[Fibrosing alveolitis in a 7-year-old girl with autoimmune hemolytic anemia and autoimmune hepatitis]. 1991

K Paul, and L Wille, and D Feist, and K Kayser
Kinderklinik der Universität Heidelberg.

A Coombs-positive haemolytic anaemia with proof of heat autoagglutinins and autoantibodies against cell nuclei was diagnosed in a girl, now 7 1/2 years of age, in the sixth month of her life as a sequel to jaundice. Shortly afterwards liver biopsy yielded a confirmed diagnosis of giant cell hepatitis. The signs of the disease receded under treatment with decortin and imurek. An attempt to discontinue the medication at 5 1/2 years of age resulted in a histologically confirmed recurrence of autoimmune hepatitis. When the patient was 5 years old, the x-ray revealed a mainly interstitial pneumonia; at 6 years of age, bronchiectases in the region of the left lower lobe were confirmed by bronchography. Resection of the lower lobe was performed after occurrence of atelectasis. Histopathological findings confirmed fibrotic alveolitis associated with giant cells. Based on the overall clinical course and the histological and immunological findings, autoimmune etiology of fibrotic alveolitis must be discussed. Progression of the disease could be avoided during the past 6 months by intensifying the immunosuppressive prednisolone therapy.

UI MeSH Term Description Entries
D011239 Prednisolone A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states. Di-Adreson-F,Predate,Predonine,Di Adreson F,DiAdresonF
D011658 Pulmonary Fibrosis A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death. Alveolitis, Fibrosing,Idiopathic Diffuse Interstitial Pulmonary Fibrosis,Fibroses, Pulmonary,Fibrosis, Pulmonary,Pulmonary Fibroses,Alveolitides, Fibrosing,Fibrosing Alveolitides,Fibrosing Alveolitis
D001995 Bronchography Radiography of the bronchial tree after injection of a contrast medium. Bronchographies
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005260 Female Females
D006505 Hepatitis INFLAMMATION of the LIVER. Hepatitides
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000744 Anemia, Hemolytic, Autoimmune Acquired hemolytic anemia due to the presence of AUTOANTIBODIES which agglutinate or lyse the patient's own RED BLOOD CELLS. Anemia, Hemolytic, Idiopathic Acquired,Autoimmune Haemolytic Anaemia,Autoimmune Hemolytic Anemia,Cold Agglutinin Disease,Hemolytic Anemia, Autoimmune,Acquired Autoimmune Hemolytic Anemia,Anemia, Hemolytic, Cold Antibody,Cold Antibody Disease,Cold Antibody Hemolytic Anemia,Idiopathic Autoimmune Hemolytic Anemia,Agglutinin Disease, Cold,Anaemia, Autoimmune Haemolytic,Anemia, Autoimmune Hemolytic,Autoimmune Haemolytic Anaemias,Autoimmune Hemolytic Anemias,Cold Agglutinin Diseases,Cold Antibody Diseases,Haemolytic Anaemia, Autoimmune
D001261 Pulmonary Atelectasis Absence of air in the entire or part of a lung, such as an incompletely inflated neonate lung or a collapsed adult lung. Pulmonary atelectasis can be caused by airway obstruction, lung compression, fibrotic contraction, or other factors. Atelectasis, Congestive,Lung Collapse,Atelectasis,Compression Atelectasis,Compression Pulmonary Atelectasis,Congestive Atelectasis,Congestive Pulmonary Atelectasis,Contraction Pulmonary Atelectasis,Postoperative Pulmonary Atelectasis,Resorption Atelectasis,Resorption Pulmonary Atelectasis,Atelectases,Atelectases, Compression,Atelectases, Compression Pulmonary,Atelectases, Congestive,Atelectases, Congestive Pulmonary,Atelectases, Contraction Pulmonary,Atelectases, Postoperative Pulmonary,Atelectases, Pulmonary,Atelectases, Resorption,Atelectases, Resorption Pulmonary,Atelectasis, Compression,Atelectasis, Compression Pulmonary,Atelectasis, Congestive Pulmonary,Atelectasis, Contraction Pulmonary,Atelectasis, Postoperative Pulmonary,Atelectasis, Pulmonary,Atelectasis, Resorption,Atelectasis, Resorption Pulmonary,Collapse, Lung,Compression Atelectases,Compression Pulmonary Atelectases,Congestive Atelectases,Congestive Pulmonary Atelectases,Contraction Pulmonary Atelectases,Postoperative Pulmonary Atelectases,Pulmonary Atelectases,Pulmonary Atelectases, Compression,Pulmonary Atelectases, Congestive,Pulmonary Atelectases, Contraction,Pulmonary Atelectases, Postoperative,Pulmonary Atelectases, Resorption,Pulmonary Atelectasis, Compression,Pulmonary Atelectasis, Congestive,Pulmonary Atelectasis, Contraction,Pulmonary Atelectasis, Postoperative,Pulmonary Atelectasis, Resorption,Resorption Atelectases,Resorption Pulmonary Atelectases
D001327 Autoimmune Diseases Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides. Autoimmune Disease,Disease, Autoimmune,Diseases, Autoimmune

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