Epilogue: 40 years in paediatrics.
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Biomedical subjects
Publications and source records attributed to O C Ward.
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Between January 1976 and December 1987 42 children with Down syndrome and congenital heart disease underwent cardiac surgery. Four children had two operations. Age at the time of surgery ranged from 11 days to 14 years. The commonest operative procedure was repair of a patent ductus arteriosus. Four patients died post-operatively, two following repair of a complete atrio-ventricular canal defect (CAVD), one following correction of tetralogy of Fallot in association with a CAVD, and a fourth following closure of ventricular septal defect and atrial septal defect. The mortality for those who had open heart surgery was 13.3% and for the series as a whole the mortality was 6.6% over a period of follow-up ranging from two months to four years. A relatively conservative approach has been adopted with regard to surgery, based on the shorter natural expectation of life in Down syndrome, the complexity of many of the cardiac lesions involved and the recognition of the frequency of early intellectual deterioration in Down patients.
Thirteen patients with Kawasaki disease were reviewed. The average age at presentation was 31 months (range: 6 weeks to 8 years). Mean follow up was 36 months, with a range of 5 to 53 months. Cardiovascular manifestations were present in 9 patients. These comprised pericarditis with myocarditis (2), pericardial effusion (4), coronary artery ectasia (3), coronary artery aneurysms (3), peripheral artery aneurysm (1) and peripheral vasculitis (1). Treatment consisted of aspirin alone in 11 and gammaglobulin with aspirin in 2 patients. One patient with coronary artery involvement died following a myocardial infarction. A large coronary artery aneurysm persists, 31 months after initial detection in another patient. Spontaneous resolution of the cardiovascular complications occurred in the remaining seven patients. The importance of clinical awareness and detailed evaluation and follow up of the cardiovascular system in Kawasaki disease is emphasised. There is also a need, however, to exclude other diagnoses which may have a similar clinical presentation.
Paracetamol suppositories in two different bases were given to children who had fever after operations. Plasma concentrations and the effect on temperature were compared. There was a significant correlation between peak plasma concentrations and maximum drop in temperature. A lipophilic base produced better results than a hydrophilic base.
One hundred and thirty children with Down's syndrome were screened for the presence of atlantoaxial instability, using both clinical examination and radiographs of the cervical spine taken in flexion and hyperextension views. Seven children were found to have radiological evidence of atlantoaxial instability, with an atlanto-dens interval greater than 5.0 mm in one or all positions. Although a full clinical history was obtained from the attending parent and each child underwent a complete neurological examination, there were no factors detected which differentiated between those with radiological evidence of atlantoaxial instability and those with a normal atlantodens distance. It is recommended that children with Down's syndrome be screened twice, at the ages of 5-10 years and at 15 years.
Two children whose illness initially fulfilled the clinical criteria for the diagnosis of Kawasaki disease were both subsequently found to have acute bacterial endocarditis and one child had a disseminated non-Hodgkins lymphoma. We describe their clinical course and emphasize the importance of the exclusion of other diagnosis as well as strict adherence to the accepted diagnostic criteria of Kawasaki disease.
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Rectal acetylsalicylic acid was given to 14 children who had undergone open heart surgery. The effect on their temperatures was similar whether 15--30 or 30--50 mg/kg was given. Either dose was more effective than no treatment. The greatest fall in temperature occurred after 4 or 5 hours. Rectal aspirin in a triglyceride base is effective in lowering postoperative temperature. It should also be of use in treating other fevers. A dose of 20--25 mg/kg is suggested.
A new and simple technique is described which clearly defines the aortic anatomy in infantile coarctation. The technique greatly helps to select those neonates whose lives can be salvaged by early surgery. There have been no serious adverse effects.
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The effect of bumetanide in infants with congenital heart disease presenting with cardiac failure was studied. The study was divided into acute (3 days) and long-term (mean 10.5 weeks) cases. A total of 12 male infants was included in the acute study and 13 cases were evaluated in the long-term study. The dose used in the acute study (0.015 mg/kg) was suboptimal; notwithstanding it was found to cause significant natriuresis and chloruresis. Bumetanide in doses varying in different infants from as little as 0.015 mg/kg on alternate days to as much as 0.10 mg/kg daily was shown to be an effective diuretic for long-term use. No side effects were observed in either study.