Oranje A.P.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Janmohamed S.R.,Erasmus MC Sophia Childrens Hospital Rotterdam |
De Laat P.C.J.,Erasmus MC Sophia Childrens Hospital
Dermatology | Year: 2011
Background: Haemangioma of infancy (HOI) on the face may be disfiguring and alarming for parents. Usually they are not treated when they are small. Treatment of HOI with propranolol is a breakthrough. Timolol (topical treatment) and propranolol are closely related. Methods: We considered topical treatment with timolol 0.5% ophthalmic solution 3-4 times daily in patients with small HOI. Twenty patients with small mostly superficial HOI were included. Results: A series of 20 patients with HOI treated with timolol 0.5% ophthalmic solution are described. The treatment was effective in all superficial HOIs after 1-4 months. A quick direct inhibitory effect on the growth of the HOI followed by slower regression was observed. The children had to be treated during the whole proliferative phase. Deep HOIs on the nose (2 cases) and lower eyelid (1 case) showed no response. Conclusion: Topical timolol 0.5% ophthalmic solution is effective in HOI. Safety and effectiveness of drugs like topical timolol and topical propranolol require further investigation but they seem very safe when used in small HOIs. We recommend that small superficial HOIs should be treated in an early proliferative phase. Copyright © 2011 S. Karger AG, Basel.
PubMed | Hannover Medical School, Erasmus MC Sophia Childrens Hospital Rotterdam, Dutch Childhood Oncology Group DCOG, Princess Maxima Center for Pediatric Oncology and 3 more.
Type: Journal Article | Journal: Oncotarget | Year: 2016
The most important reason for therapy failure in pediatric acute myeloid leukemia (AML) is relapse. In order to identify miRNAs that contribute to the clonal evolution towards relapse in pediatric AML, miRNA expression profiling of 127 de novo pediatric AML cases were used. In the diagnostic phase, no miRNA signatures could be identified that were predictive for relapse occurrence, in a large pediatric cohort, nor in a nested mixed lineage leukemia (MLL)-rearranged pediatric cohort. AML with MLL- rearrangements are found in 15-20% of all pediatric AML samples, and reveal a relapse rate up to 50% for certain translocation partner subgroups. Therefore, microRNA expression profiling of six paired initial diagnosis-relapse MLL-rearranged pediatric AML samples (test cohort) and additional eight paired initial diagnosis-relapse samples with MLL-rearrangements (validation cohort) was performed. A list of 53 differentially expressed miRNAs was identified of which the miR-106b~25 cluster, located in intron 13 of MCM7, was the most prominent. These differentially expressed miRNAs however could not predict a relapse in de novo AML samples with MLL-rearrangements at diagnosis. Furthermore, higher mRNA expression of both MCM7 and its upstream regulator E2F1 was found in relapse samples with MLL-rearrangements. In conclusion, we identified the miR-106b~25 cluster to be upregulated in relapse pediatric AML with MLL-rearrangements.
Langley G.,University of Witwatersrand |
Schmollgruber S.,University of Witwatersrand |
Fulbrook P.,Australian Catholic University |
Albarran J.W.,University of the West of England |
Latour J.M.,Erasmus MC Sophia Childrens Hospital Rotterdam
Nursing in Critical Care | Year: 2014
Background: Care of patients at the end-of-life (EOL) may be influenced by the experiences, attitudes and beliefs of nurses involved in their direct care. Aim: To investigate South African critical care nurses' experiences and perceptions of EOL care. Design: Cross-sectional survey. Methods: South African critical care nurses completed a modified version of the 'VENICE' survey tool. Data were collected concerning: attitudes towards EOL care; involvement in EOL decision-making; and beliefs about EOL practices. Results: Of 149 surveys distributed, 100 were returned (response rate 67%). Seventy-six percent stated that they had had direct involvement in EOL care of patients, but a minority (29%) had participated in EOL decision-making processes. Whilst most nurses (86%) were committed to family involvement in EOL decisions, less than two thirds (62%) reported this as routine practice. When withdrawing treatment, around half (54%) of the respondents indicated they would decrease the inspired oxygen level to room air, and the majority (84%) recommended giving effective pain relief. Continued nutritional support (84%) and hydration (85%) were advocated, with most nurses (62%) indicating that they were against keeping patients deeply sedated. Most respondents (68%) felt patients should remain in intensive care at the end of life, with the majority (72%) supporting open-visiting, no restriction on number of family members visiting (70%), and the practising of religious or traditional cultural EOL rituals (93%). Conclusions: The involvement of Johannesburg critical nurses in EOL care discussions and decisions is infrequent despite their participation in care delivery and definite views about the process. Relevance to clinical practice: Use of formal guidelines and education is recommended to increase the nurses' involvement in and their confidence in participating in EOL decisions. Educators, managers, senior nurses and other members of the multi-disciplinary team should collaborate to enable critical care nurses to become more involved in EOL care. © 2013 British Association of Critical Care Nurses.
Holzinger D.,University Hospital Muenster |
Holzinger D.,The Interdisciplinary Center |
Frosch M.,University Childrens Hospital Muenster |
Kastrup A.,University Hospital Muenster |
And 14 more authors.
Annals of the Rheumatic Diseases | Year: 2012
Background: Analysis of myeloid-related protein 8 and 14 complex (MRP8/14) serum concentrations is a potential new tool to support the diagnosis of systemiconset juvenile idiopathic arthritis (SJIA) in the presence of fever of unknown origin. Objective: To test the ability of MRP8/14 serum concentrations to monitor disease activity in patients with SJIA and stratify patients at risk of relapse. Methods: Serum concentrations of MRP8/14 in 52 patients with SJIA were determined by a sandwich ELISA. The monitoring of therapeutic regimens targeting interleukin 1 and tumour necrosis factor α, and methotrexate treatment was analysed and diagnostic power to predict flares was tested. Results: MRP8/14 levels were clearly raised in active disease and decreased significantly in response to successful treatments. Serum concentrations of MRP8/14 increased significantly (p<0.001) (mean±95% CI 12.030±3.090 ng/ml) during disease flares compared with patients with inactive disease (864±86 ng/ml). During clinical remission MRP8/14 serum levels of >740 ng/ml predicted disease flares accurately (sensitivity 92%, specificity 88%). MRP8/14 levels correlated well with clinical disease activity, as assessed by physician's global assessment of disease activity (r=0.62), Childhood Health Assessment Questionnaire (r=0.56), active joint count (r=0.46) and with C-reactive protein (r=0.71) and erythrocyte sedimentation rate (r=0.72) (for all p<0.001). Conclusion: MRP8/14 serum concentrations correlate closely with response to drug treatment and disease activity and therefore might be an additional measurement for monitoring anti-inflammatory treatment of individual patients with SJIA. MRP8/14 serum concentrations are the first predictive biomarker indicating subclinical disease activity and stratifying patients at risk of relapse during times of clinically inactive disease.
Van Waas M.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Neggers S.J.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Neggers S.J.,Erasmus Medical Center |
Van Der Lelij A.-J.,Erasmus Medical Center |
And 3 more authors.
Journal of Pediatric Hematology/Oncology | Year: 2010
The number of adult survivors of childhood cancer in the general population has increased. As reports on the prevalence of the metabolic syndrome in adult survivors of childhood cancer are scarce, we reviewed the available literature on the components of the metabolic syndrome in adult survivors of childhood cancer. Although there is a lack of studies estimating the prevalence of metabolic syndrome directly, especially prevalence of insulin resistance, obesity, and dyslipidemia is increased in certain groups. Therefore, adult survivors of childhood cancer are at increased risk of developing cerebrovascular and cardiovascular diseases. Accordingly, it is important to identify the predisposing factors of the metabolic syndrome in cohorts of survivors, to introduce medical interventions, and to subsequently decrease the risk of cerebrovascular and cardiovascular events. Copyright © 2010 by Lippincott Williams & Wilkins.
van Litsenburg R.R.L.,VU University Amsterdam |
Huisman J.,VU University Amsterdam |
Huisman J.,University Utrecht |
Pieters R.,Erasmus Mc Sophia Childrens Hospital Rotterdam |
And 3 more authors.
Supportive Care in Cancer | Year: 2014
Purpose: Improvement in survival of pediatric acute lymphoblastic leukemia (ALL) has increased the attention to quality of life (QoL). QoL is impaired during maintenance treatment, but little is known about QoL during induction therapy. Identification of patients with poor QoL during induction will provide opportunities for early interventions, and may subsequently improve future QoL. This national multi-center study aimed to assess QoL and its determinants during ALL induction treatment.Methods: Proxy reports of the Child Health Questionnaire (CHQ) and the PedsQL cancer version were collected. Child, treatment, and parental characteristics were analyzed as potential determinants in a multiple regression model.Results: One hundred thirty parents of children participated (response rate 82 %), median child age was 5.7 years and 48 % were female. QoL, as measured with the CHQ, was significantly lower than the norm, the effect sizes were large, and the differences were clinically relevant. Physical QoL was more often affected than psychosocial QoL. Regression models could be constructed for 4/ 10 CHQ scales and 6/ 8 PedsQL cancer scales, accounting for 7 to 36 % of the variance in scores. Impaired QoL was most often associated with older children, girls, and time since diagnosis. Also, father respondents seem to have a lower QoL perception compared to mother respondents although this needs to be confirmed in future research.Conclusions: Specific counseling for subsets of patients with a higher risk of low QoL during the early phases of therapy is warranted. © 2014, Springer-Verlag Berlin Heidelberg.
PubMed | University of Sheffield, Erasmus MC Sophia Childrens Hospital and Erasmus MC Sophia Childrens Hospital Rotterdam
Type: Journal Article | Journal: Pediatric pulmonology | Year: 2016
With increasing survival of patients with more severe forms of congenital diaphragmatic hernia (CDH) and risk of long-term respiratory morbidity, studies on lung morphology are needed. We used hyperpolarised (3) He MRI and anatomical (1) H MRI in a cohort of young adult CDH patients to image regional lung ventilation and microstructure, focusing on morphological and micro-structural (alveolar) abnormalities.Nine patients with left-sided CDH, born 1975-1993, were studied. Regional ventilation was imaged with hyperpolarised (3) He MRI, and the (3) He apparent diffusion coefficient (ADC) was computed separately for the ipsilateral and contralateral lungs. (1) H MRI was used to image lung anatomy, total lung volume and motion during free-breathing.(3) He MRI showed ventilation abnormalities in six patients, ranging from a single ipsilateral ventilation defect (3 patients) to multiple ventilation defects in both lungs (one patient treated with extra corporeal membrane oxygenation). In eight patients, (3) He ADC values for the ipsilateral lung were significantly higher than those for the contralateral lung.Functional and micro-structural changes persist into adulthood in most CDH patients. Ipsilateral elevated (3) He ADC values are consistent with enlargement of mean dimensions of the confining lung micro-structure at the alveolar level.
Raets M.M.A.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Sol J.J.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Govaert P.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Govaert P.,Koningin Paola Childrens Hospital |
And 5 more authors.
Radiology | Year: 2013
Purpose: To report the incidence of cerebral sinovenous thrombosis (CSVT) in a prospective cohort of preterm infants with a gestational age of less than 29 weeks. Materials and Methods: The local medical ethics review board approved this study, and written parental consent was obtained. Preterm infants with a gestational age of less than 29 weeks who were admitted to the neonatal intensive care unit were prospectively studied with cranial ultrasonography (US). The scanning protocol included visualization with color Doppler imaging of the superior sagittal sinus and transverse sinuses through the anterior (8.5-MHz probe) and mastoid (13-MHz probe) fontanelles. When feasible, magnetic resonance imaging was performed to confirm cranial US-diagnosed CSVT. The differences between preterm infants with and those without CSVT were analyzed by using Mann-Whitney tests for continuous variables and Fisher exact tests for categorical data. Results: Cranial US was used to document CSVT in 11 of 249 preterm infants with a gestational age of less than 29 weeks. Transverse sinuses were most frequently affected (in all 11 patients with CSVT). All infants with CSVT were asymptomatic. Postnatal age at diagnosis ranged from 5 to 34 days. The mean gestational age was significantly lower in infants with CSVT (25.9 weeks vs 26.8 weeks, P = .038). Of the risk factors studied, only duration of mechanical ventilation was associated with CSVT; it was significantly longer in the CSVT group. Conclusion: Systematic serial cranial US of infants with a gestational age of less than 29 weeks showed a remarkably high incidence of CSVT of 4.4%. Cranial US including color Doppler imaging with scans obtained through the mastoid fontanelle can depict CSVT at an early stage. Treatment of this possibly important condition needs attention. © RSNA, 2013.
van Waas M.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Neggers S.J.C.M.M.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Neggers S.J.C.M.M.,Erasmus University Rotterdam |
Raat H.,Erasmus University Rotterdam |
And 3 more authors.
PLoS ONE | Year: 2012
Background: Reports on metabolic syndrome in nephroblastoma and neuroblastoma survivors are scarce. Aim was to evaluate the occurrence of and the contribution of treatment regimens to the metabolic syndrome. Patients and Methods: In this prospective study 164 subjects participated (67 adult long-term nephroblastoma survivors (28 females), 36 adult long-term neuroblastoma survivors (21 females) and 61 control subjects (28 females)). Controls were recruited cross-sectionally. Waist and hip circumference as well as blood pressure were measured. Body composition and abdominal fat were assessed by dual energy X-ray absorptiometry (DXA-scan). Laboratory measurements included fasting triglyceride, high density lipoprotein-cholesterol (HDL-C), glucose, insulin, low-density lipoprotein-cholesterol (LDL-C) and free fatty acids (FFA) levels. Results: Median age at follow-up was 30 (range 19-51) years in survivors and 32 (range 18-62) years in controls. Median follow-up time in survivors was 26 (6-49) years. Nephroblastoma (OR = 5.2, P<0.0001) and neuroblastoma (OR 6.5, P<0.001) survivors had more components of the metabolic syndrome than controls. Survivors treated with abdominal irradiation had higher blood pressure, triglycerides, LDL-C, FFA and lower waist circumference. The latter can not be regarded as a reliable factor in these survivors as radiation affects the waist circumference. When total fat percentage was used as a surrogate marker of adiposity the metabolic syndrome was three times more frequent in abdominally irradiated survivors (27.5%) than in non-irradiated survivors (9.1%, P = 0.018). Conclusions: Nephroblastoma and neuroblastoma survivors are at increased risk for developing components of metabolic syndrome, especially after abdominal irradiation. We emphasize that survivors treated with abdominal irradiation need alternative adiposity measurements for assessment of metabolic syndrome. © 2012 van Waas et al.
Asche S.S.,Erasmus University Rotterdam |
van Rijn R.M.,Erasmus University Rotterdam |
Bessems J.H.J.M.,Erasmus MC Sophia Childrens Hospital Rotterdam |
Krul M.,Erasmus University Rotterdam |
Bierma-Zeinstra S.M.A.,Erasmus University Rotterdam
Chiropractic and Manual Therapies | Year: 2013
Background: Transient synovitis of the hip (TS) is considered to be a self-limiting disease in childhood. However, because the etiology is unclear and some cases precede Legg-Perthes' disease, data on follow-up are important. Our aim was to summarize the knowledge on the clinical course of TS in children. Methods: The study design was a systematic review and a literature search was conducted in Medline and Embase. Studies describing short and/or long-term follow-up of TS in children were included. Case reports, reviews and studies describing traumatic hip pain were excluded. Study quality was scored and data extraction was performed. The main outcome measures were short-term and long-term clinical course, and recurrence of symptoms. Results: A total of 25 studies were included of which 14 were of high quality. At two-week follow-up, almost all children with TS were symptom free. Those with symptoms persisting for over one month were more prone to develop other hip pathology, such as Legg-Perthes' disease. The recurrence rate of TS ranged from 0-26.3%. At long-term follow-up, 0-10% of the children diagnosed with TS developed Legg-Perthes' disease. Hip pain after intensive physical effort and limited range of motion of the hip at long-term follow-up was reported in 12-28% and in 0-18% of the children, respectively.Conclusions: The majority of the studies indicate that children with TS recover within two weeks; recurrence was seen in 0-26% of the cases. Children with TS should be followed at least six months to increase the likelihood of not missing Legg-Perthes' disease. © 2013 Asche et al.; licensee BioMed Central Ltd.