A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study

Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment.We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1­75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE.     

Association of interleukin (IL)-4 gene intron 3 VNTR polymorphism with multiple sclerosis in Turkish population

Objective

Genetic risk factors are known to contribute to the etiology of multiple sclerosis (MS). Interleukin (IL)-4 gene polymorphisms have been associated with immune-mediated diseases. The aim of this study was to explore the frequency of IL-4 gene intron 3 VNTR (variable number tandem repeat) polymorphism in a cohort of Turkish patients with MS.

Methods

The study included 125 patients with MS and 160 healthy controls. Genomic DNA was isolated and genotyped using polymerase chain reaction (PCR) analyses for the IL-4 gene intron 3 VNTR polymorphism.

Results

The distribution of genotype and allele frequencies of IL-4 gene intron 3 VNTR polymorphism was statistically different between MS patients and control group (p = 0.003 and p = 0.002, respectively). There were no statistically significant association between IL-4 VNTR polymorphism and clinical and demographical characteristics of MS patients.

Conclusion

The results of this study suggest that intron 3 VNTR polymorphism of the IL-4 gene was positively associated with predisposition to develop MS in Turkish population.     

In vitro study of fracture strength of provisional crown materials

PURPOSE

The purpose of this report was to evaluate the effect of the fabrication method and material type on the fracture strength of provisional crowns.

MATERIALS AND METHODS

A master model with one crown (maxillary left second premolar) was manufactured from Cr-Co alloy. The master model was scanned, and the data set was transferred to a CAD/CAM unit (Yenamak D50, Yenadent Ltd, Istanbul, Turkey) for the Cercon Base group. For the other groups, temporary crowns were produced by direct fabrication methods (Imident, Temdent, Structur Premium, Takilon, Systemp c&b II, and Acrytemp). The specimens were subjected to water storage at 37℃ for 24 hours, and then they were thermocycled (TC, 5000×, 5-55℃) (n=10). The maximum force at fracture (Fmax) was measured in a universal test machine at 1 mm/min. Data was analyzed by non-parametric statistics (α=.05).

RESULTS

Fmax values varied between 711.09-1392.1 N. In the PMMA groups, Takilon showed the lowest values (711.09 N), and Cercon Base showed the highest values (959.59 N). In the composite groups, Structur Premium showed the highest values (1392.1 N), and Acrytemp showed the lowest values (910.05 N). The composite groups showed significantly higher values than the PMMA groups (P=.01).

CONCLUSION

Composite-based materials showed significantly higher fracture strengths than PMMA-based materials. The CAD-CAM technique offers more advantages than the direct technique.     

Tissue factor pathway inhibitor and thrombin-activatable carboxypeptidase B for prediction of early atherosclerosis in gouty arthritis

Background

Gouty arthritis (GA) is a chronic inflammatory arthritis in which both clinical and subclinical atherosclerosis are more frequent. The dynamic equilibrium between coagulation and fibrinolysis is impaired in inflammatory diseases. We determined TFPI and TAFI antigen levels in GA patients and evaluated their association with subclinical atherosclerosis.

Methods

We included 45 GA patients (41 males, 4 females; mean age: 51.6 years) and 25 asymptomatic hyperuricemic (AHU) subjects (19 males, 6 females; mean age: 48.1 years). Cardiovascular risk factors were determined. TAFI and TFPI levels were determined by ELISA. B-mode ultrasonography was used to detect subclinical atherosclerosis.

Results

Cardiovascular risk factors were similar in both groups. The carotid IMT was significantly higher in GA group than in AHU group (0.74 ± 0.23 mm vs. 0.61 ± 0.13 mm, p = 0.009). TFPI level was significantly higher in GA group than in AHU group (86.2 ± 48.9 ng/mL vs. 25.8 ± 21.4 ng/mL, p < 0.001); TAFI antigen was significantly higher in AHU group (22.6 ± 3.6 ng/mL vs. 25.7 ± 5.3 ng/mL, p = 0.006) than in GA patients. Atherosclerotic plaque formation was more frequent in GA group (p = 0.041). When GA patients with and without plaques were compared, the first group had significantly higher mean age (p = 0.01) and TFPI level (p = 0.028). TFPI level correlated with carotid IMT (r = 0.302; p = 0.028). Logistic regression analysis showed that age (OR: 1.236, 95%CI: 1.059-1.443, p = 0.007) and TFPI (OR: 1.031, 95%CI: 1.008-1.054, p = 0.008) were independent risk factors for the presence of plaques.

Conclusions

GA patients had more frequent subclinical atherosclerosis than subjects with AHU. Higher TFPI levels in GA patients –probably associated with enhanced endothelial damage- were related to subclinical atherosclerosis. Lower TAFI levels in GA pointed to impaired fibrinolysis.     

A case of cellulitis causing tissue defect during etanercept therapy

Tumor necrosis factor-alpha (TNF-α) antagonists are employed increasingly during recent years in patients with active rheumatoid arthritis who do not respond to disease-modifying anti-rheumatic drugs. Contraindications such as infections, auto-antibody formation and hypersensitive reactions can be observed during the treatment with TNF-α antagonist drugs. Our case was a 52-year-old woman, followed by several centers for a period of 21 years with a seropositive, erosive and nodular RA diagnosis. Anti TNF-α treatment was commenced due to the failure to control the disease. During the treatment, a serious cellulite developed, which required hospitalization and surgical debridement as well as intravenous antibiotics treatment. Through the present case, we aimed to draw attention to the skin infection during the use of etanercept in a patient with RA.     

Suprathel®‐assisted surgical treatment of the hand in a dystrophic epidermolysis bullosa patient

Epidermolysis bullosa (EB) is a progressive familial disorder composed of dermal mucosal blisters, flexion contractures and pseudosyndactylies. Flexion contractures and pseudosyndactyly can be treated with surgery but usually require skin grafting. Because of poor wound healing, skin graft harvesting is a challenge in these patients. In order to prevent donor‐site morbidities due to skin graft harvesting some alloplastic materials were introduced. In this study, we focused on Suprathel^® as a new allograft material for covering the skin defects of a patient with dystrophic EB.     

Prenatal diagnosis and postnatal outcome of fetal intracranial hemorrhage

Background

The aim of this study was to present our experience with six cases of fetal intracranial hemorrhage (ICH) in terms of prenatal diagnostic features, and postnatal outcome.

Methods

The database of prenatal diagnosis unit was searched for antenatally diagnosed ICH cases. Maternal characteristics, ultrasound (US), and magnetic resonance imaging (MRI) findings, clinical course, and postnatal outcome were noted.

Results

We evaluated six consecutive cases of fetal ICH. One case was terminated at 24 weeks, and remaining five cases were delivered between 34 and 38 weeks. Five cases (5/6) had intraventricular, and one (1/6) had intraparenchymal hemorrhage. Hemorrhages were right sided in five cases (5/6), left sided in one case (1/6). Dilated and echogenic ventricular wall were the common US findings. No predisposing factor was detected in four of the cases, and intrauterine growth restriction was an underlying factor in two fetuses. Intrauterine progression of the hydrocephaly, and parenchymal thinning was seen in four cases (4/6). In three of four cases (3/4) with progressive grade 3–4 hemorrhage and hydrocephaly, postnatal outcome were dismal, and one case had mild neurological impairment at three months. In one case which had non-progressive mild ventriculomegaly, the lesion regressed after 4 weeks, and had normal short-term outcome

Conclusion

Fetal ICH can be accurately identified and categorized by antenatal sonography, and fetal MRI. Although intrauterine regression or normal short-term postnatal outcome is possible, the outcome is usually poor for fetuses with high grade and/or progressive lesions. Therefore, further studies assessing long-term postnatal outcome are needed     

Therapeutic Hotline: Treatment of prurigo nodularis and lichen simplex chronicus with gabapentin

Psychocutaneous conditions are frequently encountered in dermatology practice. Prurigo nodularis and lichen simplex chronicus are two frustrating conditions that are classified in this category. They are often refractory to classical treatment with topical corticosteroids and antihistamines. Severe, generalized exacerbations require systemic therapy. Phototherapy, erythromycine, retinoids, cyclosporine, azathiopurine, naltrexone, and psychopharmacologic agents (pimozide, selective serotonin reuptake inhibitor antidepressants) were tried with some success. Here five cases with lichen simplex chronicus and four cases with prurigo nodularis, who responded well to gabapentin, are presented.