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Mishra Om P OP Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India - - 2014
Serum IgE and IL-13 levels were estimated in 40 idiopathic nephrotic syndrome and 16 controls. There were 15 first episode nephrotic syndrome (FENS), 15 infrequent relapsing nephrotic syndrome (IRNS) and 10 patients belonged to frequent relapsing nephrotic syndrome (FRNS). Serum IgE and IL-13 levels were significantly increased in active nephrotic ...
Kim Sung-Yong SY Fred Hutchinson Cancer Research - - 2014
A proportion of patients with aplastic anemia who are treated with immunosuppressive therapy develop clonal hematologic disorders, including myelodysplastic syndrome (post-aplastic anemia myelodysplastic syndrome). Many will proceed to allogeneic hematopoietic stem cell transplantation. We identified 123 patients with post-aplastic anemia myelodysplastic syndrome who from 1991 through 2011 underwent allogeneic transplantation, ...
Pereira Bernardo Dias BD Serviço de Endocrinologia e Diabetes, Hospital Garcia de Orta, E.P.E., Avenida Torrado da Silva, 2801-951, Almada-Setúbal, Portugal, - - 2014
Oncocytomas are tumours predominantly or exclusively composed of oncocytes, cells with granular and eosinophilic cytoplasm filled with mitochondria. Although they can occur in every organ, they are rare in adrenal glands, and in paediatric patients they are even rarer, with only three case reports previously published. We present a preschool ...
Damlaj Moussab M Division of Hematology, Department of Medicine, McGill University Health Centre (MUHC), Montreal General Hospital, Room A7.117.1, 1650 Cedar Avenue, Montreal, QC, H3G 1A4, - - 2014
Warm antibody autoimmune hemolytic anemia (AIHA) results from targeted antibodies towards the red blood cells (RBCs) and can be either idiopathic or secondary to certain diseases, such as autoimmune disorders or malignancy, drugs, or infection. Patients with DiGeorge syndrome are particularly vulnerable to autoimmune conditions secondary to thymic hypoplasia and ...
Waserman Jessica E JE Department of Psychiatry and Behavioural Neurosciences, Michael G. DeGroote School of Medicine, Faculty of Health Sciences, McMaster University, Hamilton, Ontario, - - 2014
Extreme neglect of one's living space and self-care is a perplexing condition termed Diogenes syndrome, where outcomes of intervention are often poor, with high relapse and mortality rates. Relatively little has been reported regarding management approaches. We present a case of Diogenes syndrome responsive to a structured behavioral paradigm during ...
Stensman Lars M LM *Department of Pediatrics, Aarhus University Hospital Skejby †Cancer Cytogenetics Laboratory, Department of Hematology, Aarhus University Hospital, Aarhus ‡Department of Pediatrics and Adolescent Medicine, University Hospital, Rigshospitalet §Institute of Clinical Medicine, University of Copenhagen, Copenhagen, - - 2014
We describe a patient diagnosed with acute myeloid leukemia (AML) and low activity of thiopurine methyltransferase (TPMT) who developed secondary myelodysplastic syndrome after treatment. A 10-year-old boy presented with AML-M2 with t(8;21)(q22;q22) and genotyping revealing 3*B TPMT heterozygosity. The patient was treated according to the NOPHO-AML 2004 protocol. Two years ...
Ekambaram Sudha S Department of Pediatric Nephrology, Mehta Childrens Hospital, Chennai, India. Correspondence to: Dr M Vijayakumar, Consultant Pediatric Nephrologist, Mehta Children's Hospitals, No.2(e) Mc Nichols Road, 3rd Lane, Chetpet, Chennai 600 031, Tamilnadu, India. - - 2014
To assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome. Retrospective analysis of hospital case records. Pediatric nephrology department of a tertiary referral pediatric hospital. 62 children with frequently relapsing nephrotic syndrome and 35 children with steroid-dependent nephrotic syndrome. Case records of children who were ...
Catarsi Eleonora E Department of Internal Medicine, Immuno-Allergology Unit, University of Pisa, via Roma 67, 56126, Pisa, Italy, - - 2014
Susac's Syndrome (SS) is a rare disease with unknown aetiology due to a microangiopathy affecting the precapillary arterioles of the brain, retina, cochlea and semicircular canals. Neurological manifestations, visual dysfunction and hearing loss represent the classical clinical triad of SS. Diagnosis is confirmed by laboratory investigations, neuroimaging findings, fluoroangiography and ...
Mihaila Romeo-Gabriel RG Romeo-Gabriel Mihaila, MD, PhD, "Lucian Blaga" University of Sibiu, Faculty of Medicine, Str Lucian Blaga, nr 2A, Cod 550169, Sibiu, - - 2014
Cardiorenal syndrome involves altering cardiac and renal function. These patients frequently develop resistance to diuretic therapy, so that ultrafiltration should be applied in emergency for saving them. Concomitant presence of an active hematologic malignancy represents an important complicating factor. We present the case of an elderly patient with acute myeloid ...
Inoue Akiko A Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, - - 2014
We herein present a case of pediatric therapy-related myelodysplastic syndrome (t-MDS) with complex karyotype who was treated with azacitidine (AZA) for AML1-EVI1 fusion transcript as minimal residual disease after allogeneic hematopoietic stem cell transplantation (HSCT). The patient was started on AZA 41 days after the HSCT without having achieved complete ...
Martinelli Sara S Section of Hematology, Azienda Ospedaliero-Universitaria S.Anna, Ferrara, Italy, - - 2014
Sweet's syndrome is a rare condition with potentially disabling implications, characterized by painful skin lesions due to neutrophilic dermal infiltration and systemic inflammatory symptoms. A significant proportion of cases is malignancy associated. Hematologic neoplasms, particularly acute myeloid leukemia and myelodysplastic syndromes, are the most commonly associated malignant conditions. Here, we ...
Sharma Bharat B Division of Pediatric Nephrology, Department of Pediatrics, Postgraduate Institute of Medical Education & Research, Dr Ram Manohar Lohia Hospital, New Delhi, - - 2014
Impaired endothelial function is the initial step in atherogenesis, which is largely responsible for ischaemic heart disease and thrombotic strokes decades later. Fourty two children with first episode nephrotic syndrome (FENS) aged 1-16 years and 40 controls were enrolled. Soluble thrombomodulin (sTM), tissue plasminogen activator (t-PA), plasminogen activator inhibitor -1 (PAI-1) ...
Sureshkumar Premala P Centre for Kidney Research, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, Sydney, NSW 2145, - - 2014
Although most children with idiopathic nephrotic syndrome will respond to corticosteroid therapy, 80-90 % suffer one or more relapses. Using Cox proportional hazard models, we analyzed predictors of remission and relapse in 1-year follow-up data on children aged below 15 years with new-onset nephrotic syndrome. Of 129 children, 107 achieved remission with ...
Sun Li L Department of Nephrology and Rheumatology, Children's Hospital of Fudan University, Shanghai, 201102, - - 2014
Idiopathic nephrotic syndrome is the most common glomerular disease in children. This study was undertaken to observe the efficacy and side-effects of rituximab (RTX) in treating children with different types of refractory primary nephrotic syndrome. Twelve patients with steroid dependent nephrotic syndrome (SDNS), frequently relapsing nephritic syndrome (FRNS), and steroid ...
Segarra Alfons - - 2014
Background: It has been reported that the circulating level of the soluble urokinase receptor (suPAR) could be useful for distinguishing idiopathic from secondary focal segmental glomerulosclerosis, but the results published are conflicting. In this study, we analyse the intraindividual variability and clinical and anatomopathological variables associated with the suPAR levels ...
Ye Wenling W Division of Nephrology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Shuifuyuan 1, Wangfujing, Beijing, 100730, China, - - 2014
Mucormycosis is a rare and fatal opportunistic infection occurring in severely immunocompromised patients. Here, we report, for the first time, on a 65-year-old man with idiopathic membranous nephropathy and moderate renal dysfunction who suffered from life-threatening pulmonary mucormycosis during immunosuppressive therapy. After amphotericin B (AmB) administration with a total accumulating ...
Konomoto Takao T Faculty of Medicine, Division of Pediatrics, Department of Reproductive and Developmental Medicine, University of Miyazaki, 5200 Kihara, Kiyokake-cho, Miyazaki, 889-1692, Japan, - - 2014
There is ongoing discussion regarding the mechanisms underlying edema formation in nephrotic syndrome (NS). Many studies published in the last decade reported that primary renal sodium retention was a major factor in edema formation. However, many of the factors influencing edema formation in NS remain unclear, including the role of ...
Sahali Djillali D Service de Néphrologie et Transplantation, AP-HP, CHU Henri Mondor, Creteil, 94010, France, - - 2014
Idiopathic change nephrotic syndrome (INS), the most frequent glomerular disease in children and young adults, is characterized by heavy proteinuria and a relapsing remitting course. Although the mechanisms underlying the pathophysiology of proteinuria remain unclear, clinical and experimental observations suggest that lymphocyte and podocyte disturbances are two sides of the ...
Uematsu Hironori H Division of Respiratory Medicine, National Fukuoka-Higashi Medical Center, Koga, Fukuoka, - - 2014
Polyangiitis overlap syndrome is defined as systemic vasculitis that cannot be classified into one of the well-defined vasculitic syndromes. In this report, a female patient who presented with vasculitis-like and asthmatic symptoms was diagnosed as having polyangiitis overlap syndrome of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and ...
Kim J J Division of Pediatric Nephrology, Department of Pediatrics, Cohen Children's Medical Center of New York, North Shore-LIJ Health System, New Hyde Park, N.Y., - - 2014
Most cases of idiopathic nephrotic syndrome in childhood are responsive to corticosteroids. However, there is a small group of children that demonstrate steroid resistance (steroid-resistant nephrotic syndrome; SRNS), steroid dependence, or that frequently relapse (frequent-relapse steroid-sensitive nephrotic syndrome; FR-SSNS) which are more clinically difficult to treat. Therefore, second-line immunosuppressants, such ...
Nascimento Ana Cláudia Mendes do AC "Júlio de Mesquita Filho" Paulista State University, Botucatu School of Medicine, Dermatology and Radiotherapy Department, BotucatuSP, Brazil, Doctor - Resident of the Dermatology and Radiotherapy Department, Botucatu School of Medicine - "Júlio de Mesquita Filho" Paulista State University (FMB-UNESP) - Botucatu, SP, - - 2014
The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease ...
Jindal Saurabh R SR Department of Dermatology, Venereology and Leprosy, Grant Medical College, Mumbai, - - 2014
A 38-year - old female came to us with sudden eruptions of painful edematous lesions which appeared pseudovesicular on cutaneous examination. Histopathology supported the diagnosis of sweet's syndrome and she responded to a combination of dapsone and oral steroids, after having relapsed on self-discontinuation of monotherapy with dapsone.
Li Xudong - - 2014
Hemophagocytic syndrome (HPS) is a fatal disease and T-cell lymphoma is a common cause of this syndrome. The authors describe a rare case of diffuse large B-cell lymphoma (DLBCL) with HPS. Hematologic investigation, bone marrow aspirate and biopsy, cytogenetic analysis and lymph node biopsy were performed. The patient obtained transient ...
Cerny Jan J Division of Hematology Oncology, Department of Medicine, UMass Memorial Medical Center, University of Massachusetts Medical School, 55 Lake Avenue North, Worcester, MA 01655, - - 2014
The optimal salvage therapy for patients with relapsed Burkitt lymphoma is unknown. Bone marrow necrosis is an underreported (<1% of bone marrow failures). Numb chin syndrome is another rare syndrome associated with aggressive malignancies. Survival of these syndromes is dictated by the underlying disease and is usually dismal. Our 35-year-old ...
Pieralli Filippo F Internal and Emergency Medicine Unit, Careggi University Hospital, Largo Brambilla 3, 50134, Florence, - - 2013
Catastrophic antiphospholipid syndrome (CAPS) is a rare variant of antiphospholipid syndrome characterized by widespread thrombotic microangiopathy and multiorgan failure. Clinically, CAPS signs and symptoms can mimic vasculitis of systemic lupus erythematosus, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura. CAPS is burdened by high mortality, nearly 50 % in most series. However, ...
Zobniw Chrystia M CM Department of Pharmacy Services, Roswell Park Cancer Institute, Buffalo, New - - 2013
Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, is characterized predominantly by fever, elevated neutrophil count, and erythematous skin lesions composed of plaques and nodules that appear on upper extremities, face, or neck. The incidence of Sweet's syndrome in the general population is unknown due to the rarity of ...
Komai Toshihiko - - 2013
A 38-year-old woman with relapsing longitudinal extensive transverse myelitis and Sjogren's syndrome (SS) was admitted with lower extremity muscle weakness. Studies showed high serum titer of anti-aquaporin4 antibody and gadolinium-enhanced-MRI T1-weighted lesions within thoracic cord. Clinical findings suggested neuromyelitis optica-spectrum disorder (NMO-SD). High-dose corticosteroids, plasma exchange and cyclophosphamide were not ...
Kleffner Ilka I Department of Neurology, University of Münster, Münster, - - 2013
Background: Susac syndrome is a rare disease characterized by the triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss mainly affecting young women. The finding of antibodies against the endothelium in the sera of these patients has supported the hypothesis of an autoimmune endotheliopathy of the brain, inner ...
Li Cui - - 2013
Tubulointerstitial nephritis and uveitis (TINU) syndrome is considered a rare cause of acute tubulointerstitial nephritis (ATIN) that is usually associated with renal recovery. This study sought to investigate the diagnosis, prognosis, and contributing factors of TINU syndrome using a large cohort of patients with prospective follow-up. This study included patients ...
El Koumi Mohamed - - 2013
Treatment of steroid-dependent nephrotic syndrome, particularly in patients who have failed to respond to multiple immunosuppressive drugs, remains challenging. Rituximab represents a new off-label therapeutic option. Here, we report the use of rituximab in 7 children with steroid-dependent nephrotic syndrome with various histological backgrounds who failed to maintain remission with ...
Hashemi Mohammad M Cellular and Molecular Research Center, Zahedan University of Medical Sciences, Zahedan, IR Iran ; Department of Clinical Biochemistry, School of Medicine, Zahedan University of Medical Sciences, Zahedan, IR - - 2013
It has been proposed that reactive oxygen species (ROS) is involved in the pathogenesis of various diseases. Paraoxonase, a high-density lipoprotein associated enzyme, prevents low-density lipoproteins from oxidation. The aim of the present study was to investigate the serum activities of paraoxonase-1 (PON-1), and aryleterase (ARE) as well as total ...
Jhamb Rajat - - 2013
Cryptococcosis, a significant opportunistic infection, has become a global concern since the advent of immunosuppressive chemotherapy or in immunodeficient patients. Host responses range from a harmless colonization to disseminated disease. An accurate or definitive diagnosis in patients with cryptococcal meningitis is often delayed because of the similar clinical presentation and ...
Chou Kuan-Nien - - 2013
Temozolomide (TMZ), an alkylating agent, is widely used for treating high-grade gliomas. TMZ has been reported to cause secondary myelodysplastic syndrome and acute myeloid leukemia. However, TMZ-related acute lymphoblastic leukemia is rare. Here we describe a 54-year-old woman with glioblastoma multiforme, who developed precursor-B acute lymphoblastic leukemia with translocation (4;11)(q21;q23) ...
Pérez-Paredes M G - - 2013
Lenalidomide is an angiogenesis inhibitor and antitumoral drug approved to treat relapsed multiple myeloma, amyloidosis and myelodysplastic syndromes. Cutaneous side effects are a known complication of lenalidomide. We are going to report the first case of lenalidomide induced photosensitivity.
Lin Tung-Liang - - 2013
Somatic mutations of TET2, IDH1, and IDH2 have been described in myelodysplastic syndrome. The impact of these mutations on outcome of myelodysplastic syndrome and their progression to secondary acute myeloid leukemia remains unclear. Mutation status of TET2, IDH1 and IDH2 was investigated in a cohort of 46 paired myelodysplastic syndrome/acute ...
Ragnarsson Oskar - - 2013
One-hundred years have passed since Harvey Williams Cushing presented the first patient with the syndrome that bears his name. In patients with Cushing's syndrome body composition, lipid-, carbohydrate- and protein-metabolism is dramatically affected and psychopathology and cognitive dysfunction is frequently observed. Untreated patients with Cushing's syndrome have a grave prognosis ...
Landau Daniel - - 2013
Hemophagocytic lymphohistiocytosis (HLH) is caused by an excessive activation of nonmalignant macrophages. Renal lesions have been described in association with, but always after, HLH diagnosis. We describe a previously healthy 26-month-old girl who presented originally with steroid-responsive nephrotic syndrome (NS), but after 4 months, on the first NS relapse, experienced numerous ...
Rajeswari Binitha - - 2013
Guillain-Barre syndrome (GBS) is rarely reported in children with acute lymphoblastic leukemia and may be difficult to differentiate from vincristine induced neuropathy. We report two children with acute lymphoblastic leukemia on induction chemotherapy who developed GBS. The diagnostic issues and potential pathogenic mechanisms underlying GBS in pediatric patients with ALL ...
Wingerchuk Dean M - - 2013
This review defines current clinical criteria for diagnosis, differential diagnosis, and clinical evaluation of acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica, and summarizes principles of treatment. Consensus criteria for transverse myelitis and acute disseminated encephalomyelitis have been proposed. A specific biomarker, aquaporin-4 autoantibody, has been discovered for neuromyelitis optica ...
Correia Ana Sofia - - 2013
Churg-Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of ...
Stoeckle Marcel - - 2013
We report on a 76-year old patient with recurrent mucosal leishmaniasis. Multiple treatment regimens were administered. After the second relapse, immunologic workup and review of the patient's history revealed the presence of Good syndrome, characterized by immunodeficiency in patients with thymoma. The third relapse was treated with oral miltefosine with ...
Kemper Markus J - - 2013
The idiopathic nephrotic syndrome (i.e. MCNS and FSGS) in children has been regarded as a disorder of T-cell function. Recent studies, however, also describe abnormalities of B-cell function. This supports the use of B-cell modulating treatment for idiopathic nephrotic syndrome (INS), especially rituximab, which has been used in other glomerular ...
Saghafi Hossein - - 2013
Castleman disease (CD) is a rare lymphoproliferative disorder, first described in 1956. This case report describes a 27-year-old man with hyaline vascular unicentric CD, first presented with edema and hypertension. On initial evaluation for edema, 24-hour urine collection revealed 8200 mg/24 h protein excretion. Pathologic examination of the kidney specimen ...
Ohashi Kota - - 2013
We report a 53-year-old male with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome who relapsed after autologous peripheral stem cell transplantation (ASCT), but responded extremely well to lenalidomide (LEN) plus low dose dexamethasone (Ld) therapy. The patient had been diagnosed with POEMS syndrome in November 2006, and ...
Konstantinov Konstantin N - - 2013
A woman diagnosed with mixed connective tissue disease (MCTD) developed an anti-myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA) and nephrotic syndrome with normal serum creatinine. Percutaneous kidney biopsy showed pauci-immune glomerulonephritis with superimposed immune complex deposition. After treatment with cyclophophamide and prednisone, proteinuria decreased progressively to a level of 0.4 g/g ...
Ravani Pietro - - 2013
In children with idiopathic nephrotic syndrome, rituximab can maintain short-term remission with withdrawal of prednisone and calcineurin inhibitors. Long-term effects including the number of repeated infusions to maintain remission are unknown. To test this, we treated 46 consecutive children with idiopathic nephrotic syndrome lasting for at least 1 year (mean ...
Sato Tomoko - - 2013
Reported herein is a case of relapse of nephrotic syndrome (NS) after intravitreal injection of bevacizumab, a monoclonal antibody that binds to vascular endothelial growth factor (VEGF), in a 16-year-old girl. She had a diagnosis of steroid-dependent NS and had been treated with prednisolone, and remained in remission. The patient ...
Kaur Sharandeep - - 2013
Acute disseminated encephalomyelitis (ADEM) has been reported in acute leukemia and after stem cell transplantation, but ADEM as a presenting feature in pediatric acute lymphoblastic leukemia is rare. We report an 8-year-old child with acute lymphoblastic leukemia who presented with features of ADEM. The diagnosis was confirmed by neuroimaging and ...
Dursun Hasan - - 2013
To investigate the association of endothelial nitric oxide synthase gene intron 4 (eNOS4) polymorphisms with nephrotic syndrome, the eNOS4 genotypes were assessed in 161 children with nephrotic syndrome in comparison with 78 healthy subjects. We classified the children with nephritic syndrome into 2 groups: as steroid-sensitive nephrotic syndrome (SSNS) (n=125) ...
Travassos Ana Rita - - 2013
: Sweet syndrome (SS) is a neutrophilic dermatosis that may be associated with malignancies, especially hematological. We describe the case of a 53-year-old woman with a clinical presentation suggestive of SS, accompanied by pancytopenia and a hypercellular marrow with signs of myelodysplasia. The histopathological findings were characterized as an SS-like ...
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