Childrens Hospital Erasmus MC Sophia

Rotterdam, Netherlands

Childrens Hospital Erasmus MC Sophia

Rotterdam, Netherlands
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Bakker N.E.,Dutch Growth Research Foundation | Bakker N.E.,Childrens Hospital Erasmus MC Sophia | Lindberg A.,Pfizer | Heissler J.,Pfizer | And 4 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2017

Context: Longitudinal data of children with Prader-Willi syndrome (PWS) treated with genotropin were registered in the Pfizer International Growth Database (KIGS). Objective: To evaluate efficacy and safety of growth hormone (GH) treatment in a large group of children with PWS. Design: Data registered in KIGS from 1987 to 2012. Setting: Worldwide retrospective cohort study. Patients: Patients included 522 prepubertal children treated with GH for three years and 173 children who had reached adult height. Safety analysis included 2332 children. Intervention involved GH treatment. Main outcome measure: Height standard deviation score (SDS), body mass index (BMI) SDS, occurrence of serious adverse events, and deaths reported in KIGS. Results: In prepubertal children, mean (standard deviation) height SDS improved to 20.31 (1.34) (P < 0.05) during three years of GH treatment. In the adolescent group, height SDS improved until the start of puberty to 20.22 (1.31) (P < 0.05) but had a loss of 20.77 (0.81) during puberty, resulting in amean adult height SDS of 21.19 (1.37). Total height gain was 0.95 (1.32) SDS. BMI SDS increased in the prepubertal group from 1.11 (2.09) to 1.53 (1.43) (P < 0.05) and did not significantly change in the adolescent group,who had a BMI SDS at an adult height of 1.78 (1.26). KIGS contained 12 death reports. Conclusions: GH treatment in children with PWS significantly improves linear growth. BMI remains on average below +2 SDS, in contrast to the natural course of increasing obesity in PWS. Safety should be closely monitored in children with PWS, with and without GH treatment. © 2017 Endocrine Society.


Balk-Leurs I.H.,Childrens Hospital Erasmus MC Sophia | Ongkosuwito E.M.,Childrens Hospital Erasmus MC Sophia | Wattel E.,VU University Amsterdam | Prahl-Andersen B.,Childrens Hospital Erasmus MC Sophia
Cleft Palate-Craniofacial Journal | Year: 2014

Purpose: Developing teeth are used to assess maturity and estimate age in a number of disciplines. The purpose of this investigation was to study the dental maturation in children with Crouzon or Apert syndrome compared with nonsyndromic controls.Patients and Methods: Records of 40 children with Crouzon syndrome (18 boys and 22 girls, aged 4.0 to 17.9 years) and 28 children with Apert syndrome (10 boys and 18 girls, aged 3.9 to 15.1 years) were referred to the Department of Orthodontics, Cleft Palate Team and Craniofacial Team, Erasmus MC-Sophia. Data from syndromic children were compared with data from 451 nonsyndromic children (225 boys and 226 girls, aged 2.9 to 16.9 years). From panoramic radiographs, dental maturation was determined for patients with Crouzon and Apert syndromes and compared with data collected from control children. Logistic functions were constructed for dental maturation over time for syndromes and gender.Results: Statistically significant gender differences in dental maturation scores were found for girls with Crouzon (P < .05) and Apert syndrome (P < .05). Patients with Apert syndrome demonstrated a significantly delayed dental maturation (P < .05), while patients with Crouzon syndrome showed a nonsignificant delay.Conclusions: Dental maturation in patients with Apert syndrome was more delayed than in patients with Crouzon syndrome. The delay of tooth formation in patients with Crouzon or Apert syndrome suggests a possible common genetic association. © Copyright 2014 American Cleft Palate-Craniofacial Association.


Bakker N.E.,Dutch Growth Research Foundation | Bakker N.E.,Childrens Hospital Erasmus MC Sophia | Kuppens R.J.,Dutch Growth Research Foundation | Kuppens R.J.,Childrens Hospital Erasmus MC Sophia | And 21 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2015

Context: Longitudinal dataonbonemineral density(BMD)in children and adolescents with Prader-Willi Syndrome (PWS) during long-term GH treatment are not available. Objective: This study aimed to determine effects of long-term GH treatment and puberty on BMD of total body (BMDTB), lumbar spine (BMDLS), and bone mineral apparent density of the lumbar spine (BMADLS) in children with PWS. Design and Setting: This was a prospective longitudinal study of a Dutch PWS cohort. Participants: Seventy-seven children withPWSwhoremained prepubertal duringGHtreatment for 4 years and 64 children with PWS who received GH treatment for 9 years participated in the study. Intervention: The children received GH treatment, 1 mg/m2/day (≅0.035 mg/kg/d). Main Outcome Measures: BMDTB, BMDLS, and BMADLS was measured by using the same dualenergy x-ray absorptiometry machine for all annual measurements. Results: In the prepubertal group, BMDTB standard deviation score (SDS) and BMDLSSDS significantly increased during 4 years of GH treatment whereas BMADLSSDS remained stable. During adolescence, BMDTBSDS and BMADLSSDS decreased significantly, in girls from the age of 11 years and in boys from the ages of 14 and 16 years, respectively, but allBMDparameters remained within the normal range. Higher Tanner stages tended to be associated with lower BMDTBSDS (P =.083) and a significantly lower BMADLSSDS (P=.016). After 9 years of GH treatment, lean body mass SDS was the most powerful predictor of BMDTBSDS and BMDLSSDS in adolescents with PWS. Conclusions: This long-term GH study demonstrates that BMDTB, BMDLS, and BMADLS remain stable in prepubertal children with PWS but decreases during adolescence, parallel to incomplete pubertal development. Based on our findings, clinicians should start sex hormone therapy from the age of 11 years in girls and 14 years in boys unless there is a normal progression of puberty. Copyright © 2015 by the Endocrine Society.


Bakker N.E.,Dutch Growth Research Foundation | Bakker N.E.,Childrens Hospital Erasmus MC Sophia | Kuppens R.J.,Dutch Growth Research Foundation | Kuppens R.J.,Childrens Hospital Erasmus MC Sophia | And 24 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2013

Background: The most important reason for treating children with Prader-Willi syndrome (PWS) with GH is to optimize their body composition. Objectives: The aim of this ongoing study wasto determine whether long-term GH treatment can counteract the clinical course of increasing obesity in PWS by maintaining the improved body composition brought during early treatment. Setting: This was a multicenter prospective cohort study. Methods: We have been following 60 prepubertal children for 8 years of continuous GH treatment (1 mg/m2/d ≡ 0.035 mg/kg/d) and used the same dual-energy x-ray absorptiometry machine for annual measurements of lean body mass and percent fat. Results: After a significant increase during the first year of GH treatment (P <.0001), lean body mass remained stable for 7 years at a level above baseline (P <.0001). After a significant decrease in the first year, percent fat SD score (SDS) and body mass index SDS remained stable at a level not significantly higher than at baseline (P =.06, P =.14, resp.). However, body mass index SDSPWS was significantly lower after 8 years of GH treatment than at baseline (P<.0001). After 8 years of treatment, height SDS and head circumference SDS had completely normalized. IGF-1 SDS increased to +2.36 SDS during the firstyear of treatment (P<.0001) and remained stable sincethen. GH treatment did not adversely affect glucose homeostasis, serum lipids, blood pressure, and bone maturation. Conclusion: This 8-year study demonstrates that GH treatment is a potent force for counteracting the clinical course of obesity in children with PWS. Copyright © 2013 by The Endocrine Society.


Ongkosuwito E.M.,Childrens Hospital Erasmus MC Sophia | Buschang P.H.,Baylor University | V Adrichem L.N.A.,Sophia Childrens Hospital | Prahl-Andersen B.,Childrens Hospital Erasmus MC Sophia
Cleft Palate-Craniofacial Journal | Year: 2013

Objective: Le Fort III osteotomy with distraction osteogenesis (DO) is used to improve the retruded midface in patients with Crouzon or Apert syndrome. This study aimed to evaluate sagittal and vertical preoperative and postoperative cephalometric changes of DO of the midface in patients with Crouzon or Apert syndrome. Design: Population-based case-control study. Patients and Methods: Records of patients with the syndrome of Crouzon (N=6) or Apert (N= 7) were compared, before and after Le Fort III DO, with a nonsyndromic untreated control group (N = 486). Main Outcome Measures: Sagittal and vertical cephalometric maxillary landmarks and measurements were used to predict and measure midface advancement and rotation after Le Fort III DO. Cephalograms were taken before surgery (T0), 4 months after surgery at removal of the distraction device (T1), and 1 year after removal of the distraction device (T2). Analysis: Z scores were performed to compare cephalometric measures of syndromic patients with control subjects. Results: Cephalograms of 13 patients with Crouzon syndrome (N = 6) or Apert (N = 7) (age range 8.2 to 19.8 years) were evaluated. Treatment changes (T1-T2) showed statistically significant maxillary advancement, with no significant differences between the patients with the Crouzon or Apert syndrome. Conclusions: DO of the midface in patients with Crouzon or Apert syndrome seems to be stable in the sagittal direction after follow-up. Although Crouzon and Apert differ after DO, anteroposterior craniofacial dimensions were significantly improved and were closer to patterns of normal subjects. © Copyright 2013 American Cleft Palate-Craniofacial Association.


Van Der Steen M.,Dutch Growth Research Foundation | Van Der Steen M.,Childrens Hospital Erasmus MC Sophia | Lem A.J.,Dutch Growth Research Foundation | Van Der Kaay D.C.M.,Dutch Growth Research Foundation | And 11 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2015

Context: Previously we showed that pubertal children born small for gestational age (SGA) with a poor adult height (AH) expectation can benefit from treatment withGH1 mg/m2 per day (∼0.033 mg/kg/d) in combination with 2 years of GnRH analog (GnRHa) and even more so with a doubleGH dose. GnRHa treatment is thought to have negative effects on body composition and blood pressure. Long-term effects and GH-dose effectsonmetabolic health in children treated with combined GH/GnRHa are unknown. Objective: This study aimed to investigate body composition, blood pressure, and lipid profile during GH treatment, either with or without 2 years of additional GnRHa. To assess whether GH 2 mg/m2 per day (∼0.067 mg/kg/d) results in a similar or even more favorable metabolic health at AH than GH 1 mg/m2 per day. Methods: This was a longitudinal, randomized, dose-response GH trial involving 107 short SGA children (58 girls) treated with GH until AH (GH randomized 1 or 2 mg/m2/d during puberty). Sixty-four children received additional GnRHa. At AH, metabolic parameters were compared between children treated with combined GH/GnRHa and those with only GH. The GH dose effect on metabolic health was evaluated in a subgroup of 47 children who started GH treatment in early puberty (randomized 1 or 2 mg/m2/d) with 2 years of GnRHa. Results: At AH, fat mass percentage (FM%) SD score (SDS), lean body mass (LBM) SDS, blood pressure SDS, and lipid profile were similar between children treated with combined GH/GnRHa and those with only GH. In the pubertal subgroup, FM% SDS was lower during treatment with GH 2 mg/m2 per day. There was no GH dose-dependent effect on LBM SDS, blood pressure, and lipid profile. Conclusions: Combined GH/GnRHa treatment has no long-term negative effects on metabolic health compared with only GH. Started in early puberty, a GH dose of 2 mg/m2 per day results in a similar metabolic health at AH and a more favorable FM% than GH 1 mg/m2 per day. Copyright © 2015 by the Endocrine Society.


Van Sorge A.J.,Leiden University | Termote J.U.M.,University Utrecht | Simonsz H.J.,Childrens Hospital Erasmus MC Sophia | Kerkhoff F.T.,Maxima Medical Center | And 3 more authors.
British Journal of Ophthalmology | Year: 2014

Purpose: Provide insight in natural history, screening and treatment policy of retinopathy of prematurity (ROP) in The Netherlands. Methods: A multicentre, prospective, population-based study (NEDROP) included all preterm infants born in 2009 in The Netherlands fulfilling the inclusion criteria for ROP screening. Anonymised data from ophthalmologists, neonatologists and paediatricians were merged on identification number. Results: Of 2033 reported infants, 1688 (83%) were screened for ROP. ROP stage was reported in 100%, zone in 94.4% and plus disease in 83%. ROP developed in 324 (19.2%), mild ROP (stage 1-2) in 294 (17.4%), severe ROP (stage 3 or more) in 30 (1.8%) and 17 (1%) were treated. The initial screening examination was not performed within the required 42 days in 641 (38%). Date for follow-up was recorded 1973 times and accomplished within 3 days from the planned date in 1957 (99.2%). The chance of not being screened increased from 12.9% without transfer to another hospital to 23.5, 18.5 and 25% after 1, 2, or 3 transfers, respectively. Conclusions: The incidence of severe ROP and infants treated was low. NEDROP emphasises that timing of initial examination and transfer to another hospital are issues of concern within the screening process.

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