Right aortic arch detected in fetal life.

OBJECTIVE: To evaluate the prenatal distribution, associated conditions and outcome of the different types of right aortic arch (RAA) detected in fetal life. METHODS: This was a retrospective review of all cases of RAA detected prenatally between 1998 and 2005 in two tertiary referral centers. RESULTS: In the study period 71 cases of RAA were detected; 26 (37%) had RAA with aberrant left subclavian artery, 23 (32%) had RAA with mirror-image branching, 20 (28%) had RAA of unknown type and two (3%) had double aortic arch. While 20/26 cases with RAA and aberrant left subclavian artery were isolated findings, all 23 cases with RAA and mirror-image branching were associated with cardiac defects, namely tetralogy of Fallot (43%) or pulmonary atresia with ventricular septal defect (22%). Of the 20 cases with RAA, 19 of unknown type were associated with heterotaxy syndromes and had additional cardiac malformations and ambiguities of the situs. The two cases with DAA were isolated findings. Seven cases in our series (10%) had a microdeletion 22q11 and these were significantly associated with extracardiac malformations. The outcome in our series depended solely on the associated cardiac and extracardiac malformations, with the exception of one infant with isolated DAA, in whom a surgical correction was warranted. CONCLUSIONS: RAA detected in fetal life is associated frequently with other cardiac/non-cardiac malformations, heterotaxy syndromes and microdeletions 22q11. The associated conditions vary depending on the branching type of the brachiocephalic vessels and the presence of extracardiac malformations.     

Advanced thyroid carcinoma: an experience of 385 cases.

AIMS: To report clinical outcomes of a large series of cases with advanced thyroid cancer. STUDY DESIGN: Three hundred and eighty-five patients at the UICC stages III and IV were selected for the study with thyroid cancer. RESULTS: Papillary carcinoma and sclerosing carcinoma have better survival than the Hürthle cell and insular types. Lymphatic metastasis does not appear to worsen the prognosis. All the tumour forms offer the chance of long survival. CONCLUSIONS: Surgical treatment is the primary treatment of thyroid carcinoma. The combined treatments of surgery, metabolic beam therapy, suppressive hormone therapy, radiotherapy and chemotherapy cure a high percentage of patients with the tumour at an advanced stage.     

Sex differences in the clinical presentation and management of airflow obstruction.

The aim of the present study was to explore differences in the clinical expression, clinical diagnoses and management of airway diseases in a primary-care setting. Patients aged >or=35 yrs who had ever smoked were enrolled when they presented for any reason to one of eight rural primary-care practices. Respiratory symptom questionnaires and spirometry were administered. In total, 1,034 patients had acceptable and reproducible spirometry, of whom 550 (53%) were males and 484 (47%) were females. Males smoked more than females (41.2 versus 29.2 pack-yrs) respectively, and were more likely to have a pre-bronchodilator forced expiratory volume in one second/forced vital capacity <0.70 at 22.4 versus 11.8%, respectively. However, more females than males reported breathlessness (51.0 versus 42.8%, respectively), a prior diagnosis compatible with airflow obstruction and taking respiratory medications (23.4 versus 14.9%, respectively). In conclusion, the current results suggest that females are more likely than males to report breathlessness and be prescribed respiratory medications independent of differences in the severity of airflow obstruction.     

Hypoxic ventilatory response in successful extreme altitude climbers.

A very high ventilatory response to hypoxia is believed necessary to reach extreme altitude without oxygen. Alternatively, the excessive ventilation could be counterproductive by exhausting the ventilatory reserve early on. To test these alternatives, 11 elite climbers (2004 Everest-K2 Italian Expedition) were evaluated as follows: 1) at sea level, and 2) at 5,200 m, after 15 days of acclimatisation at altitude. Resting oxygen saturation, minute ventilation, breathing rate, hypoxic ventilatory response, maximal voluntary ventilation, ventilatory reserve (at oxygen saturation = 70%) and two indices of ventilatory efficiency were measured. Everest and K2 summits were reached 29 and 61 days, respectively, after the last measurement. Five climbers summited without oxygen, the other six did not, or succeeded with oxygen (two climbers). At sea level, all data were similar. At 5,200 m, the five summiters without oxygen showed lower resting minute ventilation, breathing rate and ventilatory response to hypoxia, and higher ventilatory reserve and ventilatory efficiency, compared to the other climbers. Thus, the more successful climbers had smaller responses to hypoxia during acclimatisation to 5,200 m, but, as a result, had greater available reserve for the summit. A less sensitive hypoxic response and a greater ventilatory efficiency might increase ventilatory reserve and allow sustainable ventilation in the extreme hypoxia at the summit.     

无（外侧）额叶皮质是否更好？额叶病变患者解决洞察力问题

最近提出的关于额叶功能的理论认为前额叶皮质，尤其是其背外侧面在确定适合一项特殊任务的一系列反应中起重要作用，并在选择中使上述反应发生偏差。这些活动事实上是为任何类型的非常规任务而执行，而不考虑内容的差别。本研究旨在通过一项解决“洞察力”问题的测试任务（即火柴杆算术作业），来验证Frith“塑造反应空间”假说的预测效力。从Knoblich等人对健康人不能解决火柴杆问题的解释和Frith关于额叶背外侧皮质作用的理论，作者推导出与直觉相左的预测，即对这些相对复杂的任务，外侧额叶皮质局部损伤的患者可能比1组健康受试者完成得更好。要求35例经CT或MRI扫描证实为单个局部脑损伤的患者（年龄为26—65岁）和23例健康受试者（年龄为34—62岁）完成火柴杆作业。研究结果似乎与理论上的预测相一致，虽然仅有43％的健康受试者能解决最困难的火柴杆问题（“C类”），但是却有82％的额叶外侧损伤患者完成了类似问题（Fisher精确概率检验，P〈0．05）。总之，对Frith和Knoblich等人理论的结合进行了确证。     

Enzootic Nasal Adenocarcinoma of Sheep in Slovenia

Clinicopathological and electron microscopical findings of eight cases of enzootic nasal adenocarcinoma of sheep, diagnosed solely in one big flock in Slovenia between years 2001 and 2003 are described. All affected sheep were female, their mean age was 4.5 ± 1.5 years and they either belonged to the Istrian pramenka breed (five sheep) or were crossbreeds (three sheep). Tumours that arose from the ethmoid area of the nasal cavity were unilateral in six cases (75%) and bilateral in two cases (25%). All tumours were classified as adenocarcinomas by histopathological examination and they displayed either a combination of tubular and papillary growth or less often solely tubular proliferation. No metastases were detected in regional lymph nodes, brain or other organs. Electron microscopical studies performed on the reprocessed paraffin‐embedded tissues revealed the presence of the virus‐like particles with an average diameter between 70 and 90 nm.