Hydrops Fetalis, Nonimmune

Alternative Names

  • NIHF
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WHO-ICD-10 version:2010

Certain conditions originating in the perinatal period

Haemorrhagic and haematological disorders of fetus and newborn

OMIM Number

236750

Mode of Inheritance

Autosomal dominant, autosomal recessive

Gene Map Locus

13q14.3

Description

Nonimmune Hydrops Fetalis is defined as the presence of excess extracellular body fluid in two or more sites, in the absence of any identifiable circulating antibody to RBC antigens. At an incidence rate of between 1:2000 and 1:3000, it is responsible for more than 87% of cases of Hydrops Fetalis. There may be several factors that may lead to the development of Nonimmune Hydrops Fetalis. The three most common of these factors are cardiac failure, anemia, and hypoproteinemia. Approximately a quarter of the cases have no identifiable cause. Fetal infections by Parvovirus, CMV, or Syphilis, and/or lymphatic abnormalities seen in conditions such as Cystic Hygroma, Noonan Syndrome, and others are also likely causes for the development of this condition. Hydrops Fetalis is a common feature of conditions such as Achondrogenesis, Alpha-Thalassemia, Turner Syndrome, and Osteogenesis Imperfecta. Prognosis for the condition is not very good. About 50% of affected fetuses die in utero, and a further 50% of infants born with the condition also succumb to it. Very rarely do affected infants, born before the 30th week of gestation, survive.

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
236750.1.1United Arab EmiratesFemaleYesYes Pleural effusion; Ascites; Hemangioma; P...NM_018676.3:c.1322_1329delHomozygousAutosomal, RecessiveAbdelrahman et al. 2018 Proband (patient 'IV...
236750.1.2United Arab EmiratesMaleYesYes Pleural effusion; Ascites; Hydrocele tes...NM_018676.3:c.1322_1329delHomozygousAutosomal, RecessiveAbdelrahman et al. 2018 Brother of 236750.1....
236750.1.3United Arab EmiratesMaleYesYes Pleural effusion; Ascites; Hydrocele tes...NM_018676.3:c.1322_1329delHomozygousAutosomal, RecessiveAbdelrahman et al. 2018 Cousin of 236750.1.1...
236750.1.4United Arab EmiratesMaleYesYes Ascites; Hydrocele testis; Hemangioma; P...NM_018676.3:c.1322_1329delHomozygousAutosomal, RecessiveAbdelrahman et al. 2018 Cousin of 236750.1.1...
236750.2United Arab EmiratesFemaleYesYes Premature birth; Nonimmune hydrops fetal...NM_018676.4:c.1561C>THomozygousAutosomal, RecessiveAl Rawi et al. 2021

Other Reports

Kuwait

Al-Essa et al. (1997) published the case of a newborn girl with gross fetal and neonatal hydrops due to a urogenital sinus abnormality. The baby was investigated for other known causes of hydrops fetalis, but results came back to be normal.

[Al-Essa M, Kazi A, Al-Hunayan A, Deverajan LV. Non-immune hydrops associated with urogenital sinus. Med Princ Pract. 1998; 7(3):226-9.]

Qatar

Saad et al. (2000) performed a review of cases of Intra-Uterine Fetal Death (IUFD) in Qatar. Of the 10,188 births that took place in the year 1997, a total of 83 were still born. Five of these still births were attributed to Nonimmune Hydrops Fetalis, giving the condition an incidence of 0.5 per 1000 births and 6.0 per 100 still births.

Saudi Arabia

Sasidharan et al. (1992) reported two cases of nonimmune hydrops fetalis diagnosed prenatally. One fetus died in utero while the second fetus survived and the infant was discharged from the intensive care nursery at 4 weeks of age.

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