Composite resection of posterior pelvic malignancy.

Advanced pelvic cancer is a formidable challenge to surgical resection. These tumors commonly invade the bony pelvis, may involve other viscera, and usually have been irradiated previously. The authors are presenting experience with 76 patients who had composite resection of posterior or lateral pelvic malignancy. Fifty-eight patients had secondary cancers involving the musculoskeletal pelvis. This included 47 patients with advanced carcinoma of the rectum (41 curative, 6 palliative), 10 epidermoid cancers of the anorectum (8) or cervix (2), and 1 bladder cancer. Among the 18 patients with primary pelvic tumors were three patients with chordomas, six with bone tumors (osteosarcoma chondrosarcoma, grade III giant cell tumor), and nine with soft tissue tumors. All required major resection of the sacrum or pelvic side walls, and one half had an additional exenterative procedure. The overall mortality rate was 7.9%. Long-term estimated survival was 24% in patients having curative resection of recurrent rectal cancer, and 22.5% in 10 patients with advanced epidermoid cancer. Fifty per cent of patients with primary bone or soft tissue tumors survived from 13 to 88 months. Most patients had reasonable return of function, and were able to return to work or resume their normal previous lifestyle.     

Spinal MR findings in neurofibromatosis types 1 and 2.

PURPOSE: To evaluate the frequency and nature of spinal pathology, the frequency of clinically silent lesions, and the potential benefit of screening spinal MR in neurofibromatosis patients. PATIENTS AND METHODS: 28 neurofibromatosis type-1 (NF-1) patients and nine neurofibromatosis type-2 (NF-2) patients were studied with postcontrast spinal MR imaging. RESULTS: NF-1: One patient had a biopsy-proven low-grade glioma; five patients, intradural, extramedullary masses (N = 23); one patient, extradural masses (N = 2) (neurofibromas); 16 patients had bony abnormalities; and three patients thecal sac abnormalities. NF-2: Five patients demonstrated intramedullary masses (five/eight ependymomas); nine patients, intradural, extramedullary masses (meningiomas, schwannomas); and four patients, bony abnormalities. Eight/10 NF-1 and four/nine NF-2 patients had asymptomatic masses. CONCLUSION: Intradural disease is common, often asymptomatic, and often presents at a young age in NF-1 and NF-2 patients. Because of the propensity to develop significant asymptomatic as well as symptomatic intradural disease, screening of the entire spine with MR is recommended in both NF-1 and NF-2 patients.     

Stage I-II low grade non-Hodgkin's lymphoma: prognostic factors and treatment results.

48 patients with stage I-II low-grade non-Hodgkin's lymphoma were treated by radiation and/or chemotherapy between 1970 and 1986. The histologic types were diffuse lymphocytic well differentiated, eleven patients; nodular lymphocytic poorly differentiated, 28 patients; nodular mixed, nine patients. Complete remission was obtained in 45 patients (94%). Overall survival was 83% and 68% at five and ten years, respectively. Five and ten-year relapse-free survival of complete responders was 71% and 57%, respectively. Univariate analysis of potential prognosticators showed the following to significantly increase the survival rate: one or two sites of disease (p less than 0.01), stage I (p less than 0.02), age less than 65 years (p less than 0.02), complete excision of tumor mass (p less than 0.03), and the use of radiotherapy (p less than 0.02). The extent of radiotherapy field did not affect survival. Multivariate analysis by the stepwise proportional hazards model of Cox showed that the use of radiotherapy was the factor which significantly produced better survival figures (p less than 0.03). It is concluded that two thirds of stage I-II low-grade lymphoma patients are potentially curable; radiotherapy plays a major role in the management.     

Diagnostic sonography of the fetal genitourinary tract

Fetal diagnosis has vastly improved over the last decade. Ultrasound has become the imaging modality of choice. As real-time equipment has improved technologically, the ability to deduce subtle abnormalities has greatly increased. The fetal genitourinary tract may be evaluated for renal dysplasias, anomalies, or obstruction. Points of obstruction and, at times, the exact cause of obstruction may be deduced. Abnormalities of the ureter, bladder, urethra, scrotum, or reproductive system can be detected.     

T cells in the spinal cord in experimental autoimmune encephalomyelitis are matrix adherent and secrete tumor necrosis factor alpha.

We examined T cells isolated from an autoimmune tissue lesion and from lymphoid organs for their ability to secrete tumor necrosis factor-alpha (TNF-alpha) and to adhere to extracellular matrix (ECM) proteins. CD4+ T cells were obtained from spleens, popliteal lymph nodes, and spinal cords of Lewis rats that had been immunized with myelin basic protein (MBP) to induce experimental autoimmune encephalomyelitis (EAE). We now report that, irrespective of whether or not the T cells were activated with MBP or the T cell mitogen concanavalin A (ConA), the T cells isolated from the spinal cord lesions secreted greater amounts of TNF-alpha and adhered better to ECM than did T cells from the draining lymph node. Thus, the lesions of EAE concentrate a subpopulation of CD4+ T cells with enhanced ability to interact with blood vessel wall components and to secrete TNF-alpha.     

Doppler in non-immune hydrops fetalis.

Fetal ultrasound studies were performed on 24 fetuses with non-immune hydrops to evaluate echocardiographic and cardiovascular Doppler parameters that may be useful in assessing hemodynamics and in predicting outcome. Of all cardiovascular parameters analyzed, only the presence of abnormal pulsations in the umbilical vein (p < 0.001) was found to be significantly different between the 11 survivors and 13 non-survivors. In a smaller subset of 12 fetuses, in whom inferior vena caval waveforms were recorded, survivors (n = 6) had a significantly lower percentage of retrograde flow in the inferior vena cava (p < 0.001) and higher inferior vena caval E/V velocity ratio (p < 0.001) than non-survivors (n = 6). Sixteen of the 24 cases examined had abnormal umbilical venous pulsations; 12 of the 16 (75%) died including all fetuses with hydrops due to twin-to-twin transfusion or congenital heart disease. When fetuses with pulsatile flow in the umbilical vein were compared with fetuses with normal umbilical venous flow, the following significant differences were found: lower right and left ventricular output velocities, larger inferior vena caval diameter, decreased shortening fractions of the right and left ventricles, and lower peak velocities at the aortic and pulmonary valves and in the ductus arteriosus.     

Clinical decision making for discharge planning in a changing psychiatric environment.

This article reviews the clinical decision-making process involved in planning for continuity of care following discharge from inpatient psychiatric services. The decision-making frame of reference requires the identification of the players in the process, parameters of their roles, performance expectations, and boundaries of rights and obligations; clear definition of objectives; collection of data and analysis of its relevancy; and study of possible alternatives, with evaluations of their consequences. The authors present an approach to clinical decision making that includes an analysis of modalities and practice skills and a critical review of practice in a psychiatric setting that emphasizes the role of the family; the importance of continuity of care; and the need to engage the patient, family, and care systems within briefer time frames.     

Mapping of the formin gene and exclusion as a candidate gene for the autosomal recessive form of limb-girdle muscular dystrophy.

Limb-Girdle Muscular Dystrophy (LGMD) is a myopathy with clinical and transmission heterogeneity. The recessive form, LGMD2, has been recently mapped by linkage analysis to 15q. As an attempt to identify the gene involved in this pathology, we tested as candidate gene the LD locus, called LD for limb deformity. This gene has recently been identified and mapped to chromosome 15q13-q14. It is homologous to the murine formin gene which is localized to mouse chromosome 2. Mutations in this murine gene have been shown to cause limb deformity and kidney defect. YAC clones containing the LD gene were isolated and utilised to confirm the cytogenetic localisation. Internal DNA polymorphisms of the LD locus were analyzed in LGMD2 and CEPH families. The LD gene was mapped between the alpha cardiac actin gene and the D15S24 locus. Crossovers between the LGMD2 and the LD loci excluded the LD gene as a candidate for LGMD2.