What Are the Indications for Spinal Fusion Surgery in Scheuermann Kyphosis?

BACKGROUND: Surgical indications for Scheuermann kyphosis are variable. We sought to evaluate the characteristics of patients undergoing operative versus nonoperative treatment of Scheuermann kyphosis to better understand current practices and the factors which contribute to the decision for surgical management. METHODS: Multicenter prospective cohort study. We evaluated consecutive patients presenting with Scheuermann kyphosis. Patients underwent either surgical or nonoperative management according to surgeon and patient discretion. Preoperative patient-reported outcome measures (Scoliosis Research Society and Spinal Appearance Questionnaire scores), demographics, and radiographic characteristics were assessed. RESULTS: Overall, 150 patients with Scheuermann kyphosis were enrolled, with 77 choosing nonoperative treatment and 73 treated operatively. Compared with the nonoperative cohort, patients treated operatively were older (16.3±2.0 vs. 15.1±2.2, P=0.0004), and had higher body mass index (26.3±7.2 vs. 22.7±6.5, P=0.003), had greater T2-T12 kyphosis (71±14 degrees vs. 61±12 degrees, P<0.001), increased pelvic incidence (46 vs. 41 degrees, P=0.03) and pelvic tilt (10 vs. 3 degrees, P=0.03). There was no detected difference in maximal sagittal Cobb angle in the operative versus nonoperative patients (73±11 vs. 70±12 degrees, P=0.11). Functionally, the operative patients had worse Scoliosis Research Society pain scores (3.7±0.9 vs. 4.1±0.7, P=0.0027) and appearance scores (2.9±0.7 vs. 3.4±0.8, P <0.0001). CONCLUSIONS: Patients undergoing surgical management of Scheuermann disease were more likely to have large body mass index and worse pain scores. Other factors beyond radiographic measurement likely contribute to the decision for surgical management of Scheuermann kyphosis. LEVEL OF EVIDENCE: Level II.

Complications and hardware removal after open reduction and internal fixation of humeral fractures.

BACKGROUND: The purpose of this study was to review internal fixation of humerus fractures using a large national database and assess the incidence of implant removal procedures based on patient characteristics. METHODS: The 2007 Nationwide Inpatient Sample was used to identify two different sets of patients: (1) all patients who received an open internal fixation procedure for a fracture of the upper end, shaft, or lower end of the humerus; and (2) all patients with a removal of implanted devices from the humerus. Patient variables included age, gender, race/ethnicity, median household income, patient geographic region, metropolitan location, and primary expected payor. The differences of characteristics between both groups were determined using χ, Fisher's exact, or unpaired t tests. RESULTS: Open reduction with internal fixation was reported in 7,391 patients. Hardware removals were reported in 751 patients who were determined to have an associated diagnosed complication. From these numbers, the "failure" removal rate as a percentage of the total number of procedures for the year was 10.2%. Nonunion (17.5%) and Mechanical Complication of the Implant (16.2%) were the most common complications. Both payor (p=0.0023) and age (p<0.0001) were found to have significantly different distributions among the open reduction with internal fixation and removal groups. CONCLUSION: These results suggest that the age of the patient and the patient's ability to pay may influence the likelihood of an implant removal procedure. The results will help identify patients who are at high risk for revision and who may benefit from preventative care.

National hardware removal rate associated with internal fixation of facial fractures.

PURPOSE: There has not been a broad national examination of complications and demographics of facial trauma reduction procedures. The literature has reported acceptable and unacceptable hardware removal rates in localized populations. MATERIALS AND METHODS: The 2007 Nationwide Inpatient Sample was used to determine all plate removal procedures associated with common complications from facial reductions. Statistical analysis was used to compare the differences in demographics of the reduction procedure and removal procedure groups. RESULTS: Some form of open fixation was reported in 4,879 patients. Plate removals associated with complications were reported in 246 patients. The "failure" removal rate as a percentage of the total number of open procedures for the year was 5.0%. Gender, race, age, primary payer, and median income of the patient were determined to significantly affect the likelihood for hardware removal due to complications. CONCLUSION: These results suggest that decreased lower bone quality and ability to pay affect the chances that a particular patient will undergo a hardware removal procedure. There is a strong possibility that the reported removal rate underestimates the actual failure rate of the procedures and devices used to treat facial trauma.

Adductor focal laryngeal Dystonia: correlation between clinicians' ratings and subjects' perception of Dysphonia.

BACKGROUND: Although considerable research has focused on the etiology and symptomology of adductor focal laryngeal dystonia (AD-FLD), little is known about the correlation between clinicians' ratings and patients' perception of this voice disturbance. This study has five objectives: first, to determine if there is a relationship between subjects' symptom-severity and its impact on their quality of life; to compare clinicians' ratings with subjects' perception of the individual characteristics and severity of AD-FLD; to document the subjects' perception of changes in dysphonia since diagnosis; to record the frequency of voice arrest during connected speech; and, finally, to calculate inter-clinician reliability based on results from the Unified Spasmodic Dysphonia Rating Scale (USDRS) (Stewart et al, J Voice 1195-10, 1997). METHODS: Sixty subjects with AD-FLD who were receiving ongoing injections of BoNT participated in this study. Subjects' mean age was 60.78 years and their mean duration of symptoms was 16.1 years. Subjects completed the Disease Symptom Questionnaire (DSQ) (specifically designed for this study) and the Voice Handicap Index-10 (VHI-10) (Jacobson et al, Am J Speech Lang Pathol 6:66-70, 1997) to measure the symptoms of their dysphonia and the impact of the disease on their quality of life.Two speech-language pathologists and two laryngologists used the Voice Arrest Measure (VAM) (specifically designed for this study) and the USDRS to independently rate voice recordings of 56/60 subjects. RESULTS: The mean VHI-10 score was 21.3 which is clinically significant. The results of the DSQ and the USDRS were highly correlated. The most severe symptoms identified by both subjects and clinicians were roughness, strain-strangled voice quality, and increased expiratory effort. Voice arrest, aphonia, and tremor were uncommon. Subjects rated their current voice quality at the time of reinjection (i.e., at the time of the study) as significantly better than at the time of their initial AD-FLD diagnosis (p < 0.0001). Inter-clinician reliability on the USDRS was significant at the 0.001 level. CONCLUSIONS: The findings from the VHI-10 suggest that AD-FLD has a profound impact on quality of life. The results of the DSQ and the USDRS suggest that there is a strong correlation between subjects' perception and clinicians' assessment of the individual symptoms and the severity of the dysphonia. The findings from the VAM suggest that voice arrests are infrequent in subjects with AD-FLD who are receiving ongoing BoNT injections. The strong inter-clinician reliability on the USDRS suggests that it is an appropriate measure for identifying symptoms and severity of AD-FLD.

A single course of anti-CD3 monoclonal antibody hOKT3gamma1(Ala-Ala) results in improvement in C-peptide responses and clinical parameters for at least 2 years after onset of type 1 diabetes.

Despite advances in understanding autoimmune diabetes in animal models, there has been little progress in altering the natural course of the human disease, which involves progression to insulin deficiency. Studies with immunosuppressive agents have shown short-term effectiveness, but they have not induced tolerance, and continuous treatment is needed. We studied the effects of hOKT3gamma1(Ala-Ala), a humanized Fc mutated anti-CD3 monoclonal antibody, on the progression of type 1 diabetes in patients with recent-onset disease in a randomized controlled trial. In general, the drug was well tolerated. A single course of treatment, within the first 6 weeks after diagnosis, preserved C-peptide responses to a mixed meal for 1 year after diagnosis (97 +/- 9.6% of response at study entry in drug-treated patients vs. 53 +/- 7.6% in control subjects, P < 0.01), with significant improvement in C-peptide responses to a mixed meal even 2 years after treatment (P < 0.02). The improved C-peptide responses were accompanied by reduced HbA(1c) and insulin requirements. Clinical responses to drug treatment were predicted by an increase in the relative number of CD8(+) T-cells in the peripheral blood after the lymphocyte count recovered 2 weeks after the last dose of drug. We conclude that treatment with the anti-CD3 monoclonal antibody hOKT3gamma1(Ala-Ala) results in improved C-peptide responses and clinical parameters in type 1 diabetes for at least 2 years in the absence of continued immunosuppressive medications.

High-frequency pulsed electromagnetic energy in tinnitus treatment.

OBJECTIVES/HYPOTHESIS: Electromagnetic therapy has been used with reported success in multiple clinical settings, including the treatment of seizure disorders, brain edema, migraine headaches, revascularization of burn wounds, and diabetic ulcers. The purpose of the study was to investigate the effect of pulsed high-frequency electromagnetic therapy on chronic tinnitus. STUDY DESIGN: A randomized, prospective, double-blind, placebo-controlled pilot study was conducted to evaluate the effectiveness of high-frequency pulsed electromagnetic energy using the Diapulse device in the treatment of chronic tinnitus. Thirty-seven adult patients with chronic tinnitus of at least 6 months' duration were recruited and randomly assigned to either a treatment or a placebo group. METHODS: Patients received 30-minute treatments with the Diapulse device three times a week for 1 month. The unit was set to deliver electromagnetic energy at a frequency of 27.12 MHz at a repetition rate of 600 pulses per second. All subjects had pretreatment and post-treatment audiological testing, including tinnitus frequency and intensity matching. They responded to a tinnitus rating questionnaire and the Tinnitus Handicap Inventory before and after treatment. RESULTS: There was no significant change in the pretreatment and post-treatment audiometric thresholds in either group. There were no significant differences between the pretreatment and post-treatment Tinnitus Handicap Inventory scores or the tinnitus rating scores in either subject group (Student t test). No diagnosis-specific differences were identified. CONCLUSION: High-frequency pulsed electromagnetic energy (Diapulse) at the settings used in the study showed no role in the therapy of patients with chronic tinnitus.

Ethnicity and foster care: census data as predictors of placement variables.

An epidemiological approach to foster care is presented, in which two separate data sets are used to analyze issues of ethnicity and child placement. A national probability sample and a large-city sample are drawn from the 1980 Office of Civil Rights ethnic survey and from the 1980 U.S. Census in order to test several hypotheses on ethnic differences predictive of time in care.

Does higher anchor density result in increased curve correction and improved clinical outcomes in adolescent idiopathic scoliosis?

STUDY DESIGN: Retrospective review of prospectively collected data. OBJECTIVE: To determine whether anchor density is associated with curve correction and patient-reported outcomes. SUMMARY OF BACKGROUND DATA: There is limited information as to whether anchor density affects the results of adolescent idiopathic scoliosis surgery. METHODS: A total of 952 patients with adolescent idiopathic scoliosis met inclusion criteria (Lenke 1, 2, and 5 curves) with predominantly screw constructs (no. of screws/no. of total anchors >75%). Anchor density was defined as the number of screws, hooks, and wires per level fused, with less than 1.54 considered low density. Analysis of covariance was undertaken to determine association of anchor density with percent curve correction, Scoliosis Research Society (SRS), and Spinal Appearance Questionnaire (SAQ) scores, controlling for flexibility, fusion length, demographics, and surgeon. RESULTS: High- compared with low-anchor density was associated with increased percent curve correction in Lenke 1 curves at 1 year (69% vs. 66% correction, P = 0.0022), controlling for percent preoperative curve flexibility, length of fusion, and sex (model, P < 0.0001). Similar associations held at 2-year follow-up and for Lenke 2 curves. Decreased thoracic kyphosis was found with increased anchor density for Lenke 1 and 2 curve patterns. There were no associations found between anchor density and Lenke 5 curves. For Lenke 1 curve patterns at 2 years postoperatively, in the high- versus low-anchor density cohorts, there were statistically higher SRS Activity (4.3 vs. 4.2, P = 0.019), Appearance (4.3 vs. 4.1, P = 0.0005), Satisfaction (4.5 vs. 4.3, P = 0.028), and Total scores (4.3 vs. 4.2; P = 0.024). Similarly, the SAQ Appearance score at 1 year similarly was improved in the high-anchor density group (high: 14.1 vs. low: 15.0, P = 0.03) for Lenke 1 curve patterns only. CONCLUSION: For Lenke 1 and 2 curve patterns, improved percent correction of major coronal curve was noted in the high-screw density cohort. Although statistical significance was reached, it is unclear whether screw density resulted in clinically significant differences in patient-reported outcomes.

Feasibility and exploratory efficacy evaluation of the Embrella Embolic Deflector system for the prevention of cerebral emboli in patients undergoing transcatheter aortic valve replacement: the PROTAVI-C pilot study.

OBJECTIVES: This study sought to determine the feasibility, safety, and exploratory efficacy of the Embrella Embolic Deflector (EED) system (Edwards Lifesciences, Irvine, California) in patients undergoing transcatheter aortic valve replacement (TAVR). BACKGROUND: Few data exist on the value of using embolic protection devices during TAVR. METHODS: This pilot study included 52 patients who underwent transfemoral TAVR. The EED system was used in 41 patients, whereas 11 patients underwent TAVR without embolic protection (control group). Cerebral diffusion-weighted magnetic resonance imaging (DW-MRI) was performed at baseline and within 7 days and 30 days after TAVR. RESULTS: The EED system was successfully deployed at the level of the aortic arch in all patients with no complications. The deployment of the EED system was associated with high-intensity transient signals (HITS) as evaluated by transcranial Doppler (median: 48 [interquartile range: 17 to 198] HITS), and a higher total number of HITS was observed in the EED group (p < 0.001 vs. control group). DW-MRI performed within 7 days after TAVR showed the presence of new ischemic lesions in all patients in both groups, with a median number of 7 (interquartile range: 3 to 13) lesions per patient. The use of the EED system was associated with a lower lesion volume compared with the control group (p = 0.003). All new cerebral lesions had disappeared on the DW-MRI performed at 30 days after TAVR. Two strokes unrelated to the EED system occurred 2 and 29 days after TAVR. CONCLUSIONS: This study showed the feasibility and safety of using the EED system in TAVR procedures. The EED system did not prevent the occurrence of cerebral microemboli during TAVR or new transient ischemic lesions as evaluated by DW-MRI, but it was associated with a reduction in lesion volume. Further studies are warranted to determine the efficacy of using the EED system during TAVR procedures.

Discriminative Properties of the Spinal Appearance Questionnaire Compared With the Scoliosis Research Society-22 Revised.

STUDY DESIGN: Longitudinal cohort. OBJECTIVES: To determine the responsiveness of the Spinal Appearance Questionnaire (SAQ) in patients with adolescent idiopathic scoliosis (AIS) undergoing surgical correction of the deformity. SUMMARY OF BACKGROUND DATA: The SAQ has been found to be a valid and reliable measure in patients with AIS. A recently published factor analysis and scoring system has been shown to be applicable to all Lenke types and had greater correlation to the curve magnitude than the Scoliosis Research Society (SRS) Appearance and Total score. METHODS: From a prospective multicenter database, 126 AIS patients who underwent correction of the spinal deformity with complete SAQ and SRS-22 Revised data at baseline and 2-year follow-up were identified. Discriminative properties of the SAQ domains (Expectations, Appearance, and Total) and SRS domains (Appearance, Activity, Pain, Mental, Satisfaction, and Total) were compared by computing the effect size (ES) and the standardized response mean (SRM). RESULTS: The SAQ Total had the largest ES (1.8) and SRM (1.5). This was followed by the SAQ Appearance, with an ES of 1.7 and SRM of 1.4; and the SAQ Expectations, with an ES of 1.5 and SRM of 1.2. Among the different SRS domains, only the Appearance (ES = 1.2, SRM = 1.1), Satisfaction (ES = 0.8, SRM = 0.6), and Total scores (ES = 0.8, SRM = 0.9) had effect sizes that were considered large. The SRS Mental domain had a moderate effect size (ES = 0.3, SRM = 0.3), whereas the Activity (ES = 0.0, SRM = 0.0) and Pain (ES = 0.2, SRM = 0.2) domains had small effect sizes. CONCLUSIONS: The SAQ is sensitive and responsive to change, as evidenced by the large effect size for both domain and the Total score. The effect sizes are larger than those for any of the SRS domains, including Appearance and Total scores.

Hardware removal after fracture fixation procedures in the femur.

BACKGROUND: The purpose of this study was to conduct an examination of internal fixation of femoral fractures in a large national database. The study aims were to determine the percentage of fixation procedures that result in hardware removal in a single year and to assess differences in the likelihood for hardware removal procedures using patient characteristics. METHODS: The 2007 Nationwide Inpatient Sample was used to quantify all patients who received an open reduction and internal fixation procedure for a fracture of the femur and all patients with a removal of implanted devices from the femur. The differences in patient characteristics between both groups were assessed using statistical methods. RESULTS: Internal fixation of the femur was reported in 30,943 patients. Hardware removals were reported in 4,886 patients. The removal rate for the year was estimated to be 15.8%. Treatment failure was most often because of mechanical complications (18.7%), osteoarthritis (14.3%), nonunion (13.9%), refracture (10.9%), and other implant-oriented complications (10.1%). Males and younger patients composed a significantly higher percentage of removal procedures than fixation procedures (p < 0.0001 for both). Removal rates were lower in Self-Pay and Medicare patients, while the opposite was true for Medicaid and private insurance/HMO patients (p < 0.0001). CONCLUSION: The results of this study suggest that gender, age, and insurance status may influence the likelihood of an implant removal procedure. Given that removal was more likely in males and younger patients, and most often because of mechanical and implant-oriented complications, patient activity and weight bearing are likely leading factors in implant removal.

Spinal appearance questionnaire: factor analysis, scoring, reliability, and validity testing.

STUDY DESIGN: Cross sectional. OBJECTIVE: This study presents the factor analysis of the Spinal Appearance Questionnaire (SAQ) and its psychometric properties. SUMMARY OF BACKGROUND DATA: Although the SAQ has been administered to a large sample of patients with adolescent idiopathic scoliosis (AIS) treated surgically, its psychometric properties have not been fully evaluated. This study presents the factor analysis and scoring of the SAQ and evaluates its psychometric properties. METHODS: The SAQ and the Scoliosis Research Society-22 (SRS-22) were administered to AIS patients who were being observed, braced or scheduled for surgery. Standard demographic data and radiographic measures including Lenke type and curve magnitude were also collected. RESULTS: Of the 1802 patients, 83% were female; with a mean age of 14.8 years and mean initial Cobb angle of 55.8° (range, 0°-123°). From the 32 items of the SAQ, 15 loaded on two factors with consistent and significant correlations across all Lenke types. There is an Appearance (items 1-10) and an Expectations factor (items 12-15). Responses are summed giving a range of 5 to 50 for the Appearance domain and 5 to 20 for the Expectations domain. The Cronbach's α was 0.88 for both domains and Total score with a test-retest reliability of 0.81 for Appearance and 0.91 for Expectations. Correlations with major curve magnitude were higher for the SAQ Appearance and SAQ Total scores compared to correlations between the SRS Appearance and SRS Total scores. The SAQ and SRS-22 Scores were statistically significantly different in patients who were scheduled for surgery compared to those who were observed or braced. CONCLUSION: The SAQ is a valid measure of self-image in patients with AIS with greater correlation to curve magnitude than SRS Appearance and Total score. It also discriminates between patients who require surgery from those who do not.

Polymorphism in the angiotensin II type 1 receptor (AGTR1) is associated with age at diagnosis in pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare, lethal disease associated with single gene disorders, connective tissue disease, exposures to anorexigens, and often, idiopathic etiology. Genes can modify the risk of PAH: (1) monogenic disorders associated with PAH are incompletely penetrant, and (2) not all patients with associated conditions at increased risk for PAH develop the disease. The renin angiotensin aldosterone system (RAAS) provides a set of candidate genes that could modulate pulmonary vascular disease similar to its effects on renal and peripheral vasculature. METHODS: We studied 247 patients with PAH, comprising 177 with idiopathic PAH (IPAH), 63 with PAH/connective tissue disease (CTD), and 7 with PAH associated with anorexigens. Patients were genotyped for 5 common polymorphisms in angiotensinogen (AGT), angiotensin-converting enzyme (ACE), cardiac chymase A (CMA1), angiotensin II type 1 receptor (AGTR1), and aldosterone synthase (CYP11B2). Genotypes were tested for associations with age at diagnosis, hemodynamic parameters at diagnosis, and/or response to acute pulmonary vasodilator testing at diagnosis. RESULTS: Associations were demonstrated for AGTR1 and age at diagnosis in IPAH (p = 0.005). Homozygotes for the 1166C allele (n = 13) were associated with an age at diagnosis 26 years later than those with A/A (n = 139) or A/C (n = 90) genotypes. No associations were demonstrated for AGT, ACE, CMA1, or CYP11B2. CONCLUSIONS: The 1166C polymorphism in AGTR1 appears to be associated with a later age at diagnosis in IPAH, suggesting that this pathway could be involved in the biologic variability that is known to occur in PAH.

Clinical implications of determining BMPR2 mutation status in a large cohort of children and adults with pulmonary arterial hypertension.

BACKGROUND: Bone morphogenetic protein receptor type 2 (BMPR2) mutations occur in idiopathic and familial pulmonary arterial hypertension (IPAH, FPAH); however, the impact of these mutations on clinical assessment and disease severity remains unclear. We investigated the role of BMPR2 mutations on acute vasoreactivity and disease severity in IPAH/FPAH children and adults. METHODS: BMPR2 mutation types were determined in 147 IPAH/FPAH patients. Hemodynamics were obtained at baseline and with acute vasodilator testing. RESULTS: Of 147 patients (69 adults, 78 children; 114 with IPAH, 33 with FPAH), 124 (84%) were BMPR2 mutation-negative, and 23 (16%) were mutation-positive. BMPR2 mutation-positive patients were less likely to respond to acute vasodilator testing than mutation-negative patients (4% vs 33%; p < 0.003; n = 147). BMPR2 mutation-positive children also appeared less likely to respond to acute vasodilator testing than mutation-negative children. BMPR2-positive patients had lower mixed venous saturation (57 +/- 9% vs 62 +/- 10%; p < 0.05) and cardiac index (CI; 2.0 +/- 1.1 vs 2.4 +/- 1.5 liters/min; p < 0.05) than BMPR2-negative patients. CONCLUSIONS: Patients with BMPR2 mutations are less likely to respond to acute vasodilator testing than mutation-negative patients and appear to have more severe disease at diagnosis. Determination of BMPR2 mutations appears to help identify IPAH/FPAH children and adults who are unlikely to respond to acute vasodilator testing and, thus, unlikely to benefit from calcium channel blockade (CCB) treatment.

The Timed Up and Go test: predicting falls in ALS.

There are few functionally meaningful clinical measures used to guide management of patients with ALS. Falls are common, can be debilitating, and result in increased health care costs. We assessed the performance and ability to predict falls of the Timed Up and Go (TUG) test, which quantifies walking ability, in a prospective longitudinal study. Thirty-one patients underwent six monthly TUG, ALSFRS-R, forced vital capacity, muscle testing (MMT) and quality of life assessments. Linear and generalized linear mixed effects models assessed the associations among variables and ability to predict falls. The increase in TUG time was linear over six months, and TUG time was negatively associated with ALSFRS-R (p< or =0.001) and MMT scores (p< or =0.001). The TUG test was the only variable that was associated with the chance of falling (p = 0.024); patients with TUG times of 14 s had a 10% chance of falling during the study. In conclusion, TUG performance declined linearly in this longitudinal study, was correlated with standard outcome measures, and predicted falls. The TUG test can guide management of patients with ALS; a time of 14 s can be used to prompt the recommendation for mobility aids to prevent falls.

Utility in clinical practice of immunoglobulin a anti-tissue transglutaminase antibody for the diagnosis of celiac disease.

BACKGROUND & AIMS: The diagnosis of celiac disease often relies on the anti-tissue transglutaminase (tTG) antibody test. The aim of this study was to evaluate its sensitivity and specificity in clinical practice with the use of commercial laboratories, in which the test characteristics might differ from research laboratories. METHODS: We identified 122 patients with suspected celiac disease who had anti-tTG antibody serologies as well as upper endoscopy with duodenal biopsies. Those with celiac disease were classified as either classic (with diarrhea or other symptoms of malabsorption) or silent (asymptomatic). Biopsies from celiac disease patients were classified as either partial (Marsh IIIA) or total (Marsh IIIB or IIIC) villous atrophy. RESULTS: The overall sensitivity, specificity, and positive and negative predictive values of the anti-tTG antibody test were 70.6%, 65.0%, 91.1%, and 30.2%, respectively. The sensitivity was 90.0% for patients with total villous atrophy and 42.3% for patients with partial villous atrophy (P < .0001). There were differences in both sensitivity and specificity between the 2 most commonly used commercial laboratories. The sensitivity for Lab #1 was 40.0% versus 86.4% for Lab #2 (P < .0001). The specificity for Lab #1 was 100.0%, and it was 41.7% for Lab #2 (P = .02). CONCLUSIONS: The sensitivity of the anti-tTG antibody in clinical practice is not as high as previously reported in research laboratories. The sensitivity is significantly lower in patients with partial villous atrophy. There is also significant variability in test characteristics among major commercial laboratories in the United States. These results need to be confirmed in prospective studies.

The value of wireless capsule endoscopy in patients with complicated celiac disease.

BACKGROUND: Celiac disease may be complicated by symptoms that raise the suspicion of small-intestinal malignancy. The objective is to evaluate wireless capsule endoscopy (WCE) in complicated celiac disease. METHODS: This is a prospective study at a university referral center. There were 47 patients. The indications for WCE were abdominal pain (57%), cancer surveillance (23%), blood in the stool, or persistent iron deficiency (19%). RESULTS: Findings were consistent with celiac disease in 87%: atrophy (68%), fissuring (62%), and mosaic pattern (19%), extending to the ileum in 34%. Unexpected findings were ulceration in 45% (n = 21), cancer (1), polyps (1), stricture (1), submucosal mass (1), ulcerated nodular mucosa (2), and intussusception (1) were seen in 60%. CONCLUSIONS: WCE has a high yield in complicated celiac disease, by identifying mucosal abnormalities and by excluding adenocarcinoma.

Newborn hearing screening in the NICU: profile of failed auditory brainstem response/passed otoacoustic emission.

OBJECTIVE: Incidence of a specific pattern of auditory responses, absent auditory brainstem responses (ABRs) and present otoacoustic emissions (OAEs), in newborn hearing screening in a regional perinatal center neonatal intensive care unit (NICU) is described. This profile, labeled auditory neuropathy or auditory dyssynchrony (AN/AD), is a dysfunction in neural/brainstem transmission that occurs in individuals whose outer hairs cells are functioning normally. Although the AN/AD profile has been associated with various risk factors, incidence and prediction are unknown. METHOD: Analysis of electrophysiologic measures and medical record reviews of the first 22 months of the universal newborn hearing-screening program was conducted. Association of the AN/AD profile was evaluated with the following factors: gender, gestational age, ototoxic drug regimen, low birth weight, hyperbilirubinemia, hydrocephalus, low Apgar score, anoxia, respiratory distress syndrome, pulmonary hypertension, intraventricular hemorrhage, multiple birth, seizure activity, and family history. RESULTS: One hundred fifteen (24.1%) of the 477 infants failed the ABR in 1 or both ears and passed OAEs bilaterally. Comparisons of infants fitting the AN/AD profile with those not fitting the AN/AD profile were negative with 3 exceptions: those with hyperbilirubinemia and those who were administered vancomycin or furosemide. A logistic-regression analysis model failed to predict which infants would be at risk for the AN/AD profile either unilaterally or bilaterally. CONCLUSIONS: Screening of NICU infants should be conducted with ABR first, followed by OAE after failure on ABR. Because the incidence of the AN/AD profile was found to be 24% in this at-risk population, additional study is warranted.

Changing presentation of adult celiac disease.

The mode of presentation of celiac disease in the United States is not known. We investigated the demographic and clinical features of 227 patients with biopsy-proven celiac disease and determined if there had been changes over time. The patients had been entered into a database; those seen prior to 1990 were retrospectively entered while those seen subsequently were prospectively entered. A "symptomatic" presentation described the "classical" presentation of celiac disease with prominent gastrointestinal symptoms: diarrhea and weight loss. Females were younger and had a longer duration of symptoms compared to males. The modes of presentation were symptomatic (62%), anemia or reduced bone density (15%), screening first-degree relatives (13%), and incidental diagnosis at endoscopy (8%). We compared those diagnosed before and after 1993 (when serologic testing was first used), and noted a reduction in those presenting with diarrhea, 73% vs 43% (P = 0.0001) and a reduction in the duration of symptoms, from 9.0 +/- 1.1 years to 4.4 +/- 0.6 years (P < 0001). In conclusion, the percentage of celiac disease patients presenting with diarrhea has decreased, probably related to the more widespread use of serologic testing for celiac disease.

Seronegative celiac disease: increased prevalence with lesser degrees of villous atrophy.

Our aim was to assess differences in the sensitivities of serologic tests used for the diagnosis of celiac disease among patients with varying degrees of villous atrophy. Among 115 adults with biopsy-proven celiac disease who fulfilled strict criteria, including serologic testing at the time of diagnosis and response to a gluten-free diet, 71% had total villous atrophy and 29% partial villous atrophy. Endomysial antibody was positive in 77% of those with total villous atrophy, compared to 33% with partial villous atrophy (P < 0.001). There was no difference in sensitivity when the type of presentation (classical vs. silent) was compared. Endomysial antibody-positive and negative patients did not differ with respect to age at diagnosis, duration of symptoms, mode of presentation, or family history of celiac disease. All anti-tissue transglutaminase-positive patients had TVA on biopsy. Seronegative celiac disease occurs. Endomysial antibody positivity correlates with more severe villous atrophy and not mode of presentation of celiac disease. Serologic tests, in clinical practice, lack the sensitivity reported in the literature.