Using artificial intelligence-based technologies to detect clinically relevant changes of gross motor function in children with cerebral palsy.

AIM: To compare the 66-item Gross Motor Function Measure (GMFM-66) with the reduced version of the GMFM-66 (rGMFM-66) with respect to the detection of clinically relevant changes in gross motor function in children with cerebral palsy (CP). METHOD: The study was a retrospective single centre analysis of children with CP who participated in a rehabilitation programme. Overall, 1352 pairs of GMFM-66 and rGMFM66 measurements with a time interval of 5 to 7 months were available. To measure clinically relevant changes in gross motor function, the individual effect size (iES) was calculated. RESULTS: The study population consisted of 1352 children (539 females), mean age 6 years 4 months (SD 2 years 4 months). The iES based on the GMFM-66 and the rGMFM-66 showed a significant correlation (r = 0.84, p < 0.001). The analysis of the area under the receiver operating characteristic curve showed an excellent agreement for clinically relevant gross motor improvement (Cohen's d ≥ 0.5; area under the curve = 0.90 [95% confidence interval 0.88-0.92]) or deterioration (Cohen's d ≤ -0.5; area under the curve = 0.95 [95% confidence interval 0.92-0.97]). INTERPRETATION: Performing the rGMFM-66 saves time compared to the full GMFM-66. The rGMFM-66 showed good agreement with the GMFM-66 with respect to the detection of clinically relevant changes in gross motor function in children with CP, so its use in everyday clinical practice seems justifiable. WHAT THIS PAPER ADDS: The reduced version of the 66-item Gross Motor Function Measure (rGMFM-66) detects clinically relevant changes in gross motor function in children with cerebral palsy. The rGMFM-66 correlates highly with the full GMFM-66. The rGMFM-66 can be used in clinical practice when the time schedule is limited.

Motor improvement in children with PMM2-CDG syndrome following a six-month rehabilitation treatment utilising whole-body vibration; a retrospective study.

OBJECTIVE: The aim of this study was to assess the effect of a six-month interval rehabilitation treatment on motor function of children with PMM2-CDG syndrome (#212065 Congenital disorder of glycosylation, Type Ia; CDG1A, OMIM catalogue number). METHODS: The concept 'Auf die Beine' (Center for Prevention and Rehabilitation of the University of Cologne, Germany) combines two short inpatient stays (1 to 2 weeks) with a six-month whole-body vibration (WBV) home-training program. 13 patients with PMM2-CDG syndrome participated in this concept from 2006 until 2015. Assessments at start, six months and 12 months (follow-up): Gross Motor Function Measure (GMFM-66), One-Minute Walk Test (1MWT) and instrumented gait analyses. RESULTS: The GMFM-66 (9 of 13 children) improved by 5.3 (mean) points (SD 3.2) at 12 months (p=0.0039). The 1MWT (6 of 13 children) improved by 19.17 meter (SD 16.51) after 12 months (p=0.0313). Gait analysis (9 of 13 children) measured by pathlength/distance ratio improved by -0.8 (SD 1.9) at 12 months (p=0.0195). CONCLUSION: Patients with PMM2-CDG syndrome benefit from the interval rehabilitation program 'Auf die Beine' including WBV.

Effect of Whole-body Vibration frequency on muscle tensile state during graded plantar flexor isometric contractions.

Background: Acute physiological and biomechanical alterations have been reported following whole-body vibration (WBV). Stiffening of muscles has only been anecdotally reported in response to WBV. Accordingly, this study investigated active plantar flexor muscle stiffness in response to a single WBV bout at four mechanical vibration frequencies. Methods: Thirteen healthy adults (37.1 ± 14.4 years old) randomly received WBV in 4 different frequencies (6, 12, 24, and 0 Hz control) for 5 min. Shear wave speed (SWS) in longitudinal and transverse projections, architecture, and electric muscle activity were recorded in the medial gastrocnemius (MG) and soleus (SOL) muscle during graded plantar flexor contraction. Subjective rating of perceived muscle stiffness was assessed via Likert-scale. Results: SWS of the MG at rest was enhanced in response to 5 min of 24 Hz WBV (p = 0.025), while a small reduction in SOL SWS was found during contraction (p = 0.005) in the longitudinal view. Subjective stiffness rating was increased following 12 Hz intervention. After 24 Hz WBV, pennation angle for MG was decreased (p = 0.011) during contraction. As a secondary finding, plantar flexor strength was significantly increased with each visit, which, however, did not affect the study's main outcome because of balanced sequence allocation. Conclusion: SWS effects were solely limited to 24 Hz mechanical vibration and in the longitudinal projection. The observed effects are compatible with an interpretation by post-activation potentiation, warm-up, and force-distribution within the triceps surae muscles following 5 min WBV. The outcome may suggest SWS as a useful tool for assessing acute changes in muscle stiffness.

Artificial intelligence to improve efficiency of administration of gross motor function assessment in children with cerebral palsy.

AIM: To create a reduced version of the 66-item Gross Motor Function Measure (rGMFM-66) using innovative artificial intelligence methods to improve efficiency of administration of the GMFM-66. METHOD: This study was undertaken using information from an existing data set of children with cerebral palsy participating in a rehabilitation programme. Different self-learning approaches (random forest, support vector machine [SVM], and artificial neural network) were evaluated to estimate the GMFM-66 score with the fewest possible test items. Test agreements were evaluated (among other statistics) by intraclass correlation coefficients (ICCs). RESULTS: Overall, 1217 GMFM-66 assessments (509 females, mean age 8y 10mo [SD 3y 9mo]) at a single time and 187 GMFM-66 assessments and reassessments (80 females, mean age 8y 5mo [SD 3y 10mo]) after 1 year were evaluated. The model with SVM predicted the GMFM-66 scores most accurately. The ICCs of the rGMFM-66 and the full GMFM-66 were 0.997 (95% confidence interval [CI] 0.996-0.997) at a single time and 0.993 (95% CI 0.993-0.995) for the evaluation of the change over time. INTERPRETATION: The study shows that the efficiency of the full GMFM-66 assessment can be increased by using machine learning (self-learning algorithms). The presented rGMFM-66 score showed an excellent agreement with the full GMFM-66 score when applied to a single assessment and when evaluating the change over time.

Body Fat Distribution in Children and Adolescents With Cerebral Palsy.

To evaluate the body fat distribution in children with cerebral palsy (CP). The present study focusses on a monocentric retrospective analysis of body fat distribution from children diagnosed with CP. The children participated in a rehabilitation program. Reference centiles were calculated based on data from the National Health and Nutrition Examination Survey (NHANES, 1999-2004). Z-scores for trunk-to-leg fat ratio were calculated. Further, fat mass index (FMI) was evaluated based on percentiles that have already been published. 237 males and 194 females with CP were considered (mean age: 11 years and 11 months [SD 3 years]). These were compared to 1059 males and 796 females from the NHANES (mean age: 14 years and 7 months [SD 3 years and 4 months]). The z-scores for trunk-to-leg fat ratio showed the following values: mean -0.47 (SD 1.50) for males, -0.49 (SD 1.11), for females, -0.48 (SD 1.34) for all. The z-scores for FMI showed the following values: mean -0.29 (SD 0.70) for males, -0.88 (SD 2.0) for females, -0.55 (SD 1.46) for all. The results showed rather a gynoid fat distribution and a lower FMI in children with CP than in the reference population (NHANES 1999-2004).

Pediatric Reference Centiles of Bone Mineral Density and Body Composition of Lower Limbs.

Localized neurological diseases such as spina bifida are often accompanied by normal upper limb and spinal bone mineral density (BMD), whereas regional BMD of the lower limbs may be decreased. Therefore, regional BMD measurements may be more accurate to quantify regional bone health. Until now, no pediatric reference centiles of bone mineral density and body composition of the lower extremities are available for Hologic DXA systems. The objective was to generate age-and sex specific reference centiles of DXA scans of lower limbs for Hologic DXA systems. Data from the National Health and Nutrition Examination Survey of the period 1999-2004 (age 8 - 20 years) were used to generate age-specific and sex-specific reference centiles for the non-Hispanic Black, non-Hispanic White and Mexican-American NHANES study population. The LMS method was used to calculate the reference centiles. Data of DXA scans of 2233 non-Hispanic black children (880 females), 1869 non-Hispanic white children (803 females) and 2350 Mexican American children (925 females) were used to create age-specific and sex-specific reference curves. We presented age-and sex-specific reference centiles for regional bone mineral density, bone mineral content, lean body mass and fat mass at the lower limbs for children and adolescents which were ethnicity specific and directly applicable to Hologic QDR-4500A fan-beam densitometer.

Diagnostic Evaluation of the Functional Muscle-Bone Unit in Children With Cerebral Palsy With and Without Low Trauma Fractures.

Children and adolescents with cerebral palsy (CP) are at increased risk of low trauma fractures (LTF) due to low bone mineral content (BMC). The risk of LTFs might be overestimated by only age - and sex adjusted Z-scores for BMC because Z-score based DXA techniques do not take into account other relevant parameters like height, muscle and fat mass. This study aimed to present an update of the functional muscle-bone unit-algorithm (uFMBU-A) to evaluate bone health in children with CP in order to predict the risk of LTF taking into account the parameters sex, age, height, muscle and fat mass. We performed a monocentric retrospective analysis of 177 DXA-scans of children and adolescents with CP aged 8-19. Six of these 177 patients had sustained at least 1 LTF. Age-, sex- and size adjusted Z-scores of total body less head (TBLH)-BMC, lean body mass and fat mass were calculated. The uFMBU-A was applied to the study group and results were compared with established Z-score based DXA-measurements and algorithm based diagnostic techniques concerning the prediction of LTF risk. The uFMBU-A had the greatest diagnostic odds ratio (13.3 [95% CI 2.41; 72.9]) of the evaluated predictors with a sensitivity of 50.0% (95% CI 11.8; 88.2), specifity of 93% (95% CI 88.1; 96.3). The uFMBU-A was the most accurate method of the evaluated parameters to predict LTF in children with CP and is recommended when evaluating bone health.

Vibration assisted rehabilitation in patients with Pompe disease: A case series.

The results of three cases with infantile-onset Pompe disease participating in a rehabilitation program with home-based vibration training will be presented. In this retrospective observational case study, the cases participated in the neuromuscular training program "Auf die Beine", which combines two blocks of intensive, goal directed training with 6 months of home-based whole body vibration (WBV). Assessments by the means of a dual-energy X-ray absorptiometry and grip strength were applied at multiple points throughout the program. Two cases showed an increase in lean mass index of +0.319 kg/m2, +0.721 kg/m2 and bone mineral content of +0.028 kg/m2, +0.031 kg/m2 over one year. Additionally physiotherapeutic therapy goals could be achieved. In the remaining child lean mass index did not change, bone mineral content decreased by -0.03 kg. The neuromuscular rehabilitation program "Auf die Beine" has shown to be safe and effective in two of three cases for muscle and bone mass gain as well as in achievement of physiotherapeutic goals. To summarize, WBV is an innovative therapy in a rehabilitation concept, which might be helpful in Pompe disease, but further studies with larger cohorts are needed.

Effects of long-term immobilisation on endomysium of the soleus muscle in humans.

NEW FINDINGS: What is the central question of this study? While muscle fibre atrophy in response to immobilisation has been extensively examined, intramuscular connective tissue, particularly endomysium, has been largely neglected: does endomysium content of the soleus muscle increase during bed rest? What is the main finding and its importance? Absolute endomysium content did not change, and previous studies reporting an increase are explicable by muscle fibre atrophy. It must be expected that even a relative connective tissue accumulation will lead to an increase in muscle stiffness. ABSTRACT: Muscle fibres atrophy during conditions of disuse. Whilst animal data suggest an increase in endomysium content with disuse, that information is not available for humans. We hypothesised that endomysium content increases during immobilisation. To test this hypothesis, biopsy samples of the soleus muscle obtained from 21 volunteers who underwent 60 days of bed rest were analysed using immunofluorescence-labelled laminin γ-1 to delineate individual muscle fibres as well as the endomysium space. The endomysium-to-fibre-area ratio (EFAr, as a percentage) was assessed as a measure related to stiffness, and the endomysium-to-fibre-number ratio (EFNr) was calculated to determine whether any increase in EFAr was absolute, or could be attributed to muscle fibre shrinkage. As expected, we found muscle fibre atrophy (P = 0.0031) that amounted to shrinkage by 16.6% (SD 28.2%) on day 55 of bed rest. ENAr increased on day 55 of bed rest (P < 0.001). However, when analysing EFNr, no effect of bed rest was found (P = 0.62). These results demonstrate that an increase in EFAr is likely to be a direct effect of muscle fibre atrophy. Based on the assumption that the total number of muscle fibres remains unchanged during 55 days of bed rest, this implies that the absolute amount of connective tissue in the soleus muscle remained unchanged. The increased relative endomysium content, however, could be functionally related to an increase in muscle stiffness.

Effect of Long-Term Repeated Interval Rehabilitation on the Gross Motor Function Measure in Children with Cerebral Palsy.

BACKGROUND: The efficacy of interventions for cerebral palsy (CP) has been frequently investigated with inconclusive results and motor function measured by the Gross Motor Function Measure (GMFM-66) is common. OBJECTIVE: In this observational analysis, we quantify the GMFM-66 change scores of the second and third year of a multimodal rehabilitation program (interval rehabilitation including home-based, vibration-assisted training) in children with CP. METHODS: The study was a retrospective analysis of children with CP (2-13 years) participating for a second (n = 262) and third year (n = 86) in the rehabilitation program with GMFM-66 scores at start (M0), after 4 months (M4) of intensive training, and after 8 months of follow-up (M12). A method was previously developed to differentiate between possible treatment effects and expected development under standard of care for GMFM-66 scores using Cohen's d effect size (ES; size of difference). RESULTS: After the treatment phase of 4 months (M4) in the second year, 125 of 262 children were responder (ES ≥ 0.2) and 137 children nonresponder (ES < 0.2); mean ES for nonresponder was -0.212 (trivial) and for responder 0.836 (large). After M4 in the third year, 43 children of 86 were responder (ES = 0.881 [large]) and 43 nonresponder (ES = -0.124 [trivial]). DISCUSSION AND CONCLUSION: Repeated rehabilitation shows a large additional treatment effect to standard of care in 50% of children which is likely due to the intervention, because in the follow-up period (standard of care), no additional treatment effect was observed and the children followed their expected development.

Reference Centiles for the Evaluation of Nutritional Status in Children using Body Fat Percentage, Fat Mass and Lean Body Mass Index.

BACKGROUND: Body fat percentage (BF%), fat mass index (FMI), and lean body mass index (LBMI) are often used to evaluate the nutritional status of children. Until now, no pediatric FMI reference centiles are applicable for GE Healthcare Lunar DXA systems. The aim of the study was to generate age-specific BF%, FMI, and LBMI references centiles for GE Healthcare Lunar DXA systems. METHODOLOGY: Published values from the National Health and Nutrition Examination Survey 1999-2004 (age 8-20 years) were used to generate the reference centiles for the non-Hispanic black, non-Hispanic white, and Mexican American NHANES population. The LMS and LMSP methods were used to generate the reference centiles. RESULTS: Data of 2433 non-Hispanic black children (972 females), 2026 non-Hispanic white children (873 females), and 2547 Mexican American children (1010 females) were eligible. CONCLUSIONS: We presented age-specific reference centiles for BF%, FMI, and LBMI for children and adolescents which were ethnicity specific (non-Hispanic black, non-Hispanic white, and Mexican American) and directly applicable to Prodigy and iDXA GE Healthcare Lunar systems with software version 14.0. We proposed the use of BF%, FMI, and LBMI together to evaluate nutritional status in children.

Reliability of a radiation-free, noninvasive and computer-assisted assessment of the spine in children with cerebral palsy.

PURPOSE: The radiation-free, noninvasive and computer-assisted Spinal Mouse® (SM) is a reliable and valid measuring instrument for functional analysis of the pediatric spine. The aim of this study was to examine the intra-rater reliability of the SM measurements in children with cerebral palsy (CP) and to investigate differences after a 1 week of the rehabilitation program. METHODS: A total of 168 SM investigations in the sagittal plane and frontal plane at three measurement times from a sample of 28 children (n = 10 girls, age 9.7 ± 3.1 years) with CP were eligible for evaluation. For the verification of reliability, the measurement results from the first and second measurement times (t1, t2) were used at intervals of 1 day. In addition, differences after the rehabilitation program the patients underwent (t3) were evaluated using the measurement results of the first and third measurements (5-day interval). RESULTS: The results show good to excellent intra-rater reliability for the SM measurements, both in the sagittal and in the frontal plane (ICC values = 0.69-0.99). Furthermore, significant changes may occur after only 1 week of therapeutic intervention for total spinal inclination (t1: 12.82 ± 5.40, t3: 11.11 ± 5.60, p = 0.014, Cohen's d = 0.43) and spine length (t1: 401.75 ± 69.05, t3: 409.25 ± 63.58, p = 0.030, Cohen's d = 0.43). CONCLUSIONS: SM can be used to generate reliable values for functional analysis of the spine in children with CP. Furthermore, significant posture differences can be demonstrated by therapeutic interventions, especially in the spine inclination (Inc) and spine length (SL). These slides can be retrieved under Electronic Supplementary Material.

The Appendicular Lean Mass Index Is a Suitable Surrogate for Muscle Mass in Children with Cerebral Palsy.

BACKGROUND: Densitometrically measured lean body mass (LBM) is often used to quantify skeletal muscle mass in children with cerebral palsy (CP). Since LBM depends on the individual's height, the evaluation of $\frac{{{\rm{LBM}}}}{{heigh{t^2}}}\ $ (lean BMI) is often recommended. However, LBM includes not only skeletal muscle mass but also the mass of skin, internal organs, tendons, and other components. This limitation applies to a far lesser extent to the appendicular lean mass index (LMIapp). OBJECTIVES: The aim of the study was to evaluate skeletal muscle mass in children with CP using total lean BMI (LMItot) and LMIapp. METHODS: The present study was a monocentric retrospective analysis of prospectively collected data among children and adolescents with CP participating in a rehabilitation program. In total, 329 children with CP [148 females; Gross Motor Function Classification Scale (GMFCS) I, 32 children; GMFCS II, 73 children; GMFCS III, 133 children; GMFCS IV, 78 children; and GMFCS V, 13 children] were eligible for analysis. The mean age was 12.3 ± 2.75 y. Pediatric reference centiles for age-adjusted LMIapp were generated using data from NHANES 1999-2004. Low skeletal muscle mass was defined as a z score for DXA determined LMItot and LMIapp less than or equal to -2.0. RESULTS: The z scores for LMIapp were significantly lower than LMItot in children with CP, GMFCS levels II-V (P < 0.001), with the exception of GMFCS level I (P = 0.121), where no significant difference was found. The prevalence of low LMItot (16.1%; 95% CI: 16.1, 20.1%) was significantly lower (P < 0.001) than the prevalence of LMIapp (42.2%; 95% CI: 36.9, 47.9%) in the study population. CONCLUSIONS: The prevalence of low skeletal muscle mass in children with CP might be underestimated by LMItot. LMIapp is more suitable for the evaluation of skeletal muscle mass in children with CP.

Anthropometric measurements to identify undernutrition in children with cerebral palsy.

AIM: To evaluate the diagnostic performance of anthropometric indicators to identify undernutrition in children with cerebral palsy (CP). METHOD: The present study was a monocentric retrospective analysis of prospectively collected data among children and adolescents with CP participating in a rehabilitation program. Undernutrition was defined as a z-score for dual-energy X-ray absorptiometry (DXA) determined body fat percentage less or equal to -2.0. The cut-off values for body mass index (BMI) of the World Health Organization (WHO) and the Centers for Disease Control and Prevention (CDC), and the cut-off values for BMI and height for age of the Robert Koch Institut (RKI) were evaluated. RESULTS: In total, 329 children with CP (181 males, 148 females, Gross Motor Function Classification System levels I-V) were eligible for analysis. The mean age was 12 years 4 months (SD 2y 9mo). The BMI cut-off values showed the following sensitivities and specificities: WHO, sensitivity of 0.474 (95% confidence interval [CI] 0.244-0.711), specificity of 0.897 (95% CI: 0.857-0.928); CDC, sensitivity of 0.632 (95% CI: 0.384-0.837), specificity of 0.819 (95% CI: 0.772-0.861); RKI, sensitivity of 0.789 (95% CI: 0.544-0.939), specificity of 0.732 (95% CI: 0.679-0.781); and for height for age, sensitivity of 0.263 (95% CI: 0.091-0.512), specificity of 0.668 (95% CI: 0.612-0.720). INTERPRETATION: BMI had a high specificity but very low sensitivity in identifying undernutrition in children with CP. Z-scores for height for age had even lower specificity and sensitivity and seemed not to be appropriate for predicting undernutrition in children with CP. WHAT THIS PAPER ADDS: Body mass index (BMI) z-scores had a high specificity but very low sensitivity in identifying undernutrition in children with cerebral palsy (CP). Height z-scores were not appropriate for predicting undernutrition in children with CP. Undernutrition assessed by BMI was overestimated in children with CP versus when assessed by dual-energy X-ray absorptiometry (DXA).

MEDICIONES ANTROPOMÉTRICAS PARA IDENTIFICAR DESNUTRICIÓN EN NIÑOS CON PARÁLISIS CEREBRAL: OBJETIVO: Evaluar el rendimiento diagnóstico de los indicadores antropométricos para identificar la desnutrición en niños con parálisis cerebral (PC). MÉTODO: El presente estudio realizado en un solo centro de atención, fue un análisis retrospectivo de datos recopilados prospectivamente entre niños y adolescentes con PC que participan en un programa de rehabilitación. La desnutrición se definió como una puntuación z para la absorciometría de rayos X de energía dual (DXA), y porcentaje de grasa corporal determinado menor o igual a -2,0. Fueron evaluados los valores de corte para el índice de masa corporal (IMC) de la Organización Mundial de la Salud (OMS) y los Centros para el Control y la Prevención de Enfermedades (CDC), y los valores de corte para el IMC y la altura para la edad del Robert Koch Institut (RKI). RESULTADOS: En total, 329 niños con PC (181 varones, 148 mujeres, con niveles I - V del Sistema de clasificación de la función motora gruesa) fueron elegibles para el análisis. La edad media fue de 12 años 4 meses (DS 2a 9m). Los valores de corte del IMC mostraron las siguientes sensibilidades y especificidades: OMS, sensibilidad de 0,474 (intervalo de confianza del 95% [IC] 0,244-0,711), especificidad de 0,897 (IC del 95%: 0,857-0,928); CDC, sensibilidad de 0,632 (IC del 95%: 0,384 a 0,837), especificidad de 0,819 (IC del 95%: 0,772 a 0,861); RKI, sensibilidad de 0,789 (IC 95% 0,544-0,939), especificidad de 0,732 (IC 95% 0,679-0,781); y para la altura para la edad, la sensibilidad de 0,263 (IC del 95%: 0,091 a 0,512), la especificidad de 0,668 (IC del 95%: 0,612 a 0,720). INTERPRETACIÓN: El IMC tenía una alta especificidad, pero una sensibilidad muy baja para identificar la desnutrición en niños con PC. Las puntuaciones Z para la altura para la edad tenían una especificidad y sensibilidad aún más bajas y no parecían ser adecuadas para predecir la desnutrición en niños con PC.

MEDIDAS ANTROPOMÉTRICAS PARA IDENTIFICAR SUBNUTRIÇÃO EM CRIANÇAS COM PARALISIA CEREBRAL: OBJETIVO: Avaliar o desempenho diagnóstico de indicadores antropométricos para avaliar subnutrição em crianças com paralisia cerebral (PC). MÉTODO: O presente estudo foi uma análise monocêntrica retrospectiva de dados coletados prospectivamente entre crianças e adolescentes com PC que participavam de um programa de reabilitação. A subnutrição foi definida como um escore z para porcentagem de gordura corporal determinada por absorciometria de dupla energia de raio-X (DXA) menor ou igual a -2.0. Os valores de corte para o índice de massa corporal IMC) da Organização Mundial de Saúde (OMS) e dos Centros para Controle e Prevenção de Doenças (CCPD), e os valores de corte para IMC e altura por idade do Robert Koch Institut (RKI) foram avaliados. RESULTADOS: No total, 329 crianças com PC (181 do sexo masculino, 148 do sexo feminino, níveis do Sistema de Classificação da Função Motora Grossa I-V) foram elegíveis para análise. A média de idade foi 12 anos e 4 meses (DP 2a 9m). Os valores de corte do IMC mostraram as seguintes sensibilidades e especificidades: OMS, sensibilidade de 0,474 (intervalo de confiança [IC] a 95% 0,244-0,711), especificidade de 0,897 (IC 95% 0,857-0,928); CCPD, sensibilidade de 0,632 (IC 95% 0,384-0,837), especifididade de 0,819 (IC 95% 0,772-0,861); RKI, sensibilidade de 0,789 (IC 95% 0,544-0,939), especificidade de 0,732 (IC 95% 0,679-0,781); e de altura por idade, sensibilidade de 0,263 (IC 95% 0,091-0,512), especificidade de 0,668 (IC 95% 0,612-0,720). INTERPRETAÇÃO: O IMC teve alta especificidade mas sensibilidade muito baixa para identificar subnutrição em crianças com PC. Os escores z para altura por idade tiveram especificidade ainda menor e não pareceram apropriados para predizer subnutrição em crianças com PC.

Development of disorder-specific normative data for growth in children with cerebral palsy.

The purpose of this study was to create growth-percentiles for Caucasian children with cerebral palsy (CP). The studied parameters were height and age. In a retrospective analysis, we converted measurements collected in our center to create disorder-specific percentiles of normative data. Patients were stratified due to sex (male and female) and to mobility levels using the gross motor function classification system (GMFCS) (A = walking; GMFCS I-III, B = non walking; GMFCS IV-V) into four groups. In total, 2363 measurements in patients 0-18 years were collected. The mean age for group "Am" was 6.8 years (n = 862), group "Bm" 7.6 years (n = 563), group "Af" 7.7 years (n = 600), and group "Bf" 8.2 years (n = 366). The created percentiles for all groups were below the reference percentiles for healthy Caucasian children (KiGGS). The median curve for children with GMFCS levels I-III is slightly above the 3rd percentile, whereas the 50th percentile for GMFCS levels IV-V is mostly below the 3rd KiGGS centile.Conclusion: In conclusion, children with cerebral palsy are smaller than healthy children. The difference between 50th percentile of CP patients compared to healthy children supports the need for the use of disorder-specific growth charts. Those charts can help clinicians differentiate growth disorders in patients with CP. What is Known: • Children with cerebral palsy are shorter than healthy children and height is influenced by level of ambulation. • Currently, only reference percentiles of American children with mixed ethical backgrounds are available to evaluate growth. What is New: • This paper presents disorder-specific reference percentiles for longitudinal growth of Caucasian children with cerebral palsy depending on motor function. • These percentiles allow to asses longitudinal growth in children with cerebral palsy to detect other additional diseases impairing growth.

TBS as a Tool to Differentiate the Impact of Antiresorptives onCortical and Trabecular Bone in Children With OsteogenesisImperfecta.

INTRODUCTION/BACKGROUND: Osteogenesis imperfecta is a hereditary connective tissue disorder, resulting in low bone mass and high bone fragility. Dual-energy X-ray absorptiometry (DXA) and in adulthood also the trabecular bone score (TBS) are well established to assess bone health and fracture risk. The purpose of this investigation was to assess the usefulness of TBS in respect to different treatment regimes in children with osteogenesis imperfecta. Changes of areal bone mineral density (aBMD) and TBS using DXA scans of children treated with antiresorptive therapies were evaluated. METHODOLOGY: DXA scans (aBMD, TBS) of 8 children with OI were evaluated. The scans were taken during a 1 yr period of treatment with bisphosphonates and during 1 yr pilot trial using denosumab. Changes of aBMD and TBS during both treatment regimens were compared. RESULTS: During bisphosphonate treatment aBMD increased about 6.2%, while TBS increased about 2.1%. The difference between aBMD and TBS before and after bisphosphonate treatment was not significant (p = 0.25). During denosumab treatment aBMD increased around 25.1%, while TBS increased 6.7%. The change of aBMD was significant (p = 0.007), as was the difference between aBMD and TBS (p < 0.001). CONCLUSIONS: Denosumab had a significant effect on both aBMD and TBS but was significantly more pronounced in aBMD. These results suggest a stronger effect of denosumab on cortical bone and the growth plate in comparison to bisphosphonates. Beside the lack of paediatric reference data and the small sample size, the results suggest TBS to be a useful tool for monitoring skeletal changes during development, growth, and antiresorptive therapy in children with OI.

Motor Function Improvement in Children with Ataxia Receiving Interval Rehabilitation, Including Vibration-Assisted Hometraining: A Retrospective Study.

BACKGROUND: Physiotherapy, including vibration-assisted therapy, has been proven to be effective for patients with ataxic cerebral palsy. Herewith, we studied the effect of a functional, goal-oriented interval rehabilitation program, including vibration-assisted home-training on the motor function of children with congenital ataxias. PATIENTS: 45 children (mean age 7.7 years, SD 4.70) with ataxia, having received a 6-month home-based side-alternating vibration-assisted therapy combined with intensive, goal-oriented, functional rehabilitation intervals, were included in the study, classified according to the progressive or non-progressive ataxia character. METHOD: Retrospective analysis of the prospectively collected data of the registry of the Cologne rehabilitation program "Auf die Beine". Motor abilities have been assessed prior to the intervention (M0), after 6 months of home-training (M6) as well as in a follow-up 6 months later (M12). We performed a gait analysis, a 1-minute walking test (1-MWT), and the Gross Motor Function Measure (GMFM-66). RESULTS: The GMFM-66 improvement (M6-M0 vs. M12-M6) was statistically significant with median improvement of 2.4 points (non-progressive) and 2.9 points (progressive) respectively, and clinically relevant. The 1-MWT improvement was statistically significant and clinically relevant for non-progressive ataxia. CONCLUSION: The intensive training, including vibration-assisted therapy significantly improved the motor function of children with ataxia. Six months later the skills were preserved in children with progressive ataxia and could be further developed in non-progressive forms.

Effect of layer thickness on the marginal and internal adaptation of laser-sintered metal frameworks.

STATEMENT OF PROBLEM: Laser sintering is commonly used for fabricating metal-ceramic restorations. The layer thickness of the sintering process may affect restoration adaptation. However, limited information is available regarding its impact. PURPOSE: The purpose of this in vitro study was to compare the marginal and internal adaptation of laser-sintered cobalt-chromium single crown frameworks sintered with layer thicknesses of 25 and 50 µm. MATERIAL AND METHODS: Thirty resin dies that represented prepared single molar abutment teeth were prepared by using a 3-dimensional printer and were divided into 3 groups (n=10) according to the method used for fabricating metal frameworks: group C, metal frameworks fabricated by using the lost-wax method (control); group L25, metal frameworks fabricated by using direct metal laser melting with a layer thickness of 25 µm; and group L50, metal frameworks fabricated by using direct metal laser melting with a layer thickness of 50 µm. After fabricating the metal frameworks, 15 vertical marginal discrepancy measurements were made in each axial region (mesial, distal, buccal, and lingual) using a stereomicroscope. Next, all the specimens were sectioned from the midline, and 5 internal discrepancy measurements were made in each internal region (inner marginal, axial, and occlusal). The data were analyzed statistically by using 1-way ANOVA, the Tukey honestly significant difference, and Tamhane T2 tests (α=.05). RESULTS: The highest marginal and internal discrepancy values were obtained for metal frameworks in group C, and these values were significantly different (P<.001) from those obtained for metal frameworks in the other 2 groups. No significant difference was observed in the marginal and internal discrepancy values of metal frameworks in groups L25 and L50. CONCLUSIONS: These results indicate that layer thickness does not affect the adaptation of laser-sintered metal frameworks, yet both sintering parameters yielded significantly lower mean marginal discrepancy values than the cast group.

Diagnostic performance of body mass index to identify excess body fat in children with cerebral palsy.

AIM: To assess the diagnostic performance of body mass index (BMI) cut-off values according to recommendations of the World Health Organization (WHO), the World Obesity Federation (WOF), and the German Society for Adiposity (DAG) to identify excess body fat in children with cerebral palsy (CP). METHOD: The present study was a monocentric retrospective analysis of prospectively collected data among children and adolescents with CP participating in a rehabilitation programme. Excess body fat was defined as a body fat percentage above the 85th centile assessed by dual-energy X-ray absorptiometry. RESULTS: In total, 329 children (181 males, 148 females) with CP were eligible for analysis. The mean age was 12 years 4 months (standard deviation 2y 9mo). The BMI cut-off values for 'overweight' according to the WHO, WOF, and DAG showed the following sensitivities and specificities for the prediction of excess body fat in our population: WHO: sensitivity 0.768 (95% confidence interval [CI] 0.636-0.870), specificity 0.894 (95% CI 0.851-0.928); WOF: sensitivity 0.696 (95% CI 0.559-0.812), specificity 0.934 (95% CI 0.898-0.960); DAG: sensitivity 0.411 (95% CI 0.281-0.550), specificity 0.993 (95% CI 0.974-0.999). INTERPRETATION: Body mass index showed high specificity, but low sensitivity in children with CP. Thus, 'normal-weight obese' children with CP were overlooked, when assessing excess body fat only using BMI. WHAT THIS PAPER ADDS: Excess body fat in children with cerebral palsy (CP) is less common than previously reported. Body mass index (BMI) had high specificity but low sensitivity in detecting excess body fat in children with CP. BMI evaluation criteria of the German Society for Adiposity could be improved in children with CP.

Color match of a feldspathic ceramic CAD-CAM material for ultrathin laminate veneers as a function of substrate shade, restoration color, and thickness.

STATEMENT OF PROBLEM: The final color of a ceramic restoration, especially an ultrathin veneer, is important, but selecting the correct shade is difficult because the substrate can affect the final color of the restoration. PURPOSE: The purpose of this in vitro study was to investigate the effect of substrate shade and thickness on the final color of ultrathin laminate veneers milled from feldspathic ceramic and to present a straightforward methodology with which a clinician can visualize the effects of substrate color, ceramic thickness, and prefabricated computer-aided design and computer-aided manufacturing (CAD-CAM) block color on the final color of the restoration. MATERIAL AND METHODS: All specimens were fabricated by slicing CAD-CAM feldspathic ceramic material with a precision cutter into 12×10-mm slices of approximately 0.35, 0.55, 0.75, and 1.55 mm in thickness with 10 different colors (0M1-1M1-1M2-2M1-2M2-2M3-3M1-3M2-3M3-4M1). As a background substrate, composite resin disks (12×10×2 mm) were fabricated with different shades (0M1 S, 1M1 S, 2M3 S, 3M2 S, 4M3 S, 5M3 S). The CIELab values of the polished surfaces of each specimen were measured on a background (white or simulated foundation) with a spectrophotometer by a single experienced operator, and color differences (ΔE) were calculated. Mean ±SD values were calculated and subjected to ANOVA with 2 variables (substrate and ceramic color) (α=.05). RESULTS: Combinations of a lighter substrate shade and a lighter value ceramic restoration and of a darker substrate shade and darker value ceramic restoration only changed the final color of the restoration minimally. CONCLUSIONS: The final color of a dental restoration is affected by the thickness of the restoration, the substrate color, and the ceramic color. Lighter and darker substrate colors show more color changes, and thin veneers cannot mask the substrate color.