Prognostic factors in non-metastatic limb osteosarcoma: A 20-year experience of one center.

The purpose of this study was to evaluate the prognostic significance of variables in osteosarcoma. We performed a retrospective analysis of 35 patients with non-metastatic limb osteosarcoma that were treated between 1973 and 1994. The following variables were evaluated: age, sex, ethnic group, tumor histology and primary site, alkaline phosphatase (ALP) and lactate dehydrogenase (LDH) levels at diagnosis, treatment regimen, and the histologic response to treatment. Three variables showed significant correlation with prognosis: i) histologic response to preoperative treatment. Disease-free survival (DFS) was 89% in patients with grade III-IV histologic response after a median follow-up (MFU) of 64 months, 67% in patients with grade II after an MFU of 64 months, the patients with grade I response died within 15 months (p<0.0001); ii) treatment regimen. DFS was 83% after an MFU of 42 months, 62% after an MFU of 82 months, and 30% after an MFU of 177 months in patients treated by the 90's, 80's, and 70's protocols, respectively (p<0.05); iii) corrected ALP (cALP) levels at diagnosis. DFS was 78% after an MFU of 88 months in patients with cALP levels <200, and 32% after an MFU of 56 months in patients with cALP levels >200 (p=0.01). Low ALP levels, good histologic response to preoperative chemotherapy, and the new therapeutic regimen correlated with good prognosis in patients with osteosarcoma.

The natural history of osteonecrosis of the femoral head in children and adolescents who have Gaucher disease.

We reviewed the cases of eight patients (thirteen hips) with Gaucher disease who had had osteonecrosis of the femoral head and had been managed with bed rest and non-weight-bearing with crutches only in the symptomatic stage of the bone crisis. The mean age of the patients at the onset of the first crisis in each hip was ten years (range, six to fourteen years). The mean age at the most recent follow-up examination for the six living patients (ten hips) was twenty-three years (range, nineteen to thirty-three years). The Mose rating was good for one hip, fair for two, and poor for seven. Despite the over-all unfavorable radiographic ratings, the six patients were asymptomatic and did not need assistance with daily activities. There is no known treatment that effectively prevents the development of deformities of the femoral head. Thus, we recommend symptomatic management for osteonecrosis of the femoral head in Gaucher disease with bed rest and analgesics followed by non-weight-bearing on the involved limb, if it makes the patient more comfortable, during the symptomatic stage of bone crisis.

Diurnal rhythm of prostanoid secretion from bone/marrow organ in the rat.

The secretion of prostanoids from an adult diaphysial rat bone organ was assessed throughout a 24 h period at 4 h intervals as well as the 24 h activities of bone alkaline phosphatase isoenzyme and serum corticosterone levels. Femurs were removed from 16 rats at each interval and incubated in the absence or presence of indomethacin (100 micrograms). The levels of prostaglandin E2 (PGE2) prostaglandin F2 alpha (PGF2 alpha), and the stable metabolites of thromboxane and prostacyclin, thromboxane B2 (TxB2), and 6-keto prostaglandin F1 alpha (6-keto PGF1 alpha) were measured by radioimmunoassay (RIA). The patterns for each of the variables was subjected to cosinor analysis for predetermined various periods. The chronograms obtained indicated that PGF2 alpha, PGE2, and TxB2 showed 24 h rhythms with computed peak hour acrophases at 1700, 1800, and 2200 h, respectively, and prostacyclin demonstrated a 19 h rhythm with a peak secretion at 1100 h. Corticosterone levels and bone alkaline phosphatase isoenzyme activity in the serum were at peak at 1630 and 2200 h, and at nadir at 0500 and 1000 h, respectively, both exhibiting 24 h rhythms similar to those of PGF2 alpha, PGE2, and TxB2. Bone alkaline phosphatase isoenzyme activity in the femurs' incubation media showed a 12 h diurnal rhythm with peaks at 1230 and 2330 h and nadirs at 0600 and 2330 h (p < 0.01). In summary, this study demonstrated for the first time a 24 h rhythm of prostanoid secretion from diaphysial bone.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary bone tumor resectability: the value of serial MRI studies in the determination of the feasibility, timing, and extent of tumor resection.

We report the prospective analysis of 119 magnetic resonance imaging (MRI) studies in 41 patients with primary bone tumors performed from January 1984 to December 1990 to evaluate the contribution of serial MRI studies in the determination of feasibility, timing, and extent of tumor resection. Long repetition time (TR)-long echo time (TE) T2-weighted (T2, second echo) imaging was the most useful in assessing soft-tissue involvement, and short TR-short TE T1-weighted (T1) imaging for documenting the bone marrow changes. The feasibility, timing, and extent of resection was determined with the help of MRI in 20 cases with only two local recurrences. One study falsely suggested active tumor. In four other cases, a treatment decision was made based on the MRI. The MRI added significantly to the evaluation by computed tomography, radiographs, and bone scans.

Aminohydroxy propylidene bisphosphonate (APD) treatment improves the clinical skeletal manifestations of Gaucher's disease.

OBJECTIVE: To evaluate the long-term effects and safety of aminohydroxy propylidene bisphosphonate (APD) treatment on the frequency and severity of the clinical skeletal manifestations of Gaucher's disease. METHODOLOGY: Five adolescents who suffered from recurrent bone crisis episodes and atraumatic bone fractures due to Gaucher's disease were treated with APD for 14 to 83 months. RESULTS: During the 6 years before treatment, the patients suffered from 6 to 17 bone crisis episodes, or 1 to 2.8 episodes per patient per year. Three patients were free from bone crisis episodes during 14 to 32 months of ADP treatment, while two patients had two such episodes during 60 and 83 months of APD treatment (these represent a decrease in bone crisis episodes from 1 and 2.8 per year to 0.4 and 0.3 per year, respectively). Although four patients suffered from 1 to 3 atraumatic bone fractures during the 6 years preceding treatment (a total of 10 fractures), only one patient sustained a fracture on APD treatment (total of 219 months of treatment). Using APD was not associated with clinical side effects, biochemical aberrations, significant changes in liver and kidney function, or changes in serum levels of the hormones regulating mineral metabolism. In all patients, a band-like metaphyseal sclerosis appeared on radiography of the long bone. However, APD did not interfere with bone growth. CONCLUSIONS: The marked clinical improvement in the clinical skeletal manifestations of Gaucher's disease and the absence of toxic side effects in adolescent patients treated with APD support previous findings in three adult patients on the efficacy of APD and indicate possibilities for its use in inducing prolonged remissions in affected patients.

Upper limb involvement in patients with Gaucher's disease.

Seventeen of 55 children and adolescents with Gaucher's disease presented with upper limb pain. Seven had nonspecific pain, 11 had bone crisis, and 2 had arthritis symptoms in the proximal interphalangeal joints. X-ray films of both hands showed osteopenia and thinning of the cortex in half of the patients.

Acute calcific periarthritis of the hand and elbows in women. A study and review of the literature.

OBJECTIVE: To study the clinical and radiological features of acute calcific periarthritis (ACP) of the hand and elbow in women and to review the literature. METHODS: A retrospective study of ACP of the hand and elbow was conducted in 10 women, and the literature since 1970 reviewed, using Medline data. RESULTS: Ten women fulfilled the criteria of a single focus ACP. Mean age was 36.5 years (range 25-53). X-ray diffraction in 3 patients who underwent aspiration biopsy showed a pattern of calcium hydroxyapatite. The clinical course was short and self-limited. Most cases mimicked septic conditions and were initially misdiagnosed. All patients demonstrated gradual resolution of calcific deposits after 1-3 weeks. CONCLUSIONS: We conclude that calcific tendinitis of the hand and elbow is a self-limiting disease affecting mainly pre or perimenopausal women.

Spinal involvement in children and adolescents with Gaucher disease.

Nineteen patients with type I Gaucher disease with spinal involvement first diagnosed during childhood, were followed for 2 to 24 years (average 9 years), and their clinical and radiologic history reviewed. Patients presented with three types of pain: mild pain that was defined as nonspecific, severe pain due to bone crisis, and pain associated with vertebral collapse. Collapse of vertebra occurred gradually anywhere along the thoraco-lumbar spine, and usually more than one vertebra was involved. In three patients, rectangular collapse was noted. Six patients suffered from central vertebra collapse, and two from anterior wedge compression. Further vertebral collapse with signs of root and cord compression developed in three patients as they grew. Awareness to the possibility of progressive vertebral collapse and periodic follow-up might prevent severe complications.

Adhesion characteristics of chondrocytes cultured separately and in co-cultures with synovial fibroblasts.

The present study was designed to investigate the adherence mechanism(s) and behaviour of cultured chondrocytes under various culturing conditions, co-culturing with fibroblasts, or growth in the presence of conditioned medium either of fibroblasts or chondrocytes. The findings obtained indicate that chondrocyte time-adhesion curves and the final percentiles of attached cells to a plastic substrate are much slower and lower respectively than those of anchorage dependent cell types. The poorest adhesion occurs employing chondrocytes originated from suspension cultures, as compared to chondrocytes grown in monolayers. No interference with chondrocyte adhesion was found by inhibiting the production of proteoglycan (PG). Puromycin and to a lesser degree actinomycin but not cytosine arabinoside interfered with chondrocyte adhesion, suggesting the importance of protein synthesis in this process. The nature of proadhesion modifying molecules in synoviocytes conditioned media and antiadhesive agents in chondrocyte conditioned media suggests that both substances are heat labile, non-dialyzable, protein containing factors.

Improved results in osteogenic sarcoma 1973-79 vs. 1980-86: analysis of results from a single center.

Analysis of treatment results in osteogenic sarcoma patients with classical limb primary tumors and without metastasis at diagnosis or major protocol violations showed improved prognosis with a minimum follow-up of over 5.5 years when divided by years of treatment for all event-free survivors. Twelve patients treated in 1980-86 had a 5-year disease-free survival of 67% and 9 treated in 1973-79 had a 5-year disease-free survival of 33% (P = 0.0368). The improvement appeared to reflect the increase in the intensity of the chemotherapy utilized. Definitive surgery, amputation or limb salvage did not affect the outcome. With these surgical approaches the disease-free survival was 67% and 60% respectively.

Genu valgum in children who received renal transplant.

In 19 children who received renal transplants, 6 developed genu valgum one to two years after the transplantation. Except for one patient, biochemical blood and urine parameters were normal and bone radiograph showed no signs of renal osteodystrophy during this period. Two patients had bilateral varus supracondylar osteotomy, and in one patient medial hemistapling of the distal femur was performed.

Calcaneal ostectomy for Haglund disease.

From 1967 to 1987, operative treatment was performed on 28 feet in 22 patients with retrocalcaneal bursitis due to prominent posterior superior margin of the calcaneus (Haglund disease). Through a lateral or medial approach, the prominent bone was removed. The results indicated that excision of the retrocalcaneal bursa was not necessary. Preoperative planning and intraoperative examination was necessary in order to remove an adequate amount of bone, which was the key for a successful result.

Open reduction of acetabular fracture in pregnancy. A case report.

A 24-year-old woman in her 20th week of pregnancy sustained a severely displaced acetabular fracture. Skeletal traction failed to achieve reduction. Open reduction and internal fixation were performed through an extended iliofemoral approach without affecting the pregnancy. At full term, she had a normal delivery. Four years after the injury, the patient had a pain-free hip with full range of motion and a healthy child. The potential benefit or danger of surgery to the patient and her pregnancy was thoroughly considered.

Normal ranges of popliteal angle in children.

Measurement of the popliteal angle is used to assess hamstring contracture in children with cerebral palsy. The popliteal angle in 482 normal children, 1-10 years of age, was measured. Using a 360 degrees goniometer, the popliteal angle was measured with the hip held at 90 degrees flexion to indicate hamstring muscle tightness. Between the ages of 1 and 3 years, the mean angle was 6 degrees (range, 0-15). At age 4, the angle rose to 17 degrees in girls and 27 degrees in boys (range, 5-45). At greater than or equal to 5 years the mean angle was 26 degrees with little change (range, 0-50). A popliteal angle of greater than 50 degrees in the above age groups indicates abnormal hamstring tightness.

The distribution of peptide-containing nerves in the synovia of the cat knee joint.

Synovial capsule in cats is extensively innervated by a network with axonal diameter ranging from 0.6-3 microns according to its position and neuropeptide content. Nerve markers such as Neuron Specific Enolase (NSE) and Neurofilament triplet protein (NF) could be observed only when the axonal fibre attained a critical diameter of over the 3 microns limit. The relatively thick fibres (1-3 microns) show positive immunoreactivity for Substance P (SP), 5-hydroxytryptamine (5-HT), and Vasoactive Intestinal Peptide (VIP), and seldom coreact with NSE and NF, whereas, the thinnest fibres (0.6-0.8 microns) characterized to contain either Methionine or Leucine Enkephalin (M-Enk, L-Enk) did not coreact positively with axonal markers. We found that different anesthetics may effect variably the immunoreactivity of some neuropeptides (SP, L-Enk, 5-HT) while others (VIP, M-Enk) remained unaffected. Based on our data and the few reported ones in the pertinent literature, it is judged that urethane is the anesthetic of choice in experimental studies of neuropeptides. Our findings of isolated positive immunoreactive cell bodies to enkephalin in synovia might suggest the presence of intrinsic relay system, where the enkephalin acts as suppressor of SP and VIP release from the sunovium nerve terminals. Such a local inter-relationship between different neuropeptide systems might have a practical role on the understanding of the pathogenesis of different arthritic processes as well as therapeutic strategy in the future.

Trigger finger in young patients with insulin dependent diabetes.

Two-hundred-and-fifty patients with juvenile diabetes mellitus aged 3-38 years, were examined for trigger finger. Thirteen patients (5%) were found to have trigger finger--10 women and 3 men aged 14-38 years (mean 26 years). The ring, middle fingers, and thumb were the most affected. Two patients had bilateral trigger finger. There was a significant correlation between duration of diabetes and trigger finger (p less than 0.001) but no correlation with the control of diabetes. Our work indicates for the first time the prevalence of trigger finger in young patients with insulin dependent diabetes mellitus.