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Biomedical subjects

G E Herman

Publications and source records attributed to G E Herman.

68 records · Page 4Linked to original sources

Multiple congenital anomaly/mental retardation (MCA/MR) syndrome with Goldenhar complex due to a terminal del(22q).

We present a case of terminal del(22q) with Goldenhar complex including hemifacial microsomia, bilateral epibulbar dermoids, preauricular tags with sensorineural hearing loss, vertebral anomalies, and CNS and renal malformations. The case illustrates causal heterogeneity of the Goldenhar complex and a previously unreported associated chromosome deletion.

Abnormalities, Multiple↗

Linitis plastica of the esophagus.

A 62-yr-old woman was evaluated for a 2-wk history of regurgitation and weight loss. Radiologic studies demonstrated a stenotic region in the distal third of the esophagus. Multiple endoscopic biopsies were nondiagnostic. Esophagectomy and esophagogastrostomy revealed a diffusely infiltrating adenocarcinoma ("linitis plastica") of the distal esophagus, sparing the gastroesophageal junction and stomach. Despite the absence of metastatic disease at laparotomy, the patient developed metastases unresponsive to chemotherapy. The pathologic, radiographic, and endoscopic findings are consistent with primary linitis plastica of the esophagus.

Adenocarcinoma↗

Teratogenic effects of first-trimester cyclophosphamide therapy.

Intravenous cyclophosphamide was administered for severe exacerbation of systemic lupus erythematosus to a patient not known to be in the first trimester of pregnancy. The patient received no other medication except prednisone. Her neonate was born with multiple anomalies, including absent thumbs, cleft palate, low-set ears, and multiple eye abnormalities. These anomalies probably reflect teratogenic effects of cyclophosphamide, and indicate that judgment is required before its use in the first trimester. Furthermore, this case illustrates the need for effective contraception and repetitive pregnancy testing when potentially teratogenic agents are administered to presumably nonpregnant women in the reproductive age group.

Abnormalities, Drug-Induced↗

Fatal thyrotoxic crisis.

Graves' disease is a form of hyperthyroidism. A rare complication of Graves' disease is thyrotoxic crisis. Although the crisis is frequently fatal, it is a distinctly unusual cause of sudden death. We investigated the case of a young woman with Graves' disease who died suddenly. Radioimmunoassay tests of thyroid function were used to establish the diagnosis.

Adult↗

Escherichia coli K-12 clones that overproduce dam methylase are hypermutable.

A strain of Escherichia coli K-12 that overproduces dam methylase 50-fold was found to be hypermutable, and mutations which resulted in loss of excess methylase activity restored mutation frequencies to wild-type levels. These results are consistent with involvement of this deoxyribonucleic acid methylase in mismatch correction.

Cloning, Molecular↗

Effects of autoprothrombin II-A on epinephrine-induced platelet aggregation of normal- and coumadin-treated dogs.

Platelet-rich plasma from dogs and from coumadin-treated dogs aggregated at the same optimum concentration of epinephrine. Neither protein C nor its active form called autoprothrombin II-A was necessary for aggregation of dog platelets with epinephrine. For platelet aggregation, suboptimal concentrations of epinephrine were potentiated by addition of purified autoprothrombin II-A. The latter, by itself, induced platelet aggregation in high concentration.

Animals↗

Binaural analysis in the aging auditory system.

In a simulation of the "cocktail party" problem, subjects listened to recordings of a target voice, obscured by four background voices from adjacent locations in space. Listening conditions were either dichotic (with interaural directional cues preserved) or diotic (same input to both ears, directional cues removed). Elderly subjects were unable to use binaural directional cues as effectively as young subjects to improve intelligibility of the target voice. The results suggest that there is an age related deficit in the ability to perform the binaural signal analysis necessary to effectively separate speech signals from noise.

Adult↗

Autoprothrombin ii-a, thrombin, and epinephrine: interrelated effects on platelet aggregation.

Platelets aggregate with thrombin-free autoprothrombin II-A. Aggregation was dependent on an intact release mechanism since inhibition of aggregation occurred with adenosine, colchicine, or EDTA. Autoprothrombin II-A reduced the sensitivity of platelets to aggregate with thrombin, but enhanced epinephrine-mediated aggregation. Autoprothrombin II-A directly competes for membrane sites sensitive to thrombin. It allows complexes to from with epinephrine. There was no absolute requirement for autoprothrombin II-A since epinephrine aggregated dog platelets at the identical optimum concentration following Coumadin treatment.

Animals↗