Successful Treatment with Cyclosporine A of HCV-Driven Chronic Liver Disease Mimicking Autoimmune Hepatitis in a Patient with Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) is the commonest primary immunodeficiency disease characterized by defective antibody production and various degrees of T cell numbers abnormality or impaired proliferation to mitogens. Clinical features include recurrent bacterial sinopulmonary and gastrointestinal infections. Autoimmunity is very common in CVID, occurring in approximately 25% of the patients particularly with autoimmune thrombocytopenia, hemolytic anemia, inflammatory bowel disease, and rheumatoid arthritis. Persistent antigen stimulation, secondary to a defective eradication of pathogens followed by a compensatory exaggerated chronic inflammatory response, is the primary cause leading to autoimmunity. Here we describe a girl with CVID in whom a chronic liver disease mimicking autoimmune hepatitis developed after hepatitis C virus infection. The immunosuppressive treatment with cyclosporine A proved effective in reversing liver disease.     

Leukocyte and platelet activation in patients with giant cell arteritis and polymyalgia rheumatica: A clue to thromboembolic risks?

Ischemia is a leading causes of morbidity in giant cell arteritis (GCA). We studied circulating platelets and leukocytes in patients with GCA and with polymyalgia rheumatica. Normal healthy donors (>60 a) served as controls. Patients had a significantly greater fraction of platelets expressing P-selectin, of platelet–Nph and platelet–Mo aggregates, and of Nph and Mo expressing tissue factor. These differences were correlated with the percentage of platelets expressing P-selectin and were not influenced by clinical features or by systemic inflammation. Activated circulating leukocytes and platelets could contribute to indolent vessel inflammation and possibly to thromboembolic events in patients with systemic large vessel vasculitis.     

Open-source open-access reaction time test (OORTT): an easy tool to assess reaction times

Neurological Sciences - The speed of information processing is one of the most reliable indices of cognitive efficiency. The most common way to evaluate this ability is to assess reaction times...     

Tooth loss in complying and non-complying periodontitis patients with different periodontal risk levels during supportive periodontal care

Clinical Oral Investigations - To evaluate yearly tooth loss rate (TLR) in periodontitis patients with different periodontal risk levels who had complied or not complied with supportive periodontal...     

Neural Crest‐Specific TSC1 Deletion in Mice Leads to Sclerotic Craniofacial Bone Lesion

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutations in either TSC1 or TSC2. TSC has high frequency of osseous manifestations such as sclerotic lesions in the craniofacial region. However, an animal model that replicates TSC craniofacial bone lesions has not yet been described. The roles of Tsc1 and the sequelae of Tsc1 dysfunction in bone are unknown. In this study, we generated a mouse model of TSC with a deletion of Tsc1 in neural crest‐derived (NCD) cells that recapitulated the sclerotic craniofacial bone lesions in TSC. Analysis of this mouse model demonstrated that TSC1 deletion led to enhanced mTORC1 signaling in NCD bones and the increase in bone formation is responsible for the aberrantly increased bone mass. Lineage mapping revealed that TSC1 deficient NCD cells overpopulated the NCD bones. Mechanistically, hyperproliferation of osteoprogenitors at an early postnatal stage accounts for the increased osteoblast pool. Intriguingly, early postnatal treatment with rapamycin, an mTORC1 inhibitor, can completely rescue the aberrant bone mass, but late treatment cannot. Our data suggest that enhanced mTOR signaling in NCD cells can increase bone mass through enlargement of the osteoprogenitor pool, which likely explains the sclerotic bone lesion observed in TSC patients. © 2015 American Society for Bone and Mineral Research.     

Secondary prevention of cryptogenic stroke in patients with patent foramen ovale: a systematic review and meta-analysis

The aim of our study is to compare patent foramen ovale (PFO) closure versus medical treatment and antiplatelet versus anticoagulant therapy in patients with cryptogenic stroke (CS) and PFO. We conducted a systematic review and meta-analysis with trial sequential analysis (TSA) of randomized trials. Primary outcomes are stroke or transient ischemic attack (TIA) and all-cause mortality. Secondary outcomes are peripheral embolism, bleeding, serious adverse events, myocardial infarction and atrial dysrhythmias. We performed an intention to treat meta-analysis with a random-effects model. We include six trials (3677 patients, mean age 47.3 years, 55.8% men). PFO closure is associated with a lower recurrence of stroke or TIA at a mean follow-up of 3.88 years compared to medical therapy [risk ratio (RR) 0.55, 95% CI 0.38–0.81; I²?=?40%]. The TSA confirms this result. No difference is found in mortality (RR 0.74, 95% CI 0.35–1.60; I²?=?0%), while PFO closure is associated with a higher incidence of atrial dysrhythmias (RR 4.55, 95% CI 2.16–9.60; I²?=?25%). The rate of the other outcomes is not different among the two groups. The comparison between anticoagulant and antiplatelet therapy shows no difference in terms of stroke recurrence, mortality and bleeding. There is conclusive evidence that PFO closure reduces the recurrence of stroke or TIA in patients younger than 60 years of age with CS. More data are warranted to assess the consequences of the increase in atrial dysrhythmias and the advantage of PFO closure over anticoagulants.     

Doppler echocardiography in assessing mechanical and biological heart valve prostheses

The study was performed to assess Doppler echocardiographic features of mitral and aortic prosthetic valves of different types with both normal and abnormal function. Two hundred and twenty-three patients with 250 prostheses were studied. Two hundred eight valves (111 mitral, 95 aortic and 2 tricuspid) were considered to be functioning normally after clinical examination, phonocardiography and M-mode and 2D echocardiography. This group enabled us to define normal Doppler echocardiographic findings for different types of prosthesis. In mitral position, peak (p) and mean (m) gradients were lower for disc prostheses and higher for ball and biological prosthetic valves; values of effective orifice area (A), calculated by pressure half-time method, were lower for biological and ball prostheses and higher in disc valves. Results were as follows: St. Jude (p 10.6 mmHg, m 3.9 mmHg, A 2.7 cm2), Duromedics (p 10.6, m 4.3, A 2.8), Bj?rk-Shiley (p 10.4, m 4, A 2.3), Omniscience (p 14.2, m 6.2, A 2.1), Starr-Edwards (p 15.9, m 5.4, A 2.1), Hancock (p 14.7, m 6, A 2), Carpentier (p 13.2, m 5.4, A 1.9). Mild regurgitation, considered "physiological", was found in 2/8 Carpentier valves and in 3/34 St. Jude prostheses. In aortic valves lower peak gradients were found in Lillehei (18.3 mmHg), St. Jude (23.8 mmHg), Bj?rk-Shiley (26 mmHg), Duromedics (27 mmHg) and higher values in Starr-Edwards (30.2 mmHg), Hancock (30 mmHg) and Omniscience (35.5 mmHg) prostheses. Mild regurgitation, considered "physiological", was found in 17% of Omniscience valves, 21% of Hancock, 33% of Duromedics, 45% of St. Jude, 60% of Bj?rk-Shiley prostheses. Hancock mitral valves implanted for over 7 years had a mean gradient higher than valves with a shorter period of implantation (7.6 vs 4.85 mmHg, p less than 0.1), whereas the effective orifice area was similar. Hancock aortic valves implanted for over 7 years had a peak gradient slightly higher than the other group (implantation less than 7 years previously), but the difference was not statistically significant. Forty-two valves (19 aortic and 23 mitral) were considered to be malfunctioning. Regurgitation Doppler signals of malfunctioning valves appeared different from those of "physiological" reverse flow; in the former cases forward gradient was higher than normal prostheses. In stenotic aortic prostheses, peak systolic gradient was greatly increased; in stenotic mitral prostheses, a very significant increase in mean gradient and a great decrease in effective orifice area were found. In 14 patients who underwent surgical re-operation and in the patient who died before operation, Doppler echocardiographic findings were confirmed.(ABSTRACT TRUNCATED AT 400 WORDS)     

Lymph node pick up by separate stations: Option or necessity

AIM: To evaluate whether lymph node pick up by separate stations could be an indicator of patients submitted to appropriate surgical treatment. METHODS: One thousand two hundred and three consecutive gastric cancer patients submitted to radical resection in 7 general hospitals and for whom no information was available on the extension of lymphatic dissection were included in this retrospective study. RESULTS: Patients were divided into 2 groups: group A, where the stomach specimen was directly formalinfixed and sent to the pathologist, and group B, where lymph nodes were picked up after surgery and fixed for separate stations. Sixty-two point three percent of group A patients showed 16 retrieved lymph nodes compared to 19.4% of group B(P 0.0001). Group B(separate stations) patients had significantly higher survival rates than those in group A [46.1 mo(95%CI: 36.5-56.0) vs 27.7 mo(95%CI: 21.3-31.9); P = 0.0001], independently of T or N stage. In multivariate analysis, group A also showed a higher risk of death than group B(HR = 1.24; 95%CI: 1.05-1.46).CONCLUSION: Separate lymphatic station dissection increases the number of retrieved nodes, leads to better tumor staging, and permits verification of the surgical dissection. The number of dissected stations could potentially be used as an index to evaluate the quality of treatment received.