Pulmonary Manifestations in Cerebrotendinous Xanthomatosis
スポンサーリンク
概要
- 論文の詳細を見る
We investigated five cases with cerebrotendinous xanthomatosis (CTX) with particular reference to biochemical and pathological pulmonary disorders. To date, few reports discuss the pathophysiology of pulmonary disorders of CTX patients. This study is the first investigation of such pulmonary disorders. All 5 patients had no pulmonary symptoms and no disturbances on radiological studies and pulmonary function tests. However, in bronchoalveolar lavage (BAL) fluids, many cells with cruciform reflexes, which is characteristic of intracellular sterol accumulation, were found under phase contrast microscopy. Biochemically, cholestanol was found to be increased in the BAL fluid as well as in serum. Pathological findings of transbronchial lung biopsy (TBLB) samples disclosed foamy macrophages and small granulomas in alveolar septa. In conclusion, the lung was apparently involved in CTX, and the lesions were characterized with the accumulation of foamy and giant cells with a high concentration of cholestanol, which likely results in the formation of foreign body granulomas.(Internal Medicine 37: 922-926, 1998)
- 社団法人 日本内科学会の論文
著者
-
OSAME Mitsuhiro
The Third Department of Internal Medicine, Faculty of Medicine,Kagoshima University
-
Osame Mitsuhiro
The Third Department Of Internal Medicine Faculty Of Medicine Kagoshima University
-
Kuriyama Masaru
The Third Department Of Internal Medicine Faculty Of Medicine Kagoshima University
-
KAWABATA Masaharu
the Third Department of Internal Medicine, Kagoshima University
-
Kuriyama Masaru
The Third Department Of Internal Medicine Kagoshima University School Of Medicine
-
MORI Shinitirou
the Third Department of Internal Medicine, Kagoshima University School of Medicine
-
SAKASHITA Izumi
the Third Department of Internal Medicine, Kagoshima University School of Medicine
-
Mori Shinitirou
The Third Department Of Internal Medicine Kagoshima University School Of Medicine
-
Kawabata Masaharu
The Third Department Of Internal Medicine Kagaoshima University School Of Medicine
-
Kawabata Masaharu
The Third Department Of Internal Medicine Kagoshima University School Of Medicine
-
Sakashita Izumi
The Third Department Of Internal Medicine Kagoshima University School Of Medicine
-
Osame Mitsuhiro
The Third Department Of Internal Medicine Faculty Of Medicine
関連論文
- Clinical Course of HTLV-I-Associated Uveitis
- ASSESSMENT OF MITOCHONDRIAL GENE IN PROLIFERATIVE VITREORETINAL TISSUES FROM PATIENTS WITH FAMILIAL DIABETES MELLITUS
- Statistically Significant Differences in the Number of CD24 Positive Muscle Fibers and Satellite Cells between Sarcoglycanopathy and Age-Matched Becker muscular Dystrophy Patients
- LYSOSOMAL GLYCOGEN STORAGE DISEASE WITHOUT ACID MALTASE DEFICIENCY : LECTIN HISTOCHEMICAL STUDIES
- Logistic Model Analysis of Neurological Findings in Minamata Disease and the Predicting Index
- Adult onset limb-girdle type mitochondrial myopathy with a mitochondrial DNA np8291 A-to-G substitution
- Facioscapulohumeral Muscular Dystrophy: Clinical Diversity and Genetic Abnormalities in Japanese Patients
- Nationwide Survey of Neurologic Manifestations of Acquired Immunodeficiency Syndrome in Japan
- A Hemophiliac with Human Immunodeficiency Virus (HIV)-1-Associated Dementia Complex
- IMMUNOHISTOCHEMICAL STUDY OF CYTOCHROME C OXIDASE WITH A MONOCLONAL ANTIBODY IN MITOCHONDRIAL MYOPATHY
- Molecular Analysis of Hereditary Deficiency of the Third Component of Complement (C3) in TWO Sisters
- Phenotypes of X-linked Charcot-Marie-Tooth disease and altered trafficking of mutant Connexin 32 (GJB1)
- Familial Skeletal Myopathy with Atrioventricular Block
- An Autopsy Case of Peripheral Neuroepithelioma in Posterior Mediastinum with p53 Point Mutation
- Hermansky-Pudlak Syndrome with a Novel Mutation
- CHANGES IN SERUM LIPOPROTEIN(A) AND C4B-BINDING PROTEIN LEVELS AFTER ACUTE MYOCARDIAL INFARCTION
- RISK FACTORS IN NORMOLIPIDEMIC MALE PATIENTS WITH CORONARY ARTERY DISEASE IN JAPANESE
- Pulmonary Dirofilariasis with Serologic Study on Familial Infection with Dirofilaria immitis
- Midkine (MK) Expression in Extraembryonic Tissues, Amniotic Fluid, and Cerebrosphinal Fluid during Mouse Embryogenesis
- HTLV-I-associated myelopathy
- Neuropathology of human T lymphotropic virus type I-associated myelopathy
- II-C-12 The reduced expression of capillary tight junction comprised proteins in a patient with myopathy(THE 45TH ANNUAL MEETING OF THE JAPAN SOCIETY OF HISTOCHEMISTRY AND CYTOCHEMISTRY)
- Pleural Cryptococcosis with Idiopathic CD4 Positive T-lymphocytopenia
- A Clinical Study of Adult Human Parvovirus B19 Infection
- Adult T-cell Leukemia (ATL) Cells which Express Neural Cell Adhesion Molecule (NCAM) and Infiltrate into the Central Nervous System
- Expression of Vascular Endothelial Growth Factor in Pulmonary Aspergilloma
- Circulating levels of MMP-1, -2, -3, -9, and TIMP-1 are increased in POEMS syndrome
- VEGF Is Causative for Pulmonary Hypertension in a Patient with Crow-Fukase (POEMS) Syndrome
- Eosinophilic Pneumonia with Eosinophilic Gastroenteritis
- Two Cases of Idiopathic CD4^+ T-lymphocytopenia in Elderly Patients
- Bronchiectasis with Myeloperoxidase Antineutrophil Cytoplasmic Antibody and Bactericidal/Permeability-Increasing Protein Antineutrophil Cytoplasmic Antibody
- Idiopathic CD4+ T-Lymphocytopenia in Chronic Obstructive Pulmonary Disease
- Pulmonary Manifestations in Cerebrotendinous Xanthomatosis
- A Patient with Acute-Onset HAM/TSP after Blood Transfusion Complicated with Pseudopseudohypo-parathyroidism
- Predictive Value of Serial Platelet Count and VEGF Determination for the Management of DIC in the Crow-Fukase (POEMS) Syndrome
- A Clinical Study of Adult Human Parvovirus B19 Infection
- Ehlers-Danlos Syndrome Combined with von Recklinghausen Neurofibromatosis
- Human T-Cell Lymphotropic Virus Type-I (HTLV-I)-Associated Myelopathy/Tropical Spastic Paraparesis with Acute Type of Adult T-Cell Leukemia
- Adenosine and Neopterin Levels in Cerebrospinal Fluid of Patients with Neurological Disorders
- Increased Midkine Gene Expression in Human Gastrointestinal Cancers
- Idiopathic Hypoparathyroidism with Fungal Seminal Vesiculitis
- Human T Lymphotropic Virus Type I (HTLV-I)-Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP): Recent Perspectives
- Angiographic Morphology of the Posterior Communicating Artery and Basilar Artery in Patients with ICA-PComA Aneurysm
- Human T-cell Lymphotropic Virus Type I and Systemic Lupus Erythematosus
- MOLECULAR PATHOLOGY OF HTLV-I-ASSOCIATED MYELOPATHY/TROPICAL SPASTIC PARAPARESIS
- Multifocal Relapsing-remitting Myelitis in a Patient with Atopic Dermatitis: Multiple Sclerosis or Atopic Myelitis?
- Expression of Vascular Endothelial Growth Factor in Pulmonary Aspergilloma.
- Lymphocyte Subsets in Muscle Biopsies of Human T-Lymphotropic Virus Type I Carriers with Polymyositis.
- Dystrophin-Related Protein in Becker Muscular Dystrophy.
- Diseases among Men Living in Human T-Lymphotropic Virus Type I Endemic Areas in Japan.