ABSTRACT
Objective To discuss the clinical features, diagnosis, treatment and prognosis of primary tracheobronchial amyloidosis. Methods We retrospectively studied 50 primary tracheobronchial amyloidosis cases reported in Chinese literature during 1989-2012 and one in our hospital. The clinical information, symptoms, imaging findings, endoscopic manifestations and treatment were analyzed. Results The patient in our hospital was admitted due to "short breath". Chest CT, bronchoscopy and pulmonary funetion examination were done; bronchoscopy biopsy specimen confirmed the diagnosis of PTA; and the patients responded to cyclophosphamide treatment. The main clinical symptoms of the 50 patients included cough (96%, 48/ 50), short breath (70%, 35/50), sputum (66%, 33/50), fever (24%, 12/50), and hemoptysis (22%, 11/50). Thiekening of lung markings (45. 5 %, 15/33) was themost common ehest X-ray finding; airway wall thiekening and stenosis (76. 2%, 32/42) was the most common CT manifestation; and the most common endoscopic finding was also airway wall thiekening and stenosis (40. 8%, 20/49), followed by mucosal congestion and edema (32. 7%, 16/49). Obstructive ventilatory dysfunetion was found to be the major problem during pulmonary funetion tests. The diagnoses in 47(94%) patients were confirmedby bronchoscopic biopsy. Twenty-one literatures reported complications in 3 cases and misdiagnosis in 24 cases. Forty-two of the 50 patients reeeived drug treatment, bronchoscopic interventional therapy, external beam radiation therapy and surgieal treatment, and they responded well. Conclusion Primary tracheobronchial amyloidosis is rare and is prone to be misdiagnosed. Correct diagnose needs pathological examination and currently there is no specific treatment for it.
ABSTRACT
Objective To discuss the clinical features, diagnosis, treatment and prognosis of primary tracheobronchial amyloidosis. Methods We retrospectively studied 50 primary tracheobronchial amyloidosis cases reported in Chinese literature during 1989-2012 and one in our hospital. The clinical information, symptoms, imaging findings, endoscopic manifestations and treatment were analyzed. Results The patient in our hospital was admitted due to "short breath". Chest CT, bronchoscopy and pulmonary funetion examination were done; bronchoscopy biopsy specimen confirmed the diagnosis of PTA; and the patients responded to cyclophosphamide treatment. The main clinical symptoms of the 50 patients included cough (96%, 48/ 50), short breath (70%, 35/50), sputum (66%, 33/50), fever (24%, 12/50), and hemoptysis (22%, 11/50). Thiekening of lung markings (45. 5 %, 15/33) was themost common ehest X-ray finding; airway wall thiekening and stenosis (76. 2%, 32/42) was the most common CT manifestation; and the most common endoscopic finding was also airway wall thiekening and stenosis (40. 8%, 20/49), followed by mucosal congestion and edema (32. 7%, 16/49). Obstructive ventilatory dysfunetion was found to be the major problem during pulmonary funetion tests. The diagnoses in 47(94%) patients were confirmedby bronchoscopic biopsy. Twenty-one literatures reported complications in 3 cases and misdiagnosis in 24 cases. Forty-two of the 50 patients reeeived drug treatment, bronchoscopic interventional therapy, external beam radiation therapy and surgieal treatment, and they responded well. Conclusion Primary tracheobronchial amyloidosis is rare and is prone to be misdiagnosed. Correct diagnose needs pathological examination and currently there is no specific treatment for it.
ABSTRACT
<p><b>OBJECTIVE</b>To introduce the application of Neuroform stent in the treatment of intracranianl wide neck aneurysm.</p><p><b>METHODS</b>Eight patients (9 aneurysms), including 3 males and 5 females, aged from 41 to 74, were treated. Among the 9 aneurysms, there were 3 wide-neck aneurysms in internal carotid artery (ICA), 4 vertebral aneurysms (in 3 cases), and 2 basilar tip giant aneurysms. Heparinization were given for all procedures after femoral artery Seldinger's puncture. Stents were released through 3 m Transcend 0.014 guide wires, which were posited in the aneurysm-carry arteries first, or, through a 205 cm Transcend 0.014 guiding wire. Further coiling was selected for some cases. After the treatment 24 h heparinization were maintained. For coiling cases, aspirin (300 (mg/d) and ticlopiding (250 mg/d) were given at first 6 weeks, and aspirin (300 mg/d) was given following 6 months. For stent alone cases, only 1 month aspirin (300 mg/d) was given.</p><p><b>RESULTS</b>In one ICA aneurysm, the stent moved to the bifurcation of ICA while a coil was pushed into the aneurysm lumen, and the ICA spasmed. Partial occlusion achieved in the aneurysm. The patient died due to bleeding of the aneurysm 20 h after anticoagulation treatment with heparin. One dissection aneurysm and 2 fusiform aneurysms (in bilateral vertebral arterys of one patient) were treated with stents only. The dissection aneurysm was completely occluded after 3 months. In 1 basilar artery (BA) tip giant aneurysm, the distal part of BA spasmed immediately after stenting. Complete occlusion was achieved with coils 4 months later. Another BA tip aneurysm was partially occluded after the stent was deployed. The other 3 aneurysms were completely embolized with the protection of the Neuroform stents. There is no further DSA follow-up of the cases.</p><p><b>CONCLUSIONS</b>The Neuroform stent is easy to pass through the tortuous vessels. Combine with coils, it may be used in the treatment of wide neck aneurysms. However, it has the risk of migration because of the softness.</p>