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Saul J Karpen - One of the best experts on this subject based on the ideXlab platform.
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guideline for the evaluation of cholestatic Jaundice in infants joint recommendations of the north american society for pediatric gastroenterology hepatology and nutrition and the european society for pediatric gastroenterology hepatology and nutriti
Journal of Pediatric Gastroenterology and Nutrition, 2017Co-Authors: Rima Fawaz, Björn Fischler, Udeme D Ekong, Ulrich Baumann, Jean P Molleston, Nedim Hadzic, Cara L Mack, Valerie Anne Mclin, Ezequiel Neimark, Saul J KarpenAbstract:Cholestatic Jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic Jaundice. Cholestatic Jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic Jaundice in the first months of life are biliary atresia (25%-40%) followed by an expanding list of monogenic disorders (25%), along with many unknown or multifactorial (eg, parenteral nutrition-related) causes, each of which may have time-sensitive and distinct treatment plans. Thus, these guidelines can have an essential role for the evaluation of neonatal cholestasis to optimize care. The recommendations from this clinical practice guideline are based upon review and analysis of published literature and the combined experience of the authors. The committee recommends that any infant noted to be Jaundiced after 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin, and that an elevated serum direct bilirubin level (direct bilirubin levels >1.0 mg/dL or >17 μmol/L) warrants timely consideration for evaluation and referral to a pediatric gastroenterologist or hepatologist. Of note, current differential diagnostic plans now incorporate consideration of modern broad-based next-generation DNA sequencing technologies in the proper clinical context. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all infants with cholestasis. Broad implementation of these recommendations is expected to reduce the time to the diagnosis of pediatric liver diseases, including biliary atresia, leading to improved outcomes.
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update on the etiologies and management of neonatal cholestasis
Clinics in Perinatology, 2002Co-Authors: Saul J KarpenAbstract:The early detection of cholestatic liver disease is one of the major challenges facing pediatricians when evaluating the Jaundiced infant. Early recognition of liver disease greatly facilitates the care and outcome of infants, because several serious life-threatening disorders may have cholestasis as a major presenting sign of underlying neonatal liver disease. A key component of the work-up is measurement of serum conjugated bilirubin levels, which if elevated should prompt the clinician to initiate a work-up to determine the cause of neonatal cholestasis. In general, if a patient is developing progressive Jaundice soon after birth, is still Jaundiced at 2 weeks of life, or develops Jaundice within the first month of life, a work-up for neonatal cholestasis should begin. A number of previously undiagnosed causes of neonatal cholestasis are beginning to be assigned genetic and infectious etiologies, with significant implications for the work-up and management of cholestatic infants.
Melvin B Heyman - One of the best experts on this subject based on the ideXlab platform.
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guideline for the evaluation of cholestatic Jaundice in infants recommendations of the north american society for pediatric gastroenterology hepatology and nutrition
Journal of Pediatric Gastroenterology and Nutrition, 2004Co-Authors: Virginia Moyer, Richard B Colletti, Deborah K Freese, Peter F Whitington, Alan D Olson, Fred Brewer, Melvin B HeymanAbstract:For the primary care provider, cholestatic Jaundice in infancy, defined as Jaundice caused by an elevated conjugated bilirubin, is an uncommon but potentially serious problem that indicates hepatobiliary dysfunction. Early detection of cholestatic Jaundice by the primary care physician and timely, accurate diagnosis by the pediatric gastroenterologist are important for successful treatment and a favorable prognosis. The Cholestasis Guideline Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition has formulated a clinical practice guideline for the diagnostic evaluation of cholestatic Jaundice in the infant. The Cholestasis Guideline Committee, consisting of a primary care pediatrician, a clinical epidemiologist (who also practices primary care pediatrics), and five pediatric gastroenterologists, based its recommendations on a comprehensive and systematic review of the medical literature integrated with expert opinion. Consensus was achieved through the Nominal Group Technique, a structured quantitative method. The Committee examined the value of diagnostic tests commonly used for the evaluation of cholestatic Jaundice and how those interventions can be applied to clinical situations in the infant. The guideline provides recommendations for management by the primary care provider, indications for consultation by a pediatric gastroenterologist, and recommendations for management by the pediatric gastroenterologist. The Cholestasis Guideline Committee recommends that any infant noted to be Jaundiced at 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin. However, breast-fed infants who can be reliably monitored and who have an otherwise normal history (no dark urine or light stools) and physical examination may be asked to return at 3 weeks of age and, if Jaundice persists, have measurement of total and direct serum bilirubin at that time. This document represents the official recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition on the evaluation of cholestatic Jaundice in infants. The American Academy of Pediatrics has also endorsed these recommendations. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all patients with this problem.
Jeffrey M Maisels - One of the best experts on this subject based on the ideXlab platform.
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the natural history of Jaundice in predominantly breastfed infants
Pediatrics, 2014Co-Authors: Jeffrey M Maisels, Sarah Clune, Kimberlee Coleman, Brian Gendelman, Ada Kendall, Sharon Mcmanus, Mary SmythAbstract:BACKGROUND AND OBJECTIVES: Breastfed newborns are more likely to develop prolonged hyperbilirubinemia than those fed formula, but the prevalence of prolonged hyperbilirubinemia in a largely white, North American breastfed population is unknown. In this population, we documented the natural history of Jaundice and the prevalence of prolonged hyperbilirubinemia, and we evaluated the utility of assessing the cephalocaudal progression of Jaundice in office-based practices. METHODS: We measured transcutaneous bilirubin (TcB) levels during the first month in 1044 predominantly breastfed infants ≥35 weeks of gestation and assigned a cephalocaudal zone score to each infant at the time of the TcB measurement. RESULTS: TcB level was ≥5 mg/dL in 43% of infants at age 21 ± 3 days and 34% were clinically Jaundiced. At 28 ± 3 days, the TcB was ≥5 mg/dL in 34% and 21% were Jaundiced. There was a strong correlation between the TcB level and the Jaundice zone score, but there was a wide range of TcB levels associated with each score. CONCLUSIONS: Practitioners can be reassured that it is normal for 20% to 30% of predominantly breastfed newborns to be Jaundiced at age 3 to 4 weeks and for 30% to 40% of these infants to have bilirubin levels ≥5 mg/dL. The Jaundice zone score does not provide an accurate assessment of the bilirubin level, but a score of zero (complete absence of Jaundice) suggests that the level is unlikely to be >12.9 mg/dL, whereas a score of ≥4 usually predicts a level of ≥10 mg/dL.
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what s in a name physiologic and pathologic Jaundice the conundrum of defining normal bilirubin levels in the newborn
Pediatrics, 2006Co-Authors: Jeffrey M MaiselsAbstract:Because at some point during the first week after birth almost every newborn has a total serum bilirubin (TSB) level that exceeds 1 mg/dL (17 μmol/L), the upper limit of normal for an adult, and ∼2 of every 3 newborns are Jaundiced to the clinician's eye, this type of transient bilirubinemia has been called “physiologic Jaundice.” When TSB levels exceed a certain value, the infant is often described as having “pathologic Jaundice.” I would like to argue that these terms have limited utility and are often used incorrectly, with potentially damaging consequences. They should be abandoned. The physiology of the newborn differs from that of older children and adults in many ways. Newborns breathe 40 to 60 times per minute, and their hearts beat 120 to 160 times per minute. Their hematocrit levels are frequently >60%. In time, all of these values return to normal levels, as does their bilirubin level. However, we don't talk about physiologic tachypnea, tachycardia, or polycythemia, so why pick on Jaundice? Some like the term “physiologic Jaundice” because it has a reassuring sound for parents and physicians. Presumably, physiologic Jaundice should apply to newborns whose TSB levels fall within a certain range, but what is that range? Because very few (if any) newborns have peak TSB levels <2 mg/dL, should an infant with a peak TSB of 1.5 mg/dL be considered abnormal or hypobilirubinemic? Unlike serum sodium levels, the range of normal TSB levels varies widely depending on the racial composition of the population, the incidence of breastfeeding, and other genetic and epidemiologic factors.1 There are also significant variations between different laboratories in their measurements of serum bilirubin.2 Term, healthy, North American, formula-fed infants have mean peak TSB levels between 5 and 6 mg/dL (86 and 103 μmol/L),3,4 whereas Japanese … Address correspondence to M. Jeffrey Maisels, MB, BCh, Department of Pediatrics, William Beaumont Hospital, 3601 W 13 Mile Rd, Royal Oak, MI 48073. E-mail: jmaisels{at}beaumont.edu
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the contribution of hemolysis to early Jaundice in normal newborns
Pediatrics, 2006Co-Authors: Jeffrey M Maisels, Elizabeth KringAbstract:OBJECTIVE. Neonatal Jaundice is the result of an imbalance between bilirubin production and elimination, and our objective was to clarify the contribution of an increase in bilirubin production to hyperbilirubinemia in newborns. METHODS. We measured the end-tidal carbon monoxide concentration corrected for ambient carbon monoxide concentration in 108 Jaundiced newborns (total serum bilirubin level >75th percentile) and 164 control newborns in our well-infant nursery, for the first 4 days after birth. RESULTS. Mean end-tidal carbon monoxide levels decreased in the control infants in the first 4 days but increased in the hyperbilirubinemic group. The differences between the Jaundiced and nonJaundiced infants were statistically significant on all days. CONCLUSIONS. Before hospital discharge, most infants with bilirubin levels >75th percentile are producing significantly more bilirubin than those with lower bilirubin levels. Because the ability of newborns to conjugate bilirubin is significantly impaired in the first few days, even a small increase in the rate of production can contribute to the development of hyperbilirubinemia. These data suggest that increased heme catabolism is an important mechanism responsible for hyperbilirubinemia in the first 4 days after birth.
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Jaundice in a newborn answers to questions about a common clinical problem first of two parts
Contemporary pediatrics, 2005Co-Authors: Jeffrey M MaiselsAbstract:Yes, Jaundice in newborns is prevalent and usually benign, but these babies still need ongoing clinical assessment. Part 1 reviews ways to identify and categorize hyperbilirubinemia and sets out the testing that a Jaundiced infant requires.
Nuray Urhan - One of the best experts on this subject based on the ideXlab platform.
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cea ca 19 9 and ca 125 in the differential diagnosis of benign and malignant pancreatic diseases with or without Jaundice
Journal of Surgical Oncology, 2007Co-Authors: Nuvit Duraker, Semih Hot, Yucel Polat, Anil Hobek, Nur Gencler, Nuray UrhanAbstract:Background and Objectives In this study, the value of the serum tumor markers carcinoembryonic antigen (CEA), CA 19-9, and CA 125 was assessed in the differential diagnosis of benign and malignant pancreatic diseases with and without obstructive Jaundice. Methods Serum levels of CEA, CA 19-9, and CA 125 were measured by immunoradiometric assay before the treatment in 123 patients with pancreatic carcinoma and 58 patients with a benign pancreatic disease. Results The sensitivity of CEA, CA 19-9, and CA 125 in the diagnosis of pancreatic carcinoma was 39.0%, 81.3%, and 56.9%; and specificity was 91.4%, 75.9%, and 77.6%, respectively. Although there was no significant difference between the CA 19-9 positivity ratios of the Jaundiced (84.3%) and nonJaundiced (73.5%) patient subgroups of the pancreatic carcinoma, this ratio was significantly higher in the Jaundiced subgroup (64.7%) than the nonJaundiced subgroup (7.3%) of the benign pancreatic diseases (P < 0.001). The CEA and CA 125 positivity ratios of Jaundiced and nonJaundiced subgroups of patients with benign and malignant pancreatic diseases were not significantly different. Conclusions In the differential diagnosis of pancreatic carcinoma from benign pancreatic diseases, CA 19-9 can be useful in the nonJaundiced patients, whereas CA 125 provides a limited contribution in Jaundiced patients. J. Surg. Oncol. 2007;95:142–147. © 2007 Wiley-Liss, Inc.
Ryan C Fields - One of the best experts on this subject based on the ideXlab platform.
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gallbladder cancer presenting with Jaundice uniformly fatal or still potentially curable
Journal of Gastrointestinal Surgery, 2017Co-Authors: Thuy B Tran, Jeffrey A Norton, Cecilia G Ethun, Timothy M Pawlik, Stefan Buettner, Carl Schmidt, Eliza W Beal, William G Hawkins, Ryan C FieldsAbstract:Jaundice as a presenting symptom of gallbladder cancer has traditionally been considered to be a sign of advanced disease, inoperability, and poor outcome. However, recent studies have demonstrated that a small subset of these patients can undergo resection with curative intent. Patients with gallbladder cancer managed surgically from 2000 to 2014 in 10 US academic institutions were stratified based on the presence of Jaundice at presentation (defined as bilirubin ≥4 mg/ml or requiring preoperative biliary drainage). Perioperative morbidity, mortality, and overall survival were compared between Jaundiced and non-Jaundiced patients. Of 400 gallbladder cancer patients with available preoperative data, 108 (27%) presented with Jaundice while 292 (73%) did not. The fraction of patients who eventually underwent curative-intent resection was much lower in the presence of Jaundice (n = 33, 30%) than not (n = 218, 75%; P < 0.001). Jaundiced patients experienced higher perioperative morbidity (69 vs. 38%; P = 0.002), including a much higher need for reoperation (12 vs. 1%; P = 0.003). However, 90-day mortality (6.5 vs. 3.6%; P = 0.35) was not significantly higher. Overall survival after resection was worse in Jaundiced patients (median 14 vs. 32 months; P < 0.001). Further subgroup analysis within the Jaundiced patients revealed a more favorable survival after resection in the presence of low CA19-9 < 50 (median 40 vs. 12 months; P = 0.003) and in the absence of lymphovascular invasion (40 vs. 14 months; P = 0.014). Jaundice is a powerful preoperative clinical sign of inoperability and poor outcome among gallbladder cancer patients. However, some of these patients may still achieve long-term survival after resection, especially when preoperative CA19-9 levels are low and no lymphovascular invasion is noted pathologically.
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Jaundice an important poorly recognized risk factor for diminished survival in patients with adenocarcinoma of the head of the pancreas
Hpb, 2014Co-Authors: Steven M Strasberg, William G Hawkins, Ryan C Fields, Dominic E Sanford, David C Linehan, Danielle Carpenter, Elizabeth M Brunt, Carolyn PhillipsAbstract:Objectives: Jaundice impairs cellular immunity, an important defence against the dissemination of cancer. Jaundice is a common mode of presentation in pancreatic head adenocarcinoma. The purpose of this study was to determine whether there is an association between preoperative Jaundice and survival in patients who have undergone resection of such tumours. Methods: Thirty possible survival risk factors were evaluated in a database of over 400 resected patients. Univariate analysis was used to determine odds ratio for death. All factors for which a P-value of <0.30 was obtained were entered into a multivariate analysis using the Cox model with backward selection. Results: Preoperative Jaundice, age, positive node status, poor differentiation and lymphatic invasion were significant indicators of poor outcome in multivariate analysis. Absence of Jaundice was a highly favourable prognostic factor. Interaction emerged between Jaundice and nodal status. The benefit conferred by the absence of Jaundice was restricted to patients in whom negative node status was present. Five-year overall survival in this group was 66%. Jaundiced patients who underwent preoperative stenting had a survival advantage. Conclusions: Preoperative Jaundice is a negative risk factor in adenocarcinoma of the pancreas. Additional studies are required to determine the exact mechanism for this effect.