Congenital alveolar proteinosis caused by a novel mutation of the surfactant protein B gene and misalignment of lung vessels in consanguineous kindred infants.

Unlabelled: Congenital alveolar proteinosis and misalignment of lung vessels are rare disorders. We report on five infants of consanguineous kindred. All infants were delivered at term after uneventful pregnancies.

Pulmonary hypertension after transjugular intrahepatic portosystemic shunt (TIPS).

We reported the case of a patient in whom severe, and ultimately fatal, pulmonary hypertension developed 1.5 yrs after transjugular intrahepatic portosystemic shunt (TIPS). Pulmonary artery pressures were not affected by 100% oxygen, prostacyclin or nifedipine.

A stable model of respiratory distress by small injections of oleic acid in pigs.

Objective: Development of a stable model of respiratory distress in pigs with oleic acid, fulfilling clinical criteria of the adult respiratory distress syndrome (ARDS).

Design: Eight pigs (9.1 +/- 0.

Pathology of pulmonary thromboembolism.

The incidence of pulmonary thromboembolism is hard to assess by the pathologist as a result of seasonal variation of embolism and disappearance of emboli by thrombolysis. However, the great differences in estimates of the incidence in routine hospital autopsies is mainly related to variation in scrutiny of the investigation and in size of area searched microscopically. Obstruction of major pulmonary arteries almost always results from thromboembolism which is most often found in its acute stage.

Pulmonary vasoconstriction in oleic acid induced lung injury. A morphometric study.

Distribution and severity of active vasoconstriction of muscular pulmonary arteries were morphometrically assessed in anaesthetized, paralysed and mechanically ventilated pigs with respiratory distress, induced by oleic acid. Vasoconstriction was deduced from the medial thickness which was measured and expressed as a percentage of external diameter. Six pigs received oleic acid (0.

Pulmonary veno-occlusive disease and Hodgkin's lymphoma.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension, and is of unknown aetiology. It has seldom been described in association with malignant disease and cytotoxic chemotherapy, and there have been only two previous reports of an association with Hodgkin's disease [1, 2]. We report a third case which developed 14 yrs after Hodgkin's disease had been diagnosed and treated, initially with radiotherapy and subsequently with chemotherapy.

Thrombotic lesions in primary plexogenic arteriopathy. Similar pathogenesis or complication?

In 78 patients with primary plexogenic arteriopathy (PPA), numbers of organized and recanalized thrombi were established in histologic slides of lung tissue and expressed per square centimeter of section. Three control groups of ten individuals each were used: normal, plexogenic arteriopathy secondary to ventricular septal defect, and hypoxic pulmonary hypertension. Thrombotic lesions were scarce in normal individuals but numerous in all three groups with pulmonary hypertension.

Histological features of a polymer endovascular prosthesis after transcatheter implantation in porcine arteries.

A novel approach for the treatment of acute complications and the prevention of restenosis after percutaneous transluminal angioplasty may be the placement of endovascular prostheses (stents). Stents constructed of metal have proven to be thrombogenic, and although they show a tendency to reduce restenosis, they do not prevent it. Pursuant to the search for stents with improved material and surface characteristics, we report in this paper the histological results obtained with a synthetic polymer (polyethylene terephthalate) stent after placement in porcine peripheral arteries.

Polyalveolar lobe and congenital cystic adenomatoid malformation type II: are they related?

A left lower polyalveolar lobe in a 28-day-old infant and a right lung with congenital cystic adenomatoid malformation type II in a 17-day-old infant are described. The adenomatoid malformation involved all lobes, but the lower lobe appeared to be mostly replaced by a polyalveolar area, a complication hitherto undescribed. The pronounced increase in number of alveoli in both cases was established by two different morphometric methods and was compared with three normal controls of the same age.

Ultrastructural heterogeneity of lung carcinomas: representativity of samples for electron microscopy in tumor classification.

Histologic heterogeneity of tumors is a well-known phenomenon, which has been repeated studied at the light microscopic level. Electron microscopy has been advocated as an adjunct in classification of tumors which pose difficulties on light microscopic classification. However, in view of tumor heterogeneity, it might be anticipated that the problem of sample error could detract from the usefulness of electron microscopy in tumor typing.

[Arterial hypertension and veno-occlusive disease. Connections with pulmonary hemorrhage].

Primary pulmonary hypertension, as defined by the World Health Organization, specifically includes three entities: primary plexogenic arteriopathy, pulmonary veno-occlusive disease, and silent recurrent thromboembolism. Pulmonary hemorrhage may occur in each of these syndromes but is not usually a prominent symptom. The hemorrhage is probably the result of a variety of causes and related to the specific morphologic characteristics of the pulmonary vasculature in these diseases.

Morphological substrate for the reversibility and irreversibility of pulmonary hypertension.

The reversibility of pulmonary hypertension in hypertensive pulmonary vascular disease depends, in the first place, on the feasibility of eliminating the cause of the elevation of pressure. Equally important in this respect are the type, the severity, and the extent of the pulmonary vascular lesions. This implies that various forms of pulmonary hypertension have completely different tendencies for regression.

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.

Medial defects of lung vessels: a new cause of pulmonary hypertension.

Defects, abrupt thinning of the media of muscular pulmonary arteries and pulmonary veins, were observed in two female patients, one 20 and the other 11 years of age. This congenital anomaly was associated with vasoconstriction and medial hypertrophy of adjacent segments of the vascular wall, as well as with a peculiar type of intimal proliferation. The subsequent intimal fibrosis was essentially limited to the hypertrophied segments and caused arterial obstruction and pulmonary hypertension in one of the patients.

The 'grey area' between small cell and non-small cell lung carcinomas. Light and electron microscopy versus clinical data in 14 cases.

We studied 14 lung tumours which on light microscopy had posed difficulties on classification as either small cell or non-small cell carcinomas. The light and electron microscopical features were compared with patient follow-up data. Electron microscopy showed neuroendocrine granules in 12 cases, and adeno- and squamous cell differentiation but no neuroendocrine granules in the remaining two cases.

Comparison of primary plexogenic arteriopathy in adults and children. A morphometric study in 40 patients.

Pulmonary vascular changes were studied in histological sections from 15 children and 25 adults with primary plexogenic arteriopathy. The severity of medial hypertrophy and degree of vasoconstriction were measured in histological sections and there was a close correlation between these two variables in both children and adults. More advanced arterial changes, expressed as an index of pulmonary vascular disease, were more common in adults, and their severity correlated positively with the degree of medial hypertrophy.

Pulmonary veno-occlusive disease: involvement of pulmonary arteries and review of the literature.

Pulmonary vessels from 26 patients with pulmonary veno-occlusive disease were studied histologically and morphometrically. In addition to the well-known obstruction of veins and venules, pulmonary arteries were also narrowed or obliterated in approximately half of the patients. It is unlikely that the arterial intimal fibrosis, which was sometimes as severe as the fibrosis in the veins, was secondary to the venous obstruction; rather, like the venous alterations, it probably resulted from organization of thrombi.