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Congenital lupus

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https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#1
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...
February 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28196317/common-skin-conditions-in-children-noninfectious-rashes
#2
Brian Z Rayala, Dean S Morrell
Cutaneous adverse drug reactions are among the most common noninfectious rashes of childhood. Cutaneous adverse drug reactions are classified as morbilliform, urticarial, bullous, pustular, or psoriasiform. Atopic dermatitis is one of the most common inflammatory cutaneous eruptions, and is characterized by pruritus and flexural distribution. Emollients and topical corticosteroids are first-line therapies. Topical calcineurin inhibitors are second-line, steroid-sparing drugs for certain conditions, such as face and eyelid eczema...
February 2017: FP Essentials
https://www.readbyqxmd.com/read/28179389/homonymous-hemianopia-in-the-primary-antiphospholipid-syndrome
#3
Derek Kwun-Hong Ho, Rishi Ramessur, Mradul Gupta, John P Mathews
A woman aged 26 years was referred by her GP to the eye casualty department with sudden-onset left homonymous hemianopia and right-sided headache. Full ophthalmic examination was normal with the exception of a left homonymous hemianopia confirmed with automated perimetry. Urgent CT imaging revealed a non-haemorrhagic cerebral infarct in the right parieto-occipital region. Subsequent blood tests confirmed a diagnosis of antiphospholipid syndrome with positivity in IgG anticardiolipin antibody, IgG anti-β2-GP1 antibody and the Lupus anticoagulant screen...
February 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28179384/a-complicated-multisystem-flare-of-systemic-lupus-erythematosus-during-pregnancy
#4
Philip Webster, Catherine Nelson-Piercy, Liz Lightstone
We report a case of systemic lupus erythematosus (SLE) in a young woman who became pregnant amid a severe flare. She continued to have active disease in the face of aggressive treatments complicated by several side effects of immunosuppressive drugs including recurrent sepsis and gestational diabetes. Her fetus was at risk for congenital heart block during the second and third trimesters. Despite an extremely guarded prognosis, she delivered a healthy baby girl. This case highlights the complexities of SLE management during pregnancy...
February 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28138914/obstetric-and-perinatal-outcome-in-anti-ro-ssa-positive-pregnant-women-a-prospective-cohort-study
#5
Nuria Martínez-Sánchez, Sergio Pérez-Pinto, Ángel Robles-Marhuenda, Francisco Arnalich-Fernández, María Martín Cameán, Edurne Hueso Zalvide, Jose Luis Bartha
: Anti-Ro/SS-A is one specific type of antinuclear antibodies. They are in the majority of cases associated with primary Sjögren syndrome (SS) but also in Systemic Lupus Erythematosus (SLE), rheumatoid arthritis (RA), and in healthy people. During pregnancy, they are mainly associated to congenital heart block (CHB) and neonatal lupus (NL). The aim of this study was to compare the rate of maternal and fetal complications between a series of anti-Ro/SS-A positive pregnant women prospectively followed...
January 30, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28118945/ro52-autoantibodies-arise-from-self-reactive-progenitors-in-a-mother-of-a-child-with-neonatal-lupus
#6
Joanne H Reed, Miroslaw K Gorny, Liuzhe Li, Timothy Cardozo, Jill P Buyon, Robert M Clancy
The detection of cardiac conduction defects in an 18-24 week old foetus in the absence of structural abnormalities predicts with near certainty the presence of autoantibodies against 60kD and 52kD SSA/Ro in the mother regardless of her health status. Previous studies have emphasized these autoantibodies as key mediators of tissue injury. The aim of this study was to focus on the anti-Ro52 response to determine whether these autoantibodies originate from progenitors that are inherently self-reactive or from B-cells that acquire self-reactivity during an immune response...
January 21, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28106024/-a-rare-cause-of-2-1-atrioventricular-block-and-congestive-heart-failure-in-preterm-infants-hypocalcemia
#7
Emine Azak, Hatice Tatar Aksoy, Handan Ünsal, İbrahim İlker Çetin
Atrioventricular (AV) block in the neonatal period is a rare disorder. It is frequently associated with underlying structural congenital heart disease and maternal lupus. Presently described is premature baby who developed 2:1 AV block and congestive heart failure due to hypocalcemia. Dramatic clinical improvement was observed following treatment of intravenous 10% calcium gluconate. Therefore, it is suggested that serum calcium level of newborns with AV block and congestive heart failure be measured.
January 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28092260/-down-syndrome-and-heart-block-secondary-to-neonatal-lupus
#8
Carla Lucila Illana-Bravo, Socorro Méndez-Martínez, María Enriqueta Juan-Martínez
In Down syndrome, so far, has not been reported it's association with congenital heart block, this entity is rare and occurred in only 1% of mothers who have systemic lupus erythematosus, the presence of anti-Ro antibodies cross the placenta presenting neonatal lupus with skin lesions and congenital heart block, bradycardia, which is why we describe the following case. This is a new male end product of asymptomatic young mother, but serological birth with stigmata of Down syndrome, birth presents congenital bradycardia rare manifestation abnormalities, but common in children of mothers with lupus are initiated study protocol, realizing you including laboratory tests and immunological studies cabinet as electrocardiogram and echocardiogram, which gave tone to take the mother immunological studies, being positive antiRo...
January 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28051234/neonatal-lupus-erythematosus-with-congenital-heart-block-in-twins
#9
Lamia Gargouri, Faiza Safi, Bayen Maalej, Souad Mallek, Fatma Turki, Imen Majdoub, Malek Akrout, Dorra Abid, Samir Kamoun, Abdelmajid Mahfoudh
Background - Neonatal lupus erythematosus is an uncommon acquired autoimmune disease caused by transplacental passage of maternal antibodies SSA/Ro, SSB/La or U1 ribonucleoproteins. The most common clinical manifestations are skin rash, cardiac lesions, thrombocytopenia, anemia and hepatosplenomegaly. Complete congenital heart block is usually irreversible needing a pacemaker implantation in two-thirds of cases. Cases report - We report neonatal lupus erythematosus with complete congenital heart block in twins...
July 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27924730/congenital-malformations-attributed-to-prenatal-exposure-to-cyclophosphamide
#10
Padmanabhan Rengasamy
Cyclophosphamide (CPA) remains one of the most widely prescribed anticancer drugs. It is also used in the treatment of rheumatoid arthritis, childhood nephrotic syndrome and systemic lupus erythematosus. It is a potent immunosuppressive agent. It is commonly used in blood and bone marrow transplantation. With the growing trend among women postponing childbearing, the number of women who are diagnosed with breast cancer is also increasing thus escalating the chances of exposure of the unborn child to antineoplastic drugs...
December 6, 2016: Anti-cancer Agents in Medicinal Chemistry
https://www.readbyqxmd.com/read/27922246/neonatal-lupus-erythematosus-report-of-a-case-with-cutaneous-hematological-and-hepatobiliary-findings
#11
Andaç Salman, Merve Hatun Sarıçam, Ayşe Deniz Yücelten, Cuyan Demirkesen, Tülin Ergun
Neonatal lupus erythematosus is an autoimmune disorder mainly affecting the heart and skin. It is the most common cause of congenital heart block. In addition, hematological, hepatobiliary and neurological involvement may occur. Herein, we report a 23-day-old infant presented with annular, erythematous, and scaly and atrophic lesions on the face and trunk. Based on the clinical, laboratory and histopathological findings, she was diagnosed as neonatal lupus erythematosus. Neonatal lupus eryhtematosus should be considered in infants presenting with annular skin lesions, and we present this case to highlight the value of high index of clinical suspicion in diagnosis...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27913462/pure-red-cell-aplasia
#12
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27881371/pure-red-cell-aplasia
#13
REVIEW
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
November 24, 2016: Blood
https://www.readbyqxmd.com/read/27803518/successful-preventive-treatment-of-congenital-heart-block-during-pregnancy-in-a-woman-with-systemic-lupus-erythematosus-with-anti-la-ro-antibody
#14
Vishwakarma Pratibha, Shankar Kundavi, Varma R Thangam, S Ramakrishnan
No abstract text is available yet for this article.
October 2016: Journal of Obstetrics and Gynaecology of India
https://www.readbyqxmd.com/read/27651588/correlation-of-maternal-autoantibodies-with-fetal-congenital-heart-block
#15
Dey Madhusudan, Agarwal Raju, Nambula Vijaya
BACKGROUND: Autoimmune fetal congenital heart block (CHB) is the most severe manifestation of neonatal lupus, and it is seen when maternal autoimmune antibodies cross the placenta and damage the AV node of the fetus. CHB is mainly associated with maternal SLE with anti-Ro/SSA- and anti-La/SSB-positive status, and incidence of CHB increases when both the antibodies are present. This study was conducted to know the incidence of fetal CHB in patients of SLE who had ANA, anti-Ro/SSA and anti-La/SSB positivity...
October 2016: Journal of Obstetrics and Gynaecology of India
https://www.readbyqxmd.com/read/27648302/proteolytic-inactivation-of-nuclear-alarmin-high-mobility-group-box-1-by-complement-protease-c1s-during-apoptosis
#16
J G Yeo, J Leong, T Arkachaisri, Y Cai, B H D Teo, J H T Tan, L Das, J Lu
Effective clearance of apoptotic cells by phagocytes prevents the release of intracellular alarmins and manifestation of autoimmunity. This prompt efferocytosis is complemented by intracellular proteolytic degradation that occurs within the apoptotic cells and in the efferosome of the phagocytes. Although the role of extracellular proteases in apoptotic cells clearance is unknown, the strong association of congenital C1s deficiency with Systemic Lupus Erythematosus highlights the protective nature that this extracellular protease has against autoimmunity...
2016: Cell Death Discovery
https://www.readbyqxmd.com/read/27635094/outcome-of-pregnancy-in-patients-with-systemic-lupus-erythematosis-at-korle-bu-teaching-hospital
#17
Ida Dzifa Dey, Jerry Coleman, Harriet Kwarko, Michael Mate-Kole
OBJECTIVE: To study maternal and fetal outcomes in Ghanaian women with systemic lupus erythematosus (SLE). METHODS: Retrospective study of pregnancies in women with SLE in a single centre in Ghana. RESULTS: The mean age was 30.1 years and all were nulliparous. Two out of the seven pregnancies were in disease remission at the time of booking. Nephritis without renal impairment was present in 7 pregnancies (6 women). One woman developed intrapartum eclampsia...
June 2016: Ghana Medical Journal
https://www.readbyqxmd.com/read/27510310/surgical-treatment-for-thoracoabdominal-intra-aortic-thrombus-with-multiple-infarctions-a-case-report
#18
Kenichiro Uchida, Mitsuharu Hosono, Toshihiko Shibata, Daisuke Kaku, Tomonori Yamamoto, Takafumi Terada, Naoki Shinyama, Yasumitsu Mizobata
BACKGROUND: Mobile intra-aortic thrombus without atherosclerosis, aneurysm, or congenital coagulopathy is very rare, and there are few reports especially in young or middle-aged patients. Furthermore, there are presently no established guidelines or common strategies for the treatment of mobile intra-aortic thrombus. In this case report, we describe the first case of intra-aortic thrombus caused by secondary erythrocytosis and describe the recommended treatment strategy for intra-aortic thrombus...
August 10, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27475948/neonatal-lupus-what-we-have-learned-and-current-approaches-to-care
#19
REVIEW
Marisa S Klein-Gitelman
Neonatal lupus results from the passive transfer of autoantibodies; however, this transfer is not sufficient to cause disease. This article reviews clinical presentation with a focus on autoimmune-mediated congenital heart disease. Recent data looking for additional disease mechanisms and biomarkers as well as latest information on interventions will be reviewed. Our understanding of this rare disease is often dependent on patient participation in disease registries and biorepositories. Future participation in registries including descriptive as well as biophysical data is critical to our knowledge...
September 2016: Current Rheumatology Reports
https://www.readbyqxmd.com/read/27387686/pregnancy-outcomes-in-women-with-childhood-onset-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study
#20
Miguel Ángel Saavedra, Dafhne Miranda-Hernández, Antonio Sánchez, Sara Morales, Pilar Cruz-Domínguez, Gabriela Medina, Luis Javier Jara
To compare the maternal and fetal outcomes between childhood-onset and adult-onset systemic lupus erythematosus (SLE), we reviewed the medical records of SLE pregnant women treated from January 2005 to August 2013. For comparison, patients were allocated to one of the two groups, those pregnant patients with SLE onset before 18 years of age (childhood-onset) and ≥18 years (adult-onset). The patients were evaluated at least once in each trimester and postpartum. Relevant maternal and fetal outcomes were extracted, such as lupus flare, preeclampsia/eclampsia, rate of liveborns, fetal loss (spontaneous abortion and stillbirth), term delivery, preterm birth, neonatal death, low birth weight, low birth weight at term, and congenital malformations...
October 2016: Rheumatology International
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