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Biomedical subjects

V Blair

Publications and source records attributed to V Blair.

At least 55 records · Page 3Linked to original sources

Cancer in the families of children with soft tissue sarcoma.

The cancer experience among 754 first-degree relatives (mothers, fathers, and siblings) of a population-based series of 177 children with soft tissue sarcoma is reported. The current study represents an extension of our earlier work in which the authors found an excess of breast cancer in the mothers of 143 of these children. There were 40 cancers among all first-degree relatives, compared with 24.82 expected (relative risk [RR] 1.61, P = 0.006). There was no excess in fathers, but an excess of borderline significance was seen in mothers (RR 1.67, P = 0.0545), and a significant excess in siblings (RR 4.55, P = 0.0002), mainly due to carcinoma of the breast and pediatric tumors. Results of a step forward Cox multivariate analysis identified three variables in the index child which were independently associated with high cancer risk in relatives, as follows: age younger than 24 months at diagnosis; histologic type, embryonal rhabdomyosarcoma or other and unspecified soft tissue sarcoma; and male sex. It was possible, therefore, to identify a subgroup of children whose relatives are at high risk of early onset cancer (RR in this group 10.14). The pattern of cancers is consistent with the Li-Fraumeni syndrome. The authors conclude that a marked proportion of childhood soft tissue sarcoma has a genetic basis.

Adolescent↗

Epidemiological and familial aspects of hepatoblastoma.

Data on the 20 cases of hepatoblastoma registered with the Manchester Children's Tumour Registry between 1954 and 1988 are presented. The cases represent about 0.6% of childhood malignancies and show a male:female ratio of 2.3:1. Age at presentation was almost always under 2 1/2 years. Cancer rates in close relatives were not significantly different from those expected. One family was confirmed as having Gardner syndrome, but the presence of other syndromes e.g., Beckwith-Wiedemann, could not be reliably detected in this retrospective series. The need to take an extensive pedigree and for clinical examination of other family members at the time of the child's diagnosis is emphasized.

Adult↗

Identification of factors associated with high breast cancer risk in the mothers of children with soft tissue sarcoma.

Information on the past medical history of the mothers of a population-based series of 177 children with soft tissue sarcoma was obtained by interview and from medical records. Eight mothers developed breast cancer, six premenopausally, compared with 3.26 expected (P = .04), but no excess of other types of cancers was detected. High breast cancer risk was associated with the following factors in the index child: age at diagnosis less than 24 months (relative risk [RR], 7.84), embryonal rhabdomyosarcoma (RR, 3.74), and male sex (RR, 3.02). Characteristics in the mother associated with high breast cancer risk were the following: late age at first birth (RR, 5.13), late age at birth of index child (RR, 5.69), and high birth-rank order of index child (RR, 4.08). The results suggest there may be a subset of childhood soft tissue sarcoma with a predominantly genetic etiology. The association between premenopausal breast cancer in the mother, late age at birth of index child, and early onset of soft tissue sarcoma in the index child suggests that these three events are not independent and that interactions between genetic and other factors may be important. The identification of a group of women at high risk for breast cancer affords an opportunity for screening and early detection. The study of cancer family syndromes may provide insights into underlying mechanisms in cancer genetics.

Adult↗

The inter-regional epidemiological study of childhood cancer (IRESCC): case-control study of children with central nervous system tumours.

Tumours of the central nervous system comprise 23% of all childhood cancers and form the most common group of solid malignancies. Little is know about their aetiology. The present report concerns the results of a case-control study of 78 incident cases of central nervous system tumours in children. No case-control differences were detected for the following: pre-natal diagnostic X-rays, general anaesthetics during pregnancy, pregnancy infections, pregnancy drugs (including sedatives, tranquillizers and anti-convulsants), alcohol consumption in pregnancy, child's birthweight, breast-feeding, childhood illnesses, previous medication in the child. A significant excess of case mothers had suffered from diseases of the nervous system (RR 2.6). There was a deficit of children who had been immunised among the case children which approached significance, and an excess of congenital abnormalities among cases which also approached significance. There was a small excess of neoplastic disease among case parents. The results of this study suggest that in our patients genetic rather than environmental factors are more important, but the small numbers included in the present study meant that no definite conclusions could be reached.

Abnormalities, Multiple↗

Absence from school related to children's and parental smoking habits.

A sample of 2885 children aged 12 and 13 who completed a questionnaire survey in school in January 1987 were given a second questionnaire on a specified date in May 1987. The smoking habits, parental smoking habits, sex, and social background of the children who were present on both dates were compared with those of the children who were absent on the second occasion. Regular smoking was significantly more common among those absent for the second questionnaire: among boys 181/877 (21%) who never smoked, 109/486 (22%) who sometimes smoked, and 21/45 (47%) who regularly smoked were absent, and among girls the figures were 157/947 (17%), 117/487 (24%), and 17/43 (40%) respectively. Thus the odds ratio for those who sometimes smoked was 1.29 and for regular smokers 3.09 against those who never smoked. Whatever the children's smoking habits, the proportion who were absent was higher when both parents or at least the mother smoked, the odds ratio being 1.39; the proportions absent were 203/1180 (17%) if neither parent or only the father smoked v 135/644 (21%) if both parents or only mother smoked for children who never smoked; 105/529 (20%) v 121/444 (27%) for those who sometimes smoked; and 10/27 (37%) v 28/61 (46%) for those who regularly smoked. Sex and social background had little effect, though there was an overall higher rate of absence among boys from industrial areas. The findings show a higher rate of minor ailments in children who smoke and in children whose mother smokes. If children are having frequent days off school for minor ailments possibly they or their parents would benefit from advice and help in stopping smoking.

Absenteeism↗

Predicting the onset of smoking in boys and girls.

The problem of the high prevalence of smoking among girls and young women is of great concern. In an attempt to identify the factors which influence girls and boys respectively to attempt smoking, the study examines social background, advertising and brand awareness, knowledge, teaching and personal beliefs in conjunction as predictors of smoking. In this study which involved the administration of identical pre- and post-test questionnaires to a sample of boys and girls aged 12 and 13 years, nine variables expressed by never-smokers at pre-test stage were assessed as predictors of immediate future smoking. The two tests were administered 4 months apart to 1125 boys and 1213 girls in northern England. The nine variables included were parental smoking, best friends' smoking, perceived positive values of smoking, perceived negative values of smoking, correct health knowledge, cigarette-brand awareness, having a favourite cigarette advertisement, having a cigarette-brand sponsored sport in four top favourites on television. One group received teaching about smoking between the pre- and post-tests and this was also included as a variable. For boys, no variable investigated had any consistently statistically significant correlation with the uptake of smoking. The most important predictor of smoking for boys, having a best friend who smoked, was significant on application of the chi 2 test (P 0.037), although it was non-significant when included singly in a logistic regression model (0.094); the discrepancy was probably due to the small number of best friends known to smoke. For girls, four variables were found to be significant predictors of smoking when included singly in a logistic regression.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

A review of astrocytoma in childhood.

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.

Adolescent↗

Improvements in survival from childhood cancer: results of a population based survey over 30 years.

Survival from cancer of children whose cancer was diagnosed during the 30 years 1954-83 was analysed. The study was population based with nearly 3000 cases covering about 30 million child years at risk. When survival during the three decades 1954-63, 1964-73, and 1974-83 was compared striking improvements were observed. For all childhood cancer five year survival increased from 21% in the first decade to 49% in the third decade. During the first and third decades five year survival rates for acute lymphocytic leukaemia increased from 2% to 47%, Hodgkin's disease from 44% to 91%, non-Hodgkin's lymphoma from 18% to 45%, Wilms's tumour from 31% to 85%, and germ cell tumours from 10% to 64%. Twenty patients developed second primary tumours, but otherwise there were few late deaths. Less than 1% of children who survived without a relapse for 10 years subsequently died of their initial cancer. Survival from childhood cancer is no longer rare, and people who have been cured of cancer during childhood should be accepted as normal members of society.

Adolescent↗

Malignant disease in the mothers of children with Ewing's tumour.

Previous research has shown that mothers of children with soft tissue sarcoma, osteosarcoma, and chondrosarcoma are at excess risk of developing breast cancer. The occurrence of malignant disease in the mothers of a population-based series of children with Ewing's sarcoma was investigated in order to determine whether these mothers were at excess risk of cancer and of breast cancer in particular. Sixty-one mothers were traced; there were two cases of breast cancer and two other registrable neoplasms. Risk of malignancy in the mothers was not in excess of expectation.

Adult↗

The Inter-Regional Epidemiological Study of Childhood Cancer (IRESCC): case control study of children with bone and soft tissue sarcomas.

The Inter-Regional Epidemiological Study of Childhood Cancer included 43 cases of soft tissue and 30 cases of bone sarcomas, together with their 146 matched controls. Analysis of a wide range of aetiological factors revealed few risk factors relating to events during the index pregnancy, the earlier medical experiences of the case child, or parental medical, occupational and smoking history. Associations which did emerge included: lower birth weight in children with Ewing's tumour, an excess of mothers of children with soft tissue sarcoma with symptoms of toxaemia in pregnancy; and more children with rhabdomyosarcoma who received antibiotics soon after birth. There was some evidence that mothers of children with soft tissue sarcoma may have had reduced fertility with a significant excess of the case mothers having no other pregnancies. Slight excesses of congenital malformations in the case children and of malignant and benign/borderline neoplastic disease in the older mothers were consistent with the existence of a degree of genetic predisposition in the development of the tumours in this series.

Adult↗

The Inter-Regional Epidemiological Study of Childhood Cancer (IRESCC): past medical history in children with cancer.

The Inter-Regional Epidemiological Study of Childhood Cancer (IRESCC) collected interview and medical information relating to the child's past medical experiences from parents of 555 children diagnosed with cancer and parents of 1110 unaffected matched controls. No significant associations emerged overall for ante-natal care, place and mode of delivery, length of gestation, birth weight, condition at birth, special care, neonatal procedures or breast-feeding. Few risk factors relating to previous illnesses and medication were found, although increasing numbers of illnesses appeared to be associated with an increased risk of childhood cancer, particularly acute lymphoblastic leukaemia. A highly significant excess of case children had not been immunised (p = 0.005). In general, these results indicate that past medical experiences have little influence on the development of cancer in children.

Adolescent↗

Neurofibromatosis in children with soft tissue sarcoma.

Case records of the 157 children with soft tissue sarcoma in the Manchester Children's Tumour Registry diagnosed between 1954 and 1983 were reviewed for reference to diagnostic features of neurofibromatosis (NF). Interviews were carried out with 124 families of these children. Four children in the series were identified as having NF. All four were boys, very young at diagnosis, and had rhabdomyosarcomas of the bladder or prostate. In addition, there were indications that a further nine children may have been affected. It is suggested that NF may be more common in children with soft tissue sarcoma than previously thought and that clinicians should be alert for signs and symptoms of NF in such children and their families. Affected children may be at increased risk of developing further malignancies.

Adolescent↗

Cervical carcinoma: prognosis in younger patients.

Retrospective analysis of 2870 patients with invasive carcinoma of the cervix treated by radiotherapy from 1971 to 1978 showed that the prognosis for younger patients (defined as either under 35 or under 40) was better than that for older age groups, but young patients presented with earlier disease. When the effect of stage on prognosis was also considered the improved survival of patients under 35 was confirmed, although the result was of only borderline significance. The better survival of younger patients was particularly noticeable for stage IB disease, the corrected five year survival of those under 35 being 93% compared with 79% for those over 35. On the basis of this analysis and a review of previous reports it is concluded that age alone is a poor indicator of prognosis and should not be used as an indication for adjuvant treatment. There is no evidence in this series of an aggressive form of cervical carcinoma in younger patients during the 1970s.

Adult↗

Pretreatment prognostic factors and scoring system in 407 small-cell lung cancer patients.

In 407 patients with small-cell lung cancer (SCLC), 61 pretreatment variables were evaluated in a Cox multiple regression analysis to assess their prognostic value. All patients received short-term intensive regimens (cyclophosphamide, etoposide and methotrexate or ifosfamide and etoposide, both followed by thoracic irradiation if complete response was noted). Lactate dehydrogenase (p = 0.001), tumour stage (p = 0.0001), serum sodium (p = 0.0009), pretreatment Karnofsky performance score (p = 0.0121), alkaline phosphatase (p = 0.0186) and serum bicarbonate (p = 0.0321) were the important prognostic factors. Once these variables were taken into account no other variable provided additional prognostic information. A simple scoring system ("Manchester Score") using these variables was established and shows little loss of information compared to the Cox analysis. The score distinguishes 3 prognostic groups, the best of which contains all long-term survivors, whereas the bad prognostic group contains no patient surviving longer than one year. The scoring system may help to design new treatment strategies and may also facilitate the comparison of different studies.

Carcinoma, Small Cell↗

Mycosis fungoides--response to therapy and survival patterns in 85 cases.

The clinical course and management of 85 cases of mycosis fungoides presenting over a 20-year period are described. The extent of skin involvement and presence of lymphadenopathy were seen to be important prognostic factors. The following survival patterns were observed. In eight cases with limited plaque disease there was prolonged disease-free survival after treatment with kilovoltage X-rays; after a median follow-up of 64 months there was only one case of progression to generalized disease. In 49 cases of generalized skin disease (plaque disease +/- tumours; erythroderma) treated with total skin electron beam therapy there was a 24% 5-year disease-free survival; this potential for long-term survival was seen in cases with generalized plaque disease without lymphadenopathy. The remainder of the cases showed a continuously relapsing course. The extent of skin involvement and presence of lymphadenopathy can both be used as known prognostic factors to adopt a logical plan of treatment.

Adult↗

Adrenal cortical tumours: epidemiological and familial aspects.

Epidemiological data on the 14 cases of adrenal cortical tumour registered with the Manchester Children's Tumour Registry from 1954 and 1985 are presented. The incidence of adrenal cortical carcinomas was 0.3%, mainly in girls, most of whom presented with virilisation. The incidence of neoplastic disease among close relatives was ascertained, but, except in siblings, this was not significantly higher than would be expected. Evidence from extended pedigrees, however, indicates that at least four of the children could be members of families with the SBLA (sarcoma, breast and brain tumour, leukaemia, laryngeal and lung cancer, and adrenal cortical carcinoma) cancer family syndrome, and that other relatives may be at risk of developing such neoplasms.

Adolescent↗

A clinical trial of two conceptually different radical radiotherapy treatments in stage III carcinoma of the cervix.

Two hundred and ninety-six consecutive patients under the age of 70 years and having Stage III carcinoma of the cervix were randomised to receive one of two radical radiotherapy techniques. The first was a small-field, wedged, inhomogeneous 3-week X-ray treatment, followed by two radical radium insertions delivering 80% of a radical intracavity dose. The second was a 4-week homogeneous, large-field X-ray technique, supplemented by a single intracavitary insertion giving 50% of a radical intracavitary dose. Mature 5-year survival rates for the two techniques were 38.6% and 40.3% respectively (p = 0.76). Corrected 5-year results were 44.5% and 45% (p = 0.8). The time to pelvic recurrence and the sites of pelvic recurrence were not significantly different. The rate of major morbidity was eight out of 148 in the first group and five out of 148 in the second. This large, prospective, randomised trial failed to identify any significant advantage to two conceptually different techniques which explored different volumes, fractionation and balance between X-ray therapy and intracavitary therapy. The overall results remain very satisfactory when compared with recently published European results.

Aged↗