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The article presents a case study of a 13-year-old male with abdominal pain, jaundice, and liver injury of unknown cause, eventually traced back to the consumption of Herbalife nutritional supplements. It mentions the challenges in diagnosing drug-induced liver injury (DILI), the importance of comprehensive history-taking, and the potential risks associated with dietary supplements, particularly in pediatric patients.

Multiple causes of congenital neonatal cholestasis have been identified, and are classified as extrahepatic or intrahepatic. Biliary atresia (BA), Alagille syndrome (AGS), and progressive familial intrahepatic cholestasis (PFIC) are the most common of these. Many factors associated with cholestatic diseases are known to degrade the oral health of these children. What are the oral manifestations associated with these diseases in the pediatric population? The aim of this article was to evaluate the impact of congenital cholestasis on oral health in pediatric patients. A systematic review of case reports and case series was carried out in PubMed, the Cochrane Library, and the Web of Science to identify relevant articles in French and English published up to April 2022. The review included 19 studies, 16 case reports, and three case series. Only studies dealing with BA and AGS were found. These studies showed an impact on jaw morphology, dental structure, and periodontal health. The facial dysmorphism observed in AGS was specific. Exposure to high levels of bilirubin during the period of dental calcification led to particular coloration. Regarding periodontal status, gingival inflammation was common in these patients, probably resulting from the use of certain treatment-associated drugs and poor oral hygiene. Cohort studies are needed to confirm the classification of these children as being at high individual risk of caries. Many major oral manifestations are found in children with AGS and BA, confirming the need to include a dentist in the care team of patients with congenital cholestatic disease as early as possible. It appears necessary to carry out individual prospective studies of each phenotype in order to confirm and better describe the oral impact of these cholestatic diseases and provide adequate medical care. [ABSTRACT FROM AUTHOR]

Interferences in the clinical laboratory may lead physicians misinterpret results for some biological analytes. The most common analytical interferences in the clinical laboratory include hemolysis, icterus and lipemia. Lipemia is defined as turbidity in a sample caused by the accumulation of lipoproteins, mainly very-low density lipoproteins (VLDL) and chylomicrons. Several methods are available for the detection of lipemic samples, including the lipemic index, or triglyceride quantification in serum or plasma samples, or mean corpuscular hemoglobin (MCHC) concentration in blood samples. According to the European Directive 98/79/CE, it is the responsibility of clinical laboratories to monitor the presence of interfering substances that may affect the measurement of an analyte. There is an urgent need to standardize interference studies and the way interferences are reported by manufacturers. Several methods are currently available to remove interference from lipemia and enable accurate measurement of biological quantities. The clinical laboratory should establish a protocol for the handling of lipemic samples according to the biological quantity to be tested. [ABSTRACT FROM AUTHOR]

Massive hemobilia, an acute life-threatening condition, remains a diagnostic challenge in pediatric patients due to its rarity. In this study, the difficulties encountered in the diagnosis and treatment of acute massive hemobilia and acute obstructive jaundice in a 3-year-old boy are discussed in the light of the literature. [ABSTRACT FROM AUTHOR]

A 7-day-old male child presented with abdominal distention and jaundice. Radiological investigations revealed an encapsulated sac encasing three fetus-in-fetu (FIF) in the retroperitoneum. Laparotomy revealed a sac occupying almost the whole of the abdomen. The sac was stretching the duodenum and barely visible common bile duct, which were carefully separated. The rest of the bowel was displaced to the left. The sac containing three FIFs was excised intact. One of the fetuses was highly differentiated and had thoracic meningomyelocele, which has never been reported in FIF. [ABSTRACT FROM AUTHOR]

AbstractMorning Report is a time-honored tradition where physicians-in-training present cases to their colleagues and clinical experts to collaboratively examine an interesting patient presentation. The Morning Report section seeks to carry on this tradition by presenting a patient's chief concern and story, inviting the reader to develop a differential diagnosis and discover the diagnosis alongside the authors of the case. This report examines the story of a 52-year-old man who presented to the emergency department with subacute abdominal pain and jaundice. Fever, worsening liver function tests, and multi-organ failure developed. Using questions, physical examination, and testing, an illness script for the presentation emerges; the differential is refined until a final diagnosis is made.

We report a case of a 22-year-old woman who presented with recurrent episodes of quadriparesis, often accompanied by jaundice. Neurologic examination showed symmetrical proximal predominant quadriparesis with generalized hyporeflexia. The differential diagnoses included were of metabolic, inflammatory, genetic (including channelopathies), and autoimmune causes. Serum creatine phosphokinase levels and electrophysiologic studies helped narrow the differential. The final diagnosis was one that was responsive to vitamin supplementation. This report provides a systematic clinical approach to a case of episodic weakness with jaundice and respiratory failure.

Despite substantial progress in the diagnosis of jaundice/hyperbilirubinemia as the most common disease and cause of hospitalization of newborns, on the eve of Industry/Healthcare 5.0, the development of accurate and reliable wearable diagnostic sensors for noninvasive smart monitoring of bilirubin (BIL) is still in high demand. Aiming to fabricate a smart wearable sensor for early diagnosis of neonatal jaundice and its therapeutic monitoring, we here report a fluorescent dermal nanotattoo that further coupled with an IoT-integrated wearable optoelectronic reader for minimally invasive, continuous, and real-time monitoring of BIL in interstitial fluid. Selective recovery of quenched fluorescence of the dermal tattoo sensor, composed of biocompatible dissolving/hydrogel microneedles loaded with fluorescent carbon quantum dots, upon blue light exposure used for jaundice phototherapy was utilized for highly selective BIL sensing. The fascinating features of our developed smart wearable tattoo sensor and its successful results with high correlation with blood BIL results make it a highly promising sensor for easy, minimally invasive, reliable, and smart eDiagnostics and continuous therapeutic eMonitoring of jaundice and other BIL-induced diseases at the point of care. We envision that the developed nanotattoo sensing bioplatform will inspire the development of future smart tattoo sensors in various diagnostic and monitoring scenarios.

Background: Cholestasis is characterized by the accumulation of bile in the liver or biliary system due to obstruction or impaired flow, necessitating lipid profiling to assess lipid metabolism abnormalities. Intrahepatic cholestasis, being the most significant type of cholestasis, further complicates the assessment of lipid abnormalities. However, the accuracy of low-density lipoprotein cholesterol (LDL-C) measurement in intrahepatic cholestasis patients remains uncertain., Objective: This study aimed to evaluate the consistency of the homogeneous assay and the Friedewald formula in detecting LDL-C levels and identify factors influencing LDL-C test results in intrahepatic patients with cholestasis., Methods: Retrospective analysis of laboratory data was conducted on intrahepatic cholestatic patients. Correlations between LDL-C values obtained using the homogeneous method (LDL-C(D)) and the Friedewald formula (LDL-C(F)), as well as associations between high-density lipoprotein cholesterol (HDL-C) and apolipoprotein A1 (ApoA1), LDL-C(D) and LDL-C(F), and apolipoprotein B (ApoB), were analyzed. Logistic regression analyses were employed to identify diagnostic indicators for inaccurate LDL-C measurements in intrahepatic cholestatic patients., Results: Compared to patients with intrahepatic cholestasis without jaundice, the correlation between LDL-C(F) and LDL-C(D) was weaker in those with jaundice. Additionally, HDL-C exhibited a strong correlation with ApoA1 in both jaundice and non-jaundice cholestasis cases. Elevated non-HDL-C to APOB ratio (NH-C/B Ratio) levels (>4.5) were identified as a reliable predictor of inaccurate LDL-C measurements in patients with chronic intrahepatic cholestasis accompanied by jaundice., Conclusions: LDL-C measurement reliability is moderately weaker in patients with intrahepatic cholestasis accompanied by jaundice. Elevated levels of the NH-C/B ratio serve as a significant predictor of inaccurate LDL-C measurements in this chronic patient population, highlighting its clinical relevance for diagnostic assessments., Competing Interests: The authors declare that they have no competing interests., (© 2024 Cheng et al.)

Objectives: The aims of this study were to (1) establish the maximum allowable interference limits for hemolysis, lipemia, and icterus for chemistry analytes tested in body fluid samples and (2) assess the effectiveness of serial dilution to mitigate spectral interferences., Methods: Residual body fluids from clinically ordered testing were mixed (<10% by volume) with stock solutions of interferent (spiked) and compared with a control spiked with an equal volume of 0.9% saline. The analytes were measured on the Roche cobas c501 instrument. Difference and percentage difference were calculated and compared with allowable total error limits. A subset of samples were serially diluted with 0.9% saline. Mean (SD) difference and percentage difference were calculated., Results: The interference thresholds were lower than the package insert for lactate dehydrogenase, cholesterol, triglycerides, and total protein for hemolysis; amylase, cholesterol, and total protein for icterus; and albumin for lipemia. Only cholesterol and triglyceride results returned to baseline upon dilution of icteric samples., Conclusions: Interference thresholds in body fluids were lower than blood for 6 analytes. Diluting interferences that surpass these limits does not produce reliable results that are comparable to the baseline results before spiking in the interferent., (© The Author(s) 2024. Published by Oxford University Press on behalf of American Society for Clinical Pathology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Anemia is a common finding in our population. Females are highly predisposed to develop anemia. Evaluation of anemia due to a single cause may be straightforward in most of the situations, however rare causes cannot be ruled out. In this case report, we present to you a case of a 35-year-old female who came with complaints of breathlessness. On further evaluation, she had pallor, icterus, and mild splenomegaly. Her hemoglobin (Hb) was later found to be 8.1 g/dl, pyruvate kinase activity was normal, normal liver enzymes, Heinz body positive, and elevated retics count. Thus, she was found to have a rare form of anemia, i.e., Heinz body hemolytic anemia. She was advised for splenectomy and started on medications. Following the procedure, her Hb started to rise to 9.6 g/dl and reached to 13.8 in the following months. [ABSTRACT FROM AUTHOR]

Introduction: It is not known if the nature, number and duration of presenting symptoms at diagnosis of hepatocellular carcinoma impact on overall survival. This study examines whether the presenting symptoms of hepatocellular carcinoma have a significant impact on prognosis., Methods: The study cohort comprised 725 patients with symptomatic hepatocellular carcinoma seen in our department since October 1983. Another 545 patients were diagnosed on surveillance or from incidental findings. Presenting symptoms at diagnosis were documented. A survival census was performed on 31 October 2015 with the national registry of deaths. Presenting symptoms were examined for association with overall survival using multivariable Cox regression analysis. Survival analysis was done by Kaplan-Meier method with log-rank testing. Bivariate Pearson correlation was used to look for any association between duration of symptoms and overall survival., Results: Patients with symptomatic hepatocellular carcinoma had a significantly shorter survival than those diagnosed incidentally or on screening (94.0 vs. 786.0 days, P < 0.001). Survival was shorter in patients presenting with fluid retention (56.0 vs. 118.0 days, P < 0.001), jaundice (48.0 vs. 94.0 days, P = 0.017) and two or more symptoms ( P = 0.010). Pain was associated with better survival ( P < 0.001). On multivariable Cox regression analysis, only fluid retention (hazard ratio [HR] 1.56, 95% confidence interval [CI] 1.30-1.87) and jaundice (HR 1.36, 95% CI 1.07-1.74) were independently associated with shorter survival. There was no significant relationship between the duration of symptoms and overall survival., Conclusion: Patients with hepatocellular carcinoma who present with fluid retention or jaundice have significantly shorter overall survival. This is useful in assessing patients at the time of diagnosis., (Copyright © 2024 Copyright: © 2024 Singapore Medical Journal.)

Objectives: The thiol-disulphide (TDH) homeostasis was investigated in a number of disorders. The aim of the present study is to investigate the dynamic thiol-disulphide homeostasis in patients diagnosed with obstructive jaundice and to compare the dynamic thiol-disulphide homeostasis with ischemia-modified albumin. Materials and Methods: A total of 146 participants who were admitted to the emergency department and who consisted of 69 patients and 77 healthy volunteers were included in the study. Detailed blood samples were obtained from the patients at the time of admission. Thiol/disulphide levels were examined using a brand-new method developed by Erel and Neselioglu on the patients diagnosed with obstructive jaundice during the admission. Data were evaluated in the computer system. Results: The levels of total thiol (p =0.025) and native thiol (p =0.023) were statistically significantly lower in the patient with obstructive jaundice than in the control group. It was observed that there was a significant negative correlation between the native thiol levels and the ALP (r =-0.262, p<0.01), urea (r =-0.313, p<0.01), total bilirubin (r =-0.388, p<0.01), direct bilirubin (r =-0.351, p<0.01) levels. At the same time, the level of disulphide (p = 0.235) was lower in the patient group than in the control group but not statistically significant. It was found that the ischemia modified albumin (IMA) values were lower in the control group than the obstructive jaundice group (p = 0.03). Conclusion: Total thiol and native thiol levels decrease in obstructive jaundice pathogenesis. Thiol-disulphide homeostasis may be a new biomarker of oxidative stress in obstructive jaundice. [ABSTRACT FROM AUTHOR]

OBJECTIVES: We aimed to determine whether mean platelet volume value and/or neutrophil/lymphocyte ratio values are useful as a predictive marker for gestational cholestasis. STUDY DESIGN: Retrospective analysis of the data of patients diagnosed with pregnancy cholestasis between 2018-2019 in a perinatology clinic. 352 pregnant women were enrolled in the study (122 pregnant women with Intrahepatic cholestasis as study group and 230 pregnant women with no morbidity as the control group). RESULTS: Mean platelet volume was significantly higher in pregnant women with intrahepatic cholestasis compared to the healthy controls, 9.30 (7-18) and 8.6 (6.7-11.5), respectively. The neutrophil/lymphocyte ratio was significantly higher in pregnant women with intrahepatic cholestasis compared to the healthy controls, 3.93 (0.46-13.75) and 4.25 (0.87-17.1), respectively. There was a statistically significant difference between the two groups for mean platelet volume (p <0.001). In the roc analysis, 8.85 fL for mean platelet volume had a sensitivity of 65% and a specificity of 59%. CONCLUSION: When compared with healthy pregnancies, mean platelet volume value in gestational cholestasis increases significantly. However, the predictive strength of mean platelet volume for cholestasis is not strong enough to recommend its usage as a single parameter in clinical practice. [ABSTRACT FROM AUTHOR]

Introduction Endoscopic ultrasound (EUS) is a diagnostic procedure that continues to have greater clinical applications. EUS performed before endoscopic retrograde cholangiopancreatography (ERCP) in patients with obstructive jaundice can reduce morbidity and mortality. Aim This study aimed to compare the diagnostic accuracy of both EUS and ERCP as combined or individual procedures in 30 patients diagnosed with biliary obstruction. Patients and methods From January 2017 to December 2019 in Al Azhar University Hospitals in Cairo, we recruited 30 patients with obstructive jaundice. All the patients underwent clinical examination, laboratory investigations, and abdominal US followed by EUS and ERCP procedures at the same session, and EUS findings were compared with that of ERCP. Results The diagnostic accuracy of EUS was as follows: choledocholithiasis in 92%, and malignant lesions in 95%. EUS showed pathology in 47.3% of patients who had an unknown pathology in biliary system at initial investigations. The overall diagnostic performance of EUS shows that the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 90.9, 89, 91.7, 95, and 93.5%, respectively. Regarding common bile duct stone (s), by ERCP, it was present in 13 (43.3%) cases, whereas by EUS, it was present in 12 (40%) cases. Regarding malignant lesions (ampullary tumor, cholangiocarcinoma, and pancreatic tumor), by ERCP, they were present in four (13.3%), seven (23.3%), three (10%) cases, respectively whereas by EUS, they were present in five (16.6), six (20%), and five (16.6) cases, respectively. The complication rate was 3.3%. Conclusions EUS established high diagnostic accuracy in diagnosing the causes of obstructive jaundice, with no statistically significant difference between ERCP and EUS findings. This accurately guided ERCP need and avoided needless ERCP. EUS and ERCP in a single session for treatment of obstructive jaundice is scientifically valuable. [ABSTRACT FROM AUTHOR]

Jaundice occurs as a symptom of various diseases, such as hepatitis, the liver cancer, gallbladder or pancreas. Therefore, clinical measurement with special equipment is a common method that is used to identify the total serum bilirubin level in patients. Fully automated multi-class recognition of jaundice combines two key issues: (1) the critical difficulties in multi-class recognition of jaundice approaches contrasting with the binary class and (2) the subtle difficulties in multi-class recognition of jaundice represent extensive individuals variability of high-resolution photos of subjects, huge coherency between healthy controls and occult jaundice, as well as broadly inhomogeneous color distribution. We introduce a novel approach for multi-class recognition of jaundice to detect occult jaundice, obvious jaundice and healthy controls. First, region annotation network is developed and trained to propose eye candidates. Subsequently, an efficient jaundice recognizer is proposed to learn similarities, context, localization features and globalization characteristics on photos of subjects. Finally, both networks are unified by using shared convolutional layer. Evaluation of the structured model in a comparative study resulted in a significant performance boost (categorical accuracy for mean 91.38%) over the independent human observer. Our work was exceeded against the state-of-the-art convolutional neural network (96.85% and 90.06% for training and validation subset, respectively) and showed a remarkable categorical result for mean 95.33% on testing subset. The proposed network makes a performance better than physicians. This work demonstrates the strength of our proposal to help bringing an efficient tool for multi-class recognition of jaundice into clinical practice. [ABSTRACT FROM AUTHOR]

Even though significant advances have been made, there is still a lack of reliable sensors capable of noninvasively monitoring bilirubin and diagnosing jaundice as the most common neonatal disease, particularly at the point-of-care (POC) where blood sampling from infants is accompanied by serious challenges and concerns. Herein, for the first time, using an easy-to-fabricate/use assay, we demonstrate the capability of curcumin embedded within paper for noninvasive optical monitoring of bilirubin in saliva. The highly selective sensing of the developed sensor toward bilirubin is attributed to bilirubin photoisomerization under blue light exposure, which can selectively restore the bilirubin-induced quenched fluorescence of curcumin. We also fabricated an IoT-enabled hand-held optoelectronic reader to measure and quantify the fluorescence and color signals of our sensor. Clinical analysis on the saliva of 18 jaundiced infants by using our developed smart salivary sensor proved that it is amenable to be widely exploited in POC applications for bilirubin monitoring as there are good correlations between its results with those of reference methods in saliva and blood. Meeting all WHO's REASSURED criteria by our developed sensor makes it a highly promising sensor for smart noninvasive diagnosis and therapeutic monitoring of jaundice, hepatitis, and other bilirubin-induced neurologic diseases at the POC.

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Background: The ability of mothers to correctly detect jaundice in their newborns is largely unknown. The objective was to ascertain the ability of mother to correctly detect the presence of significant jaundice in her newborn. Materials and Methods: This cross-sectional study was conducted in a tertiary care hospital in eastern India from February 2015 to July 2016. All inborn neonates more than 34 weeks' gestation were included. Congenital malformations, perinatal asphyxia, neonatal sepsis, readmission after discharge, and isoimmunization were excluded. A total of 505 inborn newborns were independently assessed by the mother and the treating pediatrician for significant jaundice every day till discharge. Each newborn underwent total serum bilirubin estimation on suspicion of significant jaundice by either of the two or at discharge, whichever was earlier. Results: The sensitivity, specificity, positive predictive value (PPV), and negative predictive value of maternal detection of significant jaundice was 51.47%, 88.33%, 39.29%, and 92.12%, respectively. Conclusion: Mothers have poor sensitivity and PPV to detect significant neonatal jaundice in the Indian population. [ABSTRACT FROM AUTHOR]

A 39-year-old female presented with a one-week history of jaundice and nausea after taking an over-the-counter herbal supplement containing ashwagandha root extract. Initial investigations revealed a hepatocellular pattern of liver enzyme abnormality with jaundice. Investigations, including viral serology, liver specific autoantibodies and an ultrasound scan of the abdomen, were unremarkable. Liver biopsy showed an acute cholestatic hepatitis with confluent necrosis but no features of chronicity. These histopathological findings differ to that of a previously reported case. Review of recent literature revealed that some clinical features and the time course of liver injury were similar to previous reports of ashwagandha drug-induced liver injury (DILI). The patient received treatment with ursodeoxycholic acid. We compare this case to previous reported cases of ashwagandha DILI and discuss the biochemical and histopathological features of ashwagandha DILI, therapeutic strategies and the importance of recognising herbal supplements as a possible cause of DILI. [ABSTRACT FROM AUTHOR]

Background: Neonatal jaundice is a condition caused by elevated levels of bilirubin in the bloodstream. Laboratory determination of serum bilirubin concentration by total serum bilirubin (TSB) test is still considered as gold standard for clinical guidance and practice. In developed countries, diagnosis of neonatal jaundice is shifting towards point-of-care medical devices. BiliDx is a device developed to allow a fast, blood-based determination of bilirubin levels at the point of care. This study aimed to determine the accuracy of the BiliDx device relative to a standard laboratory total serum bilirubin to diagnose and monitor jaundice among neonates admitted at Muhimbili National Hospital (MNH)., Material and Methodology: This was a prospective hospital-based observational study conducted at the Neonatal Ward - MNH, Dar-es-Salaam, Tanzania from November 2022 to January 2023. A total of 180 neonates admitted at the neonatal ward with jaundice and whose parents consented were enrolled in the study. Blood samples were collected; 2 ml of venous blood into the vacutainer bottle for standard laboratory measurement of total serum bilirubin (TSB) and 25µL blood collected into a transfer pipette tube and applied to BiliDx. STATA version 15.1 was used for data analysis., Results: Out of 180 neonates, 39.4% (71/180) had birth weight between 1500 - 2499.9 g, approximately 2/3rd (120/180) were preterm, 92/180 (51.1%) were males and 100/180 (55.6%) were undergoing phototherapy treatment the moment sample taken. The mean bilirubin concentration was 92 mmol/l for BiliDx and 118 mmol/l for standard laboratory TSB. The minimum and maximum values obtained with BiliDx were, 3.4 and 427.5 mmol/l respectively, compared with 10.7 and 382.1 mmol/l using standard laboratory TSB. A linear relationship and correlation coefficient of 0.8408 (p = 0.000) between BiliDx and standard laboratory TSB was found. The regression analysis showed the presence of constant error [coefficient of BiliDx/slope = 0.91, 95% CI (0.82-0.99), p = 0.000] and random error exclusively [coefficient of constant/y-intercept = 48.52, 95%CI (37.70-59.34), p = 0.000]. The Bland-Altman plot showed an acceptable mean difference of 39.1mmol/l, limits of agreement of -48.3mmol/l to 126.4mmol/l, and 179 points (179/180 = 99.4%) lying inside the limits of agreement., Conclusion: The results support the use of BiliDx for rapid and accurate testing of elevated levels of bilirubin in the bloodstream among neonates since 99.4% of the differences between BiliDx and standard laboratory TSB lie between the lines of agreement., (© 2024. The Author(s).)

Hemophagocytic lymphohistiocytosis (HLH) is a rare, usually fatal and underdiagnosed autoimmune-activated disease. The present study aimed to perform a macroscopic, histopathological and immunohistochemical evaluation for CD68 and CD57 in organs of autopsied adults with HLH. A total of 604 autopsy reports were analyzed, and all the patients that filled the diagnostic criteria for HLH (n = 2) were selected. These patients were 18 and 37 years old. Were evaluated both clinical and autopsy reports and performed histopathological and immunohistochemical analysis of the liver and spleen. Both patients filled the diagnostic criteria for HLH, as well as presented common signs and symptoms of this disease, such as chills, abdominal pain, diaphoresis, and jaundice. Hemophagocytosis was observed in the spleen, bone marrow, and lymph nodes of the two patients at autopsy. Immunostaining in the liver and spleen of both patients was mainly severe for CD68, and predominantly mild for CD57, indicating a decrease in NKC numbers and an increase in the number of macrophages, respectively. This was the first study to evaluate CD57 and CD68 in autopsies of adults with HLH. Thus, more studies are required, not only to better elucidate the pathogenetic mechanisms involved in the secondary HLH, but also to disseminate the results in the clinical environment, contributing to the early diagnosis and treatment with consequent reduction of mortality rate. [ABSTRACT FROM AUTHOR]

Abstract Diffuse reflectance spectroscopy is a non-destructive method to obtain biochemical and physiological information by investigating the optical properties of skin. Transcutaneous bilirubin (TcB) measurement utilizes reflectance spectroscopy to determine the jaundice level in newborns. Although TcB measurement has some advantages over total serum bilirubin (TSB) measurement such as being non-invasive, noninfectious, painless, and instantaneous, the existing TcB devices cannot yet replace TSB devices due to the inaccuracy of measurements. In this paper, we propose the use of reflectance spectroscopy in conjunction with regression tools such as multiple polynomial regression (MPR), artificial neural network (ANN), and support vector regression (SVR) to predict the jaundice level. The proposed methods were tested on TcB measurement data obtained from 314 babies. TcB measurements were collected by two devices: a commercially available product, Draeger JM-103, and a prototype device on which we can implement the proposed algorithms. The results are encouraging towards increasing the clinical usage of transcutaneous bilirubinometers as all the three methods accurately predict the jaundice level with a correlation value between 0.932 and 0.943. The proposed use of ANN improves the non-invasive transcutaneous approach, with results converging to more accurate invasive serum bilirubin measurements by blood sampling. [ABSTRACT FROM AUTHOR]

Context: Reflection in practice is assumed to enhance interest in knowing more about a topic, increasing engagement in learning and learning outcomes. However, this claim lacks empirical evidence, particularly in medical education. The authors investigated the effects of deliberate reflection upon clinical cases on medical students' engagement in a learning activity and learning outcomes. Methods: A three‐task (diagnostic task; learning activity; test) experiment was conducted in August 2017. Seventy‐two fourth‐year students from UNIFENAS‐BH Medical School, Brazil, diagnosed two clinical cases with jaundice as the chief complaint, either by following a deliberate reflection procedure or making differential diagnosis. Subsequently, all participants received the same study material on the diagnosis of jaundice. Finally, they took a recall test on the study material. Outcome measurements were study time and test scores. Results: There was a significant effect of experimental condition on students' engagement in the learning activity and on learning outcomes. Students who deliberately reflected upon the cases invested more time in studying the material than those who made a differential diagnosis (respectively, mean = 254.97, standard deviation = 115.45 versus mean = 194.96, standard deviation = 111.68; p = 0.02; d = 0.53). Deliberate reflection was also related to higher scores in the test relative to differential diagnosis (respectively, mean = 22.08, standard deviation = 14.94 versus mean = 15.75, standard deviation = 9.24; p = 0.03; d = 0.51). Medium effect sizes (Cohen's d) were observed in both measurements. Conclusions: Relative to making differential diagnosis, deliberate reflection while diagnosing cases fostered medical students' engagement in learning and increased learning outcomes. Teachers can employ this relatively easy procedure, possibly both with simulated and real scenarios, to motivate their students and help them expand their knowledge, an important requirement for their professional development. Deliberate reflection has long been known to offer a useful intervention for improving learning. This experiment offers one mechanism by indicating that it fosters medical students' engagement. [ABSTRACT FROM AUTHOR]

Background/Aims: Biliary atresia (BA) is a cholangio-destructive disease of the infant liver presenting with features of obstructive cholangiopathy. The Kasai portoenterostomy (KPE) is the first line of management. The aim of our study was to identify the characteristic features of liver histology in BA that impact the outcome of KPE. Patients and Methods: Data from 30 consecutive children was retrieved from our prospectively maintained database of children undergoing KPE. This included basic demographics, laboratory values and histopathological data from liver biopsy. The stages of fibrosis, presence of ductal plate malformation (DPM), giant cell transformation, extramedullary hematopoiesis and area percentage of α-SMA (α-smooth muscle actin) expression was correlated with jaundice clearance after KPE using standard statistical tests. Native liver survival was computed. Results: Overall, 13 (43%) children cleared jaundice in this series and 10 (33%) are alive with native liver. Lower area percent expression of α-SMA correlated with increased probability of jaundice clearance after KPE (P < 0.001). There was no correlation between stage of fibrosis and jaundice clearance (P = 0.52). DPM, giant cell transformation and extramedullary hematopoiesis did not correlate with outcome. All children who are alive with native liver had lower expression of α-SMA. Conclusion: α-SMA expression may be a potential predictor of jaundice clearance and native liver survival after KPE. [ABSTRACT FROM AUTHOR]

In children, acute viral hepatitis-A infection is usually a self-limiting illness. In most children, it resolves uneventfully within weeks from the onset of disease. However, rare presentations like acute acalculous cholecystitis and persistent cholestatic jaundice have been reported. We report on a 7-year-old boy with prolonged cholestatic jaundice, which resolved without steroid treatment. [ABSTRACT FROM AUTHOR]

Background/objectives Individuals with Gilbert's syndrome (GS) harbor mutations in the UGT1A1 gene and are known to have elevated levels of bilirubin, which enhances the risk for gall stone formation. The aim of this study is to screen Indian patients with GS for the incidence of gall stone disease. Methods Individuals with persistently elevated serum bilirubin levels were genotyped for two polymorphisms (rs8175347; rs4148323) in UGT1A1 gene to confirm GS in them. Flanking regions of the above polymorphisms were amplified followed by direct sequencing. Ultrasonography was done to detect gallstone disease. Clinical data, including assessment of liver function, circulating levels of total and direct bilirubin, as well as routine hematological parameters were obtained as per standard procedures (Autoanalyzer). Results Of the total 1621 individuals subjected to genotyping, 1191 (1149 males of 29.6 ± 11.3 years with mean BMI of 22.1 ± 3.7 kg/m2 and 42 females of 30.8 ± 14.8 years with mean BMI of 20.9 ± 3.7 kg/m2) were confirmed to have GS. Gall bladder abnormalities including cholelithiasis (n = 106/1191; 8.9%), polyps (n = 18/1191; 1.5%) and gallbladder wall thickening (n = 17/1191; 1.4%) were noted. Incidence of gall stone disease was observed in 103 males (out of 1149) and 3 females (out of 42) indicating the risk of the disease to be 9.0% and 7.1% respectively in males and females with GS. Conclusion Early recognition of GS by genetic analysis is required before these patients with intermittent episodes of jaundice run the risk of unnecessary operations on their bile ducts from the mistaken assumption ascribing the jaundice to a stone which has been left behind. [ABSTRACT FROM AUTHOR]

Background: Most patients with distal extrahepatic cholangiocarcinoma have developed jaundice or cholangitis at the time of initial diagnosis, which can delay surgery. We aim to evaluate the actual EB-RFA ablation volume and validated the clinical feasibility of preoperative endobiliary radiofrequency ablation (EB-RFA) for resectable distal extrahepatic cholangiocarcinoma. Methods: The medical records of patients who underwent EB-RFA from July 2016 to June 2017 at a single tertiary academic medical center were reviewed. Inclusion criteria were patients with resectable distal extrahepatic cholangiocarcinoma who required preoperative biliary decompression. Clinical outcomes of EB-RFA were reviewed retrospectively and the surgical specimens were reevaluated. Results: Of the eight patients who required a delayed operation, preoperative EB-RFA was successfully performed without serious complications including peritonitis, hemobilia, or perforation. Although curative resection was attempted in all patients, one patient underwent open and closure due to hepatic metastasis. Seven patients underwent curative surgical resection and the histology revealed that median maximal ablation depth was 4.0 mm (range, 1–6) and median effective ablation length (histological ablation length/fluorosocopic ablation length) was 72.0% (range, 42.1–95.3). Conclusions: EB-RFA partially ablated human cancer tissue and preoperative EB-RFA might be a safe and feasible in patients with distal extrahepatic cholangiocarcinoma who require a delayed operation. Ablation of the target lesion longer than the estimated length by fluoroscopy may improve the efficacy of EB-RFA. [ABSTRACT FROM AUTHOR]

Background: Afferent loop syndrome (ALS) is traditionally managed surgically and, more recently, endoscopically. The role of endoscopic ultrasound-guided entero-enterostomy (EUS-EE) has not been well described. The aim of this study was to assess the technical and clinical success and safety of EUS-EE.Methods: This was a multicenter, retrospective series at six centers in patients with ALS treated by EUS-EE. Data on patients treated with enteroscopy-assisted luminal stenting (EALS) at a single center were also collected.Results: 18 patients (mean age 64.2 years, 72 % post-pancreaticoduodenectomy, 10 female) underwent EUS-EE. The most common symptoms were vomiting (27.8 %) and jaundice (33.3 %). Clinical success included resolution of symptoms in 88.9 % and improvement to allow hospital discharge in 11.1 %. Technical success was achieved in 100 % of cases, with a mean procedure time of 29.7 minutes. The most common procedure was a gastro-jejunostomy (72.2 %). Three adverse events (16.7 %) occurred (two mild, one moderate). When compared with data on EALS, patients treated with EUS-EE needed fewer re-interventions (16.6 % vs. 76.5 %; P < 0.001).Conclusion: EUS-EE seems to be safe and effective in the treatment of ALS. Indirect comparison with EALS suggested that EUS-EE is associated with a reduced need for re-intervention. [ABSTRACT FROM AUTHOR]

Background For the determination of total bilirubin in serum the candidate reference method developed by Doumas et al. has international recognition. The primary standard SRM 916a (NIST) was recommended for use as the primary reference material for calibration. Nowadays, no primary standard is anymore commercially available. Further, a description of uncertainty components was missing. Methods Two reference laboratories have re-investigated the candidate reference measurement procedure. Beside minor modifications, mainly the use of a molar absorption coefficient instead of calibration by use of bilirubin standard solutions has facilitated the operating, and improved the analytical performance. All relevant sources of measurement uncertainty were investigated. Results A measurement range of 5–525 μmol/L and a CV of 0.5% to 1.4% (long term imprecision) were determined. Excellent agreement was obtained comparing to Doumas procedure ( r  = 0.9999) and during a two laboratory comparison participating at IFCC RELA ring trials (mean deviation: 0.6%). The combined expanded measurement uncertainty (probability 95%) for bilirubin concentrations >30 μmol/L was estimated as 2.2%. Conclusion A reference system for total bilirubin based on the described reference procedure shall enable metrological traceability and optimized standardization of the values obtained in clinical routine laboratories. [ABSTRACT FROM AUTHOR]

The porphyrias are a group of rare metabolic disorders that result from defects in heme biosynthesis. Erythropoietic protoporphyria (EPP) is the most common inherited porphyria in children and is diagnosed in most individuals after the onset of cutaneous manifestations. Hepatobiliary disease affects the minority of individuals with EPP and usually manifests in patients with an established diagnosis of EPP. We report on a classic but rare case of EPP that masqueraded as cholestasis. An 8-year-old boy was referred to the Hepatology Clinic after an abrupt onset of jaundice with a longstanding history of dermatitis. The diagnosis of EPP was established with liver biopsy, which revealed dense, dark-brown pigment in hepatocytes and Kupffer cells that, on polarization, displayed bright-red birefringence and centrally located Maltese crosses. Plasma total porphyrins and erythrocyte protoporphyrin were elevated and confirmed a diagnosis of EPP. We hope to raise awareness of this diagnosis among pediatricians, hepatologists, and pathologists and increase the consideration of EPP in patients with cholestatic liver disease and chronic dermatitis. [ABSTRACT FROM AUTHOR]

The presence of hemolysis (H), icterus (I) and lipaemia (L) in serum or plasma samples may be regarded as an important preanalytical drawback, since an abnormal concentration of these substances may derange the global quality and reliability of the total testing process. Although the assessment of sample quality before testing by means of the so-called HIL indices has become an essential part of routine practice in most clinical laboratories worldwide, no clear indications have been provided about the quality assurance of these measures. This article is hence aimed to provide an expert opinion about management of internal quality control (IQC) assessment for HIL indices. In summary, we support the use of in-house prepared IQC materials with at least two different levels for each interfering substances. IQC testing should be performed for validating performance of HIL indices in routine and urgent conditions, at least 2 times per day, and systematically recorded. We also suggest that results may be unacceptable when a single value of IQC material exceeds the mean target value by ±3 standard deviations. When results do not meet their set performance goals, the ensuing procedure mirrors that of conventional laboratory IQC management. [ABSTRACT FROM AUTHOR]

Alagille syndrome is a multisystem disorder classically involving the liver, heart, vertebrae, facial features, and the eyes. In this case report, we document a case of Alagille syndrome with an atypical clinical and histopathologic presentation and subsequent identification of a novel JAG1 missense mutation. This case highlights that there may be both atypical clinical and pathologic findings in mutation-proven Alagille syndrome and that the diagnosis of Alagille syndrome should be considered in cases of ongoing bile duct damage in the setting of early-onset jaundice, cholestasis, hepatosplenomegaly, posterior embryotoxon in the eyes, and butterfly vertebrae. [ABSTRACT FROM AUTHOR]

Self-explanation while diagnosing clinical cases fosters medical students' diagnostic performance. In previous studies on self-explanation, students were free to self-explain any aspect of the case, and mostly clinical knowledge was used. Elaboration on knowledge of pathophysiological mechanisms of diseases has been largely unexplored in studies of strategies for teaching clinical reasoning. The purpose of this two-phase experiment was to investigate the effect of self-explanation of pathophysiology during practice with clinical cases on students' diagnostic performance. In the training phase, 39 4th-year medical students were randomly assigned to solve 6 criterion cases (3 of jaundice; 3 of chest pain), either self-explaining the pathophysiological mechanisms of the findings (n = 20) or without self-explaining (n = 19). One-week later, in the assessment phase, all students solved 6 new cases of the same syndromes. A repeated-measures analysis of variance on the mean diagnostic accuracy scores showed no significant main effects of study phase (p = 0.34) and experimental condition (p = 0.10) and no interaction effect (p = 0.42). A post hoc analysis found a significant interaction (p = 0.022) between study phase and syndrome type. Despite equal familiarity with jaundice and chest pain, the performance of the self-explanation group (but not of the non-self-explanation group) on jaundice cases significantly improved between training and assessment phases (p = 0.035) whereas no differences between phases emerged on chest pain cases. Self-explanation of pathophysiology did not improve students' diagnostic performance for all diseases. Apparently, the positive effect of this form of self-explanation on performance depends on the studied diseases sharing similar pathophysiological mechanisms, such as in the jaundice cases. [ABSTRACT FROM AUTHOR]

Background: Febrile jaundice results clinically in generalized yellow coloration of the teguments and mucous membranes due to excess plasma bilirubin, accompanied by fever. Two types are found: conjugated and unconjugated bilirubin jaundice. Jaundice is a sign in several diseases due to viruses (viral hepatitis and arbovirus), parasites (malaria) and bacteria (leptospirosis). In the Central African Republic (CAR), only yellow fever is included on the list of diseases for surveillance. The aim of this study was to identify the other pathogens that can cause febrile jaundice, for better management of patients.Methods: Between 2008 and 2010, 198 sera negative for yellow fever IgM were randomly selected from 2177 samples collected during yellow fever surveillance. Laboratory analyses targeted four groups of pathogens: hepatitis B, C, delta and E viruses; dengue, chikungunya, Zika, Crimean-Congo haemorrhagic fever, West Nile and Rift Valley arboviruses; malaria parasites; and bacteria (leptospirosis).Results: Overall, 30.9% sera were positive for hepatitis B, 20.2% for hepatitis E, 12.3% for hepatitis C and 8.2% for malaria. The majority of positive sera (40.4%) were from people aged 16-30 years. Co-infection with at least two of these pathogens was also found.Conclusion: These findings suggest that a systematic investigation should be undertaken of infectious agents that cause febrile jaundice in the CAR. [ABSTRACT FROM AUTHOR]

Background/aim Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening disorder of extreme inflammation and unregulated immune response which require prompt recognition and early introduction of definitive therapy. HLH can present with wide range of hepatic dysfunction ranging from mild elevation of transaminases to liver failure. This study is carried out to describe the clinical and laboratory presentation of HLH. Methods Patients who were diagnosed with HLH between January 2013 and December 2015 were retrospectively included in this study. Results Six patients were diagnosed as secondary HLH with median age of 28.5 years at diagnosis. All patients were presented with history of deep jaundice and high grade fever with pancytopenia and splenomegaly. Underlying diagnosis was viral infections in 4 and probable viral infection in remaining two. Bone marrow hemophagocytosis was present in 3 cases. Three patients were treated with corticosteroids only and one each with corticosteroids with cyclosporine or intravenous immunoglobulin (IVIG) and HLH treatment protocol. One patient died due to acute respiratory distress syndrome (ARDS); another patient died in follow-up due to respiratory failure due to pneumonia. Conclusions HLH is rare and potentially life-threatening cause of prolonged fever, jaundice and pancytopenia. Early diagnosis and initiation of specific therapy can improve clinical outcome. [ABSTRACT FROM AUTHOR]

The article focuses on a study regarding evidence for a gene conversion in a Hb Arya Carrier. It mentions that adult hemoglobin (HbA) is a tetramer formed by the association of α and β chains (α2β2) which was encoded onto different loci. It also mentions that α genes encode for an identical amino acid sequence and arise from a common ancestor.

Bilirubin, a key biomarker for the jaundice and its clinical diagnosis needs a better analytical tool. A novel and simple fluorescent platform based on (2,2′-((1E,1′E)-((6-bromopyridine-2,3-diyl) bis(azanylylidene)) bis(methanylylidene diphenol) ( BAMD) was designed. BAMD showed a remarkable fluorescent intensity with a very good quantum yield of 0.85 and lifetime of 870 ps. Hence, it was applied for the determination of bilirubin using both colorimetric and fluorimetric techniques in physiological and basic pH. Under optimized experimental conditions, the probe detects bilirubin selectively in the presence of other interfering biomolecules and metal ions. The linear range of detection is 1 pM–500 µM at pH=7.4 and LOD is 2.8 and 3.3 pM at pH=7.4 and 9.0, respectively, which were reported so far. The probe detects the bilirubin through FRET mechanism. The practical application of the probe was successfully tested in the human blood and urine samples. Based on all above advantages, this simple idea can be applied to design a simple clinical diagnostic tool for jaundice. [ABSTRACT FROM AUTHOR]

Ethnopharmacological relevance Liver disorders are a major health problem in Cambodia, where some patients prefer to seek treatment from traditional healers. The aim of the study was to document the knowledge and practices of these healers in four Southern Cambodian provinces. Materials and methods An ethnopharmacological survey was carried out from September 2015 to January 2016 in Cambodian urban and rural areas. Thirty-three Khmer traditional healers (KTH) were interviewed using a semi-structured questionnaire including socio-demographic data, healer's formation and their professional practice conditions, perception of liver diseases (types and causes of liver disorders, diagnostic methods and symptoms of liver problems), dietary recommendations given to patients, and herbal remedies used to treat them. For each medicinal plant mentioned in herbal remedies, the local name, part of the plant, mode of preparation and administration, and their properties, according to the healers, were recorded. The plants mentioned by the traditional therapists were collected and later identified by specialists. Results Different types of liver disease are identified by the healers, and diagnosis was mostly based on reading medical records, and by observing the yellow discoloration of the skin and eyes. A total of 42 herbal remedies including 83 medicinal plants belonging to 40 families were mentioned for treating liver disorders. The most predominant families were Leguminosae and Poaceae. Among the plants reported, Cananga latifolia , Andrographis paniculata , Smilax aff. glabra , Gomphrena celosioides, Passiflora foetida and Physalis minima were the most cited species. A large part of the herbal remedies used were multi-ingredient recipes, and were prepared mainly by a decoction administered orally. Plants are combined in multi-ingredient recipes, and selected on the basis of their properties ( trocheak , psah , somrap mé rok , ktchol ) which originate from Khmer medical concepts. Most of the plants used by healers have a wide ethnobotanical use for liver disorders, and have been studied for their hepatoprotective activity and related activities on the liver. Conclusion In the diagnosis and treatment of liver diseases, KTH have incorporated biomedical concepts and new practices, which suggest that they could be defined as neotraditional healers. Medicinal plants constitute the core of traditional medicine practice by these healers, and these plants play a very important role in the health care of people with liver problems in Cambodia. Therefore, more attention should be paid to the integration of healers in national health care programs for the development of combined therapies. Furthermore, two plant species (i.e. Cananga latifolia and Willughbeia edulis ) were found to be widely used for treating liver disorders in our survey, and should be studied for their pharmacological potential for liver problems. [ABSTRACT FROM AUTHOR]

Jaundice in adults can be an indicator of significant underlying disease. It is caused by elevated serum bilirubin levels in the unconjugated or conjugated form. The evaluation of jaundice relies on the history and physical examination. The initial laboratory evaluation should include fractionated bilirubin, a complete blood count, alanine transaminase, aspartate transaminase, alkaline phosphatase, ?-glutamyltransferase, prothrombin time and/or international normalized ratio, albumin, and protein. Imaging with ultrasonography or computed tomography can differentiate between extrahepatic obstructive and intrahepatic parenchymal disorders. Ultrasonography is the least invasive and least expensive imaging method. A more extensive evaluation may include additional cancer screening, biliary imaging, autoimmune antibody assays, and liver biopsy. Unconjugated hyperbilirubinemia occurs with increased bilirubin production caused by red blood cell destruction, such as hemolytic disorders, and disorders of impaired bilirubin conjugation, such as Gilbert syndrome. Conjugated hyperbilirubinemia occurs in disorders of hepatocellular damage, such as viral and alcoholic hepatitis, and cholestatic disorders, such as choledocholithiasis and neoplastic obstruction of the biliary tree. [ABSTRACT FROM AUTHOR]

Background: Outcomes in biliary atresia (BA) have been well-documented in large national cohorts from Europe, North America, and East Asia. Understanding the challenges that preclude success of the Kasai portoenterostomy (KPE) is the key to improve the overall outcomes of BA and implementing intervention strategies. Here, we analyzed the data from the Saudi national BA study (204 BA cases diagnosed between 2000 and 2018) to identify the prognostic factors of BA outcomes., Methods: One hundred and forty-three cases underwent KPE. Several prognostic factors (center case load, congenital anomalies, serum gamma-glutamyl transferase, use of steroids, ascending cholangitis post-operatively, and degree of portal fibrosis at time of KPE) were investigated and correlated with the primary outcomes of interest: 1) success of KPE (clearance of jaundice and total serum bilirubin <20 mmol/l after KPE), 2) survival with native liver (SNL), and 3) overall survival., Results: Use of steroids after KPE was associated with clearance of jaundice, 68% vs. 36.8% in the BA cases that did not receive steroids (P = 0.013; odds ratio 2.5) and a significantly better SNL rate at 2 - and 10-year of 62.22% and 57.77% vs. 39.47% and 31.57%, respectively (P = 0.01). A better 10-year SNL was observed in centers with caseload <1/year (group 1) as compared to centers that performed ≥1/year (group 2) [45.34% vs. 26.66%, respectively; P = 0.047]. On comparison of the 2 groups, cases in group 1 had KPE at significantly earlier age (median 59.5 vs. 75 days, P = 0.006) and received steroids after KPE more frequently than group 2 (69% vs. 31%, P < 0.001). None of the remaining prognostic variables were identified as being significantly related to BA outcome., Conclusion: Steroids use post-KPE predicted clearance of jaundice and better short- and long-term SNL. There is a need to establish a national BA registry in Saudi Arabia aiming to standardize the pre- and post-operative clinical practices and facilitate clinical and basic research to evaluate factors that influence BA outcome., Competing Interests: None