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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.

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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.

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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.

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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.

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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.

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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.

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BACKGROUND & AIMS: Gaucher disease (GD) is a multisystemic disease. Liver involvement in GD is not well characterised and ranges from hepatomegaly to cirrhosis and hepatocellular carcinoma. We aim to describe, and assess the effect of treatment, on the hepatic phenotype of a cohort of patients with GD types I and II. METHODS: Retrospective study based on the review of the medical files of the Gaucher Reference Centre of the Hospital de Clínicas de Porto Alegre, Brazil. Data from all GD types I and III patients seen at the centre since 2003 were analysed. Variables were compared as pre- ("baseline") and post-treatment ("follow-up"). RESULTS: Forty-two patients (types I: 39, III: 3; female: 22; median age: 35 y; enzyme replacement therapy: 37; substrate reduction therapy: 2; non-treated: 3; median time on treatment-MTT: 124 months) were included. Liver enzyme abnormalities, hepatomegaly, and steatosis at baseline were seen in 19/28 (68%), 28/42 (67%), and 3/38 patients (8%), respectively; at follow-up, 21/38 (55%), 15/38 (39%) and 15/38 (39%). MRI iron quantification showed overload in 7/8 patients (treated: 7; MTT: 55 months), being severe in 2/7 (treated: 2/2; MTT: 44.5 months). Eight patients had liver biopsy (treated: 6; MTT: 58 months), with fibrosis in 3 (treated: 1; time on treatment: 108 months) and steatohepatitis in 2 (treated: 2; time on treatment: 69 and 185 months). One patient developed hepatocellular carcinoma. CONCLUSIONS: GD is a heterogeneous disease that causes different patterns of liver damage even during treatment. Although treatment improves the hepatocellular damage, it is associated with an increased rate of steatosis. This study highlights the importance of a follow-up of liver integrity in these patients.

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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.

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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.

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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.

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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

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La angiopatía amiloide cerebral (AAC) consiste en el depósito de amiloide en la pared de los vasos sanguíneos intracraneales, y conlleva a la aparición de hemorragia, isquemia o leucoencefalopatía. Las manifestaciones clínicas de la AAC son muy variables, tales como alteraciones cognitivas, alteraciones comportamentales, déficit neurológico focal, cefalea o crisis epilépticas. Un subtipo de angiopatía amiloide cerebral con inflamación relacionada (AAC-IR), se ha reportado recientemente en la literatura mundial. Presentamos el caso de una paciente de 74 años de edad, con un cuadro de demencia rápidamente progresiva de aproximadamente tres meses de evolución, asociada a cefalea, meningismo, disminución de la fuerza en hemicuerpo derecho, múltiples lesiones hemorrágicas parenquimatosas, hemosiderosis difusa, edema cerebral focal y estudio histológico con evidencia de amiloide intracerebral. El diagnóstico de la AAC se basa en una historia clínica compatible; neuroimagenes que demuestren hemosiderosis o múltiples hemorragias de predominio en fosa posterior, y en algunos casos estudio histológico que confirme la presencia de amiloide en la microcirculación intracraneal. Los criterios de Boston modificados unifican los hallazgos para el diagnóstico de AAC con diferentes grados de certeza. En algunas ocasiones, como en el caso presentado, la AAC se asocia a un componente inflamatorio, y se manifiesta con una demencia rápidamente progresiva, constituyéndose en un verdadero reto diagnóstico.

Cerebral amyloid angiopathy (CAA) is the deposition of amyloid in the wall of intracranial blood vessels, and leads to the appearance of hemorrhage, ischemia or leukoencephalopathy. The clinical manifestations of the CAA are highly variable, such as cognitive impairment, behavioral abnormalities, focal neurological deficits, headache or seizures. A subtype, Cerebral amyloid angiopathy-related inflammation (CAA-RI) has recently been reported in the world literature. We report the case of a 74-year-old with a rapidly progressive dementia about three months duration, associated with headache, meningismus, decreased strength in right hemisphere, multiple parenchymal hemorrhagic lesions, diffuse hemosiderosis, cerebral edema focal and histological study with evidence of intracerebral amyloid. The diagnosis of CAA is based on a clinical history compatible, neuroimaging showing hemosiderosis or multiple bleeding, predominantly in posterior fossa, and in some cases histological examination to confirm the presence of amyloid in intracranial microcirculation. Modified Boston criteria, unify the findings for the diagnosis of CAA, with varying degrees of certainty. Sometimes, as in the case presented, the CAA is associated with an inflammatory component, and is manifested by a rapidly progressive dementia, becoming a true diagnostic challenge.

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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

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Introducción: Se presenta la evaluación de la asociación entre la reserva de hierro (Fe) y los polimorfismos del gen de la hemocromatosis (HFE) en neonatos de alto riesgo perinatal. Métodos: Se incluyó una serie de neonatos de alto riesgo perinatal en los que se evaluó la reserva de Fe con la medición de la ferritina sérica (FS). Se dividieron en tres grupos: sobrecarga de Fe (SoFe), con FS >1,000 µg/l; reserva normal de Fe, con FS de 154-1,000 µg/l; y reserva baja de Fe, con FS <154 µg/l. Mediante PCR en tiempo real se buscaron las mutaciones C282Y, H63D y S65C del gen HFE. Resultados: Se estudiaron 97 neonatos. De ellos, 24 casos presentaron SoFe (proporción 0.247) y FS de 1,789 µg/l (IC 95% 1,376-2,201); 36 casos, reserva normal de FS (0.371), FS de 461 µg/l (389-533); y 37 casos, reserva baja de FS (0.381) y FS 82 µg/l (69-96). No hubo casos detectados para las mutaciones C282Y o S65C. Se identificó la variante H63D HFE en 18 neonatos (frecuencia génica de 0.185): la condición de heterocigoto (H63D/WT) en doce casos (frecuencia génica 0.124) y de homocigoto (H63D/H63D) en seis casos (frecuencia génica 0.062). La frecuencia alélica de H63D fue de 0.092. Los variante H63D HFE no mostró asociación con los neonatos de reserva normal de Fe contra reserva baja (OR 1.2; IC 95% 0.3-4.3) ni los de reserva normal contra neonatos con SoFe (OR 2.5; 0.7-9.2). Conclusiones: Cerca del 25% de neonatos de alto riesgo tendrá sobrecarga de Fe. Aún con el posible sesgo de selección, las variantes del gen HFE no influyen sobre el estado de la reserva de Fe.

Background: The association between iron stores (Fe) and HFE gene polymorphisms on high-risk neonates is shown. Methods: We included newborns with high perinatal risk. Newborns were divided into three groups for measurements of serum ferritin (SF): iron overload (IO) with SF 1000 µg/L, normal iron stores (NIS) with SF 154-1000 µg/L and low iron stores (LIS) with SF <154 µg/L. We used real-time PCR for identification of polymorphisms C282Y, H63DE, and S65C of the HFE gene. Results: We studied 97 newborns with IO in 24 cases (ratio 0.247) and SF 1789 µg/L (95% CI 1376-2201), NIS in 36 cases (0.371), and SF of 461 µg/L (389-533) and LIS in 37 cases (0.381) and SF 82 µg/L (69-96). There were no cases detected for C282Y or S65C mutations. We identified 18 neonates with H63D HFE variant (gene frequency 0.185) with heterozygous condition (H63D/ WT) in 12 cases (gene frequency 0.124) and homozygote (H63D/H63D) in six cases (gene frequency 0.062). H63D allele frequency was 0.092. The HFE H63D variant showed no association for comparing infants with NIS vs. LIS (OR 1.2, 95% CI 0.3-4.3) and NIS vs. IO newborn infant (OR 2.5, 0.7-9.2). Conclusions: In high-risk neonates ∼25% show IO even with the possible selection bias. HFE gene variants do not influence on the neonatal iron stores.

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Living donor liver transplantation (LDLT) demands a careful assessment of abnormal findings discovered during the evaluation process to determine if there will be any potential risks to the donor or recipient. Varying degrees of elevated hepatic iron levels are not uncommonly seen in otherwise healthy individuals. We questioned whether mild expression of hemosiderin deposition presents a safety concern when considering outcomes of living donation for both the donor and the recipient. We report on three LDLT patients who were found to have low- to moderate-grade hemosiderin deposition on liver biopsy. All other aspects of their evaluation proved satisfactory, and the decision was made to proceed with donation. There were no significant complications in the donors, and all demonstrated complete normalization of liver function postoperatively, with appropriate parenchymal regeneration. The recipients also had unremarkable postoperative recovery. We conclude that these individuals can be considered as potential donors after careful evaluation.

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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.

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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(AU)

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(AU)

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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.

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Fundamento: la neumonía recurrente en la infancia se define como la presencia de dos episodios de neumonía en un año o tres en cualquier período de tiempo, con radiografía de tórax normal entre los episodios. Existen múltiples causas que pueden producirla, entre ellas la hemosiderosis pulmonar idiopática, enfermedad poco frecuente en la edad pediátrica. La triada clásica que caracteriza a la hemosiderosis pulmonar idiopática no siempre es observada en los pacientes con esta enfermedad. En la actualidad existen resultados divergentes sobre el tratamiento, pronóstico y supervivencia de los pacientes estudiados. El objetivo es ilustrar la importancia de la identificación de los niños con criterios de neumonía recurrente, y así permitir el diagnóstico precoz de enfermedades subyacentes. Presentación de caso: niña de 3 años valorada por presentar tos e infiltrados recurrentes en la radiografía de tórax. Se realizó el diagnóstico de hemosiderosis pulmonar a partir de los datos clínicos, radiológicos y la obtención de macrófagos cargados con hemosiderina en el lavado bronquioalveolar. Conclusiones: la identificación oportuna de una niña de tres años con neumonía recurrente permitió el diagnóstico precoz de hemosiderosis pulmonar idiopática(AU)

Background: Recurrent pneumonia in childhood is defined as the presence of two episodes of pneumonia in a year or three in any period of time, with a normal thorax X-ray between the two episodes. There are several causes that may produce it, among them we find pulmonary idiopathic hemosiderosis, infrequent disease in pediatric age. The classic triad which characterizes the pulmonary idiopathic hemosiderosis is not always observed in patients with this disease. Today, there are divergent results about treatment, prognosis and survival of the patients studied. The objective of the work is to illustrate the importance of the identification in children with recurrent pneumonia criteria as well as to permit an early diagnosis of underlying diseases. Case presentation: A 3 year old girl was assessed for having cough and recurrent infiltrates in the thorax X-ray. The diagnosis of pulmonary hemosiderosis was given from the clinical data, radiological data and the procurement of macrophages loaded with hemosiderin at bronchi alveolar washing. Conclusion: The timely identification of a 3 years old girl with recurrent pneumonia permitted the early diagnosis of pulmonary idiopathic hemosiderosis(AU)

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Se denomina hemosiderosis pulmonar a los procesos caracterizados por depósitos anormales de hemosiderina en el parénquima pulmonar, secundarios a sangrados alveolares difusos y repetidos. Es una enfermedad de causa desconocida, poco frecuente, y en muchas ocasiones grave. En la mayoría de los pacientes se presenta en la primera década de la vida, sin predilección en cuanto a sexo. Se presenta una paciente de 7 años de edad, femenina, de piel blanca, con antecedentes de 22 ingresos desde la etapa de lactante por episodios recurrentes de dificultad respiratoria, interpretados como bronconeumonías, asociados a anemia aguda. Para el diagnóstico se realizó lavado broncoalveolar, y se observaron los macrófagos cargados de hemosiderina. La evaluación clínica y de laboratorio permitió excluir causas secundarias. Se instauró tratamiento con prednisona, con lo cual se logró una mejoría de la enfermedad. Se discuten los elementos clínicos, diagnósticos y terapéuticos de esta entidad(AU)

Idiopathic pulmonary hemosiderosis is those processes characterized by anomalous depots of hemosiderin in the pulmonary parenchyma, secondary to diffuse and repeated alveolar bleedings. It is an unknown disease, uncommon and mostly severe. It occurs in the first decade of life of most of the patients, regardless of sex. Here is a 7 years-old patient, female, Caucasian, with a history of 22 hospitalizations since she was a baby, due to recurrent episodes of respiratory distress diagnosed as bronchial pneumonias associated to acute anemia. For the diagnosis of this disease, bronchoalveolar lavage was performed and hemosiderin-loaded macrophages were observed. The clinical and lab evaluation excluded secondary causes. She was treated with prednisone and she improved her condition. The clinical, diagnosing and therapeutic elements of this disease were discussed(AU)

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