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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The literature suggests that 10% to 20% of adult patients with neurofibromatosis have associated interstitial lung disease. Characteristics of such involvement, as present in the case reported herein, include bilateral lower lobe fibrosis and may include bullous and cystic changes in advanced cases. In addition to pulmonary fibrosis, neurofibromatosis may have other intrathoracic associations; including "dumbbell" neurofibromas, intercostal neurofibromas, and intrathoracic meningoceles.
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PMID:Neurofibromatosis and interstitial lung disease. 9 88

Fibrosing alveolitis, or interstitial pulmonary fibrosis, is a common manifestation of neurofibromatosis, and was observed in 7 of 70 patients with the disease. Though neurofibromatosis is congenital, fibrosing alveolitis does not appear until adulthood, and occurs in 20% of patients with the disease who are over 30 years old. Characteristic radiographic findings include linear, interstitial density, and large upper lobe bullae; this combination limits the differential diagnosis. Pathological examination demonstrates alveolar wall thickening progressing to fibrosis and lung destruction. Pulmonary function tests can show obstructive or restrictive lung disease.
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PMID:Fibrosing alveolitis in patients with neurofibromatosis. 40 16

We report three patients with neurofibromatosis, lung parenchymal, and pleural changes. Lung fibrosis occurs in 10% of patients with neurofibromatosis. Pleural involvement is less common. We are presenting one such case.
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PMID:[Pulmonary parenchymal and pleural fibrosis as an expression of Recklinghausen's neurofibromatosis]. 313 68

The basic abnormality of neurofibromatosis consists not only of a maldevelopment of the neuroectoderm but also of the mesoderm. Therefore any organ or system of the body may be involved. Following a short clinical review of the central (acoustic) and peripheral type the well known and the more unusual radiographic findings are summarized. They include cranial and intracranial manifestations (orbitosphenoid dysplasia, bone defects of the skull, acoustic neuroma, glioma of the optic nerve and chiasm, meningioma), spinal lesions (scoliosis, vertebral scalloping, meningocele, neuroma, ependymoma), skeletal abnormalities (pseudarthrosis), cardiovascular manifestations, pulmonary fibrosis, tumors of the gastrointestinal and urinary tract and different endocrinopathies. Some of the roentgenologic symptoms are very characteristic and allow definitive diagnosis.
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PMID:[Radiological findings in Recklinghausen's neurofibromatosis]. 641 23

The neurofibromatosis type I (NF I, von Recklinghausen's disease) is an autosomal dominant neurocutaneous disorder with systemic involvement. The respiratory manifestations include: chest wall deformities, upper airway obstruction by neurofibromas, parenchymal neurogenic tumours, pulmonary fibrosis, cystic lung disease, primary pulmonary hypertension, central hypoventilation, diaphragm paralysis. We present 4 cases of NF I associated with chronic respiratory diseases. The common feature is the presence of severe chest deformities. The bullous emphysema was present in 2 of them. All 4 patients suffered from depressive syndrome with neuroleptic use.
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PMID:[Respiratory manifestations in von Recklinghausen's disease]. 1706 22