IgG4-related disease and ANCA positive vasculitis in childhood: a case-based review

Clinical Rheumatology - Autoimmune pancreatitis (AIP) type 1 is an IgG4-related disease (IgG4-RD), characterized by inflammatory pseudotumors and histologically by dense lymphoplasmacytic...     

Blastic Plasmacytoid Dendritic Cell Neoplasm: Single Center Experience on a Rare Hematological Malignancy

PurposeBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and poor prognostic hematological malignancy. There is still no standard treatment established for BPDCN patients. We aim to summarize the main clinical, biological features and treatment of 9 BPDCN patients.MethodsNine patients with BPDCN who had been diagnosed between July 2008 and December 2018 in Ankara University School of Medicine, were retrospectively evaluated.ResultsAll patients (n = 9) were male, median age was 64 (21–80). Five patients (55.6%) had bone marrow infiltration, 5 patients (55.6%) cutaneous lesions, 6 patients (66.7%) lymph node involvement, 2 patients (22.2%) central nervous system involvement and 2 patients (22.2%) spleen involvement at time of diagnosis. Complex karyotype was observed in 2 patients. CHOP was given to 5 patients (55.6%), hyper-CVAD to 2 patients (22.2%), fludarabine, cyclophosphamide and mitoxantrone to 1 patient (11.1%) and cyclophosphamide, etoposide, methylprednisolone to 1 patient (11.1%) as first line chemotherapy. Four patients (44.4%) underwent allogeneic hematopoietic stem cell transplantation (AHSCT) in complete remission (CR) 1. Venetoclax was given to a transplant ineligible patient who had skin and lymph node involvement, with the off-label use. The median follow-up time was 15.9 months (3–48.6 months). Estimated median overall survival was 15.9 + 1.6 (95% CI 12.7–19.1) months.ConclusionIntensive induction therapies followed by AHSCT in CR seems to be best approaches for patients with BPDCN. Thus, more effective treatment strategies particularly targeted therapies should be warranted to improve the survival of patients with this rare disease.     

Microbiological and SEM assessment of atraumatic restorative treatment in adult dentition

Objectives

To investigate the practicability of atraumatic restorative treatment (ART) in adults in terms of marginal adaptation of restorations and microbiological changes in residual carious dentin.

Materials and methods

The occlusal dentin caries of 25 permanent molar teeth were removed with hand instruments. The total counts of bacteria (TCB) and the facultative anaerobic bacteria (FAB), mutans streptococci (MS), and Lactobacillus spp. (LB) counts in the affected dentin were evaluated quantitatively. The weights of the samples were measured with an electronic balance (Shimadzu, Type AX200, Japan). The cavities were restored with glass ionomer cement (Ketac^TM Molar Easymix, ESPE Dental AG, Seefeld, Germany). Twenty replicas of randomly selected ART restorations were prepared and marginal adaptation was evaluated by scanning electron microscopy (SEM). After 6 months, the same protocols were repeated. Data were analyzed with paired sample t-tests, Wilcoxon t-tests, Pearson and Spearman correlations, and chi-square tests (p<0.05).

Results

In the sixth month, restoration loss and pulpitis were not observed. The mean weight of samples removed from the cavity floor was less than the baseline (0.014±0.009 and 0.023±0.013 g, respectively) (p<0.01), and the counts of total bacteria, FAB, MS, and LB significantly decreased compared to baseline (p<0.01). The frequency of marginal gaps was increased (p< 0.01).

Conclusions

ART showed that the counts of microorganisms decreased after 6 months although the marginal gap rates of restorations increased.

Clinical relevance

ART can be a reliable treatment approach in adults for 6 months due to the decrease in microorganism counts, although gaps exist.

     

Evaluation of Salivary Procalcitonin Levels in Different Periodontal Diseases

Background: The present study aims to investigate the levels of salivary procalcitonin (ProCT) in patients with different periodontal diseases. Methods: Seventy‐two non‐smokers are included in this study: 21 individuals with chronic periodontitis (CP), 14 individuals with generalized aggressive periodontitis (GAgP), 18 individuals with gingivitis (G), and 19 periodontally healthy (H) participants. Clinical periodontal parameters, including probing depth (PD), clinical attachment level (CAL), plaque index, and gingival index (GI), were assessed in all participants. Saliva samples were collected and examined for evaluating ProCT levels. Results: It was found that the median (interquartile range) salivary ProCT level was lowest in the H group: 0.00 (0.09) ng/mL; followed by the G group: 0.09 (0.11) ng/mL; the CP group: 0.15 (0.29) ng/mL; and highest in the GAgP group 0.28 (0.68) ng/mL. These differences were statistically significant between the H group and the other groups (P <0.05). There were positive correlations between the mean salivary ProCT level and GI, CAL, and PD. Conclusion: According to the present results, ProCT might play a role during periodontal inflammation, and an elevated salivary ProCT level is suggested as a potential biomarker for periodontal diseases.     

No alteration in the PFA-100 in vitro bleeding time induced by the Ginkgo biloba special extract, EGb 761, in elderly patients with mild cognitive impairment.

EGb 761 is widely used in the management of mild cognitive impairment in the elderly population. Elucidation of the effects of EGb 761 on primary haemostasis via PFA-100 could represent an important step for better understanding of the haemostatic safety of EGb 761. The purpose of this prospective study is to assess the effects of Ginkgo biloba special extract, EGb 761, on PFA-100 in vitro bleeding time in elderly patients with mild cognitive impairment. A total of 40 elderly patients aged 65-79 years who were referred for geriatric assessment and who were diagnosed as having mild cognitive impairment were included. Patients were started on 80 mg EGb-761 three times daily. The complete set of PFA-100 in vitro bleeding time and coagulation parameters including prothrombin time, activated partial thromboplastin time and International Normalized Ratio were assessed before and on the seventh day of treatment with EGb 761. There was no statistically significant prolongation in PFA-100 in vitro bleeding time or coagulation parameters in patients receiving EGb 761 after 7 days. The data about the safety of EGb 761 from the point of primary haemostasis in our elderly patient population with mild cognitive impairment casts hope for the future management of this 'difficult-to-treat' population with the promising Ginkgo extracts.     

Small-volume gallbladders and decreased motility in patients with achalasia

AIM/BACKGROUND: Achalasia may be associated with extraesophageal dysmotility. However, this relation is still poorly understood. In the present study, we used noninvasive real-time ultrasonography to examine the motility function of the gallbladder in the patients with achalasia. MATERIALS AND METHODS: Thirty-three achalasic patients and 33 healthy volunteers were included in the study. All subjects were investigated after 12 hours of fasting and 30 minutes after a standard test meal. Premeal and postmeal gallbladder volumes were used for calculation of the ejection fraction of the gallbladder and fasting gallbladder volume. RESULTS: The mean fasting volume (18.52+/-1.45 vs. 24.63+/-1.84 cm; P<0.05) and ejection fractions of gallbladder (35.84+/-4.12 vs. 54.47+/-2.47; P<0.05) in the patients with achalasia were lower than the control group. CONCLUSIONS: Such a finding may confirm the possible extraesophageal extension of primary achalasia. Achalasic patients have smaller gallbladders than do others. It could be speculated that it is congenital and/or achalasic patients' gallbladder has incomplete relaxation (as in the lower esophageal sphincter of the achalasia).     

The comparison of high and standard definition computed tomography techniques regarding coronary artery imaging

ObjectiveThe aim was to compare coronary high-definition CT (HDCT) with standard-definition CT (SDCT) angiography as to radiation dose, image quality and accuracy.ResultsThe intraclass correlation coefficient (ICC) of measured vessel attenuation values in SDCT between the two radiologists was exceedingly good. The ICC was higher in HDCT. The radiation dose of HDCT was higher than that of SDCT. The mean tube current was 180 (mA) in HDCT and 147(mA) in SDCT with the same tube voltage (kVp). There was no significant difference between image quality.ConclusionHDCT has a higher radiation dose but has much more atenuation and the spatial resolution which improve measurement accuracy for imaging coronary arteries.