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Biomedical subjects

J R Cabrera

Publications and source records attributed to J R Cabrera.

8 recordsLinked to original sources
PubMed1999

Idiopathic dilated cardiomyopathy. Elastic parallel element dysfunction as a physiopathological hypothesis for ventricular failure.

BACKGROUND: Idiopathic dilated cardiomyopathy is a disease of unknown etiology, although the viral-immunologic pathogenesis has recently emerged as an important hypothesis. Its distinctive anatomopathologic features are: macroscopically, a great ventricular dilation with little hypertrophy, and microscopically marked diffuse interstitial fibrosis not observed in other pathologic entities with dilation. Hemodynamically, its main characteristic is a progressive loss of the systolic function, although the diastolic function is also impaired. To date it is accepted that in dilated states ventricular remodeling occurs due to sliding of fiber with a maximal sarcomere distention; it is also assumed that the ventricular dysfunction is due to a primary deficit in contractility caused by the injury and loss of myocites. HYPOTHESIS: The aggressive agent mainly attacks the interstitial tissue, thus damaging the elastic parallel element structures. This results in a loss of absorbing power during diastole, starting a progressive dilation which results in maximum sarcomere distention, and compromises the ventricular function. The organ response is to create a new parallel element, which results in an increased fibrosis which also compromises this function.

Cardiomyopathy, Dilated↗
PubMed1996

Donor leukocyte infusions for treatment of relapsed acute myeloid leukemia after allogeneic bone marrow transplantation.

A 24-year-old man with acute myelomonocytic leukemia (AML-M4) who relapsed 6 months after an allogeneic BMT was treated with chemotherapy followed by donor leukocyte infusions (4.19 x 10(8) mononuclear cells/kg). The patient developed grade II acute GVHD that responded to therapy with CsA and prednisone. Chimerism was assessed by PCR amplification of the MCT 118 hypervariable region. Fourteen months after donor leukocyte infusions the patient remains in complete remission, without any morphologic and cytogenetic evidence of leukemia, and with a complete donor chimerism. This case shows that donor leukocyte infusions are an effective therapy for some acute myeloid leukemia patients who relapse after allogeneic BMT.

Adult↗
PubMed1995

[Treatment with fludarabine of chronic refractory lymphoid leukemia].

PURPOSE: New antimetabolic drugs, purine-analogous, have been introduced in the treatment of advanced or refractory cases of chronic lymphocytic leukaemia (CLL), fludarabine (FLU) being one of such drugs. The results attained with FLU in 6 cases of refractory CLL are reported. PATIENTS AND METHODS: The efficacy and toxicity of FLU was evaluated in 6 patients (median age 63 years) between March 1993 and March 1994. Five patients were in stage III-IV and one in stage II of the Rai's system, and they were refractory to the usual treatment. The dosis used here was 30 mg/sq m/day, for 5 days every 4 weeks, up to a total of 6 therapeutic courses. The response was assessed after 3 and 6 courses. Anti-infectious prophylaxis was made with co-trimoxazole, monthly benzathine penicillin G and isoniazid in Mantoux-positive patients. The response criteria were those given by the NIC Working Group for CLL. RESULTS: The patients received 2 to 6 courses. None of them attained complete remission: 1 had nodular remission, 2 had partial remission, 2 responded partially although not reaching criteria for partial remission (1 died of pancytopenia), and 1 had disease progression and died after the second course. Fast and important reduction of the lymphocyte count as well as the CD4 lymphocytes was present in all cases. Bone-marrow infiltration decreased strikingly in 2 cases and the platelet count improved in 3 cases and worsened in 2 others. The following toxicity was recorded: 2 patients had nausea, 5 had neutropenia (below 500 x 10(9)/L in 3 instances) and 4 had thrombocytopenia (lower than 40 x 10(9)/L in one case). In 26 therapeutic courses there were 10 febrile episodes (1 for pneumonia, 1 for gastroenteritis and 8 without any septic foci); 1 patient developed pulmonary tuberculosis after completing the treatment and one patient died of posttransfusion graft versus host disease after splenectomy once she had completes six courses. CONCLUSION: This experience confirms the efficacy of FLU in the treatment of refractory B-CLL patients and is in agreement with previous reports as no response is initiated after the 3rd course. The lymphocyte count decreases quickly and strikingly. Depletion of CD4 lymphocytes along with neutropenia and hypogammaglobulinaemia make these patients highly sensitive to all types of infection, chiefly by opportunistic germs, so adequate anti-infectious prophylaxis is of great importance. The two patients with pre-treatment haemoglobin and platelet values lower than 10 g/dL and 40 x 10(9)/L, respectively, were the only ones in need of transfusion. No cumulative myelosuppression was appreciated.

Aged↗
PubMed1989

Gastric Castleman's disease with a lupus-like circulating anticoagulant.

We report the case of a 13-yr-old patient with retarded growth and a 2-yr background of asthenia, anorexia, and fever, whose laboratory data revealed anemia, thrombocytosis, an elevated erythrocyte sedimentation rate, ferropenia, hyperglobulinemia, hyperfibrinogenemia, and presence of a lupus-like circulating anticoagulant. Clinical studies revealed a tumor-like overgrowth in the gastric wall, and surgery confirmed its subserosal localization in the gastric fundus. After total removal of the mass, the systemic manifestations disappeared. The pathological study revealed the existence of the hyaline-vascular variety of Castleman's disease. Having reviewed the medical literature, we have not found a single unquestionable case of gastric Castleman's disease, although three other cases have been described as gastric pseudolymphoma which, when analyzed, could correspond to typical cases of Castleman's disease. Likewise, this is the second case associated with a circulating anticoagulant of lupoid characteristics. We conclude that Castleman's disease should be included in the differential diagnosis of gastric lesions of lymphoid nature and in the series of processes associated with lupus anticoagulant.

Adolescent↗
PubMed1981

Defective neutrophil mobility in the May-Hegglin anomaly.

A case of May-Hegglin anomaly is reported in which functional studies of PMN cells showed abnormalities consisting of impairment of chemotactic and chemokinetic responses, random mobility being otherwise normal. These abnormalities seem unrelated to microtubule system dysfunction or to abnormal cell deformability, since no defect was observed in the concanavalin A (Con A) surface receptors and no improvement of directional movement resulted when filters of larger pore size were used in the assays. Other possible mechanisms of the functional defect, such as abnormal membrane receptors to kinetic signals or metabolic abnormalities cannot be excluded. PMN function should be studied in additional cases in order to ascertain if these findings are a constant feature in the May-Hegglin anomaly, a syndrome in which undue susceptibility to infection has not been reported.

Adult↗