Outcome of adults with asymptomatic severe aortic stenosis

Objectives: To evaluate clinical and echocardiographic variables that could be used to predict outcomes in patients with asymptomatic severe aortic valve stenosis. Management of asymptomatic severe aortic stenosis is controversial. Because prophylactic surgery may be protective, independent predictors of events that could justify early surgery have been sought. Methods: Outpatients (n= 133; mean [+/- SD] age, 66.2 +/- 13.6 years) with isolated severe asymptomatic aortic stenosis but normal left ventricular function and no previous myocardial infarction were followed up prospectively at a tertiary care hospital. Interventions: We use a ""wait-for-events"" strategy. Clinical and echocardiographic variables were analyzed. Results: Nineteen patients developed angina; 40, dyspnea; 5, syncope; and 7, sudden death during a mean follow-up period of 3.30 +/- 1.87 years. Event-free survival was 90.2 +/- 2.6% at 1 year, 73.4 +/-.9% at 2 years, 70.7 +/- 4.3% at 3 years, 57.8 +/- 4.7% at 4 years, 40.3 +/- 5.0% at 5 years, and 33.3 +/- 5.2% at 6 years. The mean follow-up period until sudden death (1.32 +/- 1.11 years) was shorter than that for dyspnea (2.44 +/- 1.84 years), syncope (2.87 +/- 1.26 years) and angina (3.03 +/- 1.68 years). Cox regression analysis disclosed only reduced but within normal limits ejection fraction as independent predictor of total events. Conclusions: Management on ""wait-for-events"" strategy is generally safe. Progressive left ventricular ejection fraction reduction even within normal limits identified patients at high risk for events in whom valve replacement surgery should be considered. (c) 2007 Elsevier Ireland Ltd. All rights reserved.

Diagnosis, Assessment, and Treatment of Non-Pulmonary Arterial Hypertension Pulmonary Hypertension

The 4th World Symposium on Pulmonary Hypertension was the first international meeting to focus not only on pulmonary arterial hypertension (PAH) but also on the so-called non-PAH forms of pulmonary hypertension (PH). The term ""non-PAH PH"" summarizes those forms of PH that are found in groups 2 to 5 of the current classification of PH, that is, those forms associated with left heart disease, chronic lung disease, recurrent venous thromboembolism, and other diseases. Many of these forms of PH are much more common than PAH, but all of them have been less well studied, especially in terms of medical therapy. The working group on non-PAH PH focused mainly on 4 conditions: chronic obstructive lung disease, interstitial lung disease, chronic thromboembolic PH, and left heart disease. The medical literature regarding the role of PH in these diseases was reviewed, and recommendations regarding diagnosis and treatment of PH in these conditions are provided. Given the lack of robust clinical trials addressing PH in any of these conditions, it is important to conduct further studies to establish the role of medical therapy in non-PAH PH. (J Am Coll Cardiol 2009;54:S85-96) (C) 2009 by the American College of Cardiology Foundation

Derivation and external validation of a simple prediction model for the diagnosis of type 2 Diabetes Mellitus in the Brazilian urban population

A risk score model was developed based in a population of 1,224 individuals from the general population without known diabetes aging 35 years or more from an urban Brazilian population sample in order to select individuals who should be screened in subsequent testing and improve the efficacy of public health assurance. External validation was performed in a second, independent, population from a different city ascertained through a similar epidemiological protocol. The risk score was developed by multiple logistic regression and model performance and cutoff values were derived from a receiver operating characteristic curve. Model`s capacity of predicting fasting blood glucose levels was tested analyzing data from a 5-year follow-up protocol conducted in the general population. Items independently and significantly associated with diabetes were age, BMI and known hypertension. Sensitivity, specificity and proportion of further testing necessary for the best cutoff value were 75.9, 66.9 and 37.2%, respectively. External validation confirmed the model`s adequacy (AUC equal to 0.72). Finally, model score was also capable of predicting fasting blood glucose progression in non-diabetic individuals in a 5-year follow-up period. In conclusion, this simple diabetes risk score was able to identify individuals with an increased likelihood of having diabetes and it can be used to stratify subpopulations in which performing of subsequent tests is necessary and probably cost-effective.

Frequency of 22q11.2 microdeletion in sporadic non-syndromic tetralogy of Fallot cases

Background: Tetralogy of Fallot (TOF) is a congenital conotruncal heart defect commonly found in DiGeorge (DGS) and velocardiofacial (VCFS) syndromes. The deletion of chromosome 22q11 has also been demonstrated in sporadic or familial cases of TOF. The aim of the present study was to investigate the frequency of del22q11 in patients with non-syndromic TOF seen at a tertiary Pediatric Cardiology care center. Method: One hundred and twenty three non-syndromic TOF patients were selected and evaluated by history, physical examination and review of medical records. Venous blood was drawn for genomic DNA extraction after informed consent 22q11 microdeletion diagnosis was conducted through a standardized SNP genotyping assay and consecutive homozygosity mapping. Phenotype-genotype correlations regarding cardiac anatomy were conducted. Results: We evaluated 123 non-syndromic TOF patients for a 22q11 deletion. 105 (85.4%) patients presented pulmonary stenosis and 18 (14.6%) had pulmonary atresia. Eight patients (6.5%) were found to have a deletion. Of the deleted patients, three (37.5%) presented pulmonary atresia. We have verified a tendency towards a higher prevalence of pulmonary atresia when comparing TOF patients with and without 22q11 microdeletion. Conclusions: 22q11.2 deletion in non-syndromic TOF patients is present in approximately 6% of patients. We suggest a tendency towards a higher prevalence of pulmonary atresia in non-syndromic TOF patients with 22q11 microdeletion. Molecular genetic screening of non-syndromic TOF patient may be important for the correct care of these patients and a more specific genetic diagnostic and counseling. (C) 2007 Elsevier Ireland Ltd. All rights reserved.

Observer agreement of spine stenosis on magnetic resonance imaging analysis of patients with cervical spine myelopathy

Objectives: The purpose of this study was to measure the intraobserver and interobserver reliability of magnetic resonance detection of cervical spondylotic myelopathy with and without operational guidelines. Methods: Seven radiologists examined images from 10 patients with cord signal abnormalities and clinical signs of myelopathy. Radiologist examined films twice, with and without operational guidelines designed to define stenotic changes, while blinded to the clinical findings of the patients. Analyses included a Fleiss kappa assessment of intraobserver and interobserver reliability. Results: Results demonstrated high percentage of agreement and strong intraobserver reliability and variable Fleiss kappa, values for interobserver assessment. Operational guidelines did not improve the intraobserver or interobserver agreement. Conclusion: Although the percentage of agreement was high in some cases, the kappa agreement was low-most likely a result of the base rate problem of a kappa analysis. Sample bias toward severe degenerative changes resulted in highly prevalent selections and kappa adjusted values. Nonetheless, the results do suggest that substantial intraobserver kappa agreement and a wide range of interobserver kappa agreement exists among trained radiologists during detection of stenotic changes associated with cervical spondylotic myelopathy.

Real-time and Doppler US after pediatric segmental liver transplantation

Background Accurate diagnosis of portal vein (PV) stenosis by real-time and color Doppler US (CD-US) after segmental liver transplantation in children can decrease morbidity by avoiding unnecessary biopsy, PV hypertension, thrombosis and loss of the graft. Objective To evaluate CD-US parameters for the prediction of PV stenosis after segmental liver transplantation in children. Materials and methods We retrospectively reviewed 61 CD-US examinations measuring the diameter at the PV anastomosis, velocities at the anastomosis (PV1) and in the segment proximal to the anastomosis (PV2), and the PV1/PV2 velocity ratio. The study group comprised patients with stenosis confirmed by angiography and the control group comprised patients with a good clinical outcome. Results PV stenosis was seen in 12 CD-US examinations. The mean PV diameter was smaller in the study group (2.6 mm versus 5.7 mm) and a PV diameter of < 3.5 mm was highly predictive of stenosis (sensitivity 100%, specificity 91.8%). Conclusion A PV diameter of < 3.5 mm is a highly predictive CD-US parameter for the detection of hemodynamically significant stenosis on angiography.

Sonographic Diagnosis of a Ureteral Inguinal Hernia in a Renal Transplant

Complications related to renal transplants have been widely reported in the literature. The most common complications include acute tubular necrosis, rejection, perirenal fluid collections, vascular complications, and urinary tract obstruction, which are promptly identified by imaging studies. Here we report a case of a patient with a rare cause of obstruction: a ureteral inguinal hernia. This is the sixth report of this condition, and, to our knowledge, no previous case has been reported in which sonography played an important role in promptly identifying the underlying condition and allowing additional less hazardous studies, therefore aiding case management.

Accuracy of store-and-forward diagnosis in leproy

Telemedicine might increase the speed of diagnosis for leprosy and reduce the development of disabilities. We compared the accuracy of diagnosis made by telemedicine with that made by in-person examination. The cases were patients with suspected leprosy at eight public health clinics in outlying areas of the city of Sao Paulo. The case history and clinical examination data, and at least two clinical images for each patient, were stored in a web-based system developed for teledermatology. After the examination in the public clinic, patients then attended a teaching hospital for an in-person examination. The benchmark was the clinical examination of two dermatologists at the university hospital. From August 2005 to April 2006, 142 suspected cases of leprosy were forwarded to the website by the doctors at the clinics. Of these, 36 cases were excluded. There was overall agreement in the diagnosis of leprosy in 74% of the 106 remaining cases. The sensitivity was 78% and the specificity was 31%. Although the specificity was low, the study suggests that telemedicine may be a useful low-cost method for obtaining second opinions in programmes to control leprosy.

Breast cancer in Western Australia in 1989 .5. Outcome at 5 years after diagnosis

Background: A follow-up study was undertaken of all Western Australian women who had a new diagnosis of boast cancer during 1989. The aims were to determine survival, frequency of recurrence and quality of life (QoL) of Western Australian women 5 years after a diagnosis of breast cancer; to determine reasons for choice ol rejection of reconstructive surgery in those women treated by mastectomy, and to determine if the choice of lumpectomy or mastectomy affects subsequent QoL. Methods: The vital status as at Ist June 1994 of all 692 women who had a new diagnosis of breast cancer in 1989 was ascertained by electronic linkage to official mortality registrations. A subsample of 215 survivors who had originally been treated by the nine surgeons who had managed 20 or more cases each was sent a reply-paid postal questionnaire asking about follow-up treatment since diagnosis, recurrence of disease, current QoL and attitudes to, and use of, reconstructive surgery. Results: The overall survival rate at 5 years was 80.8% (85.9% and 78.8% for Stage I and II, respectively). Cumulative mortality was 35% lower among the third of patients treated by the nine most active surgeons (14% vs 22%, P < 0.02), but this may be subject to referral bias. The subsample was representative of all surviving cases except for being an average of 2.7 years younger at diagnosis (mean ages 55.2 and 57.9 years). The response rate of the subsample to the postal questionnaire was 78%. Of women who had had a mastectomy. 40% had considered having a reconstruction, but only nine (78%) had undergone this operation. Median QoL on the Rosser scale (maximum = 1.0) was 0.9. QoL was worse for the 23% of patients with a recurrence of breast cancer. Patients treated by breast-conserving surgery showed a trend toward a better QoL compared with those treated by mastectomy. Conclusion: At 5 years after the diagnosis of breast cancer, one in five women had died and an estimated one in four of the survivors had recurrent disease. Quality of life in the remaining patients, half of whom had undergone adjuvant treatment, was very good. These are important baseline data against which to judge the impact of mammographic screening.

Adult starvation and disease-related malnutrition: A proposal for etiology-based diagnosis in the clinical practice setting from the International Consensus Guideline Committee

Background & aims: Multiple definitions for malnutrition syndromes are found in the literature resulting in confusion. Recent evidence suggests that varying degrees of acute or chronic inflammation are key contributing factors in the pathophysiology of malnutrition that is associated with disease or injury. Methods: An International Guideline Committee was constituted to develop a consensus approach to defining malnutrition syndromes for adults in the clinical setting. Consensus was achieved through a series of meetings held at the ASPEN and ESPEN Congresses. Results: It was agreed that an etiology-based approach that incorporates a current understanding of inflammatory response would be most appropriate. The Committee proposes the following nomenclature for nutrition diagnosis in adults in the clinical practice setting. ""Starvation-related malnutrition"", when there is chronic starvation without inflammation, ""chronic disease-related malnutrition"", when inflammation is chronic and of mild to moderate degree, and ""acute disease or injury-related malnutrition"", when inflammation is acute and of severe degree. Conclusions: This commentary is intended to present a simple etiology-based construct for the diagnosis of adult malnutrition in the clinical setting. Development of associated laboratory, functional, food intake, and body weight criteria and their application to routine clinical practice will require validation. (C) 2009 European Society for Clinical Nutrition and Metabolism and ASPEN American Society for Parenteral and Enteral Nutrition. Published by Elsevier Ltd. All rights reserved.

Adult Starvation and Disease-Related Malnutrition: A Proposal for Etiology-Based Diagnosis in the Clinical Practice Setting From the International Consensus Guideline Committee

Background & Aims: Multiple definitions for malnutrition syndromes are found in the literature resulting in confusion. Recent evidence suggests that varying degrees of acute or chronic inflammation are key contributing factors in the pathophysiology of malnutrition that is associated with disease or injury. Methods: An International Guideline Committee was constituted to develop a consensus approach to defining malnutrition syndromes for adults in the clinical setting. Consensus was achieved through a series of meetings held at the ASPEN. and ESPEN Congresses. Results: It was agreed that an etiology-based approach that incorporates a current understanding of inflammatory response would be most appropriate. The Committee proposes the following nomenclature for nutrition diagnosis in adults in the clinical practice setting. ""Starvation-related malnutrition,"" when there is chronic starvation without inflammation, ""chronic disease-related malnutrition"", when inflammation is chronic and of mild to moderate degree, and ""acute disease or injury-related malnutrition"", when inflammation is acute and of severe degree. Conclusions: This commentary is intended to present a simple etiology-based construct for the diagnosis of adult malnutrition in the clinical setting. Development of associated laboratory, functional, food intake, and body weight criteria and their application to routine clinical practice will require validation. (JPEN J Parenter Enteral Mar. 2010;34:156-159)

Solid pseudopapillary neoplasm of the pancreas: distinct patterns of onset, diagnosis, and prognosis for male versus female patients

Background. Solid pseudopapillary neoplasm of the pancreas is a distinctive pancreatic neoplasm with low metastatic potential. This study examines clinical differences and prognosis between male and female Patients. Methods. The medical records of 34 consecutive patients with pancreatic solid pseudopapillaly neoplasms between 1990 and 2006 were reviewed. Whenever feasible, organ-preserving operation was performed. Statistical analysis was performed using chi-square and Student t test. Results. There were 27 women (79%) and seven men (21%) with median age of 23 years. Mean diameter of the tumor was 7 cm. Tumor size tended to be smaller in patients treated in more recent years. Conservative surgery was possible in I I patients including spleen-preserving distal pancreatectomy in 3, central pancreatectomy in 5, and enucleation in 3 patients. Median hospital stay was 11 days, morbidity rate was 62%, including 17 patients with grade A pancreatic fistula, and there was no operative mortality. Mean follow-up time was 84 months. Tumor recurred in 2 patients (6%). Overall late morbidity rate was 12%. At the time of diagnosis, age was ((x) over bar +/- SD) higher among male patients (25 +/- 2 years vs 37 +/- 7 years, P < .05) with no difference in tumor size. The neoplasms were more aggressive in male Patients; therefore, conservative surgery was less likely. There was no correlation between tumor aggressiveness and age of the patient or size of tumor. Conclusion. This is the first single center study to demonstrate that solid pseudopapillary neoplasms in male patients have distinct Patterns Of onset and aggressiveness when compared with female patients. Although valid prognostic criteria are still lacking, it appears that male patients may be best treated by more radical operation and should be observed more closely during follow-up.

Immunohistochemistry and polymerase chain reaction on paraffin-embedded material improve the diagnosis of cutaneous leishmaniasis in the Amazon region

Background Recently, there has been an increase in the incidence of cutaneous leishmaniasis (CL), which represents an important health problem. This increase may be related to the epidemiologic expansion of the infective agent and the increase in tourism in tropical areas. The difficulty in clinical diagnosis, mainly in areas in which CL is not the first consideration of local physicians, has intensified efforts to describe diagnostic tests, which should be specific, sensitive, and practical. Amongst the new tests described are those including nucleic acid amplification (polymerase chain reaction, PCR) and immunohistochemistry (IHC). Methods In this study, we evaluated the sensitivity of a PCR based on small subunit (SSU) ribosomal DNA, in comparison with IHC using Leishmania spp. antibodies, in biopsies embedded in paraffin. Result The results indicated a total sensitivity of 96% (90.9% with PCR and 68.8% with IHC), showing the possibility of using paraffin-embedded biopsies to diagnose CL. Conclusion We propose the use of the two tests together as a routine protocol for diagnosis. This would require the provision of local medical services to perform molecular biology techniques and adequate Leishmania antibodies.