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Correlation of BCR/ABL transcript variants with patients' characteristics in childhood chronic myeloid leukaemia
Adler, Ronald; Viehmann, Susanne; Kuhlisch, Eberhard; Martiniak, Yvonne; Rottgers, Silja; Harbott, Jochen; Suttorp, Meinolf
BACKGROUND AND OBJECTIVE: The characteristic chromosomal translocation t(9;22)(q34;q11) in chronic myeloid leukaemia (CML) mainly results in the two different BCR/ABL fusion transcripts b2a2 or b3a2. Both transcript variants can occur simultaneously due to alternative splicing of the b3a2 transcript. Conflicting results have been reported on the influence of the transcripts on haematological findings at diagnosis and the course of the disease in adults while data concerning these topics on childhood CML are still missing. This paper reports on a correlation of BCR/ABL transcript variants with patients' characteristics in childhood CML. DESIGN AND METHODS: Transcript types were determined in 146 paediatric patients with CML enrolled in trial CML-paed-I. Fifty-five patients (38%) expressed b2a2, 53 patients (36%) b3a2 and 38 patients (26%) both transcripts, respectively. These findings were correlated with patients' characteristics (sex, age, WBC, Hb, platelet count, hepatosplenomegaly, etc.) assessed at diagnosis. RESULTS: While the co-expression of both transcripts was evenly distributed among genders [b2a2 + b3a2: 22 females (28%), 16 males (24%)] a highly significant difference (P = 0.007) was found concerning the expression of the b2a2 transcript [34 male (51%) vs. 21 female (27%)] and vice versa of the b3a2 transcript [17 male (25%) vs. 36 female (45%)]. High platelet counts and the combination of high platelet counts in conjunction with pronounced leukocytosis were observed more often in patients expressing the b3a2 transcript. CONCLUSIONS: These findings demonstrate that in children like in adults specific BCR/ABL transcript types present at diagnosis are associated with distinct haematological alterations (e.g. a high platelet count with the transcript b3a2). However, the sex-dependent skewed distribution of the BCR/ABL transcript types observed so far in this paediatric cohort only deserves further investigation.
PMID: 19067742
ISSN: 0902-4441
CID: 157814
Deep vein thrombosis after reconstructive shoulder arthroplasty: a prospective observational study
Willis, Andrew A; Warren, Russell F; Craig, Edward V; Adler, Ronald S; Cordasco, Frank A; Lyman, Stephen; Fealy, Stephen
This clinical study was performed to document the prevalence of deep vein thrombosis (DVT) after prosthetic shoulder replacement surgery. We prospectively followed 100 consecutive shoulder arthroplasty procedures (total shoulder replacement in 73 and hemiarthroplasty in 27) in 44 male and 56 female patients for 12 weeks (mean age, 67 years; range, 17-88 years). Risk factors for venous thromboembolic disease were assessed preoperatively and postoperatively. A 4-limb surveillance color flow Doppler ultrasound was performed at 2 days (100 patients) and 12 weeks (50 patients randomly selected) after surgery, and the presence and location of DVT were recorded. Postoperative symptomatic or fatal pulmonary emboli (PE) were also recorded. The overall prevalence of DVT was 13.0%, consisting of 13 DVTs in 12 patients. These included 6 ipsilateral and no contralateral upper extremity DVTs and 5 ipsilateral and 2 contralateral lower extremity DVTs. The prevalence of DVT was 10.0% (10/100) at day 2 after surgery and 6.0% (3/50) at week 12 after surgery. The incidence of symptomatic nonfatal PE was 2.0% (2/100), and that of fatal PE was 1.0% (1/100). Risk factors associated with venous thromboembolic disease did not reach statistical significance because of the small study population sample size. At our institution, the prevalence of DVT after reconstructive shoulder arthroplasty was 13.0%, a rate comparable to that after hip arthroplasty (10.3%) but lower than that after knee arthroplasty (27.2%). Shoulder arthroplasty surgeons should be aware of the potential risk of perioperative thromboembolic complications in both the acute and subacute postoperative periods.
PMID: 19095183
ISSN: 1058-2746
CID: 157815
Prospective analysis of arthroscopic rotator cuff repair: prognostic factors affecting clinical and ultrasound outcome
Nho, Shane J; Brown, Barrett S; Lyman, Stephen; Adler, Ronald S; Altchek, David W; MacGillivray, John D
The purpose of this study was to identify potential predictors of function and tendon healing after arthroscopic rotator cuff repair that will enable the orthopaedic surgeon to determine which patients can expect a successful outcome. Between 2003 and 2005, the Arthroscopic Rotator Cuff Registry was established to collect demographic, intraoperative, functional outcome, and ultrasound data prospectively on all patients who underwent primary arthroscopic rotator cuff repair. At total of 193 patients met the study criteria, and 127 (65.8%) completed the 2-year follow-up. The most significant independent factors affecting ultrasound outcome were age (odds ratio [OR], 1.08; 95% confidence interval [CI], 1.02-1.14; P = .006) and tear size (OR, 2.29; 95% CI, 1.55-3.38; P < .001). After adjustment for age and tear size, the intraoperative factors found to be significantly associated with a tendon defect were concomitant biceps procedures (OR, 11.39; 95% CI, 2.90-44.69; P < .001) and acromioclavicular joint procedures (OR, 3.85; 95% CI, 1.46-10.12; P = .006). In contrast to the ultrasound data, the functional outcome variables, such as satisfaction (OR, 3.92; 95% CI, 2.00-7.68; P < .001) and strength (OR, 10.05; 95% CI, 1.61-62.77; P = .01), had a greater role in predicting an American Shoulder and Elbow Surgeons score greater than 90. The progression from a single-tendon rotator cuff tear to a multiple-tendon tear with associated pathology increased the likelihood of tendon defect by at least 9 times, and therefore, earlier surgical intervention for isolated, single-tendon rotator cuff tears could optimize the likelihood of ultrasound healing and an excellent functional outcome.
PMID: 18799326
ISSN: 1058-2746
CID: 157816
Power Doppler sonography in the diagnosis of hemophilic synovitis--a promising tool
Acharya, S S; Schloss, R; Dyke, J P; Mintz, D N; Christos, P; DiMichele, D M; Adler, R S
BACKGROUND: Recurrent hemarthroses in hemophilia results in synovitis and joint arthropathy. Primary prophylaxis when universally instituted at current doses can prevent joint deterioration but is expensive. Alternatively, the selective implementation of prophylaxis would require a more sensitive tool for detecting synovitis than possible with clinical surveillance or plain radiographs. Magnetic resonance imaging (MRI) is such a tool and is utilized for the evaluation of hemophilic joint disease (HJD). However, it is expensive, and requires sedation in younger children precluding its utility for monitoring of synovitis. Ultrasonography (USG) with power Doppler (USG-PDS) has been utilized to detect and quantitate synovial vascularity in other arthritides and could provide an equally effective but less costly tool for HJD, particularly in children who would not require sedation. OBJECTIVES: To determine whether USG-PDS is comparable to MRI in the evaluation of hemophilic synovitis. PATIENTS: A prospective cohort of 31 subjects including 33 joints (knees, elbows, ankles) underwent dynamic contrast enhanced (DCE)-MRI and USG-PDS. RESULTS: USG-PDS measurements of synovial thickness(r = 0.70, P < 0.0001) and synovial vascularity (r = 0.73, P < 0.0001) correlated strongly with those obtained with DCE-MRI. A cutoff of PDS intensity of 1.3 decibels (dB) per mm(2) was found to yield a sensitivity of 100% and a specificity of 94.1% in 17 joints with/without a history of hemarthroses. Pettersson radiographic scores correlated significantly with synovial thickness in adults but not children. CONCLUSIONS: Our data suggest that USG-PDS may be an inexpensive and easily implemented imaging tool for detecting hemophilic synovitis and could be useful in tailoring effective prophylaxis.
PMID: 18823337
ISSN: 1538-7836
CID: 157857
Utility of portable ultrasound in a community in Ghana
Spencer, Jacqueline K; Adler, Ronald S
OBJECTIVE: In many developing countries, access to medical imaging is limited by availability of resources. Portable ultrasound shows great promise to meet the needs in these countries because it is transportable and relatively inexpensive, and it has a wide range of applications. As part of the Ghana Health Mission, Sekondi-Takoradi, Ghana, we explored the utility of ultrasound in primary care and hospital settings during March 2004. Our objective was to evaluate the clinical utility of a portable ultrasound machine in a variety of physical conditions and multiple clinical scenarios. METHODS: Ultrasound examinations were performed at 2 primary care sites and 2 hospitals using a portable ultrasound machine with linear and curved linear phased array transducers. Most ultrasound examinations were musculoskeletal, with the remainder being obstetric, pelvic, breast, vascular, abdominal, and genitourinary examinations. RESULTS: In clinic settings, musculoskeletal ultrasound represented 46% (16) of the ultrasound examinations performed, and 29% (10) of the cases were a combination of abdominal, pelvic, and genitourinary examinations. In hospital settings, abdominal, pelvic, and genitourinary ultrasound examinations combined were 56% (18), and musculoskeletal was 41% (13). Of the 67 ultrasound examinations performed, 81% (54) showed abnormal findings, 81% (54) were considered to add to the clinical diagnosis, and 40% (27) influenced medical care for the patients. CONCLUSIONS: This experience shows the usefulness of portable ultrasound examinations performed by a skilled radiologist in a clinical setting in Ghana; the challenge is to address how to best incorporate ultrasound into the current practice of medical professionals in developing countries.
PMID: 19022999
ISSN: 0278-4297
CID: 157817
The use of spiral computed tomography scans for the detection of pulmonary embolism
Kim, Han Jo; Walcott-Sapp, Sarah; Leggett, Kristi; Bass, Anne; Adler, Ronald S; Pavlov, Helene; Westrich, Geoffrey H
Total joint arthroplasty carries a risk of symptomatic pulmonary embolism (PE). This study examined symptoms and risk factors of patients who underwent postoperative spiral computed tomography (CT) scans. The presenting symptoms and risk factors of total hip arthroplasty and total knee arthroplasty patients who had a postoperative CT scan were reviewed and the location of PE/deep vein thrombosis (DVT) was noted for positive scans. Of the 10209 patients who had total hip or total knee arthroplasty, 478 (4.7%) underwent a spiral CT scan to rule out PE and 136 (1.3%) had scans positive for PE. Twenty-two and four tenths percent of scans were positive for PE only, 2.1% for proximal DVT only, and 6.1% for PE and proximal DVT. Patients taking estrogen and patients presenting with low oxygen saturation postoperatively were significantly (P = .010, P < .001) more likely to have a positive scan. Spiral CT is the gold standard for detection of PE. Thirty-one and one half percent of scans were positive for PE and/or DVT, yet only 19.2% of patients had a predisposing risk factor for PE. Total knee arthroplasty carried a greater risk for PE than THA.
PMID: 18722301
ISSN: 0883-5403
CID: 157818
Rotator cuff in asymptomatic volunteers: contrast-enhanced US depiction of intratendinous and peritendinous vascularity
Adler, Ronald S; Fealy, Stephen; Rudzki, Jonas R; Kadrmas, Warren; Verma, Nikhil N; Pearle, Andrew; Lyman, Stephen; Warren, Russell F
PURPOSE: To test the hypothesis that regional variations in supraspinatus tendon vascularity exist and can be imaged and quantified in asymptomatic individuals by using contrast material-enhanced ultrasonography (US). MATERIALS AND METHODS: After institutional review board approval and informed consent were obtained, 31 volunteers aged 22-65 years (mean age, 41.5 years) underwent lipid microsphere contrast-enhanced shoulder US performed with an L8-4 transducer operating in contrast harmonic mode and a mechanical index of 0.07 in a HIPAA-compliant protocol. Images were obtained in the volunteers at rest and after exercise. Quantitative analysis was performed by using the time-enhancement postcontrast data derived from four regions of interest (ROIs): bursal medial, articular medial, bursal lateral, and articular lateral. Two 2-minute acquisitions were performed after each contrast material bolus. Baseline enhancement and peak enhancement for each ROI were estimated from these acquisitions. Baseline gray-scale and power Doppler US images of the supraspinatus tendon were obtained by using an L12-5 transducer. The Mann-Whitney nonparametric test was used to test for significant differences between ROIs in all volunteers. RESULTS: In the volunteers at rest before exercise, significant variations in regional enhancement between the articular medial zone and both the bursal medial zone (P = .002) and the bursal lateral zone (P = .003) were observed. Differences in enhancement between the articular medial and articular lateral zones approached significance. Greater differentiation (P < .001) was observed after exercise, with a significant increase in apparent enhancement in each ROI in all volunteers. CONCLUSION: This study revealed the spatial distribution of the blood supply to the supraspinatus tendon in asymptomatic individuals. The addition of exercise to the protocol resulted in a significantly increased level of enhancement compared with that at rest and enabled more sensitive assessment of intratendinous and peritendinous vascularity. SUPPLEMENTAL MATERIAL: http://radiology.rsnajnls.org/cgi/content/full/2483071400/DC1.
PMID: 18647848
ISSN: 0033-8419
CID: 157819
Muscle sonography in six patients with hereditary inclusion body myopathy
Adler, Ronald S; Garolfalo, Giovanna; Paget, Stephen; Kagen, Lawrence
OBJECTIVE: To evaluate the morphological changes of muscle with sonography in six patients affected by hereditary inclusion body myopathy (HIBM). MATERIALS AND METHODS: We studied a group of six Persian Jews diagnosed with HIBM. All were homozygous for the GNE mutation M712T. Ultrasonographic examinations of the quadriceps femoris and hamstring muscle groups were performed. A follow-up ultrasound examination was performed, after an interval of 3 years, in four of these patients. Muscles were assessed subjectively as to echogenicity, determined by gray-scale assessment, and loss of normal muscle morphology. Power Doppler sonography (PDS) was used to assess vascularity. RESULTS: A sonographic finding of central atrophy and peripheral sparing resulting in a target-like appearance was noted in the hamstring compartment of all six patients. The quadriceps compartment also showed involvement of the rectus femoris of all patients, which, in some cases, was the only muscle involved in the quadriceps. Vascularity was markedly reduced in the affected areas, with blood flow demonstrated in the peripherally spared areas. The severity of atrophy increased with disease duration. CONCLUSION: In this case series, we describe a new sonographic finding as well as document progression of HIBM disease, which has generally been described as quadriceps sparing. The myopathic target lesion, as well as isolated rectus femoris atrophy, may provide a useful adjunct to disease diagnosis.
PMID: 17962939
ISSN: 0364-2348
CID: 157821
Contrast-enhanced ultrasound characterization of the vascularity of the rotator cuff tendon: age- and activity-related changes in the intact asymptomatic rotator cuff
Rudzki, Jonas R; Adler, Ronald S; Warren, Russell F; Kadrmas, Warren R; Verma, Nikhail; Pearle, Andrew D; Lyman, Stephen; Fealy, Stephen
The natural history of the blood supply to the rotator cuff and its role in the etiology of rotator cuff disease has not been definitively established. To date, there has not been an in-vivo dynamic assessment of the baseline vascularity of the asymptomatic rotator cuff. This study was designed to test the hypothesis that regional variations in supraspinatus tendon vascularity exist with an age-dependent decrease in asymptomatic individuals with intact rotator cuffs. Lipid microsphere, contrast-enhanced ultrasound shoulder examinations were done in 31 patients with a mean age of 41.5 years (range, 22-65 years). Images were obtained at baseline, after contrast administration at rest, and after contrast administration following exercise to visualize the intratendinous blood flow to the supraspinatus tendon. Qualitative and quantitative analysis was performed by determining 4 regions of interest (bursal medial, articular medial, bursal lateral, and articular lateral) with quantification and analysis software (QLAB Philips, Andover, MA) to examine each region of interest and normalize data for interpretation of the mean intensity per pixel. A statistically significant decrease in blood flow to the supraspinatus tendon with age was observed in a comparative analysis of patients aged younger than 40 and older than 40, (P < .05 for all 4 zones after exercise and for the bursal medial, articular medial, and bursal lateral zones after exercise; P = .07 for the articular lateral zone after exercise). A statistically significant increase in blood flow with exercise was observed in an analysis of all patients (P < .001). The age-related decrease in the vascular supply of the tendon observed in this study is consistent with published reports demonstrating an increasing prevalence of rotator cuff pathology with age and may predispose to the development of rotator cuff tendinopathy and, ultimately, attritional tears.
PMID: 18069013
ISSN: 1058-2746
CID: 157820
The use of compact ultrasound in anesthesia: friend or foe [Comment]
Adler, Ronald S
PMID: 18042843
ISSN: 0003-2999
CID: 157822