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Hepatic cardiac syndrome children

Kimberly Lewis, Ryan Butts, J Antonio Quiros, Michelle Hudspeth, Katherine Twombley, Andrew Savage, Sally Self, Ali Burnette, Shaoli Sun
AIE is a rare disorder in children that presents with severe diarrhea and malabsorption, caused by immune-mediated damage to intestinal mucosa. AIE is often associated with various syndromes of immunodeficiency including IPEX syndrome (immune dysregulation, polyendocrinopathy and enteropathy, X-linked). Dysfunctional T regulatory cells are the source of pathology in both IPEX syndrome and AIE as they are essential in maintaining tolerance to self-antigens and eliminating autoreactive B cells. This case report describes a 10-year-old cardiac transplant and total thymectomy patient on chronic immunosuppression with tacrolimus that presented with AIE and extraintestinal manifestations of cyclical hepatitis...
March 2017: Pediatric Transplantation
Aya M Fattouh, Engy A Mogahed, Nehal Abdel Hamid, Rodina Sobhy, Noha Saber, Hanaa El-Karaksy
BACKGROUND: There is deficiency of data about congenital heart defects (CHDs) in cholestatic disorders of infancy other than Alagille syndrome (AGS). We aimed to define the prevalence and types of CHDs in infants with various causes of cholestatic disorders of infancy. METHODS: This cross-sectional study was conducted on 139 infants presenting with cholestasis whether surgical or non-surgical. The study was carried out at the Pediatric Hepatology Unit, Cairo University Children's Hospital, Egypt...
September 2016: Archives of Disease in Childhood
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
Samuel Ademola Adegoke, John Akintunde Okeniyi, Adeseye Abiodun Akintunde
BACKGROUND: Lipid and electrocardiographic (ECG) abnormalities have been reported in adults with sickle cell anaemia (SCA) and may reflect underlying structural and/or functional damage. However, the relationship between ECG and lipid abnormalities among children with sickle cell disease is not fully understood. OBJECTIVES: To compare the steady-state lipid and ECG abnormalities in children with SCA to the controls and examine the hypothesis that lipid abnormalities are closely related to electrocardiographic abnormalities, and therefore are a reflection of cardiac damage among these children...
January 2016: Cardiovascular Journal of Africa
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
Eduardo Cuestas, María Isabel Gaido, Raúl Horacio Capra
OBJECTIVE: Transient neonatal hyperthyrotropinemia (TNH) is defined as a neonatal abnormality of thyroid function, which reverts to normal at re-examination after 2 weeks of life. The thyroid function of these infants has not been sufficiently studied in terms of the risk of developing persistent hyperthyrotropinemia (PH) in later childhood and its impact on growth and development. DESIGN: A prospective cohort study included all babies born in our hospital between 2001 and 2006 and screened for hypothyroidism, whose thyroid function was re-examined 6 years later...
April 2015: European Journal of Endocrinology
Ana Maria Tudor
INTRODUCTION: The shift in epidemic trends in recent years in Romania shows new problems in regard of HIV vertical transmission, firstly in intravenous drug user's mothers co-infected with hepatitis viruses and with social problems, and secondly the children of young mothers with an old HIV infection and long antiretroviral therapy history. MATERIALS AND METHODS: We studied all HIV perinatal exposed children routinely followed up in the Paediatric Department of the National Institute of Infectious Diseases, since January 1st 2006 till December 31st 2012...
2014: Journal of the International AIDS Society
Lucia Pacifico, Claudio Chiesa, Caterina Anania, Antonio De Merulis, John Frederick Osborn, Sara Romaggioli, Eugenio Gaudio
Over the last two decades, the rise in the prevalence rates of overweight and obesity explains the emergence of nonalcoholic fatty liver disease (NAFLD) as the leading cause of chronic liver disease worldwide. As described in adults, children and adolescents with fatty liver display insulin resistance, glucose intolerance, and dyslipidemia. Thus NAFLD has emerged as the hepatic component of the metabolic syndrome (MetS) and a strong cardiovascular risk factor even at a very early age. Several studies, including pediatric populations, have reported independent associations between NAFLD and markers of subclinical atherosclerosis including impaired flow-mediated vasodilation, increased carotid artery intima-media thickness, and arterial stiffness, after adjusting for cardiovascular risk factors and MetS...
July 21, 2014: World Journal of Gastroenterology: WJG
Rachana Kathuria, Anshu Srivastava, Surender K Yachha, Ujjal Poddar, Sanjay S Baijal
OBJECTIVES: 'Radiological intervention' to restore venous patency is the preferred therapy in adults with Budd-Chiari syndrome (BCS). The published literature on pediatric BCS is scarce. This study evaluated the clinical profile and role of a therapeutic radiological intervention in children with BCS. PATIENTS AND METHODS: Forty-six BCS children [29 boys, median age 10.5 (2-16) years] were enrolled. Standard medical therapy was administered to all. A radiological intervention, angioplasty [hepatic vein (HV) (n=3)], stenting [HV (n=18), inferior vena cava (IVC) (n=5)], transjugular intrahepatic portosystemic shunt (TIPS) (n=3), was performed in 25 cases...
September 2014: European Journal of Gastroenterology & Hepatology
Rabea Alhosh, Yuri Genyk, Sophoclis Alexopoulos, Daniel Thomas, Shengmei Zhou, George Yanni, Nanda Kerkar
HPS is a significant complication of portal hypertension in children with chronic liver disease and is an established indication for LT. It is characterized clinically by the triad of pulmonary vascular dilatation causing hypoxemia in the setting of advanced liver disease. NRH, a cause of non-cirrhotic portal hypertension, is characterized by diffuse benign transformation of the hepatic parenchyma into small regenerative nodules with minimal or no fibrosis. Development of NRH and HPS in pediatric LT recipients has not been reported, although occasional cases have been reported in adult LT recipients...
August 2014: Pediatric Transplantation
Hiromi Matsuda, Masaya Toda, Yasuharu Kosaka, Masayasu Arai, Hirotsugu Okamoto
It is known that acute liver dysfunction is one of the complications after Fontan operation. We tend to overlook it because their laboratory abnormalities are typically mild and hepatic dysfunction is an uncommon complication in children after cardiac surgery. However, this complication is likely to be an important indicator of poor prognosis. We report a patient who showed a prominent elevation of liver enzymes after Fontan operation. A year and 5 month old boy was scheduled for Fontan operation due to hypoplastic left heart syndrome...
April 2014: Masui. the Japanese Journal of Anesthesiology
Cresio Alves, Julia Constança Fernandes, Silvana Sampaio, Raquel de Melo Alves Paiva, Rodrigo Tocantins Calado
Herein the first molecular diagnosis of a Brazilian child with Shwachman-Diamond Syndrome is reported. A 6-year-old boy was diagnosed with cystic fibrosis at the age of 15 months due to recurrent respiratory infections, diarrhea and therapeutic response to pancreatic enzymes. Three sweat tests were negative. At the age of 5 years, he began to experience pain in the lower limbs, laxity of joints, lameness and frequent falls. A radiological study revealed metaphyseal chondrodysplasia. A complete blood cell count showed leukopenia (leukocytes: 3...
2013: Revista Brasileira de Hematologia e Hemoterapia
Binita M Kamath, Nancy B Spinner, Norman D Rosenblum
Alagille syndrome is an autosomal dominant disorder with variable multisystem organ involvement that is caused by mutations in one of two genes in the Notch signalling pathway, JAG1 or NOTCH2. Alagille syndrome is characterized by bile duct paucity, along with at least three of the following features: cholestasis, cardiac defects, skeletal abnormalities, ocular abnormalities and characteristic facies. However, the clinical features of Alagille syndrome are highly variable, and children or adults may also present with predominantly renal findings and little or no hepatic involvement...
July 2013: Nature Reviews. Nephrology
Zain Husain, Bobby Y Reddy, Robert A Schwartz
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, also referred to as drug-induced hypersensitivity syndrome, is a distinct, potentially life-threatening adverse reaction. It is seen in children and adults most often as a morbilliform cutaneous eruption with fever, lymphadenopathy, hematologic abnormalities, and multiorgan manifestations. Historically, it was most frequently linked with phenytoin and known as phenytoin hypersensitivity syndrome. However, because many other medications were found to produce the same reaction, another name was in order...
May 2013: Journal of the American Academy of Dermatology
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
Jennifer S Kicker, Julie A Haizlip, Marcia L Buck
A former 34-week-old female infant with Down syndrome underwent surgical correction of a congenital heart defect at 5 months of age. Her postoperative course was complicated by severe pulmonary hypertension and junctional ectopic tachycardia. Following treatment with amiodarone infusion, she developed laboratory indices of acute liver injury. At their peak, liver transaminase levels were 19 to 35 times greater than the upper limit of normal. Transaminitis was accompanied by coagulopathy, hyperammonemia, and high serum lactate and lipid levels...
April 2012: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
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
C Pienkowski, A Cartault, S Caula-Legriel, Z Ajaltouni, M Daudin, M Tauber
Klinefelter's syndrome (KS) affects one in 600 men and Turner's syndrome (TS), one in 2500 women. These 2 diseases are the most sex chromosome disorders characterized by one extra X in the SK male (47XXY) and the loss of an X in the girls with ST (45 X). Their common characteristic is the gonadal dysgenesis, which is the main cause of male or female infertility. Called "the forgotten syndrome", KS is under-diagnosed because apart from the large size, there are no dysmorphic features, along with a great ignorance of cognitive and language disorders in children...
September 2011: Gynécologie, Obstétrique & Fertilité
Jordan M Wright, Raymond G Watts
Venous thrombosis is an infrequent but serious cause of hospitalization in children. The epidemiology and natural history remains incompletely defined, especially in geographically distinct regions of the United States. We thus evaluated thrombosis in a single children's hospital over a 3-year period. Of 41,906 hospitalizations, 92 children were identified for review. The incidence of thrombosis was 21.9 per 10,000 admissions (0.22%). Venous thrombosis was of equal incidence in African-American and white patients...
May 2011: Journal of Pediatric Hematology/oncology
Dorota Broniszczak, Artur Apanasiewicz, Piotr Czubkowski, Piotr Kaliciński, Hor Ismail, Anna Ostoja-Chyzynska, Malgorzata Markiewicz-Kijewska
BACKGROUND: Children with biliary atresia and polysplenia syndrome (BA-PS) have always been considered as high risk liver transplant recipients due to technical problems during transplant surgery. We report single-center experience with liver transplantation in children with this syndrome. MATERIAL/METHODS: Between 2000 and 2010, 401 liver transplantations were performed in 358 children, including 6 patients with BA-PS, who underwent living (5 patients) or deceased (1 patient) donor liver transplantation...
January 2011: Annals of Transplantation: Quarterly of the Polish Transplantation Society
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