No abstract available.
Immunoglobulins ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome

Toxic epidermal necrolysis (TEN) is a rare, life-threatening, mucocutaneous drug reaction, which causes extensive epidermal detachment and serious complications involving ocular structures and internal organs. Recently, intravenous immunoglobulin (IVIG) was suggested to be effective in treating TEN through the blockage of Fas receptors which initiate keratinocyte apoptosis. Herein, we tried IVIG teratment (0.6 g/kg/day for 4 consecutive days) for a case of TEN. As a result, the progression of epidermal detachment was interrupted within 2 days and epithelialization was completed in 3 weeks without significant side effects.
Antigens, CD95 ; Apoptosis ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Keratinocytes ; Stevens-Johnson Syndrome*

Toxic epidermal necrolysis (TEN) is a rare, life-threatening, mucocutaneous drug reaction, which causes extensive epidermal detachment and serious complications involving ocular structures and internal organs. Recently, intravenous immunoglobulin (IVIG) was suggested to be effective in treating TEN through the blockage of Fas receptors which initiate keratinocyte apoptosis. Herein, we tried IVIG teratment (0.6 g/kg/day for 4 consecutive days) for a case of TEN. As a result, the progression of epidermal detachment was interrupted within 2 days and epithelialization was completed in 3 weeks without significant side effects.
Antigens, CD95 ; Apoptosis ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Keratinocytes ; Stevens-Johnson Syndrome*

Intravenous immunoglobulin (IVIG) is used in treating many cases of autoimmune and inflammatory conditions thanks to its multiple anti-inflammatory and immunomodulatory properties. The clinical use of IVIG has been for the patients with primary immunodeficiencies, but lately it is expanding its usage to the realms of treating patients with neurological conditions. Both the efficacy and safety of IVIG treatment in chronic inflammatory demyelinating polyradiculoneuropathy and Guillain–Barré syndrome have been studied successfully. However, the use of IVIG treatment in other neurological conditions still remains investigational despite several successful reports. Considerable numbers of mechanisms have been suggested in order to explain the effects of IVIG, but the exact mechanisms are not understood yet. This review covers the new developments in clinical fields and the possible ways in which IVIG could help in the future.
Humans ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Neurology ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating

Background: Kawasaki disease is a self-limited disease but it can lead to potentially fatal cardiac complications if not detected and managed accordingly. Objective: To determine the incidence of cardiac involvement in patients with Kawasaki disease admitted in PGH Methods: Medical records of patients with KD admitted from January 2012 to December 2013 were reviewed. Demographic, clinical, laboratory, chest radiographic, electrocardiographic, and echocardiographic data were recorded. The course, management, length of hospital stay, clinical outcome, duration of OPD follow-up, and medications were evaluated. Results: Thirty-eight patients with mean age of 2.67 ± 2.26 years old, 66% males with KD were included. Fifty-nine percent had cardiac involvement, and among those with cardiac involvement, 68% have coronary artery dilatation. Seventy-six percent of cases received intravenous immunoglobulin (IVIG) with 55% receiving IVIG within 10 days of illness. The initial cardiac findings resolved in the subsequent 2d-echo after IVIG except for some coronary artery abnormalities, which resolved in 5 ± 3.11 months during follow-up. There was no mortality. The possible predictive factors for the development of cardiac abnormalities published in other studies were not found to be significantly associated in this study population. Conclusion The incidence of cardiac involvement in patients with Kawasaki disease among children admitted in PGH is 59% with68% having coronary artery dilatation, higher than in other published studies.
Mucocutaneous Lymph Node Syndrome ; Immunoglobulins, Intravenous

Polymyositis is one form of inflammatory myopathy. In some patients, this disease does not entirely respond to conventional initial therapy with glucocorticoid, methotrexate and azathioprine. Multiple options exist for treating these patients, but only intravenous immune globulin has been subjected to a randomized clinical trial. We report here on a case of polymyositis that did not respond to multiple drug therapy, but it did respond to tacrolimus. After treatment with tacrolimus, the patient's disease has been well controlled for many years.
Azathioprine ; Humans ; Immunoglobulins, Intravenous ; Methotrexate ; Myositis ; Polymyositis ; Tacrolimus

OBJECTIVE: To investigate the curative effect of simply hormone and combined gamma globulin and thrombopoietin(TPO) on primary immune thrombocytopenia(PITP). METHODS: 100 patients with PITP were divided into simply drug groups, and combined drug group each for 50 cases. The patients in single drug group were given simply hormone therapy, the patients in combined drug group were given gamma globulin and thrombopoietin. The levels of TPO, platelet activating factor (PAF) were detected by DAS-ELISA. The differences of clinical curative effect, clinical indicators, biochemical indexes and adverse reactions between the two groups were compared. RESULTS: The total effective rate of combined drug group (90.00%) was obviously higher than that in single drug group (66.00%)(P<0.05). Amount of platelet infusion in combined drug group was obviously less than that in single drug group, platelet recovery time and effect onset time in combined drug group were significantly shorter than those in single drug group, and the maintaining time in combined drug group was obviously longer than that in single drug group. At the same time, the platelet peak in combined drug group was higher than that in single drug group (P<0.05). The levels of TPO, PAF between the two groups did not show statisticall significant differences before treatment (P>0.05), however, the above-mentioned indexes of two groups after treatment were lower than those before treatment (P<0.05), among them, the indexes in combined drug group were obviously lower ttan those in sigle drug group (P<0.05). The adverse reaction and mortality rate between the two groups did not show statistically significant differences(P>0.05), the recurrence rate in combined drug group(2%) was obviously lower than that in single group(14.00%) (P<0.05). CONCLUSION The curative effect of hormone, as well as gamma globulin combined with TPO to treat PITP are satisfying, can obviously improve the levels of TPO, PAF, and the drug safety is higher. but the efficacy of combined drug is surperior to single drug.
Humans ; Immunoglobulins, Intravenous ; Purpura, Thrombocytopenic, Idiopathic ; Thrombopoietin ; gamma-Globulins

From November, 2010 to February, 2018, 11 children with variants of Guillain-Barré syndrome (GBS) were treated in Xiangya Hospital of Central South University. Clinical manifestations included cranial nerve involvement in 9 cases, limb weakness in 9 cases, ataxia in 8 cases, abnormal sensation in 7 cases, weakened or disappeared tendon reflex in all cases, albuminocytologic dissociation of cerebrospinal fluid in 6 cases, and neurogenic changes in electromyography in 8 cases. Immune globulin and steroids were given to 5 cases and 1 case, respectively, while the combination of immune globulin with steroids was given to 1 case, and symptomatic treatment was given to 4 cases. All treatments were effective, remaining no apparent side effect for nervous system. The diagnosis of GBS variants was based on clinical manifestation. Patients who had cranial nerve involvement, ataxia and weakened or disappeared tendon reflex should be paid attention. Albuminocytologic dissociation of cerebrospinal fluid and electromyography were helpful to diagnosis. GBS variants were self-limited disease. Immunotherapy can ease the patients' critical condition.
Child ; Electromyography ; Guillain-Barre Syndrome ; Humans ; Immunoglobulins, Intravenous ; Immunotherapy

Background: There is limited information available regarding the management of IVIG-refractory Kawasaki Disease (KD). Objective: This study aimed to evaluate the safety and efficacy of a second intravenous immunoglobulin (IVIG) infusion versus intravenous methylprednisolone (IVMP) in patients with IVIG-refractory KD. Methodology: Cochrane Library, PubMed, Medline, Elsevier (Science Direct), Springer Link and BMJ databases were searched from May 1, 2020 to December 31, 2020. We included randomized controlled trials (RCTs) and high-quality prospective and retrospective studies, with population restricted to children 0 months to 18 years, with KD refractory to initial IVIG at 2g/kg, who remained febrile for 24-48 hours after completion of initial IVIG, and who received second-line monotherapy with either a second dose IVIG or IVMP. We conducted a meta-analysis using Review Manager [RevMan] 5.4.1 software. Results: A total of six studies (n=188 patients) were analyzed. The incidence of coronary artery lesions was comparable between a second dose of IVIG and IVMP (RR 0.82, 0.34-1.96, P=0.66) in patients with IVIG-refractory KD. The rate of fever resolution to a second IVIG, compared to IVMP, was not significantly different between groups (RR 0.97, 0.84-1.13, P=0.72). There was a significantly higher incidence of adverse events in the IVMP group (RR 0.42, 0.26-0.57, P=0.0002), but these were all transient and resolved without further treatment. Conclusion There is no significant difference in the incidence of coronary artery lesions and rate of fever resolution post-retreatment with a second dose of IVIG versus IVMP in IVIG-refractory KD. More adverse events were reported in the IVMP group.
Mucocutaneous Lymph Node Syndrome ; Immunosuppressive Agents ; Immunoglobulins, Intravenous ; Methylprednisolone

PURPOSE: Patients with Kawasaki disease (KD) who remain febrile after first dose of intravenous immunoglobulin (IVIG) are usually treated with a second dose of IVIG. However, the optimal dosage of IVIG in the retreatment of KD is still controversial. We evaluated the impact of IVIG dose difference on the coronary artery lesion (CAL) in the retreatment of KD. METHODS: We performed a prospective study on 24 patients with KD requiring IVIG retreatment from January 2000 to December 2004. The patients were assigned to receive IVIG in the retreatment of KD either as a single infusion of 2 g/kg (A group, 12 patients) or 1 g/kg (B group, 24 patients). We compared the CAL of B group to that of A group for comparable risk of both groups. RESULTS: The incidence of CAL was significantly higher in the 5 of 24 (20.9%) patients with IVIG retreatment vs the 18 of 315 (5.8%) afebrile patients after first course of IVIG treatment (P=0.016). There were no significant differences in age, sex, and other clinical and laboratory findings known as risk factors of CAL between the two groups. Among 24 patients with IVIG retreatment, there were no significant difference in the incidence of CAL between the two groups(Group A, 3/12=25%, vs Group B, 2/12=16.7%) (P>0.05). CONCLUSION: Single 1 g/kg IVIG therapy is comparable outcome to single 2 g/kg IVIG therapy in the retreatment of KD, although the efficacy of this practice requires assessment with a randomized controlled prospective trial in more patients.
Coronary Vessels ; Humans ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Incidence ; Mucocutaneous Lymph Node Syndrome* ; Prospective Studies ; Retreatment* ; Risk Factors