Etiology analysis for term newborns with severe hyperbilirubinemia in eastern Guangdong of China.

BACKGROUND: Neonatal hyperbilirubinemia is one of the common diseases of newborns that typically presents with yellow staining of skin, resulting in sequelaes such as hearing loss, motor and intellectual development disorders, and even death. The pathogenic factors of neonatal hyperbilirubinemia are complex. Different cases of hyperbilirubinemia may have a single or mixed etiology. AIM: To explore the etiological characteristics of severe hyperbilirubinemia in term newborns of eastern Guangdong of China. METHODS: Term newborns with severe hyperbilirubinemia in one hospital from January 2012 to December 2021 were retrospectively analyzed. The etiology was determined according to the laboratory results and clinical manifestations. RESULTS: Among 1602 term newborns with hyperbilirubinemia in eastern Guangdong of China, 32.20% (580/1602) was severe hyperbilirubinemia. Among the causes of severe hyperbilirubinemia, neonatal hemolysis accounted for 15.17%, breast milk jaundice accounted for 12.09%, infection accounted for 10.17%, glucose-6-phosphate dehydrogenase (G6PD) deficiency accounted for 9.14%, and the coexistence of multiple etiologies accounted for 6.55%, unknown etiology accounted for 41.72%. ABO hemolysis and G6PD deficiency were the most common causes in the 20 cases with bilirubin encephalopathy. 94 severe hyperbilirubinemia newborns were tested for uridine diphosphate glucuronosyl transferase 1A1 (UGT1A1)*6 variant (rs4148323, c.211G>A, p.Arg71Gly), 9 cases were 211 G to A homozygous variant, 37 cases were 211 G to A heterozygous variant, and 48 cases were wild genotypes. CONCLUSION: The main cause for severe hyperbilirubinemia and bilirubin encephalopathy in eastern Guangdong of China were the hemolytic disease of the newborns, G6PD deficiency and infection. UGT1A1 gene variant was also a high-risk factor for neonatal hyperbilirubinemia. Targeted prevention and treatment according to the etiology may reduce the occurrence of bilirubin encephalopathy and kernicterus.

Clinical analysis of severe hyperbilirubinemia caused by hemolytic disease of the newborn and G6PD deficiency / 中国新生儿科杂志

Objective:To study the clinical characteristics and differences of severe hyperbilirubinemia caused by hemolytic disease of the newborn (HDN) and glucose-6-phosphate dehydrogenase (G6PD) deficiency.Methods:From January 2014 to December 2021, newborns (gestational age ≥ 35 weeks and postnatal age ≤ 28 d) admitted to the Department of Neonatology of Hunan Children's Hospital with severe hyperbilirubinemia caused by HDN or G6PD deficiency were retrospectively analyzed. According to the etiology of hyperbilirubinemia, they were assigned into HDN group and G6PD deficiency group. The general conditions, clinical manifestations, laboratory results, treatment and prognosis of the two groups were compared using SPSS 26.0 software.Results:A total of 532 cases were in the HDN group and 413 cases in the G6PD deficiency group. The HDN group reached peak hyperbilirubinemia earlier than the G6PD deficiency group [3(2,5) d vs. 6(4,8)d, P<0.05]. The HDN group had lower peak value of total serum bilirubin [379.5(345.6,426.7) μmol/L vs. 486.4 (413.5,577.4) μmol/L] and lower incidence of anemia [37.4% (199/532) vs. 55.0% (227/413)]than the G6PD deficiency group.The incidence of anemia with elevated reticulocyte percent(Ret%) in the HDN group was higher than the G6PD deficiency group[66.3%(132/199) vs. 5.7%(13/227), P<0.05]. Compared with the G6PD deficiency group, the incidences of exchange transfusion and repeated (≥2 times) exchange transfusion, acute bilirubin encephalopathy(ABE) and the mortality rate after withdrawal of treatment in the HDN group were significantly lower ( P<0.05). Conclusions:Neonatal severe hyperbilirubinemia caused by HDN has early onset. G6PD deficiency caused hyperbilirubinemia has higher incidences of anemia, more severe jaundice and ABE, without increased Ret%.

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OBJECTIVES: To investigate the preadmission follow-up condition of neonates hospitalized due to severe hyperbilirubinemia after discharge from the department of obstetrics and the influencing factors for follow-up compliance. METHODS: A multicenter retrospective case-control study was performed for the cases from the multicenter clinical database of 12 units in the Quality Improvement Clinical Research Cooperative Group of Neonatal Severe Hyperbilirubinemia in Jiangsu Province of China from January 2019 to April 2021. According to whether the follow-up of neonatal jaundice was conducted on time after discharge from the department of obstetrics, the neonates were divided into two groups: good follow-up compliance and poor follow-up compliance. The multivariate logistic regression model was used to identify the influencing factors for follow-up compliance of the neonates before admission. RESULTS: A total of 545 neonates with severe hyperbilirubinemia were included in the study, with 156 neonates (28.6%) in the good follow-up compliance group and 389 (71.4%) in the poor follow-up compliance group. The multivariate logistic regression analysis showed that low gestational age at birth, ≥10% reduction in body weight on admission compared with birth weight, history of phototherapy of siblings, history of exchange transfusion of siblings, Rh(-) blood type of the mother, a higher educational level of the mother, the use of WeChat official account by medical staff to remind of follow-up before discharge from the department of obstetrics, and the method of telephone notification to remind of follow-up after discharge were associated with the increase in follow-up compliance (P<0.05). CONCLUSIONS: Poor follow-up compliance is observed for the neonates with severe hyperbilirubinemia after discharge from the department of obstetrics, which suggests that it is necessary to further strengthen the education of jaundice to parents before discharge and improve the awareness of jaundice follow-up. It is recommended to remind parents to follow up on time by phone or WeChat official account.

Preadmission follow-up condition of neonates hospitalized due to severe hyperbilirubinemia after discharge from the department of obstetrics and influencing factors for follow-up compliance: a multicenter investigation / 中国当代儿科杂志

OBJECTIVES@#To investigate the preadmission follow-up condition of neonates hospitalized due to severe hyperbilirubinemia after discharge from the department of obstetrics and the influencing factors for follow-up compliance.@*METHODS@#A multicenter retrospective case-control study was performed for the cases from the multicenter clinical database of 12 units in the Quality Improvement Clinical Research Cooperative Group of Neonatal Severe Hyperbilirubinemia in Jiangsu Province of China from January 2019 to April 2021. According to whether the follow-up of neonatal jaundice was conducted on time after discharge from the department of obstetrics, the neonates were divided into two groups: good follow-up compliance and poor follow-up compliance. The multivariate logistic regression model was used to identify the influencing factors for follow-up compliance of the neonates before admission.@*RESULTS@#A total of 545 neonates with severe hyperbilirubinemia were included in the study, with 156 neonates (28.6%) in the good follow-up compliance group and 389 (71.4%) in the poor follow-up compliance group. The multivariate logistic regression analysis showed that low gestational age at birth, ≥10% reduction in body weight on admission compared with birth weight, history of phototherapy of siblings, history of exchange transfusion of siblings, Rh(-) blood type of the mother, a higher educational level of the mother, the use of WeChat official account by medical staff to remind of follow-up before discharge from the department of obstetrics, and the method of telephone notification to remind of follow-up after discharge were associated with the increase in follow-up compliance (P<0.05).@*CONCLUSIONS@#Poor follow-up compliance is observed for the neonates with severe hyperbilirubinemia after discharge from the department of obstetrics, which suggests that it is necessary to further strengthen the education of jaundice to parents before discharge and improve the awareness of jaundice follow-up. It is recommended to remind parents to follow up on time by phone or WeChat official account.

Hemolytic Disease of the Fetus and Newborn: Historical and Current State.

Hemolytic disease of the fetus and newborn (HDFN) is an immune-mediated disorder affecting neonates globally, with a range of clinical presentations from severe and life threatening to mild or even asymptomatic. Historically, HDFN has been responsible for a large proportion of perinatal mortality, and, despite advances in diagnosis and management, this morbidity and mortality has not been eradicated. Blood banking techniques and blood transfusion have contributed to improved prophylaxis and management, drastically improving the outcome of newborns with HDFN over the last century.

Hiperbilirrubinemia severa en recién nacidos, factores de riesgo y secuelas neurológicas / Severe hyperbilirubinemia in newborns, risk factors and neurological outcomes

INTRODUCCIÓN: La hiperbilirrubinemia es altamente prevalente en los recién nacidos, con riesgo de compromiso neurológico con bilirrubinemias mayor a 20-25 mg/dl. Esta progresión es prevenible con detección y tratamiento precoz. OBJETIVO: Describir incidencia y factores asociados en pacientes hospitalizados con hiperbilirrubinemia mayor de 20 mg/dl, y el seguimiento de casos sintomáticos durante hospitalización. PACIENTES Y MÉTODO: Estudio retrospectivo de pacientes con hiperbilirru- binemia severa, entre el 2013 y 2016. Se evaluaron factores de riesgo, estratificándose por nivel de bilirrubina, edad de ingreso y edad gestacional. Se compararon los datos con test exacto de Fisher, chi cuadrado y riesgo relativo (RR) en una base de excel, con un error alfa de un p<0.05. Los datos fueron obtenidos a través de la epicrisis electrónica y de la ficha de control a nivel secundarios. RESULTADOS: Durante el periodo, de 25.288 recién nacidos vivos (RNV), 593 se hospitalizaron por hiperbilirrubinemia mayor de 20 mg/dl, 1 por cada 42 RNV; y 59 con bilirrubinemia mayor a 25 mg/dl, 1 por cada 428 RNV. La hiperbilirrubinemia fue más frecuente en varones, con RR 1,22 (IC 95% 1,04-1,44) y en pretérminos tardíos, con un RR 2,39 (IC 95% 1,96-2,93) comparado con RN de término. En los ingresados con más de 4 días, el principal factor asociado fue la baja de peso excesiva, y en los primeros 3 días, la incompatibilidad de grupo clásico. Tres de 10 pacientes con encefalopatía aguda, persistieron con compromiso neurológico, lo que significa 11,8 por 100.000 nacidos vivos. CONCLUSIONES: Los principales factores de riesgo para desarrollar hiperbilirrubinemia severa fueron prematurez, baja de peso excesiva, incompatibilidad de grupo clásico y sexo masculino. Estos hallazgos permiten focalizar la atención en grupos de riesgo y disminuir la probabilidad de daño neurológico.

INTRODUCTION: Hyperbilirubinemia is highly prevalent in newborns, with risk of neurological invol vement with bilirubinemia higher than 20 to 25 mg/dl. This progression is preventable with early de tection and treatment. OBJECTIVE: To describe the incidence and associated factors in hospitalized pa tients with hyperbilirubinemia higher than 20 mg/dl, and the follow-up of symptomatic cases during hospitalization. OATIENTS Y METHOD: Retrospective study of patients with severe hyperbilirubine mia, between 2013 and 2016. Risk factors were evaluated, stratifying by bilirubin level, admission age, and gestational age. The data were compared with Fisher's exact test, chi-square test, and relative risk (RR) in an Excel database, with an alpha error of p <0.05. The data were obtained from the electronic discharge summary and the medical record of secondary level follow-up. RESULTS: During the studied period, out of 25,288 live newborns (NB), 593 were hospitalized due to hyperbilirubinemia higher than 20 mg/dl, one per each 42 live NB; and 59 with bilirubinemia higher than 25 mg/dl, one per each 428 live NB. Hyperbilirubinemia was more frequent in males, with RR 1.22 (95% CI 1.04-1.44), and in late preterm newborns, with RR 2.39 (95% CI 1.96-2.93) compared with term NB. In those admitted with more than four days, the main associated factor was excessive weight loss, whereas in the first three days was classic group incompatibility. Three of ten cases with acute encephalopathy persisted with neurological involvement, which means 11.8 per 100,000 live births. CONCLUSIONS: The main risk factors for developing severe hyperbilirubinemia were prematurity, excessive weight loss, classic group incompatibility, and male sex. These findings allow to focus attention on risk groups and decrease the probability of neurological damage.

Application of cerebral regional oxygen saturation monitoring in neonatal blood exchange transfusion / 中华实用儿科临床杂志

Objective@#To observe the changes in cerebral regional oxygen saturation (CrSO2) during neonatal blood exchange transfusion(BET) and its effect on the level of bilirubin in neonates with severe hyperbilirubinemia.@*Methods@#From January 2017 to March 2018, 52 newborns with severe hyperbilirubinemia were hospitalized in the Department of Neonatology, Children′s Hospital of Fudan University.Every newborn was treated with BET.Near infrared spectroscopy was used to monitor CrSO2 in the process of BET.The monitoring lasted from 2 hours before the beginning of BET to 2 hours after the completion of BET.The CrSO2 were recorded every 2 minutes and total surem bilirubin (TSB) and transcutaneous bilirubin(TCB) was measured.During this period, it is accompanied by the monitoring of neonatal body temperature, heart rate, respiration and bolld oxygen saturation(SpO2). The differences in CrSO2 changes at different time points during BET were compared.At the same time, the correlations between CrSO2 and blood oxygen saturation, TSB and TCB levels were analyzed.The results of repeated measurement analysis of variance compared between the two groups were corrected by Bonfferoni.@*Results@#Among the 52 children, there were 33 males (63.46%) and 19 females (36.54%). The gestational age, average birth weight and average head circumference of newborns were (38.6±2.1) weeks, (3 338±444) g and (33.6±3.2) cm, respectively.The Apgar score of newborn was (8.1±1.6) scores at 1 minute after birth.The level of TSB detected for the first time after admission was (457.9±97.8) μmol/L.The CrSO2 after BET (74.6%-76.0%) was significantly higher than that before BET (69.4%-69.0%), and the difference was statistically significant (P<0.05). Correlation analysis showed that during BET, CrSO2 showed a gradual upward trend, SpO2 also showed a synchronous increase, while the level of bilirubin showed a downward trend, and the downward trend of TSB level was more obvious than that of TCB.@*Conclusions@#CrSO2 can reflect the improvement of cerebral oxygenation during neonatal blood exchange transfusion and avoid cerebral hypoxia in the course of treatment.

Tokyo-1 Mutation: Hereditary Spherocytosis in a Hispanic Newborn Presenting as Early Onset Severe Hyperbilirubinemia.

BACKGROUND: Hereditary spherocytosis in the Hispanic population does not often present with severe hyperbilirubinemia. Spectrin and band 3 mutations are most frequent in this population. CASE REPORT: We present a Hispanic full-term female newborn with early onset significant hyperbilirubinemia without a history of familial hemolytic disorders. She was diagnosed with hereditary spherocytosis based on laboratory findings, including presence of spherocytes on a peripheral smear, and was later found by next-generation sequencing to have Tokyo-1 mutation, an ANK1 gene mutation, that was previously only reported in Japanese population. CONCLUSION: Our report adds to the currently limited literature of the genetic spectrum and characteristics of hereditary spherocytosis in the Hispanic population. The absence of a positive family history does not preclude hereditary spherocytosis as a differential for pathologic neonatal hyperbilirubinemia.

Diagnostic Performance Analysis of the Point-of-Care Bilistick System in Identifying Severe Neonatal Hyperbilirubinemia by a Multi-Country Approach.

IMPORTANCE: The real prevalence and clinical burden of severe neonatal jaundice are undefined due to difficulties in measuring total serum bilirubin (TSB) outside secondary and tertiary clinical centers. OBJECTIVE: To assess the diagnostic performance of the point-of care Bilistick System (BS) in identifying neonatal jaundice patients requiring treatment. DESIGN: Between April 2015 and November 2016, 1911 neonates, were recruited to participate in the study. Blood samples were simultaneously collected for the TSB determination by BS and by hospital laboratory (Lab). Data were collected and sent to the Bilimetrix headquarter in Trieste where statistical analysis was performed. Newborns with neonatal jaundice were treated with phototherapy according to each center's guidelines. SETTING: 17 hospitals from Nigeria, Egypt, Indonesia, and Viet Nam. PARTICIPANTS: 1911 newborns were included, of which 1458 (76·3%) fulfilled the inclusion criteria. RESULTS: TSB level measured by BS agreed (p < .0001) with the lab result in all four countries. The diagnostic performance of BS showed a positive predictive value (PPV) of 92·5% and a negative predictive value (NPV) of 92·8%. CONCLUSIONS AND RELEVANCE: BS is a reliable system to detect neonatal jaundice over a wide range of bilirubin levels. Since Bilistick is a point-of-care test, its use may provide appropriate and timely identification of jaundiced newborns requiring treatment.

Severe neonatal hyperbilirubinemia leading to exchange transfusion.

BACKGROUND: Severe neonatal hyperbilirubinemia is associated with significant morbidity and mortality. This study was conducted to investigate the causes of severe hyperbilirubinemia leading to Exchange Transfusion (ET) from March 2009 to March 2011 in Bahrami children hospital, Tehran, Iran in order to establish guidelines to prevent profound jaundice & ET. METHODS: 94 neonates underwent ET for severe hyperbilirubinemia data for demographic data, and onset of jaundice, history of severe hyperbilirubinemia in siblings, blood group of both mother and neonate, G6PD activity, hemoglobin, hematocrite, reticulocyte count, peripheral blood smear, total and direct bilirubin before and after ET, direct and indirect Coombs, times of transfusion and the cause of hyperbilirubinemia were all recorded for analysis. RESULTS: Ninety four neonates (56.4% boys and 43.6% girls) underwent ET with a mean birth weight of 1950±40 g and a mean gestational age of 35.2±1.4 weeks. Premature labor, breastfeeding jaundice, ABO incompatibility and G6PDD with the frequency of 59(63%), 33(35%), 25(24/5%) and 12(12.8%) were of major causes of ET. CONCLUSIONS: Predisposing factors for severe hyperbilirubinemia in this study were premature labor, breastfeeding jaundice, ABO incompatibility and G6PDD. The authors recommend prevention of premature labor, reevaluation of successful breastfeeding education for mothers and screening infants for blood group and G6PD In the first of life. Arranging earlier and continuous visits in neonates with these risk factors during the first four days of life is also recommended.