bluuebunny/crossref_metadata_2025_split huggingface dataset:
Limb Girdle Muscular Dystrophy: An Interval Study of Weakness and Functional Impairment
Authors: Stübgen Joerg-Patrick 
Published: March 2009

ABSTRACT

Objective:
Prospective studies of the natural history of a myopathy are effective means of measuring the status and progression of disease. This 10-year interval study of progressive weakness and functional decline was aimed to document the natural course of clinically well-characterized limb girdle muscular dystrophy in South Africa.

Methods:
The manual muscle test (MMT) was used to evaluate limb strength; global strength was measured by the average muscle score (AMS). A nonlinear functional grading system was used to measure impairment of the arms and legs. An activities of daily living (ADL) scale was used to assess disability across multiple functional domains in a home environment.

Results:
The mean AMS deteriorated from 5.9 to 4.9 (on a 10-point scale) and was unrelated to patient age and disease duration. Most (78%) patients lost arm function. All patients maintained useful hand function. Two thirds of patients lost leg function. Half of the patients were wheelchair bound, and they reached this level of function around the end of their fourth decade of life. Every patient showed functional deterioration on the ADL scale. The mean score worsened from 65.6 to 77.2 (out of 156 points). The disability change was most pronounced in patients who became wheelchair bound.

Conclusion:
This interval analysis showed a significant decline of limb strength that was independent of patient age or duration of disease and was more rapid in stronger patients. A functional grading system detected decline and was a useful measure of deterioration when applied to patients over intervals. An ADL assessment ascertained a significant functional decline across multiple functional domains in a home environment and allowed us to advise patients on compensatory strategies to limit dependence.
Gustatory Impairment in Patients Undergoing Head and Neck Irradiation
Authors: Mirza Natasha , Machtay Mitchell , Devine Pamela A. , Troxel Andrea , Abboud Soo K. , Doty Richard L. 
Published: March 2009

AbstractObjectives: To determine whether radiation alters taste function and structure.Research Design: Prospective, longitudinal study.Methodology: Testing prior to starting radiation, and 2 weeks, 2 months, and 6 months after completing radiation.Results: Relative to controls, patients had lower taste identification test scores for bitter, salty, and sour tastes. Sour taste also showed a significant group‐by‐time interaction (P = .03). Taste pores were decreased in the irradiated group, with a significant group‐by‐time interaction (P = .03).Conclusion: Head and neck cancer patients have decreased taste function, and radiation adversely affected sour taste and taste pores.
Clinical Features and Outcome of the Tall Cell Variant of Papillary Thyroid Carcinoma
Authors: Leung Alex Kwok‐Cheung , Chow Sin‐Ming , Law Stephen C. K. 
Published: March 2009

AbstractObjectives: To study the clinical features and outcome of the tall cell variant (TCV) of papillary thyroid carcinoma (PTC).Study Design and Methods: A single‐institution retrospective analysis was performed to review patients with TCV and the usual type of PTC diagnosed from 1960 to 2000.Results: Fourteen of 1,108 patients (median follow‐up, 8.9 yr) diagnosed with PTC had TCV. Ten were female, and four were male, with a mean age of 53.7 (33‐81) years. All were ethnic Chinese. Compared with the usual PTC cohort, TCV patients presented at an older age (mean, 53.7 vs. 45.2 yr; P = .015). They had a higher rate of extrathyroidal extension (78.6% vs. 43.4%, P = .009), tracheal invasion (28.6% vs. 9%, P = .034), and carotid vessel invasion (14.3% vs. 1.5%, P = .021). TCV patients had more frequent gross (42.9% vs. 17.2%) and microscopic (14.3% vs. 6%) postoperative locoregional residual disease (P = .008). They also had a higher percentage of stage III and IV disease (American Joint Committee on Cancer, 6th ed) (74.3% vs. 31.3%, P = .009). Ten‐year local failure‐free, regional failure‐free, and metastasis‐free survival were worse in the TCV group (78.6% vs. 88.8%. P = .017; 53.0% vs. 85.9%, P < .0001; 35.7% vs. 92.1%, P < .0001, respectively). The 10‐year cause‐specific survival was also lower in TCV patients (48.2% vs. 93.4%, P < .0001).Conclusion: TCV presents at a higher stage with more advanced local disease. It has a higher risk of locoregional and distant relapse and a worse overall survival rate. Stratification by stage reveals that TCV has significantly higher mortality compared with PTC for stage IV disease. Aggressive treatment and close follow‐up of these patients is necessary.
The Use of Dexamethasone to Reduce Pain After Tonsillectomy in Adults: A Double‐Blind Prospective Randomized Trial
Authors: Lachance Marianne , Lacroix Yolène , Audet Nathalie , Savard Patrick , Thuot François 
Published: March 2009

AbstractObjectives/Hypothesis: To determine the effectiveness of dexamethasone to reduce pain after tonsillectomy in adults by at least 13 mm on the visual analogue scale. The secondary objective was to reduce the use of narcotics by at least 20%.Study Design: This multicentric study is a prospective double‐blind randomized controlled trial.Methods: A total of 102 patients were enrolled and received a 4‐day trial either of dexamethasone in decreasing doses or placebo. The patients were asked to note the level of pain on the visual analogue scale daily for 7 days. They also had to record their consumption of analgesic and any eventual side effects.Results: There were no statistically or clinically significant differences between the two groups for the level of pain noted on the visual analogue scale for the first 4 and 7 days. There were no statistical differences for the consumption of hydromorphone between the two groups.Conclusion: We cannot recommend the use of dexamethasone on a routine basis following tonsillectomy in adults for the reduction of pain or narcotics consumption.
Need for Intensive Histopathologic Analysis to Determine Lymph Node Metastases When Using Sentinel Node Biopsy in Oral Cancer
Authors: Bilde Anders , von Buchwald Christian , Therkildsen Marianne Hamilton , Mortensen Jann , Kirkegaard Jørgen , Charabi Birgitte , Specht Lena 
Published: March 2009

AbstractObjective: To determine the predictive value of sentinel node biopsy (SNB)‐assisted neck dissection in patients with oral squamous cell carcinoma (SCC) stage T1 to 2N0M0 and to determine the incidence of subclinical metastases.Study Design: Prospective cohort study.Methods: Fifty‐one patients with clinically N0 neck underwent SNB‐assisted neck dissection. The localization of the sentinel node (SN) was determined using dynamic and planar lymphoscintigraphy and single photon emission computed tomography‐computed tomography. Histopathologic examination of the harvested SN was performed using step‐serial sectioning with hematoxylin‐eosin (H&E) and immunohistochemistry on formalin‐fixed, paraffin‐embedded tissue.Results: A total of 181 SNs were excised with a median of 3 (range 1–7) SNs per patient. Four percent (2 of 51) of patients with subclinical (occult) lymph node metastasis would have been identified using routine H&E staining, whereas the 18% (9 of 49) were upstaged as a result of additional histopathology when the H&E evaluation was negative. Overall, the incidence of subclinical metastases was 22% (11 of 51).Conclusion: In this study, SNB‐assisted neck dissection proved to be technically feasible in identifying subclinical metastasis, thus accurately staging the neck with a high degree of sensitivity in patients with oral SCC T1 to 2N0M0 when additional histopathology was performed. The vast majority of patients in this study would have been spared selective neck dissection had reliance on SNB been used and selective neck dissection performed only in the case of a positive SN. Future studies should focus on determining whether SNB alone reduces patient morbidity and whether this is as equally effective in the treatment of cervical nodal metastases as compared with selective neck dissection in patients with oral SCC.
Identification of Distant Metastases With Positron–Emission Tomography‐Computed Tomography in Patients With Previously Untreated Head and Neck Cancer
Authors: Gourin Christine G. , Watts Tammara L. , Williams Hadyn T. , Patel Vijay S. , Bilodeau Paul A. , Coleman Teresa A. 
Published: March 2009

AbstractObjectives: To investigate the utility of positron–emission tomography‐computed tomography (PET‐CT) in identifying distant metastatic disease in patients with previously untreated head and neck squamous cell cancer (HNSCC) prior to definitive treatment.Materials and Methods: Retrospective analysis of 27 consecutive patients with previously untreated HNSCC who underwent PET‐CT imaging in addition to chest radiography (CXR) as part of their metastatic workup.Results: The majority of patients (89%) had TNM stage III or IV disease. PET‐CT was suspicious for pulmonary malignancy in four (15%) patients and indeterminate in one (4%) patient. CXR was suspicious for pulmonary malignancy in two (7%) patients. Pulmonary metastases or a new lung primary was present in 3 (11%) patients: 3 of 4 (75%) patients with positive PET‐CT scans and 0 of 23 (0%) patients with negative or indeterminate PET‐CT scans compared with 2 of 2 (100%) patients with positive CXR and 1 of 25 (4%) patients with negative CXR. The sensitivity and specificity of PET‐CT in predicting pulmonary malignancy was 100% and 96%, respectively, with a positive predictive value of 75% and a negative predictive value of 100%. The sensitivity and specificity of CXR in predicting pulmonary malignancy was 67% and 100%, respectively, with a positive predictive value of 100% and a negative predictive value of 96%. Including nonpulmonary sites, the overall incidence of distant metastatic disease was 19% (5/27) with 11% (3/27) unsuspected prior to PET‐CT.Conclusions: PET‐CT improves detection of metastatic disease in the high‐risk patient and should be performed as part of the routine pretreatment evaluation of patients with advanced stage HNSCC.
The Natural History of Idiopathic Unilateral Vocal Fold Paralysis: Evidence and Problems
Authors: Sulica Lucian 
Published: March 2009

AbstractObjectives/Hypothesis: To identify clinical evidence regarding outcome and duration of unilateral idiopathic vocal fold paralysis (IVFP).Study Design: Literature review.Methods: Medline search using the terms “vocal fold paralysis,” “laryngeal paralysis,” “vagal paralysis,” and “recurrent nerve paralysis” was performed. Results were cross‐checked by substituting “palsy” and “paresis” in place of “paralysis,” “cord” in place of “fold,” and adding “idiopathic” to each term. Furthermore, papers antedating the period of time covered by Medline were identified in references of publications. Case series identified by means of these searches were examined for information regarding duration and outcome of IVFP.Results: Seven hundred seventeen cases are reported. Complete recovery of motion occurred in 36 ± 22% (range, 13–83%) of individuals. Some recovery of motion (complete and partial) occurred in 39 ± 20% (range, 19–83%). Complete recovery of voice occurred in 52 ± 17% (range, 25–87%) of cases, some degree of recovery in 61 ± 22% (range, 25–87%). Most cases appear to recover in well under a year, with rare instances of markedly delayed recovery.Conclusions: Review of available evidence reveals a highly variable rate of return of motion in IVFP. The rate of return of normal voice is consistently higher, although similarly variable. The marked differences in rates of recovery among reports appears to be the result of variable definitions of recovery, oversimplified all‐or‐none notions of paralysis and recovery, and inconsistent reporting of time elapsed from onset of paralysis to evaluation. All of these factors should be addressed in future prospective studies to shed further light on the natural history of vocal fold paralysis.
Pathology of Paediatric Gastrointestinal Neuromuscular Disease
Authors: Smith Virpi V 
Published: March 2009

ABSTRACTCongenital and acquired disorders of the enteric neuromusculature are relatively uncommon in childhood, with the exception of Hirschsprung disease. Despite this, a number of histologically well‐defined phenotypes of enteric neuromuscular disease are now recognised in paediatric populations, essentially comprising enteric neuropathies and enteric myopathies. This classification is briefly discussed.
Cost and Consultation Patterns of Abdominal Pain in Uruguayan Children
Authors: Saps Miguel , Bolioli Pablo , Espana Magali , Marshall Beth M , Di Lorenzo Carlo 
Published: March 2009

ABSTRACTBackground:Children with abdominal pain (AP) have worse quality of life and poorer social functioning and school attendance than their healthy peers. This is the first investigation of consultation patterns and costs of AP in South American children.Patients and Methods:All data were collected from Unidad Coronaria Movil in Montevideo, Uruguay. Diagnoses of all house calls during a 4‐year period (January 2002 through December 2005) were analyzed. Variances in consultation patterns based on sex and age were investigated. Supply and personnel costs were analyzed and prorated to obtain an accurate estimate of the cost per house call and outpatient visit.Results:A total of 125,945 in‐home visits and 1588 outpatient consultations were analyzed. Consultation rates for AP peaked among patients 7 to 9 years of age. Female subjects 9 to 14 years of age consulted significantly more frequently for AP than male subjects in the same age group. The average AP consultation accounted for approximately 3.8% of the per capita health care spending in Uruguay in 2005.Conclusions:AP is a global health problem that is present across ethnicities, nationalities, and geographic locations and is associated with significant health care expenditure.
Gastrointestinal Complications of Inherited Epidermolysis Bullosa: Cumulative Experience of the National Epidermolysis Bullosa Registry
Authors: Fine Jo‐David , Johnson Lorraine B , Weiner Madeline , Suchindran Chirayath 
Published: March 2009

ABSTRACTBackground:Portions of the gastrointestinal (GI) tract may be severely involved in patients with inherited epidermolysis bullosa (EB). Evidence‐based data are lacking as to the frequency and time of onset of these complications.Patients and Methods:Cross‐sectional and longitudinal data were analyzed on 3,280 and 450 patients with EB, respectively, who were followed from 1986–2002 as part of the National EB Registry, an epidemiological study that attempted to identify, enroll, and collect data on every EB patient residing within the continental United States. Frequencies of abnormalities arising within the esophagus, stomach, small and large intestines, rectum, and anus were determined for each major EB subtype. Cumulative risks were similarly calculated for esophageal stenoses or strictures, and for severe growth retardation.Results:Esophageal strictures and growth retardation were commonly seen among the more severe EB subtypes, most notably Hallopeau‐Siemens recessive dystrophic EB, and occurred as early as within the first year of life. EB subtype‐specific differences were also observed in the frequency of occurrence of other GI complications.Discussion:A variety of GI complications arise in patients with inherited EB, varying across the major EB subtypes in their relative severity, frequency, and time of onset.Conclusions:Data generated by the National EB Registry should provide a sound basis whereby evidence‐based strategies can be implemented for more effective surveillance and treatment of specific GI complications.
12‐Month Follow‐up After Successful Infliximab Therapy in Pediatric Crohn Disease
Authors: Wynands Jan , Belbouab Reda , Candon Sophie , Talbotec Cécile , Mougenot Jean‐François , Chatenoud Lucienne , Schmitz Jacques , Cézard Jean‐Pierre , Goulet Olivier , Hugot Jean‐Pierre , Ruemmele Frank M 
Published: March 2009

ABSTRACTAim:Infliximab (IFX) therapy is highly efficacious for the induction and maintenance of remission in pediatric Crohn disease (CD). However, to date it is unclear how long patients should be given IFX. Given the increasing safety concerns about the concomitant and prolonged use of IFX and azathioprine in CD, we wanted to address the clinical outcome in pediatric CD patients who responded to IFX medication, once IFX was stopped.Patients and Methods:Upon induction therapy with 3 IFX infusions, 36 of 38 patients with CD were in clinical remission at 3 months. These 36 responders were separated into 2 groups: 16 patients received no further IFX infusions, whereas 20 patients received scheduled maintenance therapy with IFX for 12 months.Results:Among the 16 patients who received no further IFX infusions, 12 experienced relapse during the 12‐month follow‐up interval after IFX was stopped. In the group receiving maintenance therapy, 11 of 20 patients remained in clinical remission at 12 months of therapy, whereas 8 patients required adjustment of IFX doses or intervals. Among the 11 children who were in clinical remission and receiving maintenance therapy without dose adjustment, 8 experienced relapse within 12 months after IFX maintenance therapy was stopped. Overall, the relapse rates after IFX induction or maintenance therapy was stopped were 75% and 72%, respectively.Conclusions:These data indicate that IFX is efficacious in controlling severe pediatric CD; however, to induce and maintain clinical remission, repeated IFX infusions are required, with a need for dose adjustment in a substantial number of patients.
Safety and Efficacy of Granulocyte and Monocyte Adsorption Apheresis in Paediatric Inflammatory Bowel Disease: A Prospective Pilot Study
Authors: Martín de Carpi Javier , Vilar Pere , Prieto Gerardo , Novo María Dolores García , Ribes Carmen , Varea Vicente 
Published: March 2009

ABSTRACTObjective:Selective granulocyte‐monocyte adsorption (GMA) apheresis is a safe technique that has shown efficacy in inflammatory bowel disease (IBD), especially in adult steroid‐dependent and steroid‐refractory ulcerative colitis. GMA apheresis is performed with Adacolumn, a direct blood perfusion system that selectively adsorbs circulating granulocytes and monocytes. Studies on efficacy of GMA apheresis in paediatric IBD are scarce. Our aim was to evaluate efficacy, safety, and tolerability of GMA apheresis in paediatric IBD patients followed for 1 year.Patients and Methods:Nine patients with a mild to moderate flare‐up (6 boys, 3 girls; 5 ulcerative colitis [UC], 4 Crohn disease [CD]) were included. Mean age at inclusion was 13 years and 9 months, and mean disease duration before inclusion was 28 months. All of our patients with UC were steroid‐dependent; patients with CD had been unsuccessfully treated with other therapies. GMA apheresis consisted of 5 consecutive weekly sessions lasting 60 minutes each.Results:After the 5 sessions, 4 of 5 patients with UC and 1 of 4 patients with CD achieved remission. This remission was maintained in 2 of 4 patients with UC and in the single patients with CD. Patients taking steroids could begin to taper their daily doses after the second apheresis, and 3 of 5 of these patients reached the end of the study steroid‐free. GMA apheresis was well tolerated and no severe side effects related to the technique were observed.Conclusions:GMA apheresis is a safe, well‐tolerated technique in paediatric IBD. As previously reported, we have observed a better efficacy in promoting and maintaining remission, and reducing conventional drugs in patients with UC than in patients with CD.
Clinical Response to 2 Dosing Regimens of Lansoprazole in Infants With Gastroesophageal Reflux
Authors: Khoshoo Vikram , Dhume Pooja 
Published: March 2009

ABSTRACTProton pump inhibitors such as lansoprazole are used in the treatment of gastroesophageal reflux disease (GERD), but dosing guidelines for infants have not been determined. The objective of this study was to assess the clinical efficacy of 2 dosing regimens of lansoprazole in infants with GERD using the revised infant gastroesophageal reflux questionnaire scores (I‐GERQ‐R). Thirty consecutive infants (3–7 months) with GERD, whose conditions were diagnosed by I‐GERQ‐R scores of ≥16, were randomly assigned to receive 1 of 2 lansoprazole dosing regimens: 15 mg given once per day (group A) or approximately 7.5 mg given 2 times per day (group B). Matched infants in a control group were treated with an extensively hydrolyzed formula (group C). Daily I‐GERQ‐R scores were gathered, and the scores after 1 and 2 weeks of treatment were used for analysis. The mean pretreatment scores were similar in groups A, B, and C (26.6, 26.9, and 25.9, respectively). After treatment there was a similar drop in the mean scores in groups A and B (20.6 and 20.0, respectively), but not in group C (25.8). At the end of the first week of treatment, in group A, 5 of 15 infants (33%) had a significant reduction in their I‐GERQ‐R scores, whereas in group B, 10 of 15 infants (67%) had a significant reduction in their I‐GERQ‐R scores (P < 0.05). At the end of the second week of treatment, groups A and B had similar numbers of patients with significant improvement (60% and 67%), which was higher than in group C (3/15, 20%). Overall, there was no difference in the symptom response, as measured by I‐GERQ‐R scores, between 15 mg of lansoprazole given once per day and 7.5 mg given twice per day in infants with GERD, but the twice‐daily regimen produced a faster symptom response. Both regimens were significantly better than treatment of infants with an extensively hydrolyzed formula.
Breast Development in the First 2 Years of Life: An Association With Soy‐based Infant Formulas
Authors: Zung Amnon , Glaser Tamar , Kerem Zohar , Zadik Zvi 
Published: March 2009

ABSTRACTObjective:To evaluate the estrogenic effect of soy‐based formulas in female infants. These formulas contain significant amounts of phytoestrogens, compounds with structural similarity to estradiol.Patients and Methods:A cross‐sectional study consisting of 694 female infants ages 3 to 24 months that consecutively attended 10 general pediatric clinics, none of them having been referred for breast development. The presence of breast buds served as a marker for the endocrine effect of soy‐derived phytoestrogens.Results:Of the participants, 92 had consumed soy formulas for more than 3 months. Breast tissue was more prevalent in the second year of life in infants fed soy‐based formula vs those that were breast‐fed and those fed dairy‐based formula (22.0% vs 10.3%; P = 0.02) with an odds ratio of 2.45 (95% confidence interval 1.11–5.39). No differences in breast bud prevalence were observed during the first year of life. Unlike infants on dairy‐based formulas and breast‐feeding, infants fed a soy‐based formula did not demonstrate a decline in the prevalence of breast during the second year of life.Conclusions:We suggest that phytoestrogens impose a preserving effect on breast tissue that is evolved in early infancy, leading eventually to a slower waning of infantile breast tissue.
α‐Defensin Expression in the Gastric Tissue of Children with Helicobacter pylori–Associated Chronic Gastritis: An Immunohistochemical Study
Authors: Soylu Ozlem Bekem , Ozturk Yesim , Ozer Erdener 
Published: March 2009

ABSTRACTIn this study, we evaluated the expression of α‐defensin and its correlation with histological criteria in children with and without Helicobacter pylori–associated gastritis. Forty‐five children were included. Immunohistochemical staining was performed and the relationship between α‐defensin immunoscoring and H pylori status and histological criteria was evaluated. Expression of α‐defensin was significantly higher in the H pylori–positive group (P < 0.001) and it was significantly associated with higher grades of chronic inflammation and neutrophil density (P < 0.001 for both). Our data show that α‐defensin expression is increased in H pylori infection in childhood and is associated with inflammatory tissue damage.