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.

Early Initiation of Steroid-sparing Drugs in Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis is conventionally treated withsteroids, prolonged usage of which maybe deleterious anddisease often recurs on tapering. We initiated hydroxy-chloroquine and azathioprine early in treatment along withsteroids in seven children with idiopathic pulmonaryhemosiderosis, and observed that early introduction of secondline immunosuppressants helped in reducing disease flare andsteroid toxicity without serious adverse effects.

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 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.

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.

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.

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.

Lane-Hamilton Syndrome: Association or Coincidence.

The combination of idiopathic pulmonary hemosiderosis (IPH) and celiac disease (CD) is rare. The clinical importance of this association is that a significant improvement can be obtained with gluten free diet not only in intestinal but also in pulmonary symptoms. A four and half-years old girl was admitted with complaints of cough, difficulty in breathing and paleness. She had intermittent episodes of abdominal pain and diarrhea. She had dyspnea and tachycardia, and oxygen saturation 88%. The patient was diagnosed with CD and concomitant IPH. With gluten-free diet and corticosteroid treatment, both intestinal and pulmonary symptoms were controlled.

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.

Serodiagnosis of celiac disease in children referred for evaluation of anemia: A pediatric hematology unit's experience.

Background: Children presenting with typical clinical features of celiac disease (CD) are diagnosed relatively easily, however, diagnosis remains challenging and is often delayed when they present with 'difficult to treat anemia' without overt gastrointestinal manifestations. Index study was undertaken to report profile of patients referred to pediatric hematology unit with 'difficult anemia' who subsequently were diagnosed with CD. Materials and Methods: The records of 83 patients (1988-2008) with CD were scrutinized retrospectively who had presented with predominant hematological manifestations. Results: CD was confirmed histologically in 31 (37%), while 52 (63%) were diagnosed by serology alone. The mean age at diagnosis was 8.0 ± 2.8 years. The mean duration of symptom-diagnosis interval was 40.9 ± 30.6 months. Eighty-one (98%) children had anemia (Hb < 11 g/dl) and 55 (66%) had received iron supplements without discernible benefit. Thirty-nine (47%) patients received a blood transfusion. Thirty-six (43%) patients did not have diarrhea. Majority of the patients had either a microcytic-hypochromic (48%) or dimorphic (43%) anemia. Twenty-four (33%) had thrombocytosis, while 5 (7%) had thrombocytopenia. Mean duration of follow-up for patients on roll in the clinic for more than six months was 17.7 ± 20.9 months. Conclusion: Pediatricians and hematologists need to be aware of the extra-intestinal manifestations of CD. Prolonged duration of symptoms and a diagnosis at a relatively older age is striking in children presenting with predominantly hematological manifestations. Investigations for CD are recommended in children presenting with iron deficiency anemia refractory to hematinics or who have coexisting growth retardation. Necessity for biopsy in overtly symptomatic cases is discussed.

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.

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.

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.

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.

Idiopathic Pulmonary Hemosiderosis in 50 Children / 实用儿科临床杂志

Objective To explore the feature of clinical and laboratory and therapy in childern with idiopathic pulmonary hemosiderlsis(IPH).Methods The clinical and laboratory datas of 50 cases with IPH were studied from Jan.1996 to Jan.2006.The hemogram,the metabolism of ferrum,the cellular test of bone marrow,finding the hemosiderin cells in sputum or gastric juice or bronchovesicular juice and ma-nifestation were analyzed.Results IPH were often found in children aged 6 to 14 years old.Coungh and spitting blood and anemia were the most important clinical feature.X-ray manifestations were various.The diagnosis of IPH was based on clinical feature and finding out the he-mosiderin cells in sputum or gastric juice or bronchovesicular juice.X-ray and CT manifestations were important.At the same time,the secon-dary IPH was removed.The clinical symptom of IPH were relieved by using adrenal cortical hormone.Conclusions There is no specificity in children with IPH.X-ray,CT manifestations and finding the hemosiderin cells in sputum or gastric juice are helpful in diagnosis.Using adrenal cortical hormone will release symptom and prognosis.

The Correlation Between Imaging Findings and Pulmonary Function Changes of Idiopathic Pulmonary Hemosiderosis / 实用放射学杂志

Objective To analyze imaging findings of IPH and to study the correlation between the HRCT findings and the pulmonary function of IPH.Methods The information of 15 children with IPH were reviewed.Results The X-ray findings of IPH included ground glass opacity(n=9),exudative shadows(n=10),reticulonodular shadows(n=7),nodular shadows(n=6).The HRCT signs included abnormal bronchovascular bundles(n=12),ground glass opacity(n=12),exudative shadows ( n=11 ) ,disseminated micronodular shadows ( n=8 ) , thickened pulmonary lobular septa ( n=9 ) . The correlation between ground glass opacity and pulmonary function was 0.5394 ( P=0.134); The correlation between thickened pulmonary lobular septa and the pulmonary function was 0.9963(P=0.0001).Conclusion The HRCT examination is better than the X-ray examination. The positive correlation between thickened pulmonary lobular septa and pulmonary function change is significant,it is beneficial to estimate the damage of the palmonary.

A Case of Idiopathic Pulmonary Hemosiderosis: Long Term Follow-up / 소아알레르기및호흡기학회지

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology; is characterized by bleeding into the alveoli and progressive pulmonary fibrosis. The clinical and paraclinical findings consist of recurrent hemoptysis, pulmonary infiltrates, and iron deficiency anemia. Examinations of sputum disclose large numbers of hemosiderin-laden alveolar macrophage. Pulmonary interstitial fibrosis may develop. We experienced a case of recurrent IPH in an eight and half year-old girl who had been initially admitted at the age of 12 months old for the same complaints: cough, fever, and recurrent episodes of hemoptysis and iron deficiency anemia. Hemosiderin laden macrophages were demonstrated in smears of material obtained from gastric juices. There were no specific causes for the pulmonary hemorrhage. We reported a case of IPH with sequential findings on plain chest radiograph and high resolution computed tomography.