Síndrome Stevens Jonhson: reporte de caso clínico / Stevens Johnson syndrome: clinical case report

Resumen Introducción: El síndrome Stevens Johnson (SSJ) es una dermatosis potencialmente fatal caracterizada por una extensa necrosis epidérmica y de mucosas que se acompaña de ataque al estado general, y junto con la necrólisis epidérmica tóxica (NET) se consideran reacciones de hipersensibilidad tipo IV, relacionadas con ciertos fármacos en 60% de los casos, siendo uno de los diagnósticos pocos frecuentes, pero con una alta mortalidad hasta del 40%. Caso clínico: El siguiente caso clínico es un masculino de 34 años de edad que inició un cuadro de eritema generalizado inmediatamente tras la administración del medicamento trimetoprima/sulfametoxazol. Se le solicitó un hemograma mostrando leucocitosis, neutrofilia, VSG elevada, PCR elevada, IgE elevada, y tras el interrogatorio clínico se realiza el algoritmo ALDEN dando positivo con 10 puntos asociado al medicamento previamente dicho. Por lo tanto se le inicia tratamiento con metilprednisolona, difenhidramina, inmunoglobulina humana intravenosa y un plan terapéutico cutáneo, dando como resultado una mejoría clínica, evitando complicaciones y secuelas, hasta el día de su egreso. A manera de conclusión, se requiere un manejo multidisciplinario para atender las manifestaciones clínicas del inmunoglobulina humana intravenosa.

Abstract Introduction: Stevens Johnson Syndrome (SJS) is a potentially fatal dermatosis characterized by extensive epidermal and mucosal necrosis accompanied by an attack on the general condition, which together with Toxic Epidermal Necrolysis (TEN) are considered type IV hypersensitivity reactions, related to certain drugs in 60% of cases, being one of the rare diagnoses, but with a high mortality of up to 40%. Case report: The following clinical case is a 34 year old male who started a generalized erythema picture immediately after administration of the medication trimethoprim/sulfamethoxazole, for which a complete blood count was requested showing leukocytosis, neutrophilia, elevated ESR, elevated PCR, elevated IgE, and after the clinical questioning, the ALDEN algorithm was performed, giving positive with 10 points associated with the previously mentioned medication, for which treatment was started with methylprednisolone, diphenhydramine, intravenous human immunoglobulin and a skin therapeutic plan, resulting in clinical improvement, avoiding complications and sequelae, until the day of discharge. In conclusion, a multidisciplinary management is required to attend to the clinical manifestations of the patient, helping him to a quick and effective recovery.

Development of the method for determinating ADCC biological activity of IVIG / 中国输血杂志

【Objective】 To establish a method for determinating the antigen-dependent cell-mediated cytotoxicity (ADCC) of human immunoglobulin (pH4)for intravenous injection (IVIG) on luciferase reporter gene-modified cell assay. 【Methods】 As effector cells, Jurkat-NFAT-Luc-CD16 cells were used in the assay, and PLC/PRF/5 cells were used as target cells. After incubation of effector cells and target cells with IVIG, the method for determinating ADCC biological activity of IVIG was established by detecting luciferase released by activated T nuclear factor after binding of IVIG Fc fragment to effector cells. Meanwhile, the experimental assay conditions were optimized, and the methodology was verified subsequently. 【Results】 IVIG had a dose-response relationship in this method, which was consistent with four parameter logistic model. And the PLC/PRF/5 cells were finally determined as the target cells. The initial dilution concentration of antibody was 20 mg/mL, and the ratio dilution was 1∶2, and the effector to target ratio was 1∶3, and co-incubation time of two cells and IVIG was 24 hours. Within-run and between-run analysis including three independent tests, initial working concentration relative light unit (RLU) and the relative standard deviation (RSD) of the concentration for 50% of maximal effect(EC50) were less than 11%. The relative titers of the recovery samples of the two different dilution groups were (23.50±1.69)% and (49.30±2.97)%, respectively, and the corresponding recovery rates were (93.50±6.30)% and (96.24±5.43)%, respectively, with RSD less than 11%. 【Conclusion】 The method for determinating ADCC biological activity of IVIG based on luciferase reporter gene-modified cell assay was successfully established. It could be applied in determinating the ADCC biological activity of IVIG, and has the advantages of satisfactory linearity, accuracy, precision and specificity.

Stability of human immunoglobulin(pH 4) for intravenous injection after process optimization / 中国生物制品学杂志

@#Objective To evaluate the stability of human immunoglobulin(pH 4)for intravenous injection(IGIV)after process optimization.Methods A filter plate and B filter membrane were used to filter the protein components in different separation stages to reduce the residue of immunoglobulin A(IgA)in the product in multi-batch large-scale production. The finished product was examined for the physical properties(appearance,visible foreign body,insoluble particle examination and thermal stability test)and the chemical properties[protein content,purity,molecular size distribution,titers of antiHBs,diphtheria antibody,prokallikrein activator(PKA),anti-complement activity(ACA),anti-A and anti-B hemagglutinin,and IgA residue]. The accelerated and long-term stability tests were performed.Results There was no significant difference in the key quality indicators between IGIV batches produced by the optimized process and the normal process,while the IgA residue decreased significantly(t = 3. 992 and 11. 215 respectively,each P < 0. 05). In the accelerated stability and long-term stability tests,all the test results of IGIV after process optimization were qualified,which met the relevant regulations in the third part of Chinese Pharmacopoeia(2020 edition).Conclusion IGIV after process optimization can effectively reduce IgA residue with good stability,which is of great significance for blood product manufacturers to improve the quality of IGIV products.

Systematic evaluation of the efficacy and safety of intravenous human immunoglobulin combined with cyclophosphamide in the treatment of systemic lupus erythematosus / 中国药房

OBJECTIVE To systematically evaluate the safety and effectiveness of intravenous human immunoglobulin （IVIG） combined with cyclophosphamide in the treatment of systemic lupus erythematosus （SLE）， and to provide an evidence-based basis for clinical medication. METHODS Retrieved from PubMed， Embase， Cochrane Library， CBM， CNKI and Wanfang database， randomized controlled trials （RCTs） about IVIG combined with cyclophosphamide versus cyclophosphamide alone based on glucocorticoids were collected. The quality of the included literature was evaluated with Cochrane 5.1.0 risk of bias assessment tool after literature screening and data extraction， and meta-analysis was performed by using RevMan 5.4 software. RESULTS A total of 13 RCTs were included， involving 842 patients. Meta-analysis showed that compared with cyclophosphamide alone， IVIG combined with cyclophosphamide improved the overall response rate of systemic lupus erythematosus [RR＝1.23， 95%CI（1.15， 1.32）， P＜0.000 01]， lowered the systemic lupus erythematosus disease activity index [MD＝－2.05， 95%CI（－2.51， －1.60）， P＜0.000 01]， relieved 24 h proteinuria [MD＝－1.29， 95%CI（－1.57， －1.01）， P＜0.000 01]， reduced the inflammatory factor MCP-4 [MD＝－28.04， 95%CI（－32.72， －23.37， P＜0.000 01）]， IL-4 [MD＝－1.66， 95%CI（－1.96， －1.36）， P＜0.000 01]， and boosted immune complement C3 [SMD＝0.74，95%CI（0.34，1.14）， P＝0.000 3] and complement C4 [SMD＝0.99，95%CI （0.31，1.67）， P＝0.004]； it had similar incidence of adverse drug reactions to cyclophosphamide therapy alone [RR＝0.81， 95%CI （0.57， 1.17）， P＝0.26]. CONCLUSIONS Compared with cyclophosphamide alone， IVIG combined with cyclophosphamide has a positive role in improving the overall response rate of treating SLE， improving clinical symptoms， reducing inflammatory factors， improving immune function， but the results should be interpreted with caution.

One-year intravenous immunoglobulin replacement therapy: efficacy in reducing hospital admissions in pediatric patients with Inborn Errors of Immunity

Abstract Objectives: To compare the frequency of hospitalization in children with Inborn Errors of Immunity with antibody deficiency previous to intravenous immunoglobulin (pre- IVIG) with a one-year period after initial IVIG (post-IVIG). Methods: Medical reports of 45 patients during an eight-year period were reviewed from 2018 to 2019. Wilcoxon-test was used for related samples. Results: Forty-five children were included in the study, aged 29-249 months of age, and most of them (64.4%) were males. Median ages at onset symptoms and at diagnosis were 6 and 73 months old, respectively. Specific antibody deficiency and unclassified hypogammaglobulinemia were the predominant diagnoses (31.1% and 17.8%, respectively). X-linked agammaglobulinemia, Hyper IgE syndrome, Hyper IgM, transient hypogammaglobulinemia of infancy, and Common Variable Immunodeficiency (CVID) were also reported, in a low frequency. Forty-four (97.8%) patients were hospitalized before IVIG, and 10 patients (22.2%) after. Annual mean hospital admission reduced from 2.5 to 0.5, pre and post-IVIG, respectively (p < 0.0001). Mean length of stay (LOS) reduced from 71 to 4.7 days/year (p < 0.0001) in general ward and in the PICU from 17.2 days/year to zero (p < 0.0002). Pneumonia was the main cause of hospital admission with a reduction in the number of episodes per patient from an average of 2.2-0.1 per year (p < 0.001). Concomitant use of antibiotic prophylaxis did not influence the number of hospital admission. Conclusion: One-year intravenous IVIG significantly decreased the number of hospitalizations and length of stay in children with impaired antibody production. Social and economic impacts would be required.

Effect of IgG fragments in IVIG(pH4)on phagocytosis of sensitized erythrocytes by macrophage / 中国输血杂志

【Objective】 To research the effect of the Fc, Fab and F(ab′)2 fragments of immunoglobulin G, the main components of Human Immunoglobulin(pH4) for Intravenous Injection(IVIG), on the phagocytic function of macrophages derived from THP-1 cells. 【Methods】 First of all, IVIG was digested with papain and pepsin to obtain Fc, Fab and F(ab′)2, and these components were then identified by sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE). Afterwards, propylene glycol monomethyl ether acetate (PMA) was used to induce THP-1 cells to differentiate into M0 macrophages. Finally, the sensitized erythrocytes were labeled with carboxy fluorescein succinimidyl ester (CFSE), and the effect of the above components on the phagocytic ability of M0 macrophages to engulf sensitized erythrocytes was detected by flow cytometry. 【Results】 The identification results of SDS-PAGE showed that the prepared IgG fragments met the requirements of subsequent experiments. Flow cytometry performs showed that the phagocytosis model of M0 macrophages had been successfully established. When the concentration of Fc increased from 0.1μg/ mL to 10μg/ mL, the phagocytosis rate of erythrocytes sensitized by M0 macrophages decreased from (24.21±0.58) % to (12.27±0.19) %. When the concentration of IVIG protein increased from 0.1 μg/ml to 10 μg/ml, the phagocytosis rate decreased from (20.57±0.39) % to (0.20±0.03) %. Meanwhile, at the same protein concentration (10 μg/ml), the inhibitory effect of Fc on phagocytosis was only half that of IVIG. In addition, Fab, F(ab′)2, and human serum albumin could not inhibit phagocytosis of M0 macrophages. 【Conclusion】 IVIG can effectively inhibit the phagocytosis of THP-1 derived M0 macrophages, which is mainly dependent on the Fc, but not related to the Fab of IgG and F (ab′)2.

Errores innatos de la inmunidad en adultos / Inborn errors of immunity in adults

Introducción: La inmunodeficiencia común variable es un error innato de la inmunidad que tiene su pico de incidencia en la edad adulta. Se caracteriza por una susceptibilidad aumentada a padecer infecciones respiratorias, autoinmunidad y malignidad, secundario a un estado de hipogammaglobulinemia e inmunodisregulación, causado por mutaciones e interacciones genéticas parcialmente comprendidas. El diagnóstico es de exclusión, tiene una gran heterogeneidad clínica y comúnmente es diagnosticado de forma errónea. Objetivo: Describir un caso clínico de un paciente afectado por un error innato de la inmunidad. Caso clínico: Hombre de 35 años que se presenta a la consulta de Medicina Interna - Inmunología refiriendo un cuadro clínico de 3 años de evolución consistente en múltiples episodios de infecciones sino-pulmonares en los últimos meses, presentaba tos productiva, dificultad respiratoria y pérdida de peso no intencional de aproximadamente 8 kg. Conclusiones: La inmunodeficiencia común variable debe considerarse dentro de los diagnósticos diferenciales en todo paciente que presente alguna de sus manifestaciones clínicas, principalmente aquellas relacionadas con infecciones respiratorias a repetición, antecedente que el paciente puede presentar como relevante en sus consultas de primer nivel con medicina general o con especialistas. Su aproximación diagnóstica consiste en la solicitud de niveles séricos de inmunoglobulinas, prueba de laboratorio de fácil acceso para cualquier clínico independiente de su nivel de atención y su tratamiento se fundamenta en la administración periódica de inmunoglobulina humana exógena de forma endovenosa o subcutánea(AU)

Introduction: Common variable immunodeficiency is an inborn error of immunity that has its peak incidence in adulthood. It is characterized by an increased susceptibility to respiratory infections, autoimmunity and malignancy, secondary to a state of hypogammaglobulinemia and immunodysregulation, caused by mutations and partially understood genetic interactions. The diagnosis is one of exclusion, has great clinical heterogeneity and is commonly misinterpreted. Objective: To describe a clinical case of a patient affected by an inborn error of immunity. Methods: Retrospective description of a case report. Conclusions: Common variable immunodeficiency disorder should be considered within the differential diagnoses in every patient who presents any of its clinical manifestations, mainly those related to recurrent respiratory infections, an antecedent that the patient may present as relevant during the first-level consultations with general medicine physicians or with specialists. Its diagnostic approach consists in measuring serum immunoglobulin levels, an easily accessible laboratory test for any clinic physician regardless of their healthcare level, while its treatment is based on the periodic administration of exogenous human immunoglobulin intravenously or subcutaneously(AU)

Study of the use of IVIG Normal Human Immunoglobulin in the La Samaritana University Hospital during the period of 2017-2019 / Estudio de utilización de Inmunoglobulina Humana Normal IGIV en el Hospital Universitario de la Samaritana durante el periodo de 2017-2019 / Estudo de utilização de Imunoglobulina Humana Intravenosa Inespecífica (IGIV) no Hospital Universitário da Samaritana (HUS) durante o período 2017-2019

SUMMARY Introduction: Non-specific intravenous human immunoglobulin (IVIG) is a valuable therapeutic resource in specific pathologies, however, there are pathological conditions for which there is not yet a defined therapeutic strategy and due to similarity or biological plausibility, some prescribers end up using it. Aim: To identify the indications for which IVIG has been prescribed in the HUS. Methodology: A descriptive observational cross-sectional study was performed with retrospective collection of information between January 1, 2017, and December 31, 2019. Materials and methods: 180 medical records were reviewed, demographic characterization of the study, clinical and pharmacological population of patients who have been prescribed at least one dose of immunoglobulin. In addition, the indications proposed in the clinical history were compared with the indications approved by regulatory authority (Invima). Results: It was determined that the population with the highest predominance to which IVIG was prescribed in the HUS, were women in young adulthood. Likewise, the use of IVIG presented a higher frequency in patients with idiopathic purple thrombocytopenia (ITP), Guillain-Barré Syndrome (GBS), Systemic Lupus Erythematosus (SLE) and neonatal jaundice due to other existing hemolysis specified as main diagnoses. The indications for which there is an Invima approval are nonspecific, which means that there is no defined therapeutic strategy with IVIG and therefore that prescribers for similarity or biological plausibility turn out to be using it, for uses not indicated in the sanitary registry (UNIRS), which represented 71 % of indications for which IVIG was prescribed in HUS. Conclusion: The indications for the most prescription with IVIG were idiopathic purple thrombocytopenia (ITP) and Guillain-Barré syndrome (GBS). The non-prescribed use of IVIG in this study reached a figure of 71%.

RESUMEN Introducción: La inmunoglobulina humana intravenosa inespecífica (IGIV) es un valioso recurso terapéutico en patologías específicas, sin embargo, existen condiciones patológicas para las que aún no existe una estrategia terapéutica definida y por similitud o plausibilidad biológica algunos prescriptores terminan usándola. Objetivo: Identificar las indicaciones para las cuales se ha prescrito inmunoglobulina humana inespecífica intravenosa (IGIV) en el HUS. Metodología: Se realizó un estudio observacional descriptivo de corte transversal con recolección retrospectiva de la información entre enero 1 de 2017 hasta diciembre 31 de 2019. Materiales y métodos: Se revisaron 180 historias clínicas, de pacientes a quienes se les prescribió al menos una dosis de inmunoglobulina, se realizó una caracterización demográfica, clínica y farmacológica de la población estudio. Además, se comparó las indicaciones descritas en la historia clínica con las indicaciones aprobadas por el Invima. Resultados: Se identificó a mujeres adultas jóvenes como población a quienes se le prescribió IGIV con mayor frecuencia. Asimismo, las indicaciones para la que se prescribe IGIV con mayor frecuencia fueron la purpura trombocitopenia idiopática (PTI), síndrome de Guillain-Barré (SGB), lupus eritematoso sistémico (LES) e ictericia neonatal debida a otras hemolisis existentes. Las indicaciones aprobadas por la agencia regulatoria colombiana (Invima) son inespecíficas por lo que el uso de la IGIV es prescrito ya sea por similitud o plausibilidad biológica, lo cual puede explicar el uso no prescrito que en este estudio alcanzó la cifra del 71%. Conclusión: Las indicaciones de mayor prescripción con IGIV fue purpura trombocitopenia idiopática (PTI) y síndrome de Guillain-Barré (SGB). El uso no prescrito de IGIV en este estudio alcanzó la cifra del 71%.

RESUMO Introdução: A imunoglobulina humana intravenosa inespecífica (IGIV) é um recurso terapêutico valioso em patologias específicas, porém existem condições patológicas para as quais ainda não há estratégia terapêutica definida e devido à semelhança ou plausibilidade biológica alguns prescritores acabam por indica-la. Objetivo: Identificar as indicações para as quais a imunoglobulina humana intravenosa inespecífica (IGIV) foi prescrita no HUS. Metodologia: Foi realizado um estudo observacional transversal descritivo com coleta retrospectiva de informações entre 1° de janeiro de 2017 e 31 de dezembro de 2019. Materiais e métodos: foram revisados 180 prontuários de pacientes que receberam prescrição de pelo menos uma dose de imunoglobulina, foi realizada uma caracterização demográfica, clínica e farmacológica da população em estudo. Além disso, as indicações descritas no prontuário foram comparadas com as indicações aprovadas pelo Invima. Resultados: Mulheres adultas jovens foram identificadas como a população que recebeu IGIV com maior frequência. Da mesma forma, as indicações para as quais a IGIV foi mais frequentemente prescrita foram trombocitopenia roxa idiopática (PTI), síndrome de Guillain-Barré (SGB), lúpus eritematoso sistémico (LES) e icterícia neonatal devido a outras hemólises existentes. As indicações aprovadas pela agência reguladora colombiana (Invima) são inespecíficas, portanto o uso de IGIV é prescrito seja por semelhança ou plausibilidade biológica, o que pode explicar o uso off" label, que neste estudo atingiu a cifra de 71%. Conclusão: As indicações para a maior prescrição de IGIV foram a trombocitopenia roxa idiopática (PTI) e a síndrome de Guillain-Barré (SGB). O uso off" label de IGIV neste estudo atingiu a cifra de 71%.

Treatment of COVID-19 with COVID-19-human immunoglobulin: A case report / 中国胸心血管外科临床杂志

@#At present, there has been no report in China that novel coronavirus specific immune globulin has been used to treat coronavirus disease 2019 (COVID-19). Recently, we had successfully treated one COVID-19 patient with intravenous injection of human immunoglobulin (COVID-19-IVIG). The female patient, aged 57 years, had clinical diagnosis: (1) COVID-19, common type; (2) postoperative colon cancer; (3) leukopenia; (4) low cellular immunity. 75 mL COVID-19 human immunoglobulin (Sinoptic Wuhan Blood Products Co., Ltd.) was intravenously injected twice. The patient was hospitalized for 49 days and had a good prognosis.

Effect of human immunoglobulin manufacturing process on polymer content / 中国输血杂志

【Objective】 To investigate the changing pattern of polymer content in the manufacturing process of human immunoglobulin, especially the correlation between pH value and polymer content, so as to provide basis for the control of polymer in human immunoglobulin products. 【Methods】 IVIG and HBIG low pH intermediate samples were taken and their pH values were adjusted to different gradients with 0.5 or 0.1 mol/L HCl. The polymer content was detected by HPLC, and the influence of different pH values on polymer content was observed and analyzed. 【Results】 With low pH value, the polymer content of samples increased from <1% to 10% with the decrease of pH value. When pH value was in the range of 3.85~3.10, the polymer content in the samples increased from 0.38% to 15.5% exponentially with the decrease of pH value. The logarithm of polymer was linearly correlated with pH value. 【Conclusion】 When pH value is low, polymer content of human immunoglobulin is significantly affected by pH value. Keeping pH value above 4.0 during the whole manufacturing process of human immunoglobulin is suggested.

Síndrome de choque tóxico por Streptococcus pyogenes en un hospital de la Ciudad de México / Toxic shock syndrome caused by Streptococcus in a hospital from the Mexico City / Síndrome de choque tóxico por Streptococcus pyogenes em um hospital da Cidade do México

Resumen: El síndrome de choque tóxico por Streptococcus se describió por primera vez en 1978 por Todd y su equipo. Éste incluye datos de hipotensión, disoxia celular, aislamiento de Streptococcus pyogenes, así como la inclusión de dos o más de los siguientes criterios: síndrome de dificultad respiratoria aguda, insuficiencia renal, anormalidad hepática, coagulopatía y erupción cutánea con descamación. Es una enfermedad relativamente rara entre niños y adultos; representa una alta mortalidad, la cual oscila de 23 al 70%. En más de la mitad de los casos, se desconoce la causa de entrada de este patógeno al torrente sanguíneo, aunque los pacientes con alcoholismo, en los extremos de la vida, con comorbilidades (como enfermedad renal crónica, insuficiencia hepática y leucemias) y que hayan sido sometidos a esplenectomía son más susceptibles al contagio y a infecciones severas. En los pacientes que desarrollan infecciones de tejidos blandos profundos, como fascitis necrosante o infección intrauterina, el dolor severo es el síntoma inicial más frecuente.

Abstract: Streptococcal toxic shock syndrome was first described by Todd and collaborators in 1978, this syndrome includes hypotension, traces of cellular dysoxia, isolation Streptococcus pyogenes; as well as two or more of the following criteria: acute respiratory distress syndrome, renal failure, liver abnormality, coagulopathy, skin rash with necrosis flaking of soft tissues. It is a relatively rare disease among children and adults, representing a high mortality ranging from 23 to 70%. In more than half of the cases the cause of entry of this pathogen into the bloodstream is unknown, although patients with alcoholism, on the edge of death, with splenectomy, with comorbidities (chronic kidney disease, liver failure, leukemia) are more susceptible to infection, to acquire severe infections and shock. Cutaneous infections usually are followed by minor skin irritations (insect bites), 20% of the cases experience a flu-like syndrome (fever, chills, myalgia and diarrhea). In patients who develop deep soft tissue infections such as necrotizing fasciitis or intrauterine infection, the severe pain is the most common initial symptom of streptococcal TSS.

Resumo: A síndrome do choque tóxico estreptocócico foi descrita pela primeira vez por Todd et al em 1978, incluindo dados sobre hipotensão, disxia celular, isolamento de Streptococcus pyogenes, bem como 2 ou mais dos seguintes critérios: síndrome do desconforto respiratório agudo, insuficiência renal, anormalidade hepática, coagulopatia, erupção cutânea com descamação. É uma doença relativamente rara em crianças e adultos, representa uma alta mortalidade que varia de 23 a 70%. Em mais da metade dos casos, a causa desse patógeno na corrente sanguínea é desconhecida, embora pacientes com alcoolismo, extremos da vida, com esplenectomia e comorbidades (como doença renal crônica, insuficiência hepática, leucemia) sejam mais suscetíveis à infecção e infecções graves. Em pacientes que desenvolvem infecções profundas dos tecidos moles, como fasceíte necrozante ou infecção intra-uterina, a dor intensa é o sintoma inicial mais frequente.

Tratamento de pênfigo vulgar com imunoglobulina humana como adjuvante ao corticoide oral: um relato de caso / Pemphigus vulgaris treatment with human immunoglobulin as adjuvant to oral corticoid: a case report

Introdução: o pênfigo vulgar é uma doença de caráter autoimune, que leva à formação de bolhas locais ou generalizadas causadas pelo ataque de autoanticorpos às estruturas da epiderme. A terapêutica Recebido em: 25/09/2018 com imunoglobulina humana como adjuvante é uma excelente opção para os casos em que há resistência ao tratamento habitual, além de poder diminuir o tempo de tratamento com imunossupressores. Objetivo: descrever um relato de caso sobre um paciente portador de pênfigo vulgar submetido ao tratamento com imunoglobulina humana como adjuvante ao corticoide oral. Relato do caso: paciente 49 anos, foi internado apresentando lesões erosivas em mucosa oral e conjuntival, com piora sistêmica após início de tratamento com antibióticos e anti-inflamatório. Inicialmente realizou-se o tratamento convencional com corticoterapia, porém sem resultados satisfatórios. Devido ao agravamento da clínica, e ao estabelecimento do diagnóstico de pênfigo vulgar, foi incluída no tratamento a imunoglobulina humana como adjuvante, o que culminou em uma melhora progressiva do paciente. Conclusão: apesar de o tratamento do pênfigo vulgar ter os corticoides como primeira opção, é importante conhecer tratamentos adjuvantes e/ou alternativos, como as imunoglobulinas humanas, para auxiliar no tratamento dos pacientes que não respondem ao corticoide.

Introduction: Pemphigus Vulgaris is an autoimmune disease that leads to the formation of local or generalized blisters as a result of autoantibodies against epidermal structures. Therapy with human immunoglobulin as an adjuvant is an excellent option for cases where there is resistance to usual treatment, in addition to being able to reduce the time of treatment with immunosuppressant. Thus, it is an alternative treatment for patients with severe infections or immunological deficiencies. Aim: to describe a case report about a carrier patient of pemphigus vulgaris treated with human immunoglobulin as adjuvant to oral corticosteroids. Case report: patient with 49 years old, was hospitalized in the city of Belo Horizonte (MG) presenting erosive lesions in the oral and conjunctival mucosa, with systemic worsening after starting treatment with antibiotics and anti-inflammatory. Initially, conventional steroid therapy was performed, but with no satisfactory results. Due to worsening symptoms and the diagnosis of Pemphigus Vulgaris, human immunoglobulin was included in the treatment as an adjuvant, which resulted in a progressive improvement of the patient. Conclusion: although treatment of Pemphigus Vulgaris has steroids as the first option, it is vital that the physician knows adjuvant and/or alternative therapies, such as human immunoglobulins, to assist in the treatment of patients who do not respond to steroids.

Effect of Human Immunoglobulin for Intravenous Injection in the treatment and intervention of neonatal infectious pneumonia / 中国生化药物杂志

Objective To discuss the effect of Human Immunoglobulin for Intravenous Injection in the treatment of neonatal pneumonia treatment and intervention. Methods 100 cases of newborns in our hospital from July 2015 to 2016 to receive the treatment of infectious pneumonia patients, and were randomly divided into control group and observation group, 50 cases in each group. The control group used routine treatment, the observation group on the basis of intravenous immunoglobulin and take corresponding nursing measures. The treatment were compared between the two groups of the total efficiency of disappearance of hospitalization time and pulmonary rales and difficulty breathing time. Results In the control group than the treatment in the observation group the total efficiency significantly comparison between the two groups of children have statistical significance(P<0.05); disappeared in the observation group hospitalization time and pulmonary rales and dyspnea was significantly better on time According to the group, there were significant differences between the two groups have statistical significance(P<0.05). Conclusion The use of Human Immunoglobulin for Intravenous Injection on the treatment of neonatal pneumonia, can effectively promote the treatment efficiency, and is helpful to the patient's recovery, worthy of promotion in clinical use.

Clinical Observation of Prednisone Combined with Human Immunoglobulin(pH4)for Intravenous Injection in the Treatment of Idiopathic Thrombocytopenic Purpura / 中国药房

OBJECTIVE:To observe the efficacy and safety of prednisone combined with human immunoglobulin(pH4)for intra-venous injection in the treatment of idiopathic thrombocytopenic purpura. METHODS:85 patients with idiopathic thrombocytopenic purpura were divided into control group(42 cases)and observation group(43 cases). Control group received 1.6 mg/(kg·d)Predni-sone tablet,orally,for continuous 4 weeks;observation group received 400 mg/(kg·d)human immunoglobulin(pH4)for intravenous injection,intravenous injection,for continuous 5 d,then 1.6 mg/(kg·d)Prednisone tablet,orally,for continuous 4 weeks. All pa-tients were given Adrenal color hydrazone tablet,Vitamin C tablet and other conventional treatment. Clinical efficacy,platelet number, T lymphocyte subsets(CD3+,CD3+CD4+,CD3+CD8+,CD19+),TNF-α,IL-6 before and after treatment,time of platelet number reached normal and reached peak value,peak value of platelet number and the incidence of adverse reactions in 2 groups were ob-served. RESULTS:The total effective rate and peak value of platelet number in observation group were significantly higher than control group,time of platelet number reached normal and reached peak value were significantly shorter than control group,the differences were statistically significant(P0.05);after treatment,platelet number,CD3+and CD3+CD4+in 2 groups were significant-ly higher than before,and observation group was higher than control group,IL-6,TNF-αlevel,CD3+CD8+and CD19+were signifi-cantly lower than before,and observation group was lower than control group,the differences were statistically significant(P0.05). CONCLUSIONS:Prednisone com-bined with human immunoglobulin(pH4) for intravenous injection shows better efficacy than prednisone alone in the treatment of idio-pathic thrombocytopenic purpura,it can increase platelet number,adjust immune function,and do not increase the incidence of ad-verse reactions.

Specific human immunoglobulin in prevention and treatment of infectious diseases / 军事医学

The specific human immunoglobulin is a hyperimmune globulin against a particular pathogen or biotoxin .It′s an important variety in plasma derivatives .Specific human immunoglobulin is usually used to prevent and treat pathogen in -fections with high morbidity , severe outcomes and no efficient treatment available .Thus it has unique advantages in preven-tion and management of infectious diseases .A variety of specific human immunoglobulins have been licensed abroad , but the development of new specific human immunoglobulins is slow in China due to technical constraints , limited economic benefits or for other reasons .Here we reviewed some specific human immunoglobulins and their preventive and therapeutic effect on infectious diseases .

Síndrome de Guillain-Barré en la infancia / Guillain-Barré syndrome in the childhood

Se realizó un estudio descriptivo y prospectivo de 25 pacientes con síndrome de Guillain-Barré, atendidos en el Hospital Infantil Docente Sur de Santiago de Cuba, desde enero de 2006 hasta diciembre de 2012, con vistas a identificar sus características clinicoepidemiológicas y determinar la utilidad del tratamiento empleado. Predominaron los niños menores de 5 años (40,0 %), el sexo masculino y la infección respiratoria alta previa (ambas con 60,0 %), así como la debilidad muscular y arreflexia osteotendinosa (76,0 % en cada caso). La polirradiculoneuropatía desmielinizante inflamatoria aguda fue la variante clínica más frecuente (72,0 %). Tanto el tratamiento con inmunoglobulina humana intravenosa como la rehabilitación resultaron beneficiosos y la mortalidad baja (4,0 %).

A descriptive and prospective study of 25 patients with Guillain-Barré syndrome, assisted in the Teaching Southern Children Hospital in Santiago de Cuba was carried out from January, 2006 to December, 2012, with the aim of identifying their clinical and epidemiological characteristics and to determine the usefulness of the treatment applied. Children younger than 5 years (40.0%), male sex and previous upper respiratory infection (both with 60.0%), as well as the muscular weakness and osteotendinous areflexia (76.0% in each case) prevailed. The inflammatory and acute desmyelinating polyrradiculoneuropathy was the most frequent clinical variant (72.0%). Either the treatment with intravenous human immunoglobulin as rehabilitation were beneficial and the mortality was low (4.0%).

Entecavir combined with immunoglobulin prevents hepatitis B recurrence after liver transplantation / 中国组织工程研究

BACKGROUND:Prolonged therapy with lamivudine has been associated with tyrosine-methionine-aspartate-aspartate mutation, which results in hepatitis B recurrence. Recently, antiviral agents, such as entecavir, have high efficacy and low resistance rate in hepatitis B-related liver disease. However, the researches on the effect of entecavir in preventing hepatitis B recurrence after liver transplantation are rare. OBJECTIVE:To investigate the effect of entecavir combined with low-dose hepatitis B immunoglobulin in preventing hepatitis B recurrence after liver transplantation. METHODS:The fol ow-up data of 253 patients who had liver transplantation for hepatitis B virus related liver disease were retrospectively analyzed. Al patients received nucleoside analogues therapy formal y before liver transplantation. The effects of entecavir+hepatitis B immunoglobulin and lamivudine+hepatitis B immunoglobulin were compared in al the patients and the patents with hepatitis B recurrence risk factors (positive preoperative HBeAg, DNA-positive hepatitis B virus, hepatoma and tyrosine-methionine-aspartate-aspartate mutation). RESULTS AND CONCLUSION:A total of 253 patients received hepatitis B virus-related liver transplantation, and 29 patients died. There were 202 patients in lamivudine group in which 26 patients were dead and 16 patients had hepatitis B virus recurrence, and the recurrence rate was 7.92%(16/202). However, entecavir group had 51 patients without hepatitis B virus recurrence in which three patients were dead. There were significant differences in the mortality rate and recurrence rate between two groups. Compared with the lamivudine+hepatitis B immunoglobulin, entecavir+hepatitis B immunoglobulin could effectively reduce the recurrence rate of the patients with hepatitis B virus-related risk factors. Hepatitis B immunoglobulin was terminated and nucleoside analogues were modulated when recurrence appeared. Al patients hepatitis B virus DNA were control ed less than 500 IU/mL and liver function returned to normal level. Log-rank test showed that there was no significant difference in the long-term survival rate after timely treatment of hepatitis B virus recurrence. With the prevention of nucleoside analogues combined with hepatitis B immunoglobulin therapy, timely treatment of hepatitis B recurrence has little influence on the prognosis. Entecavir combined with hepatitis B immunoglobulin can effectively prevent the hepatitis B recurrence. For the patients with hepatitis B virus-related risk factors, entecavir combined with hepatitis B immunoglobulin can better reduce the recurrence rate of hepatitis B than lamivudine+hepatitis B immunoglobulin after liver transplantation.

Quality investigation of insoluble particles in freeze-dried and liquid human immunoglobulin for intravenous injection / 中国药学杂志

OBJECTIVE: To determine the insoluble particles in human immunoglobulin (pH 4) for intravenous injection and human hepatitis B immunoglobulin(pH 4) for intravenous injection in both liquid and freeze-dried forms from 14 domestic manufacturers. METHODS: Thirty-two batches of human immunoglobulin products including 19 batches of human immunoglobulin (pH 4) for intravenous injection, 5 batches of human immunoglobulin (pH 4) for intravenous injection (freeze-dried), 5 batches of human hepatitis B immunoglobulin (pH 4) for intravenous injection, and 3 batches of human hepatitis B immunoglobulin(pH 4) for intravenous injection (freeze-dried) were tested by light obscuration particle count test method recommend by appendix of 2010 CHP by GWF-8JA laser particle size analyzers. Insoluble particles greater than 10 and 25 μm were counted, respectively. Microscopy counting was carried out if the test results from light blockage method did not meet the qualification criteria in 2010 ChP. Trend analysis and comparison of the results from NIFDC and enterprises were done. RESULTS: The test results of insoluble particles in 32 batches of human immunoglobulin products indicate that 90.6%(29/32) of the products complied with the requirement of 2010 ChP and the results were in agreement between NIFDC and enterprises. Unfortunately, 3 batches of human immunoglobulin(pH 4) for intravenous injection(freeze-dried) failed in light obscuration particle count test, however, they met the requirement of 2010 ChP when microscopy counting method was used. CONCLUSION: The quality control of human immunoglobulin products in China market is generally fine, and the test results from different laboratories are basically consistent. Test result of insoluble particles in freeze-dried IVIG by microscopy counting method and light obscuration particle count test method show significant difference.

Excretion-secretion products and proteases from live Sporothrix schenckii yeast phase: immunological detection and cleavage of human IgG / Produtos da excreção-secreção e proteases da fase leveduriforme do Sporothrix schenckii: detecção imunológica e clivagem de IgG humana

Antigenic preparations from Sporothrix schenckii usually involve materials from mixed cultures of yeast and mycelia presenting cross-reactions with other deep mycoses. We have standardized pure yeast phase with high viability of the cells suitable to obtain specific excretion-secretion products without somatic contaminations. These excretion-secretion products were highly immunogenic and did not produce noticeable cross-reactions in either double immunodiffusion or Western blot. The antigenic preparation consists mainly of proteins with molecular weights between 40 and 70 kDa, some of them with proteolytic activity in mild acidic conditions. We also observed cathepsin-like activity at two days of culture and chymotrypsin-like activity at four days of culture consistent with the change in concentration of different secreted proteins. The proteases were able to cleave different subclasses of human IgG suggesting a sequential production of antigens and molecules that could interact and interfere with the immune response of the host.

As preparações antigênicas de Sporothrix schenckii provêm geralmente de cultivos mistos de leveduras e micélios e apresentam reações cruzadas com outras micoses profundas. Foi padronizada a obtenção da fase leveduriforme pura, com alto índice de células viáveis, o que permite, por sua vez, obter produtos específicos da excreção-secreção sem contaminantes somáticos. Estes produtos da excreção-secreção são altamente imunogênicos, e não apresentam reações cruzadas visíveis em dupla difusão e sem Western blot. O preparado antigênico consiste principalmente em proteínas com peso molecular entre 40 e 70 kDa, sendo que algumas apresentam atividade proteolítica em meios levemente ácidos. Foi observada atividade do tipo catepsina em produtos da excreção-secreção obtidos a partir de leveduras de dois dias de cultivo, e atividade do tipo quimiotripsina aos quatro dias de cultivo, consistente com a mudança de concentração de proteínas secretadas. As proteases puderam clivar diferentes subclasses de IgG humanas, o que sugere uma produção seqüencial de antígenos e moléculas que podem interagir com a resposta imune do hospedeiro.

Preparation of fluorescent polyclonal antibody against human IgG and its application in the diagnosis of nephrosis / 医学研究生学报

Objective:To prepare polyclonal antibody against human immunoglobulin G(human IgG) and to use this antibody in the clinical diagnosis of nephrosis. Methods:New Zealand rabbit was immunized subcutaneously with human IgG.Enzyme linked immunosorbent assay(ELISA) was applied to reveal the titer of the prepared polyclonal antiserum against human IgG.Antiserum was purified with affinity chromatography,and the purified antibody was confirmed for its specificity by Western blot and immunohistochemical staining.The purified antbodies which have been indentified were fused with FITC(fluorescein isothiocyanate) and then analyzed by immunohistochemical staining.Finally the fused antibody was useed in the clinical diagnosis of nephrosis. Results: The titer of the obtained antiserum was up to(1∶128 000,) double agar diffusion test showed the titer of the antibody was 1∶32.By the method of affinity chromatography,we obtained purified antibody with the purity of 85%.ELISA,double agar diffusion and immunohistochemical staining tests showed that the specificity and titer of the antibody were not decreased sharply.The purified antibody fused with FITC also kept the specificity of the primitive antibody.When the FITC fused antibody was tried in nephrosis patients,it detected human IgG effectively. Conclusion:The polyclonal antibody can specifically recognize human IgG.This purified antibody fused with FITC can be used in the diagnosis of nephrosis.