Sociodemographic, clinical, laboratory, treatment and prognostic characteristics of 156 generalized pustular psoriasis patients in Turkey: a multicentre case series.

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and severe inflammatory disease characterized by widespread and superficial sterile pustules on an erythematous background. OBJECTIVES: This multicentre study aimed to determine the clinical profile and course in a large cohort of patients with GPP. METHODS: One hundred and fifty-six GPP patients (mean age, 44.2 ± 18.7 years) who met the diagnostic criteria of the European Consensus Report of GPP were included in the study. Sociodemographic characteristics, quality of life, triggering factors of the disease, clinical, laboratory, treatment and prognostic features were evaluated. RESULTS: 61.5% of the patients were female. The rate of working at or below the minimum wage (≤$332.5/month) was 44.9%. Drugs (36.5%) were the most common trigger. While hypocalcaemia (35.7%) was the most important cause of GPP during pregnancy, systemic steroid withdrawal (20%) was the most frequently reported trigger for infantile/juvenile and mixed-type GPP (15%) (P < 0.05). Acute GPP (53.8%) was the most common clinic. Nails were affected in 43.6% of patients, and subungual yellow spots (28.2%) were the most common change. In annular GPP, fever (P < 0.001) and relapse frequency (P = 0.006) were lower than other subtypes, and the number of hospitalizations (P = 0.002) was lower than acute GPP. GPP appeared at a later age in those with a history of psoriasis (P = 0.045). DLQI score (P = 0.049) and joint involvement (P = 0.016) were also higher in this group. Infantile/juvenile GPP was observed in 16.02% of all patients, and arthritis was lower in this group (24.4 vs. 16%). GPP of pregnancy had the worst prognosis due to abortion observed in three patients. CONCLUSIONS: Recent advances in treatment have improved mortality associated with GPP, but abortion remains a significant complication. Although TNF-α inhibitors have proven efficacy in GPP, they can also trigger the disease. Mixed-type GPP is more similar to acute GPP than annular GPP with systemic manifestations and course.

Clinical characteristics, quality of life and risk factors for severity in palmoplantar pustulosis: a cross-sectional, multicentre study of 263 patients.

BACKGROUND: Palmoplantar pustulosis (PPP) is a rare, chronic, inflammatory skin disease characterized by sterile pustules on palmar or plantar areas. Data on PPP are scarce. AIM: To investigate the clinical characteristics and risk factors for disease severity in a large cohort of Turkish patients with PPP. METHODS: We conducted a cross-sectional, multicentre study of patients with PPP recruited from 21 tertiary centres across Turkey. RESULTS: In total, 263 patients (165 women, 98 men) were evaluated. Most patients (75.6%) were former or current smokers. The mean Palmoplantar Pustulosis Area and Severity Index (PPPASI) was 8.70 ± 8.06 and the mean Dermatology Life Quality Index (DLQI) score was 6.87 ± 6.08, and these scores were significantly correlated (r = 0.52, P < 0.001). Regression analysis showed that current smoking was significantly associated with increased PPPASI (P = 0.03). Coexisting psoriasis vulgaris (PsV) was reported by 70 (26.6%) patients. Male sex prevalence, PPP onset incidence, disease duration, DLQI, and prevalence of nail involvement and psoriatic arthritis (PsA) were significantly increased among patients with PPP with PsV. Of the 263 patients, 18 (6.8%) had paradoxical PPP induced by biologic therapy, and these patients had significantly increased mean DLQI and prevalence of PsA (r = 0.03, P = 0.001). CONCLUSION: Our data suggest that smoking is a risk factor for both PPP development and disease severity. Patients with PPP with PsV present distinct clinical features and patients with biologic therapy-induced paradoxical PPP have reduced quality of life and are more likely to have PsA.

Evaluation of neurodegenerative and inflammatory processes in temporomandibular joint disorders using optical coherence tomography.

INTRODUCTION: Temporomandibular joint disorders (TMD) is a process with systemic effects rather than local effects. The purpose of this study is to examine the retinal layer structure using optical coherence tomography (OCT) method in order to detect possible degenerative and inflammatory process in patients with TMD. MATERIAL AND METHODS: Thirty-five healthy controls and 34TMD patients were evaluated bilaterally in this study. The retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL) and choroidal thickness were analyzed using OCT. RESULTS: When TMD patients were compared with healthy controls, GCL and IPL volumes and RNFL thickness decreased; while choroidal thickness increased. In the unilateral TMD patients, there was no statistically significant difference in GCL, IPL, RNFL and choroidal thickness between the affected and the unaffected regions. DISCUSSION: OCT findings showed GCL and IPL volumes and RNFL thickness were decreased while choroidal thickness was increased in TMD patients. These findings suggest that TMD may cause neurodegeneration and inflammation.

Evaluation of biochemical variables in patients with trigeminal neuralgia.

The aim of this study was to evaluate the calcium, sodium, potassium, serum iron, vitamin B12, and albumin concentrations, and alkaline phosphatase (ALP) activity, in samples of serum from patients with primary trigeminal neuralgia (TN), and investigate the associations between them. Results from 73 patients who had been diagnosed with primary TN between December 2015 and 2017 were compared with those of 70 healthy subjects. Calcium (p=0.013), iron (p=0.004), and albumin (p=0.001) concentrations in the primary TN group were significantly lower than those in the control group, whereas the ALP activity was significantly higher in the TN group than in the control group (p=0.007). However, there was no significant difference in the sodium, potassium, or vitamin B12 concentrations. Biochemical variables have a role in the pathogenesis and treatment of primary TN, but there are few studies that characterise the relations between the condition and the biochemical changes. Further studies are therefore necessary to gain more information.

A comparison of the effects of Methylprednisolone Acetate, Sodium Hyaluronate and Tenoxicam in the treatment of non-reducing disc displacement of the temporomandibular joint.

BACKGROUND: This clinical study aimed to radiologically and clinically compare the effect of intra-articular injection of methylprednisolone, sodium hyaluronate or tenoxicam following arthrocentesis with that of arthrocentesis alone in patients with non-reducing disc displacement. MATERIAL AND METHODS: A total of 44 patients radiographically diagnosed with non-reducing disc displacement of the temporomandibular joint (TMJ) were randomly divided into four treatment groups, as follows: Group 1, arthrocentesis alone; Group 2, arthrocentesis plus methylprednisolone acetate; Group 3, arthrocentesis plus sodium hyaluronate; Group 4, arthrocentesis plus tenoxicam. Maximum mouth opening (MMO), lateral movement, pain severity and tenderness of TMJ and muscles of mastication on palpation were measured before treatment and at 1 week and 1, 3 and 6 months after treatment. Disc position, presence or absence of disc reduction, level of effusion, joint movement and joint space were also evaluated using magnetic resonance imaging (MRI) before treatment and 6 months after treatment. RESULTS: No significant differences in treatment success were found among the four groups. MRI findings did not vary significantly among the groups, but pre- and post-operative MRI findings varied significantly within all four groups (p<0.001). CONCLUSION: According to the data from this study, it may be concluded that either arthrocentesis alone or arthrocentesis with methylprednisolone acetate or sodium hyaluronate or tenoxicam intra-articular injections are similarly effective and promising methods in the treatment of TMJ with non-reducing disc displacement.

Expression of chemerin in the synovial fluid of patients with temporomandibular joint disorders.

The synovial membrane and fluid are significantly involved in the pathogenesis of temporomandibular joint (TMJ) disorders. This study aimed to investigate the relation between levels of chemerin in the synovial fluid (SF) of patients with TMJ disorder and their relationship. Sixty samples of SF were obtained from patients with an internal derangement (ID) or osteoarthritis (OA). Chemerin in the SF was examined by enzyme-linked immunosorbent assay (ELISA). The results showed greater levels of chemerin in the SF of patients with OA than ID. While chemerin levels were positively correlated with pain scores, they were inversely correlated with MMO. Chemerin levels increased progressively as the disorder stage became more severe. The findings of this study suggest that chemerin in SF may play role as a predisposing factor and may represent a novel potential prognostic biochemical marker in the pathogenesis of TMJ disorders.

The evaluation of hormonal and psychological parameters that affect bone mineral density in postmenopausal women.

OBJECTIVE: In this study we aimed to investigate the relationships between serum levels of DHEAS, reproductive hormones and low bone mineral density (BMD) in postmenopausal women. We also examined the relationship between psychological status of patients and their BMD results. PATIENTS AND METHODS: This study included postmenopausal female patients. BMD measurements were performed with dual-energy X-ray absorptiometry (DEXA). Psychological assessments of all cases were performed using the Hamilton Anxiety and Hamilton Depression scales. All patients provided fasting venous blood samples in order to determine serum levels of follicle stimulating hormone (FSH), luteinizing hormone (LH), estradiol (E2), and DHEAS. RESULTS: There were 33 cases (45.2%) with normal BMD levels and 40 cases (54.8%) with abnormal BMD levels (osteopenia and osteoporosis). DHEAS levels did not show any statistically significant difference according to BMD results (p = 0.431). The Hamilton Anxiety and Depression scores also did not show statistically significant differences in accordance with the BMD results (p = 0.889 and p = 0.706, respectively). CONCLUSIONS: According to our results, anxiety, depression and circulating DHEAS levels are not significantly associated with low levels of BMD, particularly at osteopenic levels. So these parameters are not useful for clinical practice in patients with low BMD in the middle-aged postmenopausal women.

Central field perimetry of discriminated targets: I. Results for normal individuals using high-contrast targets.

PURPOSE: A new perimetry method is described to evaluate central vision under multiple hue, illumination, and contrast conditions; results are described for normal individuals using high-contrast targets. METHODS: For 70 normal eyes of individuals with varying age, the ETDRS chart acuity was determined as well as discriminated target perimetry using high-contrast targets presented for 250 ms at locations up to 10 degrees eccentricity. Retesting was performed to evaluate for learning and repeatability for the thresholded acuity at each eccentricity. RESULTS: The ETDRS acuity averaged 1.15+/-0.37 arc min minimum angle of resolution (MAR) with best correction (20/23 equivalence). At fixation, the thresholded acuity for high-contrast targets paralleled the ETDRS acuity and averaged 1.75+/-0.85 arc min MAR (20/35 equivalence), and declined with increasing eccentricity in a linear fashion to 5.81+/-3.97 arc min (20/116 equivalent) at 10 degrees eccentricity. Linear regression showed Vmar=1.74+0.330 (*) eccentricity in degrees (R (2)=0.966, P<0.0001). Testing time required approximately 8-10 min per eye. Repeated testing showed only minimal learning experience at the most peripheral locations. The test-retest 95% confidence limits of difference measured 1.12 arc mins MAR at fixation (64% of the mean) and remained approximately the same proportion to the mean thresholded acuity outwards to 10 degrees eccentricity. CONCLUSION: The methodology seems rapid and reliable for measuring discriminatory visual function of optotype targets throughout the central 20 degrees diameter visual field.

Interactive, computer-based, self-reported, visual function questionnaire: the PalmPilot-VFQ.

PURPOSE: To evaluate the testing performance, reliability, and validity of a self-administered visual function questionnaire designed for a Palm Pilot in comparison with the interviewer-administered-National Eye Institute Visual Function Questionnaire-25 (NEI-VFQ-25) (interviewer-NEI-VFQ-25) and self-administered-NEI-VFQ-25 (self-NEI-VFQ-25). METHOD: The interviewer-NEI-VFQ-25 was administered to 135 sequential patients who visited a retina clinic, followed on separate days by the Palm Pilot-Visual Function Questionnaire (PalmPilot-VFQ) and self-NEI-VFQ-25. Rasch analysis of ordinal difficulty ratings for the PalmPilot-VFQ was used to estimate interval measures of perceived visual ability. Reliability was determined by calculating Cronbach's alpha and test-retest intraclass correlation coefficients (ICCs). Concurrent validity was determined by calculating correlations of the PalmPilot-VFQ score with that of a general vision question. For evaluating convergent validity, the PalmPilot-VFQ was compared with the interviewer-NEI-VFQ-25, habitual-correction visual acuity (HCVA), and with two visual analogue scale (VAS) questions. Performance time and testability were compared among the three questionnaires. RESULTS: Rasch analysis eliminated two items in the PalmPilot-VFQ due to poor-fit statistics. The final items showed internal consistency (Cronbach's alpha=0.89) and test-retest reliability (ICC=0.79), as well as an excellent separation index (3.23 and 4.01) for item parameters with significant concurrent correlation (P<0.0001). On evaluating convergent validity, the PalmPilot-VFQ showed strong correlations with interviewer-NEI-VFQ-25, with the HCVA of the better-seeing eye, and with the VAS questions (P=0.0001). Ninety percent of the 135 patients (HCVA >20/200 in the better-seeing eye) could perform the PalmPilot-VFQ with their habitual correction or high-plus spectacles, but in significantly less time than either interviewer-NEI-VFQ-25 or self-NEI-VFQ-25 (P<0.0001). CONCLUSION: The PalmPilot-VFQ seems to be a reliable, valid, interactive, computer-based, self-administered questionnaire that can be used routinely by physicians to evaluate functional vision disability in populations with a high prevalence of macular disease.

Relationships between clinical measures of visual function and anatomic changes associated with bevacizumab treatment for choroidal neovascularization in age-related macular degeneration.

PURPOSE: This pilot study was undertaken to examine the relationships between clinical measures of visual function and anatomic changes occurring in the eyes treated with bevacizumab for choroidal neovascularization (CNV) due to age-related macular degeneration (AMD). METHODS: A retrospective review was conducted for 50 eyes that had been treated with at least three injections of bevicizumab for CNV due to AMD, and followed for at least 6 months. Vision outcomes included best-corrected ETDRS chart acuity, scored by best-line read (ETDRS line) and by total letters read (ETDRS letter), and two measures obtained from central acuity perimetry with 98% Michelson contrast targets, the best acuity within 6 degrees of fixation (BA6 degrees ), and global macular acuity (GMA), representing a weighted average of the acuities thresholded at all intercepts within a 10 degrees radius of fixation. Assessment of anatomic outcomes included fibrosis, atrophy, and subretinal hemorrhage grading on fundus photography, CNV size, pigment epithelial detachment (PED) size and grading of CNV leakage on fluorescein angiography, and central retinal PED, and subretinal fluid (SRF) thickness on optical coherence tomography. RESULTS: Logistic regression analysis showed an association between the vision outcomes of EDTRS letter and BA6 degrees with the change in SRF thickness (R (2): 0.47 and 0.35, respectively). The outcome of the vision measurement of GMA was associated with the change in SRF thickness, in CNV thickness, and in CNV fibrosis grade (R (2): 0.34). No association was noted between the outcomes of ETDRS line with the change in any anatomic outcomes. CONCLUSION: Acuity perimetry outcomes in this study seemed to offer improved understanding of the relationship between the vision outcomes and the measured anatomic changes. It seemed that neither ocular coherence tomography nor fluorescein angiography alone offered sufficient morphologic markers for prediction of functional outcomes.

657del5 mutation in the NBS1 gene is associated with Nijmegen breakage syndrome in a Turkish family.

We report on a consanguineous Turkish family whose first son died of anal atresia and whose second son presented with severe pre- and post-natal growth retardation as well as striking microcephaly, immunodeficiency, congenital heart disease, chromosomal instability and rhabdomyosarcoma in the anal region. The proband was found to carry the homozygous 657del5 mutation in the NBS1 gene, which is responsible for Nijmegen breakage syndrome (NBS) in most of the Slav populations. Our family, the first diagnosed with NBS in the Turkish population, represents one of the most severely affected examples of the syndrome, with profound pre- and post-natal growth retardation associated with structural abnormalities, and expands the clinical spectrum of this rare disorder.

Serum levels and differential expression of CD44 in childhood leukemia and malignant lymphoma: correlation with prognostic criteria and survival.

BACKGROUND: The CD44, a cell surface proteoglycan, participates in a variety of function including tumor dissemination and metastasis. However, there are no available data on the prognostic significance of CD44 expression of tumor tissue correlated with serum sCD44 level in childhood leukemias and lymphomas. METHODS: Serum levels and leukemic cell tumor tissue expression of CD44 were detected in 54 children with acute leukemia and malignant lymphoma. Serum samples were obtained from all patients before treatment and during remission. Twelve age-matched healthy children were included as a control group. RESULTS: The serum CD44 levels were significantly higher in patients with Hodgkin's disease (HD), non-Hodgkin's lymphoma (NHL), Burkitt's lymphoma (BL) and acute lymphoblastic leukemia (ALL) than those in the control group. The median values were 1627.0, 1336.0, 1318.5, 1730.4, 902.7 ng/mL, respectively, and P<0.001, P<0.01, P<0.01, P<0.05 in comparisons, respectively. However, there was no significant difference between acute myeloid leukemia (AML) and the control group (median values: 900.3 and 902.7 ng/mL, respectively, P>0.05). Serum sCD44 levels significantly declined in HD, NHL and ALL patients who were in complete remission (median values: 684.0, 573.8 and 1101.1 ng/mL, respectively, P<0.05 in each comparison). Patients with HD had higher levels of serum sCD44 and correlated well with higher erythrocyte sedimentation rate (ESR), B-symptoms and advanced-stage disease (P<0.05, P<0.05 and P<0.01, respectively). Expression of CD44 was significantly high in patients with HD and NHL who were in advanced stages of disease. High serum CD44 level was also associated with high tumor tissue expression of CD44 in patients with HD and BL. In addition, patients with higher levels of serum sCD44, had a poorer outcome and survival than those with lower sCD44 levels in HD and NHL groups. CONCLUSIONS: A high serum sCD44 level and/or tumor tissue expression at diagnosis is associated with poor prognostic criteria and/or unfavorable outcome in childhood leukemias and lymphomas.

Anaesthesia for Friedreich's ataxia. Case report.

Friedreich's ataxia is an inherited neuromuscular disorder often associated with significant cardiac disease and requiring special care during anaesthesia because of increased sensitivity to muscle relaxants. We report a 37 years old female patient with Friedreich's ataxia who underwent anaesthesia for total hip replacement because of degenerative hip arthritis. Anaesthesia was induced with alfentanil and propofol. Endotracheal intubation was achieved without the use of any muscle relaxants and muscle relaxants were avoided throughout the operation. Anaesthesia was maintained with propofol infusion and intermittent bolus doses of alfentanil. At the end of the procedure recovery from anaesthesia was fast and uneventful. When there is no absolute indication for neuromuscular blocking agents as its the case for many orthopaedic operations, avoiding these drugs would simply avoid many potential complications due to muscle relaxant use in this group of patients.

Natural killer cell numbers and cytotoxic activity in pediatric Hodgkin disease.

In this study peripheral blood natural killer (NK) cell activity was evaluated in 17 pediatric cases with Hodgkin disease (HD) (9 untreated, 8 in remission) and 20 age-matched healthy children. Peripheral blood CD16 and CD56 molecule expressions were also examined. No difference related to NK cell numbers and cytotoxic activity was detected at either stage of the disease. In cases in which long-term remission has been achieved (> or = 5 years) NK cell activity was slightly but not significantly increased in parallel with remission duration. Finally, no relation between NK cell activity and the etiology, prognosis, and severity of the disease has been established in children with HD.

Immune thrombocytopenia and hemolytic anemia as a presenting manifestation of Hodgkin disease.

A very unusual clinical presentation of Hodgkin disease with immune thrombocytopenia and autoimmune hemolytic anemia is reported. A 6.5-year-old boy presented with thrombocytopenia, Coombs' positive hemolytic anemia, and multiple small posterior cervical lymph nodes. After a course of high-dose methylprednisolone therapy with a diagnosis of Evans syndrome, complete response for thrombocytopenia and partial response for anemia was achieved. Six weeks later there was a sudden increase in the size of left posterior cervical lymph nodes and a biopsy was compatible with Hodgkin disease, mixed cellularity type. The child was successfully treated with chemotherapy and radiation therapy. He has been off therapy for 28 months and has no clinical or laboratory evidence of autoimmune cytopenia. A combination of immune thrombocytopenia and autoimmune hemolytic anemia may be associated with Hodgkin disease. The recognition of this clinical picture as a complication of Hodgkin disease has important implications. This complication appeares to be managed best by the definitive treatment of Hodgkin disease.

Right atrial catheter-related complications in pediatric oncology patients: the situation in a developing country.

The complications of right atrial catheters (RACs) in pediatric oncology patients are unknown for centers in developing countries. This study examined the complications of RACs at Ankara University Medical School, Turkey. A total of 90 RACs were placed in 61 children for long-term chemotherapy with a total experience of 15,536 catheter days. The rate of catheter-related sepsis was 4.9 episodes per 1000 catheter days. Coagulase-negative staphylococci and Candida species were the most common organisms, accounting for 25.0 and 13.1% of all organisms, respectively. The most common reasons for the removal of the RACs were infection (42.4%) and dislodgement (32.2%). The rates of complications were significantly higher in this study than in western studies. This increase could be explained by the differences in catheter care practices in the Turkish center. In conclusion, the use of RACs in a developing country necessitates an appraisal of the benefits and risks for each patient and improvement of catheter care procedures.