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Biomedical subjects

G Vawter

Publications and source records attributed to G Vawter.

At least 19 recordsLinked to original sources
PubMed1986

Secondary (AA) amyloidosis in cystic fibrosis. A report of three cases.

The authors report the pathologic features of three cases of amyloidosis associated with cystic fibrosis. Renal biopsy led to the diagnosis (case 1) or suspicion (case 2) of amyloidosis in patients who were 23 and 21 years old, respectively. The third patient died at age 22 years, and amyloidosis was not discovered until autopsy. Immunohistochemical staining and potassium-permanganate pretreatment of histologic sections in all three cases provided evidence that the amyloid seen in these patients is of the secondary (AA) type. Congo red staining in each case and electron microscopy in case 1 confirmed the initial diagnosis of amyloidosis. A markedly elevated serum amyloid A protein (160 micrograms/mL; normal less than 1 microgram/mL) in case 1 indicated the presence of large quantities of the precursor protein from which the AA fibrils of secondary amyloid are derived. The kidneys, spleen, and liver contained amyloid deposits in autopsy material from all three cases. Involvement of other organs by amyloid was variable. Review of autopsy material in Boston from 23 additional cystic fibrosis patients with long-term survival did not reveal any evidence of amyloidosis. It appears that secondary amyloidosis is emerging as a significant, although rare, complication of cystic fibrosis as greater numbers of these patients survive into adulthood.

Adult↗
PubMed1984

Mesothelioma following Wilms' tumor in childhood.

A high percentage of children with Wilms' tumor are cured with multimodal treatment. A small percentage of these children will develop second tumors, perhaps related to a genetic predisposition to neoplasia or possibly secondary to the treatment utilized for Wilms' tumor. Malignant mesothelioma has been associated with contact with asbestos but has also been reported after radiation exposure. Two patients are reported who developed malignant mesothelioma of the pleura after treatment for Wilms' tumor in childhood. Both received orthovoltage radiation; one patient also received triethylenemelamine (TEM), an alkylating agent closely related to nitrogen mustard, for 5 years. Factors in the development of second tumors are discussed.

Adult↗
PubMed1984

Improved survival in neuroblastoma using multimodality therapy.

One hundred and thirty-six patients with neuroblastoma have been treated at our center from 1970 to 1982, using various combinations of surgery, radiation therapy, and chemotherapy. Choice of therapy was individualized but depended primarily on age and stage. The overall disease-free survival was 60% (minimum follow-up of one year). Patients with stage I disease and younger patients with stage II disease usually received less intensive therapy and fared extremely well (100% survival). Patients with stage III disease and older patients with stage II disease also did extremely well (survival of 85% and 90%, respectively). These patients may have benefited from intensive treatment with all three modalities. Patients under one year of age with stage IV neuroblastoma were treated with surgery and multiagent chemotherapy, and 92% (11/12) survived free of disease. Patients over one year old with stage IV disease represented the only group for which therapy was unsuccessful (10% survival). With combination approaches and with more effective multiagent chemotherapeutic regimens, a real impact on the survival of older stage II patients, stage III patients, and younger stage IV patients appears to have been made. However, older stage IV patients are rarely cured with conventional therapy, and better approaches will be needed for this group.

Adolescent↗
PubMed1984

Lung structure in asphyxiating thoracic dystrophy.

It has been assumed that asphyxiating thoracic dystrophy (ATD) is always associated with abnormal lung development due to the small thoracic cage. Applying precise quantitative morphometric techniques to the injected and inflated lung from an infant with ATD who died at 4 months of age, normal lung growth was demonstrated. The number of bronchial generations and preacinar arteries was normal, indicating normal development in the first 16 weeks of intrauterine life. Alveolar number and acinar complexity suggested that alveolar growth was normal at birth and had continued with only minor impairment after birth. Arterial changes compatible with hypoxic pulmonary hypertension were also present.

Bronchi↗
PubMed1983

Breast carcinoma after cancer therapy in childhood.

Among 910 survivors of childhood cancer, four developed infiltrating carcinoma of the breast and another had noninfiltrating breast tumor. Expected frequency was 0.3 cases of breast cancer in the series. The affected women developed breast carcinoma at ages 20, 25 and 38 years, and the men at ages 38 and 39 years, respectively. Each patient had received orthovoltage chest irradiation for treatment of Wilms' tumor or bone sarcoma between seven and 34 years previously, and estimated radiation dose to the breast exceeded 300 rad in each instance. Four patients also received diverse forms of chemotherapy. Survivors of childhood cancer have increased risk of developing breast cancer and should undergo periodic screening, particularly after breast tissue had been irradiated. Individualized radiotherapy planning can help exclude the breasts from treatment fields for some thoracic neoplasms.

Adult↗
PubMed1981

Histiocytosis-X.

Twelve of 17 patients with histiocytosis-X were immunologically abnormal, as shown by the presence of circulating lymphocytes spontaneously cytotoxic to cultured human fibroblasts or of antibody to autologous erythrocytes. The patients also had a notable lack of histamine H2 surface receptors on their T lymphocytes, suggesting a suppressor-cell deficiency. The lymphocyte abnormalities were reversed in vitro after incubation in a crude extract of calf thymus gland, and therefore all 17 patients were treated with daily intramuscular injections of this extract. With this therapy, 10 patients entered complete remission -- a response at least as good as that observed in historical controls treated with chemotherapy. A positive clinical response was associated with an increase in the number of T-cell histamine H2 receptors to normal levels and with correction of the other immunologic abnormalities. The results of this preliminary study justify a larger prospective clinical trial of thymic extract and further investigation of the immunoregulatory mechanisms in histiocytosis-X.

Cell Membrane↗
PubMed1981

High-dose methotrexate in osteogenic sarcoma adjuvant chemotherapy and limb salvage results.

This communication provides a description and update of the three adjuvant regimens utilized at the Sidney Farber Cancer Institute. The disease-free survivals are: Study I--42% at 4 years; Study II--58% at 3 years; and Study III--currently 78%. In the limb salvage experience, among the evaluable patients, 6 of 12 treated with preoperative chemotherapy are disease free (median 32 months) and 12 of 14 (86%) treated without preoperative chemotherapy are disease free (median 13 months).

Amputation, Surgical↗
PubMed1980

Early pulmonary vascular changes in congenital heart disease studied in biopsy tissue.

Patients with congenital heart defects who had or were at risk for developing pulmonary artery hypertension underwent lung biopsy at the time of intracardiac repair. In 95 consecutive patients with either ventricular septal defect, d-transposition of the great arteries, or a defect of the atrioventricular canal, the pulmonary arteries were evaluated microscopically by quantitative morphologic techniques, and the findings were correlated with hemodynamic data obtained at a recent preoperative cardiac catheterization. Three grades of severity of early pulmonary vascular changes were identified, which correlated with hemodynamic evidence of progressive functional impairment. Grade A denotes cases with abnormal extension of muscle into peripheral arteries only; pulmonary blood flow is increased but pulmonary artery pressure is normal. In grade B an increased medial wall thickness of the normally muscular arteries is also present, and in these cases pulmonary artery pressure is also increased. Grade C denotes cases in which in addition to the findings in grade B disease there is a reduction in the number of small peripheral arteries; in these cases pulmonary vascular resistance is increased. Follow-up postoperative hemodynamic evaluation will reveal the significance of these changes in reflecting irreversible functional impairment of the pulmonary vascular bed.

Adolescent↗
PubMed1979

Increased muscularity of the pylmonary circulation in victims of sudden infant death syndrome.

It has recently become apparent that some victims of sudden infant death syndrome (SIDS) are chronically hypoxemic before death. In this study, the structural alterations in the pulmonary circulation of 15 victims of SIDS were examined using precise quantitative morphometric techniques applied to the injected and inflated lung. Increased muscularity of the pulmonary circulation has been demonstrated, as evidenced by the extension of muscle into arteries not usually muscularized during the first year of life, and by an increased thickness of the medial wall in some patients. This confirms the findings of Naeye and further justifies the shift of investigative efforts from the events immediately surrounding death to a search for chronic abnormalities.

Humans↗
PubMed1978

A family with acute leukemia, hypoplastic anemia and cerebellar ataxia: association with bone marrow C-monosomy.

The eldest brother in a sibship of five children died of acute myelogenous leukemia at 10 years of age. The second and third eldest brothers died of hypoplastic anemia at ages five and nine years, respectively. A surviving 6 year old brother, the proband of the study, has abnormalities that suggest a preleukemic state: mild pancytopenia, platelet dysfunction, immunodeficiency, and bone marrow hypoplasia with approximately 18 per cent blast forms. His 17 year old sister has a mild normochromic normocytic anemia. Cytogenetic studies revealed C-group monosomy in the bone marrows of the proband and the third brother (45, XY, -C); band studies demonstrated that a No. 8 chromosome was missing in the proband (45, XY, -8). At least four of the siblings and their father had cerebellar ataxia, and evidence of a small cerebellum at autopsy examination or by computerized axial tomography. The disorder in this family has major features of two autosomal recessive preleukemic diseases, ataxia-telangiectasia and Fanconi's anemia. However, these and other inherited conditions were excluded by clinical or laboratory criteria, and no environmental causes of the familial disorder were found. The constellation of abnormalities in the family may constitute a new genetic syndrome.

Acute Disease↗
PubMed1977

The relationship between nephroblastoma and neurofibromatosis (Von Recklinghausen's disease).

Three cases with co-existent Wilm's tumor and Von Recklinghausen's Disease from a series of 342 nephroblastomas are discussed as to the significance of this association. Biological arguments favor a positive link because of the multiplicity of associative lesions which parallel each other, the co-existence of which suggests a common genetic bond. An embryologic association is described whereby early mutational events, i.e. neural induction of nephrogenesis, may link these two entities in utero. Further interconnection is stated statistically, in that nephroblastoma patients in this series had a 29 fold higher incidence of multiple neurofibromatosis than predicted for in the general population. The co-existent diseases in the three cases observed are approximately 33-50% more than can be accounted for by chance in the United States population. Malignant transformation in neurofibromatosis is discussed because of the therapeutic implications presented by two of the three Wilms' tumor patients studied.

Child, Preschool↗
PubMed1977

Hematopathology and Pathogenesis of the X-linked recessive lymphoproliferative syndrome.

Subtle immunodeficiency to infectious agents including measles virus and ten Epstein-Barr virus (EBV) has been described in the X-linked recessive lymphoproliferative syndrome. This syndrome has affected six male cousins and possibly another boy. Three brothers died of an infectious mononucleosis syndrome, in a maternal cousin agammaglobulinemia developed three years after infectious mononucleosis, and two half-brothers of the Duncan kindred died of lymphoma of the brain and intestinal tract, respectively. In three of the boys, unusual measles viral infections had developed. Paramyxovirus-like particles suggestive of measles virus were seen at necropsy in the atrophic lymphoid tissue of two boys. Also, numerous plasma cells were seen in the brains, visceral organs and the thymus glands, and thymic-dependent lymphocytes were sparse in lymph nodes and spleen. The abnormal lymphopoiesis in the syndrome probably results from a subtle immunodeficiency, and concurrent measles and EB virus infections.

Agammaglobulinemia↗
PubMed1977

Immunological disorders and malignancies in five young brothers.

Five brothers of from 6 to 18 years of age experienced immunological or neoplastic disorders during an 8-year interval. 2 boys succumbed to glioblastoma multiforme, another to metastatic carcinoma, and the 2 surviving brothers had a histiocytic lymphoma and idiopathic thrombocytopenia purpura, respectively. The mother of the boys was healthy, but her twin sister died in utero of birth defects. We suggest that an intrinsic cellular defect inherited from their mother rendered the boys vulnerable to oncogenesis.

Adolescent↗