Neurophysiological follow-up of two siblings with Crigler-Najjar syndrome type I and review of literature

Crigler-Najjar syndrome type I is an autosomal recessive inherited disease and rarely seen in childhood. Bilirubin neurotoxicity is the morbidity of the disease due to the elevated unconjugated bilirubin levels. Mental retardation, seizures, cognitive dysfunction, oculomotor nerve palsy, ataxia, choreoathetosis, and spasticity may be seen. Due to the high bilirubin levels, alterations in the neurophysiological studies may be detected. In this study, we describe two siblings who were diagnosed with Crigler-Najjar syndrome type I who underwent a successful liver transplantation using a single cadaveric organ, together with their neurophysiological follow-up and review of the literature

The risk factors for mechanical complication in endoprosthetic reconstruction of knee osteosarcoma

Background: The increased limb survival rates and higher functional demands have also increased the mechanical complication rates of megaprostheses. This study aimed to analyse possible risk factors which can predispose patients to mechanical complications. Methods: Patients with knee osteosarcoma referred to our clinic from 1992 to 2014 were investigated retrospectively. The patients who underwent surgical resection and endoprosthetic reconstruction with at least 5 years of follow up were analysed. The revision of the megaprosthesis due to a mechanical complication was accepted as an endpoint. The possible risk factors, including cement usage, implant material, joint mechanism, neoadjuvant radiotherapy, and anatomical localization of the lesion, were analysed for any association with mechanical complication rates. Results: A total of 118 patients were included for final analysis. The average age was 24.5 years (standard deviation +/- 10.1, range: 15-64). Mechanical complication rate was 22% which included 19 aseptic loosenings and seven implant failures. The average time to mechanical complication was 32.5 months. Overall, 5-year implant survival was 78%. Multiple regression analysis revealed that cement usage is an independent risk factor for mechanical complication (P = 0.007). Although the 5-year implant survival was higher in rotating hinge and titanium implants compared with fixed hinge and cobalt chrome, the multiple regression model did not yield a correlation with mechanical complication rates. Conclusion: Cemented implants showed significantly higher mechanical complication rates compared with cementless ones in this series of knee osteosarcoma patients who underwent megaprosthetic reconstruction. Hinge mechanism and implant material did not have a significant effect on mechanical complication rates. (c) 2021 Elsevier B.V. All rights reserved

The Effect of Preoperative Albendazole Treatment on Intramuscular Cyst Structure in a Patient with Simultaneous Cystic Echinococcusis of Liver and Vastus Lateralis Muscle

We report a 44-years-old woman with cystic echinococcosis (CE) who presented with simultaneous involvement of liver and vastus lateralis muscle to Istanbul University-Cerrahpasa, Istanbul, Turkey in 2020. Although she underwent surgery for the liver, the intramuscular mass was ignored. While the patient was under post-operative albendazole treatment, she was investigated for the mass on her right thigh which was excised later. The comparison of magnetic resonance imaging before and after albendazole treatment showed that albendazole eliminated the intramuscular vesicle structures by accelerating the degeneration process of the cyst. The comparison of pathology samples sent from both liver and intramuscular CE also revealed that the albendazole has left the laminar membrane intact, degenerate the germinative membrane, thereby reducing the intra-vesicle pressure and also caused the scolex structures to disappear. To the best of our knowledge, this is the first paper to report the effect of preoperative albendazole treatment on the structure of intramuscular CE

The effect of preoperative radiotherapy on local control and prognosis in high-grade non-metastatic intramedullary osteosarcoma of the extremities

Introduction This study aimed to analyse the effect of preoperative radiotherapy on survival, recurrence and necrosis rates in osteosarcoma patients treated with neoadjuvant chemotherapy and limb-salvage surgery. Materials and methods Osteosarcoma patients who referred to our clinic from 1998 to 2015 were investigated retrospectively. 141 patients with non-metastatic osteosarcoma of the limb who received neoadjuvant chemotherapy and underwent limb-sparing surgery with or without preoperative radiotherapy were included in the study. 73 patients had neoadjuvant chemotherapy only (CT group), while 68 patients had additional preoperative radiotherapy (CT + RT group). 5-year survival, disease-free survival, limb survival, local recurrence, metastasis, complication and necrosis rates were compared between the two groups. Results Overall 5-year survival, 5-year disease-free survival, 5-year limb survival rates were 68.5, 64.3, 65.7 for CT group and 72.1, 67.6, 64.7 for CT + RT group, respectively. Preoperative radiotherapy gave similar survival and disease-free survival rates. Limb survival, recurrence and metastasis rates were similar between the groups. The necrosis rate of the lesions was significantly higher in the CT + RT group compared to the CT group. Conclusion Preoperative radiotherapy can provide a higher necrosis rate and may allow the resection of unresectable lesions in the treatment of osteosarcoma. CT + RT provided similar 5-year survival despite the larger tumour size compared to CT group. Due to the higher wound complication rate (8.2% in CT, 23.5% in CT + RT group, p = 0.01), CT + RT should be restricted to the lesions with large size or close proximity to neurovascular structures

Radiographic and pathological stages of the changes at the bone-cement interface: an in-vivo experimental study

WOS: 000259456100028PubMed ID: 18560854Introduction Chemical and physical effects of cementation cause radiographic and histological changes at bone-cement interface. These changes can be of interest in the assessment of the residual lesions and subsequent recurrences after local resection and cementation of local aggressive tumours. Aim The aim of the study was to evaluate the evolution and determine the stages of the changes that occur at the bone-cement interface after cementation of cavitary lesions. Materials and methods We operated on 16 hind legs of 8 sheep (Ovies Aries) under general anaesthesia (Xylasin HCl, Ketamin HCl and Forane). A bone cavity of 12 cm(3) was produced by curettage of the distal femoral condyle and was filled with cement. Control radiographs were taken at 2 days; 3, 6 and 12 weeks, and again at 6 months. One sheep each time was killed after second day and sixth month and two sheep each time after the third, sixth and 12th week and the specimens underwent pathological examination. Results After the first 3 weeks, a reactive fibrous membrane was detected on pathological examinations. This membrane consisted of granulation tissue, necrotic bone and bone marrow, which were replaced gradually by fibrous tissue. The radiographic revelation of this fibrous membrane was a radiolucent zone of 0.5-1.5 mm at 3 weeks. A Sclerotic rim appeared around this radiolucent zone at 6 weeks. With new bone formation the fibrous membrane disappeared at 3 months. This was seen on radiographs as the replacement of the radiolucent zone by a sclerotic ring of 0.5-2 mm. This sclerotic ring disappeared at 6 months, when a diffuse sclerosis and cortical bone thickening was detected on radiographs. Discussion According to our findings we suggest to consider the pathological processes at the bone-cement interface in 3 phases: (1) Reactive phase (first 3 weeks); (2) Resorption phase (3-6 weeks), and (3) Formation phase (6 weeks to 6 months). We have distinguished five different radiographic stages: Stage 1-Early stage with no apparent zone (first 3 weeks); Stage 2-Radiolucent zone (3-6 weeks); Stage 3-Radiolucent zone with a sclerotic rime (6 weeks to 3 months); Stage 4-sclerotic ring (after 3 months) and Stage 5-Diffuse cortical thickening (after 6 months). Determining the phases of tissue reaction after cementation and its radiographic revelation will ease the diagnosis of residual lesions and subsequent recurrences after local resection and cementation of local aggressive tumors.Research Fund of Istanbul University [1357/280799]This experiment was supported by Research Fund of Istanbul University (Project number: 1357/280799)

Relationship between Anti-CCP Antibodies and Oxidant and Anti-Oxidant Activity in Patients with Rheumatoid Arthritis

<p><b>Objective/Aim: </b>A new group of autoantibodies in Rheumatoid Arthritis (RA), the anti-cyclic citrullinated peptide (anti-CCP) antibodies directed to citrulline-containing proteins, which are of value for the severity of RA. Up to date, the relationship between anti-CCP antibodies and oxidant, anti-oxidant activity in patients with RA has not been elucidated in the previous studies. In this study we aimed to investigate the effect of anti-CCP antibodies in the circulation on whole blood, serum and synovial fluid oxidant and anti-oxidant activity in patients with RA. <b>Materials and Methods:</b> RA patients with anti-CCP (+) (n=25) and anti-CCP (-) (n=24) were recruited into the study. All patients had a positive rheumatoid factor (RF). The patients who were under treatment with only non-steroidal antiinflammatory drugs (NSAID) at the study time included in the study. Catalase (CAT), Glutathione peroxidase (GSHPx), Myeloperoxidase (MPO) activities and the levels of Malondialdehyde (MDA) were measured in whole blood, serum and synovial fluid in both groups. <b>Results: </b>There were no significant differences in terms of the mean whole blood and serum antioxidative activity (CAT, GSHpx) and the mean blood and serum MDA and MPO values (oxidative activity), between the patients with anti-CCP(+) and those with anti-CCP(-). There was increased synovial oxidant activity (MDA and MPO levels) (p&#60;0.05) in anti-CCP(+) RA patients with or without ESR negativity when compared with anti-CCP(-) RA patients. There was positive correlation between anti-CCP antibody levels and synovial MDA and MPO levels (r=0.435, p&#60;0.05, r=0.563, p&#60;0.05 respectively) in anti-CCP (+) group.<b> Conclusions:</b> In conclusion, anti-CCP antibody positivity seems to be associated with increased synovial fluid oxidant activity (increased MDA and MPO levels) in patients with RA. These conclusions need to be validated in a larger controlled study population.</p

The frequency and effect of fibromyalgia in patients with Behcet's disease

Conclusion: Our study results suggest that patients with BD have an increased severity of depression and anxiety, higher frequency of FM, and worsened QoL than healthy individuals. In addition, the presence of FM seems to be related with female sex, longer disease duration, older age, depression, sleep disturbance, and poor QoL in BD patients