Acute disseminated encephalomyelitis after para-influenza infection post bone marrow transplantation.

Acute disseminated encephalomyelitis (ADEM) is a parainfectious or postvaccination demyelinating condition, characterized by rapid onset of multifocal neurological deficits, usually occurring in childhood or adolescence. We report case of ADEM in an allogeneic bone marrow transplant recipient, who presented with rapid onset of paraplegia and widespread neurological deficits 6 weeks after parainfluenza pneumonia. Magnetic resonance imaging (MRI) showed typical features of ADEM, involving the subcortical white matter, brain steam and spinal cord. There was a rapid and complete response to pulse high-dose corticosteriod and intravenous immunoglobulin. The importance of recognition and early treatment of this rare condition in transplantation practice is emphasized.

Computed tomographic evaluation of the role of craniofacial and upper airway morphology in obstructive sleep apnea in Chinese.

OBJECTIVES: To evaluate the relationship between cephalometric parameters, upper airway morphological factors and obstructive sleep apnea (OSA) in Chinese subjects. DESIGN: Polysomnogram (PSG) were performed and scored using standard criteria. Supine lateral cephalometric parameters and pharyngeal cross-sectional areas at the level of velopharynx (VA) and hypopharynx (HA) were measured from computed tomographic scans. The roles of these parameters and other anthropometric/demographic characteristics in OSA (apnea hypopnea index, AHI > or = 5) and their relationship with severity of OSA were explored by multiple logistic and multinominal regression analysis. RESULTS: Ninety-two subjects, ranging from normal (n = 36), mild/moderate OSA (n = 34) to severe OSA (n = 22), were evaluated. Compared with normal subjects, OSA subjects were heavier (body mass index 27 vs. 24 kg/m2) and older (47 vs. 42 years of age); had smaller VA size and VA to HA ratio, lower positioned hyoid bone, longer and thicker soft palate, and more retropositioned mandible relative to maxilla. After controlling for body mass index and age, subjects with severe OSA (AHI > 30) had more retropositioned mandible relative to maxilla (odds ratio, OR 1.31, P = 0.044) and longer soft palate (OR 1.16, P = 0.01), while those with mild/moderate OSA had larger VA to HA ratio (OR 0.17, P = 0.018). CONCLUSIONS: Craniofacial factors and upper airway morphology contributed to severity of OSA in Chinese subjects. Having controlled for obesity, more retropositioned mandible was associated with more severe OSA.

Magnetic resonance spectroscopy and analysis of MECP2 in Rett syndrome.

We studied the in vivo cerebral metabolites and documented the presence of MECP2 gene mutations in six Chinese females with Rett syndrome. Magnetic resonance spectroscopy spectra from the frontal lobe (gray and white matter) and deep gray nuclei (basal ganglia and thalamus) of either side were obtained. N-acetylaspartate/total creatine, choline/total creatine, and N-acetylaspartate/choline ratios were analyzed and compared with six healthy age-matched female control subjects. MECP2 gene mutation was identified in four patients; one patient had polymorphism and one patient did not have gene mutation. N-acetylaspartate/total creatine of the frontal lobe of all patients (mean: 2.63, S.D. = 0.33) was decreased compared with age-matched control subjects (mean: 3.15, S.D. = 0.27), and the difference was statistically significant (P = 0.017) with a mean difference of 0.52 (95% CI = 0.68-0.36). The difference in all other metabolite ratios in the frontal lobe and deep gray nuclei were not statistically significant compared with age-matched control subjects. Mild frontal lobe and anterior temporal lobe atrophy was present in three patients. Proton-magnetic resonance spectroscopy is a sensitive method capable of detecting the biochemical changes in Rett syndrome and is able to detect changes before conventional magnetic resonance imaging. Our preliminary results suggest that reduction in N-acetylaspartate/total creatine ratio may not be related to the MECP2 mutation.

Sputum cytology examination followed by autofluorescence bronchoscopy: a practical way of identifying early stage lung cancer in central airway.

BACKGROUND: The prognosis of early stage lung cancer was superior to that of late stages. We hypothesize that by using sputum cytology as the first screening method followed by autofluorescence bronchoscopy could detect early stage lung cancer in the central airway. METHODS: During 18-month recruitment period, subjects at high risk for lung cancer (ever smoker accumulated more than 20 pack-year and above 40 years) followed up at Chest Clinics were invited to submit sputum for cytological examination. Subjects with sputum atypia were invited to have bronchoscopy, and CT thorax. After a mean follow-up of 39+/-14 months, the characteristics of lung cancers detected in the group with sputum atypia and the group with normal sputum at baseline were assessed. RESULTS: 181 subjects submitted sputum and primary lung cancer were diagnosed in 13. 46.2% of the lung cancers were in early stages. Bronchoscopy were performed in 85, and seven were confirmed to have lung cancer (six were in early stages). 81 had CT done and 92.6% had radiological abnormalities, though three lung cancers (all stage 0) were missed by CT. Five more primary lung cancers were diagnosed during the follow-up period: one in sputum atypia group and the other four (three were advanced adenocarcinoma) in normal sputum group. The overall sensitivity of sputum cytology in detecting lung cancer was 71.4% for all histology and 100% for squamous cell lung cancer. CONCLUSIONS: Sputum cytology examination followed by bronchoscopy was a practical way of detecting early stage lung cancer in central airway.

Use of the oral chelator deferiprone in the treatment of iron overload in patients with Hb H disease.

Seventeen non-transfusion-dependent Chinese haemoglobin H (Hb H) disease patients (age 29-76 years) with serum ferritin >900 microg/l were treated with deferiprone for up to 18 months. One patient withdrew and data from 16 patients were analysed. Sixteen other Hb H patients with ferritin <900 microg/l, matched for age and genotype, acted as controls. Treatment was well tolerated except for mild arthralgia. Serum ferritin fell with treatment, reaching significance at 6 and 18 months (from 1492.3 +/- 901.4 to 519.4 +/- 405.4 microg/l at 18 months, P = 0.0008). Nine of 16 patients had levels below 397 microg/l before 18 months. Serum ferritin remained stable 6 months after stopping treatment. In contrast, there was no change in ferritin levels in the control group. Magnetic resonance imaging was used for measurement of liver iron content. Spin echo T(1)-signal intensity ratio (T(1)-SIR) and gradient echo T(2)-signal intensity ratio (T(2)-SIR) increased with treatment. T(2)-SIR rose from 0.17 +/- 0.08 pretreatment to 0.58 +/- 0.50 at 2 years (P = 0.0055). Improvement occurred in 12 of 16 patients, reaching normal in three patients. Using echocardiography, peak early diastolic : late diastolic blood flow (E/A) remained unchanged with treatment, but isovolumic relaxation time (IVRT) was prolonged at 2 years indicating mild impairment of diastolic function. All systolic function parameters were normal. A longer treatment period is desirable to demonstrate improvement in cardiac function.

Endocardial visualization of esophageal-left atrial anatomic relationship by three-dimensional multidetector computed tomography "navigator imaging".

BACKGROUND: The close proximity of left atrium (LA) and esophagus during radiofrequency ablation for atrial fibrillation (AF) predisposes to thermal injury resulting in atrio-esophageal fistula. This work proposes to study the anatomic relationship between the esophagus and the LA wall using multidetector computed tomography (MDCT) three-dimensional (3D) "Navigator" reconstruction technique. METHODS: Forty-five consecutive patients (37 men, mean age 52.7+/-14.1 years) with preradiofrequency ablation MDCT scans of the thorax for AF were recruited. Length and type (continuous or interrupted) of fat pad between esophagus and LA were evaluated. The position, width, and length of the esophagus in contact (without fat pad) with the LA were determined by using "Navigator" software on the endocardial view of LA. RESULTS: The fat pad was continuous in 4% (2 of 45) and interrupted in 96% (43 of 45) patients. The mean width and length of esophageal-LA contact in 43 cases with interrupted fat pad was 24.0+/-5.8 mm (range 10.5-35.3 mm) and 41.9+/-11.6 mm (5.4-64 mm), respectively. There was an inverse relationship between the lengths of the esophageal-LA contact and the upper fat pad (r=-0.50, P=0.001). The esophagus was located to the left, right, and midline of the LA in 40, 2, and 1 patients, respectively, and the esophagus was in contact with and overrode the PV orifice in 22 and 4 patients, respectively. CONCLUSION: Direct esophageal-LA contact without the intervening fat pad was present in 96% of the cases, with 93% of esophagi lying to the left of the LA and 51% in contact with a PV orifice. Three-dimensional Navigator imaging technique has enhanced the visualization of the anatomical information of the esophagus, LA wall, and PV orifices that may be used to avoid thermal injury to the esophagus during LA ablation procedure.

Radiographic-clinical correlation in severe acute respiratory syndrome: study of 1373 patients in Hong Kong.

PURPOSE: To retrospectively analyze serial chest radiographs in all patients with severe acute respiratory syndrome (SARS) in Hong Kong for temporal changes and differences between patients who died and those who were discharged from the hospital and to compare radiographic and clinical parameters. MATERIALS AND METHODS: This retrospective study had ethics review board endorsement, and the need for informed consent was waived. Selected serial chest radiographs obtained from the time of presentation until discharge or death in 1373 patients with laboratory-confirmed SARS were scored. Scoring was based on the area and location of lung opacification on radiographs obtained at each of five milestones (presentation, beginning of ribavirin therapy, beginning of corticosteroid therapy, time of most severe radiographic appearance of disease, and before discharge or death). Extents of lung opacification at these five milestones were compared between patients who died and those who survived (by using a repeated-measures analysis of variance model), and the temporal trend of the radiographic-clinical parameters was analyzed (by using Cochran-Armitage trend testing, Kendall tau correlation coefficients, and descriptive graphic analysis). RESULTS: The final cohort consisted of 1373 patients (1212 of whom [485 male and 727 female patients; mean age, 38.4 years] survived and 161 of whom [84 male and 77 female patients; mean age, 63.0 years] died). Among survivors, older patients had more extensive radiographic changes than younger ones. However, among patients who died, older patients had less extensive radiographic opacification at the worst stage of disease and just before death than did younger patients. Despite a higher mortality risk for male patients, both sexes in the same outcome group had similar radiographic findings. For both outcome groups, the rate of radiographic progression was similar for the first 11 days but diverged afterwards. The extent of opacification increased by approximately one zone every 4-5 days for the initial 11 days. Radiographic scores correlated with the ratio of PaO2 to the fraction of inspired oxygen, lymphocyte count, lactate dehydrogenase level, and neutrophil count at each milestone and in terms of changes between milestones (P < .01 for all correlation coefficients, except for radiographic score and neutrophil count between the first two milestones). CONCLUSION: The initial extent of radiographic opacification may be useful for prognostic prediction. Radiographic progression correlates well with that of important clinical and laboratory parameters and may be used as an objective prognostic indicator early in SARS.

Clinical, Laboratory, and Radiologic Manifestation of SARS.

Severe acute respiratory syndrome (SARS) is a highly contagious and predominantly pneumonic illness caused by a novel coronavirus now commonly known as SARS-CoV. This article describes the key diagnostic clinical features, radiologic features, and investigation profiles of affected patients. We summarize our understanding from anecdotal experience and limited published data on the use of antiviral and corticosteroid therapy in the management of this highly contagious disease.

Severe acute respiratory syndrome: radiographic evaluation and clinical outcome measures.

PURPOSE: To evaluate the relationship among chest radiographs, oxygen supplementation requirement, and treatment response in severe acute respiratory syndrome (SARS). MATERIALS AND METHODS: Forty patients (20 women, 20 men; mean age, 42.90 years +/- 14.01 [SD]; median age, 41.5 years; age range, 25-82 years) with SARS were evaluated. Daily chest radiographs were graded according to percentage of lung involvement during 20.15 days +/- 5.56 (median, 20 days; range, 14-38 days). Times between symptoms and treatment and time to reach maximal radiographic score from admission and treatment day were determined. Daily oxygen saturation (Sao2) and oxygen supplementation including mechanically assisted ventilation were recorded. Treatment response was defined as good, fair, and poor. Patterns of radiographic opacity at admission and at maximal radiographic score were noted. Differences in radiographic and clinical parameters with respect to oxygen supplementation and treatment response were respectively evaluated with Mann-Whitney and Kruskal-Wallis tests. RESULTS: Larger maximal radiographic scores, lower Sao2 at maximal radiographic change, longer time from treatment to maximal radiographic score (P <.01), and diffuse consolidation at maximal radiographic score were associated with oxygen supplementation. Parameters that influenced treatment response were time from symptom onset to treatment day (P =.003), time from admission to treatment day (P <.001), time to maximal radiographic score from treatment day (P =.001), maximal radiographic score (P =.009), Sao2 at maximal radiographic score (P =.13), and treatment radiographic score (P =.03). Fair responders had shorter time between admission and treatment than did either good (P <.001) or poor responders (P =.002) and shorter time between symptoms and treatment (P <.001) and lower treatment radiographic score (P =.012) than did good responders. Good (82%), poor (36%), and fair (33%) responders developed maximal chest radiographic scores within 4 days of treatment (P =.008). Radiographic patterns at both admission and maximal radiographic score did not influence treatment response. CONCLUSION: There are significant relationships among radiographic parameters, oxygen supplementation, and treatment response, and these relationships appear to be clinically useful in the treatment of SARS.

Severe acute respiratory syndrome: relationship between radiologic and clinical parameters.

PURPOSE: To quantify severity of severe acute respiratory syndrome (SARS) on chest radiographs and to determine its relationship with clinical parameters. MATERIALS AND METHODS: Forty patients (mean age, 42.90 years +/- 14.01 [SD]; median age, 41.5 years; age range, 25-82 years) with clinically diagnosed SARS were evaluated. Heart rate, oxygen saturation, temperature, and alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were recorded daily. Severity of lung changes on chest radiographs was scored according to percentage of involved lung. Radiographic scores at days of admission, treatment, and maximal radiographic score were extracted for statistical analysis with clinical parameters. Time to maximal radiographic score from admission and days between onset and beginning of treatment were determined. Correlations between radiographic and clinical parameters were evaluated with Spearman rank correlation. Sex differences with respect to clinical and radiographic parameters were evaluated with Mann-Whitney test. RESULTS: Median chest radiographic scores peaked 5 days after beginning of treatment before they declined. Maximal and treatment radiographic scores were inversely related to oxygen saturation (r = -0.67, P <.001; r = -0.35, P =.03). Admission radiographic score was correlated with admission AST level (r = 0.53, P =.003); treatment radiographic score, with treatment ALT and AST levels (r = 0.43, P =.007; r = 0.42, P =.019); and time to maximal radiographic score, with AST level at maximal radiographic score (r = -0.45, P =.006), admission radiographic score (r = -0.55, P <.001), treatment radiographic score (r = -0.58, P <.001), and admission ALT and AST levels (r = -0.44, P =.007; r = -0.58, P =.001). Treatment delay was associated with AST level at maximal radiographic score (r = 0.53, P =.001), treatment radiographic score (r = 0.60, P <.001), and time to maximal radiographic score (r = -0.36, P =.02). No sex differences occurred with respect to radiographic and clinical parameters (P >.05). CONCLUSION: Severity of lung abnormalities quantified on chest radiographs correlates with clinical and laboratory parameters.