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Paediatrics and child health 10건

  1. [해외논문]   Editorial Board  


    Paediatrics and child health v.27 no.12 ,pp. i - i , 2017 , 1751-7222 ,

    초록

    원문보기

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  2. [해외논문]   Metabolic disorders presenting as liver disease  

    Guilder, Laura , Pula, Shpresa , Pierre, Germaine
    Paediatrics and child health v.27 no.12 ,pp. 533 - 539 , 2017 , 1751-7222 ,

    초록

    Abstract Many Inherited Metabolic Diseases (IMDs) have hepatic manifestations due to the highly metabolically active nature of the liver. IMDs are responsible for up to a third of acute liver failure in childhood. Manifestations include cholestatic jaundice, hepatomegaly and acute and chronic liver failure. It is important to consider and identify metabolic causes of liver disease early, particularly as some may present early for example with a self-limiting period of cholestasis before presenting in later childhood with irreversible disease, and in many early intervention improves outcomes. This review highlights features in the history and presentation which should raise suspicion for an IMD, and the specialist metabolic investigations to consider when evaluating the child with liver disease. The review also discusses the clinical course and management of specific IMDs including glycogen storage disorders, congenital disorders of glycosylation, cholesterol ester storage disease, galactosaemia, neonatal haemochromatosis, hereditary tyrosinemia, fatty acid oxidations disorders, urea cycle defects, Niemann Pick C, Wilson disease, citrin deficiency and disorders of bile acid synthesis. The role of liver transplantation is discussed briefly.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  3. [해외논문]   Portal hypertension in children  

    Chiou, Fang Kuan , Abdel-Hady, Mona
    Paediatrics and child health v.27 no.12 ,pp. 540 - 545 , 2017 , 1751-7222 ,

    초록

    Abstract Portal hypertension (PH) is an important complication of chronic liver disease. It can also be caused by a wide range of extrahepatic pathologies in children, and is often clinically silent. Acute variceal haemorrhage (VH) is the most serious consequence of portal hypertension associated with significant morbidity and mortality. Management of PH in children consists of medical, endoscopic and surgical approaches which are mainly focused on acute treatment as well as reducing the risk of variceal haemorrhage. Current treatment strategies for children with PH are mostly based on extrapolation of data from adult studies and expert opinion and consensus. A structured protocol, consisting of surveillance endoscopy with primary and secondary prophylactic therapy by endoscopic variceal ligation or sclerotherapy, is increasingly becoming the standard of care. This article discusses the causes and current treatment options for PH in childhood.

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    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

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  4. [해외논문]   Paediatric liver transplantation: an overview  

    Martin, Benjamin , Ong, Evelyn G.P.
    Paediatrics and child health v.27 no.12 ,pp. 546 - 551 , 2017 , 1751-7222 ,

    초록

    Abstract The only definitive treatment for end stage liver disease is transplantation. This has been made possible by advances in vascular surgery and immunosuppression. In the paediatric population, liver transplantation is most commonly performed for liver failure secondary to biliary atresia. Successful transplantation depends on patient preparation by a dedicated multidisciplinary team and meticulous peri-operative care. Improvements in timing of transplantation, immunosuppression advances and peri-operative intensive care have all contributed to improvements in patient survival. Currently the survival rates for children undergoing deceased donor liver transplant in the UK is 95.7% at 1 year and 91.8% at 5 years. These outcomes are reliant on multi-disciplinary care, which is best provided in centres that provide the expertise in each of the specialist fields required to care for these children. This article provides an overview of the entire process. It describes the selection process, the surgery undertaken and early and late post-operative complications.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  5. [해외논문]   Liver disease in cystic fibrosis  

    van Mourik, Indra D.M.
    Paediatrics and child health v.27 no.12 ,pp. 552 - 555 , 2017 , 1751-7222 ,

    초록

    Abstract Cystic fibrosis (CF) is a multiorgan disorder, caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, resulting in abnormal regulation of salt and water movement across membranes. In the liver this leads to hyperviscous bile accumulating in the biliary tree, causing cholangiocyte and hepatocyte injury and inflammation, stimulating focal fibrosis. With increased life expectancy CF related liver disease (CFLD) has become a leading cause of morbidity and mortality in patients with CF. Most patients with CFLD remain asymptomatic even though approximately 5–10% develop multilobular cirrhosis during the first decade of life. Most will develop signs of portal hypertension. Active screening for CFLD and introduction of non-invasive imaging techniques and novel biomarkers would identify individuals at risk for cirrhosis prior to its development in order to institute therapy to prevent or reduce disease progression, and can detect patients who have developed clinically silent cirrhosis to allow monitoring and interventions to reduce or mitigate complications. Liver transplant should be considered for those who develop hepatic dysfunction or advanced portal hypertension, although deteriorating pulmonary function and quality of life should also be taken into account. Current research in CFLD focuses on repairing the basic defect and reducing inflammation thus aiming to find treatment modalities that prevent development and/or progression of CLFD.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  6. [해외논문]   Non-alcoholic fatty liver disease  

    Rajwal, Sanjay , McClean, Patricia
    Paediatrics and child health v.27 no.12 ,pp. 556 - 560 , 2017 , 1751-7222 ,

    초록

    Abstract Non-alcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs in the setting of insulin resistance and increased adiposity. It is thought to be the hepatic manifestation of more widespread metabolic dysfunction. NAFLD is also associated with dyslipidaemia and increased risk of cardiovascular disease. A “multiple-hit” pathogenic model has been suggested to explain the progressive liver damage that occurs among children with NAFLD. The mainstay of NAFLD therapy is lifestyle interventions which aim to improve obesity. Effective pharmacological treatments are still under development. Bariatric surgery may have a role in treating morbid obesity. In this article, we briefly review the currently available knowledge on NAFLD.

    원문보기

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    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  7. [해외논문]   Paediatric pancreatitis  

    Yeo, M. , Kirkham, S.
    Paediatrics and child health v.27 no.12 ,pp. 561 - 566 , 2017 , 1751-7222 ,

    초록

    Abstract Paediatric pancreatitis results from an insult to the pancreas leading to an acute inflammatory response with variable involvement of other local tissues or remote organs. The incidence of acute paediatric pancreatitis has been rising in the last 20 years and is now approaching the incidence in adults. However, most of our understanding of paediatric pancreatitis has been extrapolated from adult studies. Studies informing optimal management in children are lacking. A significant proportion of children who get acute pancreatitis go on to have recurrent acute pancreatitis or chronic pancreatitis. It is essential therefore to provide optimal management in the acute phase of the condition as this may prevent secondary complications. In this article we summarise the limited available evidence in the diagnosis and management of children focussing on acute pancreatitis. We briefly discuss recurrent and chronic pancreatitis and introduce the role of genetics in defining the missing links in our understanding of the development of chronic pancreatitis.

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    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

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  8. [해외논문]   Update on restrictive cardiomyopathy  

    Kucera, Filip , Fenton, Matthew
    Paediatrics and child health v.27 no.12 ,pp. 567 - 571 , 2017 , 1751-7222 ,

    초록

    Abstract Restrictive cardiomyopathy (RCM) is a rare form of heart muscle disease accounting for just 2 to 5% of paediatric cardiomyopathies. It is characterised by increased myocardial stiffness and restrictive filling pattern in to the left ventricle. Idiopathic RCM with no obvious identifiable cause is the most common form of the disease. RCM can also be a secondary manifestation of miscellaneous systemic conditions. Typical examples include infiltrative and storage disorders, systemic inflammatory diseases, tumors and drug toxicity. RCM-specific mutations have been identified in several genes encoding sarcomeric proteins. Patients with RCM have heart failure symptoms related to diastolic dysfunction. Unfortunately, there is no curative treatment for RCM and the prognosis is generally very poor. Cardiac transplantation is the only effective treatment. High risk of sudden cardiac death justifies early listing especially in symptomatic children. When making the diagnosis of RCM, it is crucial to rule out constrictive pericarditis which presents with a similar clinical and haemodynamic picture. Unlike RCM it can often be cured surgically.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  9. [해외논문]   Adoption – the role of the medical advisor  

    Teh, Corina , Bugarski, Lyn
    Paediatrics and child health v.27 no.12 ,pp. 572 - 574 , 2017 , 1751-7222 ,

    초록

    Abstract Restrictive cardiomyopathy (RCM) is a rare form of heart muscle disease accounting for just 2 to 5% of paediatric cardiomyopathies. It is characterised by increased myocardial stiffness and restrictive filling pattern in to the left ventricle. Idiopathic RCM with no obvious identifiable cause is the most common form of the disease. RCM can also be a secondary manifestation of miscellaneous systemic conditions. Typical examples include infiltrative and storage disorders, systemic inflammatory diseases, tumors and drug toxicity. RCM-specific mutations have been identified in several genes encoding sarcomeric proteins. Patients with RCM have heart failure symptoms related to diastolic dysfunction. Unfortunately, there is no curative treatment for RCM and the prognosis is generally very poor. Cardiac transplantation is the only effective treatment. High risk of sudden cardiac death justifies early listing especially in symptomatic children. When making the diagnosis of RCM, it is crucial to rule out constrictive pericarditis which presents with a similar clinical and haemodynamic picture. Unlike RCM it can often be cured surgically.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  10. [해외논문]   Self-assessment  

    Heinz, Peter , Sharrad, Alexander
    Paediatrics and child health v.27 no.12 ,pp. 575 - 579 , 2017 , 1751-7222 ,

    초록

    Abstract Restrictive cardiomyopathy (RCM) is a rare form of heart muscle disease accounting for just 2 to 5% of paediatric cardiomyopathies. It is characterised by increased myocardial stiffness and restrictive filling pattern in to the left ventricle. Idiopathic RCM with no obvious identifiable cause is the most common form of the disease. RCM can also be a secondary manifestation of miscellaneous systemic conditions. Typical examples include infiltrative and storage disorders, systemic inflammatory diseases, tumors and drug toxicity. RCM-specific mutations have been identified in several genes encoding sarcomeric proteins. Patients with RCM have heart failure symptoms related to diastolic dysfunction. Unfortunately, there is no curative treatment for RCM and the prognosis is generally very poor. Cardiac transplantation is the only effective treatment. High risk of sudden cardiac death justifies early listing especially in symptomatic children. When making the diagnosis of RCM, it is crucial to rule out constrictive pericarditis which presents with a similar clinical and haemodynamic picture. Unlike RCM it can often be cured surgically.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

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