Sclerodermiform Cell Epithelioma of the Palpebromalar Region.

This report describes a recurrent sclerodermiform basal cell epithelioma of the malar region next to the inferior eyelid in a 57-year-old woman. Three interventions were necessary to obtain a clear margin of resection. The area of resection was closed with a local cutaneous flap.

Enhanced pro-apoptosis gene signature following the activation of TAp63α in oocytes upon γ irradiation.

Specialized surveillance mechanisms are essential to maintain the genetic integrity of germ cells, which are not only the source of all somatic cells but also of the germ cells of the next generation. DNA damage and chromosomal aberrations are, therefore, not only detrimental for the individual but affect the entire species. In oocytes, the surveillance of the structural integrity of the DNA is maintained by the p53 family member TAp63α.

Corneal Abnormalities Are Novel Clinical Feature in Wolfram Syndrome.

Purpose: To evaluate corneal morphology among patients with Wolfram syndrome (WFS).

Design: Comparative observational longitudinal case series of WFS patients with a laboratory approach in the WFS1 gene knockout (Wfs1KO) mouse model.

Methods: A group of 12 patients with biallelic mutations in the WFS1 gene recruited from the whole country and a control group composed of 30 individuals with type 1 diabetes (T1D) were evaluated in a national reference center for monogenic diabetes.

The Small Non-coding Vault RNA1-1 Acts as a Riboregulator of Autophagy.

Vault RNAs (vtRNA) are small non-coding RNAs transcribed by RNA polymerase III found in many eukaryotes. Although they have been linked to drug resistance, apoptosis, and viral replication, their molecular functions remain unclear. Here, we show that vault RNAs directly bind the autophagy receptor sequestosome-1/p62 in human and murine cells.

Electron microscopic and confocal laser microscopy analysis of amyloid plaques in chronic wasting disease transmitted to transgenic mice.

We report here on the ultrastructure of amyloid plaques in chronic wasting disease (CWD) transmitted to Tg20 transgenic mice overexpressing prion protein (PrP). We identified three main types of amyloid deposits in mCWD: large amyloid deposits, unicentric plaques similar to kuru plaques in human prion diseases and multicentric plaques reminiscent of plaques typical of GSS. The most unique type of plaques were large subpial amyloid deposits.

[Abdominal necrotizing fasciitis after caesarean delivery].

Necrotizing fasciitis (NF) is a rare infection (0,2 to 0,4/100,000 adults) of the dermis and hypodermis extending along muscular fascia1. The absence of pathognomonic symptoms makes its diagnosis difficult. Rapidly progressive, it is a life-threatening emergency whose prognosis is letal in 30 % of cases.

Distal airway stem cells yield alveoli in vitro and during lung regeneration following H1N1 influenza infection.

The extent of lung regeneration following catastrophic damage and the potential role of adult stem cells in such a process remains obscure. Sublethal infection of mice with an H1N1 influenza virus related to that of the 1918 pandemic triggers massive airway damage followed by apparent regeneration. We show here that p63-expressing stem cells in the bronchiolar epithelium undergo rapid proliferation after infection and radiate to interbronchiolar regions of alveolar ablation.

DNA damage in oocytes induces a switch of the quality control factor TAp63α from dimer to tetramer.

TAp63α, a homolog of the p53 tumor suppressor, is a quality control factor in the female germline. Remarkably, already undamaged oocytes express high levels of the protein, suggesting that TAp63α's activity is under tight control of an inhibitory mechanism. Biochemical studies have proposed that inhibition requires the C-terminal transactivation inhibitory domain.

Conformational stability and activity of p73 require a second helix in the tetramerization domain.

p73 and p63, the two ancestral members of the p53 family, are involved in neurogenesis, epithelial stem cell maintenance and quality control of female germ cells. The highly conserved oligomerization domain (OD) of tumor suppressor p53 is essential for its biological functions, and its structure was believed to be the prototype for all three proteins. However, we report that the ODs of p73 and p63 differ from the OD of p53 by containing an additional alpha-helix that is not present in the structure of the p53 OD.

Ultrasound bone measurements in pre-adolescent girls: interaction between ethnicity and lifestyle factors.

Aim: We obtained calcaneal ultrasound measurements in 198 girls between 7.5 and 11.7 y of age, representing ethnic groups (black [n = 80], white [n = 41], mixed ancestral origin [n = 77]) in South Africa.

Verrucous carcinoma or epithelioma cuniculatum plantare.

Verrucous carcinoma is a rare form of squamous cell carcinoma. It behaves as a slow-growing, locally aggressive and rarely metastasizing tumour. Three clinical cases are presented, with emphasis on clinical presentation, pathological diagnosis and therapeutic approach.

[Analysis of 156 breast reconstructions by transverse rectus abdominis muscle flap (TRAM)].

The myocutaneous rectus abdominis flap described by Hartrampf was used for 156 breast reconstructions between 1982 and 1992. 107 reconstructions were done with one pedicle. 142 were delayed reconstructions.

Blood cadmium and lead levels among school children in Cracow.

An assessment of school children's exposure to cadmium and lead was carried out in Cracow and in a rural area in the years 1987-1989. The metal concentrations were determined in blood, which seems to be the best indicator of recent and current exposure. Simultaneously the levels of cadmium and lead in the air and dust-fall were measured.

Chronic pain: illusion or pathology? A case report of a vascular leiomyoma in the leg.

Patients with chronic pain constitute a major problem for the surgeon as the cause of their pain is often difficult to diagnose and is only rarely a well circumscribed lesion which is amenable to surgical or other treatment. We report a case of chronic pain due to a vascular leiomyoma beneath the deep fascia of the leg. Angioleiomyomas are rare tumours that are usually found in the subcutaneous tissue.