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Biomedical subjects

S O Lie

Publications and source records attributed to S O Lie.

At least 37 records · Page 2Linked to original sources
PubMed1995

[Neonatal screening for metabolic diseases--a task without priority in the Norwegian health policy?].

The Norwegian national screening programme for early diagnosis of phenylketonuria and congenital hypothyroidism was established in 1978-79. The organization and implementation of the programme is based on a marginal cost policy profile and has kept almost the same structure throughout the period of national neonatal screening. We focus on practical measures aimed at increasing the quality of the programme, and suggest a health political reconsideration of public preference and financial support for neonatal screening in Norway.

Congenital Hypothyroidism↗
PubMed1995

RG1, a new murine monoclonal antibody recognizing a "supertypic" determinant on HLA-A molecules.

Monomorphic and polymorphic anti-HLA monoclonal antibodies (mAb) are valuable reagents for assessment of the structural and functional importance of different class I determinants. We have generated a new mAb, RG1, reacting with an epitope variably expressed on normal and leukemic hematopoietic cells of different lineages. Immunoprecipitation of the RG1 antigen disclosed a bimolecular complex characteristic of class I proteins. The RG1 epitope was expressed on an HLA-A2 transfected cell line but not on cells transfected with HLA-E, -F or -G molecules. MAb reactivity with reference B-lymphoblastoid cell lines and HLA typing of RG1 reactive and unreactive cells demonstrated that the epitope was expressed in conjunction with defined HLA-A molecules. Cells expressing HLA-A2, -A24(9) and -A68(28) proteins were brightly stained with RG1 whereas mAb binding to HLA-A1, -A11 and a split of A3 molecules was significantly lower. In contrast, the RG1 epitope was apparently not expressed on HLA-A23(9), -A25(10), -A26(10), -A29(19), -A30(19), -A31(19), -A32(19), -A33(19) and some HLA-A3 molecules. Based on class I alpha sequence data, these results suggest that the RG1 epitope is localized to a region of the alpha 2 helix accessible to the T cell receptor for antigen on cytotoxic T lymphocytes. Lys in position 144 and His in position 151 are apparently critical for RG1 binding.

Amino Acid Sequence↗
PubMed1994

[Second cancer. Risk of a second malignant neoplasm in persons with cancer in childhood and adolescence].

Cancer treatments in early life have in previous studies been associated in with high risks of developing a second malignant neoplasm. This study reports on the relative and attributable risks of second malignant neoplasms among 30,880 people under the age of 20, who had been identified in the files of any of the five Nordic cancer registers, 1943-1987. Overall, 247 cases of second malignant neoplasms were observed in 238 patients, yielding a relative risk for cancer of 3.6 (95% confidence interval 3.1-4.1). The risk changed significantly from 2.6 in people first diagnosed during the 1940s and 1950s to 6.9 among cohort members included in the late 1970s and 1980s. Highest levels of the relative risk were seen during the ten years immediately after first malignant diagnosis. The incidence of second malignant neoplasms attributable to the first cancer and associated treatments, however, showed a consistent rise throughout the 45 years of follow up. It was concluded that the estimated risks for second malignant neoplasms were significantly lower than those found in most large hospital based studies but compatible with the results from a similar population based study in the United Kingdom. Extent of risk and cancer pattern were similar among the Nordic countries and are believed to be representative for a large part of the European population.

Adolescent↗
PubMed1994

Uptake and storage of retinol and retinyl esters in bone marrow of children with acute myeloid leukemia treated with high-dose retinyl palmitate.

Twenty-one children with AML, who achieved complete remission with cytostatics, were treated with high doses of retinoids after remission was achieved. They were given 52 mumol retinyl palmitate/m2 (50,000 International Units/m2) daily for at least 2 years thereafter. Thirteen of the children are still in complete remission with a mean observation time of 103 months. Due to the positive effects of retinoids seen in the treatment of AML, we have studied uptake of lipoprotein-associated retinyl esters in bone marrow cells and peripheral leukocytes in vivo. An oral load of 104 mumol/m2 (100,000 International Units/m2) retinyl palmitate resulted in a doubling of the concentration of total retinol in bone marrow cells and peripheral leukocytes after 5 hours. However, in the fasting state no significant difference was observed between the content of total retinol in bone marrow cells from controls and from patients receiving retinyl palmitate daily for years. Our study suggests that bone marrow cells take up retinyl esters postprandially, but do not store retinoids.

Anticarcinogenic Agents↗
PubMed1993

Severe congenital neutropenia: abnormal growth and differentiation of myeloid progenitors to granulocyte colony-stimulating factor (G-CSF) but normal response to G-CSF plus stem cell factor.

Several mechanisms have been proposed to explain the pathogenesis of severe congenital neutropenia (SCN); however, the mechanism(s) still remains unknown. In particular, clinical observations suggest that abnormal responsiveness of myeloid progenitors to hematopoietic growth factors (HGFs) is a possible mechanism. Therefore, to better define the status of hematopoietic progenitors in the bone marrow (BM) of patients with SCN, the responsiveness of myeloid progenitors to HGFs from two SCN patients was compared with the responsiveness of progenitors from healthy individuals. BM cells (BMCs) from the first SCN patient required higher (10- to 100-fold) concentrations of granulocyte colony-stimulating factor (G-CSF) to achieve maximal and half-maximal colony growth in vitro compared with BMCs from controls. In contrast, the dose-response of interleukin-3 (IL-3) and granulocyte-macrophage-CSF (GM-CSF) in colony formation was normal. Interestingly, IL-3, GM-CSF, and G-CSF at optimal doses showed reduced ability to induce neutrophil differentiation of BMCs from a SCN patient compared with BMCs from controls. Despite an abnormal responsiveness of mature myeloid progenitors to G-CSF in this SCN patient, myeloid progenitors responsive to the combination of stem cell factor (SCF) and G-CSF showed normal dose-response. In contrast to G-CSF alone, the combination of G-CSF and SCF induced the formation of neutrophils almost to the same extent compared with cultures of normal BMCs. Furthermore, also on BM progenitor cells obtained from the second patient with SCN, SCF highly synergized with G-CSF to promote neutrophil progenitor cell growth and differentiation in vitro. Thus, these results indicate that one mechanism of the pathogenesis in SCN patients is reduced responsiveness of neutrophil progenitor cells to G-CSF and that SCF can enhance the responsiveness of these cells to G-CSF.

Bone Marrow↗
PubMed1993

Second malignant neoplasms after cancer in childhood or adolescence. Nordic Society of Paediatric Haematology and Oncology Association of the Nordic Cancer Registries.

OBJECTIVE: To assess the relative risk of developing a second malignant neoplasm in people with a diagnosis of cancer in childhood and adolescence. DESIGN: Register based follow up study. SETTING: Populations of Nordic countries. SUBJECTS: 30,880 people under the age of 20 with a first malignant neoplasm diagnosed during the period 1943-87. MAIN OUTCOME MEASURES: Relative and attributable risks of second malignant neoplasms by type of first cancer, age at first diagnosis, calendar period, sex, and country. Expected figures were based on the appropriate national incidence rates for cancer. RESULTS: 247 cases of second malignant neoplasms were observed in 238 patients, yielding a relative risk for cancer of 3.6 (95% confidence interval 3.1 to 4.1). The risk changed significantly from 2.6 in people first diagnosed during the 1940s and 1950s to 6.9 among cohort members included in the late 1970s and 1980s. Increases were observed for most types of cancer. Highest levels of the relative risk were seen during the 10 years immediately after first malignant diagnosis. The incidence of second malignant neoplasms attributable to the first cancer and associated treatments, however, showed a consistent rise throughout the 45 years of follow up. CONCLUSION: The estimated risks for a second malignant neoplasm were significantly lower than those found in most large hospital based studies but compatible with the results from a similar population based study in the United Kingdom. Extent of risk and cancer pattern were similar among the Nordic countries and are believed to be representative for a large part of the European population.

Adolescent↗
PubMed1993

[100 years of pediatrics in Norway. The history up to 1950].

The first department of pediatrics in Norway was opened at Rikshospitalet (The National Hospital of Norway) in January 1893. This marked the start of pediatrics as an independent discipline and specialty in Norwegian medicine. Upon this centennial we present the history of the department (and of pediatrics in Norway) up to 1950.

History, 19th Century↗
PubMed1993

Prophylactic cranial irradiation increases the risk of testicular damage in adult males surviving ALL in childhood.

By combining three series of Scandinavian patients, we were able to compare the late testicular sequelae in 41 adult males whose therapy had included chemotherapy alone or chemotherapy with cranial irradiation without other radiotherapy for ALL in childhood. In multivariate analysis, cranial irradiation was associated with a decrease of 5.7 (95% confidence limits 1.5-9.9) cm (P = 0.010) in height, and a decrease of 4.8 (0.3-9.2) ml (P = 0.036) in testicle size. Cyclophosphamide was associated with increases of 8.2 (-0.5-16.9) (P = 0.065) and 3.9 (0.3-7.4) U/L (P = 0.036) in serum FSH and LH concentrations, respectively. Of the 12 patients who had received both cranial irradiation and cyclophosphamide therapy, 4 (33%) had delayed pubertal development as compared with 1 (3.5%) of the other 29 patients (P = 0.008). Patients 12-16 years of age at diagnosis had larger testicles (P = 0.051) and higher testosterone concentrations (P = 0.026) than others. Neither sexual activity nor semen findings correlated with the preceding treatment. Our data indicate that prophylactic cranial irradiation may be associated with impaired growth and pubertal development in boys with ALL.

Adolescent↗
PubMed1993

Leukemic blasts with markers of four cell lineages in Down's syndrome ("megakaryoblastic leukemia").

Among 16 patients with Down's syndrome (DS) and acute leukemia admitted to our department during a ten year period, 6 were diagnosed as acute megakaryoblastic leukemia (AMkL). The diagnosis was based on clinical and hematologic criteria, confirmed in three patients with the use of monoclonal antibodies (MoAb) specific for megakaryocytic antigens. In these three, and in a fourth patient, the leukemic blasts were positive for other myeloid, lymphoid and erythroid markers in MoAb testing. We suggest that AMkL in DS is a mixed lineage leukemia with blasts presenting a variety of cell surface antigens, indicating origin from an early progenitor cell with the capability of megakaryocytic differentiation. Of the 6 patients with AMkL, 4 treated with standard AML protocols are in complete continuing remission (CCR) with observation periods from 57+ to 148+ months.

Adolescent↗
PubMed1992

Differential chromatin structure-dependent binding of 7-aminoactinomycin D in normal and malignant bone marrow hematopoietic cells.

Chromatin structure-dependent binding of the DNA-specific dye 7-aminoactinomycin D (7-AMD) in leukemic and normal cells in bone marrow aspirates from childhood acute leukemia patients and patients without bone marrow neoplasia was assessed by multiparameter flow cytometry. Simultaneous staining with fluorescein isothiocyanate-labeled antibodies was needed in many cases for determination of the immunophenotype of the cells that exhibited differential binding of 7-AMD. 7-AMD binding was enhanced in normal (4 patients) and malignant (8 patients) myeloid cells, and was generally low in normal and leukemic lymphocytes and normoblasts. Four of 18 aspirates from 16 patients with acute lymphoblastic leukemia contained neoplastic cells with increased 7-AMD binding capability. The 7-AMD binding of the leukemic cells was not correlated to S-phase fraction (P = 0.07), but was significantly correlated to cell size as measured by forward angle light scattering (r = 0.49, P = 0.007). Patients with tumor cells exhibiting low 7-AMD binding at last aspirate survived significantly longer than the patients with leukemic cells binding high amounts of 7-AMD (P = 0.03). Neither cell size, S-phase fraction, nor ploidy status predicted patient survival in this small scale study.

Antigens, CD↗
PubMed1992

VACTERL or MURCS association in a girl with neurenteric cyst and identical thoracic malformations in the father: a case of gonosomal mosaicism?

We report on a female infant with lethal congenital malformations including extreme hydrocephalus due to aqueductal stenosis, vertebral segmentation anomalies, fused costae, anal atresia, renal dysplasia, and bicornuate uterus with a double blind vagina. The VACTERL and the MURCS associations are possible diagnoses. Her father had a neurenteric cyst in infancy. He has identical vertebral and costal malformations as his daughter but is otherwise healthy. The possibility of dominant inheritance with gonosomal mosaicism in the father is discussed.

Abnormalities, Multiple↗
PubMed1992

Superior treatment results in females with high-risk acute lymphoblastic leukemia in childhood.

In this population-based study, 808 children aged 1-15 years from Denmark, Finland, Iceland, Norway and Sweden, were diagnosed between July 1981 and June 1986 as suffering from non-B-cell acute lymphoblastic leukemia (ALL). The total population was 4.5 million children. Remission was achieved in 770/808 of the patients (95%). No sex difference in the remission rate was observed. The event free survival (EFS) at 102 months was 0.47 for males and 0.62 for females (p less than 0.001). There was no difference in EFS between males and females with standard-risk (0.58 and 0.60) or intermediate-risk (0.47 and 0.60) ALL, respectively. The EFS for females with high-risk ALL (0.68) was superior to that of males with high-risk ALL (0.31). Cox multivariant analysis showed that white blood cell count, sex, age and thrombocyte count were significant prognostic factors in all children. The intensified treatment according to the prognostic factors used in this study led to equal EFS for females with ALL from all risk groups. Males with high-risk ALL, however, did not benefit from the intensified treatment.

Age Factors↗
PubMed1992

Progress in the treatment of childhood leukaemias.

The Nordic Society for Pediatric Hematology and Oncology (NOPHO) has run a population-based registry on all cases of acute leukaemias in the Nordic countries since 1981. Data on close to 2000 children with these diagnoses are presented and used as a background for a general discussion of progress in the therapy of these challenging conditions. Our material is unique in that it is population based. The results obtained are comparable to those obtained by all other major cooperative groups. Since January 1992, the treatment protocols for all types of acute leukaemias in childhood have been harmonized in the Nordic countries.

Adolescent↗
PubMed1991

Interleukin-7 differentiates a subgroup of acute lymphoblastic leukemias.

The bone marrow stromal cell-derived growth factor interleukin-7 (IL-7) is known to stimulate growth of normal human B-cell precursors. In the present report, we have examined the effect of IL-7 on neoplastic B-cell precursors. Leukemic cells from 20 patients with common acute lymphoblastic leukemia (ALL) were highly purified by removing contaminating T cells and monocytes by rosetting with immunomagnetic beads. IL-7 markedly reduced the DNA synthesis in leukemic cells from three patients. This inhibition of DNA synthesis was accompanied by maturation of the cells, as demonstrated by the induced expression of the differentiation antigens CD19, CD20, CDw75, and surface mu-chain, and a decreased expression of terminal deoxynucleotidyl transferase. By examining G1 parameters, such as MYC, 4F2, and transferrin-receptor levels analyzed by flow cytometry as well as RNA and the cell cycle regulated antigen Ki67, it appeared that the cells were inhibited late in G1. Leukemic cells from the majority of the cases (12 of the 20 patients) responded to IL-7 with enhanced DNA synthesis without detectable maturation, as has been reported for their normal counterparts. Low molecular weight B-cell growth factor greatly potentiated the IL-7-induced growth stimulation of these cells. Thus, we have shown that IL-7 is capable of inhibiting proliferation of leukemic cells isolated from a subgroup of ALLs, and that this growth inhibition is accompanied by maturation of the cells.

Antibodies, Monoclonal↗
PubMed1991

[Children and diseases in the Third World].

Each year approximately 137 million children are born, 70 million of them in countries where the mortality rate for under-fives exceeds 100 per 1,000. In worst-off Afghanistan 300 per 1,000 children under five die. We review some of the most common causes of this catastrophy: diarrhoea, acute respiratory infections, measles, malaria, and neonatal tetanus. All of the numerically significant contributors to under-five mortality are treatable and/or preventable by simple and inexpensive methods. Medical personnel from industrialized countries, and there is an increasing need for transfer of financial resources from highly developed to developing countries. The living conditions of children in the third world involve many factors that are detrimental to both physical and mental health. In order to effect a change, all countries and societies must become involved.

Age Factors↗
PubMed1991

Management of pediatric pontine gliomas.

We present 36 consecutive patients with intrinsic glioma of the pons. Tumors with exophytic expansion were excluded. There were 16 females and 20 males, ranging in age from 2 to 13 years, median 6 years. The most common presenting symptoms were cranial nerve dysfunction, unsteadiness of gait, and hemiparesis. Computed tomography (CT) showed a hypodense (17/21) or isodense (4/21) expansion of the pons. Five tumors had areas of contrast enhancement. Following information about prognosis and possible types of management, parents decided for or against radiation therapy: twenty-four children underwent irradiation and 12 did not. Median survival among children receiving a full course of irradiation was 280 days, compared to 140 days in an equivalent group of non-irradiated children. Hemiparesis presenting without cranial nerve symptoms and contrast enhancement on CT scan were poor prognostic factors, whereas sex, age, and duration of symptoms at diagnosis were unrelated to prognosis.

Adolescent↗