PubMed Health⌕ Search

Biomedical subjects

C Engels

Publications and source records attributed to C Engels.

11 recordsLinked to original sources
PubMed2001

Gsalpha gene mutations in monostotic fibrous dysplasia of bone and fibrous dysplasia-like low-grade central osteosarcoma.

A series of nine cases with monostotic fibrous dysplasia (FD) and five cases of fibrous dysplasia-like low-grade central osteosarcoma (fd-like lgcOSA) were applied in a mutational analysis. Restriction digestion analysis, single-stranded conformational polymorphism (SSCP) analysis, and repeated sequencing demonstrated a R201H mutation in six cases and a R201C mutation in three cases of patients with monostotic FD. These results demonstrate that the presence of Gsalpha gene mutations is a constant finding in monostotic FD. To our knowledge, this is the first report in the literature investigating Gsalpha gene mutations in lgcOSA, which is one of the most important differential diagnoses of FD because of its low-grade malignant behavior. In four of five cases of fd-like lgcOSA, no mutation has been detected. In one case of this tumor, a R201C mutation could be demonstrated. Because our results demonstrate a low prevalence of Gsalpha gene mutations in this tumor in contrast to monostotic FD, mutational analysis may be an additional helpful parameter in individual cases for the differential diagnosis of FD and fd-like lgcOSA.

Adolescent↗
PubMed2001

The nature of giant cell tumor of bone.

Giant cell tumor of bone (GCT) is a locally osteolytic tumor with variable aggressiveness. In rare cases, pulmonary metastasis can be observed. The lesion most frequently occurs in the epiphysis of long tubular bones of the knee region, predominantly affecting young adults after closure of the growth plate. The characteristic histological appearance of GCT displays a high number of osteoclast-like multinucleated giant cells, which resulted in the classification "osteoclastoma" or "giant cell tumor". Apart from the multinucleated giant cells, there are two mononuclear cell types in GCT. The first one has a round morphology and resembles monocytes. The second cell type is the spindle-shaped, fibroblast-like stromal cell. Cell culture experiments with GCT cells revealed the stromal cell to be the proliferating component of the GCT. The other two cell types, the monocyte and the multinucleated giant cell, were lost after a few cell culture passages. Furthermore, latest results from GCT reveal that the stromal cells secrete a variety of cytokines and differentiation factors, including MCP1, ODF, and M-CSF. These molecules are monocyte chemoattractants and are essential for osteoclast differentiation, suggesting that the stromal cell stimulates blood monocyte immigration into tumor tissue and enhances their fusion into osteoclast-like, multinucleated giant cells. The multinucleated giant cell itself resembles a normal osteoclast that is able to resorb bone leading to extended osteolysis. This new model of GCT genesis supports the hypothesis that the stromal cell is the neoplastic component whilst the monocytes and the multinucleated giant cells are just reactive components of this tumor. Taking this into consideration, the nomenclature of the "giant cell tumor" needs to be reconsidered.

Bone Neoplasms↗
PubMed2001

Tobacco plants that lack expression of functional nitrate reductase in roots show changes in growth rates and metabolite accumulation.

When tobacco is provided with a high nitrate supply, only a small amount of the nitrate taken up by the roots is immediately assimilated inside the roots, while the majority is transported to the leaves where it is reduced to ammonium. To elucidate the importance of root nitrate assimilation, tobacco plants have been engineered that showed no detectable nitrate reductase activity in the roots. These plants expressed the nitrate reductase structural gene nia2 under control of the leaf-specific potato promoter ST-LS1 in the nitrate reductase-mutant Nia30 of Nicotiana tabacum. Homozygous T2-transformants grown in sand or hydroponics with 5.1 mM nitrate had approximately 55-70% of wild-type nitrate reductase acivity in leaves, but lacked nitrate reductase acivity in roots. These plants showed a retarded growth as compared with wild-type plants. The activation state of nitrate reductase was unchanged; however, diurnal variation of nitrate reductase acivity was not as pronounced as in wild-type plants. The transformants had higher levels of nitrate in the leaves and reduced amounts of glutamine both in leaves and roots, while roots showed higher levels of hexoses (3-fold) and sucrose (10-fold). It may be concluded that the loss of nitrate reductase acivity in the roots changes the allocation of reduced nitrogen compounds and sugars in the plant. These plants will be a useful tool for laboratories studying nitrate assimilation and its interactions with carbon metabolism.

Biological Transport↗
PubMed2000

[Chondroblastoma of the patella with pathological fracture].

Chondroblastoma is a rare benign bone tumor of cartilaginous origin. The typical localization of a chondroblastoma is the epiphysis of long tubular bones--the patella is a very unusual site with an estimated occurrence of 2%. We report a case of a 16-year-old patient with a chondroblastoma of the patella associated with a pathologic fracture. Partial resection of the patella was performed. This is the sixth case in the literature that associates patellar chondroblastoma with fracture.

Adolescent↗
PubMed2000

[Clear-cell chondrosarcoma].

Clear-cell chondrosarcoma is a rare, low-malignant bone tumor. The lesion most commonly occurs in adults, generally in the 3rd or 4th decade. Clear-cell chondrosarcoma has a predilection for the epiphyses of long tubular bones, particularly the femoral head. Radiologically, it is a sharply defined radiolucent lesion. Histologically, it is characterized by large tumor cells with distinct boundaries and a clear cytoplasm. In addition to areas of conventional chondrosarcoma, there are partially mineralized trabecular osteoid formations. On the basis of 16 cases of clear-cell chondrosarcoma, we present the observations of the Hamburg Bone Tumor Register. The cases were examined according to age distribution, location, and radiological and pathomorphological criteria. The proximal femur was the most frequent location; rare manifestations in the rib, os ilium, and distal phalanx of the hand were also present. In 50% of the cases high-malignant osteosarcoma or conventional chondrosarcoma was considered. In addition to the epiphysis of the proximal femur, clear-cell chondrosarcoma can also involve other, rather unusual sites in the skeleton. Knowledge of the histomorphology of this tumor is therefore of crucial importance for the diagnosis.

Adult↗
PubMed2000

Differential regulation of three functional ammonium transporter genes by nitrogen in root hairs and by light in leaves of tomato.

To elucidate the role of NH4+ transporters in N nutrition of tomato, two new NH4+ transporter genes were isolated from cDNA libraries of root hairs or leaves of tomato. While LeAMT1;2 is closely related to LeAMT1;1 (75.6% amino acid identity), LeAMT1;3 is more distantly related (62.8% identity) and possesses two short upstream open reading frames in the 5' end of the mRNA and a particularly short N-terminus of the protein as unique features. When expressed in yeast mutants defective in NH4+ uptake, all three genes complemented NH4+ uptake. In roots of hydroponically grown plants, transcript levels of LeAMT1;2 increased after NH4+ or NO3- supply, while LeAMT1;1 was induced by N deficiency coinciding with low glutamine concentrations, and LeAMT1;3 was not detected. In aeroponic culture, expression of LeAMT1;1 and LeAMT1;2 was higher in root hairs than in the remaining root fraction. Growth of plants at elevated CO2 slightly decreased expression of LeAMT1;2 and LeAMT1;3 in leaves, but strongly repressed transcript levels of chloroplast glutamine synthetase and photorespiratory serine hydroxymethyl-transferase. Expression of LeAMT1;2 and LeAMT1;3 showed a reciprocal diurnal regulation with highest transcript levels of LeAMT1;3 in darkness and highest levels of LeAMT1;2 after onset of light. These results indicate that in tomato at least two high-affinity NH4+ transporters, LeAMT1;1 and LeAMT1;2, are differentially regulated by N and contribute to root hair-mediated NH4+ acquisition from the rhizosphere. In leaves, the reciprocally expressed transporters LeAMT1;2 and LeAMT1;3 are supposed to play different roles in N metabolism, NH4+ uptake and/or NH3 retrieval during photorespiration.

Amino Acid Sequence↗
PubMed2000

Rapid effects of nitrogen form on leaf morphogenesis in tobacco.

Ammonium (NH4+) instead of nitrate (NO3-) as the nitrogen (N) source for tobacco (Nicotiana tabacum L.) cultivated in a pH-buffered nutrient solution resulted in decreased shoot and root biomass. Reduction of shoot fresh weight was mainly related to inhibition of leaf growth, which was already detectable after short-term NH4+ treatments of 24 h, and even at a moderate concentration level of 2 mM. Microscopic analysis of the epidermis of fully expanded leaves revealed a decrease in cell number (50%) and in cell size (30%) indicating that both cell division and cell elongation were affected by NH4+ application. Changes in various physiological parameters known to be associated with NH4(+)-induced growth depression were examined both in long-term and short-term experiments: the concentrations of total N, soluble sugars and starch as well as the osmotic potential, the apparent hydraulic conductivity and the rate of water uptake were not reduced by NH4+ treatments (duration 1-12 d), suggesting that leaf growth was neither limited by the availability of N and carbohydrates, nor by a lack of osmotica or water supply. Although the concentration of K+ in leaf press sap declined in expanding leaves by approximately 15% in response to NH4+ nutrition, limitation of mineral nutrients seems to be unlikely in view of the fast response of leaf growth at 24 h after the start of the NH4+ treatment. No inhibitory effects were observed when NH4+ and NO3- were applied simultaneously (each 1 mM) resulting in a NO3-/NH4+ net uptake ratio of 6:4. These findings suggest that the rapid inhibition of leaf growth was not primarily related to NH4+ toxicity, but to the lack of NO3(-)-supply. Growth inhibition of plants fed solely with NH4+ was associated with a 60% reduction of the zeatine + zeatine riboside (Z + ZR) cytokinin fraction in the xylem sap after 24 h. Furthermore Z + ZR levels declined to almost zero within the next 4 d after start of the NH4+ treatment. In contrast, the concentrations of the putative Z + ZR precursors isopentenyl-adenine and isopentenyl-adenosine (i-Ade + i-Ado) were not affected by NH4+ application. Since cytokinins are involved in the regulation of both cell division and cell elongation, it seems likely that the presence of NO3- is required to maintain biosynthesis and/or root to shoot transfer of cytokinins at a level that is sufficient to mediate normal leaf morphogenesis.

Carbohydrate Metabolism↗
PubMed1999

[Chondromyxoid fibroma. Morphological variations, site, incidence, radiologic criteria and differential diagnosis].

Chondromyxoid fibroma is a rare benign bone tumor, accounting for less than 1% of all bone tumors. The peak age incidence is the second and third decade of life. Chondromyxoid fibrom occurs in the metaphyseal parts of the major tubular bones, predominantly of the lower extremity. Roentgenograms show, in most cases, a well-demarcated radiolucent lesion. The classic histological feature of a chondromyxoid fibroma is stellate or spindle-shaped cells arranged in lobules in a myxoid or chondroid background. Analysis of 40 chondromyxoid fibromas demonstrates the morphological variation of this tumor. Cases were examined for age distribution, localization, and radiological and histological features. In 85% we found the typical histomorphological pattern. Recurrence rate was 12.5%. In four cases the appearance was uncharacteristic and differentiation from other tumors such as chondroblastoma or chondrosarcoma was quite difficult. By adhering to strict histomorphological criteria, definite diagnosis of chondromyxoid fibroma can be made in most cases.

Adolescent↗
PubMed1993

[Change in the procedure from external fixator to intramedullary nailing osteosynthesis of the femur and tibia].

Shaft fractures of femur and tibia can be treated successfully by intramedullary nailing. In recent years the use of interlocking nails widened the indication for nailing fractures of the proximal and distal bone and more difficult fractures. There are still limits in polytrauma patients, chain fractures with or without participation of joints and in fractures with severe soft tissue injury (open or closed). In these cases primary treatment with external fixation has proved worthwhile. The main problems and risks of primary nailing osteosynthesis occur in the early phase of treatment, whereas complications in external fixation are more likely to occur in later phases of treatment. In changing from external fixation to intramedullary nailing one can see the advantages of both methods. In the literature only small studies have been made mostly with patient groups below 50 in number. The change of method was rarely standardised and the time period between procedures was either late (more than 3 weeks) or arbitrary. The advantages and disadvantages as well as the risk in changing methods are controversial. In a 2-year prospective study from August 1989 to July 1991, patients with II and III degree open and closed femur and tibia fractures as well as trauma patients with fractures were initially treated by external fixation. A change of method from external fixation to intramedullary nailing was performed at the earliest possible time under exact criteria. 61 femur and 106 tibia fractures were accordingly treated and followed. In comparison to early studies there were no differences in bone healing or in functional results. The infection rate in tibia fractures was 1.9% (2 of 106); no infection was seen in femur fractures. The contamination rate at the time of method change was substantially higher at 14%. The difference between contamination and infection rate can be explained by experience in the technique of nailing, considering biological aspects (no or little reaming), the standardized change of method and the prophylactic use of antibiotics. In a follow-up of additional 37 femur and 58 tibia fractures that were treated accordingly, a total infection rate of 1.9% was achieved (2% in femur fractures [n = 98], 1.8% in tibia fractures [n = 164], 1.9% total [n = 262]). In femur and tibia fractures with open or closed soft tissue damage and in multiple trauma patients the treatment with initial stabilisation by external fixation and secondary change to intramedullary nailing can be recommended under certain conditions.

Adolescent↗