Fall 2016, Volume 7, Issue 5

EBSCO Health Nursing Newsletter including topics on Diabetic Ketoacidosis in Children, Angelman Syndrome, and Pressure Injuries in Surgical Patients

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Welcome back to EBSCO Health's free evidence-based nursing newsletter. We will periodically send news on the latest evidence in nursing. Please share this with your colleagues, students, practitioners and others who would benefit from this information.

Nursing Reference CenterPlus in Daily Practice

Diabetic Ketoacidosis in Children

Anderson is a 15-year-old patient recovering from diabetic ketoacidosis in the intensive acute care unit. The nurse caring for this patient wants to clarify the nursing plan for him, so she consults Nursing Reference Center Plus, keying in the words "diabetic ketoacidosis children." She retrieves the quick lesson "Diabetic Ketoacidosis in Children."

The nurse reads about the appropriate nursing care for a child with diabetic ketoacidosis, including assessment, laboratory tests, treatment goals and interventions. Based on the information in the quick lesson, the nurse carefully monitors the patient's vital signs and physiologic systems, and reviews laboratory test results. She then administers prescribed medications and provides emotional support and education for the patient and family.

Note: The above-referenced quick lesson is freely accessible to all readers of the EBSCO Health Nursing Newsletter.

Quick Overview

Caring for Patients with Angelman Syndrome

Angelman syndrome (AS) is an inherited condition caused by defects in genes involved in the development of the nervous system resulting in profound developmental and cognitive disability, deficits in expressive and receptive language, excessive smiling and laughing independent of the environmental context, ataxic movements, disrupted sleep patterns and epilepsy. Other manifestations that occur in some individuals include microcephaly (i.e., abnormally small head), deep-set eyes, thin upper lip, protruding tongue, macrostomia (i.e., wide mouth) and postnatal onset hypopigmentation (i.e., lighter skin, eyes and hair than family members).

AS results from the disruption of function or expression of the maternal copy of the gene that encodes the enzyme ubiquitin protein ligase E3A (UBE3A gene), which is located on the long (q) arm of chromosome 15. The ubiquitin protein ligase E3A plays a central role in synaptic development and maturation of the cerebral cortex. The paternal copy of the UBE3A gene is normally imprinted (i.e., having undergone a process that silences the expression of genes inherited from one parent). In approximately 70-75% of patients, there is evidence of deletion of the maternal copy of the UBE3A. Paternal uniparental disomy occurs in 2-5% of patients, when two inactive copies of the paternal gene are inherited and the mother's gene is not passed along. Imprinting defect (2-5% of cases) occurs when the maternally inherited gene is wrongly silenced or imprinted, and the father's (inactive) gene is used as a replacement.

Children with AS usually appear normal at birth; clinical manifestations, including signs of developmental delay, develop during the first year of age and become apparent in late infancy. Clinical diagnosis can be confirmed by molecular genetic testing in 90% of cases. Treatment is supportive. Lifelong management of patients with AS involves use of antiepileptic drugs (AEDs) for seizure control and melatonin as a sleep aid; physical, speech and occupational therapy; and specialized education to support the development of motor and language skills.

Please log in to your Nursing Reference Center™ or Nursing Reference Center Plus subscription to read the quick lesson on "Angelman Syndrome."

Evidence-Based Content Update

Recently, the evidence-based care sheet "Pressure Injuries in Surgical Patients" was revised following review under the Systematic Literature Surveillance Program. Among new information of value to nursing practice were new guidelines from the National Pressure Ulcer Advisory Panel. The terminology has been changed from 'pressure ulcer' to 'pressure injury.' Stages are now described with Arabic numbers rather than Roman numerals.

We invite you to log in to Nursing Reference Center or Nursing Reference Center Plus to read updated content as it becomes available.


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