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1/26. Prospective diagnosis of alveolar capillary dysplasia in infants with congenital heart disease.

    Alveolar capillary dysplasia (ACD) is a lethal pulmonary disorder found in newborns that is characterized by severe pulmonary hypertension and hypoxemia. We report on the clinical behavior of this disorder in a series of patients and its association with congenital heart disease, especially left heart obstructive disease; we also report a prospective diagnosis of ACD by lung biopsy in a newborn with congenital heart disease, which prevented futile and prolonged medical intervention.
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ranking = 1
keywords = dysplasia
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2/26. Congenital misalignment of pulmonary vessels and alveolar capillary dysplasia: how to manage a neonatal irreversible lung disease?

    Congenital misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia is a rare condition consisting of anomalous veins in bronchovascular bundles, a decreased number of alveolar capillaries, and increased muscularization of pulmonary arterioles. In the literature, infants reported as having such a malformation developed respiratory distress with persistent pulmonary hypertension and ultimately died. We report the case of an infant with MPV and alveolar capillary dysplasia who was unresponsive to maximal cardiorespiratory support, including high-frequency oscillatory ventilation and inhaled nitric oxide; the infant died of pulmonary hemorrhage after 19 days, during venoarterial extracorporeal membrane oxygenation bypass. We conclude that the diagnosis of MPV and alveolar capillary dysplasia should be considered during autopsy of infants who have died of irreversible persistent pulmonary hypertension. If a lung biopsy in infants with prolonged refractory hypoxemia confirms such diagnosis before death, expensive and invasive treatments such as extracorporeal membrane oxygenation could be avoided.
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ranking = 1.4
keywords = dysplasia
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3/26. cystic fibrosis in three children with bronchopulmonary dysplasia.

    cystic fibrosis (CF) and bronchopulmonary dysplasia (BPD) are two common causes of chronic lung disease in children. patients with BPD or CF often have recurrent respiratory symptoms, failure to thrive, and/or metabolic alkalosis during infancy and childhood. Thus, recognizing the diagnosis of CF in an infant with BPD can be difficult. We present three infants with both BPD and CF. The infants shared a history of respiratory distress and prolonged oxygen requirements. All three also had difficulty gaining weight, even after pancreatic enzyme supplementation was instituted. Metabolic alkalosis was observed in two infants. Previous studies in children with CF suggest that early diagnosis may impact both lung health and nutritional status. A high index of suspicion is necessary for clinicians to identify these children early and intervene with appropriate therapy.
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ranking = 6002.8208740224
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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4/26. Alveolar capillary dysplasia with misalignment of pulmonary veins and anterior segment dysgenesis of the eye: a report of a new association and review of the literature.

    The association of alveolar capillary dysplasia with misalignment of pulmonary veins (ACD-MPV) and ocular abnormalities has not been previously reported. We present a case of ACD-MPV and anterior segment dysgenesis of the eye in a full-term infant as well as a review of the relevant literature.
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ranking = 1
keywords = dysplasia
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5/26. decompression of multiple pneumatoceles in a premature infant by percutaneous catheter placement.

    Pneumatoceles due to acquired localized overinflation as a form of pulmonary interstitial emphysema are complications of advanced bronchopulmonary dysplasia. Different ventilation modes, selective bronchial intubation, balloon obstruction of the affected bronchus and steroids have been reported with success. Lobectomy has also been used. We present a premature infant with multiple large pneumatoceles causing respiratory compromise. In our case percutaneous decompression under fluoroscopy guidance resulted in a permanent cure.
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ranking = 1200.5641748045
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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6/26. The use of inhaled glucocorticosteroids and recovery from adrenal suppression after systemic steroid use in a VLBW premature infant with BPD: case report and literature discussion.

    Despite development of many prevention and treatment modalities for bronchopulmonary dysplasia (BPD), a form of chronic respiratory insufficiency in premature infants recovering from respiratory distress syndrome, BPD remains a treatment challenge and a significant cause of long-term morbidity. A ventilator-dependent very low birth weight infant in our newborn special care unit was receiving multiple courses of systemic dexamethasone for severe respiratory failure. The infant demonstrated adrenal suppression manifested by a baseline cortisol concentration below reported levels in infants of similar birth weight and postnatal age. We hypothesized that he had developed adrenal insufficiency as a result of the prolonged systemic steroid administration used to treat his respiratory problems. We further hypothesized that inhaled beclomethasone therapy would aid in the infant's recovery phase during relative adrenal insufficiency--and so substituted inhaled for systemic steroids. Inhaled corticosteroid treatment improved the clinical respiratory course and postnatal growth of this premature infant with BPD without inhibiting his recovery from adrenal insufficiency.
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ranking = 1200.5641748045
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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7/26. A case of acampomelic campomelic dysplasia.

    Acampomelic campomelic dysplasia is a rare variant of campomelic dysplasia syndrome affecting bone and connecting tissue. This syndrome is implicated by the absence of bowed limbs. Affected children have a characteristically smooth facial profile and are born with respiratory distress. A 15 day old Turkish boy presented with a small flat face, dolicocephalic head, proptotic eyes, short neck, low-set ears and a small thoracic cage. Limbs were mesomelically short and bilateral talipes equinovarus was present. The radiological findings indicated hypoplastic scapulae, narrow ribs, small thorax, thin claviculaes, and small iliac wings. Angulation of the femur, tibia and humerus was not observed. Our case, suited to acampomelic campomelic dysplasia, is discussed with differential diagnosis and compared with previously reported cases of the syndrome.
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ranking = 1.4
keywords = dysplasia
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8/26. Misalignment of lung vessels: diagnostic role of conventional histology and immunohistochemistry.

    Misalignment of lung vessels represents a rare congenital anomaly that may cause respiratory failure in the newborn. It is characterized by abnormal position of pulmonary veins and venules that lie adjacent to arteries and bronchi, and it is usually associated with a decreased number of alveolar capillaries (i.e., alveolar capillary dysplasia), although these two conditions have been separately described. awareness of this anomaly is required by pathologists because it can be easily overlooked on lung biopsy or autopsy, and because definite diagnosis relies on histology. We report the case of a newborn male baby who developed respiratory distress 18 h after an uncomplicated delivery. The patient died on the 7th day, after high frequency oscillatory ventilation, nitric oxide inhalation and extracorporeal membrane oxygenation were unsuccessful. On autopsy, histology and immunohistochemistry demonstrated diffuse changes, fulfilling diagnostic criteria of misalignment of lung vessels and of alveolar capillary dysplasia in both lungs, with muscularization of very peripheral pulmonary arteries and a prominent interstitial and periadventitial fibrosis. Diffuse distribution of vessel misalignment could explain the rapid onset of respiratory failure, and the presence of diffuse fibrosis might have contributed to irreversible respiratory dysfunction by impairment of lung parenchyma extensibility.
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ranking = 0.4
keywords = dysplasia
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9/26. High-frequency flow interruption in the rescue of preterm infants with severe RDS.

    High-frequency flow interruption (HFFI) was used successfully to rescue three preterm infants with severe respiratory distress syndrome (RDS) whose clinical condition continued to deteriorate while on the conventional mechanical ventilation. Had the HFFI not been used, the survival chances might have been 25 per cent for Case 1 and 2, and 45.5 per cent for Case 3. A dramatic, immediate, and sustained improvement in ventilation and oxygenation was demonstrated once the critical frequency and amplitude of HFFI were established. bronchopulmonary dysplasia which was already evidenced in one infant before the HFFI attempt was detected in two infants. This study demonstrates that HFFI is capable of achieving adequate gas exchange and improving survival in infants with severe RDS.
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ranking = 0.2
keywords = dysplasia
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10/26. Lethal skeletal dysplasia owing to double heterozygosity for achondroplasia and spondyloepiphyseal dysplasia congenita.

    A male infant with lethal short limbed dwarfism is described. His father had spondyloepiphyseal dysplasia congenita and his mother had achondroplasia. It is believed that the infant inherited both of these disorders and that their combined effects resulted in early death owing primarily to severe pulmonary hypoplasia.
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ranking = 1.8
keywords = dysplasia
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