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Biomedical subjects

D A Spencer

Publications and source records attributed to D A Spencer.

At least 19 recordsLinked to original sources
PubMed2005

The effects of intravenous tobramycin on renal tubular function in children with cystic fibrosis.

BACKGROUND: Tobramycin, used to treat respiratory exacerbations in cystic fibrosis (CF), is also a renal tubular toxin. Tubular dysfunction leads to increased urinary levels of the proximal tubular lysosomal enzyme, N-acetyl-beta-D-glucosaminidase (NAG) and the proximal tubular protein, retinol-binding protein (RBP). Hypermagnesuria and resulting hypomagnesaemia are indicative of more severe tubular damage, occasionally seen following repeated courses of intravenous tobramycin. Using these biochemical markers we studied the effect of a 2-week course of this agent on tubular function. METHODS: Twenty-two children (11 boys) with CF were studied. Median age = 10.9 years, range 3.1-16.4 years. All had a normal predicted glomerular filtration rate (pGFR). They received tobramycin 3 mg/kg/dose tds. Urinary NAG, RBP, creatinine and plasma magnesium and creatinine were assayed: a) immediately before commencing tobramycin, b) immediately following the course, c) 4 weeks after the end of the course. RESULTS: Mean log UrNAG and UrRBP rose significantly between time points a) and b) before falling to almost pre-treatment levels by time c). Using two way ANOVA analysis the results for UrNAG and UrRBP were both highly statistically significant (p<0.0001). Paired t-tests on the logged values revealed highly significant differences between all time points for UrNAG and in the case of UrRBP for all other than a) compared to c). In all patients plasma magnesium and pGFR remained within normal limits. CONCLUSIONS: Intravenous tobramycin produces acute tubular injury, which showed evidence of almost complete recovery after 4 weeks. The insult to the tubules was not sufficient to produce hypomagnesaemia in our study group. To assess cumulative tubular damage in more detail it would be necessary to repeat this study after further courses of tobramycin. We recommend monitoring plasma magnesium during courses of intravenous tobramycin.

Acetylglucosaminidase↗
PubMed2005

From hemp seed and porcupine quill to HRCT: advances in the diagnosis and epidemiology of bronchiectasis.

Over the last decade there has been a significant improvement in our ability to recognise non-cystic fibrosis (CF) bronchiectasis in children. The precise incidence is uncertain, and it varies greatly depending on the populations studied and the methods used to make the diagnosis. It is unlikely that many of the underlying causes of non-CF bronchiectasis will be eradicated in the near future, and so it must be expected that with ever improving technology this diagnosis will be made with increasing frequency. This emphasises the need to improve our understanding of the aetiology, pathophysiology, epidemiology, and management options for children with this group of conditions.

Bronchiectasis↗
PubMed2005

Postmortem diagnosis of chronic granulomatous disease: how worthwhile is it?

A previously healthy 11 year old boy died unexpectedly after a rapid course of progressive pneumonia. Postmortem microbiology and histopathology suggested an underlying diagnosis of chronic granulomatous disease. This was confirmed by neutrophil oxidative burst and gene mutation analysis of other family members, one of whom benefited from early bone marrow transplantation.

Burkholderia Infections↗
PubMed2004

The need to redefine non-cystic fibrosis bronchiectasis in childhood.

BACKGROUND: Non-cystic fibrosis (CF) bronchiectasis has previously been reported to be rare and progressive in children living in western societies. METHOD: A clinical and radiological review was undertaken of 93 children with non-CF bronchiectasis defined by high resolution computed tomographic (HRCT) scanning presenting to a tertiary paediatric respiratory centre since 1996. RESULTS: Cases constituted 9.6% of all new referrals. Male to female ratio was 2:1. Median age at symptom onset was 1.1 years (range 0-16) and of HRCT diagnosis was 7.2 years (1.6-18.8). The most common referral diagnosis of asthma was refuted in 39 of 45 cases. Associations were previous pneumonic illness (30%), immunocompromise (21%), obliterative bronchiolitis (9%), congenital lung abnormality (5%), chronic aspiration (3%), eosinophilic oesophagitis (2%), familial syndrome (2%), primary ciliary dyskinesia (1%), and right middle lobe syndrome (1%). 8% had two associated diagnoses and 18% were idiopathic. There was agreement between the chest radiograph and HRCT scan for diagnosis and lobe affected in only five cases (5%). A repeat HRCT scan in 18 cases at a minimum interval of 18 months showed total resolution of the changes in six, improvement in one, progression in five, and was unchanged in six. CONCLUSIONS: Radiologically defined non-CF bronchiectasis in children is not uncommon. Diagnostic delay is a problem. The most common association is a previous pneumonia. Chest radiography is of little diagnostic value, but resolution is possible on HRCT scanning. Bronchiectasis is currently defined as a condition which is both permanent and progressive. This term is not necessarily appropriate for all paediatric patients for whom we suggest an alternative nomenclature.

Adolescent↗
PubMed2004

Clinical features, aetiology and outcome of empyema in children in the north east of England.

BACKGROUND: The incidence of empyema in children in the UK is increasing. The reason for this is unclear. A prospective study was undertaken to investigate the clinical features, aetiology, and outcome of cases of empyema and parapneumonic effusion presenting to a tertiary paediatric respiratory centre between February 1997 and August 2001. METHOD: Routine bacterial culture of blood and pleural fluid was performed for 47 cases. Forty three pleural fluid specimens, culture negative for pneumococcus, were analysed for pneumococccal DNA by real time polymerase chain reaction (PCR). Penicillin susceptibility was determined for DNA positive specimens using complementary PCR assay. Capsular serotype specific antigen detection was by enzyme immunoassay (EIA) using monoclonal antibodies to serotypes 1, 3, 4, 5, 6A, 6B, 7F, 9V, 14, 18C, 19A, 19F, and 23F. Clinical data were obtained from patient notes, supplemented by a postal questionnaire. RESULTS: The median (range) age of the patients was 5.6 (0.6-16.9) years and 70% were male. The median (range) duration of illness before referral to hospital was 5 (0-25) days. Forty five (96%) had received antibiotics before referral; 32 (68%) required decortication and eight (21%) thoracocentesis. Median postoperative stay was 4 days (2-8). Thirty two (75%) pneumococcal culture negative specimens were pneumococcal DNA positive; 17 (53%) of these were serotype 1. All were penicillin sensitive. CONCLUSIONS: Pneumococcus is the major pathogen in childhood empyema and serotype 1 is the prevalent serotype. This has implications for vaccine development and immunisation strategy as the current 7-valent pneumococcal conjugate vaccine does not protect against serotype 1.

Adolescent↗
PubMed2003

Comparison of lung deposition of colomycin using the HaloLite and the Pari LC Plus nebulisers in patients with cystic fibrosis.

AIM: To examine the effectiveness of delivery of nebulised colistin by the HaloLite nebuliser compared to the Pari LC Plus in patients with cystic fibrosis. METHODS: Randomised crossover trial of 15 patients aged >6 years. Inhalation of one mega unit of colistin in 3 ml diluent, labelled with technetium-99m DTPA, was used to assess lung deposition. The Pari was nebulised to dryness and one button press of the HaloLite was completed. Following a seven day washout period, patients inhaled colistin twice daily for seven days through the first device. Sputum specimens were analysed for colistin levels and pseudomonas load. This procedure was repeated with the alternative device. RESULTS: Lung uptake of radiolabelled colistin was significantly higher with the Pari. However, lung uptake calculated as a percentage of the amount of drug used was significantly higher for the HaloLite. Time to nebulise was significantly shorter with the HaloLite. Sputum levels of colistin were higher following use of the Pari; this was close to significance. CONCLUSION: The manufacturer's recommended dosages for nebulising antibiotics with a HaloLite result in a lower delivery than patients receive when using a Pari nebuliser. The concept of adaptive aerosol delivery has several theoretical advantages but the recommended doses for the HaloLite need to be modified in order to improve effectiveness.

Administration, Inhalation↗
PubMed1999

Aminoglycoside-associated hypomagnesaemia in children with cystic fibrosis.

Hypomagnesaemia in children with cystic fibrosis (CF) is under-recognized. We report a child with CF who developed significant hypomagnesaemia following intravenous (i.v.) treatment with aminoglycosides for exacerbations of Pseudomonas aeruginosa infection. Three additional cases have also been observed. Investigations in two patients have revealed excessive renal loss of magnesium. It is postulated that renal tubular damage secondary to the cumulative effects of repeated courses of aminoglycosides resulted in hypomagnesaemia, and we suggest screening for this problem by monitoring serum magnesium regularly in all patients with CF receiving multiple courses of aminoglycosides.

Acute Disease↗
PubMed1998

Empyema thoracis: a role for open thoracotomy and decortication.

BACKGROUND: Thoracentesis and antibiotics remain the cornerstones of treatment in stage I empyema. The management of disease progression or late presentation is controversial. Open thoracotomy and decortication is perceived to be synonymous with protracted recovery and prolonged hospitalisation. Advocates of thoracoscopic adhesiolysis cite earlier chest drain removal and hospital discharge. This paper challenges traditional prejudice towards open surgery. METHODS: A five year audit of empyema cases referred to a regional cardiothoracic surgical unit analysing previous clinical course, surgical management, and outcome. RESULTS: Between February 1992 and February 1997, the number of referrals to this centre increased dramatically. Twenty-two children were referred for surgery (15 boys, seven girls; age range, 0.5-16 years). Before referral, patients had been unwell for 6-50 days (median, 15), had been treated with several antibiotics, and had undergone chest ultrasound (15 patients), computed tomography (five patients), pleural aspiration attempts (13 patients), and intercostal drainage (seven patients). The organism responsible was identified in only two cases (Streptococcus pneumoniae). Three patients had intraparenchymal abscess formation. Eighteen patients underwent open thoracotomy and decortication. Drain removal was performed on the first or second day. Fever resolved within 48 hours. Median hospital stay was four days. All patients had complete clinical and radiological resolution. CONCLUSIONS: Treatment must be tailored to the disease stage. In stage II and III diseases, open decortication followed by early drain removal results in rapid symptomatic recovery, early hospital discharge, and complete resolution. In the early fibrinopurulent phase, alternative strategies should be considered. However, even in ideal cases, neither fibrinolysis nor thoracoscopic adhesiolysis can achieve more rapid resolution at lower risk.

Adolescent↗
PubMed1996

Combined percutaneous lung biopsy and high-resolution computed tomography in the diagnosis and management of lung disease in children.

Computed tomography-guided percutaneous lung biopsy is a well-recognized procedure for obtaining tissue for diagnosis in adults with interstitial lung diseases. Recently this methodology has been extended to pediatric practice. We have further refined this technique by employing high-resolution computed tomography (HRCT) under general anesthesia to obtain maximum anatomical detail. High-quality images are obtained that accurately define the extent of disease, and percutaneous biopsies are then taken from a suitable area of radiological abnormality using an 18G Monopty needle. Twenty-six investigations have been performed on 24 patients. The diagnosis was established from 14 biopsies, and histological and/or radiological information that contributed to patient management was obtained from a further 4 procedures. In 4 patients the histological findings were inconclusive, and the final diagnosis was only confirmed by open lung biopsy and/or other investigations. The procedure was generally well tolerated, although chest drainage for pneumothorax was required in two patients. HRCT-guided percutaneous lung biopsy is a useful initial approach to the diagnosis of interstitial lung disease in selected patients; the necessity of more invasive procedures such as open, thoracoscopic, or transbronchial lung biopsy can thus generally be avoided.

Adolescent↗
PubMed1995

Clinical benefit from nebulized human recombinant DNase in Kartagener's syndrome.

Nebulized recombinant human DNase (rhDNase) reduces sputum viscosity, improves pulmonary function, and results in a small reduction in acute respiratory exacerbations requiring intravenous antibiotics in many patients with cystic fibrosis (CF). rhDNase is now recommended for use in CF patients with moderately severe suppurative lung disease. A 14-year-old girl with suppurative lung disease [forced expiratory volume in 1 second (FEV1) 69% and forced vital capacity (FVC) 81% predicted] secondary to Kartagener's syndrome and severe gastroesophageal reflux had worsening spirometry together with intractable gastrointestinal symptoms over the previous 18 months despite conventional treatment. She was, therefore, started on 2.5 mg rhDNase once daily. Her cough lessened and the volume of sputum decreased within 72 hours of commencement of treatment; this improvement was strongly associated with a dramatic reduction in gastrointestinal symptoms. Spirometry after 4 weeks of treatment demonstrated a 20% improvement in FEV1 and a 13% improvement in FVC. These improvements have been maintained after 4 months of rhDNase therapy. The use of rhDNase should be considered in patients with Kartagener's syndrome and a multicenter trial may be justified.

Administration, Inhalation↗
PubMed1995

Carbohydrate deficient glycoprotein syndrome; multiple abnormalities and diagnostic delay.

A 3 week old boy presented with abnormal thyroid function, and was treated with thyroxine. He developed multisystem disease including deafness and nephrotic syndrome, and died aged 3 months. Carbohydrate deficient glycoprotein syndrome (CDGS) was diagnosed post-mortem. CDGS should be considered in all infants with apparently unrelated multiple clinical or biochemical abnormalities.

Abnormalities, Multiple↗