Pulmonary hypoplasia: lung weight and radial alveolar count as criteria of diagnosis.

Askenazi, S S; Perlman, Max

doi:10.1136/adc.54.8.614

Cited by 294 publications

(156 citation statements)

References 19 publications

Supporting

Mentioning

146

Contrasting

Unclassified

Order By: Relevance

“…When autopsy was performed, the diagnosis of pulmonary hypoplasia was made when the lung/body weight ratio was less than 0.012. 20,21 Imaging of the pulmonary arteries was performed with combined power Doppler and 2-dimensional real-time sonography. The examinations were performed with either a PowerVision 7000 machine with a 5-MHz transducer and a pulse repetition frequency of 3 to 4.5 kHz (Toshiba Medical Systems Co, Ltd, Tokyo, Japan) or a Voluson 730 machine with a 3-to 8-MHz transducer and a pulse repetition frequency of 3 to 5 kHz (Kretztechnik, Zipf, Austria).…”

Section: Methodsmentioning

confidence: 99%

Evaluation of Fetal Pulmonary Vasculature by Power Doppler Imaging in Congenital Diaphragmatic Hernia

Michelet

Aubry

Sayed

et al. 2004

J of Ultrasound Medicine

View full text Add to dashboard Cite

Objective. To evaluate the feasibility of prenatal power Doppler imaging of pulmonary arteries in congenital diaphragmatic hernia and to study its potential to predict outcome. Methods. A prospective observational study was conducted. Forty-two cases of congenital diaphragmatic hernia (32 left and 10 right) without associated anomalies were analyzed. Qualitative evaluation of pulmonary vasculature was based on power Doppler imaging performed at 26 to 38 weeks. The pulmonary arteries were studied in the lung contralateral to the hernia. Pulmonary Doppler angiography was considered satisfactory when 3 levels of bifurcation defining 3 distinct segments of the pulmonary arteries were imaged and was otherwise considered poor. We also recorded the gestational age at diagnosis, side of the hernia, abdominal circumference below the third percentile, amniotic fluid volume, lung/thoracic area ratio, left/right ventricle ratio, and, in left-sided hernias, stomach position, and we carried out a multivariate analysis to determine the contribution of each factor to predict neonatal mortality. Results. More than 3 divisions of the fetal pulmonary arteries were imaged in 20 cases; 1 or 2 divisions or none were imaged in 22. Neonatal mortality was significantly greater when fewer than 3 divisions of the pulmonary arteries were imaged (18 [82%] of 22) than when 3 divisions could be identified on power Doppler imaging (5 [25%] of 20; P = .0005). However, the lung/thoracic area ratio was the only factor that remained significantly associated with mortality in the multivariate analysis. Conclusions. An altered pulmonary power Doppler image is associated with neonatal mortality, but estimation of the lung area remains the best predictor of neonatal outcome. Key words: congenital diaphragmatic hernia; lung/head ratio; lung/thorax ratio; power Doppler imaging; prenatal diagnosis; pulmonary hypoplasia; sonography. ongenital diaphragmatic hernia (CDH) diagnosed in utero has a neonatal mortality rate of 50% to 80%, 1-7 death being mainly due to pulmonary hypoplasia. Predicting the neonatal outcome prenatally remains a challenge. Moreover, prenatal staging of CDH is becoming increasingly important as new intrauterine therapeutic interventions emerge, including minimally invasive fetal surgery.8 So far, research on prenatal prediction of pulmonary hypoplasia has focused on using sonography or magnetic resonance imaging to assess the size of the lungs, 7,9-14 the degree of herniation of abdominal organs, 6,15,16 and the consequences of fetal hemodynamic changes on left ventricular size. 17,18 In CDH, however, pulmonary hypoplasia is associated with alterations of the pulmonary vasculature, accounting for potentially lethal postnatal pulmonary hypertension.19

show abstract

Section: Methodsmentioning

confidence: 99%

Evaluation of Fetal Pulmonary Vasculature by Power Doppler Imaging in Congenital Diaphragmatic Hernia

Michelet

Aubry

Sayed

et al. 2004

J of Ultrasound Medicine

View full text Add to dashboard Cite

show abstract

“…RAC is a useful indicator of the anatomical maturity of the lung (12), and the severity of pulmonary hypoplasia (13). In addition, RAC has been reported to correlate well with gestational age (14).…”

Section: Discussionmentioning

confidence: 99%

Expression of Intrapulmonary Surfactant Apoprotein-A in Autopsied Lungs: Comparative Study of Cases with or without Pulmonary Hypoplasia

et al. 2000

View full text Add to dashboard Cite

To investigate the functional maturity of the lungs of infants with pulmonary hypoplasia, we measured the expression of surfactant apoprotein-A (SP-A) in the autopsied lungs. Autopsied lungs were taken from 16 infants who died at birth or soon after. A lung-to-body weight ratio of less than 1.2% was defined as pulmonary hypoplasia. Eight infants were classified as belonging to the normal group, and eight as belonging to the pulmonary hypoplasia group. Many of the pulmonary hypoplasia group were complicated not only by pulmonary hypoplasia, but also by amniotic fluid volume abnormalities or an anatomical malformation. We measured the expression of SP-A immunologically using murine anti-human SP-A MAb in the autopsied lung tissue, and subjected the tissue to SP-A staining by the direct staining method. The expression of SP-A was assessed as one of four grades: Ϫ, Ϯ, 1ϩ, 2ϩ. The staining intensity of SP-A was high at 1ϩ or stronger in five infants of the normal group. SP-A expression was significantly reduced, however, in all infants of the pulmonary hypoplasia group except for one infant with normal amniotic fluid volume and relatively mild pulmonary hypoplasia. There was a significant negative correlation between the staining intensity of SP-A and two factors: pulmonary hypoplasia and abnormal amniotic fluid volume (p ϭ 0.039 and p ϭ 0.0063, respectively). In the present study, we demonstrated that SP-A expression was significantly reduced in infants with pulmonary hypoplasia. We speculate that the functional maturity of the lungs of infants with pulmonary hypoplasia is also suppressed. The alveolar lining is composed of type I and type II epithelial cells. Human pulmonary surfactants are lipid-protein complexes (lipoproteins) synthesized in type II cells. These surfactants are secreted into the alveolar space in order to cover the surface of alveoli and thus reduce alveolar surface tension. The functional expression of pulmonary surfactants requires specific proteins (apoproteins). To date, four types of surfactant apoproteins (SP) have been identified: SP-A, SP-B, SP-C, and SP-D. Of these, SP-A is the most prevalent form of SP (1). SP-A is a hydrophilic glycoprotein with a molecular weight of approximately 28 -36 kD, and the level of SP-A in the amniotic fluid, gastric juice, and respiratory aspirate is a useful indicator of the functional maturity of fetal and neonatal lung tissue (2, 3).In the present study, to assess the functional maturity of the lungs of infants with pulmonary hypoplasia, we measured the expression of SP-A immunohistologically using murine antihuman SP-A MAb in the autopsied lungs of infants who died at birth or soon after. METHODS Subjects.Autopsied lungs were taken from 16 infants at some point during a 21-y period between November 1976 and February 1998. Of the 16 infants, 14 came from the Division of Neonatal Intensive Care or the Department of Gynecology and Obstetrics at our hospital. These subjects had died at birth or within 5 d of birth. The remaining two infants came from the Neo...

show abstract

“…6 The incidence of congenital pulmonary hypoplasia is 1 in 1000 live birth; this includes both primary and secondary pulmonary hypoplasia. 4 But the incidence of pulmonary hypoplasia due to CAD is unknown and seems to be under recognized, because of diagnostic difficulty.…”

Section: Discussionmentioning

confidence: 99%

Congenital Acinar Dysplasia (CAD): Case Report of an Extremely Rare Cause of Pulmonary Hypoplasia

Mizanurrahman¹,

Zoani²,

Ameri³

2017

Bangladesh J Child Health

View full text Add to dashboard Cite

show abstract

Pulmonary hypoplasia: lung weight and radial alveolar count as criteria of diagnosis.

Cited by 294 publications

References 19 publications

Evaluation of Fetal Pulmonary Vasculature by Power Doppler Imaging in Congenital Diaphragmatic Hernia

Evaluation of Fetal Pulmonary Vasculature by Power Doppler Imaging in Congenital Diaphragmatic Hernia

Expression of Intrapulmonary Surfactant Apoprotein-A in Autopsied Lungs: Comparative Study of Cases with or without Pulmonary Hypoplasia

Congenital Acinar Dysplasia (CAD): Case Report of an Extremely Rare Cause of Pulmonary Hypoplasia

Contact Info

Product

Resources

About