RESUMO
High neonatal bilirubin is a common phenomenon responding to phototherapy. We report a case of a newborn with a highly elevated bilirubin of 37.3 mg/dL due to ABO incompatibility between the mother (Group O) and the newborn (Group A) requiring whole blood exchange, a procedure performed rarely to treat newborn hyperbilirubinemia. The newborn (38.8 weeks of gestation) initially showed a total bilirubin of 8.4 mg/dL and was discharged after being stabilized by phototherapy. However, the baby returned to the hospital with highly elevated bilirubin and was admitted to the Neonatal Intensive Care Unit (NICU). Emergent reconstituted whole blood exchanger therapy was initiated due to refractoriness to phototherapy and IVIG. Markedly elevated anti-A titer was found in the mother's blood (1:512) and cord blood (1:128). The baby was stabilized and eventually discharged with a serum bilirubin of 13.8 mg/dL. This case demonstrates the possible predictive value of mother/cord blood anti-A titers in severe newborn hyperbilirubinemia, which may prevent premature discharge and trigger early initiation of lifesaving therapy.
Assuntos
Sistema ABO de Grupos Sanguíneos , Bilirrubina , Transfusão Total , Humanos , Recém-Nascido , Bilirrubina/sangue , Transfusão Total/métodos , Feminino , Hiperbilirrubinemia Neonatal/sangue , Hiperbilirrubinemia Neonatal/terapia , Eritroblastose Fetal/sangue , Eritroblastose Fetal/terapia , Fototerapia/métodos , Masculino , Incompatibilidade de Grupos SanguíneosRESUMO
BACKGROUND: Hemolytic disease of the newborn (HDN) is a common condition that can have a severe impact on the health of newborns due to the hemolytic reactions it triggers. Although numerous studies have focused on understanding the pathogenesis of HDN, there are still many unanswered questions. METHODS: In this retrospective study, serum samples were collected from 15 healthy newborns and 8 infants diagnosed with hemolytic disease. The relationship between different metabolites and various IgG subtypes in Healthy, HDN and BLI groups was studied by biochemical technique and enzyme-linked immunosorbent assay (ELISA). Metabolomics analysis was conducted to identify the differential metabolites associated with HDN. Subsequently, Pearson's correlation analysis was used to determine the relation of these differential metabolites with IgG isoforms. The relationship between the metabolites and IgG subtypes was observed after treatment. RESULTS: The study results revealed that infants with hemolytic disease exhibited abnormal elevations in TBA, IgG1, IgG2a, IgG2b, IgG3, and IgG4 levels when compared to healthy newborns. Additionally, differences in metabolite contents were also observed. N, N-DIMETHYLARGININE showed negative correlations with TBA, IgG1, IgG2a, IgG2b, IgG3, and IgG4, while 2-HYDROXYBUTYRATE, AMINOISOBUTANOATE, Inosine, and ALLYL ISOTHIOCYANATE exhibited positive correlations with TBA, IgG1, IgG2a, IgG2b, IgG3, and IgG4. Through metabolomics-based research, we have discovered associations between differential metabolites and different IgG isoforms during the onset of HDN. CONCLUSION: These findings suggest that changes in metabolite and IgG isoform levels are linked to HDN. Understanding the involvement of IgG isoforms and metabolites can provide valuable guidance for the diagnosis and treatment of HDN.
Assuntos
Imunoglobulina G , Metabolômica , Isoformas de Proteínas , Humanos , Imunoglobulina G/sangue , Recém-Nascido , Metabolômica/métodos , Feminino , Masculino , Estudos Retrospectivos , Eritroblastose Fetal/sangue , Eritroblastose Fetal/metabolismo , Eritroblastose Fetal/diagnósticoRESUMO
INTRODUCTION: Anti-D detection and titration plays a major role in RhD negative antenatal cases both, for monitoring maternal as well as fetal status as well as initiation of early therapeutic interventions, such as intra-uterine transfusions (IUT) to improve maternal as well as fetal morbidity and mortality and reduce the adverse effects of haemolytic disease of fetus and newborn (HDFN). We conducted a survey focusing on the policies and procedures of anti-D detection and titration among major tertiary care centres across India. METHODOLOGY: The survey was drafted by a working group of transfusion medicine and immunohematology specialists from six different centres in India. Data were obtained via the use of an online questionnaire. RESULTS: Results were categorised into four categories, Hospital information, immuno-haematological testing methodology, clinical significance of anti-D testing and the role of transfusion medicine specialists. The survey highlighted the modalities as well as the methodologies of anti-D detection and titration in antenatal women across different major tertiary care centres in India. CONCLUSION: This survey provided a unique snapshot of the prevalent methodologies being employed by major tertiary care centres across the country for detection and titration of anti-D levels as well as the important role it plays in the therapy of affected antenatal women to minimise adverse effects on the fetus.
Assuntos
Imunoglobulina rho(D) , Humanos , Índia , Feminino , Gravidez , Inquéritos e Questionários , Imunoglobulina rho(D)/sangue , Isoanticorpos/sangue , Centros de Atenção Terciária , Eritroblastose Fetal/sangue , Eritroblastose Fetal/terapia , Eritroblastose Fetal/diagnóstico , Recém-NascidoRESUMO
Since publication of the original Immunohematology review of the Kidd blood group system in 2015 (Hamilton JR. Kidd blood group system: a review. Immunohematology 2015;31:29-34), knowledge has mushroomed pertaining to gene structure, alleles causing variant and null phenotypes, clinical significance in renal transplant and hemolytic disease of the fetus and newborn, and physiologic functions of urea transporters in non-renal tissues. This review will detail much of this new information.
Assuntos
Sistema do Grupo Sanguíneo Kidd , Transplante de Rim , Humanos , Sistema do Grupo Sanguíneo Kidd/genética , Sistema do Grupo Sanguíneo Kidd/imunologia , Transportadores de Ureia , Eritroblastose Fetal/genética , Eritroblastose Fetal/imunologia , Eritroblastose Fetal/sangue , Recém-Nascido , Proteínas de Membrana Transportadoras/genética , Alelos , Antígenos de Grupos Sanguíneos/genética , Antígenos de Grupos Sanguíneos/imunologiaRESUMO
In pregnancy, D- pregnant women may be at risk of becoming immunized against D when carrying a D+ fetus, which may eventually lead to hemolytic disease of the fetus and newborn. Administrating antenatal and postnatal anti-D immunoglobulin prophylaxis decreases the risk of immunization substantially. Noninvasive fetal RHD genotyping, based on testing cell-free DNA extracted from maternal plasma, offers a reliable tool to predict the fetal RhD phenotype during pregnancy. Used as a screening program, antenatal RHD screening can guide the administration of antenatal prophylaxis in non-immunized D- pregnant women so that unnecessary prophylaxis is avoided in those women who carry a D- fetus. In Europe, antenatal RHD screening programs have been running since 2009, demonstrating high test accuracies and program feasibility. In this review, an overview is provided of current state-of-the-art antenatal RHD screening, which includes discussions on the rationale for its implementation, methodology, detection strategies, and test performance. The performance of antenatal RHD screening in a routine setting is characterized by high accuracy, with a high diagnostic sensitivity of ≥99.9 percent. The result of using antenatal RHD screening is that 97-99 percent of the women who carry a D- fetus avoid unnecessary prophylaxis. As such, this activity contributes to avoiding unnecessary treatment and saves valuable anti-D immunoglobulin, which has a shortage worldwide. The main challenges for a reliable noninvasive fetal RHD genotyping assay are low cell-free DNA levels, the genetics of the Rh blood group system, and choosing an appropriate detection strategy for an admixed population. In many parts of the world, however, the main challenge is to improve the basic care for D- pregnant women.
Assuntos
Sistema do Grupo Sanguíneo Rh-Hr , Imunoglobulina rho(D) , Humanos , Gravidez , Feminino , Sistema do Grupo Sanguíneo Rh-Hr/imunologia , Sistema do Grupo Sanguíneo Rh-Hr/genética , Sistema do Grupo Sanguíneo Rh-Hr/sangue , Imunoglobulina rho(D)/uso terapêutico , Imunoglobulina rho(D)/sangue , Diagnóstico Pré-Natal/métodos , Isoanticorpos/sangue , Isoanticorpos/imunologia , Eritroblastose Fetal/prevenção & controle , Eritroblastose Fetal/diagnóstico , Eritroblastose Fetal/sangue , Eritroblastose Fetal/imunologiaRESUMO
This guideline provides recommendations for the prevention of Rh D alloimmunization (isoimmunization) in pregnancy, including parental testing, routine postpartum and antepartum prophylaxis, and other clinical indications for prophylaxis. Prevention of red cell alloimmunization in pregnancy with atypical antigens (other than the D antigen), for which immunoprophylaxis is not currently available, is not addressed in this guideline. All Rh D-negative pregnant individuals at risk for Rh D alloimmunization due to potential exposure to a paternally derived fetal Rh D antigen. Routine postpartum and antepartum Rh D immunoprophylaxis reduces the risk of Rh D alloimmunization at 6 months postpartum and in a subsequent pregnancy. This guideline details the population of pregnant individuals who may benefit from Rho(D) immune globulin (RhIG) immunoprophylaxis. Thus, those for whom the intervention is not required may avoid adverse effects, while those who are at risk of alloimmunization may mitigate this risk for themselves and/or their fetus. For recommendations regarding use of RhIG, Medline and Medline in Process via Ovid and Embase Classic + Embase via Ovid were searched using both the trials and observational studies search strategies with study design filters. For trials, the Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, and Database of Abstracts of Reviews of Effects via Ovid were also searched. All databases were searched from January 2000 to November 26, 2019. Studies published before 2000 were captured from the grey literature of national obstetrics and gynaecology specialty societies, luminary specialty journals, and bibliographic searching. A formal process for the systematic review was undertaken for this update, as described in the systematic review manuscript published separately. The authors rated the quality of evidence and strength of recommendations using the SOGC's modified GRADE approach. See Appendix A (Tables A1 for definitions and A2 for interpretations of strong and conditional [weak] recommendations). The intended users of this guideline include prenatal care providers such as obstetricians, midwives, family physicians, emergency room physicians, and residents, as well as registered nurses and nurse practitioners. An updated Canadian guideline for prevention of Rh D alloimmunization addresses D variants, cffDNA for fetal Rh type, and updates recommendations on timing of RhIG administration.
Assuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Sistema do Grupo Sanguíneo Rh-Hr , Isoimunização Rh , Imunoglobulina rho(D)/uso terapêutico , Eritroblastose Fetal/sangue , Sangue Fetal/imunologiaRESUMO
BACKGROUND: Prehospital and early in-hospital use of low titer group O whole blood (LTOWB) for life-threatening bleeding has been independently associated with improved survival compared to component therapy. However, when RhD-positive blood products are administered to RhD-negative females of childbearing potential (FCP), there is a small future risk of hemolytic disease of the fetus and newborn (HDFN). This raises important ethical questions that must be explored in order to justify the use of RhD-positive blood products, including LTOWB, both in clinical practice and research. METHODS: This essay explores the ethical challenges related to RhD-positive blood product administration to RhD-negative or RhD-unknown FCPs as a first-line resuscitation fluid in the trauma setting. These ethical issues include: issues related to decision-making, ethical analysis based on the doctrine of double effect (DDE), and attendant obligations incurred by hospitals that administer RhD-positive blood to FCPs. RESULTS: Ethical analysis through the use of the DDE demonstrates that utilization of RhD-positive blood products, including LTOWB, in the early resuscitation of FCPs is an ethically appropriate approach. By accepting the risk of HDFN, hospitals generate obligations to promote blood donation, evaluate for alloimmunization and counsel patients on the future risk of HDFN, and maintain an understanding of the ethical rationale for RhD-positive blood transfusion.
Assuntos
Sistema do Grupo Sanguíneo Rh-Hr , Ferimentos e Lesões , Humanos , Ferimentos e Lesões/terapia , Feminino , Transfusão de Sangue/ética , Gravidez , Eritroblastose Fetal/sangueRESUMO
The current recommended testing algorithm for assessing the alloimmunized pregnancy utilized by many obstetricians in the United States (US) fails to consider the most recent evidence, placing fetuses, and mothers at unnecessary risk of poor outcome or death. This narrative review of the current landscape of fetal red blood cell (RBC) antigen testing evaluates the history of hemolytic disease of the fetus and newborn (HDFN) and how its discovery has continued to influence practices in the US today. We compare current US-based HDFN practice guidelines with those in Europe. We also provide transfusion medicine and hematology perspectives and recommendations addressing the limitations of US practice, particularly regarding paternal RBC antigen testing, and discuss the most valuable alternatives based on decades of data and evidence-based recommendations from Europe.
Assuntos
Eritroblastose Fetal , Guias de Prática Clínica como Assunto , Feminino , Humanos , Recém-Nascido , Gravidez , Eritroblastose Fetal/diagnóstico , Eritroblastose Fetal/sangue , Eritroblastose Fetal/prevenção & controle , Europa (Continente) , Isoanticorpos/sangue , Isoanticorpos/imunologia , Medição de Risco/métodos , Estados UnidosRESUMO
Objetivo: Determinar el grupo RhD fetal a través del estudio del gen RHD en ADN fetal que se encuentra libre en plasma de embarazadas RhD negativo. Método: Se analizó la presencia de los genes RHD, SRY y BGLO en ADNfl obtenido de plasma de 51 embarazadas RhD negativo no sensibilizadas, utilizando una qPCR. Los resultados del estudio genético del gen RHD se compararon con el estudio del grupo sanguíneo RhD realizado por método serológico en muestras de sangre de cordón, y los resultados del estudio del gen SRY fueron cotejados con el sexo fetal determinado por ecografía. Se calcularon la sensibilidad, la especificidad, los valores predictivos y la capacidad discriminativa del método estandarizado. Resultados: El gen RHD estaba presente en el 72,5% de las muestras y el gen SRY en el 55,5%, coincidiendo en un 100% con los resultados del grupo RhD detectado en sangre de cordón y con el sexo fetal confirmado por ecografía, respectivamente. Conclusiones: Fue posible deducir el grupo sanguíneo RhD del feto mediante el estudio del ADN fetal que se encuentra libre en el plasma de embarazadas con un método molecular no invasivo desarrollado y validado para este fin. Este test no invasivo puede ser utilizado para tomar la decisión de administrar inmunoglobulina anti-D solo a embarazadas RhD negativo que portan un feto RhD positivo.
Objective: To determine the fetal RhD group through the study of the RHD gene in fetal DNA found free in plasma of RhD negative pregnant women. Method: The presence of the RHD, SRY and BGLO genes in fetal DNA obtained from plasma of 51 non-sensitized RhD negative pregnant women was analyzed using qPCR. The results of the genetic study of the RHD gene were compared with the RhD blood group study performed by serological method in cord blood samples, and the results of the SRY gene study were compared with the fetal sex determined by ultrasound. Sensitivity, specificity, predictive values and discriminative capacity of the standardized method were calculated. Results: The RHD gene was present in 72.5% of the samples and the SRY gene in 55.5%, coinciding 100% with the results of the RhD group detected in cord blood, and with the fetal sex confirmed by ultrasound, respectively. Conclusions: It was possible to deduce the RhD blood group of the fetus through the study of fetal DNA found free in the plasma of pregnant women with a non-invasive molecular method developed and validated for this purpose. This non-invasive test can be used to make the decision to administer anti-D immunoglobulin only to RhD-negative pregnant women carrying an RhD-positive fetus.
Assuntos
Humanos , Feminino , Gravidez , Sistema do Grupo Sanguíneo Rh-Hr/genética , DNA , Eritroblastose Fetal/diagnóstico , Eritroblastose Fetal/genética , Fenótipo , Diagnóstico Pré-Natal , Sistema do Grupo Sanguíneo Rh-Hr/sangue , Valor Preditivo dos Testes , Sensibilidade e Especificidade , Imunoglobulina rho(D) , Genes sry/genética , Eritroblastose Fetal/sangue , Doenças Fetais/diagnóstico , Doenças Fetais/genética , Doenças Fetais/sangue , GenótipoRESUMO
El uso posnatal de altas dosis de inmunoglobulina endovenosa (IgGEV) aparece promisorio, en los escasos trabajos publicados, en reducir la necesidad de exsanguinotransfusiones (EXT) en el recién nacido (RN) con enfermedad hemolítica RH (EHRH). Objetivo: Evaluar la eficacia de la IgGEV para moderar la severidad de la hemólisis: necesidad de EXT, tiempo de luminoterapia (LMT) y número de transfusiones (T) en el RN con EHRH. Diseño: Ensayo clínico controlado y randomizado. Lugar de estudio: Hospital Materno Infantil Ramón Sardá. Población y métodos: Todos los RN con EHRH [Coombs directa (+)], fueron estratificados en 3 grados (leve, moderado y severo) según la severidad de la hemólisis evaluada intraútero. Solicitado el consentimiento informado a los padres, los RN de cada grado se randomizaron en: Grupo tratado: recibió IgGEV (500mg/kg/día por tres días, la primera dosis antes de las 2 horas posnatales) más LMT, y grupo control: solo LMT. Las variables estudiadas fueron: mínimo hematocrito (Hto), bilirrubina (Bi) máxima, n° de EXT y/o T, LMT (hs), internación (días) y tiempo hasta la EXT. Resultados: Entre febrero/99 y mayo/00 fueron enrolados 46 RN (grado leve: 24 y grado moderado-severo (gM-S): 22). 23 RN pertenecieron al grupo tratado y 23 al control. Ambos grupos fueron comparables en sexo, peso, EG y Hto, Bi, Hb y prot. totales de cordón. No se observaron diferencias significativas excepto en n° de EXT. De los 23 RN tratados, 5 requirieron EXT (21.74%) cuando su bilirrubina alcanzó el nivel establecido en las guías del hospital; 18 de los 23 RN controles requirieron EXT (78,26%), Chi2 0.0011. El número necesario a tratar (NNT) fue 1,8 para prevenir una EXT. La probabilidad de sufrir EXT es significativamente menor en un paciente tratado que en un control, en cualquier momento del período de estudio (Log Rank test p= 0,0024). Conclusiones: Estos resultados apoyan el beneficio clínico de tratar con IgGEV a los RN con incompatibilidad RH.
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Eritroblastose Fetal/tratamento farmacológico , Eritroblastose Fetal/sangue , Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas/uso terapêutico , Transfusão Total , Eritroblastose Fetal/terapia , ImunoterapiaRESUMO
Un individuo con un fenotipo eritrocitario raro carece de uno o varios antígenos presentes en la mayor parte de la población de pertenencia. Cuando presenta el anticuerpo correspondiente, se pueden producir complicaciones perinatales, transfusionales y/o transplantológicas. Se presenta el caso de una embarazada aloinmunizada derivada a nuestro servicio en la semana 12 de su tercera gesta para su evaluación y seguimiento. El diagnóstico inmunohematológico le asignó el excepcional fenotipo "p" (aproximadamente 1/200 000 individuos), asociado con una mayor tasa de abortos espontáneos y a reacciones transfusionales graves cuando se transfunden unidades incompatibles. El estudio del gen A4GALT demostró la presencia de la mutación c.752C > T en doble dosis. Esta mutación lleva a un cambio de una prolina por una leucina en el residuo 251 de la 4-α-galactosiltransferasa. Por parto inducido por sufrimiento fetal, nace a las 36 semanas una bebé con prueba de antiglobulina (Coombs) directa negativa, eluido reactivo, con ictericia que requirió luminoterapia. Una semana después el neonato fue externado sin secuelas aparentes. Posteriormente, a raíz de una cirugía inminente y la improbabilidad de encontrar sangre compatible, se elaboró un plan para cubrir las posibles demandas. Este caso pone en evidencia la necesidad de contar a nivel nacional con un laboratorio de referencia de inmunohematología y un banco de sangre de grupos raros, que permita resolver con celeridad situaciones que requieran transfundir a estos individuos.
A rare blood group is usually defined as the absence of a high prevalence antigen or the absence of several antigens within a single blood group system. These individuals may develop clinically significant red cell antibodies to the high incidence red cell antigens they lack. A 33-year-old alloimmunized woman was referred to our center at the 12th week of her third pregnancy for evaluation and follow up. The laboratory work-up grouped her as belonging to "p" phenotype, associated with difficulties to find compatible blood for transfusion and a high incidence of recurrent miscarriage. At 36 weeks, a baby girl was born by induced labor due to fetal suffering. With a negative direct antiglobulin test but a positive elution test, she was in the neonatology ward for one week receiving luminotherapy. Homozygosity for a missense mutation at position 752 (c.752C > T) in the A4GALT gene was found to be responsible for the p phenotype. This mutation changes a proline to a leucine at codon 251 of the 4-α-galactosyltransferase. Recently, due to an imminent chirurgical intervention and the impossibility to have compatible blood available for transfusion, an autologous donation plan was designed to satisfy probable demand. This case showed the need for blood bank facilities capable to respond satisfactorily to these situations in Argentina. This would facilitate the storage of cryopreserved blood from individuals with rare blood groups for homologous use or to develop rare blood donors programs.
Assuntos
Adulto , Feminino , Humanos , Gravidez , Eritroblastose Fetal/sangue , Galactosiltransferases/genética , Mutação de Sentido Incorreto , Sistema do Grupo Sanguíneo P/genética , Fenótipo , Sequência de Bases , Transfusão de Sangue , Glicosiltransferases/análiseRESUMO
En el feto los antígenos Duffy pueden ser detectados a las 6 o 7 semanas de gestación y están bien desarrollados al nacimiento. A pesar de su temprana expresión, la enfermedad hemolítica por incompatibilidad de grupo sanguíneo Duffy no es usual. Se presenta el caso un recién nacido con enfermedad hemolítica por incompatibilidad Duffy. Para su tratamiento se empleó fototerapia unida a un procedimiento hemoterapéutico: la exanguinotransfusión. Aunque la incompatibilidad por este sistema de grupo sanguíneo suele ser moderada, se debe estar alerta ante la ocurrencia de un conflicto con curso inusual, para brindar un tratamiento óptimo en el momento adecuado y disminuir la morbilidad de esta enfermedad.
Duffy antigens may be detected in the fetus at 6 or 7 weeks of gestation and be well developed at birth. Despite its early expression, the hemolytic disease due to Duffy blood group incompatibility is rare. The case of a newborn with hemolytic disease caused by Duffy incompatibility is presented. For its treatment, it was used phototherapy combined with a hemotherapeutic procedure: exanguinotransfusion. Although the incompatibility produced by this blood group system is usually moderate, one should be alert to the occurrence of a conflict with unusual course in order to apply an optimum treatment at the right time and to reduce the morbidity of this disease.
Assuntos
Humanos , Recém-Nascido , Eritroblastose Fetal/sangue , Eritroblastose Fetal/terapia , Fototerapia/métodos , Sistema do Grupo Sanguíneo Duffy/análiseRESUMO
En el feto los antígenos Duffy pueden ser detectados a las 6 o 7 semanas de gestación y están bien desarrollados al nacimiento. A pesar de su temprana expresión, la enfermedad hemolítica por incompatibilidad de grupo sanguíneo Duffy no es usual. Se presenta el caso un recién nacido con enfermedad hemolítica por incompatibilidad Duffy. Para su tratamiento se empleó fototerapia unida a un procedimiento hemoterapéutico: la exanguinotransfusión. Aunque la incompatibilidad por este sistema de grupo sanguíneo suele ser moderada, se debe estar alerta ante la ocurrencia de un conflicto con curso inusual, para brindar un tratamiento óptimo en el momento adecuado y disminuir la morbilidad de esta enfermedad(AU)
Duffy antigens may be detected in the fetus at 6 or 7 weeks of gestation and be well developed at birth. Despite its early expression, the hemolytic disease due to Duffy blood group incompatibility is rare. The case of a newborn with hemolytic disease caused by Duffy incompatibility is presented. For its treatment, it was used phototherapy combined with a hemotherapeutic procedure: exanguinotransfusion. Although the incompatibility produced by this blood group system is usually moderate, one should be alert to the occurrence of a conflict with unusual course in order to apply an optimum treatment at the right time and to reduce the morbidity of this disease(AU)
Assuntos
Humanos , Recém-Nascido , Eritroblastose Fetal/sangue , Eritroblastose Fetal/terapia , Sistema do Grupo Sanguíneo Duffy/análise , Fototerapia/métodosAssuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Eritroblastose Fetal/diagnóstico , Eritroblastose Fetal/imunologia , Eritroblastose Fetal/sangue , Análise Química do Sangue , Imunoglobulina rho(D)/análise , Imunoglobulina rho(D)/imunologia , Complicações Hematológicas na Gravidez , Diagnóstico Pré-Natal , Sistema do Grupo Sanguíneo Rh-Hr/imunologiaRESUMO
The Kidd blood group is clinically significant since the Jk antibodies can cause acute and delayed transfusion reactions as well as hemolytic disease of newborn (HDN). In general, HDN due to anti-Jk(b) incompatibility is rare and it usually displays mild clinical symptoms with a favorable prognosis. Yet, we apparently experienced the second case of HDN due to anti-Jk(b) with severe clinical symptoms and a fatal outcome. A female patient having the AB, Rh(D)-positive boodtype was admitted for jaundice on the fourth day after birth. At the time of admission, the patient was lethargic and exhibited high pitched crying. The laboratory data indicated a hemoglobin value of 11.4 mg/dL, a reticulocyte count of 14.9% and a total bilirubin of 46.1 mg/dL, a direct bilirubin of 1.1 mg/dL and a strong positive result (+++) on the direct Coomb's test. As a result of the identification of irregular antibody from the maternal serum, anti-Jk(b) was detected, which was also found in the eluate made from infant's blood. Despite the aggressive treatment with exchange transfusion and intensive phototherapy, the patient died of intractable seizure and acute renal failure on the fourth day of admission. Therefore, pediatricians should be aware of the clinical courses of hemolytic jaundice due to anti-Jk(b), and they should be ready to treat this disease with active therapeutic interventions.
Assuntos
Feminino , Humanos , Recém-Nascido , Bilirrubina/sangue , Eritroblastose Fetal/sangue , Evolução Fatal , Isoanticorpos/sangue , Sistema do Grupo Sanguíneo Kidd/imunologiaRESUMO
OBJETIVO: Relatar associação infreqüente de patologia que cause aumento considerável de produção de bilirrubina e outra diminuição importante na sua excreção. DESCRIÇÃO: Mãe tercigesta, Rh negativo. Na primeira gestação, gerou recém-nascido normal, de termo, não tendo recebido imunoglobulina humana anti-RhD. A segunda gestação complicou-se por isoimunização Rh, dando à luz neonato de termo, o qual necessitou três exsanguinotransfusões e faleceu com 8 dias de vida. Na gestação atual, conseguiu dar à luz a termo recém-nascido tipo ORh positivo, Coombs direto positivo, bilirrubina de cordão 6,5 mg/dl e hematócrito 44 por cento. Com 5 horas de vida, estava ictérico, tendo sido iniciados fenobarbital (por 3 dias) e fototerapia intensiva. A hiperbilirrubinemia foi logo controlada, porém ascendia rapidamente sempre que a fototerapia era suspensa. No 10° dia de vida, a criança foi transfundida por anemia importante. Em vista da persistência da icterícia, no 13° dia de vida pensou-se em associação com síndrome de Gilbert, e o seqüenciamento de DNA foi solicitado. O resultado mostrou genótipo mutante homozigoto UDPT1A1[TA]7TAA. Permaneceu em fototerapia até o 17° dia de vida. Recebeu alta no dia seguinte, após controle de bilirrubinemia. Voltou para acompanhamento ambulatorial e apresentou desenvolvimentos pondo-estatural e neurológico normais. COMENTARIOS: O caso ressalta a importância da associação do aumento de produção/diminuição de excreção de bilirrubina na gênese de hiperbilirrubinemias prolongadas, intensas e passíveis de causar kernicterus, se não tratadas vigorosamente. Demonstra, ainda, a eficácia da fototerapia intensiva, reduzindo os riscos de tratamentos mais agressivos. Ressalta, também, a importância do acompanhamento das icterícias neonatais até a completa remissão dos sintomas.
Assuntos
Adulto , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Eritroblastose Fetal/sangue , Doença de Gilbert/complicações , Hiperbilirrubinemia Neonatal/etiologia , Bilirrubina/sangue , Doença de Gilbert/sangue , Hiperbilirrubinemia Neonatal/sangue , Hiperbilirrubinemia Neonatal/terapia , Icterícia Neonatal/sangue , Icterícia Neonatal/complicações , Icterícia Neonatal/terapia , Fototerapia , Fatores de TempoRESUMO
Anti-U is a rare red blood cell alloantibody that has been found exclusively in blacks. It can cause hemolytic disease of the newborn and hemolytic transfusion reactions. We describe the case of a female newborn presenting a strongly positive direct antiglobulin test due to an IgG antibody in cord blood. Anti-U was recovered from cord blood using acid eluate technique. Her mother presented positive screening of antibodies with anti-U identified at delivery. It was of IgG1 and IgG3 subclasses and showed a titer of 32. Monocyte monolayer assay showed moderate interaction of Fc receptors with maternal serum with a positive result (3.1 percent). The newborn was treated only with 48 hours of phototherapy for mild hemolytic disease. She recovered well and was discharged on the 4th day of life. We conclude that whenever an antibody against a high frequency erythrocyte antigen is identified in brown and black pregnant women, anti-U must be investigated
Assuntos
Humanos , Recém-Nascido , Eritroblastose Fetal , Isoanticorpos , Eritroblastose Fetal/sangue , Eritrócitos/imunologia , Doenças Hematológicas , Imunoglobulina G/sangue , Isoanticorpos/sangueRESUMO
Se estudiaron 251 recién nacidos a término y sus respectivas madres en la Maternidad Concepción Palacios, Caracas-Venezuela, con el fin de establecer la frecuencia de enfermedad hemolítica (EH) y tratar de determinar parámetros útiles para la predicción de la severidad de la EH-ABO. Hubo 23 casos de incompatibilidad ABO con los siguientes hallazgos serológicos: 9 (39) por ciento presentaron la prueba de autoaglutinación positiva, 5(21 por ciento) el Coombs directo positivo, 20 (36 por ciento) el eluido positivo. De cuatro (2 por ciento) casos con EH-ABO, dos (50 por ciento) tuvieron Coombs directo positivo y tres (75 por ciento) la prueba de autoaglutinación y el eluido positivo. El título de anticuerpos maternos varió entre 1:12 y 1:4096. La determinación semicuantitativa de las subclases IgG en 3 casos de EH-ABO demostró en forma constante la presencia de IgG3 e IgG4 y en 2 casos se asoció la IgG2. Se determinó la presencia de sustancia A en el suero de 14 niños con incompatibilidad ABO, no observándose aparentemente el efecto protector del carácter secretor. Se presentaron 2 casos de EH-Rh, los cuales tuvieron un comportamiento diferente, lo cual podría atribuirse a las subclases de IgG presentes en el suero materno
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Adolescente , Adulto , Eritroblastose Fetal/sangue , Antígenos de Grupos Sanguíneos/análise , Hiperbilirrubinemia/patologiaRESUMO
Os autores relatam a experiencia com 65 transfusoes intravasculares no tratamento da Doenca Hemolitica Perinatal realizada em 25 gestacoes complicadas por aloimunizacao Rh no periodo de janeiro a dezembro de 1991. As transfusoes comecaram entre 20-34 semanas e foram repetidas ate 6 vezes, com intervalos de 3-28 dias...