Pediatrics
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The aim of this study was to define the role of possible risk factors for the development of cystic fibrosis (CF)-related liver disease and to analyze the association between liver disease and the different genotypes present in the Israeli CF patient population. ⋯ CF patients who have pancreatic insufficiency and carry mutations associated with a severe or a variable genotype are at increased risk to develop liver disease.
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Hydrops fetalis (HF) consists of an abnormal accumulation of fluid in two or more fetal compartments, including ascites, pleural effusion, pericardial effusion, and skin edema. Almost all observed cases of HF are of the nonimmune type, the causes of which remain undetermined in 15% of patients. We report a newborn infant with nonimmune hydrops fetalis (NIHF) and congenital hypothyroidism. ⋯ These observations support our hypothesis that deficient adrenergic activity in congenital hypothyroidism might lead to chylothorax with NIHF in the fetus. We speculate that thyroid hormone may play a role in the regulation of adrenergic receptors in the lymphatic system and lungs, thus modulating both the lymphatic flow rate and lung liquid clearance, and facilitating the resolution of chylothorax. Examination of thyroid functions should be included in the investigation of fetuses and neonates with NIHF of an obscure origin.
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Clinical practice guidelines have a long and distinguished tradition in pediatrics. Currently, the American Academy of Pediatrics has developed more than 15 practice guidelines and more than 250 clinical policy statements. In the past, practice guidelines have been used to improve care through the dissemination of evidence-based, clinically effective practices to pediatric practitioners. ⋯ In the past, effective dissemination of new knowledge has been a long process, often taking years. This cycle time can be dramatically shortened through the development of networks of practice sites that share knowledge and experience in the implementation of practice guidelines and the use of strategies that take advantage of key groups in the dissemination process. When used appropriately, practice guidelines can provide an important adjunct to clinical research by facilitating the dissemination of new clinical findings and can provide an important platform for encouraging innovations in patient care.