Inflammatory activity assessment by F18 FDG-PET/CT in persistent symptomatic sarcoidosis

SummaryBackgroundEstablishing inflammatory activity in sarcoidosis patients with persistent disabling symptoms is important. Whole body F18-FDG PET/CT (PET) appeared to be a sensitive method to detect inflammatory activity in newly diagnosed symptomatic sarcoidosis. The aim was to assess the presence of inflammatory activity using PET in sarcoidosis patients with unexplained persistent disabling symptoms and the association between PET findings and serological inflammatory markers.MethodsSarcoidosis patients who underwent a PET between June 2005 and June 2010 (n = 89), were retrospectively included. All PET scans were examined and positive findings were classified as thoracic and/or extrathoracic. As serological markers of inflammatory activity angiotensine-converting enzyme (ACE), soluble interleukin-2 receptor (sIL-2R), and neopterine were considered.ResultsIn 65/89 (73%) of the studied patients PET was positive, 52 of them (80%) had serological signs of inflammatory activity. In 14/15 patients with a Chest X-ray stage IV PET was positive. In 80% of the PET positive patients extrathoracic inflammatory activity was found. Sensitivity of combined serological inflammatory markers for the presence of inflammatory activity as detected by PET was 80%, specificity 100%, positive predictive value 100%, negative predictive value 65%.ConclusionsThe majority of sarcoidosis patients with persistent disabling symptoms, even those with radiological stage IV, had PET positive findings with remarkably 80% extrathoracic lesions. In 20% PET was positive without signs of serological inflammatory activity. PET appeared to be of additional value to assess inflammatory activity in patients with persistent symptoms in the absence of signs of serological inflammatory activity and to detect extrathoracic lesions

Hormonal protection of spermatogenic stem cells during irradiation: a histological, hormonal and flow cytometric study in rats

Contains fulltext : mmubn000001_007641850.pdf (publisher's version ) (Open Access)Promotores : W. van Daal en R. Rolland131 p

Proposal for the prevention of osteoporosis in paediatric patients with classical galactosaemia.

Decreased bone mass in early childhood is an increasingly recognized problem in classical galactosaemia as in many other chronic diseases. Peak bone mass is reached in late adolescence; thus, increasing peak bone mass in childhood can prevent osteoporosis. Regular bone mass measurements and preventive treatment should begin in childhood. In the absence of evidence-based guidelines for identification and treatment of decreased bone mass in children, we provide a proposal based on our experience and the available literature. Dual-energy x-ray absorptiometry (DXA) should be used for bone mass assessment. Because cooperation is required, measurements can usually be performed from the age of 4 years. Interpretation of bone mass measurements is crucial for the diagnosis of osteopenia or osteoporosis. In children and adolescents, total body bone mineral content (BMC) as well as lean tissue mass (LTM) should be measured. Comparison of BMC corrected for LTM of the patient with the BMC corrected for LTM of healthy controls allows correction for the confounding effect of bone size. DXA should be repeated every two years in case of normal BMC, as this is the time window in which abnormalities become measurable. If BMC is between 0 and -1 SD, lifestyle factors such as physical activity, intake of calcium and vitamins K and D and oestrogen supplementation (in girls) should be optimized. If BMC is below -1 SD, we advise to start with supplementation of calcium, vitamin K(1) and vitamin D(3). DXA should be repeated yearly in case of BMC below 0 SD in order to identify deteriorations and improvements early

Body composition in children with galactosaemia

Body composition in classical galactosaemia has not been studied. Patients with classical galactosaemia, an inherited disorder of galactose metabolism caused by deficiency of galactose-1-phosphate uridyltransferase (GALT, EC 2.7.7.10), might be at risk for an abnormal body composition because of intrinsic factors related to galactosaemia and/or diet-related factors. The aim of this study was to evaluate the body composition of children with classical galactosaemia. The studied population was a previously reported group of classical galactosaemia patients (13 male and 27 female, ages 3-17 years) with decreased height, weight, weight-for-height and insulin-like growth factor-I (IGF-I) Z-scores. Body composition data were obtained by dual-energy X-ray absorptiometry (DXA). In order to correct for height, fat mass (FM) and lean tissue mass (LTM) were divided by squared height. Mid-parental target height Z-scores were assessed and compared to actual height Z-scores. Linear and multiple regression analysis were done to investigate the relationship between body composition and IGF-I, dietary intake and growth data. We found decreased height Z-scores when compared to mid-parental target height Z-scores. Mean scores for FM and LTM (both adjusted for height) were decreased. LTM (adjusted for height) and height Z-score were correlated with IGF-I Z-score. FM (adjusted for height) was correlated with soy intake. No correlation was found between soy intake and IGF-I Z-score. In this limited group of patients, height is decreased and body composition is abnormal. The decreased levels of IGF-I and/or soy nutrition might play a role in these findings

Methylphenidate down-regulates the dopamine receptor and transporter system in children with attention deficit hyperkinetic disorder (ADHD).

Methylphenidate down-regulates the dopamine receptor and transporter system in children with attention deficit hyperkinetic disorder (ADHD). Vles JS, Feron FJ, Hendriksen JG, Jolles J, van Kroonenburgh MJ, Weber WE. Department of Child Neurology, University Hospital Maastricht, Maastricht, The Netherlands. [email protected] Adults suffering from Attention Deficit Hyperactivity Disorder (ADHD) are known to have disturbed central dopaminergic transmission. With Single Photon Emission Computed Tomography (SPECT) we studied brain dopamine transporter and receptor activity in six boys with ADHD. Three months after initiation of treatment with methylphenidate we found a down-regulation of the post-synaptic dopamine receptor with a maximum of 20 % and a down-regulation of the dopamine transporter with a maximum of 74.7 % in the striatal system. This corresponded to a positive clinical response evaluated by neuropsychological questionnaires and tests. We suggest that dopamine transporter imaging by SPECT might be used to monitor psychostimulant treatment in children suffering from ADH

Eosinophilic enterocolitis diagnosed by means of technetium-99m albumin scintigraphy and treated with budesonide (CIR).

Eosinophilic enterocolitis diagnosed by means of technetium-99m albumin scintigraphy and treated with budesonide (CIR). Russel MG, Zeijen RN, Brummer RJ, de Bruine AP, van Kroonenburgh MJ, Stockbrugger RW. Department of Gastroenterology, University Hospital Maastricht, The Netherlands. A patient with a 15 year history of diarrhoea of unknown origin is described. Scintigraphy with technetium-99m labelled albumin suggested albumin loss at the terminal ileum and caecum; subsequent colonoscopic biopsies of these macroscopically normal looking areas showed abundant infiltration with eosinophils. A diagnosis of eosinophilic enterocolitis was made. Treatment with prednisolone had good results, but had to be stopped because of severe side effects. Oral cromoglycate and mesalazine were not effective. Budesonide (CIR), a new topically active corticosteroid with very little systemic effects, was at least as effective as prednisolone without producing side effect

MR imaging, Single-photon emission CT and Neurocognitive performance after mild traumatic brain injury.

BACKGROUND AND PURPOSE: Mild traumatic brain injury (mTBI) (Glasgow Coma Scale = 14-15) is a common neurologic disorder and a common cause of neurocognitive deficits in the young population. Most patients recover fully from mTBI, but 15% to 29% of patients have persistent neurocognitive problems. Although a partially organic origin is considered likely, little brain imaging evidence exists for this assumption. The aims of the present study were to establish the prevalence of posttraumatic lesions in mTBI patients on MR images and to assess the relation between these imaging findings and posttraumatic symptoms, Secondly, we explored the value of early posttraumatic single-photon emission CT (SPECT) for the evaluation of mTBI,METHODS: Twenty-one consecutive patients were included in the study. Patients underwent MR examination, technetium-99m hexamethylpropylene amine oxime SPECT, and neurocognitive assessment within 5 days after injury. Neurocognitive follow-up was conducted 2 and 6 months after injury, and MR imaging was repeated after 6 months. Lesion size and brain atrophy were measured on the MR studies.RESULTS: Twelve (57%) of 21 patients had abnormal MR findings, and 11 (61%) of 18 had abnormal SPECT findings. Patients with abnormal MR or SPECT findings had brain atrophy at follow-up. The mean neurocognitive performance of all subjects was within normal range. There was no difference in neurocognitive performance between patients with normal and abnormal MR findings, Patients with abnormal MR findings only showed significantly slower reaction times during a reaction-time task. Seven patients had persistent neurocognitive complaints and one patient met the criteria for a postconcussional syndrome,CONCLUSION: Brain lesions are common after mTBI; up to 77% of patients may have abnormal findings either on MR images or SPECT scans, and these lesions may lead to brain atrophy, The association between hypoperfusion seen on acute SPECT and brain atrophy after 6 months suggests the possibility of (secondary) ischemic brain damage. There is only a weak correlation between neuroimaging findings and neurocognitive outcome.<br/

Bone metabolism in galactosemia

Bone metabolism in galactosemia. Panis B, Forget PP, van Kroonenburgh MJ, Vermeer C, Menheere PP, Nieman FH, Rubio-Gozalbo ME. Department of Pediatrics, Metabolic Diseases, University Hospital Maastricht, 6202 AZ Maastricht, The Netherlands. Classical galactosemia is an autosomal recessively inherited disorder of galactose metabolism. Treatment consists of life-long dietary restriction of galactose. Despite treatment, long-term complications occur such as a decreased bone mineral density (BMD). A decreased BMD might be the result of either dietary deficiencies secondary to the galactose-restricted diet or unknown intrinsic factors. In this study, 40 children with classical galactosemia (13 males and 27 females, aged 3-17 years) on dietary treatment were included to gain insight in the bone metabolism of galactosemics. We found weight and height Z scores significantly decreased in galactosemics. Mean areal BMD Z scores of lumbar spine and of femoral neck as measured by Dual energy X-ray Absorptiometry (DXA) were -0.6 (P < 0.001) and -0.3 (P = 0.066), respectively. Mean volumetric BMD of the femoral neck was significant lower in galactosemics (P < 0.001). The recommended dietary allowances (RDA) for calcium, magnesium, zinc, vitamin D, and protein were met in all patients. Mean serum levels of calcium, phosphate, magnesium, zinc, 1,25-dihydroxy vitamin D (1,25OHD), parathormone (PTH), 17-beta estradiol, bone alkaline phosphatase (BAP), and under-carboxylated osteocalcin (ucOC) were normal. Serum levels of IGF-1 Z score, carboxylated osteocalcin (cOC), N-terminal telopeptide (NTX), and C-terminal telopeptide (CTX) were significantly lower in galactosemics than in control subjects. The different bone markers were strongly correlated. The low levels of IGF-1 Z score, formation marker cOC, and resorption markers NTX and CTX suggest a decreased bone metabolism in galactosemics