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Yunhua Tang, Zhiheng Zhang, Maogen Chen, Weiqiang Ju, Dongping Wang, Fei Ji, Qingqi Ren, Zhiyong Guo, Xiaoshun He
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder of unknown pathogenesis. LCH diseases may occur in a single organ or multisystem organ. The patients with multisystem involvement usually have a poor prognosis. Liver involvement in multisystem LCH results in severe complications, such as obvious sclerosing cholangitis (SC) with jaundice. METHODS: We reported a 31-year-old man developed severe SC due to multisystem LCH and was successfully treated by liver transplantation (LT)...
March 2017: Medicine (Baltimore)
Melinda Zsuzsanna Szabó, Emese Kiss
Giant cell arteritis and Takayasu arteritis classified to large vessel vasculitides have similar histopathology in the vascular wall proposing that these entities can be different phenotypes on a spectrum of a single disorder. Glucocorticoids are the mainstay of therapy combined with cyclophosphamide, azatioprine and mycofenolate mofetil, when it is required. However, a significant proportion of patients are glucocorticoid-dependent despite of the conventional disease-modifying antirheumatic drugs and suffer from serious side effects of the steroids, therefore alternate options for more effective disease management are needed...
January 2017: Orvosi Hetilap
Melinda Zsuzsanna Szabó, Emese Kiss
The authors present the latest guideline for the treatment of lupus nephritis and their own single-centre results with mycofenolate mofetil treated lupus nephritis. Lupus nephritis and mainly its proliferative form is a frequent and potentially life-threatening manifestation of systemic lupus erythematosus that can lead to end-stage renal disease. The treatment of lupus nephritis greatly improved in the last decades; mycofenolate mofetil has become an alternative of cyclophosphamide both in remission induction and as a maintenance regimen as well in the treatment of Class III and IV glomerulonephritis...
August 2016: Orvosi Hetilap
M Smith, A Gonzalez-Estrada, J Fernandez, A Subramanian
The use of MMF has become standard practice in many solid organ transplant recipients due its efficacy and favorable risk profile compared to other immunosuppressants. There has been a single case report of successful MMF desensitization. However, this protocol did not follow current Drug practice parameters. We report a successful desensitization to MMF in a double heart-kidney transplant recipient.
July 2016: European Annals of Allergy and Clinical Immunology
M Guarella, A-L Jurquet, K Retornaz, N Bardin, M-C Chastang, M Desjonquere, N Fabien, A Belot
Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children. Its diagnosis is usually made on a clinical basis following the criteria of Bohan and Peter (1975). Recently, the presence of myositis-specific autoantibodies (MSAs) have started to be associated with specific outcome in adult patients; the diagnosis and prognosis value of these autoantibodies remains to be identified in children. We report four cases of JDM with MSAs focusing on clinical, biological, and radiological manifestations, and then we describe associated treatment...
December 2015: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Tasnim F Imran, Frederick Yick, Suneet Verma, Christopher Estiverne, Chinonye Ogbonnaya-Odor, Srikanth Thiruvarudsothy, Alluru S Reddi, Neil Kothari
Lupus nephritis (LN) is an inflammatory condition of the kidneys that encompasses various patterns of renal disease including glomerular and tubulointerstitial pathology. It is a major predictor of poor prognosis in patients with systemic lupus erythematosus (SLE). Genetic factors, including several predisposing loci, and environmental factors, such as EBV and ultraviolet light, have been implicated in the pathogenesis. It carries a high morbidity and mortality if left untreated. Renal biopsy findings are utilized to guide treatment...
February 2016: Clinical and Experimental Nephrology
Giovanna Vitaliti, Omidreza Tabatabaie, Nassim Matin, Caterina Ledda, Piero Pavone, Riccardo Lubrano, Agostino Serra, Paola Di Mauro, Salvatore Cocuzza, Raffaele Falsaperla
Immunotherapeutic strategies to treat neurodegenerative disorders have inspired the scientific community. The aim of our review is to address the translational aspects of neuroimmunology to describe the efficacy of immunotherapy in the treatment of pediatric neurodegenerative disorders. In the studies we analyzed IVIG were found to be efficient in the treatment of post-streptococcal neurodegenerative disorders, even if in PANDAS, plasma-exchange (PE) showed a higher efficiency. IVIG were also successfully used in ADEM and Guillan-Barré syndrome...
2015: Human Vaccines & Immunotherapeutics
W Arcese, A Picardi, S Santarone, G De Angelis, R Cerretti, L Cudillo, E Pennese, P Bavaro, P Olioso, T Dentamaro, L Cupelli, A Chierichini, A Ferrari, A Mengarelli, M C Tirindelli, M Testi, F Di Piazza, P Di Bartolomeo
Ninety-seven patients affected by high-risk hematological malignancies underwent G-CSF primed, unmanipulated bone marrow (BM) transplantation from a related, haploidentical donor. All patients were prepared with an identical conditioning regimen including Thiotepa, Busilvex, Fludarabine (TBF) and antithymocyte globulin given at myeloablative (MAC = 68) or reduced (reduced intensity conditioning (RIC) = 29) dose intensity and received the same GvHD prophylaxis consisting of the combination of methotrexate, cyclosporine, mycofenolate-mofetil and basiliximab...
June 2015: Bone Marrow Transplantation
Andreas A Schnitzbauer, Johannes Sothmann, Lucia Baier, Thomas Bein, Edward K Geissler, Marcus N Scherer, Hans J Schlitt
BACKGROUND: Renal impairment and high model of end-stage liver disease scores before liver transplantation (LT) are increasingly common. PATIENTS AND METHODS: This was a single-arm, 2-step prospective trial of bottom-up calcineurin inhibitor (CNI)-free de novo immunosuppressive treatment (mycofenolate mofetil, steroids, basiliximab) with delayed introduction of sirolimus in patients with renal impairment. Primary endpoint was immunologic safety assessed by the incidence of steroid-resistant rejection within the first 30 days after liver transplantation...
December 2015: Transplantation
Rujikan Ausavarungnirun, Chutika Srisuttiyakorn
We present a 45-years-old suspected systemic lupus erythematosus (SLE) woman who had papulonodular mucinosis (PNM), without other cutaneous LE lesion. The lesions of PNM distributed on both legs which were an unusual location. In addition, the renal involvement was found and suspected from lupus nephritis. The patient was treated with prednisolone, mycofenolate mofetil and chloroquine. After 2 months of follow-up, the renal involvement was improved along with the disappearance ofskin lesions.
February 2014: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
Yo Ueda, Yuko Takahashi, Hiroyuki Yamashita, Tosikazu Kano, Akio Mimori
We report a 33-year-old female who developed a movement disorder during maintenance therapy for systemic lupus erythematosus (SLE). She was diagnosed with SLE at the age of 25, and experienced an episode of SLE-associated hemophagocytic syndrome at age 27, which was successfully treated with intensive immunosuppressive therapy. In November 2012, during maintenance therapy with prednisolone (PSL) 5 mg/day and tacrolimus 0.5 mg/day, she developed acute-onset involuntary movements that were classified as chorea in combination with athetosis in her right limbs and right homonymous hemianopia, which subsided after about 1 h...
2013: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
Joanna Sułowicz, Anna Wojas-Pelc, Ewa Ignacak, Alina Betkowska-Prokop, Marek Kuźniewski, Władysław Sułowicz
UNLABELLED: Due to graft preserving immunosuppressive therapy, renal transplant recipients are predisposed to the development of a variety of skin infections and skin cancers. The aim of the study was to compare prevalence frequency of infective skin changes among patients after kidney transplantation and the dialyzed population. Clinical dermatological examination was performed in 486 patients after renal transplantation. The group consisted of 296 men (60.9%) and 190 women (30.1%), of mean age 46...
2013: Przegla̧d Lekarski
Takahiko Kurasawa, Hayato Nagasawa, Eiko Nishi, Hirofumi Takei, Ayumi Okuyama, Tsuneo Kondo, Koji Nishimura, Ryota Sakai, Akiko Shibata, Kentaro Chino, Hiroe Ogawa, Tatsuya Ito, Koichi Amano, Hitoshi Kato
A substantial number of patients with lupus nephritis (LN) are refractory to conventional glucocorticoid (GC) treatment. Although many of these patients respond to immunosuppressive drugs such as intravenous cyclophosphamide (IVCY), azathioprine (AZA), mizoribine, tacrolimus, cyclosporine A (CSA) and mycofenolate mofetil (MMF), some remain refractory to such therapies. Recent studies of multi-target therapies have reported effective outcomes for immunosuppression following renal transplantation and refractory LN when therapy consists of two or more immunosuppressive drugs with different mechanisms of action...
2013: Internal Medicine
T Miyazaki, T Tagawa, N Yamasaki, T Tsuchiya, K Matsumoto, T Nagayasu
BACKGROUND: Lung transplantation cases have immunosuppression maintained using a calcineurin inhibitor, anti-metabolites, and steroid. CASE REPORT: We report 2 clinical cases in which anti-metabolites (mycophenolate mofetil) were successfully withdrawn after living donor lobar lung transplantation by monitoring immune function using the ImmuKnow® assay. In the first case, a 43-year-old woman underwent living donor lobar lung transplantation for pulmonary alveolar proteinosis...
January 2013: Transplantation Proceedings
E I Brokalaki, N Voshege, O Witzke, A Kribben, D Schadendorf, U Hillen
Severe psoriasis is a rare condition under immunosuppressive therapy. We describe a 42-years-old man with psoriasis since the age of 22 years. The patient underwent a combined pancreas-kidney transplantation at the age of 32 because of Goodpasture syndrome with renal and pulmonary involvments and type 1 diabetes mellitus. Seven years later a pancreas retransplantation was performed due to nonfunction of the original pancreas allograft. Despite intensive systemic immunosuppression, consisting of prednisone, tacrolimus, and mycofenolate mofetil, and topical treatment with dithranol and steroids, there was significant worsening of psoriasis...
November 2012: Transplantation Proceedings
Elizaveta M Orlova, Maria A Kareva, Maria A Melikyan, Elena Boyakova, Valentina A Peterkova, Alexey A Maschan
A 26-year-old female with the classic major and minor components of autoimmune polyglandular syndrome type 1 was diagnosed as having pure red cell aplasia. Treatment with 1.5 g/d mycofenolate mofetil for 3 months failed to restore erythroid production. Treatment with cyclosporine A produced a good partial response but led to renal toxicity and was therefore substituted with cyclophosphamide, which had a good partial effect and lasted for 18 months. The relapse of anemia was not observed during the 6-month follow-up period after the cessation of treatment...
November 2013: Journal of Pediatric Hematology/oncology
Jose Antonio Perez-Simón, Rodrigo Martino, Rocío Parody, Mónica Cabrero, Lucía Lopez-Corral, David Valcarcel, Carmen Martinez, Carlos Solano, Lourdes Vazquez, Francisco J Márquez-Malaver, Jordi Sierra, Dolores Caballero
Different types of graft-versus-host disease prophylaxis have been proposed in the setting of reduced intensity and non-myeloablative allogeneic stem cell transplantation. An alternative combination with sirolimus and tacrolimus has recently been tested although comparative studies against the classical combination of a calcineurin inhibitor and mycophenolate mofetil or methotrexate are lacking. We describe the results of a prospective, multicenter trial using sirolimus + tacrolimus as immunoprophylaxis, and compare this approach with our previous experience using cyclosporine + mycophenolate in the setting of unrelated donor transplantation setting after reduced-intensity conditioning...
April 2013: Haematologica
Aruna V Vanikar, Hargovind L Trivedi
BACKGROUND: We present our experience of living-donor renal transplantation (LDRT) using pretransplant stem cell transplantation (SCT) where we have successfully achieved minimization of immunosuppression. METHODS: Nine hundred sixteen patients underwent LDRT between 2007 and 2011: 606 under tolerance induction protocol (TIP) and 310 with our usual triple immunosuppression of calcineurin inhibitors (CNI), mycofenolate sodium (MMF), and prednisone (controls). The test group (TIP) was stratified into group 1, 1 haplomatch or greater (n=392), group 3, less than 1 haplomatch (n=214); controls were similarly stratified to group 2, 1 haplomatch or greater (n=179) and group 4, less than 1 haplomatch (n=131)...
October 27, 2012: Transplantation
N Zbiti, K Souly, Z Errami, L Guendouz, L Benamar, F Ezaitouni, N Ouzeddoun, R Bayahia, A Chabraoui, H Rhou
To determine the prevalence of post-kidney transplantation diabetes (PTDM) and to assess its risk factors, we retrospectively studied 92 non-diabetic kidney transplant patients. The immunosuppressive drugs used to prevent rejection included prednisone, a calcineurin inhibitor (cyclosporine or tacrolimus) and an antimetabolite (azathioprine or mycofenolate mofetil). Diabetes was defined according to the WHO criteria and the American Diabetes Association. The mean age of our patients was 35.8 ± 10.5 years, and there was a clear male predominance (56 men and 36 women)...
September 2012: Saudi Journal of Kidney Diseases and Transplantation
Taxiarchis V Kourelis, Adam Boruchov, David Hull, John Polio, John Scholes, Michael Moustakakis, Syed F Bilgrami
Organ transplant recipients are at an increased risk for subsequent malignancies including hematologic malignancies. The development of acute myeloid leukemia (AML) after solid organ transplantation is a rare but well-documented event. It is thought to be a consequence of immune dysregulation secondary to the use of immunosuppressive agents. Herein, we present the management of a liver transplantation recipient who presented with AML and comprehensively review the relevant literature. A 59-year-old male patient presented with fever and cough eight years after an orthotopic liver transplantation for cirrhosis and hepatocellular carcinoma...
March 2012: Connecticut Medicine
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