The natural history of hereditary multiple exostoses.

We established a database of hereditary multiple exostoses for the state of Washington, on the basis of a retrospective review of the medical records and a clinical evaluation of family members, to determine the prevalence, clinical range of expression, and rate of malignant degeneration. The database comprised forty-six kindreds with 113 affected members; all kindreds had at least one member living in the state of Washington. The over-all prevalence was at least one in 50,000.

Effect of sterilization and storage treatments on screw pullout strength in human allograft bone.

Many conflicting studies have been performed evaluating the effects of sterilization and storage treatments on the mechanical properties of allograft bone. In the current study, four right and left matched, sterile, deep-frozen, tibial pairs from human donors with an average age of 32 years were tested. One tibia from each pair served as a matched control for the opposite side.

MR appearance of skeletal neoplasms following cryotherapy.

Cryotherapy is an increasingly popular mode of therapy adjunctive to surgical curettage in the treatment of certain skeletal neoplasms, such as giant cell tumors or chondrosarcomas. The magnetic resonance (MR) findings following cryotherapy have not been previously reported. We reviewed the MR findings in seven patients with skeletal neoplasms following curettage and cryotherapy.

Measurement and prediction of the short-term response of soft tissue sarcomas to chemotherapy.

Background: Preoperative chemotherapy has been advocated for the treatment of soft tissue sarcomas, yet there is little information about how these tumors respond pathologically to therapy or whether tumor response can be predicted from a pretreatment biopsy.

Methods: Biopsy was done of 25 intermediate- or high-grade soft tissue sarcomas before they were treated with three cycles of doxorubicin and cisplatin and resected. The authors compared the pathologic features of the treated tumors with clinical and radiologic evidence of response to identify the pathologic features that best reflected chemotherapeutic effect.

Intradural recurrence with chondrosarcoma of the spine. A case report and review of the literature.

Known for its resistance to chemotherapy and radiation therapy, chondrosarcoma remains largely a surgically managed tumor predisposed to local recurrence and late distant metastasis. A 42-year-old man with known thoracic chondrosarcoma developed progressive low back pain, lower extremity weakness, and urinary incontinence. Magnetic resonance imaging and computed tomographic myelography demonstrated multiple filling defects throughout the lumbosacral region.

Genetic heterogeneity in families with hereditary multiple exostoses.

We have carried out a linkage analysis on 11 families segregating gene(s) for hereditary multiple exostoses (EXT). Four highly informative, short tandem-repeat (STR) markers that have been physically mapped to an interval surrounding the Langer-Giedion chromosomal region (8q24.11-q24.

The effects of freeze-drying and rehydration on cancellous bone.

Processing technique significantly affects the strength and stiffness of bone for use as a structural alloimplant in reconstructive surgery. The effects of the rehydration of freeze-dried human cancellous bone were studied using 14-mm-diameter cancellous dowels taken from matched cadaveric sites. Three different methods of rehydration were evaluated and compared with unrehydrated freeze-dried and frozen grafts.

Pediatric spine tumors with spinal cord compromise.

Twenty-nine pediatric spine tumor patients with neurologic deficit were reviewed retrospectively to evaluate tumor control, neurologic course, spinal deformity, and response to treatment. Diagnoses included 10 neuroblastomas, nine sarcomas, eight astrocytomas, and two lymphomas. Mean duration of symptoms at presentation was 8 weeks.

Comparison of STIR and spin-echo MR imaging at 1.5 T in 45 suspected extremity tumors: lesion conspicuity and extent.

Short inversion time inversion recovery (STIR) imaging and a double-echo spin-echo (SE) sequence at 1.5 T in 45 sequential patients with suspected extremity tumors were compared to assess the number of lesions detected, subjective conspicuity of lesions, approximate volume of abnormality detected in each lesion, and identification of peritumoral brightening in tissues adjacent to each lesion. STIR sequences enabled detection of all 45 lesions; 44 were detected with the SE sequence.

[Non-epidermidis coagulase-negative staphylococci in infectious diseases of the compromised host].

Today Staphylococcus epidermidis has been recognized as the etiological agent of infectious diseases such as endocarditis, sepsis and meningitis that mainly come out in compromised hosts because of the breaching of the mechanical barrier (cardiosurgical, neurosurgical patients and central venous catheter carriers). Other "non-epidermidis coagulase negative Staphylococci" are more and more frequently isolated from patients at high risk of infection. Faced with these isolations, it is difficult for the clinician and the microbiologist to give these microorganisms their effective pathogenic role.

[Severe course of delirium tremens. Results of treatment and late prognosis].

80 patients (P) (68 men and twelve women) with the diagnosis of delirium tremens were retrospectively analyzed and reexamined over a period of ten years (1974 to 1984). Included were only patients who--after failure of oral medication--required intravenous therapy with Chlomethiazol and thereby intensive care treatment. Mean age was 46.

Contralateral total hip arthroplasty or ipsilateral total knee arthroplasty in patients who have a long-standing fusion of the hip.

We studied the cases of twenty patients who had had an ipsilateral total knee arthroplasty or a contralateral total hip arthroplasty, or both, long after one hip had been fused in an acceptable position. Between 1972 and 1986, we performed twenty-one total joint arthroplasties (on thirteen hips and eight knees) and followed two additional patients (one hip and one knee) in whom the operation had been performed elsewhere. The average age of the patients at the time of arthroplasty was fifty-seven years (range, thirty-one to eighty-one years), and the average time from arthrodesis to arthroplasty was thirty-two years (range, eleven to fifty-four years).

[The influence of click polarity and stimulation rate on the auditory evoked response in relation to age].

In order to study the changes which occur in phase of the click stimulus and its relation to the stimulus repetition rate on the auditory brainstem response (ABR) as a function of age, the Authors recorded the ABR from the scalp's surface of 10 newborns and 40 infants, 3 months, 6 months, 1 year and 3 years old as well as from 10 normal adults. The stimulus was a square wave of 0.1 msec duration and 90 dBHL level.

Pitfalls in diagnosis: pediatric musculoskeletal tumors.

In general, the 5-year survival for patients with most types of sarcomas has doubled since the 1970s; improved chemotherapy, surgery, radiographic, and pathologic techniques all have contributed to this accomplishment. The 5-year survival for patients with osteosarcoma, like most sarcomas, is approximately 60% when the disease is treated with appropriate chemotherapy, surgery or radiation therapy. The details of treatment are complex and many controversies remain.