{"title":"12th International Symposium on Growth and Growth Disorders. Geneva, 25-26 October 1991.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"379 ","pages":"1-184"},"PeriodicalIF":0.0,"publicationDate":"1991-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12852194","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":"10th International Symposium on Growth and Growth Disorders. Proceedings of a meeting. Madrid, Spain, 19-20 October 1990.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"372 ","pages":"1-168"},"PeriodicalIF":0.0,"publicationDate":"1991-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13086677","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 carbohydrate-deficient glycoprotein syndrome. A new inherited multisystemic disease with severe nervous system involvement.","authors":"J Jaeken, H Stibler, B Hagberg","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"375 ","pages":"1-71"},"PeriodicalIF":0.0,"publicationDate":"1991-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12884316","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":"Prevention and treatment of primary malnutrition in developing and industrialized countries. Papers presented at the Milupa Nutrition Workshop. Cairo, February 18-20, 1990.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"374 ","pages":"5-182"},"PeriodicalIF":0.0,"publicationDate":"1991-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13116868","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}
Growth retardation is common in children with chronic renal failure (CRF). To investigate the efficacy and safety of recombinant human growth hormone treatment in such children and in children after renal transplantation, 43 prepubertal children with CRF, and 30 prepubertal and 25 pubertal patients with a renal transplant were studied. Data are reported for 31, 26 and 17 of these patients, respectively. Median height velocity increased from 4.2 to 9.8 cm/year during the first year of treatment, and to 6.8 cm/year during the second year of treatment in the patients with CRF. In the prepubertal transplant group, median height velocity changed from 3.5 to 8.4 cm/year during the first year and to 5.4 cm/year during the second year. In the pubertal transplant group, median height velocity increased from 3.2 to 6.6 cm/year during the first year and was 4.5 cm/year during the second year. The gain in height SDS for the prepubertal children in both the CRF and transplant groups was approximately 1 SD over 2 years. Treatment was well tolerated, and renal function did not change significantly in any group.
{"title":"Growth hormone treatment in short children with chronic renal failure and after renal transplantation: combined data from European clinical trials. The European Study Group.","authors":"A Van Es","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Growth retardation is common in children with chronic renal failure (CRF). To investigate the efficacy and safety of recombinant human growth hormone treatment in such children and in children after renal transplantation, 43 prepubertal children with CRF, and 30 prepubertal and 25 pubertal patients with a renal transplant were studied. Data are reported for 31, 26 and 17 of these patients, respectively. Median height velocity increased from 4.2 to 9.8 cm/year during the first year of treatment, and to 6.8 cm/year during the second year of treatment in the patients with CRF. In the prepubertal transplant group, median height velocity changed from 3.5 to 8.4 cm/year during the first year and to 5.4 cm/year during the second year. In the pubertal transplant group, median height velocity increased from 3.2 to 6.6 cm/year during the first year and was 4.5 cm/year during the second year. The gain in height SDS for the prepubertal children in both the CRF and transplant groups was approximately 1 SD over 2 years. Treatment was well tolerated, and renal function did not change significantly in any group.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"379 ","pages":"42-8; discussion 49"},"PeriodicalIF":0.0,"publicationDate":"1991-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12977141","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}
From the large database of patients enrolled in the Kabi Pharmacia International Growth Study (KIGS), 289 prepubertal patients with idiopathic growth hormone deficiency (GHD), treated for 2 years with growth hormone (GH) substitution therapy, were selected. A multiple regression analysis was performed to determine both the auxological factors characterizing the patients at the beginning of the first and second years on GH therapy and the respective treatment modalities relevant to the magnitude of the growth response. It was observed that during the first year on GH therapy the magnitude of the growth response was negatively correlated with chronological age and height SDS, and positively correlated with target height SDS, GH dose (IU/kg/week) and frequency of GH injections. During the second year the growth response was negatively correlated with chronological age and the first-year GH dose (IU/kg/week), and positively correlated with height velocity during the first year, GH dose (second year), and injection frequency (second year). The data suggest that the forces of 'catch-up'--auxologically entrenched within the distance between target height SDS and height SDS--no longer prevail during the second year of GH therapy. The inverse influence of the first-year GH dose in the two yearly phases of growth suggests that optimizing GH treatment must be attempted by analysing growth in response to GH over longer periods of time and considering that the growth process is influenced by interactive factors.
{"title":"Growth response in prepubertal children with idiopathic growth hormone deficiency during the first two years of treatment with human growth hormone. Analysis of the Kabi Pharmacia International Growth Study.","authors":"M B Ranke, O Guilbaud","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>From the large database of patients enrolled in the Kabi Pharmacia International Growth Study (KIGS), 289 prepubertal patients with idiopathic growth hormone deficiency (GHD), treated for 2 years with growth hormone (GH) substitution therapy, were selected. A multiple regression analysis was performed to determine both the auxological factors characterizing the patients at the beginning of the first and second years on GH therapy and the respective treatment modalities relevant to the magnitude of the growth response. It was observed that during the first year on GH therapy the magnitude of the growth response was negatively correlated with chronological age and height SDS, and positively correlated with target height SDS, GH dose (IU/kg/week) and frequency of GH injections. During the second year the growth response was negatively correlated with chronological age and the first-year GH dose (IU/kg/week), and positively correlated with height velocity during the first year, GH dose (second year), and injection frequency (second year). The data suggest that the forces of 'catch-up'--auxologically entrenched within the distance between target height SDS and height SDS--no longer prevail during the second year of GH therapy. The inverse influence of the first-year GH dose in the two yearly phases of growth suggests that optimizing GH treatment must be attempted by analysing growth in response to GH over longer periods of time and considering that the growth process is influenced by interactive factors.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"379 ","pages":"109-15; discussion 116"},"PeriodicalIF":0.0,"publicationDate":"1991-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12977204","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":"11th International Symposium on Growth and Growth Disorders. Proceedings of a meeting, Stockholm, Sweden, 26-27 April 1991.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"377 ","pages":"1-175"},"PeriodicalIF":0.0,"publicationDate":"1991-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12851445","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}
Early normal human linear growth is unstable and oscillates owing to an increase in growth rate between 6 and 12 months of age. This defines the onset of the infancy-childhood growth spurt (IC spurt). The onset is identical with the onset of the childhood component of the infancy-childhood-puberty growth model. The IC spurt is completed when the infancy component of this model has virtually ceased, at around 3 years of age. Additional observations suggest that its onset represents the age at which growth hormone (GH) begins to regulate growth significantly. The negative impact of late onset of the IC spurt on attained height is illustrated and discussed. A delayed spurt is observed in 30-50% of children with growth-related disorders and of infants living in a poor area of a developing country. The IC spurt is absent in children with GH deficiency who receive no hormonal therapy.
{"title":"The infancy-childhood growth spurt.","authors":"J Karlberg","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Early normal human linear growth is unstable and oscillates owing to an increase in growth rate between 6 and 12 months of age. This defines the onset of the infancy-childhood growth spurt (IC spurt). The onset is identical with the onset of the childhood component of the infancy-childhood-puberty growth model. The IC spurt is completed when the infancy component of this model has virtually ceased, at around 3 years of age. Additional observations suggest that its onset represents the age at which growth hormone (GH) begins to regulate growth significantly. The negative impact of late onset of the IC spurt on attained height is illustrated and discussed. A delayed spurt is observed in 30-50% of children with growth-related disorders and of infants living in a poor area of a developing country. The IC spurt is absent in children with GH deficiency who receive no hormonal therapy.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"367 ","pages":"111-8"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13373797","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}
The study dealt with a representative sample of the epileptic population in southwestern Finland, followed up prospectively for 23-39 years. At the end of the follow-up period, 55.5% of the original sample, i.e. 63.2% of the subjects who participated in the last follow-up evaluation, or 76.4% of those who were alive at that time, had not had epileptic attacks for at least the previous three years. A total of 60% lived independently; 21% of subjects, on the other hand, were not gainfully employed and lived in institutions. The intermediate group was the smallest (less than 18%); these were receiving a disability pension but had not been institutionalized. A certain polarization thus seemed to take place here: the epileptic patient either lead a normal life or was in institutional care. The intermediate group showed a steady decline in size in the course of the follow-up.
{"title":"Children with epilepsy as adults: outcome after 30 years of follow-up.","authors":"M Sillanpää","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The study dealt with a representative sample of the epileptic population in southwestern Finland, followed up prospectively for 23-39 years. At the end of the follow-up period, 55.5% of the original sample, i.e. 63.2% of the subjects who participated in the last follow-up evaluation, or 76.4% of those who were alive at that time, had not had epileptic attacks for at least the previous three years. A total of 60% lived independently; 21% of subjects, on the other hand, were not gainfully employed and lived in institutions. The intermediate group was the smallest (less than 18%); these were receiving a disability pension but had not been institutionalized. A certain polarization thus seemed to take place here: the epileptic patient either lead a normal life or was in institutional care. The intermediate group showed a steady decline in size in the course of the follow-up.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"368 ","pages":"1-78"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13391223","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}
Rett syndrome (RS), a complex and severely disabling neuromotor disorder affecting young girls, was not internationally recognized until in the middle eighties. In Austria and Sweden only, there are clinical experiences since the sixties. The aims of this study were to 1) define the nucleus group of RS in Sweden, 2) develop a frame of defined stages for describing the clinical progress of disease, 3) identify early developmental patterns and their predictivity, 4) give the natural history of neuromotor impairments and disability in adults, 5) analyse the period of clinical regression as to developmental profiles, patterns and progression of symptoms, 6) search informative neurophysiologic correlates to clinical symptoms and signs. By a 6 year (1984-90), nationwide search procedure, comprising an information and tracing program, a travelling consultant program, and a neuropediatric and laboratory examination program, 88 females were added to those 17, observed in Sweden during the sixties and seventies. By March, 1990, 105 females, aged 19 months-44 years (median 17 1/2 years), had entered this series, which was considered representative for the nucleus group of RS in Sweden. 97/105 females (92.3%) fulfilled the criteria for classic RS, according to internationally accepted clinical criteria, and 8 (7.7%), represented so called "formes frustes". The prevalence for classic RS in the south-west region of Sweden was 1:12.000-13.000 girls, the birth years 1965-76 and 1960-84. Based on 29 females with RS, aged 13-28 years, a clinical staging system was constructed and evaluated in 1985, emphasizing the main different phases of the disorder throughout life. This staging system was found a valuable tool for clinical assessment, documentation, and research approach, and has become internationally accepted and widely used. Further experiences have given rise to minor adjustments, which are proposed. By scrutinizing early documentation from 10 girls, at referral aged 20 months-6 1/4 years (median 3 1/4 years) and retrospectively applying the Denver Developmental Screening Test frames, early achievements, signs and symptoms were reconstructed in 1986. Although characteristic neuromotor and neurobehavioural patterns of help for early suspicion, and even subtle signs, possibly reflecting a disorder already during the first months of life, could be identified, predictive clinical manifestations of diagnostic value were not found.(ABSTRACT TRUNCATED AT 400 WORDS)
{"title":"Rett syndrome in Sweden. Neurodevelopment--disability--pathophysiology.","authors":"I W Engerström","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Rett syndrome (RS), a complex and severely disabling neuromotor disorder affecting young girls, was not internationally recognized until in the middle eighties. In Austria and Sweden only, there are clinical experiences since the sixties. The aims of this study were to 1) define the nucleus group of RS in Sweden, 2) develop a frame of defined stages for describing the clinical progress of disease, 3) identify early developmental patterns and their predictivity, 4) give the natural history of neuromotor impairments and disability in adults, 5) analyse the period of clinical regression as to developmental profiles, patterns and progression of symptoms, 6) search informative neurophysiologic correlates to clinical symptoms and signs. By a 6 year (1984-90), nationwide search procedure, comprising an information and tracing program, a travelling consultant program, and a neuropediatric and laboratory examination program, 88 females were added to those 17, observed in Sweden during the sixties and seventies. By March, 1990, 105 females, aged 19 months-44 years (median 17 1/2 years), had entered this series, which was considered representative for the nucleus group of RS in Sweden. 97/105 females (92.3%) fulfilled the criteria for classic RS, according to internationally accepted clinical criteria, and 8 (7.7%), represented so called \"formes frustes\". The prevalence for classic RS in the south-west region of Sweden was 1:12.000-13.000 girls, the birth years 1965-76 and 1960-84. Based on 29 females with RS, aged 13-28 years, a clinical staging system was constructed and evaluated in 1985, emphasizing the main different phases of the disorder throughout life. This staging system was found a valuable tool for clinical assessment, documentation, and research approach, and has become internationally accepted and widely used. Further experiences have given rise to minor adjustments, which are proposed. By scrutinizing early documentation from 10 girls, at referral aged 20 months-6 1/4 years (median 3 1/4 years) and retrospectively applying the Denver Developmental Screening Test frames, early achievements, signs and symptoms were reconstructed in 1986. Although characteristic neuromotor and neurobehavioural patterns of help for early suspicion, and even subtle signs, possibly reflecting a disorder already during the first months of life, could be identified, predictive clinical manifestations of diagnostic value were not found.(ABSTRACT TRUNCATED AT 400 WORDS)</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"369 ","pages":"1-60"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12865984","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}