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Mineral and bone disorder in chronic kidney disease (CKD-MBD) is a triad of biochemical imbalances of calcium, phosphate, parathyroid hormone and vitamin D, bone abnormalities and soft tissue calcification. Maintaining optimal bone health in children with CKD is important to prevent long-term complications, such as fractures, to optimise growth and possibly also to prevent extra-osseous calcification, especially vascular calcification. In this review, we discuss normal bone mineralisation, the pathophysiology of dysregulated homeostasis leading to mineralisation defects in CKD and its clinical consequences. Bone mineralisation is best assessed on bone histology and histomorphometry, but given the rarity with which this is performed, we present an overview of the tools available to clinicians to assess bone mineral density, including serum biomarkers and imaging such as dual-energy X-ray absorptiometry and peripheral quantitative computed tomography. We discuss key studies that have used these techniques, their advantages and disadvantages in childhood CKD and their relationship to biomarkers and bone histomorphometry. Finally, we present recommendations from relevant guidelines-Kidney Disease Improving Global Outcomes and the International Society of Clinical Densitometry-on the use of imaging, biomarkers and bone biopsy in assessing bone mineral density. Given low-level evidence from most paediatric studies, bone imaging and histology remain largely research tools, and current clinical management is guided by serum calcium, phosphate, PTH, vitamin D and alkaline phosphatase levels only., Author(s): A.D. Lalayiannis [sup.1] , N.J. Crabtree [sup.2] , M. Fewtrell [sup.1] , L. Biassoni [sup.1] , D.V. Milford [sup.2] , C.J. Ferro [sup.3] , R. Shroff [sup.1] Author Affiliations: [...]

Measurement of bone mass in children is performed for both clinical and research purposes, generally using bone densitometry. Quantitative ultrasound (QUS) predicts fracture risk in adults and has the advantage [...]

We present the case of a child with a thoracic scoliosis causing respiratory impairment in whom pre-surgical ventilation–perfusion lung scintigraphy in different postures was able to predict improvement in ventilation and perfusion after surgery. [ABSTRACT FROM AUTHOR]

The purpose of these guidelines is to offer to the nuclear medicine team a framework that could prove helpful in daily practice. These guidelines contain information related to the indications, acquisition, processing and interpretation of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) in paediatric oncology. The Oncology Committee of the European Association of Nuclear Medicine (EANM) has published excellent procedure guidelines on tumour imaging with 18F-FDG PET (Bombardieri et al., Eur J Nucl Med Mol Imaging 30:BP115–24, 2003 []. These guidelines, published by the EANM Paediatric Committee, do not intend to compete with the existing guidelines, but rather aim at providing additional information on issues particularly relevant to PET imaging of children with cancer. The guidelines summarize the views of the Paediatric Committee of the European Association of Nuclear Medicine. They should be taken in the context of “good practice” of nuclear medicine and of any national rules, which may apply to nuclear medicine examinations. The recommendations of these guidelines cannot be applied to all patients in all practice settings. The guidelines should not be deemed inclusive of all proper procedures or exclusive of other procedures reasonably directed to obtaining the same results. [ABSTRACT FROM AUTHOR]

Previous studies have shown that high uptake of (18)F-fluoro-2-deoxy-glucose in head and neck cancer, as determined by the standardized uptake value on positron emission tomography scan, was associated with poor survival. The aim of this study was to confirm the association and to establish whether a high standardized uptake value had prognostic significance. Seventy-three consecutive patients with newly diagnosed squamous cell carcinoma of the head and neck underwent a positron emission tomography study before treatment. Age, gender, performance status tumour grade, stage, maximal tumour diameter and standardized uptake value were analyzed for their possible association with survival. The median standardized uptake value for all primary tumours was 7.16 (90% range 2.30 to 18.60). In univariate survival analysis the cumulative survival was decreased as the stage, tumour diameter and standardized uptake value increased. An standardized uptake value of 10 was taken as a cut-off for high and low uptake tumours. When these two groups were compared, an standardized uptake value >10 predicted for significantly worse outcome (P=0.003). Multivariate analysis demonstrated that an standardized uptake value >10 provided prognostic information independent of the tumour stage and diameter (P=0.002). We conclude that high FDG uptake (standardized uptake value>10) on positron emission tomography is an important marker for poor outcome in primary squamous cell carcinoma of the head and neck. Standardized uptake value may be useful in distinguishing those tumours with a more aggressive biological nature and hence identifying patients that require intensive treatment protocols including hyperfractionated radiotherapy and/or chemotherapy. [ABSTRACT FROM AUTHOR]

We undertook this phase II study to evaluate the efficacy and toxicity of epidoxorubicin and ifosfamide in the treatment of locally advanced and/or metastatic soft-tissue sarcomas. We used escalating doses of epidoxorubicin (from 60 to 75 mg/m2) on day 1 and 1.2 g/m2 ifosfamide on days 1-5. Chemotherapy courses were repeated every 3-4 weeks. A total of 16 patients--13 who had not previously been treated and 3 who had undergone prior therapy with anthracyclines--entered the study. In all, 15 patients were evaluable for response and 16, for toxicity. At least two courses of chemotherapy were given. A complete remission (CR) was seen in 1 patient, a partial remission (PR) in 5, and a minor response (MR) in 1, for an objective response rate (CR + PR) of 40% (6/15); this value reached 50% in non-pretreated patients (6/12). Stable disease (SD) was observed in 40% (6/15) of patients. The relative dose intensity of epidoxorubicin ranged from 10 to 23.3 mg/m2 (median, 16.6 mg/m2). The time to objective response ranged from 4 to 12 weeks (median, 8.5 weeks). The duration of response was 4 months for the single CR, and that for the five PRs was 6+ months (range, 4-18 months). Toxicity was evaluated according to WHO criteria in 16 patients; it was mild and consisted mainly of alopecia, nausea and vomiting, and leucopenia. In only three patients did we observe grade 3 leucopenia. In one case an ifosfamide-associated encephalopathy occurred, but it regressed after 24 h. Neither chronic nor acute cardiac toxicity was reported. In this preliminary analysis, the response rate obtained with the combination of epidoxorubicin and ifosfamide was encouraging and the toxicity was acceptable. [ABSTRACT FROM AUTHOR]

Abstract: Dual X-ray absorptiometry (DXA) is increasingly used in a clinical setting to evaluate bone mass in children. Areal Bone Mineral Density (aBMD) measurements are known to be influenced by body size, but there is no consensus on the optimal way to deal with this for individual patients. Aim: : To compare parameters of bone mass with varying degrees of size correction and to determine the effect on the categorisation of patients as normal or abnormal. Subjects and methods: : Healthy children (n = 78) and 4 groups of patients (n = 194) underwent DXA scans of the lumbar spine (L2–4, GE Lunar Prodigy). Five measures of bone mass were derived, all adjusted for age and sex: aBMD, BMAD (BMC/BA 1.5), BMCh (BMC/height3), BMCa (BMC adjusted for BA), BMCt (BMC adjusted for BA and height). SD scores were calculated for each parameter for patients using data from healthy controls. Results: : Compared to healthy children, all patient groups had significantly reduced BMD SD scores (P < 0.001). Mean BMAD, BMCa and BMCt SD scores were significantly lower in only 2/4 patient groups, whilst BMCh SD scores were low only in one group. BMCt showed no advantage over BMCa. The proportion of patients with SD scores <−2 was 27% for aBMD but between 10–13% for BMAD, BMCh and BMCa. Conclusions: : All size-corrected parameters of bone mass performed similarly and classified significantly fewer patients as abnormal than did aBMD. The use of one of these parameters should reduce the number of patients diagnosed inappropriately with ‘low bone mass’. However, without validation against an outcome measure or ‘gold standard’ of bone density or structure, it is not possible to determine which parameter is most correct. [Copyright &y& Elsevier]