Down Syndrome Children Research Articles

The Quality of Mainstreaming in Preschool: The Views of Parents of Children with Down Syndrome

Down syndrome (DS), one of the developmental (cognitive) deficits, is the most common syndrome that arises from genetic disorders. The mothers of children with DS who encounter the most intense emotional situations since the tendency to take responsibility the children’s care and development usually belongs them. Among these intensive feelings, mothers are also responsible for providing needs, such as special education support, searching for a suitable preschool institution to accept their children, and access to specialists who can understand their medical needs. This case study aims to investigate the opinions of the mothers whose children with DS aged between 36-72 months were attending preschool. The data were collected using semi-structured interview techniques in three different preschool institutions where the mainstreaming was carried out in the district center of Gaziantep, Şahinbey. Interviews were conducted with five mothers whose DS children went to the preschool institution in the spring semester during 2015-2106 academic year. Descriptive and content qualitative analyses were used to analyze the data. The findings regarding the opinions of the mothers of DS children on the quality of the mainstreaming practices revealed the following salient themes: Early experiences before mainstreaming, quality of mainstreaming at preschool and inclusion. The findings showed that inclusion practices have not been adopted in mainstreaming practices in our country; therefore, the most reliable sources mothers preferred to follow was the experienced families who had children with DS in the preschool.

Low risk of treatment resistance in Down syndrome with Kawasaki disease

A Japanese nationwide survey has reported that Down syndrome (DS) is a less-frequently occurring comorbidity in Kawasaki disease (KD). Although altered immune responses are frequently observed in DS, no studies have focused on the treatment response and risk for coronary artery abnormalities (CAA) in DS patients with KD. The aim of this study was therefore to evaluate the clinical manifestations, treatment response and prevalence of CAA in DS with KD. We retrospectively reviewed the medical records of DS patients with KD from 2005 through 2012. The survey questionnaires were sent to facilities nationwide, and clinical data regarding KD in DS were collected. A control group consisted of non-DS patients with KD who were managed at Toho University. Of the 94233 children diagnosed with acute KD from 2005 to 2012, 16 children with acute KD also had DS (0.017%). The DS-KD patients were significantly older than the non-DS patients (median, 8years vs 1year, P<0.05, respectively). Half of the DS patients had incomplete KD. Although 50% of the DS children were at high risk of immunoglobulin resistance, all children responded to initial treatment and none had CAA. All DS-KD patients responded to initial i.v. immunoglobulin (IVIG) or aspirin despite having a high risk of IVIG resistance, and none of the DS patients had CAA. This suggests that the risk of treatment resistance and development of CAA may be not higher in DS patients with acute KD.

Lingual tonsillectomy in children with Down syndrome: Is it safe?

ObjectiveEvaluate peri-operative course and morbidity in children with Down syndrome (DS) who underwent a lingual tonsillectomy (LT) for residual obstructive sleep apnea (rOSA). MethodsRetrospective case series for children with DS who underwent LT for rOSA from April 2011 to July 2016. Our primary outcomes were length of stay, readmission and complications. Surgical effectiveness was evaluated by change in the obstructive apnea-hypopnea-index(OAHI) and oxygen saturation nadir. ResultsThirty-nine patients underwent LT. The mean length of stay was 1.3 days with n = 21(72%) staying one night. One subject (2.6%) had a post-operative bleed that did not require operative intervention. No other major complications occurred. In terms of effectiveness of surgery, twenty–nine children had sufficient data for inclusion. Median OAHI did not appreciably change (p = 0.07) from before surgery. Five subjects (17%) were cured of OSA (OAHI < 2/hour) and a mix of improvement and worsening was identified. The lowest oxygen saturation improved from 78% (SD = 7) before surgery to 82% (SD = 6) after surgery (p = 0.003). ConclusionLT has a favorable post-operative course but its effectiveness at curing rOSA in the DS population has not been established/proven. Further research is indicated to determine optimal surgical management for DS children with LTH. Level of evidence4.

Dolphin therapy

Dolphin therapy emerged during the 1950s. The work of Dr. John Lilly played an important role, since he investigated the impact of dolphins on disabled people. From 1978 onwards, the therapeutic aid of dolphins was further advanced, especially after the success of Dr Davis Nathanson with Down-syndrome children and Dr. Ludmila Lukina, who developed many therapeutic methods. The therapeutic method is based on the hypersonic stimulus transmitted by the dolphin sonar (a strong organ at the top of the head, which helps dolphins to produce and receive sounds) towards the central nervous system of humans, resulting in the synchronization of brain hemispheres for a certain period (brain waves are concerted in both hemispheres regarding frequency and phase), which is in turn contemplated as neurologically uncommon. The scientific experience of supplementary therapeutic approaches using dolphins reinforces the classical medical therapies and has provided solid proofs of positive effects of such programmes on: autism, Rett syndrome, Down syndrome, mental delay, brain paralysis, depression, cognitive difficulties, hyperkinetic syndrome. Therapeutic centres, such as Dolphin Assisted Therapy, provide various therapeutic programs of different duration, targeting to assist people with a variety of problems. Scientific results documented that dolphins affect the speech during interaction with humans, decrease the depression symptoms and reduce the pain in people suffering from multiple sclerosis. The present work provides up to date data regarding dolphin therapy in a worldwide context. It is actually a thorough literature review, which attempts to cover a gap in the field of therapeutic contribution of animals to human's health.

Dynamics in prevalence of Down syndrome in children with congenital heart disease.

Our findings suggest a growing number of CHD and Down syndrome, which may be the result of improved medical management and progress in educational, social, and financial support. This development is noteworthy as it adds new aspects to present discussions in the media and political settings. What is known: • Congenital heart disease is regarded to be the most important clinical phenomenon in children with Down syndrome, due to its significant impact on morbidity and mortality. • New developments in prenatal diagnostic and therapy management of congenital heart disease continue to influence the number of patients diagnosed with congenital heart disease and Down syndrome. What is New: • This study provides essential data giving the first overview of the dynamics in the prevalence of congenital heart disease and Down syndrome over an extended length of time up to 2015 in a large patient cohort, taking recent developments into account. • Our data suggest a growing prevalence of congenital heart disease and Down syndrome, which may be the result of improved medical management for Down syndrome patients and progress in educational, social, and financial support for their families; this development is noteworthy as it adds new aspects to the present discussion in the media and political settings.

Pedometer-determined physical activity levels of healthy children and children with Down’s syndrome

Purpose Children with Down’s syndrome (DS) are considered sedentary and less engaged in recommended physical activity (PA) levels. This study compared the PA levels between children with DS and healthy children in Saudi Arabia. Methods The study included 85 children divided into two groups. The DS group comprised 37 children with DS aged 8–12 years recruited from the Down Syndrome Charitable Association and Al-Nahda Schools for DS. The healthy group comprised 41 healthy children aged 8–12 years recruited from regular schools in the same region. PA levels were measured over 7 days using a pedometer. Results The healthy group was more active than the DS group (p < 0.05). The total PA steps per minute had significant differences between the DS (M = 7.979, SD = 2.21) and healthy groups (M = 17.512, SD = 3.08; p < 0.05). The daily step count differed significantly on weekdays and weekends between the groups (p < 0.05). Conclusions The DS group had a high body mass index and physical inactivity compared with the second group. Obesity and physical inactivity among Saudi Arabian children with and without DS are major health concerns. Therefore, concerted efforts are needed to combat childhood obesity, promote PA, improve patient quality of life, and reduce the sedentary lifestyle among Saudi children and adolescents.

DNA (cytosine-5)-methyltransferase 3B (DNMT 3B) polymorphism and risk of Down syndrome offspring.

Down syndrome (DS) is the most common form of human genetic mental retardation. Several polymorphisms in genes coding folic acid cycle enzymes have been associated to the risk of bearing a DS child; however, the results are controversial. S-adenosyl-l-methionine (SAM) is an important intermediate of folic acid pathway and acts as methyl donor and substrate for DNA (cytosine-5)-methyltransferase 3B (DNMT3B – EC 2.1.1.37) de novo methylation processes during embryogenesis. Recent studies suggest that a functional polymorphism of DNMT 3B in maternal genotype may be associated with a decreased risk of having a DS child. We herein investigate the association of this polymorphism with the occurrence of DS in a Brazilian population. We have genotyped 111 mothers of DS infants (MDS) and 212 control mothers (CM) through PCR-RFLP. The observed genotypic frequencies were CC = 0.22; CT = 0.49 and TT = 0.29 in CM, and CC = 0.30; CT = 0.52 and TT = 0.18 in MDS. Allelic frequencies were C = 0.47 and T = 0.53 in CM and C = 0.56 and T = 0.44 in MDS. No deviation of HWE was observed, and both DNMT 3B rs2424913 genotype (χ2 = 4.53; DF = 1; P = 0.03) and allelic (χ2 = 4.90; DF = 1; P = 0.03) frequencies show significant differences between MDS and CM. The presence of the mutant DNMT 3B T allele decreases 30% the risk of bearing a DS child (OR = 0.69; 95% CI: 0.50–0.96; P = 0.03), and the risk is diminished up to 45% in association with the homozygous genotype (OR = 0.54; 95% CI: 0.31–0.96; P = 0.04). Our results suggest that women harboring the single nucleotide polymorphism DNMT 3B rs2424913 have a decreased risk of a DS pregnancy, and further studies are necessary to confirm this protective effect.

Accuracy of Parental Perception of Nighttime Breathing in Children with Down Syndrome.

Objective In 2011, the American Academy of Pediatrics published a guideline for children with Down syndrome (DS), recommending a polysomnogram (PSG) by age 4 years regardless of symptoms. Their rationale was based on 2 publications with small cohorts, where at least 50% of the children had no obstructive sleep apnea (OSA) symptoms but their PSG results were abnormal. The American Academy of Otolaryngology-Head and Neck Surgery Foundation published a clinical practice guideline recommending PSG prior to adenotonsillectomy for these children. This study aimed to assess parents' accuracy of their children's breathing patterns as compared with PSGs in a larger cohort of children with DS. Study Design Case series with chart review. Setting Tertiary care academic pediatric hospital. Subjects and Methods Sleep intake forms assessing frequency of parent-observed apnea, snoring, and restless sleep were analyzed. None of the children had a previous tonsillectomy. Two groups were analyzed according to symptoms: infrequent (<3 nights per week on all questions answered) and frequent (≥6 nights per week on at least 1 question). OSA severity was categorized as follows: normal, <2 events per hour; mild, 2 to 4.9; moderate, 5 to 9.9; and severe, ≥10. Results A total of 113 children met inclusion criteria: 34% (n = 38) had infrequent symptoms, and 66% (n = 75) had frequent symptoms. Parents were unable to predict the presence or absence of OSA by nighttime symptoms ( P = .60). The risk of OSA for children with frequent symptoms versus those with infrequent symptoms was 1.04 (95% CI, 0.89-1.3). Conclusion Parents of DS children are unable to predict the presence or absence of OSA by nighttime symptoms, nor are they able to determine its severity.

Correlations between Chronological Ages and Dental Ages on a Group of Children with Down Syndrome

Abstract Background: The number of studies on oral complications in children with Down syndrome is substantial, but they are focused rather on the prevalence of dental caries, periodontal disease, and hypodontia. The relationship between Down syndrome and dental eruption has been rarely approached. The causes of delayed eruption in children with Down syndrome are incompletely elucidated due to the incomplete identification of the factors that intervene in the physiological process of dental eruption. Aim of the study: To evaluate the correlation between Down syndrome and the delayed eruption of permanent teeth, in relation to the chronological age, in this category of patients. Material and methods: The study group included 94 children with mixed dentition, of ages between 6 and 12 years: 36 children with Down syndrome and 58 healthy children. Clinical and radiological examinations were performed, focusing on the relation between dental age and chronological age. Results: The presence of Down syndrome in children has a significant influence (p &lt;0.001) on the delayed eruption of permanent teeth, considering the chronological age, compared to healthy children. The weighted average of this delay in our study group was 1.27. Conclusions: It is necessary to monitor children with Down syndrome for an extended period of time, in order to ensure a high quality of life and to optimize their health as much as possible.

Parenting children with Down syndrome: Societal influences.

Most studies of parenting children with Down syndrome (DS) have been conducted in industrialized countries. They suggest that sensitive communication on the part of professionals, and social support, can lead to acceptance and positive adjustments in the family. This study examined the impact of a diagnosis of DS on Ecuadorian families, in particular at how the diagnosis had been communicated and received, as well as the feelings and experiences which followed. Despite considerable progress in recent years, Ecuador is still marked by discriminatory attitudes which affect children with disabilities and their families, and by the persistence of widespread poverty. This qualitative study, conducted in Cuenca, Ecuador’s third largest city, is based on a focus group discussion and four in-depth interviews with Ecuadorian parents of DS children attending a specialist center in the city. The study shows that, reflecting the effects of status differences and lack of appropriate training, professionals rarely communicate a DS diagnosis in an appropriate manner. Further, it is shown that lack of social support, and the widespread stigmatization confronting children with DS and their families, hinder development of positive and empowering adjustments that would best serve the child’s and the family’s interest.

Declining antibody levels after hepatitis B vaccination in Down syndrome: A need for booster vaccination?

We determined the anti-HBs titer in 227 children of all ages with Down syndrome (DS). Only 48.1% (95%CI: 35.1-61.3) of the DS children aged 7-10 years and 31.9% (95%CI: 22.1-43.6) of the DS children aged >10 years had a protective anti-HBs titer (≥10 IU/L). The geometric mean anti-HBs titer was significantly lower in the DS children; this suggests booster vaccination for HBV may be needed.

Prevalence of pressure equalization tube placement and hearing loss in children with down syndrome

ObjectiveTo determine the prevalence of pressure equalization tube (PET) placement and hearing loss in children with Down syndrome (DS). Material and methodsWe evaluated 90 DS children births between 1 and 11 years old and compared to 90 children without DS paired in sex and age. Medical records were analyzed consecutively. Were collected data about proceedings PET placement, age of the patient at each PET, adenoidectomy, tonsillectomy and results for audiometry and tympanometry. ResultsAmong the 90 patients with DS, 49 (54.4%) were male, median age of 58 months (15–143 months). In this group, 75 PET were placed in 26/90 children (28.9%) mostly between 3 and 5 years old. In 10/26 (38.5%) was necessary PET replaced. When compared to the control group- 6/90 (6.7%)- children with DS presented OR = 13.7 (95% CI 4.0–47.3) times more likely to use PET. Adenoidectomy and tonsillectomy (44.4% and 42.2% respectively) were significantly more frequent in DS group. The prevalence of hearing loss was 32.1% in the right ear and 26.9% in the left ear. Type B timpanometry was found in more than half of the patients with DS. ConclusionWe found a 13-fold higher risk of PET in DS children, especially between the ages of 3–5 years. The high prevalence of hearing loss and PET placement in patients with DS reinforcing the importance of early and regular follow-up for hearing screening in this population, mostly in preschool-aged children.

The effect of adenotonsillectomy on obstructive sleep apnea in children with Down syndrome

Objective: Children with Down syndrome (DS) are liable to develop obstructive sleep apnea (OSA) due to many anatomical airway abnormalities. The tonsils and adenoid occupy part of the airway space, and their removal may be helpful in relieving airway obstruction. The aim of this study was to assess the effectiveness of adenotonsillectomy in the treatment of OSA in those children.Methods: Fifty DS children with difficult breathing were recruited, and they were subjected to polysomnographic examination (PSG). Patients with apnea-hypopnea index (AHI) > 1 were considered to have OSA. Adenotonsillectomy was performed for patients who had OSA and adenotonsillar hypertrophy, and after 3 months PSG was done for them with recording of the same preoperative parameters.Results: Forty-three children demonstrated OSA on PSG, and they were included in the study. The preoperative mean AHI was 9.18 (± 6.17) that improved postoperatively to 2.72 (± 3.80) with its normalization in 72% of patients. Also, significant improvement of arousal index, minimum oxygen saturation, desaturation index, and peak end-tidal CO2 was achieved postoperatively.Conclusion: Adenotonsillectomy is an effective method for the treatment of OSA in children with DS. However, the condition may persist in some children who usually have airway narrowing at multiple levels.

Immunoglobulin G1 and G2 profile in children with Down syndrome

Background and objectives: It is well known that children with Down syndrome (DS) suffer from frequent infections. There is an association of certain IgG subclass abnormalities with the predisposition to recurrent infection of the respiratory tract. Therefore, the study was conducted to determine the immunoglobulin G1 and G2 (IgG1, IgG2) profile in children with DS.Material and methods: Forty children between the ages of 6 months to 12 years with DS (47 XX/XY, +21) attending the Department of Immunology, BIRDEM were enrolled in the study. Age and sex matched 30 healthy normal children with 46 XX/XY were included as control. Enrolled DS and healthy children were divided into two age groups namely 6 months to 6 years and 7 years to 12 years. Serum IgG1 and IgG2 concentrations were determined by enzyme linked immunosorbent assay (ELISA) method.Results: The mean serum IgG1 concentrations of children with DS in both age groups did not differ significantly from that of normal healthy children. But the IgG2 level was significantly less (p&lt;0.003 and p&lt;0.004) in both age groups of children with DS compared to that of control healthy children.Conclusion: The study has demonstrated that the serum IgG2 level was significantly less in children with DS than that of matched normal healthy control children while there was no deficiency of IgG1.IMC J Med Sci 2017; 11(1): 1-4

Salivary secretory IgA concentration and dental caries in children with Down syndrome

To evaluate the relationship between salivary secretory immunoglobulin A (sIgA) concentration and dental caries in children with Down syndrome (DS) and compare it with findings in non-DS children. The sample comprised 61 DS children and 52 non-DS children, aged 6 to 14 years. Caries experience, plaque index (PI), and gingival bleeding index (GBI) were recorded. Saliva samples were collected from all children. Total salivary sIgA concentrations were determined using an enzymatic assay method. Caries experience in primary and permanent dentitions were similar in DS and non-DS children. However, PI and GBI values were significantly lower in DS compared to non-DS children. DS children had higher salivary sIgA concentrations compared to non-DS children. No difference in sIgA concentration was observed between children with and without caries experience in either group. DS children have higher salivary sIgA concentrations than non-DS children. However, this finding did not correlate with caries experience in the study population.

Concentrations of leptin, adiponectin and other metabolic parameters in non-obese children with Down syndrome.

Recent data indicates that adults with Down syndrome (DS) are at increased risk for cardiovascular disease (CVD) that significantly contributes to their morbidity and mortality. Although identification of cardiometabolic risk factors during childhood is desirable to design preventive interventions, the data on such risk factors in children with DS is scarce. The aim of this study was to study the cardiometabolic risk factors such as insulin resistance (IR), leptin and adiponectin concentrations, lipid abnormalities and leptin resistance in non-obese children with DS. This cross-sectional case control study included karyotype confirmed trisomy-21 DS children aged 2-12 years and their matched healthy controls. After detailed anthropometry, weight, height and body mass index (BMI) standard deviation scores (SDSs) were calculated with reference data. Laboratory evaluation included determination of fasting lipid parameters, insulin, glucose, leptin and adiponectin concentrations. The homeostasis model assessment method (HOMA-IR) was used to assess IR and the ratio of leptin to BMI was used as an index of leptin resistance. Seventy-seven children (39 with DS and 38 controls) comprised the study cohort. The anthropometric parameters were similar in the two groups. Children with DS showed significantly higher mean leptin concentrations (2.098±1.68 ng/mL vs. 1.44±0.52 ng/mL, p-value: 0.00) and higher indices of leptin resistance (0.127±0.085 vs. 0.09±0.03, p-value: 0.001) as compared to controls. Fasting adiponectin concentrations were lower (20.64±19.87 ng/mL vs. 32.58±34.25 ng/mL, p-value: 0.21) and fasting glucose higher (89.25±8.12 mg/dL vs. 85.71±5.52 mg/dL, p-value: 0.06) in the DS group as compared to the controls but the differences did not reach statistical significance. The concentrations of insulin, various lipid parameters and calculated HOMA-IR values were similar in the two groups. In the DS group, five children were identified to have high (>75th centile) leptin levels and four as impaired fasting glucose as compared to none in the controls. Alterations of several cardiometabolic risk factors, in particular, leptin concentrations and leptin resistance are present in children with DS. The presence of hyperleptinemia without hyperinsulinemia suggests a probable inherent genetic basis for increased leptin resistance in patients with DS. There is a need for larger studies to further understand increased leptin resistance in DS that may contribute to increased CVD related morbidity and mortality in these patients.

Characterization of Sleep Architecture in Down Syndrome Patients Pre and Post Airway Surgery.

ObjectivesTo define obstructive sleep architecture patterns in Down syndrome (DS) children as well as changes to sleep architecture patterns postoperatively.Study designThe study was a retrospective review.MethodsForty-five pediatric DS patients who underwent airway surgery between 2003 and 2014 at a tertiary children’s hospital for obstructive sleep apnea (OSA) were investigated. Postoperative changes in respiratory parameters and sleep architecture (SA) were assessed and compared to general pediatric normative data using paired t-tests and Wilcoxon signed-rank test.ResultsTwenty-two out of 45 of the participants were male. Thirty participants underwent tonsillectomy and adenoidectomy, four adenoidectomy, 10 tonsillectomy, and one base of tongue reduction. The patients were divided into two groups based on age (<6 years & >6 years) and compared to previously published age matched normative SA data. DS children in both age groups spent significantly less time than controls in rapid eye movement (REM) and N1 (p<0.02). Children younger than six spent significantly less time in N2 than previously published healthy controls (p<0.0001). Children six years of age or older spent more time than controls in N3 (p=0.003). Airway surgery did not significantly alter SA except for an increase in time spent in N1 (p=0.007). Surgery did significantly reduce median apnea hypopnea index (AHI) (p=0.004), obstructive apnea-hypopnea index (OAHI) (p=0.006), hypopneas (p=0.005), total apneas (p<0.001), and central apneas (p=0.02), and increased the lowest oxygen saturation (p=0.028).ConclusionsDS children are a unique population with different SA patterns than the general pediatric population. Airway intervention assists in normalizing both central and obstructive events as well as sleep architecture stages.

Comorbidities in Down syndrome livebirths and health care intervention: an initial experience from the birth defects registry in Southern Thailand.

Down syndrome (DS) is the most common chromosomal disorder causing mental retardation with a worldwide average prevalence of 1-2 cases per 1000 births. This study aimed to determine the comorbidities associated with DS and the coverage of health care services and developmental interventions for DS livebirths in Southern Thailand. A total of 149 livebirth DS infants, recruited through the prospective birth defects registry system during 2009-2013 in 3 provinces in Southern Thailand, were regularly followed-up every 3-6 months. The data collection form included the infants' demographic data, associated congenital anomalies, and developmental interventions. The DS infants were born at an average gestational age of 38.5±2.3 weeks with average birth weight of 2760±478 g, length 48.5±2.2 cm, and head circumference 32.7±1.2 cm. Congenital heart diseases, gastrointestinal defects and congenital hypothyroidism were found in 43.0%, 6.7%, and 12.1% of the cases, respectively. The percentage of DS infants who received developmental interventions in this current study were significantly greater than in a previous study covering the years 1992-2002: early stimulation program 90.0% vs. 65.6% (P<0.01), and speech training program 74.8% vs. 38.9% (P<0.01), respectively, and the infants in our study began intervention programs significantly earlier, 0.58±0.39 years vs. 1.69±0.66 years, respectively. Congenital heart disease was the most common comorbidity associated with DS. The coverage of health care services and developmental interventions for DS children has generally improved in Southern Thailand. One hundred percent coverage of health services and interventions for children with special needs is expected in the near future.

Characteristic of Malocclusion among Saudi Special Need Group Children.

The present study analyzed the characteristics of malocclusions, occlusal traits among Special Health care Needs (SHCN) children with Down syndrome (DS) and autism disorder (AD) in Riyadh City, Kingdom of Saudi Arabia. A total of 100 DS and 100 AD children from five rehabilitation centers in and around Riyadh, Kingdom of Saudi Arabia, were included in the study. Any children with history of ongoing medical treatment, extraction, or orthodontic treatment were excluded from the study. Out of the 200 patients examined, 131 were males and 69 were females and the age of the children ranged from 6 to 14 years. The children were examined for malocclusion characteristics using the Angle's classification of malocclusion, and also other occlusal traits, such as overjet, overbite, cross bite, and open bite were also determined. The data obtained were analyzed using Statistical Package for the Social Sciences, version 16 to generate descriptive statistics for each variable. The analyzed data of the right and left permanent molar relation showed higher incidence of class III malocclusion (66%) in DS children as compared with (3-4%) AD children. The AD children presented with higher percentage of class I malocclu-sion (40-41%) as compared with (10-14%) DS children. During examination of the primary molars, the analyzed data showed that left primary molar had more mesial shift in AD children as compared with DS children. Down syndrome children had high incidence of class III malocclusion and autistic children had high incidence of class I malocclusion. Overall, the DS children were more prone to malocclusion. This study provides database for health professionals in Saudi Arabia in regard to malocclusion of autis-tics and DS patients.

Congenital Visceral Malformations in Children with Down Syndrome (DS) Followed in the Pediatric Unit of the CNHU, Cotonou, Bénin

Introduction: Mortality in Down syndrome (DS) remain high and often in relation with visceral malformations. The aim of this work was to describe the congenital visceral malformations associated with DS in children followed in the pediatric unit of National Health Teaching Hospital of Cotonou, in order to improve their management.Patients and methods: It was a prospective and descriptive survey conducted on children who were treated for DS at the pediatric unit of the National Health Teaching Hospital of Cotonou from October 2015 through July 2016. All children had a diagnosis of DS based on karyotype with trisomy 21 and underwent heart, gastrointestinal and kidney ultrasonographies.Results: A total of 36 children were included. The sex ratio was 1.25 and infants under 12 months were the most represented age group (55.56 %). The mean age at diagnosis was 18.8 months. The majority of mothers were aged than 35 years. Clinical features were dominated by up slanting palpebral fissures and hypertelorism. The visceral malformations were observed only in heart with a frequency of 55.55%. Atrio-ventricular septal defect (27.78%) and patent ductus arteriosus (16.76%) were predominant. Nondisjunction trisomy 21 was found in all children.Conclusion: Congenital visceral malformations associated to DS in children at pediatric unit of the National Health Teaching Hospital of Cotonou were exclusively cardiac with atrioventricular septal defect as major type. Early diagnosis of DS with malformations screening are essential for better management of affected children.