[A case of Wegener's disease with rare complications: anuria and exophthalmos].
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Biomedical subjects
Publications and source records attributed to F Pene.
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From January 1979 to December 1987, 35 cases of primary central nervous system lymphoma (CNS-L) were treated. We recently reviewed these cases focusing on treatment results, treatment modalities, and radiotherapy (RT) or chemotherapy-radiotherapy (CT-RT). Variables such as age, risk factors, presenting symptoms, and histologic condition (all were high-grade or intermediate-grade non-Hodgkin's lymphomas [NHL]) and radiologic data were similar to those of series reported previously. The median survival time was 36 months (+/- 0.2 months) and the disease-free survival (DFS) time was 16 months (+/- 0.12 months). Twelve of 32 patients evaluable for treatment results experienced a recurrence (all but one occurred in the CNS). The DFS rate was 70% for the CT-RT group and 50% for the RT group (median follow-up time, 24 months). Therapeutic results in CNS-L are discussed with special emphasis on a putative role of CT in the management of this rare type of tumor.
UNLABELLED: From 1972 to 1985, 260 cases of anal canal epidermoid carcinoma were irradiated. Eighteen cases treated for palliation were excluded from the study; 242 (93%) were treated with curative intent. The sex ratio was 1/5.5; mean age was 66 years. HISTOLOGY: 60.3% were well differentiated epidermoid carcinoma; 31.0% moderately differentiated and 8.7%, cloacogenic cases. Staging: T1: 11.5%; T2: 16.1%; T3a: 17%; T3b: 33.5%; and T4: 21.9%. Abnormal inguinal nodes were present in 15.3% of cases. Crude overall survival (Kaplan-Meier) for the 242 cases is 86.4% at 1 year, 63.9% at 3 years, 51.2% at 5 years, and 30.8% at 10 years. Radiation therapy was the sole treatment for 193 cases. No chemotherapy was given. Patients were irradiated by external beam. They received a first course of X rays (mostly 18 MV, some 6 MV) 40 to 45 Gy (box technique) over 4 to 5 weeks in the pelvis. Age and size of tumor were considered when deciding on the target volume. After a rest period of 4 to 6 weeks, a second course of 15 to 20 Gy in 2 weeks was given through a perineal field by electron-beam of suitable energy. The mean total dose was 60.56 Gy and median was 62.5 Gy; the mean overall treatment duration was 85.3 days (median 82 days) and the mean Time Dose Factor including decay factor was 98.96. In this group, 5-year determinate survival was: T1-T2, 84.5%; T3a, 74.8%; T3b, 64.9%; T4, 58.9%. In 147/193 patients (76.2%) local control was achieved. The overall anal conservation rate was 62.6%. In 106 cases (55%), the anus had maintained normal function. The 5-year survival rate by N was 73.3% in the absence of inguinal nodes (169 cases) and 36.1% if such nodes were present. There was no significant difference in survival rate according to histological type. In the second group, receiving radiation therapy plus surgery, 33/49 cases (T3b-T4) were irradiated before surgery (median dose 40.5 Gy). Post operative radiation therapy was administered in 16 cases (T3b-T4) (median dose 49.6 Gy). The 5-year determinate survival is 53.2% for T3b and 79% for T4. According to the log-rank test, there was no significant difference between survival with radiation therapy alone and radiation therapy plus surgery. Multivariate analysis of the whole group indicated that T stage is the only predictive variable.(ABSTRACT TRUNCATED AT 400 WORDS)
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Fourteen adult patients in first complete remission of acute leukemia (A.L.) [6 with acute lymphoblastic leukemia (ALL), 8 with acute non lymphoblastic leukemia (ANLL)] were consolidated with high dose cyclophosphamide and total body irradiation followed by autologous bone marrow transplantation (ABMT) with marrow cleansed in vitro by Asta Z 7557. According to our previously described protocol showing evidence for a wide range of sensitivity from patient to patient, the marrow of each individual patient was incubated with the highest tolerable dose of Asta Z 7557. This dose, individually determined, was defined as the dose sparing between 0 and 10% of CFU-GM (CFU-GM DL95). ABMT was not followed by maintenance therapy. Hematological reconstitution was significantly faster in ALL patients when compared to ANLL patients. Out of these 14 patients: 2 relapsed on months 5 and 15 respectively after ABMT, and 2 died in complete remission on months 3 and 16 respectively, of veno-occlusive disease and encephalitis. Ten patients (70%) remain in complete remission up to a median of 15 months +, with 4 patients over 24 months +.
Between June 1979 and October 1983, 14 autografts were performed in 13 patients with CML (ten blast crisis, four accelerated phase). Results were disappointing: four patients died during aplasia; seven returned to chronic phase, but three died of hemorrhage, four relapsed, and three did not reverse. The main problem was the very low rate of successful engraftment. Both the collection of bone marrow after treatment with busulfan and a particular sensitivity of CFU-GM to cryoinjury were responsible for the infusion of very low doses of CFU-GM. However, we observed some promising results: In one patient in acute blast crisis, the Ph 1 chromosome disappeared, as well as the cytogenetic marker of transformation; in another patient with acute pure cytogenetic acceleration, the abnormal clone disappeared for 27 months; a third patient was maintained in a second chronic phase for 20 months. Thus we suggest that the results of autografting in chronic myeloid leukemia would be improved by infusing the largest possible dose of stem cells collected before or long after treatment by busulfan, and freezing them following a careful program.
To improve surgical results of potentially operable rectal cancer (T2, T3, T4, Mo), the European Organization for Research on Treatment of Cancer (EORTC) conducted a two-arm randomized clinical trial to evaluate the effect of administering radiotherapy before radical surgery. Four hundred ten patients were allocated to be treated either by surgery alone or by 34.5 Gy of radiotherapy (in 19 days overall) followed by surgery. The tolerance of the adjuvant radiation therapy was fairly good. The 5-year survival rate was 65% overall and showed no difference between both therapeutic regimens. Similarly, the metastases-free rate was the same in both groups. In contrast, the preoperative radiation therapy showed a marked effect on local control of the disease, the comparison of the time to local recurrence being highly significant between the two treatment groups (P = 0.001). The proportion of patients free of local recurrence at 5 years was 85% in the combined treatment versus 65% in the group of patients treated by surgery alone.
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A double-blind trial to evaluate the antiemetic effects of adrenocorticotropic hormone (ACTH) in patients treated with cisplatin was performed. Thirty-seven adults with advanced cancer who were treated with cisplatin were randomly assigned to receive either synthetic long-acting ACTH (1 mg IM given 24 hours, 12 hours, and immediately preceding the administration of cisplatin) or a placebo given under the same conditions. All of the patients received chlorpromazine (50 mg IM) 30 minutes before cisplatin infusion. Patients receiving ACTH and chlorpromazine had significantly fewer episodes of vomiting (p less than 0.01) and shorter periods of nausea (p less than 0.02) than patients receiving placebo and chlorpromazine. Patients receiving ACTH and chlorpromazine were significantly more comfortable than patients receiving placebo and chlorpromazine. No important side effects were observed. ACTH may be an effective agent in preventing nausea and vomiting induced by cisplatin.
Osteopetrosis was induced in lethally irradiated normal rats by cell infusion prepared from spleens of the osteopetrotic (op) mutant littermates. However, similar attempts to induce osteopetrosis in another strain of osteopetrotic rats, namely the "toothless" (tl) rats, were unsuccessful. This discrepancy between op and tl rats with regard to the transmission of the disease to normal littermates, parallels the response of the respective mutants to treatment by normal bone marrow cells: the op rat is cured, not the tl rat. The present findings confirm the cellular origin of osteopetrosis in the op rat, whereas the origin of the defect in the tl mutant remains obscure.
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It is often necessary to reconstruct the bony defects that result from the resection of bone tumours with cortico-cancellous bone grafts. In addition, post-operative radiotherapy is often given to prevent tumour recurrence. We have attempted to show that the rational use of radiotherapy after the insertion of a bone graft does not endanger the rehabilitation of the graft. We do not know of any clinical publication related to this subject. We are thus reporting eight cases of irradiated cortico-cancellous grafts which were given precise doses of irradiation and we have been able to draw the following conclusions: a) when doses of 4 000 Rads or less were given, there was no delay in graft consolidation, b) when doses of more than 6 000 Rads were used, graft rehabilitation was jeopardised. c) it is possible that 6 000 Rads given over a limited area will not endanger the graft but we have only one example. We have reviewed the literature concerning experimental studies on the progress of bone grafts, the effect of irradiation on normal bone and on cortico-cancellous grafts. The experiments have been performed must frequently in vitro and in vivo on small laboratory animals. The conclusions drawn from these studies agree with our clinical impressions: following irradiation the osteogenic activity of a cortico-cancellous graft and of normal bone are the same. They are subjected to the same risks and the same complications.
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Between 1958 and 1973, 131 patients with carcinoma of the posterior pharyngeal wall were treated at the Institute Gustave-Roussy (127 men, 4 women, mean age 61 years). Nine had a tumor of the oropharynx, 37 of the hypopharynx and 85 of the oro-hypopharynx. Nine had T1 tumors, 19 T2 and 103 T3 (TNM-UICC 1975). 122 patients were treated by irradiation: 83% were T3 lesions, 81% were more than 4 cm in diameter, 80% were ulcerated and 42% were staged N3. The survival rate of these patients was very poor (3% at five years). The nine patients who had been treated by surgical excision had a better survival rate and three are alive at 5 years, but the initial prognostic factors were better in this group. It seems that the indications for surgery should be widened to increase the survival rate in these poor prognosis cancers.
Fifty patients with non-Hodgkin's lymphoma were treated with total abdominal irradiation to a dose of 3000 rad by anterior and posterior fields treated the same day. Fourteen patients developed complications, four with intestinal obstruction due to stenosis and three with total or partial intestinal irradiation damage. These bowel complications were only seen in patients who had previously had a laparotomy. The ways of preventing these complications are discussed. We have studied renal function by means of clinical examination, biologic and radiologic investigations, and scanning in 75 patients who have received splenic irradiation to a dose of 4000 rad. With a mean follow-up time of 36 months, this study has shown that there was no significant change in blood pressure or in biochemical measurements. However, at the 17th month, nephrotomograms demonstrated cortical atrophy of the upper role of the kidney; at the 8th month, low uptake in the irradiated area was seen on 197Hg neohydrine scanning and a decrease in renal plasma flow in the left kidney during dynamic studies with Hippuran.