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Congenital lupus

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https://www.readbyqxmd.com/read/29617782/epicardial-permanent-pacemaker-implantation-in-the-retrosternal-space-of-a-2-3-kg-infant
#1
Bongyeon Sohn, Jae Gun Kwak, Jae Hong Lim, Woong-Han Kim
Permanent pacemaker implantation in small infants can be challenging because of patient size and expected somatic growth. In our case, we used the retrosternal space as a generator pocket for an extremely low-birth-weight premature baby with autoantibody-associated congenital heart block born to a systemic lupus erythematosus mother.
March 29, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29576632/maternal-thrombophilia-and-recurrent-miscarriage-is-there-evidence-that-heparin-is-indicated-as-prophylaxis-against-recurrence
#2
Ana-Luisa Stefanski, Christoph Specker, Rebecca Fischer-Betz, Wolfgang Henrich, Ekkehard Schleussner, Thomas Dörner
Background: Recurrent miscarriage, also referred to as recurrent spontaneous abortion (RSA), affects 1 - 5% of couples and has a multifactorial genesis. Acquired and congenital thrombophilia have been discussed as hemostatic risk factors in the pathogenesis of RSA. Method: This review article was based on a selective search of the literature in PubMed. There was a special focus on the current body of evidence studying the association between RSA and antiphospholipid syndrome and hereditary thrombophilia disorders...
March 2018: Geburtshilfe und Frauenheilkunde
https://www.readbyqxmd.com/read/29538673/recurrent-pregnancy-loss-evaluation-combined-with-24-chromosome-microarray-of-miscarriage-tissue-provides-a-probable-or-definite-cause-of-pregnancy-loss-in-over-90-of-patients
#3
F Popescu, C R Jaslow, W H Kutteh
STUDY QUESTION: Will the addition of 24-chromosome microarray analysis on miscarriage tissue combined with the standard American Society for Reproductive Medicine (ASRM) evaluation for recurrent miscarriage explain most losses? SUMMARY ANSWER: Over 90% of patients with recurrent pregnancy loss (RPL) will have a probable or definitive cause identified when combining genetic testing on miscarriage tissue with the standard ASRM evaluation for recurrent miscarriage...
March 12, 2018: Human Reproduction
https://www.readbyqxmd.com/read/29520672/features-associated-with-pulmonary-arterial-hypertension-in-chinese-hospitalized-systemic-lupus-erythematosus-patients
#4
Shu-Zhen Xu, Yan Liang, Xiang-Pei Li, Xiao-Mei Li, Zong-Wen Shuai, Rui-Xue Leng, Hai-Feng Pan, Dong-Qing Ye
Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of hospitalized SLE patients. We have collected the medical records of patients hospitalized with SLE at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital. Resting transthoracic echocardiography (TTE) was used to estimate pulmonary artery pressure (PAP) and PAH was defined as systolic PAP (PASP) > 30 mmHg...
March 8, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29483988/van-lohuizen-syndrome-a-case-report-with-a-diagnostic-delay-of-four-years
#5
Uwe Wollina, Katlein França, Torello Lotti, Georgi Tchernev
BACKGROUND: Cutis marmorata telangiectatic congenital or Van Lohuizen syndrome is a rare vascular disorder that may be associated with other congenital malformations. Around 300 cases have been reported so far. CASE REPORT: We present a 4-year-old girl with Van Lohuizen syndrome of the leg, but without any other malformations. CONCLUSION: Neonatal lupus erythematosus may resemble congenital vasculopathy, but histopathology and immune-serology are characteristic...
January 25, 2018: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29468569/illness-identity-in-adults-with-a-chronic-illness
#6
Leen Oris, Koen Luyckx, Jessica Rassart, Liesbet Goubert, Eva Goossens, Silke Apers, Seher Arat, Joris Vandenberghe, René Westhovens, Philip Moons
The present study examines the concept of illness identity, the degree to which a chronic illness is integrated into one's identity, in adults with a chronic illness by validating a new self-report questionnaire, the Illness Identity Questionnaire (IIQ). Self-report questionnaires on illness identity, psychological, and physical functioning were assessed in two samples: adults with congenital heart disease (22-78 year old; n = 276) and with multisystem connective tissue disorders (systemic lupus erythematosus or systemic sclerosis; 17-81 year old; n = 241)...
February 21, 2018: Journal of Clinical Psychology in Medical Settings
https://www.readbyqxmd.com/read/29314753/functional-mannose-binding-lectin-levels-in-patients-with-pediatric-onset-systemic-lupus-erythematosus-in-remission
#7
Siyaram Didel, Deepti Suri, Amit Rawat, Surjit Singh
BACKGROUND: Complement system plays a crucial role in the pathogenesis of systemic lupus erythematosus (SLE). Excessive activation of complement causes tissue injury and SLE occurrence is typically associated with congenital deficiencies of the early components of the classical complement pathway. Mannose-binding lectin (MBL) is recognized as important causative factor in the etio-pathogenesis of SLE and MBL-2 gene has evolved as a candidate gene for SLE susceptibility. AIM: This study was done to assess the functional MBL (fMBL) level in SLE patients in remission and its co-relation with disease severity and outcome...
March 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29224680/pregnancy-in-systemic-lupus-erythematosus-and-antiphospholipid-syndrome
#8
REVIEW
Rebecca Fischer-Betz, Christof Specker
Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with a high prevalence in females of childbearing age. Pregnancy in SLE nowadays has favorable outcomes for the majority of women. However, flares of disease activity, preeclampsia, fetal loss, and preterm birth are well-known risks in such pregnancies. Anti-SS-A(Ro)/SS-B(La) antibodies put fetuses at risk for congenital heart block and neonatal lupus. Several risk factors for adverse pregnancy outcomes have been identified. Women with antiphospholipid antibodies or antiphospholipid syndrome and lupus nephritis represent a group with high risk for obstetric complications...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29027258/acquired-factor-xi-deficiency-and-therapeutic-plasma-exchange
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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...
September 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28649905/juvenile-idiopathic-arthritis-in-a-patient-with-previous-diagnosis-of-severe-congenital-lupus
#19
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 2018: Lupus
https://www.readbyqxmd.com/read/28598777/no-histologic-evidence-of-foetal-cardiotoxicity-following-exposure-to-maternal-hydroxychloroquine
#20
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
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