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https://www.readbyqxmd.com/read/28214296/abnormally-high-and-heterogeneous-bone-matrix-mineralization-after-childhood-solid-organ-transplantation-a-complex-pathology-of-low-bone-turnover-and-local-defects-in-mineralization
#1
Nadja Fratzl-Zelman, Helena Valta, Renata C Pereira, Barbara M Misof, Paul Roschger, Hannu Jalanko, Kathrine Wesseling-Perry, Klaus Klaushofer, Outi Mäkitie
Chronic renal, liver and heart failure in children associates with multiple skeletal complications. Increased fracture incidence often persists after transplantation and could be related to alterations in bone material properties. In the present cohort study we evaluated bone mineralization density distribution (BMDD) by quantitative backscattered electron imaging (qBEI) in 23 pediatric solid organ allograft recipients with suspected osteoporosis. We measured BMDD in the entire cross-sectional area of transiliac bone biopsies obtained from kidney (n = 9), liver (n = 9) and heart (n = 5) transplant recipients (aged 7...
February 18, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28213954/human-herpes-virus-6-infection-in-pediatric-organ-transplant-patients
#2
Elisa Ylinen, Silja Lehtinen, Timo Jahnukainen, Teemu Karlsson, Raisa Loginov, Laura Mannonen, Irmeli Lautenschlager, Hannu Jalanko
Transplant patients need lifelong immunosuppressive medication, but this reduces their defense mechanisms, making them prone to viral infections and reactivations. We aimed to clarify the prevalence and clinical manifestations of the human herpes virus 6 (HHV-6) infection in children after pediatric solid organ transplants. Clinical findings and viral loads were compared between primary HHV-6 infections and reactivations. The study comprised 47 kidney, 25 liver, and 12 heart transplant patients who underwent surgery from 2009 to 2014...
February 18, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28213931/successful-resumption-of-peritoneal-dialysis-following-living-donor-liver-transplantation-in-children-with-end-stage-renal-disease
#3
Hiroyuki Kanazawa, Akinari Fukuda, Mai Sato, Shingo Ishimori, Kengo Sasaki, Hajime Uchida, Takanobu Shigeta, Vidyadhar Padmakar Mali, Seisuke Sakamoto, Kenji Ishikura, Mureo Kasahara
Children with ESRD in need of RRT are commonly managed by PD due to difficulty with vascular access for HD and the relatively large extracorporeal blood volume required. Major abdominal surgery may result in injury to the peritoneum and consequent adhesion, thereby resulting in a reduction in the anatomical capacity and transport capability across the peritoneal membrane. Here, we report successful resumption of PD after LDLT in two pediatric patients. The causes of ESRD were PH1 and juvenile nephronophthisis, respectively...
February 18, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28211259/liver-transplantation-from-a-deceased-donor-with-%C3%AE-thalassemia-intermedia-is-not-contraindicated-a-case-report
#4
Ersin Gumus, Osman Abbasoglu, Cahit Tanyel, Fatma Gumruk, Hasan Ozen, Aysel Yuce
The use of extended criteria donors who might have previously been deemed unsuitable is an option to increase the organ supply for transplantation. This report presents a pediatric case of a successful liver transplantation from a donor with β-thalassemia intermedia. A patient, 6-year-old female, with a diagnosis of cryptogenic liver cirrhosis underwent deceased donor liver transplantation from a thalassemic donor. Extreme hyperferritinemia was detected shortly after transplantation. The most probable cause of hyperferritinemia was iron overload secondary to transplantation of a hemosiderotic liver...
February 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28203453/association-of-osteoprotegerin-with-obesity-insulin-resistance-and-non-alcoholic-fatty-liver-disease-in-children
#5
Meltem Erol, Ozlem Bostan Gayret, Hikmet Tekin Nacaroglu, Ozgul Yigit, Oguzhan Zengi, Mehmet Salih Akkurt, Mehmet Tasdemir
BACKGROUND: Osteoprotegerin (OPG) is a member of the tumor necrosis factor superfamily. Reduced OPG levels are related to obesity, insulin resistance, and non-alcoholic fatty liver disease (NAFLD). OBJECTIVES: The aim of this study was to evaluate the relationship between OPG levels, obesity, insulin resistance, and NAFLD in pediatric patients. METHODS: This was a prospective, cross-sectional, controlled study that was conducted in the department of pediatrics at Bagcilar training and research hospital in Istanbul, Turkey, between April and August 2015...
November 2016: Iranian Red Crescent Medical Journal
https://www.readbyqxmd.com/read/28203111/unresectable-hepatoblastoma-current-perspectives
#6
REVIEW
Angela D Trobaugh-Lotrario, Rebecka L Meyers, Allison F O'Neill, James H Feusner
Although rare, hepatoblastoma is the most common pediatric liver tumor. Complete resection is a critical component for cure; however, most patients will have tumors that are not resected at diagnosis. For these patients, administration of neoadjuvant chemotherapy renders tumors resectable in most patients. For patients whose tumors remain unresectable after chemotherapy, liver transplantation is indicated (in the absence of active unresectable metastatic disease). In patients whose tumors remain unresectable after conventional chemotherapy, interventional techniques may serve as a promising option to reduce tumor size, decrease systemic toxicity, decrease need for liver transplantation, and increase feasibility of tumor resection...
2017: Hepatic Medicine: Evidence and Research
https://www.readbyqxmd.com/read/28201980/procalcitonin-for-infections-in-the-first-week-after-pediatric-liver-transplantation
#7
Vladimir L Cousin, Kalinka Lambert, Shahar Trabelsi, Annick Galetto-Lacour, Klara M Posfay-Barbe, Barbara E Wildhaber, Valérie A McLin
BACKGROUND: Procalcitonin (PCT) has become a commonly used serum inflammatory marker. Our aim was to describe the kinetics and usefulness of serial post-operative PCT measurements to detect bacterial infection in a cohort of children immediately after pediatric liver transplantation (pLT). METHODS: We performed a retrospective chart review of a cohort of pLT recipients with serial serum PCT measurements in the first week following pLT. The presence of infection was determined on clinical and biological parameters...
February 15, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28199778/a-national-retrospective-study-of-peld-as-a-predictor-of-change-to-second-line-therapy-in-children-with-autoimmune-hepatitis
#8
Andréanne N Zizzo, Carolina Jimenez-Rivera, Joseph Kim, Richard A Schreiber, Simon C Ling, Jason Yap, Jeff Critch, Najma Ahmed, Fernando Alvarez, Binita M Kamath
BACKGROUND & AIMS: Adult studies of autoimmune hepatitis (AIH) have shown that the model of end-stage liver disease (MELD) is associated with resistance to first-line treatment. Using a multi-centre retrospective database, we sought to determine if the pediatric end-stage liver disease (PELD) score would similarly predict treatment resistance in pediatric AIH. METHODS: One hundred and seventy-one children from 13 Canadian centres who fulfilled the International Autoimmune Hepatitis Group (IAIHG) criteria were included and assessed for change to second-line therapy within 24 months of primary treatment onset...
February 15, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28197039/surgical-illustration-of-en-bloc-dual-kidney-transplant-from-a-16-month-old-brain-dead-donor-to-an-adult-recipient
#9
Vikas Jain, Saurabh Jain, Paras Singhal, Suman Lata Nayak, Rajendra P Mathur
Transplantable organs from pediatric donors have been contributing significantly to donor pool worldwide. Pediatric donors are excellent resources that should be procured whenever available, and with the recent increase in deceased donations in India, more pediatric donors will be available for organ harvesting. We share a rare instance of multi-organ harvesting from a 16-month old brain dead donor and implanting both kidneys en-bloc in an adult male, while liver went to a 4-year old child. The report provides the surgical illustration of salient steps of transplanting both kidneys from pediatric donor into an adult, in an en-bloc manner...
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28194394/assessment-of-school-readiness-in-chronic-cholestatic-liver-disease-a-pilot-study-examining-children-with-and-without-liver-transplantation
#10
Anna Gold, Alaine Rogers, Elizabeth Cruchley, Stephanie Rankin, Arpita Parmar, Binita M Kamath, Yaron Avitzur, Vicky Lee Ng
Background. Assessment of school readiness evaluates physical, social-emotional, and neuropsychological domains essential for educational success. Cognitive testing of preschool aged children with chronic liver disease may guide more timely interventions and focused efforts by health care providers. Patients and Methods. Children with chronic cholestatic liver disease diagnosed as an infant and still with their native liver (NL) and children who received a liver transplant (LT) before age of 2 years underwent testing with a battery of well-validated pediatric psychometric measures...
2017: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28192105/adolescent-obesity-and-insulin-resistance-roles-of-ectopic-fat-accumulation-and-adipose-inflammation
#11
Sonia Caprio, Rachel Perry, Romy Kursawe
As a consequence of the global rise in the prevalence of Adolescent Obesity, an unprecedented phenomenon of Type 2 Diabetes (T2D) has emerged in pediatrics. At the heart of T2D development lies a key metabolic derangement: Insulin Resistance (IR) . Despite the widespread occurrence of IR affecting an unmeasurable number of youths worldwide, its pathogenesis remains elusive. IR in obese youth is a complex phenomenon that defies explanation by a single pathway. In this review we first describe recent data on the prevalence, severity, and racial/ethnic differences in Pediatric Obesity...
February 9, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28184367/novel-immunotherapies-for-autoimmune-hepatitis
#12
REVIEW
Shamir Cassim, Marc Bilodeau, Catherine Vincent, Pascal Lapierre
Autoimmune hepatitis (AIH) is a multifactorial autoimmune disease of unknown pathogenesis, characterized by a loss of immunological tolerance against liver autoantigens resulting in the progressive destruction of the hepatic parenchyma. Current treatments are based on non-specific immunosuppressive drugs. Although tremendous progress has been made using specific biological agents in other inflammatory diseases, progress has been slow to come for AIH patients. While current treatments are successful in the majority of patients, treatment discontinuation is difficult to achieve, and relapses are frequent...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28181361/a-systematic-review-of-parent-and-family-functioning-in-pediatric-solid-organ-transplant-populations
#13
REVIEW
Melissa K Cousino, Kelly E Rea, Kurt R Schumacher, John C Magee, Emily M Fredericks
The process of pediatric solid organ transplantation (SOT) places new and increased stressors on patients and family members. Measures of family functioning may predict psychological and health outcomes for pediatric patients and their families, and provide opportunity for targeted intervention. This systematic review investigated parent and family functioning and factors associated with poorer functioning in the pediatric SOT population. Thirty-seven studies were identified and reviewed. Studies featured a range of organ populations (eg, heart, liver, kidney, lung, intestine) at various stages in the transplant process...
February 8, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28180024/ciliopathies-genetics-in-pediatric-medicine
#14
REVIEW
Machteld M Oud, Ideke J C Lamers, Heleen H Arts
Ciliary disorders, which are also referred to as ciliopathies, are a group of hereditary disorders that result from dysfunctional cilia. The latter are cellular organelles that stick up from the apical plasma membrane. Cilia have important roles in signal transduction and facilitate communications between cells and their surroundings. Ciliary disruption can result in a wide variety of clinically and genetically heterogeneous disorders with overlapping phenotypes. Because cilia occur widespread in our bodies many organs and sensory systems can be affected when they are dysfunctional...
March 2017: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/28173725/contrast-enhanced-ultrasound-using-sulfur-hexafluoride-is-safe-in-the-pediatric-setting
#15
Alvaro Torres, Seppo K Koskinen, Henrik Gjertsen, Björn Fischler
Background Contrast-enhanced ultrasound (CEUS) by using sulfur hexafluoride microbubbles is not licensed for use in children, but its off-label use is widespread. Purpose To outline our experience with the off-label use of CEUS in children, specifically with regards to safety. Material and Methods We retrieved all records of 10681 patients aged under 18 years who underwent abdominal ultrasound (US) January 2004 to December 2014. We then identified those who underwent an abdominal CEUS using sulfur hexafluoride microbubbles...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28167225/no-child-left-behind-liver-transplantation-in-critically-ill-children
#16
Abbas Rana, Michael Kueht, Moreshwar Desai, Fong Lam, Tamir Miloh, Jennifer Moffett, N Thao N Galvan, Ronald Cotton, Christine O'Mahony, John Goss
BACKGROUND: Advances in critical care prolong survival in children with liver failure, allowing more critically ill children to undergo liver transplantation (OLT). In order to justify the use of a scarce donor resource and avoid futile transplants, we sought to determine the survival in children who undergo OLT while receiving pre-OLT critical care. STUDY DESIGN: We analyzed 13,723 pediatric OLTs using the UNOS database from 1987-2015, including 6,746 recipients in the MELD/PELD era (2002-2015)...
January 5, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/28162781/intra-articular-corticosteroids-versus-intra-articular-corticosteroids-plus-methotrexate-in-oligoarticular-juvenile-idiopathic-arthritis-a-multicentre-prospective-randomised-open-label-trial
#17
Angelo Ravelli, Sergio Davì, Giulia Bracciolini, Angela Pistorio, Alessandro Consolaro, Evert Hendrik Pieter van Dijkhuizen, Bianca Lattanzi, Giovanni Filocamo, Sara Verazza, Valeria Gerloni, Maurizio Gattinara, Irene Pontikaki, Antonella Insalaco, Fabrizio De Benedetti, Adele Civino, Giuseppe Presta, Luciana Breda, Valentina Marzetti, Serena Pastore, Silvia Magni-Manzoni, Maria Cristina Maggio, Franco Garofalo, Donato Rigante, Marco Gattorno, Clara Malattia, Paolo Picco, Stefania Viola, Stefano Lanni, Nicolino Ruperto, Alberto Martini
BACKGROUND: Little evidence-based information is available to guide the treatment of oligoarticular juvenile idiopathic arthritis. We aimed to investigate whether oral methotrexate increases the efficacy of intra-articular corticosteroid therapy. METHODS: We did this prospective, open-label, randomised trial at ten hospitals in Italy. Using a concealed computer-generated list, children younger than 18 years with oligoarticular-onset disease were randomly assigned (1:1) to intra-articular corticosteroids alone or in combination with oral methotrexate (15 mg/m(2); maximum 20 mg)...
February 2, 2017: Lancet
https://www.readbyqxmd.com/read/28162008/clinical-implications-of-understanding-the-association-between-oxidative-stress-and-pediatric-nafld
#18
Jake P Mann, Massimiliano Raponi, Valerio Nobili
Oxidative stress is central to the pathogenesis of non-alcoholic steatohepatitis. The reactive oxygen species (ROS) that characterise oxidative stress are generated in several cellular sites and their production is influence by multi-organ interactions. Areas covered: Mitochondrial dysfunction is the main source of ROS in fatty liver and is closely related to endoplasmic reticulum stress. Both are caused by lipotoxicity and together these three factors form a cycle of progressive organelle damage, resulting in sterile inflammation and apoptosis...
February 6, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28160250/thiopurines-and-methotrexate-use-in-ibd-patients-in-a-biologic-era
#19
REVIEW
Gerassimos J Mantzaris
Although we have been living in the era of biologic therapy for several decades, the use of immunomodulators (primarily thiopurines [azathioprine and mercaptopurine] and less so methotrexate) still remains an important component of the inflammatory bowel disease (IBD) pharmaceutical arsenal. Thiopurines as monotherapy exert corticosteroid-sparing effects and can maintain long-term remission in a considerable proportion of patients who have frequent relapses and are or have become mesalazine and/or corticosteroid intolerant or refractory...
February 3, 2017: Current Treatment Options in Gastroenterology
https://www.readbyqxmd.com/read/28159955/respiratory-morbidity-following-pediatric-orthotopic-liver-transplantation
#20
Shahzad Alam, Anilkumar Sapare, Sanjay Rao, Rajiv Aggarwal, Ashley L D'cruz
We evaluated the pulmonary complications following orthotopic liver transplantation in children (age <18 y). Twenty-two patients (49%) developed respiratory complications. Pediatric end-stage liver disease(PELD) score >25 and positive fluid balance were independent risk factors. Patients with respiratory complication had significantly higher mortality and intensive care unit stay.
February 2, 2017: Indian Pediatrics
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