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Hydrops fetalis

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
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 30, 2016: Hemoglobin
Jenny M Held, Ryan Restrepo, Robert Ricca
No abstract text is available yet for this article.
September 2016: American Surgeon
Z Xie, H Yuan
No abstract text is available yet for this article.
September 2016: Ultrasound in Obstetrics & Gynecology
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
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 these effects should include changes in the pulmonary neuroendocrine system...
September 6, 2016: Pediatric and Developmental Pathology
Ali Seven, Emine Esin Yalinbas, Rahmi Ozdemir
Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease with severe pulmonary insufficiency, characterized with aneurysmal dilation in the pulmonary artery and one or both of its branches. We presented a rare case with APVS and literature review in this letter. Prenatal USG examination of the fetus at the 26th week of gestation revealed severe polyhydramnios, dilatation at right ventricle, and abnormal appearance of the heart. At the 31st gestational week, the baby was born with cesarean section...
2016: Case Reports in Obstetrics and Gynecology
Marivi Cervera-Gaviria, Miguel Angel Alcántara-Ortigoza, Ariadna González-Del Angel, Paola Moyers-Pérez, Blanca Gabriela Lizet Legorreta-Ramírez, Nancy Barrera-Carmona, Jaime Cervera-Gaviria
BACKGROUND: Niemann-Pick disease type C (NP-C) is a fatal lysosomal neurodegenerative and neurovisceral disease. It is caused by defects in intracellular lipid trafficking, which lead to the accumulation of lipids and glycosphingolipids within the endosomes and lysosomes of affected individuals. Pathogenic variants of the NPC1 or NPC2 genes yield highly variable phenotypes with a time course that ranges from fetal onset (i.e., hydrops fetalis) to progressive dementia in adults. NP-C is typically inherited in an autosomal-recessive manner...
2016: BMC Neurology
Cheryl K Roth, Annmarie Puttbrese, Charlotte Ottley
Thalassemia syndromes are becoming more common in the United States as the population becomes more diverse. To provide appropriate care to this patient population, nurses must know that thalassemia syndromes are classified into two main types, α-thalassemia and β-thalassemia. α-Thalassemia is further delineated into four clinical patterns: silent carrier state, mild α-thalassemia, hemoglobin H disease, and hydrops fetalis. Understanding each of these complex anemias and their potential effects on a pregnant woman and her fetus will enable nurses to interpret these women's unique laboratory test results...
August 2016: Nursing for Women's Health
S Voleva, St Ivanova, B Marinov, P Genova-Kalou, V Manolov, V Vasilev
UNLABELLED: Viral infections during pregnancy, along with some form of accompanying pregnancy diseases such as diabetes, cardiovascular, gastrointestinal, kidney and others, are a major cause of arising complications and mortality of mother and fetus. AIM: To improved the laboratory diagnostic approach in the study of women with pathological pregnancy, including improve treatment and prognostic character of the outcome of pregnancy, with the inclusion of two infectious agent parvovirus B19 and Chlamydia trachomatis...
2016: Akusherstvo i Ginekologii︠a︡
Kobkorn Pongjantharasatien, Wansa Banyatsuppasin, Sonnarong Pounsawat, Sumalee Jindadamrongwech
α-Thalassemia (α-thal) is one of the most common genetic diseases in Thailand. Homozygosity of α-thal-1 (- -/- -) and compound heterozygosity of α-thal-1/α-thal-2 (- -/-α) leads to Hb Bart's (γ4) hydrops fetalis and Hb H (β4) disease, respectively. In order to better control and provide prevention of α-thal disease, the prevalence of α-thal-1 carriers and the types of genotypes in the Thai population should be known. A 7-year retrospective study, employing multiplex gap-polymerase chain reaction (gap-PCR) of 31,632 blood samples from Ramathibodi Hospital, Mahidol University, Bangkok, revealed an α-thal-1 carrier rate of 14...
August 2016: Hemoglobin
Mana Taweevisit, Paul Scott Thorner
INTRODUCTION: Placental ischemia can be pre-placental (maternal), placental or post-placental (fetal), with corresponding changes in villous vasculature. Hydrops fetalis (HF) resulting from hemoglobin (Hb) Bart disease can serve as a model for intrauterine hypoxia, and placentas from such cases show a distinctive peripheral villous stromal myofibroblastic hypercellularity (PVSH). We hypothesized that Hb Bart disease, which results in profound fetal hypoxia, would lead to placental hypoxia on a post-placental basis...
August 2016: Placenta
Javier Galve, Ana Martín-Santiago, Carmen Clavero, Carlos Saus, Ramona Alfaro-Arenas, Angeles Pérez-Granero, Pere R Balliu, Juan Ferrando
Silvery hair is a characteristic finding of 3 rare autosomal recessive disorders: Chédiak-Higashi syndrome (CHS), Elejalde syndrome (ES), and Griscelli syndrome (GS). We report the case of a 2-month-old male infant with transient silvery hair and generalized hypopigmentation of the skin and eyes who did not have one of these classic causative disorders. The patient was delivered at 35 weeks' gestation with congenital hydrops fetalis associated with a chromosomal abnormality (46,XY,add[2],[p23]), hypothyroidism, hypoproteinemia, and hypogammaglobulinemia...
June 2016: Cutis; Cutaneous Medicine for the Practitioner
Ameeta Yaksh, Lisette Jme van der Does, Eva Ah Lanters, Natasja Ms de Groot
Tachyarrhythmias are the most frequently observed cardiac complications during pregnancy. The majority of these maternal and foetal arrhythmias are supraventricular tachyarrhythmias; ventricular tachyarrhythmias are rare. The use of anti-arrhythmic drugs (AADs) during pregnancy is challenging due to potential foetal teratogenic effects. Maintaining stable and effective therapeutic maternal drug levels is difficult due to haemodynamic and metabolic alterations. Pharmacological treatment of tachyarrhythmias is indicated in case of maternal haemodynamic instability or hydrops fetalis...
May 2016: Arrhythmia & Electrophysiology Review
Adnan I Qureshi, Thomas Kohl
No abstract text is available yet for this article.
June 2016: Journal of Vascular and Interventional Neurology
Silvia Martin-Almedina, Ines Martinez-Corral, Rita Holdhus, Andres Vicente, Elisavet Fotiou, Shin Lin, Kjell Petersen, Michael A Simpson, Alexander Hoischen, Christian Gilissen, Heather Jeffery, Giles Atton, Christina Karapouliou, Glen Brice, Kristiana Gordon, John W Wiseman, Marianne Wedin, Stanley G Rockson, Steve Jeffery, Peter S Mortimer, Michael P Snyder, Siren Berland, Sahar Mansour, Taija Makinen, Pia Ostergaard
Hydrops fetalis describes fluid accumulation in at least 2 fetal compartments, including abdominal cavities, pleura, and pericardium, or in body tissue. The majority of hydrops fetalis cases are nonimmune conditions that present with generalized edema of the fetus, and approximately 15% of these nonimmune cases result from a lymphatic abnormality. Here, we have identified an autosomal dominant, inherited form of lymphatic-related (nonimmune) hydrops fetalis (LRHF). Independent exome sequencing projects on 2 families with a history of in utero and neonatal deaths associated with nonimmune hydrops fetalis uncovered 2 heterozygous missense variants in the gene encoding Eph receptor B4 (EPHB4)...
August 1, 2016: Journal of Clinical Investigation
Christine Vianey-Saban, Cécile Acquaviva, David Cheillan, Sophie Collardeau-Frachon, Laurent Guibaud, Cécile Pagan, Magali Pettazzoni, Monique Piraud, Antonin Lamazière, Roseline Froissart
Inborn errors of metabolism (IEMs) that present with abnormal imaging findings in the second half of pregnancy are mainly lysosomal storage disorders (LSDs), cholesterol synthesis disorders (CSDs), glycogen storage disorder type IV (GSD IV), peroxisomal disorders, mitochondrial fatty acid oxidation defects (FAODs), organic acidurias, aminoacidopathies, congenital disorders of glycosylation (CDGs), and transaldolase deficiency. Their biological investigation requires fetal material. The supernatant of amniotic fluid (AF) is useful for the analysis of mucopolysaccharides, oligosaccharides, sialic acid, lysosphingolipids and some enzyme activities for LSDs, 7- and 8-dehydrocholesterol, desmosterol and lathosterol for CSDs, acylcarnitines for FAODs, organic acids for organic acidurias, and polyols for transaldolase deficiency...
September 2016: Journal of Inherited Metabolic Disease
N Lin, Y Lin, H L Huang, X L Lin, D Q He, S Q He, D H Guo, Y Li, L P Xu
Objective: To conduct analysis and prenatal diagnosis on 11 couples carrying Thailand deletion (--(THΑI)) α-thalassemia 1, so as to provide information for clinical genetic counseling on α-thalassemia 1. Methods: Altogether 11 Thailand deletion (--(THΑI)) α-thalassemia 1 families were collected from Fujian Maternal and Children Health Hospital from May 2009 to September 2015. Gap-polymerase chain reaction (gap-PCR) and reverse dot blot (RDB) technology were used to detect the thalassemia mutations in the couples and fetuses...
June 28, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Pei-Hsuan Lin, Hsin-Hung Wu, Horng-Der Tsai, Charles Tsung-Che Hsieh
OBJECTIVE: We report a case of nonimmune hydrops fetalis caused by atrial flutter, which was successfully treated by intraperitoneal and intra-amniotic injections of amiodarone. CASE REPORT: A 27-year-old woman presented at 30 weeks of pregnancy with hydrops fetalis caused by a fetal atrial flutter. As the transplacental passage of antiarrhythmic agents is impaired in hydrops fetalis, we chose direct treatment using fetal intraperitoneal and intra-amniotic injections (75-300 mg) of amiodarone...
June 2016: Taiwanese Journal of Obstetrics & Gynecology
Adriana Nicholson Vest, Li Zhou, Yaniv Bar-Cohen, Gerald Eli Loeb
We have developed a rechargeable fetal micropacemaker in order to treat severe fetal bradycardia with comorbid hydrops fetalis, a life-threatening condition in pre-term non-viable fetuses for which there are no effective treatment options. The small size and minimally invasive form factor of our design limit the volume available for circuitry and a power source. The device employs a fixed-rate and fixed-amplitude relaxation oscillator and a tiny, rechargeable lithium ion power cell. For both research and clinical applications, it is valuable to monitor the electrode-myocardium interface in order to determine that adequate pacemaker output is being provided...
July 2016: Physiological Measurement
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