eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | alveolar capillary dysplasia with misalignment of pulmonary veins |
| Comorbidity | C0031190|persistent pulmonary hypertension of the newborn |
| Sentences | 41 |
| PubMedID- 24429387 | One study2 did note that maternal depression may not be independently associated with persistent pulmonary hypertension of the newborn, as the study did not find an association with the condition for non-ssri antidepressants or when exposure to ssris was restricted to the first half of pregnancy. |
| PubMedID- 20540801 | Pphn: persistent pulmonary hypertension of the newborn; no (ino): nitric oxide (inhaled); rds: respiratory distress syndrome; pao2: arterial partial oxygen pressure; paco2: arterial partial co2 pressure; sato2: arterial oxygen saturation; hfov: high frequency oscillatory ventilation; fio2: inspired oxygen fraction; map: mean airway pressure; ecmo: extracorporeal membrane oxygenation; aado2: alveolar-arterial oxygen gradient; oi: oxygenation index; ninos: neonatal inhaled nitric oxide study group; aga: adequate for gestational age; mdi: motor developmental index; pdi: performance developmental index; nicu: neonatal intensive care unit. |
| PubMedID- 23056879 | Caplan et al reported high paf plasma levels in newborns with persistent pulmonary hypertension of the newborn (pphn), correlation between plasma paf level and disease severity, and a fall in paf levels as they improved clinically [39]. |
| PubMedID- 25521916 | persistent pulmonary hypertension of the newborn (pphn) represents a failure of the normal postnatal adaptation that occurs at birth in pulmonary circulation. |
| PubMedID- 21766014 | Hypoxemic respiratory failure is commonly associated with persistent pulmonary hypertension of the newborn (pphn), a syndrome characterized by failure to achieve or maintain the normal decrease in pulmonary vascular resistance (pvr) that occurs after birth [1]. |
| PubMedID- 24400269 | persistent pulmonary hypertension of the newborn is suspected clinically in term and near-term infants who have variable hemoglobin oxygen saturation. |
| PubMedID- 23304599 | This entity may occasionally remain unrecognized and untreated; however, in most cases pat presents as respiratory failure [3], severe persistent pulmonary hypertension of the newborn, or cyanotic congenital heart disease [8]. |
| PubMedID- 25657596 | Infants recorded with a surgical procedure must have at least one diagnosis recorded after the procedurepulmonary hypertension (i27) at two occasions or morepersistent pulmonary hypertension of the newborn (p293x*) on two or more occasionssurgery on heart or major thoracic vessels (fa, fb, fc, fd, fe, ff, fg, fh, fi, fj, fk, fl, fm, fn, fo, fq, fr, fs, ft, fu, fv, fy, fz) followed by a diagnosis of pulmonary hypertension (i27 or p293xinfants below 12 months of age with a diagnosis of cardiomyopathy and heart failure who are on triple medication. |
| PubMedID- 20932343 | Mice deficient in endothelial nitric oxide synthase (e-nos) displayed defective lung vascular development, which resembles the alveolar capillary dysplasia in infants with persistent pulmonary hypertension of the newborn (pphn). |
| PubMedID- 23970432 | The morbidities analysed among the infants were respiratory distress syndrome (rds), air leak syndrome, pulmonary haemorrhage, persistent pulmonary hypertension of the newborn (pphn), cld at 28 days after birth, cld at 36 weeks of corrected age, symptomatic pda, late-onset adrenal insufficiency of prematurity, intraventricular haemorrhage (ivh), ivh grade iii/iv, periventricular leukomalacia, sepsis and necrotising enterocolitis (nec)/intestinal perforation. |
| PubMedID- 25785205 | persistent pulmonary hypertension of the newborn (pphn) is a life threatening condition mostly occurring with multiple severe neonatal conditions but hardly ever associated with congenital viral infection. |
| PubMedID- 23662203 | Some phvds are unique to childhood, such as persistent pulmonary hypertension of the newborn (pphn), bronchopulmonary dysplasia, lung hypoplasia and alveolar capillary dysplasia. |
| PubMedID- 22655195 | Most commonly, it presents immediately after birth, a condition referred to as persistent pulmonary hypertension of the newborn (pphn), when pulmonary vascular resistance fails to decrease at birth. |
| PubMedID- 25887340 | persistent pulmonary hypertension of the newborn (pphn), first described as “persistence of fetal circulation” by gersony and sinclair in 1969 [1], is a cyanogenic disorder characterized by the lack of postnatal drop of pulmonary vascular resistance and by the persistence of the typical intrauterine right-to-left shunting of blood through foramen ovale and ductus arteriosus. |
| PubMedID- 25877290 | This is especially important for term infants with conditions such as mas who will benefit from higher oxygen saturations to prevent persistent pulmonary hypertension of the newborn. |
| PubMedID- 24339037 | persistent pulmonary hypertension of the newborn has been associated to antenatal exposure to nsaid (manchester et al., 1976; csaba et al., 1978; goudie and dossetor, 1979; rubaltelli et al., 1979; wilkinson et al., 1979; turner and levin, 1984; besinger et al., 1991; norton et al., 1993; alano et al., 2001; tarcan et al., 2004; hernández-díaz, 2007), even though a recent case–control study could not confirm this risk (van marter et al., 2013). |
| PubMedID- 22518190 | Mas is frequently accompanied by persistent pulmonary hypertension of the newborn (pphn) [32], with many factors contributing to its development, including low po2 and ph, coexistent intrauterine asphyxia, and possibly vasoactive substances in the meconium itself [33]. |
| PubMedID- 25905096 | The youngest patient is an hiv-infected very-low birth-weight infant, which presented with neonatal pah: his condition resolved with haart treatment but confounding factors, such as persistent ductus arteriosus, prolonged mechanical ventilation, extreme prematurity, systemic infection, and persistent pulmonary hypertension of the newborn, may have contributed to the disease (105). |
| PubMedID- 21829408 | Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (pphn) and other pulmonary conditions are necessary. |
| PubMedID- 20224752 | It has been shown to be beneficial in pediatrics for idiopathic pulmonary hypertension [22–25], persistent pulmonary hypertension of the newborn [26–28], and for pulmonary hypertension related to congenital heart disease [29–34]. |
| PubMedID- 20727133 | Alveolar capillary dysplasia is a rare disorder, presenting with persistent pulmonary hypertension of the newborn [179]. |
| PubMedID- 24671232 | Yet, numerous studies are now available that suggest antidepressant use during pregnancy may be associated with spontaneous abortion [85], birth defects [86], persistent pulmonary hypertension of the newborn [87], and newborn behavioral syndrome [88]. |
| PubMedID- 22539968 | Unlike those developing persistent pulmonary hypertension of the newborn (pphn) from other causes (notably meconium aspiration syndrome), infants with cdh do not respond consistently well to inhaled nitric oxide (ino) [8], [37]. |
| PubMedID- 21485749 | However, maternal antidepressant use during pregnancy has been associated with documented risks to exposed infants including persistent pulmonary hypertension of the newborn (pphn) and a neonatal withdrawal/toxicity syndrome. |
| PubMedID- 24587948 | persistent pulmonary hypertension of the newborn was excluded when the level of preductal oxygen saturation was <5% above postductal oxygen saturation. |
| PubMedID- 24353699 | persistent pulmonary hypertension of the newborn (pphn) is a clinical syndrome that results from failure of normal fetal- to- neonatal circulatory transition and is associated with substantial neonatal morbidity and mortality.1although persistent pulmonary hypertension is less common, but more significant cause of respiratory distress in newborns than others like transient tachypnea of newborn, respiratory distress syndrome and etc. |
| PubMedID- 24049749 | persistent pulmonary hypertension of the newborn (pphn) is a serious neonatal illness, which in the past was associated with high mortality and morbidity. |
| PubMedID- 22614905 | persistent pulmonary hypertension of the newborn is not a single disease but rather a pathophysiologic condition. |
| PubMedID- 25814954 | Failure to adapt to postnatal life results in persistent pulmonary hypertension of the newborn (pphn), characterized by elevated pulmonary vascular resistance, right-to-left extrapulmonary shunting of deoxygenated blood and severe hypoxemia (steinhorn, 2010). |
| PubMedID- 24701562 | Moreover, h2o2 decreased sgc expression and consequently no-dependent cgmp generation in pulmonary arterial smcs from lambs with persistent pulmonary hypertension of the newborn and in rat aortic smcs or freshly isolated vessels [130, 131]. |
| PubMedID- 20169087 | He developed meconium aspiration syndrome (mas) and resultant persistent pulmonary hypertension of the newborn (pphn) and was placed on a conventional ventilator. |
| PubMedID- 20412580 | We also did not encounter any patient with pah in venice group 1.5 (persistent pulmonary hypertension of the newborn). |
| PubMedID- 20416033 | The altered alveolar development in the current study is consistent with lung pathologies seen in humans with alveolar capillary dysplasia [33], persistent pulmonary hypertension of the newborn [4,33] and disrupted alveolarization in infants with bpd [34]. |
| PubMedID- 22984626 | Infants admitted with persistent pulmonary hypertension of the newborn (pphn) to a regional neonatal intensive care units (the university of alabama at birmingham) from 2002–2009 were evaluated. |
| PubMedID- 25477529 | In persistent pulmonary hypertension of the newborn, the majority of intra-acinar arteries in the lung have this constricted appearance. |
| PubMedID- 21189937 | In the newborn with structurally normal heart, a differential cyanosis may be associated with persistent pulmonary hypertension of the newborn. |
| PubMedID- 22124769 | Exclusion criteria were congenital anomalies, severe circulatory shock, or persistent pulmonary hypertension of the newborn. |
| PubMedID- 25565893 | Infants recorded with a surgical procedure must have at least one diagnosis recorded after the procedure.○pulmonary hypertension (i27) at two occasions or more○persistent pulmonary hypertension of the newborn (p293x*) at two occasions or more○surgery on heart or major thoracic vessels (fa, fb, fc, fd, fe, ff, fg, fh, fi, fj, fk, fl, fm,fn, fo, fq, fr, fs, ft, fu, fv, fy, fz) followed by a diagnosis of pulmonary hypertension (i27 or p293xinfants below 12 months of age with a diagnosis of cardiomyopathy and heart failure, whom are on triple medication. |
| PubMedID- 22190957 | persistent pulmonary hypertension of the newborn has also been described with an absolute risk of <1%. |
| PubMedID- 23946901 | persistent pulmonary hypertension of the newborn (pphn) is a syndrome of failed circulatory adaptation at birth, characterized by high pulmonary vascular resistance causing extrapulmonary left-to-right shunting of blood across the ductus arteriosus and/or foramen ovale.1 selective pulmonary vasodilatation with inhaled nitric oxide (ino) has been proven to improve oxygenation in term newborns with severe hypoxemic respiratory failure (hrf) and pphn.2,3 randomized controlled trials have demonstrated that ino significantly decreased the need for extracorporeal membrane oxygenation in newborns greater than 34 weeks' gestational age with hrf.4,5 traditionally, ino has been delivered to intubated and mechanically ventilated babies when they continue to be hypoxemic despite administration of high levels of inspired oxygen. |
| PubMedID- 24404382 | He successfully applied ecmo in newborn infants with acute respiratory failure and persistent pulmonary hypertension of the newborn, which usually resolves within a few days of birth. |
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