Familial cystic nephroma and pleuropulmonary blastoma

BACKGROUND: Cystic nephroma (CN) and pleuropulmonary blastoma (PPB) are rare tumors without any previously recognized familial association. METHODS: Two cases of CN and one case of PPB in three siblings are reported. RESULTS: A 27-month-old girl and a 31-month-old boy underwent nephrectomy for CN and are free of disease in the contralateral kidney 16 and 14 years later, respectively. Their 28-month-old sister underwent pleuropneumonectomy with postoperative chemotherapy for PPB and died of recurrent disease 9 months later. CONCLUSION: To the knowledge of the authors, these cases represent the first reported familial occurrence of CN and the second of CN and PPB among siblings. The inheritance of a germline mutation predisposing to the development of these tumors is postulated.     

Similarities between pleuropulmonary blastoma and Askin tumor

AIM: The purpose of the study was to determine the risk of postoperative complications and the functional outcome after a hand-sewn ileal pouch-anal anastomosis (IPAA) for ulcerative colitis using a single J-shaped pouch design. METHODS: Preoperative function, operative morbidity and long-term functional outcome were assessed prospectively in 1310 patients who underwent IPAA between 1981 and 1994 for ulcerative colitis. RESULTS: Three patients died after operation. Postoperative pelvic sepsis rates decreased from 7 per cent in 1981-1985 to 3 per cent in 1991-1994 (P = 0.02). After mean follow-up of 6.5 (range 2-15) years, the mean number of stools was 5 per day and 1 per night. Frequent daytime and nighttime incontinence occurred in 7 and 12 per cent of patients respectively, and did not change over a 10-year period. The cumulative probability of suffering at least one episode of 'clinical' pouchitis was 18 and 48 per cent at 1 and 10 years and the cumulative probability of pouch failure at 1 and 10 years was 2 and 9 per cent respectively. CONCLUSION: These results indicate that increased experience decreases the risk of pouch-related complications and that with time the functional results remain stable, but the failure rate increases.     

Alpha-fetoprotein producing pulmonary blastoma in a patient with systemic sclerosis: pathogenetic analysis

We describe a rare case of pulmonary blastoma complicated with systemic sclerosis. The serum alpha-fetoprotein level was elevated at presentation and the biopsied tumour stained positively against anti-alpha-fetoprotein antibody. The alpha-fetoprotein produced autonomously by tumour cells was of yolk-sac origin. Although the pathogenesis of pulmonary blastoma has not been clarified, we suggest that this pulmonary blastoma is a type of yolk-sac tumour.     

Pulmonary thromboembolism

Pulmonary embolism (PE) is a serious complication of chronic obstructive pulmonary disease (COPD). Retrospective studies on patients with COPD treated in the intensive care unit (ICU) were performed to determine: 1) the frequency of PE; 2) the clinical course of PE in cases of COPD in the ICU; and 3) the frequency of PE as a cause of death in the studied group. The frequency of PE was 10.9% in COPD patients. In the group analysed, clinical presentation of PE was characterized by acute severe, life-threatening complications leading to death in 86.7% of cases. PE was the most frequent cause of death (40.6%) in COPD patients in the ICU. The results of treatment of pulmonary embolism in chronic obstructive pulmonary disease are poor and mortality in this group of patients is very high. We believe that improvement of management can be achieved by antithromboembolic prophylaxis, which should be instituted as soon as possible in all patients with chronic obstructive pulmonary disease in the intensive care unit.     

Pulmonary surfactant

Study of the pulmonary surfactant system has brought important information about its composition, metabolism and functions in the last decades. Besides phospholipids an important role is played by surfactant proteins (SP-A, B, C, D). These proteins aid not only the reduction of the surface tension of the alveoli, but they are involved in regulation of surfactant synthesis, secretion and in local immune defense in the lungs. Impairment of surfactant was found not only in premature newborns (IRDS), but also in cases of meconium aspiration, pneumonia and in other lung diseases in adults. Therapy by stimulation of surfactant synthesis and secretion and by intrapulmonary application of exogenous surfactant was proved effective in therapy of surfactant-deficiencies.     

Pulmonary sarcoidosis

Sarcoidosis involves the bronchi or lung in more than 90 percent of patients. Intrathoracic manifestations are protean, ranging from asymptomatic bilateral hilar lymphadenopathy to chronic, progressive, (ultimately fatal), respiratory insufficiency. The clinical course is highly variable, and optimal management and treatment are controversial. We review the salient radiographic, physiologic, and histopathologic features of pulmonary sarcoidosis and discuss rare intrathoracic complications (e.g., bronchostenosis, mycetomas, nodular sarcoidosis, necrotizing sarcoid angiitis and granulomatosis, pulmonary vascular and pleural involvement). We discuss the chest radiographic staging system and the role of ancillary diagnostic modalities including high resolution thin section computed tomographic scans (HRCT), bronchoalveolar lavage, radionuclide scan, and serum angiotensin enzyme converting enzyme. Indications for therapy and an overview of therapeutic options are outlined.     

Pulmonary melioidosis

Melioidosis is the name given to all diseases caused by the bacterium Pseudomonas pseudomallei. Melioidosis is a tropical disease and prevails in parts of Southeast Asia, northern Australia, and Central and South America. However, in recent years, cases of melioidosis have been reported in the United States and other areas. The organism can infect any organ system, although the lung is the most common organ affected. Pulmonary melioidosis presents either as an acute fulminant pneumonia or as an indolent cavitary disease. In northeastern Thailand, the incidence of P pseudomallei infection is extremely high with significant mortality. One of the key problems with treating melioidosis is its recalcitrance to therapy and high relapse rate. In addition, this Gram-negative rod is resistant to aminoglycosides. In nonendemic regions, patients with melioidosis more typically present with reactivation disease occurring months to years after initial exposure to the organism. The pulmonary disease is mainly in the apices and resembles tuberculosis. With the increasing mobility of people throughout the world and the influx of immigrants from endemic to nonendemic areas, it is important that clinicians be aware of this disease. This article will review the epidemiology, clinical presentations, diagnosis, and treatment of pulmonary melioidosis.     

Pulmonary botryomycoma

A patient with a mobile mass of anaerobic organisms in the pulmonary parenchyma is presented. Radiographically it resembled a fungous ball. This observed botryomycoma may be part of the spectrum of aspiration pneumonia.     

Pulmonary transplantation

OBJECTIVE: More than 2700 lung transplants have been performed since the initial clinical success in 1983. The evolution in the techniques of lung transplantation and patient management and the effects on results are reviewed. SUMMARY BACKGROUND DATA: Improvements in donor management, lung preservation, operative techniques, immunosuppression management, infection prophylaxis and treatment, rejection surveillance, and long-term follow-up have occurred in the decade following the first clinically successful lung transplant. A wider spectrum of diseases and patients treated with lung transplant have accentuated the shortage of suitable lung donors. The organ shortage has led to the use of marginal donors and a limited experience using living, related donors. METHODS: Changes in techniques and patient selection and management are reviewed and controversial issues and problems are highlighted. RESULTS: One-year survival of greater than 90% for single-lung transplant recipients and greater than 85% for bilateral lung transplant recipients have been achieved. Complications caused by airway complications has been reduced greatly. Obliterative bronchiolitis develops in 20% to 50% of long-term survivors and is the leading cause of morbidity and mortality after the first year after transplant. CONCLUSIONS: Lung transplantation has evolved into an effective therapy for a wide variety of causes of end-stage lung disease. Wider applicability requires solutions to the problems of donor shortage and development of obliterative bronchiolitis.     

Pulmonary vasculitis

Pulmonary vascular inflammation may be seen in a variety of primary lung diseases and in the setting of numerous systemic illnesses. This article reviews those entities in which pulmonary vasculitis represents a central feature of the pathologic process (Wegener's granulomatosis, Churg-Strauss syndrome, and pulmonary capillaritis). In addition, features of pulmonary involvement in other systemic vasculitides (Giant Cell Arteritis, Takayasu's Arteritis, and Beh?et's disease) are described. Finally, general principles for the treatment of vasculitis are reviewed.     

Pulmonary hemosiderosis

The cercarial emergence rhythms of Schistosoma mansoni were analyzed for mixed infection of the snail Biomphalaria glabrata with two chronobiological strains, one with an early shedding pattern and the other with a late shedding pattern. Under these conditions, each parasite strain kept its own circadian cercarial emergence pattern. When the chronobiological variants were of the same species, no interference between the trematode larvae occurred during the cercarial emergence process. These results confirm that within the host-parasite system the parasite component is directly responsible for the cercarial emergence pattern.