摘要:

ABSTRACT. Aim, J. and Larsson. A. (Department of Paediatrics, Karolinska Institute, St. Goran's Children's hospital, and the PKU Section, Department of Bacteriology, National Bacteriological Laboratory, Stockholm, Sweden). Evaluation of a nation‐wide neonatal metabolic screening programme in Sweden 1965–1979. Acta Paediatr Scand, 70:601,.–In Sweden, neonatal screening for phenylketonuria was started in 1965 and a total of 1326000 infants were studied up to 1979. During various periods of time, screening was also carried out for galactosaemia, hereditary tyrosinaemia, histidinaemia, and homocystinuria. In screening for phenylketonuria and galactosaemia no false‐negative results were obtained and the incidences were 1/30850 and 1/81100, respectively. In screening for hereditary tyrosinaemia only 1 out of 6 patients was identified by screening and the incidence was 1/106 710. Two cases of histidinaemia were detected, which corresponds to an incidence of 1/36 840. Both children developed normally without any treatment. No child with homocystinuria was detected in the screened population of more than 300 000 newborn infants. A screening programme involving phenylketonuria and galactosaemia was considered to be optimal among the tested di

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05753.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Gustafsson, G., Kreuger, A. and Dohlwitz, A. (Departments of Paediatrics, University Hospital, Uppsala, and County Hospital, Nykoping, Sweden). Acute lymphoblastic leukemia in Swedish children 1973–1978. Acta Paediatr Scand, 70:609,.–Three hundred and sixty‐seven children with acute lymphoblastic leukemia have been diagnosed in Sweden 1973–1978, 345 of whom were treated according to the national uniform regimens of the Swedish Child Leukemia Group (SCLG). The patients were classified into an SR (standard risk) and an IR (increased risk) group. Remission was obtained in 354 patients (96%). With 12–84 months observation time the total survival was 54% and the diseasefree survival 44 %. A more intensive cytostatic regimen in the induction period increased considerably the diseasefree survival for the SR and to some extent also for the IR patients. Relapses were significantly more common in the IR group in spite of a more intensive cytostatic regimen. The most decisive IR criteria were B‐LPK and age at diagnosis. Prognosis was significantly worse for boys in all groups. After 3 years in CCR treatment was discontinued in 95 out of 246 children (38%) of whom 19 later re

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05754.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Rechnitzer, C, Scheibel, E. and Hendel, J. (University Clinic of Paediatrics, Rigshospitalet and Department of Clinical Chemistry, Finsen Institute, Copenhagen, Denmark). Methotrexate in the plasma and cerebrospinal fluid of children treated with intermediate dose methotrexate. Acta Paediatr Scand, 70:615,.–Serious complications can follow treatment with intermediate dose methotrexate of acute lymphoblastic leukemia in childhood. Toxicity has been shown to be correlated to plasma methotrexate concentrations. During intravenous infusions of methotrexate (500 mg/m2) the mean concentrations achieved 1 to 41/2hours after the start of infusion were 1.3×10‐7mol/l in cerebrospinal fluid and 1.7×10‐5mol/1 in plasma. At 72 hours after start of methotrexate infusion, plasma methotrexate concentrations were significantly higher in cases with symptoms of toxicity. In all the children who developed toxic symptoms 72‐hour plasma methotrexate concentration was above 1×10‐7mol/l. Assuming that leucovorin is given 48 hours after the start of methotrexate infusion, 72‐hour plasma methotrexate is suitable for detection of patients at risk for toxicity. In children treated with intermediate dose methotrexate we therefore recommend estimating plasma methotrexate concentration 72 hours after the start of infusion, and instituting supplementary leucovorin when plasma methotrexate concentration excee

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05755.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Borgström, A., Sveger, T., Lindberg, T., Kullander, S. and Svanberg, L. (Departments of Clinical Chemistry, Paediatrics and Obstetrics, University of Lund, Malmö General Hospital, Malmö, Sweden). Immunoreactive trypsin, chymotrypsin and pancreatic secretory trypsin inhibitor in cord blood from infants with cystic fibrosis. Acta Paediatr Scand, 70: 619,.–Immunoreactive trypsin, chymotrypsin, and pancreatic secretory trypsin inhibitor in cord blood were measured in six infants with cystic fibrosis and in ten control infants for each index child. These three pancreatic proteins were 2.5–6.7, 2.2–7.5, and 1.3–4.2 times higher, respectively, than the corresponding mean levels of the contr

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05756.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Petersen, N. T., Høiby, N., Mordhorst, C. H., Lind, K., Flensborg, E. W. and Bruun, B. (Paediatric Department TG, Rigshospitalet, and Statens Seruminstitut, Copenhagen, Denmark). Respiratory infections in cystic fibrosis patients caused by virus, chlamydia and mycoplasma–possible synergism withPseudomonas aeruginosa.Acta Paediatr Scand, 70: 623,.–116 cystic fibrosis patients were observed, by monthly examinations over an eight‐month period, to investigate the importance of non‐bacterial respiratory infections (NBI) in exacerbations of the respiratory disease. Sputum was examined for bacteria, and serum investigated for antibody response against virus, mycoplasma and chlamydia and for antibodies againstPseudomonas aeruginosa.During this period each patient had, on an average, 2.9 exacerbations of which 76 % were associated with bacteria, most frequentlyP. aeruginosa(51 %), and 20 % with NBI, although bacteria were also present in most of these cases. No etiology was established in 18 % of the exacerbations. The NBI were caused by respiratory syncytial virus (RSV) (9 %), parainfluenza virus (5 %), influenza virus (3.6 %), adenovirus (2.4 %), mycoplasma (0.6 %) and chlamydia (0.6 %). The incidence of exacerbations was higher in patients with chronicP. aeruginosainfection. RSV infections were more common in patients who developed chronicP. aeruginosainfection during the study period, and RSV infections were frequently associated with a rise ofP. aeruginosaantibodies in patients who harboured these bacteria. The important role of NBI as mediators of onset of chronicP. aeruginosainfections in cystic fibrosis patients is s

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05757.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Tegner, H., Ceder, O., Roomans, G. M., Kollberg, H. and Torcmalm, N. G. (Department of Otolaryngology, University of Lund, Malmo General Hospital, Malmo, Department of Pediatrics, University of Umea, Umea, and the Wenner‐Gren Institute, University of Stockholm, Stockholm, Sweden). Effects of cystic fibrosis serum and cell culture medium on the mucociliary activity of the respiratory tract. Acta Paediatr Scand, 70:629,.–As previously reported a cystic fibrosis factor (CFF) is associated with the dyskinetic ciliary motion induced by serum and cell culture medium from patients with cystic fibrosis (CF). In this study a sensitive, standardized method, for the photoelectric recording of mucociliary activity was used to examine the effect of sera and media from cell cultures taken from patients with CF and healthy controls, on the mucociliary activity of rabbit trachea in vitro. No signs of decreased mucociliary activity were observed and electron microscopy showed normal ultrastructure and orientation of ci

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05758.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Cavell, B. (Department of Paediatrics, University Hospital, Lund, Sweden). Gastric emptying in infants with cystic Fibrosis. Acta Paediatr Scand, 70:635,.–Gastric emptying of meals of pasteurized human milk or infant formula was studied in 4 infants aged 5 weeks to 4 months with cystic fibrosis (CF) and pancreatic insufficiency. A marker dilution technique was used. Three of the patients showed gastric emptying rates higher than those of controls during the first hour after receiving the meal. The gastric emptying rate of the remaining patients, both after a meal of human milk and of infants formula, was similar to that of the control

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05759.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Cavell, B. (Department of Paediatrics, University Hospital, Lund, Sweden). Gastric emptying in infants fed human milk or infant formula. Acta Paediatr Scand, 70:639,.–Gastric emptying of meals of human milk or infant formula was studied in 17 healthy infants aged 4 weeks to 6 months using a marker dilution technique. In the 24 studies performed gastric emptying followed a hiphasic pattern in 11 and a linear pattern in 12 studies. The average gastric half‐emptying time for meals of human milk was 48 min, and for meals of infant formula 78 min. After 1 hour an average of 29.5 ml of human milk and 22.7 ml of infant formula per 0.1 m2of body surface area had emptied from the stom

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05760.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Ebbesen F. (Department of Neonatology, Rigshospitalet, Copenhagen, Denmark). Effect of exchange transfusion on serum reserve albumin for binding of bilirubin and index of serum bilirubin toxicity. Acta Paediatr Scand, 70:643,.–Seventeen newborn infants, who received their first exchange transfusion due to hyperbilirubinaemia and/or rhesus haemolytic disease, were studied. The exchange transfusions were performed with fresh, citrated blood. During the exchange transfusion a marked increase in the serum reserve albumin concentration for binding of bilirubin measured by the [,4C]‐MADDS method was observed, followed by a smaller decrease after the transfusion. Plasma pH increased both during and after the exchange transfusion. During the exchange transfusion a drastic fall in index of serum bilirubin toxicity was observed, followed by a smaller increase after the transfusion. Citrate was not found to interfere in the binding of bilirubin to albumin. The results are in agreement with the clinical finding that an exchange transfusion performed with fresh, citrated blood effectively reduces the risk of bilirubin encephalopathy. The ratio in serum of binding albumin, i.e. bilirubin plus reserve albumin, to total albumin failed to be increased by the exchange transfusion, and a decrease occurred after the transfusion. These findings indicate the presence in infant serum of non‐binding albumin. Donor albumin with intact binding potential is partly transformed into the non‐binding variety in the course of one hour after the transfusion. In the most severely rhesus sensitized infant a drastic decline of the serum albumin binding capacity was seen during the first day

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05761.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

ABSTRACT. Ebbesen, F., and Brodersen, R. (Department of Neonatology, Rigshospitalet, Copenhagen and Institute of Medical Biochemistry, Aarhus, Denmark). Albumin administration combined with phototherapy in treatment of hyperbilirubinaemia in low‐birth‐weight infants. Acta Paediatr Scand, 70:649,.–Fifty‐nine jaundiced light treated newborn infants with low birth weight were studied. At onset of phototherapy 30 infants received 1 g human serum albumin per kg body weight as a 9 % solution containing sodium caprylate and N‐acetyltryptophan as stabilizers. 29 infants did not receive human serum albumin and served as controls. Blood samples were taken before initiation of the therapy and again 24 and 48 h thereafter, and the following determinations were made: Serum concentrations of unconjugated bilirubin, albumin, reserve albumin for binding of bilirubin by the [l4C]‐MADDS method, packed cell volume and pH. Before infusion of albumin it was found that the binding fraction of serum albumin, i.e. the sum of the serum concentrations of bilirubin‐albumin and reserve albumin, constituted about half of the total serum albumin concentration. The other half was non‐binding, in agreement with previous findings in neonates. The effect of albumin therapy was mainly an unexpected increase of the non‐binding fraction of serum albumin, while the increase of the serum reserve albumin concentration was small and the concentration of bilirubin‐albu

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1981.tb05762.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY