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https://www.readbyqxmd.com/read/29146706/the-clinical-and-laboratory-evaluation-of-familial-hemophagocytic-lymphohistiocytosis-and-the-importance-of-hepatic-and-spinal-cord-involvement-a-single-center-experience
#1
Burcin Beken, Selin Aytac, Gunay Balta, Baris Kuskonmaz, Duygu Uckan, Sule Unal, Mualla Cetin, Fatma Gumruk
Familial hemophagocytic lymphohistiocytosis is an autosomal recessive, life-threatening condition characterized by defective immune response. A retrospective analysis was performed on 57 patients diagnosed with familial hemophagocytic lymphohistiocytosis at Hacettepe University Pediatric Hematology Department. Mutation analysis was performed on 37 patients and of these; 11 had UNC13D, 10 had PRF1 and 3 had STX11 gene mutation. Of these patients 44% were found to have central nervous system involvement on admission and spinal cord involvement was also seen in 5 patients...
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29140938/immunity-following-childhood-vaccinations-in-perinatally-hiv-exposed-children-with-and-without-hiv-infection-in-latin-america
#2
Regina Cm Succi, Margot R Krauss, D Robert Harris, Daisy M Machado, Maria I de Moraes-Pinto, Marisa M Mussi-Pinhata, Noris Pavia Ruz, Russell B Pierre, Lenka A Kolevic Roca, Esaú Joao, Irene Foradori, Marcelo C Scotta, Rohan Hazra, George K Siberry
BACKGROUND: Perinatally HIV-infected (PHIV) children are at risk for undervaccination and poor vaccine response at four years of age. Childhood vaccine coverage and immune response were compared between PHIV and HIV-exposed uninfected (HEU) children in Latin America and the Caribbean. METHODS: PHIV and HEU children were enrolled prospectively at fifteen sites from 2002-2009. Full vaccination by age four years was defined as: three hepatitis B virus (HBV) vaccine doses; four tetanus toxoid-containing vaccine doses; three doses of Haemophilus influenzae type b (Hib) vaccine by age 12 months or ≥1 dose given after age 12 months; one measles-containing vaccine dose; one rubella-containing vaccine dose...
November 14, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29138738/spatial-distribution-and-trends-of-waterborne-diseases-in-tashkent-province
#3
Veluswami Saravanan Subramanian, Min Jung Cho, Siwei Zoe Tan, Dilorom Fayzieva, Christian Sebaly
Introduction: The cumulative effect of limited investment in public water systems, inadequate public health infrastructure, and gaps in infectious disease prevention increased the incidence of waterborne diseases in Uzbekistan. The objectives of this study were: (1) to spatially analyze the distribution of the diseases in Tashkent Province, (2) to identify the intensity of spatial trends in the province, (3) to identify urban-rural characteristics of the disease distribution, and (4) to identify the differences in disease incidence between pediatric and adult populations of the province...
2017: Central Asian Journal of Global Health
https://www.readbyqxmd.com/read/29132458/-efficacy-of-analgesic-and-sedative-treatments-in-children-with-mechanical-ventilation-in-the-pediatric-intensive-care-unit
#4
Xiao-Fang Cai, Fu-Rong Zhang, Long Zhang, Ji-Min Sun, Wen-Bin Li
OBJECTIVE: To compare the efficacy and safety of different analgesic and sedative treatments in children with mechanical ventilation in the pediatric intensive care unit (PICU). METHODS: Eighty children with mechanical ventilation in the PICU who needed analgesic and sedative treatments were equally and randomly divided into midazolam group and remifentanil+midazolam group. The sedative and analgesic effects were assessed using the Ramsay Scale and the Face, Legs, Activity, Cry and Consolability (FLACC) Scale...
November 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29120853/ultrastructural-characteristics-of-the-respective-forms-of-hepatic-stellate-cells-in-chronic-hepatitis-b-as-an-example-of-high-fibroblastic-cell-plasticity-the-first-assessment-in-children
#5
Joanna Maria Lotowska, Maria Elzbieta Sobaniec-Lotowska, Dariusz Marek Lebensztejn
PURPOSE: Activation of hepatic stellate cells (HSCs), mainly responsible for extracellular matrix synthesis, is assumed to be central event in the process of liver fibrogenesis. The major objective of the research was to analyze the ultrastructural profile of activated HSCs in children with chronic hepatitis B (chB), with respect to fibrosis intensity. MATERIALS/METHODS: Ultrastructural investigations of HSCs were conducted on liver bioptates from 70 children with clinicopathologically diagnosed chB before antiviral treatment...
November 6, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/29115027/clinical-features-of-pediatric-autoimmune-hepatitis-in-japan-a-nationwide-survey
#6
Tsuyoshi Sogo, Atsushi Takahashi, Ayano Inui, Tomoo Fujisawa, Hiromasa Ohira, Hajime Takikawa
AIM: The purpose of this study was to determine the characteristics of children with autoimmune hepatitis (AIH) in Japan. METHODS: Questionnaires, which asked about patients newly diagnosed with AIH from 2009 to 2013 were sent to hospitals certified as training facilities for pediatrics in January 2015. RESULTS: A total of 35 patients were enrolled. The median age at diagnosis was 10 years (ranging from 3 months to 15 years), and the male-to-female ratio was 2:3...
November 8, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29110981/distributive-shock-in-a-neonate-with-diffuse-infantile-hepatic-hemangioma
#7
James Brown, Rajan Arora, Usha Sethuraman
BACKGROUND: Hemangiomas are common cutaneous findings on healthy infants. These vascular malformations are generally benign, though in rare circumstances they can potentially be fatal. This is particularly true when the hemangiomas are large or numerous and occurring in visceral organs. Previously unrecognized visceral hemangiomas are part of the differential for any neonate presenting unexpectedly in shock. CASE REPORT: A 10-day-old neonate presented to the pediatric emergency department with difficulty breathing...
October 27, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29106926/increasing-prevalence-of-hepatitis-c-among-hospitalized-children-is-associated-with-an-increase-in-substance-abuse
#8
A Sidney Barritt, Brian Lee, Thomas Runge, Monica Schmidt, Ravi Jhaveri
OBJECTIVE: To evaluate the impact of substance abuse on pediatric hepatitis C virus (HCV) prevalence, we examined geographic and demographic data on inpatient hospitalizations in children with HCV. STUDY DESIGN: We examined hospitalizations in children using the Kids' Inpatient Database, a part of the Healthcare Cost and Utilization Project. We identified cases using the International Classification of Diseases, 9th edition, codes for HCV infection during 2006, 2009, and 2012...
October 26, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29105236/liver-abnormalities-and-post-transplant-survival-in-pediatric-fontan-patients
#9
Sophie C Hofferberth, Tajinder P Singh, Heather Bastardi, Elizabeth D Blume, Francis Fynn-Thompson
The impact of liver parenchymal abnormalities on survival post-heart transplant remains unknown in pediatric Fontan patients. We assessed pediatric Fontan patients who underwent heart transplant and had documented pretransplant hepatic ultrasound (U/S) studies. Liver U/S findings were classified as normal (Group 1), mildly abnormal (Group 2, hepatomegaly/vascular congestion), or severely abnormal (Group 3, heterogeneous echotexture/nodularity). Among 30 study patients, 8 were classified as Group 1, 14 as Group 2, while 8 met Group 3 criteria...
November 3, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29097861/biliary-complications-after-hepatic-trauma-in-children
#10
Riccardo Guanà, Carbonaro Giulia, Andrea Brunati, Salvatore Garofalo, Jurgen Schleef
Aim of the Study: In pediatric patients with liver trauma and hemodynamic stability, conservative treatment is acknowledged as the gold standard. Patients and Methods: We conducted a retrospective analysis of 116 consecutive pediatric patients (<14-year-old) observed at our institution for closed abdominal trauma from January 2010 to January 2016. Among these, 16 patients (13%) had hepatic trauma Grade II or more, according to Moore liver trauma injury score...
October 2017: Journal of Emergencies, Trauma, and Shock
https://www.readbyqxmd.com/read/29089679/embryonal-rhabdomyosarcoma-of-the-biliary-tree-a-rare-cause-of-obstructive-jaundice-in-children-which-can-mimic-choledochal-cysts
#11
Dhara J Kinariwala, Andrew Y Wang, Patrick D Melmer, William P McCullough
Jaundice in children is more often due to hepatic disease than obstruction. Differential considerations for obstructive jaundice in children include choledocholithiasis, choledochal cysts and rare neoplasms. Rhabdomyosarcoma, the most common soft tissue sarcoma in pediatric patients, typically involves the head and neck, genitourinary system and extremities. Embryonal rhabdomyosarcoma of the biliary tree is a rare entity. We present a 3-year-old boy with abrupt onset obstructive jaundice. Although initial imaging suggested a dilated biliary system with fusiform common bile duct, sludge, and possible cholelithiasis, endoscopic retrograde cholangiopancreatogram (ERCP) diagnosed a common bile duct embryonal rhabdomyosarcoma and further imaging showed involvement of the cystic duct...
July 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/29080066/choosing-first-line-biologic-treatment-for-moderate-to-severe-psoriasis-what-does-the-evidence-say
#12
Mina Amin, Daniel J No, Alexander Egeberg, Jashin J Wu
An advanced understanding of the pathogenesis of psoriasis has led to the development of multiple therapeutic options for moderate-to-severe psoriasis. Tumor necrosis factor inhibitors, ustekinumab, interleukin-17 inhibitors, and guselkumab (an interleukin-23 inhibitor recently approved for psoriasis) are commercially available biologic agents for psoriasis. Evidence from clinical trials provides pertinent information regarding the safety and efficacy of biologic agents for psoriasis, which should be integrated into clinical decision making...
October 27, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/29062832/mucinous-cystadenoma-a-rare-hepatic-tumor-in-a-child
#13
Danielle A Ferraguti, Molly McGetrick, Ivan Zendejas, David Hernandez-Gonzalo, Regino Gonzalez-Peralta
Mucinous cystadenomas (MCAs) of the liver (also called hepatic biliary cystadenomas) are rare tumors that comprise about 5% of cystic masses of the liver in adults. These slow-growing lesions most commonly occur in middle-aged individuals, with a female sex predominance. Herein, we present a MCA in a 6-year-old male, one of only very few such cases described in the pediatric literature to date. Although MCAs are generally considered benign lesions, malignant transformation rarely occurs. The recurrence rate is high when partial cyst excision is performed...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29045974/-echocardiographic-diagnosis-of-infracardiac-total-anomalous-pulmonary-venous-connection
#14
S T Ren, Y Wang, J H Zhou, J Long, C H Wang, D B Li, Y Z Huang
OBJECTIVE: To investigate the clinical usefulness of echocardiography in the diagnosis of infracardiac total anomalous pulmonary venous connection (ITAPVC) in neonates and infants. METHODS: Retrospective analysis on 8 patients with ITAPVC was performed using echocardiography between April 2006 and December 2016. There were 4 boys and 4 girls with a mean age of 79.8 days (ranging from 15 to 195 days). A combined scanning via parasternal, subcostal and apical acoustic windows had been employed to diagnose ITAPVC and to trace the course and site of the anomalous pulmonary venous drainage, and to confirm the direction of the inter-atrial shunt and enlargement of right atrium and right ventricle...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29041934/h-syndrome-5-new-cases-from-the-united-states-with-novel-features-and-responses-to-therapy
#15
Jessica L Bloom, Clara Lin, Lisa Imundo, Stephen Guthery, Shelly Stepenaskie, Csaba Galambos, Amy Lowichik, John F Bohnsack
BACKGROUND: H Syndrome is an autosomal recessive disorder characterized by cutaneous hyperpigmentation, hypertrichosis, and induration with numerous systemic manifestations. The syndrome is caused by mutations in SLC29A3, a gene located on chromosome 10q23, which encodes the human equilibrative transporter 3 (hENT3). Less than 100 patients with H syndrome have been described in the literature, with the majority being of Arab descent, and only a few from North America. CASE PRESENTATION: Here we report five pediatric patients from three medical centers in the United States who were identified to have H syndrome by whole exome sequencing...
October 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29039050/the-protective-effect-of-fish-oil-lipid-emulsions-on-intestinal-failure-associated-liver-disease-in-a-rat-model-of-short-bowel-syndrome
#16
Seiro Machigashira, Tatsuru Kaji, Shun Onishi, Waka Yamada, Keisuke Yano, Koji Yamada, Ryuta Masuya, Takafumi Kawano, Kazuhiko Nakame, Motoi Mukai, Satoshi Ieiri
PURPOSE: Pediatric patients with intestinal failure need long-term parenteral nutrition (PN), but this nutritional support causes liver dysfunction, such as intestinal failure-associated liver disease (IFALD). Several studies have shown that the lipid emulsion produced by soybean oil (SO) is associated with the occurrence of IFALD. In this study, we evaluated the effect of SO and fish oil (FO) lipid emulsion on hepatic steatosis. METHODS: Sprague-Dawley rats underwent jugular vein catheterization and were divided into three groups: sham operation with normal chow (Sham group), 80% small bowel resection (80% SBR) + TPN with SO lipid emulsion (SO group), and 80% SBR + TPN with FO lipid emulsion (FO group)...
October 16, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29036392/update-from-the-advisory-committee-on-immunization-practices
#17
Sean T O'Leary, David W Kimberlin
The Advisory Committee on Immunization Practices (ACIP), a group of medical and public health experts, meets 3 times per year to develop recommendations for vaccine use in the United States. The group has 15 voting members, and each member's term is 4 years. ACIP members and Centers for Disease Control and Prevention (CDC) staff discuss the epidemiology of vaccine-preventable diseases and vaccine research, effectiveness, safety data, and clinical trial results. Representatives from the American Academy of Pediatrics (Carrie L...
September 25, 2017: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29028128/diagnostic-accuracy-of-magnetic-resonance-imaging-hepatic-proton-density-fat-fraction-in-pediatric-nonalcoholic-fatty-liver-disease
#18
Michael S Middleton, Mark L Van Natta, Elhamy R Heba, Adina Alazraki, Andrew T Trout, Prakash Masand, Elizabeth M Brunt, David E Kleiner, Edward Doo, James Tonascia, Joel E Lavine, Wei Shen, Gavin Hamilton, Jeffrey B Schwimmer, Claude B Sirlin
We assessed the performance of magnetic resonance imaging (MRI) proton density fat fraction (PDFF) in children to stratify hepatic steatosis grade before and after treatment in the Cysteamine Bitartrate Delayed-Release for the Treatment of Nonalcoholic Fatty Liver Disease in Children (CyNCh) trial, using centrally-scored histology as reference. Participants had multi-echo 1.5T or 3T MRI on scanners from three manufacturers. Of 169 enrolled children, 110 (65%) and 83 (49%) had MRI and liver biopsy at baseline and at end-of-treatment (EOT; 52-weeks), respectively...
October 13, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29024228/de-novo-hepatocellular-carcinoma-post-multivisceral-transplantation-in-a-child
#19
P Tran, S Zhou, L Wang, M Finegold, L Mascarenas, S Alexopolous, Y Genyk, N Kerkar
De novo hepatocellular carcinoma (HCC) post-transplantation in patients without viral hepatitis is extremely rare, with only three reported adult cases in the English literature. Here, we present a case of de novo HCC that developed in a 7-year-old female, who at 8 months of age received a liver, small bowel, spleen, and pancreas transplantation 6.5 years ago for gastroschisis and total parenteral nutrition (TPN)-related cirrhosis. The post-transplant course was complicated by Epstein-Barr virus (EBV) infection, post-transplant lymphoproliferative disease, and subsequent development of multifocal EBV-associated post-transplant smooth muscle tumors (EBV-PTSMT) in the small bowel 1 year and 10 months after transplantation, respectively...
November 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29022907/p38%C3%AE-mapk-antagonizing-jnk-to-control-the-hepatic-fat-accumulation-in-pediatric-patients-onset-intestinal-failure
#20
Yongtao Xiao, Jun Wang, Weihui Yan, Kejun Zhou, Yi Cao, Wei Cai
The p38α mitogen-activated protein kinase (MAPK) has been related to gluconeogenesis and lipid metabolism. However, the roles and related mechanisms of p38α MAPK in intestinal failure (IF)-associated liver steatosis remained poor understood. Here, our experimental evidence suggested that p38α MAPK significantly suppressed the fat accumulation in livers of IF patients mainly through two mechanisms. On the one hand, p38α MAPK increased hepatic bile acid (BA) synthesis by upregulating the expression of the rate-limiting enzyme cholesterol 7-α-hydroxylase (CYP7A1) and peroxisome proliferator-activated receptor γ coactivator-1α (PGC-1α), which in turn activated the transcription of the CYP7A1...
October 12, 2017: Cell Death & Disease
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