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Biomedical subjects

Robert Dinwiddie

Publications and source records attributed to Robert Dinwiddie.

8 recordsLinked to original sources
PubMed2005

Cystic fibrosis and Russell-Silver syndrome in a child with maternal isodisomy of chromosome 7.

A 6-year-old Asian girl was diagnosed with cystic fibrosis at 3 months of age, following investigations for failure to thrive. She had intrauterine growth retardation and continued to have restricted postnatal growth, despite adequate caloric intake and enzyme replacement therapy. Further investigations were initiated when she was 5 years old, as her growth was not responding to the usual treatment measures. These tests revealed that she had maternal isodisomy of chromosome 7.

Child↗
PubMed2005

Anti-inflammatory therapy in cystic fibrosis.

Inflammation plays a major role in the pathophysiology of lung disease in CF. This response is probably triggered primarily as a reaction to the inability of the affected lung to resist the invasion of the most common bacterial pathogens seen in this disease, namely, Staphylococcus aureus, Haemophilus influenzae and Pseudomonas aeruginosa. Debate continues as to whether there may or may not be a pre-inflammation of the lungs as part of the basic functional defect of CFTR. The anti-inflammatory treatment modalities most tested to date are: oral corticosteroids, effective but associated with significant long-term side effects, inhaled corticosteroids, so far not proven to be effective probably because of difficulty with absorption through the viscid surface secretions of the lung and ibuprofen, potentially effective but inhibited by the need to monitor drug levels invasively and potential gastrointestinal side effects. The most promising newcomer is macrolide antibiotics such as azithromycin acting as a long-term anti-inflammatory agent with an excellent safety profile.

Administration, Inhalation↗
PubMed2005

Systemic diseases and the lung.

Systemic diseases affecting the lung are fortunately relatively rare in paediatric practice. A number of conditions do, however, cause significant respiratory complications, which can result in serious morbidity and mortality in this age group. These include connective tissue disorders such as systemic lupus erythematosus, dermatomyositis and scleroderma, inherited connective tissue disorders such as Ehlers-Danlos and Marfan's syndrome, lysosomal storage disorders such as mucopolysaccharidoses, familial dysautonomia, Langerhans cell histocytosis, pulmonary lymphangiomatosis, sarcoidosis and sickle cell disease. The investigations of these conditions are often complex but form part of the overall multisystem review of each individual patient. Treatment is individualised but often requires the extended use of corticosteroids and other immunosuppressants. The outcome is variable and depends on the ability to control the underlying condition. Long-term chronic lung damage is not unusual and these diseases, when they affect the lung, carry a small but significant mortality.

Anemia, Sickle Cell↗
PubMed2004

The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis.

This study aimed to investigate the evolution of airway function in infants newly diagnosed with cystic fibrosis (CF). FEV(0.5) was measured soon after diagnosis (median age of 28 weeks) and 6 months later in subjects with CF and on two occasions 6 months apart (median ages of 7.4 and 33.7 weeks) in healthy infants, using the raised-volume technique. Repeated measurements were successful in 34 CF and 32 healthy subjects. After adjustment for age, length, sex, and exposure to maternal smoking, mean FEV(0.5) was significantly lower in infants with CF both shortly after diagnosis and at the second test, with no significant difference in rate of increase in FEV(0.5) with growth between the two groups. When compared with published reference data, FEV(0.5) was reduced by an average of two z scores on both test occasions in those with CF, with 72% of individuals having an FEV(0.5) of less than 1.64 z-scores (i.e., less than the fifth percentile) on one or both test occasions. On longitudinal analysis, subjects with CF experienced a mean (95% confidence interval) reduction in FEV(0.5) of 20% (11, 28). Airway function is diminished soon after diagnosis in infants with CF and does not catch up during infancy and early childhood. These findings have important implications for early interventions in CF.

Body Height↗
PubMed2004

Congenital upper airway obstruction.

Most causes of upper airway obstruction are rare in the neonatal period and during infancy. They may, however, cause major respiratory problems either initially or during the first few weeks of life. It is important to recognise these problems at an early stage so that appropriate measures to overcome airway obstruction can be initiated, thus avoiding significant hypoxia-related complications. Specific treatment includes the use of nasal stents, a nasopharyngeal airway or, in severe cases, tracheostomy. Many such infants have associated feeding problems secondary to the airway obstruction and associated swallowing difficulties, which can lead to recurrent aspiration. The anomalies seen in clinical practice are best classified anatomically. Treatment is aimed at overcoming the mechanical effects of airway obstruction and maximising nutritional input so as to promote growth of the airway and long-term recovery of normal upper airway function.

Airway Obstruction↗
PubMed2003

An alternative to lung transplantation.

Lung volume reduction surgery (LVRS) has been used increasingly in adults for treatment of breathlessness caused by severe emphysema.1 It is of particular benefit to patients with a heterogenous anatomic distribution of emphysema, with obvious target areas for resection,2 as it allows an improved chance of reclaiming function from surrounding compressed lung.3 We report on an 8-year-old male with obliterative bronchiolitis in whom LVRS has been used as a measure to significantly improve quality of life and avoid the immediate need for lung transplantation.

Bronchiolitis Obliterans↗
PubMed2003

Primary thoracoscopic treatment of empyema in children.

OBJECTIVE: The optimal treatment of pediatric empyema remains controversial. The objective of this study is to compare the use of conventional management versus primary thoracoscopic drainage and decortication in children with empyema. METHODS: Conventional management has consisted of chest drain insertion under general anesthesia plus intravenous antibiotics. Thoracoscopic drainage and decortication has consisted of primary thoracoscopic drainage and decortication plus antibiotics. The clinical course of 54 patients treated conventionally between 1989 and 1997 was compared with that of 21 patients treated by means of thoracoscopic drainage and decortication between September 2000 and September 2001. RESULTS: Results of the study demonstrated that patients in the drainage-decortication group had fewer invasive interventions per patient than those in the conventional management group (1.0 vs 1.26). Patients undergoing thoracoscopic drainage and decortication also had significantly shorter durations of intravenous antibiotic therapy (7.6 +/- 1.2 vs 18.2 +/- 7.5 days), chest tube drainage (4.0 +/- 0.5 vs 10.2 +/- 6.1 days), and hospital stays (7.4 +/- 0.8 vs 15.4 +/- 7.4). Moreover, there were no open thoracotomies and decortications in the thoracoscopic drainage and decortication group, whereas in the conventional management group 39% (21/54) of patients underwent an open procedure. CONCLUSION: Although the 2 groups were not prospectively randomized and they were treated in different time periods, the results of this study support the use of thoracoscopic surgery as the primary therapeutic modality in children presenting with pleural empyema. This strategy appears to offer significant benefits over conventional treatment in terms of duration of treatment and the need for more invasive surgery.

Chest Tubes↗