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Myasthenia Gravis and Pregnancy

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Population Pharmacokinetic Analysis of Tacrolimus in Adult Chinese Patients with Myasthenia Gravis: A Prospective Study.

Jue Liu, Yun-Peng Guo, Zheng Jiao, Chong-Bo Zhao, Hui Wu, Zi-Ran Li, Xiao-Jun Cai, Jian-Ying Xi,

BACKGROUND AND OBJECTIVES:Tacrolimus is a widely used immunosuppressive agent with narrow therapeutic window. Nowadays, tacrolimus has gained acceptance as a therapeutic option in myasthenia gravis (MG) treament, however, little is known about its pharmacokinetic characteristics in MG population. In this study, we aimed to investigate the population pharmacokinetic (PopPK) of tacrolimus in patients with MG and to develop model-informed dosing regimens. METHODS:Trough concentrations ... Read more >>

Eur J Drug Metab Pharmacokinet (European journal of drug metabolism and pharmacokinetics)
[2020, :]

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Suitable indications of eculizumab for patients with refractory generalized myasthenia gravis.

Munenori Oyama, Kensuke Okada, Masayuki Masuda, Yuko Shimizu, Kazumasa Yokoyama, Akiyuki Uzawa, Naoki Kawaguchi, Ryotaro Ikeguchi, Yasunobu Hoshino, Taku Hatano, Yukiko Ozawa, Jin Nakahara, Hitoshi Aizawa, Kazuo Kitagawa, Nobutaka Hattori, Satoshi Kuwabara, Hiroyuki Murai, Shigeaki Suzuki,

Background:Eculizumab is a humanized monoclonal antibody that targets complement protein C5 and inhibits terminal complement-mediated damage at the neuromuscular junction. Recently, the REGAIN study showed that eculizumab was effective and well tolerated in patients with anti-acetylcholine receptor antibody-positive refractory generalized myasthenia gravis (gMG). However, there is no consensus regarding which ... Read more >>

Ther Adv Neurol Disord (Therapeutic advances in neurological disorders)
[2020, 13:1756286420904207]

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A Comparison of Immunoglobulin Variable Region N-Linked Glycosylation in Healthy Donors, Autoimmune Disease and Lymphoma.

Esther M Vletter, Marvyn T Koning, Hans Ulrich Scherer, Hendrik Veelken, Rene E M Toes,

N-linked glycans play an important role in immunity. Although the role of N-linked glycans in the Fragment crystallizable (Fc) region of immunoglobulins has been thoroughly described, the function of N-linked glycans present in Ig-variable domains is only just being appreciated. Most of the N-linked glycans harbored by immunoglobulin variable domain ... Read more >>

Front Immunol (Frontiers in immunology)
[2020, 11:241]

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PREPL Deficiency: A Homozygous Splice Site PREPL Mutation in a Patient With Congenital Myasthenic Syndrome and Absence of Ovaries and Hypoplasia of Uterus.

Qi Yang, Rong Hua, Jiale Qian, Shang Yi, Fei Shen, Qiang Zhang, Mengting Li, Sheng Yi, Jingsi Luo, Xin Fan,

Prolyl endopeptidase-like (PREPL) deficiency (MIM 616224) is a very rare congenital disorder characterized by neonatal hypotonia and feeding difficulties, ptosis, neuromuscular symptoms, cognitive impairments, growth hormone deficiency, short stature, and hypergonadotropic hypogonadism. This syndrome is an autosomal recessive disease resulting from mutations in the PREPL gene. Previous reports have associated ... Read more >>

Front Genet (Frontiers in Genetics)
[2020, 11:]

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A Randomized Control Study on the Efficacy of Thymectomy in Patients with Nonthymomatous (without Chest Tumor) Myasthenia Gravis.

Ji Chen, Zhiming Chen, Feng Miao, Yang Song, Gang Chen, Yongjun Zhu, Liewen Pang, Jianying Xi, Chongbo Zhao, Xiaofeng Chen,

Ann Indian Acad Neurol (Annals of Indian Academy of Neurology)
[2020, 23(1):141-144]

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Levothyroxine Treatment of Pregnant Women with Hypothyroidism: Retrospective Analysis of a US Claims Database.

Maureen J Lage, Jamie Vora, Zsolt Hepp, Ramon Espaillat,

BACKGROUND:Approximately 2-4% of women of reproductive age have hypothyroidism. This study characterizes pregnant women with hypothyroidism and examines adherence to guidelines during pregnancy. METHODS:Women age 18 to 49 who were pregnant in 2014 and identified with hypothyroidism (N = 3448) were included in the retrospective study. The analyses examined differences in characteristics ... Read more >>

Adv Ther (Advances in therapy)
[2020, 37(2):933-945]

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Myasthenia Gravis and Congenital Myasthenic Syndromes.

Emma Ciafaloni,

PURPOSE OF REVIEW:Myasthenia gravis (MG) is an autoimmune neuromuscular disease that causes fluctuating weakness in ocular, bulbar, and limb muscles and can, in 15% of cases, cause myasthenic crisis, a neurologic emergency characterized by respiratory failure. Although infrequent, MG needs to be promptly recognized and treated because the potential for ... Read more >>

Continuum (Minneap Minn) (Continuum (Minneapolis, Minn.))
[2019, 25(6):1767-1784]

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First maternal uniparental disomy for chromosome 2 with PREPL novel frameshift mutation of congenital myasthenic syndrome 22 in an infant.

Ping Zhang, Bingbing Wu, Yulan Lu, Qi Ni, Renchao Liu, Wenhao Zhou, Huijun Wang,

BACKGROUND:Congenital myasthenic syndrome 22 (CMS22) is a rare autosomal recessive disorder due to isolated PREPL deficiency and characterized by neonatal hypotonia, muscular weakness, and feeding difficulties. Eight such cases have already been reported, while maternal uniparental disomy with a PREPL pathogenic mutation has never been involved. METHODS:Trio whole-exome sequencing (WES), ... Read more >>

Mol Genet Genomic Med (Molecular Genetics & Genomic Medicine)
[2020, 8(3):e1144]

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Sugammadex in the management of myasthenic patients undergoing surgery: beyond expectations.

Michele Carron, Alessandro De Cassai, Federico Linassi,

Ann Transl Med (Annals of translational medicine)
[2019, 7(Suppl 8):S307]

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Congenital Vocal Cord Paralysis and Late-Onset Limb-Girdle Weakness in MuSK-Congenital Myasthenic Syndrome.

Marcus V Pinto, Jacqui-Lyn Saw, Margherita Milone,

A 30-year-old woman with congenital vocal cord paralysis presented for evaluation of fatigable proximal upper limb weakness and difficulty maintaining the neck erect. Neurologic examination showed bilateral asymmetric eyelid ptosis, mild weakness (MRC 4/5), and atrophy of neck extensors and shoulder girdle muscles, whereas lower limb muscle strength was normal. ... Read more >>

Front Neurol (Frontiers in neurology)
[2019, 10:1300]

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Adult Intestinal Toxemia Botulism.

Richard A Harris, Fabrizio Anniballi, John W Austin,

Intoxication with botulinum neurotoxin can occur through various routes. Foodborne botulism results after consumption of food in which botulinum neurotoxin-producing clostridia (i.e., Clostridium botulinum or strains of Clostridium butyricum type E or Clostridium baratii type F) have replicated and produced botulinum neurotoxin. Infection of a wound with C. botulinum and ... Read more >>

Toxins (Basel) (Toxins)
[2020, 12(2):]

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Patient characteristics, clinical manifestations, prognosis, and factors associated with gastrointestinal cytomegalovirus infection in immunocompetent patients.

Thanaboon Chaemsupaphan, Julajak Limsrivilai, Chenchira Thongdee, Asawin Sudcharoen, Ananya Pongpaibul, Nonthalee Pausawasdi, Phunchai Charatcharoenwitthaya,

BACKGROUND:Gastrointestinal (GI) cytomegaloviral (CMV) infection is common among patients with immunocompromised status; however, data specific to GI-CMV infection in immunocompetent patients are comparatively limited. METHODS:This retrospective study included patients diagnosed with GI-CMV infection at Siriraj Hospital (Bangkok, Thailand) during 2008-2017. Baseline characteristics, presentations, comorbid conditions, endoscopic findings, treatments, and outcomes ... Read more >>

BMC Gastroenterol (BMC gastroenterology)
[2020, 20(1):22]

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Minimal breast milk transfer of rituximab, a monoclonal antibody used in neurological conditions.

Kristen M Krysko, Sara C LaHue, Annika Anderson, Alice Rutatangwa, William Rowles, Ryan D Schubert, Jacqueline Marcus, Claire S Riley, Carolyn Bevan, Thomas W Hale, Riley Bove,

OBJECTIVE:To determine the transfer of rituximab, an anti-CD20 monoclonal antibody widely used for neurologic conditions, into mature breast milk. METHODS:Breast milk samples were collected from 9 women with MS who received rituximab 500 or 1,000 mg intravenous once or twice while breastfeeding from November 2017 to April 2019. Serial breast ... Read more >>

Neurol Neuroimmunol Neuroinflamm (Neurology(R) neuroimmunology & neuroinflammation)
[2020, 7(1):]

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Ectopic thymus as a cause of Horner's syndrome.

Margeaux L Berroth, Lyudmila V Morozova, Jeffery M Pollock,

Ectopy of the thymus is a rare anomaly arising during fetal development, where the thymus does not make a complete decent into the thoracic cavity where it should involute in adolescence. The most common complications of an ectopic thymus include tracheal or esophageal compression presenting in childhood. This is a ... Read more >>

Radiol Case Rep (Radiology case reports)
[2020, 15(1):23-25]

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Use of sugammadex in patients with neuromuscular disorders: a systematic review of case reports.

Usha Gurunathan, Shakeel Meeran Kunju, Lisa May Lin Stanton,

BACKGROUND:Sugammadex is a modified gamma-cyclodextrin that acts by selectively encapsulating free amino-steroidal neuromuscular relaxants. Several case reports have been published on the use of sugammadex in patients with neuromuscular disorders that include neuromuscular junction diseases, myopathies, neuropathies, and motor neurone disorders. The primary aim of this review is to systematically ... Read more >>

BMC Anesthesiol (BMC anesthesiology)
[2019, 19(1):213]

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Toxicity of tumor immune checkpoint inhibitors-more attention should be paid.

Yu Liu, Hao Wang, Juan Deng, Chenglong Sun, Yayi He, Caicun Zhou,

In recent years, immunotherapy, especially immune checkpoint inhibitors (ICIs), has achieved amazing results in the treatment of lung cancer, melanoma, renal clear cell carcinoma and other malignant tumors. Although ICIs have achieved significant efficacy in tumor treatment, the immune-related adverse events (irAEs) caused by non-specific immune activation of ICIs can ... Read more >>

Transl Lung Cancer Res (Translational lung cancer research)
[2019, 8(6):1125-1133]

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Overview of international naturopathic practice and patient characteristics: results from a cross-sectional study in 14 countries.

Amie Steel, Hope Foley, Ryan Bradley, Claudine Van De Venter, Iva Lloyd, Janet Schloss, Jon Wardle, Rebecca Reid,

BACKGROUND:Naturopathy is a distinct system of traditional and complementary medicine recognized by the World Health Organization and defined by its philosophic approach to patient care, rather than the treatments used by practitioners. Worldwide, over 98 countries have practicing naturopaths, representing 36% of all countries and every world region. The contributions ... Read more >>

BMC Complement Med Ther (BMC complementary medicine and therapies)
[2020, 20(1):59]

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Determining How Far an Adult Rare Disease Patient Needs to Travel for a Definitive Diagnosis: A Cross-Sectional Examination of the 2018 National Rare Disease Survey in China.

Xiang Yan, Shenjing He, Dong Dong,

Background: To investigate the multidimensional difficulties in accessing a definitive diagnosis of adult rare diseases and the associated impact factors in China. Methods: A total of 1010 adult rare disease patients from the 2018 China Rare Disease Survey were used for analysis. The Structural Equation Models examined the interrelationships among ... Read more >>

Int J Environ Res Public Health (International journal of environmental research and public health)
[2020, 17(5):]

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Intravenous Magnesium Sulfate for Pain Management in Patients with Acute Renal Colic; a Randomized Clinical Trial.

Alireza Majidi, Fatemeh Derakhshani,

Introduction:It seems that magnesium (Mg) sulfate can be helpful in controlling the acute pain caused by the stone passing from the ureter by reducing ureter smooth muscle contractions. The present study has been designed with the aim of assessing the role of Mg sulfate in controlling the renal colic pain ... Read more >>

Arch Acad Emerg Med (Archives of academic emergency medicine)
[2020, 8(1):e5]

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Ectopic thymoma: report of 2 cases and review of the literature.

Xia Wu, Jiannan Guo, Xuemei Zhou, Yijie Li, Yuanli Huang, Ligao Wu,

In this report, we present two cases of ectopic thymoma, aiming to explore the clinicopathologic features, diagnosis, and differential diagnosis of ectopic thymoma. Case 1 was a female 56-years-old. For 6 months' time, there was no obvious cause of cough, expectoration, chest tightness, or asthma with chest pain. PET-CT showed ... Read more >>

Int J Clin Exp Pathol (International journal of clinical and experimental pathology)
[2019, 12(12):4297-4302]

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Comment on: Takotsubo cardiomyopathy, or 'broken-heart syndrome', with concomitant myasthenic crisis.

Josef Finsterer,

Singapore Med J (Singapore medical journal)
[2019, 60(10):551]

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Myogenic Disease and Metabolic Acidosis: Consider Multiple Acyl-Coenzyme A Dehydrogenase Deficiency.

A Dernoncourt, J Bouchereau, C Acquaviva-Bourdain, C Wicker, P De Lonlay, C Gourguechon, H Sevestre, P-E Merle, J Maizel, C Brault,

Background:Multiple acyl-coA dehydrogenase deficiency (MADD) is a rare, inherited, autosomal-recessive disorder leading to the accumulation of acylcarnitine of all chain lengths. Acute decompensation with cardiac, respiratory or hepatic failure and metabolic abnormalities may be life-threatening. Case Presentation:A 29-year-old woman presented with severe lactic acidosis associated with intense myalgia and muscle ... Read more >>

Case Rep Crit Care (Case reports in critical care)
[2019, 2019:1598213]

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Biological characteristics of transcription factor RelB in different immune cell types: implications for the treatment of multiple sclerosis.

Meng-Ge Yang, Li Sun, Jinming Han, Chao Zheng, Hudong Liang, Jie Zhu, Tao Jin,

Transcription factor RelB is a member of the nuclear factror-kappa B (NF-κB) family, which plays a crucial role in mediating immune responses. Plenty of studies have demonstrated that RelB actively contributes to lymphoid organ development, dendritic cells maturation and function and T cells differentiation, as well as B cell development ... Read more >>

Mol Brain (Molecular brain)
[2019, 12(1):115]

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Takotsubo syndrome in patients with myasthenia gravis: a systematic review of previously reported cases.

Devarajan Rathish, Minuri Karalliyadda,

BACKGROUND:Myasthenia gravis associated takotsubo syndrome is a rare condition. This study aimed to explore its typical presentation, investigations and treatment through a systematic review of previously reported cases. METHODS:Databases and reference lists of the selected articles were searched for case reports on Myasthenia gravis associated takotsubo syndrome. CARE guidelines were ... Read more >>

BMC Neurol (BMC neurology)
[2019, 19(1):281]

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Therapeutic apheresis during pregnancy: A single center experience.

Anna Colpo, Piero Marson, Francesca Pavanello, Tiziana Tison, Maria Teresa Gervasi, Alessandra Zambon, Amelia Ruffatti, Giustina De Silvestro, Ariela Hoxha,

INTRODUCTION:Therapeutic apheresis (TA) represents a treatment option for pre-existing conditions or diseases occurring during gestation. Although pregnancy is not a contraindication per se, due to the lack of evidence-based guidelines and presumed risk of maternal/fetal adverse events there is a general resistance to its application. MATERIAL AND METHODS:Between January 2005 ... Read more >>

Transfus. Apher. Sci. (Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis)
[2019, 58(5):652-658]

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