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Biomedical subjects

R Dinwiddie

Publications and source records attributed to R Dinwiddie.

At least 19 recordsLinked to original sources
PubMed2005

Growth and lung function in Asian patients with cystic fibrosis.

BACKGROUND: The incidence of cystic fibrosis (CF) in Asians is rare. How these patients fare in terms of morbidity and mortality in the UK compared to their non-Asian peers is not well documented. AIMS: To retrospectively study annual reviews of 31 Asian CF patients from three London paediatric CF centres. METHODS: Disease severity was assessed by lung function, age at first infection with Pseudomonas aeruginosa, and body mass index (BMI). The Asian children were compared with 143 matched non-Asian patients with CF. Matching criteria used were same sex and treatment centre as the Asian index patient. In addition, the controls were matched so that their date of birth, date of diagnosis, and date at annual review were within 12 months of the index patient. RESULTS: There was no significant difference in age at diagnosis or age at annual review between the Asian and non-Asian children. Mean Z-scores for FEV1 and FVC were significantly lower for the Asian girls. There was no significant difference in Z-scores for BMI between the Asian children and their controls. Age at first isolation of Pseudomonas aeruginosa in Asian girls was significantly later than for their controls (8.3 years compared to 5.6 years for non-Asian girls). CONCLUSIONS: While the Asian boys' lung function seems comparable with that of their non-Asian peers, the Asian girls emerge as a potentially vulnerable group and more work is required to discover why this is the case.

Adolescent↗
PubMed2005

The Chrispin-Norman score in cystic fibrosis: doing away with the lateral view.

The Chrispin-Norman chest radiograph scoring (CNS) system is widely used to assess respiratory disease progression in cystic fibrosis (CF). Frontal and lateral chest radiographs were performed. The present authors developed a modified CNS, which obviates the need for the lateral film. This study compares the original and the current authors' modified scoring system. A total of 50 chest radiographs from CF children, taken between August and December 2003, were scored according to the original and modified CNS. Two observers scored all 50 chest radiographs, scoring in random order the frontal radiographs, and separately the frontal and lateral radiographs together. There was no evidence of a difference between the methods for either observer, using the Bland and Altman 95% limits of agreement as follows: observer 1 (-2.0-1.9), and observer 2 (-1.77-2.2). No evidence of a difference between the observers for either method was found, comparing the 95% limits of agreement (-5.5-5.7) with the modified CNS (-5.6-6.4). In conclusion, in terms of the final score, good agreement was found between the use of the original and modified Chrispin-Norman score. In addition, low inter-observer variability was shown for both methods. The use of the modified Chrispin-Norman chest radiograph scoring system to stage disease severity in cystic fibrosis removes the need for a lateral chest radiograph.

Adolescent↗
PubMed2004

Treatment of interstitial lung disease in children.

The treatment of interstitial lung disease in children depends on the nature of the underlying pathology. In approximately 50% of cases a specific aetiology can be found such as: chronic viral infection, an auto-immune process, sarcoidosis or alveolar proteinosis. In the remainder, the process is idiopathic and the pathological findings are based on the descriptive morphological features seen in the diagnostic lung biopsy. If a specific cause is found then targeted treatment with antivirals, steroids or other immunosuppressive agents is available. Alveolar proteinosis can be treated by bronchial lavage and GM-CSF. Idiopathic cases are treated primarily with intravenous pulsed methylprednisolone or oral prednisolone backed up hydroxychloroquine. Other immunosuppressive agents such as azathioprine, methotrexate or ciclosporin have been used successfully in individual patients. The prognosis is very variable and includes no response to any therapy, partial response with chronic long term morbidity, to virtually complete recovery. The overall mortality rate is 15%. There are no controlled therapeutic trials available because of the rarity of these conditions in childhood. Unlike in adult practice, no correlation has as yet been demonstrated between the initial pattern of chest x-ray change or the degree of pathological change on the lung biopsy and the clinical outcome. The recurrence rate within families is 1 in 8.

Child↗
PubMed2003

Primary pulmonary lymphangiectasis in a premature infant: resolution following intensive care.

Primary pulmonary lymphangiectasis is a rare disease, often described as fatal within the neonatal period. We report on an infant who developed severe bilateral chylous pleural effusions with computed tomography (CT) features of pulmonary lymphangiectasis. She recovered spontaneously with supportive treatment only. We discuss aspects of her intensive care which might have contributed to the good outcome, and conclude that modern intensive care may allow spontaneous recovery in conditions described in the literature as having a very poor outcome.

Female↗
PubMed2002

Idiopathic interstitial pneumonitis in children: a national survey in the United Kingdom and Ireland.

Interstitial lung disease (ILD) of unknown etiology in immunocompetent patients is rare in children. A national survey was carried out in the United Kingdom and Ireland over a 3-year period in order to identify prevalence, age distribution, histopathology, natural history of the illness, and response to current treatment.Forty-six cases were identified, including 29 males and 17 females. Seventy-six percent presented in the first year of life. Nine (16%) occurred within four families. Conventional treatment with pulsed methylprednisolone, prednisolone, or hydroxychloroquine, singly or in combination, resulted in an excellent response in 65% of cases. Seven children died (15%). The recurrence risk for further children within the same family to develop ILD is estimated to be approximately 10%. The prevalence rate of this condition in the United Kingdom and Ireland during the period of study for children aged 0-16 years is estimated to be 3.6 cases/million.

Adolescent↗
PubMed2001

Disordered breathing during sleep in patients with mucopolysaccharidoses.

OBJECTIVE: Obstructive sleep apnoea (OSA) has been reported as a feature of children with mucopolysaccharidoses (MPS). However, the incidence and severity of OSA with respect to disease type is poorly defined. The aim of the present study was to measure objectively the degree of OSA in a group of children with a range of MPS syndromes. METHODS: In a cross-sectional study, cardiopulmonary sleep studies were performed during unsedated sleep in 26 children with MPS over a period of 2 years. Scores of OSA severity based upon clinical history and upon objective sleep study data were made in each case and compared. RESULTS: OSA was present in 24/26 patients, and ranged in severity from mild to severe. OSA was most marked in MPS type IH (Hurler syndrome) followed by types IHS (Hurler--Scheie syndrome) and II (Hunter syndrome). Frequent arousals and poor sleep quality, not suspected clinically, were noted in several patients. There was agreement between the clinical and objective scoring systems in only 17/26 patients (65%) with clinical history scores tending to underestimate the most severe cases (5/26 cases) and overestimate the severity in the mild cases (4/26 cases). CONCLUSIONS: Obstructive respiratory problems are frequent in MPS patients and there are differences in severity of OSA between the different MPS types. Assessments of the severity of OSA based upon clinical history alone are inadequate. Our results suggest that objective sleep studies are necessary to evaluate these cases, to monitor clinical outcome and to assess the effects of therapeutic intervention. Prospective studies in larger numbers of patients are needed to validate these observations.

Adolescent↗
PubMed2001

Exercise tolerance in children with cystic fibrosis undergoing lung transplantation assessment.

The aim of this study was to compare the 6-min walk test against the recently developed 3-min step test, as measures of exercise tolerance in children with moderate to severe cystic fibrosis (CF) lung disease referred for lung transplantation assessment. Twenty-eight children with CF (16 girls, 12 boys), with a mean age of 13.7 yrs (range 7.2-17.8 yrs) and mean forced expiratory volume in one second of 34% predicted (range 17%-67%) were recruited. All subjects performed both the 6-min walk and 3-min step-tests. Outcome measures were maximum rise in heart rate (HR), and maximum fall in arterial oxygen saturation (Sa,O2). There was no significant difference in resting HR or Sa,O2 prior to starting the two tests. Both step and walk tests produced significant rises in median HR (from 114-149 min(-1), p<0.0005, and 119-138 min(-1), p<0.0005, respectively) and significant falls in Sa,O2 (both from 94-92%, p<0.0005). The step test produced a significantly greater percentage rise in HR (30% versus 18%, p<0.0005) and a significantly greater percentage fall in Sa,O2 (4% versus 2%, p=0.002). Bland-Altman analysis gave wide 95% limits of agreement (10.7-29.3% for rise in HR, -2.14.6% for fall in Sa,O2). The step test was well tolerated. The 3-min step test produced a greater fall in Sa,O2 and a greater rise in HR than the 6-min walk test in children with moderate to severe CF lung disease. It may be of value when assessing a child's suitability for lung transplantation.

Adolescent↗
PubMed2000

Interstitial lung disease in children: a multicentre survey on diagnostic approach.

Chronic interstitial lung disease (ILD) is a rare disorder in the paediatric age group, with a poor prognosis. The diagnostic approach to ILD is based on more or less invasive methods. This study was implemented to verify which methods are the most often used in children. Questionnaires (333) were sent to members of the European Respiratory Society Paediatric Assembly belonging to 187 European and non-European centres. Questions concerned the use of noninvasive diagnostic methods, e.g. history taking, physical examination, routine laboratory tests, respiratory function tests and radiology (chest radiography, high-resolution computed tomography (HRCT)), and the use of invasive techniques such as bronchoalveolar lavage (BAL), transbronchial biopsy (TBB), open lung biopsy (OLB), video-assisted thoracoscopic biopsy (VAT) and HRCT with fine-needle aspiration biopsy (FNAB). Thirty eight centres returned the questionnaires and 131 children with ILD were studied. A diagnosis of ILD was achieved in five (3.8%) patients using noninvasive techniques alone. Using the various biopsy methods, histological assessment was performed on a total of 98 (74.8%) children. The most frequently used invasive technique both alone and in combination was BAL (83, 63.3%), followed by OLB (64, 48.8%), TBB (26, 19.8%) and VAT (11, 8.4%); FNAB was used in one patient. In conclusion a diagnosis of interstitial lung disease was reached on the basis of aetiological and/or histological findings in 117 (89%) of the 131 patients studied.

Adolescent↗
PubMed2000

Treatment of pleural empyema.

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.

Anti-Bacterial Agents↗
PubMed2000

The lung in multi-system disease.

Lung involvement in multi-system disease is rare in children. When it does occur it is associated with significant morbidity and mortality. Auto-immune diseases such as systemic lupus erythematosis, scleroderma and acute rheumatic disease can cause major lung pathology. Chronic illness such as sickle cell disease, mucopolysaccharidosis or familial dysautonomia can result in long-term recurrent respiratory problems. Rare conditions such as sarcoidosis, pulmonary lymphangiomatosis or Langerhan's histiocytosis occasionally result in major lung disease in this age group. The prognosis for all of these conditions is very variable depending on the severity of the disease itself and its response to treatment which often involves the use of oral corticosteroids or other powerful immuno-suppressants.

Autoimmune Diseases↗
PubMed2000

Pathogenesis of lung disease in cystic fibrosis.

Lung disease in cystic fibrosis is primarily due to a defect in the cystic fibrosis transmembrane regulating protein (CFTR). This results in abnormal chloride transfer across epithelial membranes causing an excessively viscid mucus lining of the airways. Bacterial invasion particularly with Staphylococcus aureus, Haemophilus influenzae and Pseudomonas aeruginosa stimulates a vigorous and excessive primarily neutrophil-driven inflammatory response throughout the lungs. Products of this inflammation not only damage incoming bacteria but also the host tissue itself. Over a period of years this chronic suppurative process results in permanent ongoing lung destruction principally manifested as bilateral bronchiectasis.

Cystic Fibrosis↗
PubMed1999

The relative importance of socio-economic status, parental smoking and air pollution (SO2) on asthma symptoms, spirometry and bronchodilator response in 11-year-old children.

The aim of this study was to evaluate the relative contribution of several risk factors to the prevalence of allergic respiratory symptoms, and the positivity of the bronchodilator test with fenoterol, and to establish the relative importance of these factors on the variability of FVC, FEV1, PEF, MEF25, MEF50 and MEF75. A total of 340 11-year-old children attending school in polluted and non-polluted areas of the city of Cartagena, Spain, were studied. The polluted area had had an annual mean of 75 microg/m3 of SO2 over the last 10 years and the non-polluted area had < 20 microg/m3 during this period. A questionnaire about allergic respiratory symptoms was completed by the parents. Specific questions about parental smoking habits and socio-economic level were included. Each child's performance in spirometry before and after administration of 0.2 mg of inhaled fenoterol was evaluated. The only significant predictive variables in the logistic regression (for suffering any symptom or a positive bronchodilator response) were male sex for nasal symptoms (RR 1.37; p = 0.04) and housing near heavy traffic for eye symptoms (RR 1.45; p = 0.01). Living in the polluted area reduced the risk of a positive bronchodilator response (RR 0.61; p = 0.004). Maternal smoking, even though not statistically significant, tended to increased the risk of suffering any symptom (RR 1.26; p = 0.07) or of having a positive bronchodilator response (RR 1.23; p = 0.1). None of the risk factors studied was of significant importance in explaining the variability of spirometry results. Although none of the risk factors were specifically determinant to the symptom questions, bronchodilator test or spirometric measurements, having a mother who smokes seems more important than living in a polluted area if statistically non-significant trends are considered.

Adult↗