RESUMO
The start of newborn screening for phenylketonuria (PKU) during the early 1970s has given rise to an increasing number of women who have been identified and successfully treated for the disease in childhood and are now preparing to have children of their own. Early detection and initiation of nutritional therapy before conception is key to a successful pregnancy outcome. Nurses who understand the pathophysiology, dietary limitations, and management of PKU in pregnancy can provide the care necessary for optimal maternal and neonatal health.
Assuntos
Fenilcetonúrias , Complicações na Gravidez , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Triagem Neonatal , Fenilcetonúrias/diagnóstico , Fenilcetonúrias/dietoterapia , Fenilcetonúrias/enfermagem , Cuidado Pré-Concepcional , Gravidez , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/dietoterapia , Complicações na Gravidez/enfermagem , Ultrassonografia Pré-NatalRESUMO
Cyst fluid samples obtained from eight patients with autosomal dominant polycystic kidney disease (ADPKD) were analysed for the presence of the basement membrane component laminin and its breakdown products, using ELISA and immunoblotting techniques. Whole laminin was not detected, whereas laminin fragments of 270, 155, 87, 56, and 14 kDa were detected at a mean total value of approximately 0.5 microgram/ml. The laminin fragments were assessed for their effect on cultured normal and ADPKD epithelial cells. Both cell types showed accelerated growth under these conditions. These findings suggest that basement membrane degradative fragments present in cyst fluid may contribute to cystic epithelial cell proliferation and may therefore be important in the pathogenesis of ADPKD.
Assuntos
Laminina/análise , Rim Policístico Autossômico Dominante/química , Adulto , Divisão Celular/efeitos dos fármacos , Eletroforese em Gel de Poliacrilamida , Ensaio de Imunoadsorção Enzimática , Células Epiteliais/citologia , Células Epiteliais/efeitos dos fármacos , Exsudatos e Transudatos/química , Feminino , Humanos , Túbulos Renais/citologia , Túbulos Renais/efeitos dos fármacos , Laminina/química , Laminina/fisiologia , Masculino , Pessoa de Meia-Idade , Peso Molecular , Dodecilsulfato de SódioRESUMO
Laminin isolated from human placenta was subjected to prolonged pepsin digestion. Seven peptide fragments (designated N1 to N7) were separated by ion-exchange chromatography and gel filtration and characterised by SDS-polyacrylamide gel electrophoresis and immunoblotting. The molecular size of the laminin fragments varied from approx. 900,000 (N1) to 28,000 (N7). Epithelial cells obtained from normal kidneys and patients with autosomal dominant polycystic kidney disease (ADPKD) were cultured. The incorporation of [3H]thymidine was measured over 96 h to determine the effect of the addition of the different fragments and whole laminin from EHS tumour to the cells. The rate of growth of both normal and polycystic cells was increased in the presence of the laminin fragments but this effect was more pronounced in the ADPKD cells.