Glucose-6-phosphate dehydrogenase deficiency and neonatal indirect hyperbilirubinemia: a retrospective cohort study among 40,305 consecutively born babies.

BACKGROUND AND OBJECTIVE: Glucose-6-phosphate dehydrogenase deficiency (G6PDD) being highly prevalent in the Middle East, the primary objective was to estimate the incidence of neonatal jaundice among G6PD-deficient neonates and to explore its association with various risk factors. METHODS: This retrospective cohort study includes 7 years data of neonates diagnosed with G6PDD between 1st January 2015, and 30 September 2022, from Al Wakra Hospital, HMC Qatar. RESULTS: Among the 40,305 total births, 1013 had G6PDD with an incidence of 2.51%. Of all the G6PDD babies, 24.6% (249/1013) received phototherapy and three babies required exchange transfusion. Statistically significant associations were noted between the need for phototherapy and gestational age, gestational age groups, birth weight, and birth weight groups, but logistic regression analysis showed significant association for phototherapy only with the gestational age group. CONCLUSION: Universal screening and proper follow-up is essential for G6PDD as it plays crucial role in neonatal jaundice.

Neurodevelopmental outcome at 6 months of age of full-term neonates with hyperbilirubinemia necessitating exchange transfusion.

BACKGROUND: Bilirubin neurotoxicity involves a spectrum of varying severity that could result in adverse long-term sequelae. AIMS: To compare the neurodevelopmental outcome of full-term neonates who underwent exchange transfusion with those who did not. STUDY DESIGN: A retrospective cohort study. SUBJECTS: This study included a retrospective review of records of sixty neonates who were matched in admission ages and serum bilirubin levels and the comparison groups were those who received an exchange transfusion (n = 30) versus those where exchange transfusion was planned, but the bilirubin levels dropped sufficiently during the period where the exchange blood was being prepared (n = 30). History, clinical examination, and laboratory investigations were documented. OUTCOME MEASURES: Neurodevelopmental outcome, at 6 months of age, using Bayley scales of infant development was assessed. RESULTS: The exchange group had statistically significant lower cognitive scores (p-value 0.005). The higher the rate of bilirubin decline, the better the language and motor scores in the phototherapy group (p-values 0.020 and 0.024 respectively). Infants with longer duration to exchange transfusion had lower cognitive, language, and motor scores (p-values 0.01, 0.001, and 0.003 respectively). CONCLUSIONS: Slower rates of bilirubin decline and longer duration before intervention increase the chances of adverse neurodevelopmental outcomes.

Intensive phototherapy vs. exchange transfusion for the treatment of neonatal hyperbilirubinemia: a multicenter retrospective cohort study / 中华医学杂志(英文版)

BACKGROUND@#Intensive phototherapy (IPT) and exchange transfusion (ET) are the main treatments for extreme hyperbilirubinemia. However, there is no reliable evidence on determining the thresholds for these treatments. This multicenter study compared the effectiveness and complications of IPT and ET in the treatment of extreme hyperbilirubinemia.@*METHODS@#This retrospective cohort study was conducted in seven centers from January 2015 to January 2018. Patients with extreme hyperbilirubinemia that met the criteria of ET were included. Patients were divided into three subgroups (low-, medium-, and high- risk) according to gestational week and risk factors. Propensity score matching (PSM) was performed to balance the data before treatment. Study outcomes included the development of bilirubin encephalopathy, duration of hospitalization, expenses, and complications. Mortality, auditory complications, seizures, enamel dysplasia, ocular motility disorders, athetosis, motor, and language development were evaluated during follow-up at age of 3 years.@*RESULTS@#A total of 1164 patients were included in this study. After PSM, 296 patients in the IPT only group and 296 patients in the IPT plus ET group were further divided into the low-, medium-, and high-risk subgroups with 188, 364, and 40 matched patients, respectively. No significant differences were found between the IPT only and IPT plus ET groups in terms of morbidity, complications, and sequelae. Hospitalization duration and expenses were lower in the low- and medium-risk subgroups in the IPT only group.@*CONCLUSIONS@#In this study, our results suggest that IPT is a safe and effective treatment for extreme hyperbilirubinemia. The indication of ET for patients with hyperbilirubinemia could be stricter. However, it is necessary to have a contingency plan for emergency ET as soon as IPT is commenced especially for infants with risk factors. If IPT can be guaranteed and proved to be therapeutic, ET should be avoided as much as possible.

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.

Importancia de la resonancia magnética en la encefalopatía bilirrubínica / Importance of magnetic resonance imaging in bilirubin encephalopathy

Con el objetivo de resaltar el valor diagnóstico de la resonancia magnética en la encefalopatía bilirrubínica se presenta el caso clínico de un recién nacido de 37 semanas de edad gestacional que reingresa al octavo día de vida por ictericia constatándose cifras de bilirrubina indirecta de 30,1 mg/dl. La disminución de las mismas se logró con exanguinotransfusión, fototerapia intensiva y adecuado aporte. Se solicita resonancia magnética de cráneo que informa, a nivel de ambos globos pálidos y subtalámico, un aumento de la señal en T1 y T2; contribuyendo al diagnóstico de encefalopatía bilirrubínica aguda.

In order to highlight the value of magnetic resonance imaging in the diagnosis of bilirubin encephalopathy, the clinical case of a 37 week of gestational age newborn is presented. The newborn was readmitted to hospital with jaundice on the eighth day of life, indirect bilirubin being 30.1 mg/dl. This level was decreased with exchange transfusion, intensive phototherapy and the appropriate oral supply. Magnetic resonance imaging of the skull was requested, revealing T1 and T2 hyperintensity within the globus pallidus, and the subthalamic nuclei; which contributed to the diagnose of acute bilirubin encephalopathy.

Prevalência de marcadores imuno-hematológicos em recém-nascidos ao nascimento e em suas respectivas mäes e incidência de doença hemolítica numa maternidade de São Paulo / Prevalence of immunohematologic tests at birth and the incidence of hemolytic disease in the newborn

A introduçäo da imunoglobulina anti-D diminuiu a incidência da doença hemolítica neonatal por isoimunizaçäo Rh, porém persiste este diagnóstico por outros anticorpos mais raros e o avanço tecnológico tornou possível a detecçäo destes anticorpos. OBJETIVOS: Verificar a prevalência de marcadores imuno-hematológicos, representados pelos testes de Coombs indireto, direto e de eluiçäo com identificaçäo do anticorpo detectado; incidência de doença hemolítica e de tratamento entre os recém-nascidos sensibilizados. MÉTODOS: Estudo do tipo Coorte retrospectiva, de janeiro de 1996 a julho de 1998, consistiu na descriçäo da análise dos perfis imuno-hematológicos de 1698 pares de mäes e recém-nascidos como fator de risco para doença hemolítica, subdivididos de acordo com os marcadores. A metodologia empregada para identificaçäo dos marcadores foi o da microplaca com hemácias de triagem, soro antiglobulina humana e gel centrifugaçäo. Para tipagens e fenotipagens utilizou-se o método de microplaca com soros monoclonais. Para o estudo da incidência e seguimento neonatal foram realizadas bilirrubinas totais e frações, por método enzimático colorímetro, hemoglobina e hematócrito, automatizado e reticulócitos, por coloraçäo supra vital, azul cresil brilhante e leitura por microscopia óptica. RESULTADOS: A prevalência de marcadores imuno-hematológicos associados à doença hemolítica foi de 9,07 por cento. Por grupos estratificados obtivemos no grupo com Coombs indireto (grupo I) 0,43 por cento; no grupo com Coombs direto (grupo D), 4,10 por cento e no grupo com eluiçäo (grupo E) 4,53 por cento. A incidência de doença hemolítica no estudo foi de 36,23 por cento. Quando estratificada por grupos, obtivemos no grupo I, 33,56 por cento, no grupo D, 44,43 por cento e no grupo E, 29,24 por cento. O tratamento com fototerapia foi necessário em 36,23 por cento dos RN, sendo maior sua indicaçäo no grupo D e a exsangüíneotransfusäo foi necessária em 0,88 por cento dos RN, sendo maior sua indicaçäo no grupo I. CONCLUSÕES: O grupo I, onde se concentram as incompatibilidades Rh, apresentou maior incidência de doença hemolítica e maior necessidade de tratamento com exsangüíneotransfusäo, o que mostra ainda a gravidade deste sistema em nosso meio. O grupo D, onde se concentram as incompatibilidades ABO, apresentou maior incidência de doença hemolítica e tratamento com fototerapia e menor necessidade de exsangüíneotransfusäo

Ictericia e infección perinatal / Jaundice and perinatal infection

Al iniciarse la vida extrauterina se suceden una serie de adaptaciones fisiológicas en todos los órganos de la economía, en respuesta a las demandas del nuevo medio. Los ajustes que ocurren a nivel gastrointestinal y hemático contribuyen a que en cerca del 50% de recién nacidos a término, y un número considerablemente mayor a pretérmino, manifiesten ictericia durante la primera semana de vida. La expresión bioquímica de ictericia es la hiperbilirrubinemia (bilirrubinas sericas mayores de 2 mg/dl). Con frecuencia en el neonato la ictericia se observa cuando la hiperbilirrubinemia es mayor de 5 a 7 mg/dl en sangre

Protocolo de atencion de enfermeria en la prevencion primaria de la enfermedad hemolitica por incompatibilidad del factor RH en el recien nacido Hospital La Victoria de Bogota D.E / Nursing care protocol for primary prevention of haemolitic disease due to RH factor incompatibility in the newborn, Hospital La Victoria. Bogota D.E

La enfermedad hemolitica del recien nacido por incompatibilidad de Rh negativo es una de las patologias que afecta al neonato y que se ha venido estudiando desde 1939; esta incompatibilidad se refiere a la diferencia del Rh en una pareja donde la madre es Rh negativa y el padre Rh positivo. Cuando sangre Rh positiva se transfunde a una mujer Rh negativa, por error, o cuando pequenas cantidades (mas de un milimetro) de sangre fetal Rh positiva que contiene antigeno D heredado de un padre Rh positivo; entra en la circulacion materna durante el embarazo, o bien por aborto espontaneo, o inducido, o en el momento del parto, la madre no sensibilizada Rh negativa produce anticuerpos anti D. Una vez que ha ocurrido la isoinmunizacion, pequenas dosis de antigeno pueden estimular el incremento del titulo de anticuerpos, produciendo un aumento de la destruccion de los eritricitos y causando sintomatologia de enfermedad hemolitica, manifestaciones que pueden ir desde la simple evidencia de laboratorio con moderada hemolisis hasta anemia severa, acompanada de hepatoesplenomegalia como mecanismo compensador; al aumentar esta capacidad compensatorio por anemia profunda dara lugar a palidez, signos de descompensacion cardiaca, anasarca masiva y colapso cardiocirculatorio. Este cuadro clinico denominado hidropesia fetal da lugar frecuentemente a muerte intrauterina o inmediatamente despues de nacer. Generalmente al nacimiento no presenta ictericia, puesto que la placenta ha representado el papel de aclaramiento a la bilirrubina no conjugada..

Glucose 6 phosphate dehydrogenase deficiency and neonatal jaundice in Jamaica

Glucose-6- phosphate dehydrogenase (G6PD) deficiency was detected in 16 (60.6 percent) of a group of23 neonates who had unexplained moderate or severe jaundice. This proportion is significantly more than the 9.4 percent expected in Jamaican neonates who are not moderately or severely jaundiced (P<0.003), and significantly more than the 12.6 percent observed or the 21.0 percent expected in older Jamaican children and adults (P<0.003). Phenobarbitone therapy and phototherapy reduced the need for exchange transfusion but this was necessary in eight patients. Two babies developed kernicterus and one died. On the other hand, only two of 21 neonates who were identified as G6PD deficient at birth subsequently became moderately or severely jaundiced, and this could be attributed to other causes in both cases. These findings indicate that apparently spontaneous neonatal juandice is important in infants who have the G6PD A-enzyme. However, the jaundice is probably precipitated by unknown factors to which the G6PD deficient neonate is more susceptible than the infant who is not G6PD deficient. There is also a slightly increased incidence of G6PD deficiency in neonates who develop jaundice because of ABO or Rh(D) iso-immune disease, infection or prematurity (AU)