Cases reported "Hyaline Membrane Disease"

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1/8. Potter sequence complicated by congenital cystic lesion of the bladder.

    We report a case complicated by oligohydramnios, pulmonary hypoplasia, bilateral renal dysplasia, and cystic lesion of the bladder. He was clinically compatible with Potter sequence. Congenital cystic bladder is the rarest form of the bladder. We can find no report of Potter sequence complicated by cystic lesion of the bladder. This lesion was similar to multilocular bladder. The diagnosis was confirmed it by autopsy, magnetic resonance imaging, and urography after his death.
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ranking = 1
keywords = dysplasia
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2/8. Bronchoplasty for bronchial stenosis in a neonate: a case report.

    Bronchial stenosis is an uncommon problem in children. Management can be difficult because of the small luminal diameter and proximity of the lung parenchyma to the bronchial lesion. Bronchoplasty procedures have obvious advantages in children because of their long life expectancy. Long-term functional results are superior compared with pneumonectomy because of preserved lung tissue. A premature neonate weighing only 779 g at birth was born with severe respiratory distress syndrome complicated with hyperbillirubinemia and bronchopulmonary dysplasia. The child underwent bronchoplasty using a costal cartilage graft for a right main bronchus stenosis after 2 failed attempts at bronchoscopic dilatations. In spite of all complications, the child improved sufficiently to be discharged without any oxygen dependency. Our case highlights the successful application of bronchoplasty in a very small baby. Preoperative and intraoperative bronchoscopy was helpful in localization of the endobronchial lesion. Understanding the principle, precise attention to technical detail and meticulous postoperative care contribute toward long-term benefit for pulmonary conservation.
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ranking = 4365.7929328827
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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3/8. Partial bronchial stenosis following inadvertent right bronchial intubation in a neonate.

    This case reports difficulties encountered in weaning a premature infant with bronchopulmonary dysplasia from prolonged mechanical ventilation. On chest X-ray alternating atelectasis and hyperinflation of the right lung were observed. This resulted from a short episode of misplaced endotracheal tube that produced a traumatic bronchial stenosis. Treatment by prednisolone allowed the detubation.
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ranking = 4365.7929328827
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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4/8. Abnormal lung surfactant related to essential fatty acid deficiency in a neonate.

    A low-birth-weight infant, suffering from chronic bronchopulmonary dysplasia following hyaline membrane disease and recurrent episodes of necrotizing enterocolitis, developed biochemical evidence of essential fatty acid (EFA) deficiency in the plasma. Fatty acid composition of phosphatidylcholine and phosphatidylglycerol in the lung lavage fluid was abnormal. plasma changes included a decrease in the level of linoleic acid and an increased level of palmitic, palmitoleic, oleic, and 5,8,11-eicosatrienoic acid to arachidonic acid being greater than 0.4:1. A lower than normal level of palmitic acid and an increased level of palmitoleic and oleic acids were seen in pulmonary sufactant phospholipid components. Upon treatment and recovery from EFA deficiency, the fatty acid pattern both in plasma and surfactant phospholipids returned to normal along with clinical improvement. An association between EFA deficiency and altered fatty acid composition of pulmonary surfactant phospholipids is suggested.
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ranking = 4365.7929328827
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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5/8. A distinct chondrodysplasia resembling Kniest dysplasia: clinical, roentgenographic, histologic, and ultrastructural findings.

    Two sibs, one girl and one boy, were observed in infancy with a severe lethal skeletal dysplasia syndrome that radiologically and histologically resembled Kniest dysplasia but clearly differed in clinical course and inheritance. Kniest dysplasia is a nonlethal syndrome, whereas both of these infants died in the neonatal period. Kniest dysplasia appears to be inherited as an autosomal dominant trait; the likely transmission in this family was autosomal recessive. Roentgenograms revealed dumbbell-shaped long bones superficially similar to Kniest dysplasia, but with markedly shortened diaphyses and metaphyseal irregularities. Chondro-osseous morphology demonstrated a superficially similar foamy "Swiss cheese" appearance to the cartilage matrix, as seen in Kniest dysplasia, but there were distinctly different changes in the growth plate and resting cartilage. Ultrastructurally, the chondrocytic endoplasmic reticulum was found to have an appearance different from that observed in either normal or Kniest cartilage. These cases likely represent a distinct chondrodysplasia.
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ranking = 15
keywords = dysplasia
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6/8. bronchopulmonary dysplasia: a case report.

    An unusual case of an extremely low birth weight and very preterm infant who developed bronchopulmonary dysplasia is presented. She required artificial (assisted) ventilation via a manual ambubag using a maximum concentration of 60% O2. Despite previous reports implicating mechanical ventilators and elevated peak airway pressures greater than 35cm of water, our infant still developed the disease with O2 delivery from an ambubag. Outcome was favourable. At 16 1/2 months follow-up, she appeared neurologically normal, and despite her prolonged neonatal respiratory problem, had not been troubled by chest disease or hospital readmissions. The observed etiopathogenesis is worthy of consideration in the 'developing world'.
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ranking = 4369.7929328827
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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7/8. Pulmonary pathology in surfactant-treated preterm infants with respiratory distress syndrome: an autopsy study.

    The present study examines the histological features of the lungs of neonates who died of respiratory distress syndrome or related complications after surfactant therapy. Our aim was to determine whether these lungs showed any unusual histological findings. Complete autopsies were performed 6-12 h after death in 10 surfactant-treated preterm infants and in 30 infants who died before surfactant therapy was available. Representative paraffin sections of all pulmonary lobes, stained with haematoxylin and eosin, were examined microscopically. A few selected slides were also stained with periodic acid-Schiff, Vierhoff-van Gieson, and Mallory trichrome. hyaline membrane disease and bronchopulmonary dysplasia were present in each group, although there was an increased incidence of intra-alveolar haemorrhage in surfactant-treated babies (in 8 of 10 surfactant-treated as compared with 7 of 30 untreated babies). Amongst those treated with surfactant, we observed the persistence of acute alveolar damage with unresolved hyaline membrane disease in 5 infants who died at the ages of 5, 6, 10, 12, and 13 days, respectively, and histological evidence of pneumocyte type 2 hyperplasia and dysplasia in 2 infants who died at 22 and 41 days of age, respectively. These observations reveal that surfactant-treated infants who fail to respond to therapy have continuing alveolar injury and an increased incidence of intra-alveolar haemorrhage. Since oxygen radicals can induce pneumocyte damage and necrosis and since free radicals provoke alveolar haemorrhage in animal models, we propose that the lesions we observed may stem from a lack, in some preterm babies, of specific mechanisms that detoxify oxygen radicals.
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ranking = 4366.7929328827
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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8/8. Bilateral multicystic kidney dysplasia in a fetus with neonatal pulmonary hyaline membrane disease: a case report.

    Multicystic kidney disease is the most common cause of neonatal renal mass and either the most or second most common neonatal abdominal mass. This disease is a congenital dysplasia of the kidney characterized by large nonhomogeneous dilations of the collecting tubules. It may occur unilaterally or bilaterally, and the incidence is about 1 out of 5,000 to 10,000 births. Severe bilateral defects are lethal. Partial dysplastic involvement of both kidneys eventually leads to renal function impairment. This is a case of bilateral multicystic kidney dysplasia diagnosed prenatally at 34 weeks of gestation. Hyaline membrane disease was found in both lungs after delivery. The antenatal diagnostic criteria for multicystic kidney disease and treatment after diagnosis are also discussed.
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ranking = 6
keywords = dysplasia
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