系统性硬皮病患者的手部X线表现

目的 回顾性分析系统性硬皮病患者的手部X线表现.方法 收集7例确诊为系统性硬皮病患者手部阳性X线平片,分析各病例影像学表现,总结其影像学特点.结果 7例病例中5例指骨末端不同程度的骨质吸收,2例骨质吸收不明显,7例均存在软组织钙化、骨质疏松,3例有关节挛缩征象.结论 指骨末端骨质吸收及软组织沙粒样钙化是系统性硬皮病的手部主要X线表现.     

Esophageal transit study using a sliding sum image: application to patients with probable and definite systemic sclerosis

Purpose  

Esophageal complication is common in systemic sclerosis (SSc), but scintigraphic transit patterns based on each subtype have not been understood well. The aim of this study was to develop a new algorithm for integrating a dynamic esophageal transit study and to apply the method to patients with SSc.     

Ultrasound examination of skin thickness in patients with progressive systemic sclerosis (scleroderma)

A new ultrasound technique for measuring skin thickness in 40 patients with progressive systemic sclerosis (scleroderma) and in 10 healthy control subjects has been evaluated. High frequency (10 MHz) dynamic ultrasound examination was performed at the proximal and middle phalanges of both second fingers. Measurements of the control subjects on two separate occasions demonstrated a good reproducibility of the technique with a standard deviation of 0.06 mm. The skin was thicker in patients with scleroderma 3.3 +/- 0.7 mm (mean +/- SD) compared with control subjects 2.5 +/- 0.2 mm (p less than 0.001). No relation between skin thickness and duration of disease was observed. This method can be used to monitor changes in skin thickness over time.     

P-31 MR spectroscopy of skeletal and cardiac muscle metabolism in patients with systemic sclerosis: A multiple case study

Three-dimensionally localized proton-decoupled phosphorus-31 magnetic resonance (MR) spectroscopy of skeletal and cardiac muscle was performed in six patients with systemic sclerosis. Cardiac (n = 9) and skeletal (n = 6) spectra were also obtained in healthy volunteers. Metabolite ratios and intracellular pH were determined from the spectra of skeletal and cardiac muscle. The phosphocreatine-to-adenosine triphosphate ratio was normal for both skeletal and cardiac muscle in patients with systemic sclerosis. The pH values of skeletal muscle were similar in patients and control subjects (7.13 ± 0.02 vs 7.12 ± 0.01, respectively). In skeletal muscle, the inorganic phosphate-to-phosphocreatine ratio in patients was increased relative to that of control subjects (0.106 ± 0.014 vs 0.086 ± 0.006, respectively; P =.02). P-31 MR spectroscopy showed no abnormalities in the myocardium of patients with systemic sclerosis. Assessment of the inorganic phosphate-to-phosphocreatine ratio in peripheral skeletal muscle may be helpful for assessing disease activity.     

Role of videofluorography swallow study in management of dysphagia in neurologically compromised patients

PURPOSE: The aim of this study was to demonstrate the key role of the videofluorography swallow study (VFSS) in the management of patients with dysphagia and varying degrees of neurological deficit. MATERIALS AND METHODS: In 1 year (March 2004-March 2005) 47 patients with oropharyngeal dysphagia due to different types of neurological deficit and who required rehabilitation were studied. All patients underwent: (1) clinical history assessment, (2) speech therapy assessment and (3) VFSS using digital fluoroscopy (25 frames per second). Patients were divided according to the Waxman classification into seven levels of dysphagia, and the most suitable type of feeding was selected (normal diet, restricted diet, artificial nutrition). At discharge, the possibility of changing the dietary regimen followed in hospital was evaluated based on clinical progress, radiological follow-up and the degree of improvement obtained. RESULTS: VFSS confirmed aspiration in 21/47 (44%) patients, of whom four (8%) had not been suspected at clinical-speech therapy assessment. In 13/47 (28%) patients, VFSS identified changes at the oral (three patients) or pharyngeal stage (three patients) or both (seven patients) but with no signs of silent aspiration. In the remaining 13 (28%) patients, VFSS did not show any changes in swallow dynamics. On the basis of these data, together with the follow-up at the end of rehabilitation treatment, different nutritional strategies were adopted: artificial nutrition [percutaneous endoscopic gastrostomy (PEG) or nasogastric tube], a restricted-consistency diet or normal diet. CONCLUSIONS: Our experience shows that VFSS precisely classifies the degree of dysphagia that conditions the dietary management of each neurologically compromised patient.     

Esophageal transit scintigraphy and structured questionnaire in patients with systemic sclerosis with endoscopically proven reflux esophagitis

Objectives  

Esophageal complications are common in patients with systemic sclerosis (SSc). The relationship between gastroesophageal reflux (GER) symptoms and dysmotility was examined in endoscopically confirmed patients suspected of having reflux esophagitis.     

Osteolysis of the mandible associated with progressive systemic sclerosis

A case of progressive systemic sclerosis presenting with extensive osteolysis of both coronoid processes of the mandible together with the right angle is presented. MRI demonstrated that the adjacent muscles were unchanged in size.     

Digital cineradiographic study of swallowing in patients with amyotrophic lateral sclerosis

PURPOSE: This study was performed to evaluate the usefulness of digital cineradiography in detecting swallowing disorders in dysphagic patients affected by amyotrophic lateral sclerosis (ALS) with a view to planning an adequate therapeutic approach. MATERIAL AND METHODS: From January 2005 to September 2006, 23 patients (10 men and 13 women; mean age 41.3+/-8.6 years) affected by ALS were evaluated with digital cineradiography to assess the grade of dysphagia. All patients were classified using the Hillel ALS Severity Scale (ALSSS). All examinations were performed with radiocontrolled equipment provided with a digital C-arm. RESULTS: The cineradiographic technique enabled us to differentiate patients with disorders of the oral (17/23) and/or pharyngeal (19/23) swallowing phase from those without swallowing dysfunction (4/23). In 14/23 patients, passage of contrast medium into the upper airways was observed during swallowing, whereas in 5/23 cases, aspiration of contrast medium into the lower airways was recorded. CONCLUSIONS: The videofluoroscopic swallowing study has high diagnostic capabilities in the evaluation of swallowing disorders, as it is able to identify the degree and causes of impairment. In addition, the study proved useful for planning speech therapy and for follow-up in patients with ALS.     

Bilateral mandibular condylysis in a patient with systemic sclerosis

A case of progressive osteolysis of the mandibular condyles in a 55-year-old female patient with systemic sclerosis (SSc) is described. Orofacial involvement and radiological manifestations of SSc are discussed.     

Digital cineradiographic swallow study: our experience

PURPOSE: Swallowing disorders can be secondary to different types of diseases in which, at least initially, patients succeed in establishing voluntary or involuntary compensatory mechanisms that enable them to maintain a sufficient nutritional state. When the compensatory mechanisms become insufficient massive food aspiration into the airways can occur and suffocation may prove to be the main pathology. It has been calculated that in the USA about 8,000-10,000 people die each year due to suffocation. The dynamic radiological examination of swallowing is considered important not only for diagnosis, but also for planning a rehabilitation therapy and type of nutrition for the patient and for verifying the results of the therapy. The aim of this study is to analyse the results of our experience in the use of the digital cineradiography system to evaluate patients with normal and pathological swallowing. MATERIALS AND METHODS: We reviewed the digital cineradiography of 220 patients that at no time had undergone surgery and presented no organic pharyngeal or oesophageal disease (excluding hiatus hernia). All the exams followed a standard protocol that included the dynamic evaluation of the larynx, soft palate, pharynx, and gastro-oesophageal junction with a cineradiographic sequence of 12 frames/second with a 512x1024 matrix. There was also an archive of the film in a post-processing console. The patients received single photograms (printed on laserfilm), videotape recordings or CD-ROM of the dynamic exam. RESULTS: 137 (62%) of the patients did not present swallowing alterations although only 7 patients had a negative examination. In 35 cases hiatus hernia was appreciable while in 69 cases the hernia was associated with gastro-oesophageal reflux. In 23 cases aspecific functional disorders of the oesophagus were demonstrated and in 3 cases achalasia. The remaining 83 patients (38%) (37 males and 46 females, average age 57.02 yrs) presented alterations of the oral and/or pharyngeal stages of swallowing: reduction in soft-palate motility (2 cases), unilateral paralysis of the vocal chords (1 case), incontinence of the bolus during the oral stage (8 cases), lingual movement anomalies (4 cases), subepiglottic penetration (62 cases), asymmetric epiglottic tilt, aspiration of the contrast medium in the airway (17 cases), reduction of laryngeal and hyoid bone movement (9 cases), bolus retained in the valleculae and pyriform sinus (13 cases), cricopharyngeal spasm (6 cases), pharyngeal paralysis (1 case); hiatus hernia was also evident in 20 cases and gastro-oesophageal reflux was associated in 13 of them. Overall, 36% of the cases presented an isolated form while 64% of the cases presented a complex dysfunction with several simultaneous alterations. DISCUSSION AND CONCLUSIONS: The videofluorographic swallow study is an important step in the diagnostic evaluation of a dysphagic patient not only as regards the analysis of the main alteration and its capacity to confirm the presence or absence of contrast medium aspiration in the airway, but also because it provides important information on rehabilitation and nutritional orientation (oral/no oral), as well as on the results of the therapy. The recent diffusion of the digital X-ray equipment has made possible its use for the study of the organic and functional diseases of the upper alimentary tract. Currently a standard protocol for the study of swallowing with digital fluorography is not available. The technique we applied, already verified in a significant number of dysphagic patients, has allowed us to distinguish patients with normal swallowing from those with disorders of the oral and pharyngeal stage, and thus to identify disturbance and establish an appropriate rehabilitation treatment.     

Interstitial lung disease in systemic sclerosis

PURPOSE: To evaluate high-resolution CT (HRCT) parameters of inflammation and fibrosis in systemic sclerosis (SSc), for correlation with lung function, skin scores and exercise tolerance. MATERIAL AND METHODS: 45 SSc patients (40 women, 48.5+/-13.4 years), underwent thoracic HRCT, lung function assessment, and modified Rodnan skin scores. Exercise tolerance was also graded. HRCT were scored for extent of 4 HRCT patterns of interstitial lung disease (ILD): ground glass opacification (GGO), reticular, mixed and honeycomb pattern in each lobe. Total HRCT score, inflammation index (GGO and mixed score) and fibrosis index (reticular and honeycomb scores) were correlated with lung function and clinical parameters. RESULTS: ILD was present in 39/45 (86.7%) patients. Abnormal (<80% predicted) forced vital capacity (FVC), total lung capacity (TLC) and carbon monoxide diffusion factor (DLco) were detected in 30%, 22% and 46% of patients. Total HRCT score correlated with FVC (r=-0.43, p=0.008), FEV1 (forced expiratory volume) (r=-0.37, p=0.03), TLC (r=-0.47, p=0.003), and DLCO (r=-0.43, p=0.008); inflammatory index with DLCO (r=-0.43, p=0.008) and exercise tolerance (r=-0.39, p < 0.05); and fibrosis index with FVC (r=-0.31, p=0.05) and TLC (r=-0.38, p=0.02). Higher total HRCT score, and inflammation and fibrosis indices were found in patients with abnormal lung function. CONCLUSION: Qualitative HRCT is able to evaluate inflammation and fibrosis, showing important relationships with diffusion capacity and lung volume, respectively.