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Jackmann, N., Harila-Saari, A. H., Gustafsson, J. & Frisk, P. (2020). Vitamin D status in children with leukemia, its predictors, and association with outcome. , 67(4), e28163
Open this publication in new window or tab >>Vitamin D status in children with leukemia, its predictors, and association with outcome
2020 (English)In: ISSN 1545-5009, Vol. 67, no 4, p. e28163-Article in journal (Refereed) Epub ahead of print
Abstract [en]

BACKGROUND: Children and adolescents with leukemia are potentially at high risk of vitamin D inadequacy, which may have clinical relevance for skeletal morbidity, infections, and cancer outcome. This study aimed to evaluate vitamin D status at the time of diagnosis to investigate its predictors and association with overall survival in children with leukemia. PROCEDURE: We included all 295 children and adolescents diagnosed with leukemia at our institution between 1990 and 2016 who had available serum sample from the time of diagnosis. We analyzed serum 25-hydroxyvitamin D and parathyroid hormone levels and correlated them with clinical data. RESULTS: The 25-hydroxyvitamin D level was deficient (< 25 nmol/L), insufficient (25-50 nmol/L), sufficient (50-75 nmol/L), and optimal (> 75 nmol/L) in 6.4%, 26.8%, 39.7%, and 27.1% of the children, respectively. Older age and a more recent time of sampling (calendar year) predicted lower 25-hydroxyvitamin D level. In preschool children (age </=6 years), lower 25-hydroxyvitamin D level was also associated with acute myeloid leukemia, and a 25-hydroxyvitamin D level < 50 nmol/L was associated with inferior overall survival. In school-aged children (age > 6 years), the 25-hydroxyvitamin D level showed significant seasonal variation. CONCLUSION: It remains unclear whether vitamin D supplementation in pediatric leukemia patients will improve outcome.

Keywords
adolescents, leukemia, overall survival, vitamin D, pediatric
National Category
Medical and Health Sciences
Research subject
Pediatrics
Identifiers
urn:nbn:se:uu:diva-407995 (URN)10.1002/pbc.28163 (DOI)
Available from: 2020-04-02 Created: 2020-04-02 Last updated: 2020-04-03
Wester Oxelgren, U., Westerlund, J., Myrelid, Å., Annerén, G., Johansson, L., Åberg, M., . . . Frenell, E. (2019). An intervention targeting social, communication and daily activity skills in children and adolescents with Down syndrome and autism: a pilot study. Neuropsychiatric Disease and Treatment, 15, 2049-2056
Open this publication in new window or tab >>An intervention targeting social, communication and daily activity skills in children and adolescents with Down syndrome and autism: a pilot study
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2019 (English)In: Neuropsychiatric Disease and Treatment, ISSN 1176-6328, E-ISSN 1178-2021, Vol. 15, p. 2049-2056Article in journal (Refereed) Published
Abstract [en]

Purpose: To evaluate whether an intervention, targeting deficits in social communication, interaction and restricted activities in children and adolescents with Down syndrome and autism could lead to enhanced participation in family and school activities.

Methods: The intervention included education for parents and school staff about autism, and workshops to identify social-communication and daily living activities that would be meaningful for the child to practice at home and at school. Thereafter, a three-month period of training for the child followed. Outcome measures comprised evaluation of goal achievement for each child, the “Family Strain Index” questionnaire and a visual scale pertaining to the parents´ general opinion about the intervention.

Results: On average, more than 90% of the goals were (to some extent or completely) achieved at home and at school. The mean scores of the “Family Strain Index” were almost identical at the follow-up to those before intervention. The evaluation supported that the use of strategies, intended to facilitate activities and communication, remained largely 18 months after start of the intervention.

Conclusion: Despite the group involved in this study being comprised of older children and adolescents, most of whom had severe and profound intellectual disability, the goal achievements and parents’ views on the intervention were encouraging.

National Category
Pediatrics
Research subject
Pediatrics
Identifiers
urn:nbn:se:uu:diva-381069 (URN)10.2147/NDT.S205721 (DOI)000476857800001 ()31410008 (PubMedID)
Available from: 2019-04-04 Created: 2019-04-04 Last updated: 2019-08-30Bibliographically approved
Wester Oxelgren, U., Åberg, M., Myrelid, Å., Annerén, G., Westerlund, J., Gustafsson, J. & Fernell, E. (2019). Autism needs to be considered in children with Down syndrome. Acta Paediatrica, 108(11), 2019-2026
Open this publication in new window or tab >>Autism needs to be considered in children with Down syndrome
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2019 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 11, p. 2019-2026Article in journal (Refereed) Published
Abstract [en]

Aim: To compare levels and profiles of autistic symptoms in children with Down syndrome (DS) with diagnosed autism spectrum disorder (ASD), with those with DS without ASD and with children with idiopathic autism.

Methods From a population-based cohort of 60 children with DS (age 5-17 years) with 41 participating, those with ASD were compared to those without ASD using the scores obtained with the Autism Diagnostic Observation Schedule (ADOS) Module-1 algorithm.

Results: Children with both DS and ASD had significantly higher ADOS scores in all domains compared to those without ASD. When the groups with DS, with and without ASD, were restricted to those with severe intellectual disability (ID), the difference remained. When the children with DS and ASD were compared with a group with idiopathic autism, the ADOS profile was broadly similar.

Conclusion: A considerable proportion of children with DS, exhibit autism in addition to severe ID. In addition, there is also a group of children with DS and severe ID, but without autism. There is a need to increase awareness of the high prevalence of autism in children with DS. Recognizing the prevalence of autism is important for the appropriate diagnosis and care of children with DS.

National Category
Psychiatry
Research subject
Pediatrics
Identifiers
urn:nbn:se:uu:diva-381066 (URN)10.1111/apa.14850 (DOI)000489595000013 ()31090964 (PubMedID)
Available from: 2019-04-04 Created: 2019-04-04 Last updated: 2020-02-21Bibliographically approved
Decker, R., Albertsson-Wikland, K., Kriström, B., Halldin, M., Gustafsson, J., Nilsson, N.-Ö. & Dahlgren, J. (2019). GH Dose Reduction Maintains Normal Prepubertal Height Velocity After Initial Catch-Up Growth in Short Children. Journal of Clinical Endocrinology and Metabolism, 104(3), 835-844
Open this publication in new window or tab >>GH Dose Reduction Maintains Normal Prepubertal Height Velocity After Initial Catch-Up Growth in Short Children
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2019 (English)In: Journal of Clinical Endocrinology and Metabolism, ISSN 0021-972X, E-ISSN 1945-7197, Vol. 104, no 3, p. 835-844Article in journal (Refereed) Published
Abstract [en]

Context: GH responsiveness guides GH dosing during the catch-up growth (CUG) period; however, little is known regarding GH dosing during the prepubertal maintenance treatment period.

Objective: To evaluate whether SD score (SDS) channel parallel growth with normal height velocity can be maintained after CUG by reducing the GH dose by 50% in children receiving doses individualized according to estimated GH responsiveness during the catch-up period.

Design and Settings: Prepubertal children (n = 98; 72 boys) receiving GH during CUG (GH deficient, n = 33; non-GH deficient, n = 65), were randomized after 2 to 3 years to either a 50% reduced individualized dose (GHRID; n = 27; 20 boys) or unchanged individualized dose (GHUID; n = 38; 27 boys). Another 33 children (25 boys) continued a standard weight-based dose [43 μg/kg/d (GHFIX)].

Main Outcome Measures: The primary endpoint was the proportion of children with ΔheightSDS within ±0.3 at 1 year after GH dose reduction compared with two control groups: GHUID and GHFIX. The hypothesis was that heightSDS could be maintained within ±0.3 with a reduced individualized GH dose.

Results: For the intention-to-treat population at 1 year, 85% of the GHRIDgroup maintained ΔheightSDS within ±0.3 vs 41% in the GHUIDgroup (P = 0.0055) and 48% in the GHFIXgroup (P = 0.0047). The ΔIGF-ISDS in the GHRID group was -0.75 ± 1.0 at 3 months (P = 0.003) and -0.72 ± 1.2 at 1 year compared with the GHUID group (0.15 ± 1.2; P = 0.005) and GHFIX group (0.05 ± 1.0; P = 0.02).

Conclusions: Channel parallel growth (i.e., normal height velocity) and IGF-ISDS levels within ± 2 were maintained after completed CUG using a 50% lower individualized dose than that used during the CUG period.

National Category
Pediatrics Endocrinology and Diabetes
Identifiers
urn:nbn:se:uu:diva-381823 (URN)10.1210/jc.2018-01006 (DOI)000462737000034 ()30339244 (PubMedID)
Funder
Swedish Research Council, 522-2005-7238Swedish Research Council, 7509
Available from: 2019-04-18 Created: 2019-04-18 Last updated: 2019-04-18Bibliographically approved
Ostman, J. R., Mullner, E., Eriksson, J., Kristinsson, H., Gustafsson, J., Witthoft, C., . . . Moazzami, A. A. (2019). Glucose Appearance Rate Rather than the Blood Glucose Concentrations Explains Differences in Postprandial Insulin Responses between Wholemeal Rye and Refined Wheat Breads-Results from A Cross-Over Meal Study. Molecular Nutrition & Food Research, 63(7), Article ID 1800959.
Open this publication in new window or tab >>Glucose Appearance Rate Rather than the Blood Glucose Concentrations Explains Differences in Postprandial Insulin Responses between Wholemeal Rye and Refined Wheat Breads-Results from A Cross-Over Meal Study
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2019 (English)In: Molecular Nutrition & Food Research, ISSN 1613-4125, E-ISSN 1613-4133, Vol. 63, no 7, article id 1800959Article in journal (Refereed) Published
Abstract [en]

Scope Ingestion of rye bread leads to lower postprandial plasma insulin concentrations than wheat bread ingestion, but most often not too different glucose profiles. The mechanism behind this discrepancy is still largely unknown. This study investigates whether glucose kinetics may explain the observed discrepancy. Methods and results Nine healthy men participated in a crossover study, eating 50 g of available carbohydrates as either refined wheat (WB) or traditional wholemeal rye bread (WMR) during d-[6,6-H-2(2)]glucose infusion. Labeled glucose enrichment is measured by an HPLC-TOF-MS method. The calculated rate of glucose appearance (RaE) is significantly lower after ingestion of WMR during the initial 15 min postprandial period. Additionally, the 0-90 min RaE area under the curve (AUC) is significantly lower after ingestion of WMR, as is plasma gastric inhibitory polypeptide (GIP) at 60 and 90 min. Postprandial glycemic responses do not differ between the breads. Postprandial insulin is lower after ingestion of WMR at 45 and 60 min, as is the 0-90 min AUC. Conclusion Ingestion of WMR elicits a lower rate of glucose appearance into the bloodstream compared with WB. This may explain the lower insulin response observed after rye bread ingestion, commonly known as the rye factor.

Keywords
glucose flux, rye factor, stable isotope labeled glucose, time-of-flight mass spectrometry, wholemeal rye bread
National Category
Nutrition and Dietetics
Identifiers
urn:nbn:se:uu:diva-383481 (URN)10.1002/mnfr.201800959 (DOI)000466411200004 ()30636184 (PubMedID)
Funder
Swedish Research Council Formas
Available from: 2019-05-16 Created: 2019-05-16 Last updated: 2019-05-16Bibliographically approved
Wahlström Johnsson, I., Ahlsson, F. & Gustafsson, J. (2019). High birthweight was not associated with altered body composition or impaired glucose tolerance in adulthood. Acta Paediatrica, 108(12), 2208-2213
Open this publication in new window or tab >>High birthweight was not associated with altered body composition or impaired glucose tolerance in adulthood
2019 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 12, p. 2208-2213Article in journal (Refereed) Published
Abstract [en]

Aim

To investigate whether a high birthweight was associated with an increased proportion of body fat or with impaired glucose tolerance in adulthood.

Methods

Our cohort comprised 27 subjects with birthweights of 4500 g or more, and 27 controls with birthweights within ±1 standard deviation scores, born at Uppsala University Hospital 1975‐1979. The subjects were 34‐40 years old at the time of study. Anthropometric data was collected, and data on body composition was obtained by air plethysmography and bioimpedance and was estimated with a three‐compartment model. Indirect calorimetry, blood sampling for fasting insulin and glucose as well as a 75 g oral glucose tolerance test were also performed. Insulin sensitivity was assessed using homoeostasis model assessment 2 and Matsuda index.

Results

There were no differences in body mass index, body composition or insulin sensitivity between subjects with a high birthweight and controls.

Conclusion

In this cohort of adult subjects, although limited in size, those born with a moderately high birthweight did not differ from those with birthweights within ±1 standard deviation scores, regarding body composition or glucose tolerance.

Keywords
body composition, glucose tolerance, high birthweight, insulin sensitivity, obesity
National Category
Pediatrics
Identifiers
urn:nbn:se:uu:diva-398820 (URN)10.1111/apa.14928 (DOI)000479894000001 ()31295357 (PubMedID)
Funder
Novo Nordisk
Available from: 2019-12-13 Created: 2019-12-13 Last updated: 2019-12-13Bibliographically approved
Gustafsson, J. (2019). Metabolic syndrome in children. Acta Paediatrica, 108(3), 394-395
Open this publication in new window or tab >>Metabolic syndrome in children
2019 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 3, p. 394-395Article in journal, Editorial material (Other academic) Published
Place, publisher, year, edition, pages
John Wiley & Sons, 2019
National Category
Pediatrics
Identifiers
urn:nbn:se:uu:diva-378232 (URN)10.1111/apa.14677 (DOI)000458391200002 ()30576010 (PubMedID)
Available from: 2019-03-15 Created: 2019-03-15 Last updated: 2019-03-15Bibliographically approved
Wester Oxelgren, U., Myrelid, Å., Annerén, G., Westerlund, J., Gustafsson, J. & Fernell, E. (2019). More severe intellectual disability found in teenagers compared to younger children with Down syndrome.. Acta Paediatrica, 108(5), 961-966
Open this publication in new window or tab >>More severe intellectual disability found in teenagers compared to younger children with Down syndrome.
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2019 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 5, p. 961-966Article in journal (Refereed) Published
Abstract [en]

AIM: We investigated the severities and profiles of intellectual disability (ID) in a population-based group of children with Down syndrome and related the findings to coexisting autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD).

METHODS: There were about 100 children with Down syndrome living in Uppsala County, Sweden, at the time of the study who all received medical services from the same specialist outpatient clinic. The 60 children (68% male) were aged 5-17 years at inclusion: 41 were assessed within the study and 19 had test results from previous assessments, performed within three years before inclusion. We compared two age groups: 5-12 and 13-18 years old.

RESULTS: Of the 60 children, 49 were assessed with a cognitive test and the 11 children who could not participate in formal tests had clinical assessments. Mild ID was found in 9% of the older children and in 35% of the younger children. Severe ID was found in 91% of the older children and 65% of the younger children. Verbal and nonverbal domains did not differ.

CONCLUSION: Intellectual level was lower in the older children and patients with Down syndrome need to be followed during childhood with regard to their ID levels.

Keywords
Attention deficit hyperactivity disorder, Autism spectrum disorder, Cognitive profile, Down syndrome, Intellectual disability
National Category
Pediatrics
Identifiers
urn:nbn:se:uu:diva-380997 (URN)10.1111/apa.14624 (DOI)000465091200027 ()30372566 (PubMedID)
Available from: 2019-04-03 Created: 2019-04-03 Last updated: 2019-05-14Bibliographically approved
Johnsson, I. W., Naessén, T., Ahlsson, F. & Gustafsson, J. (2018). High birth weight was associated with increased radial artery intima thickness but not with other investigated cardiovascular risk factors in adulthood. Acta Paediatrica, 12, 2152-2157
Open this publication in new window or tab >>High birth weight was associated with increased radial artery intima thickness but not with other investigated cardiovascular risk factors in adulthood
2018 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 12, p. 2152-2157Article in journal (Refereed) Published
Abstract [en]

AIM: This study investigated whether a high birth weight was associated with increased risk factors for cardiovascular disease when Swedish adults reached 34-40.

METHODS: We studied 27 subjects born at Uppsala University Hospital in 1975-1979, weighing at least 4500 g, and compared them with 27 controls selected by the Swedish National Board of Welfare with birth weights within ±1 standard deviations scores and similar ages and gender. The study included body mass index (BMI), blood pressure, lipid profile, haemoglobin A1c (HbA1c), C-reactive protein (CRP) and high-frequency ultrasound measurements of intima-media thickness, intima thickness (IT) and intima:media ratio of the carotid and radial arteries.

RESULTS: Subjects with a high birth weight did not differ from controls with regard to BMI, blood pressure, lipid profile, high-sensitivity CRP, HbA1c or carotid artery wall dimensions. However, their radial artery intima thickness was 37% greater than the control group and their intima:media ratio was 44% higher.

CONCLUSION: Our findings indicate that a high birth weight was associated with increased radial artery intima thickness, but not with other investigated cardiovascular risk factors, at 34-40 years of age. The clinical implications of these findings should be investigated further, especially in subjects born with a very high birth weight.

Keywords
Cardiovascular risk factors, High birth weight, Intima thickness, Intima:media ratio, Large for gestational age
National Category
Pediatrics Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:uu:diva-364690 (URN)10.1111/apa.14414 (DOI)000449638100022 ()29791055 (PubMedID)
Funder
Novo Nordisk
Available from: 2018-10-31 Created: 2018-10-31 Last updated: 2019-01-22Bibliographically approved
Gyllenhammar, I., Diderholm, B., Gustafsson, J., Berger, U., Ridefelt, P., Benskin, J. P., . . . Glynn, A. (2018). Perfluoroalkyl acid levels in first-time mothers in relation to offspring weight gain and growth. Environment International, 111, 191-199
Open this publication in new window or tab >>Perfluoroalkyl acid levels in first-time mothers in relation to offspring weight gain and growth
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2018 (English)In: Environment International, ISSN 0160-4120, E-ISSN 1873-6750, Vol. 111, p. 191-199Article in journal (Refereed) Published
Abstract [en]

We investigated if maternal body burdens of perfluoroalkyl acids (PFAAs) at the time of delivery are associated with birth outcome and if early life exposure (in utero/nursing) is associated with early childhood growth and weight gain. Maternal PFAA body burdens were estimated by analysis of serum samples from mothers living in Uppsala County, Sweden (POPUP), sampled three weeks after delivery between 1996 and 2011. Data on child length and weight were collected from medical records and converted into standard deviation scores (SDS). Multiple linear regression models with appropriate covariates were used to analyze associations between maternal PFAA levels and birth outcomes (n=381). After birth Generalized Least Squares models were used to analyze associations between maternal PFAA and child growth (n=200). Inverse associations were found between maternal levels of perfluorononanoic acid (PFNA), perfluorodecanoic acid (PFDA), and perfluoroundecanoic acid (PFUnDA), and birth weight SDS with a change of -0.10 to -0.18 weight SDS for an inter-quartile range (IQR) increase in ng/g PFAA. After birth, weight and length SDS were not significantly associated with maternal PFAA. However, BMI SDS was significantly associated with PFOA, PFNA, and PFHxS at 3 and 4years of age, and with PFOS at 4 and 5years of age. If causal, these associations suggest that PFAA affects fetal and childhood body development in different directions.

National Category
Clinical Medicine
Identifiers
urn:nbn:se:uu:diva-343486 (URN)10.1016/j.envint.2017.12.002 (DOI)000423441500020 ()29223808 (PubMedID)
Funder
Swedish Environmental Protection Agency, 219 1202Swedish Research Council Formas, 2010-1300Swedish Civil Contingencies Agency, dnr 1637/2012
Available from: 2018-02-27 Created: 2018-02-27 Last updated: 2018-03-07Bibliographically approved
Organisations
Identifiers
ORCID iD: ORCID iD iconorcid.org/0000-0002-7540-438x

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