PubMed Health⌕ Search

Biomedical subjects

B Emanuel

Publications and source records attributed to B Emanuel.

At least 37 records · Page 2Linked to original sources

Growth factor requirements of childhood acute leukemia: establishment of GM-CSF-dependent cell lines.

Eight permanent cell lines were established from cells of 50 consecutive patients with childhood acute leukemia. Three cell lines required growth factor-containing conditioned media. Analysis using blocking antisera and recombinant granulocytic macrophage (GM) colony-stimulating factor (CSF) identified GM-CSF as a growth factor required to establish the latter three cell lines and necessary for their continuous proliferation in chemically defined medium. Two of the GM-CSF-dependent cell lines were derived from patients with undifferentiated T- and a biphenotypic B-myelomonocytic leukemia, which suggests that GM-CSF might maintain proliferation of leukemias originating from immature progenitor cells. Cytogenetic analysis indicated that all established leukemic cell lines were aneuploid, with six lines containing chromosomal alterations related to those observed in the leukemic cells of the patient. Two patients did not have an abnormal clone identified in the marrow but did yield an aneuploid cell line. These studies indicate that GM-CSF-dependent leukemic cell lines can be established in a fraction of childhood leukemia. These cell lines lend themselves to studies aimed at the evaluation in vitro of the role of growth factors in controlling proliferation and differentiation of leukemic cells.

Acute Disease↗

A major human histone gene cluster on the long arm of chromosome 1.

A human histone gene cluster was assigned to chromosome 1 by Southern blot analysis of DNA's from a series of mouse-human somatic cell hybrids with 32P-labeled cloned human H4 and H3 histone DNA as probes. Localization of this histone gene cluster on the long arm of chromosome 1 was confirmed by in situ hybridization of this DNA probe to metaphase chromosomes.

Animals↗

Two adult siblings with thrombocytopenia and a familial 13;14 translocation.

A woman with thrombocytopenia that progressed to aplastic anemia, and her brother, who had persistent thrombocytopenia, both had a constitutional t(13;14) translocation. Six other family members, in three generations, had the same translocation, but no hematologic disorder. There was evidence suggestive of increased chromosomal fragility in lymphocyte cultures from two members of the kindred, but not in the two patients. The findings support a postulated association between the t(13;14) and hematologic disorders, but whether the mechanism involves an inherited defect in chromosomal stability is unproved.

Chromosome Fragility↗

Progressive preleukemia with a chromosomally abnormal clone in a kindred with the Estren-Dameshek variant of Fanconi's anemia.

One of two sisters with hypoplastic anemia and increased chromosomal fragility, but no clinical stigmata of Fanconi's anemia (FA), progressed to preleukemia (with a clone of chromosomally abnormal cells in the bone marrow), and then to acute nonlymphocytic leukemia. The findings indicate that constitutional genetic instability can lead to karyotypically aberrant neoplastic clones in the Estren-Dameshek variant of FA as well as in the more typical chromosomal fragility syndromes.

Adolescent↗

Translocation of an immunoglobulin kappa locus to a region 3' of an unrearranged c-myc oncogene enhances c-myc transcription.

We have studied somatic cell hybrids between mouse myeloma and JI Burkitt lymphoma cells carrying a t(2;8) chromosome translocation for the expression of human kappa chains. and for the presence and rearrangements of the human c-myc oncogene and kappa chain genes. Our results indicate that the c-myc oncogene is unrearranged and remains on the 8q+ chromosome of JI cells. Two rearranged C kappa genes were detected: the expressed allele on normal chromosome 2 and the excluded kappa allele that was translocated from chromosome 2 to the involved chromosome 8 (8q+). The distribution of V kappa and C kappa genes in hybrid clones retaining different human chromosomes indicated that C kappa is distal to V kappa on 2p and that the breakpoint in this Burkitt lymphoma is within the region carrying V kappa genes. High levels of transcripts of the c-myc gene were found when it resided on the 8q+ chromosome but not on the normal chromosome 8, demonstrating that translocation of a kappa locus to region distal to the c-myc oncogene enhances c-myc transcription.

Animals↗

Benign neoplasmia and pseudoneoplasia of the small bowel in children.

Fourteen cases of benign tumorous conditions in children are reported. Each new growth described represents a true neoplasm, one simulating a true neoplasm, or one simulating a true tumor, clinically and/or radiographically. The series contains 10 female and 4 male patients. Most patients were under 1 year of age when manifestations--most often, gastrointestinal hemorrhage or bowel obstruction-- of the condition became evident. Nine patients had roentgenographic findings which indicated the presence of an abnormality, but the specificity of the findings was low. Some patients had cutaneous or other somatic clinical stigmata found in known syndromes also containing gastrointestinal pathology. These stigmata and the awareness of the clinical and roentgenographic manifestations of small bowel tumors in children should lead to an early diagnosis and hopefully to the usually successful surgical therapy.

Adolescent↗

Perforation of the gastrointestinal tract in infancy and childhood.

Eighty-eight patients with perforations of the gastrointestinal tract in infancy and childhood, excluding those caused by appendicitis, are presented. Fifty-five patients were four weeks of age or younger. Fourteen of the perforations occurred during the first year of life and 19 occurred between one and 15 years of age. The locations of perforations by order of frequency were the ileum, rectosigmoid, stomach and duodenum. The causes in order of frequency were necrotizing enterocolitis, ulcers, unknown causes, Hirschsprung's disease, atresia of the small intestine, volvulus, trauma, gastroschisis and ventriculoperitoneal shunts for hydrocephalus. A high index of suspicion, aided by roentgenograms, is essential for an early diagnosis. The over-all mortality was 49 of 88 patients. No reduction in mortality was observed in the last ten years, despite improved surgical techniques and better antimicrobial agents. Early recognition and rapid transport of the child to a pediatric intensive care unit with better supportive measures plus antimicrobial agents effective against both anaerobic and aerobis bacteria should reduce this high mortality.

Adolescent↗

Renal trauma in children.

Recent experience with two cases of renal trauma led us to review 57 admissions to Children's Memorial Hospital, CHICAGO, DURING THE LAST 15 YEARS. It became apparent that a clearly defined approach to the management of renal trauma was lacking. A uniform investigation of such patients to include early intravenous pyelography and followed by renal scan or arteriography when pyelography is inconclusive in assessing the exact nature of the renal damage is recommended. The importance of these studies in diagnosing associated congenital anomalies and solitary kidney is also shown. Criteria for management by early surgical intervention or conservative approach are presented. The ultimate aim in management is to preserve the greatest possible amount of functioning renal tissue with the least risk and morbidity to the patient.

Accidents, Traffic↗

Mediastinitis in a child caused by perforation of pharynx.

Mediastinitis in infants and children secondary to trauma is rarely reported as evidenced by a literature search. A case of mediastinitis occurred following perforation of the pharynx by a pencil. In neonates, traumatic perforation may mimic esophageal atresia. In older children with a history of trauma to the mouth or pharynx by a sharp object, the possibility of secondary mediastinitis exists.

Abscess↗