MCID: HYD012
MIFTS: 43

Hydrops Fetalis malady

Categories: Rare diseases, Fetal diseases, Immune diseases, Genetic diseases

Aliases & Classifications for Hydrops Fetalis

Aliases & Descriptions for Hydrops Fetalis:

Name: Hydrops Fetalis 50 29 52 69
Hydrops Fetalis Nonimmune 50 29
Familial Non-Immune Hydrops Fetalis 50
Hydrops Fetalis, Non-Immune 69
Idiopathic Hydrops Fetalis 50

Classifications:



External Ids:

ICD10 33 P56 P56.0 P56.9

Summaries for Hydrops Fetalis

NIH Rare Diseases : 50 hydrops fetalis is a serious condition in which abnormal amounts of fluid build up in two or more body areas of a fetus or newborn. there are two types of hydrops fetalis: immune and nonimmune. immune hydrops fetalis is a complication of a severe form of rh incompatibility. rh compatibility causes massive red blood cell destruction, which leads to several problems, including total body swelling. severe swelling can interfere with how the body organs work. nonimmune hydrops fetalis occurs when a disease or medical condition disrupts the body's ability to manage fluid. there are three main causes for this type: heart or lung problems, severe anemia (thalassemia), and genetic defects, including turner syndrome. the exact cause depends on which form a baby has. last updated: 1/25/2012

MalaCards based summary : Hydrops Fetalis, also known as hydrops fetalis nonimmune, is related to hydrops fetalis, nonimmune and hydrops fetalis, non-immune, and/or atrial septal defect, and has symptoms including edema and joint swelling. An important gene associated with Hydrops Fetalis is GUSB (Glucuronidase Beta), and among its related pathways/superpathways are Glycosaminoglycan metabolism and Galactose metabolism. The drugs Darbepoetin alfa and Hematinics have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and placenta.

Wikipedia : 71 Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at... more...

Related Diseases for Hydrops Fetalis

Diseases related to Hydrops Fetalis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 133)
id Related Disease Score Top Affiliating Genes
1 hydrops fetalis, nonimmune 12.6
2 hydrops fetalis, non-immune, and/or atrial septal defect 12.4
3 immune hydrops fetalis 12.4
4 hydrops fetalis anemia immune disorder absent thumb 12.0
5 mucopolysaccharidosis vii 11.5
6 fetal edema 11.4
7 congenital dyserythropoietic anemia 11.3
8 pyruvate kinase deficiency 11.3
9 glucose phosphate isomerase deficiency 11.3
10 hemoglobin h disease, nondeletional 11.1
11 achondrogenesis, type ii or hypochondrogenesis 11.1
12 semmekrot haraldsson weemaes syndrome 11.0
13 thalassemias, alpha- 11.0
14 gaucher disease, perinatal lethal 11.0
15 greenberg skeletal dysplasia 11.0
16 chondrodysplasia, blomstrand type 11.0
17 lymphangiectasia, pulmonary, congenital 11.0
18 porphyria, congenital erythropoietic 11.0
19 short-rib thoracic dysplasia 12 11.0
20 hypochondrogenesis 11.0
21 fetal parvovirus syndrome 11.0
22 thalassemia 10.4
23 chorioangioma 10.3
24 teratoma 10.2
25 mucopolysaccharidosis 10.2
26 hepatitis 10.1
27 lysosomal storage disease 10.1
28 hemophagocytic lymphohistiocytosis 10.1
29 syphilis 10.1
30 down syndrome 10.1
31 pulmonary sequestration 10.1
32 diamond-blackfan anemia 10.0
33 cardiomyopathy 10.0
34 cerebritis 10.0
35 congenital syphilis 10.0
36 lymphedema 10.0
37 mediastinitis 10.0
38 polyhydramnios 10.0
39 sacrococcygeal teratoma 10.0
40 hemoglobinopathy 10.0
41 herpes simplex 10.0
42 cytomegalovirus infection 10.0
43 endocardial fibroelastosis 10.0
44 thrombosis 10.0
45 hydrocephalus due to congenital stenosis of aqueduct of sylvius 10.0 GUSB HBA1
46 intracranial hypotension 10.0 HBA1 RASA1
47 galactosialidosis 9.9
48 hemangioma 9.9
49 congenital hypothyroidism 9.9
50 histiocytosis 9.9

Graphical network of the top 20 diseases related to Hydrops Fetalis:



Diseases related to Hydrops Fetalis

Symptoms & Phenotypes for Hydrops Fetalis

Human phenotypes related to Hydrops Fetalis:

32
id Description HPO Frequency HPO Source Accession
1 hydrops fetalis 32 HP:0001789

UMLS symptoms related to Hydrops Fetalis:


edema, joint swelling

Drugs & Therapeutics for Hydrops Fetalis

Drugs for Hydrops Fetalis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 15)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Darbepoetin alfa Approved, Investigational Phase 2, Phase 3 11096-26-7, 209810-58-2
2 Hematinics Phase 2, Phase 3
3
Betamethasone Approved, Vet_approved Phase 1, Phase 2 378-44-9 9782
4 Betamethasone Valerate Phase 1, Phase 2 2152-44-5
5 glucocorticoids Phase 1, Phase 2
6 Hormone Antagonists Phase 1, Phase 2
7 Hormones Phase 1, Phase 2
8 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 1, Phase 2
9 Anti-Asthmatic Agents Phase 1, Phase 2
10 Anti-Inflammatory Agents Phase 1, Phase 2
11 Respiratory System Agents Phase 1, Phase 2
12 Betamethasone benzoate Phase 1, Phase 2
13 Betamethasone sodium phosphate Phase 1, Phase 2
14 Betamethasone-17,21-dipropionate Phase 1, Phase 2
15
Menthol Approved Phase 1 2216-51-5 16666

Interventional clinical trials:


id Name Status NCT ID Phase
1 EPO-4-Rhesus: Role of Darbepoetin Alfa in Reducing Late Anaemia in Neonates With Red Blood Cell Alloimmunization After Intrauterine Transfusions Not yet recruiting NCT03104426 Phase 2, Phase 3
2 Prenatal Steroids for Treatment of Congenital Cystic Adenomatoid Malformations (CCAM) Terminated NCT00670956 Phase 1, Phase 2
3 In Utero Hematopoietic Stem Cell Transplantation for Alpha-thalassemia Major (ATM) Not yet recruiting NCT02986698 Phase 1
4 Amniotic Fluid Tandem Mass Spectrometry for Pregnancies Complicated by NIH and Severe Symmetrical IUGR Terminated NCT00143039 Phase 1
5 Precision Medical Research of Non-immune Fetal Hydrops (NIFH)-From Prenatal Diagnosis to Intrauterine Treatment Not yet recruiting NCT02956564

Search NIH Clinical Center for Hydrops Fetalis

Genetic Tests for Hydrops Fetalis

Genetic tests related to Hydrops Fetalis:

id Genetic test Affiliating Genes
1 Nonimmune Hydrops Fetalis 29
2 Hydrops Fetalis 29

Anatomical Context for Hydrops Fetalis

MalaCards organs/tissues related to Hydrops Fetalis:

39
Heart, Lung, Placenta, Brain, Testes, Bone, Endothelial

Publications for Hydrops Fetalis

Articles related to Hydrops Fetalis:

(show top 50) (show all 559)
id Title Authors Year
1
An international registry of survivors with Hb Bart's Hydrops Fetalis Syndrome. ( 28057638 )
2017
2
Chylous Ascites in a Neonate with Hydrops Fetalis. ( 27670562 )
2016
3
EP03.14: Using spatiotemporal image correlation with M-mode display to monitor ventricular systolic function of hydrops fetalis. ( 27644483 )
2016
4
Hemoglobin Bart hydrops fetalis: A model for studying vascular changes in placental hypoxia. ( 27452444 )
2016
5
Spontaneous repigmentation of silvery hair in an infant with congenital hydrops fetalis and hypoproteinemia. ( 27416089 )
2016
6
Pulmonary neuroendocrine cell hyperplasia in hemoglobin Bart-induced hydrops fetalis: A model for chronic intrauterine hypoxia. ( 27598399 )
2016
7
EPHB4 kinase-inactivating mutations cause autosomal dominant lymphatic-related hydrops fetalis. ( 27400125 )
2016
8
Cerebral Venous Engorgement in Hydrops Fetalis. ( 27403227 )
2016
9
Lysosomal Storage Disorders in Nonimmune Hydrops Fetalis (NIHF): An Indian Experience. ( 27928775 )
2016
10
Perinatal and one-year outcomes of non-immune hydrops fetalis by etiology and age at diagnosis. ( 26712114 )
2016
11
Fetal Anemia and Hydrops Fetalis Associated with Homozygous Hb Constant Spring (HBA2: c.427Ta88>a88C). ( 26757782 )
2016
12
Congenital Chylothorax: A Unique Presentation of Nonimmune Hydrops Fetalis in a Preterm Infant. ( 26945279 )
2016
13
Zika Virus Infection and Stillbirths: A Case of Hydrops Fetalis, Hydranencephaly and Fetal Demise. ( 26914330 )
2016
14
Hydrops fetalis in a congenital chagas case in a non-endemic area. ( 26940819 )
2016
15
A Rare Association of Non-Compaction of the Ventricular Myocardium, and Non-Immune Hydrops Fetalis. ( 27328555 )
2016
16
Hydrops Fetalis Associated with Compound Heterozygosity for Hb Zurich-Albisrieden (HBA2: C.178Ga88>a88C) and the Southeast Asian (- -(SEA)/) Deletion. ( 27686733 )
2016
17
Optimizing chronic transfusion therapy for survivors of hemoglobin Barts hydrops fetalis. ( 26732098 )
2016
18
Antenatal diagnosis of idiopathic arterial calcification with hydrops fetalis. ( 25851317 )
2015
19
Single-tube nonaplex microsatellite PCR panel for preimplantation genetic diagnosis of Hb Bart's hydrops fetalis syndrome. ( 25641621 )
2015
20
Society for maternal-fetal medicine (SMFM) clinical guideline #7: nonimmune hydrops fetalis. ( 25557883 )
2015
21
Non-immune Hydrops fetalis due to Parvovirus B19 Infection in 2 Extremely Preterm Infants: Perinatal Management and Long-term Neurodevelopmental Outcome. ( 25830498 )
2015
22
Novel mutations in PIEZO1 cause an autosomal recessive generalized lymphatic dysplasia with non-immune hydrops fetalis. ( 26333996 )
2015
23
Perinatal-lethal Gaucher disease presenting as hydrops fetalis. ( 26327947 )
2015
24
Congenital Disorder of Glycosylation (CDG) Presenting as Non-immune Hydrops Fetalis. ( 26365158 )
2015
25
Familial Hemophagocytic Lymphohistiocytosis Presenting as Hydrops Fetalis. ( 26199792 )
2015
26
Rapid and reliable preimplantation genetic diagnosis of common hemoglobin Bart's hydrops fetalis syndrome and hemoglobin H disease determinants using an enhanced single-tube decaplex polymerase chain reaction assay. ( 26043722 )
2015
27
Fetal critical aortic stenosis with natural improvement of hydrops fetalis due to spontaneous relief of severe restrictive atrial communication. ( 25772579 )
2015
28
Hb H Hydrops Fetalis Syndrome Caused by Association of the -a88-(SEA) Deletion and Hb Constant Spring (HBA2: c.427Ta88>a88C) Mutation in a Chinese Family. ( 25897478 )
2015
29
Etiology of non-immune hydrops fetalis: An update. ( 25712632 )
2015
30
Clinical Features of Neonates with Hydrops Fetalis. ( 26070120 )
2015
31
Maternal and fetal capillary malformation-arteriovenous malformation (CM-AVM) due to a novel RASA1 mutation presenting with prenatal non-immune hydrops fetalis. ( 26096958 )
2015
32
Neuropathologic Characterization of Pontocerebellar Hypoplasia Type 6 Associated With Cardiomyopathy and Hydrops Fetalis and Severe Multisystem Respiratory Chain Deficiency due to Novel RARS2 Mutations. ( 26083569 )
2015
33
KLF1-null neonates display hydrops fetalis and a deranged erythroid transcriptome. ( 25724378 )
2015
34
Intracardiac Fetal Transfusion for Parvovirus-Induced Hydrops Fetalis: A Salvage Procedure. ( 26446819 )
2015
35
Hydrops fetalis in a preterm newborn heterozygous for the c.4A>G SHOC2 mutation. ( 24458587 )
2014
36
Transient myeloproliferative disorder and non-immune hydrops fetalis in a neonate with trisomy 21. ( 24473698 )
2014
37
Primary Endocardial Fibroelastosis and Nonimmune Hydrops Fetalis: Case Report with Autopsy. ( 25517884 )
2014
38
Non-immune hydrops fetalis without anemia due to parvovirus B19. ( 24140219 )
2014
39
Inferior vena cava Doppler indices in fetuses with hemoglobin Bart's hydrops fetalis. ( 24609794 )
2014
40
I+-thalassemia-associated hydrops fetalis: A rare cause of thyrotoxic cardiomyopathy. ( 25510540 )
2014
41
Nonimmune hydrops fetalis with ebstein anomaly. ( 25148508 )
2014
42
Prenatal control of Hb Bart's hydrops fetalis: a two-year experience at a mainland Chinese hospital. ( 24766075 )
2014
43
Thoracic ganglioneuromas resulting in nonimmune hydrops fetalis. ( 25032061 )
2014
44
Foetal supraventricular tachycardia with hydrops fetalis: a role for direct intraperitoneal amiodarone. ( 24811422 )
2014
45
Coronary arteriovenous fistula causing hydrops fetalis. ( 25247099 )
2014
46
A catecholamine-secreting neuroblastoma leading to hydrops fetalis. ( 24776602 )
2014
47
Histological, biochemical, and genetic characterization of early-onset fulminating sialidosis type 2 in a Korean neonate with hydrops fetalis. ( 23433491 )
2014
48
Mediastinal teratoma with hydrops fetalis in a newborn and development of chronic respiratory insufficiency. ( 25435854 )
2014
49
Accessory diaphragm associated with non-immune hydrops fetalis. ( 25100808 )
2014
50
De novo Reciprocal Translocation t(5;11)(q22;p15) Associated with Hydrops Fetalis (Reciprocal Translocation and Hydrops Fetalis). ( 25289481 )
2014

Variations for Hydrops Fetalis

Expression for Hydrops Fetalis

Search GEO for disease gene expression data for Hydrops Fetalis.

Pathways for Hydrops Fetalis

GO Terms for Hydrops Fetalis

Cellular components related to Hydrops Fetalis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 ficolin-1-rich granule lumen GO:1904813 8.62 GPI GUSB

Biological processes related to Hydrops Fetalis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 negative regulation of neuron apoptotic process GO:0043524 8.96 GPI RASA1
2 carbohydrate metabolic process GO:0005975 8.8 GBE1 GPI GUSB

Sources for Hydrops Fetalis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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