Severe Pediatric COVID-19 and Multisystem Inflammatory Syndrome in Children From Wild-type to Population Immunity: A Prospective Multicenter Cohort Study With Real-time Reporting.

BACKGROUND: SARS-CoV-2 variant evolution and increasing immunity altered the impact of pediatric SARS-CoV-2 infection. Public health decision-making relies on accurate and timely reporting of clinical data. METHODS: This international hospital-based multicenter, prospective cohort study with real-time reporting was active from March 2020 to December 2022. We evaluated longitudinal incident rates and risk factors for disease severity. RESULTS: We included 564 hospitalized children with acute COVID-19 (n = 375) or multisystem inflammatory syndrome in children (n = 189) from the Netherlands, Curaçao and Surinam. In COVID-19, 134/375 patients (36%) needed supplemental oxygen therapy and 35 (9.3%) required intensive care treatment. Age above 12 years and preexisting pulmonary conditions were predictors for severe COVID-19. During omicron, hospitalized children had milder disease. During population immunity, the incidence rate of pediatric COVID-19 infection declined for older children but was stable for children below 1 year. The incidence rate of multisystem inflammatory syndrome in children was highest during the delta wave and has decreased rapidly since omicron emerged. Real-time reporting of our data impacted national pediatric SARS-CoV-2 vaccination- and booster-policies. CONCLUSIONS: Our data supports the notion that similar to adults, prior immunity protects against severe sequelae of SARS-CoV-2 infections in children. Real-time reporting of accurate and high-quality data is feasible and impacts clinical and public health decision-making. The reporting framework of our consortium is readily accessible for future SARS-CoV-2 waves and other emerging infections.

Clinical practice - latest insights in optimizing the care of children with Down syndrome.

An essential part of the care of children with Down syndrome is secondary screening for comorbidity. It is well known that comorbidity frequently occurs in these children. A new update of the Dutch Down syndrome medical guideline was developed to create a sound evidence base for several of these conditions. We present the latest insights and recommendations from this Dutch medical guideline which are based on the most relevant literature currently available and developed with rigorous methodology. The main focus of this revision of the guideline was on obstructive sleep apnea and other airway problems and hematologic disorders, such as transient abnormal myelopoiesis, leukemia, and thyroid disorders. Conclusion: This is a short summary of the latest insights and recommendations from the updated Dutch medical guideline for children with Down syndrome.

Mainstream and special school attendance among a Dutch cohort of children with Down Syndrome.

OBJECT: To determine the level of mainstream education in a nationwide cohort of adolescents with Down Syndrome (DS), and to find characteristics related to mainstream or special school attendance. METHOD: Dutch children with DS born in 1992, 1993 or 1994, were assessed when 16-19 years old. Parents scored school enrolment between the age of 4-18 years, general characteristics and the levels of intellectual disability using the Dutch Social Competence Rating Scale. Associations between disability and years in mainstream school were assessed by ordinal logistic regression, adjusting for sex and parental education. RESULTS: We collected data from 170 boys and 152 girls (response 63%); mean age 18.3 years (ranges 16.8-19.9). Intellectual disability was mostly moderate (43%). Most children (74%) entered mainstream education between 4 and 6 years of age. At 13 years 17% was in mainstream school and 7% stayed in up to 16 years. From the age of 8 years onwards the majority was in special education, while 6% never attended school. Girls were more often in mainstream school and stayed in longer. Level of disability was significantly associated with number of years in mainstream education. CONCLUSION: Three out of four Dutch children with DS entered mainstream primary education, however late entry and high dropout are common.

Practical and social skills of 16-19-year-olds with Down syndrome: independence still far away.

Survival of children with Down syndrome (DS) has improved considerably, but insight into their level of daily functioning upon entering adulthood is lacking. We collected cross-sectional data from a Dutch nationwide cohort of 322 DS adolescents aged 16-19 (response 62.8%) to assess the degree to which they master various practical and social skills, using the Dutch Social competence rating scale and the Children's Social Behavior Questionnaire. Up to 60% mastered some of the skills required for independent functioning, such as maintaining adequate standards of personal hygiene and preparing breakfast. Less than 10% had achieved basic skills such as basic cooking and paying in a shop. It is difficult for DS people to master all the skills necessary to live independently. Ninety percent of adolescents with DS experience significant problems in social functioning.

Problem behavior of individuals with Down syndrome in a nationwide cohort assessed in late adolescence.

OBJECTIVE: To assess problem behavior in adolescents with Down syndrome and examine the association with sex and severity of intellectual disability. STUDY DESIGN: Cross-sectional data of a Dutch nationwide cohort of Down syndrome children aged 16-19 years were collected using a written parental questionnaire. Problem behavior was measured using the Child Behavior Checklist and compared with normative data. The degree of intellectual disability was determined using the Dutch Social competence rating scale. RESULTS: The response rate was 62.8% (322/513), and the mean age 18.3 years (SD ± 0.8). The total score for problem behavior was higher in adolescents with Down syndrome than in adolescents without Down syndrome (26.8 vs 16.5; P < .001). Overall, 51% of adolescents with Down syndrome had problem scores in the clinical or borderline range on 1 or more Child Behavior Checklist subscales; this is more than twice as high as adolescents without Down syndrome. Adolescents with Down syndrome had more internalizing problems than their counterparts without Down syndrome (14% and 9%, respectively, in the clinical range); the percentages for externalizing problems were almost equal (7% and 9%, respectively, in the clinical range). The highest problem scores in adolescents with Down syndrome were observed on the social problems and thought problems subscales (large to very large standardized differences). Male sex and/or more severe mental disabilities were associated with more behavioral problems. CONCLUSIONS: Serious problem behavior is more prevalent in adolescents with Down syndrome. This demonstrates the need for a focus on general behavior improvement and on the detection and treatment of specific psychopathology in individuals with Down syndrome.

Management of undescended testis: a decision analysis.

BACKGROUND: Undescended testis (UDT) or cryptorchidism is the most common genital anomaly seen in boys and can be treated surgically by orchidopexy. The age at which orchidopexy should be performed is controversial for both congenital and acquired UDT. METHODS: A decision analysis is performed in which all available knowledge is combined to assess the outcomes of orchidopexy at different ages. RESULTS: Without surgery, unilateral congenital UDT and bilateral congenital UDT are associated with average losses in quality-adjusted life-years (QALYs) of 1.53 QALYs (3% discounting 0.66 QALYs) and 5.23 QALYs (1.91 QALYs), respectively. Surgery reduces this QALY loss to on average 0.84 QALYs (0.21 QALYs) for unilateral UDT and 1.66 QALYs (0.40 QALYs) for bilateral UDT. Surgery at detection will lead to the lowest QALY loss of 0.91 (0.34) and 1.73 (0.60) QALYs, respectively, for unilateral and bilateral acquired UDT compared with surgery during puberty and no surgery. No sensitivity analysis is able to change the preferences for these strategies. CONCLUSIONS: Based on our decision analytic model using societal valuations of health outcomes, surgery for unilateral UDT (both congenital and acquired) yielded the lowest loss in QALYs. Given the modest differences in outcomes, there is room for patient (or parent) preference with respect to the performance and timing of surgery in case of unilateral UDT. For bilateral UDT (both congenital and acquired), orchidopexy at any age provides considerable benefit, in particular through improved fertility. As there is no strong effect of timing, the age at which orchidopexy is performed should be discussed with the parents and the patient. More clinical evidence on issues related to timing may in the future modify these results and hence this advice.

[Youth Healthcare guideline 'Skin disorders']. / Jeugdgezondheidszorgrichtlijn 'Huidafwijkingen'.

There is a high incidence of skin disorders; these are also frequently encountered within Youth Healthcare (YHC). Some skin disorders are caused by an underlying disease, syndrome or child abuse. Therefore, detection of these causes in an early stage is important. Skin disorders can have a huge psychosocial impact on both child and parents. This is one of the reasons why prevention, detection, diagnosis, treatment, referral, and uniform advice and guidance are of great importance. The YHC Guideline examines counselling and advice, criteria for referral to primary or secondary healthcare, and skincare in general. It also describes the disorders that should be actively detected. The Guideline also looks at specific aspects of dark skins and ethnic diversity, and the impact of skin disorders on general wellbeing. The accompanying web-based tool includes argumentation and opinions from experts on more than 75 skin disorders, including illustrations and decision trees, to aid the drawing up of a treatment plan.

Prevalence of overweight in Dutch children with Down syndrome.

OBJECTIVE: Prevalence of overweight in children is increasing, causing various health problems. This study aims to establish growth references for weight and to assess the prevalence rates of overweight and obesity in a nationwide sample of Dutch children with Down syndrome (DS), taking into account the influence of comorbidity. METHODS: In 2009, longitudinal growth data from Dutch children with trisomy 21 who were born after 1982 were retrospectively collected from medical records of 25 Dutch regional specialized DS centers. "Healthy" was defined as not having concomitant disorders or having only a mild congenital heart defect. Weight and BMI references were calculated by using the LMS method, and prevalence rates of overweight and obesity by using cutoff values for BMI as defined by the International Obesity Task Force. Differences in prevalence rates were tested by multilevel logistic regression analyses to adjust for gender and age. RESULTS: Growth data of 1596 children with DS were analyzed. Compared with the general Dutch population, healthy children with DS were more often overweight (25.5% vs 13.3% in boys, and 32.0% vs 14.9% in girls) and obese (4.2% vs 1.8%, and 5.1% vs 2.2%, respectively). Prevalence rates of overweight between DS children with or without concomitant disorders did not vary significantly. CONCLUSIONS: Dutch children with DS have alarmingly high prevalence rates of overweight and obesity during childhood and adolescence. Health care professionals should be aware of the risk of overweight and obesity in children with DS to prevent complications.

Unchanged prevalence of Down syndrome in the Netherlands: results from an 11-year nationwide birth cohort.

OBJECTIVE: This study aims to evaluate trends in prevalence of Down syndrome (DS) births in the Netherlands over an 11-year period and how they have been affected by maternal age and introduction of prenatal screening. METHOD: Nationwide data of an 11-year birth cohort (1997-2007) from the Netherlands Perinatal Registry were analyzed. First-trimester combined screening was introduced in 2002, free of charge only for women 36 years of age or older and only on patients' request. Changes in maternal age, prevalence of DS births, and rates of births at <24 weeks (legal limit for termination of pregnancy in the Netherlands) during the study period were evaluated using logistic and linear regression analyses. RESULTS: In total, 1,972,058 births were registered (91% of the births in 1997-2007). Mean prevalence of DS was 14.57 per 10,000 births (95% confidence interval 14.43; 14.73); 85% of DS were live births. No significant trend in overall prevalence of DS births was observed (p = 0.385), in spite of a significant increase of mean maternal age during the same period (p < 0.001). The increased prevalence of DS births at ≥ 24 weeks among women ≥ 36 years of age (p = 0.011) was offset by a significant increase in the proportion of DS births at <24 weeks among women aged <36 years (p = 0.013). CONCLUSION: The proportion of DS births in the Netherlands has not changed during the period 1997-2007.

Healthy growth in children with Down syndrome.

OBJECTIVE: To provide cross-sectional height and head circumference (HC) references for healthy Dutch children with Down syndrome (DS), while considering the influence of concomitant disorders on their growth, and to compare growth between children with DS and children from the general population. STUDY DESIGN: Longitudinal growth and medical data were retrospectively collected from medical records in 25 of the 30 regional hospital-based outpatient clinics for children with DS in The Netherlands. Children with Trisomy 21 karyotype of Dutch descent born after 1982 were included. The LMS method was applied to fit growth references. RESULTS: We enrolled 1,596 children, and collected 10,558 measurements for height and 1,778 for HC. Children with DS without concomitant disorders (otherwise healthy children) and those suffering only from mild congenital heart defects showed similar growth patterns. The established growth charts, based on all measurements of these two groups, demonstrate the three age periods when height differences between children with and without DS increase: during pregnancy, during the first three years of life, and during puberty. This growth pattern results in a mean final height of 163.4 cm in boys and 151.8 cm in girls (-2.9 standard deviation (SD) and -3.0 SD on general Dutch charts, respectively). Mean HC (0 to 15 months) was 2 SD less than in the general Dutch population. The charts are available at www.tno.nl/growth. CONCLUSIONS: Height and HC references showed that growth retardation in otherwise healthy children with DS meanly occurs in three critical periods of growth, resulting in shorter final stature and smaller HC than the general Dutch population shows. With these references, health care professionals can optimize their preventive care: monitoring growth of individual children with DS optimal, so that growth retarding comorbidities can be identified early, and focusing on the critical age periods to establish ways to optimize growth.

Development, problem behavior, and quality of life in a population based sample of eight-year-old children with Down syndrome.

OBJECTIVE: Children with Down syndrome (DS) have delayed psychomotor development. We investigated levels of development, problem behavior, and Health-Related Quality of Life (HRQoL) in a population sample of Dutch eight-year-old children with DS. Developmental outcomes were compared with normative data of eight-year-old children from the general population. METHOD: Over a three-year-period all parents with an eight-year-old child with DS were approached by the national parent organization. Developmental skills were assessed by means of the McCarthy Scales of Children's Ability. To measure emotional and behavioral problems we used the Child Behavior Checklist. HRQoL was assessed with the TNO-AZL Children's Quality of Life questionnaire. Analyses of variance were applied to compare groups. RESULTS: A total of 337 children participated. Mean developmental age was substantially lower than mean calendar age (3.9 years, SD 0.87 and 8.1 years, SD 0.15 respectively). Mean developmental age was significantly lower among boys than girls (3.6 (SD 0.85) and 4.2 years (SD 0.82) respectively; p<0.001). Compared with the general population, children with DS had more emotional and behavioral problems (p<0.001). However on the anxious/depressed scale, they scored significantly more favorably (p<0.001). Significantly lower HRQoL scores for the scales gross motor skills, autonomy, social functioning and cognitive functioning were found (p-values<0.001). Hardly any differences were observed for the scales physical complaints, positive and negative emotions. CONCLUSION: Eight-year-old children with DS have an average developmental delay of four years, more often have emotional and behavioral problems, and have a less favorable HRQoL compared with children from the general population.