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Biomedical subjects

D Samson

Publications and source records attributed to D Samson.

At least 37 records · Page 2Linked to original sources
PubMed2000

CCNU (lomustine), idarubicin and dexamethasone (CIDEX): an effective oral regimen for the treatment of refractory or relapsed myeloma.

We report the results of a non-randomized pilot study of an oral regimen comprising CCNU (lomustine; 25 or 50 mg/m2 on day 1), idarubicin (4-demethoxydaunorubicin) (10 mg/m2 on days 1-3) and dexamethasone (10 mg b.d. on days 1-4) in patients with relapsed or refractory myeloma. Treatment was given every 28 d for a maximum of six courses. Sixty patients were entered of whom 57 were evaluable. Overall response rate (partial or minor response) was 49% with 30% of patients achieving a partial response (50% tumour reduction). Response rates were higher in patients with untested relapse than in those with refractory disease (overall response rates 56% vs. 31%). The major toxicity was neutropenia and the regimen was otherwise well tolerated. The median survival from entry of all patients was 15 months, with 30% of patients alive at 2 years. This regimen represents a useful addition to available treatment options.

Adult↗
PubMed1999

A fine integrated map of the SPG4 locus excludes an expanded CAG repeat in chromosome 2p-linked autosomal dominant spastic paraplegia.

Autosomal dominant hereditary spastic paraplegia (AD-HSP) is a genetically heterogeneous disorder characterized by progressive spasticity of the lower limbs. A major locus (SPG4) causing AD-HSP in about 40% of the families was mapped to chromosome 2p. The analysis of six SPG4-linked AD-HSP families using the RED procedure previously showed the expansion of a CAG repeat in affected individuals. To identify the gene responsible for this form of HSP, we have constructed a 3.5-Mb YAC contig flanked by loci D2S400 and D2S367, have subcloned five of these YACs spanning the candidate region into cosmids, and screened these cosmid libraries for the presence of CAG repeat sequences. Four CAG repeats have been identified but none of them is expanded in 26 patients from 13 SPG4-linked AD-HSP families. A gene map comprising 21 transcripts was established using expressed sequence tags (ESTs) assigned previously to this region of 2p21-p22 with radiation hybrid panels GeneBridge 4 and G3. Full-length cDNAs corresponding to the 14 ESTs mapping to the SPG4 interval flanked by loci D2S352 and D2S2347 were isolated and sequenced. None contains a CAG repeat in its coding sequence. Finally, we have assembled a BAC contig composed of 37 clones that were also screened for the presence of CAG repeats; this failed to detect additional repeats to those identified on YACs.

Chromosomes, Bacterial↗
PubMed1999

Dural waisting as a sign of subarachnoid extension of cavernous carotid aneurysms: a follow-up case report.

OBJECTIVE: Even when augmented by CT and MRI, it can be difficult on angiography to predict which intracavernous carotid artery aneurysms (ICCAA) have subarachnoid extension and therefore pose a higher risk of subarachnoid hemorrhage. Previously we reported a case of an ICCAA, which on angiogram had a focal tapering of the dome that we termed a "waist." At surgery this lesion was found to have subarachnoid extension. We postulated that this dural "waisting" on the arteriogram was a predictor of subarachnoid extension. Herein we report a second case of an ICCAA with the angiographic appearance of a waist that was also confirmed to have subarachnoid extension at surgery, thereby strengthening our original hypothesis. CLINICAL PRESENTATION: A 40-year-old woman presented with a 3-month history of bitemporal headache, diplopia, and a left sixth nerve palsy. MRI showed a lesion in the vicinity of the left cavernous carotid sinus and an arteriogram confirmed the presence of a large cavernous carotid aneurysm. As in the previous case, the aneurysm fundus was indented, creating a waist on the aneurysm dome. INTERVENTION: After passing a trial balloon occlusion of the involved carotid artery, the patient was brought to the operating room for lesion trapping. The aneurysm fundus was seen to extend beyond the falciform ligament and with subsequent dissection, the neck of the aneurysm was seen to incorporate the carotid artery distal to the ophthalmic artery. The aneurysm was trapped by ligating the internal carotid artery in the neck and by placing a clip on the intracranial carotid proximal to the posterior communicating artery. CONCLUSION: The presence of subarachnoid extension of an ICCAA can be difficult to elucidate on an arteriogram. This is an additional case in which a focal narrowing or "waisting" of the aneurysm dome seen on an angiogram served as a marker of subarachnoid extension.

Adult↗
PubMed1999

Laparoscopic surgery in gynecology: randomized prospective study comparing pneumoperitoneum and abdominal wall suspension.

OBJECTIVE: To compare laparoscopic surgery using insufflation of carbon dioxide gas with laparoscopic surgery using abdominal wall retractor in gynecology. SETTING: University hospital. DESIGN: Prospective randomized study. MATERIAL AND METHODS: Fifty-one patients were assigned to the gasless laparoscopy group, and 52 patients were assigned to the laparoscopy group with pneumoperitoneum. Patients presented with ovarian cyst, endometriosis, acute salpingitis, hydrosalpinx and extra-uterine pregnancy. RESULTS: No severe complications were noted. One conversion to laparotomy was performed in each group. Eight gasless laparoscopic procedures were converted into laparoscopy with pneumoperitoneum; difficulties appeared in patients with adhesions following laparotomy and in cases of unsatisfactory exposure of the pelvis. No differences appeared between the two groups in terms of complications, quality of the operative sequelae, and duration of hospitalization. CONCLUSION: Gasless surgery is a recent technique and progress in modifying the equipment is necessary to reduce conversions.

Abdominal Muscles↗
PubMed1999

Spastin, a new AAA protein, is altered in the most frequent form of autosomal dominant spastic paraplegia.

Autosomal dominant hereditary spastic paraplegia (AD-HSP) is a genetically heterogeneous neurodegenerative disorder characterized by progressive spasticity of the lower limbs. Among the four loci causing AD-HSP identified so far, the SPG4 locus at chromosome 2p2-1p22 has been shown to account for 40-50% of all AD-HSP families. Using a positional cloning strategy based on obtaining sequence of the entire SPG4 interval, we identified a candidate gene encoding a new member of the AAA protein family, which we named spastin. Sequence analysis of this gene in seven SPG4-linked pedigrees revealed several DNA modifications, including missense, nonsense and splice-site mutations. Both SPG4 and its mouse orthologue were shown to be expressed early and ubiquitously in fetal and adult tissues. The sequence homologies and putative subcellular localization of spastin suggest that this ATPase is involved in the assembly or function of nuclear protein complexes.

Adenosine Triphosphatases↗
PubMed1999

Extracorporeal photopheresis (ECP) in the treatment of chronic graft-versus-host disease (GVHD)

The aim of our study was to assess the efficacy of extracorporeal photopheresis (ECP) in chronic graft-versus-host disease (GVHD). Eleven patients with chronic cutaneous GVHD were studied. Four had mucosal involvement and five had pulmonary involvement. All had failed to improve on first- and second-line therapy. Three patients received ECP alone; the remainder continued to receive steroids and/or immunosuppressive therapy. Patients received ECP twice monthly for 4 months and then once monthly for 3 months. They were evaluated by serial skin scores, mucosal and skin photography, pulmonary function tests, biochemical and haematological parameters. Nine patients showed objective evidence of cutaneous improvement with a mean reduction in skin score of 48% overall. In the 10th patient, skin scores and oral involvement improved on twice monthly ECP but deteriorated when reduced to once monthly. The final patient died from renal failure secondary to cyclosporin toxicity. Two out of five patients with lung involvement showed a mild improvement in pulmonary function tests. Liver function tests were abnormal in five patients; they improved in one and deteriorated in three. All patients receiving concomitant immunosuppressive/steroid therapy were able to reduce drug dosages by trial completion. Our results indicate that ECP can benefit patients with cutaneous and mucosal chronic GVHD who have failed on first- and second-line therapies. The effect on the systemic manifestations of GVHD is less consistent.

Adolescent↗
PubMed1999

CD34+-selected peripheral blood progenitor cell transplantation in patients with multiple myeloma: tumour cell contamination and outcome.

Thirty-six patients with multiple myeloma (23 PR1, nine PR2, four stable disease) were entered into a pilot study evaluating the use of CD34+-selected peripheral blood progenitor cell transplantation (PBPCT) following high-dose melphalan alone or high-dose melphalan and total body irradiation. Peripheral blood progenitor cells (PBPCs) were mobilized with cyclophosphamide and granulocyte colony stimulating factor (G-CSF). CD34+ selection using the Cellpro Ceprate-SC system was performed in 22 cases with an adequate yield in 20. 10 patients failed to mobilize sufficient cells to permit selection and in four cases selection was not performed for other reasons. 16 patients therefore received unselected PBPC. Tumour cell contamination was evaluated by IgH gene fingerprinting (fpPCR). Harvested PBPC were fpPCR positive in 13/20 CD34+-selected cases and remained positive after selection in seven. Harvested PBPC were studied in 9/16 patients receiving unselected cells; fpPCR was positive in five and negative in four. There was no difference in event-free survival (EFS) between the CD34+-selected group and the unselected group (median 21 and 26 months, respectively, P=ns). The CD34+-selection process therefore reduced contamination but did not eliminate it completely, and in this small non-randomized study there was no apparent clinical benefit of CD34+ selection.

Adult↗
PubMed1999

Phenotypic and molecular analysis of six human cell lines derived from patients with plasma cell dyscrasia.

Cell lines RPMI 8226, JJN3, U266 B1, NCI-H929 (all EBV-) and ARH77 and HS-Sultan (both EBV+) have been extensively characterized in this study. EBV- lines expressed the phenotype (CD138-, CD19+, CD20+) whereas EBV+ were (CD138+, CD19-, CD20-). CD56 expression was restricted to EBV- cell lines, with the exception of U266 B1, whereas PCA-1 was strongly expressed on five of the six cell lines. Only EBV+ cell lines bound peanut-agglutinin (PNA). However, all cell lines bound the lectin Jacalin that binds the same receptor as PNA, irrespective of the receptors sialylation status. By RT-PCR and direct sequencing of their IgH V/D/J domains, ARH77 was demonstrated to use the germline sequence VH4-34/dm1/JH6b, whereas no arrangement was demonstrated for RPMI 8226, suggesting IgH gene deletion or mutation. HLA class I and II antigens were detected using HLA typing on all cell lines warranting their use as suitable targets for HLA-restricted cytotoxic T cells. By sensitive RT-PCR, mRNA for IL-6, IL-6R and TNFbeta was found expressed in all cell lines. IL-1 mRNA expression was predominantly associated with the EBV+ phenotype. Although mRNA for IL-3 and GM-CSF was never detected, transcripts for c-kit ligand and, more commonly, its receptor were. Likewise GM-CSF, M-CSF and erythropoietin mRNA transcripts were detected in the majority of cell lines.

Base Sequence↗
PubMed1999

Current results of the surgical management of aneurysms of the basilar apex.

OBJECTIVE: To provide current information regarding the expected clinical outcomes and sources of morbidity and mortality in the modern surgical management of basilar apex aneurysms. METHOD: A retrospective review was conducted of 303 cases of such aneurysms that were treated surgically during 18 years at one institution. Postoperative angiography was performed in 81% of the cases. Clinical grading using the Glasgow Outcome Scale was conducted at the time of hospital discharge and for 91% of the surviving patients at 6 months after surgery. The preoperative parameters that were linked statistically to poor clinical outcome were identified through the use of single and multivariate analyses. RESULTS: More than 80% of the patients were operated on using some modification of the trans-sylvian exposure, and temporary arterial occlusion was used routinely. Good outcomes (Glasgow Outcome Scale scores of 4 or 5) were achieved in 76% of the patients at the time of discharge and in 81% of the patients at 6 months after surgery. There was no incidence of postoperative subarachnoid hemorrhage. Residual aneurysm was revealed by postoperative angiography in 6% of the cases. Factors found to be statistically linked to poor outcome included poor admission grade (Hunt and Hess Grades IV and V), patient age older than 65 years, computed tomographic demonstration of thick basal cistern clot, aneurysm size greater than 20 mm, and symptoms attributable to brain stem compression. CONCLUSION: Direct microsurgical repair of basilar apex aneurysms should result in good clinical outcomes in 80 to 85% of cases, with reliable prevention of subarachnoid bleeding and routine elimination/reduction of symptoms secondary to mass effect. Those patients who are at high risk for poor outcomes can be identified by the presence of certain clinical, radiographic, and demographic features before undergoing surgery and can be considered for alternative or adjunctive modes of therapy if long-term efficacy of such treatment is demonstrated.

Adolescent↗
PubMed1999

Aneurysm retreatment after Guglielmi detachable coil and nondetachable coil embolization: report of nine cases and review of the literature.

OBJECTIVE: Guglielmi detachable coil embolization of cerebral aneurysms is becoming increasingly used to manage certain intracranial lesions based on aneurysm geometry, patient condition, and patient and surgeon preferences. Aneurysm recurrences or incomplete initial treatments are not uncommon, making repeat treatment necessary using either surgical or endovascular techniques. METHODS: Between January 1993 and June 1998, 1025 cerebral aneurysms were managed by the authors at a single hospital. One hundred twenty-four of these lesions were treated using Guglielmi detachable coils, and one was managed with nondetachable coils. During the follow-up period, eight patients who underwent embolization at our institution and one who underwent embolization elsewhere received repeat treatment. Five were approached surgically, and four underwent re-embolization. All charts and films were reviewed retrospectively to determine patient outcome and clinical success. RESULTS: No patient in the subgroup of this clinical study suffered a permanent complication from initial aneurysm coiling, no episodes of subsequent bleeding occurred, and no complications resulted from any subsequent therapies. The anatomic results were excellent, and all aneurysms were totally or near totally obliterated. CONCLUSION: Subtotal initial coil embolization of aneurysms can be managed safely using a variety of surgical and endovascular techniques. Our approach to this predicament, lessons we have learned, and a review of the literature are herein discussed.

Adult↗
PubMed1998

A transcriptional Map of the FMF region.

Familial Mediterranean fever (FMF) is a recessively inherited disorder characterized by attacks of fever and serositis, which affects primarily non-Ashkenazi Jews, Armenians, Turks, and Arabs. We present here a transcriptional map covering the FMF locus that we constructed in the course of the positional cloning of the gene responsible for this disease. This map was established from a contig constructed with YAC, BAC, and cosmid clones and covers about 500 kb of 16p13.3. It contains nine transcriptional units corresponding to known genes or to genes belonging to known gene families, 23 gene fragments characterized by partial sequences, and an endogenous retrovirus sequence. It thus considerably increases the number of genes in this interval and improves our knowledge concerning some of the genes or gene families present in this region. Data accumulated in this region were also used in a comparative study of different methods of exon detection.

Base Sequence↗
PubMed1998

High-dose therapy including carboplatin adjusted for renal function in patients with relapsed or refractory germ cell tumour: outcome and prognostic factors.

Thirty-one consecutive patients with relapsed or refractory GCT received an HDT schedule including carboplatin, the dose of which was adjusted to measured glomerular filtration rate. There was one HDT-associated death (3%), due to acute renal failure. The 3-year probability of overall and disease-free survival for 21 patients with primary refractory disease or responsive relapse was 60% and 42%, respectively, while none of ten patients with refractory relapse have survived disease free.

Carboplatin↗
PubMed1998

High-dose etoposide with granulocyte colony-stimulating factor for mobilization of peripheral blood progenitor cells: efficacy and toxicity at three dose levels.

High-dose etoposide (2.0-2.4 g m(-2)) with granulocyte colony-stimulating factor (G-CSF) is an effective strategy to mobilize peripheral blood progenitor cells (PBPCs), although in some patients this is associated with significant toxicity. Sixty-three patients with malignancy were enrolled into this non-randomized sequential study. The majority (55/63, 87%) had received at least two prior regimens of chemotherapy, and seven patients had previously failed to mobilize following high-dose cyclophosphamide with G-CSF. Consecutive patient groups received etoposide at three dose levels [2.0 g m(-2) (n = 22), 1.8 g m(-2) (n = 20) and 1.6 g m(-2) (n = 21)] followed by daily G-CSF. Subsequent leukaphereses were assayed for CD34+ cell content, with a target total collection of 2.0 x 10(6) CD34+ cells kg(-1). Toxicity was assessed by the development of significant mucositis, the requirement for parenteral antibiotics or blood component support and rehospitalization incidence. Ten patients (16%) had less than the minimum target yield collected. Median collections in the three groups were 4.7 (2 g m(-2)), 5.7 (1.8 g m(-2)) and 6.5 (1.6 g m(-2)) x 10(6) CD34+ cells kg(-1). Five of the seven patients who had previously failed cyclophosphamide mobilization achieved more than the target yield. Rehospitalization incidence was significantly lower in patients receiving 1.6 g m(-2) etoposide than in those receiving 2.0 g m(-2) (P = 0.03). These data suggest that high-dose etoposide with G-CSF is an efficient mobilization regimen in the majority of heavily pretreated patients, including those who have previously failed on high-dose cyclophosphamide with G-CSF. An etoposide dose of 1.6 g m(-2) appears to be as effective as higher doses but less toxic.

Acute Disease↗
PubMed1998

Posteroinferior cerebellar artery aneurysms: surgical results for 38 patients.

OBJECTIVE: Posteroinferior cerebellar artery aneurysms have an incidence of approximately 0.49%. Reports in the literature are sparse concerning outcomes in this patient population. We report our results for 38 consecutive patients who were treated during the last 6.5 years. METHODS: All patients (n = 38) with posteroinferior cerebellar artery aneurysms that were surgically treated at Zale-Lipshy University Hospital between January 1990 and May 1997 were retrospectively reviewed. Data were collected and analyzed relating to demographics, condition at presentation, lesion characteristics, associated medical problems, postsurgical complications, and outcome. RESULTS: Sixty-six percent of the patients (n = 25) experienced neurological sequelae, which included symptomatic vasospasm, hydrocephalus, dysarthria, paresis, diplopia, ataxia, and facial paralysis. Many, however, showed significant improvement during their hospitalization and during the course of the ensuing year. Seventy-four percent of the patients had a Glasgow Outcome Scale score of 1 or 2 at the time of discharge, 91% at 6 months after surgery, and 89% at 1 year after surgery. CONCLUSION: This review summarizes the presentations and outcomes of 38 consecutive surgical cases during a 6.5-year period and concludes that posteroinferior cerebellar artery aneurysms are not benign entities. The study does, however, also demonstrate that patients have significant recuperative potential after the treatment of these lesions.

Adult↗