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Hepatic cardiac syndrome heart failure children

Huabo Cai, Baoxing Huang, Zhongsheng Zhu, Dongling Dai, Shaoming Zhou
OBJECTIVE: To explore the etiology and clinical characteristics of hypoxic hepatitis (HH) in children. METHOD: Clinical data of 7 patients with HH in Shenzhen Children's Hospital from January 2011 to March 2014 were retrospectively reviewed. RESULT: Seven cases diagnosed as HH, age from 4 months to 11 years, were admitted to pediatric intensive care unit (PICU), and accounted for 0.32% of patients in PICU during the same period. The primary causes of HH were respiratory failure and cardiac shock caused by severe hand-foot-and-mouth disease, fulminant myocarditis, infant muggy syndrome ...
March 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Jin Min Cho, Seak Hee Oh, Hyun Jin Kim, Joon Sung Kim, Kyung Mo Kim, Gu-Hwan Kim, Eunsil Yu, Beom Hee Lee, Han-Wook Yoo
BACKGROUND: Alagille syndrome (AGS) is a multisystem autosomal dominant disorder that affects the liver, heart, eyes, face, bone, and other organs. AGS is caused by mutations in one of two genes, JAG1 or NOTCH2. We evaluated clinical features, outcomes, and the presence of JAG1 and NOTCH2 mutations in Korean children with AGS. METHODS: Between January 1997 and December 2013, 19 children were diagnosed with AGS at Asan Medical Center, Seoul, Korea. Their clinical features, outcomes, and JAG1 and NOTCH2 mutation status were retrospectively analyzed...
August 2015: Pediatrics International: Official Journal of the Japan Pediatric Society
Yusuf Abdullah, Jonathan Karpelowsky, Alan Davidson, Jenny Thomas, Andre Brooks, John Hewitson, Alp Numanoglu, Sharon Cox, Alastair J W Millar
AIM: The aim of this study was to review the management of children with Wilms' tumour who have intracardiac extension. PATIENTS AND METHODS: Data were collected from patient notes regarding presentation, operative details, and outcome. RESULTS: From 1984 through 2011, 264 children with Wilms' tumour were treated at our hospital. Nine (3.4%) had cavo-atrial extension of the tumour thrombus. The thrombus extended into the right ventricle in two children and involved the hepatic veins in both, and also two others...
February 2013: Journal of Pediatric Surgery
Julien Baruteau, Philippe Sachs, Pierre Broué, Michèle Brivet, Hendy Abdoul, Christine Vianey-Saban, Hélène Ogier de Baulny
INTRODUCTION: Mitochondrial fatty acid β-oxidation defects (FAODs) are a group of severe inherited metabolic diseases, most of which can be treated with favorable prognosis following diagnosis. A description of the broad range of phenotypes resulting from these defects remains incomplete, and for this study, we sought to investigate the semiology at diagnosis in a country without a newborn screening program for FAODs. METHODS: Using a retrospective French multicentre study, we analyzed 187 children aged <6 years at diagnosis with FAOD confirmed by enzymatic and/or molecular analyses...
September 2013: Journal of Inherited Metabolic Disease
Stéphanie Franchi-Abella, Sophie Branchereau, Virginie Lambert, Monique Fabre, Clarisa Steimberg, Jean Losay, Jean-Yves Riou, Danièle Pariente, Frédéric Gauthier, Emmanuel Jacquemin, Olivier Bernard
BACKGROUND AND OBJECTIVE: Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature. PATIENTS AND METHODS: Twenty-two children with a complicated congenital portosystemic shunt were studied in our institution...
September 2010: Journal of Pediatric Gastroenterology and Nutrition
Amir-Reza Hosseinpour, Shay Cullen, Victor T Tsang
Heart transplantation is a recognised treatment for end-stage heart failure of any cause including congenital heart disease. Congenital heart disease has contributed relatively little to the adult heart transplant activities in the past two decades. However, this is likely to change as an increasing number of children with congenital heart disease reach adulthood because of the advances in paediatric cardiology and surgery. Some of these grown-ups with congenital heart disease (GUCH patients) will need transplantation for late myocardial dysfunction either secondary to uncorrected lesions, or despite previous repair or palliative surgery...
September 2006: European Journal of Cardio-thoracic Surgery
Shirley Hui-Stickle, Eileen D Brewer, Stuart L Goldstein
BACKGROUND: Previous epidemiological data for pediatric patients with acute renal failure (ARF) predate current intensive care unit (ICU) technology and practice, and do not reflect newer disease therapies for bone marrow, hepatic, and cardiac transplantation and congenital heart disease surgery. METHODS: We conducted a retrospective review of 254 ARF episodes in 248 children discharged from a tertiary referral center, Texas Children's Hospital (Houston, TX), between January 1998 and June 2001 to update current pediatric ARF epidemiological characteristics...
January 2005: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
C B Lim, Y S Choy
We retrospectively studied the records of 6 Malaysian children who were diagnosed with Alagille Syndrome (AGS) according to this criteria from January 1999 to January 2001, at the Institute of Paediatrics, Kuala Lumpur Hospital. Four patients (66%) had a positive family history. Thirteen individuals (6 patients and 7 relatives) were diagnosed with AGS in these 5 families. Only 6/13 (46%) of them presented with liver involvement. All 6 patients presented with typical facies and cholestasis (100%). Three (50%) presented with portal hypertension (PHT) with synthetic liver dysfunction (1 died), 1/6 (17%) have PHT and normal synthetic liver function...
December 2003: Medical Journal of Malaysia
Ara Kassarjian, David Zurakowski, Josée Dubois, Harriet J Paltiel, Steven J Fishman, Patricia E Burrows
OBJECTIVE: This study was undertaken to determine different imaging patterns in infantile hepatic hemangiomas and to explore the relationship between clinical presentations, imaging findings, and response to therapy. MATERIALS AND METHODS: The imaging studies and clinical records of all patients with infantile hepatic hemangiomas from two tertiary children's hospitals were reviewed. Univariate and multivariate stepwise logistic regression techniques were used to determine whether clinical presentation and imaging variables differentiated the type of treatment required...
March 2004: AJR. American Journal of Roentgenology
Agostino Pierro, Simon Eaton
Intestinal ischemia-reperfusion is a common pathway for many diseases in infants, children, and adults, and this may lead to multiple organ dysfunction syndrome and death. While several studies have investigated reperfusion injury in cardiac, cerebral, and hepatic disease, limited work has been published on intestinal ischemia-reperfusion and its multiorgan effects. The authors have developed models of intestinal ischemia-reperfusion in rats and have demonstrated that intestinal reperfusion causes liver energy failure at normothermia...
February 2004: Seminars in Pediatric Surgery
R Chaturvedi, M de Leval, I D Sullivan
OBJECTIVE: To assess the results of early homograft aortic root replacement in infants and children with an aortic root abscess. DESIGN: Descriptive study of all patients with an aortic root abscess during 1987-97, identified by retrospective review of the echocardiographic and surgical registries. SETTING: A tertiary referral centre. PATIENTS: Five patients (age 0.6 to 13 years; two female) were identified with an aortic root abscess...
January 1999: Heart: Official Journal of the British Cardiac Society
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