bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effect of growth hormone (GH) during puberty in GH‐deficient children: preliminary results from an ongoing randomized trial with different dose regimens
Authors: Wikland K Albertsson , Alm F , Aronsson S , Gustafsson J , Hagenäs L , Häger A , Ivarsson S , Kriström B , Marcus C , Moëll C , Nilsson KO , Ritzén M , Tuvemo T , Westgren U , Westphal O , Åman J 
Published: April 2008

Albertsson Wikland K, Alm F, Aronsson S, Gustafsson J, Hagenas L, Hager A, Ivarsson S, Kristrom B, Marcus C, Moe11 C, Nilsson KO, RitzCn M, Tuvemo T, Westgren U, Westphal 0, Aman J. Effect of growth hormone (GH) during puberty in GH‐deficient children: preliminary results from an ongoing randomized trial with different dose regimens. Acta Pxdiatr 1999; Suppl 428:80‐4. Stockholm. ISSN 0803‐5326This paper reports results from an ongoing, randomized, multicentre national trial. The aim is to elucidate whether a dose of growth hormone (GH) of 0.2 IUkg (0.07 mgkg), given either as once‐daily or twice‐daily injections during puberty, is more effective than a once‐daily dose of 0.1 IU/kg/day (0.03 mg/kg/day) in improving final height in children with GH deficiency (GHD). The twice‐daily regimen comes closer to the spontaneous GH secretion pattern in puberty. Ninety‐two children with GHD who had been receiving GH therapy for at least 1 year, and with spontaneous puberty or who were prepubertal and due to be started on replacement therapy to induce puberty, were randomly assigned to receive GH as follows: group A, 0.1 IU/kg/day (0.03 mg/kg/day), administered once daily; group B, 0.2 IU/kg/day (0.07 mg/kg/day), administered once daily; and group C, 0.2 IUkg/day (0.07 mg/kg/day), divided into two equal injections given at 12‐hour intervals. Pubertal height gain was 0.7, 0.7 and 1.3 SDS for groups A, B and C, respectively. The gain in height during puberty was thus most marked in group C. Mean final height, when corrected for parental height, was between 0 and 1 SDS in all treatment groups. All but seven children reached a final height within 2 SD of the general population. There was a wide range of final heights in all three treatment groups. This variation in response suggests the need to individualize treatment in order to achieve an appropriate final height for most individuals. □Dose, final height, growth hormone treatment, puberty, randomized treatment trial
Growth in Crohn's disease
Authors: Savage MO , Beattie RM , Camacho‐Hübner C , Walker‐Smith JA , Sanderson IR 
Published: April 2008

Savage MO, Beattie RM, Camacho‐Hubner C, Walker‐Smith JA, Sanderson IR. Growth in Crohn's disease. Acta Paediatr 1999; Suppl 428:89‐92. Stockholm. ISSN 0803‐5326Abnormal linear growth is frequent in children and adolescents with Crohn's disease. The typical pattern is of growth retardation associated with delayed skeletal maturation. Puberty is also frequently delayed. Over 50% of patients may have a subnormal height velocity, and approximately 25% will have short stature. The endocrine status is characterized by normal growth hormone secretion and a slightly subnormal serum level of insulin‐like growth factor I, which is related to nutritional status. Principal therapeutic options are intestinal resection for localized disease, and enteral nutrition ‐ using a polymeric diet ‐ for more widespread disease, particularly involving the small intestine. Growth responses to both modalities are often excellent and produce considerable psychological benefit. Optimum therapy is achieved by close collaboration between gastroenterologists and endocrinologists, and by the use of auxologicalmethods to document pre‐ and post‐therapeutic management. □Crohn 's disease, enteral nutrition, growth, intestinal resection, puberty
Growth retardation in chronic diseases: possible mechanisms
Authors: Underwood LE 
Published: April 2008

Underwood LE. Growth retardation in chronic diseases: possible mechanisms. Acta Pædiatr 1999; Suppl 428: 93–6. Stockholm. ISSN 0803–5326Multiple and complex mechanisms are likely to be involved in producing the growth retardation that occurs in children with chronic disease. The principal mechanisms in the pathway leading to growth arrest include too little substrate available to the child, excessive need for and over‐consumption of substrate, and inefficient management of body components needed for growth. It is proposed that alterations in the growth hormone (GH)‐insulin‐like growth factor (IGF) system play a major role at each of the steps between insult from chronic disease and growth retardation. When substrate is insufficient, the production and action of IGF‐I are attenuated at multiple points in the GH‐IGF cascade. When over‐consumption of substrate occurs, a situation of‘internal starvation’probably develops ‐ leading to events similar to those that take place when substrate supply is inadequate. Finally, conditions that cause inefficient management of body components needed for growth, as characterized by increased proteolysis, appear to be attenuated by GH and IGF‐I. □Chronic disease, growth hormone, growth retardation, insulin‐like growth factor I
Growth hormone treatment of short stature and metabolic dysfunction in juvenile chronic arthritis
Authors: Simon D , Touati G , Prieur AM , Ruiz JC , Czernichow P 
Published: April 2008

Simon D, Touati G, Prieur AM, Ruiz JC, Czernichow P. Growth hormone treatment of short stature and metabolic dysfunction in juvenile chronic arthritis. Acta Pædiatr 1999; Suppl 428: 100–5. Stockholm. ISSN 0803–5326Severe growth retardation and profoundly altered body composition are observed in children treated with glucocorticoids for systemic forms of juvenile chronic arthritis. The aim of this study was to assess the effects of 1 year of treatment with growth hormone (GH) on height velocity and body composition, and the potential effects of such treatment on glucose tolerance. Fourteen children receiving steroid therapy for juvenile chronic arthritis were treated with GH, 1.4 IU/kg/week (0.47 mg/kg/week), for 1 year and were then followed up for 1 year after cessation of treatment. Baseline GH secretion and plasma levels of insulin‐like growth factor I (IGF‐I) and IGF‐binding protein‐3 (IGFBP‐3) were at the lower limit of normal, but increased with GH treatment. During the year of GH treatment, mean height velocity increased from 1.9 to 5.4 cm/year (p < 0.001), mean lean body mass increased by 12% (p < 0.01) and mean fat mass fell by 20% (p < 0.01) compared with baseline. Decreased glucose tolerance and increased glycosylated haemoglobin levels were also observed. This may have been a result of insulin resistance, as indicated by induced hyperinsulinaemia. Following the 1‐year GH treatment period, height velocity fell to pretreatment values, and the height SDS at the end of the second year was lower (p 0.01) than before treatment. Weight and fat mass increased markedly after cessation of GH treatment. These results suggest that GH may partially counteract the adverse effects of glucocorticoids on growth and metabolism in patients with chronic inflammatory disease. Long‐term controlled studies are needed to determine the risks and benefits of GH therapy in such patients. □Body composition, growth hormone, juvenile chronic (rheumatoid) arthritis, steroid therapy
Health‐related quality of life in intersexuality
Authors: Meyer‐Bahlburg HFL 
Published: April 2008

Meyer‐Bahlburg HFL. Health‐related quality of life in intersexuality. Acta Pædiatr 1999; Suppl 428: 114–15. Stockholm. ISSN 0803–5326Standardized methods for the assessment of health‐related quality of life in patients with intersexuality are yet to be developed. This paper makes recommendations as to the kind of measures that should be included. □Gender role/identity, health‐related quality of life, intersexuality, sexuality
Growth hormone therapy in hypochondroplasia
Authors: Ramaswami U , Hindmarsh PC , Brook CGD 
Published: April 2008

Ramaswami U, Hindmarsh PC, Brook CGD. Growth hormone therapy in hypochondroplasia. Acta Pædiatr 1999; Suppl 428: 116–17. Stockholm. ISSN 0803–5326Patients with hypochondroplasia present with variable phenotypes. Children with severe short stature and disproportion of the body segments usually have the mutation Asn540Lys. They respond to growth hormone (GH) therapy with an increase in spinal length and, coupled with a surgical leg‐lengthening procedure, it is possible for some patients to achieve adult heights within the normal range. Some children who present with proportionate short stature and hypochondroplasia fail to increase their growth rate at puberty, although the growth spurt can be restored by GH therapy. Others, with an identical presentation, seem to grow normally during puberty. At present, there is no way of predicting who will undergo a normal pubertal growth spurt. We therefore monitor all patients during childhood and give GH treatment only to those patients who fail to develop a growth spurt at puberty. Severe cases may occasionally need treatment before puberty if their growth velocity is compromised, but these will probably also be candidates for a surgical leg‐lengthening procedure. □Growth, growth hormone therapy, hypochondroplasia, puberty, short stature
Molecular defects in achondroplasia and the effects of growth hormone treatment
Authors: Seino Y , Moriwake T , Tanaka H , Inoue M , Kanzaki S , Tanaka T , Matsuo N , Niimi H 
Published: April 2008

Seino Y, Moriwake T, Tanaka H, Inoue M, Kanzaki S, Tanaka T, Matsuo N, Niimi H. Molecular defects in achondroplasia and the effects of growth hormone treatment. Acta Pa; diatr 1999; Suppl 428: 118–20. Stockholm. ISSN 0803–5326Achondroplasia is a common skeletal dysplasia with severe growth retardation. Recently, mutations in the fibroblast growth factor receptor 3 (FGFR3) were identified in patients with achondroplasia. In the present study, 70 of 75 Japanese patients with achondroplasia were found to have a G1138A mutation in FGFR3, and two patients had a G1138C mutation. Growth hormone therapy was given to 145 patients with achondroplasia. Significant dose‐dependent effects on skeletal growth were obtained, with no long‐term adverse effects. □Achondroplasia, fibroblast growth factor receptor, growth hormone therapy, growth, skeletal dysplasia
Natural history of growth hormone receptor deficiency
Authors: Rosenbloom AL , Martinez V , Kranzier JH , Bachrach LK , Rosenfeld RG , Guevara‐Aguirre J 
Published: April 2008

Rosenbloom AL, Martinez V, Kranzier JH, Bachrach LK, Rosenfeld RG, Guevara‐Aguirre J. Natural history of growth hormone receptor deficiency. Acta Pædiatr 1999; Suppl 428: 153–6. Stockholm. ISSN 0803–5326This review discusses the natural history of growth hormone receptor deficiency (GHRD) in relation to epidemiology, mortality, growth, certain aspects of body composition, and intellectual development. The majority of affected individuals are of Semitic origin and 90% come from the Indian peninsula, the Middle East, or elsewhere in the Mediterranean. There is a twofold increased mortality before the age of 7 years for children with GHRD. Affected adults may have increased cardiovascular risk resulting from increased total cholesterol and low‐density lipoprotein cholesterol, unrelated to adiposity or insulin resistance. Intrauterine growth is affected minimally, if at all. Within a genetically homogeneous population in Ecuador, postnatal growth effects are as variable as in a large genetically heterogeneous population. There is no influence of parental heights. Areal bone mineral density is reduced in adults with GHRD, but estimated volumetric bone density (bone mineral apparent density) is normal. Intellectual development is unaffected by GHRD. □Body composition, growth, growth hormone receptor deficiency, insulinlike growth factor I deficiency, intellectual development, Laron syndrome, natural history
New growth hormone receptor exon 9 mutation causes genetic short stature
Authors: Ayling RM , Ross RJM , Towner P , Laue S Von , Finidori J , Moutoussamy S , Buchanan CR , Clayton PE , Norman MR 
Published: April 2008

Ayling RM, Ross RJM, Towner P, Von Laue S, Finidori J, Moutoussamy S, Buchanan CR, Clayton PE, Norman MR. Acta Pa; diatr 1999; Suppl 428: 168–72. Stockholm. ISSN 0803–5326.A novel form of congenital growth hormone insensitivity syndrome (GHIS), which lacks the classic phenotype associated with this condition, is described. Dominant inheritance is shown to result from a heterozygous 876–1 G to C transversion of the 3′ splice acceptor site preceding exon 9 in the growth hormone receptor (GHR) gene. The result of this mutation is a severely truncated cytoplasmic domain of the GHR, which is incapable of transmitting a signal. The mutant receptor is shown to form a heterodimer with the wild‐type GHR, the activity of which is inhibited in a dominant‐negative manner. □Dominant‐negative mutation, growth hormone receptor
Signal transduction defects in growth hormone insensitivity
Authors: Clayton PE , Freeth JS , Whatmore AJ , Ayling RM , Norman MR , Silva CM 
Published: April 2008

Clayton PE, Freeth JS, Whatmore AJ, Ayling RM, Norman MR, Silva CM. Signal transduction defects in growth hormone insensitivity. Acta Pædiatr 1999; Suppl 428: 174–8. Stockholm. ISSN 0803–5326Growth hormone (GH) insensitivity is a heterogeneous condition that can result from mutations within the GH receptor (GHR) and that can be inherited as both an autosomal recessive and a dominant trait. However, evidence from a small number of growth hormone binding protein (GHBP)‐positive families indicates that their GH insensitivity is independent of GHR mutations. Two of these families appear to have distinct abnormalities in GH signal transduction. Studies suggest that one family (classic Laron syndrome phenotype; designated family H) have a signalling defect close to the GHR, preventing activation of both the STAT and MAPK pathways, whereas the other family (less marked phenotype; family M) have a defect in activating MAPK but not the STAT pathway. The children studied here are specifically insensitive to GH and their defect must be exclusive to this signalling system. Thus, families with GHBP‐positive GH insensitivity without GHR mutations are likely to be important models in which to study the specificity of GH signal transduction and the relationship between GH insensitive phenotype and signalling defect. □Growth hormone insensitivity, growth hormone receptor, Laron syndrome, mitogen activated protein kinase, signal transducers and activators of transcription
Effects of insulin‐like growth factor I treatment on statural growth, body composition and phenotype of children with growth hormone insentivity syndrome
Authors: Underwood LE , Backelijauw P , Duncan V 
Published: April 2008

Underwood LE, Backelijau P, Duncan V, and the GHIS Collaborative Group. Effects of insulinlike growth factor I treatment on statural growth, body composition and phenotype of children with growth hormone insensitivity syndrome. Acta Paediatr 1999; Suppl 428:182‐4. Stockholm. ISSN 0803‐5326Eight children with growth hormone insensitivity syndrome (GHIS) have been treated with injections of recombinant human insulin‐like growth factor I (rh1GF‐I) for more than 5 years each. After good acceleration of growth in the first year of therapy, the growth rade decreased to an average of 5‐6 cm/year. In general, growth with IGF‐I therapy is less exuberant than that observed with growth hormone (GH) therapy in GH‐deficient children. IGF is well tolerated, though there may be overgrowth of the lymphoid tissues and the kidneys. Bone mineral density is improved by treatment. The benefits of therapy appear to exceed the risks. □ Growth hormone insensitivity syndrome, insulin‐like growth factor I therapy, Laron syndrome
Short‐term increments of insulin‐like growth factor I (IGF‐I) and IGF‐binding protein‐3 predict the growth response to growth hormone (GH) therapy in GH‐sensitive children
Authors: Schwarze CP , Wollmann HA , Binder G , Ranke MB 
Published: April 2008

Schwarze CP, Wollmann HA, Binder G, Ranke MB. Short‐term increments of insulin‐like growth factor I (IGF‐I) and IGF‐binding protein‐3 predict the growth response to growth hormone (GH) therapy in GH‐sensitive children. Acta Paediatr 1999; Suppl 428:200‐8. Stockholm. ISSN 0803‐5326The present study included a cohort of 42 children aged between 1.7 and 15.4 years, who presented with short stature and growth failure. Basal and generated serum levels of insulin‐like growth factor‐I (IGF‐I) and IGF‐binding protein‐3 (IGFBP‐3), measured in an IGF generation test following four or seven daily injections of growth hormone (GH), 0.1 IU/kg (0.033 mg/kg), were analysed in these patients. The growth response to 1 year of GH treatment, 0.6 IU/kg/week (0.2 mg/kg/week), was also investigated. Median height velocity of these patients increased from‐1.6 SDS (range, ‐4.6 to ‐0.3 SDS) to 3.3 SDS (range, ‐0.2 to 7.1 SDS) after 1 year of GH treatment, and median height SDS increased by 0.7 SDS (range, 0.1 to 2.2 SDS). Strong correlations were observed between basal and generated IGF‐I and IGFBP‐3 levels. The increase in IGEBP‐3 levels in response to GH in the generation test was a strong predictor of the growth response to GH therapy. All the patients in the present study could be differentiated from patients with GH insensitivity syndrome (GHIS) using the criteria of a diagnostic scoring system for GHIS. The most valuable parameters were the increases in IGF‐I and IGFBP‐3 levels in the generation test, which excluded 95.2% of the patients from a diagnosis of GHIS. □ Growth hormone treatment, insulin‐like growth factor I, insulin‐like growth factor binding protein‐3, insulin‐like growth factor generation test
Endothelial function in children with a history of premature prolonged rupture of membranes and bronchopulmonary dysplasia – a pilot study
Authors: Liljedahl Magnus , Martin Helena , Magnuson Anders , Montgomery Scott M , Schollin Jens 
Published: April 2008

AbstractAim: To investigate vascular function in children with a neonatal history of generalised inflammation indicated by premature prolonged rupture of membranes (PPROM) and bronchopulmonary dysplasia (BPD).Methods: Children born at ≤ 30 weeks 1994–2000 were investigated at a present age of 6–12 years. Twenty‐eight children participated and were divided into two groups with regard to BPD/no BPD (n = 15/13) and PPROM/no PPROM (n = 10/18). Vascular endothelial function was assessed by acetylcholine (ACh)‐induced skin vasodilatation.Results: Maximum ACh‐induced skin perfusion was statistically significantly lower in the PPROM group compared with the non‐PPROM group (p = 0.045) after adjustment for confounders. We found no association between BPD and maximum ACh‐induced skin perfusion (p = 0.404), after adjustment for confounders.Conclusion: A neonatal history of prolonged premature rupture of membranes was associated with later impairment of vascular endothelial function in childhood. This association was not observed with BPD. Some forms of perinatal inflammation may be associated with later cardiovascular function.
Guidelines for diagnosing and treating pulmonary infiltrates in children with acute leukaemia: impact of timely decisions
Authors: Furuya MEY , González‐Martínez F , Vargas MH , Miranda‐Novales MG , Bernáldez‐Ríos R , Zúñiga‐Vázquez G 
Published: April 2008

AbstractAim: Children with leukaemia are at increased risk of pulmonary complications, often with unspecific clinical data, delayed diagnosis and a high mortality rate. We evaluated the usefulness of diagnostic–therapeutic guidelines (DTG) in which specific times for decision making were incorporated.Methods: Clinical charts of children with acute leukaemia and suspicion of pulmonary involvement were reviewed. Patients were allocated to group I if their diagnostic and therapeutic decisions were in accordance with the DTG, and to group II if not.Results: Children from group I (n = 32) and group II (n = 28) did not differ with respect to age (9.3 ± 0.5 years old, mean ± SEM), gender, type, risk and stage of leukaemia, anaemia and neutropenia. Total length of hospital stay and hospitalization due to the pulmonary disease were shorter in group I than in group II (14.8 ± 2.1 vs. 28.5 ± 3.7 days, p = 0.0016; and 10.8 ± 1.0 vs. 18.4±1.8 days, p = 0.0003, respectively). Two patients (6.3%) died due to the pulmonary pathology in group I, and nine (32.1%, p = 0.016) in group II.Conclusions: Diagnostic–therapeutic guidelines that incorporate timely decisions constitute a useful algorithm to reduce the length of hospital stay and mortality in children with acute leukaemia and pulmonary infiltrates. A prospective study is needed to validate these results.
Reduction of dissolved organic matter and trihalomethane formation potential during laboratory‐scale soil‐aquifer treatment
Authors: Xue S. , Zhao Q. L. , Wei L. L. , Wang L. N. 
Published: April 2008

AbstractDissolved organic matter (DOM) in recovered groundwater from soil‐aquifer treatment (SAT) has the potential to generate harmful disinfection by‐products. This study investigated the reduction of mass and trihalomethane formation potential (THMFP) of DOM fractions from secondary effluent during laboratory‐scale SAT. Using XAD‐8 and XAD‐4 resins, DOM was fractionated into three fractions: hydrophobic acid (HPO‐A), transphilic acid (TPI‐A) and hydrophilic fraction (HPI). HPO‐A was removed by 61.1%, TPI‐A by 54.9% and HPI by 75.0% as dissolved organic carbon (DOC) during the laboratory‐scale SAT, respectively. The reduction of THMFP from HPO‐A, TPI‐A and HPI was 27.24, 26.24 and 36.08%, respectively. Specific THMFP for each DOM fraction increased across the soil columns. HPO‐A was found to be the major precursor of THMs. THMFP was strongly correlated to ultraviolet light at 254 nm (UV‐254) for HPO‐A and HPI, while the relationship between THMFP and UV‐254 for TPI‐A was significantly poor.
Measurement of sediment oxygen demand for modelling dissolved oxygen distribution in tidal Keelung River
Authors: Liu Wen‐Cheng 
Published: April 2008

AbstractThe Keelung River is one of the major branches of the Danshuei River estuarine system, which runs the metropolitan capital city of Taipei, Taiwan, and receives a large of amount of wastewater. The dissolved oxygen (DO) concentration is generally low in the tidal portion of the Keelung River. Hypoxia/anoxia occurs often, particularly during a low‐flow period. The sediment oxygen demand (SOD) amounts to a significant value, hence increasing the total oxygen demand load of the river. The present work reports on laboratory SOD made on grab sediment core samples in situ that are undisturbed. The results reveal that SOD values fluctuate with variations in seasons and are higher in summer due to a high‐temperature effect. It was found in the laboratory tests that the average SOD (at 20 °C) value is 0.76 g/m2/day and the maximum SOD (at 20 °C) value reaches 1.58 g/m2/day. The mean values of measured SOD at each station were adopted in the vertical two‐dimensional water quality model to simulate the DO distribution along the tidal Keelung River. The simulated results are in reasonable agreement with the measured DO distribution in the river. Model sensitivity analyses were also conducted with increasing and decreasing SOD. It reveals that SOD is an important parameter that affects the DO distribution in the tidal estuary.