Rare Diseases Symptoms Automatic Extraction

Prenatal Prediction Of Pulmonary Arterial Hypertension In Congenital Diaphragmatic Hernia.

[congenital diaphragmatic hernia]

To evaluate the relevance of routinely prenatal prognostic markers as side of the defect, liver position, lung-to-head ratio (LHR), observed/expected lung-to-head ratio (o/e-LHR) and observed/expected total fetal lung volume (o/e-TFLV) at magnetic resonance imaging to predict the development of postnatal pulmonary arterial hypertension (PAH) in isolated congenital diaphragmatic hernia (CDH).110 isolated CDH were referred to our fetal medicine unit between January 2004 and April 2013. Mortality and morbidity were reviewed according to the presence of postnatal PAH. The following prenatal markers were evaluated as potential predictive factors of PAH: liver position, side of the defect, LHR and o/e-LHR measured by ultrasound, o/e-TFLV measured by magnetic resonance imaging. Univariate logistic regression was used to measure association.PAH was significantly associated with perinatal mortality and morbidity (P < 0.001). The occurrence of PAH significantly decreased with increasing LHR, o/e-LHR and o/e-TFLV, while significantly increased with intra-thoracic liver, but not in right-sided defects. Univariate regression found o/e-TFLV (OR = 0.9 [0.86-0.95] P < 0.05 for percentage unit change in o/e), LHR (OR = 0.19 [0.09-0.40] P < 0.05 per unit change), o/e-LHR (OR = 0.95 [0.93-0.98] P < 0.05 for percentage unit change in o/e), liver location (OR = 2.82 [1.13-7] P < 0.05 for liver up) were significant predictors of subsequent PAH. No differences were found after adjusting for gestational age at delivery. The area under the ROC curve was 0.80 and 0.75 for o/e-TFLV and o/e-LHR respectively.In CDH, PAH is associated with a high rate of mortality and morbidity. Routinely prenatal markers usually used for the assessment of pulmonary hypoplasia are also relevant for postnatal prediction of PAH.

Diseases presenting "hypertension" symptom

  • achondroplasia
  • acute rheumatic fever
  • adrenal incidentaloma
  • aniridia
  • aromatase deficiency
  • cadasil
  • child syndrome
  • cohen syndrome
  • congenital adrenal hyperplasia
  • congenital diaphragmatic hernia
  • cushing syndrome
  • cystinuria
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • esophageal adenocarcinoma
  • fabry disease
  • familial hypocalciuric hypercalcemia
  • gm1 gangliosidosis
  • heparin-induced thrombocytopenia
  • hereditary cerebral hemorrhage with amyloidosis
  • holt-oram syndrome
  • homocystinuria without methylmalonic aciduria
  • hydrocephalus with stenosis of the aqueduct of sylvius
  • inclusion body myositis
  • kallmann syndrome
  • kindler syndrome
  • lamellar ichthyosis
  • lymphangioleiomyomatosis
  • pendred syndrome
  • primary effusion lymphoma
  • scrub typhus
  • severe combined immunodeficiency
  • sneddon syndrome
  • typhoid
  • von hippel-lindau disease
  • well-differentiated liposarcoma
  • werner syndrome
  • x-linked adrenoleukodystrophy
  • zellweger syndrome

This symptom has already been validated