Biomedical subjects

E C Alport

Publications and source records attributed to E C Alport.

5 recordsLinked to original sources

PubMed1993

Fahr's disease associated with astrocytic proliferation and astrocytoma.

This report documents the neuropathological findings of astrocytic proliferation and astrocytoma in a patient with Fahr's disease. At autopsy, there was extensive bilateral symmetrical calcification involving basal ganglia, sulcal depth of the cerebral cortex, and dentate nuclei of the cerebellum. A large low-grade astrocytoma was identified in the left parietal lobe. Astrocytic proliferation was also noted in the areas of early calcification and at the margins of large calcareous deposits, away from the tumor.

Adult↗

PubMed1986

Telangiectatic osteosarcoma: unusual behaviour.

The telangiectatic variant of osteogenic sarcoma is rare. Its biologic behaviour, treatment and prognosis are controversial. The case of a 15-year-old girl with this tumour is described. Both the location and clinical behaviour of the tumour were unusual. The tumour, which involved the distal ulna, was initially treated by a limited resection of the distal 8 cm of ulna. Sixty-nine months later the tumour recurred locally; there was no evidence of metastases. The forearm was amputated and the patient was then treated aggressively by chemotherapy. She was well 42 months later. This case illustrates the tendency for telangiectatic osteosarcoma to recur locally if it is not radically excised.

Adolescent↗

PubMed1984

Pigmented villonodular synovitis of the temporomandibular joint.

Pigmented villonodular synovitis ( PVNS ) is a benign disease of unknown etiology arising from the synovial membranes of joints, bursae, and tendon sheaths. Though histologically benign, it is a very aggressive lesion, capable of bone destruction and widespread infiltration of surrounding tissues. It is a rare disease, usually confined to large joints, particularly the knee. Its propensity for recurrence is emphasized in this report. Involvement of the temporomandibular joint (TMJ) is unusual. It most commonly presents in the TMJ region as a left pre-auricular mass. Symptoms of TMJ dysfunction are usually present, but may be subtle. These may consist of painful mastication, clicking, trismus, malocclusion, and deep pain in the TMJ. Radiological evidence of bone destruction is frequently present, and together with the pre-auricular mass, should suggest the diagnosis. Surgical excision is the preferred mode of treatment. While cosmesis is a consideration, radical excision, including involved bone, is mandatory to prevent recurrence. The effect of radiotherapy on this disease in the head and neck region is documented.

Diagnosis, Differential↗

PubMed1983

The syndrome of gastric argyrophil carcinoid tumors and nonantral gastric atrophy.

Records of the 30 cases of gastric carcinoid at the Mayo Clinic showed that the gastric mucosa was normal, hyperplastic, or atrophic (nonantral) in 12, 2, or 16 patients, respectively. In the atrophic group, the tumors were in the gastric body and fundus; small, polypoid, and multicentric; and associated with fundal argyrophil cell hyperplasia. In immunocytochemical studies, minor tumor cell populations stained positively for 5-hydroxytryptamine, gastrin, and somatostatin in 1 case and for 5-hydroxytryptamine in 3 others. Metastasis occurred in 3 patients. Twelve patients had pernicious anemia. Parietal cell or intrinsic factor antibodies or both were present in all 12 patients tested. Each of the 7 patients with an intact antrum had massive hypergastrinemia. No common HLA-A, -B, or -DR antigen pattern was detected among the 10 patients tested. The results suggest that nonantral gastric atrophy predisposes to gastric carcinoid as well as to gastric carcinoma.

Adult↗

PubMed1980

An immunoenzyme assay for quantitation of human IgG antibodies to honeybee venom phospholipase A2.

A new method to measure the concentrations of IgG antibodies to phospholipase A2 in sera from patients treated with honeybee venom immunotherapy is described. This method utilizes a microcentrifugal analyzer to detect inhibition of PLA2 enzymatic activity by antibodies in serum standards and unknowns. Sera from beekeepers with known concentrations of specific antibodies, measured by radioimmunoprecipitation, were used to construct a logit-log standard curve for the immunoenzyme assay. The standard curve was linear for concentrations between 2.3 and 20.0 microgram/ml. The concentrations of specific antibodies measured by enzyme inhibition correlated well with the concentrations as measured by radioimmunoprecipitation, r = 0.959 (least squares linear regression), n = 32. Interassay variation was 10.1% at 10 microgram/ml. The immunoenzyme method is rapid and does not require radiolabeled reagents.

Antibodies↗