Kawasaki disease is a systemic vasculitis that mainly affects younger children. Although the definite cause still remains unknown but the clinical and epidemiologic findings discuss an infectious cause. The prevalence of incomplete Kawasaki disease reported 15 to 36.2%, and it is more frequent in the extremes of the age spectrum. Non delayed treatment of disease should be initiated because of critical cardiac vascular complications. Up to 15%-25% of patients with Kawasaki disease who remain febrile after administration of first dose of Intravenous immunoglobin plus aspirin are classified as refractory disease. These Intravenous immunoglobin resistant cases are at increasing risk for coronary artery complications. The strategy on prediction of potentially non responder and treatment of Intravenous immunoglobin resistant patients is now controversial but some useful points were recommended.

Intravenous Immunoglobulin (IVIG) is a safe biological product used in many cases, such as primary and secondary immune deficiency, infectious diseases, critically ill patients and autoimmune disorders. IVIG has many advantages compared with intramuscular Immunoglobulin and is obtained from pooled plasma of 10.000-60.000 donors. Today, IVIG use in clinical settings has increased. Despite its use in many diseases, FDA only has approved its use in patients suffering from ITP, primary immunodeficiency, pediatric HIV, Kawasaki disease, bone marrow transplantation and B-cells chronic lymphocytic leukemia. But, it has been used for other cases like Guillain–Barré syndrome, premature infants, critically ill patients admitted to ICU, severe asthma and uncontrolled seizures which FDA had not approved yet.This biological product affects immune deficiency and autoimmune diseases in different ways. Despite the relative safety of this product, the patient may experience mild but acute complications.In most cases acute reaction is due to high speed infusion rate which is mild but systemic side effects in the form of allergic and non-allergic anaphylaxis are seen, especially in patients with CVID and IgA selective deficiency. The patient should be under medical supervision during infusion and to deal with anaphylaxis shock, facilities should be available.Today, the subcutaneous type of Immunoglobulin is used at home under supervision of a nurse and the patient satisfaction is high, because of its lower cost and also it does not need hospital admission.

Myocarditis is an inflammatory disease of the myocardium with lymphocyte infiltration and myocyte necrosis leading to a wide range of clinical presentations including heart failure, arrhythmia, and cardiogenic shock. Infectious and noninfectious agents may trigger the disease. The fact that immunosuppressive drugs are useful in several kinds of autoimmune myocarditis is proof of the autoimmune mechanisms involved in the development of myocarditis. Pathogenic mechanisms in myocardial inflammation are including inflammasome activation followed by myocyte destruction, myocarditis, and pericarditis. Intravenous Immunoglobulin (IVIG) is a serum product made up of Immunoglobulins, widely used in a variety of diseases. This product is effective in several immune-mediated pathologies. As well as the determined usage of IVIG in Kawasaki disease, IVIG may be useful in several kinds of heart failure including fulminant myocarditis, acute inflammatory cardiomyopathy, Giant Cell Myocarditis, and peripartum cardiomyopathy. Generally, IVIG is used in two different doses of low dose (200 to 400 mg/kg) and high dose (2 g/kg) regimen. The exact therapeutic effects of IVIG are not clear, however over the last decades, our knowledge about its mechanism of function has greatly enhanced. IVIG administration should be based on the accepted protocols of its transfusion. In this review article, we try to provide an overview of the different kinds of myocarditis, pathologic mechanisms and their common treatments and evaluation of the administration of IVIG in these diseases. Furthermore, we will review current protocols using IVIG in each disease individually.

Background: Evidence indicating that Intravenous Immunoglobulin (IVIG) could prove effective against neonatal immune hemolytic hyperbilirubinemia, reducing the need for exchange transfusion. Objective: To determine the prophylactic effect of IVIG in decreasing the incidence and severity of neonatal immune hemolytic jaundice. Methods: A case-control prospective randomized trial in Hafez and Nemazi Hospitals in Shiraz, Iran, was undertaken between September 1998 through August 1999. Fulfilling the inclusion criteria forty full-term, healthy neonates with Rh or ABO incompatibility, entered the study and were randomly assigned into 2 equal groups. IVIG was administered in a single dose of 500 mg/kg to 20 neonates in the treatment group 4-6 hours during the first 24 hours of life. Nothing was given to 20 controls. All neonates were evaluated prospectively to detect jaundice or pallor till 10-days-old. Results: The IVIG-treated neonates had a smaller rise in their bilirubin levels, required significantly less phototherapy (0% vs. 35%) (p<0. 05) and a shorter duration of hospitalization than those in the control group. In both groups, the hematocrit and hemoglobin values remained fairly stable and none of the infants had bilirubin levels exceeding the exchange levels. No side effects of IVIG were seen. Conclusions: Our results indicates that IVIG administration may be effective in prevention of neonatal immune hemolytic hyperbilirubinemia. However, further studies are required to validate the efficacy of this treatment and to determine the optimal dose, number of infusions and the best preparation of IVIG treatment.

Background and Objectives: Intravenous Immunoglobulin (IVIG) preparations are used in several disorders including primary and secondary immunodeficiencies, autoimmune and systemic inflammatory diseases; it is also applied in effective therapy of infectious diseases. IVIG is currently the most widely used plasma component in the world. The addition of multiple steps to the manufacturing process of IVIG lowers the yield of IgG and raises the manufacturing costs. Therefore, manufacturers attempt to employ a cost effective method with respect to safety and quality of the product. In this study we proposed a method which not only reduced the manufacturing steps but also raised product safety by the treatment of pasteurization as a virus inactivation method.Materials and Methods: For this experimental study, the fraction II paste (rich in IgG) was obtained from fresh frozen plasma (FFP) by the modified cold ethanol fractionation method (Cohn’s method). For further purification, filtration was used to remove impurities. Before performing virus inactivation by pasteurization, protein solution was diafiltered and then the stabilizer was added. Pasteurized solution was diafiltered again and final product was prepared after sterile filtration.Results: The quality control results of the finished product obtained by the proposed method revealed that the purity was 100% (determined by cellulose acetate electrophoresis) and the polymer amount was less than 1% (evaluated by HPLC chromatography). The secondary and tertiary structures of IgG molecule were also examined by circular dichroism spectroscopy technique and were then compared to the commercial IVIG products bringing about approximately similar and satisfactory results. The yield calculated for the initial amount of IgG of plasma was 39.1% as measured by nephelometery.Conclusions: The high purity of the finished product confirmed that the proposed purification process was satisfactory in despite of fewer steps when compared to the current methods. Indeed, the costeffective preparation together with quatily and safety are taken into account in the proposed method. If the complementary experiments are carried out, the proposed method can escalate at the industry scale.

Purpose: To report a case of non-paraneoplastic autoimmune retinopathy (npAIR) treated with Intravenous Immunoglobulin (IVIG). Case report: A 12-year-old boy presented with progressive visual field loss, nyctalopia, and flashing for three months. He had suffered from common cold two weeks before the onset of these symptoms. On the basis of clinical history and paraclinical findings, he was diagnosed with npAIR, and IVIG without immunosuppressive therapy was started. During the one-year follow-up period after the first course of IVIG, flashing disappeared completely. Visual acuity remained 10/10, but nyctalopia did not improve. Multimodal imaging showed no disease progression. Conclusion: Although established retinal degenerative changes seem irreversible in npAIR, IVIG may be a suitable choice to control the disease progression.

Background: Presence of infectious complications in oncohematological patients significantly complicates treatment and worsens the prognosis of the underlying disease. In such situations Immunoglobulin (IVIG) is applied. The aim of our study was to examine the efficiency of the use of IVIG in 67 children with oncohematological pathology.Methods: IVIG for the treatment of purulent-septic complications (neutropenia) used in patients with ALL - 47% (32), AML - 40% (26), histiocytosis 3% - (2), AA - 6% (4), MDS -3% (2) and PID is -1 (1, 4%), were administered a single dose of 0.2 g/kg/day, daily 1 per day, duration ranged from 1 to 7 days. The effectiveness of the drug in patients with oncohematological pathology with infectious complications was estimated on the normalization of body temperature, the results of the general analysis of peripheral blood, the general analysis of urine, triple culture of blood.Findings: Patients receiving of IVIG due to the presence of infectious complications were at various stages of therapy. Patients with acute lymphoblast leukemia received therapy program ALL-BFM-2002; patients with c myeloblastic leukemia (CML) received therapy program AML-BFM-2004; patients with Langerhans LCH-III or aplastic anemia received immunosuppressive therapy. Infectious complications in the study patients included sepsis, which took place in the form of sepsis without localized foci of infection in 15.3%, septicemia in 2.6%, sepsis with septicopyemia in 10.2%, and febrile neutropenia in 78.6%. Localization panicucci foci include bacterial carditis in 2.8%, ulcero-necrotic colitis in 8.5%, pneumonia in -10.8%, and necrotic lesions disease in 20.8%. The extension of infection occurred at the background of deep cytopenia-neutropenia (leukocytes less than 1×109, granulocytes less than 0, h). Microbial landscape of the patients was presented in 78.4% of the cases as gram-positive microorganisms and in 21.6% as gram-negative microorganisms. From blood of patients, gram-positive pathogens was seen in 55%. Among them the most oftenorganisms was Staphyloccocus aureus in 71.2%, Streptococcus Epidermidis in 66.7%, and Streptococcus in 67, 5%. Among fungal infection Candida (36.4%), Peniccillium (31.8%), Mucor (22.7%), and Aspergilius (9.1%) were seen. Mushrooms more often were detected from the blood (72.6%) than from other sources of infection. The markers to the virus Abstain-Barr (EBV) were seropositive in 48.9% of children, of Herpes virus in 93.3% of children, and of cytomegalovirus (CMV) from 80.2%. The dynamics of the temperature reaction was the following: the initial temperature of 38.4±of 0.36 Degrees; it was normalized after 1 day from the beginning of introduction of IGW treatment, in 18 patients (26%), within 3 days from the introduction of IGW in 25 patients (39%), within 5-7 days in 17 patients (25%), and was inefficient in 7 (10%) cases among whom were patients with progression of the underlying disease, recurrence ALL (1 case) and AML (2 cases), one patient with myelodysplastic resistant to therapy with manifestations of fungal sepsis not having achieved remission.Conclusion: Use of Immunoglobulin in complex therapy of oncohematological patients, in the period of agranulocytosis has allowed to achieve improvement in 92.3% of cases, decreased the incidence and severity of infectious complications, achieved more rapid recovery of hemopoiesis, accelerated normalization of blood, and reducing interruptions in the treatment course.