Term:persistent fetal circulation syndrome
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Accession:DOID:13042 term browser browse the term
Definition:A syndrome of persistent PULMONARY HYPERTENSION in the newborn infant (INFANT, NEWBORN) without demonstrable HEART DISEASES. This neonatal condition can be caused by severe pulmonary vasoconstriction (reactive type), hypertrophy of pulmonary arterial muscle (hypertrophic type), or abnormally developed pulmonary arterioles (hypoplastic type). The newborn patient exhibits CYANOSIS and ACIDOSIS due to the persistence of fetal circulatory pattern of right-to-left shunting of blood through a patent ductus arteriosus (DUCTUS ARTERIOSUS, PATENT) and at times a patent foramen ovale (FORAMEN OVALE, PATENT).
Synonyms:exact_synonym: ACD MPV;   ACDMPV;   Alveolar Capillary Dysplasia;   Alveolar Capillary Dysplasia With Misalignment Of Pulmonary Veins;   Alveolar Capillary Dysplasia With Misalignment Of Pulmonary Veins And Other Congenital Anomalies;   Congenital alveolar capillary dysplasia;   Familial Persistent Pulmonary Hypertension of the Newborn;   Fetal circulation;   Misalignment of the Pulmonary Vessels;   Persistent Fetal Circulation;   Persistent Pulmonary Hypertension of Newborn;   congenital alveolar capillary dysplasia with misalignment of pulmonary veins;   persistent pulmonary hypertension of the newborn
 primary_id: MESH:C536590;   MESH:D010547;   RDO:0002218
 alt_id: OMIM:265380;   RDO:0002217
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persistent fetal circulation syndrome term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Cps1 carbamoyl-phosphate synthase 1 JBrowse link 9 74,113,437 74,236,274 RGD:1600716
G Foxf1 forkhead box F1 JBrowse link 19 53,012,306 53,016,100 RGD:7240710
G Nos3 nitric oxide synthase 3 JBrowse link 4 7,321,908 7,342,404 RGD:13592920
G Pten phosphatase and tensin homolog JBrowse link 1 251,421,814 251,487,634 RGD:13592920
G Stra6 stimulated by retinoic acid 6 JBrowse link 8 62,925,364 62,944,438 RGD:11554173

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  disease 14875
    syndrome 4220
      persistent fetal circulation syndrome 5
Path 2
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  disease 14875
    disease of anatomical entity 14051
      cardiovascular system disease 4056
        vascular disease 3039
          artery disease 1963
            hypertension 1452
              pulmonary hypertension 221
                persistent fetal circulation syndrome 5
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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.