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kawasaki cyclosporine

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
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
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
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
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
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
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
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
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
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
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
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
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
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
Hiromichi Hamada, Hiroyuki Suzuki, Jun Abe, Yoichi Suzuki, Tomohiro Suenaga, Takashi Takeuchi, Norishige Yoshikawa, Shoichi Shibuta, Masakazu Miyawaki, Ko Oishi, Hironobu Yamaga, Noriyuki Aoyagi, Seiji Iwahashi, Ritsuko Miyashita, Takafumi Honda, Yoshihiro Onouchi, Masaru Terai, Akira Hata
BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis occurring in medium-sized arteries, especially coronary arteries. Patients with KD who fail to respond to standard therapy with intravenous immunoglobulin (IVIG) face a higher risk of developing coronary artery lesions. Cyclosporin A (CsA) is one treatment option for IVIG-resistant KD. However, the mechanism of its suppression of inflammation in patients with KD remains unknown. METHODS AND RESULTS: We analyzed time-line profiles of multiple inflammatory cytokines in sera of 19 patients treated with CsA (4 mg/kg/day, p...
December 2012: Cytokine
F Bajolle, D Laux
Kawasaki disease (KD) is an acute systemic vasculitis syndrome occurring mostly in children younger than 5 years of age. Especially young infants (<1 year) have an increased risk of coronary artery lesions (CAL). Whereas the etiology of KD is still unknown, progress in treatment during its acute phase has decreased the incidence of CAL from 25-30% to 3-5%. In "atypical KD", the clinical picture is dominated by an unusual symptom as seizure, bloody diarrhea, compressive cervical adenopathy, nephrotic syndrome or hyponatremia...
November 2012: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Adriana H Tremoulet, Paige Pancoast, Alessandra Franco, Matthew Bujold, Chisato Shimizu, Yoshihiro Onouchi, Alyson Tamamoto, Guliz Erdem, Debra Dodd, Jane C Burns
OBJECTIVE: To describe the clinical course and outcome of 10 patients with Kawasaki disease (KD) treated with a calcineurin inhibitor after failing to respond to multiple therapies. STUDY DESIGN: Demographic and clinical data were prospectively collected using standardized case report forms. T-cell phenotypes were determined by flow cytometry, and KD risk alleles in ITPKC (rs28493229), CASP3 (rs72689236), and FCGR2A (rs1801274) were genotyped. RESULTS: Intravenous followed by oral therapy with cyclosporine (CSA) or oral tacrolimus was well tolerated and resulted in defervescence and resolution of inflammation in all 10 patients...
September 2012: Journal of Pediatrics
Hiroyuki Suzuki, Masaru Terai, Hiromichi Hamada, Takafumi Honda, Tomohiro Suenaga, Takashi Takeuchi, Norishige Yoshikawa, Shoichi Shibuta, Masakazu Miyawaki, Ko Oishi, Hironobu Yamaga, Noriyuki Aoyagi, Seiji Iwahashi, Ritsuko Miyashita, Yoshihiro Onouchi, Kumiko Sasago, Yoichi Suzuki, Akira Hata
BACKGROUND: There are still no definite treatments for refractory Kawasaki disease (KD). In this pilot study, we evaluated the use of cyclosporin A (CyA) treatment in patients with refractory KD. METHODS: We prospectively collected clinical data of CyA treatment (4-8 mg/kg/d, oral administration) for refractory KD patients using the same protocol among several hospitals. Refractory KD is defined as the persistence or recurrence of fever (37.5°C or more of an axillary temperature) at the end of the second intravenous immunoglobulin (2 g/kg) following the initial one...
October 2011: Pediatric Infectious Disease Journal
Mitsuhito Ota, Hiroyuki Kawasaki, Masashi Horimoto
No abstract text is available yet for this article.
December 2010: American Journal of Medicine
N Sakai, Y Kawasaki, T Imaizumi, S Kanno, H Go, M Mitomo, Y Ushijima, K Suyama, M Ito, K Hashimoto, M Hosoya
Reversible posterior leukoencephalopathy syndrome (RPLS) is a distinctive clinicoradiological entity observed in a variety of clinical settings. Cyclosporine (CyA)-RPLS has been reported in a few patients with focal segmental glomerulosclerosis (FSGS); however, there had been no reports on developed RPLS after the re-administration of CyA treatment. We report two patients with FSGS who developed CyA-induced RPLS and summarize the results of a literature review for similar patients. The two patients with FSGS presented here were a 4-year-old boy and a 9-year-old boy, who presented with steroid-resistant nephrotic syndrome (NS) and were treated with CyA...
June 2010: Clinical Nephrology
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