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ABSTRACT: Background: The surgical indication for chronic thromboembolic pulmonary hypertension (CTEPH) has been modified due to recognition of peripheral type CTEPH and changes in surgical methods and skill. Bosentan and sildenafil are used as modern oral therapy (mod Tx) in patients with inoperable CTEPH, although it remains unknown whether they have positive effects on survival. Methods and Results: A total of 202 patients were diagnosed with CTEPH at Chiba University Hospital between 1986 and 2010, 100 of whom underwent pulmonary endarterectomy. Seven medically treated patients with pulmonary vascular resistance (PVR) ≤300dyn·s·cm(-5) were regarded as having mild disease. Survival rate was stratified by date of diagnosis (group 1, 1986-1998; group 2, 1999-2004; group 3, 2005-2010), and prognostic factors in the remaining 95 medically treated patients were investigated. Group 3 included the most patients treated with mod Tx (group 1, 9.1%; group 2, 24.2%; group 3, 65.0%) and had significantly better survival than either group 1 or 2 (5-year survival: group 1, 54.6%; group 2, 69.7%; group 3, 87.3%). Patients receiving mod Tx had significantly better survival than those not on mod Tx (5-year survival: 88.9% vs. 60.2%). Multivariate analysis showed that mod Tx, lower PVR, and lack of comorbidity were significant predictors of better outcome. Conclusions: Medically treated patients with CTEPH had a better survival rate, and the use of mod Tx contributed to improved survival.
Circulation Journal 04/2013; · 3.77 Impact Factor
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ABSTRACT: Lung-specific toxicity induced by epidermal growth factor receptor (EGFR) tyrosine kinase inhibitors (TKIs) for the treatment of non-small cell lung cancer (NSCLC) has emerged as a critical side-effect. Although the clinical features of the pulmonary side-effects of TKIs have been characterized, the details of the molecular mechanisms in the development of this lung-specific toxicity remain to be elucidated. EGFR-dependent epithelial regeneration and restoration plays an important role in the recovery process from lung injury. The lung comprises a unique environment where epithelial cells are exposed to internal agents in the systemic circulation and to airborne particles through the mouth and nose. This unique environment may also be associated with the development of lung-specific toxicity induced by EGFR-TKIs. Therefore, the aim of this review was to provide further insight into the molecular mechanisms of lung-specific toxicity in the context of treatment with EGFR-TKIs.
Oncology letters 11/2012; 4(5):865-867. · 0.11 Impact Factor
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ABSTRACT: ABSTRACT BACKGROUND: Right heart catheterisation (RHC) and pulmonary digital subtraction angiography (PDSA) are the standard method used to diagnose patients with suspected or definite chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to evaluate the ability of 320-slice computed tomography (CT) for simultaneous diagnosis of chronic thoromboembolic findings in the pulmonary arteries and pulmonary hemodynamics based on the curvature of the interventricular septum (IVS) in CTEPH. METHODS: Forty-four patients with high clinical suspicion of CTEPH underwent RHC, PDSA and enhanced double volume retrospective electrocardiogram-gated 320-slice CT. We measured the sensitivity and specificity of CT to detect thrombi in the pulmonary arteries compared with PDSA. We also compared IVS bowing (expressed as curvature) measured on the short-axis cine heart image with pulmonary arterial pressure (PAP) obtained by RHC. RESULTS: Compared with PDSA, the sensitivity and specificity of CT to detect chronic thromboembolic findings were 97.0% and 97.1% at the main/lobar level and 85.8% and 94.6% at the segmental level, respectively. The correlation coefficients of IVS curvature with systolic PAP and mean PAP were -0.79 (P<0.001) and -0.86 (P<0.001), respectively. CONCLUSIONS: The use of 320-slice CT allows less invasive and simultaneous detection of thrombi and evaluation of pulmonary hemodynamics for the diagnostic workup of CTEPH.
Chest 10/2012; · 5.25 Impact Factor
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ABSTRACT: In general, intravascular thrombus formation in the pulmonary arteries is considered to be the most common cause of chronic thromboembolic pulmonary hypertension (CTEPH). The current mainstay of therapy for patients with CTEPH is pulmonary endarterectomy (PEA). Recently, the existence of myofibroblast-like cells in endarterectomized tissues has been demonstrated. At the 2nd passage of these myofibroblast-like cells, a pleomorphic cell type was isolated. Pulmonary intimal sarcoma is a very uncommon neoplastic tumor thought to originate from subendothelial-mesenchymal cells of the pulmonary vascular wall. Because these pleomorphic cells were isolated from the pulmonary vascular beds, it is believed that the analysis of these cells may contribute to the understanding of pulmonary intimal sarcoma. We isolated cells from the endarterectomized tissue from patients with CTEPH and identified one type as sarcoma-like cells (SCLs). The SCLs were characterized as hyperproliferative, anchorage-independent, invasive and serum-independent. Moreover, C.B-17/lcr-scid/scidJcl mice injected subcutaneously with SCLs developed solid, undifferentiated tumors at the site of injection, and those injected intravenously with SCLs via the tail vein developed tumors which grew along the intimal surface of the pulmonary vessels, thus, demonstrating the high tumorigenic potential of these cells. The behavior of SCLs indicated that these cells may have a vascular cell-like potential which can affiliate them with the intimal surface of the pulmonary artery, and which may be shared with pulmonary intimal sarcoma. A further investigation of this mouse model with SCLs may elucidate the mechanism(s) underlying the development of pulmonary intimal sarcoma.
International Journal of Oncology 05/2012; 41(2):701-11. · 2.40 Impact Factor
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Internal Medicine 01/2012; 51(14):1947-8. · 0.94 Impact Factor
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ABSTRACT: Objective Although endothelin receptor antagonists (ERAs) and phosphodiesterase type 5 (PDE5) inhibitors have become the most commonly used treatments for pulmonary arterial hypertension (PAH) since their introduction in 2005, it remains unknown whether these medications play a significant role in the survival of Japanese patients with PAH. Methods The cardiac catheterization and survival data of 103 PAH patients were retrospectively reviewed. A comparison of survival benefits with regard to the type of PAH was completed in PAH patients diagnosed between 2005 and 2012 and those diagnosed between 1983 and 2004 and in patients undergoing treatment with ERAs and/or PDE5 inhibitors and those being treated with conventional therapy and/or oral beraprost. Although pulmonary vascular resistance (PVR) at baseline differed, the more recent group showed better survival rates compared with those observed in the early group (5-year survival: 70.1% vs. 44.8) (p<0.05). In addition, the survival of PAH patients treated with ERAs and/or PDE5 inhibitors was superior to that of the patients treated without these medications (5- and 8-year survival: 77.8% and 66.7% vs. 39.0% and 37.0%, respectively) (p<0.05), especially in patient with idiopathic and heritable PAH. Conclusion Superior survival rates are observed in patients with idiopathic and heritable PAH after introduction of ERAs and PDE5 inhibitors, and the use of these drugs provides benefits for survival.
Internal Medicine 01/2012; 51(19):2721-6. · 0.94 Impact Factor
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ABSTRACT: Small vessel disease is a major determinant of poor outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension (CTEPH). Out-of-proportion pulmonary vascular resistance (PVR) may indicate the presence of small vessel disease, but it is a very subjective evaluation. We investigated poor subpleural perfusion as a marker for small vessel disease and assessed its association with disease severity and surgical outcome of CTEPH.
We assessed the subpleural perfused area in the capillary phase of pulmonary angiography in 104 consecutive patients, including 45 who underwent surgery, and then divided the patients into either the well-perfused group (the subpleural space in at least one segment was well perfused [n = 75]) or the poorly perfused group (subpleural spaces were either unperfused or minimally perfused in all segments [n = 29]). We compared the pulmonary hemodynamics, degree of distal thrombi, and surgical outcome between these two groups.
The poorly perfused group had significantly higher PVR (937 ± 350 dyne/s/cm(5) vs 754 ± 373 dyne/s/cm(5), P = .02) and more distal thrombi, resulting in fewer surgically treated patients (27.6% vs 49.3%, P = .04) compared with the well-perfused group. This group showed a higher surgical mortality (62.5% vs 2.7%) and higher postoperative PVR (656 ± 668 dyne/s/cm(5) vs 319 ± 223 dyne/s/cm(5), P = .04). Even in a multivariate analysis, poor subpleural perfusion was associated with surgical mortality.
Poor subpleural perfusion in the capillary phase of pulmonary angiography might be related to small vessel disease and a poor surgical outcome of CTEPH.
Chest 09/2011; 141(4):929-34. · 5.25 Impact Factor
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Masashi Kantake,
Nobuhiro Tanabe,
Toshihiko Sugiura,
Ayako Shigeta,
Noriyuki Yanagawa,
Takayuki Jujo,
Naoko Kawata,
Hiroyuki Amano,
Yukiko Matsuura,
Rintaro Nishimura,
Ayumi Sekine, Seiichiro Sakao,
Yasunori Kasahara,
Koichiro Tatsumi
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ABSTRACT: BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) has been considered to be caused by single or recurrent pulmonary embolism (PE) arising from deep vein thrombosis (DVT). In Japan, female predominance and association of HLA-B*5201 with CTEPH unrelated to DVT were reported. In acute PE residual proximal DVT is associated with larger obstruction of pulmonary arteries. However, it remains uncertain whether DVT and the type of DVT are associated with clinical phenotype of CTEPH. PURPOSE: To clarify the association of DVT and DVT type with clinical phenotype of CTEPH. METHODS: Among 98 consecutive patients who underwent 16 or 64-slice multidetector CT angiography and indirect venography, 91 patients (66% female, age: 56±3years) with adequate images were enrolled. The associations of DVT and DVT type with pulmonary hemodynamics, CT obstruction index and other clinical parameters were analyzed. RESULTS: DVT was found in 45 patients (49.5%) (distal: 12, proximal: 33), and was significantly associated with male gender and recurrent type. Furthermore, it was more frequent in HLA-B*5201-negative, and d-dimer positive patients. Compared with distal DVT, proximal DVT was associated with male gender, larger CT obstruction index (48.6±13.0 vs. 34.1±13.2%, p=0.004), and higher mean pulmonary arterial pressure (48.2±12.8 vs. 40.8±7.9mmHg, p=0.03). Proximal DVT was significantly associated with the central type of CTEPH only in HLA-B*5201-negative patients. CONCLUSIONS: The existence and type of DVT were associated with clinical phenotype of CTEPH, and proximal DVT might contribute to the central type of CTEPH in only HLA-B*5201-negative patients.
International journal of cardiology 09/2011; · 7.08 Impact Factor
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ABSTRACT: The cause of chronic thromboembolic pulmonary hypertension is unknown and there is no specific circulating biomarker for its detection. The aim of the present study was to use proteomic analysis to detect serum biomarkers by evaluating the serum profiles of low-molecular-weight peptides using matrix-assisted laser desorption/ionization time-of-flight/time-of-flight mass spectrometry in patients with chronic thromboembolic pulmonary hypertension.
Serum low-molecular-weight peptide profiling using the spectrophotometric technique was studied retrospectively in patients with chronic thromboembolic pulmonary hypertension and in controls matched for sex and age. The serum level of a 2989-Da peptide in the sera of patients was significantly higher compared to that of controls. Tandem mass spectrometry indicated that the peptide was a fragment of fibrinogen Aα chain (KMADEAGSEADHEGTHSTKRGHAKSRPV). The serum level of fibrinogen Aα chain fragment, measured using a heavy isotope internal standard, tended toward negative correlation with plasmin-α2-plasmin inhibitor complex (P=0.073) and had a positive correlation with thrombin-anti-thrombin complex (P=0.031).
This fragment may be a potential diagnostic biomarker for chronic thromboembolic pulmonary hyper-tension.
Circulation Journal 09/2011; 75(11):2675-82. · 3.77 Impact Factor
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ABSTRACT: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by intravascular thrombus formation in the pulmonary arteries.Recently, it has been shown that a myofibroblast cell phenotype was predominant within endarterectomized tissues from CTEPH patients. Indeed, our recent study demonstrated the existence of not only myofibroblast-like cells (MFLCs), but also endothelial-like cells (ELCs). Under in vitro conditions, a few transitional cells (co-expressing both endothelial- and SM-cell markers) were observed in the ELC population. We hypothesized that MFLCs in the microenvironment created by the unresolved clot may promote the endothelial-mesenchymal transition and/or induce endothelial cell (EC) dysfunction.
We isolated cells from these tissues and identified them as MFLCs and ELCs. In order to test whether the MFLCs provide the microenvironment which causes EC alterations, ECs were incubated in serum-free medium conditioned by MFLCs, or were grown in co-culture with the MFLCs.
Our experiments demonstrated that MFLCs promoted the commercially available ECs to transit to other mesenchymal phenotypes and/or induced EC dysfunction through inactivation of autophagy, disruption of the mitochondrial reticulum, alteration of the SOD-2 localization, and decreased ROS production. Indeed, ELCs included a few transitional cells, lost the ability to form autophagosomes, and had defective mitochondrial structure/function. Moreover, rapamycin reversed the phenotypic alterations and the gene expression changes in ECs co-cultured with MFLCs, thus suggesting that this agent had beneficial therapeutic effects on ECs in CTEPH tissues.
It is possible that the microenvironment created by the stabilized clot stimulates MFLCs to induce EC alterations.
Respiratory research 08/2011; 12:109. · 3.36 Impact Factor
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ABSTRACT: It is generally accepted that genetic predisposition plays a role in COPD development in susceptible individuals. Therefore, many candidate genes that could be linked to the development of disease have been examined in COPD. However, inconsistent results in different study populations often limit this approach, suggesting that not only genetics, but also other factors, may be contributed to the susceptibility to COPD. Epigenetic mechanisms can affect the transcriptional activity of specific genes, at different points in time, and in different organs. Moreover, these mechanisms can have an effect on people's health. Recently, there is emerging evidence supporting a role of epigenetics for the regulation of inflammatory genes in diseases such as asthma and COPD. Moreover, recent studies suggest that the currently used treatments including corticosteroids may work through epigenetic mechanisms. Epigenetic regulation can be reprogrammed, potentially affecting the risk, aetiology and treatment of various disease states. The epigenetically influenced phenotype could be reversed with demethylating or deacetylating agents, consistent with epigenetic plasticity. The postnatal reversibility of these methylation or acetylation events may therefore provide good opportunities for intervention. The recognition of the role of genetic and epigenetic mechanisms in the development of COPD may identify novel targets that hatch new therapies for patients with COPD.
Respirology 08/2011; 16(7):1056-63. · 2.42 Impact Factor
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Miki Maruoka, Seiichiro Sakao,
Masashi Kantake,
Nobuhiro Tanabe,
Yasunori Kasahara,
Katsushi Kurosu,
Yuichi Takiguchi,
Masahisa Masuda,
Ichiro Yoshino,
Norbert F Voelkel,
Koichiro Tatsumi
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ABSTRACT: It has been generally accepted that chronic thromboembolic pulmonary hypertension (CTEPH) results from pulmonary embolism arising from deep vein thrombosis. An unresolved question regarding the etiology of CTEPH is why pulmonary thromboemboli are stable and resistant to effective anticoagulation. Recently non-resolving pulmonary thromboemboli in CTEPH have been shown to include myofibroblasts. This study investigates the cellular characteristics of myofibroblasts included in the organized thrombotic tissues of CTEPH.
Organized thrombotic tissues of patients with CTEPH were obtained following pulmonary endarterectomy. We isolated cells from endarterectomized tissue from patients with CTEPH and identified them as endothelial-like cells and myofibroblast-like cells.
Myofibroblast-like cells were characterized as hyperproliferative, anchorage-independent, invasive and serum-independent.
Here we report the presence of active myofibroblast-like cells in endarterectomized tissue of CTEPH. We suggest that the formation of myofibroblasts with a high growth potential in the organized thrombotic tissues may be an important event in the pathobiology of this disease.
International journal of cardiology 03/2011; 159(2):119-27. · 7.08 Impact Factor
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ABSTRACT: Most patients with severe pulmonary arterial hypertension (PAH) demonstrate persistent structural alterations in small pulmonary arterioles at the time of diagnosis, including marked proliferation of pulmonary artery endothelial cells (ECs), smooth muscle cells (SMCs) and fibroblasts. Rai et al. have recently proposed a paradigm shift to explain the pathobiology of small vessel disease in severe PAH patients as a quasi-neoplastic process. Indeed, the vascular lesions of patients with severe PAH exhibit some cancer-like characteristics: decreased population of apoptotic cells and overexpression of antiapoptotic proteins. Nevertheless they lack the capability for tissue invasion and metastasis. The article reviews pathomechanisms of vascular lesions in PAH comparing them with each of the cancer defining mechanisms and indicates the potential utility of antineoplastic drugs as antiproliferative treatment in PAH. PDGF has been identified as a novel potential therapeutic target and the successful treatment of experimental PAH with a PDGF receptor tyrosine kinase inhibitor has been demonstrated recently. These findings justify further clinical trials concerning thyrosine kinase inhibitors as future PAH therapies. However, the drugs currently developed for malignant neoplasms to target neoplastic proliferation should be tested carefully in PAH patients due to their cardiac and pulmonary toxicity.
International journal of cardiology 02/2011; 147(1):4-12. · 7.08 Impact Factor
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International journal of cardiology 02/2011; 146(3):449-50. · 7.08 Impact Factor
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Takashi Yamamichi,
Toshihiko Sugiura,
Yasunori Kasahara,
Takashi Higashide,
Takayuki Jyujyo,
Masanori Tsukahara, Seiichiro Sakao,
Katsushi Kurosu,
Nobuhiro Tanabe,
Yuichi Takiguchi,
Koichiro Tatsumi
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ABSTRACT: The patient was a 34-year-old man, who was referred to our hospital because of abnormal shadows in the right lower lung field on a chest radiograph during a medical screening. Chest computed tomography (CT) showed a pulmonary arteriovenous fistula 23 x 17 mm in size in the anterior basal segment of the right lung, together with a single artery and single vein. He had no symptoms and did not have Osler-Weber-Rendu syndrome. Coil embolization was performed in order to decrease the risk of complications associated with right-to-left shunting. Transcatheter embolization using interlocking detachable coils and detachable fibered coils was successfully performed without severe complications. Then, 320-row multidetector CT revealed that the blood flow from the pulmonary artery disappeared just after coil embolization, the blood flow from the pulmonary vein flowed backward, and the fistula was contrasted. The fistula had almost completely disappeared 8 months after embolization. We confirmed that blood flows were interrupted by 320-row CT and pulmonary angiography. 320-row CT was useful for the evaluation of pulmonary arteriovenous fistula and coil embolization.
Nihon Kokyūki Gakkai zasshi = the journal of the Japanese Respiratory Society. 01/2011; 49(1):62-5.
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ABSTRACT: Several lines of evidence indicate that vascular endothelial growth factor (VEGF) plays a prosurvival and antiapoptotic role in endothelial cells. SU5416 is the first VEGF receptor 2 inhibitor to enter clinical development for cancer therapy. A phase I/II study of SU5416 has been completed, and the results show that SU5416 is well tolerated in patients with terminal cancers. It has been shown that VEGF receptor blockade using SU5416 combined with chronic hypoxia results in severe angioproliferative pulmonary hypertension (PAH) with neointimal changes in adult rats. Although classic animal models of pulmonary hypertension (that is, the monocrotaline and hypoxic models) do not form obstructive intimal lesions in the peripheral pulmonary arteries, the SU5416 model has shown pulmonary arterial changes resembling plexiform lesions. Therefore, the SU5416 model of PAH has been used for some time, and it has thus contributed to a better understanding of the pulmonary hypertensive process. However, the mechanism by which SU5416 combined with chronic hypoxia can result in PAH with plexiform-like lesions in adult rats is complex and still remains to be fully elucidated. The most likely explanation is that there is increased apoptosis of endothelial cells in response to the loss of the survival signaling, creating conditions favoring the emergence of apoptosis-resistant cells with increased growth potential, that is, the endothelial cell hyperproliferation that might characterize the plexiform lesions of human PAH. The aim of the present review is to provide information useful for understanding a potent inhibitor of VEGF receptor tyrosine kinase, SU5416, and to better understand its use for generating animal models of PAH.
Respiration 11/2010; 81(3):253-61. · 2.26 Impact Factor
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ABSTRACT: We report a case of a 70-year-old man with chronic thromboembolic pulmonary hypertension (CTEPH) in whom bronchial asthma had been clinically diagnosed and treated, and who showed remarkable improvement by pulmonary endarterectomy. He had dyspnea on exertion and had been clinically treated for bronchial asthma for 15 years. However, his symptoms did not improve after oral and inhaled corticosteroid therapy, and he had dyspnea at rest. CTEPH was suspected by echocardiography and computed tomography (CT) and he was admitted to our hospital. Perfusion scans showed multiple segmental perfusion defects with normal ventilation study, and contrast-enhanced CT showed intramural thrombi in both pulmonary arteries. Right cardiac catheterization revealed a mean pulmonary arterial pressure of 70 mm Hg and pulmonary vascular resistance of 1699 dyn.s.cm(-5) with chronic thromboembolic findings on pulmonary angiography. After surgery his pulmonary hemodynamics and symptoms significantly improved. CTEPH is rarely diagnosed at the initial visit because the only symptom is dyspnea on exertion, and it is often misdiagnosed as other respiratory diseases. But it is important to suspect and diagnose CTEPH in patients with unexplained dyspnea because this disease can be cured by surgery.
Nihon Kokyūki Gakkai zasshi = the journal of the Japanese Respiratory Society. 11/2010; 48(11):836-41.
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ABSTRACT: Idiopathic pulmonary arterial hypertension (PAH) is a disabling condition characterized by PA vasoconstriction and remodeling as well as in situ thrombosis and eventual right heart failure. Idiopathic PAH occurs more frequently in females than in males. The female:male ratio is 1.64 ∼ 3.88:1. Although endogenous sex hormones including estrogen have been suggested to account for the observed gender differences in PAH, a precise pathobiology for the gender differences remains uncertain. Recent studies demonstrated that estrogen exerts beneficial effects on the pulmonary vasculature. However, it seems to contradict the female predominance that is observed in idiopathic PAH. Moreover, Sweeney and Voelkel (Sweeney L and Voelkel NF. Eur J Med Res 14: 433-442, 2009) showed that early and long-term estrogen exposure might be correlated with an increased risk of the development of PAH. Here we ask the question: Is estrogen a friend or a foe? According to accumulating evidence, we postulate that the different effects of estrogens on different target cells could account for this paradox, i.e., estrogens may exert beneficial effects only on the increased muscularization of vessel walls, but not on phenotypically altered endothelial cells. The effects of estrogens on the pulmonary vasculature are potent and complex, yet not fully understood. A better mechanistic understanding may allow for future therapeutic interventions in patients with PAH.
AJP Lung Cellular and Molecular Physiology 10/2010; 299(4):L435-8. · 3.66 Impact Factor
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ABSTRACT: A deletion/insertion (Del/Ins) polymorphism of 28 base pairs (bp) in the 3' untranslated region (UTR) of fibrinogen alpha gene (FGA) was associated with thromboembolic diseases, but the underlying mechanisms remain unknown. Computational predication reveals that the 28 bp polymorphic fragment is complementary to the sequence of a microRNA, miR-759. In this study, we aim to investigate the association and implicated mechanisms between FGA polymorphisms and the susceptibility to chronic thromboembolic pulmonary hypertension (CTEPH). The Del/Ins polymorphism was analyzed in 190 patients with CTEPH and 628 controls. The FGA 3'UTR and miR-759 interaction was investigated using luciferase assay and quantitative RT-PCR method. Expression of miR-759 and FGA in human tissues was investigated by RT-PCR. The results reveal that the allele frequency of Ins was significantly higher in the patients than in the controls (55.8 vs. 47.1%, P=0.003, odds ratio=1.42, 95% confidence interval: 1.13-1.79). Both miR-759 and FGA were expressed in human liver. Co-transfection of miR-759 decreased the expression and mRNA stability of reporter gene containing the FGA 3'UTR. The effect of miR-759 was stronger on the Ins allele than on the Del allele. These observations suggest that the expression of FGA was regulated by miR-759 through its interaction at the polymorphic 3'UTR sequence, which was associated with the susceptibility to CTEPH.
Human Genetics 10/2010; 128(4):443-52. · 5.07 Impact Factor
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ABSTRACT: Selective right pulmonary arteriography and 3-dimensional computed tomography revealed multiple severe stenoses of the peripheral pulmonary artery associated with poststenotic aneurysms in a 65-year-old woman. She was referred to the hospital for evaluation of dry cough, gradually increasing dyspnea and multiple nodular shadows on a chest radiograph. Echocardiography and cardiac catheterization showed severe pulmonary hypertension, though other structural heart diseases or well-characterized congenital syndromes were ruled out. She was diagnosed as isolated peripheral pulmonary artery branch stenosis. Recent advances in CT technology enable a less-invasive assessment of pulmonary artery, and can be useful in the management of pulmonary arterial hypertension.
Internal Medicine 01/2010; 49(17):1895-9. · 0.94 Impact Factor
