[Erythropoietin therapy in premature infants with very low birth weight].
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Anemia↗
Biomedical subjects
M A Siimes
Publications and source records attributed to M A Siimes.
Chemotherapy of acute myelocytic leukemia in children.
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Adolescent↗
Childhood leukemia and body image: interview reveals impairment not found with a questionnaire.
Twenty-eight adolescent leukemia survivors and 34 healthy controls were examined by means of a self-report questionnaire and a semi-structured face-to-face interview to appraise the quality of their body image. To protect against observer bias a blind-rater was used. The self-report inventory suggested that survivors have a perfectly normal body image, whereas in the interview 36% of the survivors were rated as having an impaired and 36% a diffuse, body image. The survivors' characteristic attitude towards their physical appearance--to evade the difficult issue--because evident when there were no ready answers from which to choose. Thus, in evaluating the impact of cancer on body image the choice of method of inquiry is vital.
Acute Disease↗
Collagen formation and degradation increase during growth hormone therapy in children.
A comprehensive set of serum markers of collagen turnover and growth was investigated in a longitudinal study of short children during growth induced by growth hormone (hGH) treatment. The study comprised 18 prepubertal children with short stature who had no other current illness or continuous medication. The growth rates and endogenous GH secretions covered a continuum from subnormal to normal. Before treatment, the concentrations of carboxyterminal propeptide of type I procollagen (PICP), reflecting type I collagen formation, of carboxyterminal telopeptide of type I collagen (ICTP), a degradation product of type I collagen, of amino-terminal propeptide of type III procollagen (PIIINP), a marker for type III collagen formation, of alkaline phosphatase (AP), and of insulin-like growth factor binding protein-3 (IGFBP-3) were within the lower limits of normal. The median IGF-I concentration was lower than the reference. One week after the start of treatment, the serum concentrations of ICTP, PIIINP, and osteocalcin (OC), and the increments in ICTP, PIIINP, and IGF binding protein-3 (IGFBP-3) correlated with the subsequent height velocity. During the 12-month treatment, all markers were higher than those of age-matched references, but only the three collagen markers paralleled the changes in height velocity. In molar concentrations, ICTP increased less than PICP. Throughout the study period, the serum level of ICTP correlated with that of PIIINP, but not with that of PICP. The findings suggest that during hGH treatment, linear body growth is closely associated with collagen formation and degradation.
Alkaline Phosphatase↗
Very low rate of type I collagen synthesis and degradation in newly diagnosed children with acute lymphoblastic leukemia.
In children with acute lymphoblastic leukemia (ALL), the metabolism of type I collagen, the major collagen of bones, may be changed at diagnosis and during early chemotherapy. In the present study, bone formation and degradation rates were evaluated longitudinally in 35 children with ALL, using two serum markers of bone collagen formation: the amino-terminal (PINP) and carboxyterminal (PICP) propeptides; and a marker of degradation: the carboxyterminal telopeptide of type I collagen (ICTP). These serum markers were determined at diagnosis, during induction treatment (at 1, 4, and 6 weeks), and during consolidation treatment (at 8 and 12 weeks). The changes in the serum markers suggested that, at diagnosis, type I collagen turnover (i.e., both synthesis and degradation) was remarkably low. The median serum levels of PINP, PICP, and ICTP were -2.6 SDS (standard deviation score), -1.5 SDS, and -2.5 SDS, respectively. The PICP and PINP levels declined further during the first week of therapy (p < 0.001), whereas the ICTP levels had risen by end of the induction phase (p < 0.05). By the end of the 12 week interval, the concentrations of the formation and degradation markers had returned to normal (p < 0.01). Our findings suggest that ALL is accompanied by low turnover of bone collagen. The abnormalities are at first aggravated, but then corrected, by treatment.
Adolescent↗
Recurrent gains of 1q, 8 and 12 in the Ewing family of tumours by comparative genomic hybridization.
Comparative genomic hybridization (CGH) was used to detect copy number changes of DNA sequences in the Ewing family of tumours (ET). We analysed 20 samples from 17 patients. Fifteen tumours (75%) showed copy number changes. Gains of DNA sequences were much more frequent than losses, the majority of the gains affecting whole chromosomes or whole chromosome arms. Recurrent findings included copy number increases for chromosomes 8 (seven out of 20 samples; 35%), 1q (five samples; 25%) and 12 (five samples; 25%). The minimal common regions of these gains were the whole chromosomes 8 and 12, and 1q21-22. High-level amplifications affected 8q13-24, 1q and 1q21-22, each once. Southern blot analysis of the specimen with high-level amplification at 1q21-22 showed an amplification of FLG and SPRR3, both mapped to this region. All cases with a gain of chromosome 12 simultaneously showed a gain of chromosome 8. Comparison of CGH findings with cytogenetic analysis of the same tumours and previous cytogenetic reports of ET showed, in general, concordant results. In conclusion, our findings confirm that secondary changes, which may have prognostic significance in ET, are trisomy 8, trisomy 12 and a gain of DNA sequences in 1q.
Adolescent↗
Impaired androgen production in female adolescents and young adults after total body irradiation prior to BMT in childhood.
Pubertal development and androgen production were evaluated 1-10 years after bone marrow transplantation (BMT) in 15 females aged 14-23 (mean 17) years. Before BMT, these patients had received combination chemotherapy for hematologic malignancy, and all had had a transplant program including total body irradiation (TBI). Of the nine patients who were pre-menarcheal at BMT, two had subsequently experienced spontaneous menarche at 11.5 and 13.3 years of age. Six were post-menarcheal, but became amenorrheic after BMT. Menstruation subsequently started spontaneously in one of them 6 years after BMT. At the time of the study, three patients were early to mid-pubertal and 12 late pubertal or post-pubertal. Twelve patients were receiving sex steroid substitution therapy. Serum concentrations of testosterone, androstenedione, dehydroepiandrosterone (DHEA) and DHEA sulfate (DHEAS) were determined. Androgen levels of late pubertal and post-pubertal transplanted patients were compared with 19 post-menarcheal patients aged 14-21 (mean 17) years who had been treated for hematologic malignancy with conventional chemotherapy. Testosterone levels of 52 healthy post-menarcheal females aged 14-29 (mean 19) years were measured as controls. Androgen levels of the BMT patients were lower than those of the conventionally treated patients. Differences in testosterone, androstenedione and DHEA levels were significant. Three spontaneously menstruating BMT patients had normal androgen levels. Testosterone levels of the conventionally treated patients and healthy controls were similar. Subnormal androgen production might be one factor behind the problems in pubertal development and sex life experienced by females after BMT. The use of these hormone levels for follow-up purposes and the potential value of androgen replacement therapy in females after TBI merit further study.
Adolescent↗
Body iron stores in relation to growth and pubertal maturation in healthy boys.
During male puberty, erythropoiesis is exceptionally active. Pubertal development and iron status were followed in 60 healthy boys at 3-month intervals for 24 months to evaluate changes in body iron stores with the serum transferrin receptor-ferritin ratio. The estimated amount of stored iron declined by about 50% over a 2-year period. Remarkable changes in iron stores were found even after as short an interval as 3 months and pubertal development was closely linked with a decrease in stored iron. The annual increments of estimated red blood cell (RBC) iron showed strong positive correlations with velocities in testicular volume and certainly in body height and weight. In contrast, the estimated changes in individual iron stores were not associated with any of those parameters. The lacking associations between iron stores and growth parameters are probably indicative of increasing intestinal absorption. Despite the relatively small quantitative role of iron stores in supplying iron for growth, falling iron stores probably play a more important regulatory role by stimulating iron absorption.
Adolescent↗
Assessment of plasma fibronectin in malnourished Nigerian children.
BACKGROUND: Severe malnutrition is associated with septic infections. The concentrations of fibronectin, albumin, and transferrin in plasma were measured in three groups of children with protein-energy malnutrition, aged 1-3 years, each group comprising 20 children. The aim of which was to test whether plasma fibronectin, being an opsonic protein, was reduced in such children, and if it was a useful index for assessing the severity of malnutrition. METHODS: The concentrations of fibronectin, albumin, and transferrin in plasma were studied by enzyme immunoassay/ immunoblotting, spectrophotometry and immunoturbidimetry respectively. RESULTS: All values were significantly lower in the patients with malnutrition than in the age- and sex-matched well-nourished Nigerian reference children. Within the malnourished group, the fibronectin value was evenly reduced in all subgroups. Albumin and transferrin values were lowest in the patients with kwashiorkor, highest in the marasmic patients, and intermediate in the patients with marasmic kwashiorkor; the values correlated mutually in individual cases as well, but not with the fibronectin levels. Neither plasma fibronectin fragmentation nor tissue fibronectin was detected in any patient with malnutrition or in the reference subjects. CONCLUSIONS: The reduced plasma fibronectin values in these patients may be due to reduced synthesis by the liver, as evidenced by the equally reduced albumin and transferrin concentrations and/or to the multiple infections characteristic of the patients.
Biomarkers↗
Iron status of children with short stature during accelerated growth due to growth hormone treatment.
We determined the influence of human growth hormone (hGH) treatment on blood soluble transferrin receptor (sTfR) in 35 children with short stature. Whereas the serum concentration of ferritin decreased from 29.6 micrograms/l to 19.7 micrograms/l, and that of transferrin increased from 2.9 g/l to 3.2 g/l during 6 months (p < 0.001), only a minimum rise in the sTfR concentration was observed (7.12 +/- 0.20 mg/l vs 7.51 +/- 0.19 mg/l, p = 0.025). The prevalence of anaemia or microcytosis did not increase. Most of the changes in serum ferritin and transferrin concentrations occurred during the first week. The study demonstrates that rapid body growth per se does not affect the sTfR concentration, but it may affect the serum transferrin and ferritin concentrations. Alternatively, GH may have a specific effect on serum ferritin and transferrin concentrations.
Body Constitution↗
Cholesterol and carotid artery wall in children and adolescents with familial hypercholesterolaemia: a controlled study by ultrasound.
The carotid artery wall was studied with ultrasound in 23 children and adolescents with familial hypercholesterolaemia and in 23 age-matched healthy controls. The study revealed changes in the carotid artery wall related both to familial hypercholesterolaemia and to age. In the control subjects, the carotid artery wall became stiffer with age. In the patients with hypercholesterolaemia, no clear age-dependence was found, but wall stiffness correlated with total and low-density lipoprotein cholesterol. The intimal-medial wall thickness was associated with serum total cholesterol, low-density lipoprotein and triglyceride concentrations, and correlated inversely with the ratio of high-density lipoprotein to total cholesterol. Carotid artery wall properties seem to be associated with the degree of hypercholesterolaemia and the high-density lipoprotein-to-total cholesterol ratio even in children. In childhood and adolescence it is already possible, with ultrasound, to detect changes in the arterial wall related both to familial hypercholesterolaemia and to age.
Adolescent↗
Sexuality of young women surviving leukaemia.
This study was designed to assess the sexuality of young women surviving acute leukaemia in childhood or early adolescence. Thirty of 31 survivors were compared with 50 healthy age matched controls. Three methods were used: a self report questionnaire, a face to face interview conducted by a psychiatrist, and a projective psychological test. The age at initiation of dating and sexual activity, the frequency of sexual intercourse, and opinions on sexual behaviour were similar in the two groups. With regard to inner sexuality, however, the survivors differed significantly from the healthy controls. Their images of sexuality were more restrictive, and their attitudes, especially those concerning sexual pleasure, were more negative than those of the controls. Sexual identity among the survivors was less often feminine and more often infantile as compared with the controls. The findings obtained with the three methods of assessment were concordant.
Adult↗
Body iron stores decrease in boys during pubertal development: the transferrin receptor-ferritin ratio as an indicator of iron status.
The transferrin receptor in serum provides a useful measure of tissue iron deficiency and the rate of erythropoiesis, whereas serum ferritin reflects the amount of storage iron in normal subjects. We studied the serum transferrin receptor and the transferrin receptor-ferritin ratio in 57 healthy prepubertal or early pubertal boys and followed them at 3-mo intervals for 24 mo to evaluate their iron status during puberty. The mean laboratory parameters changed as follows: Hb from 13.0 to 13.3 g/dL (p = 0.01), mean corpuscular volume from 85 to 84 fL (p = 0.0001), transferrin receptor from 6900 to 7200 micrograms/L (p = 0.03) ferritin from 36 to 23 micrograms/L (p = 0.0001), and transferrin receptor-ferritin ratio from 230 to 400 (p = 0.0001). At the start of the investigation, the serum transferrin receptor was elevated (> 9000 micrograms/l) or ferritin low (< = or 12 micrograms/L) in fewer than 2% of the boys. During the subsequent 2 y the proportion of boys with an elevated transferrin receptor or low ferritin value increased. The two parameters were simultaneously abnormal in none of the boys initially, but in about 3% of the boys 2 y later. The change in transferrin receptor-ferritin ratio was closely correlated with genital development. The proportion of elevated transferrin receptor-ferritin ratios increased 4.5-fold during the 2 y, indicating the high responsiveness of the ratio. At the end of the study, iron therapy was started to eliminate any iron deficiency. In response to the therapy, the mean transferrin receptor-ferritin ratio fell to 210 +/- 19, i.e. close to the level at the beginning of the study. The marked responses of the transferrin receptor and the receptor-ferritin ratio to iron therapy reflect the dependence of these parameters on iron status rather than on physiologic differences in the rate of erythropoiesis.
Adolescent↗
Serum markers of collagen metabolism and serum osteocalcin in relation to pubertal development in 57 boys at 14 years of age.
We investigated whether levels of serum collagen markers and serum osteocalcin are related to pubertal growth and development in a cross-sectional study of 57 healthy boys at 14 y of age. The level of the soft tissue marker, serum amino-terminal propeptide of type III procollagen (PIIINP) was higher in boys at Tanner stages G3 versus G2 (p < 0.01). The levels of the markers of bone collagen matrix differed only at a more advanced pubertal stage: the formation markers, carboxy-terminal and amino-terminal propeptides of type I procollagen, and the degradation marker, carboxy-terminal telopeptide of type I collagen were higher only at stage G4 versus G3 (p < 0.01). The marker of bone mineralization, serum osteocalcin was also higher only at stage G4 versus G3 (p < 0.01). Stage G4 was associated with the pubertal growth spurt. The results demonstrate that pubertal development should be taken into account when serum levels of collagen markers and osteocalcin are evaluated, and suggest that an increase in serum PIINP in boys at G3 might predict a normal pubertal growth spurt, but the finding remains to be confirmed in longitudinal studies.
Adolescent↗
[Continuing medical education for pediatricians, a pilot project].
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Adult↗
[Leukemia treatment results have improved, but prognosis is still questionable].
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Child↗
Apoprotein E phenotype determines serum cholesterol in infants during both high-cholesterol breast feeding and low-cholesterol formula feeding.
Our objective was to establish the role of the apoprotein (apo) E phenotype in determining serum cholesterol levels in infants fed exclusively on high-fat, high-cholesterol human milk and in those fed a low-cholesterol, high-unsaturated fat formula. The total and lipoprotein cholesterol, apoB, and triglyceride concentrations in serum were quantified and related to the apoE phenotype in 151 infants at birth and at 2, 6, 9, and 12 months of age. Forty-four had the E3/4 or 4/4 phenotype (E4 group), 94 had the E3/3 phenotype (E3 group), and 13 had the E2/3 or 2/4 phenotype (E2 group). In cord blood, cholesterol concentrations tended to be higher in the E4 than in the E2 group. With exclusive breast-feeding, the concentrations rose significantly faster and higher in the E4 group than in the E3 group or, especially, the E2 group. The values (mmol/L, mean +/- SEM) were 1.6 +/- 0.15, 1.5 +/- 0.05, 1.4 +/- 0.1 (P = n.s.) at birth; 4.2 +/- 0.1, 3.8 +/- 0.08, 3.4 +/- 0.2 (P < 0.001) at 2 months; 4.4 +/- 0.15, 3.9 +/- 0.1, 3.4 +/- 0.15 (P < 0.001) at 4 months; 4.3 +/- 0.17, 4.0 +/- 0.13, 3.7 +/- 0.26 (P < 0.001) at 6 months; 4.8 +/- 0.28, 4.4 +/- 0.11, 3.8 +/- 0.05 (P < 0.001) at 9 months; and 4.7 +/- 0.11, 4.4 +/- 0.08, 4.1 +/- 0.19 (P < 0.001) at 12 months, for the E4, E3, and E2 groups, respectively. Increases in LDL cholesterol and LDL apoB behaved similarly. The total triglyceride, and total HDL, HDL2, and HDL3 cholesterol concentrations did not depend on the apoE phenotype. Among infants fed high-fat, high-cholesterol human milk, the total and LDL-cholesterol concentrations and the LDL apoB concentration of those with the apoE phenotype 4/4 or 3/4 rose faster and to higher levels than in other infants. Among formula-fed infants, receiving a low-cholesterol, high-unsaturated fat diet, the differences between the apoE groups were smaller.
Aging↗
Hemoglobin level is linked to growth hormone-dependent proteins in short children.
Erythropoiesis was investigated in 32 children wih short stature and in eight children with skeletal dysplasia by studying blood hemoglobin in relation to growth and to serum concentrations of insulin-like growth factor I (IGF-I), IGF binding protein-3 (IGFBP-3), and erythropoietin (EPO) before, during, and after 12 months of recombinant human growth hormone (GH) treatment. Blood hemoglobin concentration was positively correlated with relative body height and with serum IGF-I and IGFBP-3 levels (P = .001 to .02), but not with the concentrations of EPO. The normal age-dependency of hemoglobin was lacking. Hemoglobin levels and their responses to GH treatment were similar in the patients with GH deficiency and those with normal GH secretion. Treatment with GH accelerated growth and elevated the concentrations of hemoglobin, IGF-I, and IGFBP-3. In the eight patients with skeletal dysplasia, body mass increased similarly, but gain in height was less than in the other patients, and the increase in hemoglobin was markedly pronounced. In this group, the correlations between hemoglobin, IGF-I, and IGFBP-3 were extremely close (r = 0.80 to 0.85, P = .031 to .008). These findings are in accord with earlier observations from in vitro and animal studies, and suggest that the GH-IGF axis is involved in the physiologic elevation of hemoglobin levels during childhood.
Achondroplasia↗