ABSTRACT
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.
Subject(s)
Humans , Female , Infant, Newborn , Hemosiderosis/drug therapy , Hemosiderosis/diagnostic imaging , Methylprednisolone , Prednisone , Radiography, Thoracic , Adrenal Cortex Hormones/therapeutic use , Fluticasone , Hemoptysis/etiology , Hemosiderosis/complicationsABSTRACT
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.
ABSTRACT
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.
Subject(s)
Child , Humans , Body Mass Index , Cough , Dyspnea , Ferritins , Hemoptysis , Hemosiderosis , Obesity , Pathology , Prednisolone , Weight GainABSTRACT
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.
Subject(s)
Child , Humans , Body Mass Index , Cough , Dyspnea , Ferritins , Hemoptysis , Hemosiderosis , Obesity , Pathology , Prednisolone , Weight GainABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
Subject(s)
Child , Humans , Anemia, Iron-Deficiency , Hemoptysis , Hemorrhage , Hemosiderosis , Lung Diseases , Macrophages , Pulmonary Fibrosis , Rare Diseases , Recurrence , Respiratory Insufficiency , SeasonsABSTRACT
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.
ABSTRACT
Idiopathic pulmonary hemosiderosis (IPH), a rare disease of unknown etiology, is characterized by iron deficiency anemia and chronic recurrent pulmonary symptoms such as cough, hemoptysis, and dyspnea. Recurrent intra-alveolar hemorrhage resulting in hemosiderin accumulation and progressive fibrosis often leads to death. We experienced a case of IPH in a two-year-old male who was presented with cough, dyspnea, hemoptysis and anemia. The diagnosis was confirmed by hemosiderin-laden macrophages in a gastric aspirate. He was initially treated with oral iron and prednisolone for one year. But pulmonary symptoms such as cough and hemoptysis recurred five times while he was on oral prednisolone and the side effects of moon face and truncal obesity developed. So inhaled steroid (budesonide) was administered for 18 months, with improvement of pulmonary symptoms. This suggests that early treatment with inhaled steroids may work in IPH without the serious side effects of systemic steroid. The effect of inhaled steroid should be evaluated as the initial treatment of IPH.
Subject(s)
Child , Humans , Male , Anemia , Anemia, Iron-Deficiency , Cough , Diagnosis , Dyspnea , Fibrosis , Hemoptysis , Hemorrhage , Hemosiderin , Hemosiderosis , Inhalation , Iron , Macrophages , Obesity , Prednisolone , Rare Diseases , SteroidsABSTRACT
Idiopathic pulmonary hemosiderosis (IPH), a rare disease of unknown etiology, is characterized by iron deficiency anemia and chronic recurrent pulmonary symptoms such as cough, hemoptysis, and dyspnea. Recurrent intra-alveolar hemorrhage resulting in hemosiderin accumulation and progressive fibrosis often leads to death. We experienced a case of IPH in a two-year-old male who was presented with cough, dyspnea, hemoptysis and anemia. The diagnosis was confirmed by hemosiderin-laden macrophages in a gastric aspirate. He was initially treated with oral iron and prednisolone for one year. But pulmonary symptoms such as cough and hemoptysis recurred five times while he was on oral prednisolone and the side effects of moon face and truncal obesity developed. So inhaled steroid (budesonide) was administered for 18 months, with improvement of pulmonary symptoms. This suggests that early treatment with inhaled steroids may work in IPH without the serious side effects of systemic steroid. The effect of inhaled steroid should be evaluated as the initial treatment of IPH.
Subject(s)
Child , Humans , Male , Anemia , Anemia, Iron-Deficiency , Cough , Diagnosis , Dyspnea , Fibrosis , Hemoptysis , Hemorrhage , Hemosiderin , Hemosiderosis , Inhalation , Iron , Macrophages , Obesity , Prednisolone , Rare Diseases , SteroidsABSTRACT
Idiopathic pulmonary hemosiderosis is characterized by cough, hemoptysis, dyspnea, diffuse pulmonary infiltrates, and microcytic and hypochromic anemia. The cause of this illness is unknown. We experienced a case of idiopathic pulmonary hemosiderosis in a 2 year and 8 month-old boy. Hemosiderin-laden macrophages are demonstrated in smears of material obtained from tracheal aspirates. There were no specific causes for pulmonary hemorrhage. We report a case of idiopathic pulmonary hemosiderosis with brief review of related literatures.
Subject(s)
Humans , Infant , Male , Anemia, Hypochromic , Cough , Dyspnea , Hemoptysis , Hemorrhage , Hemosiderosis , MacrophagesABSTRACT
Objective To explore the clinical characteristics,diagnosis,treatment and misdiagnosis matter of children with idiopathic pulmonary hemosiderosis(IPH).Methods The data of clinical characteristics,laboratory examination,treatment and follow-up of 21 children admitted from Jun.1993 to May 2007 were retrospectively analyzed,included 9 males,12 females,aged 1-14 years old,course of di-sease were 1-6 months.Twenty-one patients were diagnosed as IPH by chest X-ray radiography,CT scan,bone marrow biopsy,hemosiderin-laden macrophages in either sputum or gastric juice or bronchoalveolar lavage fluid.Then,therapy and prognosis of IPH were analyzed.Results All patients had varied degrees of anemia,11(52.38%) children had cough,9 (42.86%) children had fever,6(28.50%) cases had shortage of orexia,4( 19.05%) children had hemoptysis.Chest X-ray radiography and CT scan demonstrated diffuse patchy,nodular,reticulate pattern.Eighteen children received bone marrow biopsy and presented hyperplastic erythropoiesis,4(22.2%) cases were accompanied iron deficiency anemia.Nineteen (90.80%) cases shown the presence of hemosiderin-laden macrophages in either sputum or gastric juice or bronchoalveolar lavage fluid.Twenty-one misdiagnosed patients consisted of bronchopneumonia combined anemia(8 cases),lung tuberculosis combined anemia(5 cases),nulli-iron anemia(4 cases),hemolytic anemia(3 cases),myelodysplastic syndrome(1 case)and received corticosteroid therapy.Four cases of all patients were associatated with large-dose human-?-globulin and 3 cases were associatated with vincristine therapy.The therapeutic effect was significant.Eighteen patients were followed-up,3 patients were of which cured and had stopped treatment for over 2 years,11 patients presented clinically persistent remission,4 patients were recurred and aggravated.Conclusions Early diagnosis and long-term therapy of corticosteroid are very important for controlling acute onset,lessening the frequency of IPH recurrence and improving prognosis of the disease.