Swallowing kinematics and submental muscles activation during a newly designed maneuver called Mouth Open Swallowing Maneuver: A comparative study.

The aim of this study was to design a new maneuver called the Mouth Open Swallowing Maneuver (MOSM), and to compare swallowing kinematics and submental muscles activation (SMA) between MOSM and two current approaches used in dysphagia rehabilitation. Fifty healthy volunteers were asked to perform three repetitions of dry swallowing (DS) (control task), the MOSM, the Mendelsohn Maneuver (MM), and the Tongue-Hold Maneuver (THM) during videofluoroscopic swallowing study accompanied with simultaneous SMA recording. Swallowing kinematics were measured by frame-by-frame analysis on hyolaryngeal movement using ImageJ. Swallowing with maximum hyolaryngeal movement and SMA during these tasks was used for comparative analysis. Vertical movement of the hyoid during the MOSM was significantly greater than those observed during the DS and the THM (p<0.001, p<0.001). Horizontal movement of the hyoid during DS and the THM was significantly greater than that observed during the MM (p = 0.001, p = 0.001). Vertical movement of the larynx during the MOSM was significantly greater than those observed during DS, MM, and THM (p<0.001). There was no significant difference between tasks in horizontal movement of the larynx (p = 0.785). SMA during the THM was significantly greater than that observed during MOSM (p = 0.002). No significant difference was found between other tasks in terms of SMA (p>0.05). The MOSM as a newly designed maneuver was significantly superior to other maneuvers in increasing vertical hyolaryngeal movement. The THM has as much effect on hyolaryngeal movement as the MM. In this study, the MOSM was shown to be effective in increasing hyolaryngeal movement. ClinicalTrials.gov Protocol Registration and Results System (PRS); the clinical trial registration number (NCT05579041).

Are sucking patterns and early spontaneous movements related to later developmental functioning outcomes? A cohort study.

Sucking patterns and early spontaneous movements have an important role in the determination of later developmental problems, but the relationship of the two together with long-term outcomes has not been investigated. The objectives of this study were to (i) examine the relationship between sucking patterns using the Neonatal Oral Motor Assessment Scale (NOMAS) and fidgety movements and other movement patterns using detailed General Movements Assessment (GMA), and (ii) investigate the relationship between these early assessment methods and developmental functioning outcomes at later ages. We analyzed the NOMAS from 34 weeks' postmenstrual age up to 10 weeks post-term and GMA between 9 and 20 weeks post-term age, and the Bayley Scales of Infant and Toddler Development-Third Edition (Bayley-III) was applied for the developmental functioning outcomes to 62 infants (61%, 62/102) between 12 and 42 months of age. Among 102 infants at-risk, 70 (69%) showed a normal sucking pattern, and 85 (83%) had fidgety movements. The median Motor Optimality Score-Revised (MOS-R), as determined by GMA, of all infants was 24. The NOMAS was related to the MOS-R and its subcategories (p < 0.05) in all infants at-risk. The NOMAS, MOS-R and its subcategories were also related to cognitive, language, and motor development at later ages according to Bayley-III (p < 0.05).   Conclusion: This longitudinal study showed that the quality of sucking patterns, fidgety movements, and MOS-R were related to later developmental functioning, indicating that abnormal sucking patterns, aberrant fidgety movements, and lower MOS-R might predict developmental disorders. What is Known: • Sucking patterns and early spontaneous movements in which central pattern generators play an important role are related. • Sucking patterns and early spontaneous movements might be used separately to predict developmental outcomes. What is New: • Sucking patterns and early spontaneous movements, when used together, were related to later developmental functioning, including cognitive, language, and motor development in at-risk infants. • Predictive value of sucking patterns was lower for each developmental functioning outcome than early spontaneous movements.

Effectiveness of Conventional Dysphagia Therapy (CDT), Neuromuscular Electrical Stimulation (NMES), and Transcranial Direct Current Stimulation (tDCS) in Acute Post-Stroke Dysphagia: A Comparative Evaluation.

This study aims to compare the effectiveness of conventional dysphagia therapy (CDT), neuromuscular electrical stimulation (NMES), and transcranial direct current stimulation (tDCS) in the treatment of post-stroke dysphagia. A single-blind randomized controlled trial was conducted with 40 acute stroke patients - 18 females and 22 males with a mean age of 65.8 ± 11.9. The subjects were grouped into 4, with 10 individuals in each. The procedures administered to groups were as follows: the first group, sham tDCS and sham NMES; the second group, tDCS and sham NMES; the third group, NMES and sham tDCS; and the fourth group, all therapy procedures. CDT was applied to all groups either as a standalone procedure or combined with one or two of the instrumental techniques. Gugging Swallowing Screen (GUSS) and Videofluoroscopic Swallowing Study (VFSS) were employed to determine the severity of dysphagia and the effectiveness of treatment modalities. Additionally, the Penetration Aspiration Scale (PAS), Functional Oral Intake Scale (FOIS), and Dysphagia Severity Rating Scale (DSRS) were administered to interpret VFSS data. Pre- and post-treatment comparisons of all groups have revealed a statistically significant difference for all parameters except for the PAS scores at International Dysphagia Diet Standardization Initiative (IDDSI)-Level 4 consistencies. However, the differences between pre- and post-treatment scores of the fourth group across all parameters were significant - GUSS (p = 0.005), FOIS (p = 0.004), DSRS (p = 0.005), PAS IDDSI-4 (p = 0.027), PAS IDDSI-0 (p = 0.004). Inter-group comparisons, on the other hand, pointed out that the difference between pre- and post-treatment GUSS, FOIS, DSRS, and PAS scores at IDDSI Level-0 consistencies was statistically significant for all groups - GUSS (p = 0,009), FOIS (p = 0,004), DSRS (p = 0,002), PAS IDDSI-0 (p = 0,049). Closer examination of treatment groups indicated that the tDCS + CDT group, the NMES + CDT group, and the group that underwent the combination of three modalities made better progress than the one that was treated with only CDT. Though not statistically significant, the NMES + CDT group achieved better improvement than the tDCS + CDT group. This study has yielded that the group in which NMES, tDCS, and CDT were applied in combination has achieved better results than all the other groups. All treatment modalities applied to accelerate the general recovery process in acute stroke patients with dysphagia were found to be effective for the treatment of post-stroke swallowing disorders. The use of instrumental treatments such as NMES and tDCS enhanced the effectiveness of the treatment and provided more significant progress. Furthermore, combining treatment modalities such as NMES and tDCS was more effective when compared to using only conventional therapy. As a result, the most effective treatment outcomes were obtained by the group receiving CDT, NMES, and tDCS in combination. Therefore, the use of combined approaches has been recommended in appropriate patients; yet the provisional results should be tested in randomized trials with more participants.

Dynamic Imaging Grade of Swallowing Toxicity in Children with Esophageal Atresia.

INTRODUCTION: The Dynamic Imaging Grade of Swallowing Toxicity (DIGEST) scale was developed to evaluate the safety, efficiency, and overall pharyngeal swallowing performance in patients with dysphagia (DIGESTs, DIGESTe, and DIGESTt, respectively). Although various types of swallowing dysfunction are encountered in children with esophageal atresia (EA), oropharyngeal dysphagia poses risk for aspiration. Therefore, a retrospective study was performed to evaluate the safety and efficacy of swallowing by using DIGEST score in children with EA. PATIENTS AND METHODS: Thirty-nine EA patients were included. The demographic features, respiratory problems, results, and outcomes of surgical treatment were evaluated from medical records. The videofluoroscopic swallowing evaluation investigated for both airway protection and bolus residuals at the level of vallecula, posterior pharyngeal wall, and pyriform sinus at liquid and pudding consistencies. The penetration and aspiration scale (PAS) was used to define penetration and aspiration severity, and DIGEST was used to evaluate DIGESTs, DIGESTe, and DIGESTt. RESULTS: The median age of the patients were 13 months (7-39 months), and male-to-female ratio was 25:14. Sixty-seven percent of patients were type-C EA and 61% of them has associated anomalies; 38% of patients had aspiration (PAS = 6-8) in liquids and 10% in pudding consistency. Life-threatening/profound swallowing dysfunction in DIGESTe (DIGEST = 4) was seen in 13% (n = 5) of patients; 40% of EA patients showed severe problems in DIGESTt. CONCLUSION: DIGEST is a valid and reliable tool to define the efficacy and safety of swallowing in children with EA.

The Role of Bolus Residue and Its Relation with Respiratory Problems in Children with Esophageal Atresia.

Bolus residue is significant risk factor for postswallow aspiration. A retrospective study was performed to evaluate the role of bolus residue and its relation with respiratory problems in children with esophageal atresia. Children were evaluated for demographic features, type of esophageal atresia, associated anomalies, and respiratory problems. The videofluoroscopic swallowing evaluation (VFSE) was performed, and scored by using the penetration aspiration scale (PAS), bolus residual score (BRS) and normalized residual ratio scale (NRRS). Children with and without respiratory problems were also compared in terms of aspiration and bolus residue. Forty-one children with a median age of 15 months (1-138 months), male:female ratio of 26:15 was included. 65.9% (n = 27) of children were type-C and 24.4% (n = 10) were type-A EA. In 61% (n = 25) of children had liquid aspiration (PAS ≥ 6) and 9.8% (n = 4) had aspiration in pudding consistencies. Children with aspiration in liquids had significantly higher NRRS and BRS scores in vallecular residue for pudding consistencies when compared to children without aspiration (p < 0.05). No difference was detected in terms of PAS scores and bolus residual parameters between children with and without respiratory problems (p > 0.05). Children with aspiration in liquids have higher scores of BRS and NRRS at the level of vallecular especially in pudding consistencies. VFSE findings for bolus residue did not show significant relation with respiratory problems. Respiratory morbidity in children with EA is multifactorial and may not only explained by bolus residuals and aspiration.

Surface electromyographic activity of submental muscles during different types of swallow-specific tasks: A comparative study.

BACKGROUND: Repetitive performance of a motor task has been proposed to increase the ability to perform that motor task. Therefore, exercise training including swallow-specific task may be an optimal training to improve swallowing function. OBJECTIVES: To determine the submental muscles activity during different types of swallow-specific tasks (effortful swallow, ES; tongue-hold swallow, THS; swallow with Kinesio taping, SwKT; and head extension swallowing exercise, HESE) compared to normal swallow (NS), and also compare these tasks within themselves. METHODS: Thirty-five healthy adults participated in this prospective experimental study with one participant group. The surface electromyography (sEMG) was used to evaluate submental muscles activity while performing swallow-specific tasks. RESULTS: While the greatest submental muscles activity was revealed during the ES, HESE, NS and THS tasks, respectively, the lowest was obtained during SwKT. When compared to NS, ES caused a statistically greater submental muscles activity (p1  = .000 and p2  = .000), although SwKT revealed a lower submental muscles activity (p1  = .002 and p2  = .000). When swallow-specific tasks were compared within themselves, ES caused the highest muscles activity (p < .005) than all other tasks, and HESE caused higher muscles activity than THS and SwKT (p < .005). CONCLUSION: Different swallow-specific tasks can cause increased or decreased submental muscles activity compared to NS task. Since ES and HESE are superior in selective submental muscles, they appear to be more promising in terms of submental muscles gains in long-term exercise training.

The Role of Oral Feeding Time and Sham Feeding on Oropharyngeal Swallowing Functions in Children with Esophageal Atresia.

A retrospective study was performed to evaluate the role of oral feeding (OF) time and sham feeding (SF) on oral-pharyngeal swallowing functions in children with esophageal atresia (EA). Patients with EA were evaluated for age, sex, and types of atresia, time to start OF and surgical complications. Three to six weeks after full OF, videofluoroscopic swallowing evaluation (VFSE) was performed, and Penetration Aspiration Score (PAS, no aspiration = 1, penetration = 2-5, aspiration = 6-8), delay in swallowing reflex and residue after liquid and pudding consistency were noted. Patients were divided into three groups according to repair time; early primary repair (EPR, < 1 month of age), delayed primary repair (DPR, ≥ 1 month of age) and colonic interposition (CI). VFSE findings were compared between groups. In CI group, patients without aspiration in VFSE were encouraged to sham SF before CI. Patients with and without SF in CI group were also compared for oro-pharyngeal dysphagia (OPD). PAS scores were significantly higher in DPR (n = 13) group when compared to CI group (n = 12) in liquid swallowing (p = 0.032) and higher than EPR (n = 30) in pudding consistency (p = 0.03). Patients with CI showed significant OPD when compared to EPR (p = 0.017). Swallowing reflex delay was similar in both groups (p > 0.05). DPR group had significantly higher liquid residue at the level of vallecula (p = 0.028). The residue at other levels (oral, tongue base, pharyngeal wall and pyriform sinus) were similar in all groups in both liquid and pudding consistencies. There was no significant difference between sham-fed (n = 6) and not sham-fed (n = 6) infants in VFSE of OPD (p > 0.05). Patients with DPR without SF had significantly higher incidence of OPD and PAS scores when compared to EPR and CI. Although CI groups has the latest OF time, SF may improve oral motor abilities and cause better OF swallowing functions than patients with delayed repair.

The cause of dysphagia in patients with esophageal atresia: a systematic review and meta-analysis.

PURPOSE: Dysphagia is the most common symptom in patients with esophageal atresia (EA) of all ages. There is no study addressing the direct relation between dysphagia and surgical results. Therefore, a systematic review was performed to define the relationship between surgical complications and dysphagia in patients with EA. METHODS: The systematic review was drafted under PRISMA guidelines. Systematic literature search was performed for the period 2000 (Jan) to 2019 (Dec)-20 years-in the databases: MEDLINE, EMBASE, and PubMed. Statistical analysis was performed using Comprehensive Meta-Analysis Version 3.0 software. RESULTS: Among 767 articles, 486 abstracts were screened for the inclusion criteria. The full-texts of 64 articles were assessed for eligibility. The sub-group analysis could be performed in 4 articles for anastomotic strictures. Heterogeneity was calculated by I2 statistic as 18,487 and pooled odds ratio was measured under the fixed effect model (Q = 3.68; P = 0.298, I2 = 18,487). There was no significant relationship with an odds ratio of 1.37 between anastomotic stricture (AS) and dysphagia (95% CI 0.631-2.973, p = 0.426). There was no publication bias for the data (Begg's test, p = 0.496; Egger's tests, p = 0.335). CONCLUSION: This meta-analysis did not reveal a significant relationship between AS and dysphagia in children with EA. Since many other factors contribute to dysphagia, comprehensive variable information such as detailed standardized registry systems for rare diseases for pooling analysis is needed regarding other potential factors including surgical complications.

Relationship between dysphagia severity and head and neck proprioception in patients with neurological disorders.

PURPOSE: The aim of this study was to examine the relationship between dysphagia severity and head and neck proprioception in patients with neurological diseases. METHODS: Twenty-six patients with neurological diseases who received the modified barium swallowing study (MBSS) were included. Dysphagia severity was assessed with the penetration aspiration scale (PAS). Patients were divided into two groups according to their PAS scores as "with dysphagia" (n = 15) and "without dysphagia" (n = 11). Active range of motion (AROM) and muscle strength were measured. Proprioception measurement of the cervical region was performed with a laser marker placed on the head of the patients, and the deviation from the middle target was noted in centimeters. RESULTS: There was no difference between groups in terms of gender, age, height, weight, diagnosis, AROM, and the muscle strength of the cervical region (p > 0.05). A moderate, positive correlation was found between dysphagia severity and the results of proprioception in terms of neck flexion, extension, and left rotation (r = 0.48, p = 0.01; r = 0.58, p = 0.002; r = 0.42, p = 0.02, respectively). There was a statistically significant difference in proprioception measurements of neck flexion, extension, and left rotation between groups (p < 0.05). CONCLUSION: In conclusion, patients' decreased head and neck proprioception is related to severe dysphagia. Therefore, a holistic approach should be followed for swallowing function, and head and neck proprioception should be considered in dysphagia management.

Orofacial functions and oral health: An analysis on children aged 5-8 years old.

The aim of this study is to assess the orofacial function performance and oral health status of healthy children as well as their potential correlations. In this descriptive study, the oral functions of four hundred systemically healthy children who applied for the pediatric dentistry clinic were evaluated. Three scales, namely Karaduman Chewing Performance Scale (KCPS), Pediatric version of the Eating Assessment Tool (PEDI-EAT-10), and Nordic Orofacial Test Screening (NOT-S) protocol, are used to collect the data. These children's carious lesions were evaluated by using the decayed, missing, filled tooth/surface (DMFT/S, dmft/s) indices, the International Caries Detection and the Assessment-II System (ICDAS-II). In total, 400 (6.5 years [78.02 months]) children were participated in the study. Their KCPS scores indicate that 58.0% of the children were at level 0 and 35.8% were at level 1. The results of the KCPS levels and the DMFT, dmft, DT, dt, MT, mt (as =0 and ≥1) were found to be statistically significant; p = .044, p = .009, p = .008, p = .000, p = .032, and p = .003, respectively. The total PEDI-EAT-10 score of 13.4% of the children was found to be 3 or higher, suggesting that they experience a problem in swallowing. According to the NOT-S, the most affected domains were "habits" (51.0%), "facial expression" (49.3%), and "chewing and swallowing" (45.3%). Among healthy children without a defined problem in swallowing and orofacial functions, the oral health status may affect orofacial functions. Decayed (particularly, extensive caries existence) or missing tooth may have critical importance for adequate chewing in children. Especially in the presence of so many decayed or missing teeth, evaluating the orofacial functions through different scales may considerably contribute to early diagnosis of functional problems.

The Status of Dysphagia Clinics During the COVID-19 Pandemic.

The study aimed to determine the status of dysphagia clinics and procedures applied in dysphagia clinics during the COVID-19 pandemic. Clinicians working in an outpatient dysphagia clinic were included. A 30-question survey inquiring about the descriptive information of the participants and their clinics, their clinical practice, and the tele-health applications during the COVID-19 pandemic. The survey was administered via Google forms. The participants were asked to fill out the survey on behalf of their clinics. One survey was completed per dysphagia clinic. Twenty-three clinicians responded on behalf of their clinics. The number of patients and dysphagia evaluations decreased during the COVID-19 pandemic (p < 0.05). The COVID-19 screening procedures mostly performed before dysphagia evaluations were temperature check (n = 14, 60.9%), nasopharyngeal swab test (n = 9, 39.1%), anamnestic risk assessment (n = 6, 26.1%), and saturation test (n = 6, 26.1%). Protective equipments mostly used while dysphagia evaluations were surgical mask, FFP3 mask, standard gloves, glasses, and face shield. It was found that 69.6% (n = 16) of the dysphagia clinics were reported to be suitable for working under pandemic conditions, and 30.4% (n = 7) were reported to be unsuitable. The use of tele-health applications significantly increased from 13.0% (n = 3) to 52.2% (n = 12) (p = 0.003). The present study provides a general overview of the status of dysphagia clinics and procedures applied in dysphagia clinics during the COVID-19 pandemic period. The study showed that working conditions, the number of patients, and the total number of evaluations have changed throughout the pandemic, and the use of tele-health applications increased.

Assessment of swallowing function in pediatric patients with Wilson's disease: Results of a videofluoroscopic swallowing study.

BACKGROUND AND STUDY AIMS: Wilson's disease (WD) is a complex disorder related to copper metabolism and neurological involvement may lead to swallowing disorders. The purpose of this study was to evaluate swallowing function in pediatric patients with WD by using videofluoroscopic swallowing study (VFSS). PATIENTS AND METHODS: A total of 21 patients were included in the study, prospectively. The VFSS was conducted to evaluate swallowing function of the patients. The penetration-aspiration scale (PAS) was used to assess penetration-aspiration severity. RESULTS: According to the VFSS, abnormal results were detected in nine patients (42.9%) with WD. Of these nine patients, oral phase dysfunction was present in one patient, laryngeal penetration was present in one patient and moreover, abnormal esophageal body function was detected in all nine patients. Of these nine patients, five had neurological presentation at the time of diagnosis, and remaining four patients had hepatic presentation. Mean PAS score of the patients was 1. CONCLUSION: The current study results suggest that subclinical swallowing dysfunction may be observed in patients with either neurological or hepatic WD. Further studies are necessary to reveal the real incidence of esophageal phase problems of swallowing function in pediatric patients with WD.

Pediatric dentists can determine chewing performance level in children.

OBJECTIVES: The current study was aimed to verify whether pediatric dentists could determine chewing performance level in children by using Karaduman Chewing Performance Scale (KCPS). MATERIALS AND METHODS: Typical developing children and children with cerebral palsy (CP) who were referred to pediatric dentistry above the age of 2 years were included in the study. The chewing performance level was scored according to KCPS. One experienced physical therapist and three pediatric dentists independently assessed the chewing videos of the children and scored each child's chewing function. The correlation between the KCPS scores of the physical therapist and the pediatric dentists was used for reliability. The agreement between the scorings of the physical therapist and pediatric dentists was assessed using Fleiss kappa statistics. RESULTS: Fifty-four typical developing children and 43 children with CP were included. A strong positive correlation between the KCPS scoring of the physical therapist and pediatric dentists was found (r=0.911-0.939, p<0.001). An excellent agreement in the KCPS scoring between the physical therapist and the 1st and 3rd dentists (p<0.001, κ 0.754-0.763), and a good agreement in the KCPS scoring between the physical therapist and the 2nd dentist was detected (p<0.001, κ 0.687). CONCLUSIONS: The study results show that the KCPS is reliable for pediatric dentists in determining the chewing performance level in children. Therefore, it could be suggested that pediatric dentists could use the KCPS in their clinical settings and research studies. CLINICAL RELEVANCE: The study may have clinical implications in the evaluation of children with chewing difficulty in dental practice. CLINICAL TRIAL NUMBER: NCT04407455.

A Comparative Analysis of Chewing Function and Feeding Behaviors in Children with Autism.

The present study was aimed to compare chewing performance level and feeding behaviors of children with autism to their typically developing peers. A total of 56 children (37 children with autism, 19 typically developing children) participated in the study. Feeding-related characteristics and observational oral-motor characteristics of children were recorded. The Karaduman Chewing Performance Scale (KCPS) was used to assess chewing performance level, the Behavioral Pediatrics Feeding Assessment Scale (BPFAS) was used to assess feeding behaviors of children, and the Turkish version of the Feeding/Swallowing Impact Survey (T-FS-IS) was used to evaluate the effect of the child's feeding and swallowing problem on their parents. Results showed that transition time to solid food intake for children with autism was later than typically developing children (p = 0.014), and they had more tongue thrusting (p = 0.009). There were differences between groups in terms of KCPS (p = 0.002), BPFAS (Total frequency score, p = 0.008; Child frequency score, p = 0.017; Parent frequency score, p = 0.021; Restriction score, p = 0.004), and T-FS-IS (Daily activities, p = 0.004; Worry, p = 009; Feeding difficulties, p = 0.031; Total score, p = 0.001). The present study shows that children with autism had worse chewing function and worse mealtime functioning compared to typically developing children. Their parents perceived mealtime behavior as more problematic, and parents' quality of life related to feeding/swallowing disorders was worse compared to parents of typically developing children. The study results reveal the importance of early assessment and intervention of chewing function and feeding behaviors in children with autism.

Dysphagia in Children with EA-TEF From the Perspective of Pediatric Surgeons in Clinical Settings.

The current study was aimed to define clinical practice, knowledge and awareness, and best practice regarding dysphagia in children with esophageal atresia with/without tracheoesophageal fistula (EA-TEF) from the perspective of pediatric surgeons in Turkey. Pediatric surgeons practice EA repair were included. A survey related to clinical practice, knowledge and awareness, and best practice regarding dysphagia in EA-TEF was used. The survey was electronically sent to potential participants. Seventy-two pediatric surgeons with a mean professional experience of 14.73 ± 9.66 years (min = 1, max = 41) completed the survey. 19.4% (n = 14) had a standardized protocol for dysphagia screening and %51.4 (n = 37) provided swallowing rehabilitation to their patients. Most of the participants (80.6%) reported that they do not have an appropriate team approach for dysphagia management. The mean knowledge and awareness score was 11.04 ± 1.27 (min = 7, max = 12). The mean VAS score related to the need for standardized protocol in dysphagia management was 9.23 ± 1.44 (min = 3, max = 10). The mean VAS score related to the need for routine screening for dysphagia after surgery was 8.67 ± 2.17 (min = 1, max = 10). The mean score for the degree of the contribution of diagnosis and management of dysphagia to the surgical treatment in children operated for EA-TEF was 7.98 ± 2.08 (min = 3, max = 10). The participants mostly suggested the necessity of a standardized protocol in dysphagia and routine dysphagia screening in children with EA-TEF. Knowledge and awareness is found to be high among pediatric surgeons in Turkey. However, their clinical practice does not include a standardized protocol regarding dysphagia screening and management.

Cystinosis beyond kidneys: gastrointestinal system and muscle involvement.

BACKGROUND: Cystinosis is a multisystemic disease resulting from cystine accumulation primarily in kidney and many other tissues. We intended to study the evolution of less commonly seen extrarenal complications of cystinosis in a group of patients who have periods without cysteamine treatment. METHODS: Gastrointestinal and muscular complications of cystinosis were studied in a group of 21 patients. RESULTS: Twenty one patients were included in the study. Among them, 14 were homozygous and 3 were compound heterozygous for CTNS mutations. The median age of diagnosis was 15 months (range; 5 months-14 years) and the mean age at last visit was 11.3 ± 6.5 years. Nine patients (42%) had end stage renal disease at a mean age of 10.6 years (6.5-17 years). Abdominal ultrasonography and portal vein doppler ultrasonography were performed in19 patients, 14 of them (74%) had hepatomegaly, 10 patients (53%) had granular pattern or heterogeneity of liver. Only one patient had high transaminase levels and liver biopsy showed cystine crystals in the liver. Eleven patients (58%) had borderline or increased portal vein minimum and maximum flow velocities. One patient had CK level of 9024 U/L and electromyographic study showed active myopathic involvement. Two patients were found to have gastroesaphageal reflux only and 4 patients were found to have esophageal remnants in addition to reflux. CONCLUSIONS: In addition to renal functions, extrarenal organs may be affected from cystine accumulation even in childhood, especially in patients who are incompliant to treatment, resulting in complications such as swallowing difficulty, hepatomegaly and portal hypertension.

Turkish Version of the Mastication Observation and Evaluation (MOE) Instrument: A Reliability and Validity Study in Children.

The Mastication Observation and Evaluation (MOE) instrument is an objective assessment of the chewing process in children. This study aimed to translate the MOE into Turkish and to test its reliability and validity in children with cerebral palsy (CP). A total of 53 children with CP and 27 typical children were included in the study. The MOE was translated from Dutch into Turkish by using the forward, backward, forward translation method. The internal consistency, intra- and inter-observer reliability, criterion, and discriminant validity of the Turkish version of the MOE (T-MOE) were investigated. Internal consistency was excellent with a Cronbach's alpha value of 0.98. The Intraclass correlation coefficient ranged from 0.89 to 0.97 for intra-rater reliability and from 0.86 to 0.94 for inter-rater reliability. The median score from Karaduman Chewing Performance Scale (KCPS) was 7 (min = 1, max = 8). All of the items in the T-MOE and the total T-MOE score had a negative and strong correlation with the KCPS score. Typical children without chewing disorders had greater T-MOE scores than the children with CP suffering from chewing disorders (p < 0.01). The T-MOE is a reliable and valid instrument for evaluating the observed oral motor behaviors of chewing function in children. It can be used in clinical practice and research.Clinical trial number: NCT03811353.

The functional chewing training for chewing dysfunction in children with repaired EA-TEF.

BACKGROUND/PURPOSE: Chewing disorders (CD) may cause restrictions in solid food intake and can be seen in 37% of children with esophageal atresia-tracheoesophageal fistula (EA-TEF). The Functional Chewing Training (FCT) is a holistic approach to improve chewing function (CF) in children. The study aimed to evaluate the effects of FCT on CF in children with EA-TEF. METHODS: Twenty children with CD were included. Patients received 12 weeks FCT including impairment-based and adaptive components. Chewing performance level was scored with Karaduman Chewing Performance Scale (KCPS), and tolerated food texture was determined by the International Dysphagia Diet Standardization Initiative (IDDSI). The baseline and final levels of KCPS and IDDSI were compared to evaluate the effects of FCT on CF. RESULTS: Forty-five percent of cases were isolated-EA, and 55% were EA-distal TEF with a median age of 31 (min = 25, max = 84) months, of which 65% (n = 13) were male. Baseline evaluation showed that 12 cases were in level-1, 6 cases in level-3 and 2 cases in level-4 according to KCPS. Eight children with CD (40%) had IDDSI level-3 and 12 (60%) had level-7. There was a significant improvement in KCPS scores and IDDSI scores after 12 weeks of training (p < 0.01, p = 0.005,respectively). KCPS scores showed level-0 in 15 cases, and level-1 in 5 cases. All children had IDSSI level-7. CONCLUSIONS: The FCT is an effective method to improve chewing function in children EA-TEF who had CD. TYPE OF STUDY: Single group treatment study. LEVEL OF EVIDENCE: Level 4.