Incontinentia pigmenti with painful subungual tumors: a two-generation study.

We report 2 cases of painful subungual dyskeratotic tumors occurring in a mother and daughter with incontinentia pigmenti (IP) as a late manifestation of the disease. Both patients had a history of similar lesions appearing over a period of years on the digits of both the hands and feet. Biopsy specimens of the current lesions were examined and compared with the previous material available on both patients.

Splenogonadal fusion with limb deficiency and micrognathia.

Splenogonadal fusion (SGF) is a rare abnormality with two known types. In the continuous type, the spleen is connected to the gonad, and there are often limb defects, micrognathia, or other congenital malformations such as ventricular septal defect, anal atresia, microgastria, spina bifida, craniosynostosis, thoracopagus, diaphragmatic hernia, hypoplastic lung and abnormal lung fissures, polymicrogyria, deficient coccyx, and bifid spine C6-T3. The discontinuous type is usually not associated with congenital defects, and the gonad that fused with an accessory spleen has no connection with the native spleen.

Disseminated strongyloidiasis in a World War II Veteran with metastatic undifferentiated carcinoma of neuroendocrine type.

This case of disseminated strongyloidiasis illustrates the need for a high index of suspicion and effective screening for this pathogen in certain high-risk patient populations before giving immunosuppressive therapy. A complete travel and military history should be obtained. In the United States, persons with a history of military service in endemic areas, emigrants from endemic areas, patients in institutions, and residents of the Southeast should have careful evaluation for strongyloidiasis.

Extracellular matrix biosynthesis by cultured fetal rat lung epithelial cells. IV. Effects of chronic exposure to retinoic acid on growth, differentiation, and collagen biosynthesis.

Effects of chronic exposure to retinoic acid on a cell line (FRLE cells) established from the fetal rat lung type II alveolar epithelial cell have been studied. Chronic exposure to retinoic acid inhibited proliferation and altered the pattern of culture morphology at the light microscopic level. At the ultrastructural level, the development of lamellar body-like structures was inhibited.

Extracellular matrix biosynthesis by cultured fetal rat lung epithelial cells. III. Effects of chronic exposure to epidermal growth factor on growth, differentiation, and collagen biosynthesis.

Studies have been performed to evaluate the effects of chronic exposure to epidermal growth factor (EGF) on a cell line (FRLE cells) established from the fetal rat lung type II alveolar epithelial cell. Chronic exposure to EGF enhanced proliferation and altered the culture morphology at the light microscopic level. At the ultrastructural level, chronic exposure to EGF inhibited the expression of lamellar body-like structures that occurs in the absence of EGF.

Extracellular matrix biosynthesis by cultured fetal rat lung epithelial cells. II. Effects of acute exposure to epidermal growth factor and retinoic acid on collagen biosynthesis.

The effects of acute exposure to epidermal growth factor (EGF), retinoic acid, and EGF + retinoic acid on collagen biosynthesis by fetal rat lung epithelial (FRLE) have been evaluated. Acute exposure to either EGF or retinoic acid resulted in a small increase in the amount of radioactive substrate incorporated into total proteins, but simultaneous exposure to EGF + retinoic acid increased total protein synthesis approximately 2-fold. Quantitative evaluation of the genetic types of collagen synthesized by FRLE cells (types I, III, IV, and V) revealed that each experimental condition affected collagen production in a different manner.

Acute endotoxin-induced lymphocyte subset sequestration in sheep lungs.

Endotoxemia is associated with an early phase of pulmonary hypertension and a later increase in microvascular permeability. These physiologic changes are attended by peripheral blood and lung lymph leukopenia and a rapid accumulation of both granulocytes and lymphocytes in the peripheral lung. In the present study, the numbers of lymphocytes in blood, lung lymph, and lung tissue after infusion of endotoxin were determined by fluorescent labeling of lymphocyte populations with monoclonal antibodies to sheep T1, T4, T8, or leukocyte common antigen and with rabbit anti-sheep immunoglobulins (B cells).

Extracellular matrix biosynthesis by cultured fetal rat lung epithelial cells. I. Characterization of the clone and the major genetic types of collagen produced.

A new cell line of fetal rat lung origin has been established using the outgrowth procedure. One clone (2G3) isolated by this procedure exhibited during early passages some of the transmission electron microscopic features (e.g.

Morphology of noncardiogenic pulmonary edema induced by Perilla ketone in sheep.

A single infusion of Perilla ketone (PK) into sheep causes marked increases in lung fluid and solute exchange in the absence of any alteration in either pulmonary arterial or left atrial pressures. These alterations are most compatible with increased pulmonary microvascular permeability. The present paper describes the morphologic changes that accompany the previously described alterations in lung function.

Early structural changes in sheep lung following thoracic irradiation.

Using a large animal model of radiation lung injury--the sheep exposed to bilateral thoracic irradiation--we have recently shown the development of sustained pulmonary hypertension during the first 4 weeks following radiation. This is the period prior to the onset of pneumonitis and pulmonary fibrosis. In the present study, we have examined biopsy and autopsy lung tissue from these same sheep and assessed the sequential changes in lung morphology.

An elderly man with bilateral lung disease.

A 60-year-old man with a past history of sprue was admitted with cough and dyspnea and was found to have diffuse, bilateral interstitial lung disease with Kerley-B lines and pleural effusions. An open lung biopsy revealed a diffuse lymphatic infiltration of the interstitium with lymphocytes which were judged to be neoplastic rather than inflammatory. Thus non-Hodgkin's lymphoma was diagnosed.

A comparison of bronchiolitis obliterans with organizing pneumonia, usual interstitial pneumonia, and small airways disease.

This report is based on 43 cases where a diagnosis of either bronchiolitis obliterans with organizing pneumonia (BOOP), usual interstitial pneumonia (UIP), or small airways disease (SAD) was established by lung biopsy. The severity of histologic abnormalities in the peripheral airways and interstitial spaces were measured on these biopsies using semiquantitative techniques and compared with the clinical data available in 42 of 43 cases, preoperative chest radiographs in 31 of 43, and preoperative pulmonary function tests in 29 of 43. The data show that when a diagnosis of BOOP was made, there was a higher total pathologic score for membraneous bronchiolitis (MB) and respiratory bronchiolitis (RB) than for UIP and SAD (p less than 0.

Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings.

To assess the role of chest radiography in the differential diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP) and usual interstitial pneumonia (UIP), records of 34 patients with biopsy-proved BOOP (16 patients) or UIP (18 patients) were reviewed. Chest radiographs taken before biopsy were available in 26 patients, clinical information in 33, and pulmonary function data in 22. These data were reviewed independently, without knowledge of the pathologic diagnosis.