Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This work reviews the most frequent late effects seen in long-term survivors and how they relate to individual therapeutic modalities: a) Growth: severe growth retardation is seen in patients treated by radiation therapy, related to dose, anatomical site and age of patient, along with bony abnormalities (scoliosis, atrophy or hypoplasia, osteoporosis). b) Fertility: chemotherapy, in particular alkylating agents and the methylhydrazine procarbazine, can interfere with gonadal function, especially when administered with abdomen and pelvic irradiation. This effect is often seen in Hodgkin disease. c) Cardiovascular function: the anthracyclines cardiotoxicity is well known and most commonly presents with cardiomyopathy, pericarditis or both. d) Pulmonary function: pulmonary fibrosis and recurring pneumonitis are the most common effects when more than a total dose 3000 cGy has been delivered to more than 50% of the lung. Chemotherapeutic agents (bleomycin, busulfan and many others) appear to be dose-related responsible for pulmonary disease in long-term survivors. e) Gastrointestinal function: fibrosis and enteritis are the most common pathologic abnormalities of the gastrointestinal tract, particularly after radiation therapy. The hepatotoxicity of anticancer therapy is well known: fibrosis-cirrhosis is seen after radiation therapy when a total dose between 1200 and 5800 cGy is administered, but abnormal liver function is also found after chemotherapy, being methotrexate implicated as cause of chronic hepatopathy. f) Urinary tract: hemorrhagic cystitis has been associated with cyclophosphamide and iphosfamide, but today this complication has been reduced by the use of prophylactic measures such as vigorous hydration and diuresis. Radiation in dose exceeding 2000 cGy is a well-defined cause of renal failure.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Late data in pediatric oncology]. 207 95

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

Pulmonary fibrosis is a severely disabling disease often leading to death. CCN2 (Cellular Communication Network factor 2, also known as CTGF) is a known mediator of fibrosis and clinical trials studying anti-CCN2 efficacy in pulmonary fibrosis are currently underway. Fork head box D1 (FoxD1) transcription factor is transiently expressed in several mesenchymal cell types, including those of fetal lungs. Differentiation of FoxD1-progenitor derived pericytes into myofibroblasts involves CCN2 expression and contributes importantly to maladaptive tissue remodeling in e.g. kidney and lung fibrosis models. To generate a model for studying the contribution of CCN2 expression in FoxD1-progenitor derived cells to development of fibrotic tissue remodeling, we set out to establish a FoxD1Cre - CCN2flox/flox mouse colony. However, all double-transgenic mice died soon after birth due to asphyxia. Histopathological examination revealed a reduction in alveolar space and lung weight, and subtle axial (thoracic and cervical) skeletal deformities. Together with the previously reported association of a FoxD1 containing locus with human adolescent idiopathic scoliosis, our data suggest that the development of fatal pulmonary hypoplasia caused by selective deletion of CCN2 from FoxD1-progenitor derived mesenchymal cells was secondary to aberrant axial skeletogenesis.
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PMID:FoxD1-driven CCN2 deletion causes axial skeletal deformities, pulmonary hypoplasia, and neonatal asphyctic death. 3229 73

Pulmonary fibrosis is a severely disabling disease often leading to death. CCN2 (Cellular Communication Network factor 2, also known as CTGF) is a known mediator of fibrosis and clinical trials studying anti-CCN2 efficacy in pulmonary fibrosis are currently underway. Fork head box D1 (FoxD1) transcription factor is transiently expressed in several mesenchymal cell types, including those of fetal lungs. Differentiation of FoxD1-progenitor derived pericytes into myofibroblasts involves CCN2 expression and contributes importantly to maladaptive tissue remodeling in for example kidney and lung fibrosis models. To generate a model for studying the contribution of CCN2 expression in FoxD1-progenitor derived cells to development of fibrotic tissue remodeling, we set out to establish a FoxD1Cre - CCN2flox/flox mouse colony. However, all double-transgenic mice died soon after birth due to asphyxia. Histopathological examination revealed a reduction in alveolar space and lung weight, and subtle axial (thoracic and cervical) skeletal deformities. Together with the previously reported association of a FoxD1 containing locus with human adolescent idiopathic scoliosis, our data suggest that the fatal pulmonary hypoplasia resulting from selective deletion of CCN2 from FoxD1-progenitor derived mesenchymal cells developed secondary to impaired breathing movements due to aberrant axial skeletogenesis.
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PMID:Correction to: FoxD1-driven CCN2 deletion causes axial skeletal deformities, pulmonary hypoplasia, and neonatal asphyctic death. 3202 Apr 19