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1.
Haemophilia ; 29(1): 240-247, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36395791

RESUMO

INTRODUCTION: Reproductive-age women with bleeding disorders (BDs) are underdiagnosed and understudied, despite their increased risk for adverse health outcomes and pregnancy complications. AIM: This study examines pregnancy outcomes and obstetric complications of Utah women with BDs. METHODS: This retrospective cohort study utilized linked birth records and clinical billing data from two large Utah healthcare systems. Utah residents who had their first birth at > 20 weeks gestation (2008-2015) and who received non-emergent care within either system before delivery were included (n = 61 226). Multivariable logistic regression models were used to examine relationships between BDs and neonatal and obstetric outcomes. RESULTS: A total of 295 women (.48%) were included in the BD study population. Women with BDs had significantly increased odds of preterm birth (aOR 1.85, 95% CI 1.32-2.60), Caesarean delivery (aOR 1.38, 95% CI 1.06-1.79), postpartum blood transfusion (aOR 2.55, 95% CI 1.05-6.22), unplanned postpartum hysterectomy (aOR 33.96, 95% CI 7.30-157.89) and transfer to an intensive care unit (aOR 18.18, 95% CI 7.17-46.08). All of the women with BDs who experienced these serious complications were not diagnosed with a BD until the year of their first birth. Additionally, those with BDs were more likely to experience maternal and infant mortality. CONCLUSION: Women with BDs had an increased risk for preterm birth, Caesarean delivery, blood transfusion, unplanned hysterectomy, intensive care unit admission, maternal and infant mortality. Those who were not diagnosed with a BD before the year of their first birth were at an increased risk for serious pregnancy complications.


Assuntos
Transtornos da Coagulação Sanguínea , Transtornos Hemorrágicos , Complicações na Gravidez , Nascimento Prematuro , Gravidez , Lactente , Recém-Nascido , Humanos , Feminino , Nascimento Prematuro/epidemiologia , Nascimento Prematuro/etiologia , Estudos Retrospectivos , Parto , Cesárea/efeitos adversos , Complicações na Gravidez/epidemiologia , Transtornos da Coagulação Sanguínea/complicações , Transtornos Hemorrágicos/complicações
2.
BMJ Case Rep ; 15(10)2022 Oct 07.
Artigo em Inglês | MEDLINE | ID: mdl-36207057

RESUMO

A term Caucasian neonate with an uncomplicated birth history presented with persistent umbilical stump bleeding unresponsive to extensive topical haemostatic measures initially. He subsequently developed hypovolaemic shock. Routine full blood count and basic coagulation screen were unremarkable. He received packed red cell and cryoprecipitate transfusions. Further specialist coagulation studies performed revealed factor XIII deficiency. Genetic investigations demonstrated a compound heterozygosity for the disorder. He was later started on monthly prophylactic treatment of plasma-derived factor XIII. Clinicians should have a high index of suspicion for factor XIII deficiency for newborns with abnormal umbilical stump bleeding in the presence of no bleeding risk factors and normal routine blood investigations.


Assuntos
Deficiência do Fator XIII , Transtornos Hemorrágicos , Hemostáticos , Testes de Coagulação Sanguínea , Fator XIII/uso terapêutico , Deficiência do Fator XIII/complicações , Deficiência do Fator XIII/diagnóstico , Deficiência do Fator XIII/genética , Hemorragia/complicações , Transtornos Hemorrágicos/complicações , Humanos , Recém-Nascido , Masculino
3.
J Pediatr Adolesc Gynecol ; 35(6): 647-652, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35917902

RESUMO

STUDY OBJECTIVE: To examine the clinical characteristics and prevalence of congenital bleeding disorders (CBDs), with emphasis on congenital factor VII (FVII) deficiency and other rare bleeding disorders, in adolescent and young adult females referred to a hemophilia treatment center (HTC) for evaluation and management of heavy menstrual bleeding (HMB) and iron deficiency anemia (IDA) DESIGN: In this single-center retrospective study, we reviewed the clinical characteristics and prevalence of CBDs in postmenarchal females, younger than 22 years of age, referred to an HTC from 2015 to 2021 for evaluation of HMB with or without IDA. RESULTS: One hundred females, with a mean age of 15 years (range 9-20 years), met initial study criteria, and 95 were included in the final analysis. Forty-five (47%) females were ultimately diagnosed with a CBD. The most prevalent diagnoses were FVII deficiency and type 1 von Willebrand disease (VWD) (42.3%, n = 19 each). Forty-two percent of patients with FVII deficiency had a low-for-age FVII activity level, 21.1% were only positive for the FVII R353Q variant associated with borderline FVII levels, whereas 36.8% had both a low-for-age FVII activity level and a positive R353Q variant. Eighty percent of patients with a CBD were found to have relatives with abnormal bleeding symptoms. CONCLUSION: Congenital FVII deficiency is prevalent among female adolescents experiencing HMB with or without IDA. In addition to VWD, evaluation for this specific factor deficiency should be considered as part of the initial CBD workup. Presence of abnormal bleeding history in the family could also help to predict presence of a CBD.


Assuntos
Anemia Ferropriva , Deficiência do Fator VII , Transtornos Hemorrágicos , Deficiências de Ferro , Menorragia , Adolescente , Criança , Feminino , Humanos , Adulto Jovem , Anemia Ferropriva/epidemiologia , Fator VII , Deficiência do Fator VII/complicações , Deficiência do Fator VII/epidemiologia , Hemorragia , Transtornos Hemorrágicos/complicações , Menorragia/etiologia , Menorragia/complicações , Prevalência , Estudos Retrospectivos
4.
Am J Hematol ; 97(1): 60-67, 2022 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-34710246

RESUMO

Iron deficiency anemia is associated with heavy menstrual bleeding (HMB) and, by extension, a bleeding disorder (BD). It is unknown if iron deficiency without anemia is associated with a BD in adolescents. Moreover, the threshold of ferritin associated with fatigue in adolescents with HMB is unclear. In this multicenter study, we enrolled adolescents with HMB without BD. Participants underwent BD and anemia work-up in Young Women's Hematology Clinics and completed the Peds QL™ fatigue scale. BDs were defined as von Willebrand Disease, platelet function defect, clotting factor deficiencies, and hypermobility syndrome. Two hundred and fifty consecutive adolescents were enrolled, of whom 196 met eligibility criteria. Overall, 43% (95% confidence interval: 36%-50%) were diagnosed with BD. A total of 61% (n = 119) had serum ferritin levels < 15 ng/mL, 23.5% (n = 46) had iron deficiency only, and 37% (n = 73) had iron deficiency anemia. Low ferritin or ferritin dichotomized as < 15 or ≥ 15 ng/mL was not associated with BD on univariable analysis (p = .24) or when accounting for age, race, ethnicity, body mass index, and hemoglobin (p = .35). A total of 85% had total fatigue score below the population mean of 80.5, and 52% (n = 102) were > 2 SD (or < 54) below the mean, the cut-off associated with severe fatigue. A ferritin threshold of < 6 ng/mL had a specificity of 79.8% but a sensitivity of 36% for severe fatigue. In conclusion, iron deficiency without anemia is not a predictor of BD in adolescents with HMB in a specialty setting. Severe fatigue, especially sleep fatigue, is prevalent in adolescents with BD. Ferritin of < 6 ng/mL has ~80% specificity for severe fatigue in adolescents with HMB.


Assuntos
Fadiga/complicações , Transtornos Hemorrágicos/complicações , Deficiências de Ferro/complicações , Adolescente , Adulto , Fadiga/sangue , Feminino , Ferritinas/análise , Transtornos Hemorrágicos/sangue , Humanos , Deficiências de Ferro/sangue , Masculino , Menorragia/sangue , Menorragia/complicações , Adulto Jovem , Doenças de von Willebrand/sangue , Doenças de von Willebrand/complicações
5.
Platelets ; 33(1): 168-170, 2022 Jan 02.
Artigo em Inglês | MEDLINE | ID: mdl-33426985

RESUMO

B-cell lymphoproliferative diseases may be associated with acquired hemostasis disorders, such as acquired hemophilia A (AHA) caused by autoantibodies that neutralize factor VIII activity, and δ-storage pool deficiency, an abnormality of platelet function due to defective dense granules and impaired secretion. We describe the case of a 67-year-old man in whom these two acquired bleeding disorders were concomitantly present as the first clinical manifestation of an indolent non-Hodgkin lymphoma. Immunosuppressive therapy with prednisone was initially started to eradicate anti-FVIII antibodies, subsequently boosted with cyclophosphamide and rituximab, these medications being also chosen to treat the associated indolent lymphoma. Bleeding symptoms were first tackled with limited benefit by using rFVIIa and then rescued using recombinant porcine FVIII. After a 6 month's follow-up lymphoma and AHA were in remission and platelet function was improved. This case underlines the need of multiple and complex diagnostic and therapeutic approaches to rare acquired bleeding disorders associated with lymphoproliferative diseases.


Assuntos
Albinismo/complicações , Hemofilia A/etiologia , Transtornos Hemorrágicos/complicações , Síndrome de Hermanski-Pudlak/complicações , Linfoma não Hodgkin/complicações , Idoso , Hemofilia A/fisiopatologia , Humanos , Masculino
6.
Viruses ; 13(12)2021 11 25.
Artigo em Inglês | MEDLINE | ID: mdl-34960630

RESUMO

Diagnosis of SARS-CoV-2 infections is mostly based on the nasopharyngeal swabs (NPS). However, this collection is invasive and uncomfortable, especially for children and patients with coagulopathies, whose NPS collection often causes bleeding. Thus, the aim of this study was to evaluate the usefulness and accuracy of saliva for the diagnosis of COVID-19 in patients presenting bleeding disorders. Samples of NPS, oropharyngeal swabs (OPS), and saliva were collected simultaneously from 1159 hospitalized patients with hematological diseases and from 524 healthcare workers, both symptomatic and asymptomatic for SARS-CoV-2. All samples were evaluated for SARS-CoV-2 by qRT-PCR. SARS-CoV-2 was detected in NPS, OPS and saliva from 16.9%, 14.4% and 15.6% individuals, respectively. Tests in saliva showed sensitivity, specificity, and overall agreement of 73.3%, 96.9% and 92.7% (=0.74), respectively. Salivary tests had good accuracy (AUC = 0.7) for discriminating negative and positive qRT-PCR for SARS-CoV-2. Higher sensitivity was observed in symptomatic than in non-symptomatic patients, as well as in healthy subjects than in patients with hematological disease, in both OPS and saliva. The mean viral load in NPS was significantly higher than in OPS and in saliva samples (p < 0.001). Saliva is a good diagnostic tool to detect SARS-CoV-2, especially among patients symptomatic for COVID-19, and is a valuable specimen for mass screening of hospitalized patients with hematological diseases, especially for those that with bleeding disorders.


Assuntos
Transtornos da Coagulação Sanguínea/complicações , COVID-19/complicações , COVID-19/diagnóstico , Transtornos Hemorrágicos/complicações , SARS-CoV-2/isolamento & purificação , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , COVID-19/virologia , Teste para COVID-19 , Criança , Pré-Escolar , Estudos de Coortes , Testes Diagnósticos de Rotina , Feminino , Pessoal de Saúde , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Saliva , Carga Viral , Adulto Jovem
8.
Blood Coagul Fibrinolysis ; 32(6): 366-372, 2021 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-33973892

RESUMO

Unclassified bleeding disorders account for 2.6% of all new bleeding disorder registrations in the UK. The management of the bleeding phenotype associated with these disorders is poorly described. Systematic review and meta-analysis to determine the bleeding rates associated with tranexamic acid, desmopressin, platelet transfusion, plasma transfusion and recombinant activated factor VII, for patients with unclassified bleeding disorders undergoing surgery, childbirth or with menorrhagia. We searched for randomized controlled trials in MEDLINE, Embase, The Cochrane Central Register of Controlled Trials, PubMed, ISI Web of Science and the Transfusion Evidence Library from inception to 24 February 2020. Wherever appropriate, data were pooled using the metaprop function of STATA. Two studies with 157 participants with unclassified bleeding disorders were identified. The pooled risk of minor bleeding for patients undergoing surgery treated with peri-operative tranexamic acid was 11% (95% confidence interval 3--20%; n = 52; I2 = 0%); the risk for desmopressin and tranexamic acid in combination was 3% (95% confidence interval 0--7%; n = 71; I2 = 0%). There were no instances of major bleeding. In one procedure, 1 of 71 (1.4%), treated with a combination of desmopressin and tranexamic acid, the patient had a line-related deep vein thrombosis. There were too few patients treated to prevent postpartum haemorrhage or for menorrhagia to draw conclusions. The GRADE quality of evidence was very low suggesting considerable uncertainty over the results. However, both tranexamic acid, and the combination of tranexamic and desmopressin have high rates of haemostatic efficacy and have few adverse events. PROTOCOL REGISTRATION: PROSPERO CRD42020169727.


Assuntos
Hemorragia/terapia , Transtornos Hemorrágicos/terapia , Menorragia/terapia , Hemorragia Pós-Parto/terapia , Antifibrinolíticos/uso terapêutico , Transfusão de Componentes Sanguíneos , Desamino Arginina Vasopressina/uso terapêutico , Gerenciamento Clínico , Feminino , Hemorragia/complicações , Transtornos Hemorrágicos/complicações , Hemostáticos/uso terapêutico , Humanos , Menorragia/complicações , Hemorragia Pós-Operatória/complicações , Hemorragia Pós-Operatória/terapia , Gravidez , Ácido Tranexâmico/uso terapêutico
10.
Prensa méd. argent ; 106(10): 618-624, 20200000. fig, tab
Artigo em Inglês | LILACS, BINACIS | ID: biblio-1362699

RESUMO

All health care providers should be aware of the impact of bleeding disorders on their patients during any surgical procedures. The knowledge of the mechanisms of hemostasis and optimized management are very important. Initial recognition of a bleeding disorder, in such patients with a systemic pathologic process, may occur in surgical practice. The surgical treatment of those patients might be complicated during the surgery due to the use of anticoagulant and/or antiplatelet medications raises a challenge in the daily practice of surgical professionals. Adequate hemostasis is critical for the success of any surgical procedure because bleeding problems can give rise to complications associated with important morbidity-mortality. Besides, prophylactic, restorative, and surgical care of patients with any bleeding disorders is handled skillfully by practitioners who are well educated regarding the pathology, complications which could arise, and surgical options associated with these conditions. The purpose of this paper is to review common bleeding disorders and their effects on the surgical aspect. Many authors consider that patient medication indicated for the treatment of background disease should not be altered or suspended unless so indicated by the prescribing physician. Local hemostatic measures have been shown to suffice for controlling possible bleeding problems resulting from surgery.


Assuntos
Humanos , Procedimentos Cirúrgicos Operatórios , Inibidores da Agregação Plaquetária/administração & dosagem , Hemorragia/cirurgia , Transtornos Hemorrágicos/complicações , Hemostasia Cirúrgica/mortalidade , Anticoagulantes/administração & dosagem
11.
Prehosp Disaster Med ; 35(6): 690-692, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33023694

RESUMO

Pulmonary embolism is a clinical condition with high mortality rates in all age groups. The treatment includes anticoagulation and fibrinolytic therapy, and clinical management is challenging in cases of bleeding diathesis. Sepsis-induced coagulopathy (SIC), which has been recently defined to cause disruption of coagulation cascade accompanied by organ dysfunctions, is regarded as a major cause of mortality. It is noteworthy that there is no decrease in fibrinogen levels, unlike disseminated intravascular coagulopathy (DIC). This study aimed to present the management of a 70-year-old female patient who was admitted to emergency department with atypical complaints and diagnosed with pulmonary embolism due to deep vein thrombosis and septic shock. The clinical success of fibrinolytic therapy following the administration of fresh frozen plasma (FFP), although the patient had elevated international normalized ratio (INR), is presented in this case report. Since elevated INR and thrombocytopenia, which are observed in SIC, are caused by the inhibition of fibrinolysis, fibrinolytic therapy can be a rational treatment choice considering the profit/loss rate.


Assuntos
Transtornos Hemorrágicos/complicações , Embolia Pulmonar/terapia , Choque Séptico/complicações , Trombose Venosa/complicações , Idoso , Tomada de Decisões , Feminino , Humanos , Plasma , Embolia Pulmonar/complicações , Terapia Trombolítica
12.
Lancet Haematol ; 7(9): e671-e678, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32659214

RESUMO

BACKGROUND: COVID-19 is an ongoing global pandemic. Changes in haematological characteristics in patients with COVID-19 are emerging as important features of the disease. We aimed to explore the haematological characteristics and related risk factors in patients with COVID-19. METHODS: This retrospective cohort study included patients with COVID-19 admitted to three designated sites of Wuhan Union Hospital (Wuhan, China). Demographic, clinical, laboratory, treatment, and outcome data were extracted from electronic medical records and compared between patients with moderate, severe, and critical disease (defined according to the diagnosis and treatment protocol for novel coronavirus pneumonia, trial version 7, published by the National Health Commission of China). We assessed the risk factors associated with critical illness and poor prognosis. Dynamic haematological and coagulation parameters were investigated with a linear mixed model, and coagulopathy screening with sepsis-induced coagulopathy and International Society of Thrombosis and Hemostasis overt disseminated intravascular coagulation scoring systems was applied. FINDINGS: Of 466 patients admitted to hospital from Jan 23 to Feb 23, 2020, 380 patients with COVID-19 were included in our study. The incidence of thrombocytopenia (platelet count <100 × 109 cells per L) in patients with critical disease (42 [49%] of 86) was significantly higher than in those with severe (20 [14%] of 145) or moderate (nine [6%] of 149) disease (p<0·0001). The numbers of lymphocytes and eosinophils were significantly lower in patients with critical disease than those with severe or moderate disease (p<0·0001), and prothrombin time, D-dimer, and fibrin degradation products significantly increased with increasing disease severity (p<0·0001). In multivariate analyses, death was associated with increased neutrophil to lymphocyte ratio (≥9·13; odds ratio [OR] 5·39 [95% CI 1·70-17·13], p=0·0042), thrombocytopenia (platelet count <100 × 109 per L; OR 8·33 [2·56-27·15], p=0·00045), prolonged prothrombin time (>16 s; OR 4·94 [1·50-16·25], p=0·0094), and increased D-dimer (>2 mg/L; OR 4·41 [1·06-18·30], p=0·041). Thrombotic and haemorrhagic events were common complications in patients who died (19 [35%] of 55). Sepsis-induced coagulopathy and International Society of Thrombosis and Hemostasis overt disseminated intravascular coagulation scores (assessed in 12 patients who survived and eight patients who died) increased over time in patients who died. The onset of sepsis-induced coagulopathy was typically before overt disseminated intravascular coagulation. INTERPRETATION: Rapid blood tests, including platelet count, prothrombin time, D-dimer, and neutrophil to lymphocyte ratio can help clinicians to assess severity and prognosis of patients with COVID-19. The sepsis-induced coagulopathy scoring system can be used for early assessment and management of patients with critical disease. FUNDING: National Key Research and Development Program of China.


Assuntos
Infecções por Coronavirus/patologia , Transtornos Hemorrágicos/patologia , Pneumonia Viral/patologia , Adulto , Idoso , Betacoronavirus/isolamento & purificação , COVID-19 , Infecções por Coronavirus/classificação , Infecções por Coronavirus/complicações , Infecções por Coronavirus/virologia , Coagulação Intravascular Disseminada/complicações , Coagulação Intravascular Disseminada/patologia , Eosinófilos/citologia , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Produtos de Degradação da Fibrina e do Fibrinogênio/metabolismo , Transtornos Hemorrágicos/complicações , Humanos , Modelos Lineares , Linfócitos/citologia , Masculino , Pessoa de Meia-Idade , Razão de Chances , Pandemias/classificação , Pneumonia Viral/classificação , Pneumonia Viral/complicações , Pneumonia Viral/virologia , Tempo de Protrombina , Estudos Retrospectivos , Fatores de Risco , SARS-CoV-2 , Índice de Gravidade de Doença , Trombocitopenia/complicações , Trombocitopenia/patologia
13.
JBJS Rev ; 8(6): e0001, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32487975

RESUMO

Perioperative management of orthopaedic patients with a hematologic disorder is a complex endeavor that requires a multidisciplinary team-based approach. A team composed of an experienced orthopaedic surgeon, an anesthesiologist, and a hematologist is necessary to achieve optimal outcomes. Patients with hemophilia and other complex hematologic disorders should be managed at, or in consultation with a hematologist at, a comprehensive hemophilia center. Bleeding disorders and inherited thrombophilia present unique challenges for the perioperative management of orthopaedic surgery. Comprehensive preoperative planning and familiarity with treatment guidelines can help to minimize these risks. Knowledge of the disease processes outlined in this article will provide orthopaedic surgeons with the requisite background knowledge that is needed to initiate safe and effective treatment strategies involving this high-risk patient population.


Assuntos
Transtornos Hemorrágicos/complicações , Procedimentos Ortopédicos , Assistência Perioperatória , Humanos
14.
Cardiovasc Intervent Radiol ; 43(3): 362-368, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31690982

RESUMO

PURPOSE: In this systematic review, we report the safety and efficacy of embolization for treating hemarthrosis in patients with bleeding diatheses with or without a history of prior arthroplasty. MATERIALS AND METHODS: A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analysis guidelines. A structured search was performed in PubMed, Web of Science, MEDLINE, and SCOPUS databases of patients undergoing embolization for hemarthrosis. Demographic data, clinical history, angiographic findings, interventions, and outcomes were extracted and tabulated. RESULTS: The search identified 97 unique titles of which 15 were deemed relevant comprising 78 patients with coagulopathies undergoing 93 embolization procedures. Mean follow-up time was 29 months (range 3-116.5). In the 29 patients who underwent arthroplasty prior to embolization for hemarthrosis, there were 11 bleeding recurrences (37.9%). Of these patients who experienced recurrences, 10 underwent repeat embolization as coagulation factor replacement was not sufficient to address the re-bleeding. In the 49 patients with bleeding diatheses who underwent embolization for hemarthrosis with no history of prior arthroplasty, there were 11 recurrences (22.4%) and 4 of these patients underwent repeat embolization with favorable results. There were four major procedure-related complications. CONCLUSION: Transarterial embolization is safe and effective in addressing recurrent hemarthrosis in patients with bleeding diatheses with or without a history of arthroplasty of the affected joint.


Assuntos
Embolização Terapêutica/métodos , Procedimentos Endovasculares/métodos , Hemartrose/complicações , Hemartrose/terapia , Transtornos Hemorrágicos/complicações , Feminino , Humanos , Masculino
15.
J Pediatr Adolesc Gynecol ; 33(2): 139-143, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31765796

RESUMO

STUDY OBJECTIVE: To describe the adolescent population that seeks care in the emergency department (ED) for heavy menstrual bleeding (HMB), and to compare those who are discharged to those who are admitted to the hospital. DESIGN: Retrospective study. SETTING: Emergency department and inpatient unit at a national tertiary care hospital from 2006-2018. PARTICIPANTS: Adolescents 11-19 years old with ICD-9 and ICD-10 codes for HMB. INTERVENTIONS: Chart abstraction for demographic data, symptoms, laboratory tests, outcomes, and treatments. MAIN OUTCOME MEASURE: Adolescents who were admitted were compared to girls who were treated as outpatients. RESULTS: There were 258 adolescents who sought care for HMB in the ED during the study period. A total of 44 patients (17%) were admitted to the hospital, whereas 214 (83%) were discharged. The average age of those admitted was 15 years, compared to 17 years for those discharged (P < .001). In the admitted group, the mean initial hemoglobin (Hgb) was 6.3 g/dL compared to 12.0 g/dL in the discharged group (P < .0001). Only 23% of the discharged patients were released with medications; the remainder did not receive treatment. Anovulation was the etiology of HMB in the majority (56%) of both inpatients and outpatients. Of the 44 adolescents admitted to the hospital for HMB, 12 (27%) had a bleeding disorder (BD) and 32 (73%) did not. CONCLUSION: The majority of adolescents who presented to the emergency department for HMB were not anemic and did not receive any treatment. Of those admitted, almost one-third had an underlying BD, which is higher than previously reported.


Assuntos
Serviço Hospitalar de Emergência/estatística & dados numéricos , Hospitalização/estatística & dados numéricos , Menorragia/etiologia , Adolescente , Transtornos da Coagulação Sanguínea/complicações , Criança , Feminino , Transtornos Hemorrágicos/complicações , Humanos , Estudos Retrospectivos
17.
Res Vet Sci ; 125: 305-308, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31351200

RESUMO

Multiple myeloma (MM) is a tumor of plasma cells representing approximately 1% of all canine tumors. Clinical evident bleeding is often referred to as the main finding. The aim of the study was to evaluate the occurrence of clinical bleedings in dogs with MM and its prognostic implications compared to a population of dogs not affected by MM. Two groups of dogs (# 78 each) individually matched for breed, age and gender were considered. Group-1 (exposed) was affected by MM and group-2 (unexposed) was affected by other diseases. They were compared for bleeding and mortality at 90 days after diagnosis (relative risk, RR; attributable risk, AR). Among group-1, bleeding patients (B) were compared with non-bleeding patients (NB) in terms of mortality at 90 days (RR, AR). Incident cases of MM were 78/57,694 (0.13%). Signs of bleeding up to 30 days before the referral presentation were found in 33 (42.3%) group-1 dogs in comparison to 6 (7.7%) group-2 dogs (RR, 5.50, CI 95% 2.55-12.3, p = 0.0001; AR, 0.34, CI 95% 0.22-0.47, p = 0.0001). Epistaxis was the most frequent sign of bleeding recorded. Nineteen dogs from group-1 (24.3%) and eight from group-2 (10.2%) were non-survivors (RR = 2.37, CI 95% 1.14-5.06, p = 0.01; AR = 0.14, CI 95% 0.02-0.26, p = 0.01). Among the group-1, the B dogs, 4/33 (12.1%) were non-survivors, while 15/45 NB dogs (33.3%) were non-survivors (RR = 2.75, CI 95% 1.08-7.44, p = 0.03; AR = 0.21, CI 95% 0.20-0.38, p = 0.03). Epistaxis at diagnosis was frequent in MM dogs, and signs of bleeding were associated with a more favorable 90-day prognosis.


Assuntos
Doenças do Cão/epidemiologia , Transtornos Hemorrágicos/veterinária , Mieloma Múltiplo/veterinária , Animais , Estudos de Coortes , Doenças do Cão/diagnóstico , Doenças do Cão/etiologia , Cães , Feminino , Transtornos Hemorrágicos/complicações , Transtornos Hemorrágicos/diagnóstico , Transtornos Hemorrágicos/epidemiologia , Masculino , Mieloma Múltiplo/complicações , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/epidemiologia , Prevalência , Prognóstico
18.
World J Gastroenterol ; 25(8): 1031-1036, 2019 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-30833808

RESUMO

BACKGROUND: Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism, platelet storage pool deficiency and systemic complications associated with ceroid deposition in the reticuloendothelial system. HPS types 1 and 4 are associated with Crohn's disease (CD)-like gastrointestinal disorders, such as granulomatous enterocolitis or perianal disease. Cases of colitis can be particularly severe and, before the use of anti-tumor necrosis factor alpha (TNFα) therapy had become common, were reported as showing poor responsiveness to medical treatment. CASE SUMMARY: We present the case of a 51-year-old albino woman who presented with acute severe colitis that led to the diagnosis of HPS. Histologic findings of biopsy samples showed chronic inflammation with deep ulcerations, and granulomas without caseous necrosis. Molecular genetic analysis confirmed HPS type 1, with a homozygous 27 base-pair deletion in exon 20 of the HPS1 gene. Once the patient's bleeding diathesis was corrected by platelet transfusion, the granulomatous colitis responded dramatically to a medical treatment regimen that included corticosteroids, azathioprine and infliximab; this regimen is similar to that used in CD treatment. Although it remains unclear if the granulomatous enterocolitis in HPS is due to ceroid deposition or reflects the co-existence of CD and HPS, the fact that this case of HPS-related granulomatous colitis responded to the same therapeutic approach used in CD suggests that this type of colitis may result from HPS patients' genetic susceptibility to CD. CONCLUSION: We report a case of severe colitis that led to the diagnosis of HPS, which was responsive to azathioprine and infliximab.


Assuntos
Albinismo/complicações , Hemorragia Gastrointestinal/tratamento farmacológico , Transtornos Hemorrágicos/complicações , Síndrome de Hermanski-Pudlak/complicações , Imunossupressores/uso terapêutico , Doenças Raras/complicações , Choque Hemorrágico/tratamento farmacológico , Doenças do Colo Sigmoide/tratamento farmacológico , Azatioprina/uso terapêutico , Colite/diagnóstico , Colite/tratamento farmacológico , Colite/etiologia , Colo Sigmoide , Evolução Fatal , Feminino , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiologia , Humanos , Infliximab/uso terapêutico , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Choque Hemorrágico/diagnóstico , Choque Hemorrágico/etiologia , Doenças do Colo Sigmoide/diagnóstico , Doenças do Colo Sigmoide/etiologia , Sigmoidoscopia
19.
J Pediatr Adolesc Gynecol ; 32(2): 122-127, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30472382

RESUMO

STUDY OBJECTIVE: Heavy menstrual bleeding (HMB) is a common gynecological complaint among young women with up to 40% having experienced HMB. Bleeding disorders are increasingly being recognized in adolescents and young adults with HMB. The aim of this study was to determine the prevalence of bleeding disorders in adolescents with HMB, among patients who presented to the Queensland Statewide Paediatric and Adolescent Gynaecology Service between July 2007 and July 2017. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: The study was a retrospective review of 124 female adolescents aged 8 to 18 years with HMB who presented to the Queensland Paediatric and Adolescent Gynaecology Service, Brisbane, Australia. The primary outcome measure was diagnosis of a bleeding disorder, with secondary outcomes including iron deficiency and/or anemia and treatment modalities. RESULTS: Screening for bleeding disorders was performed in 77/124 (62.1%) of patients with HMB. Twenty-seven adolescents were diagnosed with a bleeding disorder, giving a prevalence of 27/124 (21.7%) in those with HMB, and 27/77 (35%) with HMB who were screened. Of these 35%, von Willebrand disease was the most common bleeding disorder, found in 14/27 (51.6%), followed by inherited platelet function disorders diagnosed in 9/27 (33.3%), thrombocytopenia (inherited or acquired) in 3/27 (11.1%), and Factor IX deficiency in 1/27 (3.7%). Iron deficiency and/or anemia was diagnosed in 53/107 (49.5%) of patients with HMB who were screened for this, and 19/27 (70.3%) of those diagnosed with a bleeding disorder. CONCLUSION: Adolescents with HMB who present to a tertiary pediatric and adolescent gynecology service should be screened for bleeding disorders, because of the considerably high prevalence in this at-risk population.


Assuntos
Transtornos Hemorrágicos/epidemiologia , Programas de Rastreamento/métodos , Menorragia/etiologia , Adolescente , Anemia/epidemiologia , Anemia/etiologia , Anemia/terapia , Criança , Feminino , Ginecologia , Transtornos Hemorrágicos/complicações , Transtornos Hemorrágicos/terapia , Humanos , Prevalência , Queensland/epidemiologia , Estudos Retrospectivos , Fatores de Risco
20.
Blood ; 132(20): 2134-2142, 2018 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-30429157

RESUMO

Heavy menstrual bleeding (HMB) is frequently reported by adolescents. The role of the hematologist is threefold in the evaluation of such patients: 1) perform a clinical and laboratory evaluation for an underlying bleeding disorder based on the degree of clinical suspicion, 2) identify and manage any concomitant iron deficiency, and 3) provide input to the referring provider regarding the management of HMB, particulary for patients with identified hemostatic defects. Several clues in the menstrual history should raise suspicion for an underlying bleeding disorder, such as menses lasting >7 days, menstrual flow which soaks >5 products daily or requires product change during the night, passage of large blood clots, or failure to respond to conventional therapies. A detailed personal and family history of other bleeding symptoms should also be obtained. Iron deficiency with and without anemia is commonly found in young women with HMB. Therefore, it is important to obtain not only a hemoglobin, but also a ferritin level, when evaluating these patients. Iron supplementation is often a key component of management in the adolescent with heavy menses, and is still needed even in those who have received packed red cell transfusions due to severe anemia. Strategies for decreasing menstrual blood flow are similar between adults and adolescents with heavy menses, with combined hormonal contraceptives recommended as first-line therapy. However, adolescent-specific considerations exist for many of these agents, and must be incorporated into shared decision making when selecting the most appropriate treatment.


Assuntos
Transtornos Hemorrágicos/diagnóstico , Menorragia/diagnóstico , Menorragia/etiologia , Adolescente , Anemia Ferropriva/etiologia , Anemia Ferropriva/terapia , Feminino , Hematologia/métodos , Transtornos Hemorrágicos/complicações , Transtornos Hemorrágicos/terapia , Humanos , Menorragia/terapia
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