Showing papers on "Alveolar capillary dysplasia published in 1997"

Combined therapy with inhaled nitric oxide and intravenous prostacyclin in an infant with alveolar-capillary dysplasia

Abstract: Severe persistent pulmonary hypertension of the newborn (PPHN) remains a significant cause of neonatal morbidity and mortality with limited effective treatment options. We present the first case of a neonate with PPHN treated concurrently with inhaled nitric oxide (iNO) and intravenous prostacyclin (PGI2). He ultimately was diagnosed with alveolar-capillary dysplasia, a rare and fatal cause of pulmonary hypertension. However, his partial response to treatment demonstrates a possible role for combined therapy with iNO and PGI2 in infants with severe PPHN.

Alveolar capillary dysplasia: lung biopsy diagnosis, nitric oxide responsiveness, and bronchial generation count.

Abstract: Alveolar capillary dysplasia, a rare cause of neonatal pulmonary hypertension characterized by a developmental abnormality in the pulmonary vasculature, was diagnosed by lung biopsy in a male newborn maintained on nitric oxide therapy for 18 days. Autopsy confirmed the pulmonary vascular defect and demonstrated deficient airspace formation. In addition, a bronchial generation count was low, suggesting that the abnormal lung vascular development in this condition represents a special form of pulmonary hypoplasia that starts in early fetal life.

Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension

Abstract: We report on a rare case of fatal congenital alveolar capillary dysplasia. The newborn boy of a 37 weeks' normal gestation suffered from persistent pulmonary hypertension without any cardiovascular malformation and died at the age of 4 weeks despite intensive treatment. The autopsy tissue was examined histologically, immunohistochemically, and ultrastructurally. Moreover, a three-dimensional tissue reconstruction based on serial sections was performed comparing the affected lung with normal lung tissue. We observed a unique pattern of pulmonary dysplasia: An extreme decrease of capillaries was localized centrally within thickened intra-acinar septa instead of capillaries intensely neighboring pneumocytes; ectatic veins normally running in the interlobular septa were found to accompany intralobular bronchovascular bundles, denying a clear distinction between pulmonary and bronchial veins; small muscular pulmonary arteries extended to the precapillary level and type 2 pneumocytes exceeded by far the type 1 pneumocytes, inverting the normal ratio. In summary, alveolar capillary dysplasia is assumed to be a primary capillary disorder of unknown origin, which possibly involves the regular differentiation of pneumocytes, according to the close alveolocapillary relationship during pulmonary ontogenesis. We consider the venous alterations as being part of the dysplasia, whereas the arterial phenomena might occur secondarily. Recent reports on affected siblings suggest a genetic component of pathogenesis.

Alveolar capillary dysplasia with and without misalignment of the veins. 1604

Abstract: Alveolar capillary dysplasia (ACD) has assumed clinical importance because of the associated pulmonary hypertension that fails to respond to ECMO. We present two term infants with ACD and congenital heart disease who both failed ECMO therapy. The lungs were filled and fixed postmortem with 10% formalin at 24 cm H2O pressure. Tissue blocks from the left lung were later fixed with glutaraldehyde, cut 1 mm thick and stained with toluidine blue to assess alveolar capillary density. Regular 5-μm sections were stained with hematoxylin and eosin for lung histology. The two infants had pulmonary lobules with round, immature air spaces, lined by cuboidal epithelium separated by hypocapillarized thickened walls (ACD). One infant also had small malpositioned veins accompanying small pulmonary arteries (misalignment of the pulmonary veins; MPV), cortriatrium, and a hypoplastic left heart. The other had no MPV, pulmonary lymphangiectasia, right lung isomerism, and double outlet right ventricle. In conclusion, ACD may occur with and without MPV, possibly depending upon the stage of development during which the abnormality occurs. Eighty-five percent of infants with ACD have additional congenital abnormalities. ACD with and without MPV should be suspected in those newborns with persistent pulmonary hypertension, especially when congenital abnormalities are present.