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Biomedical subjects

J Alba

Publications and source records attributed to J Alba.

32 records · Page 2Linked to original sources

[Fractionated bone marrow transplantation in a case of severe combined immunodeficiency].

Authors present a male infant affected by severe combined immunodeficiency with a high percentage of circulating B lymphocytes. Pathogenetic mechanism underlying our patient's disease seems to be an intrinsic defect in the lymphoid stem cell. As no histocompatible donor could be found, an hemiallogeneic bone marrow transplantation of maternal origin, fractionated with soybean agglutinin, was performed. The patient died 27 days after transplantation. At this time the percentage of T cells in his peripheral blood rose to near normal values, and the percentage of B cells normalized, karyotype analysis demonstrated circulating cells of female origin. Necropsy showed a spleen with lymphoid follicles and germinal centres but no signs of graft-versus-host could be found. Fractionated bone marrow could be of paramount importance in the treatment of these patients, since it could circumvent graft-versus-host disease.

Adult↗

[Transformation of a rib plasmocytoma into an amyloid tumor (author's transl)].

The appearance of amyloidosis during the course of multiple myeloma is a well known fact and has an overall incidence of 6 to 15%. However, the total transformation of a plasmocytoma into a voluminous amyloid tumor is a very rare event. A female patient was diagnosed of lambda light chain disease after developing a conspicuous rib plasmocytoma over the same region where a pathological fracture had appeared three years before. She was treated with discontinuous courses of melphalan and methyl-prednisolone, and developed a reversible nephrotic syndrome and a pathological fracture of the right clavicle. At necropsy there was generalized amyloidosis and complete substitution of the rib plasmocytoma by amyloid substance, with another important accumulation of amyloid in the region of the clavicular fracture. The present concepts on amyloidogenesis in multiple myeloma are reviewed, and the peculiarities of the present case together with the possible role of initiating factors and the effects of therapy are discussed. The case herein reported appears to represent a human model of focal amyloidogenesis in myeloma.

Aged↗

[Treatment of peritoneal hydatidosis with mebendazole. Preliminary study (author's transl)].

Authors present three cases of peritoneal hydatidosis, two of which are due to spontaneous rupture of hepatic hydatid cysts and the other due to operative rupture of ovarian hydatid cyst. A dose of 1 g./day of mebendazole was administered for 11 months with 30 day break periods every three months. Mebendazole has proved effective in the treatment of secondary echinococcosis. In authors judgement this may be an effective treatment for peritoneal spreading if mebendazole is used at an early stage since they have verified the absence of dissemination in one of the cases and an inactive dissemination in other one. If treatment is applied later, regression is achieved too, but more slowly. Immunological and ecographical results are reported in detail as well as surgical and pathologic findings. A cautious interpretation of these preliminary results is advised since data on long-term results are lacking.

Benzimidazoles↗

Neutrophil chemotactic defect and hypogammaglobulinemia.

A 15-months-old boy developed agranulocytosis after administration of Chloramphenicol and Aminopyrine. In spite of total hematological recovery, the patient's immunological study disclosed a persistent neutrophil chemotactic defect and hypogammaglobulinemia. Other studies of specific and non-specific immunity were normal. Neutrophil adherence, random and random stimulated mobility were always within the normal range. The presence of chemotactic inhibitors was discarded. In vitro incubation of his neutrophils with Cytochalasin B at 0.1 micrograms/ml final concentration, reversed the chemotactic abnormality suggesting a possible cell membrane defect.

Agammaglobulinemia↗

[Pseudomelanoma].

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Aged↗

Cellular immunodeficiency with immunoglobulins: treatment with a thymus implant in millipore diffusion chambers.

A 14 month old male affected by cellular immunodeficiency with immunoglobulins underwent implantation of a thymus enclosed in Millipore diffusion chambers. Five days after the implant the percentage of T lymphocytes forming spontaneous rosettes reached normal levels. One month later responsiveness to PHA was demonstrated in the patient's lymphocytes and IgG and IgM serum levels showed a marked increase. Positive skin tests were elicited 6 month's post-implant. An inguinal lymph node biopsy showed developing primary follicles and germinal centers. The patient's condition improved significantly. One year after the implant the patient died from irreversible neurological deterioration. It is suggested that partial immunological reconstitution was achieved by thymic humoral factors.

Antibodies↗