Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Methylprednisolone ; therapeutic use ; Mucocutaneous Lymph Node Syndrome ; diagnosis ; drug therapy ; gamma-Globulins ; therapeutic use

Immune thrombocytopenia (ITP) is an immune-mediated acquired hemorrhagic autoimmune disease. At present, the first-line therapeutic drugs for ITP include glucocorticoids and intravenous immunoglobulins. However, about 1/3 of the patients had no response to the first-line treatment, or relapsed after dose reduction or withdrawal of glucocorticoids. In recent years, with the gradual deepening of the understanding on the pathogenesis of ITP, the drugs targeting different pathogenesis continually emerge, including immunomodulators, demethylating agents, spleen tyrosine kinase (SYK) inhibitors and neonatal Fc receptor (FcRn) antagonist. However, most of these drugs are in clinical trials. This review summarized briefly the recent advances in the treatment of glucocorticoids resistance and relapsed ITP, so as to provide reference for the clinical treatments.
Infant, Newborn ; Humans ; Purpura, Thrombocytopenic, Idiopathic/drug therapy* ; Glucocorticoids/therapeutic use* ; Thrombocytopenia ; Immunoglobulins, Intravenous/therapeutic use* ; Immunologic Factors/therapeutic use*

Child ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Mucocutaneous Lymph Node Syndrome ; drug therapy ; Neutrophils

Female ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Middle Aged ; Pyoderma Gangrenosum ; drug therapy ; pathology ; Skin ; pathology

Intravenous immunoglobulin (IVIG) has been widely used in chemotherapy for hematological malignancies, targeted therapy, and hematopoietic stem cell transplantation; however, there are still no available guidelines or consensus statements on the application of IVIG in pediatric hematological/neoplastic diseases at present in China and overseas. This consensus is developed based on the research advances in the application of IVIG in pediatric hematological/neoplastic diseases across the world and provides detailed recommendations for the clinical application of IVIG in pediatric hematological/neoplastic diseases and the prevention and treatment of related adverse reactions.
Child ; China ; Consensus ; Hematologic Neoplasms/drug therapy* ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunoglobulins, Intravenous/therapeutic use*

Consensus ; Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Mucocutaneous Lymph Node Syndrome/therapy*

Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Infant ; Mucocutaneous Lymph Node Syndrome/therapy*

Kawasaki disease, an acute febrile vasculitis of unknown etiology, is usually treated with high doses of immunoglobulin (IVIG) and aspirin. However, 20% of children show persistent or recurrent fever despite IVIG, and coronary artery aneurysm progression. In such cases of resistance to IVIG treatment, repeated IVIG administration or the initiation of steroid therapy, and the use of cyclophosphamide have been reported. We aimed to show in this study that methotrexate (MTX) may be used as a treatment for Kawasaki disease resistant to IVIG treatment. We report the case of a 6-year old boy who was admitted at another hospital with an initial complaint of a fever for 5 days and skin rashes for 3 days. The patients fever persisted despite three courses of IVIG (2 gm/kg, 1 gm/kg, 1 gm/kg, respectively) over a 14-day period. On day 14 of his illness he showed a dilated right coronary artery, and on day 19 dexamethasone, at a daily dose of 0.3 mg/kg, was given but this resulted in defervescence. However, upon stopping the dexamethasone treatment, his fever recurred and he was transferred to our hospital. On days 31 and 38 of his illness, IVIG (400 mg/kg for 5 days, twice) was administered and from day 38 onwards the patient was given dexamethasone (0.6 mg/kg, daily) and MTX (10 mg/BSA, once weekly) whereupon his fever subsided and did not recur. On day 48 dexamethasone was replaced with prednisolone, which was subsequently tapered. The patient is now taking MTX and being observed on an outpatient basis. We report the case of a boy with IV-globulin resistant Kawasaki disease, who after repeated infusions of IVIG and steroid therapy showed fever recruuence, which that subsided after MTX treatment.
Case Report ; Child ; Human ; Immunoglobulins, Intravenous/*therapeutic use ; Male ; Methotrexate/*therapeutic use ; Mucocutaneous Lymph Node Syndrome/complications/*drug therapy

Aspirin ; therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; diagnosis

Kawasaki disease (KD) is one of the common acquired heart diseases in children aged <5 years and is an acute systemic vasculitis. After nearly 60 years of research, intravenous immunoglobulin combined with oral aspirin has become the first-line treatment for the prevention of coronary artery lesion in acute KD; however, there are still controversies over the role and optimal dose of aspirin. The consensus was formulated based on the latest research findings of KD treatment in China and overseas and comprehensive discussion of pediatric experts in China and put forward recommendations on the dose, usage, and course of aspirin treatment in the first-line treatment of KD.
Aspirin/therapeutic use* ; Child ; Consensus ; Coronary Vessels/pathology* ; Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Mucocutaneous Lymph Node Syndrome/pathology*