Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case is described of testicular feminisation syndrome in a 19-yr-old patient with a normal female appearance. A laparotomy with removal of the gonads was performed. The latter looked like testes with intraparenchymal nodules. Light and electron microscopy study of these nodules revealed the presence of immature seminiferous-like tubules and of Leydig cell clusters. The tubules were filled with clear Sertoli cells, few degenerative dark cells and scanty spermatogonia. Thin collagen bundles surrounded the tubules and the foci of Leydig cells without any crystals of Reinke. The immaturity of all these cellular components could be connected with a mutation at a repressive locus which could be responsible for the abnormalities observed in the different steps of androgen action at target cell level.
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PMID:A complete form of testicular feminisation syndrome; a light and electron microscopy study. 74 23

Semicarbazide, a lathyrogen, was given to growing rats to elucidate the consequences of altering the molecular structure of fibrous proteins within the lung. Static pressure-volume (P-V) measurements during deflation of saline-filled lungs showed normal recoil pressure and compliance values within the physiological range of lung volume. Quasi-static P-V measurements were also normal during slow reinflation, even beyond physiological limits to a recoil pressure of 20 cm H20. However, the lungs of experimental rats ruptured at much lower recoil pressures than controls. Histology was normal in lungs fixed at 20 cm H20. In contrast, lungs showed dilation of terminal air spaces, rupture of alveolar walls, and an increase in mean linear intercept in experimental compared with control specimens, when fixed at 30 cm H20. Biochemical analyses revealed reduced cross-linking of lung collagen without change in its total content. There were no detectable changes in the quantity or quality of lung elastin. It is concluded that semicarbazide may selectively impair the maturation of lung collagen and that immaturity of lung collagen is associated with a reduction in the tensile strength of lung tissue, without changes in elasticity within physiological volume limits.
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PMID:Effects of a molecular change in collagen on lung structure and mechanical function. 113 68

The immunohistological distribution of collagen types I, II, III, V and VI in human benign and malignant cartilaginous tumours of bone was examined with regard to their aggressiveness. The matrix of enchondromas consisted of type II collagen distributed diffusely, and type VI predominantly localized in the immediate surroundings of the cells. Types I, III and V collagen were not found. These findings were similar to the distribution of collagenous proteins in normal hyaline cartilage where each lobule was consistently rimmed by types I and V collagen. In grade 1 chondrosarcomas, the main collagenous components of matrix were also types II and VI collagen. Type II was sometimes found in the cytoplasm of tumour cells and type VI tended to lose territorial localization. In addition, type I collagen was demonstrated consistently and type V in some cases. In grade 2 chondrosarcomas, type II collagen was demonstrated not only in the matrix but occasionally in the cytoplasm of tumour cells. Type VI was dispersed in the intercellular areas. The other collagenous proteins such as types I, III and V were also present in the matrix. In grade 3 chondrosarcomas, type II collagen was localized predominantly in the cytoplasm of tumour cells and in the adjacent matrix. Type VI was markedly decreased with complete loss of pericellular distribution, whereas types I, III and V were constantly present in the matrix. Those alterations in the distribution of collagen types correlated well with the aggressive behaviour of the tumours. The findings suggest that distribution of different collagen types in cartilaginous tumours reflects the immaturity of the tumour cells and is a useful indicator of their aggressiveness.
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PMID:Immunohistological study on collagenous proteins of benign and malignant human cartilaginous tumours of bone. 212 78

We present the clinicopathologic characteristics of 110 colorectal mixed hyperplastic adenomatous polyps (MHAP) that exhibited the architectural but not the cytologic features of a hyperplastic polyp. They are compared with 60 traditional adenomas, 40 hyperplastic polyps, and five colonic polyps that contained admixed but well-defined hyperplastic and adenomatous glands (HP/AD). The patients with MHAP ranged in age from 15 to 88 years (mean, 63 years). Five patients had two or more (up to seven) lesions. MHAP measured 0.2-7.5 cm in diameter. They were distributed throughout the colorectum, but a slight preponderance of large lesions (more than 1.0 cm) occurred in the cecum and appendix. All MHAP were characterized by a serrated glandular pattern simulating that seen in hyperplasia (27% of MHAP were initially diagnosed as hyperplastic polyps). However, MHAP were distinguished by the presence of goblet cell immaturity, upper zone mitoses, prominence of nucleoli, and the absence of a thickened collagen table. Although surface mitotic activity, nuclear pseudostratification, and nuclear cytoplasmic ratio were greater in MHAP than in hyperplastic polyps, they were slightly less than in traditional adenomas. Thirty-seven percent of MHAP contained foci of significant dysplasia; 11% contained areas of intramucosal carcinoma. We conclude that these lesions reflect a morphologically unique variant of adenoma and suggest that they be termed "serrated adenoma" in order to emphasize their neoplastic nature. We further offer the hypothesis that MHAP may arise from the neoplastic transformation of a more differentiated cell in the crypt than the traditional adenoma.
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PMID:Mixed hyperplastic adenomatous polyps/serrated adenomas. A distinct form of colorectal neoplasia. 1047 80

The acid solubility of the collagen of cockerel tendon demonstrates a similar degree of intermolecular acid-labile cross-linkage to that found in adult human tendon, implying similar maturity of the collagen. By contrast, rabbit tendon collagen demonstrates a dissimilar degree of cross-linkage suggesting immaturity of the collagen. Since this cross-linkage is likely to be related to mechanical properties, the cockerel may well be a better animal model than the rabbit for the study of tendon healing. The application of ultrasound to sutured cockerel tendons produced no change in the mechanical strength of the tendon at six weeks, or its propensity to form adhesions, relative to untreated control sutured tendons. This contrasts with the decrease in strength after ultrasound found by previous workers using rabbits.
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PMID:The effect of ultrasound on the healing of repaired cockerel tendon: is collagen cross-linkage a factor? 262 4

To explain the reported predisposition to germinal matrix hemorrhage in premature infants, pathogenetically important morphological features of the germinal matrix should be present in the 3rd trimester and rapidly change near term. Such features were sought in this study of the germinal matrix and its vasculature in normal rhesus monkey fetuses. The matrix cells, glia, ependyma, and capillaries showed no important structural changes during the 3rd trimester. The terminal vein tributaries were greatly enlarged by 148 days, but cellular and collagen support in their walls was minimal at this time. The latter features developed by the final days of gestation. These findings do not support a structural immaturity or specialization of the germinal matrix predisposing to germinal matrix hemorrhage. Our results, therefore, support the recent emphasis on physiological parameters in the pathogenesis and prevention of germinal matrix hemorrhage.
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PMID:Gestational changes in the germinal matrix of the normal rhesus monkey fetus. 396 5

Severe nerve injuries may require microsurgical grafting to span a defect. Introduction of graft material into a highly vascular recipient bed is documented to aid in early regeneration of neuronal blood supply. A silicone rod (SR)-induced fibrovascular sheath was employed to evaluate the regeneration of rat tibial nerve through 2-mm-diameter collagen tubes (CT) or contralateral nerve autografts (AUTO). At first operation, 5 mm of right tibial nerve was resected from 30 retired male breeder Sprague-Dawley rats. Resected nerve was replaced with either a 5 X 2 mm SR or the nerve ends were sutured to the intermuscular fascia. Four weeks later, animals were repaired by replacing the SR with either a CT or a contralateral AUTO from the left tibial nerve. Three months later, EMG testing was performed, and histologic sections were prepared. The EMG latency and the size of the compound action potential for sheathed or non-sheathed CT or AUTO were statistically superior to controls at the 95% confidence level. All other intergroup comparisons of latency and action potential size were statistically insignificant. The proportion of nerve fibers traversing the surgical sites was not influenced by the method of repair or by the presence or absence of sheathing. Tubulized repairs most closely resembled unoperated nerves, and autografted repairs had a large diameter, but much fibrosis, whereas controls displayed immaturity and disorganization. Our observations suggest that there was no difference between repairs performed with or without a vascular pseudosheath. However, CT supported regeneration better than did AUTO repair.
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PMID:Nerve regeneration through collagen tubes. 658 89

Muscle biopsies of 5 patients with congenital muscular dystrophy (CMD) (8 months to 3 years old) were examined by electron microscopy to determine ultrastructural abnormalities of the muscle as well as of the connective tissue cells. Two populations of muscle fibers were observed in each biopsy. Besides muscle fibers with normal or enlarged diameters there were frequently very small muscle cells indicating immaturity. The most interesting findings in each biopsy were myofibroblast-like cells exhibiting active protein synthesis. Large amounts of collagen fibrils with abnormal diameters as well as accumulation of elastic fibrils within the endomysium in CMD suggest abnormalities in collagen synthesis.
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PMID:Congenital muscular dystrophy (CMD) - a collagen formative disease? 720 42

Two main types of extracellular matrix distribution was distinguished on the basis of immunohistochemistry of the periodontium connective tissue in various inflammatory-sclerotic processes. The 1st type was characterized by a significant reduction of interstitial collagen, moderate content of type V collagen and high content of fibronectin (in case of the exacerbation). The 2nd type was characterized by a high content of type V collagen, increased content of interstitial collagen and moderate amount of fibronectin (in case of reparative sclerosis). An inversion of the relation between the I and III collagen types in favour of the latter, high level of type V collagen and permanent presence of fibronectin were characteristic for the scar tissue. These properties of scar connective tissue indicate its immaturity and permanent activity.
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PMID:[Structure of connective tissue in inflammatory-sclerotic processes in the periodontium]. 767 82

Kaspareit-Rittinghausen described a rodent model of inherited polycystic kidney disease (PKD), the Han:SPRD rat [1, 2], in which heterozygotes develop renal cysts and renal failure (in males) over several months, whereas homozygous animals develop rapidly progressive renal enlargement that leads to death in a few weeks. In this study, we examined selected elements of the pathogenesis of this disease in heterozygotes and homozygotes from birth to advanced disease. Heterozygous male rats developed slowly progressive renal cystic disease with interstitial fibrosis and azotemia seen by six months of age. Female heterozygotes developed slowly progressive renal cystic disease, but did not develop interstitial fibrosis or azotemia. Epithelial cells lining cyst cavities showed various degrees of morphologic immaturity. Cyst walls also developed basement membrane thickening, especially in areas of cellular immaturity, suggesting an interrelationship between this basement membrane thickening and cellular dedifferentiation. Thickened basement membranes were associated with increased immunoreactivity for type IV collagen, laminin, and fibronectin. Homozygous rats developed massive renal enlargement, marked azotemia, and died near three weeks of age. Renal c-myc proto-oncogene expression was elevated in homozygous cystic infants and in adult heterozygotes. In situ hybridization showed high levels of c-myc mRNA in cyst epithelia, suggesting abnormal regulation of cellular proliferation in the cells lining cysts, as seen in other models of PKD. The Han:SPRD rat is the only well-documented animal model of inherited PKD with an autosomal-dominant inheritance pattern and appears to have several features which resemble human ADPKD.
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PMID:Autosomal-dominant polycystic kidney disease in the rat. 845 52


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