Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have reviewed the records of the 16,564 cases of childhood cancer diagnosed from 1971 to 1983 which were reported to the National Registry of Childhood Tumours in Great Britain for the presence of underlying genetic disease in order to estimate the proportion which results from inherited mutations. A genetic condition was listed for 509 patients, or 3.07% of the total number of tumours. The most frequently recorded diagnoses were: bilateral retinoblastoma (162 cases); Down syndrome (135); neurofibromatosis (90); hereditary Wilms' tumour (71); and tuberous sclerosis (20). The highest hereditary fractions at individual tumour sites were seen for: retinoblastoma (37.2%); kidney (7.2%); leukaemia (2.6%) and brain and spinal cord (2.0%). When information about family history from published reports was incorporated into the figures calculated from Registry data the total genetic fraction was estimated to be 4.2%. We conclude that there is a clear genetic basis for a small minority of the cancers of childhood, but ethnic variation and the lack of known environmental determinants suggest that the total influence of heredity may be higher.
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PMID:An estimate of the heritable fraction of childhood cancer. 206 56

The irradiation-fusion technique offers a means to isolate intact subchromosomal fragments of one mammalian species in the genetic background of another. Irradiation-reduced somatic cell hybrids can be used to construct detailed genetic and physical maps of individual chromosome bands and to systematically clone genes responsible for hereditary diseases on the basis of their chromosomal position. To assess this strategy, we constructed a panel of hybrids that selectively retain the portion of human chromosome band 11p13 that includes genes responsible for Wilms tumor, aniridia, genitourinary anomalies, and mental retardation (constituting the WAGR syndrome). A hamster-human hybrid containing the short arm of chromosome 11 as its only human DNA (J1-11) was gamma-irradiated and fused to a Chinese hamster cell line (CHO-K1). We selected secondary hybrid clones that express MIC1 but not MER2, cell-surface antigens encoded by bands 11p13 and 11p15, respectively. These clones were characterized cytogenetically by in situ hybridization with human repetitive DNA and were tested for their retention of 56 DNA, isozyme, and antigen markers whose order on chromosome 11p is known. These cell lines appear to carry single, coherent segments of 11p spanning MIC1, which range in size from 3000 kb to more than 50,000 kb and which are generally stable in the absence of selection. In addition to the selected region of 11p13, two cell lines carry extra fragments of the human centromere and two harbor small, unstable segments of 11p15. As a first step to determine the size and molecular organization of the WAGR gene complex, we analyzed a subset of reduced hybrids by pulsed-field gel electrophoresis. A small group of NotI restriction fragments comprising the WAGR complex was detected in Southern blots with a cloned Alu repetitive probe. One of the cell lines (GH3A) was found to carry a stable approximately 3000-kb segment of 11p13 as its only human DNA. The segment encompasses MIC1, a recurrent translocation breakpoint in acute T-cell leukemia (TCL2), and most or all of the WAGR gene complex, but does not include the close flanking markers D11S16 and delta J. This hybrid forms an ideal source of molecular clones for the developmentally fascinating genes underlying the WAGR syndrome.
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PMID:A panel of irradiation-reduced hybrids selectively retaining human chromosome 11p13: their structure and use to purify the WAGR gene complex. 215 97

Survival rates were analysed for a population-based series of over 15,000 childhood cancers registered in Great Britain during 1971-85. There were highly significant improvements (P less than 0.001 for trend) in survival for many major diagnostic groups. Between 1971-73 and 1983-85 the actuarial 5-year survival rates increased from 37% to 70% for acute lymphoblastic leukaemia, from 4% to 26% for acute non-lymphoblastic leukaemia, from 76% to 88% for Hodgkin's disease, from 22% to 70% for non-Hodgkin's lymphoma, from 61% to 72% for astrocytoma, from 24% to 42% for medulloblastoma, from 15% to 43% for neuroblastoma, from 58% to 79% for Wilms' tumour, from 17% to 54% for osteosarcoma, from 26% to 61% for rhabdomyosarcoma, from 59% to 94% for malignant testicular germ-cell tumours and from 43% to 77% for malignant ovarian germ-cell tumours. These increases in population-based survival rates reflect the substantial advances in treatment of a wide range of childhood cancers since 1970. The two principal diagnostic groups for which there was no evidence of any trend were retinoblastoma, which already had an excellent prognosis with a 5-year survival rate of over 85%, and Ewing's sarcoma, for which the survival rate remained below 45%.
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PMID:Trends in survival for childhood cancer in Britain diagnosed 1971-85. 217 43

An investigation of 749 deaths occurring among 4082 patients surviving at least five years after the diagnosis of childhood cancer in Britain before 1971 has been undertaken. Of the 738 with sufficient information the numbers of deaths attributable to the following causes were: recurrent tumour, 550 (74%), a second primary tumour, 61 (8%), a medical condition related to treatment of the tumour, 49 (7%), an traumatic death unrelated to the tumour or its treatment, 34 (5%), finally, any other cause unrelated to the tumour or its treatment, 44 (6%). Less than 10% of five year survivors of non-Hodgkin lymphomas, neuroblastoma, retinoblastoma, Wilms' tumour, or a soft tissue sarcoma died of recurrent tumour during the next 15 years, while more than 25% of five year survivors of Hodgkin's disease, ependymoma, medulloblastoma, and Ewing's tumour died of recurrent tumour during the corresponding period. Almost 50% of five year survivors of acute lymphoblastic leukaemia died of recurrent disease during the corresponding 15 years, a large proportion of deaths being due to central nervous system relapse in an era before central nervous system prophylaxis was routinely given. Comparison of the mortality observed with that expected from mortality rates in the general population indicated three times the expected number of deaths from non-neoplastic causes. Five times the expected number of deaths from cardiovascular causes were observed, these were predominantly myocardial infarction and cerebrovascular accidents. There was no evidence of an excess in the number of suicides observed, but there were three times the expected number of deaths from accidents observed after central nervous system tumours. Two groups of patients were identified whose deaths were potentially avoidable. Seven patients with craniopharyngioma and panhypopituitarism presented with addisonian crises during periods of stress not adequately covered by exogenous corticosteroids. In the other group were children who received radiotherapy and later developed problems related to radiation fibrosis. We emphasize that our investigation relates to patients diagnosed with childhood cancer before 1971. The pattern of mortality that will emerge after recent treatment regimens, in which chemotherapy is being used more extensively, is likely to be different from that observed in our study.
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PMID:Late deaths after treatment for childhood cancer. 227 Sep 44

In an effort to better understand the epidemiology of cancer in Zaire, a retrospective review of biopsy-proven malignant tumors was undertaken. Of 188 biopsies taken from children aged 0-15 years over a 4.5 year period, 73 (39%) revealed malignancy. Fifty-six percent of patients with malignant tumors were boys. Lymphoma was the most common tumor (28 patients, 15 with Burkitt's Lymphoma). Sarcoma (15 patients), carcinoma (8 patients), Wilms' Tumor (6 patients), and retinoblastoma (5 patients) were also seen. Lymphomas were most heavily represented in the first 5 years of life, while sarcoma and carcinoma accounted for most of the malignancies in children after 10 years of age. Lymphomas and sarcomas are relatively more common in Zaire than in North America and Europe, while leukemia and central nervous system tumors are notably less common in Zaire. In view of current limitations on health care in rural Zaire, cancer care should be directed toward early diagnosis, quick referral for appropriate surgical care, and use of the limited arsenal of chemotherapy.
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PMID:Malignant tumors in children of northeastern Zaire. A comparison of distribution patterns. 230 7

The authors measured the cognitive function and physical growth of 51 children who had been treated for acute lymphoblastic leukemia with chemotherapy, cranial irradiation and intrathecal methotrexate, and who had remained disease-free for five to 12 years. A comparison group of 15 children treated for Wilms' tumor was also studied. Cognitive impairment and growth retardation were greater among the leukemia group. Of potentially greater significance, however, was the finding that female sex was the pre-eminent risk factor for central nervous system toxicity resulting from treatment. Cognitive impairment, short stature and excessive weight were all more prevalent among females than males. Approximately half the children were microcephalic, but there was no sex difference. Age at evaluation and diagnosis, as well as socio-economic status, were differentially related to outcomes for the two sexes. The authors believe the sex differences were indicative of a fundamental interaction between postnatal neural development and other biological processes.
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PMID:Late effects of central nervous system treatment of acute lymphoblastic leukemia in childhood are sex-dependent. 231 27

A cancer-prone family was studied to determine if certain chromosomal abnormalities might have predisposed members to develop diverse types of malignancies. The types of neoplasia that occurred in this family included cancers of the breast and stomach, multiple myeloma, dermatofibrosarcoma, Wilm's tumor, and leukemia; the latter three occurred in children at an early age. Peripheral lymphocytes from 13 family members were examined for the presence of constitutional chromosomal abnormalities, fragile sites, and mutagen sensitivity. Our data shows that all living members of this family who had cancers were hypersensitive to chromosome breakage induced by bleomycin. In contrast, neither constitutional chromosomal abnormality nor heritable type of folate-sensitive fragile site was observed in any member. The above findings suggest that genetic defects affecting chromosomal breakage and repair may be contributing factors for cancer development in several members of this family.
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PMID:Mutagen sensitivity and cancer susceptibility. Report of a cancer-prone family. 247 11

In the past two decades a huge progress was achieved in the treatment of malignant diseases in children. The cyclical chemotherapy combined with surgery and radiotherapy gave very good results. However, the contribution of chemotherapy to paediatric neuro-oncology was not as great is it was to other branches of paediatric oncology (acute leukaemia, malignant lymphoma, Wilms' tumour). One of the reasons is that although brain tumours are usually solid in children the existence of different histological types contributes to the fact that they are not very frequent or are rare. Consequently, for many types the necessary number of representative controlled studies is lacking. On the other hand, the leading paediatric oncology associations in the world (Children's Cancer Study Group (CCSG), International Society of Paediatric Oncology (SIOP) and Paediatric Oncology Group (POG)) have applied the prospective clinical programmes of treatment of these tumours later than in other malignant diseases. On the basis of the analysis of these programmes and experience of individual oncological centres a certain experience was acquired in present-day possibilities of chemotherapy in children suffering from brain tumours.
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PMID:[Use of chemotherapy in brain tumors in children--possibilities and perspectives]. 249 Oct

To focus attention on the problem of infant mortality in Lebanon, data were compiled on infant mortality from 1978 to 1986 at the American University of Beirut Medical Center. Causes of death are analyzed for 602 males and 398 females. 54.9% deaths occurred at 1 month of age and 77.4% died within the 1st year. Autopsies were performed on .7%. 37.7% of all neonatal deaths were due to neonatal diseases such as hyaline membrane disease, asphyxia neonatorum, immaturity, necrotizing enterocolitis, hemorrhage, hemolysis, meconium aspiration, and kernicterus. Better prenatal care would reduce this group, or the administration of corticosteroids to the mother 24-48 hours prior to delivery, as well as rapid resuscitation at birth and prevention of the 5 curses: hypoxemia, hypoglycemia, hypothermia, hypotension, and acidosis. Although unavailable in Lebanon, administration of surfactants through an endotracheal tube would also help. Infections constitute 25.1% of deaths; many are preventable through adequate public health measures and strict personal hygiene, i.e., diseases such as sepsis, pneumonia, meningitis, gastroenteritis, hepatitis, encephalitis, and 1-2 cases of the following: diphtheria, measles, peritonitis, tetanus, tuberculosis, cytomegalis inclusion, herpes, parathyphoid, pertussis, poliomyelitis, and shigellosis. Congenital diseases were 21.6%. In utero diagnosis could prevent some diseases and in utero treatment is possible for hydrocephalus and hydronephrosis. Screening programs postnatally could lead to treatment. 5.9% were malignancies such as leukemia, lymphoma, brain tumors, histocytosis, Wilm's tumor, Ewing sarcoma, and Hodgkin's disease. Early diagnosis is critical if mortality is to be reduced in this group, but medical advances are still needed. 2.9% are miscellaneous diseases such as poisoning, rheumatic diseases, marasmus, Reye's syndrome, nephrosis, rickets, and epilepsy. Most of these diseases are preventable, except for rheumatic inflammation of the heart. Recommended necessary steps to reduce infant mortality are: prenatal care, diagnosis and screening, intrauterine surgery; resuscitation and intensive care centers with modern equipment and trained personnel; national vaccination and screening programs; adequate public health measures and hygiene; parental education; and well-equipped hospitals to serve all regardless of income level.
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PMID:Pediatric mortality: an avoidable tragedy. 251 28

Using chromosome walking techniques, overlapping lambda and cosmid clones from the T cell receptor alpha (TCR alpha) region have been isolated; these span the entire J alpha region and parts of the TCR delta gene. Molecular analysis of the acute childhood leukemia cells (T-ALL) 8511 revealed a rearrangement on one chromosome 14 in J alpha 58 kb 5' of C alpha; this does not result in production of alpha message. The translocation was identified 90 kb 5' of C alpha at the previously identified J delta 2 element. A probe derived from the 5' region of the translocation breakpoint hybridized to DNA from a mouse-human cell hybrid containing chromosome 11 as the only human chromosome. This probe was used to isolate cosmid clones from chromosome 11. Several rare cutting restriction enzyme sites were found in close vicinity to the translocation breakpoint, and a long-range map spanning 1000 kb of chromosome region 11p13 was established. Analysis of the DNA from 15 cases of sporadic and familial Wilms' tumor did not reveal any changes, indicating that the translocation breakpoint does not reside in this gene.
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PMID:Molecular cloning of the translocation breakpoint in T-ALL 11;14 (p13;q11): genomic map of TCR alpha and delta region on chromosome 14q11 and long-range map of region 11p13. 254 21


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