Biomedical subjects
Publications and source records attributed to R H Campbell.
Unilateral swelling of submandibular salivary gland in two cats was diagnosed as necrotising sialometaplasia. Histological features that differentiate the disease from other salivary gland lesions, particularly neoplasia are: lobular necrosis of salivary tissue; squamous metaplasia conforming to duct and/or acinar outlines; preservation of salivary lobular morphology; and variable inflammation and granulation tissue.
Recently much attention has been given to the detection of submicroscopic chromosome rearrangements in patients with idiopathic mental retardation. We have screened 27 subjects with mental retardation and dysmorphic features for such rearrangements using a genetic marker panel screening. The screening was a pilot project using markers from the subtelomeric regions of all 41 chromosome arms. The markers were informative for monosomy in both parents at 3661902 loci (40.6%, 95% confidence interval 37.0-44.2%) in the 22 families where DNA was available from both parents. In two of the 27 subjects, submicroscopic chromosomal aberrations were detected. The first patient had a 5-6 Mb deletion of chromosome 18q and the second patient had a 4 Mb deletion of chromosome 1p. The identification of two deletions in 27 cases gave an aberration frequency of 7.5% without adjustment for marker informativeness (95% confidence interval 1-24%) and an estimated frequency of 18% if marker informativeness for monosomy was taken into account. This frequency is higher than previous estimates of the number of subtelomeric chromosome abnormalities in children with idiopathic mental retardation (5-10%) although the confidence interval is overlapping. Our study suggests that in spite of the low informativeness of this pilot screening, submicroscopic chromosome aberrations may be a common cause of dysmorphic features and mental retardation.
Four children with spinal cord compression due to malignant tumours are presented. The severity of the condition was not initially recognized by parents, or the nature of the likely cause by the initial physicians. Lower limb asymmetrical weakness, clear-cut sensory levels, and marked pain indicate need for urgent imaging and exclusion of a space occupying lesion. In 1997 diagnosis of Guillain-Barré syndrome should not be made without careful prior spinal imaging.
Standard treatment for the majority of malignant brain tumours consists of surgery and radiotherapy. This treatment has late morbidity which is accentuated in the very young child. As part of a strategy to improve quality of life and overall survival of young children with brain tumours, members of the United Kingdom Children's Cancer Study Group (UKCCSG) have piloted an intensive chemotherapy regimen which aims to avoid or delay radiotherapy following surgery. Twenty eight children with a variety of malignant brain tumours have received the regimen, which contains carboplatin, vincristine, cyclophosphamide, methotrexate, and cisplatin. The treatment is toxic, resulting in one death from infection. The bulk of the toxicity was associated with the administration of carboplatin. All but three children eventually required adjuvant radiotherapy and this was given between 1.5 and 27 months from diagnosis (median delay to radiotherapy, 12 months). Using this treatment regimen, overall survival at four years is 35% (confidence intervals 10% to 60%). While there is no evidence from this study that radiotherapy can be abandoned in the management of malignant brain tumours, its introduction may be delayed using suitable chemotherapy, thus allowing time for further CNS development. This treatment strategy has been taken forward as an international clinical trial run through the International Society for Paediatric Oncology, but using a smaller dose of carboplatin to reduce toxicity.
A male infant, born following an uncomplicated pregnancy, was severely anaemic at birth following significant foeto-maternal haemorrhage. At three weeks of age a tumour was found in the liver with evidence of metastatic disease in the lungs. The infant died before treatment could be started. Postmortem revealed choriocarcinoma which led to subsequent diagnosis in the mother who also had pulmonary metastases. The mother has been successfully treated. The case is described in detail and followed by a discussion and a literature review of reported cases of simultaneous choriocarcinoma in infant and mother.
Seven new cases are described of near haploid acute lymphoblastic leukemia (ALL) and the findings reviewed together with updated complete remission duration and survival data for the 21 cases already published. The patients were four males and three females, with an age range 2-19 years; all had an immunophenotype consistent with common ALL. The poor prognostic outlook for patients with near haploid ALL is confirmed by the median remission duration of 14 months for these patients, which is comparable to that for the previously published cases. The pattern of chromosome loss was marked particularly by the presence of two copies of chromosomes 10, 14, 18, 21 and both sex chromosomes. Populations of hyperdiploid cells with double the near haploid number were observed in six of the patients, one of whom demonstrated further clonal evolution, and it is proposed that some cases classified as hyperdiploid ALL with greater than 50 chromosomes may also have arisen from a near haploid stem line.
Between 1954 and 1984, 282 children with astrocytoma were included in the Manchester Children's Tumour Registry (MCTR), giving an overall incidence of 9.3 per million person-years. There were 110 children with adult astrocytoma and 172 children with juvenile astrocytoma. The five-year survival for adult astrocytoma was 15% and 75% for juvenile astrocytoma. There were no significant improvements in survival with time. There were 21 children with neurofibromatosis (NF) and 4 children had tuberous sclerosis. Some children had other recognized syndromes and others had major or minor abnormalities. Nine children had second tumors, mainly associated with NF, and seven siblings had malignant tumors. A number of mothers of these children were found to have breast cancer. Some of these families may represent examples of the Li-Fraumeni cancer family syndrome. We conclude that astrocytomas is an important problem in childhood and that a proportion of cases may have a genetic origin.
Explore the source record for details and available documents.
The results of treatment are reviewed in 18 cases of childhood nasopharyngeal carcinoma. Since 1976 adjuvant chemotherapy with cyclophosphamide, methotrexate, and adriamycin has been used in Manchester and Leeds. The outcome is compared in 9 patients treated with radiotherapy alone and 9 patients treated later with both radiotherapy and adjuvant chemotherapy. Relapse-free survival rates were 0% for those treated with radiotherapy alone and 78% at 13-80 months after diagnosis for those treated with both radiotherapy and chemotherapy. It is concluded that adjuvant chemotherapy improves relapse-free survival in childhood nasopharyngeal carcinoma.
Gonadal function was studied in two groups of children previously treated for medulloblastoma with surgery followed by postoperative craniospinal irradiation. In group 1 but not in group 2, the children also received adjuvant chemotherapy for one to two years. All children in group 1 received a nitrosourea (BCNU or CCNU), plus vincristine in four and procarbazine in three patients. The nine children in group 1 showed clinical and biochemical evidence of gonadal damage with elevated serum FSH concentrations and, in the boys, small testes for their stage of pubertal development. In group 2 (n = 8), each child had completed pubertal development normally, the boys had adult sized testes and the girls regular menses. Gonadotropin values were normal in all eight children. We conclude that nitrosoureas were responsible for the gonadal damage in the children in group 1, with procarbazine also contributing to the damage in the three children who received this drug. In view of the limited proved value of adjuvant chemotherapy with nitrosoureas in the treatment of medulloblastoma, recognition of this serious complication of cytotoxic drug therapy may necessitate reassessing in which subgroups of children with medulloblastoma the benefits of adjuvant chemotherapy outweight the complications.
To investigate the need for and effects of phototherapy in full-term otherwise healthy babies with physiological jaundice, 40 consecutive babies with serum bilirubin levels of 250 mumol/l or more were assigned at random to two treatment groups. Phototherapy was started in the early group (n = 20) when serum bilirubin was 250 mumol/l and in the late group (n = 20) when serum bilirubin reached 320 mumol/l; however, only 3 of the late group required treatment. Phototherapy prevented a further rise in bilirubin in almost all treated babies, but the difference in peak bilirubin level between early and late treatment groups was not significant. Early phototherapy produced a more rapid decline in bilirubin; levels fell to below 250 mumol/l in a median of 28 h with early treatment and 54 h with late treatment. In each group the ratio of boys to girls was 2/1 and boys remained jaundiced for significantly longer. Phototherapy therefore curtailed the rise and duration of hyperbilirubinaemia, but the effect was small. Jaundice subsided spontaneously in most of these mature infants, especially girls. Phototherapy can separate mother from baby, and it is physiologically stressful. Treatment may be safely withheld until serum bilirubin exceeds 320 mumol/l.
Eighty-four children with acute lymphoblastic leukaemia (ALL) who had relapsed in bone marrow were studied to assess whether treatment would be more successful if relapse was detected before the disease became clinically evident. Patients whose relapse was detected by routine bone marrow examination before the disease became apparent were compared with those whose relapse was suspected from clinical examination or peripheral blood findings. In the former there was a lower percentage of blast cells in the marrow (p less than 0.02) and the patients suffered less from complications of the disease, but there was no difference in the incidence or duration of second remissions between the two groups.
We have reviewed the neurological complications not directly attributable to leukaemic infiltration in a group of 438 children with leukaemia or lymphoma. 61 children had one or more complications due chiefly to bleeding, infection, or drug toxicity. Early death from intracranial haemorrhage occurred in 1% of children with lymphoblastic leukaemia and 7% of children with myeloblastic leukaemia. Measles and chicken pox were the most serious infective complications; one child remains severely retarded after presumed measles encephalitis, one child with chicken pox died, and a second remains disabled. 2 additional cases of measles encephalitis and one of progressive multifocal leucoencephalopathy are described. Drugs which caused neurotoxicity included vincristine, cytosine arabinoside, L-asparaginase, and phenothiazines, but most problems were caused by methotrexate. Methotrexate toxicity was more prevalent and more serious in children who had had previous central nervous system leukaemia. We conclude that viral infections and methotrexate pose the greatest neurological hazards to children with leukaemia.
A case of idiopathic arterial calcification is described in a dysmature infant dying of massive pulmonary haemorrhage on the fourth day after a gestation of 36 weeks. The mother had disseminated lupus erythematosis and lupus nephritis treated with large amounts of prednisolone. Unlike most of the previously recorded cases of idiopathic arterial calcification of infancy subintimal proliferation of fibrous tissue and involvement of the coronary arteries did not occur. It is suggested that this non-occlusive form of arterial calcification may be a distinct entity.
1. Body weight was measured through forty consecutive illnesses in seventeen patients with oedema in association with chronic bronchitis and hypoxia. All patients were taking diuretic drugs at the time. 2. Body weight increased little as peripheral oedema and a raised jugular venous pressure appeared. The subsequent weight-loss during treatment was usually greater than the pre-treatment weight-gain. Body weight increased slowly in convalescence to equal or exceed hospital admission weight without a deterioration of general health or reappearance of oedema. 3. Total body water, exchangeable sodium and exchangeable potassium were measured in patients after treatment of the acute illness and clearance of oedema and again in six patients of the group 2-3 months later in convalescence. Total exchangeable sodium was normal or slightly reduced after treatment of oedema and in convalescence between recurrent acute illnesses. Even when gross oedema was present exchangeable sodium was substantially increased in only one of three patients studied at this stage. Total exchangeable potassium was invariably severely depressed. 4. Large changes of body tissue weight without comparable change in exchangeable sodium support previous evidence that oedema in hypoxic bronchitis is not simply a further form of congestive cardiac failure. 5. It is suggested that at least some of the tissue loss in acute exacerbations is a direct result of hypoxaemia and similar to that observed at high altitude. Part of the oedema fluid is thought to be derived from intracellular water released during dissolution of tissue matrix.
Explore the source record for details and available documents.
Explore the source record for details and available documents.