STUDIES ON A PATIENT WITH IN VIVO EVIDENCE OF TYPE I GLYCOGENOSIS AND NORMAL ENZYME ACTIVITIES IN VITRO

ABSTRACT. Biochemical and clinical studies on a patient with hepatic glycogen storage disease are reported. The patient showed many of the clinical and biochemical features of type I glycogenosis (glucose-6-phosphatase deficiency), but had normal activities of the following enzymes in liver tissue: glucose-6-phosphatase (EC3.1.3.9); amylo-1,6-glucosidase (EC3.2.1.33); glycogen phosphorylase (EC2.4.1.1); fructose-1,6-diphosphatase (EC3.1.3.11). The urinary excretion of 2-oxoglutaric acid was greatly increased in this patient and in a case of enzymologically proven type I glycogenosis. Abnormal 2-oxoglutaric aciduria has not been previously reported in the glycogen storage diseases. The results are discussed in relation to the possible nature of the underlying biochemical defect in patients of this type.     

Congenital smooth muscle hamartoma: a case report

We describe a case of congenital smooth muscle hamartoma with hypertrichosis and pseudo-Darier's sign, and suggest that it is a clinicohistopathological entity distinct from Becker's naevus.     

A study of the efficacy of dressings in preventing infections of continuous ambulatory peritoneal dialysis catheter exit sites

• ? This paper describes a preliminary investigation into the efficacy of dressings in preventing catheter exit site infections in patients undergoing continuous ambulatory peritoneal dialysis (CAPD). It has been carried out in the form of a pilot study.
• ? The background to the study is described and the literature is reviewed. This revealed that it is an important field of research.
• ? Exit site infections are the main cause of morbidity and treatment failure in CAPD patients.
• ? The rationale for carrying the study out, the findings and the implications for further study are reported and discussed.
• ? There is little guidance in current literature for clinicians on the prevention of catheter exit site infection. Patients are difficult to recruit to research studies. Practising nurses need training and careful management as data collectors.

     

Planning a Continuum of Care in Child Psychiatric Nursing A Collaborative Effort

A guide for providing a continuum of care for children in need of psychiatric services from the least restrictive services (outpatient care), to a moderately restrictive setting (partial hospitalization), to the most restrictive setting (inpatient care) is described. Movement along the continuum is facilitated by the nurse case manager. Concepts from three theoretical frameworks are integrated to define goals, criteria, and expected outcomes for each level of care along the continuum. They are Neuman's (1982) systems model for nursing practice and education, Caplan's (1961) model for primary prevention of mental disorders in children, and Frances, Clarken and Perry's (1984) differential therapeutics. The continuum provides a guide for nurses and other health care providers to achieve high quality care as economically as possible. It also enhances self-care and self-responsibility for families of disturbed children by offering a sequential graded system of care along the health-illness continuum.     

Assessing the knowledge and skill needs of community-based health promoters

Community-based health promoters require a range of complexskills. As part of its ongoing support to five community-basedhealth promotion programs, the Health Promotion Branch of theOntario (Canada) Ministry of Health, commissioned a study toidentify knowledge and skills needs in these programs and assesswhether resources were available to meet these needs. A NeedsAnalysis Questionnaire was developed and administered to coordinators,staff board members, and volunteers at 23 health promotion sites.Key informant interviews with site coordinators and focus groupinterviews of site staff board members and volunteers were carriedout. The needs analysis process is presented, as are illustrativefindings. Potential applications of the needs analysis proceduresare considered.     

RURAL OPHTHALMOLOGY IN WEST AFRICA

Abstract
Eye disease is common in Africa where it causes much suffering and loss of valuable man-power. Simple measures can provide immense relief at little cost The majority of the population is not wealthy and lives in rural areas. The Royal Commonwealth Society for the Blind (RCSB) has established mobile eye units as a cost-effective means of treatment and prevention of blindness, and for training national personnel to continue the good work. Ophthalmologists prepared to give a period of service are urgently needed.