The Experts below are selected from a list of 5262 Experts worldwide ranked by ideXlab platform
Steven M. Stolz - One of the best experts on this subject based on the ideXlab platform.
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Treatment of Life-threatening Primary Pulmonary Hemosiderosis with Cyclophosphamide
Chest, 1992Co-Authors: John L. Colombo, Steven M. StolzAbstract:This report describes a five-year-old boy with severe pulmonary hemorrhage caused by primary pulmonary Hemosiderosis with cow's milk sensitivity. After failing to respond to corticosteroids and azathioprine, he dramatically improved after being given cyclophosphamide. He worsened after discontinuation of cyclophosphamide on two occasions and improved significantly with its reinstitution. Cyclophosphamide was continued for 14 months without further bleeding or adverse effects. The patient has remained in remission for nearly five years. Cyclophosphamide may be a life-saving alternative therapy for refractory pulmonary hemorrhage with pulmonary Hemosiderosis.
John L. Colombo - One of the best experts on this subject based on the ideXlab platform.
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Treatment of Life-threatening Primary Pulmonary Hemosiderosis with Cyclophosphamide
Chest, 1992Co-Authors: John L. Colombo, Steven M. StolzAbstract:This report describes a five-year-old boy with severe pulmonary hemorrhage caused by primary pulmonary Hemosiderosis with cow's milk sensitivity. After failing to respond to corticosteroids and azathioprine, he dramatically improved after being given cyclophosphamide. He worsened after discontinuation of cyclophosphamide on two occasions and improved significantly with its reinstitution. Cyclophosphamide was continued for 14 months without further bleeding or adverse effects. The patient has remained in remission for nearly five years. Cyclophosphamide may be a life-saving alternative therapy for refractory pulmonary hemorrhage with pulmonary Hemosiderosis.
Antoine Deschildre - One of the best experts on this subject based on the ideXlab platform.
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Down syndrome and pulmonary Hemosiderosis: an under-recognized association
Rare ILD DPLD, 2018Co-Authors: Aurelia Alimi, Jessica Taytard, Rola Abou Taam, Véronique Houdouin, Aude Forgeron, Marc Lubrano Lavadera, Pierrick Cros, Isabelle Gibertini, Jocelyne Derelle, Antoine DeschildreAbstract:Introduction: Pulmonary Hemosiderosis is a rare cause of interstitial lung disease in children. The French experience had previously highlighted that 20% of the 25 included children also had Down syndrome (DS). In this study populations, the main features of the patients with Hemosiderosis were unspecific stigmata of autoimmune disease. This study aims to investigate the relationships between pulmonary Hemosiderosis and DS. Material and methods: Patients with pulmonary Hemosiderosis followed is one of RespiRare network and younger than 20 years old at diagnosis were selected. The following data were collected : DS status, clinical, biological, functional, and radiological findings. Results: Nine of the 34 included patients (26%) had DS. They were a girl predominance (72%) in the non-DS group, and a male predominance in the DS group (56%). The mean age at the diagnosis was 3.80±3.30 with no significant difference between DS and non-DS patients. DS patients tended to present a more severe form of the disease with more dyspnoea (p=0.03) and more pulmonary arterial hypertension (PAH) (p=0.0003). The 3 (9%) patients who died were DS patients (p=0.0003). Discussion and conclusions: DS seem to be a risk factor for Hemosiderosis. Hemosiderosis in DS patients is more severe at presentation, and at follow-up. Several hypotheses can be proposed for such association: an increased fragility of the lung capillary, a higher susceptibility to autoimmune lesions, and a higher risk of chronic hypoxia, leading to PAH. In DS, Hemosiderosis should be considered in patients with anaemia of unknown origin.
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Pulmonary Hemosiderosis in children with Down syndrome: a national experience
Orphanet Journal of Rare Diseases, 2018Co-Authors: Aurelia Alimi, Jessica Taytard, Véronique Houdouin, Aude Forgeron, Pierrick Cros, Isabelle Gibertini, Jocelyne Derelle, Rola Abou Taam, Marc Lubrano Lavadera, Antoine DeschildreAbstract:Background Pulmonary Hemosiderosis is a rare and complex disease in children. A previous study from the French RespiRare® network led to two important findings: 20% of the children presented with both pulmonary Hemosiderosis and Down syndrome (DS), and at least one tested autoantibody was found positive in 50%. This study investigates the relationships between pulmonary Hemosiderosis and DS. Methods Patients younger than 20 years old and followed for pulmonary Hemosiderosis were retrieved from the RespiRare® database. Clinical, biological, functional, and radiological findings were collected, and DS and non-DS patients’ data were compared. Results A total of 34 patients (22 girls and 12 boys) were included, among whom nine (26%) presented with DS. The mean age at diagnosis was 4.1 ± 3.27 years old for non-DS and 2.9 ± 3.45 years old for DS patients. DS patients tended to present a more severe form of the disease with an earlier onset, more dyspnoea at diagnosis, more frequent secondary pulmonary hypertension, and an increased risk of fatal evolution. Conclusions DS patients have a higher risk of developing pulmonary Hemosiderosis, and the disease seems to be more severe in this population. This could be due to the combination of an abnormal lung capillary bed with fragile vessels, a higher susceptibility to autoimmune lesions, and a higher risk of evolution toward pulmonary hypertension. A better screening for pulmonary Hemosiderosis and a better prevention of hypoxia in DS paediatric patients may prevent a severe evolution of the disease.
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Pulmonary Hemosiderosis in children with Down syndrome: a national experience
Orphanet Journal of Rare Diseases, 2018Co-Authors: Aurelia Alimi, Jessica Taytard, Véronique Houdouin, Aude Forgeron, Marc Lubrano Lavadera, Pierrick Cros, Isabelle Gibertini, Jocelyne Derelle, Rola Abou Taam, Antoine DeschildreAbstract:BACKGROUND: Pulmonary Hemosiderosis is a rare and complex disease in children. A previous study from the French RespiRare® network led to two important findings: 20% of the children presented with both pulmonary Hemosiderosis and Down syndrome (DS), and at least one tested autoantibody was found positive in 50%. This study investigates the relationships between pulmonary Hemosiderosis and DS.METHODS: Patients younger than 20 years old and followed for pulmonary Hemosiderosis were retrieved from the RespiRare® database. Clinical, biological, functional, and radiological findings were collected, and DS and non-DS patients' data were compared.RESULTS: A total of 34 patients (22 girls and 12 boys) were included, among whom nine (26%) presented with DS. The mean age at diagnosis was 4.1 ± 3.27 years old for non-DS and 2.9 ± 3.45 years old for DS patients. DS patients tended to present a more severe form of the disease with an earlier onset, more dyspnoea at diagnosis, more frequent secondary pulmonary hypertension, and an increased risk of fatal evolution.CONCLUSIONS: DS patients have a higher risk of developing pulmonary Hemosiderosis, and the disease seems to be more severe in this population. This could be due to the combination of an abnormal lung capillary bed with fragile vessels, a higher susceptibility to autoimmune lesions, and a higher risk of evolution toward pulmonary hypertension. A better screening for pulmonary Hemosiderosis and a better prevention of hypoxia in DS paediatric patients may prevent a severe evolution of the disease.
Robert R.m. Gifford - One of the best experts on this subject based on the ideXlab platform.
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Histologic resolution of documented Hemosiderosis in a renal transplant recipient. A case report.
Transplant international : official journal of the European Society for Organ Transplantation, 1992Co-Authors: Zakiyah Kadry, Harold C. Yang, Robert R.m. GiffordAbstract:A 33-year-old cadaveric renal transplant recipient showed complete histologic resolution of Hemosiderosis by liver biopsies obtained pre- and post-transplantation. Although there have been reports in the past of progression of Hemosiderosis to hemochromatosis to severe liver failure in the renal transplant population, the correlation has never been clear. This is the first case report of complete resolution of Hemosiderosis as documented histologically by liver biopsies in a cadaveric renal transplant recipient.
Dong Jik Ahn - One of the best experts on this subject based on the ideXlab platform.
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Renal Hemosiderosis secondary to intravascular hemolysis after mitral valve repair: A case report.
Medicine, 2020Co-Authors: In Hee Lee, Gun Woo Kang, Chang-yeon Kim, Sun-jae Lee, Min-kyung Kim, Dong Jik AhnAbstract:RATIONALE Renal Hemosiderosis is a disease in which hemosiderin deposits in the renal cortex as a form of iron overload. However, cases of renal Hemosiderosis due to intravascular hemolysis following mitral valve repair have been rarely reported. PATIENT CONCERNS We present the case of a 62-year-old woman who developed asymptomatic urinary abnormalities including microscopic hematuria and proteinuria due to renal Hemosiderosis following a mitral valve repair surgery performed two years earlier. DIAGNOSES A percutaneous renal biopsy showed no specific glomerular abnormality, tubular atrophy, or interstitial fibrosis but extensive deposition of hemosiderin in the proximal tubule. The patient was diagnosed with renal Hemosiderosis and chronic intravascular hemolysis following mitral valve repair. INTERVENTIONS Our patient refused a mitral valve repeat surgery and hence was treated with oral iron preparations, N-acetylcysteine, and a β-receptor blocker. OUTCOMES Moderate mitral regurgitation with the regurgitant blood striking against the annuloplasty ring was confirmed on follow-up echocardiography. After the 24-month follow-up period, hemolytic anemia persisted, but there was no significant decline of renal function. LESSONS For cases of chronic intravascular hemolysis accompanied with asymptomatic urinary abnormalities, a renal biopsy is required to exclude underlying kidney pathology and predict potential renal insufficiency.