RESUMO
La hemosiderosis pulmonar idiopática (HPI) es una patología poco frecuente; su distribución geográfica, su incidencia y prevalencia se desconocen de manera exacta a nivel mundial. Tiene una fuerte asociación con condiciones autoinmunes y una adecuada respuesta al tratamiento inmunosupresor. A pesar de ser una patología grave, presenta una tasa de morbilidad y mortalidad mediana, siempre que se realice un diagnóstico y tratamiento precoz. Se presenta el caso clínico de una paciente femenina con diagnóstico de HPI quien cursó con la triada clásica de esta enfermedad: hemoptisis, anemia ferropénica e infiltrados pulmonares difusos. Se descartaron otras causas de hemorragia pulmonar difusa y se realizó el diagnóstico por biopsia pulmonar. Se trató con esteroides sistémicos e inhalados y azatioprina. Tras casi 2 años después del diagnóstico, estando sin tratamiento por 3 meses, presentó una exacerbación con hemorragia pulmonar masiva ocasionando el fallecimiento de la paciente.
Idiopathic pulmonary hemosiderosis (IPH) is a rare pathology; its geographic distribution, incidence and prevalence are not accurately known worldwide. It has a strong association with autoimmune conditions and has an adequate response to immunosuppressive treatment. Despite being a serious pathology, it has a medium morbidity and mortality rate, provided that early diagnosis and treatment is performed. We present the clinical case of a female patient diagnosed with IPH who presented with the classic triad of this disease: hemoptysis, iron deficiency anemia and diffuse pulmonary infiltrates. Other causes of diffuse pulmonary hemorrhage were ruled out and the diagnosis was made by lung biopsy. She was managed with systemic and inhaled steroids and azathioprine. After almost 2 years before the diagnosis, being without treatment for 3 month she had a massive pulmonary hemorrhage, causing the death of the patient.
Assuntos
Humanos , Feminino , Adulto Jovem , Hemossiderose/diagnóstico , Hemossiderose/tratamento farmacológico , Pneumopatias/diagnóstico , Pneumopatias/tratamento farmacológico , Radiografia Torácica , Tomografia Computadorizada por Raios X , Fatores de Risco , Hemoptise/etiologia , Hemossiderose/diagnóstico por imagem , Imunossupressores/uso terapêutico , Pneumopatias/diagnóstico por imagemRESUMO
This study collected samples from 50 Holstein cows, most intensively bred, and from the Campos Gerais region, Paraná, with an average milk production of 30.21L/day. Samples of the liver, spleen and lymph nodes were collected to determine the levels of copper, cobalt, iron, molybdenum and zinc. Spleen and lymph nodes were subjected to histological analysis and evaluation of the degree of hemosiderosis. The average concentrations of copper (495.05ppm), molybdenum (4.19ppm), and zinc (274.49ppm) were higher than those established for the bovines. For cobalt 26% of the animals presented levels below the established level, which characterized cobalt deficiency. Only iron (299.12ppm) exhibited an adequate average level. Histopathologically hemosiderosis was observed mainly in the spleen (78%) and less frequently in the lymph nodes (20%). The observation of hemosiderin in the spleen and lymph nodes is not related to copper deficiency. Still it may be related to high levels of molybdenum, zinc, iron, or other undetermined causes.
Para este estudo foram coletadas amostras de 50 vacas, da raça Holandesa, a maior parte criada intensivamente e oriunda da região dos Campos Gerais/PR, com média individual de produção de leite de 30,21L/dia. Foram coletadas amostras de fígado, baço e linfonodos para determinação dos níveis de cobre (Cu), cobalto (Co), ferro (Fe), molibdênio (Mo) e zinco (Zn). Baço e linfonodo foram submetidos à análise histológica e avaliação do grau de hemossiderose. A média das concentrações de Cu (495,05ppm), Mo (4,19ppm) e Zn (274,49ppm) encontrava-se acima dos níveis estabelecidos para bovinos. Para o Co, observou-se que 26% dos animais apresentaram níveis abaixo do estabelecido, o que caracterizou deficiência de Co. Apenas o Fe (299,12ppm) apresentou níveis médios adequados. Na histopatologia, hemossiderose foi observada, principalmente no baço (78%) e com menos frequência nos linfonodos (20%). A observação de hemossiderina no baço e linfonodos não está relacionada à deficiência de Cu, porém pode estar relacionada a elevados níveis de Mo, Zn e Fe ou a outras causas não determinadas.
Assuntos
Animais , Feminino , Bovinos , Doenças dos Bovinos , Deficiência de Minerais/diagnóstico , Hemossiderose/veterinária , Fígado , Minerais/isolamento & purificação , Baço , Zinco , Cobalto , Cobre , Ferro , Linfonodos , MolibdênioRESUMO
Hemochromatosis (HC) is a disorder that alters the body's ability to metabolize iron, increasing its absorption, causing iron overload, and consequently an accumulation of the mineral in multiple organs such as the liver, heart, and pancreas. The amount of total iron in the body is 2-4 g in healthy individuals and remains within these limits throughout life thanks to the control of intestinal absorption. In patients with CH, this amount is increased by at least 10 times, which translates into body deposits of 20-40 grams of iron on average. Factors that increase the risk of having HC: having two copies of the mutated HFE gene, family history, ethnicity or ancestry from Northern Europe (less common in blacks, Hispanics, and Asians), and male gender.
Assuntos
Humanos , Pessoa de Meia-Idade , Hemocromatose/diagnóstico , Hemocromatose/fisiopatologia , Sinais e Sintomas , Transplante de Fígado , Insuficiência Cardíaca , Hemocromatose/terapia , Hemossiderose , FerroRESUMO
Some wild species of mammals and birds are prone to excessive iron accumulation, especially when maintained in human care. Hemosiderosis is the process of intracellular accumulation of iron without evidence of toxicity, whereas hemochromatosis is characterized by severe iron accumulation with accompanying organ damage. Iron storage disease (ISD) occurs when organ damage is severe and causing clinical signs. This retrospective study investigated the occurrence of hemosiderosis and ISD across a variety of avian taxa, including captive and free-ranging birds. Archived paraffin-embedded hepatic samples from 103 birds from Belo Horizonte Zoo that died naturally in the period of 2008 to 2018 were re-evaluated with histologic and morphometric techniques, focusing on the identification and scoring of iron deposits in hepatocytes and the quantification of total affected hepatic area. The birds represented 13 orders, 22 families, and 52 genera, and 66 (64.0%) had some degree of iron accumulation in their liver. Importantly, no statistical difference was observed in the occurrence of iron accumulation between families, orders, or origin (free-ranging or captive). Direct and positive correlation was observed between the total area affected by the iron deposits and the histologic score. In this study, there were two cases with severe iron accumulation and clinical signs compatible with ISD: a barefaced curassow (Crax fasciolata) and a channel-billed toucan (Ramphastos vitellinus). This study indicates that iron accumulation may occur in a wide range of avian species, with frequencies and intensities that are similar between free-ranging birds and those in human care. It describes for the first time the occurrence of ISD in a Galliform species.
Assuntos
Doenças das Aves , Hemocromatose , Hemossiderose , Animais , Animais Selvagens , Animais de Zoológico , Doenças das Aves/epidemiologia , Aves , Hemocromatose/epidemiologia , Hemocromatose/veterinária , Hemossiderose/epidemiologia , Hemossiderose/veterinária , Estudos RetrospectivosRESUMO
La hemosiderosis pulmonar idiopática (HPI) es una causa de hemorragia alveolar difusa. OBJETIVO: describir la evolución de niños con HPI en nuestra institución. Se realizó una revisión retrospectiva con protocolo de seguimiento. Se reclutaron 13 pacientes, 7 hombres. Procedentes de una zona agrícola (6/13). No todos presentaron la tríada diagnóstica completa: infiltrados algodonosos (9/13), anemia (11/13), hemoptisis (9/13). Todos evidenciaron un recuento de hemosiderófagos sobre 30% en el lavado broncoalveolar. Tomografía computada de tórax: normal (5/13), patrón intersticial (5/13), vidrio esmerilado (2/13) y fibrosis (1/13). Espirometría: normal (7/13), restrictiva (4/13), obstructiva (1/13) y no efectuada (1/13). Tratamiento durante la fase aguda: bolos de metilprednisolona (7/13) o prednisona (6/13) o hidrocortisona (1/13). En la fase de mantención se administró: prednisona (13/13) más un inmunosupresor, azathioprina (12/13), hidroxicloroquina (1/13), micofenolato (1/13), más budesonida MDI (13/13). Ocho pacientes detuvieron los sangrados. Dos pacientes fallecieron y hubo cinco embarazos de curso fisiológico en 3 adolescentes. Se observó: a) diferentes modalidades de presentación que retrasaron el diagnóstico; b) gran exposición a pesticidas; c) mejor pronóstico si el diagnóstico y el tratamiento eran precoces, también en niñas adolescentes; d) la mayoría detuvo los episodios de sangrado.
Idiopathic pulmonary hemosiderosis (IPH) is a cause of diffuse alveolar hemorrhage. OBJECTIVE: to describe the evolution of children with IPH in our institution. Retrospective monitoring with a follow-up protocol was carried out. 13 patients, seven males, were recruited. From an agricultural area (6/13). Not all of patients had the complete diagnostic triad: cotton infiltrates (9/13), anemia (11/13), hemoptysis (9/13). Hemosiderin-laden macrophages counting in the bronchoalveolar lavage fluid was over 30% in all the patients. Computed chest tomography was informed as normal (5/13), interstitial pattern (5/13), ground glass (2/13) and fibrosis (1/13). Spirometry: normal (7/13), restrictive (4/13), obstructive (1/13) and not performed (1/13). Treatment during the acute phase: bolus of methylprednisolone (7/13) or prednisone (6/13) or hydrocortisone (1/13). In the maintenance phase: prednisone (13/13) plus an immunosuppressant, azathioprine (12/13), hydroxychloroquine (1/13), mycophenolate (1/13), plus budesonide MDI (13/13). Eight patients stopped the bleeding episodes. Two patients died and there were five physiological pregnancies in 3 adolescents. It was observed:(a) different modes of IPH presentation that delayed its diagnosis; (b) large exposure to pesticides; (c) prognosis improved if diagnosis and treatment were early, also in adolescent girls; (d) most of the patients stopped the bleeding episodes.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Hemossiderose/tratamento farmacológico , Hemossiderose/diagnóstico por imagem , Pneumopatias/tratamento farmacológico , Pneumopatias/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Zonas Agrícolas , Evolução Clínica , Chile , Estudos Retrospectivos , Seguimentos , Corticosteroides/uso terapêutico , Idade de Início , Anemia Ferropriva/etiologia , Hemoptise/etiologia , Imunossupressores/uso terapêuticoRESUMO
We present the case-report of a one-month-old infant, admitted to the Emergency Department with hypovolemic shock secondary to pulmonary hemorrhage who required life-support measures, including vasoactive drugs and methylprednisolone pulses. She was discharged from the hospital after 13 days of evolution and then readmitted 5 days later for a new episode of hemoptysis with hemodynamic compromise. Fiberoptic bronchoscopy was performed 4 days after the first episode showed a normal anatomy, without active bleeding, with 20% of hemosiderophages in bronchoalveolar lavage. Diffuse infiltrates were found on the chest radiograph. Differents studies were performed for check-out infection, heart disease, immune disease, thrombophilia, celiac disease, swallowing disorder, vascular abnormalities and allergy to cow's milk protein were negative, which led to Idiopathic Pulmonary Hemosiderosis (IPH). It was managed with amino acid formula, daily oral prednisone until 6 months of age and then every other day, and permanent inhaled fluticasone. In subsequent controls, normal growth and development were found, with no recurrences up to the time of this report, at 1 year of age. The favorable evolution in this case is attributed to early diagnosis and timely treatment with systemic corticosteroids. A review of the topic of IPH in pediatrics is presented, and study and treatment algorithms are proposed.
Se presenta el caso de una lactante de un mes de edad, que se presentó en el Servicio de Urgencia con shock hipovolémico secundario a hemorragia pulmonar. Necesitó medidas de soporte vital, incluyendo drogas vasoactivas y pulsos de metilprednisolona. Egresó del hospital a los 13 días de evolución y reingresó 5 días después por nuevo episodio de hemoptisis con compromiso hemodinámico. La fibrobroncoscopía efectuada a los 4 días de evolución del primer episodio mostró una anatomía normal, sin sangrado activo, con 20% de hemosiderófagos en el lavado broncoalveolar. En la radiografía de tórax se encontró infiltrados difusos. Los estudios en busca de infección, cardiopatía, enfermedad inmunológica, trombofilia, enfermedad celíaca, trastorno de deglución, anomalías vasculares y alergia a la proteína de la leche de vaca resultaron negativos, por lo que se planteó una Hemosiderosis Pulmonar Idiopática (HPI). Se manejó con fórmula aminoacídica, prednisona oral diaria hasta los 6 meses de edad y después en días alternos y fluticasona inhalada permanente. En controles posteriores se constató crecimiento y desarrollo normal, sin recidivas hasta el momento de este reporte, con 1 año de edad. La evolución favorable en este caso se atribuye al diagnóstico precoz y tratamiento oportuno con corticoides sistémicos. Se presenta una revisión del tema de HPI en pediatría y se proponen algoritmos de estudio y tratamiento.
Assuntos
Humanos , Feminino , Recém-Nascido , Hemossiderose/tratamento farmacológico , Hemossiderose/diagnóstico por imagem , Metilprednisolona , Prednisona , Radiografia Torácica , Corticosteroides/uso terapêutico , Fluticasona , Hemoptise/etiologia , Hemossiderose/complicaçõesRESUMO
Resumen La hemosiderosis pulmonar idiopática es una entidad rara caracterizada por hemorragia alveolar capilar. Su tríada clásica es hemoptisis crónica o recurrente, anemia por deficiencia de hierro y opacidades en las imágenes pulmonares. El objetivo de esta revisión fue determinar la frecuencia en radiografía y tomografía de tórax, de los hallazgos de hemosiderosis pulmonar idiopática en adultos, reportados en la literatura durante los últimos 20 años, de acuerdo con los hallazgos semiológicos en imagen, localización y distribución. Se hizo una búsqueda de publicaciones en bases de datos. Se seleccionaron 42 estudios, se estratificaron variables y se recopilaron los hallazgos. La consolidación y las opacidades reticulonodulares fueron el hallazgo más frecuente en radiografía. En tomografía el hallazgo principal fue el vidrio deslustrado en la región basal. No hubo diferencias en los hallazgos semiológicos de imagen en cuanto al compromiso de acuerdo con el género, aunque las mujeres tendieron a presentar una distribución difusa. MÉD.UIS.2020;33(2):55-64.
Abstract Idiopathic pulmonary hemosiderosis is a rare entity characterized by capillary alveolar hemorrhage. Its classic triad is chronic or recurrent hemoptysis, iron deficiency anemia, and opacities in lung images. The objective of this review was to determine the frequency in chest radiography and chest tomography of the findings of idiopathic pulmonary hemosiderosis in adults, reported in the literature during the last 20 years, according to the semiological imaging findings, location and distribution. A search of publications in databases was made. Forty-two studies were collected, variables were stratified in different categories and the findings were compiled. The most frequent finding in chest radiography were consolidation and reticulonodular opacities. On the other hand, the main finding in tomography was ground glass opacities in the basal region. There were no differences in the pattern of compromise by gender, although women tended to have a more diffuse distribution. MÉD.UIS.2020;33(2):55-64.
Assuntos
Humanos , Adulto , Radiografia Torácica , Tomografia , Hemossiderose , Dispneia , AnemiaRESUMO
Abstract Introduction: Anemic patients with chronic kidney disease (CKD) can be divided into anemic patients without or with functional iron deficiency (FID). The increase in the number of cases of hemosiderosis in patients on hemodialysis (HD) attributed to excessive intravenous iron replacement has called for the investigation of the factors involved in the genesis of FID. Objectives: This study aimed to describe the prevalence of FID in patients with CKD on HD, characterize the included individuals in terms of clinical and workup parameters, and assess their nutritional, oxidative stress, and inflammation statuses. This cross-sectional study assembled a convenience sample of 183 patients with CKD on HD treated in Southern Brazil. Patients meeting the inclusion and exclusion criteria were divided into two groups, one with anemic subjects with FID and one with anemic patients without FID. Participants answered a questionnaire probing into socio-epidemiological factors, underwent anthropometric measurements, and were tested for markers of anemia, oxidative stress, inflammation, and nutrition. Statistical analysis: The date sets were treated on software package GraphPad InStat version 3.1. Variables were tested with the Kolmogorov-Smirnov, chi-square, Student's t, and Mann-Whitney tests. Statistical significance was attributed to differences with a p < 0.05. Results: Markers of inflammation were not statistically different between the two groups. Markers of anemia and nutrition were significantly lower in patients with FID. Patients with FID were prescribed higher doses of parenteral iron (p < 0,05). Discussion: FID was associated with lower nutritional marker levels, but not to increased levels of markers of inflammation or oxidative stress, as reported in the literature. Additional studies on the subject are needed.
Resumo Introdução: A anemia na DRC pode ser dividida em anemia sem deficiência funcional de ferro e com deficiência funcional de ferro (ADFF). Diante do aumento dos casos de hemossiderose em pacientes em hemodiálise, atribuídos à reposição excessiva de ferro endovenoso, maiores conhecimentos sobre os fatores envolvidos na gênese da ADFF são importantes. Objetivos: documentar a prevalência de ADFF em renais crônicos em hemodiálise. Caracterizar clínica e laboratorialmente os portadores de ADFF em HD e avaliar o estado nutricional, estresse oxidativo e inflamatório. Estudo transversal, amostra de conveniência, envolvendo 183 renais crônicos em hemodiálise no sul do Brasil. Após aplicação dos critérios de exclusão, os pacientes foram separados em dois grupos: portadores de anemia com e sem deficiência funcional de ferro. Foram submetidos a questionário socioepidemiológico, à análise antropométrica e análise laboratorial dos marcadores de anemia, estresse oxidativo, inflamatórios e nutricionais. Análise estatística: programa GraphPad InStat versão 3.1. Foram aplicados os testes: Kolmogorov-Smirnov, qui-quadrado, t de Student e Mann-Whitney. Nível de significância adotado de 5%. Resultados: não houve diferença significativa nos marcadores inflamatórios entre os dois grupos. Houve diferença significativa nos marcadores de anemia e nutrição, significativamente menores nos pacientes com ADFF. Pacientes com ADFF receberam doses mais elevadas de ferro parenteral (p < 0,05). Discussão: ADFF esteve associada a menores valores de marcadores nutricionais, mas não esteve associada a marcadores inflamatórios ou de estresse oxidativo aumentados, como relatado na literatura. Estudos adicionais sobre o tema são necessários.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Biomarcadores/metabolismo , Diálise Renal/efeitos adversos , Anemia Ferropriva/etiologia , Insuficiência Renal Crônica/complicações , Inflamação/metabolismo , Anemia/etiologia , Brasil/epidemiologia , Avaliação Nutricional , Prevalência , Estudos Transversais , Estresse Oxidativo/fisiologia , Anemia Ferropriva/epidemiologia , Administração Intravenosa , Hemossiderose/epidemiologia , Anemia/epidemiologia , Ferro/administração & dosagem , Ferro/efeitos adversos , Óxido Nítrico/metabolismoRESUMO
INTRODUCTION: Anemic patients with chronic kidney disease (CKD) can be divided into anemic patients without or with functional iron deficiency (FID). The increase in the number of cases of hemosiderosis in patients on hemodialysis (HD) attributed to excessive intravenous iron replacement has called for the investigation of the factors involved in the genesis of FID. OBJECTIVES: This study aimed to describe the prevalence of FID in patients with CKD on HD, characterize the included individuals in terms of clinical and workup parameters, and assess their nutritional, oxidative stress, and inflammation statuses. This cross-sectional study assembled a convenience sample of 183 patients with CKD on HD treated in Southern Brazil. Patients meeting the inclusion and exclusion criteria were divided into two groups, one with anemic subjects with FID and one with anemic patients without FID. Participants answered a questionnaire probing into socio-epidemiological factors, underwent anthropometric measurements, and were tested for markers of anemia, oxidative stress, inflammation, and nutrition. STATISTICAL ANALYSIS: The date sets were treated on software package GraphPad InStat version 3.1. Variables were tested with the Kolmogorov-Smirnov, chi-square, Student's t, and Mann-Whitney tests. Statistical significance was attributed to differences with a p < 0.05. RESULTS: Markers of inflammation were not statistically different between the two groups. Markers of anemia and nutrition were significantly lower in patients with FID. Patients with FID were prescribed higher doses of parenteral iron (p < 0,05). DISCUSSION: FID was associated with lower nutritional marker levels, but not to increased levels of markers of inflammation or oxidative stress, as reported in the literature. Additional studies on the subject are needed.
Assuntos
Anemia Ferropriva/etiologia , Anemia/etiologia , Biomarcadores/metabolismo , Inflamação/metabolismo , Diálise Renal/efeitos adversos , Insuficiência Renal Crônica/complicações , Administração Intravenosa , Adulto , Anemia/epidemiologia , Anemia Ferropriva/epidemiologia , Brasil/epidemiologia , Estudos Transversais , Feminino , Hemossiderose/epidemiologia , Humanos , Ferro/administração & dosagem , Ferro/efeitos adversos , Masculino , Pessoa de Meia-Idade , Óxido Nítrico/metabolismo , Avaliação Nutricional , Estresse Oxidativo/fisiologia , Prevalência , Insuficiência Renal Crônica/fisiopatologia , Oligoelementos/administração & dosagem , Oligoelementos/efeitos adversosRESUMO
Abstract Avian malaria is one of the most important diseases of captive penguins. We employed morphometric techniques to evaluate hepatic hemosiderosis in rehabilitating wild Magellanic penguins (Spheniscus magellanicus) that were negative (n = 9) or naturally infected by different subgenera of Plasmodium spp. (n = 24), according with: Plasmodium subgenera (Haemamoeba, Huffia, Other lineages, and Unidentified lineages), severity of Plasmodium histopathological lesions, and concurrent diseases, age class (juvenile or adult plumage), sex (male, female or not determined), body score (emaciated, thin, good, excellent, not available), molt, presence or absence of oil contamination upon admission, iron supplementation, and rehabilitation center. The percentage of the area occupied by hemosiderin was called 'Index of Hepatic Hemosiderosis (IHH)'. Plasmodium-positive females presented significantly higher IHH values (17.53 ± 12.95%) than males (7.20 ± 4.25%; p = 0.041). We observed higher levels of congestion (p = 0.0182) and pneumonia (p = 0.0250) severity between Unidentified lineages vs. Huffia. We believe that the hepatic hemosiderosis observed in this study was multifactorial, the result of pathological processes caused by malaria, molting, hemoglobin and myoglobin catabolism during migration, anemia, concomitant diseases, and iron supplementation, all possibly potentiated by decreased liver mass. Further studies are needed to clarify the mechanisms of these hypotheses.
Resumo Malária aviária é uma das mais relevantes doenças em pinguins cativos. Foram aplicadas técnicas morfométricas para avaliar a hemossiderose hepática em pinguins-de-Magalhães (Spheniscus magellanicus ) de vida livre em reabilitação negativos (n = 9) e naturalmente infectados por diferentes subgêneros de Plasmodium spp. (n = 24), quanto a: subgênero de Plasmodium (Haemamoeba , Huffia, Outras Linhagens, e Linhagens não identificadas), severidade das lesões histopatológicas causadas por Plasmodium e doenças concomitantes, faixa etária (plumagem juvenil ou adulta), sexo (macho, fêmea, indeterminado), condição corporal (emaciado, magro, bom, excelente, indisponível), muda, presença/ausência de óleo a admissão, suplementação de ferro, e centro de reabilitação. A porcentagem da área ocupada por hemossiderina foi denominada "Índice de Hemossiderose Hepática (IHH)". Fêmeas Plasmodium -positivas apresentaram IHH significativamente mais elevado que machos, respectivamente, 17,53 ± 12,95% e 7,20 ± 4,25% (p = 0,041). Níveis mais elevados de congestão (p = 0,0182) e pneumonia (p = 0,0250) foram observados entre Linhagens não identificadas vs. Huffia. Possivelmente, a hemossiderose hepática observada nesse estudo seja multifatorial, resultado de processos patológicos causados por malária, muda, catabolismo de hemoglobina e mioglobina durante a migração, anemia, doenças concomitantes e suplementação de ferro, potencialmente intensificados por massa hepática reduzida. Estudos complementares são necessários para esclarecer os mecanismos de tais hipóteses.