Degree Of Pulmonary Involvement Research Articles

Benign Esophagobronchial Fistula With and Without Esophageal Obstruction: Two Ends of the Surgical Spectrum

Acquired esophagobronchial fistula (EBF) is uncommon and its surgical remediation is challenging. Management depends on the cause, degree of pulmonary involvement, and existence of esophageal obstruction. We report management of two EBF cases representing extremes of the surgical spectrum. One patient with EBF secondary to mediastinal fungal infection underwent pulmonary resection and esophageal repair. Another, who was positive for human immunodeficiency virus, required esophageal resection and fistula closure, but no pulmonary resection. Successful outcome was achieved in both patients.

Endocrine and cytokine responses in humans with pulmonary tuberculosis

Endocrine responses during chronic infections such as lung tuberculosis are poorly characterized. Hormonal changes are likely to occur since some of the cytokines produced during this disease could affect endocrine mechanisms that, in turn, influence the course of infectious/inflammatory processes. A main purpose of this work was to study endocrine responses involving pituitary, adrenal, gonadal, and thyroid hormones in parallel to IFN-γ, IL-10, and IL-6 levels in tuberculosis patients with different degree of pulmonary involvement. We have also studied whether products derived from peripheral immune cells obtained from the patients can affect the in vitro production of adrenal steroids. The population studied comprised HIV-negative newly diagnosed, untreated male patients with mild, moderate, and advanced lung tuberculosis, and matched, healthy controls. IFN-γ, IL-10, and IL-6 levels were elevated in patients with tuberculosis. Dehydroepiandrosterone and testosterone levels were profoundly decreased and growth hormone levels were markedly elevated in patients, in parallel to modest increases in cortisol, estradiol, prolactin, and thyroid hormone concentrations. Supernatants of peripheral blood mononuclear cells obtained from the patients and stimulated in vitro with Mycobacterium tuberculosis antigens significantly inhibited dehydroepiandrosterone secretion by the human adrenal cell line NCI-H295-R. These results support the hypothesis that at least some of the endocrine changes observed in the patients may be mediated by endogenous cytokines. The endocrine profile of tuberculosis patients would favor a reduction of protective cell-mediated immunity and an exacerbation of inflammation leading to perpetuation of the lung injury and to the hypercatabolic condition that characterizes this disease.

Miliary Coccidioidomycosis in the Immunocompetent

Miliary coccidioidomycosis indicates hematogenous or lymphatic spread of Coccidioides immitis and is characterized by the development of multiple small granulomas throughout the lungs and other organs. Previous reports have suggested that this disorder occurs almost exclusively in immunocompromised patients, with most patients succumbing to progressive respiratory failure. In this article, we describe the largest series of immunocompetent patients with miliary coccidioidomycosis, define clinical characteristics, and outline important aspects of diagnosis and treatment. We identified eight patients (five men and three women; age range, 23 to 65 years) with miliary coccidioidomycosis diagnosed at Kern Medical Center (located in an endemic area) from 1990 to 1997. Four of the patients were white, two were African American, and two were Hispanic. A miliary pattern was defined as the presence of discrete 2- to 10-mm lesions diffusely distributed throughout both lung fields, as shown on chest radiograph. Microscopic examination and culture of C immitis from sputum, tissue, or body fluid confirmed diagnosis. Patients with HIV were excluded. These patients constituted approximately 1% of those admitted to our institution for coccidioidomycosis from 1990 to 1997. Four patients had symptoms for < or = 1 week before admission (acute), and four had symptoms for between 5 and 12 weeks (chronic). Four patients demonstrated a miliary pattern on initial chest radiograph, and two of these patients received an initial diagnosis of miliary coccidioidomycosis. Five patients required mechanical ventilation. Arterial blood gas measurements revealed a mean PO(2) of 54.2 +/- 8. 6 mm Hg and a mean PCO(2) of 32.5 +/- 3.2 mm Hg. Five patients developed ARDS. Five patients had extrapulmonary involvement, with the meninges (n = 4) and skin (n = 4) being the most common sites. All patients were treated with fluconazole and/or amphotericin B. Three patients died; all had chronic involvement and received mechanical ventilation. We present eight immunocompetent patients with a lower mortality rate and better outcome than previously reported. In our series, miliary coccidioidomycosis manifested as either an acute respiratory illness or an advanced stage of a chronic illness occurring in the context of widespread dissemination. All who died had chronic involvement. Prompt recognition of miliary coccidioidomycosis is crucial, but may be hindered by the large differential diagnosis. Important diagnostic factors include a history of travel through endemic areas, ethnicity, immunologic status, involvement of multiple organ sites, and pronounced hypoxemia not accounted for by the degree of pulmonary involvement seen on chest radiograph.

MILIARY COCCIDIOIDOMYCOSIS IN THE IMMUNOCOMPETENT

Background Miliary coccidioidomycosis indicates hematogenous or lymphatic spread of Coccidioides immitis and is characterized by the development of multiple small granulomas throughout the lungs and other organs. Previous reports have suggested that this disorder occurs almost exclusively in immunocompromised patients, with most patients succumbing to progressive respiratory failure. In this article, we describe the largest series of immunocompetent patients with miliary coccidioidomycosis, define clinical characteristics, and outline important aspects of diagnosis and treatment. Materials and methods We identified eight patients (five men and three women; age range, 23 to 65 years) with miliary coccidioidomycosis diagnosed at Kern Medical Center (located in an endemic area) from 1990 to 1997. Four of the patients were white, two were African American, and two were Hispanic. A miliary pattern was defined as the presence of discrete 2- to 10-mm lesions diffusely distributed throughout both lung fields, as shown on chest radiograph. Microscopic examination and culture of C immitis from sputum, tissue, or body fluid confirmed diagnosis. Patients with HIV were excluded. Results These patients constituted approximately 1% of those admitted to our institution for coccidioidomycosis from 1990 to 1997. Four patients had symptoms for ≤ 1 week before admission (acute), and four had symptoms for between 5 and 12 weeks (chronic). Four patients demonstrated a miliary pattern on initial chest radiograph, and two of these patients received an initial diagnosis of miliary coccidioidomycosis. Five patients required mechanical ventilation. Arterial blood gas measurements revealed a mean Po 2 of 54.2 ± 8.6 mm Hg and a mean Pco 2 of 32.5 ± 3.2 mm Hg. Five patients developed ARDS. Five patients had extrapulmonary involvement, with the meninges (n = 4) and skin (n = 4) being the most common sites. All patients were treated with fluconazole and/or amphotericin B. Three patients died; all had chronic involvement and received mechanical ventilation. Conclusion We present eight immunocompetent patients with a lower mortality rate and better outcome than previously reported. In our series, miliary coccidioidomycosis manifested as either an acute respiratory illness or an advanced stage of a chronic illness occurring in the context of widespread dissemination. All who died had chronic involvement. Prompt recognition of miliary coccidioidomycosis is crucial, but may be hindered by the large differential diagnosis. Important diagnostic factors include a history of travel through endemic areas, ethnicity, immunologic status, involvement of multiple organ sites, and pronounced hypoxemia not accounted for by the degree of pulmonary involvement seen on chest radiograph.

Circulating profile of Th1 and Th2 cytokines in tuberculosis patients with different degrees of pulmonary involvement

To investigate whether differences in the degree of pulmonary tuberculosis lesions could be accompanied by changes in the pattern of circulating cytokines, 29 untreated tuberculosis patients showing mild ( n=10), moderate ( n=5) or advanced ( n=14) pulmonary disease, and 12 age-matched healthy controls (mean±S.D., 36±15 years) were studied. ELISA methods for the evaluation of interferon-γ, interleukin-2, interleukin-4, and interleukin-10 indicated that all patients had increased serum levels of the four cytokines in relation to controls. Mean titers of interferon-γ and interleukin-2 in mild and moderate patients appeared higher than in those with advanced disease, whereas moderate and advanced patients showed the higher levels of IL-4 in comparison to mild cases. Raised levels of interleukin-10 were more prevalent in advanced disease, and statistically different from those in mild patients. This cytokine pattern may help to explain findings wherein mild tuberculosis is characterized by preserved cellular immune responses while advanced disease is accompanied by an impairment of such parameters.

Circulating profile of Th1 and Th2 cytokines in tuberculosis patients with different degrees of pulmonary involvement.

To investigate whether differences in the degree of pulmonary tuberculosis lesions could be accompanied by changes in the pattern of circulating cytokines, 29 untreated tuberculosis patients showing mild (n = 10), moderate (n = 5) or advanced (n = 14) pulmonary disease, and 12 age-matched healthy controls (mean +/- S.D., 36 +/- 15 years) were studied. ELISA methods for the evaluation of interferon-gamma, interleukin-2, interleukin-4, and interleukin-10 indicated that all patients had increased serum levels of the four cytokines in relation to controls. Mean titers of interferon-gamma and interleukin-2 in mild and moderate patients appeared higher than in those with advanced disease, whereas moderate and advanced patients showed the higher levels of IL-4 in comparison to mild cases. Raised levels of interleukin-10 were more prevalent in advanced disease, and statistically different from those in mild patients. This cytokine pattern may help to explain findings wherein mild tuberculosis is characterized by preserved cellular immune responses while advanced disease is accompanied by an impairment of such parameters.

Interrelationships Between Pulmonary and Extrapulmonary Involvement in Systemic Sclerosis: A Longitudinal Analysis

(1) To evaluate the relationship between the degree of pulmonary involvement by systemic sclerosis (SSc) and the degree of involvement of other organ systems by SSc at baseline. (2) To assess the degree of impairment in lung function at presentation and the annual rate of change in lung function to predict the rate of progression of involvement of extrapulmonary organ systems by SSc over time. (3) To determine whether survival in patients with SSc can be predicted from the degree of lung function impairment at baseline or from the annual rate of change in lung function. Semiquantitative indices of pulmonary and extrapulmonary involvement and pulmonary function tests (PFTs) were analyzed and compared in 62 nonsmoking scleroderma patients enrolled in a 3-year prospective drug trial, vs 47 in a "study group" who underwent serial evaluation. The other 16 "early withdrawals" withdrew prior to the second evaluation. The indices of organ system involvement were based on clinical, physiologic, and biochemical findings as previously published. The PFTs included total lung capacity (TLC), forced vital capacity (FVC), FEV1, and single-breath diffusing capacity for carbon monoxide (Dsb). Annualized rates of change in PFTs and indices of extrapulmonary involvement were calculated for each subject from data collected on at least 2 separate occasions at least 6 months apart. Spearman rank correlations were performed between individual baseline PFTs (expressed as percent predicted) and (a) indices of extrapulmonary involvement at baseline, (b) annualized rates of change in PFTs, and (c) annualized rates of change in indices of extrapulmonary involvement. Correlations also were performed between the rate of change in each lung function measure and rates of change in indices of extrapulmonary involvement. The ability of PFTs at baseline and their rates of change to predict cumulative survival was assessed by Cox stepwise regression. The degree of impairment in baseline PFTs was related to involvement of the right side of the heart but not to other extrapulmonary system involvement. Baseline PFTs were not related to the rate of subsequent decline of lung function or worsening of extrapulmonary organ system involvement. Subsequent annual rates of decline in lung function were related to worsening skin and upper gastrointestinal involvement. Cumulative survival may be related to the rate of decline in DCO, TLC, and FVC, but was not predicted by impairment in any measure of lung function. With the exception of involvement of the right side of the heart consistent with cor pulmonale, the degree of pulmonary involvement by SSc was not correlated with the extent of extrapulmonary involvement. The degree of pulmonary involvement by SSc did not predict subsequent worsening of either pulmonary or extrapulmonary involvement. Worsening pulmonary involvement by SSc, in general, does not correlate with worsening involvement of extrapulmonary organ systems, except for the skin and upper gastrointestinal tract. A rapid decline in DCO or lung volumes may predict poor survival.

α1-Proteinase Inhibitor, Elastase Activity, and Lung Disease Severity in Cystic Fibrosis

The potential role of neutrophil elastase in exacerbating pulmonary infection and tissue damage in cystic fibrosis (CF) has led to proposals for treatment of lung disease in CF with the elastase inhibitor, alpha 1-proteinase inhibitor (alpha 1PI). Reports that alpha 1PI is inactivated in the CF lung suggest that the effectiveness of alpha 1PI therapy depends on the quantity of elastase present and the extent of alpha 1PI inactivation, both of which are expected to vary with disease severity. In this study we assessed the elastase-alpha 1PI profile in sputum and plasma from CF patients with various degrees of pulmonary involvement. Levels of active elastase in sputum samples increased with severity of pulmonary disease (F ratio = 5.63, p < 0.01), as did sputum levels of alpha 1PI (F ratio = 4.88, p < 0.01). A positive correlation was observed between sputum levels of active elastase and alpha 1PI (r = 0.68, p < 0.005). Plasma alpha 1PI levels were also elevated in CF patients compared with control subjects (p < 0.005), indicating a compensatory increase in plasma and sputum levels of alpha 1PI in response to increased elastase load. Molar levels of total immunogenic neutrophil elastase were, on average, 12 times higher than alpha 1PI in CF sputum. These results suggest that the major contributor to the elevated levels of active elastase observed in the CF lung is an increase in elastase release rather than inactivation of alpha 1PI.

Pressure Controlled Inverse Ratio Ventilation in Respiratory Failure

Since the earliest use of mechanical ventilation for respiratory insufficiency the search has been on for optimal ventilation attuned to different degrees of pulmonary involvement. Pressure controlled inverse ratio ventilation is a recently described ventilatory modality, in which the conventional inspiratory to expiratory ratio is reversed. This method of ventilation allows for stabilization of pulmonary units and diffusion of gases.

Phenotypic heterogeneity in cystic fibrosis.

We have confirmed heterogeneity in CF using a different combination of primary clinical variables than those used in previous studies. Subgroupings of individuals with similar levels of sweat chloride were independent of the clustering based on level of pancreatic enzyme supplementation and degree of pulmonary involvement. Data from families with multiple CF children are consistent with the hypothesis that the genetic etiology of CF involves two or more genes that modify the expression of the primary gene defect.

Splenomegaly in Sarcoidosis

The clinical records of 32 patients with sarcoidosis associated with splenomegaly were reviewed. The results of this study disclosed that when compared with a matched control population without splenomegaly, patients with splenomegaly had evidence of more extensive extrathoracic sarcoidosis. In contrast, there was no difference in the degree of pulmonary involvement between patients with or without splenomegaly. Of the 32 patients with splenomegaly, seven (20%) had evidence of hypersplenism and five (16%) had abdominal symptoms. These abnormalities occurred only in patients with greatly enlarged spleens. Considering splenomegaly, we suggest that corticosteroids are indicated in the management of only large spleens and not of smaller spleens, unless there are other specific indications.

Benign metastasizing fibroleiomyoma. A case report.

SummaryA case is reported of benign metastasizing fibroleiomyoma with omental and pulmonary involvement. The patient showed a severe ventilation/perfusion respiratory defect. There have been 40 previously reported cases, only six with metastases to the lungs. This case appears to have been unique in the degree of pulmonary involvement.

A REVIEW OF 164 CHILDREN WITH MECONIUM ILEUS SEEN AT THE CHILDREN'S HOSPITAL MEDICAL CENTER, BOSTON

Meconium ileus is a complication of cystic fibrosis which still causes a high mortality rate in the first year of life. The condition is frequently complicated by meconium peritonitis, volvulus, and/or atresia, but these complications are not necessarily fatal. In the last ten years, the mortality rate for babies with meconium ileus has been about 40%. Thirty-five percent of children who survived the operation died from pneumonia in the first 6 months of life. Children who lived to 6 months had a prognosis similar to other children with cystic fibrosis who did not suffer from meconium ileus, and this will depend on the degree of pulmonary involvement. Some children with meconium ileus may have few pulmonary symptoms and, with good medical care reach adult life. Close cooperation between the surgeon and pediatrician and the early application of an intensive prophylactic and therapeutic pulmonary program are essential if we are to improve the mortality and morbidity in this disease.

Patterns of pulmonary fibrosis as related to pulmonary function.

Excerpt Discrepancies are frequently noted between the degree of lung involvement as shown by the roentgenogram and the severity of subjective symptoms of the patient. With the advent of pulmonary ...