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The respiratory system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Idiopathic pulmonary haemosiderosis is rare, affecting mainly males. There is progressive alveolar replacement by haemosiderin-laden macrophages, and interstitial and intra-alveolar fibrosis. The cause is unknown and the diagnosis is one of exclusion.
Noninfectious Pulmonary Manifestations of Renal Disease In Children
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Stephen T. Lawless, H. Jorge Baluarte
Idiopathic pulmonary hemosiderosis is a disease occurring most frequently in childhood and in young adults. It is characterized by dyspnea, cyanosis, fever, cough, blood-streaked sputum, and pallor, with laboratory evidence of iron-deficiency anemia. The basic pathologic process is blood extravasation in the pulmonary alveoli by repeated hemorrhages. A chest roentgenogram shows a variety of findings from bilateral perihilar patchy infiltration to diffuse pulmonary infiltration. Clearing of the pulmonary fields can occur with treatment but the lesions may progress to interstitial fibrosis.
Radiology of Interstitial Lung Disease in Children
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Fredric A. Hoffer, John A. Kirkpatrick
Idiopathic pulmonary hemosiderosis is a disease that often appears after the first year of life but within the first decade. It is characterized by cough, hemoptysis, and an iron deficiency anemia. It is occasionally a manifestation of allergy to cow’s milk. During acute bleeding into the lung, i.e., alveoli, there are multiple areas of consolidation. However, as the blood is reabsorbed or after multiple bouts of bleeding, there is accumulation of hemosiderin in the interstitial spaces (Figures 18A), which leads to multiple interstitial nodules (Figure 18B).
Chronic interstitial lung diseases in children: diagnosis approaches
Published in Expert Review of Respiratory Medicine, 2018
Nadia Nathan, Laura Berdah, Keren Borensztajn, Annick Clement
DAH can be observed in various clinical situations with or without systemic diseases. In the absence of systemic findings, isolated pulmonary capillaritis should be discussed with the search for positivity of the anti-glomerular basement membrane antibody with linear deposits in the lung tissue biopsy as well as suggestive serologic features such as p-ANCA antibodies. Cow’s milk allergy (Heiner’s syndrome) and diseases induced by environmental factors such as pesticide should also be evocated. Idiopathic pulmonary hemosiderosis is a diagnosis of exclusion based on patient presentation with acute, subacute, or recurrent DAH, on the results of lung biopsy showing evidence of ‘bland’ pulmonary hemorrhage (ie, without capillaritis or vasculitis), and after exclusion of the conditions listed above. In this situation, red blood cells leak into the alveolar space without evidence of damage and/or inflammation of the alveolar capillaries [50].
Assessment of liver and cardiac iron overload using MRI in patients with chronic anemias in Latin American countries: results from ASIMILA study
Published in Hematology, 2018
Rodolfo Cancado, Nora P. Watman, Clarisse Lobo, Zulay Chona, Fernando Manzur, Fabiola Traina, Miriam Park, Guillermo Drelichman, Juan Pablo Zarate, Luis Marfil
Patients aged >10 years and with a confirmed diagnosis of homozygous sickle cell anemia (SCA), low- and intermediate–1- International Prognostic Scoring System (IPSS) risk myelodysplastic syndrome (MDS), aplastic anemia (AA), Diamond-Blackfan anemia (DBA), congenital sideroblastic anemias, and other rare anemias were included in the study. Patients were required to have received (in their lifetime history) <20 units (or ∼100 mL/kg) of red blood cell (RBC) transfusions with SF levels >2000 ng/mL or ≥20 units (or ∼100 mL/kg) of RBC transfusions regardless of SF level. Patients with thalassemia and any disease with hemosiderosis not related to blood transfusion (ie, hereditary hemochromatosis, idiopathic pulmonary hemosiderosis, etc), or with a life expectancy of <12 months, or pregnant women were excluded from the study. Patients with SCA facing clinical infections or pain crisis, at the time of enrollment or during sample collection and those for which MRI evaluation was contraindicated were also excluded.
Celiac disease and idiopathic pulmonary hemosiderosis: a literature review of the Lane–Hamilton syndrome
Published in Postgraduate Medicine, 2022
Stavros Tryfon, Efthymia Papadopoulou, Georgios Psarros, Michael Agrafiotis, Maria Saroglou
Diffuse alveolar hemorrhage (DAH) is a clinical entity where alveolar hemorrhage originates from the pulmonary microcirculation in several conditions involving immune and nonimmune mechanisms. Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of DAH [5], with an estimated prevalence at 0.24–1.23/million. It is more common in children and, although in adults it had been generally diagnosed under the age of 30 [6,7], recent data showed that over half of the adult cases were diagnosed above the age of 30 years [8].