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Human pulmonary hypoplasia was studied statistically and pathologically in a large series of autopsy cases. Multiple logistic regression analysis indicated five independent risk factors from 10 statistically significant factors for pulmonary hypoplasia: (1) hydrops fetalis; (2) renal anomalies; (3) hernia, including diaphragmatic hernia and omphalocele; (4) skeletal anomalies; and (5) abnormalities of amniotic fluid, such as oligohydramnios and polyhydramnios. The characteristics of pulmonary hypoplasia for each factor were defined by morphological, morphometric, and biochemical methods. All bronchiolar branching, acinar complexity, and acinar maturation were retarded in hypoplastic lungs with hydrops fetalis, renal anomalies, affected side of diaphragmatic hernia, omphalocele, and skeletal anomalies. Only acinar complexity and maturation were impaired in the lung with oligohydramnios due to prolonged rupture of membranes. The pathogenesis of pulmonary hypoplasias should be considered differently with each associated anomaly and time of impairment. While impairment in early gestational stage before 16 weeks' gestation results in both reduced bronchiolar branching and retarded acinar development, that, at late stage, influences only acinar development.
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PMID:Human pulmonary hypoplasia. Statistical, morphological, morphometric, and biochemical study. 161 25

The accuracy of prenatal diagnosis has become increasingly critical in the field of high-risk obstetrics. Although ultrasound (US) provides adequate information in most cases and continues to be the initial prenatal examination of choice, there are instances in which the results of the US study may be equivocal. The role of magnetic resonance (MR) imaging was explored in 27 selected patients with various indications to determine its effectiveness as a complement to US. MR imaging was most helpful in the diagnosis of extrauterine gestation, evaluation of placental position, determination of extent or nature of masses associated with pregnancy, and differentiation between diaphragmatic hernia and a thoracic mass. Although MR imaging did not add information that affected the accuracy of the diagnosis of oligohydramnios, in all other cases it provided an extra dimension in diagnosis by showing clearer anatomic relationships in the pelvis. It has proved to be a valuable complement to an equivocal US study.
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PMID:MR imaging in high-risk obstetric patients: a valuable complement to US. 173 85

The fetal urinary bladder was visualized sonographically in 1254 (94%) of 1335 consecutive fetuses of 14 weeks of development or older, but it could not be seen in 81 cases (6%). Five of these cases were lost to follow-up and were excluded from the study. Of the remaining 76 cases, 69 (91%) of the pregnancies progressed to term, and the infants were normal at birth and at 6 week perinatal follow-up. Seven (9%) of the fetuses had a variety of associated obstetrical abnormalities that resulted in fetal demise or termination of the pregnancy (oligohydramnios, hydrops, intrauterine growth retardation [IUGR], ventriculomegaly, diaphragmatic hernia, cystic hygroma, and triploidy). Notably, none of these were renal tract anomalies. We concluded that (1) nonvisualization of the fetal urinary bladder with an otherwise normal sonogram, including normal volume of amniotic fluid and normal renal areas, is of no clinical concern and does not require follow-up, and (2) nonvisualization of the fetal urinary bladder in the setting of associated obstetrical abnormalities may be secondary to renal tract anomalies or may only be a consequence of the associated abnormalities.
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PMID:Significance of nonvisualization of the fetal urinary bladder. 181 Oct 78

Pulmonary acinar development was assessed in 17 autopsy cases of pulmonary hypoplasia associated with diaphragmatic hernia. Morphologic examination was conducted by light and electron microscopy, and morphometric study was achieved by radial alveolar count and biochemical quantitation of surfactant phospholipid. In most cases of unilateral diaphragmatic hernia, the ipsilateral lung was underdeveloped morphologically and biochemically compared with the contralateral lung. However, both ipsilateral and contralateral lungs were well developed in some cases of unilateral diaphragmatic hernia. Thus, acinar development of hypoplastic lung in diaphragmatic hernia is varied. The influence of other conditions, such as oligohydramnios, polyhydramnios, and other anomalies that may influence retention of lung fluid or fetal respiratory movement, should be considered to assess the acinar development of hypoplastic lung in diaphragmatic hernia.
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PMID:Pulmonary acinar development in diaphragmatic hernia. 201 98

Normal human pulmonary elastic fiber development and development in some pathological conditions were examined using elastic stains by light microscopy, electron microscopy, and immunohistochemistry. In normal development elastic fibers, composed mainly of microfibrils, first appeared around primitive bronchioles at 10 weeks of gestation. As they matured, their appearance became more amorphous, and they extended into the peripheral alveolar walls. Development of elastic fibers was retarded in the hypoplastic lungs of the oligohydramnios syndrome, diaphragmatic hernia, and hydrops fetalis. Elastic development was also retarded in congenital pulmonary lymphangiectasia and in focal areas of lungs with pulmonary dysplasia. Distribution of well-developed elastic fibers was found around the dilated bronchioles and alveoli in cases of congenital cystic adenomatoid malformation and extralobar pulmonary sequestration. Elastic fibers were distributed irregularly and unevenly in the lungs of bronchopulmonary dysplasia and ventilated cases of Wilson Mikity syndrome. In addition, four very immature infants who had progressively deteriorating respiratory function showed an almost total lack of elastic fibers in their alveolar walls.
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PMID:Pulmonary elastic fibers in normal human development and in pathological conditions. 223 57

Magnetic resonance imaging (MRI) has been used sparingly in obstetrics. The reasons for this relate mainly to cost, availability, difficulty obtaining clear images because of fetal movement, and the convenience and utility of ultrasound. However, MRI use is expanding and has the potential to play an important role in selected problem pregnancies. We studied the pregnancies of five women whose fetuses showed anomalies by ultrasound. These included cases of a large body wall defect, a diaphragmatic hernia, hydrocephalus, Meckel-Gruber syndrome, and iniencephaly with a diaphragmatic hernia (the iniencephaly sequence). Three of these examinations followed fetal neuromuscular blockade, and two were associated with oligohydramnios without fetal paralysis. Paralysis provided superior images. The fetal central nervous system, subcutaneous tissue, and liver imaged particularly well. This study illustrates that MRI can enhance and even clarify certain information provided by ultrasound.
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PMID:Magnetic resonance imaging of anomalous fetuses. 265 26

Oligohydramnios, whatever the cause, results in lungs that are 'hypoplastic' or at least smaller than normal. The fine structure in such lungs has not been reported previously. Three fetal lambs had both ureters ligated at 60, 72 and 58 days of gestation. They were sacrificed at 117, 143 and 142 days of gestation. The lungs were fixed by tracheal perfusion at 15 mm Hg. Individual lobar volumes were determined, and the lungs were sampled for electron microscopy. Total lung volumes for 2 of the lambs were very close to those previously reported for lungs of equivalent gestation with diaphragmatic hernia. The third lamb had a twin that had been unrecognised at the time of the original procedure. This lamb's lung volumes were normal. The morphological appearances of the lungs at the electron microscopy level are not normal but dramatically different from those previously reported in lambs with diaphragmatic hernia, phrenic nerve avulsion, and fetal tracheostomy. The lungs of the lamb with bilateral ureteric ligation and a normal twin were essentially normal. These preliminary findings point to the need for more studies of lung structure in oligohydramnios/obstructive uropathy models. However, the normal lungs in a lamb with grossly dysplastic kidneys and a normal twin argue against any important renal trophic factor in lung development in the fetal lamb. An interesting sidelight to this investigation was the unequivocal demonstration that early ureteric ligation results in true renal dysplasia in the fetal lamb. The kidneys of the lamb sacrificed at 117 days were smaller than normal and grossly dysplastic. The kidneys of the lambs that underwent ureteric ligation at 60 and 72 days were structurally different, with the ureters ligated at 60 days being small and dysplastic, while those with the ureters ligated at 72 days were larger and appeared almost polycystic.
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PMID:Development of fetal lamb lung and kidney in obstructive uropathy: a preliminary report. 307 15

To discriminate between different forms of pulmonary hypoplasia (PH), 24 hypoplastic lungs were studied for their development of bronchial cartilage plates and peripheral air spaces. In 6 lungs from premature infants with oligohydramnios, normal amounts of immature and irregularly shaped cartilages were distributed with mitoses concentrated toward the periphery of the bronchi. Pulmonary acini appeared markedly immature. In 5 lungs from infants with diaphragmatic hernia, large numbers of cartilage bars were clustered around the proximal bronchi, whose branching was much reduced. Peripheral air spaces were small but structurally mature. In Potter syndrome, small amounts of tiny, mature cartilages were observed irregularly around the proximal bronchi and poorly distributed into the peripheral bronchi. The acinar structure was very immature. In 4 anencephalic infants, a marked decrease in the volume of mature cartilage was present, with cartilage seen only around proximal bronchi; the acini were atelectatic and less well developed. It is suggested that the earlier the action of a teratogen, the greater the abnormality of bronchial branching, cartilage distribution, and later lung development.
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PMID:A study of cartilage development in pulmonary hypoplasia. 339 56

83 cases of pulmonary hypoplasia were found in 709 perinatal necropsies in St. Mary's Hospital between 1976 and 1983. 49 of these infants were inborn, representing an incidence of 1.4 per 1000 births. Diaphragmatic hernia was present in 43% and was the commonest reason for referral, usually to the neonatal surgical unit, from an outside hospital. Renal malformation, often with oligohydramnios, was seen in 25%, and 11% had no other major disease. The respiratory symptoms associated with pulmonary hypoplasia in the absence of diaphragmatic hernia seem to have encouraged the referral of these infants to the neonatal medical unit. However, more than half the liveborn infants with pulmonary hypoplasia died during the first postnatal day. In comparison with first week deaths from other causes, short survival was a particular feature of pulmonary hypoplasia in infants weighing less than 2.5 kg and pneumothorax and pulmonary haemorrhage were commoner in term infants with hypoplastic lungs.
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PMID:Pulmonary hypoplasia in a regional perinatal unit. 373 17

Quantitative biochemical criteria for lung growth and maturation were compared with the histological appearances in hypoplastic lungs from 20 fetuses and newborn infants. Cases associated with oligohydramnios showed a characteristic series of changes with narrow airways, retardation of epithelial and interstitial growth, delay in development of blood-air barriers, and low concentrations of phospholipid phosphorus, lecithin phosphorus, total palmitate, and lecithin palmitate. The growth and maturation arrest appeared to affect the peripheral part of the acinus. Examples of other types of lung hypoplasia showed different features. Hypoplastic lungs from infants with normal or increased amniotic fluid were of mature structure with phospholipid concentrations similar to those of infants with normally developed lungs at term. The hypoplastic left lung in 2 cases of congenital diaphragmatic hernia had an immature structure with low phospholipid concentrations, whereas the right lung has structurally and biochemically more mature. It is suggested that fetal lung growth may be impaired by any influence which reduces thoracic volume but that maturation arrest is due specifically to loss of the ability to retain lung liquid.
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PMID:Fetal lung hypoplasia: biochemical and structural variations and their possible significance. 702 90


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