Time to think again: cystic fibrosis is not an "all or none" disease.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to C Wallis.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
OBJECTIVE: To determine the clinical presentation and treatment strategies for children admitted with pleural empyema. METHODOLOGY: Retrospective review of medical and radiological records of 54 patients admitted with pleural empyema between January 1989 and April 1997. RESULTS: Fever (98%), cough (83%), chest pain (38%), clinical cyanosis (17%) and abdominal pain (16%) were common clinical features. The causative organism was identified in 17 patients (31%). Intravenous antibiotics were given for a mean of 18. 2 +/- 7.5 days. Forty-seven (87%) patients had closed chest tube drainage and 21(39%) patients underwent decortication for unsatisfactory response to medical treatment. The chest tube insertion was more likely to be delayed in patients who required decortication, although the difference was not significant (8.1 +/- 5.4 vs 6.3 +/- 5.2 days of illness, P = 0.67). All patients were discharged well, with almost complete resolution of the chest radiograph at 6 months. CONCLUSIONS: Intensive medical management with adequate chest tube drainage and appropriate antibiotics will result in full resolution for most patients. Surgical intervention is important in patients who fail to receive adequate treatment early in the disease.
Non-invasive ventilation (NIV) provides significant advantages to the paediatrician who cares for children with chronic ventilatory insufficiency and who would like to manage these children in the home environment. There is a growing experience in the use of NIV in the management of children with upper airway obstruction, ventilatory failure due to weakness or distortion of the thoracic cage and in children with abnormalities to their central respiratory drive especially during sleep. Success hinges on choosing the correct patient and the most appropriate device with a team approach to both the early assessment and follow-up. It may not be successful on the first attempt and often a phased introduction and some perseverance is required on the part of the paediatric team and the family. Home ventilation is possible, practical and advantageous compared with institutional living and non-invasive techniques can assist the paediatrician in achieving this goal.
Individually, congenital abnormalities of the lung are rare but collectively they form an important group of conditions. The range of malformations is broad and the clinical impact is very variable, depending on the degree of malformation. This article groups these conditions under their traditional headings and considers their management. However, in conditions that are atypical, it is often useful to adopt a clear anatomical approach. Consider investigations that will clearly identify the structure of the bronchial tree, lung parenchyma and the vascular supply and drainage. Describe each in logical and simple terms. Similarly, an individual assessment of function will assist in planning appropriate management. The natural history of some of the congenital lung lesions is often unknown. This gap in our knowledge has widened with the advent of detailed antenatal ultrasound scanning. Paediatricians are now faced with an increasing cohort of completely asymptomatic antenatally diagnosed congenital lung lesions and the best post-natal management is unclear. Long-term follow-up studies are required to document their natural history.
OBJECTIVES: To identify the number and current location of children, aged 0 to 16 years, requiring long term ventilation in the United Kingdom, and to establish their underlying diagnoses and ventilatory needs. DESIGN: Postal questionnaires sent to consultant respiratory paediatricians and all lead clinicians of intensive care and special care baby units in the United Kingdom. SUBJECTS: All children in the United Kingdom who, when medically stable, continue to need a mechanical aid for breathing. RESULTS: 141 children requiring long term ventilation were identified from the initial questionnaire. Detailed information was then obtained on 136 children from 30 units. Thirty three children (24%) required continuous positive pressure ventilation by tracheostomy over 24 hours, and 103 received ventilation when asleep by a non-invasive mask (n=62; 46%), tracheostomy (n=32; 24%), or negative pressure ventilation (n=9; 7%). Underlying conditions included neuromuscular disease (n=62; 46%), congenital central hypoventilation syndrome (n=18; 13%), spinal injury (n=16; 12%), craniofacial syndromes (n=9; 7%), bronchopulmonary dysplasia (n=6; 4%), and others (n=25; 18%). 93 children were cared for at home. 43 children remained in hospital because of home circumstances, inadequate funding, or lack of provision of home carers. 96 children were of school age and 43 were attending mainstream school. CONCLUSIONS: A significant increase in the number of children requiring long term ventilation in the United Kingdom has occurred over the past decade. Contributing factors include improved technology, developments in paediatric non-invasive ventilatory support, and a change in attitude towards home care. Successful discharge home and return to school is occurring even for severely disabled patients. Funding and home carers are common obstacles to discharge.
Explore the source record for details and available documents.
PURPOSE: Studies have shown that 11% to 18% of patients with an abdominal aortic aneurysm (AAA) have a first-degree relative with an AAA. A familial pattern among patients with peripheral arterial aneurysms and arteriomegaly has not been reported. The objective of this study was to examine familial patterns among patients with peripheral arterial aneurysm and arteriomegaly and compare them with patterns among patients with AAA. METHODS: Pedigrees were constructed for first-degree relatives of patients who received the diagnosis of peripheral arterial aneurysm, arteriomegaly, or AAA from 1988 through 1996. The presence of aneurysms and risk factors was confirmed for patients and relatives by means of telephone interviews and review of hospital and physician records. RESULTS: Seven hundred three first-degree relatives older than 50 years were contacted for 140 probands with peripheral arterial aneurysm, AAA, or arteriomegaly. There were differences in risk factors for hernia and diabetes mellitus among the probands with peripheral arterial aneurysm, AAA, or arteriomegaly but none for relatives. Patients with peripheral arterial aneurysm (n = 40) had a 10% (4/40) familial incidence rate of an aneurysm, patients with AAA (n = 86) had a 22% (19/86) familial incidence rate, and patients with arteriomegaly (n = 14) had a 36% (5/14) familial incidence rate. AAA (24/28, or 86%) was the aneurysm diagnosed most commonly among first-degree relatives. Most aneurysms (85%) occurred among men. CONCLUSION: There appears to be a gradation of familial patterns from peripheral arterial aneurysm to AAA to arteriomegaly among patients with degenerative aneurysmal disease, and there appears to be a predominance among men. Relatives of patients with any of the 3 lesions-peripheral arterial aneurysm, AAA, arteriomegaly--most frequently have AAA. Relatives of patients with AAA, peripheral arterial aneurysm, or arteriomegaly may be screened by means of a physical examination for peripheral aneurysmal disease. Screening by means of ultrasound examination of the aorta should be limited to first-degree relatives of patients with aortic aneurysms or arteriomegaly.
A modification to the standard quadhelix transpalatal expansion arch is presented in which the wire passes distal to the most posterior molar and enters the mesial end of the headgear tube. Laboratory measurements were carried out on small, average and large sized appliances with this modification using a special rig fitted with strain gauges. For the three sizes of appliance, the modified quadhelix is more flexible than the standard quadhelix for both displacement and rotation of the molar arms. Also it is less time consuming to fit and remove it is concluded that this appliance is capable of producing clinically useful forces and couples with increased precision.
Paediatric home ventilation is a feasible option and can be successful in a wide range of conditions and ages. Advances in ventilator technology and an ethos of optimism for home care has increased the possibilities for discharging chronically ventilated children from intensive care units and acute medical beds. With careful planning the process can succeed, but difficulties often thwart the responsible team, especially when attempting discharge for the first time. These core guidelines aim to assist a smooth, swift and successful transfer. They were developed by a working party of interested professionals spanning a wide range of health care disciplines and represent a synthesis of views accumulated from the experiences of individual teams throughout the UK. Three case scenarios provide further illustrative detail and guidance.
Explore the source record for details and available documents.
Trials of rhDNase in mixed groups of adults and children with cystic fibrosis (CF) have demonstrated improvements in lung function and well-being. This has led to many pediatric CF patients receiving regular rhDNase therapy although their response to treatment may not be the same as that seen in adults. We have retrospectively reviewed the effects of rhDNase during the first year of therapy in 65 children receiving the drug at two tertiary referral centers. Outcome measures included changes in lung function, oxygen saturation, use of intravenous antibiotics, and subjective improvement. Median baseline lung function (% of predicted) was 45% for forced expiratory volume in 1 second (FEV1) and 58% for forced vital capacity (FVC). At 3-4 months following initiation of therapy the group demonstrated median (95% CI) increases of 14.2% (95% CI 7.3; 21.1%) in FEV1 and 7% (0; 14%) in FVC. Within this wide scatter of responses, one-quarter of children deteriorated, but almost 50% showed significant improvements of > 10%. A similar pattern was seen at 9 months, with median increases for the group of 11.1% (0; 18.8%) in FEV1 and 5.6% (0; 17%) in FVC, again with approximately one-third of the group deteriorating and one-half improving significantly. Intravenous antibiotic use decreased significantly. Almost all the children (89%), including those with a fall in lung function, described subjective improvement. There were no predictive markers at baseline of a good response to the drug. However, there was a good correlation between lung function response at 3 months and that at 6, 9, and 12 months. Thus, children respond to rhDNase at least as well as adults, and a therapeutic trial is justified in those over 5 years with significantly impaired lung function. Response is highly variable, making careful individual assessment mandatory. Baseline characteristics are not useful in predicting those children who will respond well to treatment, but long-term response to the drug can be predicted on the basis of spirometric improvement at 3 months. Therefore, this would be a useful time period for a therapeutic trial.
Explore the source record for details and available documents.
OBJECTIVE: The assessment of propofol to produce diurnal sedation in critically ill patients. DESIGN: Prospective clinical study. SETTING: Intensive Care Unit, University Hospital. PATIENTS AND PARTICIPANTS: Thirty consecutive patients admitted to the Intensive Care Unit older than 18 years who were expected to be sedated for more than 50 h. INTERVENTIONS: The patients were randomised into two groups. All received sedation with a constant background infusion of morphine and a variable infusion rate of propofol, which was altered hourly to maintain the intended sedation score. The first group received constant light sedation (CLS) over 50 h aiming for a Ramsay score of 2-3. The second group received CLS between 0600 h and 2200 h and additional night sedation (ANS) with propofol between 2200 h and 0600 h, aiming for a sedation score of 4-5. MEASUREMENTS AND RESULTS: Patients were studied for 50 h from 1800 h on the first day of admission. Recordings of heart rate, blood pressure, sedation scores and propofol and morphine infusion rates were made hourly. An APACHE II score was recorded for each patient. Sedation scores were analysed by blind visual assessment and cosinor analysis, which is used in chronobiology to examine the correlation of data with a cosine curve. Patients in the ANS group had significantly better rhythmicity of sedation levels using cosinor analysis (r = 26% v 8%) p < 0.01. There was no difference between the CLS and ANS groups with respect to age, sex or APACHE II scores. Nine out of 15 patients in the ANS group achieved diurnal sedation. Three patients in the CLS group showed diurnal rhythmicity of sedation, which can be attributed to natural sleep, and had a median APACHE II score of 12. Five patients in the CLS group and three in the ANS group showed a deep constant sedation pattern. They had high APACHE II scores (median 21.5) and an obtunded conscious level on admission due to severe sepsis. CONCLUSION: Propofol can safely provide diurnal sedation in the critically ill when titrated against the Ramsay score. Sedation levels cannot be manipulated in some severely ill patients.
OBJECTIVE: To investigate the concentrations of triglyceride, cholesterol, and high-density lipoprotein during a 50-hr infusion of 2% propofol, starting within 24 hrs of admission to the intensive care unit (ICU). DESIGN: Prospective, clinical study. SETTING: ICU, university hospital. PATIENTS: Thirty adult patients, who were ventilated and expected to be sedated for >2 days, were studied for 50 hrs, beginning at 1800 hrs on the first day of ICU admission. MEASUREMENTS AND MAIN RESULTS: Triglyceride, cholesterol, and high-density lipoprotein were measured at 2000, 0400, and 0800 hrs. Tumor necrosis factor (TNF)-alpha, interleukin (IL)-6, and C-reactive protein were measured at 2000 hrs. Median cholesterol and high-density lipoprotein concentrations were at the low end of the normal range. In seven patients, peak triglyceride concentrations were >3 mmol/L up to a maximum of 4.83 mmol/L. Although there was no statistical difference in lipid concentrations between days 1 and 2, there was an apparent pattern of increasing triglyceride concentrations. There was a correlation between peak triglyceride concentration and total propofol consumption, but there was no correlation between lipids and age, gender, or Acute Physiology and Chronic Health Evaluation II scores. There was a direct correlation between triglyceride and C-reactive protein concentrations, and an inverse correlation between cholesterol and C-reactive protein. Twenty-two patients had evidence of TNF and 11 patients had an IL-6 of >1000 pg/mL, but there was no relationship between concentrations of cytokines and triglycerides in plasma. CONCLUSIONS: Infusion of 2% propofol to critically ill patients over a 50-hr period does not result in a significant increase in triglyceride concentrations. Mean cholesterol and high-density lipoprotein concentrations were low throughout the study period. There was a significant direct correlation between triglyceride and C-reactive protein and an inverse correlation between cholesterol and C-reactive protein, suggesting that the changes in lipids in critically ill patients may be partly attributable to the acute-phase response.
The efficacy of a non-steroidal anti-inflammatory agent, ibuprofen, was evaluated in pain control following periodontal surgery. This type of agent acts peripherally by inhibiting the release of prostaglandins and minimizing the local inflammatory response. Thus there may be an advantage in pre-treatment administration of the drug so as to delay or even prevent postoperative pain. The study was multicentre, involving a Public Hospital Periodontal Unit, two specialist periodontal practices in Sydney, NSW, and two in Canberra, ACT. One hundred and twenty-seven patients who were to undergo periodontal surgery were randomly given either two 200 mg tablets of ibuprofen or two matching placebo tablets at least 30 minutes before administration of local anaesthesia. The procedure was double blind: neither the patient nor the clinician was aware of the tablet identity. Postoperatively, all patients were given labelled ibuprofen for pain relief, but were randomly divided into two groups: As directed who were instructed to take the drug regularly for two days postoperatively, and As required, who were to take the drug only if needed for pain relief. All patients completed a diary recording quantity and time of medication, and regular assessment of pain experience utilizing a visual analogue scale. The As directed group showed no significant difference in pain experience between pre-operative and post-operative only medication, but the As required group experienced significantly less pain and requirement for medication if the ibuprofen was administered pre-operatively.
Explore the source record for details and available documents.
Increasingly, proteins are delivered to the respiratory tract as an aerosol, and clinical efficacy is dependent on optimal delivery of the protein in an intact form. The object of this study was to compare the in vivo and in vitro results of two aerosol delivery systems for the aerosolization of recombinant human deoxyribonuclease I (rhDNase) in patients with cystic fibrosis (CF). Patients with CF who were to be initiated on rhDNase were randomized either to the Hudson nebulizer and Pulmo-Aide compressor or to the Sidestream nebulizer driven by the CR50 air compressor. An in vitro study was performed in six sets of the two aerosol delivery systems. One hundred and seventy three patients were randomized in this open study, where rhDNase was administered for 7 days. Improvements in pulmonary function were observed in both groups following 1 week of therapy with rhDNase. Changes in the Sidestream/CR50 and Hudson/Pulmo-Aide groups, respectively, were: 16 and 11% for forced expiratory volume in one second (p=0.14); 12 and 10% for forced vital capacity (p=0.70); and 14 and 7% for forced expiratory flow at 25-75% of expiration (FEF(25-75)) (p=0.18). A greater proportion of patients in the Sidestream/CR50 group (58%) had a >10% response in FEF(25-75) compared to the Hudson/Pulmo-Aide group (42%; p=0.03). The Sidestream nebulizer had a faster nebulization rate (p<0.05), lower mass median diameter for the aerosol mass produced (p<0.001), higher percentage of particles in the respirable range (p<0.001) and greater respirable output (p<0.005), compared to the Hudson nebulizer. The Sidestream/CR50 combination is a quicker, more efficient system in vitro than the Hudson/Pulmo-Aide combination, whereas the in vivo study only suggested a difference. Clinically, the two systems have similar efficacy.