Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumour of the skin, with an estimated incidence of 0.8 to five cases per 1 million people per year. OBJECTIVE: To study epidemiological, immunohistochemical and clinical features, delay in diagnosis, type of treatment and outcome of DFSP from 1982 to 2002. METHODS: Using data from the population-based cancer registry, 66 patients with pathologically proved DFSP were included (fibrosarcomatous DFSP were excluded). Each patient lived in one of the four departments of Franche-Comté (overall population of 1 million people) at the time of diagnosis. The main data sources came from public and private pathology laboratories and medical records. The rules of the International Agency for Research on Cancer were applied. RESULTS: The estimated incidence of DFSP in Franche-Comté was about three new cases per 1 million people per year. Male patients were affected 1.2 times as often as female patients were. The trunk (45%) followed by the proximal extremities (38%) were the most frequent locations. DFSP occurred mainly in young adults between 20 and 39 years of age. Mean age at diagnosis was 43 years, and the mean delay in diagnosis was 10.08 years. Our 66 patients initially underwent a radical local excision. Among them, 27% experienced one or more local recurrences during 9.6 years of follow-up. There was one regional lymph node recurrence without visceral metastases. These recurrences were significantly related to the initial peripheral resection margins. We observed a local recurrence rate of 47% for margins less than 3 cm, vs. only 7% for margins ranging from 3 to 5 cm [P=0.004; OR=0.229 (95%, CI=0.103-0.510)]. The mean time to a first local recurrence was 2.65 years. Nevertheless, there was no death due to the DFSP course at the end of the follow-up, and the final outcome was favourable. CONCLUSION: Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.     

The Washington University-Barnes Hospital experience with lung transplantation. Washington University Lung Transplantation Group

OBJECTIVE.--To review our experience with lung transplantation, emphasizing recipient selection, choice of procedure, functional results, and outcome. DESIGN.--Retrospective review of patients who received lung transplants at Barnes Hospital, St Louis, Mo, between July 1, 1988, and January 31, 1991. SETTING.--Washington University School of Medicine, St Louis, Mo, and Barnes Hospital, a medical school and its affiliated referral hospital, respectively. PATIENTS.--Sixty-nine lung transplant procedures were performed in 66 recipients. Patients with clinically and physiologically severe lung disease were selected according to predetermined guidelines. Underlying diseases in the recipients included chronic obstructive pulmonary disease, alpha 1-antitrypsin deficiency emphysema, cystic fibrosis, pulmonary fibrosis, primary pulmonary hypertension, Eisenmenger's syndrome associated with an atrial septal defect, bronchiectasis, eosinophilic granuloma, and lymphangiomyomatosis. INTERVENTION.--Double-lung, bilateral sequential, and single-lung transplantations were performed. Eight patients underwent en bloc double-lung transplantations or a modification of this procedure with separate bronchial anastomoses. Thereafter, the bilateral sequential approach to replacement of both lungs was performed in 26 patients. Thirty-two patients underwent single-lung transplantations. MAIN OUTCOME MEASURES.--Pulmonary function tests, arterial blood gas levels, pulmonary artery pressure, pulmonary vascular resistance, and actuarial survival. RESULTS.--Actuarial survival at 1 year for the 66 lung transplant recipients was 79%. Actuarial survival at 1 year was 82% for the bilateral lung transplant recipients and was 90% for the single-lung transplant recipients. In patients with either restrictive or obstructive lung disease, pulmonary function tests and arterial blood gas levels improved markedly after lung transplantation. In patients with primary pulmonary hypertension or Eisenmenger's syndrome, the pulmonary artery pressure decreased and the cardiac index increased into the normal range after single-lung transplantation. CONCLUSIONS.--In carefully selected patients with end-stage lung disease, single-lung and bilateral lung transplantations can significantly improve functional capacity, with promising early actuarial survival statistics after 1 year.     

Factors leading to long-term hospitalization after laparoscopic appendectomy.

BACKGROUND: Randomized studies demonstrate that laparoscopic appendectomy yields better results compared with open techniques. We sought to identify factors that determine an extended hospital stay among patients undergoing laparoscopic appendectomy. METHODS: This was a prospective study including 669 patients undergoing laparoscopic appendectomy. We analyzed variables that can predict the length of hospital stay. RESULTS: Of 669 patients undergoing laparoscopic appendectomy, 141 stayed in the hospital for > or = 5 days (Group 1), and 97 stayed in the hospital for < or = 1 day after surgery (Group 2). The univariate analysis demonstrated that fever (P<0.0001), nausea and vomiting (P=0.060), leukocytosis (P<0.0001), gangrened or perforated intraoperative appearance of the appendix (P<0.0001), and appendix position behind the ileocecal junction (P<0.001) were related to a longer hospital stay. The multivariate analysis through logistical regression showed that the factors independently and significantly associated with an extended hospital stay were presurgical fever, appendix position behind the ileocecal junction, and intraoperative gangrened or perforated appearance of the appendix. CONCLUSION: Fever, appearance, and position of the appendix are factors related to an extended hospital stay.     

The impact of poverty on educational outcomes for children

Over the past decade, the unfortunate reality is that the income gap has widened between Canadian families. Educational outcomes are one of the key areas influenced by family incomes. Children from low-income families often start school already behind their peers who come from more affluent families, as shown in measures of school readiness. The incidence, depth, duration and timing of poverty all influence a child’s educational attainment, along with community characteristics and social networks. However, both Canadian and international interventions have shown that the effects of poverty can be reduced using sustainable interventions. Paediatricians and family doctors have many opportunities to influence readiness for school and educational success in primary care settings.     

Challenges in geriatric oral health research methodology concerning caregivers of cognitively impaired elderly adults

Little is known about oral health care behaviors of community-dwelling, cognitively impaired elderly (CIE) persons. Few studies have been conducted regarding the actual provision of oral care for CIE persons or the attitudes and concerns about oral health among their caregivers. The CIE person's ability to perform self-care decreases over time, and the role of the caregiver in daily oral care becomes increasingly important. The purpose of this pilot study was to explore the attitudes toward oral health and identify related concerns among caregivers who care for community-dwelling CIE persons. Caregivers were surveyed by means of a self-administered, mailed questionnaire. To maintain confidentiality, an intermediary was used for recruitment and data collection. Overall, 148 caregivers were invited by an intermediary to participate in the survey; only 21 (14%) consented to participate and returned the questionnaire. This paper discusses some of the numerous methodological challenges identified when an attempt was made to survey caregivers' attitudes toward and barriers to providing oral care for a dependent CIE.     

Naphthalimido derivatives as antifolate thymidylate synthase inhibitors

A new series of N-(substituted)benzyl-1,8-naphthalimides 4, structurally related to the previously reported thymidylate synthase (TS) inhibitor naphthaleins 3, were synthesized and compounds tested for their inhibition of several species of TS. Moreover, their in vitro cytotoxicity together with antimycotic and antibacterial properties were assayed. While no activity was detected in the antibacterial tests, the m-nitro (4ae) and the p-nitro (4af) derivatives were found able to partially inhibit TS at low micromolar concentrations. Introduction of nitro or (substituted)-amino groups in position 4 of the naphthalic ring always led to less active compounds.     

Structural and functional organization of the upper esophageal sphincter

Using traditional anatomical and histological methods, the muscle envelope of the pharynx-esophagus junction was investigated in humans and dogs. In the upper (cranial) portion of the esophagus of man and dogs, an inferior anatomical sphincter was detected which histologically can be referred to the group of rhabdo-sphincters. The upper esophageal sphincter is a purely esophageal structure which in man is located at a distance of 25-30 cm from the maxillary incisors. In adult humans, it is 25-30 mm long and is situated obliquely to the long esophageal axis. The posterior semicircle of the sphincter is located higher than the anterior one. In the area of the upper esophageal sphincter the esophageal wall is of different thickness. Due to the muscle envelope and submucous membrane of the base, the right wall is 1.7-2.0 times thicker than the left, anterior or posterior wall. The data obtained from fiber esophagoscopy of patients and electromyography of the pharynx-esophagus junction of dogs have shown that the upper (cranial) esophageal sphincter control food passage from the pharynx to the esophagus and prevents food reflux to the laryngopharynx, protecting airways from aspiration.