Management of mitochondrial disease on an intensive care unit

Patients with mitochondrial disease may present to the IntensiveCare Unit (ICU) with a variety of neurological and general medicaldisorders. Eleven patients were admitted to a neurological ICUbetween 1970 and 1992 because of respiratory insufficiency,status epilepticus and/or metabolic encephalopathy associatedwith mitochondrial disease. Respiratory impairment occurredin eight patients and was associated with nocturnal hypoventilationdue to respiratory muscle weakness, aspiration due to bulbarweakness and abnormalities of central control leading to a reducedCO₂ drive, irregular respiratory patterns and sleep apnoea.Seven patients received continuous respiratory support duringthe acute illness; three were subsequently weaned to domiciliaryventilation, and four died. Five patients had stroke-like episodes,which in two were recurrent. Four patients developed tonic-clonicgrand mal epilepsy associated with myoclonic fits (2 patients),absences (2), focal fits (1) and status epilepticus (2). Encephalopathywas associated with recurrent lactic acidosis (2 patients),cardiac failure (2), hyponatraemia (2), renal abnormalities(3) and complete heart block (1). Although rare, mitochondrialdisease should be considered in any patient with unexplainedrespiratory failure, intractable epilepsy, lactic acidosis orrecurrent stroke.     

Paving the way: Stepping stones to evidence-based nursing

Wallace MC, Shorten A, Russell KG. International Journal of Nursing Practice 1997; 3: 147–152
Paving the way: Stepping stones to evidence-based nursing
Evidence-based practice is an emerging paradigm in health care. This paper outlines the main features of this paradigm and its potential value to nursing. Evidence-based practice is based on a conceptual framework that examines the extent of evidence available in support of particular clinical practices. The Quality of Evidence Ratings adapted by the National Health and Medical Research Council (NHMRC) from the United States Preventive Services Task Force are discussed, and the strengths and weaknesses of different categories of evidence are highlighted. Potential barriers to implementation of research into practice are identified. The authors suggest that legal, ethical, economic and humane imperatives oblige nursing to develop evidence-based practice as one of several viable contributions to nursing knowledge. Suggestions for analysing current research and for the planning of the direction of future nursing research are made.     

Comparison between broad-band ultrasound attenuation of the calcaneum and total body bone mineral density in children

We measured broad-band ultrasound attenuation (BUA) in the calcaneum using the prototype Paediatric Contact Ultrasound Bone Analyser (CUBA) and total body bone mineral density (TBBMD) using dual-energy X-ray absorptiometry (DXA) (Hologic QDR-1000W) in 58, 7 17-year-old healthy children and adolescents. Calcaneal BUA was significantly related to TBBMD ( r = 0.74, p < 0.001), and both the calcaneal BUA and TBBMD were significantly correlated with age and body weight. We conclude that calcaneal BUA reflects bone mineral density (BMD) in healthy children and adolescents; however, BMD measured by CUBA appears to be less sensitive than that measured by DXA. Since BUA reflects structural properties of bone, as well as density, it may complement radiological techniques of bone density measurement in the assessment of paediatric conditions associated with fracture risk.     

New treatment modalities for paediatric kidney disease

Summary: The new modalities of treating nephrotic syndrome, chronic renal insufficiencies, renal osteodystrophy and the use of recombinant human growth hormone and insulin-like growth factors are reviewed in order to provide the nephrologist caring for children an informed choice of therapies.