Hyperbilirubinemia

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...

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...

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...

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...

BACKGROUND: Even relatively low serum bilirubin concentrations can cause neurodevelopmental impairment in extremely low birth weight (EBWL) infants, while sequelae from hyperbilirubinemia in late preterm and term infants are rare and occur only at very high serum bilirubin lev-els. Phototherapy is the current treatment of choice. OBJECTIVE: To present an update on the most important issues involved in phototherapy for jaundiced infants. RESULTS: Light absorption by bilirubin in the skin transforms the native Z,Z-bilirubin to conformational photoisomers Z,E-bilirubin and E,Z-bilirubin and structural photoisomers E,Z-lumirubin and E,E-lumirubin...

BACKGROUND: Few studies have investigated patients with severe influenza who receive intravenous peramivir for salvage therapy. METHODS: We retrospectively analyzed data from 71 patients with severe influenza who received intravenous peramivir therapy in the intensive care units of three medical centers between 2012 and 2016. All patients received oseltamivir or zanamivir before the administration of peramivir. RESULTS: A total of 44 men and 27 women with a median age of 55 years were enrolled...

Therapeutic plasma exchange (TPE) in neonates and small infants is a treatment method at the forefront that may become a potentially life-saving procedure in a wide array of severe conditions. Indications for TPE in the pediatric population have been mainly derived from adult literature, with neonatal hyperbilirubinemia being the most notable exception. The only alternative to TPE in small pediatric patients is manual blood exchange transfusion, which, however, bears an unacceptably high risk of severe complications...

Bilirubin is the end product of heme catabolism, which is produced primarily from the breakdown of erythrocyte hemoglobin in the reticuloendothelial system. Hyperbilirubinemia is induced not only by increased bilirubin synthesis, but can also be caused by decreased bilirubin clearance. There are several disorders, which can contribute to hyperbilirubinemia, including Dubin‑Johnson syndrome (DJS). DJS is a rare autosomal recessive disorder, which is characterized by predominantly conjugated hyperbilirubinemia without progression to end‑stage liver disease...

BACKGROUND: Fludarabine and clofarabine are purine nucleoside analogues with established clinical activity in patients with acute myeloid leukemia (AML). METHODS: Herein, the authors evaluated the efficacy and safety of idarubicin and cytarabine with either clofarabine (CIA) or fludarabine (FIA) in adults with newly diagnosed AML. Adults with newly diagnosed AML who were deemed suitable for intensive chemotherapy were randomized using a Bayesian adaptive design to receive CIA (106 patients) or FIA (76 patients)...

OBJECTIVES: To evaluate normative sonographic measurements of the inferior vena cava (IVC), aorta (Ao), and IVC/Ao ratio in the first 2 days of life in term neonates. METHODS: We prospectively observed 200 term (more than 36 and 6/7 weeks of gestation), single, healthy neonates born in a city hospital. The exclusion criteria were congenital abnormalities, an Apgar score of less than 8, and hyperbilirubinemia requiring phototherapy. Maximum IVC (distal to the hepatic-IVC junction) and Ao (above the superior mesenteric artery) diameters were measured in the first 2 days of life in the longitudinal plane...

BACKGROUND: Beyond the physiological levels of bilirubin may leads to alterations in autonomic regulation. Heart rate variability (HRV), is a noninvasive and quantitative marker of the activity of the autonomic nervous system (ANS). To date, few studies have been evaluated the undesirable effects of severe unconjugated hyperbilirubinemia (UHB) on autonomic functions, and only one study has used HRV as a marker of the autonomic activity. However, the relationship between altered cardiac autonomic functions and UHB by using the HRV derived from 24-hour Holter electrocardiography (ECG) recording previously...

BACKGROUND: Non-invasive transcutaneous bilirubin (TcB) monitoring has been widely used to screen for hyperbilirubinemia. TcB values measured using the recently developed BiliCare(™) system have not been fully evaluated. METHODS: One hundred and seven TcB measurements were obtained from 82 Japanese newborns ≥ 35 weeks' gestational age within 2 weeks after birth. Measurements were taken at the scaphoid fossa, conchal cavity, and lobe of the ear using BiliCare...

BACKGROUND: Neonatal hyperbilirubinemia is a frequently encountered problem. Erythrocytes, especially reticulocytes are rich in copper (Cu) and magnesium (Mg) so its serum levels increase after hemolysis. Zinc (Zn) plays an important role in synthesis of some enzymes included in the bilirubin metabolism and may cause hemolysis. Exchange transfusion is the main treatment for severe neonatal hyperbilirubinemia but can exchange transfusion affect the previous trace elements. MATERIALS AND METHODS: We measured Cu, Zn, and Mg serum levels in full-term neonates admitted to neonatal intensive care unit of Minia University hospital with severe indirect hyperbilirubinemia before and after exchange transfusion...

BACKGROUND: Eculizumab treated PNH patients (pts) show a dramatic decrease in serum LDH activities and bilirubin concentrations. However, some pts remain hyperbilirubinemic, possibly indicating an inadequate response due to extravascular hemolysis. METHODS: Mutation analyses of hepatocanalicular transporter/nuclear receptor variants (ABCB4, ABCB11, ATP8B1, NR1H4) were performed in eight (5/8 males; mean age 38 yrs (range 26-68 yrs)) out of the 174 pts with PNH/-clone at our department due to a persistent increase in total bilirubin concentrations (median 3...

All diagnostic algorithms for Neonatal Cholestasis Syndrome (NCS) focus on differentiating numerous medical causes from Biliary Atresia (BA). No preoperative diagnostic algorithm has 100% diagnostic accuracy for BA and yet, timely diagnosis is crucial to optimize surgical outcome. Markers for high index of clinical suspicion for BA are: a "usually" well thriving infant with conjugated hyperbilirubinemia, raised gamma glutamyl transpeptidase, persistently "acholic" stools, firm hepatomegaly with dysmorphic, hypoplastic gall bladder...

LESSONS LEARNED: There continues to be a lack of systemic options for advanced hepatocellular carcinoma (HCC); sorafenib and, very recently, regorafenib are the only approved options. There exists a potential to combine sorafenib with chemotherapeutic agents shown to be active in HCC, such as capecitabine, safely.Good tumor response was observed, with objective improvement in a few patients seldom seen by single agent sorafenib; however, because of the limited number of patients, meaningful conclusions on survival cannot be drawn...

OBJECTIVE: Infants whose mothers had syphilis during pregnancy were studied to determine how often exposed newborns with normal physical examinations and nonreactive nontreponemal serologic tests had abnormal laboratory or radiographic studies. STUDY DESIGN: Retrospective analysis of prospectively collected data from infants born to mothers with syphilis and had a normal examination and a nonreactive nontreponemal test. Some infants had IgM immunoblotting, PCR testing or rabbit infectivity testing (RIT) performed...

BACKGROUND: In low-resource countries, screening for D antibodies to detect pregnancies at risk for hemolytic disease of the newborn is not routine practice. Retrospective data showed that 5.5% of Surinamese newborns of D-negative women had a positive direct antiglobulin test (DAT), indicating the presence of maternal antibodies against fetal antigens. Here, the frequency and clinical relevance of DAT positivity is evaluated. STUDY DESIGN AND METHODS: Between April 2015 and June 2016, an observational, multicenter cohort study was undertaken among Surinamese newborns born to D-negative women...

BACKGROUND: Novel extracorporeal procedures are constantly being developed and evaluated for use in patients with sepsis. Preclinical evaluation of such procedures usually requires testing in large animal models. In the present work, the safety and efficacy of a recently developed ADVanced Organ Support (ADVOS) system in a newly developed large animal two-hit model of liver failure combined with endotoxemia were tested. METHODS: After establishing the model in more than 50 animals, a randomized study was performed...

OBJECTIVE: To evaluate the efficacy of oral or intravenous fluid supplementation in accelerating the decline of serum bilirubin with intensive phototherapy among healthy term and late preterm neonates with hyperbilirubinemia. STUDY DESIGN: This open-label randomized controlled trial randomized neonates with severe hyperbilirubinemia (serum total bilirubin ≥18 mg/dL or within 2 mg/dL of threshold for exchange transfusion) to receive 50 mL/kg of intravenous fluid over 8 h (IVF group, n=51), 50 mL of oral rehydration solution over 8 hours (oral rehydration solution (ORS) group, n=50) or only standard therapy (control group, n=49)...