Increased CSF-BACE1 activity associated with decreased hippocampus volume in Alzheimer's disease

The enzyme β-secretase (BACE1) is essentially involved in the production of cerebral amyloidogenic pathology in Alzheimer's disease (AD). The measurement of BACE1 activity in cerebrospinal fluid (CSF) has been reported, which may render CSF measurement of BACE1 a potential biomarker candidate of AD. In order to investigate whether BACE1 protein activity is correlated with regional brain atrophy in AD, we investigated the association between CSF levels of BACE1 and MRI-assessed hippocampus volume in patients with AD (n = 30). An increase in CSF-BACE1 activity was associated with decreased left and right hippocampus volume corrected for global head volume in the AD patients. Boot-strapped regression analysis showed that increased CSF levels of BACE1 activity were associated with increased CSF concentration of total tau but not amyloid-β1-42 in AD. White matter hyperintensities did not influence the results. BACE1 activity and protein levels were significantly increased in AD compared to 19 elderly healthy controls. Thus, the CSF biomarker candidate of BACE1 activity was associated with hippocampus atrophy in AD in a robust manner and may reflect neurotoxic amyloid-β-related processes.     

The role of human leucocyte antigens in children with hydatid disease: their association with clinical condition and prognosis

Hydatid disease (HD) is a parasitosis caused by Echinococcus granulosus, which is still an important health problem worldwide, and our country is an endemic region for HD. There is little information regarding the role of human leucocyte antigen (HLA) in genetic susceptibility or resistance to HD. In this study, we aimed to investigate the HLA profile of Turkish children with HD and to compare them with healthy individuals. We also planned to investigate whether HLAs have a potential role in the predisposition to or prevention of the occurrence of HD and to study the relationship between the clinical features of HD and the HLA profile of the patients. The study included 81 children (25 boys, 56 girls) with HD aged between 3 and 18 years. All the patients’ and control subjects’ HLA class I and II antigens were examined, antigen allele frequencies were calculated, and clinical characteristics were also evaluated. The frequency of HLA-B18, -DR1, and -DR15 alleles were significantly different between the patients and healthy groups; HLA-DR15 antigen might be associated with HD occurrence, and the presence of HLA-B18 and HLA-DR1 antigens might be associated with HD resistance. Compared with the healthy group, patients with lung HD had a significant increase in HLA-B44 frequency, and liver HD patients had a significant increase in HLA-DR15 antigen frequency. Furthermore, presence of HLA-DR11 was found to be a significant factor associated with cure of the disease. We concluded that HLA types have significant impact on the development of HD and clinical course of disease.     

Childhood intrathoracic Hodgkin lymphoma with hypertrophic pulmonary osteoarthropathy: a case report and review of the literature

Hypertrophic osteoarthropathy (HOA) is characterized by clubbing, periosteal new bone formation and polyarthritis. The pathogenesis of clubbing involves an increased expression of platelet-derived growth factor (PDGF) and vascular endothelial growth factor (VEGF) from the digitally lodged platelet clumps, which bypass the pulmonary capillary network as a result of various systemic disorders. Intrathoracic neoplasms are rare causes of HOA in children. We report here a 14-year-old boy with digital clubbing, who eventually received the diagnosis of intrathoracic Hodgkin lymphoma (HL) and HOA. Eight cases previously reported with these two diagnoses are reviewed to emphasize the prognostic significance of HOA in childhood HL. Conclusion: Given the pathogenesis of clubbing and the prognostic significance of HOA, intrathoracic disease should be considered when HOA is detected in a child with a known or suspected malignant disease, and the occurrence of HOA during follow-up should alert the physicians for possible recurrence of the neoplastic disease or intrathoracic involvement. There is no conflict of interest associated with this report nor any sponsors.     

Idiopathic isolated laryngotracheobronchial cartilage calcification in a child

Laryngeal, tracheal, and bronchial cartilage calcification is quite rare in children. It was reported to occur in congenital cardiovascular diseases, Keutel syndrome, chondroplasia punctata, warfarin embryopathy, and warfarin sodium therapy. It can occur idiopathically as well. Laryngotracheobronchial cartilage calcification is demonstrated in this report by chest radiography and multiplanar three-dimensional CT examination in a 2.5-year-old boy with nonspecific cough.     

Bcl-2 and p53 expression in insular and in well-differentiated thyroid carcinomas with an insular pattern

Expression of p53 and bcl-2 oncogenes was investigated in poorly differentiated, so-called insular carcinomas of the thyroid gland and also in the follicular and papillary carcinomas with an insular component. Hematoxylin-eosin sections of 217 thyroid carcinomas were reevaluated for insular carcinoma and also for thyroid carcinomas with an insular component. Immunohistochemical staining method was used for detecting p53 and bcl-2 expression on paraffin blocks of three pure insular, five follicular or papillary thyroid carcinomas with a major insular component (more than 50%) and six with a minor insular component (20–50%). Flow cytometry was also performed in these cases. None of the cases showed p53 immunoreactivity. Bcl-2 expression was observed in all cases and the most intense staining was seen in insular areas. All the cases were diploid. We suggest that bcl-2 plays a role in loss of differentiation of thyroid carcinomas.     

The association between individual attachment patterns,the perceived social support,and the psychological well‐being of Turkish informal caregivers

Background: This study aimed to investigate the relations among the psychological well‐being (i.e. depression and state/trait anxiety levels), attachment patterns (i.e. secure, ambivalent, avoidant), and the perceived social support from family/friends/significant others of caregivers of cancer patients in Turkey. Methods: Fifty‐one caregivers of adult cancer patients were recruited from the oncology outpatient clinic of the Marmara Medical School Hospital in Istanbul. Caregivers were assessed with the Adult Attachment Scale, the Beck Depression Inventory, State–trait Anxiety Inventories, and the Multidimensional Scale of Perceived Social Support. Results: Stepwise multiple regression analysis indicated that depression was predicted by ambivalent attachment and the perceived social support from family. The support from significant others was the significant predictor of trait anxiety and the caregivers' ambivalent attachment score was the significant predictor of state anxiety. Conclusions: We assert that ambivalent attachment pattern could confer a vulnerability to psychological distress in cancer caregivers. Assessing the psychological experiences and needs of caregivers and being aware of possible risk factors (such as attachment patterns) and protective factors (social support network) for depression and anxiety might be helpful for successful programmes and interventions that support the caregivers of cancer patients. Copyright © 2009 John Wiley & Sons, Ltd.     

Ultrasonography in patients with ulnar neuropathy at the elbow: Comparison of cross‐sectional area and swelling ratio with electrophysiological severity

The aim of this study was to determine the diagnostic value of ultrasonographic measurements in ulnar neuropathy at the elbow (UNE) and to assess the relationship between the measurements and the electrophysiological severity. The largest anteroposterior diameter (LAPD) and cross‐sectional area (CSA) measurements of the ulnar nerve were noted at multiple levels along the arm, and the distal‐to‐proximal ratios were calculated. Almost all of the measurements and swelling ratios between patients and controls showed statistically significant differences. The largest CSA, distal/largest CSA ratio, CSA at the epicondyle, and proximal LAPD had larger areas under the curve than other measurements. The sensitivity and specificity in diagnosing UNE were 95% and 71% for the largest CSA, 83% and 85% for the distal/largest CSA ratio, 83% and 81% for the CSA at the epicondyle, and 93% and 43% for the proximal LAPD, respectively. There was a statistically significant correlation between the electrophysiological severity scale score (ESSS) and the largest CSA, the CSA at the epicondyle and 2 cm proximal to the epicondyle, and the LAPD at the level of the epicondyle (P < 0.05). None of the swelling ratios showed a significant correlation with the ESSS. The largest CSA measurement is the most valuable ultrasonographic measurement both for diagnosis and determining the severity of UNE. Muscle Nerve, 2010