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https://www.readbyqxmd.com/read/28064267/chemical-pleurodesis-with-oxytetracycline-in-congenital-chylothorax
#1
Alpana Utture, Vinayak Kodur, Jayashree Mondkar
BACKGROUND: Congenital chylothorax is an accumulation of chyle in the pleural space that may present in neonatal period with respiratory distress. CASE CHARACTERISTICS: A 34-week preterm who presented with massive congenital chylothorax complicated with hydrops fetalis. OUTCOME: The neonate was treated successfully by pleurodesis with Oxytetracycline. MESSAGE: Pleurodesis with oxytetracycline seems to be effective in treatment of congenital chylothorax...
December 15, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/28057638/an-international-registry-of-survivors-with-hb-bart-s-hydrops-fetalis-syndrome
#2
Duantida Songdej, Christian Babbs, Douglas R Higgs
Hb Bart's Hydrops Fetalis Syndrome (BHFS) resulting from α(0)-thalassemia is considered a universally fatal disorder. However, over the last three decades improvements in intrauterine interventions and perinatal intensive care have resulted in increasing numbers of BHFS survivors. We have initiated an international registry containing information on 69 patients, of which 31 are previously unpublished. In this perspective we analyze the available clinical information to document the natural history of BHFS...
January 5, 2017: Blood
https://www.readbyqxmd.com/read/28043830/fetal-primary-cardiac-tumors-during-perinatal-period
#3
REVIEW
Shi-Min Yuan
Fetal primary cardiac tumors are rare, but they may cause complications, which are sometimes life threatening, including arrhythmias, hydrops fetalis, ventricular outflow/inflow obstruction, cardiac failure, and even sudden death. Among fetal primary cardiac tumors, rhabdomyomas are most common, followed by teratomas, fibromas, hemangiomas, and myxomas. Everolimus, a mammalian target of rapamycin inhibitor, has been reported to be an effective drug to cause tumor remission in three neonates with multiple cardiac rhabdomyomas...
October 28, 2016: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28040135/pallister-killian-syndrome-cytogenetics-and-molecular-investigations-of-mosaic-tetrasomy-12p-in-prenatal-chorionic-villus-and-in-amniocytes-strategy-of-prenatal-diagnosis
#4
Francesco Libotte, Domenico Bizzoco, Ivan Gabrielli, Alvaro Mesoraca, Pietro Cignini, Salvatore Giovanni Vitale, Ilaria Marilli, Ferdinando Antonio Gulino, Agnese Maria Chiara Rapisarda, Claudio Giorlandino
OBJECTIVE: Pallister-Killian syndrome (PKS) is a rare, sporadic genetic disorder caused by mosaic tetrasomy of the short arm of chromosome 12 (12p). Clinically, PKS is characterized by several systemic abnormalities, such as intellectual impairment, hearing loss, epilepsy, hypotonia, craniofacial dysmorphism, pigmentary skin anomalies, epilepsy, and a variety of congenital malformations. Prenatally, PKS can be suspected in the presence of ultrasound anomalies: diaphragmatic hernia, rhizomelic micromelia, hydrops fetalis, fetal overweight, ventriculomegaly in the central nervous system, congenital heart defects, or absent visualization of the stomach...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27981786/diagnosis-of-common-hemoglobinopathies-among-south-east-asian-population-using-capillary-isoelectric-focusing-system
#5
H Srivorakun, G Fucharoen, K Sanchaisuriya, S Fucharoen
INTRODUCTION: We have evaluated an automated capillary isoelectric focusing (cIEF)-based Hb analyzer in diagnosis of hemoglobinopathies commonly found among South East Asian population. METHODS: Study was performed on a cohort of 665 adult Thai subjects and 13 fetal blood specimens obtained at routine thalassemia diagnostic laboratory. Hb analysis was performed using the cIEF system. Thalassemia genotypes were defined by DNA analysis. RESULTS: The system revealed satisfactorily within-run and between-run precision for quantitation of Hb A2 and Hb E (CV: 0...
December 16, 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27956203/syphilis-during-pregnancy-a-preventable-threat-to-maternal-fetal-health
#6
REVIEW
Martha W F Rac, Paula A Revell, Catherine S Eppes
Syphilis remains the most common congenital infection worldwide and has tremendous consequences for the mother and her developing fetus if left untreated. Recently, there has been an increase in the number of congenital syphilis cases in the United States. Thus, recognition and appropriate treatment of reproductive-age women must be a priority. Testing should be performed at initiation of prenatal care and twice during the third trimester in high-risk patients. There are 2 diagnostic algorithms available and physicians should be aware of which algorithm is utilized by their testing laboratory...
December 9, 2016: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27943499/prenatal-diagnosis-and-prognosis-of-accelerate-idioventricular-rhythm
#7
Jean-Claude Fouron, Ariane McNeal-Davidson, Sylvia Abadir, Anne Fournier, Jean-Luc Bigras, Christine Boutin, Myriam Brassard, Marie-Josée Raboisson, Nicolaas van Doesburg, Annie Berger, Sophie Brisebois, Roxanne Gendron
OBJECTIVES: Since postnatal identification of accelerated idioventricular rhythm (AIVR) is essentially based on specific electrocardiographic patterns, its prenatal diagnosis is challenging and its genuine incidence undetermined. Therefore the objectives of this study are: 1) To evaluate the performance of specific ultrasonographic approaches in intrauterine identification of cardiocirculatory events linked to electrocardiographic signs of AIVR, including its left or right ventricular origin, and 2) To assess the prenatal prognosis of the arrhythmia...
December 10, 2016: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27928775/lysosomal-storage-disorders-in-nonimmune-hydrops-fetalis-nihf-an-indian-experience
#8
Jayesh Sheth, Mehul Mistri, Krati Shah, Mayank Chaudhary, Koumudi Godbole, Frenny Sheth
Lysosomal storage disorders (LSD) are rare inherited neurovisceral inborn errors of metabolism which may present as nonimmune hydrops fetalis (NIHF) during pregnancy. Although causes of NIHF are highly diverse, LSDs are one of the underlying causes of NIHF. The aim of this study was to elucidate most frequent causes of LSDs presenting as NIHF in Indian population. Several fetal tissues were investigated for enzymatic diagnosis of LSDs using modified fluorometric assays in the current prospective study carried out at our national tertiary center from 2006 through 2016...
December 8, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27920985/the-first-case-of-recurrent-ultra-late-onset-group-b-streptococcal-sepsis-in-a-3-year-old-child
#9
Ai Hosoda, Ryohei Gatayama, Shiori Moriyama, Noriyuki Ishii, Kenichiro Yamada, Youhei Matsuzaki, Masayoshi Shinjoh
Group B streptococcus (GBS) is a commonly recognized cause of sepsis and meningitis in neonatal and young infants. Invasive GBS infection is classified into early onset GBS disease (EOD, day 0-6), late onset GBS disease (LOD, day 7-89) and ultra late onset GBS disease (ULOD, after 3 months of age). ULOD is uncommon and recurrence is especially rare. We present the first recurrent case of ULOD GBS sepsis in 3-year-old girl with a past medical history of hydrops fetalis and thoracic congenital lymphatic dysplasia...
2017: IDCases
https://www.readbyqxmd.com/read/27876354/update-in-the-genetics-of-thalassemia-what-clinicians-need-to-know
#10
REVIEW
Xuan Shang, Xiangmin Xu
Thalassemia is a significant health problem worldwide. Prenatal diagnosis is the only effective way to prevent the birth of a fetus with severe thalassemias, which include hemoglobin Bart's hydrops fetalis and thalassemia major. However, accurate prenatal diagnosis depends on the comprehensive consideration of the molecular basis of thalassemias. To make a correct decision, the obstetrician should have a certain understanding of the genetics of thalassemias. Here we present a brief introduction of some fundamental genetic knowledge of thalassemias, including the production of hemoglobin, structure and location of globin genes, hemoglobin switch, epidemiology, clinical classification, molecular and cellular pathology, genotype-phenotype correlation, and genetic modifiers...
October 26, 2016: Best Practice & Research. Clinical Obstetrics & Gynaecology
https://www.readbyqxmd.com/read/27862048/favorable-outcomes-after-in-utero-transfusion-in-fetuses-with-alpha-thalassemia-major-a-case-series-and-review-of-the-literature
#11
Emily M Kreger, Sylvia T Singer, Russell G Witt, Nancy Sweeters, Billie Lianoglou, Ashutosh Lal, Tippi C Mackenzie, Elliott Vichinsky
OBJECTIVE: Alpha thalassemia major (ATM) is often fatal in utero due to severe hydrops fetalis. Although in utero transfusions (IUTs) are increasingly used to allow fetal survival in ATM, prenatal and postnatal outcomes are not well described. METHODS: We retrospectively reviewed cases of ATM at our institution treated with consecutive IUT. Clinical records were reviewed for transfusion history, neurodevelopmental outcomes, anatomic abnormalities, survival to hematopoietic cell transplantation, and transfusion independence...
December 2016: Prenatal Diagnosis
https://www.readbyqxmd.com/read/27857529/severe-hemolytic-disease-of-the-premature-newborn-due-to-rh1-incompatibility-a-case-report
#12
Jean Uwingabiye, Hafid Zahid, Fayçal Labrini, Abdelhak El Khazraji, Anass Yahyaoui, Rachid Hadef, Nezha Messaoudi
We report a case of dramatic outcome of severe hemolytic disease in a newborn due to RH1 incompatibility. A newborn with A RH1 blood group was admitted in the Mohammed V Military Teaching Hospital for the problem of hydrops fetalis associated with RH1 incompatibility. The blood group of his mother, aged 31, was AB RH1-negative and that of his 37 year old father was A RH1. The mother had a history of 4 term deliveries, 3 abortions, and 1 living child. There was no prevention by anti-D immunoglobulin postpartum...
2016: Clujul Medical (1957)
https://www.readbyqxmd.com/read/27853988/antenatal-manifestations-of-inborn-errors-of-metabolism-prenatal-imaging-findings
#13
Laurent Guibaud, Sophie Collardeau-Frachon, Audrey Lacalm, Mona Massoud, Massimiliano Rossi, Marie Pierre Cordier, Christine Vianey-Saban
Prenatal manifestations of inborn errors of metabolism (IEM) are related to severe disorders involving metabolic pathways active in the fetal period and not compensated by maternal or placental metabolism. Some prenatal imaging findings can be suggestive of such conditions-especially in cases of consanguinity and/or recurrence of symptoms-after exclusion of the most frequent nonmetabolic etiologies. Most of these prenatal imaging findings are nonspecific. They include mainly ascites and hydrops fetalis, intrauterine growth restriction (IUGR), central nervous system (CNS) anomalies, echogenic kidneys, epiphyseal stippling, craniosynostosis, and a wide spectrum of dysostoses...
January 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/27809499/dna-binding-and-cleavage-by-the-human-parvovirus-b19-ns1-nuclease-domain
#14
Jonathan L Sanchez, Zachary Romero, Angelica Quinones, Kristiane R Torgeson, Nancy C Horton
Infection with human parvovirus B19 (B19V) has been associated with a myriad of illnesses, including erythema infectiosum (Fifth disease), hydrops fetalis, arthropathy, hepatitis, and cardiomyopathy, and also possibly the triggering of any number of different autoimmune diseases. B19V NS1 is a multidomain protein that plays a critical role in viral replication, with predicted nuclease, helicase, and gene transactivation activities. Herein, we investigate the biochemical activities of the nuclease domain (residues 2-176) of B19V NS1 (NS1-nuc) in sequence-specific DNA binding of the viral origin of replication sequences, as well as those of promoter sequences, including the viral p6 and the human p21, TNFα, and IL-6 promoters previously identified in NS1-dependent transcriptional transactivation...
November 29, 2016: Biochemistry
https://www.readbyqxmd.com/read/27787388/hydrocephalus-secondary-to-chemotherapy-in-a-case-of-prenatally-diagnosed-giant-immature-grade-3-sacrococcygeal-teratoma-a-case-report-and-literature-review
#15
Ioan Sarbu, Demetra Socolov, Razvan Socolov, Ingrith Miron, Mioara Trandafirescu, Smaranda Diaconescu, Carmen Iulia Ciongradi
INTRODUCTION: Sacrococcygeal teratoma (SCT) is a rare tumor in the general population, arising from multipotent stem cells. Whereas most of the cases diagnosed postnatally have good prognosis, the rate of mortality and morbidities associated with prenatally diagnosed SCT remain high, with a reported mortality rate of 30% to 50%. The outcome of fetal SCT can be unpredictable, with some cases with slow growth during fetal life, whereas others grow rapidly, causing multiple complications; also, some of these tumor will develop triggering fetal (preterm delivery, high-output cardiac failure, hydrops fetalis, intrauterine death) or maternal complications (distocia, placentomegaly, maternal mirror syndrome-preeclampsia)...
October 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27712118/zika-virus-epidemic-an-update
#16
Gregory Valentine, Lucila Marquez, Mohan Pammi
Zika Virus (ZIKV), previously the cause of only rare and sporadic human infections, is now considered a Public Health Emergency of International Concern. Over the past two years, ZIKV has become a pandemic encompassing much of the Americas. ZIKV is now proven to cause microcephaly and ophthalmic anomalies in the newborn. Hydrops fetalis, developmental delay, and other anomalies are increasingly being attributed to ZIKV infection in fetuses and neonates. Sequelae of congenital infection and rapid spread of ZIKV throughout the Americas has catapulted Zika virus concerns to the forefront of the medical community...
October 7, 2016: Expert Review of Anti-infective Therapy
https://www.readbyqxmd.com/read/27686733/hydrops-fetalis-associated-with-compound-heterozygosity-for-hb-zurich-albisrieden-hba2-c-178g%C3%A2-%C3%A2-c-and-the-southeast-asian-sea-deletion
#17
Xin Yang, Jin-Mei Yan, Jian Li, Xing-Mei Xie, Jian-Ying Zhou, Yan Li, Dong-Zhi Li
Hb Zurich-Albisrieden [HBA2: c.178G > C; α59(E8)Gly→Arg (α2)] is a rare nondeletional α-thalassemia (α-thal) that results from a nucleotide substitution at codon 59 of the α2-globin gene. In this report, we present a fetus with cardiomegaly, enlarged placenta and increased middle cerebral artery-peak systolic velocity (MCA-PSV) at 25 weeks' gestation. Fetal blood sampling revealed the severe anemia [hemoglobin (Hb) level being 5.5 g/dL] and Hb H (β4) disease-like hematological findings with Hb Bart's (γ4) level of 30...
September 2016: Hemoglobin
https://www.readbyqxmd.com/read/27670562/chylous-ascites-in-a-neonate-with-hydrops-fetalis
#18
Jenny M Held, Ryan Restrepo, Robert Ricca
No abstract text is available yet for this article.
September 2016: American Surgeon
https://www.readbyqxmd.com/read/27644483/ep03-14-using-spatiotemporal-image-correlation-with-m-mode-display-to-monitor-ventricular-systolic-function-of-hydrops-fetalis
#19
Z Xie, H Yuan
No abstract text is available yet for this article.
September 2016: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27642479/theca-lutein-cysts-and-early-onset-severe-preeclampsia
#20
Mehmet Akif Sargin, Niyazi Tug, Ozgur Aydin Tosun, Murat Yassa, Evrim Bostanci
Hyperreactio luteinalis (HL) is a rare condition that is characterized by bilateral ovarian enlargement and multiple thin walled cysts. Hypersensitivity of the ovary to circulating human chorionic gonadotropin (hCG) is playing the main role in pathophysiology. HL observed in cases where there is high serum ß-HCG levels, similarly to gestational trophoblastic disease, multiple pregnancies, hydrops fetalis and after fertiliy treatment. Most of HL are self limiting condition and patients are asymptomatic. Differentiation from ovarian malignancies is important...
2016: Pan African Medical Journal
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