A multicenter report of biologic agents for the treatment of secondary amyloidosis in Turkish rheumatoid arthritis and ankylosing spondylitis patients

In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.7) and 30 RA patients (11M, 19F, mean age: 51.7) with AA amyloidosis from 16 different centers in Turkey were included. Clinical and demographical features of patients were obtained from medical charts. A composite response index (CRI) to biologic therapy—based on creatinine level, proteinuria and disease activity—was used to evaluate the efficacy of treatment. The mean annual incidence of AA amyloidosis in RA and AS patients was 0.23 and 0.42/1000 patients/year, respectively. The point prevalence in RA and AS groups was 4.59 and 7.58/1000, respectively. In RA group with AA amyloidosis, effective response was obtained in 52.2 % of patients according to CRI. RA patients with RF positivity and more initial disease activity tended to have higher response rates to therapy (p values, 0.069 and 0.056). After biologic therapy (median 17 months), two RA patients died and two developed tuberculosis. In AS group, 45.7 % of patients fulfilled the criteria of good response according to CRI. AS patients with higher CRP levels at the time of AA diagnosis and at the beginning of anti-TNF therapy had higher response rates (p values, 0.011 and 0.017). During follow-up after anti-TNF therapy (median 38 months), one patient died and tuberculosis developed in two patients. Biologic therapy seems to be effective in at least half of RA and AS patients with AA amyloidosis. Tuberculosis was the most important safety concern.     

Topiramate‐induced nephrolithiasis

Nephrolithiasis is a less common side effect of the antiepileptic drug topiramate. We report the case of a 3‐year‐old boy who presented to the emergency department with abdominal pain; examinations revealed a large calcification in the left kidney. Regular ultrasound examinations are recommended in children using topiramate.     

Embedded NiTi Wires for Improved Dynamic Thermomechanical Performance of Silicone Elastomers

The extraordinary properties of shape memory NiTi alloy are combined with the inherent viscoelastic behavior of a silicon elastomer. NiTi wires are incorporated in a silicon elastomer matrix. Benefits include features as electrical/thermal conductivity, reinforcement along with enhanced damping performance and flexibility. To gain more insight of this composite, a comprehensive dynamic thermomechanical analysis is performed and the temperature- as well as frequency-dependent storage modulus and the mechanical loss factor are obtained. The analyses are realized for the composite and single components. Moreover, the models to express the examined properties and their temperature along with the frequency dependencies are also presented.     

Rhinoplasty in the black sea region

ABSTRACT: We have retrospectively evaluated the charts of 120 patients who had undergone rhinoplasties. The noses were divided into 2 types. Type 1 (42 cases) was characterized by a normal radix, high dorsum, and prominent vault. These noses were corrected by dorsal reduction, lateral osteotomy when needed, and caudal septal resection. Type 2 (78 cases) was characterized by a dependent tip with inadequate projection and a low radix with an excessive projected dorsal hump. These noses were corrected by tip support with cartilage graft and/or various suturing techniques and dorsal reduction. Secondary revision was necessary in 26 patients (21.6% of the total number) mostly due to inadequate septal correction in the first operation and irregularities of the dorsum. The postoperative result was considered as good in 88 (81%), fair in 27 (15%), and poor in 5 (4%) by the patients. Although the cases presented in this series were mostly difficult ones, experience was found to be the major determinant in success while dealing with the challenging noses of the Black Sea Region.     

Effect of anti-TNF treatment on sleep problems in ankylosing spondylitis

Sleep disturbances and problems are increased in ankylosing spondylitis (AS). But much is not known in a quantitative way about sleep problems and effect of treatments on AS. This study is aimed first, to investigate sleep disturbances in AS and secondly, to evaluate the effects of anti-TNF treatment on SD in AS. One hundred seventy-one (Female/male: 90/81) AS patients fulfilling modified New York criteria and 86 (F/M: 56/30) age- and gender-matched controls without inflammatory diseases were included into the study. Demographic data and disease activity and treatments were recorded using The Bath Ankylosing Spondylitis Functional Index (BASFI) and The Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). The Medical Outcomes Study (MOS) Sleep Questionnaire was used for evaluating sleep and problems of sleep. AS patients had higher sleep disturbance scale (SDS) and sleep problem index (SPI) II scores. Group A (patients using NSAID and/or DMARD, 53.2% of patients) had higher BASDAI and BASFI compared with Group B (Patients using anti-TNF treatments) (4.29?±?2.38 vs. 2.46?±?2.32, p?<?0.001; 1.95?±?2.15 vs. 0.93?±?1.31, p?<?0.001, respectively). Whereas Group A had higher scores of SDS, awaken short of breath or headache, somnolence, and SPI-II than controls, none of the sleep parameters were statistically different between patients on anti-TNF treatments and controls. BASDAI was positively correlated with SPI-I, SPI-II, SDS, and somnolence scale. AS patients had increased sleep problems and disturbances compared with controls. Anti-TNF agents improve significantly these problems. Sleep problems are significantly correlated with the disease activity.     

Sonographic assessment of carpal tunnel syndrome in rheumatoid arthritis: prevalence and correlation with disease activity

Carpal tunnel syndrome (CTS) is one of the most frequent extra-articular manifestations of rheumatoid arthritis (RA). High frequency ultrasonography (US) is a sensitive and specific method in diagnosis of CTS. This study is aimed to: firstly assess diameter frequency of CTS in RA with US and compare with a control group; secondly, investigate relationship of CTS with disease activity. One hundred consecutive RA patients (women/men: 78/22) fulfilling ACR 1987 RA criteria and 45 healthy controls (women/control: 34/11) were enrolled into study. Disease activity parameters, RA and CTS patient global assessment and health assessment questionnaire (HAQ-DI) were recorded. Both patient and control group were questioned about secondary causes of CTS, and Katz hand diagram, Boston CTS questionnaire and Phalen ve Tinel tests were applied once for each hand. Wrist joint and carpal tunnel were assessed with US grey scale and power Doppler US, then cross-sectional area of median nerve (CSA) was calculated. Patients with median nerve CSA between 10.0 and 13.0 mm(2) were evaluated with electromyography (EMG). CTS was diagnosed if CSA of median nerve >13.0 mm(2) or CTS was shown with NCS. Although there was no difference between RA patients and controls in age, sex, history of DM (+) and goitre, CTS was more frequent in RA group (respectively, 17.0% vs. 4.4%, P = 0.038). In RA group with CTS, age, history of DM, disease duration, HAQ-DI score, CTS patient global score, Boston symptom severity and functional status scores were elevated compared to without CTS [respectively, 57 (36-73) vs. 50 (24-76), P = 0.041; 35.3% vs. 6.0%, P < 0.001; 108 (12-396) months vs. 72 (6-360) months, P = 0.036; 1.93 (0.75-2.87) vs. 1.125 (0-2.75), P = 0.013; 52 (1-97) vs. 25 (0-91), P = 0.001; 2.81 (1.18-4.17) vs. 2.0 (1.0-4.01), P = 0.01; 3.37 (1.37-5.0) vs. 2.25 (1.0-5.0), P = 0.008]. No difference was found between CTS (+) and (-) RA patients in acute phase reactants, disease activity and US findings (P > 0.05). Sensitivity of Katz hand diagram was higher than Tinel and Phalen tests (respectively, 100, 60.0, 66.7%). Boston symptom and functional scores of RA patients with CTS diagnosed by EMG were increased than patients CTS (-) by EMG [respectively, 3.05 (1.90-4.27) vs. 1.55 (1.0-2.90), P = 0.002; 3.25 (1.73-3.82) vs. 1.12 (1.0-2.10), P = 0.008]. CTS frequency in RA was found higher than normal population, especially in patients with additional risk factors of CTS. There was no relationship between CTS and disease activity. CTS group had long disease duration and worse functional status. CTS could be a result of the chronic course in RA. In patient with CSA between 10 and 13 mm(2), Boston CTS questionnaire might give additional idea about CTS.     

Nasopharyngeal sarcoidosis: a rare involvement

Sarcoidosis is a chronic, multisystemic, non-caseating granulomatous disease of unknown etiology. Nasopharyngeal involvement is very rare in sarcoidosis. The objective of this report was to evaluate a rare involvement of sarcoidosis. This report includes a case of nasopharyngeal sarcoidosis. A 51-year-old female with nasopharyngeal sarcoidosis was treated as sarcoidosis, and she was better. Nasopharyngeal involvement is very rare in sarcoidosis but it must be kept in mind.     

Aspirin, clopidogrel, and warfarin use and outcomes in a cohort of 580 patients discharged after hospitalization for decompensated heart failure

The benefits of taking of aspirin, clopidogrel, and warfarin in relation to cardiovascular mortality and re-hospitalization in chronic heart failure (HF) patients have been called into question. We examined the outcomes (cardiac mortality and/or HF re-hospitalization) in patients discharged from our hospital between January 2003 and July 2009 after hospitalization for chronic decompensated HF. Of 580 HF patients (mean age, 63?±?13?years; mean ejection fraction, 26?±?9%, 63% with coronary disease and 37% without coronary disease), 207 patients (36%) died due to cardiovascular reasons, and 313 (54%) required HF re-hospitalization for decompensated HF during a 39?±?14?month follow-up period. 101 (17%) patients were taking clopidogrel during enrollment in the study. When comparing patients who were on clopidogrel treatment with those who were not, clopidogrel was found to have a beneficial effect on cardiac mortality (27 vs. 38%, P?=?0.04). In conclusion, in this observational prospective study, patients who used clopidogrel showed decreased cardiac mortality [HR, 0.566 (95% CI 0.332-0.964), P?=?0.036] compared to patients who did not take clopidogrel. Clopidogrel had a beneficial effect on the survival of chronic HF patients in the long term.     

Inverse relationship between cirrhosis and massive tumours in hepatocellular carcinoma

Background

A subset of patients with hepatocellular carcinoma (HCC) present with massive tumours. It is unknown why certain patients develop these massive tumours, and whether this presentation is specific to the underlying viral aetiology or patient demographics such as gender, race and age.

Methods

All patients with HCC at Bellevue Hospital Center, New York from 1998 to 2012 were identified and relevant demographic and clinical information was collected. Computed tomography/magnetic resonance imaging (CT/MRI) images were reviewed and the maximal tumour diameter on axial sections was recorded. Cirrhosis was defined histologically or by radiographical criteria. The two cohorts of massive and non-massive HCC were compared.

Results

A total of 361 patients with HCC were identified, of which 58 were categorized as having a massive HCC using a 13 cm size cut-off. Univariate and multivariate analysis demonstrated a significant association of massive HCC with age <40 years; hepatitis B or Asian ethnicity; and a lack of cirrhosis or platelet count >100.

Discussion

Massive HCC represents a tumour subtype that is associated with young, chronic hepatitis B carriers with non-cirrhotic livers. The clinical implications of this finding are that patients with massive HCC are typically excellent resection candidates barring the presence of gross vascular invasion or distant metastases.     

The safety of a pancreaticoduodenectomy in patients older than 80 years: risk vs. benefits

Background: A pancreaticoduodenectomy (PD) offers the only chance of a cure for pancreatic cancer and can be performed with low mortality and morbidity. However, little is known about outcomes of a PD in octogenarians. Methods: Differences in two groups of patients (Group Y, <80 and Group O, ≥80 year-old) who underwent a PD for pancreatic adenocarcinoma were analysed. Study end-points were length of post-operative stay, overall morbidity, 30-day mortality and overall survival. Results: There were 175 patients in Group Y (mean age 64 years) and 25 patients in Group O (mean age 83 years). Octogenarians had worse Eastern Cooperative Oncology Group (ECOG) Performance Status (PS ≥1: 90% vs. 51%) and American Society of Anesthesiology (ASA) score (>2: 71% vs. 47%). The two groups were similar in underlying co-morbidities, operative time, rates of portal vein resection, intra-operative complications, blood loss, pathological stage and status of resection margins. Octogenarians had a longer post-operative stay (20 vs. 14 days) and higher overall morbidity (68% vs. 44%). There was a single death in each group. At a median follow-up of 13 months median survival appeared similar in the two groups (17 vs. 13 months). Conclusions: As 30-day mortality and survival are similar to those observed in younger patients, a PD can be offered to carefully selected octogenarians.