Perioperative fluid therapy in children.
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Biomedical subjects
Publications and source records attributed to M G Coulthard.
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The AXIN1 gene has been implicated in caudal duplication anomalies. Its coding region was sequenced in both members of a monozygotic (MZ) twin pair discordant for a caudal duplication anomaly, but no mutation was found. Using bisulfite sequencing, we examined methylation at the promoter region of the AXIN1 gene in these twins and in twin and age-matched singleton controls. Methylation of the promoter region in peripheral blood mononucleated cells was variable among individuals, including MZ pairs. In the MZ pair discordant for the caudal duplication, this region of the affected twin was significantly more methylated than that of the unaffected twin (P < .0001), which was significantly more methylated than those of the controls (P = .02). We have confirmed that this CpG island does function as a promoter in vitro and that its activity is inversely proportional to the extent of methylation. This finding raises the possibility that hypermethylation of the AXIN1 promoter, by mechanisms as yet undetermined, is associated with the malformation. This case may be paradigmatic for some cases of MZ discordance.
The use of central venous catheters, while advantageous, is associated with a range of complications including thrombosis and infection. These complications can pose significant physical and financial costs to the patient and health care system. A critical appraisal of the two randomized controlled trials examining this topic in critically ill patients has shown that heparin-bonded central venous catheters significantly reduced the incidence of catheter-related thrombosis and infection in children and adults. These findings suggest that heparin-bonded central venous catheters should be considered for routine use in critically ill patients.
Moderate antenatal renal pelvic dilation (5-15 mm) may suggest vesicoureteric reflux, but it is not known to predict renal scarring. Dimercaptosuccinic acid scans on such children aged over 4 years showed a scarring rate (0/133 boys, 1/56 girls) similar to our local population. Investigation and treatment of moderate dilation may not be required.
Malakoplakia typically affects the bladders of immunocompromised adults who have defective intracellular killing of Escherichia coli. Renal malakoplakia is rare in children and generally has a good outcome. In the case presented, however, it caused end stage renal failure in a 5 year old girl. The management dilemmas surrounding renal transplantation are highlighted.
An 11 year old girl developed hypertensive encephalopathy and renal failure from reflux nephropathy. Resection of her shrunken left kidney did not control her hypertension. Two selective arterial embolisations of the scarred right lower pole produced only transient benefit, but a heminephrectomy gave good control. Embolisation may delay definitive treatment.
A girl with failure to thrive and a haemoglobin of 140 g/l at 1.3 years died from a brain haemorrhage 2.5 years later. Renal artery stenosis had caused severe, chronic hypertension and increased erythropoietin secretion (haemoglobin 182 g/l). Blood pressure should be measured in all unwell children, including those failing to thrive.
AIMS: To determine the outcome of children who reach end stage renal failure before the age of 2 years. METHODS: Using a retrospective questionnaire, 10 years' data were collected from the paediatric nephrology units in Britain and Ireland (1988 to 1997, follow up 1.3-11.5 years). RESULTS: A total of 192 children were identified; 0.31/million/year. Most had congenital or inherited conditions, and there were more boys. Latterly, half were diagnosed antenatally. Ninety per cent were dialysed initially, most using home peritoneal cyclers, some by haemodialysis through central lines. Five per cent recovered sufficient function to come off dialysis. Most required tube feeding (often gastrostomy) and erythropoietin; some needed growth hormone. A total of 56% received a transplant (2% without prior dialysis) at (medians) 2.6 years and 12.3 kg. The 2 and 10 year survival of first kidneys was 78%. Growth improved following transplantation. Fourteen per cent died because treatment was not started or was withdrawn. Most had particularly complex renal conditions, or additional major non-renal diagnoses. Typically, decisions not to treat were made mutually between clinicians and families. When treatment was continued, 71% survived, and few had serious non-renal conditions. Most attended normal schools, and by 6 years of age, less than 10% still required dialysis. Infants starting treatment under and over 1 month of age fared equally well. CONCLUSIONS: By school age, most infants treated for end stage renal failure will have a functioning transplant, reasonable growth, and will attend a normal class, regardless of the age at which they commence treatment. Treatment is seldom sustained in children who have serious additional medical conditions. It is reasonable to treat infants with uncomplicated renal failure.
OBJECTIVE: To determine the type and frequency of emergencies in general practice, and the extent to which general practices are equipped to appropriately respond to emergencies. DESIGN: Random-sample, cross-sectional questionnaire survey of general practitioners, October 1999 - March 2000. SETTING: General practices in south-east Queensland. PARTICIPANTS: 512 of 900 eligible GPs in current clinical practice. MAIN OUTCOME MEASURES: The type and frequency of medical emergencies presenting to GPs, and descriptive details of emergency drugs and equipment available in their practices. RESULTS: 512 GPs (response rate, 57%) reported managing a cumulative total of 5640 emergencies over the preceding 12 months. Non-metropolitan GPs saw about 30% more emergencies than their metropolitan counterparts (median, 9 and 7, respectively; P=0.02). The most common emergencies (seen by more than 30% of all GPs) were acute asthma, psychiatric emergencies, convulsions, hypoglycaemia, anaphylaxis, impaired consciousness, shock, poisoning and overdose. Most GPs (77%) stocked 15 or more of the 16 emergency doctor's bag drugs, but a smaller proportion (67%) had all of the basic emergency equipment items considered essential. CONCLUSIONS: A substantial number of patients with potentially life-threatening emergencies present to GPs. Doctor's bag emergency drugs are available in most general practices, but availability of basic emergency equipment is suboptimal.
Haemodialysing small infants is difficult because of vascular access limitations. We show that Poiseuille's law (that flow through a tube varies with its radius4) makes it inevitable that the blood flow that can be achieved in smaller patients will fall disproportionately compared to their need for dialysis. Poiseuille's law also predicts that for single and multiple lumen cannulae of the same outside gauge, blood flow through the single lumen will be several times greater. Measurements confirmed this. It is argued that haemodialysis efficiency will therefore be improved by using a single lumen cannula to alternately withdraw and return blood, compared to sampling and returning continuously through a multiple lumen cannula, despite only withdrawing for half the time.
This paper reports the introduction of an evidence-based medicine fellowship in a children's teaching hospital. The results are presented of a self-reported 'evidence-based medicine' questionnaire, the clinical questions requested through the information retrieval service are outlined and the results of an information retrieval service user questionnaire are reported. It was confirmed that clinicians have frequent clinical questions that mostly remain unanswered. The responses to four questions with 'good quality' evidence-based answers were reviewed and suggest that at least one-quarter of doctors were not aware of the current best available evidence. There was a high level of satisfaction with the information retrieval service; 19% of users indicated that the information changed their clinical practice and 73% indicated that the information confirmed their clinical practice. The introduction of an evidence-based medicine fellowship is one method of disseminating the practice of evidence-based medicine in a tertiary children's hospital.
Though hypertension is a common feature of Henoch: Schönlein purpura (HSP) acute nephritis, it is seen only rarely and transiently if renal function is normal and abnormal urinary findings are minimal. We report a 3.4 year old girl who had the typical arthritis and rash of HSP, but also had significant hypertension with only minimal urinary findings. Imaging investigations revealed one normal kidney, and one that was almost destroyed from previous reflux nephropathy; her hypertension resolved after unilateral nephrectomy. Other causes of hypertension should be excluded in children with HSP and minimal urinary findings.
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The Eph family of receptor tyrosine kinases plays a crucial role during development and is implicated in oncogenesis. Using a partial cDNA clone of an Eph-related kinase (Esk) we isolated the complete coding region of a gene which we show to be murine EphA1 by both structural and functional criteria. The chromosomal localization is shown to be syntenic to hEphA1 and the genomic organization also shows distinct features found in the hEphA1 gene. Functionally, in keeping with findings for the human homologue, both soluble recombinant and "native" mEphA1 show preferential binding to ephrin A1. However, we also observed significant binding to other A-type ligands as has been observed for other Eph receptors. We analysed the expression of mEphA1 mRNA by in situ hybridization on tissue sections. mEphA1 was expressed in epithelial elements of skin, adult thymus, kidney and adrenal cortex. Taken together with previous Northern blotting data these results suggest that mEphA1 is expressed widely in differentiated epithelial cells.
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Thirty-eight children (aged 2-16 years) attending a regional kidney unit had a full clinical and radiological dental examination. Twenty had previously undergone a renal transplant, 11 had chronic renal failure and 7 had other renal diseases. Periodontal disease was uncommon The presence of gingival hyperplasia (gum overgrowth), as recorded in 22 of the children, did not show any relationship with the use of immunosuppressant therapy. However, gingival overgrowth was so excessive in 2 patients that surgical removal was required. The prevalence of dental caries was low. Enamel defects were common, and of an unusual pattern, with a much higher prevalence of diffuse opacities and enamel hypoplasia than in the normal child population, 83% and 22%, respectively. This increased prevalence is probably due to disordered calcium and phosphate metabolism. The prevalence of these defects may reflect an early onset of renal disease, since there were a number of very young children in the programme. Dental and medical care should be closely integrated for children with renal disease to avoid the undesirable dental sequelae of, in particular, gingival overgrowth, carcinoma and enamel hypoplasia.
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