Executive cognitive deficits in primary dystonia.

Primary dystonia is a disorder of movement for which no consistent pathophysiology has been identified; in the absence of evidence to the contrary, it is assumed to be cognitively benign. We have studied a clinically heterogeneous group of 14 patients with primary dystonia on a battery of neuropsychological tests. Despite well-preserved speed of information processing, language, spatial, memory and general intellectual skills relative to normal controls, we have identified a constellation of attentional-executive cognitive deficits on the Cambridge Neuropsychological Test Automated Battery (CANTAB). Specifically, patients demonstrated significant difficulties negotiating the extra-dimensional set-shifting phase of the IED task. The implications of these findings for the pathophysiology of primary dystonia are discussed. This is, to the best of our knowledge, the first report of a significant cognitive deficit in patients with primary dystonia.     

中国食管癌高发区食管原发性腺癌组织病理学研究Ⅰ．食管原发性腺癌的临床病理学分析

中国食管癌高发区河南省近１５年（１９８０～１９９４）病理确诊的７３６４例食管癌中，其中食管原发性腺癌（ＰＥＡ）２３９例，占３．２５％。通过对２３９例ＰＥＡ手术标本（包括早期癌２０例，其余为中、晚期癌）切片系统观察，指出中国食管癌高发区ＰＥＡ主要起源于食管固有腺体的各级导管部分。ＰＥＡ的病理组织学可分６型：腺导管癌（１０９例）、腺鳞癌（５４例）、腺样囊性癌（２９例）、粘液表皮样癌（２０例）、基底细胞样癌（１５例）、胃型腺癌（１２例）。每型的组织图象及预后均各有特点，提示ＰＥＡ组织学再分型有意义。     

Multiple primary malignant neoplasms in urologic patients

Methods: Second primary cancers constitute approximately 9–10% of malignancies diagnosed in the United States. We aimed to show the risk and incidance of second primary tumor occuring associated to urologic tumors and show the distrubution of tumors in Tracia region. We retrospectively examined the patients' files with the diagnosis of malignant disease between the years 1985–2000. Hazard function analysis was performed to estimate the relative risk of secondary malignancy occuring. Age, sex and tumor number were examined to find out if they affect on mortality rate. Results: A total number of 25 MPMNs were diagnosed. In 52 percent of the cases the second primary neoplasm developed within six months. The relative risk of development of a second neoplasm is found as increasing 1.111 times per month. The incidence of secondary malignancy occuring in the patients with one tumor was found as 6.31%. Age (p < 0.001) and tumor number (p < 0.001) are found as statistically effective predictor on mortality rate where the sex is not. Conclusions: In the patients with a primary tumor not only the metastasis must be investigated but also second primary tumors should be taken in to consideration. This revised version was published online in August 2006 with corrections to the Cover Date.     

Traumatic dental injuries and associated factors among Brazilian preschool children

Abstract –  The main objective of this study was to assess the epidemiology of traumatic dental injuries (TDI) to the primary teeth of preschool children and second, to investigate whether TDI were related to anterior open bite and, third, whether TDI are related to socio-economic circumstances in an urban Brazilian population. A clinical examination was performed during the National Day of Children's Vaccination. TDI were classified according to the modified classification proposed by Ellis. In addition to those criteria the presence of tooth discoloration was recorded. An interview was carried out with mothers or guardians. The data collected included mother's level of education and living conditions. The prevalence of TDI was 9.4%. The maxillary central incisors were the most affected teeth. Most children with a TDI experienced traumatic injuries to one tooth (6.3%), while 2.8% had two and 0.4% had three traumatized teeth. The most common crown fracture was in enamel only (68.8%), followed by crown fracture of enamel and dentin (13.8%). Missing teeth following trauma occurred in 10.9% of those with TDI. The prevalence of tooth discoloration was 5.1%. Children with anterior open bite had twice the level the level of TDI compared to those with normal occlusions ( P  = 0.001). Socio-economic factors were not statistically significantly associated with the occurrence of TDI. The prevalence of dental injuries in Brazilian preschool children was low. Children with anterior open bite experienced more dental injuries. Neither of the indicators of socio-economic status was related to the prevalence of TDI.     

Pulpal reactions to glutaraldehyde and paraformaldehyde pulpotomy dressings in monkey primary teeth

Abstract Pulpotomy was performed in primary teeth of 4 vervet monkeys to compare the effect of an experimental dressing containing glutaraldehyde (GL) to a similar formula with paraformaldehyde (PF). Forty teeth were examined histologically 2 weeks to 5 months post-operatively. In the PF group, calcifications were more frequently observed and the central core of tissue in the root canal was heavily laden with debris. Pathologic apical resorption and lesions were more frequent. The pulp in the apical third of the root canal was usually uninflamed in the GL group and peripheral calcifications were seldom observed in it. Reaction to GL seemed, therefore, more favorable clinically. Defects were not observed in the permanent teeth that erupted following shedding or extraction of the experimental primary ones of either group.     

Klinefelter's Syndrome: Hypogonadism,abnormal carbohydrate metabolism. Is it a result of biotin deficiency?

The aetiology of Klinefelter's Syndrome is not known. The causative factor(s) must explain the hypogonadism, low androgen levels, the disordered carbohydrate metabolism and the commonly associated psychiatric conditions. A biotin deficient/dependent state can account for the above. A biotin deficient Klinefelter's Syndrome patients with the above is described. The possible role of biotin in the primary, secondary and tertiary prevention of Klinefelter's Syndrome needs further research.     

Guidance of dopaminergic neuritic growth by immature astrocytes in organotypic cultures of rat fetal ventral mesencephalon.

Astrocytes, with their many functions in producing and controlling the environment in the brain, are of great interest when it comes to studying regeneration after injury and neurodegenerative diseases such as in grafting in Parkinson's disease. This study was performed to investigate astrocytic guidance of growth derived from dopaminergic neurons using organotypic cultures of rat fetal ventral mesencephalon. Primary cultures were studied at different time points starting from 3 days up to 28 days. Cultures were treated with either interleukin-1 beta (IL-1 beta), which has stimulating effects on astrocytic proliferation, or the astrocytic inhibitor cytosine arabinoside (Ara-C). Tyrosine hydroxylase (TH)-immunohistochemistry was used to visualize dopaminergic neurons, and antibodies against glial fibrillary acidic protein (GFAP) and S100 beta were used to label astrocytes. The results revealed that a robust TH-positive nerve fiber production was seen already at 3 days in vitro. These neurites had disappeared by 5 days. This early nerve fiber outgrowth was not guided by direct interactions with glial cells. Later, at 7 days in vitro, a second wave of TH-positive neuritic outgrowth was clearly observed. GFAP-positive astrocytic processes guided these neurites. TH-positive neurites arborized overlying S100 beta-positive astrocytes in an area distal to the GFAP-positive astrocytic processes. Treatment with IL-1 beta resulted in an increased area of TH-positive nerve fiber network. In cultures treated with Ara-C, neither astrocytes nor outgrowth of dopaminergic neurites were observed. In conclusion, this study shows that astrocytes play a major role in long-term dopaminergic outgrowth, both in axonal elongation and branching of neurites. The long-term nerve fiber growth is preceded by an early transient outgrowth of dopamine neurites.     

慢性肝病和肝癌中HCV核心区三种寡肽抗原的抗体检测

检测１４８例慢性肝病和肝癌患者血清中丙型肝炎病毒核心区３种寡肽抗原的抗体，结果显示其阳性率在慢性迁延型肝炎、慢性活动型肝炎、肝炎肝硬化和原发性肝癌患者分别为７．７％，２３．１％，，２３．５％和１６．３％。在抗体阳性者中８０％以上有乙型肝炎病毒感染证据，提示我国ＨＢＶ和ＨＶＣ重叠感染较为常见。     

Lymph node metastases in the neck and parotid gland from an unknown primary melanoma

A lymph node metastasis in the neck or parotid region from an unknown primary melanoma is an uncommon occurrence. Out of a total of 300 patients with head and neck melanoma treated at the Netherlands Cancer Institute between 1976 and 1992, 17 (5.7%) presented in this way. The most common site for metastatic lymph nodes (18 nodes in 17 patients) was level V (n= 7), followed by the parotid region (n= 4), level II (n= 4), level III (n= 2), and level IV (n= 7). Two patients had local excision of the neck node metastasis only, while the remaining 15 patients underwent more extensive surgical treatment. The 5-year disease-specific survival rate in this group was 48%, with a median survival of 36 months, which is more or less similar to the prognosis of stage II melanoma of the head and neck with a known, surgically treated primary tumour. No relation was found between disease-free interval and sex, the number of positive lymph nodes or the duration of symptoms.     

Primary aortoduodenal fistula caused by duodenal tuberculosis without an abdominal aortic aneurysm: Report of a case

A 66-year-old man died of massive gastrointestinal hemorrhage caused by a fistula between the third portion of the duodenum and the abdominal aorta. An autopsy revealed that duodenal tuberculosis had resulted in the development of a fistula into the aorta with no pathological changes, and no active pulmonary tuberculosis was found. Duodenal tuberculosis and primary aortoduodenal fistula (ADF) without an aneurysm are both extremely rare. Thus, we report herein a unique case of primary aortoduodenal fistula without an abdominal aortic aneurysm, but associated with duodenal tuberculosis, and review the current literature.