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Function of children with myelodysplasia and lower extremity amputations.

This is a retrospective study of the functional status of children who underwent a lower extremity amputation for complications of myelodysplasia. With a computerized surgical database, 12 children with myelodysplasia who underwent an amputation at the Boyd level or above at a single children's referral hospital between 1983 and 2001 were identified. Four patients could not be contacted, but the remaining 8 patients were evaluated through chart review and interview to assess the impact of the amputation on their function. With a mean follow-up time of 9 years (range, 5-15 years), all 6 of the patients with a below-knee or Boyd amputation continued to ambulate using a prosthesis. Most patients occasionally reported having ulcers on their residual limb, but these cases were easily managed and did not result in amputation revisions.The only patient in this series with an above-knee amputation and the only patient with a knee disarticulation were exclusively wheelchair ambulators and no longer owned a prosthesis. This study supports the notion that children with myelodysplasia can have amputations and successfully wear a prosthesis to maintain their ambulation.

Adolescent↗

Microcycle conidiation and its genetic basis in Neurospora crassa.

Some wild isolates of Neurospora show microcycle conidiation in liquid culture under continuous agitation. Macroconidia from agar-grown mycelial cultures germinated in liquid and the germlings spontaneously produced conidia with no intervening mycelial phase. Three types of microcycle conidiation were seen among progeny of N. crassa Vickramam A x N. crassa a wild-type: (1) multinucleate blastoconidia produced by apical budding and septation, (2) multinucleate arthroconidia produced by holothallic septation and disarticulation of cells, and (3) uninucleate microconidia produced directly from conidiogenous cells of the germlings. Two genes were identified which control specific patterns of microcycle conidiogenesis. A single gene mcb in linkage group VR near al-3 (3.2% recombination) controls blastoconidiation. This gene is epistatic to gene mcm located in linkage group IIL, very near ro-7 (1.4%). mcm controls both microconidiation and arthroconidiation depending on temperature. Strains of genotype mcm produce microconidia almost exclusively at 18-22 degrees C, but arthroconidia with few or no microconidia at 30 degrees C. Because they result in rapid and synchronized conidiation in liquid culture, the two genes should be useful for studies of developmental gene regulation. mcm makes it possible to obtain large quantities of pure microconidia rapidly for experimentation.

Crossing Over, Genetic↗

A morphological and genetic analysis of conidiophore development in Neurospora crassa.

The filamentous fungus Neurospora crassa responds to nutrient deprivation and dessication by producing asexual spores, or conidia. These conidia are derived from differentiated aerial structures called conidiophores. The process of conidiation was analyzed in wild-type and morphological mutants using scanning electron microscopy (SEM) and specific fluorescent probes. The first discernible morphological step of conidiation is the transition from growth by hyphal tip elongation to growth by repeated apical budding, resulting in the formation of chains of proconidia that resemble beads on a string. The initial proconidial chains are morphologically distinct from those that form later and are capable of reverting to hyphal growth, whereas the later chains are committed to conidiation. As the proconidial chains are formed, nuclei migrate into the conidiophore, and cross-walls arise between adjoining proconidia in a series of steps that have been defined by staining with Calcofluor, a fluorescent chitin-binding probe. The chains ultimately disarticulate in several discrete stages into free, morphologically mature conidia. Different conidiation-defective mutants were shown to be blocked at distinct stages in conidiation. Our observations permit us to derive a developmental timeline of conidiation relating the occurrence of morphological changes and the stage blocked in specific mutants.

Gene Expression Regulation↗

Central fibrosarcoma of bone. Report of a case.

A case of a 46-year-old Japanese male having fibrosarcoma of bone is reported. The tumor developed in the proximal metaphysis of the left femur. During the three years following onset of the disease with symptoms of local pain and mass, the patient was operated on three times (curettage and bone graft, curettage and bone graft with Jwett's nail fixation and disarticulation). The tumor was found to be an intraosseous translucent lesion on x-ray examiation. Histologically, the tumor consisted of compact or loose, atypical spindle cells, producing abundant collagen-fibers without any osteoid, bony or cartilage formation. From the clinical and pathological findings, this case is thought to be a typical central fibrosarcoma of bone.

Femoral Neoplasms↗

Primary sarcomas of the jaw.

Nine cases of primary sarcoma of the jaw seen over a period of 14 years are presented. They consisted of four osteosarcomas, three fibrosarcomas, one myxosarcoma and one Ewing's sarcoma. The treatment of choice for osteosarcomas of the mandible is hemimandibulectomy with disarticulation at the temporomandibular joint. Bone graft reconstruction from the iliac crest should be done at a second stage. Palliative maxillectomy for extensive fibrosarcoma of the maxilla served to relieve one patient of local symptoms for more than a year. Myxosarcoma, a variant of fibrosarcoma, and Ewing's sarcoma of the jaw, are very rare conditions, and since these lesions are usually very extensive radiotherapy is the treatment of choice.

Adolescent↗

Treatment of advanced and inaccessible sarcomas with continuous intra-arterial chemotherapy prior to definitive surgery or radiotherapy--a possible alternative to amputation or disabling radical surgery.

Four patients with advanced and inaccessible soft tissue sarcomas were treated with a regimen of intra-arterial chemotherapy followed by radiotherapy and/or surgical excision. Two of the patients had advanced sarcomas in the buttock and thigh regions which would otherwise have required hindquarter amputation in one case or disarticulation of the hip in the other case. These sarcomas responded significantly to intra-arterial chemotherapy to the extent that subsequent local surgery was effective in eradicating the residual tumours. No viable tumour cells were found in the resected specimens. In both patients amputation was avoided and local tumour eradication was achieved. In the other two patients, advanced and non-resectable sarcomas in the head were first treated with a similar regimen of intra-arterial chemotherapy. In both cases the tumours regressed in size prior to administration of local radiotherapy. After completion of chemotherapy and radiotherapy no viable tumour cells were detected in either lesion. In one case (originally a very extensive sarcoma of the jaw in a 5 year old child) a residual lump was resected but no viable tumour was detected in the resected specimen. These four patients represent our total experience with this plan of management. All responded well and there has been no evidence of local disease recurrence in any of the four patients. One patient (Case 2) did develop pulmonary and bone metastases from which she died 2 years later but the other three patients remain well with no evidence of residual disease, 11 years, 4 years and 20 months after presentation.

Adult↗

Rotationplasty of the lower limb for childhood osteosarcoma of the femur.

BACKGROUND: Surgical management of osteosarcoma of the limb in childhood often involves extensive surgery. Intervention ranges from local resection to amputation depending on the level and the extent of the disease process. However, where the disease is extensive the level of amputation may preclude effective prosthetic use. In these circumstances resection of the diseased segment of the limb, i.e. an intercalary amputation and reconstruction with rotation of the tibial component of the limb can provide function equivalent to a below-knee amputee. METHODS: To examine the role of rotationplasty of the lower limb for childhood osteosarcoma of the femur, a retrospective study was carried out which examined the function of five patients who had been treated by a tibial rotationplasty. The records of two patients who had died from metastatic disease 2 and 4 years after surgery were examined. The three surviving patients were examined clinically and their clinical records and radiographs were reviewed. RESULTS: Of the two patients who died from metastatic disease, neither had local recurrence of the tumour, neurovascular complications, late derotations or psychological problems. In the three patients who survived, there had been no local recurrence of tumour or other complication and each has an excellent level of activity with below-knee function using a rotationplasty prosthesis. CONCLUSIONS: For children with osteosarcoma of the femur and with extensive disease that precludes complete limb preservation, resection and tibial rotationplasty provides function far superior to a high above-knee amputation or a hip disarticulation without the risk of local recurrence.

Artificial Limbs↗

Uptake and release of D-aspartate in the guinea pig cochlear nucleus.

This study attempted to determine if L-glutamate (L-Glu) and/or L-aspartate (L-Asp) might be the transmitters of neurons that provide synaptic endings to the cochlear nucleus of the medulla. The uptake and release of D-[3H]aspartate (D-Asp), a putative marker for L-Glu and L-Asp, were measured in the guinea pig cochlear nucleus before and after destruction of the cochlear afferents by cochlear ablation. The cochlear nucleus was dissected into the anteroventral (AVCN), posteroventral (PVCN), and dorsal (DCN) cochlear nuclei. Subdivisions from unlesioned animals took up D-Asp, achieving concentrations in the tissues that were 13-20 times that in the medium. Subsequently, electrical stimulation evoked a Ca2+-dependent release of part of the D-Asp from each subdivision. Disarticulation of the middle ear ossicles, which attenuates acoustic stimulation, produced a modest inhibition of D-Asp release in each subdivision, but did not alter the uptake of D-Asp. Cochlear ablation strongly depressed both the uptake and the release of D-Asp in each subdivision, presumably as a result of destruction of the cochlear nerve endings in the cochlear nucleus. Nevertheless, after lesions, there was a preservation of the uptake and release of D-Asp in the DCN relative to the AVCN and PVCN. These residual activities in the DCN may be mediated by the axonal endings of the granule cells of the cochlear nucleus. The present findings support the hypothesis that the granule cells of the cochlear nucleus, as well as the cochlear nerve fibers, use L-Glu and/or L-Asp as transmitters.

Animals↗

An empirical bound on the compressibility of the cochlea.

Effects of a possible inner-ear compressibility on middle-ear transfer functions are explored and a small upper bound on the magnitude of that compressibility established. Consequently. the traditional two-port representation of middle-ear mechanics remains valid to within a few percent. If the compressibility of the cochlea is small but finite, a simple phenomenological model of that compressibility correctly predicts hearing thresholds in the "middleless" ear at low frequencies. Experiments to establish the value of cochlear compressibility and to explore further its possible contributions to residual hearing in patients with missing or disarticulated middle-ear ossicles are suggested.

Animals↗

Natural dissemination of Bacillus anthracis spores in northern Canada.

Soil samples were collected from around fresh and year-old bison carcasses and areas not associated with known carcasses in Wood Buffalo National Park during an active anthrax outbreak in the summer of 2001. Sample selection with a grid provided the most complete coverage of a site. Soil samples were screened for viable Bacillus anthracis spores via selective culture, phenotypic analysis, and PCR. Bacillus anthracis spores were isolated from 28.4% of the samples. The highest concentrations of B. anthracis spores were found directly adjacent to fresh carcasses and invariably corresponded to locations where the soil had been saturated with body fluids escaping the carcass through either natural body orifices or holes torn by scavengers. The majority of positive samples were found within 2 m of both year-old and fresh carcasses and probably originated from scavengers churning up and spreading the body fluid-saturated soil as they fed. Trails of lesser contamination radiating from the carcasses probably resulted from spore dissemination through adhesion to scavengers and through larger scavengers dragging away disarticulated limbs. Comparison of samples from minimally scavenged and fully necropsied carcass sites revealed no statistically significant difference in the level of B. anthracis spore contamination. Therefore, the immediate area around a suspected anthrax carcass should be considered substantially contaminated regardless of the condition of the carcass.

Animals↗

Morphology of submerged growth of streptomycetes as a taxonomic aid. I. Morphological development of Streptomyces aureofaciens in agitated liquid media.

In a study of the submerged growth morphology of 145 different streptomycetes in A C Broth, the species Streptomyces aureofaciens was observed to represent an unusual type. It was characterized by profuse mycelial segmentation, transformation of disarticulated fragments of segmented mycelium into chains of thickened arthrospores, and formation of filiform appendages on the arthrosporic cells giving an insectival appearance to these elements. This unusual morphology was determined to be a consistent feature in 110 strains of S. aureofaciens studied, hence suggesting a useful taxonomic aid in identification of this species. Preliminary studies of multiple strains of many different Streptomyces species indicate that other useful taxonomic features based on morphology of submerged growth are found in a significantly large part of the genus.

Culture Media↗

Palaeopathology of spinal osteophytosis, vertebral ankylosis, ankylosing spondylitis, and vertebral hyperostosis.

Five hundred and sixty intact skeletons and several thousand disarticulated vertebrae have been examined with special reference to spinal fusion. In period they ranged from a 21st dynasty Egyptian mummy to a mid-19th century skeleton. Osteophytes were found in about half of the specimens, as reported previously. Fifteen skeletons with extensive blocks of spinal fusion were also identified. Sacroiliitis was present in two, but the asymmetrical spinal disease and peripheral joint changes suggested Reiter's disease or psoriatic spondylitis rather than ankylosing spondylitis. The remaining 13 had typical features of Forrestier's disease, and extraspinal findings indicative of diffuse idiopathic skeletal hyperostosis (DISH) were also common. A review of the available literature suggests that many palaeopathological specimens previously reported as anklylosing spondylitis are examples of DISH or other seronegative spondylarthropathies. The antiquity and palaeopathology of AS needs reappraisal.

Adult↗

Poor accuracy and interobserver reliability of knee arthroscopy measurements are improved by the use of variable angle elongated probes.

OBJECTIVES: (a)To determine the accuracy and reliability of arthroscopic measurements of cartilage lesion diameter in an artificial right knee model; (b) to determine whether the use of a set of variable angle elongated probes improves performance; and (c) to identify other sources of variability. METHODS: Ovoid "lesions" were drawn on the five cartilage surfaces of four plastic knees models. Two observers assessed these 20 lesions arthroscopically, measuring two diameters in orientations parallel and orthogonal to the probe. Observer 1 (orthopaedic surgeon) and observer 2 (arthroscopic rheumatologist) made two sets of measurements, firstly with the conventional probe and five months later with the variable angle elongated (VAE) probes. The knees were disarticulated to determine true lesion diameter. RESULTS: Observer 1 had negligible bias and good accuracy regardless of orientation or probe type. Observer 2 demonstrated both bias and poor accuracy using the conventional probe. Both improved using VAE probes. Poor interobserver reliability with conventional probes also improved using VAE probes. Major sources of variability could be traced to the probe type, the characteristics of the operator, and the orientation of the lesion in relation to the probe; the lesion location itself did not cause variability. CONCLUSIONS: Variation in accuracy and poor interobserver reliability of measurements with conventional methods of cartilage lesion diameter measurement improved when specially designed measurement probes were used. Arthroscopic measurements performed as well as most clinical and radiographic measures. These findings have important implications for the use of arthroscopy as an outcome in multicentre trials where arthroscopists have different levels of experience.

Analysis of Variance↗

The fine structure of conidial development in the genus Torula. I. T. herbarum (Pers.) Link ex S. F. Gray and T. herbarum f. quaternella Sacc.

Conidiogenesis in Torula herbarum and T. herbarum f. quaternella was observed by scanning and transmission electron microscopy. Conidia of the former were shown to be made up of three equally sized cells capped by a distinctive, and easily recognizable, conidiogenous cell. Conidiogenous cells also arose terminally on erect hyphae and on prostrate hyphae. The single-layered conidial cell walls were differentiated into an inner hyaline zone and an outer electron-dense zone formed by the deposition of melanin. Conidiogenous cells lacked melanin at the apex and, before conidiation, the lateral walls were strengthened by a further deposition of melanin. The apex bulged outwards and was modified into a new multicelled conidium bearing another apical conidiogenous cell. Continued development of new conidia resulted in an acropetal chain which became disarticulated after cytolysis within the conidiogenous cell. The relative distinctions between holoblastic and enteroblastic development are discussed and it is concluded that the conidia should be referred to as blastoconidia.

Cell Wall↗

The fine structure of conidial development in the genus Torula. IV. T. thermophila Cooney & Emerson.

Torula thermophila produced typical chlamydospores either as intercalary chains within prostrate hyphae or as terminal swellings on short, lateral, hyphal branches. Mature chlamydo-spores were spherical, dark brown, smooth-surfaced structured structures with thick, single-layed cell walls (= secondary wall layer) usually differentiated into an outer electron-dense zone and an inner electron-transparent zone. Disarticulation and sport release occurred after the disintegration of the original hyphal wall. The thallospores of T. thermophila arise in a manner different from the blastospores produced by other species of Torula and are structurally more closely related to the spores produced by Humicola insolens. However until further work has been completed on spore development in the Torula-Humicola complex of fungi the name T. thermophila is retained.

Cell Wall↗

Clostridium septicum infection associated with colonic carcinoma and hematologic abnormality.

Six patients with Clostridium septicum sepsis seen at Duke University Medical Center over a two-year period also had other abnormalities, consisting of hematologic disorders in 3 and colon tumors in 3. Three patients died of sepsis; 2 survived following disarticulation of the arm to control gas gangrene, while the sixth patient survived the sepsis but died of metastatic disease. When anaerobic cultures are positive for C. septicum, antibiotics should be given immediately. The high incidence of underlying colon tumor, especially in the cecum, should prompt consideration of a barium-enema examination.

Adenocarcinoma↗

CT with 3D rendering of the tendons of the foot and ankle: technique, normal anatomy, and disease.

Three-dimensional rendering of computed tomographic data with volume rendering (VR), shaded surface display (SSD), and maximum intensity projection has been performed for over 20 years. In the foot and ankle, no one image reformatting technique is satisfactory for displaying every anatomic relationship or disease process. Two-dimensional multiplanar reformatted (MPR) images are the basic images used for diagnosis. MPR images are especially useful for identifying small fractures. VR is useful for demonstrating the relationships between ankle tendons and the underlying osseous structures, and SSD is useful when fractures extend to the articular cortex and a disarticulated view is desired. Three-dimensional images are helpful in patients with congenital deformities, arthritis, and trauma.

Ankle↗

Alterations in protein and mineral contents of rat skull bones, evoked by different protein levels of the diet.

Holtzman rats of both sexes were fed one of the following semisynthetic diets; 25% protein (control), 10% protein (slight malnutrition), 5% protein (medium malnutrition), and 2% protein (severe malnutrition). The skulls were cleaned and disarticulated with papain; they were defatted, dried, and the total weight (Tw) of each bone was determined. A gravimetric method of estimating bone protein-mineral ratio was performed. Following a decalcification with EDTA, the protein matrix was weighed (PMw). A protein-mineral index (PMI) was defined as: PMI = 100 (Tw-PMw)/Tw. The nutritional deficiencies imposed affected the protein and/or mineral contents of the bones according to three different patterns. The first pattern was followed by the bones of the vault and the facial region. The second one included the basicranial bones. The third one comprised the incisors. These patterns were associated with the histogenetic characteristics of the bones and teeth. A relationship between these findings and previously ascertained functional craniofacial variations in malnourished rats was found. From the differential behavior of the base, the vault, the masticatory apparatus and the incisors, it was inferred that each of these functional cranial components reacted to the experimentally unbalanced nutrition, according to a specific kind of adaptive mechanism.

Animals↗