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Hydrops fetalis

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https://www.readbyqxmd.com/read/28727978/pulmonary-neuroendocrine-cell-hyperplasia-in-hemoglobin-bart-induced-hydrops-fetalis-a-model-for-chronic-intrauterine-hypoxia
#1
Mana Taweevisit, Boochit Theerasantipong, Kanlaya Taothong, Paul Scott Thorner
The pulmonary neuroendocrine system includes pulmonary neuroendocrine cells (PNECs) and neuroepithelial bodies (NEBs) that are distributed throughout respiratory epithelium and regulate lung growth and maturation antenatally. Abnormalities in this system have been linked to many hypoxia-associated pediatric pulmonary disorders. Hemoglobin (Hb) Bart disease is a severe form of α-thalassemia resulting in marked intrauterine hypoxia with hydrops fetalis (HF) and usually death in utero. Affected fetuses can serve as a naturally occurring human model for the effects of intrauterine hypoxia, and we postulated that these effects should include changes in the pulmonary neuroendocrine system...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28672054/the-prognostic-factors-of-hydrops-fetalis-with-pleural-effusion
#2
Atsushi Nakayama, Makoto Oshiro, Yasumasa Yamada, Tetsuo Hattori, Yasuhiro Wakano, Seiji Hayashi, Minoru Kokubo, Koji Takemoto, Shigeru Honda, Kuniko Ieda, Hikaru Yamamoto, Masanori Kouwaki, Kyoko Yokoi, Osamu Shinohara, Takenori Kato, Masafumi Miyata, Taihei Tanaka, Masahiro Hayakawa
BACKGROUND: Hydrops fetalis (HF) has low survival rate, particularly in cases of preterm birth. In addition, the severity index of HF has not been fully investigated yet. This study was aimed to clarify the prognostic factors of HF patients with pleural effusion. METHODS: All live-born HF patients with pleural effusion, except for chromosomal abnormality or complex congenital heart disease, born from 2009 to 2013 in Aichi Prefecture in Japan were included. The prenatal, perinatal, and postnatal information was obtained from their medical records and was retrospectively analyzed...
July 3, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28648573/personalized-treatment-with-immunoadsorption-and-intravenous-immunoglobulin-in-a-case-of-severe-rh-alloimmunization-during-pregnancy-unresponsive-to-plasma-exchange
#3
Anna Colpo, Tiziana Tison, Maria Teresa Gervasi, Cinzia Vio, Maria Vicarioto, Giustina De Silvestro, Piero Marson
INTRODUCTION: Despite prophylaxis, a small proportion of RhD-negative women may develop anti-D antibodies after a sensitizing event occurring during pregnancy or delivery of a D-positive baby. Intrauterine transfusion (IUT) is the treatment of choice in case of fetal anemia, but it cannot be performed early during pregnancy. Combined treatment with therapeutic plasma-exchange (TPE) and intravenous immunoglobulin (IVIG) can avoid or delay IUT. Immunoadsorption (IA) could represent a more effective treatment in selected cases...
June 6, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28647738/outcome-and-treatment-of-antenatally-diagnosed-nonimmune-hydrops-fetalis
#4
Ahmed A Nassr, Amen Ness, Pardis Hosseinzadeh, Bahram Salmanian, Jimmy Espinoza, Victoria Berger, Eleonore Werner, Hadi Erfani, Stephen Welty, Zhoobin H Bateni, Amir A Shamshirsaz, Edwina Popek, Rodrigo Ruano, Alexis S Davis, Timothy C Lee, Sundeep Keswani, Darrell L Cass, Oluyinka O Olutoye, Michael A Belfort, Alireza A Shamshirsaz
INTRODUCTION: The objectives of this study were to evaluate the outcome of nonimmune hydrops fetalis in an attempt to identify independent predictors of perinatal mortality. MATERIAL AND METHODS: A retrospective cohort study was conducted including all cases of nonimmune hydrops from two tertiary care centers. Perinatal outcome was evaluated after classifying nonimmune hydrops into ten etiological groups. We examined the effect of etiology, site of fluid accumulation, and gestational age at delivery on postnatal survival...
June 24, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28621803/absent-pulmonary-valve-syndrome-diagnosis-associations-and-outcome-in-71-prenatally-diagnosed-cases
#5
Roland Axt-Fliedner, Andrii Kurkevych, Maciej Slodki, Maria Respondek-Liberska, Katarzyna Zych-Krekora, Rüdiger Stressig, Jochen Ritgen, Guiseppe Rizzo, Martin Krapp, Luc de Catte, Gunther Mielke, Stephan Bosselmann, Mathias Meyer-Wittkopf, Andreea Kawecki, Aline Wolter, Marios Mamalis, Christian Enzensberger
OBJECTIVE: The objective of this study was to analyze the spectrum of prenatally diagnosed absent pulmonary valve syndrome (APVS) and the outcome from diagnosis onwards. Fetuses with APVS and tetralogy of Fallot (TOF/APVS) and with APVS and intact ventricular septum (APVS/IVS) were included. METHOD: Multicenter retrospective study of the International Prenatal Cardiology Collaboration Group. Clinical and echocardiographic databases of nine referral centers were reviewed from 2012-2016...
June 16, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28611934/etiology-and-perinatal-outcome-of-nonimmune-hydrops-fetalis-in-southern-china
#6
Sheng He, Linlin Wang, Pingshan Pan, Hongwei Wei, Dahua Meng, Juan Du, Xiaoxian Tian, Chenguang Zheng, Xiaoxia Qiu, Guoyang Luo
Objective  This study aims to analyze the etiology and perinatal outcome of nonimmune hydrops fetalis (NIHF) in Southern China. Methods  All cases with NIHF diagnosed antenatally from January 1, 2007 to December 31, 2014 were identified and analyzed. Results  Total 482 cases of NIHF were identified during the study period. The most common cause of NIHF was hemoglobin (Hb) Bart's disease (61.8%), followed by chromosomal abnormalities (13.5%), idiopathic etiology (13.1%), cardiac abnormalities (6.4%), and others (5...
April 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/28603204/a-6-year-old-girl-diagnosed-with-mevalonate-kinase-deficiency-who-had-hydrops-fetalis-and-neonatal-onset-cholestasis
#7
Yuriko Yamashita, Shinsuke Matsumoto, Ryugo Hiramoto, Isao Komori, Takayuki Tanaka, Ryuta Nishikomori, Toshio Heike, Shuichiro Umetsu, Ayano Inui
  We experienced a 6-year-old girl diagnosed with mevalonate kinase deficiency (MKD) who had cholestasis, anemia, and elevated inflammatory markers in neonatal period. She was admitted to our hospital because of fever and elevated inflammatory markers at 5 years 11months of age. Without using antibiotics, the fever and the inflammatory markers were spontaneously resolved. MKD was suspected from elevated serum IgD level and the recurrent febrile attacks. The genetic test revealed heterozygous mutation of p...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28586146/locating-the-level-and-extent-of-congenital-high-airway-obstruction-fluid-in-the-airway-tract-as-reference-points
#8
Mamatha Gowda, Shilpa Gupta, Ashraf Ali, Shanthi Paranthaman
Described here are a series of four cases of congenital high airway obstruction in the fetus. All of the patients presented in the second trimester and all had hydrops fetalis. Three cases had bilateral hyperinflated lungs, midline shift of heart, flattening or inversion of the diaphragm, and fetal ascites. Autopsy was performed in one of these three and showed laryngeal atresia. In one fetus, there was only a unilateral huge enlargement of the lung with mediastinal shift. On autopsy, this fetus had atresia of right main bronchus...
June 6, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28580721/persistent-hemolytic-disease-of-the-fetus-and-newborn-hdfn-associated-with-passive-acquisition-of-anti-d-in-maternal-breast-milk
#9
Marissa Li, John C Blaustein
BACKGROUND: Anti-D is a well-documented, significant cause of hemolytic disease of the fetus and newborn (HDFN), but its presence in breast milk is not routinely described. Theoretically, breast milk containing anti-D could have the potential to exacerbate HDFN if ingested by the affected infant. STUDY DESIGN AND METHODS: This is a case report of a 28-week premature male neonate with hydrops fetalis born to a 32-year-old woman (gravidity 3/parity 3) with anti-D and anti-G...
June 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28561210/cardiac-etiologies-of-hydrops-fetalis
#10
Shi-Min Yuan
Cardiac etiologies of hydrops fetalis have been a topic of concern due to challenging perinatal management. The common cardiac etiologies leading to hydrops fetalis include structural cardiac anomalies, cardiac dysrhythmias, cardiac tumors, cardiomyopathy and myocarditis. The mechanisms of cardiogenic hydrops fetalis may be: 1) elevation of atrial pressure and volume overload, 2) decrease of cardiac output, and 3) development of congestive heart failure. The diagnosis of hydrops fetalis was usually made at 19-36 gestational weeks, when ultrasound is a highly effective diagnostic method...
April 2017: Zeitschrift Für Geburtshilfe und Neonatologie
https://www.readbyqxmd.com/read/28536688/isolated-fetal-lymphatic-malformation-of-the-thigh-prenatal-diagnosis-and-follow-up
#11
Huseyin Durukan, İlay Gözükara, Murside Cevikoglu, Talat Umut Kutlu Dilek
Fetal lymphatic malformation can be found in different parts of the fetal body. It occurs most frequently in the nuchal and axillary region and less frequently in the abdomen or inguinal areas. Lymphatic malformation has been associated with fetal aneuploidy, hydrops fetalis, structural malformations, and intrauterine fetal death. A 31-year-old gravida 3, para 2 woman was admitted to our hospital at 22 weeks of gestation (confirmed by ultrasonographic examination). The fetus was alive, and had a mass derived from the left inguinal region extending to the anterior left leg with fluid-filled cavities about 3-5 cm in size...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28533037/epidemiology-of-live-born-infants-with-nonimmune-hydrops-fetalis-insights-from-a-population-based-dataset
#12
Martina A Steurer, Shabnam Peyvandi, Rebecca J Baer, Tippi MacKenzie, Ben C Li, Mary E Norton, Laura L Jelliffe-Pawlowski, Anita J Moon-Grady
OBJECTIVE: To evaluate the incidence, etiology, and 1-year mortality of nonimmune hydrops fetalis (NIHF) and to identify risk factors for mortality in a contemporary population-based dataset. STUDY DESIGN: The California Office of Statewide Health Planning and Development maintains a database linking maternal and infant hospital discharge, readmissions, and birth and death certificate date from 1 year before to 1 year after birth. We searched the database (2005-2012) for infants with NIHF (identified by the International Classification of Diseases, 9th Revision, Clinical Modification code)...
May 19, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28523047/alpha-thalassemia-deletions-found-in-suspected-cases-of-beta-thalassemia-major-in-pakistani-population
#13
Saba Shahid, Muhammad Nadeem, Danish Zahid, Jawad Hassan, Saqib Ansari, Tahir Shamsi
BACKGROUND & OBJECTIVE: Alpha (α) thalassemia is a hereditary disorder and is caused by deletions or mutations in globin genes. It is present in two clinically significant forms: hemoglobin Bart hydrops fetalis (Hb Bart) syndrome and hemoglobin H (HbH) disease. It is highly prevalent in South-East Asia or Mediterranean countries. The most common deletion reported in alpha thalassemia in Pakistani population was -α(3.7) with a frequency of 8.3%, and the rare forms were -α(4.2) (0.2%) and ααα(anti3...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28512588/severe-aplastic-anemia-following-parvovirus-b19-associated-acute-hepatitis
#14
Masanori Furukawa, Kosuke Kaji, Hiroyuki Masuda, Kuniaki Ozaki, Shohei Asada, Aritoshi Koizumi, Takuya Kubo, Norihisa Nishimura, Yasuhiko Sawada, Kosuke Takeda, Tsuyoshi Mashitani, Masayuki Kubo, Itsuto Amano, Tomoyuki Ootani, Chiho Ohbayashi, Koji Murata, Tatsuichi Ann, Akira Mitoro, Hitoshi Yoshiji
Human parvovirus (HPV) B19 is linked to a variety of clinical manifestations, such as erythema infectiosum, nonimmune hydrops fetalis, and transient aplastic anemia. Although a few cases have shown HPVB19 infection as a possible causative agent for hepatitis-associated aplastic anemia (HAAA) in immunocompetent patients, most reported cases of HAAA following transient hepatitis did not have delayed remission. Here we report a rare case of severe aplastic anemia following acute hepatitis with prolonged jaundice due to HPVB19 infection in a previously healthy young male...
2017: Case Reports in Hepatology
https://www.readbyqxmd.com/read/28497611/co-inheritance-of-%C3%AE-0-thalassemia-elevates-hb-a2-level-in-homozygous-hb-e-diagnostic-implications
#15
K Singha, H Srivorakun, G Fucharoen, S Fucharoen
INTRODUCTION: Differentiation of homozygous hemoglobin (Hb) E with and without α(0) -thalassemia is subtle on routine hematological ground. We examined in a large cohort of homozygous Hb E if the level of Hb A2 is helpful. METHODS: A total of 592 subjects with homozygous Hb E were recruited from ongoing thalassemia screening program. Additionally, five couples at risk of having fetuses with Hb Bart's hydrops fetalis who were homozygous Hb E were also investigated...
May 12, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28489127/genotype-1-of-human-parvovirus-b19-in-clinical-cases
#16
Maria Isabel de Oliveira, Ana Maria Sardinha Afonso, Suely Pires Curti, Patrícia Evelin Silva, Tamyris Fernanda Barbosa, Elian Reis Silva, Cristina Adelaide Figueiredo
Introduction: Virus surveillance strategies and genetic characterization of human parvovirus B19 (B19V) are important tools for regional and global control of viral outbreak. In São Paulo, Brazil, we performed a study of B19V by monitoring the spread of this virus, which is an infectious agent and could be mistakenly reported as a rash and other types of infection. Method: Serum samples were subjected to enzyme immunoassay, real time polymerase chain reaction, and sequencing...
March 2017: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/28416051/-mirror-syndrome-is-a-rare-complication-in-pregnancy-characterized-by-oedema-and-hydrops-fetalis
#17
Signe Eiland, Eleonora Cvetanovska, Anne Høyrup Bjerre, Henrik Nyholm, Karin Sundberg, Lone Nikoline Nørgaard
We describe a case of mirror syndrome. A 41-year-old woman, para 4, was referred to hospital at 32 weeks of gestational age with excessive oedema. She developed oliguria, mild hypertension and proteinuria. Fetal ultrasound confirmed severe hydrops fetalis. Intrauterine pleural drainage was performed without improvement of the condition. Since maternal symptoms worsened, a caesarean section was performed at 34 weeks with delivery of a live hydropic girl who died after 17 hours. Maternal symptoms decreased four days after delivery with full recovery two weeks post-partum...
April 10, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28398685/parvovirus-b19-infection-during-pregnancy-and-risks-to-the-fetus
#18
REVIEW
Asher Ornoy, Zivanit Ergaz
Parvovirus B19 infects 1 to 5% of pregnant women, generally with normal pregnancy outcomes. During epidemics, the rate of infection is higher. Major congenital anomalies among offspring of infected mothers are rare, as the virus does not appear to be a significant teratogen. However, parvovirus B19 infection may cause significant fetal damage, and in rare cases, brain anomalies and neurodevelopmental insults, especially if infection occurs in the first 20 weeks of pregnancy. Parvovirus B19 is also an important cause of fetal loss, especially in the second half of pregnancy when spontaneous fetal loss from other causes is relatively rare...
March 15, 2017: Birth defects research
https://www.readbyqxmd.com/read/28383542/quantitative-clinical-characteristics-of-53-patients-with-mps-vii-a-cross-sectional-analysis
#19
Matthias Zielonka, Sven F Garbade, Stefan Kölker, Georg F Hoffmann, Markus Ries
PURPOSE: The main purpose of the study was to provide quantitative data regarding survival and diagnostic delay. Mucopolysaccharidosis (MPS) type VII (OMIM 253220) is a progressive neurometabolic disorder caused by deficiency of the lysosomal enzyme β-glucuronidase (GUS). Hard clinical end points have not been quantitatedMethods:We quantitatively analyzed published cases with MPS VII (N = 53/88 with sufficient data). Main outcome measures were onset of disease and survival. The role of biomarkers such as GUS residual enzyme activity and levels of storage material assessed as urinary excretion of glucosaminoglycans (GAG) as potential predictors of clinical outcomes were investigated...
April 6, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28381876/genetic-origin-of-%C3%AE-0-thalassemia-sea-deletion-in-southeast-asian-populations-and-application-to-accurate-prenatal-diagnosis-of-hb-bart-s-hydrops-fetalis-syndrome
#20
Wittaya Jomoui, Goonnapa Fucharoen, Kanokwan Sanchaisuriya, Patnaree Charoenwijitkul, Jitpanu Maneesarn, Xiangmin Xu, Supan Fucharoen
α(0)-thalassemia of SEA deletion (-(SEA)) is common among Southeast Asian and Chinese. Using haplotype and phylogenetic analyses, we examined the origin of this defect in Southeast Asian populations. Study was done on both normal and α(0)-thalassemia alleles in 3 ethnic groups including 96 Thai, 52 Laotian and 21 Cambodian. Five SNPs encompassing the (-(SEA)) including (rs3760053 T>G), (rs1211375 A>C), (rs3918352 A>G), (rs1203974 A>G) and (rs11248914 C>T) were examined using high-resolution melting assays...
April 6, 2017: Journal of Human Genetics
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