Ulnar Length Research Articles

Metrical parameters for approximation of height from superior extremity long bones in humans

This study entitled “Metrical parameters for approximation of height from superior extremity long bones in humans” was worked out in the Anatomy Department, GMC, Aurangabad. Students belonging to second year of M.B.B.S, B.D.S, and B.sc Nursing with other non-medico members of the department were included in the study. The age range of the study subjects was between 20 to 50 years including both males and females totaling 500 in number. The study focused on devising a linear regression equivalence to estimate height from the length of arm and forearm bones (viz. humerus, radius and ulna). The stature or height was measured with the help of an anthropometer from crown to heel, length of humerus from humeral head to the farthest point on trochlea, length of ulna from the tip of olecranon process to the distal point of its styloid process, length of radius from the most proximal margin of the head to the distal point of its styloid process. All these measurements were taken with the help of spreading calipers. Correlation coefficient (r) for length of long bones with stature was 0.565 for male humerus, 0.4699 for female humerus, 0.5434 for male radius, 0.4987 for female radius, 0.6398 for male ulna and 0.5637 for female ulna. ‘t’ test applied for testing the statistical significance of the obtained values was found to be significant. The regression equations for approximation of stature were formulated using length of humerus, radius and ulna. Keywords: Stature estimation, Humerus, Radius, Ulna.

Accurate Tidal Volume for Patients under Mechanical Ventilation: A Cross-sectional Descriptive Study

Background: In order to prevent lung injury among patients under mechanical ventilation, tidal volume should be determined based on predicted body weight. The aim of the study was to determine the accuracy of tidal volume determination for patients under mechanical ventilation and to assess nurses’ knowledge about accurate tidal volume determination. Methods: This was a cross-sectional descriptive study. This study was conducted on 250 patients under mechanical ventilation and 75 nurses who provided care to the patients. Patients’ height was estimated based on their ulna length and then, their predicted body weight and tidal volume were estimated. Nurses’ knowledge about tidal volume determination was also assessed. Results: The mean of delivered tidal volume was 9.1±1.73 mL/kg of predicted body weight. Tidal volume for 172 patients (68.8%) had been set at more than 8 mL/kg of predicted body weight. Forty nine nurses (65.3%) noted that there was no guideline in their wards for height and weight measurement. They determined patients’ weight and height through either visual estimation (21 nurses; 28.0%) or asking from their colleagues, patients, or patients’ family members (48 nurses; 64.0%). Conclusion: Nurses have limited knowledge about accurate tidal volume determination and hence, deliver high tidal volume to patients under mechanical ventilation which puts them at risk for ventilator-associated lung injury. Urgent interventions such as lung-protective strategies, staff training, and careful managerial supervision are needed to prevent ventilator-associated lung injury and improve patient safety.

The pediatric proximal ulna: a radiographic study.

The proximal ulna has been comprehensively described in the anatomic literature and imaging studies. However, to the best of our knowledge, the anatomy of the proximal ulna in children has not been fully described in the literature. The present study was conducted on 189 children aged between 0 and 12years (4.7 ± 2.7) by analysis of lateral X-rays of the forearm. Proximal ulna dorsal angulation (PUDA), tip-to-apex (TTA), and total ulnar length (TUL) were measured. The correlation between age and the various X-ray measurements was recorded and then compared with gender differences and adult measurements. Three orthopedic surgeons independently examined the X-ray films and confirmed the reliability of the original observations through intra-group correlation coefficients. There was a strong positive correlation between age and TUL (r = 0.834), and a moderately negative correlation between age and PUDA (r = -0.405). No significant differences were observed between different genders (p > 0.05). Compared with adults, all measurements were smaller except for PUDA, all such measurements statistically significantly different between children and adults (p < 0.05). Interobserver and intraobserver reliability were "very good" for TUL (0.81-1.00), and "good" for PUDA, TTA, and TTA% (0.61-0.80). Good understanding of the anatomy of the pediatric proximal ulna will help to increase the knowledge base in pediatric orthopedic surgeons allowing them to provide improved treatment of fractures. Restoration of the correct forearm anatomy should result in superior clinical and functional results.

Stature Estimation Using Ulnar Length and Shoulder Elbow Length in a Bangladeshi Population

In forensic science, the stature estimation from different parts of the human body, particularly in personal identification, has gained considerable importance due to mass disasters like cyclones, earthquakes, mass accidents, terror attacks etc. Moreover, clinically stature estimation is also essential to assess physical growth, nutrition, and calculating body surface area. Therefore, the present study was intended to develop the standard formula to evaluate the stature of a person using ulnar length and shoulder elbow length in Bangladeshi adult population. The study was carried out on arbitrarily selected 300 healthy adult individuals (150 males and 150 females). At first, several statistical tests were applied to analyze the collected data. Then, sex-specific simple linear regression, stepwise regression, and multiple regression models were applied to develop the models for estimating the stature based on shoulder elbow length and ulnar length. The results showed the insignificant bilateral differences (p > 0.05) of the ulnar length and shoulder elbow length measurements in both sexes. The coefficients of correlation between stature and all measurements were significantly positive (p < 0.001). In this study, the ulnar length showed a better relationship with stature than shoulder elbow length in both sexes with a lower value SEE and higher values of R and R2. Also, the multiple regression models gave better results than the simple and stepwise regression models due to higher R values and lower SEE. This study concludes that the ulnar length and the shoulder elbow length are significant parameters to estimate the stature in Bangladeshi adult’s population.

Estimation of Stature using Ulnar Length in a Population of Chennai

Estimation of stature plays a major role in the field of Forensic Medicine and anthropometry especially helpsin establishing the identity of an individual from mutilated, decomposed and amputated body parts. In ourstudy, stature was estimated from the percutaneous length of ulna. Study sample consisted of 96 adult malesand 66 adult females between the age group of 18 - 22 years, born and brought up in Chennai. The length ofthe of the right and left ulna was measured with a vernier caliper from the tip of the olecranon to the styloidprocess and the height of the individual was measured with a calibrated stadiometer. The measurementswere noted in the proforma. The data was statistically analyzed and linear regression formulae were derived.

A cadaveric morphometric analysis of coracoid process with reference to the Latarjet procedure using the "congruent arc technique".

Congruent arc Latarjet procedure involves rotating the coracoid process so that its inferior surface is flush with the glenoid face, owing to their matching radius of curvature (ROC). However, there has been no cadaveric study to actually measure and compare the ROC of coracoid with glenoid, especially in Indian population. 44 shoulders were dissected in 24 cadavers to measure usable length of coracoid process, width, height, ROC of coracoid and glenoid as well as ulnar length (as proxy of cadaver height). Critical coracoid height and length were estimated based on screw sizes of 2.7mm, 3.5mm, 4mm and 4.5mm, and pair concordance between height and length calculated. ROC of coracoid and glenoid were compared to measure extent of congruency. The mean usable length of coracoid process, width and height at mid-point were 21.8mm, 13.7mm and 8.6mm, respectively. Out of the different screw sizes, 2.7mm screws were found safe in 82% shoulders. 24 coracoid-glenoid pairs fulfilled the operational definition (≤ 5mm) of congruency while rest 20 were seemingly incongruent chiefly due to coracoid variations, with mean ROC difference 4.13mm (95% CI 1.51-6.74mm). The ulnar length was significantly smaller in the incongruent ROC group (p = 0.0002). The available length as well as height of the transferred coracoid must be considered when deciding optimum diameter fixation screws in Latarjet procedure. Owing to smaller anatomic dimensions of coracoid in Indian population, 2.7-mm screws provide the safest fixation option. Also, the ROC of coracoid and glenoid does not match in substantial proportion of the cadavers. Pre-operative planning should include a CT-based assessment of glenoid and coracoid dimensions to decide the technique of Latarjet procedure and the optimum diameter fixation screws required.

Ulnar length – A Tool for Stature Estimation

Stature is a important factor in identification of an unidentified dead body or any mutilated part of such body. In present study we made an attempt to estimate the stature from percutaneous length of ulna. Study consists of 94 adult males and 96 adult females between the age group of 18- 24 years, born and brought up in Maharashtra. The length of the of the right and left ulna was measured with a venier calliper from the apex of the olecranon to the styloid process and the height of the individual was measured with a calibrated standing meter rule. The measurements were noted in the proforma. The data was statistically analyzed and linear regression formulae were derived.

Estimating the Height of An Individual from The Length of Ulna in Undergraduate Students of a Nepalese Medical College

Introduction: Estimation of height from bones plays an important role in identifying unknown bodies, parts of bodies, or skeletal remains. Multiple anthropometric techniques have been used to estimate stature from skeletal remains by anthropologists, anatomists, and forensic experts. The ulna is a long bone often used for body height estimation, as it is mostly subcutaneous throughout its length and is easily approachable for measurement. Methods: The present study was carried out on 100 (57 male and 43 female) undergraduate students of a medical college of the age group of 18 to 24 years. The parameters studied were height, length of right, and left ulna. The observations were analyzed by Pearson’s correlation to examine the relationship between the length of ulna and height. Results: The mean height of males was 174.54 ± 13.32 cm and of females was 156.01±11.19 cm. The mean length of the right ulna was 27.36 ± 2.12 cm (males) and 24.35±1.97 cm (females). The mean length of the left ulna was 27.29 ± 2.13 cm (males) and 24.06 ± 2.18 cm (females). Pearson’s correlation showed a positive and statistically significant (p&lt;0.001) relation between the length of the ulna and the height. The regression equation was derived to estimate the height of an individual from the length of the ulna. Conclusion: The ulna bone length is an accurate parameter that can be used in estimating an individual's height. The regression equation derived in this study can be of great help to anatomists, clinicians, anthropologists, and forensic scientists.

A new species of Omeisaurus (Dinosauria: Sauropoda) from the Middle Jurassic of Yunyang, Chongqing, China

ABSTRACT A new species of Omeisaurus, O. puxiani sp. nov., from the Middle Jurassic of Southwest China is reported. The holotype consists of partial articulated vertebrae, forelimb, hind limb and other materials of an individual. Comparative study and cladistic analysis show that this new taxon belongs to Omeisaurus, and bears a unique combination of character states, such as all presacral vertebrae opisthocoelous, complex pneumatic fossa system in middle cervical centra, lateral fossae of dorsal centra divided by secondary septa, end of anterior caudal neural spines expanding posteriorly, edge of humerus deltopectoral crest turning posterolaterally and ratio of length of ulna to humerus of 0.69. The previously reported single middle cervical (S66) from the same locality and horizon can be referred to this new species. The discovery of Omeisaurus puxiani enriches the diversity of the genus of Omeisaurus and provides additional information to help understanding the evolutionary history of this genus in Eastern China. Abbreviations No. 208 HEGT: Chongqing Laboratory of Geological Heritage Protection and Research, No. 208 Hydrogeological and Engineering Geological Team, Chongqing Bureau of Geological and Mineral Resource Exploration and Development Chongqing, Chongqing, China; GSC: Chongqing Institute of Geological Survey, Chongqing, China; IVPP: Institute of Vertebrate Paleontology and Paleoanthropology, Chinese Academy of Sciences, Beijing, China; ZDM: Zigong Dinosaur Museum, Zigong, Sichuan, China

Forearm Deformity and Radial Head Dislocation in Pediatric Patients with Hereditary Multiple Exostoses: A Prospective Study Using Proportional Ulnar Length as a Scale to Lengthen the Shortened Ulna.

Ulnar lengthening is a consensus treatment for hereditary multiple exostoses with radial head dislocation in pediatric patients; however, the optimal amount of ulnar lengthening remains unclear. It is necessary to look for a parameter to decide the amount of ulnar lengthening that will avoid recurrence and complications. The purpose of the present study was to confirm that proportional ulnar length (ulnar length/radial length) can be used as a scale for ulnar lengthening in pediatric patients. The normal lengths of the ulna and radius in the pediatric population were measured in different age groups. The proportional ulnar length was calculated as ulnar length/radial length in each group. Thirty forearms in 26 patients with radial head dislocation were treated with ulnar lengthening and deformity correction. The goal of ulnar lengthening was to recover the normal proportional ulnar length. The function of the forearm was evaluated at the time of the latest follow-up. The preoperative and postoperative values for radial bowing, radioarticular angle, ulnar variance, and carpal slip were also compared. The value of proportional ulnar length in the normal population consistently averaged approximately 1.1. In all cases, proportional ulnar length recovered to the normal value of 1.1. Reduction of the dislocated radial head was achieved in 28 forearms (93%); in 16 of these forearms, good-quality reduction was achieved and no recurrence of radial head dislocation was observed during follow-up. The function of the forearm improved markedly (p < 0.001). Ulnar variance improved from 2.51 to -0.79 cm (p < 0.001). Radial deformities improved according to measurements of radial bowing and the radioarticular angle (p < 0.001). All parents were satisfied with the postoperative appearance and function of the forearm. Proportional ulnar length could be used as a scale to decide the amount of ulnar lengthening for radial head dislocation in pediatric patients with hereditary multiple exostoses. Ulnar lengthening according to proportional ulnar length and deformity correction can prevent recurrence of ulnar variance and avoid impingement of the wrist. Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Evaluation of Vibration Analysis to Assess Bone Mineral Density in Children

The effectiveness of vibration analysis to assess bone mineral density (BMD) in children with suspected reduction in bone density was studied. A system was designed that measured the ulna's vibration responses in vivo. The system was evaluated on the ulnae of 48 children (mean age=12.0, std=3.5 years), 31 of whom had been confirmed to have osteogenesis imperfecta (OI). All ch ildren had dual energy X-ray absorptiometry (DXA) scan as part of their routine clinical care and vibration analysis was pe rformed on t he same day. Frequency spectra of the ulnae's vibration responses were obtained and processed by principal component analysis. Four main principal components were selected and together with age, sex and right hand ulna's length were used in a regression analysis to estimate BMD. Regression analysis was repeated using the children's leave-one-out and partitioni ng methods. The percentage similarity and correlation between the DXA-derived and vibration analysis estimated BMDs using the leave-one-out were 80.34% and 0.59 and for partitioning were 74.2% and 0.64 respectively. There was correlation between vibration analysis BMD readings a nd those derived from DXA however a larger study will be needed to better establish the extent to which vibration analysis can assist in assessing bone density in clinical environments.

Wrist Joint Skeletal Changes in Children With Transfusion-dependent Thalassemia.

Arthropathies and bone deformities are well known to occur in patients with thalassemia major and have been attributed to the disease or to its therapy. Before the advent of chelation therapy, these children developed widened diploic space and "hair-on-end" pattern in skull, "cobweb" pattern in the pelvis, and the lack of the normal concave outline in the long bones because of extensive marrow proliferation. After the introduction of iron-chelation therapy, these patients were noted to develop metaphyseal abnormalities and vertebral changes resembling spondylo-metaphyseal dysplasia. Only one study has shown some association of deferiprone (chelating agent) use with distal ulnar changes in these children. Our study was done to describe the skeletal changes and deformities in wrist joints of children with transfusion-dependent thalassemia and correlate them with age, mean pretransfusion hemoglobin level, mean serum ferritin level, and type and duration of chelation therapy in these children. A total of 60 children with transfusion-dependent thalassemia from the thalassemia daycare center were examined. These children were divided into 3 groups on the basis of their age (group A: 2 to 6 y, group B: 6 to 10 y, and group C: 10 to 14 y). Detailed history, including treatment history, number of blood transfusions received over the last 1 year, clinical examination, and radiologic assessment of both forearm with wrists were done. The clinical and radiologic differences in radial and ulnar lengths increased significantly with the increasing age of these patients, the ulna being short. There was some correlation between increasing negative ulnar variance and distal radial articular angle with deferiprone consumption. Chelation therapy, particularly with deferiprone, may cause distal ulnar growth arrest causing ulnar shortening and progressive radial bowing in these children. Level IV-case series.

Estimation of Body Stature Using the Percutaneous Length of Ulna of an Individual

ObjectiveTo explore the association between the percutaneous length of ulna and height of that individual and to formulate a gender-specific formula.MethodsThis is a cross-sectional study conducted at the Nishtar Medical University, Multan from May 1 2019 to July 1 2019. Length of ulna and body height were measured for 100 participants, including an equal number of males and females. Means of age, length of ulna and body height were compared between both genders. Regression coefficient, Pearson correlation coefficient and constant were calculated and regression formulae were formed for calculating height from length of the ulna for males, females, and the whole study group, separately. Data analysis was done using Statistical Package for the Social Sciences; version 23 (SPSS Inc., Chicago, IL); p≤0.05 was considered statistically significant.ResultsPearson correlation coefficient, regression coefficient, constant were statistically significant for males, females, and the whole study group (p<0.001). Regression equations that were devised after analyzing the data to estimate the stature from ulna length are as follows: for the whole study group: 42.830+4.671 (length of ulna); for males: 70.369+3.698 (length of ulna); for females: 18.562+5.617 (length of ulna).ConclusionThe length of ulna provides a reliable and relatively accurate means for estimating an individual’s height. Regression formulae devised will be of practical use for forensic scientists, anthropologists, archeologists, clinicians and anatomists for estimating the stature from the length of the ulna.

Prediction of height using arm span and ulna length among malaysian adolescents

Prediction of height using arm span and ulna length among malaysian adolescents

Multiple Hereditary Exostosis of Distal Ulna with Dislocation of Radial Head: A Case Report

Background: Multiple hereditary exostosis of the forearm is typically formed in the distal ulna causing disturbances in the growth of the ulna and functional disability. Multiple hereditary exo­stosis inhibits the growth of the ulna leading to an acquisition of a varus deformity in the ra­dius which sometimes leads to dislocation of the radial head, the development of limitations in the pronation-supination of the forearm, and cos­me­tic problems. This study aimed to report the mul­ti­ple hereditary exostosis of distal ulna with dis­location of radial head. Case Presentation: We present a case of a 14 year-old girl with main complaint of a bony prominence on her right elbow which limits the elbow flexion range of motion. 3D CT-Scan re­vealed a dislocation of radial head, shortening and posterior bowing of ulna. We performed excision of the prominent radial head, recons­truction, and temporary arthrodesis of the elbow. Results: The forearm deformity in patients with multiple hereditary exostosis related to the cross-sectional area of the distal ulnar physis was only one-quarter of the distal radius, the distal ulna is more involved in the condition. There was more longitudinal growth at the distal ulnar physis than at the distal radial physis, the distal ulnar physis contributed more total Ulnar Length than the distal radial physis did to Radial length. Known surgical interventions including simple excision, ulnar lengthening, corrective radial os­teo­­tomy, hemi-epiphyseal stapling of the distal radius, the Sauve-Kapandji procedure. Conclusion: Simple excision could improve the range of movement of the forearm but would not halt the progression of disease, particularly in younger patients. It was not effective in con­troll­ing progression of the deformity. Mature patients did not recur, but in patients that had been excis­ed before puberty the results were varied and unpredictable. Keywords: multiple hereditary exostosis, radial head dislocation, excision Correspondence: Pamudji Utomo. Department of Orthopaedics and Traumatology Prof. Dr. R.Soeharso Ortho­paedics Hospital, Surakarta. Email: utomodr­@yahoo.com. Indonesian Journal of Medicine (2020), 05(01): 63-69 https://doi.org/10.26911/theijmed.2020.05.01.10

What Is the Normal Ulnar Bow in Adult Patients?

Rotation of the forearm is a result of the complex interaction among the radius, ulna, and interosseous membrane. Although the radius is recognized as curved, the ulna is generally thought of as a "straight bone." To better describe normal anatomy, which may lead to more successful anatomic fixation of forearm fractures, we aimed to apply a method of measuring the normal ulnar bow and determine the mean ulnar bow in adults. (1) To what degree is the ulna bowed in the coronal and sagittal planes in normal adult forearms? (2) To what degree is the radius bowed in the coronal plane in normal adult forearms? Radiographs of the forearms of adults taken during a 1-year period were initially obtained retrospectively. These radiographs were performed for various reasons, including forearm pain and routine radiographic follow-up. Radiographs were excluded if evidence of a fracture or post-fracture fixation was found, if a patient had missing AP or lateral images, or if a suboptimal technique was used. The coronal and sagittal bow of the ulna was measured with a method adapted from previous studies that assessed radial bow using AP and lateral radiographs, respectively. Similar measurements were made in the coronal plane for the radius. All measurements were performed independently by the four authors. There was excellent interobserver reliability for ulnar bow in the coronal and sagittal planes (interclass correlation coefficient = 0.96 and 0.97, respectively) and for radial bow in the coronal plane (interclass correlation coefficient = 0.90). The mean maximal coronal ulnar bow was 7 ± 2 mm and was located at 75% of the ulnar length, measured proximally to distally. The location of coronal bow was consistently distal to the radial bow location. The mean maximal sagittal ulnar bow was 6 ± 3 mm and was located at 39% of the ulnar length. The mean maximal coronal bow of the radius was 14 ± 2.0 mm and was 59% of the total length of the radius from proximal to distal. The ulna is not a "straight bone," as is commonly thought, but rather has a bow in both the coronal and sagittal planes. Knowledge of the standard ulnar bow may be pivotal to prevent malunion of the ulna during surgery. Future research using these data in preoperative planning may lead to changes in plate contouring and clinical outcomes in forearm fracture management.

Teratological Effect of Indoxacarb on Chick Embryos (Gallus gallus domesticus)

The present study was designed to assess the teratological effects of Indoxacarb on chick embryos (Gallus gallus domesticus) by administering different doses of commercial Indoxacarb (0.5, 1.0 and 1.5ml/1000ml/egg, in three experimental groups B, C and D) injected into the yolk sac of eggs before incubation. Embryos were recovered at day 12 of incubation. The mortality rate gradually increased from lower to higher doses. A total of twelve morphometric parameters viz., wet body weight, crown rump length, anterior posterior head diameter, eye diameter, beak length, neck length, humerus length, radius and ulna length, metacarpus length, femur length, fibula length and metatarsus length were recorded and compared with control group. These quantitative parameters indicated significant plessthan0.05) decrease in all treated groups compared to control group. While the qualitative anomalies such as microcephaly, hydrocephaly, edematous swelling, hematoma formation, abnormal body coloration, anophthalmia, microphthalmia, deformed beak, agnathia, wry neck, micromelia, amelia, omphalocele and ectopia cardis were also recorded in treated groups as compared to untreated groups. Therefore, it is concluded that Indoxacarb has potentially harmful effects on the development of avian embryos even at very low dose concentration. Hence, due to toxicity of this pesticide it must be used with utmost caution.

The Radioulnar Distance at the Level of the Radial Tuberosity.

Ruptures of the distal biceps brachii tendon are generally treated operatively due to their loss of supination and flexion force. A mechanical impingement at the insertion of the tendon at the radial tuberosity is discussed to play a role in the etiology of this injury. The aim of this study was to present a detailed, three-dimensional anatomical analysis of the radioulnar space at the radial tuberosity. A total of 166 imprints of the radioulnar space in neutral rotation and pronation from 84 cadaveric specimens of both arms using silicone impression material were produced for this study. Imprints were cut in slices of 3 mm and digitally measured after picture acquisition using a high-resolution digital camera. Distances were grouped into a proximal, central, and distal groups and used for correlation to morphometric data at the elbow (radial head diameter, ulna and radius length) as well as volume calculation. The mean radioulnar distance was 8.8 ± 4.0 mm in neutral rotation and 7.8 ± 3.9 mm in pronation. In pronation, the central zone was the smallest whereas in neutral rotation the proximal zone was the smallest. The volume of the radioulnar space did not reduce significantly during pronation. Little space is provided for the insertion of the distal biceps brachii tendon especially during pronation. This could play a role in the etiology of distal biceps brachii tendon ruptures and should be considered in the fixation after rupture of the tendon. Clin. Anat., 33:661-666, 2020. © 2019 Wiley Periodicals, Inc.

Obtaining spirometric reference values when height is not available – comparison of alternative anthropometric measures

IntroductionHeight is one of the variables used to derive reference values for respiratory function but it is not always available. To fulfil this need, different formulas are described in literature. AimTo understand which method best estimate height in order to obtain Forced Vital Capacity (FVC) and Forced Expiratory Volume in 1st second (FEV1) reference values. MethodsThe true stature (TS) and five alternative anthropometric measures that estimate height [arm span using a regression equation (ASR), arm span using a fix correction factor (ASF), half arm span, knee height and ulnar length] were measured for all patients. To assess the differences between the TS versus the estimated heights and the respective reference values of FVC and FEV1, T Student Test and Wilcoxon test were used. Agreement was evaluated by Intraclass Correlation Coefficient and by Bland-Altman plot. ResultsA total of 160 subjects were recruited but 14 patients were excluded due to skeletal deformities. The predicted FVC and FEV1 using all alternative anthropometric measures were statistically different from those calculated by TS, except for ASF. The limits of agreement were wide for all alternative measures. With the exception of ASF, predicted FVC and FEV1 were overestimated when using all other alternative measures. ConclusionsFor the purpose of replacing height in order to obtain predicted values of FVC and FEV1, the ASF was shown to be the most accurate and recommendable method. However, we must be aware there is a possible degree of error.

EP.82A prospective study of eteplirsen treatment for Duchenne muscular dystrophy in routine clinical practice: study design and methodology

Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder caused by mutations in the DMD gene that encode the protein dystrophin. Lack of dystrophin severely compromises muscle fiber strength, flexibility and stability. Consequently, patients with DMD experience diminished quality of life, disability, and premature death, typically resulting from cardiac or respiratory failure. Eteplirsen was approved by the US FDA in September 2016 for the treatment of DMD in patients with a confirmed mutation of the dystrophin gene amenable to exon 51 skipping. This long-term, prospective, observational study (5-year minimum follow-up) was designed to gather data on patients with DMD receiving eteplirsen, as well as on the clinical course of DMD, in routine clinical practice. A minimum of 200 patients with a confirmed DMD diagnosis will be enrolled. The study will initiate in the US, then expand to other countries. Annual interim analyses of all outcomes of interest will be performed. Efficacy assessments will include loss of ambulation, defined as having a North Star Ambulatory Assessment (NSAA) walk score of 0 and the inability to perform the 10-meter walk test; pulmonary function based on % predicted forced vital capacity and height (standing height and ulnar length); neuromuscular function assessments (NSAA, timed function tests, performance of the upper limbs, Brooke Upper Extremity Scale grade, repetitive movement outcomes, and walk pace); activities of daily living; and quality of life as measured by the Pediatric Outcomes Data Collection Instrument. Safety assessments will include monitoring of all adverse events. This presentation will provide specific details regarding study methods, including study design, sample selection, recruitment, and safety and efficacy evaluations. Details regarding the Duchenne Outcomes Research Interchange, a patient- and physician-reported outcomes data warehouse that is planned to launch in 2019, will also be provided.