Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0013911 (emaciation)
 1,059 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The longterm survival and occurrence of neoplastic and nonneoplastic lesions following total body irradiation (TBI), 8.5 Gy, with or without additional cyclophosphamide (Cy; 100 mg kg-1 i.p.) treatment as a conditioning regimen for bone marrow transplantation (BMT) were studied in male BN/BiRij rats. The two groups of rats that were treated with Cy (Cy and Cy + TBI) that survived beyond 100 days after treatment, had a severely decreased median (post treatment) survival time (Cy + TBI: 14.5 months and Cy: 14.1 months). Survival time in the TBI group was moderately decreased (18.5 months) as compared with the untreated controls (27.2 months). All treatment modalities were carcinogenic according to the raw data. After Cy-treatment a high incidence of, frequently multiple, malignant nerve-sheath tumours (Cy: 66 per cent, Cy + TBI: 31 per cent, controls: 2 per cent) was observed. TBI induced an increased occurrence of a great variety of tumours, especially mesenchymal tumours. This effect was more pronounced in animals receiving TBI alone as compared to animals receiving the combined treatment of Cy + TBI; an effect that most likely resulted from the longer median survival after TBI. The multi-target effect of TBI was also reflected in the occurrence of nonneoplastic effects in a variety of tissues, including high incidences of biliary cysts in the liver and severe testicular atrophy. The most important Cy-induced nonneoplastic lesion was incisor dysplasia, which resulted in feeding problems that could only be partly overcome by administering powdered food. Early mortality in the Cy-treated groups was associated with emaciation and generalized organ atrophy. A more definitive estimate of the late effects of supralethal chemoradiotherapy as part of a treatment of malignant disease has to await the results of various conditioning regimens for BMT in rats employing the acute BN myelocytic leukaemia (BNML) as a rat model for human acute myelocytic leukaemia (AML).
 ...
PMID:Late effects of cyclophosphamide and total body irradiation as a conditioning regimen for bone marrow transplantation in rats (a preliminary report). 329 5

The heterogeneous group of craniotubular dysplasias is characterized by modeling errors of the craniofacial and tubular bones. Some conditions in this category cause not only skeletal abnormalities but also a variety of mesoectodermal dysplasias, as exemplified in Lenz-Majewski syndrome (MIM 151050), which comprises craniodiaphyseal dysplasia, failure to thrive, mental retardation, proximal symphalangism, enamel hypoplasia, and loose skin. We report on a boy with a hitherto unknown multisystem disorder, including skeletal changes that were regarded as a form of craniotubular dysplasia. The patient had a large head, exophthalmos, a broad nasal root, anteverted nostrils, large auricles, thick lips, micrognathia, severe postnatal growth retardation with emaciation, severe mental retardation, sparse hair growth, enamel hypoplasia, and thin, loose skin with hyperlaxity. Skeletal changes consisted of thickened calvaria, sclerosis of the skull base and facial bones, thick ribs, and metaphyseal undermodeling of the tubular bones. In addition, generalized osteopenia was evident. The present disorder overlaps phenotypically with Lenz-Majewski syndrome; nevertheless, the absence of diaphyseal hyperostosis and proximal symphalangism in the present patient was not consistent with Lenz-Majewski syndrome.
 ...
PMID:Craniotubular dysplasia with severe postnatal growth retardation, mental retardation, ectodermal dysplasia, and loose skin: Lenz-Majewski-like syndrome. 921 75

In a colony of Steppe lemmings (Lagurus lagurus), of the rodent sub-family Arvicolinae, 8.6% of animals exhibited dysplastic growth of the molar teeth. Clinical findings included nodular swellings of the mandible, hypersalivation, malocclusion and emaciation. To investigate the underlying cause, two control and 10 affected animals, aged between 6 and 18 months, were examined using radiography and computed tomography and at post mortem examination. Bilaterally symmetrical masses were identified in the molar regions of the left and right mandible and maxillae. Histologically, the masses were characterised by dysplastic odontogenic epithelium, dentin, cementum, enamel and dental pulp formation that resembled odontogenic dysplasia. This tumour-like proliferative lesion has been reported in the continuously-growing incisor teeth of ageing rodents and lagomorphs but this is the first description of the clinico-pathological features of such odontogenic dysplasia of the molar teeth of Steppe lemmings.
 ...
PMID:Odontogenic dysplasia in the molar teeth of Steppe lemmings (Lagurus lagurus). 2057 34

Gammaretroviral vectors are an efficient means to effect gene therapy. However, genotoxicity from insertion at nonrandom sites can confer a competitive advantage to transduced cells, resulting in clonal proliferation or neoplasia. Six pig-tailed macaques (Macaca nemestrina) underwent total body irradiation and reconstitution with autologous stem cells genetically modified by a gammaretroviral vector overexpressing HOXB4. Two animals were euthanized owing to irradiation- or transplantation-associated toxicity, whereas the other 4 had successful reconstitution. Of the 4 macaques with successful reconstitution, 1 has no long-term follow-up information; 1 was euthanized owing to infection with simian varicella virus infection 18 months post-total body irradiation; and the 2 others are described herein as case Nos. 1 and 2. After being stable for 3 years, case No. 1 developed pancytopenia and petechiation, and after 2 years of stability case No. 2 developed anemia and thrombocytopenia. Despite therapy, the animals deteriorated and were euthanized. Gross findings included emaciation; case No. 1 also had hemorrhage, peritonitis, and cholecystitis. Histologically, bone marrow was hypercellular with predominately blast cells of all hematopoietic lineages, though with myeloid predominance, and with maturation arrest and blast cell dysplasia (myelodysplasia). Myelodysplasia was likely from a combination of insertional mutagenesis by the retroviral vector and overexpression of HOXB4. Consequences of myelodysplasia included the blood dyscrasias and, in case No. 1, hemorrhage, bacterial cholecystitis, hepatitis, and peritonitis.
 ...
PMID:Myelodysplasia in 2 pig-tailed macaques (Macaca nemestrina) associated with retroviral vector-mediated insertional mutagenesis and overexpression of HOXB4. 2087 Sep 55

Osteofibrous dysplasia is a rare and benign nonneoplastic condition of unknown etiology in humans and mammals. An adult female cockatiel (Nymphicus hollandicus) was presented with the following problems: a soft tissue mass on the left frontal area of the head that extended over the left eye, anorexia, lethargy, and emaciation. Computed tomographic imaging and ultrasonography revealed a soft tissue opacity in the left cranial area of the head with multifocal heterogeneous opacities and foci of mineralization. Cytological, histopathological, and immunohistochemistry examinations were performed on biopsy samples from tissue collected from the mass. Following surgical removal of the cranial mass the patient was treated with a nonsteroidal anti-inflammatory drug and antibiotic medication. The surgical site healed with no complications and through follow-up examinations there were no signs of reoccurrence of the abnormal tissue for 2 months. Through cytological and histopathological examination and immunohistochemistry testing of the submitted tissue, a diagnosis of osteofibrous dysplasia was determined.
 ...
PMID:Osteofibrous Dysplasia in a Cockatiel (Nymphicus hollandicus). 3223 87