Idiopathic Pulmonary Hemosiderosis
Lourdes R. Laraya-Cuasay, Walter T. Hughes in Interstitial Lung Diseases in Children, 2019
Pulmonary hemosiderosis (PH) is a rare disease which primarily affects children and adolescents, and was described first by Virchow in 1864. It is characterized by acute and chronic blood loss which occurs from pulmonary capillaries and often results in the triad of hemoptysis, interstitial pulmonary infiltrates, and anemia. The presentation and clinical course of idiopathic PH (IPH) are variable and depend upon the intensity, duration and frequency of the hemorrhages. Chronic and acute blood loss occurs in IPH, and since the iron contained in hemosiderin trapped within the lung is only scantily reutilized for erythropoiesis, iron deficiency anemia develops in most cases. Pulmonary functions improve during remissions, and may revert to normal before interstitial fibrosis supervenes. Cardiovascular disease leading to pulmonary venous hypertension, especially mitral valve stenosis, can cause diffuse pulmonary hemorrhage and PH. Pulmonary symptoms generally precede renal involvement, and although the clinical course is variable, the disease is often fatal despite treatment with corticosteroids, immunosuppressives and plasmapheresis.
Venous Thrombosis
C. Alan B. Clemetson in Vitamin C, 1989
R. Virchow outlined three variables operating in the pathogenesis of thrombosis: coagulability of the blood, impedence to the flow of blood, and changes in the vessel wall. M. Lamy reported pleural effusions, hemosiderosis, and adrenal hypertrophy, as well as venous and arterial thrombosis, in young adults who died of malnutrition following release from German prison camps at the end of World War II. Pelvic vein thrombosis presents a diagnostic problem and a serious hazard following certain forms of pelvic surgery. Abnormalities of ascorbic acid metabolism due to surgery, etc. may play a more important role than does a simple dietary deficiency of the vitamin. Much research and many theories have been devoted to the cause of deep-vein thrombosis and embolism; especially as regards the increased risk of these occurrences and other forms of cardiovascular disease in women taking the old high-dose combined estrogen-progestagen birth control pills.
Tryptophan Metabolism
C. Alan B. Clemetson in Vitamin C, 1989
The essential amino acid l-tryptophan is used in the body for many purposes besides the building of protein molecules and its complex conversion to nicotinic acid for the synthesis of NAD and NADP. Y. S. Cho-Chung and H. C. Pitot isolated and purified the enzyme tryptophan pyrrolase which catalyses the irreversible step in the degradative metabolism of tryptophan in mammalian liver to nicotinic acid and nicotinamide, a series of reactions which accounts for the niacinsparing effect of tryptophan in the rat. D. Y. Cooper isolated an enzyme which catalysed the hydroxylation of tryptophan to 5-hydroxytryptophan. In a tryptophan loading study of Bantu patients with hemosiderosis and chronic scurvy, L. V. Hankes et al. found significantly increased levels of anthranilic acid glucuronide, acetylkynurenine, kynurenine, and hydroxykynurenine in the urine. In studies of both the enzyme tryptophan-5-hydroxylase and also 5-hydroxytryptophan decarboxylase, the hydroxylation step was found to be the rate-limiting reaction in the formation of serotonin.
Severe heart failure, dilated cardiomyopathy and pulmonary haemosiderosis in coeliac disease: report of two cases
Published in Paediatrics and International Child Health, 2014
Banani Poddar, Upender Shava, Anshu Srivastava, Aditya Kapoor
Coeliac disease (CD) is a chronic inflammatory, multi-system disorder with protean manifestations which has been linked to various auto-immune-mediated disorders. Dilated cardiomyopathy (DCM) is a rare extra-intestinal manifestation that is being recognised increasingly in patients with CD. Two cases of CD are described, an 18-year-old boy and a 13-year-old girl, both of whom presented with rapid onset of congestive heart failure and severe left ventricular systolic dysfunction. Upper limb venous thrombosis and recurrent haemoptysis secondary to pulmonary haemosiderosis in the second case were the other unusual features. The importance of CD screening of patients with DCM and pulmonary haemosiderosis is emphasised.
New Concept in Natural History and Management of Diabetes Mellitus in Thalassemia Major
Published in Hemoglobin, 2009
Ratna Chatterjee, Rekha Bajoria
Diabetes Mellitus is a major endocrinopathy, which occurs due transfusional haemosiderosis and is found in 20–30% of adult patients with β-thalassaemia worldwide, accounting for significant morbidity. It is multifactorial with iron loading being the dominant cause and its management poses a clinical challenge. Diabetes in thalassaemia patients is distinct from type 2 diabetes. It is peculiar in many aspects including its pathophysiology and occurs due to insulin resistance as well as islet cell insufficiency. This article reviews the natural history of diabetes in this presentation with emphasis on prevention monitoring and management. Use of MRI techniques may be useful for future monitoring as well as biochemical monitoring to prevent complications of diabetes. Early intervention with intensified chelation may reverse pancreatic function and structural changes as evident from MRI.
Safety And Efficacy Of 4 Years Of Deferasirox Treatment For Sickle Cell Disease Patients
Published in Hemoglobin, 2013
Efthymia Vlachaki, Maria Mainou, Eleni Bekiari, Evaggelia Vetsiou, Apostolos Tsapas
Deferasirox (DFRA) is a novel oral chelator agent for treatment of iron overload. Although well established in the treatment of β-thalassemia major (β-TM), it has not yet been fully investigated in patients with sickle cell disease. The aim of this report is to present the preliminary results of a pilot study assessing the effect of 4 years of DFRA treatment in six patients with sickle cell disease who are in need of recurrent transfusions. Our results show a significant reduction of ferritin levels and improvement of liver hemosiderosis, assessed by means of magnetic resonance imaging T2* (MRI T2*). None of the patients presented any serious adverse effects and the treatment was well tolerated. These results are in accordance with previous studies about the use of DFRA in sickle cell disease.
Related Knowledge Centers
- Hemochromatosis
- Hemosiderin
- Mononuclear Phagocyte System
- Lung
- Iron Overload
- Transfusional Iron Overload
- Idiopathic Pulmonary Haemosiderosis