Biomedical subjects
Publications and source records attributed to J Sierra.
We report a case study spanning three generations of familial spastic paraplegia, distal amyotrophy and poikiloderma. This study is the first description of an association between these three disorders. The gait disorder, the sensory and motor involvement and the skin disorder coincide in all the affected members, suggesting autosomal dominant inheritance with complete penetrance.
An unusual case of Castleman's disease (CD) restricted to the left cervical area is reported. CD is rarely confined to the cervical area; when it occurs, it is usually of the hyaline-vascular subtype and small in size. This case was atypical due to its histology (plasma-cell variant), big size (15 x 11 x 6 cm) and the absence of general symptoms. In spite of surgical excision, the disease recurred 11 months later. However, subsequent surgery followed by chemotherapy resulted in a long-lasting control of the disease.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
In order to clarify the role of autologous bone marrow transplantation in adult lymphoblastic lymphoma (LBL) patients we have conducted a retrospective multi-institutional Spanish survey. Twenty-two adult patients, 20 males, two females, age 14-52 years (median 25.5) with LBL were treated with high-dose chemoradiotherapy and autologous bone marrow support. Fourteen cases were transplanted in first complete remission (CR1) and eight with other chemosensitive status (four later CR, two partial remissions and two sensitive relapses). From the 14 cases transplanted in CR1, four had previous bone marrow involvement and one meningeal infiltration; eight cases were Ann-Arbor stage IV and fulfilled accepted high-risk criteria for relapse. The conditioning regimen consisted of cyclophosphamide (60 mg/kg x 2) and total body irradiation (9-12 Gy) in 16 cases and high dose chemotherapy in six. The procedure-related mortality was 9% (7% in CR1 patients). The actuarial 2-year overall survival for CR1 patients was 85% at a median follow-up of 19 months. Disease-free survival (DFS) was 77%. In patients with less favourable disease status the 2-year overall and DFS were 73% and 50% respectively. In this study the DFS in CR1 patients was not influenced by bone marrow involvement or high-risk criteria predictive for relapse. These results support the effectiveness of this procedure, mainly for patients in CR1.
In the past 4 years we have treated four patients with a total of 19 fetal liver infusions (FLI). Two cases of refractory anemia with excess blasts in transformation (RAEB-t) were conditioned with cyclophosphamide and total body irradiation (1400 cGy) and were treated with FLI. In spite of such intensive conditioning, one patient recovered autologous hemopoiesis 3 weeks later, remaining in remission 4 years after this procedure. The second patient died with aplastic marrow on day 154, and the third suffered from severe aplastic anemia refractory to several types of conventional treatment. After FLI and without previous conditioning therapy a partial fetal engraftment was documented. This was transient and followed by autologous hemopoietic recovery and cure of the disease. The fourth patient had bone marrow failure in the setting of a severe pneumonia following autologous bone marrow transplantation. Ten days after FLI the hematological parameters dramatically improved and the pneumonia resolved. Autologous reconstitution of hemopoiesis was demonstrated. These experiences suggest that FLI might stimulate autologous hemopoiesis. This therapeutic approach may be useful to treat bone marrow failure when there is no response to first-line therapy. In hematologic malignancies with an indication for stem cell transplantation, other sources such as allogeneic or autologous bone marrow seem preferable to fetal liver cells.
By means of a mail questionnaire, information on a series of 56 pregnancies i in 48 women diagnosed with leukemia or lymphoma was collected from ten hospitals. Seven patients conceived while receiving treatment for their neoplasms; in 22 patients, the hematologic disease was diagnosed during pregnancy, and the remaining 27 patients became pregnant after completion of the antineoplastic treatment. When a comparison was made of the evolution of these pregnancies to that of pregnancies in a healthy population, no increase in the incidence of complications was observed: 64% of the pregnancies went to term, 9% resulted in spontaneous abortion, and 5% resulted in premature births. The observed incidence of one major malformation in 56 pregnancies did not differ from the frequency of malformations in the offspring of healthy individuals. There were no fetal losses in six pregnancies in which conception occurred during the first year after chemotherapy. In spite of the inherent limitations derived from the design of this type of study and the type of subject analyzed, the data here support the hypothesis that the cytostatic treatment of hematologic malignancies, if deemed necessary, should not be postponed because of pregnancy. Moreover, the authors agree with advice recommending that no antifolic or alkylating agents be used for prolonged periods and that radiotherapy be avoided, especially to those fields involving the pelvic area.
Between January 1977 and January 1989, 465 pericardial bioprostheses were implanted in 424 patients. The mean age of patients was 59.1 years (range 16-81 y.) At the time of surgery, 68% of the patients suffered from chronic atrial fibrillation. Mitral valve replacement was performed in 167 patients, aortic valve replacement in 216, multiple replacement in 40 (36 mitral and aortic, 3 mitral and tricuspid, and 1 mitral, aortic and tricuspid), and 1 pulmonary valve replacement. The different types of pericardial valve used were: Ionescu-Shiley 408, Mitral Medical 23, Bioflo 30, and Hancock 4. Hospital mortality was 10.1% with an attrition rate of 1.8 episodes per 100 patients/year. The 12-year actuarial survival rate was 65.1%. No patient underwent long-term anticoagulant treatment. The first 144 patients undergoing mitral and multiple valve replacements received temporary anticoagulation for the first 8 weeks after surgery. There was no valve thrombosis observed. Altogether 19 thromboembolic events (6 early and 13 late) were clinically documented. One patient died after an embolic event. The linearized rates of thromboembolism were 1.64 episodes per 100 patients/year for mitral and multiple valve replacements and 0.33 episodes per 100 patients/year for aortic valve replacement, with an overall rate of 1.0 episodes per 100 patients/year. Excluding early thromboembolism, the linearized rate was 1.02 episodes per 100 patients/year overall. The actuarial freedom from embolism was 92.4% overall, 88.2% for the mitral and multiple valve replacement group, and 97.6% for the aortic valve replacement group at a maximum follow-up of 12 years.(ABSTRACT TRUNCATED AT 250 WORDS)
The Labcor-Santiago pericardial valve has been designed to minimize mechanical stress and to avoid abrasion wear, at the same time preserving the concept of mounting the pericardium outside the posts for full orifice opening. The new design has preserved the traditional superior hemodynamic performance of pericardial valves, avoiding abrasion by careful padding of the stents and introducing a new concept of cusp attachment in order to increase mechanical durability. Since June 1990, 40 patients received 46 Labcor-Santiago valves: 29 in the aortic position and 17 in the mitral position. Mean age was 65.6 years. Eighty percent of the patients were women. New York Heart Association Functional Class III-IV was present in 76% of patients. Hospital mortality has been 12.5%, with 31% undergoing concomitant procedures. No anticoagulation was administered and there has been one thromboembolic event. Echo-Doppler assessment yielded the following in the aortic position: effective orifice area for 19-mm size = 1 cm2; for 21-mm size = 1.2 cm2. Mean systolic gradient for 19-mm size = 14.7 mmHg; for 21-mm size = 11.2 mmHg. In the mitral position: effective orifice area for 27-mm size = 2 cm2; for 29-mm size = 2.3 cm2. Mean diastolic gradients for 27-mm size = 4.6 mmHg; for 29-mm size = 3.3 mmHg. The low-profile flexible stent, the streamlined shape without outside prosthetic material, and the anatomical sewing rim allow a comfortable implantation of the Labcor-Santiago valve, particularly in the small aortic annulus. It is hoped that this new design will contribute to an increase in in vivo mechanical durability.
We report the case of a female aged 67 years suffering a Waldenström's macroglobulinemia which began with arthritis at the knee and pleural effusion. In both locations the anatomopathological examination showed infiltration by typical lympho-plasmatic cells. Other possible etiologies such as amyloid, infection or arthritis produced by microcrystals were ruled out. The relevant finding in this case was the presence of infiltrative reaction at the synovial tissue which has been rarely reported in the literature. We review the incidence of articular manifestations in cases of Waldenström's macroglobulinemia and the possible pathogenetic mechanisms involved.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The preliminary results of the LAL-86 protocol applied to 43 patients diagnosed of acute lymphoblastic leukaemia (ALL) or lymphoblastic lymphoma (LL) between May 1986 and April 1989 are reported. Induction treatment consisted of one or two courses of vincristine, daunorubicin, prednisone, cytosine arabinoside and 6-thioguanine combination therapy. This phase was followed by consolidation treatment, in which VM-26, cyclophosphamide, BCNU and L-asparaginase were added to the former agents. Central nervous system prophylaxis was done with intrathecal methotrexate. Patients under 45 years of age with HLA identical sibs were subjected to allogeneic bone marrow transplantation (BMT) in the first complete remission (CR); when no HLA-identical sibs were available patients were randomised into autologous BMT or maintenance therapy. The remaining patients received maintenance chemotherapy. CR was achieved in 34 ALL patients (79%), 5 were refractory to treatment and 4 died during remission induction. Allogeneic BMT was carried out in 6 cases, autologous BMT in 3, and the remainders received chemotherapy. When performing this review, 7 patients had relapsed and the actuarial probability of 2-year duration of CR was 70%. Sixteen patients have died with a two-year disease-free survival probability of 60%. The preliminary results of the LAL-86 protocol are encouraging, but greater number of patients is needed, as well as a longer follow-up, to assess the effect of chemotherapy and compare these findings to the results of autologous or allogeneic BMT in the first RC.
Myelodysplastic syndromes (MDS) are disorders characterized by a profound impairment of proliferation and maturation of hematopoietic cells. The prognosis is poor owing to the occurrence of severe cytopenia or to the common leukemic transformation of these conditions. At present there is no available effective treatment for patients with MDS. A patient is reported with simple refractory anemia which responded to therapy with high doses of maturative factors (folic acid and vitamin B12). This therapy resulted in the disappearance of transfusion requirements and in the increase of peripheral blood cell counts. It is concluded that, in view of the lack of toxicity of the treatment with madurative factors, this therapy should be attempted in a sequential fashion in all patients with MSD.
The results of bone marrow autograft (BMAG) in 20 patients with acute leukemia (AL) consecutively treated from October 1985 and May 1988 are reported. The follow up was continued until November 1988. The mean age of the patients was 20 years (range 10-48) and their diagnoses were acute myeloblastic leukemia (AML) in 12 and acute lymphoblastic leukemia (ALL) in 8. The preparation for BMAG included cyclophosphamide and whole body radiation in all cases. The procedure was carried out in the first complete remission (CR-1) in 5 patients, in CR-2 in 11, in CR-3 in 2, and in CR-4 in another 2. Two patients died as a direct consequence of BMAG, 11 relapsed and 7 are alive and free from relapse. The likelihood of survival free from prolonged illness was calculated as 24% for the whole series, 60% for the cases of BMAG in CR-1 and 50% for the group of patients with AML. The results were poor in the cases of BMAG carried out for AL in an advanced stage. On the basis of these results the experience of other authors with this therapeutic modality is reviewed, and its current indications are discussed.
Explore the source record for details and available documents.
With the aim to attesting the ability of treated pericardial tissue to resist infection we analyzed the incidence of prosthetic valve endocarditis in 541 operative survivors with the Ionescu-Shiley (I-S) valve since January 1977. A total of 580 valves were available for the study, 21 (3.8%) sustained I-S endocarditis or 0.65% patients/annum. The mean age was 53.2 years; male/female ratio was 16/5. Native endocarditis was present in 20 cases (3.6%). There were 13 aortic endocarditis (2 in cases of multiple replacement) and eight mitral. The interval between insertion and endocarditis ranged from 2 weeks to 108 months (means 31); 8 cases were early I-S endocarditis (38%) and 13 late endocarditis (62%). The organisms isolated were: Staphylococcus (epidermidis, aureus) in 10 cases. Streptococcus (viridans, fecalis) in 7 cases, gram negative in three and no organism was cultured in 3 cases. The outcome was as follows: specific medical treatment in 4 cases (one early), valve re-replacement in 11 (four early) from whom eight survived, and 6 patients died with unspecific medical treatment. The total mortality rate 9/21 (43%), in early endocarditis 5/8 (62.5%) and in late endocarditis 5/13 (38%). Operative and necropsy findings in 12/21 showed always periprosthetic abscess but no leaflet perforations. The rate of I-S valve endocarditis compares favourably with other valve substitutes. Early re-replacement combined with adequate antibiotic treatment is mandatory according to our experience.
The incidence and duration of hypertriglyceridemia (HTG) associated with allogeneic HLA-identical bone marrow transplantation (BMT) were investigated in 38 patients with normal triglyceride levels (TG) before BMT and without any evidence of prior liver disorder. Twenty-two (58%) patients developed HTG. The median of TG peak values was 350 mg/dl (range 215-908) and the median actuarial time for HTG appearance was 7 weeks (range 1-12). Among several variables analysed (age, sex, hyperglycemia, total parenteral nutrition, treatment with methylprednisolone, estrogens or cyclosporine A) only cyclosporine A administration was significantly associated with HTG development.