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Pediatric jaundice

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https://www.readbyqxmd.com/read/29672616/adherence-to-hyperbilirubinemia-guidelines-by-midwives-general-practitioners-and-pediatricians-in-indonesia
#1
Mahendra T A Sampurna, Kinanti A Ratnasari, Risa Etika, Christian V Hulzebos, Peter H Dijk, Arend F Bos, Pieter J J Sauer
Severe hyperbilirubinemia, which may result in kernicterus, is seen more frequently in low and middle-income countries, such as Indonesia, than in high-income countries. In Indonesia midwives, general practitioners (GPs), and pediatricians are involved in the care of jaundiced newborn infants. It is unknown whether the high incidence of severe hyperbilirubinemia in this country is related to a lack of awareness of existing hyperbilirubinemia guidelines issued by, for example, the World Health Organization, the American Academy of Pediatrics, or the Indonesian Health Ministry, or to a lack of adherence to such guidelines...
2018: PloS One
https://www.readbyqxmd.com/read/29668548/clinical-course-of-homozygous-hemoglobin-constant-spring-in-pediatric-patients
#2
Patcharee Komvilaisak, Arunee Jetsrisuparb, Goonnapa Fucharoen, Ratana Komwilaisak, Junya Jirapradittha, Pakaphan Kiatchoosakun
BACKGROUND: Hemoglobin (Hb) Constant Spring is an alpha-globin gene variant due to a mutation of the stop codon resulting in the elongation of the encoded polypeptide from 141 to 172 amino acid residues. Patients with homozygous Hb Constant Spring are usually mildly anemic. METHODS: We retrospectively describe clinical manifestations, diagnosis, laboratory investigations, treatment, and associated findings in pediatric patients with homozygous Hb Constant Spring followed-up at Srinagarind Hospital...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29658451/-clinical-and-genetic-analysis-of-a-pediatric-patient-with-sodium-taurocholate-cotransporting-polypeptide-deficiency
#3
Hua Li, Jian-Wu Qiu, Gui-Zhi Lin, Mei Deng, Wei-Xia Lin, Ying Cheng, Yuan-Zong Song
Sodium taurocholate cotransporting polypeptide (NTCP) deficiency is an inborn error of bile acid metabolism caused by mutations of SLC10A1 gene. This paper reports the clinical and genetic features of a patient with this disease. A 3.3-month-old male infant was referred to the hospital with the complaint of jaundiced skin and sclera over 3 months. Physical examination revealed moderate jaundice of the skin and sclera. The liver was palpable 3.5 cm below the right subcostal margin with a medium texture. Serum biochemistry analysis revealed markedly elevated bilirubin (predominantly direct bilirubin) and total bile acids (TBA), as well as decreased 25-OH-VitD level...
April 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29601463/conjugated-hyperbilirubinemia-in-the-neonate-and-young-infant
#4
Anna K Weiss, Parag V Vora
Cholestatic jaundice in the first few weeks of life may herald potentially life-threatening pathology. It is therefore incumbent upon the pediatric practitioner to have a high index of suspicion for severe disease when investigating jaundice in a young infant. This article outlines the epidemiology, pathophysiology, differential diagnosis, and diagnostic workup for both the most common and the most severe causes of cholestasis in the neonatal period.
April 2018: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29499989/clinical-pathologic-and-genetic-features-of-neonatal-dubin-johnson-syndrome-a-multicenter-study-in-japan
#5
Takao Togawa, Tatsuki Mizuochi, Tokio Sugiura, Hironori Kusano, Ken Tanikawa, Takato Sasaki, Fumio Ichinose, Seiichi Kagimoto, Takahisa Tainaka, Hiroo Uchida, Shinji Saitoh
OBJECTIVE: To clarify the clinical, pathologic, and genetic features of neonatal Dubin-Johnson syndrome. STUDY DESIGN: Ten patients with neonatal Dubin-Johnson syndrome were recruited from 6 pediatric centers in Japan between September 2013 and October 2016. Clinical and laboratory course, macroscopic and microscopic liver findings, and molecular genetic findings concerning ATP-binding cassette subfamily C member 2 (ABCC2) were retrospectively and prospectively examined...
May 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29491755/significant-hyperbilirubinemia-and-acute-hepatocellular-jaundice-in-a-pediatric-patient-receiving-deferasirox-a-case-report
#6
Elizabeth A Feldman, Christopher D Miller, Sarabeth Wojnowicz, Robert Seabury
Despite a boxed warning, postmarketing reports of deferasirox-associated hepatic injury in patients with chronic transfusions are not well described. Hepatic impairment, including failure, has been reported to occur more frequently in patients older than 55 years and in those with significant comorbidities, including liver cirrhosis and multiorgan failure. In this case report, we describe significant hyperbilirubinemia and acute hepatocellular jaundice related to deferasirox in a 7-year-old female being treated for iron overload secondary to chronic transfusions...
January 2018: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29482208/efficacy-of-subthreshold-newborn-phototherapy-during-the-birth-hospitalization-in-preventing-readmission-for-phototherapy
#7
Andrea C Wickremasinghe, Michael W Kuzniewicz, Charles E McCulloch, Thomas B Newman
Importance: Treatment of jaundiced newborns with subthreshold phototherapy (phototherapy given to newborns with bilirubin levels below those recommended in American Academy of Pediatrics [AAP] guidelines) is common. However, the use of subthreshold phototherapy may have risks and increase costs, and, to date, it has not been systematically studied in newborns. Objectives: To estimate the efficacy of subthreshold phototherapy for newborns with total serum bilirubin (TSB) levels from 0...
April 1, 2018: JAMA Pediatrics
https://www.readbyqxmd.com/read/29471012/paediatric-fatty-liver-disease-pefld-all-is-not-nafld-pathophysiological-insights-and-approach-to-management
#8
REVIEW
Robert Hegarty, Maesha Deheragoda, Emer Fitzpatrick, Anil Dhawan
The recognition of a pattern of steatotic liver injury where histology mimicked alcoholic liver disease, but alcohol consumption was denied, led to the identification of non-alcoholic fatty liver disease (NAFLD). Non-alcoholic fatty liver disease has since become the most common chronic liver disease in adults owing to the global epidemic of obesity. However, in paediatrics, the term NAFLD seems incongruous: alcohol consumption is largely not a factor and inherited metabolic disorders can mimic or co-exist with a diagnosis of NAFLD...
February 20, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29364802/-infectious-mononucleosis-and-cholestatic-hepatitis-a-rare-association
#9
Catarina Salgado, Ana Margarida Garcia, Catarina Rúbio, Florbela Cunha
Infectious mononucleosis is one of the major clinical manifestations of Epstein-Barr virus infection. In this syndrome, elevation of liver transaminase levels is common but cholestasis is rare, with few cases described in the literature. We present the case of a 14-year-old female adolescent, admitted to the Emergency Room with fever, odynophagia and cervical adenomegaly. She was treated with amoxicillin and two days later he presented with jaundice. The analytical evaluation was compatible with cholestatic hepatitis and abdominal ultrasound revealed hepatosplenomegaly without dilatation of the bile ducts...
December 29, 2017: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/29333010/sinusoidal-obstruction-syndrome-during-treatment-for-wilms-tumor-a-life-threatening-complication
#10
Sidharth Totadri, Amita Trehan, Deepak Bansal, Richa Jain
Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Settings and Design: Retrospective file review conducted in a Pediatric Hematology-Oncology unit...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29295661/neonatal-hyperbilirubinemia-secondary-to-combined-anti-e-and-anti-c-isoimmunisation-a-literature-review
#11
Deepak Sharma, Nazanin Farahbakhsh
A term male infant was admitted at 48 h of postnatal life to the neonatal unit for jaundice. The investigation showed total serum bilirubin (TSB) of 17.1 mg/dl, haemoglobin of 11 g/dl, reticulocyte count of 9.5% and peripheral smear was suggestive of macrocytic, normochromic red blood cell (RBC) with target cells and multiple spherocytes with occasional nucleated RBC. The infant's blood group was B positive. Direct antiglobulin test was strongly positive by gel method (3+). Mother's blood group was B positive and indirect antiglobulin test was positive when tested postnatally...
January 8, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29232532/reconsideration-of-laparoscopic-kasai-operation-for-biliary-atresia
#12
Sheng-Yang Huang, Chou-Ming Yeh, Hou-Chuan Chen, Chia-Man Chou
INTRODUCTION: Minimal invasive surgery for all kinds of surgical diseases had been practiced for years. The laparoscopic Kasai operation is one of the most challenging procedures and remains controversial for treating biliary atresia (BA). PURPOSE: This work presented the initial experience of our Institute and compared the outcomes of open and laparoscopic Kasai operations for BA. MATERIALS AND METHODS: Patients 18 years old and younger, and were operated in our Institute for BA from January 2011 to August 2017, were included in this study...
February 2018: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/29192742/biliary-tract-rhabdomyosarcoma-a-report-from-the-soft-tissue-sarcoma-committee-of-the-associazione-italiana-ematologia-oncologia-pediatrica
#13
Katia Perruccio, Valerio Cecinati, Angela Scagnellato, Massimo Provenzi, Giuseppe Maria Milano, Eleonora Basso, Carla Manzitti, Giovanni Cecchetto, Rita Alaggio, Martina Di Martino, Amalia Schiavetti, Fraia Melchionda, Maria Carmen Affinita, Stefano Chiaravalli, Lucia Miglionico, Rita Balter, Angela Tamburini, Gianni Bisogno, Andrea Ferrari
INTRODUCTION: Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor frequent in children. Biliary duct localization is extremely rare, but it is the most common cause of malignant obstructive jaundice in pediatric patients. METHODS: This report describes a series of 10 patients under 18 years of age with biliary tract rhabdomyosarcoma who were enrolled, from 1979 to 2004, in 3 consecutive Italian pediatric cooperative protocols that had been drawn up by the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP)...
December 1, 2017: Tumori
https://www.readbyqxmd.com/read/29184811/dermatologic-manifestations-of-endocrine-disorders
#14
REVIEW
Michael Lause, Alisha Kamboj, Esteban Fernandez Faith
The skin serves as a window for clinicians to understand, diagnose, and monitor endocrine disease. Dermatologic manifestations of endocrinopathies contribute significantly to an individual's health and quality of life. In this review, we outline various disorders of the hypothalamic-pituitary axis, thyroid gland, pancreas, adrenal gland, and androgen axis as well as hereditary endocrine syndromes. In acromegaly, glycosaminoglycan deposition contributes to a thickening of skin and soft tissue, which manifests as coarsening and enlargement of facial and acral structures...
October 2017: Translational pediatrics
https://www.readbyqxmd.com/read/29115027/clinical-features-of-pediatric-autoimmune-hepatitis-in-japan-a-nationwide-survey
#15
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 that 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 (range, 3 months-15 years), and the male-to-female ratio was 2:3...
March 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
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
#16
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/29030698/biliary-atresia-and-liver-transplantation-results-and-thoughts-for-primary-liver-transplantation-in-select-patients
#17
REVIEW
Riccardo Superina
Biliary atresia (BA) is one of the most common indications for liver transplantation in children. Despite advances in biliary atresia surgical techniques, most children will ultimately require liver transplantation. Possible pre-operative predictors of outcome after the Kasai operation are: 1. Age at operation 2. Presence of the biliary atresia splenic malformation syndrome (BASM) 3. Center specific factors 4. Liver histology and 5. Anatomic pattern of bile ducts found at surgery.Age at surgery is considered a strong predictor of success after portoenterostomy...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29022095/laparoscopic-portoenterostomy-for-biliary-atresia-single-center-experience-and-review-of-literatures
#18
Joel Cazares, Hiroyuki Koga, Hiroshi Murakami, Hiroki Nakamura, Geoffrey Lane, Atsuyuki Yamataka
AIM: The aim of this report was to present the laparoscopic portoenterostomy (LapPE) procedure developed by the Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine (JLapPE). We also attempted to obtain an understanding of the current status of laparoscopic portoenterostomy in the world as reported in the English literature to compare with our experience. METHODS: There were 22 BA patients who had JLapPE between 2009 and 2016...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983658/newborn-screening-for-biliary-atresia-in-the-united-states
#19
Cat Goodhue, Michael Fenlon, Kasper S Wang
Despite advances in our understanding of the pathogenesis of biliary atresia (BA), BA remains the most common cause of end-stage liver disease in children and the leading indication for pediatric liver transplantation. Age at time of Kasai portoenterostomy (KPE), performed to provide bile drainage, strongly correlates with transplant-free survival, mostly due to progression of intrahepatic fibrosis to cirrhosis. Unfortunately, challenges remain in recognizing that a jaundiced infant may have BA. To better diagnose infants with BA at an earlier age, population-based screening programs in countries such as Taiwan, Japan, and China have utilized stool color cards...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28982343/study-of-glucose-6-phosphate-dehydrogenase-deficiency-5-years-retrospective-egyptian-study
#20
Adel A Hagag, Ibrahim M Badraia, Mohamed S Elfarargy, Mohamed M Abd Elmageed, Ehab A Abo-Ali
BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency worldwide that causes a spectrum of diseases including neonatal hyperbilirubinemia, acute and chronic hemolysis after exposure to oxidative stress. AIM OF THE WORK: This five years retrospective study was carried out to study the demographic, clinical and laboratory data of 1000 patients with G6PD deficiency anemia registered in Hematology Unit, Pediatric Department, Tanta University Hospital...
February 13, 2018: Endocrine, Metabolic & Immune Disorders Drug Targets
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