Five patients, 4 men and 1 woman, had adult-onset and slowly progressive weakness. There was distal wasting in 2, hepatomegaly in 3, and congestive heart failure in 2. Electromyography showed a mixed pattern with abundant fibrillations. Serum creatine phosphokinase was increased 5- to 45-fold. Blood glucose failed to respond to epinephrine or glucagon, and venous lactate did not rise after ischemic exercise. Muscle biopsy showed vacuolar myopathy affecting both fiber types. By electron microscopy the vacuoles corresponded to large pools of glycogen not limited by a membrane. Glycogen concentration was 3 to 5 times normal in muscle and 7 to 21 times normal in erythrocytes. In the presence of iodine, muscle glycogen showed a spectrum characteristic of phosphorylase-limit-dextrin. Debrancher activity was measured by a spectrophotometric assay and by a radioactive reverse reaction. The activity was lacking in muscle and erythrocytes of 4 patients according to both assays; in 1 patient the reverse reaction was not impaired. Though previously reported in only 5 patients, debrancher deficiency myopathy may not be rare and should be considered in the differential diagnosis of adult-onset hereditary myopathies.

Adolescent↗

PubMed1979

Adenoma of the pigment epithelium of the ciliary body simulating a malignant melanoma.

A benign adenoma of the pigment epithelium of the ciliary body in a 27-year-old woman simulated a malignant melanoma clinically and resulted in enucleation. The correct diagnosis was not made clinically despite the use of A- and B- scan ultrasonography and the radioactive phosphorus (32P) uptake test, procedures which are usually reliable in differentiating between melanoma and pseudomelanoma. We emphasize the shortcomings of these ancillary tests in differentiating pigment epithelial tumors from malignant melanoma.

Adenoma↗

PubMed1979

Comparative aspects of nuclear proteins and nuclear composition of cultivated embryonic, neonatal and neoplastic rat brain cells of glial origin.

Differences in nuclear composition and variations in the types of acid soluble nuclear proteins were identified when cultivated neoplastic and nontransformed rat brain cells were compared. HeLa S-3 cells were used as a biological reference in these studies. The nuclei of anaplastic glioma cells were found to contain more total nuclear protein and greater amounts of nuclear RNA, than the nuclei of nontransformed embryonic and neonatal rat brain cells. Densitometer profiles of samples developed by polyacrylamide gel electrophoresis indicated that all three major histone classes were present in the nuclei of the four different cell types examined. Each type of cell was grown in the presence of 3H-lysine or 3H-lysine plus 14C-arginine to further characterize both the histones and the acid soluble nonhistone proteins. Neoplastic and nontransformed cells contained similar quantities of histones H4, H3, H2A and H2B. In contrast, transformed cells were found to contain two dominant subspecies of lysine rich H1 histone, while only one histone H1 subcomponent was extracted from the nuclei of the embryonic and neonatal rat brain cells. Nuclei isolated from HeLa cells and anaplastic glioma cells also contained increased varieties and larger quantities of acid soluble nonhistone nuclear proteins.

Animals↗

PubMed1979

[Nursing in the National Health Plan of Honduras].

After summarizing the health situation in Honduras and describing the National Health Plan launched in 1973, the authors explain the changes that have come about in nursing, the difficulties that had to be surmounted to reach a new professional position, and the administrative decisions that had to be taken to devise a health policy. An account is given of the changes made in the training of nursing staff and in the functions that staff must perform in the planning, implementation, and evaluation of the coverage extensive program. The article high-lights the role of the Nursing Division in the Ministry of Public Health and Social Welfare and the levels of coordination established with the other technical divisions and institutions responsible for the health personnel training. In closing, the article points out that the nursing sector represents the keystone of the model for the delivery of services to attain the goal of health for all by the year 2000.

Education, Nursing↗

PubMed1978

Rhegmatogenous retinal detachment following chorioretinal inflammatory disease.

Preexisting ocular inflammatory disease was responsible for 44 cases (1.7%) of rhegmatogenous retinal detachment in a large series of consecutive retinal detachments. Characteristics of retinal detachments following ocular inflammatory disease include a longer duration of the detachment, fewer observable retinal breaks, a higher incidence of visible vitreous membranes and preoperative macular puckers, a younger age distribution, and a higher incidence of phakic patients. In comparing the group of postinflammatory rhegmatogenous retinal detachments with detachments not associated with inflammation, the following characteristics did not show any statistically significant difference: sex, the eye involved, the status of the ciliary epithelium, the presence or types of retinal folds, the rate of operative complications, or the rate of reattachment at six months.

Adult↗

PubMed1978