Single-center controlled study for application of hemoperfusion and versus gammaglobulin for the treatment of Henoch-Sch?nlein purpura combined gastrointestinal bleeding in children / 中华实用儿科临床杂志

Objective:To compare the clinical efficacy and safety of hemoperfusion (HP) and gammaglobulin on the treatment of Henoch-Sch?nlein purpura (HSP) with gastrointestinal bleeding in children.Methods:Case-control study.A total of 39 HSP children combined with gastrointestinal bleeding diagnosed in the Department of Pediatric Nephrology, Rheumatology and Immunology, Shengjing Hospital of China Medical University from January 2015 to December 2019 were retrospectively recruited.They were divided into the HP group and the gammaglobulin group according to the therapeutic strategy.Clinical data were collected, and a 6-month follow-up survey was conducted for monitoring the relapse of gastrointestinal bleeding and the occurrence of kidney injury.The differences between groups were compared by Fisher′s exact test, two independent samples t-test, Mann-Whitney U-test, Kruskal-Wallis H-test, and One-Way ANOVA. Results:(1) There were 20 cases in the HP group and 19 cases were included in the gammaglobulin group.The gammaglobulin group was younger than the HP treatment group.(2) In addition to gastrointestinal bleeding, children in both groups had other clinical symptoms, such as abdominal pain, angioneurotic edema, and hematuria.(3)Comparison of laboratory indexes: Inflammatory indexes: white blood cell count (WBC), C-creative protein (CRP) and coagulation function indexes: fibrin degradation products (FDP), D-dimer (DD) were significantly elevated before treatment in the 2 groups, and there was no difference between the 2 groups ( P>0.05); WBC, CRP and FDP, DD declined in the 2 groups after treatment compared with the former, and there was no difference between the 2 groups ( P>0.05); (4) Comparison of clinical manifestations: when HP was applied with gammaglobulin in the treatment window within 3 d, the difference in the time of abdominal pain relief in the HP group was shorter than that of the gammaglobulin group [1.00(1.00, 1.00) d vs.2.00(1.75, 6.50) d, P=0.011]; comparing the time of gastrointestinal bleeding stopping when HP was applied with gammaglobulin comparison, the difference in gastrointestinal bleeding cessation time was not statistically significant ( P>0.05); (5) Comparison of hospitalization time: within 3 d application of HP compared with other window period hospitalization time were significantly reduced [(16.89±4.99) d than (19.20±2.39) d than (34.83±8.40) d, both P<0.05]; (6) Comparison of hospitalization costs: within 3 d application of HP compared with other window period hospitalization costs were significantly reduced [25 554.03 (22 168.61, 28 527.30) yuan than 33 619.48 (32 661.18, 36 971.47) yuan than 51 290.34 (34 163.04, 64 772.66) yuan, both P<0.05]; There were no statistically significant difference in the hospitalization time and hospitalization cost between and within the gammaglobulin group (all P>0.05); (7) Comparison of hormone dosages: the difference in the results of the initial dose of hormone use, pre-treatment dose of gammaglobulin/HP, and post-treatment dose of gammaglobulin/HP between the two groups of children was not statistically significant(all P>0.05). Safety profile was comparable between groups.The difference in hormone dosage before and after treatment within the gammaglobulin and HP treatment group was statistically different ( P<0.001). Conclusions:For children with severe HSP accompanied by gastrointestinal bleeding, early treatment with blood purification can rapidly relieve clinical symptoms and reduce the number of hospital days and hospitalization costs.For cases where blood purification is not available or suitable, gammaglobulin treatment is another option.

Evaluación de la zona gamma del proteinograma por electroforesis: correspondencia clínico-patológica / Evaluation of protein electrophoresis in the gamma zone: clinicopathological correspondence / Avaliação da área gamma do proteinograma por eletroforese: correspondência clínico-patológica

El proteinograma por electroforesis (PxE) sérico es solicitado para detectar modificaciones del perfil proteico. El objetivo del trabajo fue evaluar las alteraciones de la zona gammaglobulina y su correspondencia con distintos estados clínico-patológicos. Se incluyeron 7.259 pacientes (1-89 años) a los que en 2013 se les solicitó PxE. Según el trazado densitométrico, en la zona gammaglobulina se reconocieron diferentes grupos: hipogammaglobulinemia (<0,60 g/dL), hipergammaglobulinemia policlonal (≥1,80 g/dL), banda monoclonal (BM) y bandas oligoclonales. Prevaleció la hipergammaglobulinemia policlonal (4,2%), seguida por BM (1,4%) e hipogammaglobulinemia (0,8%). Hipergammaglobulinemia policlonal (>3 g/dL) se observó en: hepatitis autoinmune, cirrosis, síndrome de Sjögren, enfermedad mixta del tejido conectivo, HIV, hepatitis C y enfermedad de Castleman. El hallazgo de BM correspondió a 47% de pacientes con gammapatía monoclonal de significado incierto y 40% con mieloma múltiple; el 0,5% fueron casos nuevos. Con hipogammaglobulinemias, en adultos prevaleció la inmunosupresión terapéutica (55%), seguida por diabetes/síndrome metabólico/hipotiroidismo (23%); en niños, 22% por inmunosupresión y 78% con hipogammaglobulinemia no clasificada como inmunodeficiencia primaria. Se concluye que en 6,4% de los PxE se observó alteración de la zona gammaglobulina; prevaleció la hipergammaglobulinemia policlonal. En 1 de cada 200 PxE se pesquisó un paciente con BM. El hallazgo de hipergammaglobulinemia policlonal o BM se correspondió con distintos estados clínico-patológicos.

Serum protein electrophoresis (PEP) is requested to screen changes in the protein profile. The aim of this study was to evaluate alterations in the gamma globulin zone and correspondence with various clinical and pathological states. 7259 patients were included (1-89 years of age) who had been requested a PEP in 2013. According to the densitometric tracing, in the gamma globulin zone different groups were recognized: hypogammaglobulinemia (<0.60 g/dL), polyclonal hypergammaglobulinemia (≥1,80 g/dL), monoclonal band (MB) and oligoclonal band. The polyclonal hypergammaglobulinemia prevailed (4.2%), followed by MB (1.4%) and hypogammaglobulinemia (0.8%). Polyclonal hypergammaglobulinemia (>3 g/dL) was observed in autoimmune hepatitis, alcoholic cirrhosis, Sjögren's syndrome, mixed connective tissue disease, HIV, hepatitis C and Castleman's disease. The MB finding corresponded to a 47% of patients with monoclonal gammopathy of undetermined significance and 40% with multiple myeloma; 0.5% were new cases. In adults, hipogammaglobulinemias prevailed in therapeutic immunosuppression cases (55%), followed by patients with diabetes/ metabolic syndrome/ hypothyroidism (23%); in children, 22% with immunosuppression and 78% corresponded to hipogammaglobulinemias not classified as primary immunodeficiency. To conclude, an alteration in the gamma globulin zone was observed in 6.4% of PEP. In 1 out of 200 PEP MB was found. The finding of polyclonal hypergammaglobulinemia or MB corresponded to different clinicopathological states.

O proteinograma por eletroforese (PXE) sérico é solicitado para detectar modificações no perfil proteíco. O objetivo do trabalho foi avaliar as alterações da área gammaglobulina e sua correspondência com diversos estados clínico-patológicos. Incluíram-se 7259 pacientes (1-89 anos) aos quais, em 2013, foi solicitado um PxE. De acordo com o traçado densitométrico, na área gammaglobulina, diferente grupos foram reconhecidos: hipogammaglobulinemia (<0,60 g/dL), hipergammaglobulinemia policlonal (≥1,80 g/dL), banda monoclonal (BM) e bandas oligoclonais. Prevaleceu a hipergammaglobulinemia policlonal (4,2%), seguida por BM (1,4%) e hipogammaglobulinemia (0,8%). Hipergammaglobulinemia policlonal (>3 g/dL) foi observada em: Hepatite autoimune, cirrose, síndrome de Sjögren, doença mista do tecido conjuntivo, HIV, hepatite C e doença de Castleman. O achado de BM correspondeu a 47% de pacientes com gammapatia monoclonal de significado indeterminado e 40% com mieloma múltiplo; 0,5% eram casos novos. Com hipogammaglobulinemias em adultos prevaleceu a imunossupressão terapêutica (55%), seguida por diabete/síndrome metabólica/hipotireoidismo (23%); em crianças, 22% por imunossupressão e 78% com hipogammaglobulinemia não classificados como imunodeficiência primária. Conclui-se que em 6,4% dos PxE foi observada alteração da área gammaglobulina; prevaleceu a hipergammaglobulinemia policlonal. Em 1 de cada 200 PxE foi encontrado um paciente com BM. O achado de hipergammaglobulinemia policlonal ou BM se correspondeu com diferentes estados clínico-patológicos.

Avaliação da absorção colostral em neonatos ovinos da raça Bergamácia / Evaluation of colostral absorption in neonates of Bergamacia breed

Objetivou-se determinar o período de absorção das macromoléculas colostrais e a transferência de imunidade passiva em cordeiros da raça Bergamácia. Avaliou-se o proteinograma sérico dos cordeiros antes da ingestão de colostro até 48 horas de vida e das frações colostrais ao nascimento e 12 horas pós-parto. Foi avaliada a concentração de proteína total no soro por refratometria e sua relação com a densidade e quantidade de gamaglobulinas presentes no colostro. A concentração sérica de gamaglobulina nos cordeiros variou de 0,111±0,07g/dL antes da ingestão de colostro a 1,609±0,72g/dL às 48 horas. Nas amostras de colostro, a concentração variou de 3,125±1,27g/dL, imediatamente após o parto, para 1,378±0,82g/dL, 12 horas após. A concentração de proteína sérica total teve acréscimo de 4,46±0,58g/dL para 5,61±0,75g/dL entre o nascimento e após 48 horas, apresentando correlação positiva com a densidade e a proteína total colostral. A absorção colostral pelo cordeiro foi ascendente até 24 horas subsequentes ao parto, quando, então, iniciou-se sua estabilização. A quantificação da proteína sérica, com uso de refratômetro nos cordeiros, pode ser usada como método para avaliar a transferência de imunidade passiva, pois está diretamente relacionada com a absorção de gamaglobulina colostral...

The period of absorption of colostrum macromolecules and the passive immunity transfer of Bergamacia lambs was determined. The serum proteinogram of lambs before the intake of colostrum up 48 hours of life and colostrum fractions between delivery and twelve hours after birth were measured. The total protein concentration in serum was evaluated by refractometry and also its relationship with the density and amount in colostrum. The serum concentration of gamma globulin in lambs from 0.111±0.07g/dL before the intake of colostrum was 1.609±0.72g/dL at 48 hours. In the colostrum samples, the concentration was 3.125±1.27g/dL immediately after delivery and 1.378±0.82g/dL twelve hours after birth. Serum total protein concentration increased from 4.46±0.58g/dL to 5.61±0.75g/dL between birth and after 48 hours, there was positive correlation with the density and total protein colostrum. The lamb had ascendant colostrum absorption subsequent to delivery, for twelve hours and then began its stabilization. The quantification of serum protein with the use of the refractometer in lambs can be used as a method to evaluate the transfer of passive immunity because it is directly related to the absorption of colostral gammaglobulin...

Gammaglobulins in critical care / Gamaglobulinas em Terapia Intensiva

The use of intravenous immunoglobulin (IVIG) is relatively in frequent in patients admitted to intensive care units (ICUs). However, “off-label” IVIG prescriptions for different conditions are highly prevalent. The aim of this paper is to review the existing evidence for the use of IVIG in patients admitted to ICUs, emphasizing non-infectious diseases and complications: hypogammaglobulinemia of the critically ill, hemophagocytic lymphohistiocytosis (HLH), Guillain-Barré syndrome (GBS), Kawasaki disease (KD), chylothorax, acute myocarditis, toxic shock syndrome (TSS), Stevens-Johnson syndrome (SJS)/toxic epidermalnecrolysis (TEN), and sepsis. In conclusion, in critically ill patients, IVIG use is of benefit in KD, GBS, and TSS. It may benefit patients with fulminant acute myocarditis. The benefit is not proven in patients with HLH, chylothorax, and SJS/TEN.

O uso de imunoglobulina intravenosa (IVIG) é relativamente infrequente em pacientes internados em unidades de terapia intensiva (UTIs). Entretanto, prescrições “off-label” de IVIG para diferentes patologias são altamente prevalentes. O objetivo deste artigo é revisar as evidências existentes para o uso de IVIG em pacientes internados em UTIs, enfatizando as doenças e complicações não infecciosas: hipogamaglobulinemia do paciente crítico, linfo-histiocitose hemofagocítica (HLH), síndrome de Guillain-Barré (GBS), doença de Kawasaki (KD), quilotórax, miocardite aguda, síndrome do choque tóxico (TSS), síndrome de Stevens-Johnson (SJS)/necrólise epidérmica tóxica (TEN), e sepse. Em conclusão, em pacientes criticamente enfermos, o uso de IVIG é benéfico em KD, GBS e TSS. IVIG pode ser benéfica em pacientes com miocardite aguda fulminante. O benefício não foi comprovado em pacientes com HLH, quilotórax e SJS/TEN.

Properties and action mechanisms of intravenous gammaglobulin / Propriedades e mecanismos de ação da gamaglobulina intravenosa

Intravenous immunoglobulins (IVIG) preparations have been used as a substitutive therapy for primary and secondary immunodeficiencies for many years; now it is well know that IVIG can have two other important and opposite functions: pro and anti-inflammatory, depending on its concentration. Low doses of IVIG exert proinflammatory activities, that require complement activation or binding via Fc fragment of IgG to IgG Fcγ specific receptors present on effector cells of innate immunity. On the other hand, administrationof high IVIG doses has anti-inflammatory activity. This manuscript presents a revision of how IVIG IgG mediated this effects, how and upon which cells from the immune system acts, their capacity to scavenging complement fragments, the recently demonstrated anti-inflammatory activity of variable minor sialylated portion of IgG molecules and finally, the importance of genetic variation and expression of Fcγ receptors. To comprehend the mechanism of action of IVIG is fundamental, not only to obtain further improvements on therapeutic effectiveness, but also to discover new and unexpected capacities of this biological bomb known as IVIG.

As preparações de imunoglobulinas intravenosas (IVIG) têm sido utilizadas como terapia substitutiva para imunodeficiências primárias e secundárias durante muitos anos; agora é sabido que a IVIG pode ter duas outras funções importantes e opostas: pró e anti-inflamatória, dependendo da sua concentração. Em baixas doses a IVIG exerce atividades pró-inflamatórias, que requerem uma ativação do complemento ou uma ligação via fragmento Fc de IgG para IgG Fcγ receptores específicos presentes nas células efetoras da imunidade inata. Doutro lado, a administração de altas doses de IVIG provoca uma atividade anti-inflamatória. Este manuscrito apresenta uma revisão de como a IVIG IgG mediou nestes efeitos, como e sobre quais celulas do Sistema imune atua, a sua capacidade para limparem fragmentos do complemento, a atividade anti-inflamatória recentemente demostrada de porção sializada menor variável de moléculas de IgG e, finalmente, a importância da variação genética e expressão de receptores Fcγ. Conocer o mecanismo de ação da IVIG é fundamental para obtermos, não só uma melhoria na eficácia terapêutica, mais também para descobrirmos novas e surpreendentes capacidades desta bomba biológica chamada IVIG.

Período de absorção intestinal de macromoléculas em cabritos recém-nascidos após a ingestão de colostro bovino / Macromolecules intestinal absorption period of goat kids after bovine colostrum intake

Após o nascimento, os cabritos são dependentes das imunoglobulinas colostrais devido às características placentárias que não permitem a passagem de macromoléculas da circulação materna. De acordo com a literatura, os cabritos possuem capacidade absortiva por até quatro dias. Muitos aspectos fisiológicos de outras espécies são aceitos e utilizados para caprinos, mas aqueles relacionados à transferência de imunidade passiva precisam de investigação. Os objetivos do presente estudo foram determinar o período de passagem de macromoléculas da mucosa intestinal para a circulação e a duração da proteção humoral transferida passivamente pela ingestão de colostro bovino e caprino. Sessenta cabritos recém-nascidos foram distribuídos em seis tratamentos: T 0 (n=25), ingestão natural de colostro caprino à vontade; T 1 (n=7), colostro bovino entre o nascimento e duas horas pós-parto; T 2 (n=7), ingestão de colostro bovino entre quatro e seis horas pós-nascimento; T 3 (n=7), leite nas primeiras oito horas e colostro bovino entre 10 e 12 horas pós-parto; T 4 (n=7), ingestão de leite até 18 horas e colostro bovino entre 22 e 24 horas pós-nascimento; T 5 (n=7), leite até 30 horas e ingestão de colostro bovino entre 34 e 36 horas pós-parto. Determinaram-se as concentrações séricas de proteína total (PT), gamaglobulina, imunoglobulina G (IgG) e a atividade sérica de gama glutamiltransferase (GGT). Ao nascimento, todos os neonatos tiveram valores mais baixos das variáveis, com aumento significativo da PT e gamaglobulina, após dois dias, nos grupos T 0, T 1 e T 2; a IgG e GGT aumentaram em todos os grupos. Os tratamentos T 3, T 4 e T 5 foram considerados como indutores de falha de transferência de imunidade passiva. A absorção de macromoléculas pelo trato intestinal dos cabritos ocorreu até 36 horas pós-parto, sendo mais efetiva até 12 horas. Os níveis de anticorpos persistiram até 75 dias após a ingestão de colostro bovino, porém, com concentrações inadequadas.

After birth goat kids are dependent of colostrum immunoglobulins due to placental characteristics that don't allow macromolecules passage from dam's circulation. According to literature goat kids have colostrum immunoglobulin absorption capability for up to four days. Many physiological aspects of other species have been accepted and used for goats, but those related to passive immunity transference needs more investigation. The goals of the present study was to determine the period of macromolecules passage through gut wall to circulation until 36 hours postpartum and verify the duration of protective humoral immunity transferred by the ingestion of bovine and caprine colostrum. Sixty newborn goat kids were allocated into six treatment groups: T 0 (n=25), non-restricted natural ingestion of goat colostrum; T 1 (n=7), bovine colostrum from birth to two hours postpartum; T 2 (n=7), bovine colostrum ingestion between four to six hours after birth; T 3 (n=7), milk intake until the first eight hours and bovine colostrum administration between 10 to 12 hours postpartum; T 4 (n=7), milk ingestion for the first 18 hours and bovine colostrum ingestion between 22 and 24 hours after birth; T 5 (n=7), milk administration until 30 hours and bovine colostrum intake between 34 to 36 hours postpartum. The total protein (TP), gammaglobulin, immunoglobulin G (IgG) and gamma-glutamyltransferase (GGT) serum concentrations were determined. At birth all neonates presented lower values of the variables, with significant increase of TP and gammaglobulin at two days in groups T 0, T 1 and T 2, IgG and GGT increased in all groups. The treatments T 3, T 4 and T 5 were considered to induce failure of immunity passive transfer. The absorption of macromolecules by kid's intestinal tract occurred until 36 hours postpartum, with better effectiveness until 12 hours. Antibody levels persist up to 75 days after bovine colostrum intake, but at this time their low concentrations doesn't provide adequate protection.

Intravenous Infusion of Tranexamic Acid during Aortic Valve Replacement in a Patient with Indiopathic Thrombocytopenic Purpura / 日本心臓血管外科学会雑誌

An 82-year-old woman, who had suffered from idiopathic thrombocytopenic purpura (ITP) treated with oral steroids, was admitted to our hospital with worsening exertional dyspnea. Cardiac examinations revealed severe aortic stenosis with left ventricular dysfunction. High dose intravenous gammaglobulin therapy (400 mg/kg/day) for 5 days was conducted to increase the platelet count prior to the operation. However, a decrease was observed in the platelet count from 2.1×10⁴/mm³ on admission to 1.9×10⁴/mm³ before surgery. Without additional therapy, aortic valve replacement using a 19 mm bioprosthesis was performed with cardiopulmonary bypass (CPB). Tranexamic acid (20 mg/kg/h) was continuously infused from the skin incision to the end of the surgery. Forty units of the platelet concentrates were transfused just after weaning from CPB. The patient had no hemorrhagic complications. We believe that intraoperative administration of tranexamic acid combined with platelet transfusion is effective to reduce perioperative bleeding for a patient with ITP unresponsive to preoperative gammaglobulin therapy.

Characterization of a Hepatitis B virus strain in southwestern Paraná, Brazil, presenting mutations previously associated with anti-HBs Resistance / Caracterização de uma cepa de hepatite por vírus B no sudoeste do Paraná, Brasil, apresentando mutações previamente associadas à resistência anti-HBs

The present study investigated if hepatitis B virus (HBV) mutants circulate in the southwestern region of the State of Paraná, Brazil, by analyzing samples from children who received immunoprophylaxis but were born to HBV carrier mothers. Samples from 25 children were screened for HBV serum markers and for HBV DNA by PCR. Only one sample was positive for HBsAg, anti-HBs and HBV DNA, although the child had been vaccinated. Analysis of the S gene sequence of this sample showed the presence of a proline at position 105, a serine at position 114, three threonines at positions 115, 116 and 140, and a glutamine at position 129. The presence of these amino acids, except for serine at position 114, has been related to monoclonal or polyclonal therapy with anti-HBs after liver transplantation, whereas the presence of threonine at position 116 has been described in immunized children from Singapore. This finding demonstrates the possible circulation of HBV strains resistant to hepatitis B immunoprophylaxis in southwestern Paraná, Brazil. The genotype of the sample was identified as genotype D, which is frequently found in the region studied. Since 36 percent of the children had received incomplete or no immunoprophylaxis, more extensive follow-up of children born to HBsAg-positive mothers is needed.

O presente estudo investigou se mutantes do vírus da hepatite B (HBV) circulam na região Sudoeste do Estado do Paraná, Brasil, analisando amostras de crianças que receberam a imunoprofilaxia por terem nascido de mães portadoras do HBV. Amostras de 25 crianças foram analisadas para os marcadores sorológicos do HBV e para o DNA-HBV por PCR. Somente uma amostra foi positiva para AgHBs, anti-HBs e DNA-HBV, apesar da criança ter sido vacinada. Análises da seqüência do gene S desta amostra mostrou a presença de uma prolina na posição 105, uma serina na posição 114, três treoninas nas posições 115, 116 e 140, e uma glutamina na posição 129. A presença destes aminoácidos, exceto para Serina na posição 114, tem sido relacionada a terapia monoclonal ou policlonal com anti-HBs após transplante de fígado, enquanto a presença da treonina na posição 116 tem sido descrita em crianças imunizadas de Singapura. Este achado demonstra a possível circulação de cepas do HBV resistentes a imunoprofilaxia para hepatite B no Sudoeste do Paraná, Brasil. O genótipo da amostra foi identificado como genótipo D, o qual é frequentemente encontrado na região estudada. Desde que 36 por cento das crianças tinham recebido incompleta ou nenhuma imunoprofilaxia, um seguimento mais intensivo das crianças nascidas de mães AgHBs positivo é necessário.

Perfil eletroforético das proteínas séricas de serpentes Crotalus durissus terrificus (cascavel) criadas em cativeiro / Serum protein electrophoresis profile of the rattlesnake Crotalus durissus terrificus kept in captivity

As serpentes peçonhentas dos gêneros Bothrops e Crotalus têm sido mantidas em cativeiro visando à extração de venenos para a produção de imunobiológicos. O conhecimento da fisiologia desses animais e as alterações na concentração de proteínas e suas frações séricas são importantes para a identificação precoce de importantes enfermidades que cursam com estados de hipoproteinemia e hiperproteinemia. O objetivo do trabalho foi determinar a concentração de proteína total e o perfil eletroforético das proteínas séricas de serpentes Crotalus durissus terrificus (cascavel) criadas em cativeiro. Foram colhidas amostras de sangue da veia coccígea ventral de 21 serpentes adultas e sadias, divididas em dois grupos: Grupo 1 de 12 machos com peso médio de 588,89±193,55g, e Grupo 2 de nove fêmeas com peso médio de 708,33±194,04g. A proteína total sérica foi determinada pelo método de refratometria e a eletroforese em gel de agarose. Obtiveram-se valores da proteína total sérica (g/dL) de 4,51±0,50 para machos e de 4,82±0,72 para fêmeas, e para machos e fêmeas de 4,64±0,61. Foram identificadas pela eletroforese quatro frações protéicas (g/dL): albumina, a, b, g-globulinas e calculada a relação albumina:globulina. As serpentes fêmeas apresentaram maiores valores para as variáveis, albumina e para a relação albumina/globulina (AG) diferindo significativamente (P<0,05) do grupo de machos, porém sem significado clínico.

The poisonous snakes of the genera Crotalus and Bothrops have been kept in captivity with the purpose of extracting poison for the production of immunobiological. Knowledge of the physiology of these animals and serum proteins concentration changes are important for early identification of major diseases which lead to states of hypoproteinemia and hyperproteinemia. The objective was to determine the concentration of total protein and serum protein electrophoresis profile of Crotalus durissus terrificus (rattlesnake) in captivity. Blood samples were taken from the ventral coccygeal vein of 21 adult and healthy snakes divided into groups: Group 1 with 12 males, weighing in average 588.89±193.55g, and Group 2 with nine females, weighing in average 708.33±194.04g. The total serum concentration of protein was determined by the method of refractometry and agarose gel electrophoresis. The total protein values in the serum for females was 4.82±0.72, for males 4.51±0.50 and males and females 4.64±0.61, identified by four fractions (g/dL): albumin, a, b and g-globulin. Additionally the albumin/globulin ratio was calculated. The female snakes showed higher values for the variables, albumin and the albumin/globulin (AG) differed significantly (P<0.05) from the group of male snakes, but there was no clinical significance.

An Experience of ABO-incompatible Kidney Transplantation Using Plasmapheresis and Anti-CD20 Monoclonal Antibody / 대한진단검사의학회지

Due to an extreme shortage of cadaveric kidneys, many centers in Japan successfully performed ABO-incompatible kidney transplantations using plasmapheresis, splenectomy and immunosuppression. Recently, a protocol including anti-CD20 monoclonal antibody (rituximab) and antigen-selective immunoadsorption has been used for ABO-incompatible transplantation in Europe. In Korea, ABO-incompatible kidney transplantation has been rarely performed. We report an experience of successful ABO-incompatible kidney transplantation using plasmapheresis and rituximab. The patient was a 32-yr-old female suffering from chronic renal failure, and her blood type was O, Rh+. The donor was her husband, and his blood type was B, Rh+. A combination therapy including 5 times of plasmapheresis starting from 10 days before transplantation with 2-day interval, intravenous gammaglobulin, rituximab at 2 weeks before transplantation and potent immunosuppression successfully decreased the titers of anti-A and anti-B antibodies to 1:2 and 1:1, respectively. The kidney transplantation was successful without any sign of hyperacute or acute rejection.

Hepatitis crónica por el virus B embarazo y prevención de la transmisión perinatal: pregnancy and prevention of perinatal transmission / HBV chronic hepatitis

La probabilidad de evolucionar a hepatopatía crónica posterior a una infección por el virus B depende de la edad el 80 al 90% de los niños expuestos el primer año de vida desarrollarán la enfermedad. Se debe incluir la serología para el virus B en los exámenes de rutina del control prenatal. La inmunoprofilaxis pasiva y activa previene la infección perinatal del virus B. En las mujeres embarazadas portadoras de infección crónica por virus B con alta carga viral se documenta un 20-30% de transmisión al niño recién nacido. Las madres con alta carga viral deben ser tratadas con lamiduvina posterior a la semana 28 de gestación. El niño de madre con alta carga viral debe recibir dos dosis de HBIg y el esquema de vacunación. El niño de madre positiva con carga viral baja debe recibir dosis única de HBIg y esquema de vacunación completa. Se debe de realizar control serológico de anticuerpo y antígeno de superficie en el niño entre los 9 -15 meses de edad. La lactancia no está contraindicada.

The probability to evolve to chronic liver disease post HBV infection depends on the age. Between 80 to 90% of children exposed in their first year of life will develop this disease.The serology for HBV must be included in the routine tests of perinatal control. The active and passive immunoprophylaxis prevents the perinatal infection of HBV. It has been documented that pregnant women carrying the HBV chronic infection with high viral burden have a 2030% transmission to the new born child. Those mothers with high viral burden must be treated with lamiduvine after the 28th gestation week. The child of a mother with high viral burden must receive two unique dosages of HBIg and a complete vaccination scheme. Antibody and surface antigen serologic control must be done to the child between 9-15 months of age. Lactancy is not contraindicated.

Reevaluation of the Efficacy of Intravenous Gammaglobulin in the Prevention and Treatment of Coronary Artery Lesion in Kawasaki Disease / 华中科技大学学报（医学）（英德文版）

Summary: In order to objectively evaluate the efficacy of intravenous gammaglobulin (IVIG) in the prevention and treatment of coronary artery lesion (CAL) in Kawasaki disease (KD) and the related factors influencing the IVIG efficacy, 314 children with KD were reviewed retrospectively and comparatively and were divided into IVIG plus aspirin group and ASA group. The occurrence and restoration of CAL in these two groups as well as many laboratory and clinical indexes including average hospital stay (days), total fever duration, defervescence time, platelet count, erythrocyte sedimentation rate, C reactive protein etc. were observed. The incidence of CAL was 39.5 % in the children with KD. In the IVIG+ASA group, the incidence of CAL was 34.3 % and 56.0 % in ASA group respectively (P<0.001). The incidence of CAL was reduced in the group in which 2.0 g/kg or 1.0 g/kg IVIG was administered as compared with the group in which IVIG was administered at a dose ≤0.6 g/kg or ≥3.0 g/kg (P<0.05). CAL occurred less frequently when IVIG was administered at 3-10 days of the course than that when IVIG was administered ≤3 days or >10 days (P<0.05). About 13.4 % of the CAL treated with IVIG was not recovered at the 12 th month of the course, mostly in the groups in which only ASA was administered and IVIG treatment was started 10 days later. The hospital stay (days), defervescence time, total fever duration, platelet count, erythrocyte sedimentation rate and C reactive protein were significantly reduced in IVIG+ASA group as compared with those in the ASA group (P<0.05). IVIG treatment can remarkably shorten the course of patients with KD and decrease the incidence of CAL, but the efficacy of IVIG in the prevention and treatment of KD disease is not as expected by people, therefore, reevaluation of the practical efficacy of IVIG is required.

Clinical Features and Treatment of Human Parvovirus B_(19) Infection Associated Henoch-Schonlein Purpura / 实用儿科临床杂志

0.05),but the latter was superior to the former in extinction of exanthem.4.B_(19)-DNA clearance of hormone group was 25.0%,that of gamma globulin group was 81.82%,and there was significant difference between 2 groups(P

Comparison of Effectiveness between Two Different Protocols of Treatment of IV gamma-globulin in Idiopathic Thrombocytopenic Purpura

PURPOSE: Several methods of IV gamma-globulin(IVG) infusion are effective in the treatment of autoimmune disease, including idiopathic thrombocytopenic purpura(ITP). But it is not known which method is more effective in the treatment of ITP. The effectiveness of these two methods of IVG infusion was studied in terms of platelet recovery rate, side effects and recurrence rate. METHODS: Forty seven patients with acute ITP in the department of pediatrics, Eulji University Hospital from January 1995 to June 2001 were enrolled. We assesssed the treatment effects of 47 patients blindly selected; IV gamma-globulin 2 g/kg/day in one day(treatment group A, n=25), 400 mg/ kg/day in five days(treatment group B, n=22). RESULTS: Treatment group A increased platelet count more rapidly on the 2nd, 4th and 6th day of treatment than treatment group B. Side effects like fever, chill and vomiting were more frequent in treatment group A than treatment group B. The platelet count on the sixth day of treatment showed a greater increase in the cases which had side effects than in the cases which did not. There was no difference between the two groups in the recurrence rate. CONCLUSION: IV gamma-globulin 2 g/kg/day in one day increases platelet count more rapidly than 400 mg/kg/day in five days, and is favorable for the prevention of a severe hemorrhagic episode like early intracranial hemorrhage.

HLA Cross-match Negative Conversion by Therapeutic Plasmapheresis in a Patient with Positive HLA Cross-match / 대한진단검사의학회지

It is well accepted that kidney transplantation cannot be done if the recipient has antibodies showing a positive HLA cross-match to the donor. Recently, Schweitzer and his associates used a combination therapy with plasmapheresis, IV gamma globulin, and potent immunosuppression to induce HLA cross-match negative conversion in patients with a positive HLA cross-match to living donors and they reported good results after the trials. Therefore, we treated a patient with combination therapy who had persistent-positive HLA cross-match to multiple living donors. The patient was a 38-year-old female suffering from chronic renal failure and she showed persistent positive HLA cross-match to multiple living donors. Using a combination therapy with plasmapheresis, IV gamma globulin and immunosuppression, we have successfully achieved a HLA cross-match negative conversion in a patient and we did kidney transplantation without any sign of hyperacute or acute rejection. Although we present possibility of a HLA cross-match negative conversion by combination therapy, especially in a recipient with a low titer cross-match positive to a family donor, further long-term study with more patients is needed for evaluation of the efficacy of this trial.

Clinical Characteristics in Patients with Kawasaki Disease Who Received Intravenous Gamma-globulin Retreatment

PURPOSE: Although the use of intravenous gamma-globulin(IVGG) in Kawasaki disease(KD) is effective in reducing clinical symptoms and coronary artery complications, 20-30% of patients have persistent or recrudescent fever and ongoing clinical symptoms. In these patients, the additional infusion of IVGG is considered. The authors studied the characteristics of patients who received IVGG retreatment, and compared them with the patients who did not need IVGG retreatment, for determination of IVGG retreatment. METHODS: We reviewed the medical records of 117 KD patients who could be followed up at least six months. We studied the conventional laboratory findings, electrocardiogram(EKG), signal averaged ECG(SAECG) and echocardiogram. RESULTS: Twenty three patients had early cardiac complications during the six months of follow-up. Four patients had late cardiac complications after six months. The early cardiac complication rate was higher in the IVGG retreatment group than the single infusion group(P<0.0001). The late complication rate was also higher in the retreatment group(P<0.0001). The patients who received methyl-prednisolone(m-PD) pulse therapy had much higher rates of early and late cardiac complications than those who received a single IVGG infusion. Among the clinical data and laboratory findings, only CRP increased significantly in patients who have had the cardiac complications. The IVGG retreatment group had increased CRP than the single infusion group. CONCLUSION: The patients with increased initial CRP may have an increased incidence of complications and an increased possibility of IVGG retreatment. We thought that retreated KD patients might have inflammations severe enough to need high dose IVGG as shown by high CRP levels, and IVGG retreatment could not prevent coronary artery lesions sufficiently.

Intravenous Gamma-globulin Retreatment in Kawasaki Disease

PURPOSE: This study was designed to determine the outcome and safety of intravenous gamma-globulin(IVGG) retreatment in Kawasaki disease. METHODS: A clinical observation of the therapeutic effects, laboratory findings and echocardiograms was carried out on 72 patients with Kawasaki disease in Kosin University Hospital from 1991 to 1999. 27 patients were treated with 1g/kg/day IVGG for 2 days, 45 patients were treated with 2 g/kg for 10hours. The clinical indication for retreatment was fever. Persistent fever was defined as a temperature> or =38.3degrees C persisting beyond 48hrs after the completion of the infusion. Recrudescent fever was defined as a temperature> or =38.3degrees C for 48hrs after the completion of the infusion, followed by a temperatureup. CONCLUSION: We concluded that the IVGG retreatment of Kwasaki disease may improve the clinical course and coronary artery outcome.

Outcome of Cardiac Complication in Patients of Kawasaki Disease Treated with High-dose Gammaglobulin

PURPOSE: We performed the study to evaluate the risk factors associated with cardiac complication and the outcome of coronary lesions after high-dose (2g/kg, 1dose) gammaglobulin (IVIG) treatment in patients with Kawasaki disease (KD). METHODS: Retrospective studies were performed on 338 cases of KD treated with high-dose IVIG at this hospital from May 1994 to March 1999. RESULTS: Among 338 patients, fever was persistent for 8.09+/-3.45 days in all patients and after IVIG infusion, fever subsided in 26.5+/-26.4 hours. Forty-five patients (13.3%) with abnormal baseline echocardiogram remained febrile for 9.09+/-4.09 days, significantly longer than normal baseline echocardiogram group. There was no significant differences in age, sex and other clinical findings. In 7 patients (2.1%) usage of medications above 12 months were needed. Eleven cases(3.3%) with persistent fever after their first course of IVIG therapy became afebrile post-second course of IVIG in 5 of the 11 cases with cardiac complication and in 1 case with recurrence. Twelve (male 11, female 1) of the 338 cases recurred after 8.45+/-4.39 months. Three of those 12 cases had cardiac complication. CONCLUSION: The overall prevalence of persistent coronary abnormalities in KD patients treated with high-dose IVIG and aspirin remains low. Even if patients had cardiac complication, they improved later.

Therapeutic Response of Different Treatment Modalities in the Adult Idiopathic Thrombocytopenic Purpura / 대한혈액학회지

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a severe reduction in the number of circulating platelets. Corticosteroid therapy, which has been used in ITP for many years, has produced a complete or partial response rate of 65% to 75%, although sustained remissions have been reported in only 18% to 32% of the patients. The purpose of the present study is to define response to each treatment and ultimate outcome of adults with ITP. METHOD: A clinical study was done on 35 cases of ITP who had admitted to the department of Internal Medicine, Yeungnam University Hospital from June 1983 to July 1996. The response of each treatment modalities was based on criteria of Defino and Cooperative Latin American Group on Hemostasis and Thrombosis. RESULTS: The mean age of patients was 41.9 years old and female to male ratio was 1:0.6 (female:22 cases, male:13 cases). The complete response rate to intravenous gamma-globulin as early therapy was obtained in 72% of the patients and the duration to platelet count above 50,000/ microliter was 3 days after intravenous gamma-globulin therapy. Duration of response was 15 days. The CCR (continuing complete response) to corticosteroid was obtained in 16% of the patients, and no CCR to reinduction with corticosteroid was observed. The CR to splenectomy was obtained in 85% of the patients. The overall results of all therapeutic modalities were CCR 43%, TCR (temporary complete response) 20%, PR (partial response) 31%, and NR (no response) 6%. CONCLUSION: This analysis of ITP in adults suggests that splenectomy remains as the most effective treatment and intravenous gamma-globulin is effective for rapid elevation of platelet count.

A Study on Clinical Application of Harada's Scoring Method to Kawasaki Disease: Suggesting the revision of the criteria for IVGG treatment of KD in Korea

PURPOSE: We performed a study on clinical benefits of Harada's scoring method, problems of present criteria and requirement of the revision of that criteria for intrvenos gamma-globulin (IVGG) treatment of Kawasaki disease (KD) in Korea. METHODS: The patients of 104 cases who visited to Department of Pediatrics of Chungnam National University Hospital and diagnosed as KD were objective group. We classified the patients to high and low risk groups as two methods of Harada's scoring method and present criteria for IVGG treatment of KD in Korea, and analyzed the results after treatment by means of echocardiogram. RESULTS: 1. Characteristics of the patients 1) Duration to diagnosis: Mean duration was 6.0 3.5days after onset of fever. 2) Age of onset and male to female ratio: the patients of 84.6% were the age of 4 years or less, and male to female ratio was 1.8 to 1. 2. Coronary aitery lesion (CAL) abnormalities according to items of Harada's scoring method and classification of risk groups 1) The age of less than 12 months and CRP of 3+ or more were significant predictive factors 2) Classification of risk groups was significantly predictive in CAL development. 3. Classification of total cases The patients reserved to administration for IVGG according to Harada's scoring method and present criteria for IVGG treatment of KD in Korea were 63.5% and 50% respectively. If so in present criteria for IVGG treatment of KD in Korea, 50% of the KD patients were excluded for that application. 4. Outcome after treatment according to selective IVGG use by two methods 1) Selective IVGG use by Harada's scoring method: There was no significant difference in development of CAL between low risk group and high risk group, resulting in 1 case (14.3%) of 7 cases and 5 cases (9.4%) of 53 cases respectively. 2) Selective IVGG use by present criteria for IVGG treatment of KD in Korea: There were more significant cases at low risk group than those at high risk group in development of CAL, resulting in 3 cases (42.9%) of 7 cases and 5 cases (12.2%) of 41 cases respectively. 3) Outcome after treatment in patients not administered IVGG as low risk groups according to two methods: There were more significant cases in low risk group at present criteria for IVGG treatment of KD in Korea than those at Harada's scoring method in the development of CAL, resulting in 3 cases (42.9%) of 7 cases and 1 case (14.3%) of 7 ases respectively. 4) Outcome after treatment in patients administered IVGG as high risk groups according to two methods: There was no significant difference between high risk groups at both methods in the CAL development, resulting in 5 cases (12.2%) of 41 cases at present criteria for IVGG treatment of KD in Korea and 5 cases (9.4%) of 53 cases at Harada's scoring method. CONCLUSIONS: Harada's scoring method had more benefits in clinical application, selection of risk group, and outcome after treatment, So we propose the revision of present criteria for IVGG treatment of KD in Korea, suggesting another method as indication for selective IVGG treatment.