Effects of peripartum treatment on delivery outcomes in women with primary immune thrombocytopenia: a prospective cohort study / 中华围产医学杂志

Objective:To investigate the effects of peripartum administration of low-dose corticosteroids or intravenous immunoglobulin (IVIG) on delivery outcomes in pregnant patients with primary immune thrombocytopenia (ITP).Methods:This prospective cohort study involved pregnant women (≥34 gestational weeks) who were diagnosed with ITP in Peking University People's Hospital from January 2017 to December 2021. Their platelet counts were between 20×10 9/L to 50×10 9/L without bleeding and none of them had been treated with any medications. All patients were divided into medication group (prednisone or IVIG) and platelet transfusion group based on their preference. Differences in vaginal delivery rate, postpartum hemorrhage rate and platelet transfusion volume between the two groups were compared using t-test, Wilcoxon rank sum test and Chi-square test. Binary logistic regression was used to investigate the factors influencing the rates of vaginal delivery and postpartum hemorrhage. Multiple linear regression was used to analyze the factors influencing the platelet transfusion volume. Results:A total of 96 patients with ITP were recruited with 70 in the medication group and 26 in the platelet transfusion group. The vaginal delivery rate in the medication group was higher than that in the platelet transfusion group [60.0% (42/70) vs 30.8% (8/26), χ 2=6.49, P=0.013]. After adjusted by the proportion of multiparae and the gestational age at delivery, binary logistic regression showed that the increased vaginal delivery rate in patients undergoing the peripartum treatment ( OR=4.937, 95% CI: 1.511-16.136, P=0.008). The incidence of postpartum hemorrhage in the two groups was 22.9% (16/70) and 26.9% (7/26), respectively, but no significant difference was shown ( χ 2=0.17, P=0.789). The median platelet transfusion volume was lower in the medication group than in the platelet transfusion group [1 U(0-4 U) vs 1 U(1-3 U), Z=－2.18, P=0.029]. After adjustment of related factors including the platelet count at enrollment, obstetrical complications and anemia, multiple linear regression showed that the platelet transfusion volume was also lower in the medication group (95% CI:0.053-0.911, P=0.028). Ninety-six newborns were delivered without intracranial hemorrhage. The overall incidence of neonatal thrombocytopenia was 26.0% (25/96). There was no significant difference in birth weight, and incidence of neonatal asphyxia or thrombocytopenia between the two groups. Conclusion:Peripartum therapy in ITP patients may increase vaginal delivery rate and reduce platelet transfusion volume without causing more postpartum hemorrhage.

Application of high-dose intravenous immunoglobulin in the treatment of severe dermatoses / 中华皮肤科杂志

High-dose intravenous immunoglobulin (IVIG) has emerged as an effective treatment option for some refractory and severe dermatoses with few adverse reactions. The Fab and Fc fragments of IgG can exert anti-inflammatory effect by mediating specific downstream reactions via binding to a variety of autoantigens, autoantibodies and complements. This review summarizes action mechanisms of IVIG, focuses on the progress towards its application in severe dermatoses (such as severe drug eruption, refractory dermatomyositis and autoimmune bullous diseases) and special populations, as well as its adverse reactions.

Co-existence of Guillain-Barré syndrome and Behcet syndrome: A case report / 北京大学学报(医学版)

A 40-year-old male patient was referred to our department with complains of recurrent oral ulcer for more than 20 years and vulvar ulcer for more than 10 years. He presented with a 3-month history of right external ophthalmoplegia. More than 10 days ago, the patient received ganglioside infusion. And one week ago, he developed numbness and pain of his lambs, and progressive myasthenia, accompanied by right blepharoptosis and dysuria. On exam, motor strength was graded 0/5 in the lower and the upper extremities. Deep tendon reflexes were diminished in extremities. His admission medical examination: hemoglobin (HGB), white cell and platelet counts were normal. C-reactive protein (CRP) was negative. Erythrocyte sedimentation rate (ESR) 53 mm/h. Antinuclear antibody (ANA), anti-dsDNA antibody, anti-Smith antibody, anti-cardiolipin antibody and human leucocyte antigen B51 were all within normal range. The etiological tests of influenza A pathogen, influenza B pathogen, parainfluenza virus, enterovirus and parvovirus were all negative. He tested positive for serum anti-GM1 IgG. Cerebrospinal fluid had a normal white cell count, an elevated protein content. Gram staining, culture and PCR detection for varicella-zoster virus, cytomegalovirus and herpes simplex virus were all negative. Antibodies associated with autoimmune encephalitis and paraneoplastic syndrome were negative in cerebrospinal fluid. Electromyography and nerve conduction studies showed a severe axonal damage affecting motor nerves. No obvious abnormalities were observed in his magnetic resonance imaging of brain and cavernous sinus. The patient was diagnosed with Behcet syndrome complicated with acute Guillain-Barré syndrome. He received intravenous methylprednisolone, intravenous immunoglobulin (IVIg) therapy, plasma exchange and rituximab treatment. After treatment, the patient's muscle strength of limbs was restored to grade 1, blepharoptosis and pain disappeared. The nervous system involvement of Behcet syndrome is relatively rare, especially combined with Guillain-Barré syndrome, which is easy to cause misdiagnosis. The treatment of Behcet syndrome complicated with acute Guillain-Barré syndrome includes the treatment of primary disease, plasma exchange and IVIg therapy. In addition, supportive treatment is very important for such patients. The focus of treatment is to avoid respiratory insufficiency, prevent deep vein thrombosis, monitor cardiac function and hemodynamics. Pain-relieving, physical exercise and psychological support are often under-recognized. The rehabilitation treatment is very important to improve the prognosis and quality of life of patients. What we need to learn is that when the symptoms and signs of the nervous system are difficult to be explained by neuro-Behcet syndrome alone, we should be alert to the possibility of other nervous system diseases.

Transfusion-related acute lung injury associated to intravenous immunoglobulin infusion in a pediatric patient / Lesão pulmonar aguda relacionada à transfusão associada com infusão de imunoglobulina intravenosa em paciente pediátrico

ABSTRACT Case report of a patient with an immunodeficiency who demands regular replacement of intravenous immunoglobulin. She presented an episode of transfusion-related acute lung injury shortly after using an immunoglobulin product different than the one she usually received. The patient evolved with respiratory changes (hypoxia, dyspnea, change in pulmonary auscultation) minutes after the end of the infusion, and received non-invasive respiratory support. She was discharged after 36 hours with good outcome. The patient achieved full recovery, showing no further reactions in subsequent immunoglobulin infusions (no longer receiving the product that was used when she had the episode of transfusion-related acute lung injury). Although rare, this reaction is potentially serious and has no specific treatment other than supportive therapy. The literature is scarce regarding the risk of recurrence. The decision on whether to proceed with immunoglobulin therapy after this adverse effect should be analyzed individually, assessing the possible risks and benefits for the patient.

RESUMO Relato de caso de paciente com imunodeficiência que necessitava de reposição regular de imunoglobulina endovenosa. Ela apresentou um episódio de lesão pulmonar aguda relacionada à transfusão após uso de produto de imunoglobulina diferente daquele que recebia habitualmente. Evoluiu com alterações respiratórias (hipóxia, dispneia e alteração de ausculta pulmonar) minutos após o fim da infusão, necessitando de suporte respiratório não invasivo. A paciente recebeu alta hospitalar após 36 horas, com boa evolução. Obteve recuperação total dos sintomas, sem mais reações nas infusões subsequentes de imunoglobulina (sendo optado por não mais prescrever o produto que foi usado quando ocorreu o episódio de lesão pulmonar aguda relacionada à transfusão). Apesar de rara, essa reação é potencialmente grave, não possui tratamento específico além de terapia de suporte, e há pouca informação na literatura sobre o risco de recorrência. A decisão sobre o seguimento da terapia com imunoglobulina após esse efeito adverso deve ser analisada individualmente, avaliando os possíveis riscos e benefícios para o paciente.

Early recognition and treatment of intravenous immunoglobulin unresponsive patients with Kawasaki disease / 国际儿科学杂志

The cause of Kawasaki disease(KD) remains unclear.Coronary artery lesions (CAL)can occur in 20％ ～25％ children with untreated KD.The usage of high-dose intravenous immunoglobulin(IVIG) has reduced the incidence of CAL,but some patients will not respond to IVIG and have a higher incidence of CAL.Early recognition of IVIG unresponsiveness patients with KD may help physicians to adjust treatment and to improve the prognosis.Factors related to IVIG unresponsive including clinical characteristics and laboratory findings.Clinical scoring systems were also used to predict IVIG unresponsive KD patients.But there was a lack of a better scoring system or method that have been validated by clinicians.Retreatment with IVIG was a widely accepted therapy in IVIG unresponsive KD patients by now.Using effective agents such as corticosteroids and TNF-α antagonists in a timely way may play an important role to reduce the incidence of CAL in IVIG unresponsive KD patients.But there is no standard treatment protocol for IVIG unresponsive KD currently.This paper reviewed the progress in early recognition and treatment of IVIG unresponsive KD patients to provide reference for clinicians.

II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies / II Consenso Brasileiro sobre o uso de imunoglobulina humana em pacientes com imunodeficiências primárias

ABSTRACT In the last few years, new primary immunodeficiencies and genetic defects have been described. Recently, immunoglobulin products with improved compositions and for subcutaneous use have become available in Brazil. In order to guide physicians on the use of human immunoglobulin to treat primary immunodeficiencies, based on a narrative literature review and their professional experience, the members of the Primary Immunodeficiency Group of the Brazilian Society of Allergy and Immunology prepared an updated document of the 1st Brazilian Consensus, published in 2010. The document presents new knowledge about the indications and efficacy of immunoglobulin therapy in primary immunodeficiencies, relevant production-related aspects, mode of use (routes of administration, pharmacokinetics, doses and intervals), adverse events (major, prevention, treatment and reporting), patient monitoring, presentations available and how to have access to this therapeutic resource in Brazil.

RESUMO Nos últimos anos, novas imunodeficiências primárias e defeitos genéticos têm sido descritos. Recentemente, produtos de imunoglobulina, com aprimoramento em sua composição e para uso por via subcutânea, tornaram-se disponíveis em nosso meio. Com o objetivo de orientar o médico no uso da imunoglobulina humana para o tratamento das imunodeficiências primárias, os membros do Grupo de Assessoria em Imunodeficiências da Associação Brasileira de Alergia e Imunologia produziram um documento que teve por base uma revisão narrativa da literatura e sua experiência profissional, atualizando o I Consenso Brasileiro publicado em 2010. Apresentam-se novos conhecimentos sobre indicações e eficácia do tratamento com imunoglobulina nas imunodeficiências primárias, aspectos relevantes sobre a produção, forma de utilização (vias de administração, farmacocinética, doses e intervalos), efeitos adversos (principais efeitos, prevenção, tratamento e notificação), monitorização do paciente, apresentações disponíveis e forma de obtenção deste recurso terapêutico em nosso meio.

Multicenter, Single-Arm, Phase IV Study of Combined Aspirin and High-Dose “IVIG-SN” Therapy for Pediatric Patients with Kawasaki Disease

BACKGROUND AND OBJECTIVES: Intravenous immunoglobulin-SN (IVIG-SN) is a new human immunoglobulin product. Its safety is ensured by pathogen-elimination steps comprising solvent/detergent treatment and a nanofiltration process. This multicenter clinical study was designed to evaluate the efficacy and safety of combined aspirin and high-dose IVIG-SN therapy in pediatric patients with Kawasaki disease (KD). SUBJECTS AND METHODS: We evaluated coronary artery lesions (CALs) at 2 and 7 weeks after administering IVIG-SN; total fever duration; and variations in erythrocyte sedimentation rate, N-terminal pro B-type natriuretic peptide or B-type natriuretic peptide, and creatine kinase-myocardial band level before and after treatment with IVIG-SN (2 g/kg). Adverse events were monitored. RESULTS: Forty-five patients were enrolled, three of whom were excluded according to the exclusion criteria; the other 42 completed the study. The male:female ratio was 0.91:1, and the mean age was 29.11±17.23 months. The mean fever duration before IVIG-SN treatment was 6.45±1.30 days. Although most patients had complete KD (40 patients, 90.91%), four had atypical KD (9.09%). After IVIG-SN treatment, one patient (2.38%) had CALs, which was significantly lower than the incidence reported previously (15%) (p=0.022), but not significantly different from recent data (5%). There were no serious adverse events, though 28 patients (63.64%) had mild adverse events. Three adverse drug reactions occurred in 2 patients (eczema, anemia, and increased eosinophil count), all of which were transient. CONCLUSION: IVIG-SN treatment in patients with KD was safe and effective.

Clinical Manifestations and Treatment in Korean Patients with X-Linked Agammaglobulinemia

PURPOSE: X-linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the Bruton's tyrosine kinase (Btk) gene. The aim of this study was to investigate the clinical manifestations, molecular features, and treatment status of XLA in Korean patients at Seoul National University Children's Hospital. METHODS: Fourteen Korean boys with XLA showing serum agammaglobulinemia, non-detectable to less than 2% of peripheral B-cells, and mutation of the Btk gene were enrolled. We observed the clinical features, laboratory findings, status of treatment, and complications in these XLA patients. RESULTS: All XLA patients had a history of recurrent bacterial infections before diagnosis, and 20% of them had a neutropenia. Of the XLA patients 35.7% had a family history of XLA and 75% of their mothers were carriers. Btk gene analysis showed variable gene mutations in Xq22 including 9 amino acid substitutions, 3 frameshifts, 1 premature stop codon, and 1 splice defect. After intravenous immunoglobulin replacement therapy, infection episodes decreased, but complications such as bronchiectasis and chronic sinusitis remained. CONCLUSIONS: In patients less than 4 years of age with recurrent infection, analysis of serum gamma globulin levels and the Btk gene are recommended for the early diagnosis of XLA and for the appropriate prevention of recurrent infection.

Clinical Manifestations and Treatment in Korean Patients with X-Linked Agammaglobulinemia

PURPOSE: X-linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the Bruton's tyrosine kinase (Btk) gene. The aim of this study was to investigate the clinical manifestations, molecular features, and treatment status of XLA in Korean patients at Seoul National University Children's Hospital. METHODS: Fourteen Korean boys with XLA showing serum agammaglobulinemia, non-detectable to less than 2% of peripheral B-cells, and mutation of the Btk gene were enrolled. We observed the clinical features, laboratory findings, status of treatment, and complications in these XLA patients. RESULTS: All XLA patients had a history of recurrent bacterial infections before diagnosis, and 20% of them had a neutropenia. Of the XLA patients 35.7% had a family history of XLA and 75% of their mothers were carriers. Btk gene analysis showed variable gene mutations in Xq22 including 9 amino acid substitutions, 3 frameshifts, 1 premature stop codon, and 1 splice defect. After intravenous immunoglobulin replacement therapy, infection episodes decreased, but complications such as bronchiectasis and chronic sinusitis remained. CONCLUSIONS: In patients less than 4 years of age with recurrent infection, analysis of serum gamma globulin levels and the Btk gene are recommended for the early diagnosis of XLA and for the appropriate prevention of recurrent infection.

Guia prático para usar Thymoglobuline® (globulina antitimócito) em transplante renal / Use of Thymoglobulin® (antithymocyte immunoglobulin) in renal transplantation: practical guide

Resumo A combinação de imunossupressores faz parte do protocolo de tratamento de pacientes submetidos a um transplante renal (TR). A Thymoglobuline®, imunoglobulina policlonal de coelho dirigida contra timócitos humanos, é o agente mais usado como terapia de indução no TR nos Estados Unidos. No Brasil, a Thymoglobuline® está aprovada para uso em pacientes que foram submetidos a transplante e, apesar de ser amplamente utilizada, ainda existem controvérsias em relação ao seu modo de uso. Realizamos uma revisão sistemática da literatura avaliando os estudos que utilizaram a Thymoglobuline® na indução e no tratamento de rejeição em pacientes submetidos ao TR. A revisão utilizou os bancos de dados computadorizados da EMBASE, LILACS e MedLine e dos trabalhos selecionados foram extraídas informações sobre os dados gerais dos pacientes, as características metodológicas e as variáveis analisadas em cada estudo. Dos resultados obtidos, desenvolvemos um guia prático sobre o uso de Thymoglobuline® em pacientes transplantados renais.

Abstract The combination of immunosuppressive drugs is part of the treatment regimen of patients undergoing kidney transplantation (RT). Thymoglobulin®, a rabbit immunoglobulin directed against human thymocytes, is the most commonly agent used for induction therapy in RT in the US. In Brazil, Thymoglobulin® is approved by ANVISA for the use in patients who underwent kidney transplantation and despite being widely used, there are controversies regarding the drug administration. We prepared a systematic review of the literature, evaluating studies that used Thymoglobulin® for induction and for acute rejection treatment in patients undergoing RT. The review used the computadorized databases of EMBASE, LILACS and MedLine. Data were extracted from the studies concerning general features, methodological characteristics and variables analyzed in each study. From the results, a practical guide was prepared analyzing various aspects on the use of Thymoglobulin® in patients submitted to RT.

Síndrome de Stevens-Johnson: presentación de tres casos / Stevens-Johnson's syndrome: presentation of three cases

Fundamento: el Síndrome de Stevens-Johnson (SSJ) es una enfermedad grave, a menudo fatal, que ha sido considerada como un tipo de eritema multiforme, es causada generalmente por fármacos y de no ser diagnosticado y tratado de forma oportuna puede asociarse a secuelas importantes y la muerte. Objetivo: describir y reseñar el tratamiento y evolución clínica del cuadro clínico de tres casos con diagnóstico de Síndrome de Stevens-Johnson, ingresados en Cuidados Intensivos. Presentación de casos: se presentan tres enfermos, dos pertenecientes al sexo femenino y uno al masculino, con diagnóstico de SSJ asociados al empleo de fármacos. En los tres casos el tratamiento consistió en medidas de cuidados generales para la profilaxis y el tratamiento de complicaciones, esteroides sistémicos y además IgG intravenosa de producción nacional (intacglobin). Los tres pacientes evolucionaron de forma satisfactoria. Resultados: se destaca la utilidad del uso de inmunoglobulina G intravenosa en el tratamiento de estos pacientes, así como su importancia en una unidad de cuidados para enfermos graves. Conclusiones: en la casuística predominó el sexo femenino y el empleo precoz del Intacglobin (IgG IV) que contribuyó a una mejor evolución de los pacientes al detener la progresión de la enfermedad, evitar complicaciones y disminuir la estadía en las unidades de enfermos graves.

Background: Stevens-Johnson’s syndrome (SJS) is a serious disease, fatal most of the time, which has been considered as a type of erythema multiforme. It is generally caused by medicaments. If it is not diagnosed and treated at appropriate time it can be associated to considerable sequelae and death. Objective: to describe the treatment and evolution of the clinical manifestations of three cases with the diagnosis of Stevens-Johnson’s syndrome admitted in the intensive care unit. Cases presentation: the cases of two female patients and a male patient with the diagnosis of SJS, associated to the use of medicaments, are briefly presented. The treatment consisted of measures of general care for the prophylaxis and treatment of complications for the three cases. The patients were treated with intravenous IgG of national production (intacglobin) and systemic steroids. The three patients improved their condition satisfactorily. Results: the use of intravenous immunoglobulin G in the treatment of these patients stands out; as well as the importance of the treatment of seriously ill patients in an intensive care unit. Conclusions: female sex predominated in the casuistics. The early use of Intacglobin (IgG IV) contributed to a better improvement of the patient’s condition arresting the development of the disease, avoiding complications and decreasing the hospital stay of seriously ill patients.

Características epidemiológicas del síndrome de Guillain-Barré en población chilena: estudio hospitalario en un período de 7 años / Clinical features of Guillain-Barré syndrome in 41 patients admitted to a public hospital

Background: Guillain-Barré syndrome (GBS) is the commonest cause of acute flaccid paralysis worldwide, with an incidence of 0.6-4 per 100.000 inhabitants per year. It affects all age groups and carries an incapacity burden of up to 20%. Aim: To describe the features of GBS in adult Chilean patients admitted to a tertiary care hospital. Material and Methods: Review of medical records of 41 patients aged 17 to 81 years (30 males) admitted to a public hospital with the diagnosis of GBS between 2003 and 2009. According to clinical and electrophysiological criteria, the patients were classified into different varieties of GBS. Results: The incidence of GBS was higher in males (2.7:1) and the demyelinated GBS variety was found in 66% of cases. According to the Hughes’ disability score, patients treated with plasmapheresis, showed non-statistically significant better outcomes than those treated with intravenous immunoglobulin. Conclusions: In this group of patients the demyelinated variety of GBS was more common than the axonal type. Although not statistically significant, the better response to plasmapheresis is encouraging and should prompt a controlled study.

Comparison of the effect of different dose human immunoglobulin for intravenous injection in the treatment of Kawasaki disease / 中国基层医药

Objective To compare the effect of different dose human immunoglobulin for intravenous injec-tion( IVIG) in the treatment of Kawasaki disease ( KD) .Methods According to different time ,55 children with KD were divided into two groups.26 patients between January 2002 and February 2006 were selected as group A,while 29 patients between October 2006 and June 2013 were selected as group B .Patients in group A were given IVIG 0.4 g· kg-1 · d-1 ×5d,while group B was given IVIG 2 g· kg-1 · d-1 ×1d.Then,the time of fever-reducing and CAL of the two groups were compared .Results The time of fever-reducing in group A was (38 ±4) h,that in group B was (34 ±3)h,there was significant difference between the two groups (t=4.15,P0.05).Conclusion The treatment of KD with IVIG 2 g· kg-1 · d-1 ×1d is more superior than IVIG 0.4 g· kg-1 · d-1 ×5d.

Prediction of Resistance to Standard Intravenous Immunoglobulin Therapy in Kawasaki Disease

BACKGROUND AND OBJECTIVES: Ten to twenty percent of children with Kawasaki disease (KD) do not respond to initial intravenous immunoglobulin (IVIG) treatment. If untreated, approximately 15% to 25% of KD patients have complications. The aim of this study was to find useful predictors of responsiveness to initial IVIG treatment in KD. SUBJECTS AND METHODS: We retrospectively reviewed medical records of 91 children diagnosed with KD at Myong Ji Hospital from March 2012 to April 2014. Before and after (24 hours to 36 hours) IVIG treatment, the following laboratory data were obtained: hemoglobin (Hb) level, white blood cell count, proportion of neutrophil, lymphocyte and eosinophil, platelet count, erythrocyte sedimentation rate (ERS), C-reactive protein (CRP), creatine kinase (CK), creatine kinase MB (CK-MB), and N-terminal pro-brain natriuretic peptide (NT-proBNP). Subjects were then divided into two groups: IVIG-responsive or IVIG-resistant. RESULTS: Of 91 patients, 11 (12%) required retreatment. By univariate analysis, before-IVIG laboratory parameters of white blood cell count, % neutrophil, ERS, CRP, sodium, CK, CK-MB, and NT-proBNP were significantly different between IVIG-responsive and IVIG-resistant patient groups. In the after-IVIG laboratory parameters, Hb level, white blood cell count, % neutrophil, % lymphocyte, CRP, CK, CK-MB, and NT-pro-BNP were significantly different between the two groups. While the mean-differences were not statistically significant, fractional change (FC)-CRP and FC-% neutrophil showed significant difference. By multivariate analysis, FC-CRP was confirmed to be an independent predictor for initial IVIG resistance. CONCLUSION: Fractional change-C-reactive protein might be a useful and important value for predicting initial IVIG resistance in KD patients.

FcγR gene polymorphisms in Kawasaki disease susceptibility and the efficacy of intravenous immunoglobulin therapy / 国际儿科学杂志

Fcgamma receptors family conclude three groups(CD64、CD32、CD16),of which FcγR Ⅱa,Ⅱb,Ⅲb have Gene polymorphisms,for this reason they have an effect on affinity of FcγR to antibodies different,so FcγRⅡa,Ⅱb,Ⅲb are related to genetic susceptibility and IVIG treatment response.The Gene subtype of FcγR Ⅱ a is considered to be the susceptibility genes of KD.Polymorphisms of FcγRs were intimately connected to pathogenesis,coronary artery impairment and disease prevention of Kawasaki disease.

An in vitro study on influence of intravenous immunoglobulin on function of T cells in cord blood of preterm infants / 中华围产医学杂志

Objective To observe the influence of different doses of intravenous immunoglobulin (IVIG) on function of T cells in cord blood of premature infants and to explore the immunomodulating mechanism of IVIG.Methods Cord blood mononuclear cells (CBMC) were isolated by density gradient centrifugation method from 15 preterm infants born between June 1,2011 to December 31,2011 at 32 34 gestational weeks.Three groups were formed according to the concentrations of IVIG used for CMBC culturing in vitro (Group A,6 mg/ml; Group B,9 mg/ml; Group C,12 mg/ml).After 72 hours in sterile conditions,the levels of IFN 7,IL 4,IL-10 and TGF β1 in the supernatant were determined by enzyme-linked immunosorbent assay.The expression of CD4+ CD25+ Foxp3+ Treg cells was examined by fluorescent activated cell sorter.Differences among groups were compared by one way analysis of variance.For comparison between two groups,LSD test was used.Results (1) The level of IFN γ secreted by CBMC in Group C was 0.03 ± 0.02,which was much lower than that in Group A(0.18±0.08) and Group B(0.13±0.05) (F=5.72,both P＜0.01).The level of IFN-γ in Group B and Group A did not show statistical difference (P＞0.05).Compared with Group A(0.03±0.01),the level of IL4 was much lower in Group B (0.02±0.01) and Group C(0.01±0.01) (F=20.38,both P＜0.01),while no significant difference was shown between Group B and Group C (P＞0.05).(2) No significant difference was found in the expression of CD4+CD25+Foxp3+Treg cells among different groups (F 0.67,P＞0.05).(3) The level of IL 10 in Group C was 3.02 ± 3.79,which was significantly lower than that in Group A (10.78±5.44) and Group B (6.90±4.64)(F=4.68,P＜0.01 and 0.05).The level of IL-10 inGroup B was still lower than that in Group A (P＜0.05).The levels of TGF-β1 in Group C(8.44± 13.71) and Group B(16.15 ±13.94) were significantly lower than that in Group A(30.23 ± 16.32) (F=5.22,P＜0.01,P＜0.05),but there was no significant difference between Group B and Group C (P＞0.05).Conclusions IVIG might inhibit the function of Th cells in CBMC of preterm infants in a dose dependent manner.IVIG could inhibit the function of natural Treg cells by regulating the secretion of cytokines IL-10 and TGF-β1 in a dose dependent effect.

Anemia crônica no pós-transplante renal: parvovirose B19 / Posttransplant chronic anemia: parvovirus B19

A anemia é frequente em pacientes após o transplante renal (TxR) e sua prevalência varia conforme o tempo pós-transplante e os critérios diagnósticos empregados. A infecção pelo Parvovírus B19 (PV B19) é causa subdiagnosticada de anemia nesta população. Para ilustrar a epidemiologia e espectro clínico, apresentamos caso de PV B19 que evoluiu com aplasia pura de série vermelha (APSV), ressaltando as dificuldades do diagnóstico e tratamento. O emprego da detecção do DNA viral pela reação em cadeia da polimerase e do diagnóstico das alterações da morfologia da medula óssea são particularmente úteis para o diagnóstico no paciente transplantado imunossuprimido que falha na produção da resposta humoral contra o PV B19.

Anemia is frequent in kidney transplant patients, and its prevalence varies according to posttransplant time and the adopted diagnostic criteria. Parvovirus B19 (PV B19) infection is an underdiagnosed cause of anemia in this particular population. To illustrate epidemiologic and clinical data regarding it, we present a case of PV B19 infection complicated by pure red cell aplasia (PRCA), pointing out the pitfalls we encountered in diagnosis and treatment. The use of viral DNA detection by polymerase chain reaction (PCR), and correct interpretation of morphological features of bone marrow histology are particularly important for the diagnosis of this condition in kidney transplant patients, who fail to develop a proper humoral response against PV B19, thus importantly decreasing the sensitivity of serological methods in this setting.

Treatment of toxic epidermal necrolysis with intravenous immunoglobulin: a series of three cases / Tratamento da necrólise epidérmica tóxica com imunoglobulina endovenosa: uma série de três casos

Stevens-Johnson's syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatoses, that lead to keratinocyte apoptosis induced by interactions between Fas (cell death receptor) and soluble Fas-ligand, present in serum of Stevens-Johnson's syndrome / toxic epidermal necrolysis patients. Anti-Fas antibodies in intravenous immunoglobulin (IVIG) would block the apoptosis cascade. Three cases of toxic epidermal necrolysis occurred in one male and two female patients, after use of allopurinol, leprosy multidrug therapy concomitant with dipyrone, and diclofenac. The cases were treated with intravenous immunoglobulin 2-3 mg/kg and prednisone 20-50 mg/day. The interruption of new lesions outbreak and reepithelization were extremely fast after the use of intravenous immunoglobulin, without adverse effects. Controlled studies are needed to confirm the efficacy of intravenous immunoglobulin in Stevens-Johnson's syndrome / toxic epidermal necrolysis, but the results seem promising.

A Síndrome de Stevens-Johnson e a Necrólise Epidérmica Tóxica são dermatoses graves, que levam à apoptose dos queratinócitos induzida pela interação entre Fas (receptor de morte celular) e Fasligante solúvel, presente no soro de pacientes com Síndrome de Stevens-Johnson e Necrólise Epidérmica Tóxica. Anticorpos anti-Fas contidos na imunoglobulina endovenosa podem bloquear esta cascata apoptótica. Três casos de Necrólise Epidérmica Tóxica são descritos, ocorrendo após uso de alopurinol, diclofenaco e poliquimioterapia para hanseníase concomitante com dipirona. Os três casos foram tratados com imunoglobulina endovenosa 2-3 mg/kg, divididos em 4 ou 5 dias e prednisona 20-50 mg/dia. A interrupção no surgimento de novas lesões e a repitelização foram extremamente rápidas, sem ocorrência de efeitos adversos. Estudos controlados são necessários para confirmar a eficácia da imunoglobulina endovenosa na Síndrome de Stevens-Johnson e Necrólise Epidérmica Tóxica, porém, seus resultados parecem ser promissores.

Doença de Kawasaki / Atypical Kawasaki disease

A Doença de Kawasaki é vasculite sistêmica febril de etiologia desconhecida e importante causa cardiopatia adquirida na infância, principalmente no primeiro ano de vida, onde são mais comuns os casos atípicos. Os casos de Doença de Kawasaki Atípica (DKA) ou incompleta podem não preencher o número de critérios diagnósticos clássicos e apresentar sintomas pouco frequentes, atrasando o diagnóstico, o que aumenta o risco de doença coronariana. Descrevemos aqui um caso de DKA no qual sintomas oftalmológicos e neurológicos foram observados, além de um aumento importante de transaminases, alertando que os critérios clássicos podem ser restritivos, atrasar o diagnóstico e o tratamento precoce e efetivo dos casos atípicos.(AU)

Kawasaki disease is a systemic febrile vasculitis which etiology remains unknown and is an important cause of acquired heart disease during childhood, mainly in the first year of life, when atypical cases are more frequent. Atypical Kawasaki Disease (AKD) or incomplete cases may not fulfill the classic diagnostic criteria and present anusual symptoms, delaying the diagnosis and increasing the risk of coronary damage. Herein we report a case of atypical Kawasaki Disease, in which ophthalmologic and neurologic symptoms were observed besides an important rise in transaminases levels. We report warns that over reliance on classical criteria may be restrictive, may delay the diagnosis and prevent the effective and early treatment in atypical cases.(AU)