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Immunoglobulin iv

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https://www.readbyqxmd.com/read/29777306/respiratory-manifestations-in-lps-responsive-beige-like-anchor-lrba-protein-deficient-patients
#1
Oded Shamriz, Bella Shadur, Adeeb NaserEddin, Irina Zaidman, Natalia Simanovsky, Orly Elpeleg, Eitan Kerem, Joel Reiter, Polina Stepensky
Lipopolysaccharide (LPS)-responsive beige-like anchor (LRBA) protein deficiency is a rare syndrome of primary immune deficiency and immune dysregulation. In this study, we sought to summarize our experience with respiratory manifestations in LRBA-deficient patients. We conducted a retrospective analysis of the medical records of LRBA-deficient patients treated at Hadassah-Hebrew University Medical Center, Jerusalem, Israel. Data retrieved included pulmonary workup, disease course, treatment, and outcome. Ten patients were included...
May 18, 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29776940/successful-treatment-of-fulminant-neonatal-enteroviral-myocarditis-in-monochorionic-diamniotic-twins-with-cardiopulmonary-support-intravenous-immunoglobulin-and-pocapavir
#2
Shahnawaz M Amdani, Hannah S Kim, Anthony Orvedahl, Audrey Odom John, Ahmed Said, Kathleen Simpson
Neonatal cardiogenic shock most commonly occurs due to critical congenital heart disease, sepsis, metabolic disorder or arrhythmias. In particular, enterovirus infections are common in the neonatal period, and patients can present with fulminant myocarditis. Early recognition is imperative due to its high morbidity and mortality without prompt and aggressive treatment. We present the successful treatment of fulminant neonatal enteroviral myocarditis in a pair of monochorionic diamniotic twins with cardiopulmonary support, intravenous immunoglobulin and pocapavir, an enteroviral capsid inhibitor...
May 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29774587/intravenous-immunoglobulins-in-dermatology-part-2-clinical-indications-and-outcomes
#3
REVIEW
E Forbat, F R Ali, F Al-Niaimi
Intravenous immunoglobulin (IVIg) is a solution of human IgG, salt, sugars and solvents used to treat a multitude of diseases. Although IVIg has been known to treat many diseases safely and successfully, there are relatively few supporting randomized controlled trials. In part two of this review, we assess the clinical indications of IVIg in dermatological disorders and the outcomes of its use.
May 17, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29774583/intravenous-immunoglobulins-in-dermatology-part-1-biological-mechanisms-and-methods-of-administration
#4
REVIEW
E Forbat, F R Ali, F Al-Niaimi
Intravenous immunoglobulin (IVIg) is a solution of human IgG, salt, sugars and solvents, which is used to treat a multitude of diseases. Although IVIg has been known to treat many diseases safely and successfully, there are relatively few supporting randomized controlled trials. In this article, we review the biological mechanisms of IVIg in dermatological disorders and the practicalities of its use, including its mechanism of action, dosing, availability, costs and adverse effects.
May 17, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29774053/postherpes-simplex-encephalitis-a-case-series-of-viral-triggered-autoimmunity-synaptic-autoantibodies-and-response-to-therapy
#5
Harry Alexopoulos, Sofia Akrivou, Sotiria Mastroyanni, Maria Antonopoulou, Argirios Dinopoulos, Melpo Giorgi, Kostas Konstantinou, Evangelos Kouremenos, Maria Lariou, Dimitrios Naoumis, Efterpi Pavlidou, Evaggelos Pavlou, Konstantinos Voudris, Panayotis Vlachoyiannopoulos, Marinos C Dalakas
Background: Recent evidence suggests that patients with herpes simplex virus (HSV) encephalitis may relapse because of autoimmunity against the N-methyl-D-aspartate receptor (NMDAR). We present a case series of post-HSV relapsing encephalopathy associated with antibodies to central nervous system (CNS) synaptic antigens. Patient/Methods: Sera and cerebrospinal fluid (CSF) from five patients with HSV encephalitis who relapsed after antiviral therapy were tested for anti-NMDAR, gamma-aminobutyric acid b receptor (GABAbR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), Leucine-rich, glioma inactivated 1 (LGI1), anti -contactin-associated protein-like 2 (CASPR2) and dipeptidyl-peptidase-like protein-6 (DDPX) antibodies using cell-based assays...
2018: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/29773230/medications-received-by-patients-with-juvenile-dermatomyositis
#6
Takayuki Kishi, Nastaran Bayat, Michael M Ward, Adam M Huber, Lan Wu, Gulnara Mamyrova, Ira N Targoff, William J Warren-Hicks, Frederick W Miller, Lisa G Rider
OBJECTIVE: Few controlled studies are available to guide treatment decisions in juvenile dermatomyositis (JDM). This study evaluated therapies received, changes of treatment over time, and factors associated with medication choices in JDM. METHODS: We performed a retrospective analysis of the number and type of therapies and duration of treatment for 320 patients with JDM enrolled in a North American registry. Kaplan-Meier and logistic regression analysis were used to assess the association of demographic and clinical features and autoantibodies with medication usage...
March 28, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29771320/immunoglobulin-profiling-identifies-unique-signatures-in-patients-with-kawasaki-disease-during-intravenous-immunoglobulin-treatment
#7
Tai-Ming Ko, Kazuma Kiyotani, Jeng-Sheng Chang, Jae-Hyun Park, Poh Yin Yew, Yuan-Tsong Chen, Jer-Yuarn Wu, Yusuke Nakamura
Identifying the causes of high fever syndromes such as Kawasaki disease (KD) remains challenging. To investigate pathogen exposure signatures in suspected pathogen-mediated diseases like KD, we performed immunoglobulin (Ig) profiling using a next-generation sequencing method. After intravenous Ig (IVIG) treatment, we observed disappearance of clonally expanded IgM clonotypes, which were dominantly observed in acute-phase patients. The complementary-determining region 3 (CDR3) sequences of dominant IgM clonotypes in acute-phase patients were commonly observed in other Ig isotypes...
May 16, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29768976/predictors-of-intravenous-immunoglobulin-resistance-and-coronary-artery-aneurysm-in-patients-with-kawasaki-disease
#8
Nattika Chantasiriwan, Suchaya Silvilairat, Krit Makonkawkeyoon, Yupada Pongprot, Rekwan Sittiwangkul
BACKGROUND: Patients with Kawasaki disease (KD) who have intravenous immunoglobulin (IVIG) resistance are at increased risk for development of coronary artery abnormalities. Although in Japan several risk scoring systems are able to predict patients with IVIG-resistant (KD), they do not accurately predict non-responders in other regions. AIM: The objectives of this study were to determine the predictors of IVIG resistance and coronary artery aneurysm (CAA) and to develop risk scoring systems for predicting IVIG-resistant KD in the Thai population...
May 17, 2018: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/29766732/-remission-of-the-disease-associated-related-with-immunoglobulin-igg4-accompanied-by-multiple-lymphadenopathy-after-treatment-with-rituximab-and-dexamethasone-a-case-report
#9
Zdeněk Adam, Zita Chovancová, Markéta Nová, Pavel Fabian, Zdeněk Řehák, Renata Koukalová, Marek Slávik, Luděk Pour, Marta Krejčí, Aleš Čermák, Zdeněk Král, Jiří Mayer
A disease associated with immunoglobulin IgG4 is a rare unit with very variable symptoms. We describe the course and treatment of the disease in a patient who presented with multiple lymphadenopathy and infiltrates in the area of the retroperitoneum and pelvis and signs of chronic sclerosing pancreatitis. The disease was clinically manifested by a significant loss of weight, but also by a loss of perception of taste and smell. The diagnosis was made based on a high amount of IgG4 expressing plasma cells in the sampled tissue and an increased concentration of immunoglobulins of type IgG and mainly subclass IG4...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29765787/liver-failure-and-conjugated-hyperbilirubinemia-in-a-preterm-neonate-role-of-early-ivig-and-exchange-transfusion
#10
Jayasree Nair, Vasantha H S Kumar
Neonatal liver failure (NLF) is a rare diagnosis but carries with it significant risks of mortality and morbidity. Common etiologies for NLF include metabolic causes, gestational alloimmune liver disease (GALD or neonatal hemochromatosis), and viral infections. We report a case of liver failure in a premature infant with abnormal iron profile within 48 hours of birth. Lack of accepted guidelines for the initial management of severe jaundice with a high direct component in the first week after birth made treatment challenging...
April 2018: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29765591/a-case-of-periorbital-necrotizing-fasciitis-rapidly-progressing-to-severe-multiorgan-failure
#11
Paola Eiben, Sancho Rodriguez-Villar
Periorbital necrotizing fasciitis (PNF) is a severe suppurative infection of the subcutaneous tissue and underlying fascia of the periorbital region. Typically, the course of PNF is milder and has a better prognosis than that of necrotizing fasciitis in other parts of the body. As such, this disease is thought to be associated with a significantly smaller risk of morbidity and mortality. Nevertheless, it is a rare and devastating condition that can lead to disfigurement, blindness and death. Early recognition is critical to improved patient outcomes...
April 2018: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/29765083/adeno-associated-virus-vector-mediated-interleukin-10-induction-prevents-vascular-inflammation-in-a-murine-model-of-kawasaki-disease
#12
Jun Nakamura, Sachiko Watanabe, Hiroaki Kimura, Motoi Kobayashi, Tadayoshi Karasawa, Ryo Kamata, Fumitake Usui-Kawanishi, Ai Sadatomo, Hiroaki Mizukami, Noriko Nagi-Miura, Naohito Ohno, Tadashi Kasahara, Seiji Minota, Masafumi Takahashi
Kawasaki disease (KD), which is the leading cause of pediatric heart disease, is characterized by coronary vasculitis and subsequent aneurysm formation. Although intravenous immunoglobulin therapy is effective for reducing aneurysm formation, a certain number of patients are resistant to this therapy. Because interleukin-10 (IL-10) was identified as a negative regulator of cardiac inflammation in a murine model of KD induced by Candida albicans water-soluble fraction (CAWS), we investigated the effect of IL-10 supplementation in CAWS-induced vasculitis...
May 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29763986/early-iv-injected-human-dermis-derived-mesenchymal-stem-cells-after-transient-global-cerebral-ischemia-do-not-pass-through-damaged-blood-brain-barrier
#13
Ji Hyeon Ahn, Bai Hui Chen, Joon Ha Park, Bich Na Shin, Tae-Kyeong Lee, Jeong Hwi Cho, Jae Chul Lee, Jeong-Ran Park, Se-Ran Yang, Sungwoo Ryoo, Myoung Cheol Shin, Jun Hwi Cho, Il Jun Kang, Choong Hyun Lee, In Koo Hwang, Young-Myeong Kim, Moo-Ho Won
There is lack of researches on effects of intravenously injected mesenchymal stem cells (MSCs) against transient cerebral ischemia (TCI). We investigated the disruption of the neurovascular unit (NVU), which comprises the blood-brain barrier (BBB) and examined entry of human dermis-derived MSCs (hDMSCs) into the damaged hippocampal CA1 area in a gerbil model of TCI, and their subsequent effects on neuroprotection and cognitive function. Impairments of neurons, and BBB were examined by immunohistochemistry, electron microscopy, and Evans blue and immunoglobulin G (IgG) leakage...
May 15, 2018: Journal of Tissue Engineering and Regenerative Medicine
https://www.readbyqxmd.com/read/29761871/autophagy-may-protect-against-parenteral-nutrition-associated-liver-disease-by-suppressing-endoplasmic-reticulum-stress
#14
Tian Zhang, Junkai Yan, Nan Wang, Lina Dai, Ying Wang, Wei Cai
BACKGROUND: The objectives of this study were to address the role of autophagy in the pathogenesis of parenteral nutrition (PN)-associated liver disease (PNALD) and its possible mechanism in vivo. METHODS: Five-week-old male Sprague Dawley rats were fed Shoobree chow (Xietong Organism, Jiangsu, China) and administered intravenous 0.9% saline (sham group), PN (PN group), PN plus rapamycin (1 mg/kg; PN + Rapa group), or rapamycin (Rapa group) for 7 days. Before and after study, body weight, biochemical indicators, hepatic histology, level of autophagy, hepatocyte apoptosis, reactive oxygen species (ROS), and endoplasmic reticulum (ER) stress indicators including binding immunoglobulin protein (BIP), spliced X-box-binding protein-1 (sXBP1), and CCAAT-enhancer-binding protein homologous protein (CHOP) were measured...
May 15, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29761297/severe-neurologic-complications-of-immune-checkpoint-inhibitors-a-single-center-review
#15
Sarah Mancone, Thomas Lycan, Tamjeed Ahmed, Umit Topaloglu, Andrew Dothard, William J Petty, Roy E Strowd
BACKGROUND: Immune checkpoint inhibitors (ICIs) are a promising class of anticancer drugs associated with immune-related adverse events (IRAEs). In registration studies of selected cancer populations, neurologic IRAEs were rare. Post-marketing experience describing their prevalence in clinical practice continues to be reported. METHODS: A retrospective cohort of patients treated at our institution with ICIs from 2005 to 2017 was identified. Patients with new neurologic ICD codes documented during or after ICI treatment were enrolled...
May 14, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29761122/bortezomib-treatment-for-severe-refractory-anti-nmda-receptor-encephalitis
#16
Yong-Won Shin, Soon-Tae Lee, Tae-Joon Kim, Jin-Sun Jun, Kon Chu
Objective: To evaluate the therapeutic potential of bortezomib, a proteasome inhibitor that target plasma cells, in order to revive stalled recovery in patients with anti- N -methyl-d-aspartate (NMDA) receptor encephalitis who remain bedridden even after aggressive immunotherapy. Methods: We consecutively enrolled patients with anti-NMDA receptor encephalitis who remained bedridden after first-line immunotherapy (steroids and intravenous immunoglobulin), second-line immunotherapy (rituximab), and tocilizumab treatment, and treated them with subcutaneous bortezomib...
May 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29760626/toxic-epidermal-necrolysis-in-an-8-year-old-girl-successfully-treated-with-cyclosporin-a-intravenous-immunoglobulin-and-plasma-exchange
#17
Marzena Zielińska, Łukasz Matusiak, Waldemar Gołębiowski, Katarzyna Swiątek, Iwona Chlebicka, Joanna Maj, Jacek Szepietowski
No abstract text is available yet for this article.
April 2018: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29759327/immunotherapy-responsive-childhood-neurodegeneration-with-systemic-and-central-nervous-system-inflammation
#18
Mario Sa, Yael Hacohen, Lucy Alderson, W K Kling Chong, Glenn Anderson, Thomas S Jacques, David Neubauer, Elzbieta Szczepanik, Ming Lim, Marios Kaliakatsos
Subacute neuroregression in association with raised neopterin and overexpression of interferon stimulated genes (ISGs) could indicate a type 1 interferonopathy. Here we describe a novel immunotherapy-responsive, clinico-immunological and imaging phenotype with evidence of innate immune activation. Three children (patient 1: 22-month-old boy; patient 2: 5-year-old girl; patient 3: 4-year-old girl) presented with asymmetric bilateral mixed dystonia and spasticity, regression in language (expressive more than receptive) and bulbar symptoms with no evidence of seizures...
April 27, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29759074/dengue-fever-complicated-with-guillain-barr%C3%A3-syndrome-a-case-report-and-review-of-the-literature
#19
Chamara Dalugama, John Shelton, Mahendra Ekanayake, Indika Bandara Gawarammana
BACKGROUND: Dengue is an arboviral infection classically presenting with fever, arthralgia, headache, and rashes. It is hyperendemic in Sri Lanka and has a major impact on health. Neurological complications of dengue fever are rare but have been reported in the literature. CASE PRESENTATION: A 60-year-old Sri Lankan man presented with a history of fever, arthralgia, and generalized malaise of 2 days duration. A diagnosis of dengue was confirmed with leukopenia, thrombocytopenia, and positive NS1 antigen done on day 2 without evidence of hemoconcentration...
May 15, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29755528/hypogammaglobulinemia-observed-one-year-after-rituximab-treatment-for-idiopathic-thrombocytopenic-purpura
#20
Bilal Ahmad Shoukat, Osama Ali, Dileep Kumar, Muhammad Bilal Gilani, Adeela Zahid, Shaheer Aslam Joiya, Maqsood Anwar Malik
We present the case of a 19-year-old female with severe hypogammaglobulinemia after having had treatment with rituximab for idiopathic thrombocytopenic purpura requiring intravenous immunoglobulins. She was admitted with the diagnosis of left-sided pneumonia with parapneumonic effusion. The patient was treated with piperacillin/tazobactam after having a poor response to co-amoxiclav. The patient had been tested for immunoglobulin levels, and the levels were very low. She has a history of ITP for which she received steroids...
2018: Case Reports in Medicine
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