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      Cor triatriatum sinister: a rare underlying cause of pulmonary hemosiderosis

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          Abstract

          Pulmonary hemosiderosis is a disorder with unknown cause and characterized by hemosiderin appreciation in alveolar interstitium from decomposed hemoglobin following alveolar capillary bleeding, which finally leads to pulmonary fibrosis. It can be divided into primary and secondary types in terms of its etiology. While primary types are related to autoimmunity, secondary types can be associated with cardiovascular and pulmonary causes such as mitral stenosis leading to pulmonary congestion. We report a case of cor triatriatum sinister in a child who presented with hemoptysis as a main clinical manifestation and had been previously diagnosed with idiopathic pulmonary hemosiderosis. Based on clinical signs and imaging examinations, we considered the hemoptysis was most likely due to cor triatriatum. The child underwent corrective surgery with uneventful recovery. The hemoptysis has not recurred any more after operation. Cardiovascular disease including cor triatriatum should be considered with regards to the etiology of pulmonary hemosiderosis.

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          Idiopathic pulmonary haemosiderosis revisited.

          Idiopathic pulmonary haemosiderosis is a rare cause of diffuse alveolar haemorrhage of unknown aetiology. It occurs most frequently in children, has a variable natural history with repetitive episodes of diffuse alveolar haemorrhage, and has been reported to have a high mortality. Many patients develop iron deficiency anaemia secondary to deposition of haemosiderin iron in the alveoli. Examination of sputum and bronchoalveolar lavage fluid can disclose haemosiderin-laden alveolar macrophages (siderophages), and the lung biopsy shows numerous siderophages in the alveoli, without any evidence of pulmonary vasculitis, nonspecific/granulomatous inflammation, or deposition of immunoglobulins. Contrary to earlier reports, corticosteroids alone or in combination with other immunosuppressive agents may be effective for either exacerbations or maintenance therapy of idiopathic pulmonary haemosiderosis.
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            Prognosis in pediatric idiopathic pulmonary hemosiderosis.

            Previously, IPH patients have been reported to have an average survival of 2.5 years. However, at our institution, many IPH patients have survived longer than that. Therefore, we conducted this study to determine the clinical course and current mortality of pediatric IPH patients treated with immunosuppressants. Retrospective chart review. Children's hospital. Seventeen patients in whom IPH was diagnosed between 1972 and 1998. Mean age at diagnosis was 4.5 +/- 3.5 years, and 12 patients were female. At diagnosis, all patients had anemia and pulmonary infiltrates; 85% had hypoxemia, 65% had hemoptysis, and 70% had fever. The diagnosis was made by open lung biopsy in 13 patients (76%), hemosiderin-laden macrophages in BAL fluid in 1 patient (6%), hemosiderin-laden macrophages in gastric aspirate in 2 patients (12%), or by clinical presentation alone in 1 patient (6%). The mean duration of follow-up for all patients was 3.6 +/- 3.4 years (range, 0.7 to 10.2). Initial treatment consisted of prednisone only in 14 patients (82%), and prednisone and hydroxychloroquine in two patients (12%). Thirteen patients (76%) required long-term corticosteroids because of recurrent hemoptysis. Eight patients (47%) required other immunosuppressants (hydroxychloroquine or azathioprine) in addition to prednisone to control their hemoptysis. One patient who was not treated with prednisone remained asymptomatic for 1.8 years. Three patients (17%) died of acute massive pulmonary hemorrhage (4.1 +/- 5.0 years postdiagnosis). Five-year survival for IPH patients in our study was 86% (by Kaplan-Meier method). We conclude that these IPH patients who received long-term treatment had a better outcome than those previously reported who were not treated with extended courses of immunosuppressive therapy. We speculate that long-term immunosuppression therapy may improve the prognosis in IPH.
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              Long-term clinical course of patients with idiopathic pulmonary hemosiderosis (1979-1994): prolonged survival with low-dose corticosteroid therapy.

              Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by recurrent episodes of pulmonary symptoms such as cough, hemoptysis, and dyspnea. Our study consisted of 23 patients: 12 males and 11 females with IPH. The diagnosis was based on history, presence of anemia, and characteristic chest X-ray, and was confirmed by showing macrophages laden with hemosiderin in gastric washings or bronchoalveolar lavage and/or open lung biopsy. All but one patient were diagnosed in our department between 1979-1994. There was a history of multiple blood transfusions for anemia in 10 patients. Consanguinity between parents was noted in 11 patients. Severe pallor, cough, hemoptysis, and hepatomegaly were the most common findings on physical examination. All but 2 patients had hypochromic microcytic anemia of varying severity. In 12 children, moderate reticulocytosis was noted. Corticosteroids were administered with doses ranging from 5 mg every other day to 2 mg/kg/day depending on the severity of the episodes (duration of disease from 2-14 years). It is our impression that patients with IPH, benefit from long-term steroid treatment which in turn results in a milder course. Long-term low-dose steroid treatment appeared to prevent crises and assured a prolonged survival.
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                Author and article information

                Contributors
                409505388@qq.com
                xiaoyunbinrui@126.com
                rockyman_luo@163.com
                chenrenwei@sina.com
                hp_911@sohu.com
                liupingbo88299@sina.com
                +8615200911861 , justindxc@gmail.com
                Journal
                Springerplus
                Springerplus
                SpringerPlus
                Springer International Publishing (Cham )
                2193-1801
                24 February 2016
                24 February 2016
                2016
                : 5
                : 150
                Affiliations
                [ ]Department of Cardiothoracic Surgery, Hunan Children’s Hospital, No. 86 Ziyuan Road, Changsha, 410007 Hunan China
                [ ]Department of Cardiology, Hunan Children’s Hospital, Changsha, 410007 Hunan China
                [ ]Medical School, University of South China, Hengyang, China
                Article
                1752
                10.1186/s40064-016-1752-1
                4766185
                27026847
                8b3ab439-bba8-4686-9675-5de7d2eaea09
                © Peng et al. 2016

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

                History
                : 5 November 2015
                : 12 February 2016
                Categories
                Case Study
                Custom metadata
                © The Author(s) 2016

                Uncategorized
                cor triatriatum,pulmonary hemosiderosis,hemoptysis,surgery
                Uncategorized
                cor triatriatum, pulmonary hemosiderosis, hemoptysis, surgery

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