Diagnostic values of serum BNP, PTX3, and VEGF in acute pulmonary embolism complicated by pulmonary artery hypertension and their correlations with severity of pulmonary artery hypertension.

OBJECTIVE: This paper aimed to unveil the diagnostic values of serum brain natriuretic peptide (BNP), pentraxin 3 (PTX3), and vascular endothelial growth factor (VEGF) in acute pulmonary embolism complicated by pulmonary artery hypertension (APE-PAH) and their correlations with severity of PAH. METHODS: A total of 153 patients with APE were selected for our study and divided into the PAH and Non-PAH groups according to the measurement of pulmonary artery pressure by echocardiography. Serum BNP levels were measured by chemiluminescence immunoassay, and serum PTX3 and VEGF levels were appraised by ELISA. The predictive values of BNP, PTX3, and VEGF for APE-PAH were evaluated by applying the receiver operating characteristic (ROC) curve. Spearman test was implemented to correlate BNP, PTX3, and VEGF with the severity of PAH. RESULTS: Higher serum levels of BNP, PTX3, and VEGF were observed in the PAH group versus the Non-PAH group (p < .05). ROC curve analysis indicated that BNP, PTX3, and VEGF had acceptable diagnostic value for predicting APE-PAH. Higher serum levels of BNP, PTX3, and VEGF were witnessed in the moderate and severe PAH groups in contrast to the mild PAH group (p < .05), and the levels of these parameters were elevated in the severe PAH group versus the moderate PAH group (p < .05). Spearman correlation analysis signified that serum BNP (r = 0.377), PTX3 (r = 0.488), and VEGF (r = 0.575) levels were positively correlated with the severity of PAH in APE-PAH patients. CONCLUSION: Serum BNP, PTX3, and VEGF levels are significantly elevated in APE-PAH patients. Serum BNP, PTX3, and VEGF levels are of clinical value in the diagnosis of APE-PAH patients, and serum BNP, PTX3, and VEGF levels are positively correlated with the severity of PAH and can be used as predictors of the severity of PAH.

Metal ion assistant transformation strategy to synthesize catechol-based metal-organic frameworks from Ti3C2Tx precursors.

Chemical transformation strategy is capable of fabricating nanomaterials with well-defined structures and fascinating performance via controllable crystallization kinetics in the phase transformation. V2CTx MXene has been used as precursors to fabricate vanadium porphyrin metal-organic frameworks (V-PMOFs) via the coordination of deprotonated carboxylic acid ligands. However, the rational and in-depth exploration of synthesis mechanism with the aim of enriching the variety of MXene (i.e., Ti3C2Tx) and organic ligands (i.e., catechol-based) to design new MOFs is rarely reported. Herein, we have first developed a metal ion assistant transformation strategy to synthesize three-dimensional catechol-based TiCu-HHTP (HHTP = 2,3,6,7,10,11-hexahydroxytriphenylene) MOFs with a non-interpenetrating SrSi2 (srs) framework using two-dimensional Ti3C2Tx as precursors. The unique synergetic transformation mechanism involves the electron transfer from Ti3C2Tx to electrostatically adsorbed Cu2+ ion for redox reaction, the subsequent Ti-C bond rupture for Ti4+ ion release, and the continuous chelation coordination between Ti4+/Cu2+ and HHTP. Ti3C2Tx precursors and auxiliary metal ion could be rationally substituted by V2CTx and Mn+ (e.g., Ni2+, Co2+, Mn2+, and Zn2+), respectively. This strategy lays the foundation for the design and synthesis of innovative and multifarious MOFs derived from MXene or other unconventional metal precursors.

Human Wharton's jelly-derived mesenchymal stem cells prevent pregnancy loss in a rat by JAK/STAT-mediated immunomodulation.

AIM: Spontaneous abortion (SA) is a multiple-original syndrome with immune imbalance as one of its major risk factors. As Wharton's jelly-mesenchymal stem cells (WJ-MSCs) are considered to be able to prevent abortion, this study aims to explore the currently poorly understood underlining molecular signaling pathways and regulatory mechanisms of WJ-MSCs in pregnancy maintenance. METHODS: Abortion mode is established by subcutaneous injection of bromocriptine in rat on day 9 and abortion prevention is achieved by WJ-MSCs injection via tail vein. WJ-MSCs were cultured with/without the inhibitors of JAK/STAT or NF-κB. The uterus was collected on the 14th day of gestation and the rate of embryo absorption was calculated. The expression of Th1/Th2/Th3 cytokines in decidual, placental tissue, and peripheral blood was analyzed. RESULTS: WJ-MSCs treatment significantly reduced the abortion rate in bromocriptine-treated pregnancy such that it was not significantly different from a normal pregnancy. JAK/STAT inhibition abolished pregnancy preserving effects of WJ-MSCs but NF-κB inhibition did not. The levels of Th1-related cytokines and mRNA levels in the bromocriptine abortion model were significantly higher than the normal pregnancy group and ethanol control group, while levels of the Th2-related cytokines and mRNA levels significantly decreased. WJ-MSCs transfusion into the abortion model restored cytokine profiles such that they were not significantly different from the normal pregnancy group and ethanol control group. JAK/STAT inhibition of WJ-MSCs prevented their effect on cytokine and mRNA levels, but NF-κB inhibition did not. CONCLUSIONS: WJ-MSCs significantly lower the rate of embryo resorption of spontaneous abortion by reducing Th1-related cytokines while increasing Th2 and Th3-related cytokines in JAK/STAT-dependent manner.

Montgomery T-Tube Insertion Under ECMO in a Patient With Complete Subglottic Stenosis and Severe Lower Tracheal Collapse.

The treatment of complete subglottic stenosis (SGS) remains a challenge due to anatomic and technological limitations. Placement of the Montgomery T-tube is an effective treatment option. For complete SGS patient combined with lower trachea collapse, the ventilation management during the T-tube insertion process is more complicated. Here, we report a case with complete SGS combined with severe lower trachea collapse, which was successfully managed with T-tube insertion under extracorporeal membrane oxygenation.

Mild Hypothermia Alleviates Complement C5a-Induced Neuronal Autophagy During Brain Ischemia-Reperfusion Injury After Cardiac Arrest.

After restoration of spontaneous circulation (ROSC) following cardiac arrest, complements can be activated and excessive autophagy can contribute to the brain ischemia-reperfusion (I/R) injury. Mild hypothermia (HT) protects against brain I/R injury after ROSC, but the mechanisms have not been fully elucidated. Here, we found that HT significantly inhibited the increases in serum NSE, S100ß, and C5a, as well as neurologic deficit scores, TUNEL-positive cells, and autophagic vacuoles in the pig brain cortex after ROSC. The C5a receptor 1 (C5aR1) mRNA and the C5a, C5aR1, Beclin 1, LC3-II, and cleaved caspase-3 proteins were significantly increased, but the P62 protein and the PI3K/Akt/mTOR pathway-related proteins were significantly reduced in pigs after ROSC or neuronal oxygen-glucose deprivation/reoxygenation. HT could significantly attenuate the above changes in NT-treated neurons. Furthermore, C5a treatment induced autophagy and apoptosis and reduced the PI3K/Akt/mTOR pathway-related proteins in cultured neurons, which could be reversed by C5aR1 antagonist PMX205. Our findings demonstrated that C5a could bind to C5aR1 to induce neuronal autophagy during the brain I/R injury, which was associated with the inhibited PI3K/Akt/mTOR pathway. HT could inhibit C5a-induced neuronal autophagy by regulating the C5a-C5aR1 interaction and the PI3K/Akt/mTOR pathway, which might be one of the neuroprotective mechanisms underlying I/R injury. The C5a receptor 1 (C5aR1) mRNA and the C5a, C5aR1, Beclin 1, LC3-II, and cleaved caspase-3 proteins were significantly increased, but the P62 protein and the PI3K/Akt/mTOR pathway-related proteins were significantly reduced in pigs after ROSC or neuronal oxygen-glucose deprivation/reoxygenation. Mild hypothermia (HT) could significantly attenuate the above changes in NT-treated neurons. Furthermore, C5a treatment induced autophagy and apoptosis and reduced the PI3K/Akt/mTOR pathway-related proteins in cultured neurons, which could be reversed by C5aR1 antagonist PMX205. Proposed mechanism by which HT protects against brain I/R injury by repressing C5a-C5aR1-induced excessive autophagy. Complement activation in response to brain I/R injury generates C5a that can interact with C5aR1 to inactivate mTOR, probably through the PI3K-AKT pathway, which can finally lead to autophagy activation. The excessively activated autophagy ultimately contributes to cell apoptosis and brain injury. HT may alleviate complement activation and then reduce C5a-induced autophagy to protect against brain I/R injury. HT, mild hypothermia; I/R, ischemia reperfusion.

Early high-dose continuous veno-venous hemofiltration alleviates the alterations of CD4+ T lymphocyte subsets in septic patients combined with acute kidney injury.

BACKGROUND: This study aims to determine whether early high-dose continuous venous-venous hemofiltration (CVVH) alleviates the alterations in CD4+ T lymphocyte subsets in septic patients combined with acute kidney injury. METHODS: Enrolled septic patients combined with acute kidney injury were randomized into CVVH (n = 50) and conventional treatment (non-CVVH, n = 53) groups. Healthy volunteers (n = 21) were enrolled. CVVH was initiated within 12 h of intensive care unit (ICU) admission with doses of 35~60 ml/kg/h and maintained for at least 72 h. Th1, Th2, Th17, and Treg were measured by flow cytometry on days 1, 3, and 7 of ICU admission. Sequential organ failure assessment (SOFA) scores were calculated. RESULTS: Th1 percentages and Th1/Th2 ratios were lower, and Th2, Th17, and Treg percentages and Th17/Treg ratios were higher in septic patients compared to healthy volunteers. CVVH significantly increased Th1 percentages and Th1/Th2 ratios, and significantly decreased Th2, Th17, and Treg percentages and Th17/Treg ratios compared to non-CVVH. Th1 percentages and Th1/Th2 ratios were negatively correlated with SOFA scores, while Th2, Th17, and Treg percentages and Th17/Treg ratios were positively correlated with SOFA scores. Patients with CVVH had significantly lower SOFA scores on day 7 of ICU admission and a shorter ICU stay compared to those with non-CVVH. CONCLUSIONS: Septic patients combined with acute kidney injury exhibit different alterations of CD4+ T lymphocyte subsets. Early high-dose CVVH alleviates the alterations, which may be one of the factors associated with improved sepsis severity.

Interventional bronchoscopic therapy in adult patients with tracheobronchial schwannoma.

BACKGROUND: Tracheobronchial schwannomas are extremely rare tumors of neurogenic origin. Treatment includes surgery and interventional bronchoscopic therapy. Studies that have described interventional bronchoscopic therapy for tracheobronchial schwannoma have been reported in the published literature, but most of them are individual case reports, in which the long-term efficacy and recurrence are poorly understood. This study aimed to explore the feasibility, efficacy, and safety of interventional bronchoscopic therapy in adult patients with tracheobronchial schwannoma. METHODS: Patients with pathologically diagnosed tracheobronchial schwannoma between January 2007 and December 2020 who underwent interventional bronchoscopic therapy in a single center in China were retrospectively reviewed. The clinical features, interventional bronchoscopic therapy procedures, complications, and follow-up results were analyzed. RESULTS: A total of 7 patients (5 men and 2 women; median age, 56 years; age range, 26-63 years) were enrolled. Patients' symptoms were all significantly relieved after interventional bronchoscopic therapy, with mean American Thoracic Society Dyspnea Index score decreasing from 2.29±0.76 to 0.29±0.49 (t=6.481; P=0.001). No severe procedure-related complications were observed. Intraluminal tumors were found subsequently in three cases during a short-term follow-up (4 days to 1 month). One patient underwent surgery; thereafter got lost to follow-up, while the other 2 patients underwent a second endoscopic resection with good prognosis. Six patients received long-term follow-up (range, 2-12 years; median period, 6 years), with all patients being in stable conditions. CONCLUSIONS: Interventional bronchoscopic therapy is an acceptable approach in the management of tracheobronchial schwannoma, and is a useful alternative to surgical resection, especially for those who are at a high risk of surgery or unwilling to undergo surgery. However, long-range bronchoscopic surveillance is necessary because of the possibility of tumor recurrence. Due to its benign nature, repeat endoscopic resection can still be considered after recurrence.

Conductive Ni3(HITP)2 MOFs thin films for flexible transparent supercapacitors with high rate capability.

The flexible transparent supercapacitors have been considered as one of the key energy-storage components to power the smart portable electronic devices. However, it is still a challenge to explore flexible transparent capacitive electrodes with high rate capability. Herein, conductive Ni3(HITP)2 (HITP = 2,3,6,7,10,11-hexaiminotriphenylene) thin films are adopted as capacitive electrodes in flexible transparent supercapacitors. The Ni3(HITP)2 electrode possesses the excellent optoelectronic property with optical transmittance (T) of 78.4% and sheet resistance (Rs) of 51.3 Ω sq-1, remarkable areal capacitance (CA) of 1.63 mF cm-2 and highest scan rate up to 5000 mV s-1. The asymmetric Ni3(HITP)2//PEDOT:PSS supercapacitor (T = 61%) yields a high CA of 1.06 mF cm-2 at 3 µA cm-2, which maintains 77.4% as the current density increases by 50 folds. The remarkable rate capability is ascribed to the collaborative advantages of low diffusion resistance and high ion accessibility, resulting from the intrinsic conductivity, short oriented pores and large specific areas of Ni3(HITP)2 films.

Inflammatory anemia-associated parameters are related to 28-day mortality in patients with sepsis admitted to the ICU: a preliminary observational study.

BACKGROUND: Anemia is one of the most common complications of sepsis. Sepsis-related anemia is associated mainly with inflammation. We aimed to observe the changes in the inflammatory anemia-associated parameters of patients with sepsis in the early stage of intensive care unit (ICU) admission and to evaluate their association with 28-day mortality. METHODS: A total of 198 patients with sepsis were divided into survivor (n = 110) and non-survivor (n = 88) groups on the basis of 28-day survival. Healthy volunteers (n = 20) were enrolled as a control group. Plasma levels of iron, ferritin, erythropoietin (EPO), soluble transferrin receptor (sTfR), hepcidin, interleukin-6 (IL-6), hemoglobin and the red blood cell distribution width (RDW) were measured on days 1, 3 and 7 of ICU admission. Clinical data and laboratory findings were collected, and the Sequential Organ Failure Assessment (SOFA) score was calculated. RESULTS: Patients with sepsis showed significant decreases in hemoglobin, plasma iron and sTfR/log ferritin and significant increases in plasma EPO, sTfR, hepcidin, ferritin and IL-6 on days 1, 3 and 7 of ICU admission compared with healthy volunteers. Hemoglobin was correlated negatively with plasma IL-6 and hepcidin. In patients with sepsis, non-survivors had significantly lower plasma iron, EPO and sTfR/log ferritin, but higher plasma hepcidin, ferritin and IL-6 than survivors on days 1, 3 and 7 of ICU admission. Plasma EPO, hepcidin, ferritin, IL-6, sTfR/log ferritin, the RDW and SOFA score were associated significantly with 28-day mortality but to a varying extent. In particular, in predicting 28-day mortality, plasma hepcidin had an area under the receiver operating curve of 0.808 and 87.3% specificity, which was the highest among the inflammatory anemia-associated parameters tested. CONCLUSIONS: Inflammatory anemia-associated parameters changed significantly in patients with sepsis in the first week of ICU admission. Plasma EPO, hepcidin, ferritin, IL-6, sTfR/log ferritin, the RDW and SOFA score were associated significantly with 28-day mortality. Plasma hepcidin might have a superior predictive value, with high specificity, compared with other inflammatory anemia-associated parameters for 28-day mortality of sepsis patients in the ICU.

Interlayer Hydrogen-Bonded Metal Porphyrin Frameworks/MXene Hybrid Film with High Capacitance for Flexible All-Solid-State Supercapacitors.

2D metal-porphyrin frameworks (MPFs) are attractive for advanced energy storage devices. However, the inferior conductivity and low structural stability of MPFs seriously limit their application as flexible free-standing electrodes with high performance. Here, for the first time, an interlayer hydrogen-bonded MXene/MPFs film is proposed to overcome these disadvantages by intercalation of highly conductive MXene nanosheets into MPFs nanosheets via a vacuum-assisted filtration technology. The alternant insertion of MXene and MPFs affords 3D interconnected "MPFs-to-MXene-to-MPFs" conductive networks to accelerate the ionic/electronic transport rates. Meanwhile, the interlayer hydrogen bonds (F···HO and O···HO) contribute a high chemical stability due to a favorable tolerance to volume change caused by phase separation and structural collapse during the charge/discharge process. The synergistic effect makes MXene/MPFs film deliver a capacitance of 326.1 F g-1 at 0.1 A g-1 , 1.64 F cm-2 at 1 mA cm-2 , 694.2 F cm-3 at 1 mA cm-3 and a durability of about 30 000 cycles. The flexible symmetric supercapacitor shows an areal capacitance of 408 mF cm-2 , areal energy density of 20.4 µW h cm-2 , and capacitance retention of 95.9% after 7000 cycles. This work paves an avenue for the further exploration of 2D MOFs in flexible energy storage devices.

Wrinkled two-dimensional ultrathin Cu(ii)-porphyrin framework nanosheets hybridized with polypyrrole for flexible all-solid-state supercapacitors.

Two-dimensional (2D) metal porphyrin frameworks (MPFs) with a π-conjugated skeleton, high surface area, and more accessible active sites have been applied as active materials for supercapacitors, but the poor electrical conductivity limits the enhancement of electrochemical performance. Moreover, MPF-based flexible supercapacitors have been rarely reported. Here, for the first time, we fabricated wrinkled 2D ultrathin 5,10,15,20-tetrakis(4-carboxylphenyl)porphyrin copper(ii) (Cu-TCPP) nanosheets through the surfactant-assisted synthetic method. The as-obtained wrinkled Cu-TCPP nanosheets were further composited with conductive polypyrrole (PPy) to construct a flexible Cu-TCPP/PPy film through the combination of the electrophoretic deposition method and electrochemical polymerization technology. The wrinkled 2D ultrathin Cu-TCPP nanosheets not only offer macroporous channels for fast ion/electron transport, but also effectively reduce the transmission path of electrolyte ions. The PPy affords 3D conductive networks for rapid electron transport and enhances the electrochemical kinetics. The synergistic effect of Cu-TCPP and PPy leads to the enhanced supercapacitive performance of Cu-TCPP/PPy in comparison with the individual components. As a result, the freestanding Cu-TCPP/PPy electrode shows a capacitance of 340.6 mF cm-2 at 1 mA cm-2, 71.14 F cm-3 at 0.1 A cm-3, and 163.34 F g-1 at 0.2 A g-1, more than 45% greater than that of the pristine PPy film of 224.6 mF cm-2, 48.15 F cm-3 and 92.2 F g-1 at the corresponding current densities. Moreover, the fabricated binder-free symmetric supercapacitor possesses a maximum energy density of 2.27 µW h cm-2 at the highest power density of 50 µW cm-2. This work provides a new strategy to design 2D metal-organic framework-based flexible all-solid-state supercapacitors with high energy storage performances.

Cryptogenic organizing pneumonia masquerading as lung carcinoma: A case report and review of the literature.

Cryptogenic organizing pneumonia (COP) is a rare pulmonary disorder of unknown etiology. COP with hemoptysis as the primary presenting symptom has rarely been reported. The present study reported a case of COP that resembled lung carcinoma with hemoptysis as the only clinical symptom. The patient recovered well following thoracoscope surgery. A literature review of 119 COP cases between 1995 and 2015 was presented. Cough, fever and dyspnea were the most common clinical manifestations. The most common imaging manifestations were multiple or single consolidation, lung nodules, migratory sign, reversed halo sign, and multiple ground-glass opacity. A total of 3 cases exhibited COP accompanied by lung cancer. Glucocorticoids were effective for the majority of cases and invasive surgeries were implemented in most cases. The majority of cases recovered or relieved, and the prognosis of COP was relatively good. COP was easily confused with lung tumor and it is necessary to make differential diagnosis between COP and lung cancer. Invasive surgery should be avoided when possible to avoid or reduce patient trauma.

[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. RESULTS: There were 58 females and 41 males. The median age at the diagnosis was 53 years. The most common exposures were low-molecular-weight chemicals (42.7%) and animal proteins (37.5%). Common clinical symptoms included dyspnea on exertion (90.6%) and cough (76.0%). Pulmonary function test showed diffusion abnormality (73.5%) and restrictive ventilatory impairment (59.7%). Chest CT scan revealed patchy or diffuse bilateral ground-glass opacities (64.6%), centrilobular nodules (21.9%), and air trapping (15.6%). Reticulation (45.8%), traction bronchiectasis (21.9%) and honeycombing(9.4%) were present in chronic HP. BAL lymphocyte counts > 0.2 and CD4/CD8 < 0.9 were more commonly seen in patients with a disease course of less than 1 year. The pathologically-confirmed group and the clinically-suspected group shared many similar characteristics including age at diagnosis, gender, clinical manifestation, pulmonary function impairments and imaging findings, but significant differences existed in certain parameters. In the pathologically- confirmed group, the duration of disease was longer (24 months vs 6 months, Z = -2.492, P = 0.013) and clubbed fingers were more common (23.4% vs 8.2%, χ(2) = 4.227, P = 0.040). Diffusion abnormality was present in more patients of this group (90.7% vs 44.0%, χ(2) = 35.219, P < 0.01). By CT scan, reticulation, traction bronchiectasis and honeycombing (57.5% vs 26.5%, χ(2) = 9.434, P < 0.01) were more evident as compared to the clinically-suspected group. The value of transbronchial lung biopsy for diagnosing HP was limited, with a positive result of only 8.2%. Surgical lung biopsy was needed in uncertain cases. CONCLUSION: The diagnosis of HP was difficult. In some cases a clinical diagnosis can be made by combination of history of exposure, CT manifestations and cell classification of BAL. For atypical cases a multi-disciplinary approach including pathologists, radiologists and pulmonologists is needed.

Protective effects of penehyclidine hydrochloride on liver injury in a rat cardiopulmonary bypass model.

BACKGROUND AND OBJECTIVE: Hepatic injury after cardiac surgery is considered to be a consequence of cardiopulmonary bypass (CPB). The aim of this study was to test the hypothesis that penehyclidine hydrochloride (PHC) could attenuate hepatic injury using a rat CPB model. METHODS: Male Sprague-Dawley rats were randomly divided into six groups (eight per group), including sham-operated control, sham low-dose PHC control (0.6 mg kg), sham high-dose PHC control (2.0 mg kg), vehicle control, low-dose PHC (0.6 mg kg) and high-dose PHC (2.0 mg kg)-treated groups. Blood samples were collected from the femoral artery at the cessation of CPB and the serum levels of the liver enzymes, including alanine aminotransferase (ALT) and aspartate aminotransferase (AST), were determined. The ultrastructure of liver tissue was also examined under an electron microscope. RESULTS: In the sham-operated groups, high-dose PHC and low-dose PHC had no significant impact on the levels of respiratory rate, heart rate, blood pressure, ECG, ALT or AST. Compared with the sham group, the serum levels of ALT and AST were increased significantly in the surgical groups. PHC alleviated all the biochemical and histopathological changes in a dose-dependent manner. The ALT and AST levels in the high-dose PHC-treated groups were significantly lower than those in the vehicle control group. CONCLUSION: Treatment with penehyclidine hydrochloride could improve liver function during CPB.

[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. 2009 in this hospital were retrospectively analyzed, and the related literature was reviewed. RESULTS: In the 4 patients, there were 1 male and 3 females. The age at disease onset was 0 - 10 years, and the age at diagnosis was 15 - 53 years. The most common symptoms were productive cough (4/4), sinusitis (3/4), and shortness of breath (3/4). Other manifestations included situs inversus (2/4), infertility (1/4), and tympanitis (1/4). Hypoxemia was found in 3 cases. Obstructive ventilatory impairment accompanied with diffusion dysfunction was observed in 2 cases, while the pulmonary function tests were normal in the other 2 cases. All 4 cases received chest CT scan, and bronchiectasis was present in all of them. Bilateral diffuse micronodules and patchy infiltrates were found in 3 and 2 cases respectively. Electron microscopic examination of the endobronchial biopsy specimen showed lack of dynein arms in 4, lack of muco-cilia in 2, and abnormal arrangement of microtubules in 2 cases. CONCLUSIONS: The Kartagener syndrome is relatively easy to be diagnosed, because it is characterized by the triad of sinusitis, bronchiectasis and situs inversus. However, PCD without situs inversus often goes unrecognized. PCD should be considered in patients with childhood onset disease, bronchiectasis, centrilobular micronodules or tree-in-bud signs in CT scan. Examination of the ciliary ultrastructure is essential to the confirmation of the diagnosis.