Biomedical subjects
Publications and source records attributed to J Wyllie.
This retrospective review of present practice of administration of adenosine by paediatricians shows that current guidelines recommend starting doses that are effective in only 9% of infants and children.
The effect of humidity on measurement of neonatal urine output was assessed by weighing nappies in a clinically relevant context. Saline was used as dummy urine, on modern nappies in incubators at various humidity settings. In at least some additional humidity, no clinically relevant evaporative loss occurred.
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The European Resuscitation Council (ERC) last issued guidelines for the resuscitation of the newly born infant in 1999 [1]. This was an "Advisory Statement" of the International Liaison Committee on Resuscitation (ILCOR). Following this, the American Heart Association and the Neonatal Resuscitation Programme Steering Committee of the American Academy of Paediatrics and representatives of the World Health Organisation, together with representatives from ILCOR, undertook a series of evidence-based evaluations of the science of resuscitation which culminated in the publication of "Guidelines 2000 for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care" in August 2000 [2,3]. The Paediatric Life Support Working Party of the European Resuscitation Council has considered this document and the supporting scientific literature and presents the ERC Newly Born Guidelines in this paper. Readers will find few changes to the ILCOR Advisory Statement recommendations as the new evidence that has emerged since its publication in 1999 has been confirmatory of the ILCOR recommendations.
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BACKGROUND: Tuberculosis is a re-emerging problem in the United Kingdom. BCG immunization administered in the neonatal period is protective. National guidelines and locally published standards identify infants for whom BCG immunization is recommended. The study aimed to calculate the rate of identification of infants 'at risk' by parental ethnic group and/or family history of tuberculosis, to determine subsequent immunization uptake, and to describe characteristics associated with missed BCG immunization. METHODS: A retrospective audit was conducted. Demographic data were collected from a computer database of antenatal booking data, for 2043 pregnancies delivering between 1 October 1998 and 30 April 1999. A cohort of infants 'at risk' was defined, and infants referred for BCG immunization were identified. A manual search of immunization records determined immunization uptake. RESULTS: A cohort of 247 (12 per cent pregnancies) was 'at risk'. Fifty-five per cent of the cohort 'at risk' was correctly identified and 42 per cent correctly identified and immunized. The largest subgroup of the cohort, 48 per cent, was Caucasian and at risk because of a positive family history of tuberculosis. Family history of tuberculosis was the most important risk factor, and was missed in 86 per cent of cases. CONCLUSIONS: Despite the local publication of established guidelines, 58 per cent of infants 'at risk' failed to be immunized. Family history of tuberculosis was more important than parental ethnic group in predicting risk for the cohort, and was missed in the majority of cases. Appropriate guidelines alone do not guarantee good practice. Guidelines should be introduced in conjunction with regular audit to ensure effective implementation.
AIM: To describe the clinical phenotype in infants with ARC syndrome, the association of arthrogryposis, renal tubular acidosis, and cholestasis. METHODS: The medical records for six patients with ARC syndrome were reviewed, presenting over 10 years to three paediatric referral centres. RESULTS: All patients had the typical pattern of arthrogryposis. Renal Fanconi syndrome was present in all but one patient, who presented with nephrogenic diabetes insipidus. Although all patients had severe cholestasis, serum gamma glutamyltransferase values were normal. Many of our patients showed dysmorphic features or ichthyosis. All had recurrent febrile illnesses, diarrhoea, and failed to thrive. Blood films revealed abnormally large platelets. CONCLUSIONS: ARC syndrome exhibits notable clinical variability and may not be as rare as previously thought. The association of Fanconi syndrome, ichthyosis, dysmorphism, jaundice, and diarrhoea has previously been reported as a separate syndrome: our observations indicate that it is part of the ARC spectrum.
An infant with ectrodactyly, glaucoma, cleft palate, congenital heart defect and genital anomalies associated with a 7(q21.2q31.2) deletion is presented. Glaucoma and ectrodactyly in association with a 7q deletion has not been previously reported. We recommend that early ophthalmological assessment is required in infants with such deletions.
AIMS: To establish the extent and type of premedication used before intubation in neonatal units in the United Kingdom. METHODS: A structured telephone survey was conducted of 241 eligible units. Units were subdivided into those that routinely intubated and ventilated babies (routine group) and those that transferred intubated and ventilated babies (transfer group). RESULTS: Of the units contacted, 239 (99%) participated. Only 88/239 (37%) gave any sedation before intubating on the unit and only 34/239 (14%) had a written policy covering this. Morphine was used most commonly (66%), with other opioids and benzodiazepines used less frequently. Of the 88 units using sedation, 19 (22%) also used paralysis. Suxamethonium was given by 10/19 (53%) but only half of these combined it with atropine. Drug doses varied by factors of up to 200, even for commonly used drugs. CONCLUSION: Most UK neonatal units do not sedate babies before intubating, despite evidence of physiological and practical benefits. Only a minority have written guidelines, which prohibits auditing of practice.
OBJECTIVE: To examine the results of fetal cardiac scanning and audit the changes in performance resulting from the introduction of a training programme for obstetric ultrasonographers. METHODS: Using the database of the Northern Regional Congenital Abnormality Survey (NORCAS), fetuses with complex or significant congenital heart disease (CCHD) diagnosed prenatally in 1994 were identified. A simple programme of centralised and local training was instituted in 1995 by the department of paediatric cardiology to teach obstetric ultrasonographers in district general hospital maternity departments to identify congenital heart malformations. The results of the training programme were assessed by comparing the 1994 identification rate of CCHD with the rates for 1996 and 1997. RESULTS: Birth rate fell during the study from 35,026 in 1994 to 32,874 in 1997. Registration of CCHD also fell, from 115 in 1994 to 87 in 1997. Prenatal recognition of CCHD rose from 17% in 1994 to 30% in 1995 and 36% in 1996. In 1997 it fell slightly to 26.9%. The total number of scans did not change much year on year, but the number of parents choosing termination increased significantly (from 22.7% to 57%). CONCLUSIONS: A simple training programme for obstetric ultrasonographers increased their ability to detect serious congenital heart disease at a routine 18-20 week anomaly scan. With a termination rate of more than 50%, the incidence of CCHD in the population fell from 3.3/1000 to 2.6/1000 live births. This audit, conducted within a stable population using ascertainment by a well established fetal malformation registry, suggests that prenatal diagnosis may have a significant effect on the incidence of complex or serious congenital cardiac malformations.
AIM: To attempt to define the prevalence and significance of murmurs detected on routine clinical examination at six to eight weeks. METHODS: A retrospective review of the results of routine clinical examination of a cohort of 6 to 8 week old babies resident in Newcastle upon Tyne, was carried out in two 12 month periods. All cardiac defects diagnosed in infancy in the same cohort were ascertained. RESULTS: 7132 babies were eligible for routine examination; 83% of these were examined. Murmurs were heard in 47 of 5395 babies and in 11 of 25 referred for evaluation congenital heart disease was found. The six to eight week examination led to diagnosis of 11 of 35 cases (31%) of congenital heart disease in the study population. CONCLUSIONS: Nearly one baby in 100 had a murmur on routine examination at six to eight weeks. Nearly half of those with murmurs who were referred had a structural cardiovascular malformation.
OBJECTIVE: To predict the effect of antenatal ultrasound screening for congenital heart disease and maternal serum screening of Down's syndrome on the practice of paediatric cardiology and paediatric cardiac surgery. DESIGN: A retrospective and prospective ascertainment of all congenital heart disease diagnosed in infancy in 1985-1991. SETTING: One English health region. PATIENTS: All congenital heart disease diagnosed in infancy by echocardiography, cardiac catheterisation, surgery, or necropsy was classified as "complex", "significant", or "minor" and as "detectable" or "not detectable" on a routine antenatal ultrasound scan. RESULTS: 1347 infants had congenital heart disease which was "complex" in 13%, "significant" in 55%, and "minor" in 32%. 15% of cases were "detectable" on routine antenatal ultrasound. Assuming 20% detection and termination of 67% of affected pregnancies, liveborn congenital heart disease would be reduced by 2%, infant mortality from congenital heart disease by 5%, and paediatric cardiac surgical activity by 3%. Maternal screening for Down's syndrome, assuming 75% uptake, 60% detection, and termination of all affected pregnancies, would reduce liveborn cases of Down's syndrome by 45%, liveborn cases of congenital heart disease by 3.5%, and cardiac surgery by 2.6%. CONCLUSIONS: Screening for congenital heart disease using the four chamber view in routine obstetric examinations and maternal serum screening for Down's syndrome is likely to have only a small effect on the requirements for paediatric cardiology services and paediatric cardiac surgery.
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This study documents the presentation of acutely life threatening congenital heart disease in infancy in order to assess the performance of screening examinations soon after birth and at 6 weeks of age. All cases of obstructive left heart malformations presenting in infancy in one health region from 1987-91 were analysed retrospectively. Altogether, 120 infants presented with either hypoplastic left heart syndrome, interruption of the aortic arch, coarctation of the aorta, or aortic valve stenosis. Twelve became symptomatic or died within 24 hours. Thirty four had an abnormal neonatal examination, eight of whom were referred. Six more became symptomatic before discharge. Ninety four babies went home, 51 developed heart failure before 6 weeks, and another seven died without diagnosis. Twenty five of 36 (69%) reaching 6 weeks without diagnosis had a second examination which was abnormal in 17. Two babies died undiagnosed after 6 weeks and the other 18 presented at up to 11 months of age. The neonatal and 6 week examinations perform poorly as screening tests for congenital heart disease. A normal neonatal examination does not exclude life threatening congenital heart disease.
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Synovectomy of the knee was performed in a 27-year-old female with seropositive rheumatoid arthritis of 6 years duration. At surgery the suprapatellar pouch was found to be separate from the rest of the knee joint, where direct access to articular collagenous tissues remained. The suprapatellar pouch, presumably separate from the knee cavity since birth, was free of disease. The rest of the joint lining, and synovium of the forefoot joints, that had been operated on at the same time, had gross and histological evidence of severe rheumatoid synovitis. The sparing of this suprapatellar pouch by rheumatoid disease is not easily explained, especially since its origin and nature are similar to other synovial parts of the knee that were actively involved. These latter areas had direct access to articular collagenous tissues. The findings of this case question the predilection of rheumatoid arthritis for synovium, and suggest that synovium and articular collagenous tissues are requisites of chronic rheumatoid arthritis.
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Determinations of prothrombin time with the semiautomatic fibrometer and with a more automated machine (Electra 600) were compared in regard to reproducibility, accuracy, and speed. Prothrombin times determined for replicate samples with the two machines showed a correlation coefficient of 0.98, but the Electra 600 sensed the clot 0.5 seconds before the fibrometer. The overall coefficients of variation for multiple tests in the normal therapeutic and elevated ranges of prothrombin time were 3.5% for the fibrometer and 2.0% for the Electra 600. The average technician working time was 60% shorter with the fully automated machine than with fibrometer. It is concluded that the automated machine was more accurate and more rapid in determining prothrombin time.