1/20. Surfactant replacement therapy in RSV-induced acute respiratory distress syndrome (ARDS).Acute respiratory distress syndrome (ARDS) associated with severe respiratory syncytial virus infection is rare. We report a 5-month-old Indian girl who was admitted to our intensive care ward with severe respiratory failure who fulfilled the criteria for ARDS using both Murray's lung injury Score of > 2.5 and the American-European consensus Conference definition for ARDS. She developed diffuse bilateral alveolar infiltrates, severe hypoxaemia (PaO2/FiO2 < 100) and required high PEEP (> 15 cm H2O) 24 hours after admission. RSV was isolated from her nasopharyngeal secretion. She also had clinical features suggestive of a primary immunodeficiency and had laboratory evidence of combined T and B cell defect. There was unsustained clinical improvement with a dose of surfactant administered at 36 hours of PICU stay, and she continued to deteriorate and succumbed after 19 days in the PICU.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
2/20. 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.- - - - - - - - - - ranking = 4keywords = alveolar (Clic here for more details about this article) |
3/20. Compound SFTPB 1549C-->GAA (121ins2) and 457delC heterozygosity in severe congenital lung disease and surfactant protein B (SP-B) deficiency.Several human respiratory disorders have been linked to an abnormality of pulmonary surfactant synthesis or turnover. Among those conditions, hereditary deficiency in the hydrophobic surfactant protein B (SP-B) has been recognized as a rare cause of respiratory failure in term newborn infants. Homozygosity for a common mutation (1549C-->GAA, or 121ins2) of the SP-B-encoding gene (SFTPB) results in rapidly fatal respiratory failure, with complete absence of the mRNA and protein observed in lung fluid or biopsy specimens. Hereditary SP-B deficiency is also associated with aberrant processing of proSP-C and deficiency of the active SP-C peptide. In the present study, we characterized the SFTPB gene in an infant with severe unexplained respiratory distress and identified a paternally derived 1549C-->GAA lesion, as well as a hitherto unreported mutation (457delC) inherited from the mother. Analysis of bronchoalveolar lavage fluid demonstrated the complete absence of SP-B. However, unlike previous infants with hereditary SP-B deficiency, proSP-C was processed to the active SP-C peptide, suggesting that the defect in SP-B, rather than SP-C, caused the respiratory distress in this infant. The present findings demonstrate the importance of SFTPB in pulmonary function and support the need for further genotype-phenotype correlations in patients with SP-B deficiency.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
4/20. Deficiency of lamellar bodies in alveolar type II cells associated with fatal respiratory disease in a full-term infant.We report a case of a full-term female infant who presented with severe respiratory distress shortly after birth and died at 23 d of age with unremitting respiratory failure. Infectious and other known causes of respiratory disease in this clinical setting were excluded. Examination of a lung biopsy showed abnormal lung parenchyma with features reminiscent of desquamative interstitial pneumonitis. Ultrastructural studies revealed that alveolar type II cells lacked cytoplasmic lamellar bodies, while other organelles appeared normal. Histochemical and immunohistochemical investigations indicated normal alveolar type II cell marker expression including surfactant proteins (SP-A, SP-B, pro-SP-B, and pro-SP-C). Mutations in the coding sequences of the SP-B gene were excluded as a cause of disease. This case appears to be a novel congenital defect affecting the pulmonary surfactant system. The cellular abnormality may involve the assembly of cytoplasmic lamellar bodies in alveolar type II cells-the principal storage site of pulmonary surfactant.- - - - - - - - - - ranking = 7keywords = alveolar (Clic here for more details about this article) |
5/20. 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.- - - - - - - - - - ranking = 8keywords = alveolar (Clic here for more details about this article) |
6/20. Acute respiratory distress syndrome associated with scrub typhus: diffuse alveolar damage without pulmonary vasculitis.Pathologic findings of scrub typhus have been characterized by vasculitis of the microvasculature of the involved organ resulting from a direct invasion by orientia tsutsugamushi. We experienced a case of acute respiratory distress syndrome (ARDS) associated with scrub typhus. The case was proven by eschar and high titer of serum IgM antibody (positive at 1:1280). Open lung biopsy showed diffuse alveolar damage (DAD) in the organizing stage without evidence of vasculitis. Immunofluorescent antibody staining and polymerase chain reaction for O. tsutsugamushi failed to demonstrate the organism in the lung tissue. The patient expired due to progressive respiratory failure despite doxycycline therapy. Immunologic mechanism, without direct invasion of the organism, may participate in the pathogenesis of ARDS associated with scrub typhus.- - - - - - - - - - ranking = 5keywords = alveolar (Clic here for more details about this article) |
7/20. 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.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
8/20. 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.- - - - - - - - - - ranking = 3keywords = alveolar (Clic here for more details about this article) |
9/20. Clinical experience in using a new type of nasal prong for administration of N-CPAP.Nasal continuous positive airway pressure (N-CPAP) has been used in infants with decreased lung compliance for increasing the functional residual capacity (FRC), decreasing the work of breathing and improving the PaO2/PAO2 (arterial-alveolar PO2 ratio) without intubation. However, the currently available nasal prongs for administration of N-CPAP have presented some problems in fixation, and lesions to the nasal septum or nostrils might be induced by aggressive pressure intended to fix them. We would therefore like to report our experience in using a new type of nasal prong for administration of N-CPAP therapy. The nasal prongs we used were provided by Dr. Wung of Columbia University in new york, who first designed them, and have been used safely, effectively and without any complications.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
10/20. bile acid pneumonia: a "new" form of neonatal respiratory distress syndrome?We describe 3 cases of neonatal respiratory distress syndrome (RDS) in near-term infants, born from mothers with severe intrahepatic cholestasis of pregnancy. Common pictures of the cases were: good indices of lung maturity in the amniotic fluid; severe RDS requiring mechanical ventilation; high serum bile acid (BA) levels in the early days of life; no meconium aspiration; negative cultures; and absence of indirect laboratory signs of infection. After the first case, we hypothesized that abnormally high BA levels could have reversed the action of phospholipase A2 in the lungs, causing a degradation of phosphatidylcholines to lysophosphatidylcholines and the consequent lack of surfactant activity, leading to the severe respiratory distress. Consequently, in cases 2 and 3, we gave intratracheal surfactant to the infants, which, although administered around the first 24 hours of life, showed to be helpful. Our experience suggests that a high level of attention in the management of newborn infants (even near-term infants) born from women with intrahepatic cholestasis of pregnancy is necessary to detect as soon as possible signs and symptoms of this "unexpected" RDS, which can assume a very severe clinical picture. In such instances, we recommend that the diagnosis of BA pneumonia be kept in mind and that exogenous surfactant be given as soon as possible, even in the presence of indices of normal lung maturity in the amniotic fluid. Finding high levels of BA and lysophosphatidylcholines in the bronchoalveolar lavage of affected infants would aid in support of the diagnosis.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
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