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H J Mankin

Publications and source records attributed to H J Mankin.

At least 19 recordsLinked to original sources
PubMed1995

Osteoid osteoma: percutaneous radio-frequency ablation.

PURPOSE: To evaluate a percutaneous technique for in situ destruction of osteoid osteoma. MATERIALS AND METHODS: Radio-frequency ablation was performed in 18 patients with osteoid osteoma (17 male, one female; age range, 8-42 years). Diagnosis was established by means of clinical and radiographic features and confirmed by means of needle biopsy findings. No attempt was made to remove the lesion. A small radio-frequency electrode introduced into the lesion through the biopsy track was used to produce thermal necrosis of a 1-cm sphere of tissue. RESULTS: Symptoms were completely relieved in 16 (89%) of 18 patients. In one patient, a second procedure was required for pain relief. All but two patients underwent treatment as outpatients: These two were hospitalized for 1 night only. All patients resumed normal daily activities immediately. No casts or external supports were required; there were no complications. Twelve patients were followed up for more than 1 year. There were no recurrences CONCLUSION: Radio-frequency ablation of osteoid osteoma is a promising alternative to surgery in selected patients.

Activities of Daily Living↗
PubMed1995

Role of surgical resection in pelvic Ewing's sarcoma.

PURPOSE: The improved survival in patients with Ewing's sarcoma over the past two decades has placed increased importance on achievement of local disease control. Ewing's sarcoma that arises in the pelvis has been recognized to have a worse prognosis than that in the appendicular skeleton, and the role of surgical resection in these cases remains controversial. The current study attempts to identify a benefit to surgical resection in these patients. METHODS: We retrospectively examined 39 patients who presented with Ewing's sarcoma in a pelvic location, all of whom were treated systemically with chemotherapy. Twenty patients received radiation only as a means of local control, and 19 underwent resection with or without radiation therapy. The patients were evaluated with end points of disease-free survival and overall survival for a minimum of 24 months and a mean of 58 months. RESULTS: There was an even distribution among patients who underwent surgical resection for local control as compared with those who received only radiation therapy with respect to age, site, date of treatment, and stage of disease. Despite uncontrolled biases including tumor size and response to chemotherapy that would be expected to favor patients who undergo resection, surgery in addition to or in substitution for radiation therapy did not result in a statistically significant increase in disease-free survival or overall survival. Local disease control was comparable between those who underwent resection and those who did not: three patients in each group developed a local recurrence. CONCLUSION: Currently, morbidity of surgical resection should be weighed against the efficacy and secondary complications of radiation therapy in the decision-making process for local disease control. The issue of whether overall survival and local disease control is improved in patients who undergo surgical resection remains controversial and may require a prospective randomized trial to be answered definitively.

Adolescent↗
PubMed1995

Enzyme replacement therapy for Gaucher disease: skeletal responses to macrophage-targeted glucocerebrosidase.

OBJECTIVES: Reversal of the hematologic and visceral abnormalities characteristic of Gaucher disease, the most common lipid storage disorder, with biweekly infusions of macrophage-targeted glucocerebrosidase (glucosylceramidase) is well documented. The extent to which the skeleton responds to enzyme replacement therapy has not been systematically investigated. METHODS: To assess the skeletal response to enzyme replacement therapy, we treated 12 patients with type 1 Gaucher disease, who had intact spleens, with macrophage-targeted glucocerebrosidase. The initial dose of enzyme was 60 U/kg body weight every 2 weeks for 24 months, followed by reduction in dosage to 30 and then 15 U/kg body weight every 2 weeks, each for 9 months. RESULTS: The lipid composition of bone marrow, determined by direct chemical analysis, began to improve after 6 months of treatment at a time when noninvasive imaging studies showed no significant changes. By 42 months, improvement in marrow composition was demonstrable on all noninvasive, quantitative imaging modalities (magnetic resonance score, quantitative xenon scintigraphy, and quantitative chemical shift imaging) used in this study. Quantitative chemical shift imaging, the most sensitive technique, demonstrated a dramatic normalization of the marrow fat content in all patients. Net increases in either cortical or trabecular bone mass, as assessed by combined cortical thickness measurements and dual-energy quantitative computed tomography, respectively, occurred in 10 patients. CONCLUSIONS: Prolonged treatment over 3 1/2 years with macrophage-targeted glucocerebrosidase produces objective reversal of disease in both the axial and appendicular skeleton in patients with Gaucher disease. Marked improvement occurs in marrow composition and bone mass in both children and adults.

Adolescent↗
PubMed1992

Quantitative imaging of Gaucher disease.

Twenty-three patients with type 1 Gaucher disease were evaluated with a battery of quantitative imaging techniques. Plain radiographs were used to measure cortical thickness and Erlenmeyer flask deformity. Xenon-133 uptake was measured in scans of the lower extremities. Dual-energy quantitative computed tomography was used for calculation of trabecular bone and bone marrow fat content in the spine and long bones. Magnetic resonance (MR) imaging was performed to evaluate disease extent and three-dimensional splenic volume. MR images were also used to provide quantitative measurements of each vertebral fat fraction. Each imaging modality was correlated with the others as well as with the clinical history of skeletal complications and the hematocrit and acid phosphatase activity. There was a strong relationship between splenic volume and disease severity as measured clinically and with laboratory testing. The spinal fat fraction also correlated with these measures of disease severity and with the femoral fat fraction and xenon uptake. No measurement allowed discrimination of patients with from those without skeletal complications.

Adolescent↗
PubMed1992

Current status of allografting for bone tumors.

Over the past 20 years the Orthopaedic Oncology Unit of the Massachusetts General Hospital has performed over 660 massive cadaveric allograft transplantations, mostly for patients with bone tumors. The overall success rate for such procedures is around 80%, with most of the failures occurring in the first 3 years as a result of fracture (19%), nonunion (14%), and infection (10%). With longer follow up, late occurring osteoarthritis has been noted in approximately 17% of those patients at risk, suggesting that the articular surface begins to "wear" at 5 years. Some problems remain with the procedure, and more study of such aspects as the immune mechanism, cryopreservation of cartilage, maintaining the status and safety of the bone banking system, and improving the techniques of surgery ultimately may lead to a more uniform and predictable success rate.

Adolescent↗
PubMed1992

Fresh and frozen articular cartilage allografts.

Fresh and frozen cartilage allografts both function well to relieve joint pain due to cartilage injury. Fresh cartilage allografts appear to remain alive for many months and are most applicable to small injuries such as osteochondritis dissecans, traumatic defects, and osteonecrosis. Frozen cartilage allografts have been used mostly for treatment of bone tumors which most often involve the subchondral and the metaphyseal areas of the long bones, particularly around the knee and hip. Frozen cartilage allografts appear to survive well and maintain joint space for several years. Deterioration of either type of graft does not appear to result in joint pain, making these grafts useful for long periods of time.

Adaptation, Physiological↗
PubMed1992

Epithelioid sarcoma in the upper extremity.

Eighteen patients who had epithelioid sarcoma in the upper extremity were divided into two groups on the basis of treatment: marginal resection of the tumor or wide or radical resection of the tumor. Historical, demographic, histological, and staging data were recorded and were correlated with the type of treatment and the outcome. Marginal resection (excision through the reactive zone or pseudocapsule surrounding the tumor, with confirmation of a negative margin by a pathologist), with or without adjuvant therapy, led to a dismal outcome in patients who had been treated in this manner initially or secondarily (fifteen failures after seventeen procedures). Disease-free survival at ten years was significantly improved when wide resection (intracompartmental en bloc excision with a cuff of normal tissue of more than three centimeters) or radical resection was either the initial or the secondary treatment; success then was noted in nine of thirteen operations. Of twelve patients in whom a marginal resection had been done initially, three had had a recurrence, three had had a metastasis, and five had died, at a mean follow-up of seven years (range, twenty-seven months to sixteen years). At a mean follow-up of six years (range, two to fifteen years), only one of five patients in whom a secondary lesion had been treated with marginal resection was free of disease. Of the six patients who had been treated with wide or radical resection initially, none had died, one had had a recurrence, and one had had a metastasis, at a mean follow-up of seven years (range, twenty-two months to fourteen years).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Intraoperative electron beam radiation therapy for retroperitoneal soft tissue sarcoma.

From December 1981 to December 1989, 20 patients with primary or recurrent retroperitoneal sarcoma received 4000 to 5000 cGy of external beam radiation therapy (EBRT) in conjunction with surgical resection and intraoperative radiation therapy (IORT). Seventeen of 20 patients underwent complete (14 patients) or partial (3 patients) resection. Three patients had shown evidence of metastases after EBRT by the time of surgery. The 4-year actuarial local control and disease-free survival rates of the 17 patients undergoing resection were 81% and 64%, respectively. Twelve patients received IORT at the time of resection for microscopic disease (10 patients) or gross residual sarcoma (2 patients). Of the ten patients receiving IORT for microscopic tumor, one patient has died of local failure and peritoneal sarcomatosis and two patients have died of distant metastases only. The remaining seven patients are disease-free. One patient treated for gross residual sarcoma has experienced a local failure 1 year after IORT and is without disease 7 years after salvage chemotherapy. The other patient treated for gross residual sarcoma has died of local failure. Five patients did not receive IORT at the time of resection because of the extensive size of the tumor bed. Three of these patients are disease-free with one patient alive with lung metastases and one patient dying of hepatic metastases. Aggressive radiation and surgical procedures appear to provide satisfactory resectability and local control with acceptable tolerance.

Adolescent↗
PubMed1991

Replacement therapy for inherited enzyme deficiency--macrophage-targeted glucocerebrosidase for Gaucher's disease.

BACKGROUND AND METHODS: Gaucher's disease, the most prevalent of the sphingolipid storage disorders, is caused by a deficiency of the enzyme glucocerebrosidase (glucosylceramidase). Enzyme replacement was proposed as a therapeutic strategy for this disorder in 1966. To assess the clinical effectiveness of this approach, we infused macrophage-targeted human placental glucocerebrosidase (60 IU per kilogram of body weight every 2 weeks for 9 to 12 months) into 12 patients with type 1 Gaucher's disease who had intact spleens. The frequency of infusions was increased to once a week in two patients (children) during part of the trial because they had clinically aggressive disease. RESULTS: The hemoglobin concentration increased in all 12 patients, and the platelet count in 7. Serum acid phosphatase activity decreased in 10 patients during the trial, and the plasma glucocerebroside level in 9. Splenic volume decreased in all patients after six months of treatment, and hepatic volume in five. Early signs of skeletal improvements were seen in three patients. The enzyme infusions were well tolerated, and no antibody to the exogenous enzyme developed. CONCLUSIONS: Intravenous administration of macrophage-targeted glucocerebrosidase produces objective clinical improvement in patients with type 1 Gaucher's disease. The hematologic and visceral responses to enzyme replacement develop more rapidly than the skeletal response.

Acid Phosphatase↗
PubMed1991

Cartilage synthesizes the serine protease inhibitor PAI-1: support for the involvement of serine proteases in cartilage remodeling.

The work described here demonstrates the synthesis by human articular cartilage of plasminogen activator inhibitor-1 (PAI-1), a potent inhibitor of the serine protease tissue plasminogen activator (tPA). We also present data demonstrating an increase in PAI-1 messenger ribonucleic acid (mRNA) in chondrocytes exposed to the cytokine interleukin-1 (IL-1). Interestingly, this elevation of steady-state mRNA levels does not appear to result in an increase in synthesis of PAI-1 protein. Northern blot analysis reveals that of the two mRNA species (3.4 kb, 2.4 kb) previously reported for PAI-1, only the larger species (3.4 kb) appears to be synthesized by chondrocytes. Our data demonstrate the IL-1-stimulated production by cartilage of tissue plasminogen activator. We also show evidence for the presence of plasminogen in cartilage. A scheme is presented indicating the probable importance of the serine proteases (tPA and plasminogen) and PAI-1 in cartilage degradation.

Blotting, Northern↗
PubMed1991

Physeal reconstruction using tissue donated from early postnatal limbs in a murine model.

Physeal reconstruction was performed in a murine model by transplanting corresponding postnatal tissue from 4-day-old C57B mice to resection defects. The site of the reconstruction, the murine distal femoral epiphysis, is completely cartilaginous and avascular at this stage of development. The tissue transplanted into the defect was demonstrated to have high kinetic activity by its incorporation of tritiated thymidine. The physeal reconstruction as performed restored only 25% of normal growth. While transplanting cell populations is feasible, the method will require a great deal of work before clinical application.

Animals↗
PubMed1991

Low conductivity irrigating solutions for arthroscopy.

The purpose of this study was to find a nonconductive physiologic solution to use for arthroscopy when electrosurgery is performed. We studied the effect of several solutions on proteoglycan synthesis in bovine articular cartilage slices, and the effect of using an electrosurgical instrument in various solutions in bovine knee joints. Both HEPES buffer 0.3 M (N-2-hydroxyethylpiperazine-N'-2-ethanesulfonic acid) (Sigma Chemical Company, St. Louis, Missouri) and glycerol 2.6% gave good results in both categories. The glycerol solution appears to be the best from those tested since it supported cartilage metabolism somewhat better and is far less expensive than HEPES solution. Thus, in the use of electrosurgery during arthroscopic procedures, solutions such as Ringer's lactate and saline are too conductive to be effective or safe. Distilled water is nonphysiologic. Glycerol 2.6% is safe and effective and supports articular cartilage metabolism reasonably well for up to 2 h.

Animals↗
PubMed1991

Massive bone allografts for traumatic skeletal defects.

Large bone allograft transplants have been successfully used to reconstruct skeletal defects created by tumor resections and failed arthroplasties, but little has been reported on their use in traumatic defects. Of approximately 500 allograft procedures done at the Massachusetts General Hospital from 1979 to 1988, 11 were done for restoration of traumatic bone loss. The average age of the patients was 30 (range 11 to 71 years), and the location of the defect was the tibia or femur in 10 of the 11 patients studied. Eight osteoarticular grafts (six hemicondylar and two total condylar) and three intercalary grafts were used for six open and five closed fractures. The time from injury to reconstruction averaged 17 months (3 to 96 months). Primary reconstruction was done in three cases and a salvage procedure in eight. Patients were assessed by the operating surgeon and a physical therapist using an evaluation system that considers function, life-style, and emotional acceptance. According to the system, nine patients had excellent or good results (six hemicondylar grafts, three intercalary grafts), one patient had a fair result (total elbow graft), and one patient had failure of a total condylar graft and subsequently required an amputation. This study suggests that large bone allografts are of value in reconstructing traumatic skeletal defects, especially those involving an articular surface in a young patient.

Adolescent↗