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https://www.readbyqxmd.com/read/28730667/role-of-plasma-exchange-leukocytapheresis-and-plasma-diafiltration-in-management-of-refractory-macrophage-activation-syndrome
#1
Noriko Kinjo, Kazuya Hamada, Chika Hirayama, Masaki Shimizu
Macrophage activation syndrome (MAS) is a life-threating complication of systemic juvenile idiopathic arthritis (s-JIA). Steroid and cyclosporine (CsA) are effective for MAS, but, treatment for steroid- and CsA-resistant patients is still challenging. We report the case of steroid and CsA resistant s-JIA associated MAS misdiagnosed as Kawasaki disease (KD), who was successfully treated with the combination of plasma exchange (PE) and leukocytapheresis (LCAP) followed by plasma diafiltration (PDF). PE + LCAP effectively removed proinflammatory cytokines and reduced the number of peripheral white blood cells...
July 21, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28640392/calcineurin-inhibitors-exacerbate-coronary-arteritis-via-the-myd88-signaling-pathway-in-a-murine-model-of-kawasaki-disease
#2
Kenji Murata, Yoshitomo Motomura, Tamami Tanaka, Shunsuke Kanno, Takahisa Yano, Mitsuho Onimaru, Atsushi Shimoyama, Hisanori Nishio, Yasunari Sakai, Masatsugu Oh-Hora, Hiromitsu Hara, Koichi Fukase, Hidetoshi Takada, Satohiro Masuda, Shouichi Ohga, Sho Yamasaki, Toshiro Hara
Calcineurin inhibitors (CNIs) have been used off-label for the treatment of refractory Kawasaki disease (KD). However, it remains unknown whether CNIs show protective effects against the development of coronary artery lesions in KD patients. To investigate the effects of CNIs on coronary arteries and the mechanisms of their actions on coronary arteritis in a mouse model of KD, we performed experiments with FK565, a ligand of nucleotide-binding oligomerization domain-containing protein 1 (Nod1) in wild-type, SCID, CARD9(-/-) , and MyD88(-/-) mice...
June 22, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28588173/macrophage-activation-syndrome-in-paediatric-rheumatic-diseases
#3
M I Islam, M K Talukder, M M Islam, K Laila, S A Rahman
Macrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic disorders, which commonly occurs in systemic juvenile idiopathic arthritis (sJIA).This study was carried out with the aims of describing the clinical features, laboratory findings and outcomes of MAS associated with paediatric rheumatic diseases in the Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU) and compare these results with previous studies on MAS. This retrospective study was conducted in the paediatric rheumatology wing of the Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28580631/kawasaki-disease-medical-therapies
#4
Jane W Newburger
Medical therapies in patients with Kawasaki disease (KD) are administered to reduce the prevalence of coronary aneurysms, reduce systemic inflammation, and prevent coronary thrombosis. All patients with acute KD should be treated with intravenous immunoglobulin (IVIG) 2 g/kg, generally administered over 10-12 hours. Aspirin has never been shown to prevent aneurysms, but is given for its anti-inflammatory and antipyretic effects until the patient has been afebrile for ∼2 days, then lowered to an antiplatelet dose...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28562511/kawasaki-disease-complicated-with-macrophage-activation-syndrome-a-systematic-review
#5
Susana García-Pavón, Marco A Yamazaki-Nakashimada, Milton Báez, Karla L Borjas-Aguilar, Chiharu Murata
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD). We report 2 cases, performed a literature search, and analyze the characteristics of MAS associated with KD. A total of 69 patients were evaluated, 34 reported the date of the diagnosis of MAS and KD, 6% had a diagnosis of MAS before KD, 21% had a simultaneous presentation, and 73% had the diagnosis of MAS after KD. Different treatment approaches were observed with corticosteroids administered in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and monoclonal anti-TNF in 6% of cases...
August 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28508981/partial-remission-by-cyclosporine-monotherapy-in-a-patient-with-membranous-nephropathy-superimposed-diabetic-nephropathy
#6
Yoshiyuki Oshiro, Hisataka Tanaka, Fumiko Kawasaki, Niro Okimoto
It has been noted that cyclosporine A (CsA) is an effective drug for membranous nephropathy (MN). Diabetes is a common disease that sometimes causes nephrotic syndrome. We report the case of an 89-year-old woman with type 2 diabetes mellitus who exhibited nephrotic syndrome. Examination of a renal biopsy indicated MN and she was prescribed CsA as monotherapy. Her edema subsided and she achieved partial remission. This is the first report of a patient in diabetic condition with MN having achieved partial remission after CsA monotherapy without steroid therapy...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/27681743/the-clinical-diagnosis-and-management-of-kawasaki-disease-a-review-and-update
#7
REVIEW
Frank H Zhu, Jocelyn Y Ang
Kawasaki disease is an acute, self-limited vasculitis of childhood and has become the leading cause of acquired pediatric heart disease in the USA. Prompt treatment is essential in reducing cardiac-related morbidity and mortality. The underlying etiology remains unknown. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in susceptible hosts. The characteristic clinical features of fever for at least 5 days with bilateral nonpurulent conjunctivitis, rash, changes in lips and oral cavity, changes in peripheral extremities, and cervical lymphadenopathy remain the mainstay of diagnosis...
September 2016: Current Infectious Disease Reports
https://www.readbyqxmd.com/read/27338268/efficacy-of-combination-therapy-with-anti-thymocyte-globulin-and-cyclosporine-a-as-a-first-line-treatment-in-adult-patients-with-aplastic-anemia-a-comparison-of-rabbit-and-horse-formulations
#8
COMPARATIVE STUDY
Takahiro Suzuki, Hiroyuki Kobayashi, Yasufumi Kawasaki, Kiyoshi Okazuka, Kaoru Hatano, Shin-Ichiro Fujiwara, Iekuni Oh, Ken Ohmine, Yoshinobu Kanda
Anti-thymocyte globulin (ATG) is a key drug in immunosuppressive therapy for patients with aplastic anemia. The mainstay of ATG therapy had been a horse ATG (hATG) formulation, Lymphoglobulin or ATGAM, but Lymphoglobulin was recently discontinued, and Thymoglobulin, a rabbit ATG (rATG) formulation, is currently used as the first-line drug in many countries, including Japan. However, a recent randomized clinical trial reported significantly unfavorable outcomes associated with the use of rATG regimens. We retrospectively analyzed clinical outcomes of adult patients with moderate to severe aplastic anemia who were treated with 3...
October 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/26670024/adjunct-cyclosporine-therapy-for-refractory-kawasaki-disease-in-a-very-young-infant
#9
Seigo Okada, Yoshihiro Azuma, Yasuo Suzuki, Hiroko Yamada, Midori Wakabayashi-Takahara, Yuno Korenaga, Hideaki Akase, Shunji Hasegawa, Shouichi Ohga
Herein we describe the case of a 6-week-old boy who developed complete Kawasaki disease (KD). The cytokine profile and activation of monocytes and subsequent T cells matched the typical feature of refractory KD. The patient received a total of three courses of i.v. immunoglobulin (IVIG), but did not achieve clinical relief. Adjunctive therapy with oral cyclosporine A (CsA) led to prompt defervescence. This was continued for 7 days without serious adverse events. Coronary artery dilatations regressed within 3 months of follow up...
April 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/26628527/study-protocol-for-a-phase-iii-multicentre-randomised-open-label-blinded-end-point-trial-to-evaluate-the-efficacy-and-safety-of-immunoglobulin-plus-cyclosporin-a-in-patients-with-severe-kawasaki-disease-kaica-trial
#10
RANDOMIZED CONTROLLED TRIAL
Reiko Aoyagi, Hiromichi Hamada, Yasunori Sato, Hiroyuki Suzuki, Yoshihiro Onouchi, Ryota Ebata, Kengo Nagashima, Moe Terauchi, Masaru Terai, Hideki Hanaoka, Akira Hata
INTRODUCTION: Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown aetiology that predominantly affects infants and young children. We hypothesise that cyclosporin A (CsA) may be effective in treating KD by regulating the Ca(2+)/NFAT signalling pathway. This trial compares the current standard therapy of intravenous immunoglobulin (IVIG) and the combined IVIG+CsA therapy in paediatric patients with severe KD. METHODS AND ANALYSIS: This trial is a phase III, multicentre, randomised, open-label, blinded-end point trial that evaluates the efficacy and safety of IVIG+CsA therapy...
2015: BMJ Open
https://www.readbyqxmd.com/read/26547265/kawasaki-disease-a-comprehensive-review-of-treatment-options
#11
REVIEW
Rupal M Patel, Stanford T Shulman
WHAT IS KNOWN AND OBJECTIVE: Kawasaki disease (KD) is an acute self-limiting systemic vasculitis with specific predilection for the coronary arteries that affects previously healthy young infants and children. It is the leading cause of childhood-acquired heart disease in the developed world. Although the stimulus for the cascade of inflammation in KD is unknown, prompt treatment within 10 days of symptom onset has been shown to improve clinical outcomes and reduce the risk of coronary artery complications...
December 2015: Journal of Clinical Pharmacy and Therapeutics
https://www.readbyqxmd.com/read/26541649/management-of-tacrolimus-associated-food-allergy-after-liver-transplantation
#12
Naho Obayashi, Mitsuyoshi Suzuki, Tomoaki Yokokura, Nakayuki Naritaka, Satoshi Nakano, Yoshikazu Ohtsuka, Hiroyuki Sugo, Seiji Kawasaki, Toshiaki Shimizu
Increasingly, food allergy associated with tacrolimus after pediatric living-donor liver transplantation (LT) has been reported. Tacrolimus prevents the activation of T cells by blocking calcineurin, thus producing an immunosuppressive effect, but tacrolimus induces an imbalance in T-helper type 1 (Th1) and Th2 cells in the food allergy process. This report describes a case of tacrolimus-associated food allergy after pediatric living-donor LT. The patient was a 7-year-old Japanese girl who had undergone living-donor LT at 12 months of age, and whom we first saw in the clinic at age 18 months...
December 2015: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/26318177/recent-advances-in-kawasaki-disease-proceedings-of-the-3rd-kawasaki-disease-summit-chandigarh-2014
#13
REVIEW
Surjit Singh, Dhrubajyoti Sharma, Sagar Bhattad, Saji Phillip
Kawasaki disease (KD) is the most common cause of acquired heart disease in children in Japan, North America and Europe. It is now being increasingly recognized from the developing countries as well. If not diagnosed and treated in time, KD can result in coronary artery abnormalities in approximately 15-25% cases. The long-term consequences of these abnormalities may manifest in adults as myocardial ischemia and congestive heart failure. Intravenous immunoglobulin (IVIg) remains the drug of choice for treatment of KD, but several new agents like infliximab, cyclosporine, glucocorticoids and statins are now being increasingly used in these patients...
January 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/26275234/rituximab-and-low-dose-cyclosporine-combination-therapy-for-steroid-resistant-focal-segmental-glomerulosclerosis
#14
Kazuhide Suyama, Yukihiko Kawasaki, Kyohei Miyazaki, Syuto Kanno, Atsushi Ono, Yuichi Suzuki, Shinichiro Ohara, Mitsuaki Hosoya
BACKGROUND: We investigated the efficacy of rituximab and low-dose cyclosporine combination therapy for focal segmental glomerulosclerosis (FSGS) in children with steroid-resistant nephrotic syndrome (SRNS). METHODS: Five FSGS children with SRNS were treated twice with rituximab and low-dose cyclosporine (CyA) combination therapy (RTX-CyAT). The clinical features and laboratory data were investigated before and after RTX-CyAT, and the outcomes were assessed. RESULTS: Prednisolone (PSL) was discontinued 3 months after RTX-CyAT in all patients...
March 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/25615939/incidence-and-prognosis-of-systemic-lupus-erythematosus-in-a-35-year-period-in-fukushima-japan
#15
MULTICENTER STUDY
Yukihiko Kawasaki, Shinichiro Ohara, Kyohei Miyazaki, Shuto Kanno, Atsushi Ono, Kazuhide Suyama, Masatoki Sato, Shigeo Suzuki, Ryo Maeda, Katsutoshi Nagasawa, Mitsuaki Hosoya
BACKGROUND: We examined the epidemiology, clinical manifestations, and prognosis of pediatric systemic lupus erythematosus (SLE) in Fukushima Prefecture, Japan over a 35 year period. METHODS: We collected the medical records of 37 patients diagnosed with SLE between 1977 and 2013. These children were divided into two groups. group 1 consisted of 19 patients who were diagnosed between 1977 and 1995, and group 2 consisted of 18 patients diagnosed between 1996 and 2013...
August 2015: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/25518415/-cyclosporin-a-treatment-for-refractory-kawasaki-disease
#16
Hiroyuki Suzuki, Tomohiro Suenaga, Nobuyuki Kakimoto, Takashi Takeuchi, Shoichi Shibuta
The association between functional polymorphism of inositol 1,4,5-trisphosphate 3- kinase-C(ITPKC) and susceptibility to Kawasaki disease(KD) and formation of coronary arterial lesions was reported in 2008. Since ITPKC acts as a negative regulator of T-cell activation, activated T cells may play a pivotal role in the pathogenesis of KD. Cyclosporin A(CsA), which potently suppresses the activity of T cells through negative regulation of the nuclear factor of activated T cells(NFAT) pathway, may be a promising candidate for the treatment of refractory KD...
September 2014: Nihon Rinsho. Japanese Journal of Clinical Medicine
https://www.readbyqxmd.com/read/25518397/-present-situation-and-new-challenges-for-medical-treatment-of-kawasaki-disease
#17
REVIEW
Kazuyuki Ikeda, Kenji Hamaoka
To predict resistance to intravenous immunoglobulin (MIG) treatment in patients with severe Kawasaki disease (KD), Gunma, Kurume, and Osaka risk scores were established. Using these scores, prospective randomised trials were performed, and addition of predni- solone or intravenous methylprednisolone -pulse to the standard regimen of IVIG improves coronary artery outcomes in severe KD. Adding the evidences of new and existing therapies, such as infliximab, cyclosporin A, ulinastatin, and plasma exchange, the clinical guideline for medical treatment of the acute phase of KD was revised in 2012...
September 2014: Nihon Rinsho. Japanese Journal of Clinical Medicine
https://www.readbyqxmd.com/read/24500387/efficacy-of-cyclosporine-a-for-steroid-resistant-severe-henoch-sch%C3%A3-nlein-purpura-nephritis
#18
Shinichiro Ohara, Yukihiko Kawasaki, Kyohei Miyazaki, Atsushi Ono, Yuichi Suzuki, Kazuhide Suyama, Mitsuaki Hosoya
Aggressive treatment is necessary for continuous high-range proteinuria in cases of pediatric Henoch-Schönlein purpura nephritis (HSPN) as the long-term prognosis is sometimes poor. Cyclosporine (CyA) has immunosuppressive effects as well as a very selective inhibitory effect on T-helper cell function. Here we report two 7-year-old boys with steroid-resistant HSPN treated with CyA. After diagnosis of HSPN, we treated both patients with methylprednisolone and urokinase pulse therapy (MUT) combined with multiple drugs; however, high-range proteinuria persisted and CyA was added to the treatment regimen...
2013: Fukushima Journal of Medical Science
https://www.readbyqxmd.com/read/23606968/etanercept-suppresses-arteritis-in-a-murine-model-of-kawasaki-disease-a-comparative-study-involving-different-biological-agents
#19
Ryuji Ohashi, Ryuji Fukazawa, Makoto Watanabe, Hanako Tajima, Noriko Nagi-Miura, Naohito Ohno, Shinichi Tsuchiya, Yuh Fukuda, Shunichi Ogawa, Yasuhiko Itoh
Coronary arteritis, a complication of Kawasaki disease (KD), can be refractory to immunoglobulin (IVIG) treatment. To determine the most effective alternative therapy, we compared the efficacy of different agents in a mouse model of KD. Vasculitis was induced by injection of Candida albicans water-soluble fractions (CAWS) into a DBA/2 mouse, followed by administration of IVIG, etanercept, methylprednisolone (MP), and cyclosporine-A (CsA). At 2 and 4 weeks, the mice were sacrificed, and plasma cytokines and chemokines were measured...
2013: International Journal of Vascular Medicine
https://www.readbyqxmd.com/read/23199405/management-of-acute-and-refractory-kawasaki-disease
#20
REVIEW
Carline E Tacke, David Burgner, Irene M Kuipers, Taco W Kuijpers
Acute Kawasaki disease (KD) is treated with high-dose intravenous immunoglobulin (IVIG), which is proven to decrease the incidence of coronary artery aneurysms from 25% to less than 5%. Aspirin is also given, although the evidence base is less secure. There is increasing evidence for steroid therapy as adjunctive primary therapy with IVIG, especially in Asian children. Approximately 10-30% of patients fail to respond to the initial IVIG and are at increased risk of coronary artery aneurysms. The optimal treatment for IVIG-nonresponsive KD remains controversial...
October 2012: Expert Review of Anti-infective Therapy
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