Application of intravenous immunoglobulin G in children with rheumatic diseases / 中华实用儿科临床杂志

The indications for the application of intravenous immunoglobulin G (IVIG) mainly include the replacement therapy of antibody deficiency diseases and immunomodulatory therapy with a high dose of IVIG.IVIG is extensively used in children with rheumatic diseases due to its immunomodulatory effects.However, most of them are " off-label drugs". In the treatment of pediatric rheumatic diseases, IVIG can benefit Kawasaki disease and primary immune thrombocytopenia, and may be beneficial in treating Sj?gren′s syndrome, dermatomyositis and systemic lupus erythematosus.However, for the patients with Stevens-Johnson syndrome, antineutrophil cyctoplasmic antibody (ANCA) asso-ciated vasculitis and systemic scleroderma, there is not much chance to obtain benefits.In the treatment of rheumatic diseases in children, the application of IVIG needs to be further standardized.

Pyoderma gangrenosum: A clinico-epidemiological study.

Background: Pyoderma gangrenosum is a neutrophilic dermatosis of unknown etiology, with inconstant systemic associations and a variable prognosis. Aims: To study the clinical features and systemic associations of pyoderma gangrenosum and its response to treatment. Methods: All patients diagnosed to have pyoderma gangrenosum at the dermatology department of the Government Medical College, Kozhikode, from January 01, 2005 to December 31, 2014 were included in this prospective study. Results: During the 10-year study period, 61 patients were diagnosed to have pyoderma gangrenosum. A male predilection was noted. The most common clinical type was ulcerative pyoderma gangrenosum (90.2%). More than 60% of patients had lesions confi ned to the legs; 78.7% had a single lesion and 27.9% had systemic associations. Most patients required systemic steroids. Patients with disease resistant to steroid therapy were treated with intravenous immunoglobulin G and split-thickness skin grafts under immunosuppression induced by dexamethasone pulse therapy. All except one patient attained complete disease resolution. Limitations: The main limitation of our study was the small sample size. Conclusions: The male predilection documented by us was contrary to most previous studies. We found split-thickness skin graft to be a useful option in resistant cases. More prospective studies may enable the formulation of better diagnostic criteria for pyoderma gangrenosum and improve its management.

Drug Utilization in Korean Children with Kawasaki Disease

BACKGROUND: Kawasaki disease (KD) is an acute febrile, systemic vasculitis as a leading cause of acquired heart disease in children. Intravenous immunoglobulin G (IVIG) and aspirin are the standard initial therapy in the treatment of acute KD. The purpose of this study was to investigate drug utilization in children with KD, and to compare “IVIG + high-dose aspirin” and “IVIG + moderate-dose aspirin” in preventing cardiac complications. METHODS: We analyzed pediatric patient sample data compiled by the Health Insurance Review & Assessment Service from 2010 to 2015. We identified patients with KD using the KCD-6 code of M30.3. We excluded patients in chronic phase or ≥10 years. We also excluded patients who were diagnosed KD in November or December. Drug utilization pattern were assessed in acute KD patients and 30-day and 60-day cardiac complications were investigated between “IVIG + high-dose aspirin” group and “IVIG + moderate-dose aspirin” group. RESULTS: In acute phase, IVIG was administered to 95.8% patients, and 57.1% patients were prescribed moderate-dose aspirin and 25% patients were with highdose aspirin. Steroid use was rapidly increased from 4.0% in 2010 to 11.3% in 2015. Both 30-day and 60-day cardiac complications occurred less in “IVIG + high-dose aspirin” group compared to “IVIG + moderate-dose aspirin” group, but not statistically significant (0.9% vs 1.8%, p=0.252 for 30-day complication rate; 1.5% vs 2.7%. p=0.073 for 60-day complication rate). CONCLUSION: We were not able to demonstrate which aspirin therapy is superior for preventing cardiac complications in acute KD patients and further research is warranted.

Intravenous immunoglobulin G in women with reproductive failure: The Korean Society for Reproductive Immunology practice guidelines / 대한생식의학회지

The task force of the Korean Society for Reproductive Immunology recommends intravenous immunoglobulin G treatment in women with reproductive failure, including recurrent pregnancy loss and/or repeated implantation failure, who show cellular immune factors such as abnormal natural killer cell levels, natural killer cell cytotoxicity, and/or type 1 T helper immunity.

Toxic epidermal necrolysis associated with deflazacort therapy with nephrotic syndrome

Toxic epidermal necrolysis (TEN) is a drug-related fatal disease. Extensive necrosis of the epidermis can lead to serious complications. This report describes two cases of TEN, associated with deflazacort (DFZ), in two boys, aged 4 years and 14 years, with nephrotic syndrome (NS). The 14-year-old male teenager received DFZ following NS relapse. After 17 days, pruritic papules appeared on the lower extremities. Another case involved a 4-year-old boy receiving DFZ and enalapril. After a 41-day DFZ treatment period, erythematous papules appeared on the palms and soles. Within 3 days, both boys developed widespread skin lesions (>50%) and were admitted to the intensive care unit for resuscitative and supportive treatment. The patients showed improvement after intravenous immunoglobulin-G therapy. Owing to the rapid, fatal course of TEN, clinicians need to be aware of the adverse effects of this drug when treating cases of NS.

Kartagener's syndrome with immunoglobulin G subclass deficiency

Kartagener syndrome is characterized by the triad of situs inversus, bronchiectasis, and chronic paranasal sinusitis. Recurrent sinopulmonary infection, the major determinant for diagnosing immunodeficiency, is the most common clinical manifestation of the disease. A 17-year-old female patient presented with dyspnea, cough, sputum, nasal congestion, and rhinorrhea for more than 5 years. Nasal symptoms and dyspnea had not been controlled by intermittent treatment with mucolytics and antibiotics from primary clinics since 3 months before visiting our clinic. Chest X-ray and computed tomography showed situs inversus, dextrocardia and bronchiectasis. Paranasal sinus series revealed mucosal thickening and haziness on both maxillary sinus. Serum immunoglobulin (Ig) G4 was decreased, but total IgG was within normal range. Under the diagnosis of Kartagener syndrome with IgG4 deficiency, monthly intravenous IgG (IVIG) treatment was performed for 6 months. Her symptoms were well controlled and the frequency of antibiotics use was markedly decreased. We report a patient having the Kartagener syndrome with IgG4 deficiency that was successfully controlled with a 6-month-treatment of IVIG.

Early Prediction of Chronic Childhood Immune Thrombocytopenic Purpura According to the Response of Immunoglobulin Treatment / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is a frequently observed bleeding disorder in children. High dose intravenous immunoglobulin G (IVIG) has been used for the treatment of ITP since 1981, and now several methods of IVIG infusion are used. Since 1983, we have treated ITP patients with short-term and low-dose IVIG according to the individual patient's daily response. This study aimed to evaluate individual patient's response after IVIG for the prediction of chronic ITP.METHODS: We evaluated 259 childhood ITP patients retrospectively who were newly diagnosed at the Department of Pediatrics, Kyungpook National University Hospital from 1983 to 2012. We analyzed the individual response to treatment and current state of disease. We evaluated the time to reach desired platelet counts after treatment of IVIG, relapse rate and diagnosis of chronic ITP. The patients were classified into 2 groups according to the time to reach desired platelet counts (50,000/microL) after daily treatment of IVIG, rapid (1 or 2 doses) and slow responder (more than 3 doses).RESULTS: Among 182 patients followed up over 6 months, 41 patients (22.5%) were eventually diagnosed with chronic ITP. Hundred and two patients (56.7%) belonged to rapid response group, and 17 of them (16.7%) were diagnosed with chronic ITP. Eighty patients (44.4%) belonged to the slow response group, and 24 of them (30%) were diagnosed with chronic ITP, which were higher than the early response group (P=0.033).CONCLUSION: Individual response rate of IVIG treatment could be a useful predictor of chronic ITP, but this finding needs support from further studies.

Early Prediction of Chronic Childhood Immune Thrombocytopenic Purpura According to the Response of Immunoglobulin Treatment / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is a frequently observed bleeding disorder in children. High dose intravenous immunoglobulin G (IVIG) has been used for the treatment of ITP since 1981, and now several methods of IVIG infusion are used. Since 1983, we have treated ITP patients with short-term and low-dose IVIG according to the individual patient's daily response. This study aimed to evaluate individual patient's response after IVIG for the prediction of chronic ITP. METHODS: We evaluated 259 childhood ITP patients retrospectively who were newly diagnosed at the Department of Pediatrics, Kyungpook National University Hospital from 1983 to 2012. We analyzed the individual response to treatment and current state of disease. We evaluated the time to reach desired platelet counts after treatment of IVIG, relapse rate and diagnosis of chronic ITP. The patients were classified into 2 groups according to the time to reach desired platelet counts (50,000/microL) after daily treatment of IVIG, rapid (1 or 2 doses) and slow responder (more than 3 doses). RESULTS: Among 182 patients followed up over 6 months, 41 patients (22.5%) were eventually diagnosed with chronic ITP. Hundred and two patients (56.7%) belonged to rapid response group, and 17 of them (16.7%) were diagnosed with chronic ITP. Eighty patients (44.4%) belonged to the slow response group, and 24 of them (30%) were diagnosed with chronic ITP, which were higher than the early response group (P=0.033). CONCLUSION: Individual response rate of IVIG treatment could be a useful predictor of chronic ITP, but this finding needs support from further studies.

Early Prediction of Chronic Childhood Immune Thrombocytopenic Purpura According to the Response of Immunoglobulin Treatment / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is a frequently observed bleeding disorder in children. High dose intravenous immunoglobulin G (IVIG) has been used for the treatment of ITP since 1981, and now several methods of IVIG infusion are used. Since 1983, we have treated ITP patients with short-term and low-dose IVIG according to the individual patient's daily response. This study aimed to evaluate individual patient's response after IVIG for the prediction of chronic ITP. METHODS: We evaluated 259 childhood ITP patients retrospectively who were newly diagnosed at the Department of Pediatrics, Kyungpook National University Hospital from 1983 to 2012. We analyzed the individual response to treatment and current state of disease. We evaluated the time to reach desired platelet counts after treatment of IVIG, relapse rate and diagnosis of chronic ITP. The patients were classified into 2 groups according to the time to reach desired platelet counts (50,000/microL) after daily treatment of IVIG, rapid (1 or 2 doses) and slow responder (more than 3 doses). RESULTS: Among 182 patients followed up over 6 months, 41 patients (22.5%) were eventually diagnosed with chronic ITP. Hundred and two patients (56.7%) belonged to rapid response group, and 17 of them (16.7%) were diagnosed with chronic ITP. Eighty patients (44.4%) belonged to the slow response group, and 24 of them (30%) were diagnosed with chronic ITP, which were higher than the early response group (P=0.033). CONCLUSION: Individual response rate of IVIG treatment could be a useful predictor of chronic ITP, but this finding needs support from further studies.