摘要
Resumen Introducción: La determinación del grupo sanguíneo ABO/RhD y la prueba directa de Coombs (PDC) al nacimiento son una práctica recomendada, pero existe variabilidad en su implementación universal. Se presentan los resultados de la determinación al nacimiento del grupo ABO/RhD y la PDC en una cohorte institucional. Métodos: Se incluyeron los recién nacidos entre 2017 y 2020 en un hospital de atención a embarazos de alto riesgo. Se determinó el grupo ABO/RhD y se realizó la PDC en muestras de cordón umbilical o en las primeras 24 horas de vida. Se registraron las variables demográficas, maternas y neonatales. Se estimó la asociación entre las variables mediante la razón de probabilidad (OR). Resultados: Se incluyeron 8721 binomios. La PDC fue positiva en 239 recién nacidos (2.7%), siendo las variables asociadas a la PDC positiva la edad materna > 40 años (OR: 1.5;IC95%: 1.0-2.3), el nacimiento por vía cesárea (1.4; 1.1-2.0), la madre del grupo O (6.4; 3.8-11.8), la prematuridad (3.6; 2.6-5.0); el peso al nacer < 2500 g (2.1; 1.6-2.8); el neonato del grupo A (15.7; 10.7-23.1) o del grupo B (17.6; 11.4-27.2), la hemoglobina al nacer < 13.5 g/dl (4.5; 2.8-7.1) y la reticulocitosis > 9% (1.9; 1.2 a 3.1). Discusión: La frecuencia de PDC positiva neonatal es del 2.7%, con asociación significativa la incompatibilidad materna/neonatal al grupo ABO y RhD, con impacto significativo en diversas variables neonatales. Estos resultados apoyan la política de implementación universal al nacimiento de la determinación de ABO/RhD y PDC.
Abstract Background: Evaluating the ABO/RhD blood group and the direct antiglobulin Coombs test (DAT) at birth is recommended good practice, but there is variability in its universal implementation. This study aims to show the comparative results in various variables of clinical impact during the hospital stay of neonates with positive DAT compared with those with negative DAT, based on the systematic detection of the ABO/RhD group and DAT at birth. Methods: Newborns between 2017 and 2020 in a high-risk pregnancy care hospital were included. The ABO/RhD and DAT group was determined in umbilical cord samples or the first 24 hours of life. Demographic, maternal, and neonatal variables were recorded. The association between the variables was estimated using the odds ratio (OR). Results: 8721 pairs were included. The DAT was positive in 239 newborns (2.7%), with the variables associated with positive PDC being maternal age > 40 years (OR: 1.5; 95% CI: 1.0 to 2.3), birth by cesarean section (1.4; 1.1-2.0), mother group O (6.4; 3.8-11.8), prematurity (3.6; 2.6-5.0), birth weight < 2500 g (2.1; 1.6-2.8), newborn group A (15.7; 10.7-23.1) and group B (17.6; 11.4-27.2), hemoglobin at birth < 13.5 g/dl (4.5; 2.8-7.1) and reticulocytosis > 9% (1.9; 1.2 to 3.1). Discussion: The frequency of neonatal positive PDC was 2.7%, with a significant association with maternal/neonatal incompatibility to the ABO and RhD group, with a substantial impact on various neonatal variables. These results support the policy of universal implementation at the birth of the ABO/RhD and DAT determination.
摘要
OBJECTIVE To provide reference for safe drug use in patients with anaplastic lymphoma kinase （ALK）-positive non-small cell lung cancer （NSCLC）. METHODS Clinical pharmacists participated in the diagnosis and treatment of a patient with ALK-positive NSCLC who developed bilateral pleural effusion and hemolytic anemia after taking alectinib； regarding symptoms such as pleural effusion and hemolytic anemia in the patient， clinical pharmacists investigated the patient’s history of medication and disease， as well as potential drug interaction； to consider the correlation between the patient’s use of alectinib and the duration of pleural effusion and hemolytic anemia， clinical pharmacists suggested that clinical doctors discontinued alectinib and used reduced dose treatment after the pleural effusion improved， but the patient suffered from bilateral pleural effusion and hemolytic anemia again； after evaluating the correlation between alectinib and bilateral pleural effusion and hemolytic anemia using the Naranjo’s assessment scale， clinical pharmacists recommend permanent discontinuation of alectinib and jointly recommend replacement with ensartinib with clinical physicians. RESULTS Physicians adopted the suggestions of clinical pharmacists. The pleural effusion subsequently regressed and hemolytic anemia improved after replacing the drug. The correlation between alectinib and bilateral pleural effusion and hemolytic anemia was confirmed. CONCLUSIONS Clinical pharmacists participate in pharmaceutical monitoring of ALK-positive NSCLC patients， assist clinical doctors in developing personalized medication recommendations， and ensure the safety of patient medication.
摘要
【Objective】 To identify the specificity of alloantibody against high-frequency antigens in one case suffering with severe hemolytic diseases of the fetus and newborn (HDFN) and to screen for matching blood for transfusion. 【Methods】 The HDFN test and the antibody serological identification tests in the mother were performed. Several common high frequency antigens of maternal red blood cells (RBCs) were determined. IgG subtype coated on the RBCs of the newborn was determined. The phagocytic efficiency of the antibody was tested using the monocyte phagocytosis of sensitized erythrocyte by flow cytometry in vitro. Sanger sequencing of DI gene was performed in the mother, father and mother’s brother. The diluted maternal plasma was used for large scale screening of matching blood using IAT in Coomb’s gel card. 【Results】 Di(b-) phenotype was identified in the mother of the newborn and anti-Dib (titer: 512) related HDN was detected in the newborn. IgG1 and IgG2 subtypes of anti-Dib were detected and the rate of monocyte phagocytosis was 88.83%(74.7/84.09). The compatible blood was not detected in the maternal relatives. Subsequently, the newborn received the matching RBCs of two Di(b-) donors identified from 5 520 blood donors and discharged from the hospital. We screened out 17 Di(b-) donors out of 51 334 blood donors, indicating that the distribution frequency of Di(b-) among blood donors in Guangzhou was about 0.033% (17/51 334). 【Conclusion】 By serology and molecular biology methods, the newborn was identified with HDFN caused by anti-Dib, and an effective large-scale screening method for Di (b -) rare blood types was established to find matching blood, which supported the establishment of rare Di(b-) blood database.
摘要
【Objective】 To investigate the blood transfusion compatibility test results of a child with thalassemia after delayed hemolysis reaction, and to formulate transfusion strategies based on other clinical data to ensure the safety of clinical blood use. 【Methods】 A comprehensive analysis was conducted on the transfusion compatibility test results of a child with thalassemia, combined with Rh blood type system detection, blood transfusion history, serum bilirubin changes, clinical symptoms and other data to determine the occurrence of delayed hemolytic reaction, and formulate the subsequent appropriate blood matching strategies. 【Results】 On admission, the blood type of the patient was B DccEE, with hemoglobin (Hb) 38 g/L, reticulocyte ratio (Ret%) 2.92%, total bilirubin (TBil) 65.8 μmol/L, direct bilirubin (DBil) 12.0 μmol/L, indirect Bilirubin (IBil) 53.8 μmol/L, negative for unexpected antibody screening, no agglutination or hemolysis on both primary and secondary sides of the cross-matching over with type B DCcEe donors, negative for direct anti human globulin test(DAT), negative for indirect anti human globulin test (IAT), lactate dehydrogenase(LDH) 1 050 U/L, aspartate aminotransferase(AST) 113 U/L, urine occult blood 2+, urine bilinogen 4+, and a history of red blood cell transfusion 5 days before admission. The above results indicated that the child had a delayed hemolytic reaction, then type B DccEE leukocyte-depleted suspension red blood cell was transfused, and various indicators improved after transfusion. 【Conclusion】 Appropriate blood matching strategies should be formulated based on the results of blood transfusion compatibility testing and other clinical data before transfusion for children with thalassemia in order to effectively ensure transfusion safety.
摘要
【Objective】 To explore a transfusion therapeutic plan for hemolytic disease of the newborn(HDN) caused by anti-c antibodies in mother of Asian-type DEL. 【Methods】 The ABO blood type and Rh phenotype of the mother and child were determined using the saline tube method. The mother′s RhD negativity was confirmed through classical anti-human globulin testing and RhD adsorption-elution test. The mother′s unexpected antibodies were screened and their titers were measured using classical anti-human globulin testing, antibody card test and polyamine method. Cross-matching was conducted. The three hemolysis tests were performed by antibody card test. The mother′s RHD gene typing was conducted using a commercially available RHD negative identification gene detection kit (PCR-SSP method). 【Results】 The mother exhibited a CCee Rh serological phenotype, and the DEL gene test confirmed the presence of RHD*1227A, indicating the production of anti-c antibodies. The infant displayed a DCcEe Rh serological phenotype, positive for direct antiglobulin test and red cell elution. Based on the mother′s obstetric history, clinical manifestations of the infant and blood examination results, the diagnosis was HDN caused by anti-c antibodies. 【Conclusion】 For infants with HDN caused by anti-c antibodies in mother of Asian-type DEL, DCCee Rh phenotype red blood cell transfusion is recommended, while CCee Rh phenotype deglycerolized red blood cell transfusion is recommended for the mother.
摘要
【Objective】 To elucidate the prediction ability of monocyte monolayer assay(MMA) used in hemolytic disease of fetus and newborn(HDFN) caused by IgG anti-M. 【Methods】 Plasma from eight pregnant women containing IgG anti-M were collected, and were divided into two groups(4 cases with HDFN, with severe clinical symptoms such as fetal hydrops, and 4 cases without HDFN) according to the clinical outcomes. M antigen positive cells were sensitized with dithiothreitol(DTT) treated plasma from eight pregnant women respectively. MMA was performed by coincubation with monocytes and sensitized M cells, along with negative and positive control set up. T-test was conducted to compare the difference in phagocytic efficiency between two groups. 【Results】 The phagocytic efficiency in group with HDFN were 15.37%, 13.05%, 9.17% and 24.50% respectively, with the mean value of 15.52%, while the group without HDFN were 8.74%, 11.07%, 5.12% and 6.23% respectively, with the mean value of 7.79%.There was no significant difference in phagocytic efficiency between two groups(P>0.05). The mean values of both groups were not significantly different from the negative control(P>0.05), but both were significantly lower than positive control(P<0.05). 【Conclusion】 The low phagocytic efficiency couldn’t convince that the MMA is an effective predictor for the HDFN caused by IgG anti-M, indicating that another mechanism might be responsible for it rather than monocyte phagocytosis. The assessment of the peak systolic velocity in middle cerebral artery of the fetal should be considered in the management for pregnant women who produce IgG anti-M to estimate the situation of fetal anemia.
摘要
【Objective】 To analyze the application of serological test results in the diagnosis and treatment of anti-M-induced hemolytic disease of the fetus and newborn(HDFN), and to explore HDFN prevention strategies. 【Methods】 The serological test results of 12 cases of HDFN caused by anti-M diagnosed in our laboratory from January 2017 to December 2023 were retrospectively analyzed, including blood group identification of mothers and children, serum total bilirubin/hemoglobin/antibody titer test, and three hemolysis tests in newborns. Clinical data of the children and mothers were collected, including pregnancy history, blood transfusion history, prenatal antibody testing, history of intrauterine blood transfusion and gestational week of delivery, and the prognosis of the children was followed up. 【Results】 All 12 cases of fetal neonatal hemolytic disease due to anti-M were RhD+ MN phenotype newborn born to RhD+ NN mother, with maternal- fetal incompatiblility in MN blood groups. In the ABO blood group system, ABO incompatibility between mother and child accounted for 41.7%(5/12).None of the mothers had a history of blood transfusion, and the median titer of the test at 4℃ was 32, and the median titer at 37℃ was 4. The mothers of 3 cases had a history of multiple intrauterine blood transfusions, with an incidence of 25%(3/12). One case had an abnormal first pregnancy, with an incidence of 8.3%(1/12), and seven cases had an abnormal pregnancy with a miscarriage, with an incidence of abnormal pregnancy and birth history of 58.3%(7/12). There were 6 cases of premature labor, with an incidence of 50%(6/12). The mothers in three cases underwent regular obstetric examination and the specificity of the antibodies was determined, accounting for 25%(3/12). Twelve children had free antibodies with a median titer of 6 at 4℃ and 2 at 37℃. Two children had anti-M antibodies that were not reactive at 37℃, with a negative rate of 16.7%(2/12). The positive rate of DAT and elution test was respectively 8.3%(1/12) and 16.7%(2/12) in the children. The median minimum hemoglobin value was 75 g/L, and all 12 children received blood transfusions. The median peak total bilirubin value was 157.5 μmol/L, and none of them reached the threshold for blood exchange. The rate of delayed anemia was 16.7%(2/12), the postnatal mortality rate was 8.3%(1/12), and 11 children was free of growth and neurodevelopmental delay in prognosis. 【Conclusion】 Anti-M can cause severe HDFN, which can also occur in primigravida. The intensity of antibody titer does not correlate with the severity of the disease, and it is prone to cause delayed anemia, which should be monitored regularly according to the serological characteristics of anti-M and clinical symptoms, and should be treated timely.
摘要
【Objective】 To analyze the characteristics and related influencing factors of transfusion reactions in pediatric patients, so as to provide evidence for clinical treatment and prevention of transfusion reactions. 【Methods】 A retrospective study was conducted on children who received blood transfusions at our hospital from 2019 to 2023 in terms of the incidence, types, time of occurrence, and related influencing factors of transfusion reactions. 【Results】 A total of 69 926 transfusions were performed from 2019 to 2023, with 711 cases of transfusion reactions, resulting in an incidence of 1.02%. The incidence of transfusion reactions decreased annually from 2019 (1.89%) to 2022 (0.50%). The incidence of transfusion reactions to apheresis platelets, frozen plasma, suspended leukocyte-depleted red blood cells and cryoprecipitate coagulation factor were 2.16% (551/25 565), 0.50% (92/18 277), 0.25% (65/25 679) and 0.74% (3/405), respectively, with apheresis platelet transfusion of a significantly higher incidence compared to other blood components (P<0.05). As of transfusion reaction types, allergic reactions accounted for 86.22% (613/711), febrile non-hemolytic transfusion reactions (FNHTR) accounted for 13.08% (93/711), and acute hemolytic transfusion reactions accounted for 0.70% (5/711). Multivariate logistic regression analysis showed that apheresis platelet transfusion was an independent risk factor for allergic reactions (P<0.05), while suspended leukocyte-depleted red blood cells transfusion was an independent risk factor for FNHTR (P<0.05). In terms of the occurrence time of transfusion reactions, compared to apheresis platelets and frozen plasma, transfusion reactions caused by suspended leukocyte-depleted red blood cells transfusion occurred later (apheresis platelets P<0.05, frozen plasma P<0.05). 【Conclusion】 The results suggest that blood components transfused are significant influencing factors for the characteristics of transfusion reactions in pediatric patients, affecting the incidence, types, and occurrence time of transfusion reactions, which provides reference for clinical treatment and prevention of transfusion reactions.
摘要
【Objective】 To make transfusion management strategies for patients with history of blood transfusion and/or pregnancy by following up a patient with delayed hemolytic transfusion reactions(DHTR) caused by unexpected antibody produced after blood transfusion. 【Methods】 ABO, Rh, MN and Kidd blood group test, direct antiglobulin test, unexpected antibody screening, antibody identification, antibody titer detection, and cross-matching test were performed on a patient with DHTR. Meanwhile, suitable red blood cells were screened for subsequent treatment. 【Results】 The patient′s blood group was B, RhD(+ ) and CCDee, the antibody screening test and cross-matching test were negative before the first transfusion. After eight days, hemoglobin of the patient decreased to 57 g/L and the laboratory results indicated delayed hemolysis, the antibody screening was positive, and the antibody identification result was anti-M, as RBCs of the patient received typed as M+ N+. After the patient received M antigen negative RBCs, the laboratory test results still indicated delayed serologic transfusion reaction. A new antibody arose and was identified as anti-Jka while RBCs transfused were M-N+ and Jk(a+ b-). Afterwards, it was effective for the patient to receive B, RhD(+ ), M-N+ and Jk(a-b+ ) RBCs. 【Conclusion】 Most of the homologous antibodies produced by patients after blood transfusion will disappear within a few years. When patients undergo another transfusion, DHTR may occur because of anamnestic reaction. Establishing a transfusion management document and creating a card for patients who have already produced RBC alloantibodies can greatly reduce the occurrence of DHTR by informing doctors and staff when the next transfusion is needed.
摘要
【Objective】 To investigate the serological changes of blood type and whether hemolytic reactions occurred in a patient with ABw subtype after type A therapeutic plasma exchange(TPE) retrospectively. 【Methods】 The ABO blood group identification and genotyping was carried out by microcolumn gel method, microcolumn glass bead method, saline tube method and absorption-elution test, and the laboratory results before and after TPE were analyzed. 【Results】 The patient underwent 2 000 mL type A TPE in other hospitals and was transferred to our hospital. On the second day of hospitalization, the test showed consistency between forward and reverse blood typing. On the third day, the detection showed that Bc was ±~1+ , and discrepancy was found between forward and reverse typing. The presence of B antigen was confirmed through tube method and human anti-B absorption-elution test, and was identified as ABw03 by molecular biological method. After type A TPE, Hb and Plt both temporarily decreased, creatinine slightly increased, ALT and AST continued to decrease, while TBIL, DBIL and IBIL showed a temporary decrease followed by an increase, indicating a slight hemolytic reaction. 【Conclusion】 Infusion of large volume of plasma may cause hemolytic reactions when ABO subtype patients contain weak A or B antigens.The blood type should be strictly identified according to the ABO blood type forward and reverse typing standards. Additional laboratory tests combined with molecular biology techniques are required for weak agglutination, which can reduce the missed detection of ABO subtypes and reduce the occurrence of transfusion reactions.
摘要
【Objective】 To study the changes in serum immunoglobulin levels in children with thalassemia who undergo repeated blood transfusions and explore their correlation with delayed hemolytic transfusion reactions(DHTR). 【Methods】 Serum samples from children with thalassemia who received blood transfusion treatment from June 2022 to April 2023 (observation group) and healthy children who underwent physical examination (control group) in our hospital were collected. The levels of serum immunoglobulins (IgG subtype, IgM, IgA, IgE and IgD) were detected using flow cytometry CBA multi-factor quantitative detection technology, and the differences between the two groups were compared. The children were divided into 4 groups according to different transfusion numbers: ≤10 numbers, 11-30 numbers, 31-50 numbers and >50 numbers, and the differences between different blood transfusion numbers and serum immunoglobulin levels in each group were compared using one-way analysis of variance (ANOVA). Children with thalassemia with DHTR were in the hemolysis group, and children with thalassemia who did not experience DHTR were in the non-hemolysis group. The changes in serum immunoglobulins (IgG subtypes, IgM, IgA, IgE and IgD) between the two groups were compared to explore the correlation between serum immunoglobulins in thalassemia children with repeated transfusion and DHTR. 【Results】 The levels of IgG1, IgG3, IgG4 and IgA in the observation group were significantly higher than those in the control group, with the increase of(2.07±2.12), (0.67±2.03), (0.30±0.37)and(6.04±11.40)mg/mL, respectively, while the level of IgD in observation group was significantly lower than that in the control group, with a decrease of(0.03±0.01)mg/mL, P0.05). IgG1 and IgG4 both significantly increased with the number of blood transfusions.The IgG1 in the 4 groups increased sequentially as(0.30±0.62), (0.41±0.51)and(3.60±3.48)mg/mL, and IgG4 increased sequentially as (0.12±0.13), (0.22±0.07) and (0.21±0.38)mg/mL. IgG2, IgM and IgD showed a significant decrease, with IgG 2, IgM, and IgD in four groups decreased as(0.91±1.50), (0.14±0.10)and(0.05±0.05)mg/mL, respectively, showing significant differences with the number of blood transfusions(P0.05). IgG1, IgG3 and IgG4 in the hemolysis group were significantly higher than those in the non-hemolysis group, with an increase of (4.44±3.41), (0.73±1.26)and(0.52±0.40), respectively(P0.05). 【Conclusion】 The serum immunoglobulin levels of children with thalassemia who undergo repeated blood transfusions are abnormal. There are differences in correlation between the number of blood transfusions and serum immunoglobulin levels among children with thalassemia who undergo repeated blood transfusions. The relevant serum immunoglobulins for DHTR in children with thalassemia who undergo repeated blood transfusions are IgG1, IgG3 and IgG4.
摘要
【Objective】 To analyze the antibody types of autoimmune hemolytic anemia(AIHA) patients in Panyu district, Guangzhou and track the therapeutic effect of blood transfusion, so as to provide reference for clinical transfusion treatment strategy of AIHA patients. 【Methods】 From January 2021 to October 2023, 96 ambiguous cross-matching blood samples from Blood Transfusion Departments of local hospitals sent to Panyu Central Blood Station were analyzed, and 25 samples of AIHA patients were identified. Then blood group identification, Rh system antigen phenotyping, antibody screening and cross-matching were further performed to analyze the correlation between antibody types and transfusion efficacy in AIHA patients. 【Results】 Among the 25 samples of AIHA patients, 17 showed consistency between forward and reverse blood grouping and 8 showed discrepancy. There were 19 (19/25, 76%) samples incompatible in cross match on the major side, of which 18 (18/19, 94.7%) were positive for direct Coombs test, autoantibodies and non-specific antibodies, and 1 (1/19, 5.3%)was positive for autoantibody and alloantibody.There were 6 (6/25, 24%) samples compatible in cross match on the major side, of which 3 (3/6, 50%) were positive for autoantibodies, 3 (3/6, 50%) were positive for autoantibody and alloantibody. Of the 25 AIHA patients, 20 received blood transfusion treatment and could be traced, and 5 patients did not receive blood transfusion treatment or transferred to other hospitals and could not be traced. Blood transfusion was effective in 11 (11/20, 55%) cases, partially effective in 6 (6/20, 30%) cases, and ineffective in 3 (3/20, 15%) cases. Among the ABO blood group incompatibility samples, transfusion was effective or partially effective in 17 (17/20, 85%) cases. 【Conclusion】 The transfusion efficacy of AIHA patients is not directly related to the results of cross-matching. Under the premise of regulating the autoimmune environment and eliminating the ABO blood group incompatibility caused by unexpected alloantibodies, AIHA patients with incompatible cross-matching can be transfused when necessary, and transfusion of ABO and Rh system antigen homologous blood can improve the safety and efficiency of transfusion.
摘要
Objective To analyze the research status and trend of scarlet fever literature in China, and to provide reference for subsequent research. Methods Three major Chinese databases, CNKI, Wanfang, and VIP, as well as Web of Science English database, were used to search for literature related to scarlet fever from 2000 to 2023. Citespace6.2.R2 software was used to statistically analyze the number of publications, authors, institutions and journals, co-cited literature, keyword clustering, and other literature characteristics of the literature. Results From 2000 to 2023, a total of 1 011 Chinese literature were included in the three major Chinese databases. Since 2011, the number of publications had gradually increased, but in recent years, the number of publications had decreased. The organization with the most publications was the Shenyang Center for Disease Control and Prevention. The cluster analysis of key words mainly formed 9 cluster tags, and the high-frequency keywords mainly included epidemic characteristics, epidemiology, incidence rate, etc. A total of 84 English literature were included in the WOS database, with an overall upward trend in publication volume. The institution with the most publications was the China Center for Disease Control and Prevention, and the most frequently cited journal was “LANCET INFECT DIS”.《Resurgence of scarlet fever in China: a 13-year population-based surveillance study》 was the most cited journal. After keyword cluster analysis, 9 cluster labels were mainly formed, and the keywords were mainly outbreak，Hong Kong, and Group A streptococcus. Conclusion Compared with the English literature, which mainly focuses on spatiotemporal aggregation, etiology and strain resistance, Chinese literature focuses more on epidemic surveillance, clinical features and quality nursing.
摘要
ABSTRACT Objective: The objectives of this study were to describe the first pediatric case of cold agglutinin syndrome (CAS) triggered by human adenovirus and review the literature. Case description: This case report involves a previously healthy, 2½-year-old female child with human adenovirus isolated in a nasal swab. At 72 h after admission, the patient progressed to a severe episode of anemia (hemoglobin level: 2.6 g/dL). The laboratory findings were consistent with CAS. The patient received blood transfusion, vitamin supplementation, adequate hydration, and thermal protection. At her last follow-up, 1 year after her initial presentation, she remains clinically well without signs of hemolysis. Comments: While severe CAS is extremely uncommon in the pediatric emergency department, human adenovirus infection is a common illness in pediatrics. Recently, the adenovirus has been associated with new complications (acute hepatitis and fulminant liver failure). Pediatric physicians and hematologists should be aware of unusual evolution, signs, and symptoms of this infection that warrant more urgent medical attention. In this case, the hematologic complication suspicion was the key to early diagnosis and adequate management.
RESUMO Objetivo: Descrever o primeiro caso pediátrico de síndrome da crioaglutinina desencadeado por adenovírus humano e revisar a literatura. Descrição do caso: Paciente do sexo feminino, dois anos e seis meses, previamente hígida e diagnosticada com adenovírus humano isolado em swab nasal. Após 72 horas da admissão, a paciente evoluiu com quadro de anemia grave (hemoglobina de 2,6 g/dL). Os achados laboratoriais foram compatíveis com síndrome da crioaglutinina. A paciente recebeu transfusão de concentrado de hemácias, suplementação vitamínica, hidratação adequada e proteção térmica. Em seu último retorno ambulatorial, um ano após a apresentação inicial, permanecia clinicamente bem, sem sinais de hemólise. Comentários: Enquanto a síndrome da crioaglutinina grave é extremamente incomum na emergência pediátrica, a infecção por adenovírus humano é um quadro comum na infância. Recentemente, o adenovírus tem sido associado a novas complicações, e pediatras e hematologistas devem ficar atentos à possibilidade de uma evolução incomum dessa infecção e dos sinais e sintomas que possam necessitar de atenção urgente. No caso apresentado, a suspeita da complicação hematológica foi a chave para o diagnóstico precoce e seu manejo adequado.
摘要
ABSTRACT Objective: To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD). Case description: Both patients started with a painful crisis and had acute chest syndrome during hospitalization. They showed significant worsening of hemolytic anemia, with very high levels of lactate dehydrogenase, thrombocytopenia, lowered level of consciousness, organ damage and the presence of schistocytes in peripheral blood. Due to the possibility of TMA, despite the very rare association with SCD, they were treated with fresh frozen plasma replacement and plasmapheresis, with good response. Comments: TMA is a serious, life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. The association of SCD and TMA is difficult to diagnose, since they can share a similar clinical presentation. Recognizing this association and promptly instituting treatment may impact the survival of these patients.
RESUMO Objetivo: Descrever dois casos de pacientes que apresentaram microangiopatia trombótica (MAT) associada à doença falciforme (DF). Descrição do caso: Ambos os pacientes iniciaram com crise dolorosa e apresentaram síndrome torácica aguda durante a internação. Eles apresentaram piora significativa da anemia hemolítica, com níveis muito elevados de lactato desidrogenase, trombocitopenia, rebaixamento do nível de consciência, lesão de órgãos e presença de esquistócitos no sangue periférico. Diante da possibilidade de MAT, apesar da associação muito rara com DF, eles foram tratados com reposição de plasma fresco congelado e plasmaférese, com boa resposta. Comentários: A MAT é uma doença grave e com risco de vida, caracterizada por anemia hemolítica microangiopática, trombocitopenia e danos a órgãos. A associação de DF e MAT é de difícil diagnóstico, pois as duas podem ter apresentação clínica semelhante, portanto reconhecer essa associação e instituir o tratamento prontamente pode ter grande impacto na sobrevida desses pacientes.
摘要
El síndrome hemolítico urémico secundario a Streptococcus pneumoniae (SHU-Sp) es una complicación poco frecuente de las enfermedades invasoras por S. pneumoniae. Presenta una alta morbimortalidad, con requerimiento de transfusiones de glóbulos rojos y plaquetas, terapia de sustitución de la función renal de inicio precoz y más prolongada, así como mayores complicaciones a largo plazo, comparado con las formas secundarias a infección entérica por Escherichia coli productora de toxina Shiga. Presentamos el caso clínico de una preescolar de dos años, previamente sana, vacunada con tres dosis de PCV13, que desarrolló una insuficiencia renal aguda, anemia hemolítica y plaquetopenia, en el contexto de una neumonía con empiema y bacteriemia por S. pneumoniae.
Streptococcus pneumoniae associated hemolytic uremic syndrome (Sp-HUS) is an uncommon complication of invasive pneumococcal infections. Patients with Sp-HUS have a higher mortality and long term morbidity than those due to HUS from Shiga toxin-producing Escherichia coli infections (STEC-HUS). They often require more red blood cells and platelet transfusions, and early initiation of renal substitution therapy, presenting a higher rate of arterial hypertension and chronic renal disease in the long term, compared to STEC-HUS. We report a healthy 2 year-old infant, vaccinated with three doses PCV13, that developed acute renal failure, hemolytic anemia and thrombocytopenia in the course of a complicated pneumococcal pneumonia with empyema and bacteremia.
Subject(s)
Humans , Female , Child, Preschool , Pneumococcal Infections/complications , Hemolytic-Uremic Syndrome/etiology , Pneumococcal Infections/therapy , Pneumococcal Infections/diagnostic imaging , Streptococcus pneumoniae , Thrombocytopenia , Radiography, Thoracic , Renal Insufficiency , Hemolytic-Uremic Syndrome/therapy , Hemolytic-Uremic Syndrome/diagnostic imaging摘要
La púrpura trombótica trombocitopénica es una entidad poco frecuente en pediatría, pero de alta mortalidad sin tratamiento adecuado y oportuno. Se caracteriza por presentar anemia hemolítica microangiopática asociada a signos y síntomas neurológicos, cardíacos, abdominales y menos frecuentemente renales; puede estar acompañada de fiebre. En niños, el diagnóstico se basa en los hallazgos clínicos y de laboratorio. La actividad de ADAMTS13 <10 % apoya, pero no confirma el diagnóstico y, dada la gravedad de la patología, el resultado no debe retrasar el inicio del tratamiento. Se presenta una paciente de 15 años, previamente sana, con signos neurológicos asociados a anemia hemolítica y trombocitopenia. Durante su internación, se arribó al diagnóstico de púrpura trombótica trombocitopénica adquirida.
Thrombotic thrombocytopenic purpura is a rare disease in pediatrics, but it has a high mortality if not managed in an adequate and timely manner. It is characterized by microangiopathic hemolytic anemia associated with neurological, cardiac, abdominal, and less frequently, renal signs and symptoms; it may be accompanied by fever. In children, diagnosis is based on clinical and laboratory findings. ADAMTS13 activity < 10% supports the diagnosis but does not confirm it and, given its severity, the result should not delay treatment initiation. Here we describe the case of a previously healthy 15-year-old female patient with neurological signs associated with hemolytic anemia and thrombocytopenia. During hospitalization, she was diagnosed with acquired thrombotic thrombocytopenic purpura.
Subject(s)
Humans , Female , Adolescent , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , Anemia, Hemolytic/diagnosis , Pediatrics摘要
Introducción. Existen limitados reportes epidemiológicos del síndrome urémico hemolítico (SUH) en Latinoamérica. Objetivo. Describir la frecuencia y características de las hospitalizaciones por SUH en niños de 0 a 14 años. Métodos. Se realizó un estudio descriptivo de análisis secundario con datos de hospitalizaciones por SUH de instituciones de salud de Perú en el periodo 2015-2022. Resultados. Se registraron 228 hospitalizaciones. El 52,2% fueron varones. El 88,6% fue menor de cinco años y el 11% menor de un año. Los departamentos con más hospitalizaciones fueron Lima y Arequipa con 133 y 46 casos. Las instituciones del Ministerio de Salud (MINSA) y del Seguro Social (EsSalud) reportaron el 42,5% y 27,6% de las hospitalizaciones. Del 2015 al 2022, la incidencia varió de 0,42 a 1,13 casos/100 000 niños menores de cinco años. Conclusiones. Las hospitalizaciones por SUH fueron más frecuentes en menores de cinco años y en las instituciones del MINSA.
Introduction. There are limited epidemiological reports of hemolytic uremic syndrome (HUS) in Latin America. Objective. To describe the frequency and characteristics of hospitalizations due to HUS in children aged 0 to 14 years. Methods. Descriptive study of secondary analysis was performed with data on hospitalizations due to HUS from healthcare institutions from Peru, 2015-2022. Results. Two hundred twenty-eight hospitalizations were registered, 52.2% were male, 88.6% were under five years old, and 11% were under one year old. The departments with more hospitalizations were Lima (133 cases) and Arequipa (46 cases). The institutions of the Ministry of Health (MINSA) and Social Security (EsSalud) reported 42.5% and 27.6% of hospitalizations. From 2015 to 2022, the incidence ranged from 0.42 to 1.13 cases/100,000 children under five years of age. Conclusions. Hospitalizations due to HUS were more frequent in children under five years of age and MINSA institutions.
摘要
ABSTRACT The covid-19 vaccine confers direct protection and reduces transmission rates of the virus and new variants. Vaccines from Pfizer/BioNTech and CoronaVac have been cleared for children in Brazil. They are safe, effective, and immunogenic. There are no known complications associated with the use of steroids or vaccines in pediatric patients with covid-19 and nephrotic syndrome. With or without immunosuppression, these patients are not at increased risk of severe covid-19, and steroids are safe for them. A milder form of covid-19 occurs in patients with chronic kidney disease without the need for hospitalization. The vaccine response may be reduced and/or the duration of antibodies after vaccination may be shorter than in the general population. However, considering risk of exposure, vaccination against covid-19 is recommended. It is believed that patients with hemolytic-uremic syndrome are at higher risk of severe covid-19. Vaccination is recommended, although specific data on the safety and efficacy of the covid-19 vaccine are limited. There is agreement that the benefits of induced immunity outweigh the risks of immunization. Vaccination against covid-19 is recommended for children and adolescents needing kidney transplantation or who have undergone transplantation. These patients present decreased immune response after vaccination, but immunization is recommended because the benefits outweigh the risks of vaccination. Current recommendations in Brazil stipulate the use of the messenger RNA vaccine. This paper aims to provide pediatric nephrologists with the latest knowledge about vaccination against covid-19 for children with kidney disease.
Resumo A vacina covid-19 confere proteção direta, reduz as taxas de transmissão do vírus e de novas variantes. No Brasil, estão liberadas para a população pediátrica as vacinas Pfizer/BioNTech e a CoronaVac, ambas seguras, eficazes e imunogênicas. Pacientes pediátricos com síndrome nefrótica e covid-19 têm curso clínico regular sem complicações relacionadas ao uso de esteroides ou vacinas. Esses pacientes, com ou sem imunossupressão, não apresentam maior risco de covid-19 grave e o tratamento com esteroides é seguro. Os pacientes com doença renal crônica têm covid-19 mais leve, sem necessidade de hospitalização. A resposta vacinal pode ser reduzida e/ou a duração dos anticorpos pós-vacinação pode ser menor do que na população geral. Entretanto, a vacina covid-19 está recomendada, considerando o risco de exposição. Acredita-se que pacientes com síndrome hemolítico-urêmica teriam maior risco de covid-19 grave. A vacina é recomendada, embora dados específicos sobre segurança e eficácia da vacina covid-19 sejam limitados. Há concordância que os benefícios da imunidade induzida superam quaisquer riscos da imunização. A vacina covid-19 é recomendada para crianças e adolescentes candidatos ao transplante renal ou já transplantados. Esses pacientes têm resposta imunológica reduzida após a vacina, entretanto ela é recomendada porque os benefícios superam qualquer risco dessa vacinação. A recomendação atual no Brasil é a vacina de tecnologia RNA mensageiro. O objetivo deste documento é levar aos nefrologistas pediátricos os conhecimentos mais recentes sobre a vacinação contra contra-19 em crianças com doenças renais.
摘要
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.