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Biomedical subjects

E Gilbert

Publications and source records attributed to E Gilbert.

At least 37 records · Page 2Linked to original sources
PubMed1987

Fatal cardiac toxicity in bone marrow transplant patients receiving cytosine arabinoside, cyclophosphamide, and total body irradiation.

Three patients developed fatal cardiac toxicity from the combination of cytosine arabinoside, cyclophosphamide, and total body irradiation while undergoing preparation for a bone marrow transplant. The pattern of the toxicity was unique for this combination of ablative chemotherapy. All three patients had autopsies demonstrating characteristic myocardial and pericardial toxicity. The cardiotoxic effects of this combination may be averted by lowering the dose of the cyclophosphamide.

Adolescent↗
PubMed1987

Genitourinary abnormalities associated with the Smith-Lemli-Opitz syndrome.

The Smith-Lemli-Opitz syndrome is characterized by mental retardation, hypotonia, facial dysmorphism and abnormalities of the limbs, genitalia and kidneys. Since the latter 2 features have not been emphasized in the urological literature, the experience from the institution at which the syndrome was first described is reviewed and an illustrative case is reported. Upper urinary tract abnormalities were noted in 57 per cent and genital abnormalities in 71 per cent of the children evaluated.

Abnormalities, Multiple↗
PubMed1986

Aniridia and Wilms' tumor in a child constitutionally mosaic for 11p-;12q+: a new chromosomal change also present in Wilms' tumor cells of the blastema type.

A child with congenital aniridia was assessed closely, by repeated abdominal ultrasound examinations, beginning at birth. The Wilms' tumor subsequently discovered and removed was analyzed karyotypically and found to have some cells with a terminal deletion of chromosome 11; in other cells this deletion was associated with a duplication in the long arm of chromosome 12. These findings were identical to those observed in the patient's peripheral blood mononuclear cells. This case further substantiates the association between changes in chromosome 11 and Wilms' tumor and demonstrates how chromosomal abnormalities in early infancy may lead to the development of Wilms' tumor.

Cells↗
PubMed1984

Metastatic neuroblastoma arising in an ovarian teratoma with long-term survival. Case report and review of the literature.

A case of neuroblastoma arising in an immature teratoma of the ovary in a 22-year-old woman is reported. Differentiation was grade 3 in the primary and metastases. Metastases to retroperitoneal, mediastinal, and supraclavicular lymph nodes and to bone were diagnosed 2 years after presentation of the primary tumor. Electron microscopic study demonstrated dense-core granules of the neurosecretory elements. Treatment with combination chemotherapy followed by radiotherapy resulted in complete remission which continues to the time of this report, more than 4 years after diagnosis of the primary tumor. Prolonged survival of metastasizing grade 3 immature teratoma is distinctly uncommon. The literature pertaining to this unusual tumor and to neuroblastoma of adults is reviewed.

Adult↗
PubMed1984

Testicular histology in triad syndrome.

A twenty-year-old patient with the triad syndrome had a Sertoli-cell-only histologic appearance in his intra-abdominal testes. Testicular histology was then reviewed in 6 other boys with the triad syndrome, and no spermatogonia were seen. While fertility is doubtful in patients with this syndrome, they additionally may not be at risk for germ cell testicular tumors.

Adult↗
PubMed1984

Adverse histopathologic effects of chemotherapeutic agents in childhood leukemia and lymphoma.

Pathologic material from 84 children with leukemia or lymphoma who died and were autopsied at the University of Wisconsin Hospital between 1967 and 1980 was reviewed to assess the adverse tissue changes due to the chemotherapy administered. In each case the histopathologic findings were correlated with the chemotherapy given. Individual drug dosage is administered (per m2) correlated with the adverse tissue findings at autopsy. Data presented suggest that the drug dose administered should be correlated not only with tumor response but also with tissue effects (some of which produce no noticeable, immediate, clinically apparent changes) to decrease the likelihood of producing irreversible tissue changes.

Adolescent↗
PubMed1983

Urologic implications of the VATER association.

Among 23 children with the VATER association 21 had significant genitourinary involvement. The extent of these genitourinary anomalies is not generally appreciated. The principal renal anomalies were renal agenesis (7 cases), ureteropelvic junction obstruction (5 cases), crossed fused ectopia (5 cases) and severe reflux (9 cases). Since corrective urologic surgery is commonly beneficial for children with the VATER association, an early and appropriate urologic evaluation is indicated.

Abnormalities, Multiple↗
PubMed1982

Hypomelanosis of Ito (incontinentia pigmenti achromians)--a clinicopathologic study: macrocephaly and gray matter heterotopias.

We studied a boy with macrocephaly, hypotonia, pigmentary retinopathy, unilateral whorled hypopigmented skin lesions, and seizures. Skin biopsy confirmed the clinical diagnosis of hypomelanosis of Ito. Postmortem examination at age 22 months revealed a severe neuronal migrational defect that altered the cerebral cortex architecture of white matter. There were many gray matter heterotopias characterized by altered neurons and giant cells. Electronmicroscopy revealed the astrocytic nature of the giant cells. Embryologic migration of both melanoblasts from neural crest and cortical neurons occurs in the second trimester, suggesting a common mechanism for the developmental pathology of skin and brain.

Brain↗
PubMed1981

Erythroblastic islands in erythroleukemia.

Erythroblastic islands are distinct anatomical units allowing direct transfer of ferritin molecule into developing erythroblasts. A patient with Fanconi's anemia who developed acute erythroleukemia and hypotransferrinemia exhibited numerous erythroblastic islands in the bone marrow. This unusual finding in our patient is thought to be in response to an increased demand for iron caused by neoplastic erythropoietic proliferation in the absence of an adequate amount of transferrin.

Adolescent↗
PubMed1979

Fatal lymphoma after transplantation of cultured thymus in children with combined immunodeficiency disease.

A fatal, widespread, polyclonal, B-cell immunoblastic lymphoproliferative disorder developed in three children with combined immunodeficiency shortly after intra-abdominal transplantation of cultured thymus epithelium for immunoreconstitution. All three had surface immunoglobulin-bearing cells (15 to 20 per cent) in the peripheral blood before transplantation and polyclonally elevated immunoglobulins afterward. Abnormal immunoregulation was demonstrated by a lack of concanavalin A-induced suppressor-cell activity in mixed leukocyte culture in all three patients before transplantation and in two afterward. We suggest that the transplant acted as a promoter through immunostimulation or production of promoter factors, and that excessive polyclonal B-cell proliferation resulted because of inadequate immunoregulatory mechanisms. Although this complication occurred in only three of 30 patients with various forms of immunodeficiency treated with cultured thymus, these cases illustrate a potential problem in immunoreconstitution of combined immunodeficiency disorders.

B-Lymphocytes↗