Long-term safety and influence on growth in patients receiving sirolimus: a pooled analysis.

Background: Sirolimus is increasingly utilized in treating diseases associated with mTOR pathway overactivation. Despite its potential, the lack of evidence regarding its long-term safety across all age groups, particularly in pediatric patients, has limited its further application. This study aims to assess the long-term safety of sirolimus, with a specific focus on its impact on growth patterns in pediatric patients.

Clinical features and multiomics profiles indicate coagulation and platelet dysfunction in COVID-19 viral sepsis.

Increased cases of sepsis during COVID-19 in the absence of known bacterial pathogens highlighted role of viruses as causative agents of sepsis. In this study, we investigated clinical, laboratory, proteomic, and metabolomic characteristics of viral sepsis patients ( = 45) and compared them to non-sepsis patients with COVID-19 ( = 186) to identify molecular mechanisms underlying the pathology of viral sepsis in COVID-19. We identified unique metabolomic and proteomic signatures that suggest a substantial perturbation in the coagulation, complement, and platelet activation pathways in viral sepsis.

Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China.

Background: The high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient.

Methods: We retrospectively evaluated HRCT scores for 231 patients with autoimmune pulmonary alveolar proteinosis (APAP) from three centers of the China Alliance for Rare Diseases.

A 47-Year-Old Woman With Progressive Exertional Dyspnea and Fatigue.

A previously healthy 47-year-old nonsmoking woman was admitted to our hospital with an 8-month history of progressive exertional dyspnea and fatigue. Chest high-resolution CT (HRCT) on admission showed diffuse, bilateral, patchy ground-glass opacity (GGO) (Fig 1A). She was diagnosed with interstitial lung disease, and corticosteroid therapy with 8 weeks prednisone taper was completed, with initial good response.

Cushing's disease with pulmonary Cryptococcus neoformans infection in a single center in Beijing, China: A retrospective study and literature review.

Background: Patients with Cushing's disease (CD) with hypercortisolism have an increased risk of opportunistic infection. However, most CD patients exposed to infections are diagnostic latency, leading to a poor prognosis.

Methods: Six patients in our hospital and an additional six patients in the literature were included in this study.

Diagnosis and treatment of community-acquired pneumonia in adults: 2016 clinical practice guidelines by the Chinese Thoracic Society, Chinese Medical Association.

Community-acquired pneumonia (CAP) in adults is an infectious disease with high morbidity in China and the rest of the world. With the changing pattern in the etiological profile of CAP and advances in medical techniques in diagnosis and treatment over time, Chinese Thoracic Society of Chinese Medical Association updated its CAP guideline in 2016 to address the standard management of CAP in Chinese adults. Extensive and comprehensive literature search was made to collect the data and evidence for experts to review and evaluate the level of evidence.

Characteristics of serum lipid metabolism in patients with autoimmune pulmonary alveolar proteinosis.

Objective: To study the serum lipid panels in consecutive autoimmune pulmonary alveolar proteinosis(APAP)patients and analyze their relationship with anti-granulocyte macrophage-colony stimulating factor(GM-CSF)antibody and other markers.

Methods: Thirty-two non-diabetic APAP patients were enrolled in the study. Serum lipids of these patients and 100 healthy volunteers were tested after an overnight fasting.

[Clinical characteristic analysis of 96 cases of hypersensitivity pneumonitis].

Objective: To improve understanding of the clinical characteristics and diagnosis of hypersensitivity pneumonitis (HP).

Methods: We retrospectively analyzed the clinical data, including clinical symptoms, laboratory tests, exposure, pulmonary function tests, chest CT imaging and cytological classification of bronchoalveolar lavage (BAL) of 96 patients with HP from Jan 2001 to Jun 2011 in Peking Union Medical College Hospital. We divided the patients into 2 groups: a pathologically-confirmed group and a clinically-suspected group.

[The clinicopathological analysis of 4 cases of IgG4-related nonspecific interstitial pneumonia].

Objectives: To observe the immunohistochemical staining of IgG4 in nonspecific interstitial pneumonia (NSIP) and to study the clinicopathological features of IgG4-related NSIP.

Methods: Retrospective analysis was carried out for 32 patients with NSIP who had been admitted into Peking Union Medical College Hospital from November 2002 to October 2010. The diagnosis of NSIP was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin.

[The diagnostic value of CT-guided percutaneous needle lung biopsy in diffuse parenchymal lung diseases].

Objective: This study was to evaluate the efficacy and limitation of CT-guided percutaneous cutting needle lung biopsy in the diagnosis of diffuse parenchymal lung diseases (DPLD).

Methods: A total of 481 patients admitted in Peking Union Medical College Hospital from January 2000 to December 2008 underwent CT-guided percutaneous cutting needle lung biopsy. The patients were evaluated by clinical history, physical examination and lung HRCT.

Inactivation of mammalian target of rapamycin (mTOR) by rapamycin in a murine model of lipopolysaccharide-induced acute lung injury.

Background: The mammalian target of rapamycin (mTOR) pathway, a key cellular signaling pathway associated with various cellular functions, has distinct roles in the inflammatory process. In this study, the mTOR inhibitor rapamycin (Rapa) was used to test whether inhibition of mTOR activation attenuates lipopolysaccharide (LPS)-induced acute lung injury (ALI) in a murine model.

Methods: Mice pretreated with Rapa or vehicle were given LPS intratracheally.

[Clinical analysis of 123 cases of chylous effusion].

Objective: To analyze the clinical characteristics of chylous effusion and boost its diagnostic and therapeutic level.

Methods: A retrospective analysis was conducted for 123 cases of chylous effusion at our hospital between January 1990 and December 2009.

Results: The main clinical manifestations of chylous effusion included dyspnea (55.

The St. George's Respiratory Questionnaire in lymphangioleiomyomatosis.

Objective: To examine the correlation between the health-related quality of life measured by the St. George's Respiratory Questionnaire (SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis (LAM).

Methods: This study retrospectively analyzed the SGRQ scores and other measures (the Borg scale of breathlessness at rest, 6-minute walking distance, blood oxygen levels, and pulmonary function) of patients diagnosed and confirmed with LAM.

[Combined pulmonary fibrosis and emphysema syndrome].

Objective: To retrospectively analyze clinical presentations, pulmonary function parameters and radiological appearance in patients with combined pulmonary fibrosis and emphysema (CPFE) syndrome.

Methods: We reviewed the clinical features, imaging, and lung function indices from 8 patients diagnosed with CPFE according to the findings by high resolution computerized tomography (HRCT) since 2006 to 2009 at Peking Union Medical College Hospital.

Results: All patients were male, aged 65 (59 - 75) years, and 7 of them were smokers.

[Clinical analysis of four cases of primary ciliary dyskinesia and literature review].

Objective: To study the clinical characteristics and diagnosis of primary ciliary dyskinesia (PCD).

Methods: Four cases diagnosed as PCD by cilia electron microscopy examination from Jan. 2007 to Aug.

[ γδ T lymphocyte function and the polymorphism of T cell receptor V δ chain in lungs of asthmatic patients].

Objective: To observe the function of gamma delta T lymphocytes and the polymorphism of T cell receptor V delta chain in the lungs of asthmatic patients and explore the role of gamma delta T cells in airway inflammation.

Methods: Bronchoalveolar lavage fluid BALF was obtained from 7 asthmatic patients and 7 healthy control individuals. The percentage of gamma delta T cell in BALF was measured by flow cytometry.

[Clinical and radiological features of pulmonary tuberculosis manifested as interstitial lung diseases.].

Objective: The purpose of this paper was to investigate the clinical and radiological features of pulmonary tuberculosis presenting as interstitial lung diseases (ILD).

Methods: We analyzed the data of cases suspected of diffuse parenchyma lung diseases at this hospital between October 2003 and October 2007. The diagnosis of active pulmonary tuberculosis was based on epithelioid granuloma or positive acid-fast bacilli in lung biopsy and changes on serial radiographs obtained during treatment.

Clinical features and prognosis in 21 patients with extrinsic allergic alveolitis.

Objective: To assess the spectrum of causes, clinical features, differences between disease phases, and prognosis of extrinsic allergic alveolitis (EAA).

Methods: Patients with EAA diagnosed at Peking Union Medical College Hospital from August 1983 to May 2007 were analyzed retrospectively. Their medical records were examined to gather clinical, laboratorial, radiological, and histopathological data.

[Pathologic study of diffuse pulmonary interstitial fibrosis caused by chronic hypersensitivity pneumonitis].

Objective: To study the pathologic characteristics of chronic hypersensitivity pneumonitis, especially the pattern of pulmonary interstitial fibrosis; and to compare the histologic features with those of idiopathic interstitial pneumonitis.

Methods: The HE-stained paraffin sections of 10 cases of chronic hypersensitivity pneumonitis encountered during the period from 2000 to 2008 were retrospectively analyzed.

Results: There were altogether 6 males and 4 females, with age of patients ranging from 23 to 59 years (mean=47.

[Intravascular lymphomatosis presenting in the lung].

Objective: To investigate the clinical, radiographic and pathological characteristics of intravascular lymphomatosis primarily manifested in the lung, without skin and central nervous system involvements.

Methods: A case of T cell intravascular lymphomatosis presenting with fever and multiple pulmonary shadows on chest radiograph was described and 14 similar cases reported in the English literature were reviewed.

Results: We described a case of T cell intravascular lymphomatosis, who was a 36 year old man, complained of fever and multiple pulmonary shadows on chest radiograph and admitted to Peking Union Medical College Hospital in march, 2008.

Pulmonary manifestations of Sjögren's syndrome.

Background: Primary Sjögren's syndrome (PSS) is associated with various histological patterns of interstitial lung disease. Although chest images and lung function studies showed that lung involvement predominantly occurs in small airways, pathological findings were not consistent with the results of high-resolution CT (HRCT) and lung function tests.

Objectives: To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests.

[Predictive value of single and combined indexes in the diagnosis of sarcoidosis].

Objective: To evaluate the predictive value of single and combined indexes in the diagnosis of sarcoidosis.

Methods: Eighty-eight patients suspected of having sarcoidosis were retrospectively investigated. The diagnostic predictive value of lymphocyte percentage and CD4/CD8 ratio in bronchoalveolar lavage (BAL), angiotensin converting enzyme (ACE) and their combination was evaluated by ROC curve method and Bayes' rule.

[Primary pulmonary lymphoma: analysis of 18 cases].

Objective: To study the clinical characteristics, pathology, diagnosis and treatment of primary pulmonary lymphoma.

Methods: Eighteen cases of primary pulmonary lymphoma diagnosed from Jan 1989 to Feb 2007 were retrospectively analyzed.

Results: There were 6 males and 12 females, with a median age of 47.