Acta paediatrica Scandinavica. Supplement最新文献

{"title":"Syndromes associated with growth deficiency.","authors":"D L Rimoin, J M Graham","doi":"10.1111/j.1651-2227.1989.tb17159.x","DOIUrl":"https://doi.org/10.1111/j.1651-2227.1989.tb17159.x","url":null,"abstract":"There are literally hundreds of syndromes associated with short stature which have different prognoses, complications and responses to treatment (1 -3). A specific diagnosis is essential for accurate prognosis, treatment and genetic counselling. The first step in the clinical evaluation of short stature is to determine whether the body habitus is proportionate or disproportionate (Fig. 1). In general, children with disproportionate short stature have a skeletal dysplasia, whereas those with proportionate short stature usually have a more generalized disorder, such as intrauterine growth retardation (IUGR), malnutrition, chronic disease, psychosocial dwarfism, chromosomal anomalies or an endocrine disorder. Exceptions to this rule d o occur, however, such as the disproportionate dwarfism in severe cretinism or the proportionate shortening in osteogenesis imperfecta. A mildly disproportionate body habitus may not be apparent on casual examination, and thus anthropometric measurements, such as sitting height or upper/lower segment ratio and arm span, must be made before a relatively mild skeletal dysplasia, such as hypochondroplasia, can be excluded. Once a person with short stature is found to be proportionate, it is helpful to determine whether growth deficiency was of prenatal or postnatal onset. Prenatal onset growth deficiency usually implicates a fetal environmental insult or general cellular genetic defect. Late fetal insults are more likely to result in catch-up growth postnatally than prolonged","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"349 ","pages":"3-10"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1651-2227.1989.tb17159.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13807521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The pathophysiology of the pancreatic defect in cystic fibrosis.","authors":"P R Durie","doi":"10.1111/apa.1989.78.s363.41","DOIUrl":"https://doi.org/10.1111/apa.1989.78.s363.41","url":null,"abstract":"<p><p>Studies of the postnatal development of the pancrease in CF infants show a failure of acinar development and an increase in lumen volume with accumulation of secretory material within the pancreatic ducts. Our evaluation of functional changes within the exocrine pancreas are consistent with the pathologic findings. Impaired pancreatic fluid secretion appears to be a primary phenomenon of CF. We have shown that pancreatic secretions from CF patients also contain significantly higher concentrations of protein in comparison with pancreatic function-matched controls. Since total protein output is not increased, hyperconcentration of protein appears to be a direct result of a primary defect of fluid secretion. Protein hyperconcentration appears to predispose CF patients to protein precipitation and obstruction within small pancreatic ducts which, in turn, produces pancreatic acinar atrophy and fibrosis Our studies suggest that both bicarbonate and chloride transport within pancreatic ducts account for deficient fluid secretion in the pancreas of CF subjects.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"363 ","pages":"41-4"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/apa.1989.78.s363.41","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13840999","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"7th International Symposium on Growth and Growth Disorders. Proceedings of a meeting. Rome, Italy, 21-22 April 1989.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"356 ","pages":"1-180"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13955217","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Update on the Kabi International Growth Study, April 1989.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The efficacy and safety of recombinant human growth hormone (rhGH) treatment is under prospective evaluation in children with various short stature conditions. Of the 987 children enrolled up to April 1989, 836 (84.7%) had classic growth hormone deficiency (GHD) and 151 (15.3%) non-GHD. There was a predominance of idiopathic growth hormone deficiency (IGHD), with a ratio of IGHD to secondary or organic GHD (OGHD) of 2.2:1. There were more boys than girls in both the IGHD and OGHD groups. Isolated GHD was more common than multiple pituitary hormone deficiency except in some of the groups with OGHD. About half of the OGHD patients had GHD secondary to treatment for CNS tumours. Idiopathic short stature and Turner's syndrome were the most common diagnoses in the non-GHD group. The median age at onset of treatment in IGHD was 8.2 years for boys and 8.6 years for girls. The corresponding figures for OGHD were 14.0 years and 12.2 years, respectively. The height SDS for chronological age at the start of treatment was -3.0 for IGHD and slightly less for children with OGHD. Approximately one-third of the children had already reached puberty at the start of hGH treatment.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"356 ","pages":"173-7"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13955219","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ontogeny of digestion and absorption as related to perinatal changes in food composition in infants.","authors":"O Koldovský","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"351 ","pages":"7-12"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13626855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"On the construction of the infancy-childhood-puberty growth standard.","authors":"J Karlberg","doi":"10.1111/j.1651-2227.1989.tb11237.x","DOIUrl":"https://doi.org/10.1111/j.1651-2227.1989.tb11237.x","url":null,"abstract":"<p><p>Using the infancy-childhood-puberty (ICP) growth model, the postnatal linear growth curve is mathematically broken down into three components: infancy, childhood and puberty. The fact that each component of this model seems to represent a defined biological phase of the growth process, extends its clinical application. The principles used to construct the ICP growth standard and the basic 'clinical' ICP-based growth charts are described. These charts have a number of advantages over the conventional growth charts currently in use. Most importantly, the ICP growth chart provides reference values not only for total growth but also for each of the three individual components. Using this methodology, the magnitude and the onset of each component can be assessed in an individual child. The age at onset of the childhood component, which is a newly discovered and important feature of human growth, can easily be detected in an individual child. Furthermore, prepubertal growth, onset of puberty and pubertal growth can be accurately assessed, because the difference in pubertal maturation is taken into account when setting standards during adolescence. The methodology also offers a new and highly accurate predictor of final height and permits assessment of growth rates over shorter and longer periods using a simple and effective method of assessing change. The ICP growth model appears to be a refined instrument for detecting and understanding growth disturbances.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"356 ","pages":"26-37"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1651-2227.1989.tb11237.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13636009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Psychomotor and mental development during infancy. Relation to psychosocial conditions and health. Part IV of a longitudinal study of children in a new Stockholm suburb.","authors":"L Nordberg,&nbsp;P A Rydelius,&nbsp;I Nylander,&nbsp;G Aurelius,&nbsp;R Zetterström","doi":"10.1111/j.1651-2227.1989.tb11228.x","DOIUrl":"https://doi.org/10.1111/j.1651-2227.1989.tb11228.x","url":null,"abstract":"<p><p>This article presents the fourth substudy in a Swedish research project of a birth cohort of children in a newly-built Stockholm suburb. The aims are to follow and to describe their mental development by prospective methods. Here we present the results of the one-year follow-up. The children's mental development, measured with the Griffiths' Development Scales, and their behaviour in the test situation and during the home visit are described. These results are related to various psychosocial background factors (such as the parents' ages, number of siblings, form of custody), home environment factors (the parents' mental disease, addictions and criminality) and the children's physical health and development. Children with deviant behaviour during the home visits are described separately. Of 640 women who paid their first visit to the maternal welfare centers in a new Stockholm suburb during one prospective year, 532 (85%) were interviewed with regard to 41 stress factors forming a \"Life stress score\" (LSS). The interviews were supplemented with data from hospital, social welfare and police records concerning the expectant mother and the father. The 532 mothers were divided into three groups according to the degree of psychosocial stress (194 without psychosocial stress, 171 with severe psychosocial stress and 167 in an intermediate group). The pregnancies and deliveries of all mothers were evaluated. The physical health and development (using information from the child welfare clinics) and the mental health and development (using information from home visits and testings) were studied during infancy in 452 children (226 boys and 226 girls)--i.e. 77% of all children born in the suburb during the year. The children were tested with the Griffiths' Development Scales and their behaviour during the test was observed on home visits by the same psychologist (L.N.) at the age of 10 months (79 boys, 73 girls) or 14 months (92 boys, 107 girls), or about the age of 18 months (55 boys, 46 girls). The test results are mainly reported by descriptive methods. In summary, the results of the evaluation of the children's mental health during the first year of life, generally showed average developmental quotients. However, 20% of the children had values below the average. Thirty-two per cent of the children with low test results (less than -1 standard deviation on the total test) came from homes with serious psychosocial stress and 29% from homes with a mild degree of psychosocial stress. Of the nine children who had generally very low scores in the Griffiths' evaluation, seven came from homes with psychosocial stress.(ABSTRACT TRUNCATED AT 400 WORDS)</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"353 ","pages":"1-35"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13938645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuroendocrine control of growth hormone secretion.","authors":"V de Gennaro Colonna,&nbsp;S G Cella,&nbsp;V Locatelli,&nbsp;S Loche,&nbsp;E Ghigo,&nbsp;D Cocchi,&nbsp;E E Müller","doi":"10.1111/j.1651-2227.1989.tb17175.x","DOIUrl":"https://doi.org/10.1111/j.1651-2227.1989.tb17175.x","url":null,"abstract":"It is now a tenet of neuroendocrinology that the secretion of growth hormone (GH) is regulated by the central nervous system (CNS) (1). Two specific neuropeptides in the hypothalamus, GH-releasing hormone (GHRH) and somatostatin, exert dual control over GH secretion. These in turn are regulated by numerous neurotransmitters, which may act at the level of the GHRHor somatostatin-producing neurones or both. Both amine and amino acids neurotransmitters play a role in the control of GH release and, in general, they exert a dual influence, either stimulating or inhibiting hormone release. This dual effect on GH secretion results from actions of the same molecule at both GHRHand somatostatin-secreting neurones. This is best exemplified by catecholamines, where aand 0-adrenoceptors exert, respectively, stimulatory and inhibitory influences. Alternatively, the neurotransmitter may pass into the stalk blood to act at a receptor site located on the somatotroph (2). As a result of GH secretion, both GH itself and the GH-dependent insulinlike growth factors (IGFs) exert a feedback inhibitory action on GH secretion via hypothalamic and/or pituitary sites (Fig. 1). In addition to the classical neurotransmitters, a host of neuropeptides stimulate GH release, though their physiological function is unclear. In general, these compounds do not act directly at the level of the pituitary, but via the CNS, particularly with the mediation of classical neurotransmitters (1, 3). This paper focuses on certain aspects of the neuroendocrine control of GH secretion which the authors have investigated recently.","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"349 ","pages":"87-92; discussion 100"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1651-2227.1989.tb17175.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13712277","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Oral rehydration solutions for the children of Europe. Proceedings of a workshop held at XXI annual meeting of ESPGAN. Copenhagen 1988.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"364 ","pages":"1-50"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13783141","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neonatal septicaemia--incidence, etiology and outcome. A 6-year analysis.","authors":"E L Grauel,&nbsp;E Halle,&nbsp;R Bollmann,&nbsp;P Buchholz,&nbsp;S Buttenberg","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Between 1983 and 1988 we observed altogether 222 cases of neonatal septicemia and/or meningitis in our Department of Neonatology. The incidence was 8.46 per 1,000 liveborn infants. The case fatality rate amounted to 45.9%. The most frequently isolated causative agents were Escherichia coli (23.4%) followed by group B Streptococci (16.7%), Staphylococcus aureus (9.9%), Klebsiella pneumoniae species (8.8%), Serratia marcescens (7.9%), Pseudomonas aeruginosa and coagulase-negative Staphylococci each 5.9%. The report includes information about serotypes of Escherichia coli, group B Streptococci and plasmid patterns of Serratia marcescens. The latter was responsible for an outbreak of septicemia and meningitis with high mortality. The changing infection pattern reflects changes in the newborn population, especially in the patient structure of the neonatal intensive care unit, changes in the antibiotic policy and organizational problems.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"360 ","pages":"113-9"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13784570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}