Complicated pericardial cyst: Atypical anatomy and clinical course

Pericardial cysts are usually detected by chance and are clinically silent in most cases. Nevertheless, symptoms and serious complications may occur. We describe a case of pericardial cyst diagnosed in an 8-year-old boy who was admitted with chest pain. Echocardiography revealed a mild to moderate pericardial effusion and a 7.5 × 5.5 cm intrapericardial echo-free lesion consistent with a pericardial cyst. Surgery was carried out 3 days afterward because of the patient's worsening condition, the progressive increase of pericardial effusion, and the onset of initial signs of cardiac tamponade. The cyst showed a long and easily movable vascular pedicle and inflammatory areas involving the pericardial surface. Like the pericardial effusion, the contents of the mass appeared as serosanguineous fluid on aspiration. Histologic examination confirmed the diagnosis of pericardial cyst and showed findings according to ischemia-related lesions of the cyst. The coexistence of pericardial cyst and cardiac tamponade is very unusual. The atypical anatomy and clinical course suggest a distinct and so far undescribed pathogenetic mechanism for this association: the torsion of a vascular pedicle and the subsequent development of ischemia-related lesions of the cyst.     

A risk score and a flowchart for liver retransplantation

Rates of overall graft survival after liver retransplantation (RETX) are still 20% lower than those after primary liver transplantation (TX). On the basis of previous mathematical approaches from other authors who tried to identify prognostic variables for survival and prognostic risk scores for liver RETX, we studied 12 categorical and 17 continuous variables from the donor, the recipient, and the surgical procedure, among patients who underwent liver retransplantation. Data were retrieved in a retrospective study over the last 12 years, in order to overcome the possible gap of other series that often included RETX performed many years ago. We considered 394 consecutive cadaveric liver TXs in adult patients, namely, 351 primary TXs and 43 RETXs. Using multivariate logistic regression, we calculated the following equation for 1-year risk of death for patients undergoing liver RETX: log(Odds)= -4.81+2.23 x Recipient Sex + 1.86 x Donor Age + 1.60 x MELD Score (where: Recipient Sex: F=0, M=1; Donor Age (years): <40=0, 40-59=1; 60+ =2; MELD Score: <26=0, 26+ =1). With this formula, we built a decision tree to predict the individual risk of death based on the subject's profile. Keeping in mind that mathematical models can only help our decisional process and are not conclusive, our data needs to be validated on a larger scale.     

CD25+ T cell depletion impairs murine squamous cell carcinoma development via modulation of antitumor immune responses

Squamous cell carcinoma (SCC) constitutes a microenvironment that could modulate the antitumor immune response. Also, tumor-infiltrating lymphocytes are believed to play complex regulatory roles in antitumor immunity against SCC. The presence of regulatory T cells (Tregs) has been associated with the suppression of tumor-reactive T cells. However, the underlying mechanism for this T cell dysfunction is not clear. We used a multistage model of SCC to examine the role of Treg cells during tumor development. 7,12-dimethylbenz[a]-anthracene/phorbol 12-myristate 13-acetate treatment and systemic depletion of Treg cells using an anti-CD25 monoclonal antibody (PC61) resulted in a decrease in the number and incidence of papilloma. Furthermore, CD25 depletion increased the proportion of CD8(+) and CD4(+) T cells that were isolated from tumor lesions. The levels of interleukin (IL)-1β, IL-10, IL-12, IL-13, interferon-γ, transforming growth factor-β and tumor necrosis factor-α, but not IL-17, were increased in the tumor microenvironment after Treg depletion. Therefore, our results indicated involvement of CD25(+) T cells in SCC development and in the suppression of the inflammatory immune response.     

Systemic lupus erythematosus in a multiethnic US cohort (LUMINA L II): relationship between vascular events and the use of hormone replacement therapy in postmenopausal women.

OBJECTIVES: To examine the influence of hormone replacement therapy (HRT) in the occurrence of vascular arterial and venous thrombotic events in postmenopausal women with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: SLE women aged > or =16 years, disease duration < or =5 years from LUMINA, a multiethnic, longitudinal outcome study, were included. Menopause was defined at disease onset as the presence of amenorrhea >6 months and/or oophorectomy, and/or increased follicle stimulating hormone values, and/or HRT use regardless of the presence or absence of climacteric symptoms (hot flashes). Patients were divided into HRT ever users and nonusers. Patients with positive antiphospholipid antibodies (n = 9) or vascular arterial events (n = 1) occurring before HRT use were excluded. The occurrence of vascular arterial and venous thrombotic events was compared between HRT users and HRT nonusers and its role examined by logistic regression after adjusting for "confounding by indication" using propensity score or logistic regression analyses. RESULTS: Seventy-two postmenopausal women, 32 (44%) HRT users and 40 (56%) HRT nonusers, were studied. HRT use was associated with fewer vascular arterial but not venous thrombotic events (P = 0.021) in the univariable analyses. However, after adjusting for the propensity score, HRT use was no longer significant (P = 0.064). Comparable results were obtained by logistic regression. CONCLUSIONS: HRT use was not associated with the occurrence of vascular arterial events in the LUMINA patients. HRT use in women with SLE should be individualized, but our data suggest its use may be safe if antiphospholipid antibodies are not present or vascular arterial events have not previously occurred.     

Nutritional status and dietary patterns in disabled people

BACKGROUND AND AIM: Obesity, cardiovascular diseases, diabetes and osteoporosis are the most frequent pathologies among people with a severe reduction of physical activity. The impairment in nutritional status, consequent to quantitative and qualitative inadequacy of diet, could be one of the first steps in the development of co-morbidities in disabled subjects. In order to evaluate this hypothesis we investigated the nutritional status and the food intake in patients with physical or mental disabilities. METHODS AND RESULTS: Thirty-seven disabled subjects (24 with exclusively physical inactivity and 13 with mental retardation and physical inactivity) mean age 33.5+/-9.2 years and 25 healthy subjects (mean age 31.0+/-9.3 years) were enrolled. Anthropometric measurements, indirect calorimetry, dual-energy X-ray absorptiometry, dietary intake and biochemical parameters were collected for each subject. Forty percent of disabled were overweight and 14% were obese. Fat free mass (FFM) and bone mineral content (BMC) was lower and fat mass (FM) was higher than able-bodied control. Absolute resting energy expenditure (REE) was lower in disabled subjects, but this difference disappeared when REE was normalized to FFM. Dietary intake resulted unbalanced (16%, 31%, 50% of total daily energy intake derived from protein, lipid and carbohydrate respectively) with a distribution of dietary fatty acid quite far from the recommended ratio [3.1(SFA):4.1(MUFA):1.0(PUFA)] and an excessive consumption of simple carbohydrates (mean intake 17.5+/-4.9%). Insufficient intake of fibre, iron, calcium, potassium and zinc was also found. Finally, alterations in the cholesterol profile were evident in more than one third of the disabled subjects, whereas fasting glucose intolerance was evident in one fourth. CONCLUSION: This study shows a consistent nutritional status impairment in disabled patients resulting in an reduction of FFM and BMC, in an over-representation of FM and in a number of biochemical risk factors for cardiovascular disease. The altered nutritional status is counterparted by a widespread inadequacy of dietary patterns. This nutritional and dietary impairment occurs both in subjects with mental and physical diseases.     

Mucopolysaccharidosis type IIID: 12 new patients and 15 novel mutationsb

Mucopolysaccharidosis III D (Sanfilippo disease type D, MPS IIID) is a rare autosomal recessive lysosomal storage disorder previously described in only 20 patients. MPS IIID is caused by a deficiency of N‐acetylglucosamine‐6‐sulphate sulphatase (GNS), one of the enzymes required for the degradation of heparan sulphate. So far only seven mutations in the GNS gene have been reported. The clinical phenotype of 12 new MPS IIID patients from 10 families was studied. Mutation analysis of GNS was performed in 16 patients (14 index cases). Clinical signs and symptoms of the MPS IIID patients appeared to be similar to previously described patients with MPS III. Early development was normal with onset of behavioral problems around the age of 4 years, followed by developmental stagnation, deterioration of verbal communication and subsequent deterioration of motor functions. Sequence analysis of the coding regions of the gene encoding GNS (GNS) resulted in the identification of 15 novel mutations: 3 missense mutations, 1 nonsense mutation, 4 splice site mutations, 3 frame shift mutations, 3 large deletions and 1 in‐frame small deletion. They include the first missense mutations and a relatively high proportion of large rearrangements, which warrants the inclusion of quantitative techniques in routine mutation screening of the GNS gene. © 2010 Wiley‐Liss, Inc.