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Hyperbilirubinemia

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https://www.readbyqxmd.com/read/28818034/utility-of-hepatobiliary-scintigraphy-in-diagnosing-or-excluding-biliary-atresia-in-premature-neonates-and-full-term-infants-with-conjugated-hyperbilirubinemia-who-received-parenteral-nutrition
#1
Karine Barseghyan, Rangasamy Ramanathan, Thomas Chavez, Susan Harlan, Chuan-Hao Lin, Tania Mitsinikos, Claire McLean
PURPOSE: Hepatobiliary scintigraphy (HBS) is used to aid in the diagnosis of Biliary Atresia in full-term infants with conjugated hyperbilirubinemia. There is little information on the utility of the HBS in premature infants with conjugated hyperbilirubinemia and infants with parenteral nutrition associated cholestasis (PNAC). The purpose of this study is to assess the utility of HBS in differentiating Biliary Atresia and PNAC in premature neonates and full-term infants who received parenteral nutrition (PN)...
August 17, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28815739/kernicterus-in-a-boy-with-ornithine-transcarbamylase-deficiency-a-case-report
#2
Eduardo López-Corella, Isabel Ibarra-González, Cynthia Fernández-Lainez, Miguel Á Rodríguez-Weber, Sara Guillén-Lopez, Leticia Belmont-Martínez, David Agüero-Linares, Marcela Vela-Amieva
Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle defect associated with severe and usually fatal hyperammonemia. This study describes a patient with early onset lethal OTCD due to a known pathogenic variant (c.298+1G>A), as well as the novel autopsy finding of kernicterus with relatively low blood concentration of unconjugated bilirubin (UCB) (11.55 mg/dL). The patient was a full-term male with a family history of two previous male siblings who died as newborns after acute neurologic deterioration...
August 16, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28814249/the-neurological-sequelae-of-neonatal-hyperbilirubinemia-definitions-diagnosis-and-treatment-of-the-kernicterus-spectrum-disorders-ksds
#3
Steven Shapiro, Jean Baptiste Le Pichon, Sean M Riordan, Jon Watchkoe
Despite its lengthy history the study of benign jaundice, hyperbilirubinemia and kernicterus suffers from a lack of clarity and consistency in the key terms used to describe both the clinical and pathophysiological nature of these conditions. This has led to multiple terms being used to describe similar conditions (i.e. kernicterus and chronic bilirubin encephalopathy) or the same term being used to describe different concepts (i.e. BIND as a descriptor of the signs of bilirubin neurotoxicity, mild kernicterus, or as a scale to assess bilirubin toxicity)...
August 14, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28811238/i-index-is-not-an-accurate-indicator-of-icteria-in-conjugated-hyperbilirubinemia
#4
Nora Nikolac Gabaj, Marijana Miler, Roman Mihic
INTRODUCTION: Serum indices have become a standard in assessing degree of endogenous interferences in serum and plasma samples. The aim of this study was to evaluate accuracy of I index in comparison with total bilirubin concentration in icteric samples with ranging amount of conjugated bilirubin. MATERIALS AND METHODS: This study retrospectively analyzed data from laboratory information system. Total, conjugated bilirubin and I index are measured on Abbott Architect c8000 (N=900)...
August 12, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28790862/hyperbilirubinemia-in-atazanavir-treated-hiv-infected-patients-the-impact-of-the-ugt1a1-28-allele
#5
REVIEW
Periklis Panagopoulos, Efstathios Maltezos, Angelos Hatzakis, Dimitrios Paraskevis
Combination antiretroviral treatment (cART) has significantly improved the life expectancy of people living with HIV. The life-long nature of cART increases the risk of side effects, which in some cases may have been caused by specific genetic characteristics. Patients treated with atazanavir (ATV) boosted with ritonavir (rit), which is a protease inhibitor used for the treatment of HIV, present with elevated bilirubin levels, at high proportions. ATV/rit-related hyperbilirubinemia has been previously associated with genetic characteristics in uridine diphosphate glucuronosyltransferase (UGT) enzyme...
2017: Pharmacogenomics and Personalized Medicine
https://www.readbyqxmd.com/read/28782473/importance-of-hemolysis-and-its-clinical-detection-in-neonates-with-hyperbilirubinemia
#6
Ronald J Wong, Vinod K Bhutani, David K Stevenson
Neonatal hyperbilirubinemia is generally a benign transitional phenomenon, and may even be a protective antioxidant mechanism for human neonates who may be vulnerable to the oxidative stress (compared to life in utero) encountered during birth an extra-uterine oxidative environment (1, 2). The pathophysiologic impact of neonatal unconjugated hyperbilirubinemia has been well reviewed in this supplement (see Jon fill-in). The severity of hyperbilirubinemia varies due to the magnitude of an imbalance between two major contributing processes: (i) an increase bilirubin production rate due to an increase in heme turnover (two to three times that of an adult) due to the degradation of fetal red blood cells (RBCs), which have a shortened lifespan, after birth (3, 4); and/or (ii) a diminished ability of the immature newborn liver to conjugate bilirubin and therefore excrete bilirubin in bile...
August 7, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28782398/perinatal-outcomes-in-uncomplicated-late-preterm-pregnancies-with-borderline-oligohydramnios
#7
Erdem Sahin, Yusuf Madendag, Ahter Tanay Tayyar, Mefkure Eraslan Sahin, Ilknur Col Madendag, Gokhan Acmaz, Deniz Unsal, Vesile Senol
PURPOSE: The purpose of this study is to determine the adverse perinatal outcomes in uncomplicated late preterm pregnancies with borderline oligohydramnios. METHODS: A total of 430 pregnant women with an uncomplicated singleton pregnancy at a gestational age of 34 + 0-36 + 6 weeks were included. Borderline oligohydramnios was defined as an amniotic fluid index (AFI) of 5.1-8 cm, which was measured using the four-quadrant technique. Adverse perinatal outcomes were compared between the borderline and normal AFI groups...
August 16, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28782153/n-acetylcysteine-and-prednisolone-treatment-improved-serum-biochemistries-in-suspected-flupirtine-cases-of-severe-idiosyncratic-liver-injury
#8
Jürgen Borlak, Florian van Bömmel, Thomas Berg
BACKGROUND & AIMS: The analgesic flupirtine has been linked to cases of severe idiosyncratic drug-induced liver injury (sFILI). We therefore examined whether N-acetylcysteine (NAC) and glucocorticoid therapy is effective in the management of sFILI. METHODS: In a retrospective cohort study efficacy of NAC-infusion and oral prednisolone treatments on liver-function-tests (LFTs) and clinical outcome of 21 sFILI cases was evaluated by comparing it to an external cohort of 30 sFILI cases not receiving the antidote...
August 7, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28762541/real-world-effectiveness-and-safety-of-paritaprevir-ritonavir-ombitasvir-and-dasabuvir-with-or-without-ribavirin-for-patients-with-chronic-hepatitis-c-virus-genotype-1b-infection-in-taiwan
#9
Chen-Hua Liu, Chun-Jen Liu, Tung-Hung Su, Hung-Chih Yang, Chun-Ming Hong, Tai-Chung Tseng, Pei-Jer Chen, Ding-Shinn Chen, Jia-Horng Kao
BACKGROUND AND AIM: The real-world effectiveness and safety of paritaprevir/ritonavir, ombitasvir, and dasabuvir (PrOD) remain limited for East Asian hepatitis C virus genotype 1b (HCV-1b) patients. We aimed to evaluate the anti-viral responses of PrOD-based regimens for HCV-1b patients in Taiwan. METHODS: We performed a retrospective analysis of 103 HCV-1b patients receiving PrOD with or without ribavirin (RBV) for 12 weeks. Data were analyzed to assess the on-treatment and off-therapy HCV viral load, and on-treatment adverse events (AEs)...
August 1, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28760474/impact-of-postoperative-liver-dysfunction-on-survival-after-left-ventricular-assist-device-implantation
#10
Kaustav Majumder, John R Spratt, Christopher T Holley, Samit S Roy, Rebecca J Cogswell, Kenneth Liao, Ranjit John
BACKGROUND: Liver dysfunction in left ventricular assist device (LVAD) recipients is common both before and after implantation. Postoperative liver dysfunction (PLD) develops in some LVAD recipients without preoperative liver dysfunction. The aim of this study was to assess clinical outcomes in such patients. METHODS: Records of all patients undergoing implantation of a HeartMate II (HM II, St. Jude Medical, Inc, Minneapolis, MN) LVAD at a single center at the University of Minnesota from January 2005 through June 2014 were analyzed...
July 28, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28759390/cholestasis-and-hepatic-iron-deposition-in-an-infant-with-complex-glycerol-kinase-deficiency
#11
Diana Montoya-Williams, Meredith Mowitz
We present a 6-week-old male infant with persistent hyperbilirubinemia, hypertriglyceridemia, elevated creatine kinase levels, and transaminitis since the second week of life. When he developed hyperkalemia, clinical suspicion was raised for adrenal insufficiency despite hemodynamic stability. A full endocrine workup revealed nearly absent adrenocorticotropic hormone. Coupled with his persistent hypertriglyceridemia (peak of 811 mg/dL) and elevated creatine kinase levels (>20 000 U/L), his corticotropin level lead to a clinical diagnosis of complex glycerol kinase deficiency (GKD), also known as Xp21 deletion syndrome...
July 2017: Pediatrics
https://www.readbyqxmd.com/read/28758317/eccrine-chromhidrosis-in-an-adolescent-with-sickle-cell-disease
#12
Jong G Park, Neil S Prose, Reed Garza
Eccrine chromhidrosis can occur secondary to hyperbilirubinemia. We report an adolescent with sickle cell disease who presented with eccrine chromhidrosis on his palmar and plantar surfaces. He had extremely high levels of conjugated bilirubin but no fever. This is the youngest known reported patient with eccrine chromhidrosis and the first in a patient with sickle cell disease.
July 30, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28754279/-g6pd-deficiency-in-females-with-neonatal-revelation-report-of-four-cases
#13
A Renault, D Mitanchez, A Cortey
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human erythrocyte enzyme defect, estimated to affect approximately 4 million people worldwide. It is associated with severe neonatal hyperbilirubinemia, which may lead to bilirubin encephalopathy and kernicterus, and with hemolytic crisis. G6PD deficiency is an X-linked enzymopathy affecting hemizygous males, homozygous females, and also a subset of heterozygous females via chromosome X inactivation. We report four cases of female newborns with neonatal hyperbilirubinemia related to a G6PD deficiency and followed by the Centre national de référence en hémobiologie périnatale (CNRHP) from November 2013 to July 2014...
July 25, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28751579/promoterless-gene-targeting-without-nucleases-rescues-lethality-of-a-crigler-najjar-syndrome-mouse-model
#14
Fabiola Porro, Giulia Bortolussi, Adi Barzel, Alessia De Caneva, Alessandra Iaconcig, Simone Vodret, Lorena Zentilin, Mark A Kay, Andrés F Muro
Crigler-Najjar syndrome type I (CNSI) is a rare monogenic disease characterized by severe neonatal unconjugated hyperbilirubinemia with a lifelong risk of neurological damage and death. Liver transplantation is the only curative option, which has several limitations and risks. We applied an in vivo gene targeting approach based on the insertion, without the use of nucleases, of a promoterless therapeutic cDNA into the albumin locus of a mouse model reproducing all major features of CNSI Neonatal transduction with the donor vector resulted in the complete rescue from neonatal lethality, with a therapeutic reduction in plasma bilirubin lasting for at least 12 months, the latest time point analyzed...
July 27, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/28748879/early-clinical-manifestations-and-laboratory-findings-before-and-after-treatment-of-cytomegalovirus-infection-in-kidney-transplant-patients
#15
Wasim Ahmed Siddiqui, Issa Al Salmi, Amitabh Jha, Abbas Pakkyara, Mohammad Yasir, Faissal A M Shaheen
Cytomegalovirus (CMV) is one of the most frequently encountered opportunistic viral pathogens in kidney transplant recipients. In this study, we retrospectively reviewed all living related and unrelated kidney transplant recipients on regular follow-up from January 2006 to June 2015, who were suspected to have CMV clinically and confirmed by DNA polymerase chain reaction (PCR). CMV PCR was detected in 102 kidney transplant recipients. The median time of detection after kidney transplant was 21 months, ranging from 15 days to 84 months...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28739260/uses-and-misuses-of-albumin-during-resuscitation-and-in-the-neonatal-intensive-care-unit
#16
REVIEW
Wissam Shalish, Francois Olivier, Hany Aly, Guilherme Sant'Anna
Albumin is one of the most abundant proteins in plasma and serves many vital functions. Neonatal concentrations vary greatly with gestational and postnatal age. In critically ill neonates, hypoalbuminemia occurs due to decreased synthesis, increased losses or redistribution of albumin into the extravascular space, and has been associated with increased morbidities and mortality. For that reason, infusion of exogenous albumin as a volume expander has been proposed for various clinical settings including hypotension, delivery room resuscitation, sepsis and postoperative fluid management...
July 21, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28730953/an-update-on-predicting-severe-hyperbilirubinemia-and-bilirubin-neurotoxicity-risks-in-neonates
#17
Tom Newman, M Jeffrey Maisels
Extreme hyperbilirubinemia and kernicterus, though rare, continue to occur despite the adoption of universal screening. Unless they are known to have glucose-6-phosphate dehydrogenase deficiency, infants who currently develop kernicterus in high resource countries are often otherwise healthy newborns discharged from the well-baby nursery. In this review, we highlight risk factors that increase the risk of a newborn ≥35 weeks gestational age developing severe hyperbilirubinemia, as well as the risk factors that increase the hyperbilirubinemic infant's risk of kernicterus...
July 20, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28722598/risk-factors-for-severe-infection-hospitalization-and-prolonged-antimicrobial-therapy-in-patients-with-babesiosis
#18
Neeharik Mareedu, Anna M Schotthoefer, Jason Tompkins, Matthew C Hall, Thomas R Fritsche, Holly M Frost
Babesiosis is an emerging tick-borne disease transmitted by the hard tick Ixodes scapularis, which also transmits Lyme disease. Better gradation of prognostic indicators are needed to determine which patients may develop serious complications requiring hospitalization, and to provide early guidance on appropriate therapy. In this study, we evaluated 128 patients with smear or real time polymerase chain reaction-confirmed Babesia microti infections over a period of 16 years. Patients with asplenia or immunocompromising conditions were more likely to have severe infection (P < 0...
July 10, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28721815/conjunctival-icterus-an-important-but-neglected-sign-of-clinically-relevant-hyperbilirubinemia-in-jaundiced-neonates
#19
Jon Freeman Watchko, Abeer Azzuqa
BACKGROUND: Conjunctival icterus is a largely neglected physical sign that may be helpful in identifying neonates with clinically relevant hyperbilirubinemia by practitioners in the hospital and outpatient clinic or parents at home. OBJECTIVE: A recent NICU based study reported that conjunctival icterus is often a sign of significant (TSB ≥ 17 mg/dl) hyperbilirubinemia and TSB levels ≥ 76th-95th percentile on the Bhutani nomogram. In contrast, others report that conjunctival icterus, although frequently present at high TSB levels, may also be detected at lower TSB concentrations; suggesting instead that its absence may help to rule out significant hyperbilirubinemia...
July 18, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28721814/refractory-causes-of-kernicterus-in-developed-countries-can-we-eradicate-g6pd-deficiency-triggered-and-low-bilirubin-kernicterus
#20
Jon Freeman Watchko
BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency triggered and low-bilirubin kernicterus persist despite current prevention strategies. OBJECTIVE: Efforts to eradicate bilirubin induced brain injury in these two conditions will require novel approaches to riskassessment and hyperbilirubinemia evaluation. METHOD: In the case of G6PD deficency, a heightenedawareness of populations at risk on who expanded kernicterus preventio strategies can befocused including intensified parental engagement, education and counselling on neonataljaundice...
July 18, 2017: Current Pediatric Reviews
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