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)

The basic gene defect in the autosomal recessive disorder cystic fibrosis has not been identified, and no firm linkage of the disorder to any other marker has been reported. However, a serum protein abnormality present in unaffected heterozygotes as well as in affected homozygotes has been described, and immunological quantitation of this protein, termed cystic fibrosis antigen, allows the three genotypes to be distinguished. We show here that an immunologically indistinguishable protein is present at high concentrations in granulocytes from normal and cystic fibrosis individuals as well as in myeloid leukaemia cells. Somatic cell hybrids between the mouse myeloid stem-cell line WEHI-TG and myeloid leukaemia cells express cystic fibrosis antigen only when human chromosome I is present.
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PMID:Tissue localization and chromosomal assignment of a serum protein that tracks the cystic fibrosis gene. 258 74

The normal range of glucose-phosphate-isomerase (GPI) in the plasma of children during the first month of life is up to 80 U/l; until the end of the second year of life between 11 and 50 U/l; thereafter the upper limit is 46 U/l. In osteogenic sarcoma or medulloblastoma there is a good correlation between activity of GPI in plasma and clinical tumor stage. In a lot of other tumors sensitivity of this enzyme is either very low as in Ewing-sarcoma or myeloic leukemia or there is no consistent relation to the extent of the tumor. High activities of GPI are equally obtained in children suffering from cystic fibrosis, diabetes mellitus or muscular dystrophy. GPI is not valid as a tumor marker even being raised in sarcoma and medulloblastoma as mentioned. So it is not necessary to check GPI activity as a part of routine enzyme chemistry.
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PMID:[Behavior of glucosephosphate isomerase in children with malignant diseases]. 346 43

The principles guiding the inpatient treatment of adolescents with anorexia nervosa or bulimia are no different than those guiding the treatment of adolescents with cystic fibrosis, leukemia, or depression. The patient is treated first and foremost as an adolescent, avoiding reinforcement of the sick role. Biologic, psychologic, and social needs must all be considered. Further, the family must be included in the treatment, since the vast majority of patients will be returning to their families after discharge from the hospital. Hospitalization may be required for a number of reasons. Regardless of the indications for admission to the hospital, a consistent, individualized, positively reinforcing plan for evaluation and treatment needs to be developed and executed. By so doing, the hospital team is in a unique position to help the patient and the family develop more healthy patterns of acting and interacting.
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PMID:Inpatient management of anorexia nervosa and bulimia. 360 23

The met oncogene was previously isolated from a chemically transformed human cell line, MNNG-HOS. Recent evidence has demonstrated that two classes of transcripts are expressed from the met proto-oncogene locus. The met oncogene, however, expresses an aberrant RNA which has sequences in common with both transcripts. We now report partial nucleotide sequencing of the human met oncogene and show that met is related to the protein kinase oncogenes and growth factor receptors. The met nucleotide sequence is not identical to that of any published gene, and it is more closely homologous to the tyrosine kinases than to the serine/threonine kinases. Within the tyrosine kinase family, the sequenced met domains are most closely related to the human insulin receptor and the viral abl gene. In situ chromosome hybridization has mapped met to human chromosome 7 band 7q21-q31, a location distinct from that of other kinases. This is also a region associated with nonrandom chromosomal deletions observed in a portion of patients with acute nonlymphocytic leukaemia. The accompanying paper shows that this chromosomal locus is also tightly linked with the human heredity disease cystic fibrosis.
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PMID:The human met oncogene is related to the tyrosine kinase oncogenes. 406 11

Adolescent perceptions of the impact of illness were measured through the administration of an original questionnaire to 345 healthy adolescents and 168 adolescents with diabetes mellitus, cystic fibrosis, cancer, and cardiac, renal, or rheumatologic diseases. Total impact of illness (e.g., leukemia or colds) did not differ between ill and healthy respondents, and the nature of adolescent concerns were similar for both groups. Restriction of freedom was seen as the major disruption brought about by illness, with other areas of impact including relations with peers, siblings, and parents. Adolescents with cancer were most likely to view treatment as highly disruptive and, along with rheumatologic patients, expressed greatest disruption of body image secondary to disease and treatment. Perceived school disruption was most common in cardiac and oncologic patients, with the former also expressing a high degree of concern about sexuality. Females in all groups reported more impact of illness on physical appearance than did males; this difference was greatest in adolescents with cancer, rheumatologic diseases, and cystic fibrosis. The essentially healthy psychologic status of chronically ill adolescents is noted, as is the generally hopeful and positive quality of patient responses.
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PMID:Psychologic effects of illness in adolescence. II. Impact of illness in adolescents--crucial issues and coping styles. 696 86

Pharmacokinetic-based adjustment of individual aminoglycoside dosage regimens is currently being utilized in the clinical setting in an attempt to avoid toxicity and/or enhance efficacy. In this report, comparison have been made between initial and adjusted aminoglycoside dosage employed in toddlers and older children with cystic fibrosis or leukemia (high daily dose) and neonates (low daily dose) with suspected or proven infection. The initial versus adjusted mean aminoglycoside dosage was 14.1 and 17.0 mg/kg/day, respectively, in toddlers and older children with cystic fibrosis, and 7.1 and 11.5 mg/kg/day, respectively, in toddlers and older children with leukemia. Neonates with suspected or proven infection had an initial mean total daily dose of 7.4 mg/kg/day and the adjusted mean daily dose was 5.4 mg/kg/day. The use of a single pharmacokinetic dosing model for all patients, irrespective of evidence of increased or decreased drug elimination, results in widely differing drug dosages. Important and serious questions must be considered regarding the balance between efficacy and toxicity resulting from the rigid manner by which dosage adjustment protocols are employed.
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PMID:Aminoglycoside dosage in pediatric patients:considerations regarding pharmacokinetic-based dose adjustment in patients requiring high versus low dose therapy. 734 81

The expression of the cystic fibrosis transmembrane conductance regulator (CFTR) gene can be down-regulated by inflammatory stimuli such as phorbol myristate acetate (PMA). Since the respiratory manifestations of cystic fibrosis (CF) are characterized by intense chronic airway inflammation very early in life, successful gene therapy for CF will require that expression of the transferred normal CFTR gene be resistant to down-regulation by inflammatory mediators. To evaluate the concept that a viral promoter--human CFTR cDNA unit would be resistant to this form of down-regulation, a retrovirus promoter (5' long terminal repeat of the Moloney murine leukemia virus)--human CFTR cDNA unit was transferred to T84 human colon carcinoma cell line using a retrovirus vector. Exposure of the retrovirus-modified T84 cells to PMA resulted in down-regulation of the endogenous CFTR mRNA transcripts (6.5 kb), but did not affect the level of exogenous CFTR transcripts (8.0 kb). Importantly, in parallel with the persistence of the exogenous CFTR transcripts, the modified cells still maintained cAMP-regulated CI- secretion in the presence of PMA. These in vitro data suggest that a constitutive viral promoter--CFTR cDNA unit should be resistant to modulation by inflammatory stimuli, a likely requirement for successful gene therapy for CF.
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PMID:Transfer of a constitutive viral promoter-cystic fibrosis transmembrane conductance regulator cDNA to human epithelial cells conveys resistance to down-regulation of cAMP-regulated Cl- secretion in the presence of inflammatory stimuli. 752 42

In utero somatic gene transfer may be a useful therapeutic strategy for a variety of inherited disorders. In the present study, we demonstrate transgene expression in the airways of fetal lamb lungs, 2-3 weeks after injection of Moloney murine leukemia retrovirus based vectors containing cDNA for beta-galactosidase (lacZ) or human interleukin receptor antagonist protein (IRAP), into the fluid filled future airspace of fully catheterized twin fetal lambs (104-117 days gestational age; term 147 days). Expression of lacZ or IRAP was limited to the twin that received the respective vector and was apparent, at light microscopic level, in the epithelium and submucosal space of proximal airways, and to a lesser extent, in the respiratory epithelium of the distal airways. These data demonstrate for the first time that transfer of foreign DNA to fetal lung can be accomplished. These findings support the use of retroviral vectors for somatic lung DNA transfer and suggest that inherited disorders such as cystic fibrosis may be approached therapeutically via gene transfer, in utero.
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PMID:Retrovirus-mediated gene transfer in lungs of living fetal sheep. 767 Nov 10

In 1992 black yeasts of the species Exophiala dermatitidis were isolated for the first time from patients at the University Clinics in Dresden. Since that time this relatively rarely detected fungus has been frequently cultivated from clinical specimens. Our observations were: Patient with acute lymphatic leukaemia: In a 3 1/2 years old boy E. dermatitidis was isolated from 8 blood cultures during a septicaemic phase. Elimination of the fungus and decreasing of the fever were reached after removing a central venous catheter and treatment with amphotericin B and 5-fluorocytosine for 3 weeks. In this patient E. dermatitidis was assessed to be the cause of the septicaemia setting in via catheter. Patients with cystic fibrosis: In 8 of 51 mycologically surveyed patients E. dermatitidis was frequently - in 2 cases for a long time up to 7 months - isolated from sputum specimens. The occurrence of this fungus was considered to be a colonization with subclinical development. In these patients no fungal invasion or systemic mycosis were seen. The administration of itraconazole for 4 respectively 7 months did not succeed in eliminating E. dermatitidis out of the respiratory tract. It is recommended to include mycological longtime cultures in the surveillance of cystic fibrosis patients for detection of E. dermatitidis.
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PMID:[Detection of Exophiala dermatitidis (Kano) De Hoog 1977 in septicemia of a child with acute lymphatic leukemia and in patients with cystic fibrosis]. 785 73

In several childhood diseases which have the ensuing risk of infertility in adult life because of direct hypothalamic-pituitary-testicular axis involvement, or as a consequence of therapeutic toxicity, the role of antisperm antibodies (ASA) is rarely addressed. The aim of this study was to investigate the occurrence of ASA in a large prepubertal male population (aged 1.2-13 years) consisting of three groups: Group I, 52 patients affected by malignant diseases (lymphoblastic leukaemia, malignant lymphoma, or Wilm's tumour, n = 42), or by nephrotic syndrome (n = 10); Group II, 212 patients with either genital tract abnormalities (cryptorchidism, inguinal hernia, funicular torsion or hypospadias, n = 202), or cystic fibrosis (n = 10); Group III: 100 age-matched normal boys. Group I and II patients were investigated at diagnosis and during or after treatment (drug, radiation or surgical therapy). Group III was used as controls. ASA were detected in sera by the Tray Agglutination Test (TAT) and indirect IgG, IgA and IgM immunobead tests (iIBT). All normal boys were ASA-negative using both tests. Twenty-six out of the 264 patients (9.8%) in Groups I and II were ASA-positive: 23 (8.7%) patients had a positive TAT with a titre of 1:32 to 1:128, whilst 14 (5.3%) had IgG-ASA after iIBT. Eleven patients (4.1%) were ASA-positive in both tests. Of the 26 ASA-positive boys, 24 had genital tract abnormalities (cryptorchidism, testicular torsion, hypospadias) and two had leukaemia with testicular infiltration. Treatment did not modify antibody positivity. Our data confirm that ASA can occur in prepubertal boys, mostly among cases with urogenital pathology, but that it is rare among other cases. Therefore autoimmune reaction against spermatozoa is another factor that should be considered in the evaluation of several conditions in childhood involving reproductive tract alteration and potential impairment of the blood testis (Sertoli cell) barrier.
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PMID:Antisperm antibodies in prepubertal boys treated with chemotherapy for malignant or non-malignant diseases and in boys with genital tract abnormalities. 920 87


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