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Biomedical subjects

E Johnston

Publications and source records attributed to E Johnston.

At least 37 records · Page 2Linked to original sources

Genetic control of avian scleroderma.

The inheritance of avian scleroderma, a fibrotic autoimmune disease of chickens resembling human scleroderma, was investigated. Comb inflammations and lesions were used to determine the state of disease of 4-week-old chickens. All line 200 males and 60% of female line 200 chicks showed abnormalities. Crosses (F1) between line 200 and eight partially inbred lines of chickens maintained at the University of California at Davis were all normal. Backcrosses of F1 cocks to line 200 hens showed a higher incidence of scleroderma in males than in females for all lines. The incidence of affected birds varied between backcrosses from a low of 42% for backcross line 217 males derived from a New Hampshire line, to 88% for males of backcross line 213 derived from a partially inbred Leghorn line, demonstrating the presence of genes modifying the penetrance of presumed major genes causing the disease. Backcross genotypes segregating for haplotypes of the major histocompatibility complex (MHC) derived from inbred lines showed consistently lower penetrance of scleroderma than homozygotes carrying the line 200 haplotype. Thus B3Bs (lines 211 and 215), B14Bs (line 217), and B15Bs (lines 212, 213, 216, and 218) all had fewer affected individuals than BsBs homozygotes from the same families.

Animals↗

Ligand-dependent structural variations in Escherichia coli F1 ATPase revealed by cryoelectron microscopy.

The Escherichia coli F1 ATPase, ECF1, has been examined by cryoelectron microscopy after reaction with Fab' fragments generated from monoclonal antibodies to the alpha and epsilon subunits. The enzyme-antibody complexes appeared triangular due to the superposition of three anti-alpha Fab' fragments on alternating densities of the hexagonally arranged alpha and beta subunits. The Fab' to the epsilon subunit superimposed on a beta subunit. A density was observed near the center of the structure in the internal cavity. The position of this central density with respect to peripheral sites was not fixed. Sorting of images of ECF1 labeled with the combination of three anti-alpha Fab' fragments plus an Fab' directed to the epsilon subunit gave three classes in each of which the central density was closest to a different beta subunit. The distribution of the central density among the three classes was measured for different ligand-binding conditions. When ATP was present in catalytic sites under conditions where there was no enzyme turnover (i.e., without Mg2+ present), there were approximately equal numbers of images in each of three classes. When ATP and Mg2+ were added and ATP hydrolysis was allowed to proceed, almost two-thirds of the images were in the class in which the central density was closest to the beta subunit superimposed by the epsilon subunit. We conclude that domains within the ECF1 structure, either the central mass or a domain including the epsilon subunit, move in the enzyme in response to ligand binding. We suggest that this movement is involved in coupling catalytic sites to the proton channel in the F0 part of the ATP synthase.

Adenosine Diphosphate↗

Infratentorial subdural empyema: clinical and computerized tomography findings. Report of three cases.

Infratentorial subdural empyemas are rare. The authors report three cases encountered between 1979 and 1988, representing a 3% incidence among all subdural empyemas. The common source was an ear infection. Clinical presentation encompassed a systemic febrile illness, headaches, and a stiff neck. Only one patient had an inconspicuous focal neurological deficit that suggested a cerebral location. Initial diagnosis was acute meningitis in each case. A lumbar puncture was ordered in all three cases but was actually performed in two without developing tonsillar herniation. Cerebrospinal fluid analysis confirmed the diagnosis of meningitis in one but was normal in the other. Computerized tomography allowed a precise diagnosis and localization of the pathology. All three patients received aggressive antibiotic therapy plus suboccipital craniectomy and aspiration of pus; catheter drainage was performed in two. Cultures were positive in one case and negative in the others. Two patients were cured without sequelae; the third patient was moribund at surgery and died. Although it is known that subdural empyemas may localize in the posterior fossa, only one previous report was found. Infratentorial subdural empyema may sometimes be an unrecognized companion of acute meningitis and is cured with antibiotic therapy alone.

Acute Disease↗

Does osteomalacia contribute to development of oral complications of oxalosis?

Two renal dialysis patients with oral manifestations of oxalosis had undecalcified sections of iliac and alveolar bone and teeth examined histologically in an attempt to explain the development of tooth mobility and tooth loss. Osteomalacia was detected in all bone specimens and attributed to aluminum toxicity after the histochemical localization of aluminum at the calcification front between osteoid and calcified matrix. Aluminum was also detected histochemically in the cementum of teeth. Calcium oxalate crystals were present in bone marrow, teeth, and gingiva. It is proposed that tooth mobility and tooth loss in oxalosis result from the combined effects of osteomalacia and oxalate crystal deposition within the periodontium. To prevent avoidable tooth loss it is suggested that patients with oxalosis who develop tooth mobility should have aluminum toxicity and osteomalacia excluded as causal factors.

Adolescent↗

Focal osteomalacia due to low-dose diphosphonate therapy in Paget's disease.

Transiliac bone biopsies carried out on 13 patients with Paget's disease to evaluate the effects of low-dose diphosphonate (disodium etidronate) therapy showed focal osteomalacia in the 9 patients in whom post-therapy specimens were taken through pagetic bone. Active bone resorption persisted in 5 of these. A mineralisation defect not amounting to osteomalacia--ie, osteoid of increased thickness but of normal extent--was present in the 4 specimens taken through non-pagetic bone. Although 9 patients experienced symptomatic improvement, 2 suffered fissure fractures in affected lower limbs. In Paget's disease, the combination of osteomalacia and continuing active resorption within a lytic lesion may increase the risk of fracture in a weight-bearing bone. It is suggested that although disodium etidronate often provides effective pain relief it should be administered with caution until the optimum dose and duration of therapy are further evaluated.

Aged↗

The topography of primate retina: a study of the human, bushbaby, and new- and old-world monkeys.

The distribution of ganglion cells has been studied in the retinas of four primates: the prosimian bushbaby, the New-World squirrel monkey, the Old-World crab-eating cynamolgous monkey, and the human. The sizes of ganglion cell somas were also measured at a number of retinal locations and compared with similar measurements in the cat retina to test for the presence in primates of retinal specializations such as the visual streak, and for gradients in retinal structure, such as that between temporal and nasal retina. In all four primates, ganglion cell somas in peripheral retina ranged considerably in diameter (6-16 micrometer in the bushbaby, 8-22 micrometer in the squirrel monkey, 8-23 micrometer in the cynamolgous monkey, 8-26 micrometer in the human). It seems likely that the strong physiological correlates of soma size which have been described among cat retinal ganglion cells and among the relay cells of the macaque lateral geniculate nucleus are generally present in primates. In all four primates, evidence was also obtained of a visual streak specialization; the isodensity lines in ganglion cell density maps were horizontally elongated, and small-bodied ganglion cells were relatively more common in the region of the proposed streak than in other areas of peripheral retina. However, the visual streak seems less well developed than in the cat; among the four primate species examined it was best developed in the bushbaby, at least as assessed by the shape of the isodensity lines. All four primates showed a clear foveal specialization, but this feature seemed least developed in the bushbaby. At the fovea, ganglion cells are smaller in soma size than in peripheral retina; they also seemed more uniform in size, although some distinctly larger cells persist in the human and bushbaby. Soma size measurements also provided evidence of a difference between nasal and temporal areas of peripheral retina comparable to that reported for the cat and other species. Thus the primate retinas examined show features, such as the foveal specialization, which seem unique to them among mammals. They also show features, such as nasal-temporal differences in ganglion cell size, and (though weakly developed) a visual streak, which they have in common with other mammals with widely different phylogenetic histories.

Animals↗

Undetected latrogenic hypothyroidism: a late complication of radio-iodine therapy.

The thyroid status of 317 patients treated with radioactive iodine between 1951 and December 1971 was investigated. Information was obtained on 236 patients and hypothyroidism, either treated or untreated, was present in 48 percent of women and 37 percent of men at mean follow-up intervals of 5.5 and 4.4 years respectively. Ninety-four of the 241 patients still alive were assessed personally by clinical examination and serum thyroxine assay, and 10 were found to have previously undiagnosed hypothyroidism. Seven of these patients had symptoms and in five these symptonms were the cause of significant distress. The importance of undertaking a long term review programme following radioactive iodine therapy is emphasised.

Adenoma↗

The linkage relationships of HL-A with other genetic marker systems.

Data on the linkage relationships of HL-A with 31 autosomal genetic markers are presented. Among 40 markers for which some information on the linkage relationship with HL-A is available, four (PGM3, GBG, Ch and Pg) have lod scores greater than +3. The most likely order for the polymorphic systems tentatively assigned to chromosome 6 is Kell: Pg:(HL-A,GBG):Ch:PGM3:P.

Blood Group Antigens↗