Acute Acquired Immune Thrombocytopenia After Cardiac Surgery: A Challenging Case

Thrombocytopenia is a common condition that recognizes an infinite number of possible causes, especially in specific settings like the one covered in this case report: the postoperative period of cardiac surgery. We report a case of an old male with multiple comorbidities who underwent a coronary angioplasty procedure and aortic valve replacement. He showed severe thrombocytopenia in the postoperative days. Differential diagnosis required a big effort, also for the experts in the field. Our goal was to aggressively treat the patient with prednisolone, platelets, and intravenous immunoglobulins to maximize the prognosis. Our patient developed no complications and was discharged successfully

Características clínicas y evolución de los pacientes con enfermedad de Kawasaki en un hospital general / Clinical characteristics and course of patients with Kawasaki disease at a general hospital

La enfermedad de Kawasaki (EK) es la principal causa de cardiopatía adquirida en menores de cinco años. Nuestro objetivo fue conocer las características clínicas, el compromiso coronario y la evolución de pacientes atendidos en nuestra institución. Se revisó una serie de casos desde 2001 hasta 2018. Se incluyeron 63 pacientes, 58 % varones; la mediana de edad fue 2,6 años. La mediana de días de fiebre al diagnóstico fue 5,5 días. El 33 % presentó la forma incompleta y se detectó compromiso coronario en el 20 %. El 60 % de los pacientes con afectación coronaria presentaron EK incompleta versus el 28 % de presentación incompleta en los pacientes sin compromiso coronario (p 0,06). No se observaron diferencias en datos de laboratorio entre los grupos según el compromiso coronario. En conclusión, 33 % presentó EK incompleta y el 20 %, afectación coronaria. Hubo una tendencia de mayor riesgo para daño coronario en la forma incompleta.

Kawasaki disease (KD) is considered the leading cause of acquired heart disease in children younger than 5 years. Our objective was to know the clinical characteristics, coronary involvement, and course of patients seen at our facility. A case series from 2001 to 2018 was reviewed. Sixty-three patients were included; their median age was 2.6 years; 58% were males. The median duration of fever at the time of diagnosis was 5.5 days. The incomplete form was observed in 33% and coronary involvement, in 20%. Among patients with coronary involvement, 60% had incomplete KD versus 28% among those without coronary involvement (p: 0.06). No differences were observed between groups in laboratory data based on coronary involvement. To conclude, 33% had incomplete KD and 20%, coronary involvement. There was a trend to a higher risk for coronary artery damage in the incomplete form of KD.

Next generation sequencing screening for human parvovirus B19 infection after liver transplantation and the analysis of related risk factors / 器官移植

Objective To summarize the screening Methods for human parvovirus (HPV) B19 infection after liver transplantation and analyze the related risk factors. Methods Clinical data of 86 recipients were retrospectively analyzed. According to the Results of next generation sequencing (NGS), all recipients were divided into the HPV B19 infection group and control group. Clinical characteristics, treatment regime and clinical prognosis of patients infected with HPV B19 were analyzed. The risk factors of HPV B19 infection were analyzed using univariate and multivariate Logistic regression model by forward LR step method. Results Nine of the 86 recipients developed fever and progressive anemia with unexplained reasons at approximately 2 weeks after liver transplantation. NGS detection demonstrated that HPV B19 was positive and they were diagnosed with pure red cell aplasia (PRCA) caused by HPV B19 infection. After intravenous immunoglobulins (IVIG) was given and the immunosuppressant therapy was adjusted, the hemoglobin levels in all patients were significantly increased. The Results of multivariate analysis revealed that low serum globulin level in peripheral blood at postoperative 7 d [odds ratio (OR) =0.749, P=0.040] and young age (OR=0.937, P=0.038) were the independent risk factors of HPV B19 infection after liver transplantation. Conclusions HPV B19 infection should be considered in relatively young patients with unexplained hemoglobin decline early after liver transplantation. NGS screening is an effective method for early diagnosis of HPV B19 infection. Low serum globulin level in peripheral blood at postoperative 7 d and young age may be independent risk factors of the incidence of HPV B19 infection.

Infliximab Treatment for Intravenous Immunoglobulin-resistant Kawasaki Disease: a Multicenter Study in Korea

BACKGROUND AND OBJECTIVES: We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens. METHODS: Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire. RESULTS: Subjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p 5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p < 0.001). CONCLUSIONS: This study suggests that the early administration of infliximab may reduce the incidence of significant CAA in patients with IVIG-resistant KD. However, further prospective randomized studies with larger sample sizes are required.

Factors Predicting Resistance to Intravenous Immunoglobulin Treatment and Coronary Artery Lesion in Patients with Kawasaki Disease: Analysis of the Korean Nationwide Multicenter Survey from 2012 to 2014

BACKGROUND AND OBJECTIVES: Approximately 10–15% of children with Kawasaki disease (KD) do not respond to initial intravenous immunoglobulin (IVIG) and have higher risk for coronary artery lesion (CAL). The aim of this study was to identify predictive factors from laboratory findings in patients who do not respond to IVIG treatment and develop CAL from KD. METHODS: We retrospectively collected nationwide multicenter data from the Korean Society of Kawasaki Disease and included 5,151 patients with KD between 2012 and 2014 from 38 hospitals. RESULTS: Among 5,151 patients with KD, 524 patients belonged to the IVIG-resistant group. The patients in the IVIG-resistant group had a significantly higher serum N-terminal pro-brain natriuretic peptide (NT-proBNP) level (1,573.91±3,166.46 vs. 940.62±2,326.10 pg/mL; p < 0.001) and a higher percentage of polymorphonuclear neutrophils (PMNs) (70.89±15.75% vs. 62.38±32.94%; p < 0.001). Multivariate logistic regression analyses revealed that significantly increased PMN, NT-proBNP, C-reactive protein (CRP), aspartate aminotransferase (AST), and alanine aminotransferase (ALT) were the predictors of IVIG resistance (p < 0.05). Multivariate logistic regression analyses also showed that only CRP was associated with the risk of CAL (p < 0.01), while PMN, NT-proBNP, AST, and ALT were not. CONCLUSIONS: Elevated PMN, serum NT-proBNP, CRP, AST, and ALT levels are significantly associated with IVIG resistance in patients with KD. Moreover, serum CRP is significantly increased in patients with KD with CAL.

Tratamiento exitoso con inmunoglobulinas intravenosas en un paciente con pseudoobstrucción intestinal asociada a lupus eritematoso sistémico / Successful treatment with intravenous immunoglobulins in a patient with intestinal pseudo-obstruction associated with systemic lupus erythematosus

Resumen El compromiso gastrointestinal en pacientes con lupus eritematoso sistémico (LES) es muy diverso. Su frecuencia y ubicación a lo largo del tracto digestivo varían ampliamente. Los procesos inflamatorios secundarios a los depósitos de complejos inmunes o eventos vasculares pueden ser los causantes de este compromiso. Una de las manifestaciones gastrointestinales características en los pacientes con LES es la pseudoobstrucción intestinal, que se define como la propulsión intestinal ineficaz que se produce en ausencia de factores mecánicos u obstructivos. Esta es, sin embargo, una complicación rara y poco entendida del LES. En este artículo, reportamos el caso de un paciente masculino con diagnóstico de LES y pseudoobstrucción intestinal, que fue tratado exitosamente con esteroides e inmunoglobulinas intravenosas. Se presenta una revisión completa de la literatura y una propuesta de la fisiopatología de la manifestación.

Abstract Gastrointestinal involvement in patients with systemic lupus erythematosus (SLE) is very diverse, and the frequency of occurrence and location along the digestive tract varies widely. Inflammatory processes secondary to immune complex deposits or vascular events may cause this involvement. One of the most characteristic gastrointestinal manifestations in these patients is the intestinal pseudo-obstruction, which is defined as the ineffective intestinal propulsion that occurs in the absence of mechanical or obstructive factors. This is, however, a rare and poorly understood complication of SLE. The case is presented of a male SLE patient presenting with intestinal pseudo-obstruction, and was successfully treated with steroids and intravenous immunoglobulin. A complete review of the literature and a proposal for the pathophysiology of intestinal pseudo-obstruction are presented.

Predictors of intravenous immunoglobulin resistance in Kawasaki Disease in a tertiary children's hospital

@#<p style="text-align: justify;"><strong>BACKGROUND AND OBJECTIVE</strong>: Kawasaki Disease (KD) is the leading cause of acquired heart disease in children in developed countries. We aimed to determine the predictors of intravenous immunoglobulin (IVIG) resistance based on clinical manifestations and laboratory parameters.</p><p style="text-align: justify;"><strong>METHODOLOGY:</strong> This was a retrospective cohort study of classic KD patients.    </p><p style="text-align: justify;"><strong>RESULTS:</strong> Two hundred and ten patients were included in the study. The mean age was 2.0 ±1.8 years old with slight female predominance at 51.4%. Seven (3.3%) cases were found to be IVIG resistant. There was no significant difference in age, clinical manifestations or fever duration.  Univariate analysis revealed that IVIG resistant group had a heavier weight with mean of 16.4 kg ± 12.2 compared to the IVIG responder group 12.2kg ± 4.8. The IVIG resistant group had a higher white blood cell count of 23.9 ± 7.8 compared to the responder group of 17.9 ± 6.5.                           </p><p style="text-align: justify;"><strong>CONCLUSION AND RECOMMENDATIONS:</strong> There is an IVIG resistance rate of 3.3% among classic KD patients. A high white blood cell count and weight are probable predictors for IVIG resistant KD. We recommend a larger sample size of resistant cases and a case-control multicenter study.</p>

Uso de inmunoglobulinas intravenosas en una paciente obstétrica con choque séptico: reporte de caso y revisión de la literatura / Use of intravenous immunoglobulins in an obstetric patient with septic shock: Case report and review of the literature

Objetivo: Reportar el caso de una paciente obstétrica que desarrolló un shock séptico secundario a una infección gastrointestinal y el uso de inmunoglobulina endovenosa (IVIG) como terapia coadyuvante y hacer una revisión de la literatura publicada del uso de inmunoglobulinas en shock séptico con énfasis en su uso en el embarazo. Materiales y métodos: Se presenta el caso de una paciente con embarazo de 36 semanas, quien presentó un choque séptico secundario a una infección gastrointestinal y progresó con falla orgánica multisistémica. En vista de la pobre respuesta al tratamiento instaurado se inició terapia coadyuvante con inmunoglobulina enriquecida en IgM con una buena respuesta. Se realizó una búsqueda de la literatura en la bases de datos de Medline vía PubMed, Direct Sciencie, Biblioteca Cochrane y SciELO utilizando las palabras clave de los Descriptores en Ciencias de la Salud (DeCS) como inmunoglobulina endovenosa y sepsis en embarazo. Resultados: Se encontraron 6 artículos relacionados directamente con el uso de IVIG en sepsis en el embarazo, 1 revisión de tema, 2 reportes de caso y 3 guías diagnósticas y de manejo. Las inmunoglobulinas intravenosas se han utilizado con mayor frecuencia en pacientes no obstétricas en shock séptico como terapia coadyuvante especialmente en infección por estreptococos y estafilococos. Conclusión: Las inmunoglobulinas intravenosas podrían ser una alternativa como coadyuvante en manejo de shock séptico en gestantes con pobre respuesta al tratamiento convencional. Se necesitan estudios analíticos que incluyan gestantes para confirmar estos hallazgos.

Objective: To report the case of an obstetric patient who developed septic shock secondary to a gastrointestinal infection, and the use of intravenous immunoglobulin (IVIG) as an adjunct, and to conduct a review of the published literature on the use of immunoglobulins in septic shock with emphasis on its use during pregnancy. Materials and methods: Case report of a patient with 36 weeks of gestation who presented with septic shock secondary to a gastrointestinal infection resulting in multiple organ failure. In view of poor response to treatment, adjunctive therapy with IgM-enriched immunoglobulin therapy was initiated, achieving good response. A search of the literature was conducted in the Medline, Direct Science, Cochrane Library and SciELO databases using Health Science Descriptors (DeCS) such as intravenous immunoglobulin and sepsis in pregnancy. Results: Six articles directly related to the use of IVIG in sepsis during pregnancy were found, including 1 review, 2 case reports and 3 diagnosis and management guidelines. Intravenous immunoglobulins have been used with greater frequency in non-obstetric patients in septic shock as an adjunct especially in streptococcal and staphylococcal in fections. Conclusion: Intravenous immunoglobulins could be an alternative as adjunct therapy in the management of septic shock in pregnant women who respond poorly to conventional treatment. Analytical studies including pregnant women are needed in order to confirm these findings.

Autoimmune Hemolytic Anemia after Intravenous Immunoglobulin Therapy in a Child with Kawasaki Disease / 임상소아혈액종양

Kawasaki disease (KD) can cause acquired heart disease and systemic vasculitis in children. It is treated with intravenous immunoglobulin (IVIG). A significant complication is development of coronary artery lesions such as dilatations or aneurysms. However, uncommon complications can occur, like autoimmune hemolytic anemia when IVIG is used. We present a case of autoimmune hemolytic anemia associated with KD. Dilatation of right coronary artery was found at echocardiography and he was treated twice with IVIG (2 g/kg) with interval of 48 hours. Laboratory finding showed hemoglobin 7.1 g/dL, hematocrit 20.8%, corrected reticulocyte 5.86%, total bilirubin 0.29 mg/dL, lactate dehydrogenase 425 IU/L, and haptoglobin 5 mg/dL. Normocytic, normochromic anemia with anisopoikilocytosis was found on peripheral blood smear, and direct antiglobulin test was positive. The patient was started on oral prednisolone for 3 weeks, with which all symptoms resolved. We report this rare case, prompting consideration of IVIG associated complications when treating KD.

Meta-analysis of factors predicting resistance to intravenous immunoglobulin treatment in patients with Kawasaki disease / 소아과

PURPOSE: Studies have been conducted to identify predictive factors of resistance to intravenous immunoglobulin (IVIG) for Kawasaki disease (KD). However, the results are conflicting. This study aimed to identify laboratory factors predictive of resistance to high-dose IVIG for KD by performing meta-analysis of available studies using statistical techniques. METHODS: All relevant scientific publications from 2006 to 2014 were identified through PubMed searches. For studies in English on KD and IVIG resistance, predictive factors were included. A meta-analysis was performed that calculated the effect size of various laboratory parameters as predictive factors for IVIG-resistant KD. RESULTS: Twelve studies comprising 2,745 patients were included. Meta-analysis demonstrated significant effect sizes for several laboratory parameters: polymorphonuclear leukocytes (PMNs) 0.698 (95% confidence interval [CI], 0.469-0.926), C-reactive protein (CRP) 0.375 (95% CI, 0.086-0.663), pro-brain natriuretic peptide (pro-BNP) 0.561 (95% CI, 0.261-0.861), total bilirubin 0.859 (95% CI, 0.582-1.136), alanine aminotransferase (AST) 0.503 (95% CI, 0.313-0.693), aspartate aminotransferase (ALT) 0.436 (95% CI, 0.275-0.597), albumin 0.427 (95% CI, -0.657 to -0.198), and sodium 0.604 (95% CI, -0.839 to -0.370). Particularly, total bilirubin, PMN, sodium, pro-BNP, and AST, in descending numerical order, demonstrated more than a medium effect size. CONCLUSION: Based on the results of this study, laboratory predictive factors for IVIG-resistant KD included higher total bilirubin, PMN, pro-BNP, AST, ALT, and CRP, and lower sodium and albumin. The presence of several of these predictive factors should alert clinicians to the increased likelihood that the patient may not respond adequately to initial IVIG therapy.

Prediction of nonresponsiveness to medium-dose intravenous immunoglobulin (1 g/kg) treatment: an effective and safe schedule of acute treatment for Kawasaki disease / 소아과

PURPOSE: Medium-dose (1 g/kg) intravenous immunoglobulin (IVIG) is effective in the majority of patients with Kawasaki disease (KD) but some patients who do not respond to medium-dose IVIG are at high risk for the development of coronary artery lesions (CALs). The purpose of this study was to identify the clinical predictors associated with unresponsiveness to medium-dose IVIG and the development of CALs. METHODS: A retrospective study was performed in 91 children with KD who were treated with medium-dose IVIG at our institution from January 2004 to December 2013. We classified the patients into responders (group 1; n=68) and nonresponders (group 2; n=23). We compared demographic, laboratory, and echocardiographic data between the 2 groups. RESULTS: Multivariate logistic regression analysis identified 6 variables as predictors for resistance to medium-dose IVIG. We generated a predictive scoring system assigning 1 point each for percentage of neutrophils ≥65%, C-reactive protein≥100 mg/L, aspartate aminotransferase≥100 IU/L, and alanine aminotransferase≥100 IU/L, as well as 2 points for less than 5 days of illness, and serum sodium level≤136 mmol/L. Using a cutoff point of ≥4 with this scoring system, we could predict nonresponsiveness to medium-dose IVIG with 74% sensitivity and 71% specificity. CONCLUSION: If a patient has a low-risk score in this system, medium-dose IVIG can be recommended as the initial treatment. Through this process, we can minimize the adverse effects of high-dose IVIG and incidence of CALs.

Non-Responders to Intravenous Immunoglobulin and Coronary Artery Dilatation in Kawasaki Disease: Predictive Parameters in Korean Children

BACKGROUND AND OBJECTIVES: In Kawasaki disease (KD), high dose intravenous immunoglobulin (IVIG) significantly lowers the coronary complications. However, some patients either do not respond to initial therapy or develop coronary complications. We aimed to identify the predictive factors for unresponsiveness to initial IVIG therapy and coronary artery dilatation (CAD; defined by Z-score≥2.5) in the acute phase and convalescent phase. SUBJECTS AND METHODS: A retrospective review was conducted of 703 patients with KD, admitted to Gachon University Gil Medical Center between January 2005 and June 2013. The patients were divided into two groups-IVIG responders vs. non-responders-based on the IVIG treatments, and presence of fever after treatment. Further, these groups were divided into two subgroups based on their CAD. RESULTS: Among the 703 patients with KD, the rate of non-responders to initial IVIG was 16.8%. Serum total bilirubin, platelet count, and neutrophil proportion were independent predictive parameters of unresponsiveness (p<0.05). CAD was found in 234 patients (33.3%) in the acute phase, and in 32 patients (4.6%) in the convalescent phase. Male gender, fever duration, serum C-reactive protein, and white blood cell count were related to CAD (p<0.05). CAD was detected more frequently in non-responders than in the responders (47.5% vs. 31.5%, p=0.001). Kobayashi, Egami, and Sano scoring systems applied to our study population reflected low sensitivities (28.0-33.9%). CONCLUSION: Several independent parameters were related to unresponsiveness to the initial IVIG or CAD. These parameters might be helpful in establishing more focused and careful monitoring of high-risk KD patients in Korea.

Autoimmune Hemolytic Anemia after Intravenous Immunoglobulin Therapy in a Child with Kawasaki Disease / 임상소아혈액종양

Kawasaki disease (KD) can cause acquired heart disease and systemic vasculitis in children. It is treated with intravenous immunoglobulin (IVIG). A significant complication is development of coronary artery lesions such as dilatations or aneurysms. However, uncommon complications can occur, like autoimmune hemolytic anemia when IVIG is used. We present a case of autoimmune hemolytic anemia associated with KD. Dilatation of right coronary artery was found at echocardiography and he was treated twice with IVIG (2 g/kg) with interval of 48 hours. Laboratory finding showed hemoglobin 7.1 g/dL, hematocrit 20.8%, corrected reticulocyte 5.86%, total bilirubin 0.29 mg/dL, lactate dehydrogenase 425 IU/L, and haptoglobin 5 mg/dL. Normocytic, normochromic anemia with anisopoikilocytosis was found on peripheral blood smear, and direct antiglobulin test was positive. The patient was started on oral prednisolone for 3 weeks, with which all symptoms resolved. We report this rare case, prompting consideration of IVIG associated complications when treating KD.

Encefalomielitis aguda diseminada grave: Comunicación de un caso asociado a infección por virus parainfluenza 3 / Severe acute disseminated encephalomyelitis associated with parainfluenza 3 infection: Case report

Disseminated encephalomyelitis (ADEM) is an infrequent condition with considerable morbidity and mortality in adult patients. It requires a high level of suspicion and diagnosis emerges by gathering clinical information, laboratory exams and images studies. ADEM is related to an immunological phenomena occurring after a bacterial/viral infection or recent vaccination. Glucocorticoids are the first line treatment, reserving immunoglobulins and plasmapheresis to refractory cases. We report a male patient aged 25, with ADEM associated to parainfluenza 3 virus respiratory infection that required mechanical ventilation and that had a complete recovery only after plasmapheresis.

La encefalomielitis aguda diseminada es una enfermedad infrecuente pero de elevada morbi-mortalidad en pacientes adultos. Demanda una sospecha y diagnóstico precoz que requiere el concurso de información clínica, pruebas de laboratorio y estudio de imágenes. De sustrato inmunológico, se puede relacionar a una infección viral, bacteriana o inmunización reciente. Los glucocorticoides son el tratamiento de elección, mientras que la inmunoglobulina intravenosa y la plasmaféresis se reservan para casos refractarios. Se presenta el caso de una encefalomielitis aguda diseminada grave, en un paciente de sexo masculino de 25 años, asociado a una infección respiratoria por virus parainfluenza 3. Requirió conexión a ventilación mecánica y tuvo una respuesta completa con plasmaféresis.

Low dose intravenous immunoglobulins and steroids in toxic epidermal necrolysis: A prospective comparative open-labelled study of 36 cases.

Background: Toxic epidermal necrolysis (TEN) is a severe adverse drug reaction associated with high mortality. Though different modalities of treatment are advocated, there is no consensus regarding specific therapy. Corticosteroids have shown conflicting results and for high dose intravenous immunoglobulins (IVIG), cost is a limiting factor. Aim: To find out the effectiveness of combination therapy with low-dose IVIG and steroids versus steroids alone in our TEN patients. Methods: After obtaining Ethical Committee approval, 36 consecutive TEN patients (2008-2012) were alternately allocated to 2 groups - Group A was given combination of low-dose IVIG (0.2-0.5 g/kg) and rapidly tapering course of steroids (intravenous dexamethasone 0.1- 0.3 mg/kg/day tapered in 1-2 weeks) while Group B was given same dose of steroids alone. Outcome parameters assessed were time taken for arrest of disease progression, time taken for re-epithelization, duration of hospital stay and mortality rates. Results: Both groups had 18 patients. Baseline characteristics like age, sex ratio, SCORTEN, body surface area involvement and treatment interval were comparable. Time for arrest of disease progression and for re-epithelization was significantly lowered in Group A (P = 0.0001, P = 0.0009 respectively). Though duration of hospital stay and deaths were less in Group A, difference was not statistically significant. SCORTEN based standardized mortality ratio (SMR) analysis revealed that combination therapy reduced the probability of dying by 82% (SMR = 0.18 ± 0.36) and steroids by 37% (SMR = 0.63 ± 0.71). Difference in SMR was statistically significant (P = 0.00001). No significant side effects due to either modality were found in any of the patients. Conclusion: Combination therapy with low-dose IVIG and steroids is more effective in terms of reduced mortality and faster disease resolution when compared to steroids alone in TEN.

Síndrome hemofagocítico secundario a infecciones graves en un quemado / Secondary hemophagocytic syndrome due to recurrent infections in a severely burned patient

El síndrome hemofagocítico constituye una entidad infrecuente, heterogénea, subdiagnosticada, y muchas veces fatal. En los casos secundarios, los desencadenantes pueden ser numerosos, tales como infecciones, fármacos, enfermedades autoinmunes y neoplasias. El mecanismo fisiopatogénico se explica por la presencia de una función disminuida o defectuosa de células NK y linfocitos T citotóxicos, que resulta en una activación inmune inefectiva y descontrolada, conduciendo al daño celular, falla multiorgánica y proliferación macrofágica con hemofagocitosis. Existen diferentes opciones terapéuticas, mayormente combinaciones de citostáticos y esteroides, cuyo objetivo es la supresión de la respuesta inmune descontrolada. Ocasionalmente, la condición clínica de algunos pacientes con síndrome hemofagocítico impide la utilización de esquemas terapéuticos intensivos. Comunicamos el caso de un paciente quemado grave, que reúne los criterios diagnósticos de síndrome hemofagocítico, quien presentó una evolución favorable con el tratamiento combinado de esteroides e inmunoglobulinas endovenosas.

The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.

Adverse events of intravenous immunoglobulin infusions: a ten-year retrospective study

BACKGROUND: Intravenous immunoglobulin (IVIG) is a biological product with adverse effects that appears to vary considerably among different IVIG preparations. OBJECTIVES: To describe the adverse events of patients given intravenous immunoglobulin infusions. METHOD: Data was collected on all patients receiving IVIG infusion at a tertiary hospital from January 2001 to December 2010. Descriptive statistics was used. RESULTS: 77 patients (45 males, 32 females) received IVIG infusions. Thirty two percent (n = 25) experienced adverse reactions. The most common indication was Kawasaki disease (85.7%) followed by immunodeficiency disorders (7.8%). Majority of the patients were children, with the highest frequency of infusions among those aged 2 to 8 years old (52%). 36 infusions were associated with occurrence of adverse effects. Fever was the most common adverse event (n = 11, 30.6%), followed by rash (n = 8, 22.2%) and chills (n = 7, 19.4%). Other adverse events were cyanosis (n = 3, 8.3%), hypotension (n = 2, 5.6%), hypothermia (n = 2, 5.6%), irritability (n = 1, 2.8%), vomiting (n = 1, 2.8%), and chest pain (n = 1, 2.8%). Adverse events were observed to occur most frequently within 1 to 6 h from onset of IVIG infusion. Among the various IVIG preparations available locally (Gammagard, Kiovig, Gamimune, Veno-S & IV Globulin S), Gammagard was the brand frequently used (50.7%). It also has the most number of adverse events, with 17 out of 41 (41.5%) infusions resulting in adverse reactions. Most of the reactions occurred with fast infusion rates, and clinical manifestations subsided when the rate of infusion was reduced. CONCLUSION: In this study, thirty two percent of patients given IVIG infusions experienced adverse events. Fever was the most common manifestation. Symptoms occurred within 1 to 6 h from onset of infusion, were affected by fast infusion rates, and managed by reducing the rate of infusion.

Late-Onset Narcolepsy with Cataplexy Unresponsive to Intravenous High-Dose Immunoglobulin Treatment

Narcolepsy with cataplexy (NC) is associated with hypocretin deficiency, and is thought to be an autoimmunity condition. The mean age at onset is estimated to be in the early 20s. Recent papers have addressed the response to immunotherapies in NC, with challenging results. We report a case of late-onset NC in a patient who did not benefit from early intravenous high-dose immunoglobulin (IVIg) therapy. This is the first reported attempt at using IVIg to treat an NC patient in Korea.

Intravenous Immunoglobulin Treatment in a Child with Resistant Atopic Dermatitis

In a subgroup of patients suffering from atopic dermatitis (AD), treatment is quite difficult even after taking oral immunosuppressants. High-dose intravenous immunoglobulin (IVIG) treatment has been reported to be beneficial for them in a few uncontrolled trials. Herein we report a case of intractable AD in a 5-year-old girl who had significant clinical improvement after receiving 3 cycles of IVIG treatment (2 g/kg) without notable side effects. Since the first infusion of IVIG, the patient's skin lesions improved steadily and the improvement persisted until the 8-month follow-up. The eczema area and severity index score decreased remarkably, while immunologic parameters did not correlate with clinical improvement. This case suggests that IVIG therapy can be quite effective and safe for children with resistant AD.

Efficacy of Intravenous Immunoglobulin Therapy in Childhood Atopic Dermatitis

OBJECTIVE: Atopic dermatitis (AD) is a chronic inflammatory skin disease with significant morbidity, and for which there is a need for safe and effective alternative therapies. Although a few observations on the efficacy of intravenous immunoglobulin (IVIG) in AD have been reported, clinical evidence of effectiveness from controlled trials is lacking. Therefore, the purpose of this study was to clarify whether IVIG therapy (1.0 g/kg body weight at each monthly visit for 6 months) is effective in childhood atopic dermatitis and to analyze the clinical characteristics of IVIG responses in this disease. METHODS: Forty three atopic dermatitis patients who had characteristic clinical features of atopic dermatitis were included in this study. The patients received an injection of IVIG at 1.0 g/kg body weight at each monthly visit for 6 months. Laboratory tests were performed for blood chemistry, total immunoglobulin E, immunoglobulin G/immunoglobulin A/immunoglobulin M, blood eosinophil count, and C-reactive protein. RESULTS: In total forty three atopic dermatitis patients, only 14 patients completely underwent 6 cycles, but other 29 patients incompletely (1-5 cycles). In the 14 patients, there were just 13 records of scoring atopic dermatitis (SCORAD) index. The mean SCORAD score in the 13 patients was 39.6+/-24.4. SCORAD score decreased significantly (initial SCORAD, 39.6+/-24.4; final SCORAD, 21.3+/-15.6; P=0.016). CONCLUSION: IVIG therapy may be recommended in the treatment of recalcitrant atopic dermatitis. In addition, further investigation on predictive markers for responses of IVIG therapy in atopic dermatitis may be needed.