{"title":"Adolescent and adult cystic fibrosis patients: how to leave home when you are depending on it?","authors":"A Lampo, I Dab, A Malfroot","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Over a period of 1 year, 11 on 23 CF adolescent or adult patients were referred to the childpsychiatrist (CP) for psychological problems and a notice of total dependency of their parents. The approach of the CP was systematic and family oriented. We noticed that on 11 patients only 6 came to the appointment and only 6 on 10 parents accepted to contact the CP. Of the 6 studied cases 4 showed severe psychopathology (1 sexual abuse, 1 physical and emotional abuse, 2 extreme over-protection), 2 showed psychological problems due to their restricted life conditions. On top of this, all 6 met enormous financial and social problems to gain more independency. The dependency of CF adolescent and adult patients is a family problem and a combination of relational, emotional, but also practical and financial problems and thus not exclusively due to their illness. As the prognosis of cystic fibrosis (CF) improved significantly, every aspect of this disease also became an \"adult\" problem. Independency is only one aspect and can not be considered on its own. We have to look at all technical, psychological and social adjustment problems those patients and their parents have to cope with.</p>","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"228-30"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13285454","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":"Coping strategies in CF families.","authors":"D Kornas-Biela","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Thirty couples, parents of CF children were tested by means of the following methods: The Personal and Illness Orientation Questionnaire, The Social Support Scale, The Coping Health Inventory for Parents (McCubbin et al.). The results show that the most important matters to both parents are: confidence in mutual assistance, optimistic outlook at the health prospects of the child, active participation in the development of the child's independence. Some personal-dependent (e.i. gender-dependent) differences in the coping strategies were observed.</p>","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"233-4"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13285456","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}
B Santini, P Lerro, T Ciervo, P V Robazza, N Ansaldi
{"title":"Sensitivity assessment of chymotrypsin faecal test in controlling malabsorption in cystic fibrosis.","authors":"B Santini, P Lerro, T Ciervo, P V Robazza, N Ansaldi","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"165-8"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13285538","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}
S V Rachinsky, N I Kapranow, V K Tatochenko, O I Simonowa, J E Turina
{"title":"Submaximal physical loads in cystic fibrosis.","authors":"S V Rachinsky, N I Kapranow, V K Tatochenko, O I Simonowa, J E Turina","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"198-200"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13285546","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":"Effects of neonatal screening for cystic fibrosis on reproduction, attitudes toward reproductive behaviour and genetic knowledge.","authors":"J E Dankert-Roelse, K Knol, L P ten Kate","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>An evaluation was carried out into whether neonatal screening for Cystic Fibrosis could potentially prevent the birth of more affected children in the same family. Although CF was detected in more than 50% within the first year of life in those cases where a diagnosis was made on clinical symptoms, in these families 10 children -- among whom two with CF -- were born prior to the CF diagnosis. With neonatal screening, no pregnancies were started before the CF-diagnosis. No differences were found in attitudes toward further reproduction and understanding of important genetic facts. Although prenatal diagnosis was not yet possible at the time of the study, approximately 50% of the parents showed a positive attitude toward this option. Minimizing the delay in diagnosis by neonatal screening, together with the availability of prenatal diagnosis may lead to a significant reduction in subsequent births of CF cases.</p>","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"99-101"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13285272","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}
S Quattrucci, M Angelico, M Stancati, S Bertasi, D Cantusci, A De Sanctis, M Antonelli
{"title":"Hepatobiliary involvement in adolescents and adults with cystic fibrosis.","authors":"S Quattrucci, M Angelico, M Stancati, S Bertasi, D Cantusci, A De Sanctis, M Antonelli","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Fifty nine C.F. pts (38 males) aged 14-41 years have been considered, for all of them the following parameters were taken into account: Shwachman clinical score modified (radiological evaluation emended), steatorrhea, liver function tests (SGOT, SGPT, GT, Bilirubin, AP, Quick T.), A and B hepatitis markers and ultrasonographic scan of pancreas and hepatobiliary system. 23 pts (38%) presented well recognized hepatobiliary involvement: 2 pts (3%) evident cirrhosis, 3 pts (5%) cirrhosis with portal hypertension and esophageal varices, 5 pts (8%) were affected with signs of chronic hepatopathy, 13 pts (22%) presented gallbladder stones.</p>","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"180-2"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13285543","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}
R Szibor, R Brückner, J Gedschold, W Weise, H Gola, I Röse, K Grade, A Speer, C Coutelle, R Bollmann
{"title":"Prenatal diagnosis of cystic fibrosis in first and second trimenon (GDR report).","authors":"R Szibor, R Brückner, J Gedschold, W Weise, H Gola, I Röse, K Grade, A Speer, C Coutelle, R Bollmann","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"117-9"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13285645","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}
J Hardcastle, C J Taylor, P T Hardcastle, P S Baxter, J Goldhill
{"title":"Intestinal transport in cystic fibrosis (CF).","authors":"J Hardcastle, C J Taylor, P T Hardcastle, P S Baxter, J Goldhill","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Ion transport was measured in jejunal biopsies using short-circuit current (SCC) as an index of net electrogenic ion transport. Increases in SCC associated with stimulation of Cl- secretion were observed in control tissues, but CF tissues did not exhibit such a response, indicating a failure of intestinal Cl- secretion. The rise in SCC associated with Na(+)-linked glucose absorption was greater in CF tissues than in controls, reflecting an enhanced active absorption of glucose.</p>","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"157-8"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13285655","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":"Dissociation between cyclic AMP rise and mucin secretion in response to a beta-adrenergic agonist.","authors":"N A Bradbury, R L Dormer, M A McPherson","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>A novel hypotonic swelling method has been used to introduce cyclic AMP phosphodiesterase (cAMP PDE) into intact rat submandibular acini. Acini swollen in the presence of cAMP PDE showed no cyclic AMP rise in response to the beta-agonist isoproterenol; however, stimulation of mucin secretion was not impaired. Control experiments showed that acini swollen in the absence of cAMP PDE showed normal cyclic AMP rise and mucin secretion responses. The results showing a dissociation between cyclic AMP rise and mucin secretion suggest that cyclic AMP is not the sole mediator of beta-adrenergic stimulation of epithelial cell secretion. The method of intracellular manipulation described will allow (i) investigation of key regulators of secretory events, shown to be defective in CF cells. (ii) determination of the biochemical function of proteins which are candidates for the biochemical defect in CF.</p>","PeriodicalId":75422,"journal":{"name":"Acta Universitatis Carolinae. Medica","volume":"36 1-4","pages":"55-7"},"PeriodicalIF":0.0,"publicationDate":"1990-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13124195","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}