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Congenital lupus

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https://www.readbyqxmd.com/read/29027258/acquired-factor-xi-deficiency-and-therapeutic-plasma-exchange
#1
Geoffrey D Wool, Angela Treml, Jonathan L Miller
Congenital factor XI (FXI) deficiency is associated with a variable bleeding phenotype. Recent reports have documented the use of therapeutic plasma exchange to rapidly and isovolumetrically increase FXI levels before invasive procedures in patients with congenital FXI deficiency. We report a case of acquired FXI deficiency in a pregnant woman with lupus. We proved that the inhibitor was an IgG, therefore potentially capable of crossing the placenta. While immune suppression eliminated detectable circulating inhibitor, the woman's FXI remained quite low...
October 13, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28943081/a-large-retrospective-review-of-persistent-proteinuria-in-children
#2
Chingying Chang-Chien, Gwo-Tsann Chuang, I-Jung Tsai, Bor-Luen Chiang, Yao-Hsu Yang
BACKGROUND: Proteinuria is a common finding in children. It may be due to a benign cause, but it can also represent early renal injury. Of children with persistent proteinuria noted in mass urine screening programs, 35% have a urine protein level greater than 100 mg/dl and many of them are associated with many underlying renal diseases. The aim of this study was to identify the etiology and prognosis of persistent proteinuria in children. METHODS: We collected data on urine protein from January 2011 to December 2016 in a tertiary medical center...
September 21, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28915908/advances-in-paediatrics-in-2016-current-practices-and-challenges-in-allergy-autoimmune-diseases-cardiology-endocrinology-gastroenterology-infectious-diseases-neonatology-nephrology-neurology-nutrition-pulmonology
#3
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Silvia Montella, Sergio Bernasconi
This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated...
September 16, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28875729/role-of-cardiac-pacing-in-congenital-complete-heart-block
#4
Stephanie F Chandler, Francis Fynn-Thompson, Douglas Y Mah
Congenital complete heart block affects 1/15,000 live-born infants, predominantly due to atrioventricular nodal injury from maternal antibodies of mothers with systemic lupus erythermatosus or Sjogren's syndrome. The majority of these children will need a pacemaker implanted prior to becoming young adults. This article will review the various patient and technical factors that influence the type of pacemaker implanted, and the current literature on optimal pacing practices. Areas covered: A literature search was performed using PubMed, Embase and Web of Science...
November 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/28844688/primary-sj%C3%A3-gren-s-syndrome-and-pregnancy-a-report-of-18-cases
#5
Julia Demarchi, Silvia B Papasidero, Diana Klajn, Paula Alba, Alejandra M Babini, Virginia Durigan, Carla Gobbi, Laura Raiti
Primary Sjögren's syndrome (pSS) is a condition that predominantly affects women. Reports of pregnancy outcome in these patients are limited and contradictory. OBJECTIVE: To describe pregnancy characteristics and outcomes and newborn morbidity in women with pSS. MATERIAL AND METHODS: We included women with pSS who became pregnant after the onset of the symptoms of the disease. Clinical and serological characteristics, risk factors and previous maternal comorbidities are described...
August 24, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28843719/incidence-risk-factors-and-mortality-of-neonatal-and-late-onset-dilated-cardiomyopathy-associated-with-cardiac-neonatal-lupus
#6
Nathalie Morel, Kateri Lévesque, Alice Maltret, Gabriel Baron, Mohamed Hamidou, Pauline Orquevaux, Jean-Charles Piette, François Barriere, Jérôme Le Bidois, Laurent Fermont, Olivier Fain, Arnaud Theulin, François Sassolas, Quentin Hauet, Gaëlle Guettrot-Imbert, Sophie Georgin-Lavialle, Christophe Deligny, Eric Hachulla, Luc Mouthon, Claire Le Jeunne, Philippe Ravaud, Delphine Le Mercier, Bénédicte Romefort, Elisabeth Villain, Damien Bonnet, Nathalie Costedoat-Chalumeau
BACKGROUND: Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. METHODS: We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. RESULTS: Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]...
December 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28839394/eyelash-trichomegaly-a-rare-presenting-feature-of-systemic-lupus-erythematosus
#7
Ashish Dalal, Sudhanshu Sharma, Ajay Kumar, Nidhi Sharma
An increase in length, curling, pigmentation, or thickness of eyelashes is termed eyelash trichomegaly. It may be inherited as an isolated trait or as a feature of a congenital syndrome such as Oliver-McFarlane syndrome or oculocutaneous albinism. Acquired conditions associated with eyelash trichomegaly include HIV infection, connective tissue disorders, and the administration of drugs such as cyclosporine and topical latanoprost. We hereby report a rare case of acquired eyelash trichomegaly with diffuse hair loss and "lupus hairs" on the scalp in a 16-year-old female diagnosed with systemic lupus erythematosus...
April 2017: International Journal of Trichology
https://www.readbyqxmd.com/read/28790229/autoimmune-disease-with-cardiac-valves-involvement-libman-sacks-endocarditis
#8
Eka Ginanjar, Yulianto Yulianto
This case study aim to evaluate the response of steroid treatment for autoimmune endocarditis. Valvular heart disease is relatively rising in both congenital and acquired cases, but the autoimmune endocarditis remains rare. In this case, a 34 year old woman with clinical manifestation resembling systemic lupus erythematosus (SLE) is diagnosed with Libman-sacks Endocarditis. After six months of steroid treatment, her clinical manifestations and heart structure improved.
April 2017: Acta Medica Indonesiana
https://www.readbyqxmd.com/read/28730136/early-diagnosis-of-abcb11-spectrum-liver-disorders-by-next-generation-sequencing
#9
Su Jeong Lee, Jung Eun Kim, Byung-Ho Choe, An Na Seo, Han-Ik Bae, Su-Kyeong Hwang
PURPOSE: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. METHODS: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling...
June 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28709760/clinical-and-pathologic-implications-of-extending-the-spectrum-of-maternal-autoantibodies-reactive-with-ribonucleoproteins-associated-with-cutaneous-and-now-cardiac-neonatal-lupus-from-ssa-ro-and-ssb-la-to-u1rnp
#10
REVIEW
Peter M Izmirly, Marc K Halushka, Avi Z Rosenberg, Sean Whelton, Khodayar Rais-Bahrami, Dilip S Nath, Hilary Parton, Robert M Clancy, Sara Rasmussen, Amit Saxena, Jill P Buyon
While the relationship between maternal connective tissue diseases and neonatal rashes was described in the 1960s and congenital heart block in the 1970s, the "culprit" antibody reactivity to the SSA/Ro-SSB/La ribonucleoprotein complex was not identified until the 1980s. However, studies have shown that approximately 10-15% of cases of congenital heart block are not exposed to anti-SSA/Ro-SSB/La. Whether those cases represent a different disease entity or whether another antibody is associated has yet to be determined...
July 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28649905/juvenile-idiopathic-arthritis-in-a-patient-with-previous-diagnosis-of-severe-congenital-lupus
#11
I H Rotstein Grein, W Armbrust, A M van der Molen, A van Royen-Kerkhof
Neonatal lupus (NL) is an acquired autoimmune disease of the newborn, caused by transplacental passage of the maternal autoantibodies anti SS-A/Ro and anti SS-B/La. When the clinical picture starts directly at birth, it is known as congenital lupus (CL). The clinical manifestations are variable. Except for cardiac involvement, the other manifestations tend to be benign, and resolve with the child's clearance of maternal antibodies. We report a patient who presented at birth with very severe involvement of the skin, and subsequent contractures of hands and feet, leading to functional impairment...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28598777/no-histologic-evidence-of-foetal-cardiotoxicity-following-exposure-to-maternal-hydroxychloroquine
#12
Deborah Friedman, Leif Lovig, Marc Halushka, Robert M Clancy, Peter M Izmirly, Jill P Buyon
It is currently recommended that hydroxychloroquine (HCQ) be maintained during pregnancy in patients with systemic lupus erythematosus. Recent data suggest that this Toll-like receptor inhibitor may also reduce the recurrence rate of anti-SSA/Ro associated congenital heart block (CHB). This case report describes a unique situation in which a CHB-afflicted, HCQ-exposed pregnancy was electively terminated. The heart did not reveal any characteristic features of cardiotoxicity, providing further evidence supporting the safety of foetal exposure to HCQ...
September 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28585599/discordant-disease-expression-of-neonatal-lupus-erythematosus-in-twins
#13
Faiza Mustafa, Yawar Najam, Mohammad Tauqeer Akbar, Tahir Aziz Ahmed
Neonatal lupus erythematosus is an autoimmune disease resulting from the trans-placental passage of maternal anti-SSA/Ro, anti-SSB/La, and less frequently anti-RNP antibodies to the foetus. At the time of diagnosis 50% of mothers are asymptomatic. Neonatal manifestations of this multisystem disease may include congenital heart block, cutaneous lesions and haematological abnormalities. We present the case of congenital neonatal lupus erythematosus in non-identical twins, showing variability in clinical manifestation of this disease,despite receiving the same level of antibodies from the mother...
June 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28546762/cardiovascular-magnetic-resonance-imaging-clinical-implications-in-the-evaluation-of-connective-tissue-diseases
#14
REVIEW
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Genovefa Kolovou
Cardiovascular magnetic resonance imaging is a recently developed noninvasive, nonradiating, operator-independent technique that has been successfully used for the evaluation of congenital heart disease, valvular and pericardial diseases, iron overload, cardiomyopathies, great and coronary vessel diseases, cardiac inflammation, stress-rest myocardial perfusion, and fibrosis. Rheumatoid arthritis and other spondyloarthropathies, systemic lupus erythematosus, inflammatory myopathies, mixed connective tissue diseases (CTDs), systemic sclerosis, vasculitis, and sarcoidosis are among CTDs with serious cardiovascular involvement; this is due to multiple causative factors such as myopericarditis, micro/macrovascular disease, coronary artery disease, myocardial fibrosis, pulmonary hypertension, and finally heart failure...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28532196/fetal-and-neonatal-involvement-in-maternal-rheumatologic-disease
#15
Shigeo Iijima
A pregnancy complicated with rheumatologic diseases can have various influences on the fetus and/or neonate. Maternal systemic lupus erythematosus (SLE) may cause preterm and/or small for gestational age (SGA) delivery and neonatal lupus (NL). Some neonates with NL have congenital heart block (CHB) with increased morbidity and mortality, even requiring pacemakers. Antiphospholipid syndrome may occur with SLE and affect fetal and/or neonatal outcomes. Pregnancy involving primary Sjögren's syndrome (pSS) tends to result in preterm delivery and low birthweight infants...
June 8, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28512017/a-case-of-autoimmune-severe-acquired-von-willebrand-syndrome-type-3-like
#16
Chakri Gavva, Prapti Patel, Yu-Min Shen, Eugene Frenkel, Ravi Sarode
Von Willebrand disease (VWD) is the most common congenital bleeding disorder and is due to quantitative or qualitative defects of von Willebrand factor (VWF). Acquired defects of VWF, termed acquired von Willebrand syndrome (AVWS), are due to a host of different mechanisms. Autoantibody-mediated AVWS may be associated with lymphoproliferative or immunological disorders, such as systemic lupus erythematosus (SLE). A large majority of AVWS cases are type 1 or type 2A-like and patients tend to have a mild to moderate bleeding tendency...
June 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28474658/spontaneous-massive-hemopneumothorax-double-trouble-with-a-twist
#17
Milta Kuriakose, Arjun Khanna, Deepak Talwar
Spontaneous hemopneumothorax (SHP) is observed in 3%-7% cases of spontaneous pneumothorax where the tear of an adhesion can lead to bleeding with associated hemothorax. This condition has been reported in patients with hemophilia, sarcoidosis, congenital cystic adenomatoid malformation, systemic lupus erythematosus, etc., Here, we describe an unusual case of acute massive SHP in a 62-year-old male who underwent a percutaneous transluminal coronary angioplasty (PTCA) and presented with worsening dyspnea over the next 3 days...
May 2017: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/28331377/management-of-systemic-lupus-erythematosus-during-pregnancy-challenges-and-solutions
#18
REVIEW
Caroline L Knight, Catherine Nelson-Piercy
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal-neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Effective prepregnancy risk assessment and counseling includes exploration of factors for poor pregnancy outcome, discussion of risks, and appropriate planning for pregnancy, with consideration of discussion of relative contraindications to pregnancy...
2017: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/28256367/impact-of-systemic-lupus-erythematosus-on-maternal-and-fetal-outcomes-following-pregnancy-a-meta-analysis-of-studies-published-between-years-2001-2016
#19
REVIEW
Pravesh Kumar Bundhun, Mohammad Zafooruddin Sani Soogund, Feng Huang
BACKGROUND: Previous research has already shown systemic lupus erythematosus (SLE) to have severe consequences on pregnancy outcomes. However, insufficient number of participants, which were mainly limited to one particular region, limited outcomes analyzed and lack of evidence based analysis to support systematic reviews of the literature were the limitations observed. Therefore, by improving these limitations, we aimed to systematically show the impact of SLE on maternal and fetal outcomes following pregnancy...
May 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#20
REVIEW
Antonio Alberto Zuppa, Riccardo Riccardi, Simonetta Frezza, Francesca Gallini, Rita Maria Paola Luciano, Giovanni Alighieri, Costantino Romagnoli, Sara De Carolis
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur...
April 2017: Autoimmunity Reviews
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