OBJECTIVETo improve the diagnosis and treatment of lymphangioleiomyomatosis (LAM).

METHODSThe clinical data of four cases of lymphangioleiomyomatosis diagnosed in our hospital were analyzed and corresponding literature was reviewed.

RESULTSLymphangioleiomyomatosis, a rare disorder of unknown cause that occured almost exclusively in women of child bearing ages, was characterized microscopically by atypical smooth muscle proliferation. It could occur as a sporadic diseases or as a part of tuberous sclerosis complex (TSC). LAM was associated with progressive dyspnea, recurrent spontaneous pneumothoraces, chylous effusions, and hamartomas.

CONCLUSIONClinicians should be familiar with the characteristics of LAM so that diagnosis and treatment can be made earlier to improve the prognosis.

Adolescent ; Adult ; Biopsy, Needle ; Female ; Humans ; Lung ; diagnostic imaging ; pathology ; Lung Neoplasms ; diagnosis ; pathology ; physiopathology ; Lymphangioleiomyomatosis ; diagnosis ; pathology ; physiopathology ; Male ; Middle Aged ; Tomography, X-Ray Computed

PURPOSE: Lung size mismatch is a major cause of poor lung function and worse survival after lung transplantation (LTx). We compared predicted total lung capacity (pTLC) and TLC measured by chest computed tomography (TLC(CT)) in LTx candidates. MATERIALS AND METHODS: We reviewed the medical records of patients on waiting lists for LTx. According to the results of pulmonary function tests, patients were divided into an obstructive disease group and restrictive disease group. The differences between pTLC calculated using the equation of the European Respiratory Society and TLC(CT) were analyzed in each group. RESULTS: Ninety two patients met the criteria. Thirty five patients were included in the obstructive disease group, and 57 patients were included in the restrictive disease group. pTLC in the obstructive disease group (5.50±1.07 L) and restrictive disease group (5.57±1.03 L) had no statistical significance (p=0.747), while TLC(CT) in the restrictive disease group (3.17±1.15 L) was smaller than that I the obstructive disease group (4.21±1.38 L) (p<0.0001). TLC(CT)/pTLC was 0.770 in the obstructive disease group and 0.571 in the restrictive disease group. CONCLUSION: Regardless of pulmonary disease pattern, TLC(CT) was smaller than pTLC, and it was more apparent in restrictive lung disease. Therefore, we should consider the difference between TLC(CT) and pTLC, as well as lung disease patterns of candidates, in lung size matching for LTx.
Adolescent ; Adult ; Aged ; Female ; Humans ; Lung/*diagnostic imaging/pathology/*physiopathology ; Lung Diseases/diagnostic imaging/pathology/physiopathology/therapy ; *Lung Transplantation ; Male ; Middle Aged ; Organ Size ; *Patient Selection ; Retrospective Studies ; *Tomography, X-Ray Computed ; *Total Lung Capacity ; Young Adult

OBJECTIVETo analyze the clinical features of interstitial pneumonitis (IP) associated with interferon therapy for chronic hepatitis C.

METHODSWe report the first case of IP in China resulting from pegylated interferon alpha-2a in combination with ribavirin for treatment of hepatitis C viral infection. A statistical analysis of the related literatures documenting such IP cases was performed using SPSS 11.5 software.

RESULTSOf the 22 patients reported to develop IP after interferon therapy alone or in combination with ribavirin, 83%, 72% and 56% of the patients had the symptoms of dyspnoea, dry cough and fever, respectively. Twenty of these cases presented with restrictive pulmonary functional impairment and/or hypoxemia, and diffuse infiltration on chest radiography and/or CT. Complications were documented in 71% of the cases within 12 weeks of the treatment. The majority (85%) of the patients had favorable prognoses with an average recovery time of 7.5 weeks. Compared with the patients with mild and moderate pulmonary function impairment, 8 patients with severe pulmonary functional impairment had early onset of IP during the interferon therapy (6.6 vs 14.1 weeks, P<0.05), and a higher rate of corticosteroid treatment (75% vs 54%, P>0.05).

CONCLUSIONIP is a rare pulmonary complication associated with IFN therapy, and patients with chronic hepatitis C should be followed up closely in the first 12 weeks of interferon therapy. Prompt discontinuation of medication is mandatory in the presence of IP, and corticosteroid therapy may not be essential for patients with mild or moderate pulmonary functional impairment under close monitoring. The severity of pulmonary damage is associated with the time of complication occurrence, and corticosteroids are required when obvious pulmonary toxicity occurs in early stage of the treatment (within 6 weeks) to reduce the pulmonary damage.

Adult ; Aged ; Female ; Hepatitis C, Chronic ; drug therapy ; Humans ; Interferon-alpha ; adverse effects ; therapeutic use ; Lung ; diagnostic imaging ; drug effects ; pathology ; physiopathology ; Lung Diseases, Interstitial ; chemically induced ; diagnostic imaging ; pathology ; physiopathology ; Male ; Middle Aged ; Time Factors ; Tomography, X-Ray Computed

OBJECTIVETo investigate pulmonary function impairment and the spinal factors that may determine pulmonary function in patients with scoliosis.

METHODSSeventy-eight patients with idiopathic scoliosis or congenital scoliosis and 78 age- and gender-matched healthy subjects were enrolled in this study. The radiographic parameters of spinal deformity were obtained from patients with scoliosis. Both two groups received pulmonary function tests.

RESULTSPatients with scoliosis demonstrated a restrictive pattern of pulmonary function impairment with a proportional decrease in both forced expiratory volume in one second and forced vital capacity. Total lung capacity and functional residual capacity were reduced. Carbon monoxide diffusion capacity was decreased, while diffusion coefficient remained normal or slightly higher. Airway resistance and conductance were not affected. In addition, airway resistance and residual volume were found abnormal in patients with congenital scoliosis. Multiple linear regression analysis showed that three spinal factors including involved thoracic vertebrae, vertical height from C7 to S1, and Cobb angle were independently responsible for 40%-51% of total variances of forced vital capacity, forced expiratory volume in one second, total lung capacity, and functional residual capacity.

CONCLUSIONSPatients with scoliosis have restrictive ventilation defects. More thoracic vertebrae involvement, lower vertical height, and larger Cobb angle are associated with severer impairment of lung volume.

Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Lung ; physiopathology ; Male ; Radiography ; Scoliosis ; pathology ; physiopathology ; Thoracic Vertebrae ; diagnostic imaging ; pathology ; Young Adult

Acetates ; therapeutic use ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hyperplasia ; Infant ; Lung ; diagnostic imaging ; pathology ; physiopathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; physiopathology ; Methylprednisolone ; therapeutic use ; Neuroendocrine Cells ; pathology ; Quinolines ; therapeutic use ; Tomography, X-Ray Computed

OBJECTIVETo investigated the involvement of pulmonary function impairment in ulcerative colitis (UC), to explore a scientific basis for the Chinese medicine (CM) theory of exterior-interior correlation between Lung (Fei) and Large intestine (Dachang).

METHODSTotally 120 patients with a diagnosis of UC were recruited and the demographics, clinical data, and blood samples were collected. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) concentrations were measured. Every patient accepted pulmonary function test and took chest radiograph (CXR).> RESULTS: Pulmonary function abnormalities were present in 72 of 120 patients. The median (interquartile range) vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1 s (FEV), carbon monoxide diffusion capacity (DL) of lung, total lung capacity (TLC) and functional residual volume (FRV) were decreased in distal UC and pancolitis compared with ulcerative prochitis (P <0.0005). Male patients had increased VC, FEV/FVC, and residual volume (RV)/TLC compared with female (P <0.0005), but decreased DLand carbon monoxide iffusion capacity (K) of lung/alveolar ventilation (P <0.0005). Age was strongly correlated with RV (Spearman rank correlation coefficient (rs)=-0.57,P <0.0001), and RV/TLC (rs=0.48,P<0.0001). Age was also correlated with FEV/FVC (rs=-0.29, P=0.001), forced expiratory flow in 75% vital capacity (FEF75%, rs=-0.20, P=0.03), DL(rs=-0.21, P=0.02), TLC (rs=-0.25, P=0.006), and FRV (rs=-0.28, P=0.002). The course of disease was correlated with FEF75% (rs=-0.18, P=0.049) and K(rs=-0.19, P=0.036). Chest radiograph abnormalities were presented in 38 of 120. Pulmonary symptoms were presented in 10 of 120. Other extraintestinal complications were presented in 21 of 120.

CONCLUSIONSPulmonary function impairment was more frequently than other extraintestinal complications in UC patients, which may be affected by sex, age, extent and course of disease. These results may be a scientific basis for the theory of exterior-interior correlation between Lung and Large intestine.

Adolescent ; Adult ; Age of Onset ; Aged ; Colitis, Ulcerative ; complications ; pathology ; physiopathology ; Colon ; pathology ; Demography ; Female ; Humans ; Inflammation ; complications ; pathology ; Lung ; diagnostic imaging ; physiopathology ; Male ; Middle Aged ; Radiography, Thoracic ; Respiratory Function Tests ; Young Adult

OBJECTIVETo investigate the role of the expression of ephrinB2 and EphB4 in non-small cell lung cancer (NSCLC), and their relationship with multi-slice spiral CT pulmonary perfusion imaging.

METHODSThirty-one nodules with pathologically proven NSCLC underwent CT perfusion scan. The perfusion parameters including blood flow (BF), blood volume (BV), peak enhancement image (PEI) were collected. The expression of ephrinB2 and EphB4 in tumor cells and interstitial vasculature were detected by immunohistochemistry. Correlation analysis and trend test were used to assess the relationship between ephrinB2/EphB4 expression and clinicopathological features, and between ephrinB2/EphB4 expression and perfusion parameters.

RESULTSPositive expression of ephrinB2 and EphB4 in the NSCLC group was 83.9% and 71.0%, respectively, significantly higher than that in the internal control group (P < 0.01). The expression of ephrinB2 and EphB4 was consistently in tumor parenchyma but differently in tumor vessels. The expressions of ephrinB2 and EphB4 were positively correlated with lymphatic metastasis (P < 0.05). The expression of EphB4 was negatively correlated with blood flow (BF) and blood volume (BV), respectively (P < 0.05). There was a significant positive correlation between ephrinB2 expression and BF (r = 0.516, P = 0.003), and a positive correlation between ephrinB2 expression and BV (r = 0.448, P = 0.013). The expressions of ephrinB2 and EphB4 were not correlated with PEI (P > 0.05). The values of BF and BV in the high and moderate EphB4 expression groups were significantly decreased compared with that in the negative group (P < 0.01). The value of BF in the high ephrinB2 expression group was significantly increased compared with that in the moderately positive group and negative group (P < 0.01). The value of BV in the high ephrinB2 expression group was significantly increased compared with that in the negative group (P < 0.01).

CONCLUSIONThe CT pulmonary perfusion imaging reflects the density difference of blood vessels with functional lumen, and such difference also depends on the quantity and quality of vasculature with functional lumen.

Adult ; Aged ; Blood Volume ; Carcinoma, Non-Small-Cell Lung ; blood supply ; diagnostic imaging ; metabolism ; pathology ; physiopathology ; Ephrin-B2 ; metabolism ; Female ; Humans ; Lung Neoplasms ; blood supply ; diagnostic imaging ; metabolism ; pathology ; physiopathology ; Lymphatic Metastasis ; Male ; Middle Aged ; Perfusion Imaging ; Pulmonary Circulation ; Receptor, EphB4 ; metabolism ; Tomography, Spiral Computed ; methods

OBJECTIVETo investigate the Churg-Strauss syndrome (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis.

METHODSWe retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review. All patients underwent transbronchial lung biopsy (TBLB). Six of them underwent surgical lung biopsy as well.

RESULTSThe patients included 7 men and 9 women, aged from 14 to 61 years (median, 47.5 years). Extrathoracic organs involved included nervous system (7/16) and skin (5/16). Respiratory symptoms included cough (12/16), exertional dyspnea (11/16), hemoptysis (4/16), and chest pain (3/16). CT findings included bilateral ground-glass opacities (12/16), bilateral patchy opacities (12/16), and centrilobular nodules (6/16). The pathological findings of TBLB demonstrated increased eosinophils (3/16), vasculitis (3/16), and interstitial pneumonia (16/16). The pathological findings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period (range, 3 to 51 months; median, 15 months).

CONCLUSIONSAsthma may be present in CSS patient when there is bronchial involvement. Ground-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.

Adolescent ; Adult ; Asthma ; physiopathology ; Biopsy ; Churg-Strauss Syndrome ; diagnosis ; diagnostic imaging ; drug therapy ; pathology ; Cyclophosphamide ; Female ; Humans ; Immunosuppressive Agents ; therapeutic use ; Lung ; diagnostic imaging ; pathology ; physiopathology ; surgery ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Tomography, X-Ray Computed ; methods ; Treatment Outcome ; Young Adult