Topic
Alveolar capillary dysplasia
About: Alveolar capillary dysplasia is a research topic. Over the lifetime, 219 publications have been published within this topic receiving 5569 citations. The topic is also known as: Alveolar capillary dysplasia with misalignment of pulmonary veins.
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TL;DR: The variable clinical, genetic, and pathology findings of 4 patients with atypical late onset and/or prolonged survival of alveolar capillary dysplasia are presented.
26 citations
TL;DR: Because it is shown that such a lesion may also develop as a result of HCV infection in the liver graft of a patient not transplanted for PBC, it is believed it should not be considered as the hallmark of PBC.
Abstract: experienced a histopathologically typical acute rejection episode with marked bile duct injury prior to HCV recurrence, and it is therefore possible that the bile duct injury had, in some way, interfered with the pattern of HCV damage. Third, the use of immunosuppressive agents could have interfered with the immune mediatedor direct cytopathic HCV related injury. This granulomatous bile duct destruction was comparable to the florid duct lesion described as a characteristic feature of PBC and whose presence has recently been used as evidence of PBC recurrence liver grafts6. Because we have shown that such a lesion may also develop as a result of HCV infection in the liver graft of a patient not transplanted for PBC, we believe it should not be considered as the hallmark of PBC. References
26 citations
TL;DR: The authors adopted a policy of weaning from ECMO before repair in an effort to avoid hemorrhagic risks and reviewed their experience with CDH patients who required ECMO for stabilization before repair but for whom post-ECMO repair was planned.
25 citations
TL;DR: A prospective diagnosis of ACD by lung biopsy in a newborn with congenital heart disease, which prevented futile and prolonged medical intervention, is reported.
Abstract: 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.
24 citations
TL;DR: In full-term babies with unexplained progressive respiratory distress from birth and progress of radiological changes, both AD and SPBD should be considered.
Abstract: Background: Full-term infants with severe and prolonged respiratory distress represent a diagnostic challenge. Plain radiographic findings may be nonspecific or similar to classic surfactant deficiency disease for infants with surfactant protein B deficiency and acinar dysplasia. Objectives: To describe the similar clinical-radiolgical patterns of two rare neonatal conditions. Materials and methods: Six newborn babies with severe respiratory distress at birth demonstrated clinical and radiographically prolonged and progressive diffuse pulmonary opacification. Results: All infants demonstrated hyperinflation of the lungs. The diffuse hazy opacification, which varied from mild (n=3) to moderate (n=3), progressed to severe diffuse opacification preceding death, which occurred at 12–36 days of life. Open lung biopsy confirmed the diagnosis of primary alveolar acinar dysplasia (AD) in four infants and surfactant protein B deficiency (SPBD) in two infants. Conclusions: In full-term babies with unexplained progressive respiratory distress from birth and progress of radiological changes, both AD and SPBD should be considered.
23 citations