Radiographic evaluation of the free jejunal graft

Jejunal autotransplantation is an increasingly popular method of reconstructive surgery for the upper aerodigestive tract following laryngopharyngectomy. From 1977 to 1985, 55 free jejunal grafts were performed on 49 patients. Thirty patients (61%) experienced reconstructive complications including graft failure in 6, fistula in 18, and strictures in 10. Postoperative dysphagia occurred in 26 patients (53%). The cause of the dysphagia is often complex and related to a number of factors that are discussed. While jejunal autotransplantation is successful in selected patients, complications are not infrequent. The radiologist plays an important role in the evaluation and follow-up of these patients.     

Skin cancer in adults younger than 40 years at the General Hospital of Mexico

Skin cancer is the most common malignant neoplasm reported worldwide. Over the last 20 years, skin cancer has been recognized in a high proportion among young people not associated with genetic disorders or other diseases. In Mexico there is no epidemiological information about this topic, so we made a retrospective study from 2006 to 2009 in the Dermatology service of the General Hospital of Mexico. We included 730 patients with diagnosis of primary skin cancer; 51 cases occurred in people younger than 40 years with an average age of 38 years, and with an estimated accumulated incidence of 6.8% for the elapsed time. In this study, skin cancer in young people showed a predominance of women (67% of patients); basal cell carcinoma was the most common type. Most of the lesions were found in sun-exposed areas. Skin cancer in people younger than 40 years is increasing dramatically, so we recommend avoiding ultraviolet radiation exposure in childhood and adolescence, and implementing sun-protection campaigns in order to educate this sector of the population and make them more aware of the potential dangers.     

Cerebral amyloid angiopathy and parenchymal amyloid deposition in transgenic mice expressing the Danish mutant form of human BRI2

Familial Danish dementia (FDD) is an autosomal dominant neurodegenerative disease clinically characterized by the presence of cataracts, hearing impairment, cerebellar ataxia and dementia. Neuropathologically, FDD is characterized by the presence of widespread cerebral amyloid angiopathy (CAA), parenchymal amyloid deposition and neurofibrillary tangles. FDD is caused by a 10-nucleotide duplication-insertion in the BRI₂ gene that generates a larger-than-normal precursor protein, of which the Danish amyloid subunit (ADan) comprises the last 34 amino acids. Here, we describe a transgenic mouse model for FDD (Tg-FDD) in which the mouse Prnp (prion protein) promoter drives the expression of the Danish mutant form of human BRI₂ . The main neuropathological findings in Tg-FDD mice are the presence of widespread CAA and parenchymal deposition of ADan. In addition, we observe the presence of amyloid-associated gliosis, an inflammatory response and deposition of oligomeric ADan. As the animals aged, they showed abnormal grooming behavior, an arched back, and walked with a wide-based gait and shorter steps. This mouse model may give insights on the pathogenesis of FDD and will prove useful for the development of therapeutics. Moreover, the study of Tg-FDD mice may offer new insights into the role of amyloid in neurodegeneration in other disorders, including Alzheimer disease.     

The epsilon isoform of 14-3-3 protein is a component of the prion protein amyloid deposits of Gerstmann-Sträussler-Scheinker disease

The 14-3-3 proteins are highly conserved, ubiquitous molecules involved in a variety of biologic events, such as transduction pathway modulation, cell cycle control, and apoptosis. Seven isoforms have been identified that are abundant in the brain, preferentially localized in neurons. Remarkable increases in 14-3-3 are seen in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease (CJD), and it has been found in pathologic inclusions of several neurodegenerative diseases. Moreover, the zeta isoform has been detected in prion protein (PrP) amyloid deposits of CJD patients. To further investigate the cerebral distribution of 14-3-3 in prion-related encephalopathies, we carried out an immunohistochemical and biochemical analysis of brain tissue from patients with Gerstmann-Str?ussler-Scheinker disease (GSS) and sporadic, familial and acquired forms of CJD, using specific antibodies against the seven 14-3-3 isoforms. The study showed a strong immunoreactivity of PrP amyloid plaques of GSS patients for the 14-3-3 epsilon isoform, but not for the other isoforms. The epsilon isoform of 14-3-3 was not found in PrP deposits of CJD. These results indicate that the epsilon isoform of 14-3-3 is a component of PrP amyloid deposits of GSS and suggest that this is the sole 14-3-3 isoform specifically involved in the neuropathologic changes associated with this disorder.     

Postoperative Complications and Functional Results After Subtotal Gastrectomy with Billroth II Reconstruction for Primary Gastric Cancer

Subtotal gastrectomy is considered the preferred treatment for gastric cancer with antral location. The aim of this study was to assess the incidence of early postoperative complications and late functional results in patients who underwent subtotal gastrectomy with Billroth II reconstruction for primary gastric adenocarcinoma. The results of 310 patients were analyzed with regard to postoperative complications and death rates. Functional results as they relate to the gastric resection were evaluated in 195 disease-free patients. Of the 310 patients, 77 developed postoperative general and surgical complications (24.8%) and 13 consequently died (in-hospital mortality: 4.2%). Although infrequent (6 cases, 1.9%), anastomotic leak was the most serious complication (4 cases died during the postoperative phase). Considering functional results, weight loss continued for the first trimester after surgery, after which it stabilized. Loss of appetite was rarely observed; early after the operation the majority of patients were consuming a normal diet and regularly consumed less than five meals per day (83.6%). Dumping syndrome was uncommon and usually resolved within one year (12.3% at three months, 9.5% after one year, 5.2% after two years). On the other hand, postprandial abdominal fullness was frequently observed (43.1% at three months, 36.1% after one year, 21.3% after three years, and 16.5% after five years). Billroth II reconstruction after subtotal gastrectomy is associated with a limited risk of anastomotic complications. Anastomotic leak, although infrequent, is a life-threatening complaint and requires prompt recognition and aggressive surgical treatment. The incidence of late complications was low and the majority of patients recovered from them within one year after surgery, although the occurrence of postprandial abdominal fullness was not completely irrelevant.     

Giant liposarcoma of the mesentery. Report of a case

Liposarcomas represent the single most common type of soft tissue sarcoma. Its abdominal localization is rare, occurring in only 5% of cases. A 55 year old male was found to have a case of primary giant liposarcoma of the mesenterium with a maximum diameter of 40 cm and weight of 9 kg. Computed tomography revealed the presence of a large mass presenting a dishomogeneous density with an adipose component, probably of mesenteric origin. A xifopubic laparotomy confirmed the presence of a pedunculated growth originating from the mesentery. The mass was removed and the histopathological report noted a well differentiated sclerosing liposarcoma with the peritoneal liquid positive for malignant cells. Surgery currently represents the only possibly curative therapy for this type of tumour but close long-term follow up and accurate evaluation of the clinicopathologic parameters are needed.     

Failure to Detect the Presence of Prions in the Uterine and Gestational Tissues from a Gravida with Creutzfeldt-Jakob Disease

The vertical transmission of a prion disease from infected mothers to their offspring is believed to be one of the routes for the natural spread of animal prion diseases. Supporting this notion is the observation that prion infectivity occurs in the placenta of infected ewes. Furthermore, the prion protein (PrP), both in its cellular form (PrP^C) and its pathological isoform (PrP^Sc), has been observed at the fetal-maternal interface of scrapie-infected sheep. However, whether these features of prion infectivity also hold true for human prion diseases is currently unknown. To begin to address such an important question, we examined PrP in the uterus as well as gestational tissues, including the placenta and amniotic fluid, in a pregnant woman with sporadic Creutzfeldt-Jakob disease (CJD). Although the proteinase K (PK)-resistant prion protein, PrP27-30, was present in the brain tissues of the mother, the PrP detected in the uterus, placenta, and amniotic fluid was sensitive to PK digestion. Unlike PrP^C in the brain and adjacent cerebrospinal fluid, the predominant PrP species in the reproductive and gestational tissues were N-terminally truncated, similar to urine PrP. Our study did not detect abnormal PrP in the reproductive and gestational tissues in this case of CJD. Nevertheless, examination by a highly sensitive bioassay is ongoing to ascertain possible prion infectivity from CJD in the amniotic fluid.The transmissible prion diseases affecting both humans and animals are characterized by the accumulation of an infectious prion protein particle (PrP^Sc) mainly in the central nervous system (CNS) and occasionally in the peripheral tissues.^1,2,3,4 Animal prion diseases such as scrapie in sheep and goats, chronic wasting disease in deer and elks, and bovine spongiform encephalopathy in cattle are believed to spread naturally by oral transmission, close contact between animals, and maternal transmission. Indeed, Western blot analysis and bioassays have demonstrated that PrP^Sc and prion infectivity are present not only in the CNS, but also in many peripheral tissues including the tonsils, spleen, lymph nodes, nasal mucosa, distal colon, ovaries, uterus, skeletal muscle, placenta, and amniotic fluid of affected animals.²In humans, the transmission of prion diseases has been observed in the acquired form of the disease including kuru, iatrogenic Creutzfeldt-Jakob disease (iCJD), and variant CJD (vCJD).⁵ Kuru is associated with cannibalistic rituals,^6,7 whereas iCJD is caused by prion exposure in the course of medical or surgical procedures, and vCJD has been attributed to the consumption of prion-contaminated meat.^8,9 In addition to CNS, PrP^Sc has also been detected in the tonsils, spleen, lymph node, retina, optic nerve, rectum, adrenal gland, and thymus of vCJD and in the spleen and skeletal muscles of sporadic CJD (sCJD).^3,4 However, it remains unknown whether human prion diseases are vertically transmitted in pregnancy. For instance, none of four offspring born to four gravid women with CJD had reportedly developed the disease when they reached the respective ages of 22, 10, 7, and 3 years.¹⁰ In addition, no reports are available concerning examination of PrP in the uterus and gestational tissues from prion-affected patients.We examined PrP^Sc distribution in CNS, uterus, and gestational tissues from a woman affected with prion disease, who had become pregnant and delivered a baby boy during the time she had the disease. Typical PK-resistant PrP core fragments and neuropathological changes, characteristic of sCJD (with PrP^Sc type 1 carrying a valine/valine polymorphism at codon 129 of PrP gene), were detected in the brain tissues obtained at either biopsy or autopsy. Although PrP was detectable in the uterus, placenta, and amniotic fluid, it was PK-sensitive. Moreover, neither conventional nor enrichment-based Western blot analysis revealed the presence of abnormal PrP species. In contrast to the PrP in the brain and cerebrospinal fluid (CSF), the PrP detected in the uterus and gestational tissues, including placenta and amniotic fluid, was N-terminally truncated, similar to that normally found in the urine. Although the presence of prion infectivity in tissue remains to be determined by highly sensitive bioassay, our current study suggests that abnormal PrP species, including both PK-resistant and PK-sensitive forms, are undetectable in the uterus and gestational tissues in sporadic CJD.