[Pulmonary hemosiderosis in systemic lupus erythematosus in a child].
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The most common cause of anaemia in childhood is iron deficiency. Anaemia due to poor dietary iron is seen most often between 9-24 months of age in infants being fed with large amounts of milk during periods of high growth rate. After the age of two years one must look for other causes of iron deficiency, particularly blood loss. In the description of three cases of idiopathic pulmonary haemosiderosis, the problems of severe chronic anaemia in childhood are discussed. Idiopathic pulmonary haemosiderosis is a rare disease in children, characterized by iron deficiency, anaemia and pulmonary symptoms. It has a high mortality, mostly due to acute pulmonary haemorrhage. However, early diagnosis is important, as some therapeutical regimens have succeeded in delaying the pulmonary haemorrhages and a few patients have possibly gained complete remission.
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A case is presented of spontaneous pulmonary haemosiderosis in a four-year-old boy. During three years of follow-up a severe disease episode occurred in the child, requiring treatment in Intensive Therapy Unit. Good therapeutic effect was achieved after combination of corticosteroid treatment with imuran.
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Superficial siderosis of the central nervous system (SSC) typically involves slowly progressive ataxia, hypoacusis and dementia, possibly with pyramidal signs and sphincter disturbances in combination with xanthochromic CSF with siderophages. However, there are also atypical oligosymptomatic forms. Before the era of magnetic resonance imaging (MRI), diagnosis was only possible at autopsy. Nowadays a firm diagnosis can be made during life by demonstrating the typical marginal hypointense signal in the T2-weighted images in the cerebrum, brain stem, cerebellum and spinal cord. Contrast-enhanced computed tomography may demonstrate widespread meningeal enhancement, but this sign is not specific for SSC. We present an oligosymptomatic case of SSC with slowly progressive ataxia and slight hypoacusis. The etiology, gross pathological and histopathological findings, differential diagnosis and therapy are discussed.
Idiopathic pulmonary haemosiderosis (IPH) is a rare disease characterized by recurrent episodes of intrapulmonary bleeding, chronic iron deficiency anaemia and pulmonary fibrosis. IPH is a diagnosis made by exclusion of other causes. It occurs in both adults and children. Other conditions than IPH can cause pulmonary haemosiderosis. The etiology is unknown, but might be an immunological mechanism causing a defect in the basement membrane of the pulmonary capillary. IPH should be suspected in patients with recurrent episodes of coughing, haemoptysis, dyspnoea and anaemia. Chest X-ray shows pulmonary infiltrates during an acute attack. Examination of sputum or lung biopsy discloses large numbers of haemosiderin-laden pulmonary macrophages. The mortality-rate is high, but the prognosis is difficult to evaluate because many patients survive for a long time either with a course of recurrent attacks or with chronic symptoms, such as dyspnoea and persistent anaemia. Steroids may improve the condition of the patient during a bleeding episode.
Idiopathic pulmonary haemosiderosis (IPH) is a rare disease of unknown aetiology. The clinical and paraclinical findings consist of recurrent haemoptysis, pulmonary infiltrates and iron deficiency anaemia. Examination of sputum or bronchoalveolar lavage fluid obtained at fiberoptic bronchoscopy discloses large numbers of haemosiderin-laden alveolar macrophages. Pulmonary interstitial fibrosis may develop. As pulmonary haemosiderosis can be observed in association with several diseases, IPH is basically a diagnosis made by exclusion of other causes. The treatment consists of immunosuppression with steroids and cytotoxic drugs.
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