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Pediatric jaundice

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https://www.readbyqxmd.com/read/29333010/sinusoidal-obstruction-syndrome-during-treatment-for-wilms-tumor-a-life-threatening-complication
#1
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
#2
Deepak Sharma, Nazanin Farahbakhsh
A term male infant was admitted at 48 hours 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 2, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29232532/reconsideration-of-laparoscopic-kasai-operation-for-biliary-atresia
#3
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...
December 12, 2017: 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
#4
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
#5
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
#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/29089679/embryonal-rhabdomyosarcoma-of-the-biliary-tree-a-rare-cause-of-obstructive-jaundice-in-children-which-can-mimic-choledochal-cysts
#7
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
#8
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
#9
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
#10
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
#11
Adel Abd Elhaleim 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 an 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...
October 3, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#12
Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Caroline Caldeira Hosken, Thaís Costa Nascentes Queiroz
BACKGROUND: Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE: To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS: This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18...
October 2, 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28960043/the-clinical-differences-between-urgent-visits-and-non-urgent-visits-in-emergency-department-during-the-neonatal-period
#13
Hyung Jun Yang, Woochan Jeon, Hee Jung Yang, Jae Ryoung Kwak, Hyo Yeon Seo, Ji Sook Lee
As neonates are brought to the emergency department (ED) for various complaints, it is challenging for emergency physicians to clinically determine the urgency of the visit. We sought to explore clinical characteristics associated with urgent visits to the ED. We conducted a retrospective study by reviewing medical records of neonatal visits to a tertiary pediatric regional emergency center for 5 years. Cases of patients who were discharged after checking only chest or abdominal X-ray or discharged without workup, were classified as non-urgent visits...
November 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28947328/outcomes-of-biliary-atresia-in-the-nordic-countries-a-multicenter-study-of-158-patients-during-2005-2016
#14
Mikko P Pakarinen, Lars Søndergaard Johansen, Jan F Svensson, Kristin Bjørnland, Vladimir Gatzinsky, Pernilla Stenström, Antti Koivusalo, Nina Kvist, Markus Almström, Ragnhild Emblem, Sigurdur Björnsson, Torbjörn Backman, Runar Almaas, Hannu Jalanko, Björn Fischler, Jørgen Thorup
BACKGROUND/PURPOSE: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. METHODS: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28883277/siblings-with-congenital-thrombotic-thrombocytopenic-purpura
#15
Yasutomo Funakoshi, Masahiko Okada, Masanori Matsumoto, Koichi Kokame, Hiroyuki Moriuchi
Congenital thrombotic thrombocytopenic purpura (TTP) is a rare hereditary deficiency of ADAMTS13 (von Willebrand factor-cleaving protease) characterized by thrombocytopenia and microangiopathic hemolytic anemia. The spectrum of the clinical phenotype is wide, ranging from asymptomatic episodes of thrombocytopenia to life-threatening multiorgan failure. Reportedly, some patients develop isolated thrombocytopenia during childhood. We herein report sibling cases of congenital TTP. An 11-year-old boy with thrombocytopenia accompanied by influenza virus infection was referred to our hospital...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28731912/assessing-infant-and-maternal-readiness-for-newborn-discharge
#16
Ling Jing, Casidhe-Nicole Bethancourt, Thomas McDonagh
PURPOSE OF REVIEW: The review highlights the shift from prescribed length of stay (LOS) to mother-infant dyad readiness as the basis for making discharge decisions for healthy term newborns. We describe the components of readiness that should be considered in making the decision, focusing on infant clinical readiness, and maternal and familial readiness. RECENT FINDINGS: Although the Newborns' and Mothers' Health Protection Act of 1996 aimed to protect infants and mothers by establishing a minimum LOS, the American Academy of Pediatrics 2015 policy on newborn discharge acknowledges the shift from LOS-based to readiness-based discharge decision-making...
October 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28708635/the-management-of-pediatric-hearing-loss-caused-by-auditory-neuropathy-spectrum-disorder
#17
Nguyen S Pham
PURPOSE OF REVIEW: Auditory neuropathy spectrum disorder (ANSD) is a condition in which auditory testing reveals normal otoacoustic emissions, but auditory brainstem testing is abnormal or absent and speech discrimination is poor. This constellation of findings ostensibly suggests that the cochlea is healthy and an abnormality of conduction or processing of sound occurs along the nerve fibers. As more is learned about this condition, it is becoming clear that ANSD describes heterogeneous, distinct clinical entities that must be taken into account when devising treatment modalities...
July 13, 2017: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/28699603/infants-with-extrahepatic-biliary-atresia-effect-of-follow-up-on-the-survival-rate-at-ege-university-medical-school-transplantation-center
#18
Miray Karakoyun, Maşallah Baran, Caner Turan, Murat Kılıç, Orkan Ergun, Sema Aydoğdu
BACKGROUND/AIMS: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. MATERIALS AND METHODS: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively...
July 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28690638/fat-soluble-vitamin-deficiency-in-pediatric-patients-with-biliary-atresia
#19
Rui Dong, Song Sun, Xiao-Zhou Liu, Zhen Shen, Gong Chen, Shan Zheng
OBJECTIVE: To analyze the levels of fat-soluble vitamins (FSVs) in pediatric patients with biliary atresia (BA) before and after the Kasai procedure. METHODS: Pediatric patients with obstructive jaundice were enrolled in this study. The FSV levels and liver function before, 2 weeks after, and 1, 3, and 6 months after the Kasai procedure were measured. RESULTS: FSV deficiency was more obvious in patients with BA than in patients with other cholestatic liver diseases, especially vitamin D deficiency...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28648013/-retrospective-analysis-of-anesthetic-and-perioperative-management-in-patients-of-acute-fatty-liver-of-pregnancy
#20
Y Y Qu, H Zeng, X Y Guo, M Li
Objective: To retrospectively analyze the clinical characteristics of patients with acute fatty liver of pregnancy (AFLP), and to discuss perioperative and anesthetic management. Methods: A retrospective review was conducted on the records of pregnant patients with a diagnosis of acute fatty liver of pregnancy in Peking University Third Hospital from January 2007 to December 2015. 12 cases were identified. The clinical features, preoperative laboratory findings, types of delivery, anesthetic techniques for cesarean section, and the outcomes of parturients and fetus were collected and analyzed...
June 27, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
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