultados: Se estudiaron 100 pacientes con una mediana de edad de 11 meses. Presentaron sangrado 76; el mediastino fue la localización más frecuente; 39 precisaron revisión quirúrgica. En los primeros 3 días de ECMO, el 97% de los pacientes precisaron transfusión de hematíes (34,4 ml/kg al día), el 94% plaquetas (21,1ml/kg al día) y el 90% plasma (26,6ml/kg al día). Los pacientes posquirúrgicos, con imposibilidad de salida de la circulación extracorpórea, los que presentaron sangrado al inicio de la ECMO, los que precisaron revisión quirúrgica y los que tuvieron canulación transtorácica requirieron mayor volumen de transfusiones. Se produjeron tromboembolias en 14 pacientes y hemólisis en 33. La mortalidad de los niños que presentaron sangrado al inicio de ECMO (57,6%) fue significativamente mayor que la del resto (37,5%) (p = 0,048). Conclusiones: Los niños tratados con ECMO presentan una elevada incidencia de sangrado y precisan un gran volumen de transfusiones. El postoperatorio de cirugía, el sangrado al inicio de la ECMO, la necesidad de revisión quirúrgica, la imposibilidad de salida de la circulación extracorpórea y la canulación transtorácica se asocian a un mayor volumen de transfusiones. Los niños que sangraron al inicio de la ECMO presentaron mayor mortalidad


Objectives: To analyze the hematological complications and need for transfusions in children receiving extracorporeal life support (ECLS). Design: A retrospective study was carried out. Setting: A pediatric intensive care unit. Patients: Children under 18 years of age subjected to ECLS between September 2006 and November 2015. Interventions: None. Variables of interest: Patient and ECLS characteristics, anticoagulation, hematological and coagulation parameters, transfusions and clinical course. Results: A total of 100 patients (94 with heart disease) with a median age of 11 months were studied. Seventy-six patients presented bleeding. The most frequent bleeding point was the mediastinum and 39 patients required revision surgery. In the first 3days, 97% of the patients required blood transfusion (34.4ml/kg per day), 94% platelets (21.1ml/kg per day) and 90% plasma (26.6ml/kg per day). Patients who were in the postoperative period, those who were bleeding at the start of ECLS, those requiring revision surgery, those who could not suspend extracorporeal circulation, and those subjected to transthoracic cannulation required a greater volume of transfusions than the rest of the patients. Thromboembolism occurred in 14 patients and hemolysis in 33 patients. Mortality among the children who were bleeding at the start of ECLS (57.6%) was significantly higher than in the rest of the patients (37.5%) (P=.048). Conclusions: Children subjected to ECLS present high blood product needs. The main factors related to transfusions were the postoperative period, bleeding at the start of ECLS, revision surgery, transthoracic cannulation, and the impossibility of suspending extracorporeal circulation. Children with bleeding suffered greater mortality than the rest of the patients


Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Oxigenação por Membrana Extracorpórea/métodos , Transfusão de Sangue/tendências , Unidades de Terapia Intensiva Pediátrica , Doenças Hematológicas/sangue , Estudos Retrospectivos , Substitutos Sanguíneos/uso terapêutico , Tromboembolia/mortalidade , Mortalidade Infantil , Hemorragia/complicações , Anticoagulantes , Modelos Logísticos , Hemólise , Doenças Hematológicas/complicações , Doenças Hematológicas/etiologia
9.
Khirurgiia (Mosk) ; (5): 25-30, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31169815

RESUMO

AIM: To justify the concept of systemic membrane-destabilizing distress syndrome in surgery via analysis of phospholipid bilayer of cell membranes of various organs in urgent surgical abdominal diseases. MATERIAL AND METHODS: Experimental research on dogs (n=90) included modeling of peritonitis, pancreatitis, intestinal obstruction, obstructive jaundice, and post-hemorrhagic anemia. Clinical and laboratory studies were performed in patients (n=119) with acute peritonitis, severe pancreatitis, intestinal obstruction, post-hemorrhagic anemia, acute cholecystitis, gastrointestinal bleeding, benign mechanical jaundice. Lipid profile in tissues and blood cells was determined by extraction, fractionation and densitometry. Moreover, we assessed intensity of lipid peroxidation and phospholipase activity, endogenous intoxication, functional state of organs and blood cells. RESULTS: It was revealed that all above-mentioned acute abdominal diseases are followed by significant changes of lipid bilayer and dysfunction of tissues in target organs, blood cells and other organs (liver, kidney, colon and small intestine, heart, lungs, spleen, brain). Changes of phospholipid bilayer are correlated with severity and course of the disease. These data were used to determine a new complex in surgery - systemic membrane-destabilizing distress syndrome. Its concept, pathogenesis, and diagnosis are presented. It was analyzed its role in development and progression of dysregulation pathology and thanatogenesis. Evidence of its importance in the pathogenesis of surgical aggression was obtained.


Assuntos
Anemia/fisiopatologia , Membrana Celular/fisiologia , Doenças do Sistema Digestório/fisiopatologia , Hemorragia/fisiopatologia , Icterícia Obstrutiva/fisiopatologia , Estresse Fisiológico/fisiologia , Anemia/complicações , Animais , Doenças do Sistema Digestório/complicações , Modelos Animais de Doenças , Cães , Hemorragia/complicações , Humanos , Icterícia Obstrutiva/complicações , Lipídeos de Membrana/fisiologia , Fosfolipídeos/fisiologia , Síndrome
10.
Dis Colon Rectum ; 62(7): 794-801, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31188179

RESUMO

BACKGROUND: Carbon dioxide embolus has been reported as a rare but clinically important risk associated with transanal total mesorectal excision surgery. To date, there exists limited data describing the incidence, risk factors, and management of carbon dioxide embolus in transanal total mesorectal excision. OBJECTIVE: This study aimed to obtain data from the transanal total mesorectal excision registries to identify trends and potential risk factors for carbon dioxide embolus specific to this surgical technique. DESIGN: Contributors to both the LOREC and OSTRiCh transanal total mesorectal excision registries were invited to report their incidence of carbon dioxide embolus. Case report forms were collected detailing the patient-specific and technical factors of each event. SETTINGS: The study was conducted at the collaborating centers from the international transanal total mesorectal excision registries. MAIN OUTCOME MEASURES: Characteristics and outcomes of patients with carbon dioxide embolus associated with transanal mesorectal excision were measured. RESULTS: Twenty-five cases were reported. The incidence of carbon dioxide embolus during transanal total mesorectal excision is estimated to be ≈0.4% (25/6375 cases). A fall in end tidal carbon dioxide was noted as the initial feature in 22 cases, with 13 (52%) developing signs of hemodynamic compromise. All of the events occurred in the transanal component of dissection, with mean (range) insufflation pressures of 15 mm Hg (12-20 mm Hg). Patients were predominantly (68%) in a Trendelenburg position, between 30° and 45°. Venous bleeding was reported in 20 cases at the time of carbon dioxide embolus, with periprostatic veins documented as the most common site (40%). After carbon dioxide embolus, 84% of cases were completed after hemodynamic stabilization. Two patients required cardiopulmonary resuscitation because of cardiovascular collapse. There were no deaths. LIMITATIONS: This is a retrospective study surveying reported outcomes by surgeons and anesthetists. CONCLUSIONS: Surgeons undertaking transanal total mesorectal excision must be aware of the possibility of carbon dioxide embolus and its potential risk factors, including venous bleeding (wrong plane surgery), high insufflation pressures, and patient positioning. Prompt recognition and management can limit the clinical impact of such events. See Video Abstract at http://links.lww.com/DCR/A961.


Assuntos
Embolia Aérea/etiologia , Hemorragia/complicações , Insuflação/efeitos adversos , Complicações Intraoperatórias/etiologia , Reto/cirurgia , Cirurgia Endoscópica Transanal/efeitos adversos , Adulto , Idoso , Idoso de 80 Anos ou mais , Dióxido de Carbono , Embolia Aérea/diagnóstico , Embolia Aérea/terapia , Feminino , Humanos , Insuflação/métodos , Internacionalidade , Complicações Intraoperatórias/diagnóstico , Complicações Intraoperatórias/terapia , Masculino , Pessoa de Meia-Idade , Posicionamento do Paciente , Cuidados Pós-Operatórios , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Veias
12.
Medicine (Baltimore) ; 98(19): e15477, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31083181

RESUMO

RATIONALE: Recurrent massive hemorrhagic ascites secondary to endometriosis is extremely rare in the medical literature. PATIENT CONCERNS: We report the case of a 24-year-old nulliparous woman presenting with severe abdominal distention, massive ascites, moderate anemia, menstrual pain, and an elevated CA-125 level. DIAGNOSIS: We found a thickened peritoneum in the left lower abdomen by ultrasound during the follow-up period, and endometriosis was subsequently diagnosed by performing core needle biopsy (CNB). INTERVENTIONS AND OUTCOMES: The patient received medical treatment for endometriosis and had a good response to the treatment. LESSONS: This is the first case in which endometriosis ectopic to peritoneum was diagnosed by CNB. Endometriosis should be considered a differential diagnosis when recurrent massive hemorrhagic ascites occur. CNB should be valued as a method for diagnosing endometriosis.


Assuntos
Ascite/diagnóstico , Endometriose/diagnóstico , Hemorragia/diagnóstico , Ascite/complicações , Ascite/tratamento farmacológico , Ascite/patologia , Biópsia com Agulha de Grande Calibre , Tratamento Conservador , Diagnóstico Diferencial , Endometriose/complicações , Endometriose/tratamento farmacológico , Endometriose/patologia , Feminino , Hemorragia/complicações , Hemorragia/tratamento farmacológico , Hemorragia/patologia , Humanos , Peritônio/diagnóstico por imagem , Peritônio/patologia , Recidiva , Adulto Jovem
13.
Biomed Res Int ; 2019: 2674758, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31073523

RESUMO

Aims: To evaluate the safety and effectiveness of percutaneous transhepatic antegrade embolization (PTAE) with 2-octyl cyanoacrylate assisted with balloon occlusion of the left renal vein or gastrorenal shunts (GRSs) for the treatment of isolated gastric varices (IGVs) with large GRSs. Methods: Thirty patients with IGVs associated with large GRSs who had underwent PTAE assisted with a balloon to block the opening of the GRS in the left renal vein were retrospectively evaluated and followed up. Clinical and laboratory data were collected to evaluate the technical success of the procedure, complications, changes in the liver function using Child-Pugh scores, worsening of the esophageal varices, the rebleeding rate, and survival. Laboratory data obtained before and after PTAE were compared (paired-sample t-test). Results: PTAE was technically successful in all 30 patients. No serious complications were observed except for one nonsymptomatic pulmonary embolism. During a mean follow-up of 30 months, rebleeding was observed in 4/30 (13.3%) patients, worsening of esophageal varices was observed in 4/30 (13.3%) patients, and newly developed or aggravated ascites were observed on CT in 3/30 (10%) patients. Significant improvement was observed in Child-Pugh scores (p=0.009) and the international normalized ratio (INR) (p=0.004) at 3 months after PTAE. The cumulative survival rates at 1, 2, 3, and 5 years were 96.3%, 96.3%, 79.9%, and 79.9%, respectively. Conclusion: Balloon-assisted PTAE with 2-octyl cyanoacrylate is technically feasible, safe, and effective for the treatment of IGV associated with a large GRS.


Assuntos
Oclusão com Balão/métodos , Embolização Terapêutica/métodos , Varizes Esofágicas e Gástricas/terapia , Cirrose Hepática/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Oclusão com Balão/efeitos adversos , Cianoacrilatos/administração & dosagem , Varizes Esofágicas e Gástricas/fisiopatologia , Feminino , Fístula/fisiopatologia , Fístula/terapia , Hemorragia Gastrointestinal/complicações , Hemorragia Gastrointestinal/patologia , Hemorragia/complicações , Hemorragia/patologia , Humanos , Estimativa de Kaplan-Meier , Fígado/fisiopatologia , Cirrose Hepática/fisiopatologia , Masculino , Pessoa de Meia-Idade , Veias Renais/efeitos dos fármacos , Veias Renais/cirurgia , Adesivos Teciduais/administração & dosagem , Resultado do Tratamento
14.
Vnitr Lek ; 65(3): 211-218, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31088098

RESUMO

Hemorrhagic shock is a severe complication of conditions that are accompanied by massive bleeding and is associated with high mortality and morbidity. The authors summarize the recent knowledge in the pathophysiology of coagulopathy in the course of its development and some options in treatment strategy.


Assuntos
Choque Hemorrágico , Hemorragia/complicações , Humanos , Choque Hemorrágico/etiologia , Choque Hemorrágico/terapia
16.
Gulf J Oncolog ; 1(29): 87-90, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30956201

RESUMO

Esophageal carcinoma metastasis to the brain is very rare with less than 300 cases published worldwide. The reported incidence rates of esophageal carcinoma metastases to the brain range from 0.3% to 13%. It is associated with a worse prognosis than brain metastases from other solid tumors. The most common form of metastasis is cystic lesions while hemorrhagic metastasis represents a rare subset of an infrequent entity. It is extremely rare for esophageal carcinoma to present as an isolated brain metastasis with only three cases reported worldwide. These cases were misdiagnosed as meningitis, a pituitary tumor, and glioma. This paper reports the first case of hemorrhagic brain metastasis as the initial and only manifestation of esophageal adenocarcinoma worldwide. Although extensive work up to search for malignancy is a routine procedure in patients with metastatic brain disease, upper gastrointestinal endoscopy, which is rarely done as part of the work up should be included. Esophageal cancer continues to be challenging to manage with very limited therapeutic options. To date, the clinical outcome after treatment is unsatisfactory, and prognosis is poor. Further research is required to identify better treatment approaches and improve the survival of patients. This paper is an urgent call for action to design chemotherapeutic agents that target tumor cells in the blood and different body organs and has the ability to cross the blood-brain barrier with an acceptable side effect profile. This is mainly because metastatic brain disease has become more common in young age group.


Assuntos
Adenocarcinoma/complicações , Neoplasias Encefálicas/secundário , Neoplasias Esofágicas/complicações , Hemorragia/complicações , Adenocarcinoma/patologia , Neoplasias Esofágicas/patologia , Feminino , Humanos , Masculino , Metástase Neoplásica , Prognóstico
17.
Haemophilia ; 25(3): 382-391, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31016855

RESUMO

INTRODUCTION: Real-world data (RWD) on health-related outcomes in persons with haemophilia A (PwHA) provide insights into patient needs and can guide clinical study design. A global, prospective, non-interventional study (NIS; NCT02476942) collected detailed RWD on bleeding outcomes, health-related quality of life (HRQoL) and health status in PwHA treated per local routine clinical practice. AIM: To report HRQoL and health status in the adult/adolescent PwHA with inhibitors cohort in the NIS. METHODS: This cohort enrolled PwHA aged ≥12 years with high-titre factor VIII inhibitor history. Participants remained on their usual treatment (no protocol-specified interventions). Health-related outcomes: Haemophilia Quality of Life Questionnaire for Adults (Haem-A-QoL), Haemophilia-specific Quality of Life Questionnaire for Children Short Form (Haemo-QoL SF), EuroQol 5-Dimensions 5-Levels (EQ-5D-5L) index utility score (IUS) and visual analogue scale (EQ-VAS). RESULTS: One hundred three participants were enrolled on episodic (n = 75) or prophylactic treatment (n = 28); median (range) age, 31 (12-75) years; median (range) observation time, 26 (4-70) weeks. Haem-A-QoL scores indicated impairments in HRQoL aspects; comparable between episodic/prophylactic regimens and relatively consistent over time. Haemo-QoL SF scores with both regimens varied over time, and appeared poorer with episodic than prophylactic treatment. IUS and EQ-VAS were comparable between regimens, stable over time and lower on bleeding days. Mean proportions of missed work and school days were 16% and 23%, respectively; mean (standard deviation) number of days hospitalized was 3.2 (8.8) (comparable between groups). CONCLUSIONS: These RWD demonstrate that PwHA with inhibitors have impaired HRQoL, despite standard treatment, and that more effective treatment options are needed.


Assuntos
Nível de Saúde , Hemofilia A/epidemiologia , Qualidade de Vida , Absenteísmo , Adolescente , Adulto , Idoso , Criança , Feminino , Hemofilia A/complicações , Hemorragia/complicações , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Instituições Acadêmicas/estatística & dados numéricos , Inquéritos e Questionários , Trabalho/estatística & dados numéricos , Adulto Jovem
19.
Medicine (Baltimore) ; 98(11): e14658, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30882629

RESUMO

Autonomic dysfunction is a feature of glaucoma patients, which are reported to be related to glaucoma progression. We investigated pupil responses to a light flash using dynamic pupillometry in glaucoma patients to assess autonomic nervous system status. In total, 97 glaucoma patients, including 21 eyes of 21 glaucoma patients with cardiac autonomic dysfunction, were enrolled. Pupil reactions were assessed using 1 flash of white light after 2 minutes of dark adaptation and recorded using dynamic pupillometry. Changes in the radius of the pupil were evaluated as a function of several time-dependent and pupil/iris (P/I) diameter ratio parameters. Autonomic function was assessed using a cardiac heart-rate-variability test which performs 5 autonomic function tests and classifies patients with cardiac autonomic neuropathy (CAN). Comparison of pupil parameters between eyes with and without disc hemorrhage indicated larger P/I ratios in darkness, greater changes in the P/I ratio during examination, shorter latency to plateau, and shorter duration of constriction in eyes with disc hemorrhages. A comparison of pupil parameters between eyes with and without CAN showed larger P/I ratios in darkness, larger P/I ratios at maximum constriction, and prolonged latency to maximum constriction. The presence of CAN was significantly related to the P/I ratio in darkness and the latency of maximum constriction. Using dynamic pupillometry, we found that glaucoma patients with CAN dysfunction have larger baseline pupils in darkness and different constriction responses to light. Assessing the pupils might be a good method of identifying patients with autonomic dysfunction.


Assuntos
Doenças do Sistema Nervoso Autônomo/fisiopatologia , Vias Autônomas/fisiopatologia , Glaucoma/fisiopatologia , Frequência Cardíaca , Hemorragia/complicações , Pupila/fisiologia , Adulto , Idoso , Doenças do Sistema Nervoso Autônomo/complicações , Doenças do Sistema Nervoso Autônomo/diagnóstico , Técnicas de Diagnóstico Neurológico , Feminino , Glaucoma/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Disco Óptico , Estimulação Luminosa , Estudos Prospectivos , Reflexo Pupilar
20.
Artigo em Inglês | MEDLINE | ID: mdl-30845781

RESUMO

Acute hemorrhagic oedema of infancy (AHEI) is a cutaneous leukocytoclastic small-vessel vasculitis presenting with localized purpuric large skin plaques that are frequently associated with fever and oedema. It must be promptly differentiated from a number of diseases with similar dermatologic manifestations with potentially severe clinical courses that require adequate monitoring and prompt therapy to avoid the risk of a negative evolution. A 15-month-old girl with a negative personal medical clinical history was admitted for the sudden appearance of petechiae on the soft palate. The patient was moderately febrile during the following two days, with a maximum ear temperature of 38.3 °C. The fever disappeared on the third day, whereas the hemorrhagic rash progressively increased and extended to the limbs, face and auricles associated with a strong oedematous component. Moreover, on the second day of hospitalization, bilateral oedema of the metacarpophalangeal joints with joint pain appeared. The blood and serological tests showed an increase in C-reactive protein concentration (3.58 mg/dL) in the absence of leukocytosis and with a normal platelet count (180,000/mm³). The examination of the peripheral smear showed the presence of some large mononuclear elements with hyperbasophile cytoplasm. No alterations in platelet morphology were evidenced. The skin manifestations progressively diminished and disappeared spontaneously within 3 weeks, leaving no sequelae. Conclusion: This case shows the classic skin lesions of AHEI that require differentiation from those of more severe diseases that need prompt recognition and therapy. In this case, the age of the patient, the lack of systemic involvement and the favorable clinical course without therapy were typical. However, as these patients may present to the emergency department with an impressive clinical picture, the condition must be promptly diagnosed to avoid unnecessary diagnostic procedures and to reassure parents.


Assuntos
Edema/diagnóstico , Hemorragia/diagnóstico , Vasculite Leucocitoclástica Cutânea/diagnóstico , Doença Aguda , Criança , Diagnóstico Diferencial , Edema/complicações , Feminino , Febre/complicações , Hemorragia/complicações , Humanos , Lactente , Pele/patologia , Vasculite Leucocitoclástica Cutânea/patologia
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