Interpretation of the 2022 American Academy of Pediatrics guidelines for the management of hyperbilirubinemia in newborn infants / 中国当代儿科杂志

The American Academy of Pediatrics updated the guidelines for the management of hyperbilirubinemia in the newborn infants with a gestational age of ≥35 weeks in September 2022. Based on the evidence over the past 18 years, the guidelines are updated from the aspects of the prevention, risk assessment, intervention, and follow-up of hyperbilirubinemia in the newborn infants with a gestational age of ≥35 weeks. This article gives an interpretation of the key points in the guidelines, so as to safely reduce the risk of bilirubin encephalopathy and unnecessary intervention.

Análise macroscópica e radiográfica de dentes decíduos com hiperbilirrubinemia de crianças com doenças hepáticas crônicas e transplante hepático / Macroscopic and radiographic analysis of primary teeth with hyperbilirubinemiain children with chronic liver disease and liver transplantation

Objetivo: identificar mediante análise macroscópica e ra-diográfica as alterações estruturais em tecidos dentários afetados pela hiperbilirrubinemia, normalmente constatada a partir do sinal clínico de icterícia e provoca o desenvolvi-mento de pigmentos intrínsecos esverdeados nas estruturas dentárias. Materiais e Métodos: a amostra foi composta por 31 dentes decíduos dividida em grupo controle (n= 7) e grupo experimental (n= 24). As análises macroscópicas foram efetuadas por meio de fotografias individuais padroniza-das e as radiográficas obtidas com aquisições de imagem a 9 mA, 70 kVp, distância de 8cm, exposição 0,4 segundos e com XDR Sensor®. As imagens foram convertidas pelo software XDR Brasil 3.1.6 e padronizadas pelo programa GIMP 2.10.22. Os dados da média simples do histograma foram analisados pelo teste T-Student e Mann-Whitney (p<0,05). Resultados: demonstraram a maior intensidade de pigmentação em região cervical da raiz, com diferença de densidade radiográfica estaticamente significante na porção radicular entre os grupos experimental e controle (p=0,043). Na análise da densidade radiográfica da estrutura radicular do grupo experimental houve diferença estatica-mente significante (p=0,016) entre os terços cervical e apical. Discussão: Os dados evidenciaram que dentes pigmentados pela hiperbilirrubinemia não possuem alterações na densidade mineral nos terços coronários. Conclusão: Dentes com pigmentação esverdeada bilirrubina possuem diferenças na densidade radiográfica so-mente na região radicular.

Aim: is to identify, through macroscopic and radio-graphic analysis, structural changes in dental tissues affected by hyperbilirubinemia, usually seen from the clinical sign of icterus and causes the development of intrinsic greenish pigments in dental structure. Materials and Methods: The sample consisted of 31 primary teeth divided into a control group (n=7) and an experimental group (n=24). Macroscopic analyzes were performed using standardized individual photographs and radiographic ones obtained by image acquisition at 9 mA, 70 kVp, 8cm distance, 0.4 seconds exposure and with XDR Sensor®. The images were converted by XDR Brasil 3.1.6 software and standardized by GIMP 2.10.22 software. The Histogram's simple mean data were analyzed by T-Student and Mann-Whitney tests (p<0.05). Results: showed intensity of pigmentation in the cervical region of the root, with a statistically significant difference in the root portion between the experimental and control groups (p=0.043. In the analysis of radiographic density of the root structure of the experimental group, there was a statistically significant difference (p= 0.016) between the cervical and apical thirds. Discussion: The data showed that teeth pigmented by hyperbilirubinemia do not have changes in mineral density in the coronary thirds. Conclusion: Greenish pigments teeth have differences in radiographic density only in the root structure.

Hiperbilirrubinemia: visión del patólogo / Hyperbilirubinemia: pathologist's view

Los niveles de bilirrubina sérica normal en el adulto varían entre 0,3 mg/dL y 1,2 mg/dL, y su valor está determinado por la tasa de captación hepática, conjugación y excreción. La ictericia se hace evidente cuando los niveles de bilirrubina sérica se elevan por encima de 2,5 mg/dL a 3 mg/dL, siendo un indicador de enfermedad subyacente. La bilis es producida por los hepatocitos y fluye desde los canalículos, canales de Hering, conductos biliares intrahepáticos, conductos hepáticos derechos e izquierdos hasta llegar al duodeno. A nivel histopatológico, cualquier entidad que lleve a la acumulación intrahepática de bilis por disfunción hepatocelular u obstrucción biliar genera colestasis, que se observa en la biopsia hepática como la acumulación de tapones de color marrón verdoso de pigmento biliar en los hepatocitos, y secundariamente se observan los canalículos dilatados. Las causas de colestasis intrahepática son diversas e incluyen enfermedades como colangitis biliar primaria, colangitis esclerosante primaria, hepatitis autoinmune, hepatitis virales y toxicidad medicamentosa. Esta revisión tiene como objetivo analizar algunos tipos de hiperbilirrubinemia, resaltando sus características histopatológicas.

Normal serum bilirubin levels in adults range from 0.3 mg/dL to 1.2 mg/dL, and its value is determined by the rate of hepatic uptake, conjugation, and excretion. Jaundice becomes apparent when serum bilirubin levels rise above 2.5 mg/dL to 3.5 mg/dL and is an indicator of underlying disease. Bile is produced by hepatocytes and flows from the canaliculi, Hering's canals, intrahepatic bile ducts, and right and left hepatic ducts to the duodenum. Pathologically, any condition that leadsto intrahepatic accumulation of bile due to hepatocellular dysfunction or biliary obstruction, generates cholestasis, which is observed in liver biopsy as the accumulation of greenish-brown deposits of bile pigment in hepatocytes, with dilated canaliculi. The causes of intrahepatic cholestasis are diverse and include diseases such as primary biliary cholangitis and primary sclerosing cholangitis, autoimmune hepatitis, viral hepatitis, and drug toxicity. This review aims to analyze some types of hyperbilirubinemia, highlighting their histopathological characteristics.

Investigation of risk factors related to the development of hepatic dysfunction in patients with a low and moderate cardiac risk during open-heart surgeries

Abstract Objective: To determine the possible risk factors associated with hepatic dysfunction during open-heart surgeries. Methods: After excluding 71 patients, 307 patients with possible low and moderate cardiac risk who underwent either coronary artery bypass graft surgery (CABG) (n=176) or valve repair surgery (mitral valve, mitral and aortic valves and/or tricuspid valve) (n=131) were investigated prospectively during a 6-month period. Hyperbilirubinemia is defined as an occurrence of a plasma total bilirubin concentration >34 µmol/L (2 mg/dL) in any measurement during the postoperative period; the patients were divided into groups with or without postoperative hyperbilirubinemia. The collected parameters were: alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP), total bilirubin (TBil), gamma-glutamyl transpeptidase (GGT) and albumin. The parameters were collected preoperatively and postoperatively on days 1, 3 and 7. Preoperative, intraoperative, and postoperative risk factors were investigated. Logistic regression analysis was performed to identify the risk factors for postoperative hyperbilirubinemia. Results: Postoperative hyperbilirubinemia was observed in 7 of 176 patients (4%) who underwent CABG, and in 11 of 131 patients (8.4%) who underwent valve replacement surgeries. Independent risk factors for early postoperative hyperbilirubinemia were found as: ejection fraction (EF), aortic cross-clamp (ACC) time, intensive care unit stay and extubation time (P<0.001). In comparison to CABG procedures, postoperative hyperbilirubinemia was observed more frequently in patients undergoing valve surgeries (P=0.027). Conclusion: Low EF and prolonged ACC time are significant independent risk factors for early postoperative hyperbilirubinemia during open-heart surgeries with cardiopulmonary bypass. Valve surgeries show a higher incidence of hyperbilirubinemia in comparison to CABG.

Dientes primarios verdes por hiperbilirubinemia / Dentes decíduos verdes por hiperbilirubinemia / Green deciduous teeth due to hyperbilirubinemia

Definida como una concentración sérica de bilirrubina, la hiperbilirrubinemia es una condición rara y puede ser causada por cualquier factor que eleve la carga de bilirrubina a ser metabolizada por el hígado. Así, la bilirrubina se distribuye por los tejidos corporales y se acumula en los tejidos duros, incluso, en los dientes primarios. El objetivo de este trabajo es relatar un caso clínico sobre la repercusión severa de la hiperbilirrubinemia en dientes primarios y sus manifestaciones bucales en la primera infancia, como dientes verdosos. Paciente del sexo masculino, 3 años y 8 meses, estuvo en clínica odontológica para evaluación de "manchas verdes en los dientes". En su historia médica se observó que nació prematuro y tuvo complicaciones posparto que evolucionaron para sepsis Dientes primarios verdes por hiperbilirrubinemiaIvana Aguiar Raposo,1..... Ronilza Matos,2.....• Marta Verônica Souto de Araujo,3..... •Tamara Kerber Tedesko,4.....José Carlos Pettorossi Imparato,5..... Revista de Odontopediatría Latinoamericana¹ São Leopoldo Mandic Campinas, Sao Paulo, Brasil.2 Universidade Paulista. 3 Universidade Federal de Pernambuco.4 Universidade Federal de Santa Maria.5 Universidade São Paulo - USP.Casos ClínicosDOI: 10.47990/alop.v11i2.304y, como consecuencia del uso de varios medicamentos, desarrolló un cuadro de hiperbilirrubinemia. El examen clínico dental constató la presencia de manchas verdes en los 20 dientes primarios, ausencia de lesión de caries, encías sanas y buena condición de higiene bucal. Se concluye que los altos niveles de bilirrubina desarrollados por el niño prematuro asociados a septicemia pueden llevar a la pigmentación verde (coloración intrínseca) en los dientes primarios durante el periodo de calcificación de los mismos (odontogénesis), incluso a la afectación de los dientes permanentes. Por eso, es relevante el conocimiento de la historia médica del niño desde el nacimiento para el establecimiento del diagnóstico y pronóstico de las alteraciones dentales

Defined as a serum bilirubin concentration, hyperbilirubinemia is a rare condition and can be caused by any factor that raises the bilirubin load to be metabolized by the liver. Thus, bilirubin is distributed throughout the body's tissues and accumulates in hard tissues, including primary teeth. The aim of this work is to report a clinical case about the severe repercussion of hyperbilirubinemia in primary teeth, and its early childhood manifestations, such as greenish teeth. A male patient, 3 years and 8 months old, attended the dental clinic for evaluation of "green spots on teeth". Medical history revealed that the child had premature birth and postpartum complications that evolved into sepsis, and due to the use of several drugs, developed hyperbilirubinemia. The dental clinical examination showed the presence of green spots on all 20 deciduous teeth, absence of carious lesions, healthy gums and good oral hygiene conditions. We conclude

Association between the inter-aural latency difference of brainstem auditory evoked potential wave V and neonatal hyperbilirubinemia / 中国当代儿科杂志

OBJECTIVE@#To study brainstem auditory evoked potential (BAEP) in neonates with hyperbilirubinemia using short auditory stimuli (60 dBnHL), and to investigate the differences in the inter-aural latency difference (ILD) of wave V between neonates with different total serum bilirubin (TSB) levels.@*METHODS@#A prospective study was conducted in neonates with hyperbilirubinemia who were admitted to the Department of Neonatology, Yuhuan People's Hospital of Zhejiang Province, from May 2019 to October 2020. The neonates were divided into a severe group (@*RESULTS@#Compared with the mild group, the severe group had significantly higher proportions of neonates with abnormal hearing threshold and abnormal ILD (@*CONCLUSIONS@#Serum bilirubin in neonates affects the ILD of BAEP wave V, especially in those with severe hyperbilirubinemia. ILD at the optimal cut-off value of ≥0.4 ms shows potential value in the diagnosis of hearing impairment caused by neonatal hyperbilirubinemia.

Effectiveness of Saccharomyces boulardii combined with phototherapy in the treatment of hyperbilirubinemia in neonates: a prospective randomized controlled trial / 中国当代儿科杂志

OBJECTIVE@#To study the effectiveness of Saccharomyces boulardii combined with phototherapy in the treatment of hyperbilirubinemia in neonates.@*METHODS@#The neonates with hyperbilirubinemia who were hospitalized from January to December 2018 were enrolled and randomly divided into an observation group (n=61) and a control group (n=63). The neonates in the observation group were treated with phototherapy combined with Saccharomyces boulardii, and those in the control group were treated with phototherapy combined with placebo. Treatment outcomes were compared between the two groups. Fecal samples were collected 72 hours after treatment and 16s rRNA high-throughput sequencing was used to compare the features of gut microbiota between the two groups.@*RESULTS@#There was no significant difference in the total serum bilirubin level between the two groups before treatment (P>0.05). At 24, 48, and 72 hours after treatment, the observation group had a significantly lower level of total serum bilirubin than the control group (P<0.05). Compared with the control group, the observation group had a significantly lower proportion of neonates requiring phototherapy again [20% (12/61) vs 75% (47/63), P<0.05]. Compared with the control group, the observation group had a significantly higher abundance of Bacteroides (P<0.05) and a significantly lower abundance of Escherichia coli and Staphylococcus in the intestine at 72 hours after treatment (P<0.05).@*CONCLUSIONS@#In neonates with hyperbilirubinemia, phototherapy combined with Saccharomyces boulardii can effectively reduce bilirubin level and prevent the recurrence of jaundice. Saccharomyces boulardii can favour the treatment outcome by regulating the gut microbiota of neonates.

Hyperbilirubinemia impact on newborn hearing: a literature review

SUMMARY The increase in bilirubin levels in newborns can cause toxic effects on the auditory system, which can lead to hearing loss. This review aimed to verify the impact of hyperbilirubinemia in the hearing of newborns, relating audiological findings to serum levels of bilirubin. A literature review was conducted during October 2017, using the terms "hyperbilirubinemia", "jaundice", "infant", "newborn" and "hearing loss", on databases CAPES journals, MEDLINE and BIREME (SciELO, BBO). 827 studies were identified and 59 were selected for full-text reading, resulting in the selection of seven articles that met the inclusion criteria and were considered relevant to the sample of this study. All the reviewed studies performed brainstem auditory evoked potential as the main test for audiological evaluation. Changes in the audiological findings of neonates with hyperbilirubinemia were observed in all studies. There was no consensus on the serum bilirubin levels that may cause auditory changes; however, the relationship between hearing disorders and blood levels of bilirubin was positive. We identify the need to establish reference values for bilirubin levels considered critical for the occurrence of hearing disorders as well as the audiological follow-up of neonates with hyperbilirubinemia.

RESUMO O aumento nos níveis de bilirrubina no neonato pode provocar efeitos tóxicos no sistema auditivo, podendo levar à perda auditiva. O objetivo desta revisão foi verificar o impacto da hiperbilirrubinemia na audição de recém-nascidos, relacionando os achados audiológicos aos níveis séricos de bilirrubina. Realizou-se uma revisão sistemática de literatura durante o mês de outubro de 2017, utilizando-se os termos hyperbilirubinemia, jaundice, infant, newborn e hearing loss, nas bases de dados periódicos Capes, Medline e Bireme (SciELO, BBO). Foram identificados 827 estudos, dentre os quais 59 foram selecionados para leitura do texto na íntegra, resultando na seleção de sete artigos que atendiam aos critérios de inclusão e foram considerados relevantes para a amostra deste trabalho. Em todas as pesquisas revisadas, o potencial evocado auditivo de tronco encefálico foi o principal exame audiológico realizado. Em todos os estudos foram observadas alterações nos resultados audiológicos de neonatos com hiperbilirrubinemia. Não houve consenso quanto aos níveis séricos de bilirrubina que podem causar alterações auditivas, porém, a relação entre as alterações audiológicas e os níveis sanguíneos de bilirrubina foi positiva. Percebeu-se a necessidade de estabelecer valores de referência para os níveis de bilirrubina considerados críticos para a ocorrência de alterações audiológicas, assim como de acompanhamento audiológico dos neonatos com hiperbilirrubinemia.

Genetic Spectrum of UGT1A1 in Korean Patients with Unconjugated Hyperbilirubinemia

No abstract available.

Colestasis en el adulto: enfoque diagnóstico y terapéutico. Revisión de tema / Diagnostic and therapeutic approach for cholestasis in the adult

Resumen La colestasis es uno de los motivos de consulta más frecuentes en hepatología. Se genera por una alteración en la síntesis, la secreción o el flujo de la bilis, a través del tracto biliar. Esta se define por una elevación de enzimas como la fosfatasa alcalina (Alkaline Phosphatase, ALP) y la gamma-glutamil transferasa, y en estadios tardíos con la hiperbilirrubinemia, al igual que con otras manifestaciones clínicas, tales como el prurito y la ictericia. El enfoque diagnóstico implica establecer el origen de dicha elevación, determinando si es intrahepática o extrahepática. Si es intrahepática, se debe esclarecer si proviene de los hepatocitos o de la vía biliar de pequeño y de gran calibre. El tratamiento dependerá de la etiología, por lo cual es importante un diagnóstico preciso. En esta revisión se presenta la fisiopatología y un enfoque diagnóstico y terapéutico.

Abstract Cholestasis is one of the most frequent reasons for hepatology consultation. It is generated by altered synthesis, secretion or flow of bile through the biliary tract and is defined by elevated levels of enzymes such as alkaline phosphatase and gamma glutamyl transferase. In late stages, hyperbilirubinemia and clinical manifestations such as pruritus and jaundice develop. The diagnostic approach involves establishment of the reasons for elevated enzyme levels and determination of whether it is intrahepatic or extrahepatic. If it is intrahepatic, the source must be determined (hepatocytes, small bile ducts, or large caliber bile ducts). Treatment depends on the etiology, so accurate diagnosis is important. This review presents the pathophysiology and a diagnostic and therapeutic approach.

Bilirrubina: metabolismo, pruebas de laboratorio e hiperbilirrubinemia / Bilirubin: metabolism, laboratory tests and hyperbilirubinemia

Resumen La bilirrubina es el producto final de la degradación del grupo hem. La bilirrubina no conjugada (BNC) se forma en las células retículoendoteliales, transportada al hígado, donde es conjugada a glucurónidos y secretada a los canalículos. La BNC se solubiliza en el suero por medio de su fuerte unión con la albúmina. La unión bilirrubina-albúmina es una función de las concentraciones de la albúmina y de la bilirrubina y de la afinidad de unión por la bilirrubina. La fracción de bilirrubina no unida o bilirrubina libre plasmática (Bf) se incrementa significativamente conforme el nivel de bilirrubina sérica total (BST) alcanza la capacidad de unión de la albúmina. La Bf es considerada un mejor indicador de neurotoxicidad que la BST, a causa de que solamente la bilirrubina libre puede cruzar la barrera hematoencefálica. En la práctica médica la bilirrubina es un marcador de disfunción hepática, colestasis o enfermedad hemolítica. Una variedad de factores limita la sensibilidad y la especificidad de la medición de la bilirrubina para detectar anormalidades: lipemia, hemólisis, exposición a la luz visible y el estado de ayuno. La hiperbilirrubinemia puede ser clasificada como prehepática, hepática y poshepática, y esto brinda un marco útil para identificar la causa subyacente. Además, hay bilirrubina conjugada y no conjugada. La hiperbilirrubinemia y la ictericia neonatales se presentan en casi todos los recién nacidos y puede ser benigna si su progresión a hiperbilirrubinemia es reconocida, monitoreada y prevenida o tratada en una manera oportuna.

Abstract Bilirubin is the end product of heme breakdown. Unconjugated bilirubin (UB) is formed in reticuloendothelial cells, transported to the liver where it is conjugated to glucuronides, and then secreted into the canaliculi. UB is solubilized in serum via very tight linkage to albumin. Bilirubin-albumin binding is a function of the concentration of bilirubin and albumin and the binding affinity for bilirubin. The fraction of unbound bilirubin or plasma free bilirubin (Bf) increases significantly as the total serum bilirubin (TSB) level approaches the binding capacity of albumin. Bf is thought to be better indicator of neurotoxicity than TSB, because only plasma free bilirubin can cross the blood-brain barrier. In medical practice bilirubin is a marker of liver dysfunction, cholestasis or hemolytic disease. A variety of factors limit both the sensitivity and the specificity of bilirubin measurement to detect the abnormalities: lipemia, hemolysis, exposure of visible light and fasting state. Hyperbilirubinemia can be categorised as prehepatic, hepatic or poshepatic, and this provides a useful framework for identifying the underlying cause. In addition, there are conjugated and unconjugated bilirubin. Neonatal hyperbilirubinemia and jaundice occur in almost all newborns and may be benign if its progression to extreme hyperbilirubinemia is recognized, monitored and prevented or managed in a timely manner.

Aplicación del bilirrubinómetro no invasivo en recién nacidos / Application of non-invasive bilirubinometry in newborns

Introducción: Las pautas para prevención y tratamiento de hiperbilirrubinemia neonatal recomiendan medición de bilirrubina sérica total (BST) o bilirrubina transcutánea (BTc) para determinar el grado de ictericia antes del alta del recién nacido (RN); ésta última no invasiva, proporciona información instantánea y de calidad superior a la evaluación clínica. A pesar de ello aún no ha sido aplicada en forma sistemática en los hospitales de Paraguay. Objetivo: evaluar la aplicación en nuestro medio de la medición de bilirrubinemia transcutánea antes del alta correlancionando con la bilirrubina sérica. Materiales y Métodos: Estudio observacional, descriptivo con componente analítico, de corte transversal. Fueron incluidos RN con edad gestacional ≥ a 35 semanas, con peso ≥ a 2000 gramos, luego de las 24 hs de vida hasta los 8 días; bajo consentimiento informado de los padres, durante un año. Los datos fueron consignados en una planilla de Microsoft Excel y procesado por el software IBM SPSS Statistics ®. Resultados: De 271 RN que ingresaron al estudio, en la primera medición con el Bilirrubinómetro transcutáneo, cumplían con criterios para toma de bilirrubina sérica 90 (33,2%) de ellos. En los restantes 181 RN (66,8%), los datos emparejados no estaban disponibles debido a que siguiendo las recomendaciones de las guías actuales no fue necesario medir la bilirrubina sérica. El valor del coeficiente de correlación para la primera medición fue r = 0.574. Para la segunda medición las medidas emparejadas estaban disponibles para 131 RN. En este caso se encontró correlación positiva entre ambos métodos de 0,590. Conclusión: La bilirrubina transcutánea puede utilizarse en forma rápida, segura y válida, como un test de screening para la detección de hiperbilirrubinemia y podría evitar una proporción importante de toma de muestras sanguíneas, mejorando la seguridad del paciente.

Introduction: The guidelines for prevention and treatment of neonatal hyperbilirubinemia recommend measurement of total serum bilirubin (BST) or transcutaneous bilirubin (BTc) to determine the degree of jaundice before discharge of the newborn (NB); the latter non-invasive method provides instant information which is superior to the clinical evaluation. Despite this, it has not yet been systematically applied in hospitals in Paraguay. Objective: to evaluate transcutaneous measurement of bilirubin concentration as compared to serum bilirubin levels prior to discharge in our setting. Materials and Methods: This was an observational, cross-sectional, descriptive study with an analytical component. For a period of one year, we tracked NBs with a gestational age ≥ 35 weeks, weighing ≥ 2000 grams, from 24 hours of life until 8 days of life, obtaining the informed consent of the parents. The data was entered in a Microsoft Excel spreadsheet and processed by the IBM SPSS Statistics ® software. Results: Of 271 NBs who entered the study, 90 (33.2%) met criteria for measurement of serum bilirubin at their first measurement with the transcutaneous bilirubinometer. In the remaining 181 RN (66.8%), the paired data were not available as measurement of serum bilirubin was not required per the recommendations of current guidelines. The correlation coefficient value for the first measurement was r = 0.574. For the second measurement, paired measurements were available for 131 NBs. In this case, a positive correlation was found between both methods of 0.590. Conclusion: Transcutaneous bilirubin can be used quickly, safely and accurately as a screening test for the detection of hyperbilirubinemia and could avoid a significant proportion of blood sampling, improving patient safety.

Factors associated with clinical outcomes among neonates admitted with acute bilirubin and hypoxic-ischaemic encephalopathies at a tertiary hospital in south-west Nigeria

Background:Babies who are delivered outside hospital are most at risk of serious illnesses such as perinatal asphyxia and severe hyperbilirubinaemia. These conditions are major contributors to neonatal mortalities in resource-poor settings. Objective: To explore the relationship between pre-admission and intra-facility care and immediate outcomes among neonates with acute bilirubin and hypoxic-ischaemic encephalopathies. Methods: Using a retrospective design, the outcome of outborn babies with acute bilirubin encephalopathy (ABE) and hypoxic-ischaemic encephalopathy (HIE) were studied in a Nigerian hospital between 2012 and 2016. Results: A total of 40 and 80 babies with ABE and HIE were studied. Among babies with ABE, 67.5% arrived at the hospital on self-referral and of the babies with official referral, only 61.5% had had a serum bilirubin check prior to referral. Among the babies with ABE, 25.0% had both social and facility-related challenges, 45.0% had only facility-related challenges and 20.0% had only social challenges. All the babies with ABE who died had either social or facility-related challenges. For the babies with HIE, 56.2% came on self-referral while 70% received no care prior to presentation at the hospital. Some 40% of babies with HIE had both social and facility-related challenges while 12.5% had only facility-related challenges. None of the babies who died presented early. Comparable proportions of babies who died or survived had social challenges and facility-related challenges. Conclusion: Most of the outborn babies with HIE and ABE who arrived at the hospital on self-referral and facility-based care were hindered by social issues and facility-related challenges

The use of locally fabricated compact fluorescent lamps (CFL) phototherapy devices in the management of significant neonatal hyperbilirubinaemia

Abstract: One of the challenges of managing significant neonatal hyperbilirubinaemia in resource-poor settings is limited access to effective phototherapy devices hence the need to try appropriate technologies. The objective was to assess the effectiveness of Compact Fluorescent Lamps phototherapy devices in the management of significant neonatal hyperbilirubinaemia in a resource-poor setting. A cohort of babies with significant hyperbilirubinaemia managed with locally fabricated Compact Fluorescent Lamps (CFL) phototherapy devices (2014-2016) were compared with historical controls managed with conventional imported phototherapy devices (2007-2010) for the severity of hyperbilirubinaemia and requirements for Exchange Blood Transfusion. A total of 96 babies in the subject group and 202 babies in the control group were studied. The proportion of babies with peak TSB >30mg/dl was significantly higher among the controls compared to the subjects (p < 0.001). The interval between the commencement of phototherapy and the peak TSB was greater than 1 day among 30.2% (29/96) subjects compared to 74.3% (150/202) babies in the control group (p < 0.001). The mean duration of phototherapy was significantly shorter for the subjects compared to the controls. EBT was performed for 38.5% of the subjects and 51.5% of the controls. Single sessions of EBT were required for 78.4% of the subjects compared to 45.2% of the controls (p = 0.001). In conclusion, the locally fabricated Compact Fluorescent Lamps phototherapy devices reduced EBT rate among babies with significant hyperbilirubinaemia

A 2-day-old neonate with hyperbilirubinemia and thrombocytopenia / 中国当代儿科杂志

A girl was admitted to the hospital on day 2 after birth due to jaundice of the skin and sclera for half a day. The main clinical manifestations were persistent severe jaundice and thrombocytopenia, and she was finally diagnosed with congenital thrombotic thrombocytopenic purpura (TTP). Her conditions were improved after phototherapy, exchange transfusion, and infusion of fresh frozen plasma, red blood cells, and platelets. Gene detection showed a homozygous mutation of c.3650T>C(p.I1217T) in the ADAMTS13 gene, while her parents had a heterozygous mutation at this locus. Congenital TTP is a rare autosomal recessive disease, and timely infusion of fresh frozen plasma can achieve a good clinical outcome. This is the first case of congenital TTP caused by homozygous mutation at this locus reported in China and overseas.

Effect of red blood cell storage duration on the clinical effect of exchange transfusion and internal environment in neonates with hyperbilirubinemia / 中国当代儿科杂志

OBJECTIVE@#To study the effect of red blood cell (RBC) storage duration on the clinical effect of exchange transfusion (ET) and internal environment in neonates with hyperbilirubinemia.@*METHODS@#A retrospective analysis was performed for the clinical data of 135 neonates with hyperbilirubinemia who received ET between January 2015 and August 2018. According to RBC storage duration, the neonates were divided into short-term storage group (RBCs were stored for ≤7 days) with 56 neonates and long-term storage group (RBCs were stored for >7 days) with 79 neonates. The two groups were compared in terms of serum total bilirubin (TBIL) level and the rate of TBIL reduction at 0 and 12 hours after ET, as well as the duration of continued phototherapy and rate of repeated ET. Routine blood test parameters, electrolytes, blood glucose, and blood gas parameters were measured before ET and at 0 hour after ET.@*RESULTS@#At 0 hour after ET, there were no significant differences in the TBIL level and the rate of TBIL reduction between the two groups (P>0.05). At 12 hours after ET, the long-term storage group had a significantly higher TBIL level and a significantly lower rate of TBIL reduction than the short-term storage group (P7 days in ET for neonates with hyperbilirubinemia does not affect the immediate effect of ET, but these neonates tend to have a poor outcome after continued phototherapy and high risk of hyponatremia, hyperkalemia, and metabolic acidosis.

Sustained erroneous near-infrared cerebral oxygen saturation in alert icteric patient with vanishing bile duct syndrome during and after liver transplantation: A case report

Monitoring cerebral oxygenation using a near infrared spectroscopy (NIRS) device is useful for estimating cerebral hypoperfusion and is available during liver transplantation (LT). However, high serum bilirubin concentration can interfere with NIRS because bilirubin absorbs near infrared light. We report a patient who underwent LT with a diagnosis of vanishing bile duct syndrome, whose regional cerebral oxygen saturation (rSO₂) remained below 15% even with alert mental status and SpO2₂ value of 99%. The rSO₂ values were almost fixed at the lowest measurable level throughout the intra- and postoperative period. We report a case of erroneously low rSO₂ values during the perioperative period in a liver transplant recipient which might be attributable to skin pigmentation rather than higher serum bilirubin concentration.

Gray-brown skin discoloration following phototherapy for hyperbilirubinemia due to anti-E alloimmunization / 소아과

No abstract available.

Safety and efficacy of bendamustine in the conditioning regimen for autologous stem cell transplantation in patients with relapsed/refractory lymphoma

BACKGROUND: Bendamustine is an attractive option for the management of both de novo and relapsed lymphomas. It is being increasingly used in the conditioning regimen for autologous stem cell transplantation (SCT) and can be an alternative to the traditionally-used carmustine. In this study, we aimed to determine the safety and efficacy of bendamustine in the conditioning regimen for autologous SCT in refractory/relapsed lymphomas. METHODS: We designed a descriptive study to evaluate bendamustine in combination with etoposide, cytarabine, and melphalan (BeEAM) in the conditioning regimen for autologous SCT. RESULTS: Fourteen patients (median age, 28 yr) with Hodgkin's lymphoma (HL) (N=8), non-Hodgkin's lymphomas (NHL) (N=5), or peripheral T-cell lymphoma, not otherwise specified (PTCL NOS) (N=1) were included in the study. A median number of 5.95×10⁶ CD34+ cells/kg were transfused. Median times to absolute neutrophil count and platelet engraftment were 17 days and 24 days, respectively. The 100-day transplantation mortality rate was 28% (4 patients). Eight patients (57.14%) had GII-III acute kidney injury, four patients (28.5%) had GIII-IV hyperbilirubinemia, and twelve patients (85%) had GII-III diarrhea. After 3 months, 37% (5 patients) and 21.4% (3 patients) demonstrated complete response and partial response, respectively. The median follow-up was 5.5 months (15 days–19 mo). At the final follow-up, 7 patients (50%) were alive and in CR. CONCLUSION: Our study showed that bendamustine is a potentially toxic agent in the conditioning regimen for autologous SCT, resulting in significant liver, kidney, and gastrointestinal toxicity. Further studies are required to assess its safety and efficacy at reduced doses.

Prevalence of Unexpected Antibodies in Pregnant Korean Women and Neonatal Outcomes / 대한수혈학회지

BACKGROUND: In pregnant women, the frequency of irregular antibodies that cause hemolytic disease of the fetus and newborn (HDFN) vary between study populations. The clinical manifestations of HDFN differ according to the specificities and degree of irregular antibodies. This study examined the frequency and nature of maternal alloimmunization and neonatal outcomes. METHODS: Pregnant women, who underwent irregular antibody screening for prenatal testing at an obstetrics clinic in a single center, were enrolled. Those who screened positive for irregular antibodies were selected as the test group, and age- and obstetrics history-matched pregnant women were selected as the control group to evaluate the pregnancy outcomes according to irregular antibodies. RESULTS: The prevalence of irregular antibodies was 2.78% (42/1,508). With the exception of an unidentified antibody, anti-D was the most frequently identified antibody, followed in order by anti-E and anti-Le(a). The rate of fetal death was higher in the test group (6/37, 16.2%) than in the control group (1/37, 2.7%) (P=0.047). Eight pregnant women had anti-C or anti-D, one woman had a stillbirth, and four living neonates developed hyperbilirubinemia. Of six pregnant women with anti-E alone or with other alloantibodies, three experienced a spontaneous abortion or stillbirth. Among the six newborns with maternal anti-Le(a) and anti-Jk(a), four developed hyperbilirubinemia, but their mothers did not experience a spontaneous abortion or stillbirth. CONCLUSION: The prevalence of unexpected antibodies among pregnant Korean women was 2.78%. A significant difference in neonatal outcomes was observed, including the death rate, prematurity, and hyperbilirubinemia, depending on the specificity of the unexpected antibody.