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PubMedDate not supplied

Fetal akinesia-hypokinesia deformation sequence (FADS) in 2 siblings with congenital myotonic dystrophy.

Two premature siblings described herein had clinical features comparable to the fetal akinesia-hypokinesia deformation sequence (Pena-Shokeir syndrome) with polyhydramnios, intrauterine growth retardation, pulmonary hypoplasia, short umbilical cord and lethality. Autopsy revealed no thoracal or abdominal viscera anomalies and examination of the brain, spinal cord and peripheral nerves did not disclose any pathological changes. Light microscopy, immunohistochemistry and electron microscopy of skeletal muscles demonstrated immature muscles with some fibril disorganisation and abnormal immunoreactivity for actin and desmin. Subsequent molecular genetic analysis revealed a maternal diagnosis of myotonic dystrophy. The retarded growth and maturation of skeletal muscle observed in the presented cases correspond with previous findings in neonatal myotonic dystrophy. A well-defined myopathy can thus result in the fetal akinesia-hypokinesia deformation sequence.

Abnormalities, Multiple↗
PubMed1995

Clinical guidelines: fad, fact, or vision.

Interest in clinical guidelines is expanding significantly. Clinical guidelines have been developed by the Agency for Health Care Policy and Research, specialty organizations representing almost every health-care discipline, and individual agencies and facilities. Clinical guidelines may be used in the development of practice and patient care standards and within care delivery models such as case management. Clinical guidelines offer professional nurses an opportunity to define their practice and their contributions to outcomes-based patient care. Clinical guidelines also may enable interdisciplinary continuous quality improvement to ensure ongoing optimization of care delivery and patient outcome. Therefore, clinical guidelines can be used to design patient care that is truly driven by patient need and that empowers optimal contributions by nurses and all other members of the caregiving team.

Clinical Protocols↗
PubMed1994

Communication skills in clinical practice fad or necessity?

Communication skills of 40 final year medical students were assessed using Objective Structured Clinical Examination. The scores obtained were significantly less than those obtained in clinical skills. There was no significant correlation between the two sets of scores. It is suggested that emphasis should be placed on developing and evaluating communication skills during undergraduate medical curriculum.

Communication↗
PubMed1994

Riboflavin status of the elderly: dietary intake and FAD-stimulating effect on erythrocyte glutathione reductase coefficients.

OBJECTIVE: The present study was undertaken to investigate the relationship between riboflavin intake and its biochemical status in elderly subjects (> or = 65 years). SUBJECTS: Thirty males and 30 females were randomly selected from a larger sample of free-living elderly residents of Edmonton, Canada. Subjects were contacted through a seniors' service organization and the Medical Officer of Health. Any subject using vitamin supplements or alcohol other than the occasional drink was excluded from the study. DESIGN: Dietary intake was estimated using a 3-nonconsecutive-day food record. Biochemical status was assessed by measuring the erythrocyte glutathione reductase activity coefficient (EGRAC), which is a functional test indicative of metabolic availability of riboflavin. RESULTS: Average daily riboflavin intake of the study population was above the recommended requirement (0.5 mg/1000 kcal). According to the frequently used criterion that an EGRAC value > or = 1.20 is indicative of inadequate riboflavin status, 23% of the males and 7% of the females were classified as being deficient in the vitamin. However, based on the recently suggested normal values (1.30-1.4) for EGRAC, it appeared that all male subjects but one had adequate riboflavin status. CONCLUSION: The criteria used for EGRAC values may affect the interpretation of riboflavin status. It is therefore important that guidelines for EGRAC are standardized for more reliable interpretations.

Aged↗
PubMed1994

[Supplements during pregnancy: fad or necessity?].

Needs for vitamins and micronutriments, enzyme cofactors in various metabolic processes, are notably increased during pregnancy in order to satisfy the requirements of fetal and placental growth. Limited maternal stores and an often inadequate dietary intake lead to deficiency, the materno-foetal consequences of which are beginning to be discerned: supplements thus appear to be useful. While there is hardly any discussion regarding iron and calcium, the authors feel that it should be broader, including water-soluble vitamins, magnesium and trace elements, in such a way as to deal with widely encountered microdeficiencies and with metabolic inter-relations. Recent studies appear to show that, rather than being selective and seeking out a "high risk" population, supplements should be given routinely to all pregnant women because of their low costs in relation to expected health benefits.

Deficiency Diseases↗
PubMed1975

Covalently bound FAD in d-6-hydroxynicotine oxidase. Immunological studies of D- and L-6-hydroxynicotine oxidase: evidence for a D-enzyme precursor.

Antersera prepared against both enantiozymes, D- and L-6-hydroxynicotine oxidase, formed precipitins in double diffusion tests with their respective antigens only. A mixture of the two antisera caused spur formation of the two precipitin lines obtained with the pure enzymes. Antiserum to L-apoprotein reacted with native L-enzyme and L-apoprotein but not with the D-sspecific enzyme. D-6-hydroxynicotine oxidase activity was inhibited by the anti-D-antiserum, leaving the L-enzyme fully active, while anti-L-antiserum inhibited the L- but not the D-specific activity. The delayed induction of D-6-hydroxynicotine oxidase as compared to the other activities of the nicotine-degrading sequence and the differential immunochemical behavior of the enantiozymes allowed the search for a D-enzyme precursor. In cells harvested 3 hours after the addition of DL-nicotine, the L-enzyme activity was present, whereas no D-enzyme activity could be detected. However, an extract of these cells did form an immunoprecipitin line with anti-D-antiserum. L-6-Hydroxynicotine oxidase, but no D-6-hydroxynicotine oxidase activity, could also be induced in Arthrobacter oxidans grown in a medium with a high glucose content and DL-nicotine as the sole nitrogen source. An extract of these L-induced cells produced the specific immunoprecipitation with anti-D-antiserum. A pulse-chase experiment with cells grown first on glucose and DL-nicotine in the presence of [14C]leucine and then in an unlabeled medium which induces D-6-hydroxynicotine oxidase activity resulted in a radioactive D-enzyme-immunoprecipitin line. From these experiments it is concluded that a precursor of the active D-enzyme is induced simultaneously with the other nicotine-degrading enzymes.

Arthrobacter↗