Hemosiderosis pulmonar idiopática, caso clínico en México. Importancia de un diagnóstico y tratamiento oportuno / Idiopathic pulmonary hemosiderosis, clinical case in Mexico. The relevance of performed an early diagnosis and treatment

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.

Hemocromatosis: revisión de la literatura a propósito de un caso clínico / Hemochromatosis: case report and literature review

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.

Hemosiderosis pulmonar idiopática: seguimiento de pacientes durante 25 años (1995-2019) / Idiopathic pulmonary hemosiderosis: follow-up of patients for 25 years (1995-2019)

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.

Hemosiderosis pulmonar idiopática (HPI): presentación de un caso y revisión de la literatura / Idiopathic pulmonary hemosiderosis (IPH): a case report and review of the literature

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.

Hallazgos imagenológicos de la hemosiderosis pulmonar idiopática en el adulto: una revisión de la literatura de los últimos 20 años / Imaging findings of idiopathic pulmonary hemosiderosis in adults: Literature review of the last 20 years

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.

Functional iron deficiency in patients on hemodialysis: prevalence, nutritional assessment, and biomarkers of oxidative stress and inflammation / Deficiência funcional de ferro em pacientes em hemodiálise: prevalência, avaliação nutricional e de biomarcadores de estresse oxidativo e de inflamação

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.

Comparative morphometric evaluation of hepatic hemosiderosis in wild Magellanic penguins (Spheniscus magellanicus) infected with different Plasmodium spp. subgenera / Avaliação morfométrica comparativa da hemossiderose hepática em pinguins-de-Magalhães (Spheniscus magellanicus) de vida livre infectados por diferentes subgêneros de Plasmodium spp

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.

Patrón dermatoscópico en arcoiris (irisado) en dermatofibroma hemosiderótico: reporte de un caso / Dermoscopic pattern in rainbow (iridescent) in hemosiderotic dermatofibroma: a case report

El patrón en arcoiris ha sido un tema controvertido, dado que en un principio se planteó como un patrón dermatoscópico específico asociado al diagnóstico de Sarcoma de Kaposi. Sin embargo, esta asociación ha sido cuestionada por diversos reportes que han evidenciado la presencia de este patrón dermatoscópico en otro tipo de lesiones, tales como, otros tumores vaculares, cicatriz hipertrófica, angioqueratoma, dermatitis por estasis, pseudo-Kaposi (acroangiodermatitis), melanoma, liquen plano, dermatofibroma hemosiderótico, entre otros. Se ha propuesto que este efecto se genera por la absorción, difracción y difusión de la luz polarizada y su interferencia con diferentes componentes vasculares y fibrosos de la dermis más que por algún hallazgo histopatológico específico. Presentamos el primer caso con registro fotográfico de dermatofibroma hemosiderótico con presencia de un 'patrón irisado' en la dermatoscopía.

The dermoscopic 'rainbow pattern' has been a controversial issue; it was initially proposed as a specific dermatoscopic pattern associated with Kaposi's Sarcoma. However, this has been questioned by several reports that have shown the presence of this dermoscopic pattern in a wide variety of lesions such as other vascular tumors, hypertrophic scars, angiokeratoma, stasis dermatitis, pseudo-Kaposi acroandgiodermatitis), melanoma, lichen planus, and hemosiderotic dermatofibroma, among others. It has been proposed that this effect is generated by the absorption, diffraction and diffusion of polarized light and its interference with different vascular and fibrous components of the dermis, rather than being caused by a specific histopathologic finding. We present the first photographically recorded case of hemosiderotic dermatofibroma, characterized by the presence of a rainbow pattern in dermoscopy.

Severe iron-deficiency anemia as initial manifestation of pulmonary hemosiderosis in a child / Anemia ferropriva grave como manifestaçao inicial de hemossiderose pulmonar em criança

ABSTRACT Idiopathic pulmonary hemosiderosis is a potentially fatal disease that results from episodes of alveolar hemorrhage of unknown origin. The clinical spectrum is varied, and anemia may constitute the only manifestation of illness, preceding other signs and symptoms by several months. We present the case of a 4 year-old child presenting with fever, vomiting and prostration, associated with pallor. He had microcytic and hypochromic anemia refractory to iron therapy. Gastrointestinal bleeding was ruled out after negative extensive etiological investigation. Subsequently, pulmonary infiltrates suggestive of alveolar hemorrhage were observed in the chest radiography. The cytological exam of the bronchoalveolar lavage showed hemosiderin-laden macrophages. After the etiological study, the diagnosis of idiopathic pulmonary hemosiderosis was made by exclusion. He was initiated on corticosteroid therapy, later associated to an immunosuppressive agent, with subsequent correction of anemia and of the radiological pattern. The patient is currently asymptomatic.

RESUMO A hemossiderose pulmonar idiopática é uma doença potencialmente fatal que cursa com episódios de hemorragia alveolar de etiologia desconhecida. As manifestações clínicas são variadas, e a anemia pode constituir o único sinal de doença, precedendo em vários meses os outros sinais e sintomas. Apresenta-se o caso de criança de 4 anos, com febre, vômitos e prostração, associados à palidez. Apresentava anemia microcítica e hipocrômica, refratária à terapêutica com ferro. A hipótese diagnóstica de sangramento gastrintestinal foi excluída, após investigação etiológica extensa, inconclusiva. Posteriormente, em radiografia torácica, foram observados infiltrados sugestivos de hemorragia alveolar. O exame citológico do lavado broncoalveolar mostrou macrófagos com depósitos de hemossiderina. Após estudo etiológico, assumiu-se, por exclusão, o diagnóstico de hemossiderose pulmonar idiopática. Foi iniciada terapêutica com corticoides, associada posteriormente a imunossupressor, com correção subsequente da anemia e do padrão radiológico, encontrando-se, atualmente, assintomático.

Hemispherectomy in treatment of seizures: current perspectives of the indications, techniques and complications: literature review

Introducción: A hemisferectomía es un procedimiento valioso en el tratamiento de trastornos convulsivos causados por desordenes hemisféricos unilaterales. El hemisferectomía anatómica se ha utilizado para este fin desde 1938, sin embargo, se abandonó este procedimiento después de informes de complicaciones postoperatorias causadas por hemosiderosis superficial, ependimitis e hidrocefalia obstructiva. Así que, se ha mostrado en la literatura modificaciones en las indicaciones y técnicas de hemisferectomía anatómica cuya finalidad es la de reducir la incidencia de esta complicación sin dejar de lograr control de las convulsiones. Sobre la base de la literatura, la hemisferectomía mejora la calidad de vida de los pacientes que tiene la indicación para realizar este procedimiento, ya que permite reducir la frecuencia de las convulsiones, si tónica o átona, tónico-clónicas Objetivo: El objetivo de esta revisión de la literatura es discutir los detalles técnicos, modalidades, riesgos, complicaciones, resultados y de pronóstico de hemisferectomía basado en la revisión crítica de la literatura. Casuística y Métodos: Se realizó la consulta bibliográfica, utilizando la base de datos MEDLINE, LILACS, SciELO, que utiliza el lenguaje como criterios de selección, la elección de los artículos recientes preferiblemente en portugués, español o inglés. Conclusión: Según las referencias, hemisferectomía es un procedimiento con buen resultado para las personas con convulsiones derivadas cuando está indicado para casos seleccionados y la tasa de éxito no es proporcional a la extensión de la resección del tejido neuronal. A mayor resección puede o no reducir la frecuencia de las crisis, sin embargo, la incidencia de la morbilidad puede ser mayor.

Background: The hemispherectomy is a valuable procedure in the management of seizure disorders caused by unilateral hemispheric disease. The anatomical hemispherectomy has been used for this purpose since 1938, however, it was abandoned after reports of postoperative complications caused by superficial hemosiderosis, ependymitis and obstructive hydrocephalus. So that, it has been showed modifications in the techniques of hemispherectomy whose the purpose is reduce the incidence of this complications while still achieving seizure control. Based on literature, the hemispherectomy improves the quality of life of patients that has the indication to perform this procedure because it allows reducing the frequency of seizures, whether tonic or atonic, tonic-clonic. Aim: The aim of this literature review is discuss the indications, technical details, modalities, risks, complications, results as well de prognosis of callosotomy based on critical literature review and the authors experience. Casuistry and Methods: It was performed bibliographical consultation, using the databases MEDLINE, LILACS, SciELO, utilizing language as selection criteria, choosing preferably recent articles in Portuguese, Spanish or English. Conclusion: According to references, the functional hemispherectomy has a good outocome for those with seizures arisin when indicated to selected cases and the success rate is not proportional to the extent of neuronal tissue resection. So that, a greater resection cannot necessarily reduce the seizure frequency, however the morbidity may also be larger.

Hemosiderosis pulmonar idiopática en paciente adulto / Idiopathic pulmonary hemosiderosis in an adult patient

Resumen Se presenta el caso de una paciente de 46 años de edad con cinco años de evolución de episodios intermitentes de hemoptisis cuyo diagnóstico final fue hemosiderosis pulmonar idiopática. Su presentación y características clínicas son comparadas con los otros casos reportados en la literatura.

Abstract The case of a 46-year-old patient with five years of evolution of intermittent episodes of hemoptysis whose final diagnosis was idiopathic pulmonary hemosiderosis is reported. Its presentation and clinical characteristics are compared with the other cases reported in the literature.

Hemosiderotic dermatofibroma

Abstract: We report a rare clinical case of hemosiderotic dermatofibroma in a 36-year-old female patient. The main dermatoscopic finding was represented by homogeneous blue-gray pigmentation. The aim of this report is to demonstrate the rarity of the lesion and the dermatoscopic importance it assumes by sharing a blue-gray homogeneous pattern with other benign and malignant lesions.

Hemosiderosis pulmonar idiopática con osificación pulmonar / Idiopathic pulmonary hemosiderosis with dendriform pulmonary ossification

La osificación pulmonar es un hallazgo poco usual, generalmente asintomático, que se reporta como incidental en biopsias de pulmón. Asimismo, la hemosiderosis pulmonar idiopática es una causa poco frecuente de infiltración pulmonar. Se presenta el caso de un hombre de 64 años con síntomas respiratorios crónicos, en quien se detectaron estas dos condiciones en el estudio histopatológico.

Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged

Acute idiopathic pulmonary hemorrhage in infants. Report of two cases and literature review / Hemorragia pulmonar idiopática aguda en pediatría: reporte de 2 casos y revisión en la literatura

Acute idiopathic pulmonary hemorrhage is a rare life-threatening disease in children. The classic triad includes hemoptysis, anemia and respiratory distress. Since clinical presentation may vary, diagnosis can be difficult. Severe respiratory distress, and ventilatory failure requiring mechanical ventilation are often present. Chest X-rays usually show unilateral or bilateral infiltrates, therefore, other causes of pulmonary hemorrhage must be excluded, since most of them correspond to systemic diseases. Treatment often requires intravenous steroids to solve the respiratory failure in most cases. We present two cases involving infants treated at Hospital San José (a fourth level hospital in Bogotá, Colombia) with acute idiopathic pulmonary hemorrhage which required mechanical ventilation and responded to intravenous steroids. A literature review was conducted with special emphasis on clinical presentation, diagnosis and therapeutic approaches.

La hemorragia pulmonar idiopática aguda es una enfermedad poco común en pediatría que amenaza la vida de lactantes. La tríada clásica se caracteriza por hemoptisis, anemia y dificultad respiratoria, aunque su presentación clínica es variable, por lo que su diagnóstico presenta dificultades. Este cuadro de clínico se presenta junto a dificultad respiratoria severa y falla ventilatoria, que requiere una ventilación mecánica. En la radiografía se visualizan infiltrados unilaterales y/o bilaterales, por lo que deben excluirse otras causas de hemorragia pulmonar que en su mayoría son enfermedades sistémicas. El tratamiento requiere esteroides endovenosos que permitan resolver la falla respiratoria en la mayoría de los casos. Se presentan dos casos de lactantes menores atendidos en el Hospital de San José (hospital de cuarto nivel en Bogotá D.C.), con un cuadro agudo de hemorragia pulmonar idiopática con ventilación mecánica y que responden al tratamiento con esteroides endovenosos. Se realiza revisión de la literatura haciendo particular énfasis en la presentación clínica, el enfoque diagnóstico y terapéutico

Evaluación de efectividad y seguridad de deferasirox en Hemosiderosis Transfusional / Evaluation of effectiveness and safety of deferasirox in Hemosiderosis Transfusional

Antecedentes: Descripción de la condición de salud de interés (indicación): El cuerpo humano no posee un mecanismo activo para la excreción de hierro, y sus niveles son controlados principalmente por su absorción en el intestino delgado. Fisiológicamente, la cantidad de hierro absorbido (1-2mg/dia) se pierde mediante exudados de la mucosa intestinal y piel, así como pequeñas cantidades a través de la orina y bilis. Los pacientes que cursan con anemias crónicas y que son dependientes de transfusiones sanguíneas, reciben un exceso de hierro con cada transfusión (cada unidad de glóbulos rojos contiene aproximadamente 250mg de hierro); este hierro es acumulado de forma gradual en diferentes tejidos, tales como corazón e hígado. Descripción de la tecnología: El deferasirox es un medicamento quelante, trifdentado que se une especialmente al hierro; es empleado en el tratamiento de la sobrecarga crónica de este metal en el organismo. Está disponible en comprimidos para administración por vía oral. Cuenta con registro sanitario en Colombia. Evaluación de efectividad y seguridad: En pacientes con diagnóstico de hemosiderosis transfusional ¿cuál es la efectividad y seguridad de deferasirox comparado con deferoxamina, en la reducción de depósitos de hierro hepático o cardíaco, niveles de ferritina sérica y mortalidad? La pregunta de investigación fue validada teniendo en cuenta las siguientes fuentes de información: registro sanitario INVIMA, Acuerdo 029 de 2011, guías de práctica clínica, revisiones sistemáticas y narrativas de la literatura, estudios de prevalencia/incidencia y carga de enfermedad, libros de texto, consulta con expertos temáticos, sociedades científicas y otros actores clave. Población: pacientes con diagnóstico de hemosiderosis transfusional. Tecnología de interés: Deferasirox. Conclusiones: Efectividad: Deferasirox es una alternativa terapéutica de administración oral, efectiva para el tratamiento de la hemosiderosis transfusional. No existen diferencias significativas en mortalidad entre deferasirox y deferoxamina. La efectividad de deferasirox puede ser similar a deferoxamina dependiendo de la dosis y proporción comparada; sin embargo, la satisfacción de los pacientes es mayor en el grupo de pacientes previamente tratados con deferoxamina, que recibieron posteriormente deferasirox, lo que puede llevar a una mejor adherencia al tratamiento. Seguridad: los eventos adversos más frecuentes se encuentran relacionados con síntomas gastrointestinales, sin diferencias estadísticamente significativas entre ambos agentes. Sin embargo, se demuestra una mayor probabilidad de presentar aumento en los niveles de creatinina sérica con deferasirox en comparación con deferoxamina.

Idiopathic pulmonary hemorrhage: morphology and differential diagnosis. Case report / Hemorragia pulmonar idiopática: morfologia e diagnóstico diferencial. Relato de caso

Idiopathic pulmonary hemorrhage (IPH) is a rare cause of alveolar hemorrhage (AH) with unknown etiology that primarily affects children. The process has a variable clinical progression, and its diagnosis is established after excluding all causes of AH. Herein, the authors report a case of IPH in an adult male patient referring cough and hemoptysis. The conventional radiography computed tomography imaging identified zones of consolidation and areas of ground-glass attenuation in the lower lobes and lingula. Forced spirometry, bronchoscopy, and laboratorial tests yielded normal results. Several alveolar hemosiderin-laden macrophages were identified on bronchoalveolar lavage and lung biopsy. Thus, the histopathological findings associated with clinical data were compatible with IPH.

A hemorragia pulmonar idiopática (HPI) é uma causa rara de hemorragia alveolar (HA) que afeta principalmente crianças, com etiologia desconhecida. O processo tem evolução clínica variável e o diagnóstico é estabelecido depois que todas as causas de HA forem excluídas. Neste artigo, os autores relatam um caso de HPI em paciente do sexo masculino, adulto, referindo tosse e hemoptise. O exame radiológico convencional e a tomografia computadorizada identificaram zonas de consolidação e áreas de vidro fosco nos lobos inferiores e língula. A espirometria forçada, a broncoscopia e os testes laboratoriais revelaram resultados normais. Numerosos macrófagos alveolares contendo hemossiderina foram identificados no lavado broncoalveolar e na biópsia pulmonar. Os achados histopatológicos relacionados com os dados clínicos foram então compatíveis com HPI.

Hemosiderosis pulmonar en una lactante / Lung hemosiderosis in an infant

Se presenta el caso clínico de una lactante de 11 meses de edad con hemosiderosis pulmonar, quien fue ingresada en el Hospital Infantil Sur de Santiago de Cuba por presentar manifestaciones respiratorias recurrentes desde los 5 meses, que coincidió con la supresión de la lactancia materna e inicio de la alimentación con leche de vaca, además de anemia microcítica e hipocrómica, infiltrado radiográfico característico, así como siderófagos en el contenido gástrico, síntomas que se correspondían con el síndrome de Heiner. El diagnóstico precoz y el tratamiento oportuno garantizaron la evolución favorable de la paciente.

The case report of an 11 months female infant with lung hemosiderosis is presented. She was admitted to the Southern Hospital from Santiago de Cuba due to recurrent breathing manifestations since 5 months of age, which coincided with the discontinuation of breastfeeding and the beginning of feeding with cow milk, besides hypochromic microcytic anemia, characteristic radiographic infiltrate, as well as siderophages in the gastric content, symptoms which were in correspondence with Heiner syndrome. The early diagnosis and the opportune treatment guaranteed the favorable clinical course of the patient.

Hemorragia pulmonar asociada a enfermedad celíaca / Pulmonary hemorrhage associated with celiac disease

La hemosiderosis pulmonar idiopática (HPI) es una enfermedad grave y potencialmente fatal caracterizada por episodios recurrentes de hemorragia alveolar, hemoptisis y anemia. Su asociación con enfermedad celíaca (EC), descripta como síndrome de Lane-Hamilton, podría deberse a que ambas entidades comparten una misma vía inmunopatogénica. Se presentan dos pacientes de 13 años que consultaron por hemoptisis y anemia grave que no habían respondido al tratamiento inmunosupresor con pulsos de metilprednisolona, meprednisona e hidroxicloroquina. En ambos niños se destaca la ausencia de síntomas gastrointestinales al momento de la consulta, pero el dosaje de anticuerpos antiendomisio y antitransglutaminasa fue positivo, y la biopsia de intestino confirmó la presencia de enteropatía. En el plan de estudios de pacientes con síndrome de hemorragia alveolar difusa, aún en ausencia síntomas gastrointestinales, corresponde evaluar la presencia concomitante de enfermedad celíaca. En su presencia, la incorporación de una dieta libre de gluten favorece el control de los síntomas, permite reducir el tratamiento inmunosupresor y mejora la evolución clínica de ambas entidades.

Idiopathic pulmonary hemosiderosis is a severe and potentially fatal disease characterized by recurrent episodes of alveolar hemorrhage, hemoptysis, and anemia. His association with celiac disease, described as Lane- Hamilton syndrome, could be due to the fact that both entities share a common pathogenic immune pathway. We report two patients of 13 years who consulted for hemoptysis and severe anemia that had not responded to immunosuppressive treatment with pulses of methyl prednisolone, oral meprednisone and hydroxychloroquine. Although both children highlight the absence of gastrointestinal symptoms at the time of consultation, the dosage of anti-endomysial and anti-transglutaminase antibodies was positive and biopsy confirmed the presence of intestinal enteropathy. It is emphasized that in patients with diffuse alveolar hemorrhage, even in the absence of gastrointestinal symptoms, the concomitant presence of celiac disease should be evaluated. If celiac disease is present, the incorporation of a gluten-free diet helps to control the symptoms, allows reducing the immunosuppressive treatment and improves the clinical course of both entities.