Unusual Lung Research Articles

An unusual interstitial lung disease

An unusual interstitial lung disease

Pathological Findings and Distribution of Pandemic Influenza A (H1N1) 2009 Virus in Lungs from Naturally Infected Fattening Pigs in Norway

The Norwegian pig population was considered free from influenza A virus infections until the first case of porcine pandemic influenza A (H1N1) 2009 virus infection in October 2009. Human to pig transmission of virus was suspected. Unusual lung lesions were observed in fattening pigs, with red, lobular, multifocal to coalescing consolidation, most frequently in the cranial, middle, and accessory lobes. The main histopathological findings were epithelial degeneration and necrosis, lymphocyte infiltration in the epithelial lining and lamina propria of small bronchi and bronchioles, and peribronchial and peribronchiolar lymphocyte infiltrations. Infection with pandemic influenza A (H1N1) 2009 virus was confirmed by real-time RT-PCR and immunohistochemical detection of influenza A virus nucleoprotein in the lesions. This investigation shows that natural infection with the pandemic influenza A (H1N1) 2009 virus induces lung lesions similar to lesions described in experimental studies and natural infections with other swine-adapted subtypes of influenza A viruses.

Update on the diagnosis and treatment of Pneumocystis pneumonia

Pneumocystis is an opportunistic fungal pathogen that causes an often-lethal pneumonia in immunocompromised hosts. Although the organism was discovered in the early 1900s, the first cases of Pneumocystis pneumonia in humans were initially recognized in Central Europe after the Second World War in premature and malnourished infants. This unusual lung infection was known as plasma cellular interstitial pneumonitis of the newborn, and was characterized by severe respiratory distress and cyanosis with little or no fever and no pathognomic physical signs. At that time, only anecdotal cases were reported in adults and usually these patients had a baseline malignancy that led to a malnourished state. In the 1960-1970s additional cases were described in adults and children with hematological malignancies, but Pneumocystis pneumonia was still considered a rare disease. However, in the 1980s, with the onset of the HIV epidemic, Pneumocystis prevalence increased dramatically and became widely recognized as an opportunistic infection that caused potentially life-treating pneumonia in patients with impaired immunity. During this time period, prophylaxis against this organism was more generally instituted in high-risk patients. In the 1990s, with widespread use of prophylaxis and the initiation of highly active antiretroviral therapy (HAART) in the treatment of HIV-infected patients, the number of cases in this specific population decreased. However, Pneumocystis pneumonia still remains an important cause of severe pneumonia in patients with HIV infection and is still considered a principal AIDS-defining illness. Despite the decreased number of cases among HIV-infected patients over the past decade, Pneumocystis pneumonia continues to be a serious problem in immunodeficient patients with other immunosuppressive conditions. This is mostly due to increased use of immunosuppressive medications to treat patients with autoimmune diseases, following bone marrow and solid organ transplantation, and in patients with hematological and solid malignancies. Patients with hematologic disorders and solid organ and hematopoietic stem cell transplantation are currently the most vulnerable groups at risk for developing this infection. However, any patient with an impaired immunity, such as those receiving moderate doses of oral steroids for greater than 4 weeks or those receiving other immunosuppressive medications are at also at significant risk.

Middle lobe syndrome in the left lower lobe in chronic obstructive pulmonary disease

Middle lobe syndrome is a term that refers to a recurrent collapse of a lung segment, typically the right middle lobe. We discuss a case that middle lobe syndrome occurred in an unusual lung segment and related to an unusual cause.

Diffuse interstitial pneumonia and pulmonary hypertension: a novel manifestation of chronic granulomatous disease

The present authors report the case of an adult with chronic granulomatous disease who developed an unusual lung fibrosis associated with severe pulmonary hypertension. Histological analysis of a lung biopsy showed a diffuse infiltration with pigmented macrophages without granulomas, which particularly involved the pulmonary arterial and venular walls. Clinical and histological findings were suggestive of pulmonary veno-occlusive disease. Such a clinical association has not been previously described in the literature and might be due to the persistent expression of gp91phox at a very low level. In conclusion, the present case report illustrates a novel manifestation of chronic granulomatous disease.

Time-motion echocardiographic examination in the identification of an unusual lung artifact in the ICU

Time-motion echocardiographic examination in the identification of an unusual lung artifact in the ICU

Thyroid cystic echinococcosis: case presentation and review of the literature

Aim: In many parts of the world, including Romania, Cystic Echinococcosis (CE), is a serious health problem. The absence of a coherent survey and control program of the disease in our country can explain its high endemicity. Liver and lung represent the most common locations of the larval stage of the parasite in humans, but rare cases with unusual locations, as thyroid, spleen, pancreas, ovaries, heart etc can occur. Material and methods: An unusual thyroid and lung location of CE in a 23 year old female patient is presented. The thyroid CE, right lobe location, 3.6/2.5cm was discovered by means of ultrasound. The compression on the surrounding vessels and nerves was associated a plapebral pthosis and dysphonia. Abdominal US and chest radiography failed to discover another location, but CT scan confirmed the presence of thyroid cyst and emphasized a lung cyst, 3cm diameter. ELISA test for detection of specific antibodies anti E. granulosus was negative. Results: The thyroid CE was surgically removed and antiparasitic treatment was initiated for the lung cyst. Conclusion: Inspite the rarity of CE with thyroid location (less than 1%) in endemic areas for CE, medical doctors should carefully evaluate by imaging methods any thyroid cyst and should recommend specific investigations regarding the parasitic origin, in order to avoid the complications consequent to the puncture (spillage of protoscolices, anaphylactic reactions). According to our records, thyroid CE is present in young population and very often the serology can be negative.

Unusual cavitary lung lesion in an asymptomatic patient—Case report and differential diagnosis

Summary An unusual cavitary lung lesion in an asymptomatic and nonsmoker patient is uncommon. In these cases, infectious pathologies should be included in the differential diagnosis. Then, to correctly conduct an investigation, it is important to consider the clinical picture, the radiological images and the epidemiology. This presentation may need a wide variety of tests to establish the final diagnosis. Such a presentation occurs in the following case report.

Pulmonary Sclerosing Hemangioma in a Patient With Cowden Syndrome

We describe the case of an 18-year-old female with Cowden syndrome in whom a simple x-ray detected a solitary pulmonary nodule that was identified as a sclerosing hemangioma. Pulmonary sclerosing hemangioma is an unusual lung neoplasm which typically presents as a solitary peripheral nodule in asymptomatic women. Although the histology of this entity is well defined, its origin and treatment is debated. One of the main diagnostic problems is to histologically differentiate a pulmonary sclerosing hemangioma from a papillary lung carcinoma.

The “sugar” clear cell tumor of the lung—clinical presentation and diagnostic difficulties of an unusual lung tumor in youth

A case of the “sugar” clear cell tumor of the lung in a 16-year-old boy is presented. The course of the disease with general symptoms, never reported before, highlights diagnostic difficulties of an extremely rare lung tumor in youth. The boy presented with daily spikes of unexplained high fever of 6 weeks' duration with features of hypochromic microcytic anemia, elevated erythrocyte sedimentation rate, C-reactive protein, α 2- and β-globulins, and elevated platelet count. The lung tumor was a yellow, circumscribed mass confined to the sixth segment of the left lung. Histological examination revealed the tumor composed of cells with clear cytoplasm with large content of glycogen, with no signs of necrosis, and immunoreactive for HMB-45, but not for cytokeratin, LCA, CD34, and CD68. The performed thoracotomy and segmentectomy were both diagnostic and curative.

Cytology of an unusual lung tumour in a middle-aged female Part 1

Cytology of an unusual lung tumour in a middle-aged female Part 1

Unusual lung tumours: an illustrated review of CT features suggestive of this diagnosis

This review presents an imaging-centred approach to the diagnostic challenge of uncommon lung tumours in adults. Emphasis is placed on features that may be used to differentiate these tumours including fat content, tumour site, multifocality, calcification and predominant pattern of involvement.

Microscopic polyangiitis with unusual lung involvement

Microscopic polyangiitis (MPA) is a non-granulomatous, systemic and small vessel vasculitis accompanied by segmental necrotizing glomerulonephritis with no evidence of other small vessel disease. We report a patient with weakness, fever, and arthralgia whose CXR and thoracic CT showed widespread nodular infiltration. His proteinase-3 anti-neutrophilic cytoplasmic antibody (c-ANCA) was positive. The serum creatinine was increased and haematuria subsequently developed. Renal biopsy revealed a focal segmental necrotizing glomerulonephritis which was compatible with MPA. He was treated with high-dose corticosteroid and cyclophosphamide. Because of a worsening CXR and hypoxaemia, mechanical ventilation was applied. Despite this he died of respiratory failure following 20 days of treatment. Nodular infiltration is an unusual radiological pattern in patients with MPA and is the reason for this report.

Lung field segmenting in dual-energy subtraction chest X-ray images.

The purpose of this study was to develop and test a method to delineate lung field boundaries in dual-energy chest x-ray images. The segmenting method uses soft-tissue images and spatial frequency-dependent, background-subtracted images. Large-scale chest anatomy features are located and used to select the lung apices, the lateral lung boundaries, and the lung-mediastinum and lung-diaphragm boundaries. Extraneous parts of the contours are removed and they are joined to form complete lung boundaries. The reliability measure uses a statistical shape model to estimate the probability of occurrence of a contour. The method was experimentally tested with 30 human subject images. It has higher accuracy and specificity and a sensitivity parameter equal to the best previously reported method. The reliability measure is able to detect contours with unusual lung outlines or errors in the processing. The method exploits the characteristics of dual-energy subtraction images to improve lung field segmenting performance.

Pulmonary Langerhans' Cell Histiocytosis

Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration in the differential diagnosis of diffuse infiltrative lung disease, particular among smokers. This review highlights recent progress in our understanding of the etiology, clinical presentation, and diagnostic and therapeutic approaches to this unusual lung disorder. Evolving studies strongly link the basic pathogenesis of PLCH as an uncommon reaction to tobacco smoke. Recent progress in the clinical approach to these patients has emphasized important roles for high-resolution computed tomographic (CT) scanning and immune reactive tissue markers including cluster differentiation (CD) 1a antigen in the diagnosis of this disorder. A rationale diagnostic algorithm and current management strategies are summarized.

Unusual primary lung neoplasms.

Bronchial carcinoids and hamartomas are, respectively, the most common malignant and benign unusual primary lung neoplasms. These tumors are often asymptomatic but can cause central airway obstruction. Helical computed tomographic and radionuclide scintigraphic advances in their detection and evolution, together with newer interventional bronchoscopy techniques such as neodymium:yttrium-aluminum-garnet laser phototherapy and cryotherapy, represent important improvements in the diagnosis and management of patients with such tumors.

Cystic Fibrosis Phenotype Associated with Pancreatic Insufficiency Does Not Always Reflect the cAMP-dependent Chloride Conductive Pathway Defect: ANALYSIS OF C225R-CFTR AND R1066C-CFTR

We have previously screened the cystic fibrosis transmembrane conductance regulator (CFTR) gene and identified new disease-causing mutations. C225R and R1066C are both associated with pancreatic insufficiency, but the former mutation is associated with mild and unusual lung disease, whereas the latter is associated with severe lung disease. In the present study, we expressed these mutants heterologously in HeLa cells, and we analyzed protein synthesis by immunoprecipitation and chloride channel function by using a halide-sensitive fluorescent dye, 6-methoxy-N-ethylquinolinium. Immunoprecipitation and functional studies showed that cells transfected with C225R-CFTR exhibit cAMP-dependent chloride fluxes; C225R-CFTR protein is poorly expressed but fully glycosylated and can be compared with R117H-CFTR. R1066C-CFTR protein is not correctly processed and, unlike DeltaF508-CFTR, this defect cannot be corrected by reduced temperature or overexpression in butyrate-treated cells; defective processing may occur at a different step in the biosynthetic pathway. These results point to two different mechanisms underlying the same pancreatic status and suggest that it is unwise to use pancreatic sufficiency and insufficiency to define mild and severe cystic fibrosis (CF) disease, respectively. Finally, the experimental model described here may be helpful to predict the pulmonary status of CF patients bearing mutations located in putative membrane-spanning domains of the CFTR protein.

Intrathoracic neoplasms other than bronchogenic carcinoma.

Intrathoracic neoplasms other than bronchogenic carcinoma comprise a disparate group of tumors that have in common an anatomic location within the thorax. For the purposes of this review, they are divided into three categories: pulmonary metastases from locations other than the thorax, mediastinal neoplasms, and unusual primary lung neoplasms. This article reviews the recent literature; discusses evolving strategies for imaging, diagnosis, and treatment; and presents promising research developments.

Lung tumors of uncertain histogenesis: Shimosato Y. Clinical Laboratories Divisin, National Cancer Center Hospital, 5-1-1, Tsukiji Chuo-ku, Tokyo 104. Semin Diagn Pathol 1995; 12: 185–192

Unusual lung tumors of uncertain origin have been described briefly concerning benign clear cell (sugar) tumor and, in more detail, concerning pneumocytoma (so-called sclerosing hemangioma). Melanocytic differentiation of clear cell tumor, which is peculiar to the lung, has been suggested recently. Sclerosing hemangioma, also unique to the lung, is now considered to be a tumor (neoplasm) related to alveolar epithelial cells. It shows invasive growth and multiplicity (or aerogenous metastasis), but is very rarely capable of lymph node metastasis. It should be designated as pneumocytoma.

Pulmonary amyloidosis and unusual lung involvement in SLE

The association of systemic lupus erythematosus (SLE) with amyloidosis is exceptional. We present a 37-year-old patient who was diagnosed five months earlier for SLE. She developed an acute episode of chest pain, cough and dyspnoea. Hypoxemia and obstructive changes in respiratory tests were present. The chest X-ray was repeatedly normal. Open lung biopsy revealed lupus pneumonitis with positive stain for immunoglobulins and complement, bronchiolitis obliterans, and pulmonary amyloidosis.