Doença falciforme: cuidado com pessoas com úlcera da perna nos serviços de atenção à saúde / Sickle cell disease: caring for people with leg ulcers in heal-thcare services / Enfermedad falciforme: cuidado de personas con úlcera de pierna en los servicios de atención a la salud

Objetivo: identificar o local e os cuidados diretos recebidos por pessoas com úlceras da perna por doença falciforme nos serviços de atenção à saúde. Método: estudo transversal, realizado em 11 centros, no período de agosto de 2019 a abril de 2020. Fizeram parte do estudo 72 pessoas com úlcera da perna ativa. O estudo foi aprovado pelo Comitê de Ética em Pesquisa. Resultado: apresentavam anemia falciforme 91,7% dos participantes, com mediana de três anos de existência da úlcera; 77,8% eram redicivantes; 40,3% compravam os insumos; 66,7% trocavam o próprio curativo no domicílio; 52,8% realizavam uma ou mais trocas diárias; 45,8% dos tratamentos foram prescritos pelo médico; 37,5% eram pomada (colagenase ou antibiótico); 89% não utilizavam compressão para o manejo do edema. Conclusão: a maioria dos participantes não estava inserida na Rede de Atenção à Saúde para o tratamento da úlcera, e não recebia assistência sistematizada e nem insumos apropriados.

Objective: to identify the location and direct care received by people with leg ulcers due to sickle cell disease in health care services. Method: a cross-sectional study carried out in 11 centers from August 2019 to April 2020. The study included 72 people with active leg ulcers. The study was approved by the Research Ethics Committee. Results: a total of 91.7% of the participants had sickle cell anemia, with a median of three years of ulcer existence; 77.8% were recurrent; 40.3% bought the supplies; 66.7% changed their own dressings at home; 52.8% did one or more changes a day; 45.8% of the treatments were prescribed by physician; 37.5% were ointments (collagenase or antibiotics); and 89% did not use compression to manage edema. Conclusion: most of the participants were not included in the Health Care Network for ulcer treatment and did not receive systematized care or appropriate supplies.

Objetivo: identificar el lugar y los cuidados directos recibidos por personas con úlceras de pierna por enfermedad falciforme en los servicios de atención a la salud. Método: estudio transversal, realizado en 11 centros, en el período de agosto de 2019 a abril de 2020. Participaron 72 personas con úlcera de pierna activa. El estudio fue aprobado por el Comité de Ética en Investigación. Resultado: presentaban anemia falciforme 91,7% de los participantes, con una mediana de tres años de existencia de la úlcera; 77,8% eran recidivantes; 40,3% compraban los insumos; 66,7% cambiaban su propio vendaje en el domicilio; 52,8% realizaban uno o más cambios diarios; 45,8% de los tratamientos fueron prescritos por el médico; 37,5% eran pomada (colagenasa o antibiótico); y 89% no utilizaban compresión para el manejo del edema. Conclusión: la mayoría de los participantes no estaba integrada en la Red de Atención a la Salud para el tratamiento de la úlcera, y no recibía asistencia sistematizada ni insumos apropiados.

Relationship of hemoglobin levels with outcomes in deceased donor kidney transplant: a retrospective cohort study / Relação dos níveis de hemoglobina com desfechos em transplante renal de doador falecido: um estudo de coorte retrospectivo

ABSTRACT Introduction: Anemia is frequent in patients undergoing replacement therapy for kidney failure. Anemia in the pre- and post-transplantation period might be related to kidney transplant outcomes. The current study therefore sought to assess the relationship between anemia, delayed allograft function (DGF), chronic kidney allograft dysfunction (CAD), and death from any cause following kidney transplantation from a deceased donor. Methods: This was a retrospective study with 206 kidney transplant patients of deceased donors. We analyzed deceased donors' and kidney transplant patients' demographic data. Moreover, we compared biochemical parameters, anemia status, and medicines between DGF and non-DGF groups. Afterward, we performed a multivariate analysis. We also evaluated outcomes, such as CAD within one year and death in ten years. Results: We observed a lower frequency of pre-transplant hemoglobin concentration (Hb) but higher frequency of donor-serum creatinine and red blood transfusion within one week after transplantation in the group with DGF. In addition, there was an independent association between Hb concentration before transplantation and DGF [OR 0.252, 95%CI: 0.159-0.401; p < 0.001]. There was also an association between Hb concentration after six months of kidney transplantation and both CAD [OR 0.798, 95% CI: 0.687-0.926; p = 0.003] and death from any cause. Conclusion: An association was found between pre-transplantation anemia and DGF and between anemia six months after transplantation and both CAD and death by any cause. Thus, anemia before or after transplantation affects the outcomes for patients who have undergone kidney transplantation from a deceased donor.

RESUMO Introdução: A anemia é frequente em pacientes submetidos à terapia substitutiva para insuficiência renal. A anemia nos períodos pré e pós-transplante pode estar relacionada aos desfechos do transplante renal. Portanto, o presente estudo buscou avaliar a relação entre anemia, função retardada do enxerto (FRE), disfunção crônica do enxerto renal (DCE) e óbito por qualquer causa após transplante renal de doador falecido. Métodos: Este foi um estudo retrospectivo com 206 pacientes transplantados renais de doadores falecidos. Analisamos dados demográficos de doadores falecidos e pacientes transplantados renais. Além disso, comparamos parâmetros bioquímicos, status de anemia e medicamentos entre os grupos FRE e não-FRE. Posteriormente, realizamos uma análise multivariada. Também avaliamos desfechos, como DCE em um ano e óbito em dez anos. Resultados: Observamos menor frequência de concentração de hemoglobina (Hb) pré-transplante, mas maior frequência de creatinina sérica do doador e transfusão de hemácias no período de uma semana após o transplante no grupo FRE. Além disso, houve associação independente entre a concentração de Hb antes do transplante e a FRE [OR 0,252; IC 95%: 0,159-0,401; p < 0,001]. Houve também associação entre a concentração de Hb após seis meses de transplante renal e ambos, DCE [OR 0,798; IC95%: 0,687-0,926; p = 0,003] e óbito por qualquer causa. Conclusão: Encontrou-se uma associação entre anemia pré-transplante e FRE e entre anemia seis meses após o transplante e ambos, DCE e óbito por qualquer causa. Assim, a anemia antes ou após o transplante afeta os desfechos de pacientes que foram submetidos a transplante renal de doador falecido.

Etiología y evolución de la anemia en pacientes sometidos a cirugía cardiovascular / Etiology and evolution of anemia in patients submitted to cardiovascular surgery

Resumen Objetivo: El objetivo del presente estudio es evaluar en nuestro medio la prevalencia de anemia en el preoperatorio de la cirugía cardiovascular, su incidencia postoperatoria y su evolución durante el primer mes. Métodos: Se realizó un estudio de cohorte prospectivo en el que se incluyeron todos los pacientes sometidos a cirugía cardiovascular central intervenidos durante el periodo del 01/09/2021 al 01/09/2022 en un hospital universitario. Se realizó seguimiento clínico y de laboratorio previo a la cirugía, al quinto y al día treinta del postoperatorio. Se comparó a los grupos con y sin anemia preoperatoria. Resultados: La prevalencia de anemia en el preoperatorio fue del 32.1%. La incidencia de anemia en el postoperatorio fue del 96% en el grupo de pacientes sin anemia previa. Al mes de la cirugía un 73 y un 90% de los pacientes, con y sin anemia preoperatoria respectivamente, persistían anémicos. Los pacientes con anemia preoperatoria tuvieron una menor recuperación de sus valores de hemoglobina al mes. Se observó una tendencia a mayor mortalidad y una mayor necesidad de derivación a centros de rehabilitación postegreso hospitalario en aquellos con anemia preoperatoria. Conclusiones: En este trabajo se evidenció una alta prevalencia e incidencia de anemia en el perioperatorio de las cirugías cardiovasculares. Así como su subtratamiento y elevada persistencia durante el mes posterior a la cirugía.

Abstract Objective: The aim of this study is to evaluate the prevalence of anemia in the preoperative period of cardiovascular surgery, its postoperative incidence and its evolution during the first month in our setting. Methods: A prospective cohort study was carried out in which all patients undergoing central cardiovascular surgery operated during the period 09/01/2021-09/01/2022 in a university hospital were included. Clinical and laboratory follow-up was carried out prior to surgery, on the fifth and on the 30th postoperative day. Groups with and without preoperative anemia were compared. Results: The prevalence of anemia in the preoperative period was 32.1%. The incidence of anemia in the postoperative period was 96% in the group of patients without previous anemia. One month after surgery, 73 and 90% of the patients, with and without preoperative anemia, respectively, remained anemic. Patients with preoperative anemia had less recovery of their hemoglobin values at one month. A trend towards higher mortality and a greater need for referral to post-hospital discharge rehabilitation centers was observed in those with preoperative anemia. Conclusions: In this work, a high prevalence and incidence of anemia in the perioperative period of cardiovascular surgeries was evidenced. As well as its subtreatment and high persistence during the month after surgery.

Anemia aplásica inducida por metimazol: presentación de caso / Methimazole Induced Aplastic Anemia: Case Report / Anemia aplástica induzida por metimazol: relato de caso

Introducción: los medicamentos antitiroideos son una de las alternativas terapéuticas en el tratamiento de la enfermedad de Graves. Sin embargo, pueden generar efectos adversos severos poco frecuentes en el plano hematológico, como la anemia aplásica, la cual se ha asociado con altas dosis de estos medicamentos, aunque con reversión de esta afección ante el retiro del medicamento. Descripción del caso: mujer de 38 años con antecedente de enfermedad de Graves en tratamiento con metimazol, quien consultó por síntomas como epistaxis anterior de difícil control, petequias, astenia e hiporexia. Se documentó pancitopenia en el hemo-grama, con posterior hallazgo en biopsia de médula ósea de aplasia medular, sin respuesta ante el retiro del metimazol y soporte transfusional. Posteriormente, la paciente falleció. Conclusión: la presentación de aplasia medular asociada con metimazol es poco común y se relaciona con altas dosis de este medicamento. En la mayoría de casos, el retiro de este agente genera recuperación clínica y celular. No obstante, en algu-nos pacientes persiste el compromiso hematológico que va desde importantes repercusiones clínicas hasta desenlaces fatales. Por lo tanto, el presente caso busca hace hincapié en la importancia de vigilar este efecto adverso ante el inicio de esta medicación

Introduction: Antithyroid drugs are one of the therapeutic alternatives in the treatment of Graves' dis-ease. However, it can generate severe but infrequent adverse effects at the hematological level, such as aplastic anemia, which has been associated with high doses of these drugs, although with reversal of this hematological condition when the drug is withdrawn. Case description: A 38-year-old woman with a his-tory of Graves' disease treated with methimazole, who consult for symptoms such as anterior epistaxis, petechiae, asthenia, and hyporexia. Pancytopenia is documented in the blood count, with a subsequent finding of bone marrow aplasia in bone marrow biopsy, without response to withdrawal of Methimazole and transfusion support. The patient subsequently died. Conclusion: The methimazole-associated bone marrow aplasia is uncommon and it ́s associated with high doses of methimazole, in most cases with-drawal of methimazole leads to clinical and cellular recovery. However, in some patients hematological involvement persists with significant clinical repercussions up to fatal outcomes. Therefore, this case seeks to highlight the importance of monitoring for this adverse effect before starting this medication

Introdução: as drogas antitireoidianas são uma das alternativas terapêuticas no tratamento da doença de Graves. No entanto, pode causar efeitos adversos graves, mas infrequentes, no nível hematológico, como a anemia aplástica, que tem sido associada a altas doses desses medicamentos, embora com rever-são desse quadro hematológico quando a droga é retirada. Descrição do caso: mulher de 38 anos com história de doença de Graves tratada com metimazol, que consultou por sintomas como epistaxe ante-rior de difícil controle, petéquias, astenia e hiporexia. A pancitopenia é documentada no hemograma, com achado posterior de aplasia da medula óssea na biópsia da medula óssea, sem resposta à retirada do metimazol e suporte transfusional. O doente faleceu posteriormente. Conclusão: a apresentação de aplasia da medula óssea associada ao metimazol é pouco frequente em associação com doses elevadas de metimazol. Na maioria dos casos, a retirada do metimazol conduz à recuperação clínica e celular. No entanto, nalguns doentes, o envolvimento hematológico persiste com repercussões clínicas significati-vas, podendo mesmo ocorrer desfechos fatais. Assim, o presente caso pretende realçar a importância da monitorização deste efeito adverso antes de iniciar esta medicação

Conmemorando los cincuenta años de Leche Purita: importancia de la leche en la erradicación de la desnutrición infantil en Chile

La desnutrición ocurre cuando hay una ingesta o asimilación deficiente de la energía y los nutrientes. Si esta se produce dentro de los primeros años de vida, las consecuencias para la salud son devastadoras. El tratamiento temprano de la desnutrición es clave para reducir estas consecuencias y en este proceso la leche tiene una participación destacada. La leche es un excelente alimento debido al contenido y calidad de sus macro- y micronutrientes, lo que ha llevado a que la investigación y el uso de la leche en el tratamiento de la desnutrición hayan aumentado sostenidamente desde comienzos del siglo XX. En Chile, la desnutrición infantil fue prácticamente erradicada entre los años 1960 y 1980 debido a la aplicación exitosa de una serie de políticas públicas materno-infantiles, dentro de las cuales la Leche Purita fue fundamental. Sin embargo, la historia de la leche como parte de las políticas nutricionales en Chile comenzó mucho antes. Conmemorando los cincuenta años desde el nacimiento de Leche Purita, el objetivo de este trabajo es realizar un breve repaso acerca de la importancia histórica de la leche en las políticas nutricionales en Chile, enfatizando la contribución realizada por la Leche Purita a la erradicación de la desnutrición infantil y a la disminución de la anemia en niños.

Undernutrition occurs when there is poor intake or assimilation of energy and nutrients. If undernutrition is established within the first years of life, the health consequences are devastating. Early treatment of undernutrition is critical to reduce these consequences and milk plays an important role in this treatment. Cow's milk is an excellent food due to the content and quality of its macro- and micronutrients. This has led to a steady increase in research and the use of milk in treating undernutrition since the beginning of the 20th century. In Chile, child undernutrition was practically eradicated between the decades of 1960 and 1980 due to the successful application of maternal and child public policies, within which the product Leche Purita was fundamental. However, the history of milk as part of nutritional policies in Chile began much earlier. Commemorating fifty years since the introduction of Leche Purita, we aim to summarize the historical importance of milk in Chile's nutritional policies. We emphasize Leche Purita's role in eradicating child undernutrition and reducing anemia among children.

Factores asociados a anemia ferropénica en lactantes y preescolares / Factors associated with iron deficiency anemia in infants and preschoolers

Introducción: La anemia se define como una afección en la cual el número de glóbulos rojos o la concentración de hemoglobina dentro de estos se encuentran por debajo del límite inferior, 11 gr/dl para menores entre 6 a 59 meses. La OMS calcula que a nivel mundial la anemia presenta una prevalencia del 42% en los niños menores de 5 años. Objetivos: Realizar una revisión panorámica de diversas publicaciones científicas acerca de los factores asociados a la anemia ferropénica en preescolares. Materiales y métodos: La revisión se realizó a través de la búsqueda electrónica de diversos artículos científicos relacionados con el tema. Se utilizó la pregunta PEO: ¿Cuáles son los factores asociados a anemia ferropénica en lactantes y preescolares? Se seleccionaron los artículos publicados desde el 2017 hasta el 2022. Resultados: De los 48 artículos encontrados en la revisión, 33 fueron descartados por no cumplir con nuestros criterios de selección, quedando 15 artículos para esta revisión. Basado en 12 artículos revisados, se halló que los factores que se asocian a anemia en menores de 5 años eran por problemas socioeconómicos, déficit de micronutrientes altos en hierro en dieta, madre con antecedente de anemia, la edad materna, falta de educación, entre otros. Conclusión: Los factores predisponentes para la presencia de anemia ferropénica en los menores de 5 años más importantes fueron los relacionados con la edad materna, el nivel socioeconómico y educativo de la madre que conllevan el déficit de hierro del menor.

Introduction: Anemia is defined as a condition in which the number of red blood cells or the hemoglobin concentration within red blood cells is below the lower limit, 11 g/dL for children aged 6-59 months. The WHO estimates that worldwide anemia has a prevalence of 42% in children under 5 years of age. Objectives: To perform an overview review of various scientific publications on the factors associated with iron deficiency anemia in preschoolers. Materials and methods: The review was carried out through an electronic search of various scientific articles related to the subject. The PEO question was used: What are the factors associated with iron deficiency anemia in infants and preschoolers? Articles published from 2017 to 2022 were selected. Results: Of the 48 articles found in the review, 33 were discarded for not meeting our selection criteria, leaving 15 articles for this review. Based on 12 articles reviewed, it was found that the factors associated with anemia in children under 5 years of age were socioeconomic problems, deficiency of micronutrients high in iron in the diet, mothers with a history of anemia, maternal age, lack of education, among others. Conclusion: The most important predisposing factors for the presence of iron deficiency anemia in children under 5 years of age were those related to maternal age, socioeconomic and educational level of the mother that lead to iron deficiency in the child.

Utilidad de los anticuerpos anti-células parietales gástricas y anti-factor intrínseco en el estudio de la deficiencia de vitamina B12 asociada a gastritis autoinmune y anemia perniciosa / Usefulness of anti-gastric parietal cell and anti-intrinsic factor antibodies in the study of vitamin B12 deficiency associated with autoimmune gastritis and pernicious anemia / Utilidade de anticorpos anti-células parietais gástricas e anti-fator intrínseco no estudo da deficiência de vitamina B12 associada à gastrite autoimune e à anemia perniciosa

Resumen La gastritis autoinmune (GAI) es una afección inflamatoria progresiva de la mucosa oxíntica caracterizada por la destrucción de células parietales, pérdida de factor intrínseco, malabsorción de vitamina B12 (cobalamina), hierro y otros micronutrientes y puede progresar hacia un estado avanzado de anemia megaloblástica conocida como anemia perniciosa (AP). El objetivo de este estudio fue determinar la deficiencia de vitamina B12 debida a malabsorción utilizando la detección de anticuerpos anti-células parietales gástricas (ACPG) y anti-factor intrínseco (AFI). Se analizaron 2050 sueros de pacientes con un inmunoanálisis quimioluminiscente para vitamina B12 total y 2,8% de éstos con las pruebas de inmunofluorescencia indirecta para ACPG y enzimoinmunoanálisis para AFI. La deficiencia de vitamina B12 (<200 ng/mL) fue del 13,1%. En la detección de anticuerpos se encontró: 2 doble positivos ACPG/AFI, 17 simple positivos ACPG y 4 simple positivos AFI. Todas las muestras ACPG y/o AFI positivas tuvieron valores de vitamina B12 total <200 ng/mL. En 5 pacientes con ACPG positivos se diagnosticó gastritis crónica confirmada por biopsia. En los 6 pacientes AFI positivos se realizó el diagnóstico de AP y en 2 de ellos se confirmó por histopatología. La positividad de ACPG y/o AFI permitió la clasificación de pacientes con sospecha de GAI en candidatos para la examinación histológica y la aplicación de esquemas terapéuticos adecuados. Se destaca la importancia de las pruebas de laboratorio como parte de una estrategia de diagnóstico temprano y vigilancia endoscópica, para evitar las manifestaciones relacionadas con la deficiencia de hierro y vitamina B12 y las complicaciones de la enfermedad avanzada.

Abstract Autoimmune gastritis (AIG) is a progressive inflammatory condition of the oxyntic mucosa, characterised by gastric parietal cell destruction, loss of intrinsic factor, and malabsorption of vitamin B12 (cobalamin), iron and other micronutrients; conditioning progress to a state of megaloblastic anemia known as pernicious anemia (PA). The aim of this study was to determine vitamin B12 deficiency due to malabsorption utilizing anti-parietal cell (APCA) and anti-intrinsic factor (IFA) antibodies detection. 2050 patient serum samples were analised by chemiluminescent immunoassay for vitamin B12. A total of 2.8% of them were tested for APCA by indirect immunofluorescence and for IFA by enzyme immunoessay. Vitamin B12 deficiency (<200 ng/mL) was 13.1%. Regarding antibody detection: 2 APCA/IFA double positives, 17 APCA simple positives and 4 IFA simple positives were found. APCA and/or IFA positive samples had total vitamin B12 values <200 ng/mL. Chronic gastritis confirmed by biopsy was diagnosed in 5 patients with positive ACPG antibodies. All 6 IFA positive patients were diagnosed with PA, while 2 of them also received histopatologic confirmation. APCA and/or IFA confirmation allowed for the classification of patients with suspicion of AIG as possible candidates for histologic examination and application of appropriate therapeutic schemes. Importance of laboratory testing is to be noted; as part of a strategy that enables early diagnosis and adequate endoscopic surveillance, to avoid manifestations related to iron and vitamin B12 deficiency and the complications of advanced disease.

Resumo A gastrite autoimune (GAI) é uma doença inflamatória progressiva da mucosa oxíntica, caracterizada pela destruição das células parietais gástricas, perda do fator intrínseco, má absorção de vitamina B12 (cobalamina), ferro e outros micronutrientes pode progredir para um estado avançado de anemia megaloblástica conhecida como anemia perniciosa (AP). O objetivo deste estudo foi determinar a deficiência de vitamina B12 por má absorção usando a detecção de anticorpos anti-células parietais gástricas (ACPG) e anti-fator intrínseco (AFI). Foram analisados 2050 soros de pacientes com um imunoensaio quimioluminiscente para vitamina B12 total, 2,8% deles com testes de imunofluorescência indireta para ACPG e enzimaimunoensaio para AFI. A deficiência de vitamina B12 (<200 ng/mL) foi de 13,1%. Na detecção de anticorpos foram encontrados: 2 duplo positivos ACPG/AFI, 17 simples positivos ACPG e 4 simples positivos AFI. Todas as amostras ACPG e/ou AFI positivas apresentaram valores de vitamina B12 total <200 ng/mL. Gastrite crônica confirmada por biópsia foi diagnosticada em 5 pacientes positivos para ACPG. Nos 6 pacientes AFI positivos o diagnóstico de AP foi feito e em 2 deles foi confirmado por histopatologia. A positividade para ACPG e/ou AFI permitiu a classificação de pacientes com suspeita de GAI em candidatos para exame histológico e a aplicação de esquemas terapêuticos adequados. Destaca-se a importancia dos testes laboratoriais, como parte de uma estratégia de diagnóstico precoce e vigilância endoscópica, para evitar manifestações relacionadas à deficiência de ferro e vitamina B12 e complicações da doença avançada.

Riesgos y beneficios de la profilaxis con sulfato ferroso en lactantes nacidos a término con niveles de hemoglobina y ferremia desconocidos / Risks and benefits of iron supplementation in full-term infants with unknown hemoglobin and ferritin levels

La seguridad y eficacia de los programas de suplementación con hierro a lactantes, está actualmente en discusión. El objetivo de esta revisión fue identificar estudios sobre riesgos y beneficios de la suplementación con hierro profiláctico en lactantes menores de un año, nacidos a término, con niveles de hemoglobina (Hb) y ferremia desconocidos. Se realizó una búsqueda en Pubmed y Cochrane, identificando 3 revisiones sistemáticas y metaanálisis. Estos estudios arrojaron resultados que indican mejoras en los niveles séricos de hierro y hemoglobina como resultado de la suplementación con hierro. Sin embargo, no se observó un beneficio significativo en el desarrollo cognitivo de los lactantes. Los efectos adversos más reportados son los gastrointestinales, efectos en el crecimiento (menor ganancia de talla y peso) y menor absorción de zinc. En resumen, la evidencia en cuanto a la profilaxis con hierro en lactantes es limitada, lo que nos lleva a recomendar un seguimiento cercano de los lactantes que reciben suplementos de hierro, con el objetivo de detectar posibles eventos adversos. Es fundamental evaluar cuidadosamente los riesgos y beneficios de esta intervención antes de su implementación (AU)

The safety and efficacy of iron supplementation programs for infants are currently under discussion. The objective of this review was to identify studies on the risks and benefits of prophylactic iron supplementation in infants under one year of age, born at term, with unknown hemoglobin (Hb) and serum iron levels. The search was conducted on Pubmed and Cochrane, identifying three systematic reviews and meta-analyses. The results indicate improvements in serum iron and hemoglobin levels as a result of iron supplementation. However, a significant benefit in infant cognitive development was not observed. The most reported adverse effects were gastrointestinal, effects on growth (reduced height and weight gain), and reduced zinc absorption. In summary, the evidence regarding iron prophylaxis in infants is limited, leading us to recommend close monitoring of infants receiving iron supplements to detect potential adverse events. It is crucial to carefully assess the risks and benefits of this intervention before implementation (AU)

Síndrome torácico agudo por drepanocitosis como causa de muerte súbita: Reporte de un caso y revisión de literatura / Acute chest syndrome due to sickle cell disease as a cause of sudden death: Case report and literature review

Resumen Introducción: La anemia de células falciformes se define como la hemoglobinopatía estructural autosómica recesiva más prevalente en todo el mundo, puede presentar fenómenos vasooclusivos y la anemia hemolítica que son los responsables de las principales complicaciones de esta enfermedad, una de estas es el síndrome torácico agudo, siendo una causa importante de morbilidad y mortalidad en estos pacientes. Objetivo: correlacionar los hallazgos de la autopsia médico legal, con las generalidades de la fisiopatología de la anemia de células falciformes actualizadas y disponibles. Materiales y métodos: Se realizó el análisis del protocolo de la autopsia médico legal efectuada, y revisión bibliográfica en un caso de una femenina de 37 años que en apariencia padecía de "dolor por nervio ciático", quien falleció en su casa de habitación, tras presentar un dolor intenso localizado en la espalda y disnea. Tras la autopsia médico legal se estableció como causa de muerte el síndrome torácico agudo por drepanocitosis. Conclusiones: La anemia de células falciformes se debe tener presente al momento de la valoración médico legal como una posible causa de muerte, para ello es necesario conocer la fisiopatología de esta enfermedad, y poder identificar dichas afectaciones al momento de realizar la autopsia médico legal, para esto es necesario contar con todas las herramientas posibles para poder realizar un diagnóstico certero.

Abstract Objective: To correlate the findings of the medical legal autopsy carried out with the updated and available generalities of the pathophysiology of sickle cell anemia. Materials and methods: The analysis of the protocol of the medical-legal autopsy carried out, and a bibliographic review, were carried out. A 37-year-old female who apparently suffered from "sciatic nerve pain," who died in her home after experiencing intense pain located in her back and dyspnea. After the medical-legal autopsy, acute chest syndrome due to sickle cell disease was established as the cause of death. Conclusions: Sickle cell anemia must be kept in mind at the time of the medical-legal evaluation as a possible cause of death, for this it is necessary to know the pathophysiology of this disease, and to be able to identify these effects when performing the Medical- Legal Autopsy, for this It is necessary to have all the possible tools to be able to make an accurate diagnosis.

Asociación del bajo peso al nacer con el control prenatal, la anemia materna y ser madre soltera

Fundamento: la elevada frecuencia de recién nacidos con peso inferior al adecuado, en relación con la anemia materna, la atención prenatal insuficiente y la condición de madre soltera, puede tener importantes repercusiones en la salud de las madres y los niños. Objetivo: identificar la relación entre el control prenatal, la anemia materna y la condición de ser madre soltera con el bajo peso al nacer de los infantes. Método: se realizó un estudio observacional, analítico y retrospectivo de casos y controles en gestantes atendidas en un hospital de la región Sierra de Ecuador durante el año 2022. La muestra fue de 280 pacientes. El grupo de casos lo conformaron las gestantes con hijos con bajo peso al nacer (n = 70) y el grupo de control por gestantes con recién nacidos con peso normal (n = 210). Se realizó la prueba de Chi cuadrado de independencia y se calculó el Odds Ratio y su intervalo de confianza del 95 % para el nivel de asociación entre las variables. Resultados: la relación de la variable asistencia a controles prenatales con respecto al bajo peso al nacer mostró una asociación protectora (OR = 0,5). Se identificaron como factores de riesgo, la anemia materna (OR = 3,1) y la condición de madre soltera (OR = 2,3) por sus altos valores de asociación positiva y directa. Conclusiones: el control prenatal, la anemia materna y la condición de madre soltera resultaron tener un nivel de asociación significativa con el bajo peso al nacer.

Foundation: the high frequency of newborns with less than adequate weight, in relation to maternal anemia, insufficient prenatal care and single mother status, can have important repercussions on the health of mothers and children. Objective: to identify the relationship between prenatal control, maternal anemia and the condition of being a single mother with low birth weight of infants. Method: an observational, analytical and retrospective case-control study was carried out in pregnant women treated in a hospital in the Sierra region of Ecuador during the year 2022. The sample was 280 patients. The case group was made up of pregnant women with children with low birth weight (n = 70) and the control group was made up of pregnant women with newborns with normal weight (n = 210). The Chi square test of independence was performed and the Odds Ratio and its 95 % confidence interval were calculated for the level of association between the variables. Results: the relationship between the variable attendance at prenatal check-ups and low birth weight showed a protective association (OR = 0,5). Maternal anemia (OR = 3,1) and being a single mother (OR = 2,3) were identified as risk factors due to their high values of positive and direct association. Conclusions: prenatal control, maternal anemia and single mother status turned out to have a significant level of association with low birth weight.

Prevalence of Anemia in Patients with Diabetes Mellitus: A Systematic Review and Meta-Analysis / 生物医学与环境科学（英文）

OBJECTIVE@#Anemia is a common public health concern in patients with type 2 diabetes worldwide. This study aimed to identify the prevalence of anemia among patients with diabetes.@*METHODS@#Electronic databases, including PubMed, Scopus, Web of Sciences, and Google Scholar, were searched systematically for studies published between 2010 and 2021. After removing duplicates and inappropriate reports, the remaining manuscripts were reviewed and appraised using theNewcastle-Ottawa Scale (NOS) tool. A random-effects model was used to calculate the pooled estimates of the extracted data using Stata version 17. Heterogeneity of the studies was assessed using the Q statistic.@*RESULTS@#A total of 51 articles containing information on 26,485 patients with diabetes were included in this study. The articles were mainly from Asia (58.82%) and Africa (35.29%). The overall prevalence of anemia was 35.45% (95% CI: 30.30-40.76), with no evidence of heterogeneity by sex. Among the two continents with the highest number of studies, the prevalence of anemia in patients with diabetes was significantly higher in Asia [40.02; 95% CI: 32.72-47.54] compared to Africa [28.46; 95% CI: 21.90-35.50] ( P for heterogeneity = 0.029). Moreover, there has been an increasing trend in the prevalence of anemia in patients with diabetes over time, from [15.28; 95% CI: 9.83-22.21] in 2012 to [40.70; 95% CI: 10.21-75.93] in 2022.@*CONCLUSION@#Globally, approximately 4 in 10 patients with diabetes suffer from anemia. Therefore, routine anemia screening and control programs every 3 months might be useful in improving the quality of life of these patients.

Fanconi Anemia: Exploration of DNA Repair Pathways from Genetic Diseases to Cancer and Prospects for Treatment / 肿瘤防治研究

Fanconi anemia (FA) is an inheritable disorder that presents with bone marrow failure, developmental anomalies, and an increased susceptibility to cancer. The etiology of this condition stems from a genetic mutation that disrupts the proper repair of interstrand DNA cross-links (ICLs). The resultant dysregulation of the DNA damage response mechanism can induce genomic instability, thereby elevating the mutation rates and the likelihood of developing cancer. The FA pathway assumes a pivotal role in safeguarding genome stability through its involvement in the repair of DNA cross-links and the maintenance of overall genomic integrity. A mutation in the germ line of any of the genes responsible for encoding the FA protein results in the development of FA. The prevalence of aberrant FA gene expression in somatic cancer, coupled with the identification of a connection between FA pathway activation and resistance to chemotherapy, has solidified the correlation between the FA pathway and cancer. Consequently, targeted therapies that exploit FA pathway gene abnormalities are being progressively developed and implemented. This review critically examines the involvement of the FA protein in the repair of ICLs, the regulation of the FA signaling network, and its implications in cancer pathogenesis and prognosis. Additionally, it explores the potential utility of small-molecule inhibitors that target the FA pathway.

Anemia-related subjective symptoms in the general adult population in Japan / Journal of Rural Medicine

Objectives: Approximately 17% of Japanese women have hemoglobin concentrations less than 12 g/dL. Therefore, anemia prevention and early intervention are crucial public health issues in Japan. This study aimed to identify the symptoms and characteristics of anemic individuals in the general adult population by comparing survey responses of individuals with anemia and without anemia visiting blood donation centers.Materials and Methods: This cross-sectional study used self-administered questionnaires. Individuals who visited two Japanese Red Cross Society blood donation centers in Fukushima Prefecture, Japan were included. Hemoglobin levels were measured at blood donation, and the levels of 13 g/dL for men and 12 g/dL for women were defined as anemia.Results: Of the 857 individuals analyzed, 530 were men and 327 were women, of whom 19 (3.6%) and 12 (3.7%) had low hemoglobin levels, respectively. Logistic regression analysis was performed in men, and the results showed that “lightheadedness” (odds ratio [OR]=8.4) and “depressive symptoms” (OR=3.6) were significantly associated with hemoglobin levels. None of the evaluated items were significantly associated with hemoglobin levels in women.Conclusion: Among healthy Japanese men, those who exhibit lightheadedness and depressive symptoms have an increased risk of anemia. Lightheadedness and depressive symptoms may be indicative of undiagnosed anemia in men, which necessitates greater clinical attention.

A Case Report of Multidisciplinary Diagnosis and Treatment of a Patient with Tuberous Sclerosis Complex and Multi-Organ Involvement / 罕见病研究

Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.

Association of ABCB1 gene polymorphisms rs1128503, rs2032582, rs4148738 with anemia in patients receiving dabigatran after total knee arthroplasty / 中华创伤杂志(英文版)

PURPOSE@#Dabigatran is usually prescribed in recommended doses without monitoring of the blood coagulation for the prevention of venous thromboembolism after joint arthroplasty. ABCB1 is a key gene in the metabolism of dabigatran etexilate. Its allele variants are likely to play a pivotal role in the occurrence of hemorrhagic complications.@*METHODS@#The prospective study included 127 patients with primary knee osteoarthritis undergoing total knee arthroplasty. Patients with anemia and coagulation disorders, elevated transaminase and creatinine levels as well as already receiving anticoagulant and antiplatelet therapy were excluded from the study. The association of ABCB1 gene polymorphisms rs1128503, rs2032582, rs4148738 with anemia as the outcome of dabigatran therapy was evaluated by single-nucleotide polymorphism analysis with a real-time polymerase chain reaction assay and laboratory blood tests. The beta regression model was used to predict the effect of polymorphisms on the studied laboratory markers. The probability of the type 1 error (p) was less than 0.05 was considered statistically significant. BenjaminiHochberg was used to correct for significance levels in multiple hypothesis tests. All calculations were performed using Rprogramming language v3.6.3.@*RESULTS@#For all polymorphisms there was no association with the level of platelets, protein, creatinine, alanine transaminase, prothrombin, international normalized ratio, activated partial thromboplastin time and fibrinogen. Carriers of rs1128503 (TT) had a significant decrease of hematocrit (p = 0.001), red blood count and hemoglobin (p = 0.015) while receiving dabigatran therapy during the postoperative period compared to the CC, CT. Carriers of rs2032582 (TT) had a significant decrease of hematocrit (p = 0.001), red blood count and hemoglobin (p = 0.006) while receiving dabigatran therapy during the postoperative period compared to the GG, GT phenotypes. These differences were not observed in carriers of rs4148738.@*CONCLUSION@#It might be necessary to reconsider thromboprophylaxis with dabigatran in carriers of rs1128503 (TT) or rs2032582 (TT) polymorphisms in favor of other new oral anticoagulants. The long-term implication of these findings would be the reduction of bleeding complications after total joint arthroplasty.

Pharmaceutical care for rare ADR in a patient with ALK-positive non-small cell lung cancer induced by alectinib / 中国药房

OBJECTIVE To provide reference for safe drug use in patients with anaplastic lymphoma kinase （ALK）-positive non-small cell lung cancer （NSCLC）. METHODS Clinical pharmacists participated in the diagnosis and treatment of a patient with ALK-positive NSCLC who developed bilateral pleural effusion and hemolytic anemia after taking alectinib； regarding symptoms such as pleural effusion and hemolytic anemia in the patient， clinical pharmacists investigated the patient’s history of medication and disease， as well as potential drug interaction； to consider the correlation between the patient’s use of alectinib and the duration of pleural effusion and hemolytic anemia， clinical pharmacists suggested that clinical doctors discontinued alectinib and used reduced dose treatment after the pleural effusion improved， but the patient suffered from bilateral pleural effusion and hemolytic anemia again； after evaluating the correlation between alectinib and bilateral pleural effusion and hemolytic anemia using the Naranjo’s assessment scale， clinical pharmacists recommend permanent discontinuation of alectinib and jointly recommend replacement with ensartinib with clinical physicians. RESULTS Physicians adopted the suggestions of clinical pharmacists. The pleural effusion subsequently regressed and hemolytic anemia improved after replacing the drug. The correlation between alectinib and bilateral pleural effusion and hemolytic anemia was confirmed. CONCLUSIONS Clinical pharmacists participate in pharmaceutical monitoring of ALK-positive NSCLC patients， assist clinical doctors in developing personalized medication recommendations， and ensure the safety of patient medication.

A meta-analysis of ferric carboxymaltose versus other intravenous iron preparations for the management of iron deficiency anemia during pregnancy

Abstract Objective: We conducted a meta-analysis of randomized clinical trials evaluating the clinical effects of ferric carboxymaltose therapy compared to other intravenous iron in improving hemoglobin and serum ferritin in pregnant women. We also assessed the safety of ferric carboxymaltose vs. other intravenous iron. Data source: EMBASE, PubMed, and Web of Science were searched for trials related to ferric carboxymaltose in pregnant women, published between 2005 and 2021. We also reviewed articles from google scholar. The keywords "ferric carboxymaltose," "FCM," "intravenous," "randomized," "pregnancy," "quality of life," and "neonatal outcomes" were used to search the literature. The search was limited to pregnant women. Selection of studies: Studies related to ferric carboxymaltose in pregnancy were scanned. Observational studies, review articles, and case reports were excluded. Randomized studies in pregnant women involving ferric carboxymaltose and other intravenous iron formulations were shortlisted. Of 256 studies, nine randomized control trials were selected. Data collection: Two reviewers independently extracted data from nine selected trials Data synthesis: The final effect size for increase in hemoglobin after treatment was significant for ferric carboxymaltose vs. iron sucrose/iron polymaltose (standard mean difference 0.89g/dl [95% confidence interval 0.27,1.51]). The final effect size for the increase in ferritin after treatment was more for ferric carboxymaltose vs. iron sucrose/iron polymaltose (standard mean difference 22.53µg/L [-7.26, 52.33]). No serious adverse events were reported with ferric carboxymaltose or other intravenous iron. Conclusion: Ferric carboxymaltose demonstrated better efficacy than other intravenous iron in increasing hemoglobin and ferritin levels in treating iron deficiency anemia in pregnant women.

Anemia crónica como factor de riesgo de muerte por choque hipovolémico en población peruana. 2021-2022

Objetivo: determinar si la anemia crónica es un factor de riesgo asociado a la muerte por choque hipovolémico en adultos peruanos. Métodos: estudio observacional, de casos y controles, basado en datos del Sistema Nacional de Defunciones del Ministerio de Salud peruano entre enero de 2021 a septiembre de 2022. La población estudiada fue de 281.456 adultos cuando se analizó según sexo, y 281.416 adultos cuando se dividió según grupo etario con una diferencia de 40 datos perdidos durante el procesamiento de la información. Se incluyó a pacientes con y sin anemia (variable independiente), cuyas causas de muerte inmediata fueron divididas en muertes por choque hipovolémico (variable dependiente) y muertes por otras causas inmediatas. Se realizó la prueba de Chi-cuadrado y el coeficiente V de Cramer, así como el análisis multivariado considerando variables como la edad y el sexo. Se consideró un valor de p significativo menor a 0,05 con un intervalo de confianza al 95%. Resultados: 1627 hombres fallecieron por choque hipovolémico de los cuales 18,20% tuvo anemia crónica (p<0,001). De 1540 mujeres fallecidas, 21,30% tuvo anemia crónica (p<0,001) y en ambos grupos (3167) el 19,70% tuvo anemia crónica (p<0,001). Con la prueba V de Cramer se halló una asociación positiva y baja entre las variables estudiadas (p<0,001). Los pacientes con anemia crónica tuvieron mayor probabilidad de morir por choque hipovolémico que los fallecidos sin anemia crónica, siendo mayor en adultos a partir de los 40 años, datos reafirmados en el modelo de regresión logística binaria. Conclusiones: la anemia crónica es una comorbilidad que supone un aumento del riesgo de muerte en pacientes con choque hipovolémico. Independientemente de la etiología del choque, el control y corrección de la anemia crónica podría tener un impacto en la reducción de la mortalidad de la hipovolemia aguda en adultos peruanos.

Objective: To determine if chronic anemia is a risk factor associated with death due to hypovolemic shock in Peruvian adults. Methods: Observational, case-control study, based on data from the National Death System of the Peruvian Ministry of Health between January 2021 and September 2022. Patients with and without anemia (independent variable) were included, whose causes of immediate death were divided into deaths due to hypovolemic shock (dependent variable) and deaths due to other immediate causes. The Chi-square test and Cramer's V coefficient were performed, as well as multivariate analysis considering variables such as age and sex. A significant p value of less than 0.05 with a 95% confidence interval was considered. Results: One thousand six hundred and twenty-seven men died from hypovolemic shock, of which 18.20% had chronic anemia (p<0.001). Of 1540 deceased women, 21.30% had chronic anemia (p<0.001) and in both groups (3167) 19.70% had chronic anemia (p<0.001). With Cramer's V test, a positive and low association was found between the variables studied (p<0.001). Patients with chronic anemia were more likely to die from hypovolemic shock than those who died without chronic anemia, and this was higher in adults over 40 years of age, data reaffirmed in the binary logistic regression model. Conclusions: Chronic anemia is a comorbidity that represents an increased risk of death in patients with hypovolemic shock. Regardless of the etiology of the shock, the control and correction of chronic anemia could have an impact on reducing mortality from acute hypovolemia in Peruvian adults.

Alteraciones hematológicas en el paciente gran quemado

Introducción: Las quemaduras graves alteran de manera significativa los parámetros hematológicos y de la coagulación. Objetivo: Describir las alteraciones hematológicas en el paciente gran quemado. Métodos: Se realizó un estudio descriptivo, longitudinal y prospectivo, en pacientes ingresados en el servicio de Cirugía Plástica y Caumatología del Hospital Universitario Manuel Ascunce Domenech, de la provincia Camagüey, durante el período comprendido entre mayo de 2020 a abril de 2022. Se estudiaron 38 pacientes, se tuvieron en cuenta las siguientes variables: alteración hematológica, superficie corporal quemada, índice pronóstico, estado al egreso. Resultados: De los pacientes con 30 % o más de superficie corporal quemada (12 en total) el 91,66 % presentó anemia. La anemia se encontró con más frecuencia en los pacientes clasificados como muy graves, 11 pacientes que representan el 28,95 %, es de destacar que los pacientes con este índice de gravedad constituyeron la mayoría en esta serie, 19 para un 50 %. En 23 de los enfermos presentaron alteraciones leucocitarias. Se encontró leucocitosis en el 44,73 % de los pacientes y leucopenia en seis enfermo que representó el 15,79 %. En el 23,68 % de los enfermos se observó la presencia de trombocitopenia. Conclusiones: Las alteraciones hematológicas influyen en la buena evolución de los pacientes quemados, de no tratarse de forma oportuna pueden llevar a estos lesionados a la muerte.

Introduction: The serious burns alter of significant way the hematological and coagulation parameters. Objective: To describe the hematological alterations in the large burned patient. Methods: A descriptive, longitudinal and prospective study was carried out for the sake of describing the main hematological alterations in the large burned patient, once 2020 were deposited in the Manuel Ascunce Domenech's Universitary Hospital service of Plastic Surgery and Caumatology, of the City Camagüey, during the period understood between May, 2020 to April, 2022. They studied 38 patients in those who they had in account variables: hematological alteration, corporal burned-out surface, index forecast, state to the discharge. Results: Of the patients with 30 % or more of corporal burned-out surface (12 in total) the 91.66 % presented anemia. Anemia was found with more frequency in the patient classified as very serious condition, 11 patients that represent the 28.95 %, it is from standing out that the patients with this severity rate constituted the majority in this series, 19 for a 50 %. In 23 of the sick persons of this series they presented leukocytal alterations. Leucocytosis was found in 44.73 % of the patients, and leukopenia in six patients which represented 15.79 %. In 23.68 % of the patients the presence of thrombocytopenia was observed. Conclusions: Hematological alterations influence the good progress of burned patients, and if not treated in a timely manner can lead to death.