Acrophyseal growth arrest in a long-term survivor of acute lymphoblastic leukemia

Growth arrest at the secondary growth plate, also known as the acrophysis, is a rare phenomenon with only very few known published case reports. We report on a case of formation of ghost secondary ossification centers at the acrophyses of the knee joint in a 14-year-old female, who survived early childhood acute lymphoblastic leukemia. The patient suffered from severe side effects from both disease and subsequent treatment strategies with a 10-month immobilization period as a consequence at the age of 3 years. The ghost secondary ossification centers were encountered on radiographs and MRI 10 years later, when she presented for evaluation of chronic pain in her left knee related to sports activities, due to a meniscal cyst. Awareness of this phenomenon is nevertheless important, because it seems that endochondral bone growth recovery at the acrophyses might be different from recovery in physes, because we found no concomitant sequelae of growth arrest in the metaphyses

High fatigue scores before and after surgical treatment of bone and soft tissue tumors

Item does not contain fulltextThe first objective of the present study was to investigate fatigue severity in patients diagnosed with bone and soft tissue tumors prior to the surgical treatment of the tumor and 6 months post-operatively. The second objective was to determine which variables are associated with severe fatigue. Patients diagnosed with benign or low-grade malignant bone and soft tissue tumors, undergoing surgical therapy for the tumor only, were included in this study. The control group contained patients scheduled for knee arthroscopy for suspected meniscus tears. Fatigue, pain, anxiety and self-efficacy were measured pre-operatively and after 6 months and each patient wore an actometer to quantify physical activity. In the tumor group of 43 patients, 35% were severely fatigued pre-operatively and 33% post-operatively. The tumor group reported a significantly higher level of anxiety. No differences were observed in pain, physical limitations, self-efficacy or actometer scores. Multiple regression analysis of the tumor group revealed that higher pain scores, higher state anxiety and lower self-efficacy were asssociated with fatigue severity. In the control group of 24 knee arthroscopy patients, the percentage of severely fatigued patients decreased from 38% (n=9) prior to treatment to 29% (n=7) 6 months later. A substantial number of patients were severely fatigued in both the tumor group and the knee arthroscopy group. Self-efficacy, pain and anxiety appear to be the most important variables associated with fatigue severity in tumor patients prior to surgery

Myositis ossificans : another condition with USP6 rearrangement, providing evidence of a relationship with nodular fasciitis and aneurysmal bone cyst

Myositis ossificans is defined as a self-limiting pseudotumor composed of reactive hypercellular fibrous tissue and bone. USP6 rearrangements have been identified as a consistent genetic driving event in aneurysmal bone cyst and nodular fasciitis. It is therefore an integral part of the diagnostic workup when dealing with (myo)fibroblastic lesions of soft tissue and bone. Two cases of myositis ossificans with USP6 rearrangement were published so far. We determine herein the incidence of USP6 rearrangement in myositis ossificans using USP6 fluorescence in situ hybridization analysis (FISH). Of the 11 cases included, seven patients were female and four were male. Age ranged from 6 to 56 years (mean 27 years). Lesions were located in the thigh (n = 5), knee (n = 1), lower leg (n = 1), lower arm (n = 1), perineum (n = 1), gluteal (n = 1) and thoracic wall (n = 1). All assessable cases except one (8/9) showed rearrangement of USP6 providing evidence that myositis ossificans is genetically related to nodular fasciitis and aneurysmal bone cyst

Can Orthopedic Oncologists Predict Functional Outcome in Patients with Sarcoma after Limb Salvage Surgery in the Lower Limb? A Nationwide Study

Accurate predictions of functional outcome after limb salvage surgery (LSS) in the lower limb are important for several reasons, including informing the patient preoperatively and, in some cases, deciding between amputation and LSS. This study aimed to elucidate the correlation between surgeon-predicted and patient-reported functional outcome of LSS in the Netherlands. Twentythree patients (between six months and ten years after surgery) and five independent orthopedic oncologists completed the Toronto Extremity Salvage Score (TESS) and the RAND-36 physical functioning subscale (RAND-36 PFS). The orthopedic oncologists made their predictions based on case descriptions (including MRI scans) that reflected the preoperative status. The correlation between patient-reported and surgeon-predicted functional outcome was "very poor" to "poor" on both scores ( 2 values ranged from 0.014 to 0.354). Patient-reported functional outcome was generally underestimated, by 8.7% on the TESS and 8.3% on the RAND-36 PFS. The most difficult and least difficult tasks on the RAND-36 PFS were also the most difficult and least difficult to predict, respectively. Most questions had a "poor" intersurgeon agreement. It was difficult to accurately predict the patient-reported functional outcome of LSS. Surgeons' ability to predict functional scores can be improved the most by focusing on accurately predicting more demanding tasks

Multifocal occurrence of extra-abdominal desmoid type fibromatosis – A rare manifestation. A clinicopathological study of 6 sporadic cases and 1 hereditary case

Desmoid-type fibromatosis, also called desmoid tumor, is a locally aggressive myofibroblastic neoplasm that usually arises in deep soft tissue with significant potential for local recurrence. It displays an unpredictable clinical course. β-Catenin, the genetic key player of desmoid tumors shows nuclear accumulation due to mutations that preve

MRI for the evaluation of gastric physiology

Radiolog

End stage renal disease patients have a skewed T cell receptor Vβ repertoire

BACKGROUND: End stage renal disease (ESRD) is associated with defective T-cell mediated immunity. A diverse T-cell receptor (TCR) Vβ repertoire is central to effective T-cell mediated immune responses to foreign antigens. In this study, the effect of ESRD on TCR Vβ repertoire was assessed. RESULTS: A higher proportion of ESRD patients (68.9 %) had a skewed TCR Vβ repertoire compared to age and cytomegalovirus (CMV) – IgG serostatus matched healthy individuals (31.4 %, P < 0.001). Age, CMV serostatus and ESRD were independently associated with an increase in shifting of the TCR Vβ repertoire. More differentiated CD8(+) T cells were observed in young ESRD patients with a shifted TCR Vβ repertoire. CD31-expressing naive T cells and relative telomere length of T cells were not significantly related to TCR Vβ skewing. CONCLUSIONS: ESRD significantly skewed the TCR Vβ repertoire particularly in the elderly population, which may contribute to the uremia-associated defect in T-cell mediated immunity. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s12979-015-0055-7) contains supplementary material, which is available to authorized users

Ecologisch gericht suppleren: meetplan strandsurvey 2019

Suppleties van zand op vooroever of strand worden in opdracht van Rijkswaterstaat uitgevoerd om de Nederlandse kust tegen erosie te beschermen en om voldoende zand in het kustfundament te houden. Een groot deel van de suppleties vindt plaats in of nabij de kuststrook die binnen de Natura2000 kaders wordt beschermd, de Noordzeekustzone. Het is dus van belang de eventuele effecten van deze praktijk op de natuur zorgvuldig te bestuderen, zodat dit effect kan worden afgezet tegen het algemene nut voor de maatschappij. Betere kennis van de effecten kan leiden tot beperking van eventuele schade aan en mogelijk zelfs tot versterking van gewenste natuurwaarden en ecosysteemdiensten. Tot nog toe is er vooral aandacht geweest voor de gevolgen van suppleren op benthos en weinig voor de mogelijke effecten op vispopulaties, terwijl de ondiepe kustzone als kinderkamer voor vis een zeer belangrijke economische ecosysteemdienst levert. Kennis van de habitatfactoren die het voorkomen van juveniele vis in kinderkamers bepalen leidt tot een verbeterd inzicht van de gevolgen van suppleties op vispopulaties en van de voedselketen van viseters in de ondiepe kustzone. De kennisinstituten Deltares en Wageningen Marine Research hebben te samen in overleg met natuurorganisaties in 2016 het document `Ecologische effecten van zandsuppleties’ (Herman et al. 2016) geschreven met als doel onderzoek te formuleren naar ecologische effecten van zandsuppleties. In het onderdeel ‘uitvoeringsplan’ (deel C in Herman et al. 2016) zijn 3 onderzoekslijnen (ook wel Krachtlijnen genoemd) gedefinieerd, te weten: Vooroever, Duinen en Waddenzee. Het hier beschreven meetplan voor een strandsurvey in 2019 valt onder de onderzoekslijn Vooroever. De onderzoeksvraag luidt: “Wat zijn de cumulatieve gevolgen van reguliere suppleties op samenstelling en functioneren van het ecosysteem van de ondiepe vooroever van de Nederlandse kust?”. Deze volgt uit de prioritering van de krachtlijn Vooroever: (cumulatieve) gevolgen van reguliere suppleties op samenstelling en functioneren van het ecosysteem van de vooroever. Conform het plan van aanpak voor dit programma (Herman et al. 2016) wordt voorgesteld om een strandsurvey uit te voeren in de brandingszone (<1 m diepte), waarbij vis, benthos en habitatkarakteristieken worden bemonsterd. Deze strandsurvey is daarmee een onderdeel van een groter pakket van geplande dataverzameling in het kader van Ecologisch Gericht Suppleren II/Natuurlijk Veilig. Het hoofddoel van de strandsurvey is om gedurende het voorjaar (maart-juni) iedere 2 à 3 weken op drie verschillende locaties data te verzamelen over aantallen, conditie, groei en dieet van juveniele platvis in de brandingszone in relatie tot relevante omgevingsvariabelen, zowel abiotisch als biotisch. De survey zal inzicht verschaffen wanneer en in welke mate de brandingszone in die periode gebruikt wordt als kinderkamer voor jonge platvis en in hoeverre dit gebruik en conditie, groei, en dieet afhangen van biotische en abiotische omgevingsvariabelen en of dit verschilt per platvissoort. Dit rapport beschrijft de meetstrategie, de meetmethoden, de te meten variabelen (vis, benthos en omgevingsvariabelen) en de bemonsteringslocaties voor de strandsurvey 2019