RESUMO
Acquired hemophilia (AH) is an autoimmune hemostatic disorder mediated by autoantibodies directed against factor VIII: C. In 52% of cases, the cause is unknown or is not associated with other pathological entities; in the rest, there are concomitant factors: lupus, rheumatoid arthritis, cancer, pregnancy, and medications. In Mexico, there is not a registry of AH, and awareness of the disease among health personnel is low. The groups with the highest incidence are women of childbearing age and individuals older than 70 years. It is characterized by severe bleeding, especially after trauma and normal childbirth or cesarean delivery, and large ecchymoses in the trunk and extremities. The suspicion is simple, it just takes for sudden, severe hemorrhage and a prolonged activated partial thromboplastin time that is not corrected with plasma to concur in an individual. Treatment involves achieving hemostasis and eradicating the antibody. The former is achieved with recombinant activated factor VII or activated prothrombin complex concentrate. Cyclophosphamide, prednisone or rituximab are used to eradicate the antibody. Most cases of AH are not diagnosed, which translates into a high mortality rate. Given that awareness about the disease among physicians is low, it is not suspected, neither diagnosed, and nor is it treated. This document reviews the most recent data on AH and expands on its diagnosis and treatment.
La hemofilia adquirida (HA) es un trastorno hemostático autoinmune ocasionado por autoanticuerpos dirigidos contra el factor VIII: C. En 52 % de los casos, la causa se desconoce o no se asocia con otra entidad patológica; en el resto, existen factores concomitantes: lupus, artritis reumatoide, cáncer, embarazo y medicamentos. En México no existe registro ni conciencia de la enfermedad entre el personal de salud. Los grupos de mayor incidencia son las mujeres en edad reproductiva y los individuos mayores de 70 años. Se caracteriza por hemorragia grave, sobre todo posterior a traumatismos y parto o cesárea, y equimosis grandes en tronco y extremidades. La sospecha es simple, basta que concurran hemorragia súbita, grave y un TTPa prolongado que no se corrige con plasma. El tratamiento consiste en lograr la hemostasia y erradicar el anticuerpo; lo primero se logra con el factor VII activado recombinante o concentrado del complejo de protrombínico activado. La ciclofosfamida, prednisona o rituximab sirven para erradicar el anticuerpo. La mayoría de los casos no son diagnosticados y la mortalidad es alta. Ya que los médicos desconocen el problema, no se sospecha, no se diagnostica y no se trata. Este documento revisa los datos más recientes de la HA y abunda en el diagnóstico y tratamiento.
Assuntos
Autoanticorpos/imunologia , Fator VIII/imunologia , Hemofilia A/imunologia , Adulto , Idoso , Equimose/etiologia , Feminino , Hemofilia A/complicações , Hemofilia A/epidemiologia , Hemofilia A/terapia , Hemorragia/etiologia , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Gravidez , Complicações Hematológicas na Gravidez/etiologia , Prognóstico , Adulto JovemRESUMO
Resumen La hemofilia adquirida (HA) es un trastorno hemostático autoinmune ocasionado por autoanticuerpos dirigidos contra el factor VIII: C. En 52 % de los casos, la causa se desconoce o no se asocia con otra entidad patológica; en el resto, existen factores concomitantes: lupus, artritis reumatoide, cáncer, embarazo y medicamentos. En México no existe registro ni conciencia de la enfermedad entre el personal de salud. Los grupos de mayor incidencia son las mujeres en edad reproductiva y los individuos mayores de 70 años. Se caracteriza por hemorragia grave, sobre todo posterior a traumatismos y parto o cesárea, y equimosis grandes en tronco y extremidades. La sospecha es simple, basta que concurran hemorragia súbita, grave y un TTPa prolongado que no se corrige con plasma. El tratamiento consiste en lograr la hemostasia y erradicar el anticuerpo; lo primero se logra con el factor VII activado recombinante o concentrado del complejo de protrombínico activado. La ciclofosfamida, prednisona o rituximab sirven para erradicar el anticuerpo. La mayoría de los casos no son diagnosticados y la mortalidad es alta. Ya que los médicos desconocen el problema, no se sospecha, no se diagnostica y no se trata. Este documento revisa los datos más recientes de la HA y abunda en el diagnóstico y tratamiento.
Abstract Acquired hemophilia (AH) is an autoimmune hemostatic disorder mediated by autoantibodies directed against factor VIII: C. In 52% of cases, the cause is unknown or is not associated with other pathological entities; in the rest, there are concomitant factors: lupus, rheumatoid arthritis, cancer, pregnancy, and medications. In Mexico, there is not a registry of AH, and awareness of the disease among health personnel is low. The groups with the highest incidence are women of childbearing age and individuals older than 70 years. It is characterized by severe bleeding, especially after trauma and normal childbirth or cesarean delivery, and large ecchymoses in the trunk and extremities. The suspicion is simple, it just takes for sudden, severe hemorrhage and a prolonged activated partial thromboplastin time that is not corrected with plasma to concur in an individual. Treatment involves achieving hemostasis and eradicating the antibody. The former is achieved with recombinant activated factor VII or activated prothrombin complex concentrate. Cyclophosphamide, prednisone or rituximab are used to eradicate the antibody. Most cases of AH are not diagnosed, which translates into a high mortality rate. Given that awareness about the disease among physicians is low, it is not suspected, neither diagnosed, and nor is it treated. This document reviews the most recent data on AH and expands on its diagnosis and treatment.
Assuntos
Humanos , Masculino , Feminino , Gravidez , Adulto , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Autoanticorpos/imunologia , Fator VIII/imunologia , Hemofilia A/imunologia , Complicações Hematológicas na Gravidez/etiologia , Prognóstico , Equimose/etiologia , Hemofilia A/complicações , Hemofilia A/terapia , Hemofilia A/epidemiologia , Hemorragia/etiologia , Imunossupressores/uso terapêuticoRESUMO
Abstract Recent crisis and conflicts in African countries, the Middle East and the Americas have led to forced population migration and rekindled concern about food security. This article aims to map in the scientific literature the implications of forced migration on food and nutrition of refugees. Scoping Review, and database search: databases: PubMed Central, LILACS, SciElo, Science Direct and MEDLINE. Languages used in the survey were: English, Portuguese and Spanish, with publication year from 2013 to 2018. 173 articles were obtained and after removing of duplicates and full reading, 26 articles were selected and submitted to critical reading by two reviewers, resulting in 18 articles selected. From the analysis of the resulting articles, the following categories emerged: Food Inequity; Cultural Adaptation and Nutrition; Emerging Diseases and Strategies for the Promotion of Nutritional Health. Food insecurity is a marked consequence of forced international migration, and constitutes an emerging global public health problem, since concomitant with increasing population displacements also widens the range of chronic and nutritional diseases.
Resumo As recentes crises e conflitos em países Africanos, no Oriente Médio e Américas têm originado migração forçada de populações e reacende a preocupação com os cuidados com a alimentação. O objetivo deste artigo é mapear na literatura científica as implicações da migração forçada sobre a alimentação e nutrição de refugiados. Scoping Review, com buscas nas bases de dados: bases de dados: PubMed Central, LILACS, SciElo, Science Direct e MEDLINE. Os idiomas de pesquisa foram: Inglês, Português e Espanhol, e ano de publicação compreendido entre 2013 e 2018. Obteve-se 173 artigos e após remoção dos duplicados e leitura integral, 26 artigos foram selecionados e submetidos à leitura crítica por dois revisores independentes, resultando em 18 artigos selecionados. Da análise dos resultados dos artigos, emergiram as categorias: Iniquidade Alimentar; Adaptação Cultural e Nutrição; Doenças Emergentes e Estratégias de Promoção da Saúde Nutricional. A insegurança alimentar é consequência marcante da imigração internacional forçada, e se constitui um emergente problema de saúde pública global, pois concomitante aos crescentes deslocamentos populacionais também se ampliam a gama de doenças crônicas e nutricionais.
Assuntos
Humanos , Masculino , Feminino , Gravidez , Criança , Estado Nutricional , Emigração e Imigração/tendências , Abastecimento de Alimentos , Aculturação , Complicações Hematológicas na Gravidez/etiologia , Complicações Hematológicas na Gravidez/epidemiologia , Refugiados , Promoção da Saúde/métodos , Distúrbios Nutricionais/epidemiologiaRESUMO
Recent crisis and conflicts in African countries, the Middle East and the Americas have led to forced population migration and rekindled concern about food security. This article aims to map in the scientific literature the implications of forced migration on food and nutrition of refugees. Scoping Review, and database search: databases: PubMed Central, LILACS, SciElo, Science Direct and MEDLINE. Languages used in the survey were: English, Portuguese and Spanish, with publication year from 2013 to 2018. 173 articles were obtained and after removing of duplicates and full reading, 26 articles were selected and submitted to critical reading by two reviewers, resulting in 18 articles selected. From the analysis of the resulting articles, the following categories emerged: Food Inequity; Cultural Adaptation and Nutrition; Emerging Diseases and Strategies for the Promotion of Nutritional Health. Food insecurity is a marked consequence of forced international migration, and constitutes an emerging global public health problem, since concomitant with increasing population displacements also widens the range of chronic and nutritional diseases.
Assuntos
Aculturação , Emigração e Imigração/tendências , Abastecimento de Alimentos , Estado Nutricional , Refugiados , Criança , Feminino , Promoção da Saúde/métodos , Humanos , Masculino , Distúrbios Nutricionais/epidemiologia , Gravidez , Complicações Hematológicas na Gravidez/epidemiologia , Complicações Hematológicas na Gravidez/etiologiaRESUMO
The importance of the immunomodulatory effects of vitamin D has recently been associated with autoimmune and chronic inflammatory diseases. Vitamin D deficiency has been linked to the development of autoimmune conditions. Antiphospholipid syndrome is an autoimmune disease characterized by thrombotic events and obstetric complications in patients with antiphospholipid antibodies. Current data show that patients with antiphospholipid syndrome have a high prevalence of vitamin D deficiency even without classic risk factors. Several studies have suggested vitamin D may have anti-thrombotic functions. In antiphospholipid syndrome, low vitamin D serum levels have been associated with thrombotic manifestations, suggesting a possible protective role of vitamin D in antiphospholipid syndrome. This literature review presents current evidence on the haemostatic functions of vitamin D and their possible relationship with the clinical manifestations of antiphospholipid syndrome.
Assuntos
Síndrome Antifosfolipídica/complicações , Deficiência de Vitamina D/complicações , Vitamina D/metabolismo , Anticorpos Antifosfolipídeos/sangue , Anticoagulantes/uso terapêutico , Feminino , Humanos , Gravidez , Complicações Hematológicas na Gravidez/etiologia , Trombose/tratamento farmacológico , Trombose/etiologia , Deficiência de Vitamina D/tratamento farmacológicoRESUMO
BACKGROUND: According to data from the World Health Organization and UNICEF from year 2009, iron deficiency is the most widespread nutritional deficiency worldwide. This deficiency causes an imbalance between needs and iron supply, which consequently results in anemia. Around the world, two million people suffer from anemia, half of which is due to iron deficiency. The most impacted groups are children and teenagers, due to their highest requirements derived from the growing process, and women in their reproductive age, due to their loss of iron derived from menstruating or to their highest iron needs during pregnancy. This increase in needs is not satisfied by the regular diet, since it includes an insufficient amount and/or low bioavailability of iron. PURPOSE: To share with the medical community treating pregnant women the experience of an expert group so that they always bear in mind the repercussions caused by anemia during pregnancy, know more about the diagnostic possibilities and have a reference point for prescribing iron supplements. METHOD: The consensus method was used through the expert panel group technique. Two rounds were taken for structuring the clinical questions. The first one was to facilitate working groups their focusing in the clinical topics and the population of interest; the second one was to aid in posing specific questions observing the Patient, Intervention, Compare and Outcome (PICO) structure. The primary and clinical secondary study variables were defined by the working groups from the previously developed questions and during the face-to-face working period, according to the natural history of the disease: risk factors, diagnostic classification, (either pharmacological or non pharmacological) treatment and prognosis. The level of evidence and clinical recommendation was classified based on the Evidence Classification Level and Clinical Recommendation of the Medicine Group based on Evidence from Oxford University. RESULTS: In Mexico, 20.6% of pregnant women suffer from anemia, especially those between 15 and 16 years old, who prevail in 42.4% and 34.3% percent, respectively. Almost half the cases are due to iron deficiency. This type of anemia is associated with a higher risk of pre-term delivery, of low birth weight and perinatal death. The first assessment of an anemic pregnant woman shall include the medical history, a physical examination and the quantification of the erythrocyte indices, serum concentrations of iron and ferritin. The measurement of this last one has the highest sensitivity and specificity for diagnosing iron deficiency. Daily oral iron supplementation, at a 60-to-120 mg dosage, may correct most of mild-to-moderate anemias. The most appropriate treatment is with iron salts (iron sulfate, polimaltose iron complex or iron fumarate). In case of intolerance to iron sulfate or fumarate, polimaltose iron is a better tolerated option. Treatment shall be administered until the hemoglobin values are > 10.5 g and ferritin is between 300 and 360 microg/dL, and such levels shall be observed for at least one year. Parenteral administration is an alternative for patients with a severe intolerance to oral administration; even when the possibility of anaphylaxis shall be considered it is lower when using ferrous sacarate. Transfusion is reserved for patients with hemoglobin lower than 7 g/dL or having an imminent cardio-respiratory decompensation. CONCLUSIONS: Iron deficiency is the highest prevailing nutritional deficiency worldwide and its consequences during pregnancy may be highly risky for both the mother and her child. Anemia diagnosis may easily be achieved through a blood analysis including the serum ferritin determination. Serum iron measurement shall not be used as the only marker to set the diagnosis. It is important to rule out other causes, in addition to the deficiencies, which produce anemia in a patient. It is essential to suggest the administration of iron supplements not only during the antenatal period but also after birth o even after a miscarriage to fulfill the need for depleted iron. In severe anemias (hemoglobin being lower than 9.0 g/L), iron doses higher than 120 mg a day may be required. Treatment shall always begin orally, and if this is not well tolerated, parenteral administration shall be used.
Assuntos
Anemia/diagnóstico , Anemia/tratamento farmacológico , Complicações Hematológicas na Gravidez/diagnóstico , Complicações Hematológicas na Gravidez/tratamento farmacológico , Adolescente , Adulto , Anemia/classificação , Anemia/epidemiologia , Anemia/etiologia , Anemia/terapia , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/tratamento farmacológico , Anemia Ferropriva/epidemiologia , Anemia Ferropriva/etiologia , Anemia Ferropriva/terapia , Biomarcadores , Transfusão de Sangue , Medicina Baseada em Evidências , Feminino , Ferritinas/sangue , Morte Fetal/etiologia , Doenças Fetais/etiologia , Doenças Fetais/prevenção & controle , Ácido Fólico/administração & dosagem , Ácido Fólico/uso terapêutico , Saúde Global , Hemoglobinas/análise , Humanos , Recém-Nascido , Ferro/administração & dosagem , Ferro/efeitos adversos , Ferro/sangue , Ferro/uso terapêutico , Ferro da Dieta/farmacocinética , México/epidemiologia , Pessoa de Meia-Idade , Cuidado Pós-Natal/métodos , Cuidado Pós-Natal/normas , Gravidez , Complicações Hematológicas na Gravidez/classificação , Complicações Hematológicas na Gravidez/epidemiologia , Complicações Hematológicas na Gravidez/etiologia , Complicações Hematológicas na Gravidez/terapia , Cuidado Pré-Natal/métodos , Cuidado Pré-Natal/normas , Prevalência , Fatores de Risco , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Adulto JovemAssuntos
Hematoma Subdural/etiologia , Complicações Hematológicas na Gravidez/etiologia , Púrpura Trombocitopênica Idiopática/complicações , Encéfalo/patologia , Feminino , Hematoma Subdural/diagnóstico por imagem , Humanos , Recém-Nascido , Gravidez , Complicações Hematológicas na Gravidez/diagnóstico por imagem , Ultrassonografia Pré-Natal/métodosRESUMO
Hemodialysis is the gold standard for substitution of renal function in women with chronic or acute renal insufficiency during pregnancy or during the postpartum period. Perinatal complications are frequent. Recognition of appropriate techniques in medical treatment of patients will contribute to decrease its incidence and allow better perinatal results. We undertook this study to review the techniques and medical indications of hemodialysis in pregnant and postpartum patients. This condition requires care in a high-specialty medical center. We recommend incremental increases in intensity and frequency and duration of treatments, use of equipment to manage fluctuations in blood and dialysis fluids, new filters, microdose of heparin and limiting ultrafiltration to avoid hemodynamic, electrolytic, and metabolic alterations, obstetrical hemorrhage or premature delivery. The objective is to maintain a satisfactory clinical status and maternal blood, urea, nitrogen (BUN) levels =80 mg/dl and creatinine 5-7 mg/dl for opportune fetal development and birth. Routine pharmacological treatment should continually be individually adjusted as to number of medications and dosage. Recognition of hemodialysis techniques and indications will contribute to obtaining improved perinatal results.
Assuntos
Complicações na Gravidez/terapia , Transtornos Puerperais/terapia , Diálise Renal/métodos , Insuficiência Renal/terapia , Aborto Espontâneo/etiologia , Aborto Espontâneo/prevenção & controle , Anemia/tratamento farmacológico , Anemia/etiologia , Feminino , Doenças Fetais/etiologia , Doenças Fetais/prevenção & controle , Hospitais Especializados , Humanos , Recém-Nascido , Trabalho de Parto Prematuro/etiologia , Trabalho de Parto Prematuro/prevenção & controle , Poli-Hidrâmnios/etiologia , Poli-Hidrâmnios/prevenção & controle , Gravidez , Complicações Hematológicas na Gravidez/tratamento farmacológico , Complicações Hematológicas na Gravidez/etiologia , Resultado da Gravidez , Gravidez de Alto Risco , Cuidado Pré-Natal/métodos , Diálise Renal/efeitos adversos , Diálise Renal/instrumentação , Insuficiência Renal/sangueRESUMO
La hemodiálisis es el método de primera elección para sustituir la función renal en mujeres con insuficiencia renal crónica o aguda durante el embarazo o posparto. Las complicaciones perinatales son frecuentes, sin embargo, el conocimiento de la adecuación de la técnica en el tratamiento médico de las pacientes puede contribuir a la reducción de su presentación y mejorar los resultados perinatales. El objetivo de esta investigación fue revisar la técnica y prescripción médica de la hemodiálisis en la mujer durante el embarazo o posparto. Este tipo de enfermas debe atenderse en un centro de alta especialidad. Se recomienda la hemodiálisis intensa incrementando la frecuencia y duración de las sesiones, utilizar aparatos que manejan altos flujos de sangre y líquido de diálisis, filtros nuevos, microdosis de heparina y limitar la ultrafiltración para evitar cambios hemodinámicos, hidroelectrolíticos, metabólicos, hemorragia obstétrica o parto pretérmino. El objetivo es mantener un estado clínico satisfactorio y niveles sanguíneos maternos de nitrógeno de la urea ≤ 80 mg/dl y creatinina 5 a 7 mg/dl para favorecer el crecimiento y desarrollo fetales. El tratamiento farmacológico rutinario debe continuarse, ajustando el número de medicamentos y su dosis de manera individual. El conocimiento de la técnica de hemodiálisis y su prescripción pueden contribuir a lograr mejores resultados perinatales.
Hemodialysis is the gold standard for substitution of renal function in women with chronic or acute renal insufficiency during pregnancy or during the postpartum period. Perinatal complications are frequent. Recognition of appropriate techniques in medical treatment of patients will contribute to decrease its incidence and allow better perinatal results. We undertook this study to review the techniques and medical indications of hemodialysis in pregnant and postpartum patients. This condition requires care in a high-specialty medical center. We recommend incremental increases in intensity and frequency and duration of treatments, use of equipment to manage fluctuations in blood and dialysis fluids, new filters, microdose of heparin and limiting ultrafiltration to avoid hemodynamic, electrolytic, and metabolic alterations, obstetrical hemorrhage or premature delivery. The objective is to maintain a satisfactory clinical status and maternal blood, urea, nitrogen (BUN) levels =80 mg/dl and creatinine 5-7 mg/dl for opportune fetal development and birth. Routine pharmacological treatment should continually be individually adjusted as to number of medications and dosage. Recognition of hemodialysis techniques and indications will contribute to obtaining improved perinatal results.