keyword
https://read.qxmd.com/read/38508435/subclinical-maternal-autoimmune-disease-leading-to-congenital-high-degree-atrioventricular-block-case-report-and-review-of-the-literature
#1
Shelby E Walcott, Dr Christian H Tan, Dr Jason Wicker, Dr Silvio Litovsky
Maternal autoimmune disease is the most common cause of congenital heart block (CHB), a rare illness characterized by fibrosis and calcification of the fetal atrioventricular (AV) node due to maternal autoantibodies anti-SSA/Ro and anti-SSB/La. We report the full autopsy and clinical information on a female neonate with high degree AV block and calcification in the AV node, atrial approaches to the AV node, and both right and left bundle branches, born to a 27-year-old female with subclinical autoimmune disease...
March 18, 2024: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://read.qxmd.com/read/38507328/prevalence-of-neonatal-lupus-in-a-small-group-of-antibody-carrying-mothers-and-frequency-of-complete-atrioventricular-block
#2
JOURNAL ARTICLE
Héctor González-Zambrano, Gerardo Orozco-Barocio, María C Franco-Ramos
BACKGROUND: Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. OBJECTIVE: To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. METHODS: From January 1992 to December 2017, patients with CCAB were included...
2024: Archivos de Cardiología de México
https://read.qxmd.com/read/38479815/hydroxychloroquine-in-lupus-or-rheumatoid-arthritis-pregnancy-and-risk-of-major-congenital-malformations-a-population-based-cohort-study
#3
JOURNAL ARTICLE
Ngoc V Nguyen, Elisabet Svenungsson, Annica Dominicus, Maria Altman, Karin Hellgren, Julia F Simard, Elizabeth V Arkema
OBJECTIVES: To assess the infant risk of major congenital malformations (MCM) associated with first-trimester exposure to hydroxychloroquine (HCQ) among mothers with systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA). METHODS: This population-based cohort study utilised Swedish nationwide registers and included all singleton births (2006-2021) among individuals with prevalent SLE or RA in Sweden. The exposure was filling ≥1 HCQ prescription during the first trimester...
March 13, 2024: Rheumatology
https://read.qxmd.com/read/38345986/a-compound-heterozygosis-of-two-novel-mutations-in-vwf-exacerbates-vwd-in-a-chinese-pedigree
#4
JOURNAL ARTICLE
Jiajia Hu, He Yu, Wuchao Wang, Jinfang Li, Qinzhu Xu, Jiang Rong, Songsong Lu
BACKGROUND: von Willebrand disease (vWD), caused by mutations in the von Willebrand factor (vWF) coding gene, is a disease characterized by abnormal coagulation activity and a severe tendency for hemorrhage. Therefore, identifying mutations in vWF is important for diagnosing congenital vWD. METHODS: We studied a 23-year-old male vWD patient and his parents. Clotting methods were used to determine activated partial thromboplastin time (aPTT), prothrombin time (PT), fibrinogen (FIB) levels, FVIII activity...
February 1, 2024: Clinical Laboratory
https://read.qxmd.com/read/38252597/a-case-of-systemic-lupus-erythematosus-in-a-patient-with-noonan-syndrome-with-recurrent-severe-hypoglycemia
#5
JOURNAL ARTICLE
Shotaro Masuoka, Takashi Tanaka, Miwa Kanaji, Karin Furukawa, Keiko Koshiba, Zento Yamada, Eri Watanabe, Mai Kawazoe, Shun Ito, Ayako Fuchigami, Toshihiro Nanki
Noonan syndrome (NS) is a dominantly inherited genetic disorder with mutations in genes encoding components or regulators of the RAS/mitogen-activated protein kinase pathway. Its diagnosis is based on characteristic features, including typical facial features, a short stature, congenital heart disease, mild developmental delay, and cryptorchidism. Patients with NS sometimes develop autoimmune diseases, such as Hashimoto's thyroiditis and, rarely, systemic lupus erythematosus (SLE). We herein present a 29-year-old Japanese female with NS complicated by SLE and repeated severe hypoglycemia...
January 22, 2024: Modern rheumatology case reports
https://read.qxmd.com/read/38228120/preliminary-evaluation-of-in-vivo-reflectance-confocal-microscopy-features-of-neonatal-lupus-erythematosus
#6
JOURNAL ARTICLE
Ying Wang, LiXin Chen, Bei Qin, Xibo Gao, Qinfeng Li
BACKGROUND: Neonatal lupus erythematosus (NLE) is a rare autoimmune disease,which need to distinguish from eczema, congenital syphilis, and tinea corporis in newborns. Reflectance confocal microscopy (RCM) could be a helpful noninvasive diagnostic tool,which has been used to evaluate several inflammatory skin conditions. The aim of this study was to describe the RCM characteristics of NLE. METHODS: 9 NLE patients were included in the study,all patients were evaluated clinically with RCM...
January 16, 2024: Dermatology: International Journal for Clinical and Investigative Dermatology
https://read.qxmd.com/read/38125818/the-risk-of-pediatric-cardiovascular-diseases-in-offspring-born-to-mothers-with-systemic-lupus-erythematosus-a-nationwide-study
#7
JOURNAL ARTICLE
Jong Ho Cha, Jae Kyoon Hwang, Young-Jin Choi, Jae Yoon Na
BACKGROUND: Systemic lupus erythematosus (SLE), a common autoimmune disease predominantly affecting women, has been linked to various complications during pregnancy. The transfer of anti-Ro/SSA antibodies from SLE-affected mothers to their offspring can lead to neonatal lupus and cardiac issues. This study investigated the association between maternal SLE and the risk of pediatric cardiovascular disorders. METHODS: The study utilized South Korea's National Health Insurance Service (NHIS) database, covering 3,505,737 children born between 2007 and 2017 and tracked until 2020...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/38057018/one-advantageous-reflection-of-iron-metabolism-in-context-of-normal-physiology-and-pathological-phases
#8
REVIEW
Niraj Kumar Srivastava, Somnath Mukherjee, Vijaya Nath Mishra
PURPOSE (BACKGROUND): The presented review is an updating of Iron metabolism in context of normal physiology and pathological phases. Iron is one of the vital elements in humans and associated into proteins as a component of heme (e.g. hemoglobin, myoglobin, cytochromes proteins, myeloperoxidase, nitric oxide synthetases), iron sulfur clusters (e.g. respiratory complexes I-III, coenzyme Q10, mitochondrial aconitase, DNA primase), or other functional groups (e.g. hypoxia inducible factor prolyl hydroxylases)...
December 2023: Clinical Nutrition ESPEN
https://read.qxmd.com/read/38021813/pregnancy-and-inflammatory-rheumatological-diseases-a-single-center-retrospective-cohort-study
#9
JOURNAL ARTICLE
Abeer A Alkhodier, Abdurhman S Alsaif, Norah H Alqntash, Rakan B Alanazi, Ghaida Alotaibi, Abdulrahman Alrashid
Background Rheumatic diseases pose risks to pregnant women, leading to complications like preterm birth, congenital heart block, and pregnancy loss. These diseases are expected to deteriorate during pregnancy and further in the postpartum period. The impact of these diseases on the pregnancy will add further burden on the patient, fetus, physician, and healthcare system. Advances in diagnosis and treatment have improved outcomes making them similar to that of healthy women, but close follow-up in a multidisciplinary clinic is essential...
October 2023: Curēus
https://read.qxmd.com/read/37975160/expert-perspective-on-a-clinical-challenge-lupus-and-pregnancy
#10
JOURNAL ARTICLE
Laura Tarter, Bonnie L Bermas
Systemic lupus erythematosus (SLE), a multiorgan systemic inflammatory disorder, predominantly affects women during their reproductive years. In this review, we summarize the state of knowledge about preconception planning and management of SLE during pregnancy. Achieving remission or low disease activity for several months on medications compatible with pregnancy prior to conception is essential to decreasing the risk of disease flare and improving pregnancy outcomes, including pre-eclampsia, preterm birth, and intrauterine growth restriction...
November 17, 2023: Arthritis & Rheumatology
https://read.qxmd.com/read/37965190/a-case-of-neonatal-lupus-erythematosus-presenting-with-extensive-erosions-at-birth-healing-with-extensive-scarring
#11
Sheau Yun Kan, Sridhar Arunachalam, Selina Ho, Chong Chia Yin, Thaschawee Arkachaisri, Mark Jean Aan Koh
No abstract text is available yet for this article.
December 2023: JAAD Case Reports
https://read.qxmd.com/read/37946215/reverse-complete-heart-block-using-transcutaneous-pacing-and-repeated-plasmapheresis-in-a-neonate-with-lupus-a-case-report
#12
JOURNAL ARTICLE
Yanfei Liu, Wanwei Li, Kun Zhou, Zhangxue Hu
BACKGROUND: It has been reported that the complete heart block (CHB) in neonatal lupus (NL) cannot be reversed. This study reported a case of NL-CHB that was reversed by transcutaneous pacing and repeated plasmapheresis. CASE PRESENTATION: A 35+ 6 -week male preterm baby was transferred to the neonatal intensive care unit of the Army Medical Center in May 2020 for slight cyanosis around the lips and nose. Two days after birth, a sudden decrease in heart rate was observed during electrocardiogram (EGG) monitoring...
November 9, 2023: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/37945182/association-between-toxoplasmosis-and-autoimmune-rheumatic-diseases-in-egyptian-patients
#13
JOURNAL ARTICLE
Wafaa A Aboukamar, Samar Habib, Samar Tharwat, Mohamed Kamal Nassar, Manal A Elzoheiry, Rania Atef, Manar S Elmehankar
PURPOSE: To explore the association between T. gondii and autoimmune rheumatic diseases (ARDs). METHODS: This study involved 82 patients with ARDs: 44 rheumatoid arthritis (RA), 28 systemic lupus erythematosus (SLE), and 10 systemic sclerosis (SSc) and 61 age- and sex-matched controls. Sociodemographic, clinical, and laboratory data were collected, and disease activity was assessed. Exposure to toxoplasmosis risk factors was investigated. Serological tests for anti-Toxoplasma IgM and IgG antibodies were assessed using ELISA...
November 2023: Reumatología clinica
https://read.qxmd.com/read/37881400/congenital-heart-block-and-its-association-with-anti-ro-and-anti-la-antibodies-in-pregnancy-a-case-report-of-a-rare-entity-and-a-review-of-the-current-evidence
#14
Ioakeim Sapantzoglou, Zacharias Fasoulakis, George Daskalakis, Marianna Theodora, Panagiotis Antsaklis
Systemic lupus erythematosus (SLE) is a heterogeneous chronic, multisystem, inflammatory autoimmune disorder with variable clinical features, with its manifestations being attributed to the presence of multiple autoantibodies and their subsequent autoimmune reactions. Multiple organs may be involved, with the kidneys, the joints, and the skin being the most common, increasing maternal and fetal morbidity and mortality. Our current article describes the case of a 32-year-old primigravida who was referred to our department after the detection of fetal bradycardia and the strong suspicion of an underlying cardiac abnormality...
September 2023: Curēus
https://read.qxmd.com/read/37840528/the-prognostic-value-of-neutrophile-lymphocyte-ratio-and-serum-chemerin-to-predict-maternal-fetal-complications-in-pregnant-systemic-lupus-erythematosus-patients
#15
JOURNAL ARTICLE
Gabriela Medina, Francisco R González-Cortés, Antonio Sánchez-González, Juan G Vázquez-Rodríguez, Irvin Ordoñez-González, María P Jiménez-Arellano, Grettel García-Collinot, Susana I Morales-Montalvo, Oscar I Florez-Durante, María P Cruz-Domínguez, María F Colorado-Cruz, Miguel A Zurita-Muñoz, Gilberto Cruz-Arteaga, Carolina Sánchez-Enriquez, Luis J Jara, Miguel Á Saavedra
BACKGROUND: Pregnancy in SLE continues to be a challenge. The neutrophil-to-lymphocyte ratio (NLR) and chemerin are predictors of preeclampsia in the general population; however, their role as predictors of maternal-fetal complications in pregnant SLE patients has not been analyzed. OBJECTIVE: To investigate the prognostic value of NLR and serum chemerin, to predict maternal-fetal complications in pregnant SLE patients, and compare both biomarkers among three study groups...
October 2023: Lupus
https://read.qxmd.com/read/37767540/update-on-hydroxychloroquine-use-in-pregnancy
#16
JOURNAL ARTICLE
Wassan Nori, Nabeeha Najatee Akram, Raid M Al-Ani
It is well-known that hydroxychloroquine (HCQ) treats malaria, systemic lupus erythematosus, and rheumatoid arthritis in women for its immunomodulatory and anti-inflammatory action. Additionally, HCQ was used in cases with refractory antiphospholipid syndrome. HCQ safety was reinforced in pregnant women owing to insignificant reports of adverse pregnancy outcomes and major congenital malformation. Recently, HCQ was tested in cases with chronic placental inflammation with a promising result of increased life birth; however, its benefit needs further validation...
September 20, 2023: World Journal of Experimental Medicine
https://read.qxmd.com/read/37701161/nail-whispers-revealing-dermatological-and-systemic-secrets-an-analysis-of-nail-disorders-associated-with-diverse-dermatological-and-systemic-conditions
#17
JOURNAL ARTICLE
Mansi Satasia, Amita H Sutaria
Background and objective Nail disorders encompass a wide spectrum of conditions, spanning congenital, developmental, infectious, neoplastic, degenerative, dermatological, and systemic diseases. A comprehensive exploration of their clinical manifestations, incidence, and associations is crucial for precise diagnosis and effective management. Methods This observational cross-sectional study conducted at B.J. Medical College and Civil Hospital, Ahmedabad involved 300 consecutive patients with nail changes from July 2017 to June 2019 reporting diverse dermatological and systemic conditions...
September 2023: Curēus
https://read.qxmd.com/read/37587864/intrapancreatic-accessory-spleen-diagnosed-by-size-reduction-after-immunosuppressive-therapy-a-case-report
#18
Turgut Tursem Tokmak, Mahmut Burak Lacin, Humeyra Gencer
BACKGROUND: Intrapancreatic accessory spleen (IPAS) is a congenital entity that can be confused with malignant distal pancreatic masses. Radiologic imaging and radionuclide imaging have an important place in the diagnosis of IPAS. CASE REPORT: Blood tests were performed on a 36-year-old female patient who presented with tachypnea, tachycardia, pain in the joints, and pain in the left abdominal quadrant. Laboratory test results were as follows: hemoglobin value 6...
August 15, 2023: Current medical imaging
https://read.qxmd.com/read/37546163/neonatal-lupus-erythematosus-manifested-as-a-complete-heart-block-a-case-report
#19
Siddinath Gyawali, Suraj Prasad Gupta Rauniyar, Bindu Gyawali, Tapasya Bhusal, Srijana Basnet
KEY CLINICAL MESSAGE: Fetal bradycardia and congenital complete heart block could be the presentation of neonatal lupus. A high index of suspicion of this condition helps to identify an asymptomatic mother. ABSTRACT: Neonatal lupus erythematosus (NLE) is a rare acquired autoimmune disease of newborns due to placental transfer of Ro/SSA or La/SSB autoantibodies. Though cardiac, cutaneous, hematological, and hepatobiliary abnormalities are detected, cardiac defects cause significant morbidity and mortality...
August 2023: Clinical Case Reports
https://read.qxmd.com/read/37490686/acquired-epidermolysis-bullosa-in-a-pediatric-patient
#20
JOURNAL ARTICLE
Rosario Torres-Iberico, Katia Henostroza-Inga, Rosa Pérez-Córdova, Roxana Lipa-Chancolla
BACKGROUND: Acquired epidermolysis bullosa is a rare and chronic autoimmune subepidermal bullous disease characterized by the formation of autoantibodies against type VII collagen. Presentation in childhood is rare and with several manifestations. CASE REPORT: We report the case of a 12-year-old female patient who presented bullous and polymorphic lesions on the chest and extremities of several months of evolution. Due to the characteristics of the skin lesions, a histopathological and direct immunofluorescence study was conducted, confirming the diagnosis of acquired epidermolysis bullosa...
2023: Boletín Médico del Hospital Infantil de México
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