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Biomedical subjects

D Hollister

Publications and source records attributed to D Hollister.

At least 19 recordsLinked to original sources

Familial aortic dissecting aneurysm.

A family is described in which nine members over two generations had an aortic dissecting aneurysm or aortic or arterial dilation at a young age. The family has been followed up since 1977 after the death of a second teenager from a kindred of 11. None of the patients had the Marfan syndrome or a history of systemic hypertension. Three members died of ruptured aortic dissecting aneurysm and acute hemopericardium at 14, 18 and 24 years of age, respectively; a fourth member died suddenly at age 48 years, a few years after aortic repair for aneurysmal dilation. One member underwent surgical repair of an ascending aortic dissecting aneurysm at age 18 years and is still alive. Four members are currently under close medical observation for aortic or arterial dilation. Histologic examination of the aortic wall at autopsy or surgery in three patients revealed a loss of elastic fibers, deposition of mucopolysaccharide-like material in the media and cystic medial changes. Types I and III collagen from cultured fibroblasts appeared normal on gel electrophoresis. Results of indirect immunofluorescent studies of the elastin-associated microfibrillar fiber array in skin and fibroblast culture from multiple family members were also normal. This dramatic familial cluster of aortic dissecting aneurysm and aortic or arterial dilation suggests a genetically determined disease of autosomal dominant inheritance although the basic defect remains unknown.

Adolescent↗

Kniest dysplasia is characterized by an apparent abnormal processing of the C-propeptide of type II cartilage collagen resulting in imperfect fibril assembly.

Epiphyseal and growth plate cartilages from four cases of Kniest dysplasia have been studied. In each case collagen fibril organization appeared abnormal by electron microscopy compared with age-matched normal cartilages: fibrils were much thinner, of irregular shape and did not exhibit the characteristic banding pattern. This was associated with the absence (compared with normal cartilage) of the C-propeptide of type II collagen (chondrocalcin) from the extracellular matrix of epiphyseal cartilages, although it was detected (as in normal cartilages) in the lower hypertrophic zone of the growth plate in association with calcifying cartilage. The C-propeptide was abnormally concentrated in intracellular vacuolar sites in Kniest cartilages and its total content was reduced in all cases but not in all cartilages. Moreover, it was not a part of the procollagen molecule. In contrast, type II collagen alpha-chain size was normal, indicating the formation of a triple helix. Also type II collagen content was normal and it was present in extracellular sites and only occasionally detected intracellularly. These observations suggest that the defect in Kniest dysplasia may result from the secretion of type II procollagen lacking the C-propeptide and abnormal fibril formation, and that the C-propeptide is normally required for fibril formation.

Calcium-Binding Proteins↗

Eosinophilic meningitis in Hodgkin's disease. Report of a case and review of the literature.

Eosinophilic meningitis is rare in Hodgkin's disease. Five previous cases have been reported. We describe an additional patient whose CSF showed an eosinophilic pleocytosis with Reed-Sternberg cells while in complete systemic remission from Hodgkin's disease. The patient responded to intrathecal administration of methotrexate given by Ommaya reservoir. Six months later, systemic relapse occurred, but the CSF remained normal. Eosinophilic meningitis is a treatable complication of Hodgkin's disease, and prolonged response is possible.

Adult↗

Treatment of prolymphocytic leukemia.

Prolymphocytic leukemia is characterized by marked splenomegaly, distinctive cellular morphologic characteristics, and a poor clinical course. Five patients with typical PL were treated systematically with vincristine/prednisone, chlorambucil/prednisone, splenic irradiation, splenectomy, and other chemotherapy regimens. No patient responded to vincristine/prednisone. Two patients responded to chlorambucil/prednisone, and four patients had brief responses to splenic irradiation. Two patients underwent splenectomy, one of whom had a prolonged clinical remission. There were no complete remissions. No other chemotherapy combinations were of value. The median survival was 33 months. Recommendations are made to use chlorambucil/prednisone or splenic irradiation as initial treatment. Splenectomy should be considered in patients refractory to these modalities. The course of PL may be more protracted than originally reported.

Aged↗

Continuous infusion vincristine and bleomycin with high dose methotrexate for resistant non-Hodgkin's lymphoma.

Sixteen patients with resistant non-Hodgkin's lymphoma were treated with continuous infusions of vincristine (1-2 mg/m2 daily X 2 days) and bleomycin (0.25 mg/kg bolus dose, then 0.25 mg/kg/daily X 5 days). Responding patients received high dose methotrexate (1500 mg/m2) with citrovorum rescue on days 15, 22, 29, 36. Treatment cycles were repeated every six weeks in responding patients. The response frequency was 50% (three complete and five partial responses). Median response duration was 29 weeks. Major toxicity included stomatitis (63%) and leukopenia (44%). One episode each of possible hypersensitivity pneumonitis and paralytic ileus occurred. Continuous infusions of vincristine and bleomycin should be studied further in less critically ill patients.

Adult↗

Hematologic effects of intravesicular thiotepa therapy for bladder carcinoma.

Twenty-nine patients with bladder carcinoma treated with intravesical thiotepa administration were reviewed to assess hematologic toxicity. Ten of 25 consecutive patients had at least one episode of acute myelosuppression. Thrombocytopenia was the most common abnormality; anemia was uncommon. Acute suppression occurred most often within the first three months of therapy and was dose related. One of the 25 patients and four additional patients had chronic myelosuppression; all were male. They were older and had received more thiotepa over a longer period than those patients without chronic myelosuppression. Thrombocytopenia was again the most common abnormality. Anemia was nearly as frequent in this subpopulation and may herald chronic blood dyscrasia. One patient had refractory anemia with an excess of blast forms; another had smoldering leukemia. Acute and chronic forms of myelosuppression were not related. Thiotepa treatments should be limited to 90 mg/mo and protracted therapy avoided in elderly men.

Adult↗

An in vitro model for the study of enzymatically mediated biomechanical changes in the chorioamniotic membranes.

The effects of different incubation media on the biomechanical properties of nine chorioamniotic membranes were studied. Significant alterations in strain/rupture, work/rupture, and rupture/tension values have been demonstrated after a 24 hour incubation period in pseudoamniotic fluid (PAF). These changes can be arrested if enzyme inhibitors are added to the incubation media. This study suggests that biomechanical alterations in the chorioamniotic membranes may be biomechanical, namely, enzymatically mediated.

Amnion↗