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1.
Rheumatol Int ; 44(8): 1553-1565, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38898306

ABSTRACT

BACKGROUND: Little is known about the provision of work-related support for (self-)employed people with rheumatoid arthritis (RA) or axial spondyloarthritis (axSpA) by healthcare providers (HCPs) or employers. OBJECTIVE: This study aims to explore the experiences of (self-)employed people with RA or axSpA regarding work-related support from HCPs and employers in the Netherlands. METHODS: This cross-sectional study concerned an online survey for (self-)employed people, aged ≥ 16 years and diagnosed with RA or axSpA. The survey focused on experiences with HCPs and employers' work-related support and included questions on sociodemographic factors, health and work characteristics and work-related problems. RESULTS: The survey was completed by 884 participants, 56% with RA and 44% with axSpA, of whom 65% were employed, 8% self-employed and 27% not employed. In total, 95% (589/617) of (self-)employed participants reported work-related problems. Sixty-five percent of employed and 56% of self-employed participants had discussed these work-related problems with rheumatologists and/or other HCPs. Whereas 69% of employees with their employer. Both employed and self-employed participants reported that work-related advices or actions were more often provided by other HCPs (53%) than rheumatologists (29%). Fifty-six percent of employees reported this work-related support by the employer. CONCLUSION: This survey among (self-)employed people with RA or axSpA found that the majority reported work-related problems, but only half of them received any work-related support for these problems. Discussion of work-related problems with HCPs was more often reported by employed than self-employed participants. More attention from especially rheumatologists and other HCPs is important to identify and address work-related problems promptly.


Subject(s)
Arthritis, Rheumatoid , Axial Spondyloarthritis , Employment , Humans , Cross-Sectional Studies , Male , Female , Arthritis, Rheumatoid/psychology , Middle Aged , Adult , Netherlands , Surveys and Questionnaires
2.
J Occup Rehabil ; 2024 Feb 05.
Article in English | MEDLINE | ID: mdl-38316721

ABSTRACT

PURPOSE: Work ability of people with rheumatoid arthritis (RA) and axial spondyloarthritis (axSpA) is reduced, but underexamined as a clinical treatment target. The evidence on vocational interventions indicates that delivery by a single healthcare professional (HCP) may be beneficial. Physiotherapist (PT)-led interventions have potential because PTs are most commonly consulted by RA/axSpA patients in the Netherlands. The aim was to develop a PT-led, vocational intervention for people with RA/axSpA and reduced work ability. METHODS: Mixed-methods design based on the Medical Research Council (MRC) framework for developing and evaluating complex interventions, combining a rapid literature review and six group meetings with: patient representatives (n = 6 and 10), PTs (n = 12), (occupational) HCPs (n = 9), researchers (n = 6) and a feasibility test in patients (n = 4) and PTs (n = 4). RESULTS: An intervention was developed and evaluated. Patient representatives emphasized the importance of PTs' expertise in rheumatic diseases and work ability. The potential for PTs to support patients was confirmed by PTs and HCPs. The feasibility test confirmed adequate feasibility and underlined necessity of training PTs in delivery. The final intervention comprised work-focussed modalities integrated into conventional PT treatment (10-21 sessions over 12 months), including a personalized work-roadmap to guide patients to other professionals, exercise therapy, patient education and optional modalities. CONCLUSION: A mixed-methods design with stakeholder involvement produced a PT-led, vocational intervention for people with RA/axSpA and reduced work ability, tested for feasibility and ready for effectiveness evaluation.

3.
BMC Rheumatol ; 7(1): 31, 2023 Sep 20.
Article in English | MEDLINE | ID: mdl-37730637

ABSTRACT

BACKGROUND: Although reduced work ability is a substantial problem among people with inflammatory arthritis (IA), work ability is an underexposed area in clinical practice. Evidence on vocational interventions in IA is limited, but favourable results of delivery by a physiotherapist (PT) warrant the need for further research. Therefore, we aim to evaluate the (cost-)effectiveness of a multimodal, PT-led, vocational intervention in (self-)employed people with IA compared to usual care. METHODS: This randomized controlled trial will include 140 people with rheumatoid arthritis (RA) or axial spondyloarthritis (axSpA) who are (self-)employed and have reduced work ability (Work Ability Index - Single Item Scale (WAS) ≤ 7/10) and/or RA/axSpA related sick leave (≤ 6 months). Participants will be randomized 1:1 to the intervention or control condition (usual care). The intervention, delivered by primary care PTs, will be personalized to each patient, consisting of 10 to 21 sessions over 12 months. The intervention will be multimodal, comprising of 1) exercise therapy and a physical activity plan, 2) education/self-management support, 3) work-roadmap to guide participants in finding relevant other care, with optionally 4) online self-management course and 5) workplace examination. Assessments will be performed at baseline and after 3, 6, and 12 months. The primary outcome measure of effectiveness is work ability, as measured with the WAS at 12 months. For the cost-effectiveness analysis, the EuroQol (EQ-5D-5L), self-reported healthcare use, sick leave and productivity while at work will be used to estimate the trial based cost-utility from a societal perspective. A process evaluation, including assessments of adherence and treatment fidelity, will be undertaken using the registrations of the PTs and semi-structured interviews at 12 months follow-up in a random sample of the intervention group. DISCUSSION: The results of this study will provide insights in the (cost-)effectiveness of a multimodal, PT-led, vocational intervention in people with IA and a reduced work ability. TRIAL REGISTRATION: This study is registered in the International Clinical Trial Registry Platform (ICTRP) under number NL9343.

4.
Immunooncol Technol ; 15: 100089, 2022 Sep.
Article in English | MEDLINE | ID: mdl-35865122

ABSTRACT

Background: Adoptive cell therapy with peripheral blood T cells expressing transgenic T-cell receptors (TCRs) is an innovative therapeutic approach for solid malignancies. We investigated the safety and feasibility of adoptive transfer of autologous T cells expressing melanoma antigen recognized by T cells 1 (MART-1)-specific TCR, cultured to have less differentiated phenotypes, in patients with metastatic melanoma. Materials and methods: In this phase I/IIa trial, peripheral blood T cells from HLA-A2∗02:01-positive patients with unresectable stage IIIC/IV melanoma expressing MART-1 were selected and stimulated with anti-CD3/CD28 beads, transduced with a modified MART-1(26-35)-specific 1D3 TCR (1D3HMCys) and expanded in interleukin (IL)-7 and IL-15. Patients received a single infusion of transgenic T cells in a dose-escalating manner. Feasibility, safety and objective response rate were assessed. Results: Twelve pretreated metastatic cutaneous (n = 7) and uveal (n = 5) melanoma patients were included. Patient 1 received 4.6 × 109 1D3HMCys T cells and experienced grade 5 toxicity after 9 days. Subsequent patients received 5.0 × 107 [n = 3; cohort (c) 2], 2.5 × 108 (n = 2; c3) and 1.0 × 108 (n = 6; c4) 1D3HMCys T cells. The study was prematurely terminated because of dose-dependent toxicity, concerning skin (10/12), eyes (3/12), ears (4/12) and cytokine release syndrome (5/12), with 7 patients experiencing grade 3-5 toxicity. Partial responses were seen in 2/11 (18%) assessable patients and persistence of 1D3HMCys T cells corresponded to infused cell dose. Conclusions: Production of TCR-modified cells as described leads to highly potent T cells. Partial responses were seen in 18% of patients with dose-dependent 'on-target, off-tumor' toxicity and a maximum tolerated dose of 1.0 × 108 cells.

5.
Prev Med Rep ; 18: 101074, 2020 Jun.
Article in English | MEDLINE | ID: mdl-32226730

ABSTRACT

Drug use during sex ('chemsex') has been associated with sexually transmitted infections (STIs) and mental health harms. Little quantitative evidence exists on the health care needs of MSM practicing chemsex from a patient perspective. This study assessed self-perceived benefits and harms and the needs for professional counselling among MSM practicing chemsex. In 2018, 785 MSM were recruited at nine Dutch STI clinics and 511 (65%) completed the online questionnaire. Chemsex was defined as using cocaine, crystal meth, designer drugs, GHB/GBL, ketamine, speed and/or XTC/MDMA during sex <6 months. Chemsex was reported by 41% (209/511), of whom 23% (48/209) reported a need for professional counselling. The most reported topic to discuss was increasing self-control (52%, 25/48). Most MSM preferred to be counselled by sexual health experts (56%, 27/48). The need for professional counselling was higher among MSM who engaged in chemsex ≥2 times per month (30% vs. 17%, p = 0.03), did not have sex without drugs (sober sex) in the past three months (41% vs. 20%, p = 0.04), experienced disadvantages of chemsex (28% vs. 15%, p = 0.03), had a negative change in their lives due to chemsex (53% vs. 21%, p = 0.002), and/or had an intention to change chemsex behaviours (45% vs. 18%, p < 0.001). Our study shows that almost one in four MSM practicing chemsex expressed a need for professional counselling on chemsex-related issues. STI healthcare providers should assess the need for professional counselling in MSM practicing chemsex, especially in MSM with above mentioned characteristics, such as frequent users.

6.
World J Surg ; 43(5): 1370-1376, 2019 May.
Article in English | MEDLINE | ID: mdl-30719554

ABSTRACT

BACKGROUND: Obesity is becoming a bigger health problem every year. Current research shows that the obesity-related metabolic problems are strongly associated with visceral fat and not subcutaneous fat. Visceral obesity (VO) is associated with a worse postoperative outcome in multiple fields of abdominal surgery. On the other hand, muscle mass is related to better postoperative outcome. In rectal cancer patients, we studied the influence of visceral obesity and muscle mass on postoperative complications. METHODS: The visceral fat area (VFA) and skeletal muscle area (SMA) were determined on preoperative CT scans in 406 patients. The preoperative comorbidity, per-operative outcome and postoperative complications were extracted retrospectively from the patient files. VO was defined as a VFA > 100 cm2. Correlations between body composition, postoperative complications and LOS were studied. RESULTS: In our study, 67% of the patients were classified as visceral obese. Mean body mass index (BMI) was higher in the VO group (26.6 ± 3.5 vs 23.5 ± 2.8; p < 0.001). Visceral obese patients had a higher prevalence of cardiac comorbidity (29% vs 13% p = 0.001), hypertension (36% vs 20% p = 0.002) and diabetes mellitus (16% vs 5% p = 0.002). In addition, VO patients had more operative blood loss (431 vs 310 mL; p = 0.008), longer operating time (166 vs 149 min p = 0.003) and more wound infections (14% vs 8% p = 0.048). Visceral obesity was associated with more complications (OR: 1.63 p = 0.043) and longer LOS (risk estimate: 1.18 p = 0.009). CONCLUSION: VO patients more often had a history of cardiac disease, hypertension and diabetes mellitus. Visceral obesity correlated with a worse outcome after surgery for rectal cancer.


Subject(s)
Body Composition , Obesity, Abdominal/complications , Postoperative Complications/epidemiology , Rectal Neoplasms/surgery , Aged , Female , Humans , Length of Stay , Male , Middle Aged , Rectal Neoplasms/metabolism , Retrospective Studies , Treatment Outcome
7.
Clin Endocrinol (Oxf) ; 86(2): 297-304, 2017 Feb.
Article in English | MEDLINE | ID: mdl-27689944

ABSTRACT

CONTEXT: Patients with Prader-Willi syndrome (PWS) have an increased fat mass and decreased lean body mass. GH-treated young adults with PWS who have attained adult height benefit from continuation of growth hormone (GH) treatment, as GH maintained their improved body composition, whereas fat mass increased during the placebo period. Adults with PWS are predisposed to T2DM and cardiovascular disease. Whether GH affects metabolic health profile of this patient group is unknown. OBJECTIVE: To investigate the effects of GH vs placebo on metabolic health, in young adults with PWS who were GH-treated for many years during childhood and had attained adult height (AH). METHOD: A 2-year, randomized, double-blind, placebo-controlled crossover study with stratification for gender and BMI in 27 young adults with PWS. Intervention with GH (0·67 mg/m2 /day) and placebo, both for 1-year duration. RESULTS: Compared to placebo, GH treatment resulted in similar glucose and insulin levels during oral glucose tolerance test. Only fasting glucose and insulin were slightly higher during GH vs placebo (+0·2 mmol/l and +18·4 pmol/l), although both remained within normal ranges in both phases. Blood pressure and lipid profile were similar after GH vs placebo. At baseline (AH) and during GH, no patients had metabolic syndrome, while 1 developed it during placebo treatment. CONCLUSIONS: Growth hormone treatment has no adverse effects on metabolic health profile. Thus, GH-treated young adults with PWS who have attained AH benefit from continuation of GH treatment without safety concerns regarding metabolic health.


Subject(s)
Human Growth Hormone/therapeutic use , Prader-Willi Syndrome/drug therapy , Adolescent , Blood Glucose/analysis , Cross-Over Studies , Female , Glucose Tolerance Test , Human Growth Hormone/adverse effects , Humans , Insulin/blood , Male , Prader-Willi Syndrome/physiopathology , Young Adult
8.
Orphanet J Rare Dis ; 11(1): 153, 2016 11 16.
Article in English | MEDLINE | ID: mdl-27852283

ABSTRACT

BACKGROUND: Patients with Prader-Willi syndrome (PWS) have a cognitive impairment. Growth hormone (GH) treatment during childhood improves cognitive functioning, while cognition deteriorates in GH-untreated children with PWS. Cessation of GH treatment at attainment of adult height (AH) might deteriorate their GH-induced improved cognition, while continuation might benefit them. We, therefore, investigated the effects of placebo versus GH administration on cognition in young adults with PWS who were GH-treated for many years during childhood and had attained AH. METHOD: Two-year, randomized, double-blind, placebo-controlled cross-over study in 25 young adults with PWS. Cross-over intervention with placebo and GH (0.67 mg/m2/day), both during 1 year. RESULTS: Total (TIQ), verbal (VIQ) and performance IQ (PIQ) did not deteriorate during 1 year of placebo, compared to GH treatment (p > 0.322). Young adults with a lower TIQ had significantly more loss of TIQ points during placebo versus GH, in particular VIQ decreased more in those with a lower VIQ. The effect of placebo versus GH on TIQ, VIQ and PIQ was not different for gender or genotype. CONCLUSIONS: Compared to GH treatment, 1 year of placebo did not deteriorate cognitive functioning of GH-treated young adults with PWS who have attained AH. However, patients with a lower cognitive functioning had more loss in IQ points during placebo versus GH treatment. The reassuring finding that 1 year of placebo does not deteriorate cognitive functioning does, however, not exclude a gradual deterioration of cognitive functioning on the long term. TRIAL REGISTRATION: ISRCTN24648386 , NTR1038 , Dutch Trial Register, www.trialregister.nl . Registered 16 August 2007.


Subject(s)
Cognition/drug effects , Growth Hormone/therapeutic use , Prader-Willi Syndrome/drug therapy , Adolescent , Body Height , Cross-Over Studies , Double-Blind Method , Female , Growth Hormone/administration & dosage , Humans , Male
9.
Obes Surg ; 26(2): 251-6, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26123526

ABSTRACT

BACKGROUND: Patients qualified for gastric bypass surgery have an enlarged and fatty liver. An essential step in gastric bypass surgery is elevation of the left liver lobe to expose the gastroesophageal junction. An enlarged and fatty liver complicates the surgical procedure and increases the risk for laceration of the liver. The aim of our study was to evaluate methods to reduce liver volume in patients prior to gastric bypass surgery. METHODS: A systematic literature search of multiple databases, including PubMed, EMBASE.com, and the Cochrane Library and a hand search of reference lists, was performed. We used the search terms morbid obesity and liver, including their synonyms and controlled terms. Inclusion criteria were as follows: patients with morbid obesity who qualified for bariatric surgery, the use of a preoperative treatment to reduce liver volume, and the use of imaging techniques before and after treatment. RESULTS: In total, 281 patients in 11 different studies were included. Preoperative diets reduced liver size by an average of 14%, alternative methods including nutritional supplements, reduced liver size between 20 and 43%, and an intragastric balloon by 32%. CONCLUSIONS: This review showed that nutritional supplements and intragastric balloon are more effective than low calorie diets in reducing liver volume prior to gastric bypass surgery. However, low calorie diet is the preferable method to reduce liver volume, considering the level of evidence and practical applicability. There is a need for well-designed randomized studies with sufficient power in order to confirm the effectiveness of preoperative methods to reduce liver volume.


Subject(s)
Fatty Liver/therapy , Gastric Bypass , Liver/pathology , Obesity, Morbid/surgery , Caloric Restriction , Dietary Supplements , Fatty Liver/complications , Fatty Liver/pathology , Gastric Balloon , Humans , Obesity, Morbid/complications , Organ Size , Preoperative Care , Weight Loss
10.
J Clin Endocrinol Metab ; 100(8): 3041-9, 2015 Aug.
Article in English | MEDLINE | ID: mdl-26050733

ABSTRACT

CONTEXT: Children with Prader-Willi syndrome (PWS) attain high-serum immunoreactive IGF-1 levels during a standard-dose GH treatment, which leads to concern, but lowering the dose deteriorates their body composition. OBJECTIVE: The objective of the study was to evaluate serum IGF-1, IGF binding protein (IGFBP)-3, and acid-labile subunit (ALS) levels, complex formation, and IGF bioactivity in GH-treated PWS children. DESIGN: This was a cross-sectional study. SETTING: The setting of the study was a Dutch PWS cohort. PARTICIPANTS: Forty GH-treated PWS children compared with 41 age- and sex-matched healthy controls participated in the study. INTERVENTIONS: Interventions included GH treatment (1.0 mg/m(2) · d = ∼0.035 mg/kg · d). MAIN OUTCOME MEASURES: Serum IGF-1, IGFBP-3, and ALS levels, complex formation, and IGF bioactivity by IGF-1 receptor kinase activation assay were measured. RESULTS: Serum IGF-1, IGFBP-3, and ALS levels and IGF-1 to IGFBP-3 ratio were significantly higher in GH-treated PWS children than in healthy controls. The 150-kDa ternary complex formation was, however, also significantly higher than in controls, indicating that most of serum IGF-1 is sequestered in the ternary 150-kDa complex with ALS and IGFBP-3. Young GH-treated PWS children [median (interquartile range) aged 5.2 (4.3-7.2) y] exhibited higher serum IGF bioactivity than controls, but no difference was observed in IGF bioactivity between older GH-treated PWS children, aged 14.9 (13.8-16.2) years, and controls. The proportion of IGF bioactivity of total serum IGF-1 was, however, lower in GH-treated PWS children than in controls. Serum immunoreactive IGF-1 levels did not correlate with IGF bioactivity in GH-treated children with PWS, in contrast to a strong positive correlation in healthy controls. CONCLUSIONS: In GH-treated PWS children, most serum IGF-1 is sequestered in the 150-kDa complex. Higher IGF bioactivity was found only in young GH-treated PWS children and not in the older ones. IGF bioactivity during GH showed a wide variation, and there was a disrupted correlation with immunoreactive IGF-1 levels, which makes immunoreactive IGF-1 levels an inappropriate indicator for GH dosing in PWS children.


Subject(s)
Human Growth Hormone/therapeutic use , Insulin-Like Growth Factor I/metabolism , Prader-Willi Syndrome/drug therapy , Adolescent , Carrier Proteins/metabolism , Case-Control Studies , Child , Child, Preschool , Cross-Sectional Studies , Female , Glycoproteins/metabolism , Humans , Infant , Male , Multiprotein Complexes/metabolism , Netherlands , Prader-Willi Syndrome/metabolism
11.
Endocrine ; 50(3): 633-42, 2015 Dec.
Article in English | MEDLINE | ID: mdl-25989955

ABSTRACT

Prader-Willi syndrome (PWS) is characterized by a switch from failure to thrive to excessive weight gain and hyperphagia in early childhood. Hyperghrelinemia may be involved in the underlying mechanisms of the switch. The purpose of this study is to evaluate acylated ghrelin (AG) and unacylated ghrelin (UAG) levels in PWS and investigate their associations with hyperphagia. This is a cross-sectional clinical study conducted in three PWS expert centers in the Netherlands and France. Levels of AG and UAG and the AG/UAG ratio were determined in 138 patients with PWS (0.2-29.4 years) and compared with 50 age-matched obese subjects (4.3-16.9 years) and 39 healthy controls (0.8-28.6 years). AEBSF was used to inhibit deacylation of AG. As a group, PWS patients had higher AG but similar UAG levels as healthy controls (AG 129.1 vs 82.4 pg/ml, p = 0.016; UAG 135.3 vs 157.3 pg/ml, resp.), resulting in a significantly higher AG/UAG ratio (1.00 vs 0.61, p = 0.001, resp.). Obese subjects had significantly lower AG and UAG levels than PWS and controls (40.3 and 35.3 pg/ml, resp.), but also a high AG/UAG ratio (1.16). The reason for the higher AG/UAG ratio in PWS and obese was, however, completely different, as PWS had a high AG and obese a very low UAG. PWS patients without weight gain or hyperphagia had a similar AG/UAG ratio as age-matched controls, in contrast to those with weight gain and/or hyperphagia who had an elevated AG/UAG ratio. The switch to excessive weight gain in PWS seems to coincide with an increase in the AG/UAG ratio, even prior to the start of hyperphagia.


Subject(s)
Ghrelin/blood , Prader-Willi Syndrome/blood , Acylation , Adolescent , Age Factors , Body Mass Index , Case-Control Studies , Child , Child, Preschool , Cross-Sectional Studies , Eating , Feeding Behavior , Female , Genotype , Humans , Hyperphagia/blood , Insulin-Like Growth Factor I/metabolism , Male , Obesity/blood
12.
Horm Res Paediatr ; 83(5): 321-31, 2015.
Article in English | MEDLINE | ID: mdl-25764996

ABSTRACT

BACKGROUND/AIMS: Dietary management is a difficult but key aspect of care in children with Prader-Willi syndrome (PWS). We therefore investigated the effect of growth hormone (GH) treatment on reported energy intake in children with PWS, in relation with body composition, resting energy expenditure (REE) and hormone levels. METHODS: In a randomized controlled GH trial including 47 children with PWS, we assessed 5-day dietary records and dual-energy X-ray absorptiometry for body composition. REE was calculated by Müller's equation, based on fat mass, fat free mass and gender. RESULTS: Baseline energy intake of children with PWS was lower than normal daily energy requirements (p < 0.001), and decreased with age to 50% in prepubertal children. Energy intake in infants [m/f: 11/8; median (interquartile range [IQR]) age 2.7 years (1.5-3.2)] increased after 1 year of GH treatment (p = 0.008); this tended to be higher in the GH group than in the untreated group (p = 0.07). In prepubertal children [m/f: 14/14; median (IQR) age 6.8 years (5.1-8.1)], the increase in energy intake was higher in the GH group, but this was not different compared to the untreated group. REE was not different between the GH group and the untreated group. Increase in energy intake during 2 years of GH treatment was correlated with lower fat percentage standard deviation scores (p = 0.037) and higher adiponectin levels (p = 0.007). CONCLUSION: Our study demonstrates that parents of children with PWS are very well capable of restricting energy intake up to 50% compared to daily energy requirements for age- and sex-matched healthy children. GH treatment was associated with a slight increase in energy intake, but also improved body composition and adiponectin levels, which suggests a protective effect of GH treatment.


Subject(s)
Body Composition/physiology , Energy Intake/physiology , Energy Metabolism/physiology , Human Growth Hormone/therapeutic use , Prader-Willi Syndrome/drug therapy , Body Composition/drug effects , Child , Child, Preschool , Diet , Energy Intake/drug effects , Energy Metabolism/drug effects , Female , Human Growth Hormone/pharmacology , Humans , Infant , Male , Motor Activity/drug effects , Motor Activity/physiology , Prader-Willi Syndrome/physiopathology , Treatment Outcome
13.
J Clin Endocrinol Metab ; 100(4): 1609-18, 2015 Apr.
Article in English | MEDLINE | ID: mdl-25668198

ABSTRACT

CONTEXT: Longitudinal data on bone mineral 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 with PWS who remained prepubertal during GH treatment 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/m(2)/day (≅ 0.035 mg/kg/d). MAIN OUTCOME MEASURES: BMDTB, BMDLS, and BMADLS was measured by using the same dual-energy 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 all BMD parameters 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.


Subject(s)
Bone Density , Human Growth Hormone/therapeutic use , Prader-Willi Syndrome/drug therapy , Puberty , Adolescent , Body Composition/drug effects , Bone Density/drug effects , Child , Child, Preschool , Female , Gonadal Steroid Hormones/therapeutic use , Humans , Longitudinal Studies , Male , Netherlands , Prader-Willi Syndrome/physiopathology , Puberty/drug effects , Puberty/physiology , Time Factors
14.
J Urol ; 193(1): 291-8, 2015 Jan.
Article in English | MEDLINE | ID: mdl-25109686

ABSTRACT

PURPOSE: Boys with Prader-Willi syndrome often have undescended testes. Prospective studies on the treatment of cryptorchidism in these patients are lacking. We evaluated the effects of human chorionic gonadotropin administration on testis position in 16 males with Prader-Willi syndrome. In patients who subsequently underwent orchiopexy biopsy was taken and testis histology was evaluated. MATERIALS AND METHODS: A total of 16 boys (median age 1.6 years, IQR 1.2 to 1.8) with Prader-Willi syndrome and cryptorchidism underwent human chorionic gonadotropin stimulation test. After a positive test human chorionic gonadotropin treatment was initiated. Patients received 250 to 500 IU (depending on age) intramuscularly twice weekly for 6 weeks. RESULTS: We found 1 testis in a stable scrotal position, 1 vanished testis and 1 atrophic abdominal testis. Of 29 testes responding to human chorionic gonadotropin 23% reached a stable scrotal position, 62% reached a lower position and 14% did not change position. Thus, 22 testes required orchiopexy. Of 17 obtained biopsies in 12 patients 2 showed germ cells in more than 60% of seminiferous tubules, 3 in 30% to 60% and 7 in less than 30%. In addition, 4 boys had Sertoli cell only syndrome and 1 had a vanished testis. In patients undergoing orchiopexy younger age and increased inhibin B and testosterone levels after human chorionic gonadotropin stimulation were associated with a greater number of germ cell containing tubules. CONCLUSIONS: Human chorionic gonadotropin administration resulted in an anatomically lower testis position in most of our patients with Prader-Willi syndrome, and 23% of testes reached a stable scrotal position. Of the cases 76% required orchiopexy to ensure a stable position in the scrotum.


Subject(s)
Chorionic Gonadotropin/therapeutic use , Cryptorchidism/pathology , Cryptorchidism/therapy , Prader-Willi Syndrome/complications , Cryptorchidism/etiology , Humans , Infant , Male , Urologic Surgical Procedures, Male
15.
Eur J Vasc Endovasc Surg ; 46(5): 588-92, 2013 Nov.
Article in English | MEDLINE | ID: mdl-24012465

ABSTRACT

OBJECTIVES: To determine if the duration of wearing compression stockings after endovenous laser ablation (EVLA) of the great saphenous vein (GSV) has influence on pain and quality of life. METHODS: This was a prospective randomized controlled trial. Between December 2006 and February 2008, 109 consecutive patients with EVLA of the GSV were analyzed. Deep vein insufficiency, ulceration, more than one insufficient vein in one leg, and use of anticoagulants were exclusion criteria. Group A used compression stocking for 48 hours after therapy, group B for 7 days. Pain (visual analogue scale [VAS]) and quality of life (SF-36) were analyzed 48 hours, 1 week, and 6 weeks after therapy. Three months after treatment, duplex ultrasound imaging was performed to assess occlusion rates. RESULTS: Both groups (group A, n = 37; group B, n = 32) where comparable at baseline. After 1 week, there was a significant difference in pain (VAS score 3.7 [± 2.1] vs. 2.0 [± 1.1], p ≤ .001), and physical dysfunction (group A, 85.1 [± 11.2] vs. group B, 95.7 [± 10.1]; p < .001) as well as vitality (group A, 75 [± 13.0] vs. group B, 83.7 [± 13.4]; p = .03), all in favor of group B, which disappeared after 6 weeks. After 6 weeks, no significant differences in all endpoints were present. Duplex ultrasound imaging revealed complete GSV occlusion in all patients, while no cases of deep venous thrombosis had developed. CONCLUSIONS: Prescribing compression stockings for longer than 2 days after endovenous GSV ablation (without simultaneous phlebectomies) leads to reduced pain and improved physical function during the first week after treatment.


Subject(s)
Laser Therapy , Saphenous Vein/surgery , Stockings, Compression , Venous Insufficiency/surgery , Adult , Chi-Square Distribution , Humans , Laser Therapy/adverse effects , Middle Aged , Netherlands , Pain Measurement , Pain, Postoperative/etiology , Pain, Postoperative/prevention & control , Predictive Value of Tests , Prospective Studies , Quality of Life , Saphenous Vein/diagnostic imaging , Surveys and Questionnaires , Time Factors , Treatment Outcome , Ultrasonography, Doppler, Duplex , Venous Insufficiency/diagnosis
16.
J Clin Endocrinol Metab ; 98(10): 4013-22, 2013 Oct.
Article in English | MEDLINE | ID: mdl-24001750

ABSTRACT

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 was to 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/m(2)/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 first year of treatment (P < .0001) and remained stable since then. 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.


Subject(s)
Body Composition/drug effects , Human Growth Hormone/therapeutic use , Obesity/drug therapy , Prader-Willi Syndrome/drug therapy , Absorptiometry, Photon , Adolescent , Body Height/drug effects , Bone Density/drug effects , Child , Child, Preschool , Disease Progression , Female , Human Growth Hormone/pharmacology , Humans , Male , Obesity/diagnostic imaging , Prader-Willi Syndrome/diagnostic imaging , Prospective Studies , Treatment Outcome
17.
Med Image Comput Comput Assist Interv ; 13(Pt 1): 526-33, 2010.
Article in English | MEDLINE | ID: mdl-20879271

ABSTRACT

Recently, new techniques for minimally invasive aortic valve implantation have been developed generating a need for planning tools that assess valve anatomy and guidance tools that support implantation under x-ray guidance. Extracting the aortic valve anatomy from CT images is essential for such tools and we present a model-based method for that purpose. In addition, we present a new method for the detection of the coronary ostia that exploits the model-based segmentation and show, how a number of clinical measurements such as diameters and the distances between aortic valve plane and coronary ostia can be derived that are important for procedure planning. Validation results are based on accurate reference annotations of 20 CT images from different patients and leave-one-out tests. They show that model adaptation can be done with a mean surface-to-surface error of 0.5mm. For coronary ostia detection a success rate of 97.5% is achieved. Depending on the measured quantity, the segmentation translates into a root-mean-square error between 0.4 - 1.2mm when comparing clinical measurements derived from automatic segmentation and from reference annotations.


Subject(s)
Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortography/methods , Heart Valve Prosthesis Implantation/methods , Minimally Invasive Surgical Procedures/methods , Pattern Recognition, Automated/methods , Surgery, Computer-Assisted/methods , Computer Simulation , Humans , Models, Cardiovascular , Reproducibility of Results , Sensitivity and Specificity
18.
Ned Tijdschr Geneeskd ; 152(19): 1077-80, 2008 May 10.
Article in Dutch | MEDLINE | ID: mdl-18552058

ABSTRACT

Two patients presented with post-transplant lymphoproliferative disorder (PTLD). PTLD encompasses a broad range ofoften malignant proliferations of lymphoid tissue arising in the immunocompromised host after transplantation. The first patient, a 62-year-old woman, received a bilateral lung transplant due to end-stage emphysema and was diagnosed with PTLD 27 days after transplantation. Treatment consisted of reduction in immunosuppression and administration of rituximab. The PTLD regressed. The second patient, a 57-year-old woman, presented with a massively disseminated PTLD 12 years after kidney transplantation. Immunosuppression was reduced and rituximab was administered, but no response was observed. Despite salvage chemotherapy, the patient died due to progressive disease. These two cases illustrate the heterogeneous presentation of PTLD. The condition is caused by the proliferation of B lymphocytes infected with Epstein-Barr virus (EBV) that are no longer controlled by EBV-specific cytotoxic T lymphocytes, due to the immunosuppressive medication given to prevent transplant rejection. Regression of the lymphoma may be achieved by reducing the immunosuppression or treating with rituximab, which attacks B lymphocytes.


Subject(s)
Antibodies, Monoclonal/therapeutic use , Immunocompromised Host , Immunosuppressive Agents/adverse effects , Lymphoma/etiology , Antibodies, Monoclonal, Murine-Derived , Fatal Outcome , Female , Humans , Immunosuppressive Agents/administration & dosage , Lymphoma/drug therapy , Lymphoma/pathology , Middle Aged , Organ Transplantation , Rituximab , Severity of Illness Index
19.
Postgrad Med J ; 84(988): 93-8, 2008 Feb.
Article in English | MEDLINE | ID: mdl-18322130

ABSTRACT

BACKGROUND: Previous research has shown that sicker patients are less satisfied with their healthcare, but specific effects of adverse health outcomes have not been investigated. The present study aimed to assess whether patients who experience adverse outcomes, in hospital or after discharge, differ in their evaluation of quality of care compared with patients without adverse outcomes. METHOD: In-hospital adverse outcomes were prospectively recorded by surgeons and surgical residents as part of routine care. Four weeks after discharge, patients were interviewed by telephone about the occurrence of post-discharge adverse outcomes, and their overall evaluation of quality of hospital care and specific suggestions for improvements in the healthcare provided. RESULTS: Of 2145 surgical patients admitted to the Leiden University Medical Center in 2003, 1876 (88%) agreed to be interviewed. Overall evaluation was less favourable by patients who experienced post-discharge adverse outcomes only (average 19% lower). These patients were also more often dissatisfied (OR 2.02, 95% CI 1.24 to 3.31) than patients without adverse outcomes, and they more often suggested that improvements were needed in medical care (OR 2.07, 1.45 to 2.95) and that patients were discharged too early (OR 3.26, 1.72 to 6.20). The effect of in-hospital adverse outcomes alone was not statistically significant. Patients with both in-hospital and post-discharge adverse outcomes also found the quality of care to be lower (on average 33% lower) than patients without adverse outcomes. CONCLUSIONS: Post-discharge adverse outcomes negatively influence patients' overall evaluation of quality of care and are perceived as being discharged too early, suggesting that patients need better information at discharge.


Subject(s)
Intraoperative Care/standards , Patient Satisfaction , Postoperative Complications/psychology , Quality of Health Care , Deinstitutionalization/statistics & numerical data , Female , Hospitalization/statistics & numerical data , Humans , Male , Middle Aged , Netherlands , Prospective Studies , Treatment Outcome
20.
Qual Saf Health Care ; 17(1): 47-52, 2008 Feb.
Article in English | MEDLINE | ID: mdl-18245219

ABSTRACT

OBJECTIVE: (1) to estimate the occurrence of postdischarge adverse outcomes in surgical patients and their treatment; (2) to explore determinants during admission that may influence the occurrence of postdischarge adverse outcomes. DESIGN: Four weeks after discharge, patients were contacted by telephone and interviewed about: (1) the occurrence of adverse health outcomes after discharge; (2) their treatment and whether readmission or additional surgery was required. Data on the type of surgery and the occurrence of in-hospital adverse outcomes were taken from the routine reporting system. SETTING: Dutch university hospital Study sample: All 2145 surgical patient admissions in 2003, of whom 1960 (91%) agreed to participate. MAIN OUTCOME MEASURES: Percentage of patient admissions with postdischarge adverse outcomes, by type of treatment. RESULTS: Postdischarge adverse outcomes occurred in 487 patients (25%). Most (76%) of the 554 adverse outcomes were treated by a specialist: 165 (30%) during readmission and 257 (46%) at the outpatient clinic. Postdischarge adverse outcomes were mostly infections (39%). In-hospital adverse outcomes and complex surgical procedures increased the probability for both postdischarge adverse outcomes (odds ratio 1.43 (1.05 to 1.94) and 1.36 (1.02 to 1.82) respectively) and postdischarge adverse outcomes that require readmission (odds ratio 1.59 (1.01 to 2.52) and 1.73 (1.09 to 2.74) respectively). CONCLUSIONS: Patients should be informed at discharge that postdischarge adverse outcomes may occur, in particular if the patient had complex surgical procedures or adverse outcomes during hospitalisation. Since infections were the main type of postdischarge adverse outcomes, more attention should be given on wound treatment by patients and infection prevention after discharge.


Subject(s)
Outcome Assessment, Health Care , Postoperative Complications/epidemiology , Surgery Department, Hospital/statistics & numerical data , Health Services Research , Hospitals, University , Humans , Netherlands , Patient Discharge , Postoperative Complications/therapy , Reoperation/statistics & numerical data
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