Idiopathic pulmonary hemosiderosis associated with Kabuki syndrome.

Kabuki syndrome (KS) is a genetic disorder caused by gene mutations in either lysine-specific methyltransferase 2D (KMT2D) or lysine demethylase 6A (KDM6A). This congenital disorder exhibits characteristic facial features, developmental delays in psychomotor skills, and skeletal abnormalities. Moreover, it is classified as a congenital immunodeficient disorder under the category of combined immunodeficiency, leading to hypogammaglobulinemia and the onset of autoimmune diseases. Here, we present the first case of KS complicated by idiopathic pulmonary hemosiderosis (IPH). The KS patient, a 2-year-old Japanese girl with a history of hypoplastic left heart syndrome and recurrent bacterial infection, developed severe respiratory distress and anemia. She had autoimmune hemolytic anemia and gouty nephropathy. Hemophagocytic macrophages with hemosiderin ingestion were identified in bronchoalveolar lavage fluid, excluding differential diagnoses and leading to the diagnosis of idiopathic pulmonary hemosiderosis. Intravenous prednisolone (2 mg/kg/day) was administered, but symptoms did not improve. However, pulmonary hemorrhage disappeared with methylprednisolone pulse therapy. IPH warrants consideration in cases where individuals with KS manifest idiopathic pneumonia and concurrent anemia.

Diffuse alveolar hemorrhage in pediatrics: Etiologies and outcomes.

BACKGROUND: Many conditions, including autoimmune disease and idiopathic pulmonary hemosiderosis (IPH), can cause diffuse alveolar hemorrhage (DAH). Little is known about the epidemiology and outcomes in children. OBJECTIVES: This retrospective cohort study sought to describe the etiologies and outcomes of DAH in pediatric patients at a tertiary care center. METHODS: This study involved review of patient records with diagnostic codes or bronchoscopy reports suggestive of pulmonary hemorrhage at a large children's hospital over 11 years (2010-2020). Patients were included if they met criteria for DAH, defined as bilateral pulmonary infiltrates and at least one of the following: (1) hemoptysis, (2) blood visible on bronchoscopic exam without apparent airway source, or (3) DAH noted on biopsy or autopsy. Infants less than 10 days corrected gestational age were excluded. RESULTS: Seventy-one children with DAH were included in the analysis. Cardiovascular disease was the most common etiology. Bleeding diathesis was common, but all patients had other causes of DAH. Patients with IPH were younger than those with autoimmune disease (p < .001). Most (77%) patients required mechanical ventilation, though this was less common among patients with autoimmune disease. Overall mortality was high (37%) but varied based on underlying etiology; mortality was higher in patients with cardiovascular disease (65%) while no deaths were seen in patients with autoimmune disease or IPH (p = .002). Survivors of DAH who performed pulmonary function tests had normal lung function. CONCLUSIONS: DAH frequently causes respiratory failure in children. In our cohort, mortality was highest in patients with cardiovascular disease.

A Rare Presentation of Late-Onset Idiopathic Pulmonary Hemosiderosis: A Case Report.

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH). It is associated with a high mortality rate and recurrent episodes of widespread alveolar hemorrhage and most commonly affects children. Here, we present a rare occurrence of late-onset idiopathic pulmonary hemosiderosis in a 74-year-old male. He was admitted for non-resolving pneumonia, hemoptysis, and type 1 respiratory failure, along with sideropenic anemia. Chest imaging showed bilateral upper lobe and right middle lobe alveolar opacities. Infective and autoimmune etiologies of diffuse alveolar hemorrhage were ruled out during the evaluation. Transbronchial lung biopsy showed patchy alveolar hemorrhage and abundant hemosiderin pigment deposition, revealing idiopathic pulmonary hemosiderosis. The patient was successfully treated with oral steroids, followed by complete radiological resolution without clinical relapse at one-year follow-up.

Cardiomyopathy in Celiac Disease: A Systematic Review.

(1) Background: Cardiomyopathy in celiac disease or celiac cardiomyopathy (CCM) is a serious and potentially life-threatening disease that can occur in both adults and children. However, data supporting the causal relationship between celiac disease (CD) and cardiomyopathy (CMP) are still inconsistent. The aim of this study was to review and synthesize data from the literature on this topic and potentially reveal a more evidence-based causal relationship. (2) Methods: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to search Medline, Embase, and Scopus databases from database inception until September 2023. A total of 1187 original articles were identified. (3) Results: We identified 28 CCM patients (19 adult and 9 pediatric) with a mean age of 27.4 ± 18.01 years. Adult patients with CCM were predominantly male (84.2%) while pediatric patients were predominantly female (75%). The most common comorbidities associated with CCM were anemia (75%) and pulmonary hemosiderosis (20%). In 35% of patients, CCM occurred before the diagnosis of CD, while in 48% of patients, CCM and CD were diagnosed at the same time. Diagnosis of CD preceded diagnosis of CCM in only 18% of patients. Diagnosis of CCM is often delayed with an average, from the onset of symptoms to diagnosis, of 16 months. All patients were treated with a gluten-free diet in addition to guideline-directed medical therapy. At 11-month follow-up, cardiovascular improvement was seen in 60.7% of patients. Pediatric mortality was 33.3%, while adult mortality was 5.3%. (4) Conclusions: Clinicians should be aware of the possible association between CD and CMP, and we recommend CD work-up in all patients with CMP who have concomitant anemia. While we identified only 28 cases in the literature, many cases might go unreported due to a lack of awareness regarding CCM. A high degree of clinical suspicion and a prompt diagnosis of CCM are essential to minimizing the risks of morbidity and mortality, as the combination of a gluten-free diet and guideline-directed medical therapy can improve clinical outcomes.

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.

Pulmonary function analysis of 21 children with idiopathic pulmonary hemosiderosis / 中华实用儿科临床杂志

Objective To evaluate the injury of pulmonary function of children with idiopathic pulmonary hemosiderosis(IPH) and the changes after treatment,and to provide some guidance for the diagnosis and treatment of IPH.Methods Twenty-one children with IPH who were admiued at Children's Hospital of Fudan University between June 2012 and May 2016 were selected.The pulmonary function and clinical data of them were analyzed.Results The general pulmonary function of 21 children with IPH before treatment with glucocorticoid was reported that 4 cases (19.05％) were normal and 17 cases (80.95％) were abnormal,including 11 cases (52.38％) with restrictive ventilatory disorder,4 cases (19.05％) with mixed ventilatory disorder,1 case (4.76％) with obstructive ventilatory disorder,and 1 case (4.76％) with small airway dysfunction.Pulmonary function test was performed on 15 cases after 1-2 months of treatment with glucocorticoid.The results showed that maximal vital capacity (VCmax％) vs.the expected value was (77.91 ± 18.86)％ vs.(60.43 ± 23.70)％,forced vital capacity (FVC％) vs.the expected value was (78.96 ±19.24)％ vs.(61.03 ±24.62)％ and forced expiratory volume in one second (FEV1％) vs.the expected value was (86.03 ± 21.69) ％ vs.(65.17 ± 26.89) ％,which were significantly higher than those before treatment,and the differences were statistically significant (t =-4.13,-4.01,-4.54,all P ＜ 0.05).Three cases were followed up for 18 to 40 months by detecting pulmonary function and the results of dynamic monitoring of pulmonary function showed a fluctuation in FVC％ [case 1:(69.6-84.2) ％;case 2:(56.1-73.7) ％;case 3:(40.4-70.2) ％].Conclusion The characteristic pulmonary function changes in children with IPH are restrictive ventilatory disorder.Pulmonary function test play a significant role in diagnosis,treatment and prognosis of IPH.

Three Cases of Pulmonary Hemosiderosis with Long-term Treatment of Deflazacort in Children / 임상소아혈액종양

Idiopathic pulmonary hemosiderosis (IPH) is a rare respiratory disease with an unknown etiology, and is diagnosed with laboratory, radiology, and pathology tests. Chief complaints of IPH include hemoptysis, cough, and dyspnea. Since it is considered an immune-mediated disease, the first line of treatment is systemic corticosteroid therapy. The three cases reported here showed a decrease in ferritin level and improvement in the hemoglobin level with prednisolone treatment. However, long-term corticosteroid therapy may cause several side effects, particularly growth retardation and obesity, which can affect growing children. In the present study, all patients had cushingoid symptoms and obesity. Therefore, we switched to deflazacort (DFZ), which has lesser side-effects of weight gain. This report describes clinical courses of the disease and comparison of body mass index of three patients with IPH who took DFZ instead of prednisolone. DFZ was effective for IPH, and is useful for weight gain reduction.

Three Cases of Pulmonary Hemosiderosis with Long-term Treatment of Deflazacort in Children / 임상소아혈액종양

Idiopathic pulmonary hemosiderosis (IPH) is a rare respiratory disease with an unknown etiology, and is diagnosed with laboratory, radiology, and pathology tests. Chief complaints of IPH include hemoptysis, cough, and dyspnea. Since it is considered an immune-mediated disease, the first line of treatment is systemic corticosteroid therapy. The three cases reported here showed a decrease in ferritin level and improvement in the hemoglobin level with prednisolone treatment. However, long-term corticosteroid therapy may cause several side effects, particularly growth retardation and obesity, which can affect growing children. In the present study, all patients had cushingoid symptoms and obesity. Therefore, we switched to deflazacort (DFZ), which has lesser side-effects of weight gain. This report describes clinical courses of the disease and comparison of body mass index of three patients with IPH who took DFZ instead of prednisolone. DFZ was effective for IPH, and is useful for weight gain reduction.

Clinical follow-up studies of pediatric idiopathic pulmonary hemosiderosis / 中华实用儿科临床杂志

Objective To describe the clinical characteristics of idiopathic pulmonary hemosiderosis (IPH),and to investigate the possible risk factors for poor prognosis.Methods The clinical data of 41 patients with IPH were retrospectively analyzed,22 cases were divided into survival group and death group according to the follow-up data and prognosis,and the related clinical factors in 2 groups were statistically analyzed.Results Of the 41 patients with IPH,14 cases were male,27 cases female.The median age of onset was 3.8 years,and the diagnosed median age was 4.6 years.The common clinical features of the 41 children with IPH included pale,cough,hemoptysis,fever,and fatigue,in 34 cases (82.9％),27 cases (65.8％),16 cases (39.0％),13 cases (31.7％) and 11 cases (26.8％) children,respectively.Initial symptoms included pale,cough,hemoptysis,in 27 cases (65.9％),13 cases (31.7 ％),and 11 cases (26.8％) children,respectively.Accessory examinations revealed microcytic hypochromic anemia (average hemoglobin concentration was 65.2 g/L),and 40 patients (97.6％)had an abnormal chest X-ray.Forty patients were treated with glucocorticoids,and all of them had symptom remission.Twenty-two patients were followed up for 6 months to 9 years,and 6 cases (27.3％)died during follow-up and 16 cases (72.7％) still alive.There were no statistically significant differences between death group and survival group as to age of onset,gender distribution,age on diagnosis,degree of anemia,and clinical features(all P ＞ 0.05).The incidence of jaundice was significantly different between the 2 groups (P ＜ 0.05).Conclusions IPH has diverse clinical manifestations and a high rate of misdiagnosis.A routine chest Xray film should be taken for patients with moderate to severe microcytic hypochromic anemia.The conditions of some pediatric patients can be stabilized with glucocorticoids treatment,but the associated mortality rate remains high.History of jaundice may be a risk factor for poor prognosis.

Clinical study of 25 children with idiopathic pulmonary hemosiderosis / 中华实用儿科临床杂志

Objective To analyze the clinical features,laboratory results,treatment and prognosis of children with idiopathic pulmonary hemosiderosis (IPH).Methods The documents of 25 children with IPH,who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from Apr.1992 to Nov.2008 were reviewed.Then some of the patients were followed up.Results The median age of onset was 4 years old (6.5 months to 8 years old).Those patients aged from 3 to 6 years old ranked the first as 48％ (12 cases).The median age at diagnosis was 5.17 years old.The course of desease was between 10 days and 6 years,the median course was 1 year.Cough,pallor,fever and hemoptysis were the common clinical features of IPH patients.The chest radiological patterns for IPH were diverse.The common features of high resolution CT scan in 15 patients included declined transparency and ground glass shadows in 11 cases,cloudy patchy infiltrate in 9 cases,reticular changes in 6 cases,and nodular changes in 2 children.Twentythree cases were once misdiagnosed and 60％ of them were delayed in diagnosis as IPH for more than 1 year.Glucocorticoid therapy was effective in improving symptoms.Some patients suffered from rheumatoid arthritis later in their life.Conclusions The manifestations of IPH in children are nonspecific,therefore it is easily to be misdiagnosed.Combined chest radiographic presentations with repeatedly looking for hemosiderin-laden macrophages in sputum,gastric aspirate or bronchoalveolar lavage fluid are helpful in diagnosis.Glucocorticoid therapy can control the symptoms.Some relationships may exist between IPH and rheumatoid arthritis.

A Case of Pulmonary Hemosiderosis that was Dissolved by an Oral Prednisolone and the Milk Avoidance / 소아알레르기및호흡기학회지

A 29-month-old boy presented with fever, dyspnea, and paleness. He was initially diagnosed with pneumonia and severe sepsis. Although he was treated with intravenous antibiotics and high dose methylprednisolone, dyspnea and paleness recurred two times. Under suspicion of pulmonary hemosiderosis, we performed video-assisted thoracoscopic lung biopsy and bronchoalveolar lavage on him and found hemosiderin-laden macrophages in both specimens. Despite thorough history and laboratory examination, we could not find any pathologic or serologic evidence for primary and secondary causes of pulmonary hemosiderosis except for one that indicating Heiner's syndrome. After taking oral prednisolone he showed improvement of anemia and dyspnea, which was maintained by milk avoidance. Based on the history and the existence of immunoglobulin G antibodies against milk components, we are considering it as the case of Heiner's syndrome.

Hemosiderosis pulmonar idiopática / Idiopathic pulmonary hemosiderosis

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.

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.

Hemosiderosis pulmonar idiopática / Idiopathic pulmonary hemosiderosis

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)

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.

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.(AU)

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.(AU)

A Case of Pulmonary Siderosis Mimicking Metastatic Lung Cancer / 결핵및호흡기질환

Pulmonary siderosis is a pneumoconiosis caused by chronic iron inhalation. A diagnosis of pulmonary siderosis is based on a patient history of iron inhalation, on chest radiographic findings, and on accumulation of iron oxide in macrophages within the lung. A typical radiographic finding of pulmonary siderosis includes ill-defined micronodules that are diffusely distributed in the lung. We experienced a 52-year-woman with a 1.3x1.5-cm mass in the left upper lobe with multiple nodules in both lungs. Because the radiographic findings were atypical, we conducted a video-assisted thorascopic lung biopsy procedure to exclude the diagnosis of metastatic lung cancer. After confirming iron deposition in the lung tissue and knowing the patient's occupational history of welding iron, we concluded that this was a case of pulmonary siderosis.

A Case of Pulmonary Siderosis Mimicking Metastatic Lung Cancer / 결핵및호흡기질환

Pulmonary siderosis is a pneumoconiosis caused by chronic iron inhalation. A diagnosis of pulmonary siderosis is based on a patient history of iron inhalation, on chest radiographic findings, and on accumulation of iron oxide in macrophages within the lung. A typical radiographic finding of pulmonary siderosis includes ill-defined micronodules that are diffusely distributed in the lung. We experienced a 52-year-woman with a 1.3x1.5-cm mass in the left upper lobe with multiple nodules in both lungs. Because the radiographic findings were atypical, we conducted a video-assisted thorascopic lung biopsy procedure to exclude the diagnosis of metastatic lung cancer. After confirming iron deposition in the lung tissue and knowing the patient's occupational history of welding iron, we concluded that this was a case of pulmonary siderosis.

Maintenance therapy with dose-adjusted 6-mercaptopurine in 15 cases of idiopathic pulmonary hemosiderosis / 中国综合临床

Objective To review the diagnosis of idiopathic pulmonary hemosiderosis ( IPH),and to evaluate the efficacy of maintenance therapy with dose-adjusted 6-mercaptopurine (6MP) in IPH children. Methods The diagnosis of IPH was confirmed by in-patient examination and at least 1 year follow-up to exclude secondary causes of pulmonary hemorrhage. Fifteen children met the criteria of IPH and were enrolled. The age at diagnosis was 2-13 years ( median 7 years). Prednisone was administered at 2 mg/( kg·d) for 4 weeks in acute phase of the disease followed by taper. 6MP was also started at 60 mg/( m2·d) simultaneously and continued for 3 years. Results The diagnosis was delayed in most children, which was due to the lack of initial classical manifestation of the disease. The time between the onset of symptoms and diagnosis ranged from 2 weeks to 108 months ( median 8 months) . All the patients exhibited response to the initial treatment and prednisone was successfully tapered off. Only 1 of 8 patients with relative leucopenia (3 × 109/L -6 × 109/L) on 6MP maintenance recurred while 5 of 7 others recurred (P < 0. 05) during median 6-year (range 2. 5 - 9. 5 years) follow-up. Of the latter 5 patients who recurred,4 remained recurrence-free after adjusting the dose of 6MP upwards to keep relative leucopenia. Conclusions Diagnostic delayed is still a main problem in pediatric IPH. Most IPH children in our group tolerated maintenance treatment with 6MP and achieved long-term remission, and these suggested growth retardation on long-term steroids therapy could be avoided. Because of interindividual difference in 6MP metabolism, adjusting the dose of 6MP may be necessary for treatment of IPH children and avoid under-treatment or overtreatment in some children,and thus improve the prognosis. White blood count could be a simple and useful indicator to predict clinical response in most IPH children on 6MP.

Hemosiderosis pulmonar idiopática en adultos: presentación de tres casos y revisión de la literatura / Idiopathic pulmonary hemosiderosis in adults: a report of three cases and literature review

La hemosiderosis pulmonar idiopática (HPI) es una enfermedad rara de etiología desconocida, caracterizada por episodios recurrentes de hemorragia alveolar difusa (HAD). Existen múltiples condiciones asociadas con HAD, la mayoría de los casos ocurren en asociación con enfermedades sistémicas autoinmunes. La HPI es un diagnóstico de exclusión que se utiliza para describir los casos de HAD en los que no se encuentra una condición asociada. El objetivo del siguiente trabajo es la presentación de tres casos de HPI en mujeres adultas, revisión de la bibliografía disponible y por ultimo, remarcar la importancia de la biopsia pulmonar en el diagnostico definitivo de la HAD.

Idiopathic pulmonary hemosiderosis (IPH) is a rare lung disease of unknown etiology, characterized by recurrent episodes of diffuse alveolar hemorrhage (DAH). There are several conditions associated with DAH and most of them occur in association with systemic autoimmune diseases. IPH is diagnosed after other identifiable causes of alveolar bleeding have been excluded. The objectives of this paper is to present three cases of IPH in adult women, to review the literature and to underline the importance of lung biopsy in the definitive diagnosis of the DAH.

A case of idiopathic pulmonary hemosiderosis with seasonal recurrence / 소아과

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease affecting mostly children. This disorder is characterized by recurrent episodes of hemoptysis, bilateral diffuse pulmonary infiltrates, and iron-deficiency anemia. An acute fulminant alveolar hemorrhage can be fatal due to respiratory failure, while chronic hemorrhage leads to hemosiderin-laden macrophages and pulmonary fibrosis. Genetic, autoimmune, allergic, environmental, and metabolic mechanisms of pathogenesis have been suggested, but the etiology of IPH remains unknown. We report on a 9-year-old girl with idiopathic pulmonary hemosiderosis who showed seasonal recurrences without cause.