1/7. Persistent pulmonary hypertension of the newborn due to alveolar capillary dysplasia.Three unrelated female term infants died when less than 1 month old from intractable pulmonary hypertension associated with deficient capillaries in airspace walls, anomalous small pulmonary veins in bronchiolar-arterial rays, and medial thickening in small pulmonary arteries together with peripheral muscularization. This complex vascular abnormality in the lungs has been termed alveolar capillary dysplasia and/or misalignment of lung vessels in seven previously reported cases. Each infant also showed abnormally immature parenchymal development in the lungs, as was noted in four of the seven prior cases. One had phocomelia; four of the seven prior cases had a variety of congenital anomalies. The primary pulmonary vascular anomaly is likely to be a failure of fetal lung vascularization dating from the second trimester and to be due to action of an unknown teratogen. Centroacinar veins may represent bronchial veins that do not normally develop beyond the ends of cartilaginous bronchi. Pulmonary arterial occlusive changes are interpreted as reactive to obstruction at the level of pulmonary arterioles.- - - - - - - - - - ranking = 1keywords = parenchymal (Clic here for more details about this article) |
2/7. Basilar arterio-venous pseudoparallelism due to persistence of embryonal venous pattern.A vascular malformation, consisting of a venous vessel bridgeing the right inferior petrosal sinus and the anterior spinal veins, was found in the posterior fossa. The vessel presented a ring-like course around the right trigeminal root, and it was parallel and dorsal to the basilar artery. The malformation was associated with cutaneous and hepatic angiomas and peri-osteal lipomas. It had been clinically silent for 52 years, when it thrombosed causing death. The authors think that, within a general mesenchymopatic state, this is a result of the persistence of an embryonal cerebral venous pattern.- - - - - - - - - - ranking = 0.2243988892312keywords = cerebral (Clic here for more details about this article) |
3/7. Occipital infarction with hemianopsia from carotid occlusive disease.Extracranial internal carotid artery occlusive disease usually produces stroke in the middle cerebral artery territory or the border zone between the middle and anterior cerebral arteries. It is unusual for occipital infarction in the posterior cerebral artery territory to be caused by internal carotid artery disease despite the fact that the posterior cerebral artery may arise directly from the internal carotid artery as an anatomic variation. We describe a patient with a fetal posterior cerebral artery originating from the internal carotid artery, and the initial manifestation of his extracranial internal carotid artery occlusive disease was hemianopsia from occipital infarction.- - - - - - - - - - ranking = 1.121994446156keywords = cerebral (Clic here for more details about this article) |
4/7. Misalignment of lung vessels: a syndrome causing persistent neonatal pulmonary hypertension.The cases of two female infants with persistent neonatal pulmonary hypertension are described. Combinations of pulmonary parenchymal lesions were present, including underdevelopment of alveoli and interstitial fibrosis as well as misalignment of lung vessels. As a result of the misalignment, the pulmonary veins joined the pulmonary arteries, rather than following a course away from them. One of the infants had additional congenital malformations. This syndrome should be considered in the evaluation of infants with persistent fetal circulation.- - - - - - - - - - ranking = 1keywords = parenchymal (Clic here for more details about this article) |
5/7. Persistent pulmonary hypertension in a very low birthweight preterm infant.Persistent pulmonary hypertension of the neonate (PPHN) characteristically is seen in full-term or postterm infants. Occasionally, PPHN complicates the course of hyaline membrane disease in preterm infants. This report documents the unusual occurrence of PPHN in a preterm very low birthweight infant without apparent pulmonary parenchymal disease.- - - - - - - - - - ranking = 1keywords = parenchymal (Clic here for more details about this article) |
6/7. Congenital alveolar capillary dysplasia: a developmental vascular anomaly causing persistent pulmonary hypertension of the newborn.The clinical course and histologic findings are presented of an infant with an unusual form of pulmonary dysplasia. Characteristic sonographic findings and progressive hypoxemia led to the diagnosis of persistence of the fetal circulation. The patient expired despite ventilatory and pharmacologic intervention. Postmortem findings of severe pulmonary capillary hypoplasia, despite normal anatomical and biochemical parenchymal maturation, were observed. It is suggested that factors controlling pulmonary capillary maturation may be significantly different from those involved in airway and pulmonary parenchymal development.- - - - - - - - - - ranking = 2keywords = parenchymal (Clic here for more details about this article) |
7/7. Congenital syphilis associated with persistent pulmonary hypertension of the neonate--a clinico-pathological case study.Congenital syphilis remains a significant clinical problem, especially in developing countries. We report a fatal case of congenital syphilis complicated by persistent pulmonary hypertension and hypoxic ischaemic encephalopathy. OBJECTIVE: To describe the association of congenital syphilis with persistent pulmonary hypertension of the newborn (PPHN). METHOD: Case report of a single patient. RESULTS: fatal outcome of one baby with congenital syphilis and associated PPHN despite maximal conventional treatment. Histological examination of the lungs revealed pulmonary oedema, intra-alveolar haemorrhages, localised bronchopneumonia and marked interstitial infiltrates of lymphocytes, plasma cells, macrophages and fibroblasts with interstitial fibrosis. Examination of peripheral pulmonary arteries revealed focal excessive muscularisation with increased adventitial connective tissue. DISCUSSION: Reviewing our own experience and available literature, this case study supports the infrequent association of congenital syphilis with PPHN. However, when it occurs, this combination appears to be fatal. CONCLUSION: More research is warranted to clarify the role of inflammatory mediators in congenital syphilis of the lung.- - - - - - - - - - ranking = 0.43782009307925keywords = haemorrhage (Clic here for more details about this article) |