Pyruvate kinase deficiency in 29 Turkish patients with two novel intronic variants.

Pyruvate kinase (PK) is a key enzyme of anaerobic glycolysis. The genetic heterogeneity of PK deficiency (PKD) is high, and over 400 unique variants have been identified. Twenty-nine patients who had been diagnosed as PKD genetically in seven distinct paediatric haematology departments were evaluated. Fifteen of 23 patients (65.2%) had low PK levels. The PK:hexokinase ratio had 100% sensitivity for PKD diagnosis, superior to PK enzyme assay. Two novel intronic variants (c.695-1G>A and c.694+43C>T) have been described. PKD should be suspected in patients with chronic non-spherocytic haemolytic anaemia, even if enzyme levels are falsely normal. Total PKLR gene sequencing is necessary for the characterization of patients with PKD and for genetic counselling.

Serum sRAGE levels in children with atopic dermatitis: a prospective study.

Introduction: Atopic dermatitis (AD) is chronic inflammatory skin disorder. The receptor for advanced glycation end products (RAGE) plays a role in inflammatory reactions. The soluble form of RAGE (sRAGE) acts as a decoy to inhibit interactions of RAGE. Aim: To determine serum sRAGE levels in children with AD. Material and methods: AD diagnosis was made according to Hanifin and Rajka criteria. Disease severity was scored by the scoring atopic dermatitis (SCORAD) index. Skin prick testing (SPT), total immunoglobulin E (Ig E) and eosinophil counts were analysed. The sRAGE levels were determined using ELISA technique. Results: The children, aged 0.4 to 2.0 years with AD (n = 65) were investigated in two groups according to the presence (AD+/Atopy+ [n = 40]) or absence (AD+/Atopy- [n = 25]) of SPT positivity. The comparisons were made with a healthy control group matched for age and sex. The medians (interquartile range) of sRAGE levels in patient and control groups were 8.43 (1.04-18.37) and 14.09 (6.35-28.64), respectively (p < 0.001). The medians (interquartile range) of sRAGE levels in AD+/Atopy+, AD+/Atopy- and control groups were 8.5 (3.1-17.27), 7.75 (1.04-18.37) and 14.09 (6.35-28.64), respectively (p = 0.004). Correlation analysis failed to reach significance with the disease severity sRAGE levels, total IgE levels and eosinophil counts. Conclusions: To our knowledge, this is the first study investigating the association of sRAGE levels with AD and disease severity in childhood. Serum sRAGE levels are decreased in AD but not correlated with disease severity. sRAGE levels may be important in the AD disease process.

Assessment of extracorporeal photopheresis related cell damage.

AIM: Extracorporeal photochemotherapy (ECP) is emphasized chiefly as it has a high safety profile. However, the genotoxic effects of ECP are not known. This experimental study aimed to assess the potential genotoxic impact of ECP treatment by the AKLIDES system, a new generation standardized and automated evaluation method. MATERIALS AND METHODS: Buffy coats were obtained from the blood of 26 healthy volunteers, and ECP was applied to 2 j/cm2 UV-A for two hours. After the DNA isolation procedure, all slides were stained with DAPI to visualize lymphocytes, FITC for visualization of damage foci marker (γH2AX), and APC for visualization of repair foci marker (53BP1). With the AKLIDES imaging system, all parameters were evaluated. RESULTS: Median damage marker Foci γ-H2AX before and after ECP were 11.42 and 18.65 arbitrary units, respectively (p = 0.153). Median repair marker foci 53BP1 (repair biomarker) before and after ECP were measured as 4.17 and 6.7 arbitrary units. The difference was also not statistically significant (p = 0.088). Although 58 % of cells were affected by ECP irradiation, as shown by FITC fluorescent staining, no statistical difference was found in any genotoxicity parameters. CONCLUSION: We found an increase in the foci γ-H2AX parameter, one of the objective indicators of DNA breaks, and an increase in the foci 53BP1 parameter, which indicates the post-damage repair mechanisms after ECP. However, further in vitro, and in vivo studies are needed with large sample volumes to demonstrate the significance.

Effects of ultrasound guided leukocyte-rich platelet-rich plasma (LR-PRP) injection in patients with pes anserinus tendinobursitis.

OBJECTIVES: To demonstrate the efficacy and safety of ultrasound guided leukocyte-rich platelet-rich plasma (LR-PRP) injection in patients with pes anserinus tendinobursitis (PATB). METHODS: A prospective, randomized and single-blinded study of 60 patients with PATB were randomly assigned into 2 groups. Whereas 2 mL LR-PRP injection was applied to one grup, once accompanied by ultrasonography (USG), 2 mL LR-PRP injection was applied to the other group accompanied by USG twice with a one-week interval. Visual Analog Scale (VAS), Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), 6-minute walking test (6MWT), Likert Scale were evaluated pre-treatment, at the 4th and 12th weeks after treatment. RESULTS: There was no statistical difference between the two groups in terms of age, gender, body mass index, duration of symptoms, affected side. When both groups are compared within themselves before and after treatment, there was a significant improvement in all VAS, in all WOMAC subgroups, 6MWT, at the 4th and 12th weeks after treatment. When the two groups are compared with each other, there was no statistical difference. In addition, when all patients were evaluated with Likert scale in the 12th week after treatment, complete healing in 22(36.7 %) patients, significant relief in 25(41.7 %) patients, mild relief in 4(6.7 %) patients, 5(8.3 %) same as before treatment patients, and worsened pain in 4(6.7 %) patients were seen. CONCLUSION: Both single-dose and double-dose local LR-PRP is a safe and effective treatment option for patients with PATB syndrome. We believe that once LR-PRP injection may be sufficient for the treatment efficacy in PATB.

Umbilical cord N-terminal procollagen of type l collagen (P1NP) and beta C-terminal telopeptide (ßCTX) levels in term pregnancies with vitamin D deficiency.

AIMS: The aim of the present study was to evaluate umbilical cord N-terminal procollagen of type l collagen (P1NP) and beta C-terminal telopeptide (ßCTX) levels in term pregnancies with vitamin D deficiency. MATERIALS AND METHODS: Ninety-two pregnant women between 19 and 35-years-old who delivered at term gestational age were included in the study and divided into deficient (n = 32), insufficient (n = 30), and normal (control) vitamin D levels (n = 30). RESULTS: Maternal demographic characteristics and biochemical parameters were similar among groups. The mean umbilical cord P1NP level was 221.4 (211.7-231.0, 95%CI) pg/mL in the vitamin D deficiency group, 282.5 (271.2-293.8, 95%CI) pg/mL in the vitamin D insufficiency group, and 280.9 (270.9-290.8, 95%CI) pg/mL in the control group and significantly lower in vitamin D deficiency group than others (p < .001). Umbilical cord P1NP level was similar in the vitamin D insufficiency group and control group (p = .971). The mean umbilical cord ßCTX level was 5530, 9 (5511.5-5550.3, 95%CI) pg/mL in the vitamin D deficiency group, 5516.3 (5498.4-5534.2, 95%CI) pg/mL in the vitamin D insufficiency group, and 5510 (5491.4-5528.5, 95%CI) pg/mL in the control group, which was statistically similar among the groups (p = .251). CONCLUSION: Our results indicated that vitamin D deficiency during pregnancy affects fetal bone osteoblast activity.

Apelin-13: A Promising Biomarker for Age-Related Macular Degeneration?

PURPOSE: To investigate the value of serum apelin-13 levels in patients with age-related macular degeneration (AMD). METHODS: Patients with dry-type AMD, patients with treatment-naïve neovascular-type AMD, and healthy controls were included in this study. Diagnoses were confirmed on detailed fundus examination, optical coherence tomography (OCT), and fundus fluorescein angiography (FFA). Central foveal thickness and subfoveal choroidal thickness were evaluated. Both serum apelin-13 and vascular endothelial growth factor (VEGF) levels were measured by a competitive enzyme-linked immunosorbent assay (ELISA) principle. RESULTS: A total of 84 subjects, i.e., 24 in the dry-type AMD group (group 1), 27 in the neovascular-type AMD group (group 2), and 33 in the control group (group 3) were included in the study. Mean best-corrected visual acuity (BCVA) was 76 ± 4.5, 48.4 ± 16.3, and 83.4 ± 3.09 ETDRS letters in group 1, 2, and 3, respectively. The level of serum VEGF was 44.11 ± 26.14, 56.53 ± 53.77, and 61.47 ± 41.62 pg/mL in groups 1, 2, and 3, respectively (p = 0.553, p = 0.286, and p = 0.896, respectively). The level of serum apelin-13 was 586.47 ± 167.56, 622.18 ± 324.52, and 379.31 ± 171.96 pg/mL in groups 1, 2, and 3, respectively (p = 0.847, p = 0.04, and p ≤ 0.001, respectively). There was a negative correlation between the level of serum apelin and visual acuity (VA) and choroidal thickness. CONCLUSION: Serum apelin-13 levels were higher in both dry-type and neovascular-type AMD patients than in controls. Further studies demonstrating the relationship of the level of serum apelin-13 and AMD are needed.

Hypoxia-inducible factors in arteriovenous fistula maturation: A prospective cohort study.

BACKGROUND: Neointimal hyperplasia is the main cause of arteriovenous fistula (AVF) failure. Hypoxia-inducible factors (HIFs) factors are associated with neointimal hyperplasia. Thus, we investigated the association between HIF-2 alpha (HIF-2α) and AVF maturation in end-stage kidney disease (ESKD) patients. METHODS: This prospective cohort study was conducted in 21 voluntary healthy subjects and 50 patients with ESKD who were eligible for AVF creation. Inclusion criteria were being ESKD patients without a history of AVF surgery and dialysis. Eight patients excluded from the study due to having unavailable veins six patients were excluded due to acute thrombosis after surgery. One patient lost to follow-up. A total of 35 patients were included in final analysis. The blood samples were collected a day before the AVF surgery for biochemical parameters and HIF-2α measurement. HIF-2α levels were measured by the ELISA method. RESULTS: Compared with healthy subjects, ESKD patients had a significantly higher level of HIF-2α. [1.3 (1.0-1.9) vs 2.2 (1.6-3.0)] (P = .002). Patients were divided into two groups after the evaluation of AVF maturation, as the mature group (n = 19) and the failure group (n = 16). Serum HIF-2α level was 1.7 (1.1-1.8) in the mature group; however, it was 3.1 (2.8-3.3 in failure group (P < .001). Multiple logistic regression analyses showed that HIF-2α independently predicted AVF maturation. The ROC curve analysis showed that HIF-2α > 2.65 predicted AVF maturation failure with the 87% sensitivity and 94% specificity [AUC:0.947, 95% CI (0.815-0.994), P < .001]. CONCLUSIONS: HIF-2-α levels were higher in ESKD patients than healthy subjects. HIF-2-α could be a marker of AVF maturation failure.

Evaluation of the levels of secretory leukocyte protease inhibitor in the cervical mucus of women with unexplained infertility.

AIM: Secretory leukocyte protease inhibitor (SLPI) has specific effects on the immune system. SLPI is overexpressed in inflammation triggered by immune responses, which could have significant effects on the local immune responses in cervical mucosa. This over expression may be greater in women with unexplained infertility, which would increase the immune reaction in the cervical region against sperm. The aim of our study was to assess the levels of SLPI in cervical mucus in women with unexplained infertility. METHODS: This prospective cross-sectional study was conducted using 50 Caucasian volunteers between 20 and 40 years old. The 50 participants were divided into two groups as unexplained infertility (n = 20) and control (n = 30). The control group comprised healthy fertile women with demographic characteristics similar to those of the infertility group. The enzyme-linked immunosorbent assay method was used to assess SLPI levels in the cervical mucus of all participants. RESULTS: The median SLPI level in cervical mucus was 3767 (3541-4594, 95% CI) pg/mL (25th percentile; 3139 pg/mL, 75th percentile; 5047 pg/mL) in the unexplained-infertility group and 3204 (2602-3539, 95% CI) pg/mL (the 25th percentile = 2615 pg/mL; 75th percentile = 3990 pg/mL) in the control group, which was a significant difference (P = 0.013). CONCLUSION: Our results indicated that SLPI levels in cervical mucus were remarkably higher in patients with unexplained infertility than in the control group.

Increased asymmetric dimethylarginine in vitamin B12 deficient adolescents.

OBJECTIVE: Vitamin B12 deficiency induces hyper-hyperhomocysteinemia by inhibiting intracellular methionine re-methylation. Hyper-hyperhomocysteinemia increases the risk of atherosclerosis. Asymmetric dimethylarginine is an endogenous inhibitor of nitric oxide synthase and its level elevates in cardiovascular diseases. In this study, we aimed to examine the relationship between asymmetric dimethylarginine and arterial stiffness and atherosclerosis in adolescents with vitamin B12 deficiency. METHODS: A total of 88 adolescents with age ranging between 11 and 17 years of age were enrolled for this study. Among them, 50 patients had vitamin B12 deficiency 200 pg/ml. In all cases, the levels of asymmetric dimethylarginine were measured with high performance liquid chromatography method. The carotid artery intima media thickness and left ventricular mass index were measured using echocardiography. All these measurements of the study groups were compared. RESULTS: Both plasma levels of asymmetric dimethylarginine and carotid artery intima media thickness were significantly higher in the vitamin B12 deficiency group than in the control group. Correlation analysis showed significant negative correlation of vitamin B12 with homocysteine, asymmetric dimethylarginine, and carotid artery intima media thickness (p<0.05). CONCLUSION: Our results suggest that endothelial dysfunction starts in the early stage of adolescent vitamin B12 deficiency, and vitamin B12-deficient adolescents have increased circulating asymmetric dimethylarginine, showing that endothelial dysfunction and increased carotid artery intima media thickness be related to atherosclerosis.

Does omalizumab treatment affect serum dehydroepiandrosterone sulphate levels in chronic idiopathic urticaria?

INTRODUCTION: It is known that serum dehydroepiandrosterone sulphate (DHEA-S) levels are low in patients with chronic idiopathic urticaria. AIM: In the study, the effect of the drug on the DHEA-S serum levels and its correlation with the remission and relapse times of the disease was investigated. MATERIAL AND METHODS: Fifty-seven patients with chronic idiopathic urticaria who were referred to our hospital and 20 healthy volunteers were included in the study. A subcutaneous injection of 300 mg omalizumab was administered to the patient group. Drug injections at this dose were completed (6 injections in total, one per month). Relations between serum DHEA-S levels and relapse rates, treatment response and remission duration of the patients and control group were investigated in the groups. RESULTS: Median DHEA-S value before treatment was 116.3 (21.5-448.7) µg/dl; the median DHEA-S value measured after 3 months was 98.4 (10.0-410.0) µg/dl (p = 0.003). The median DHEA-S value before treatment was 123.1 (21.5-299.6) µg/dl when the initial and 3-month DHEA-S levels of the 34 complete remission patients were compared; after 3 months the value was 100.4 (23.1-301.9) µg/dl (p = 0.021). CONCLUSIONS: This is the first study to investigate the effect of omalizumab treatment on DHEA-S levels in the treatment of chronic urticaria according to our literature review. The DHEA-S levels were found to be significantly lower after omalizumab therapy but not related to remission and relapse times.

Cervical Local Immune Response for High-Risk Human Papillomavirus Infection: Involvement With Cervical Mucus SLPI Proteins.

PURPOSE: To evaluate cervical mucus secretory leukocyte protease inhibitor (SLPI) concentrations in patients with high-risk human papillomavirus (hrHPV) 16 or 18 positive and low-grade squamous intraepithelial lesions (LGSIL) or high-grade squamous intraepithelial lesions (HGSIL). METHOD: Patients with HPV 16 or 18 positive from 30 to 45 years of age whose cervical cancer screening results reported cytologically LGSIL or HGSIL were included in the study. In the control group, we included participants in the same age with cytology negative and HPV-negative healthy women. All cytological LGSIL or HGSIL results were histopathologically confirmed with colposcopic biopsy specimens. Finally, the study consisted of a total of 3 groups each containing 25 participants as follows: (1) Pap smear and HPV-negative control group, (2) HPV 16 or HPV 18 and LGSIL-positive participants, and (3) HPV 16 or 18 and HGSIL-positive participants. Cervical mucus SLPI levels were analyzed using the enzyme-linked immunosorbent assay method. RESULTS: The mean cervical mucus SLPI levels were 32.94 ng/mL (range: 23-41.29 ng/mL) in the hrHPV + LGSIL group, 29.40 ng/mL (range: 21.03-38.95 ng/mL) in the hrHPV + HGSIL, and 18.75 ng/mL (range: 13.58-29.24 ng/mL) in the healthy control group. Cervical mucus SLPI levels were found to be significantly higher in the hrHPV + LGSIL and hrHPV + HGSIL groups compared to the control group ( P < .001). CONCLUSIONS: The data from the present study indicate that SLPI seems to be one of the important immunomodulatory proteins that provide local immune response in cervical mucosa.

Serum and Tissue HIF-2 Alpha Expression in CIN, N-Acetyl Cysteine, and Sildenafil-Treated Rat Models: An Experimental Study.

Background and Objectives: Contrast-induced nephropathy (CIN), is acute renal damage due to contrast agents. This study is conducted to evaluate serum and renal heterodimeric nuclear transcription factor (HIF)-2 alpha levels and its tissue expression in contrast-induced nephropathy, and in N-acetyl cysteine (NAC)-and Sildenafil-treated rat models. Materials/Methods: This randomized, controlled, interventional animal study was conducted on Wistar rats. Rats (n = 36) were randomly assigned to four groups: control (n = 9), CIN group (n = 9), CIN + NAC group (n = 9), and sildenafil (n = 9). The rat model was used to form iohexol-originated CIN. During the modeling, prophylactic treatment was performed at the 24th and 48th h. After 48 h of modeling, blood, urine, and tissue samples were obtained for biochemical analyses. HIF-2-α levels were measured in renal tissue, serum, and urine samples. Renal sections were also performed for histopathologic and immunohistochemical evaluations of renal injury and HIF-2-α expression. Results: In the CIN model, HIF-2α levels and other biochemical parameters were significantly increased (p < 0.01). Both sildenafil and NAC efficiently decreased renal damage due to contrast agents, as shown in histopathologic examinations (p < 0.05). Similarly, after treatment with sildenafil and NAC, HIF-2α levels were significantly decreased (p < 0.05). Conclusions: The current study shows that serum and tissue HIF-2α levels decrease in CIN. Besides, the levels and tissue expression of HIF-2α decrease with both NAC and sildenafil treatments. With further studies, HIF-2α can be investigated as a biomarker of CIN and can be used in the follow-up of patients with CIN.

Evaluation of platelet parameters and neutrophil/lymphocyte ratio during omalizumab treatment in patients with severe chronic spontaneous urticaria

Background/aim: Spontaneous wheals and/or angioedema lasting longer than six weeks are described as chronic spontaneous urticaria (CSU). Omalizumab is used for the treatment of antihistamine-resistant CSU. The neutrophil­lymphocyte ratio (NLR), platelet­ lymphocyte ratio (PLR), mean platelet volume (MPV), and platelet distribution width (PDW) are considered important indicators of inflammation and platelet activation in chronic diseases. We aimed to determine the NLR, PLR, MPV, and PDW levels in patients with CSU compared with healthy controls. We also aimed to investigate the effects of omalizumab therapy on these parameters in CSU patients. Materials and methods: This hospital-based, retrospective study included 143 patients with CSU and 132 healthy controls with a mean age of 40.0 ± 13.17 and 42.0 ± 16.34, respectively. Patients with equal or higher-than-baseline UAS scores at week 12 of omalizumab treatment were considered nonresponders, others were considered responders. We analyzed the neutrophils, lymphocytes, platelet counts, NLR, PLR, MPV, and PDW before, during, and after omalizumab treatment and compared the results with those of healthy controls. Results: CSU patients presented higher baseline MPV (P = 0.035) and lower baseline PDW values (P < 0.001) than healthy controls. There were statistically significant increases in the MPV (P < 0.001), MPV/platelet count (P = 0.005), and PDW (P = 0.003) and there was a statistically significant decrease in the NLR (P = 0.018) during omalizumab treatment. The percent increase of MPV was low in nonresponders (P = 0.009). Nonresponders had lower PDW values than responders (P = 0.040). Conclusion: The increase in the MPV and PDW may be due to platelet activation during omalizumab treatment. The decrease in the NLR may be regarded as an antiinflammatory effect of omalizumab. The effect of omalizumab on platelet and inflammatory markers may be used to discriminate the responders from nonresponders.

Increased vitamin B12 levels in children with zinc deficiency.

Additional nutritional deficiencies may accompany zinc deficiency. We determined the vitamin B12 levels in patients with zinc (Zn) deficiency. This retrospective study enrolled 256 patients age 6 months to 16 years (176 females and 80 males) in whom Zn levels in hair samples were measured concurrently with serum vitamin B12 and ferritin levels. For all patients, we retrospectively assessed the hair Zn levels, serum ferritin and vitamin B12 levels, red blood cell count, hemoglobin, hematocrit, mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration, and red cell distribution levels. Data were analyzed to determine whether there was a significant difference between any of these parameters and the presence of vitamin B12 deficiency between patients with and without Zn deficiency. In all 118 patients had Zn levels < 100 µg/g and 138 patients had levels > 100 µg/g. No significant differences were observed in ferritin levels, red blood cell parameters, or presence of iron deficiency or anemia between the two groups (p > 0.05). The median vitamin B12 level was 323 (range 238-440) pg/mL in the Zn-deficient group and 276 (range 208-382) pg/mL in those with normal Zn levels. Vitamin B12 levels were significantly higher in the Zn-deficient group (p = 0.02). A significant negative correlation was detected between vitamin B12 levels and Zn levels (r = -0.17, p = 0.004). Vitamin B12 levels are higher in patients with Zn deficiency; thus, Zn may have a negative effect on vitamin B12 levels.

The plasma gelsolin levels in atopic dermatitis: Effect of atopy and disease severity.

BACKGROUND: Gelsolin is an actin-binding protein with several cellular functions including anti-apoptosis and is reported to have an anti-inflammatory effect. Apoptosis of keratinocytes has been implicated as a key mechanism of atopic dermatitis (AD). OBJECTIVE: We aimed to determine plasma gelsolin (pGSN) levels in children with atopic dermatitis (AD). METHOD: The diagnosis of AD was made according to Hanifin and Rajka criteria. The disease severity was scored by objective SCORAD index by the same allergist. Skin prick testing (SPT), total IgE levels, and eosinophil counts were analyzed. The pGSN levels were determined using ELISA technique. RESULTS: Children aged between 0.5 and 3.0 years were included in the study. The children with AD (AD; n=84) were analyzed in two groups according to the presence (AD+/Atopy+; n=54) or absence of SPT positivity (AD+/Atopy-; n=30). The comparisons were made with a healthy control group matched for age and sex (n=81). The median (interquartile range) of pGSN levels in AD+/A+, AD+/A- and control groups were 267µg/ml (236-368), 293 (240-498) and 547 (361-695), respectively (p<0.001). The difference between the control group and AD sub-groups remained significant after Bonferroni correction (p<0.001). Correlation analysis failed to reach significance with the disease severity total IgE levels and eosinophil counts. CONCLUSION: This is the first study investigating the association of pGSN levels with AD and disease severity. pGSN levels decreased in AD. These findings suggest that gelsolin may have a role in the disease process in AD patients.

The Importance of Nucleated Red Blood Cells in Patients with Beta Thalassemia Major and Comparison of Two Automated Systems with Manual Microscopy and Flow Cytometry.

BACKGROUND: Nucleated red blood cells (NRBC) can be used as a marker of erythropoietic stress and help optimize transfusion therapy in patients with beta thalassemia major. The aim of this study was to determine this correlation; and compare two automated systems (Sysmex XE-2100, and Advia 2120i) with manual microscopy and flow cytometry (FCM). METHODS: Absolute NRBC counts and percentages (NRBC%) from 51 patients were analyzed with both automated and reference methods. The results were compared with levels of pre-transfusion hemoglobin and ferritin levels. RESULTS: The mean age of the included patients (31 female, 20 male) were 12.9 ± 7.5 years. Mean levels of hemoglobin and ferritin were 9.5 ± 1.2 g/dL and 1896 ± 1194 ng/mL respectively. The NRBC% of two instruments did not significantly differ and correlated well (p < 0.0001, r2 = 0.984). NRBC% obtained with the XE-2100 and Advia 2120i versus manual microscopy also yielded high correlations (r2 = 0.951 and r2 0.981, respectively); however, absolute NRBC counts versus FCM yielded lower coefficients (r2 = 0.723 and r2 = 0.694, respectively). High serum ferritin levels were correlated with both NRBC% and counts with both instruments (p < 0.001) and hemoglobin levels lower than 9.0 g/dL had a negative correlation with NRBC% and NRBC counts with the Advia 2120i (p < 0.05, r = -0.495) but not with the XE-2100. CONCLUSIONS: Monitoring of NRBC% with both instruments can help optimize transfusion therapy for patients with beta thalassemia major. Following the NRBC% is more efficient than absolute counts.

Cutoff values for bacteria and leukocytes for urine sediment analyzer FUS200 in culture-positive urinary-tract infections.

OBJECTIVES: The microscopic analysis of urine is essential for the diagnosis of patients with urinary tract infections. Quantitative urine culture is the 'gold standard' method for definitive diagnosis of urinary-tract infections, but it is labor-intensive, time consuming, and does not provide the same-day results. The aim of this study was to evaluate the analytical and diagnostic performance of the FUS200 (Changchun Dirui Industry, China), a new urine sedimentation analyzer in comparison to urine culture as the reference method. METHODS: We evaluated 1000 urine samples, submitted for culture and urine analysis with a preliminary diagnosis of urinary-tract infection. Cut-off values for the FUS200 were determined by comparing the results with urine cultures. The cut-off values by the receiver operating characteristic (ROC) curve technique, sensitivity, and specificity were calculated for bacteria and white blood cells (WBCs). RESULTS: Among the 1000 urine specimens submitted for culture, 637 cultures (63.7%) were negative, and 363 were (36.3%) positive. The best cut-off values obtained from ROC analysis were 16/µL for bacteriuria (sensitivity: 82.3%, specificity: 58%), and 34/µL for WBCs (sensitivity: 72.3%, specificity: 65.2%). The area under the curve (AUC) for the bacteria and WBCs count were 0.71 (95% CI: 0.67-0.74) and, 0.72 (95% CI: 0.69-0.76) respectively. CONCLUSIONS: The most important requirement of a rapid diagnostic screening test is sensitivity, and, in this perspective, an unsatisfactory sensitivity by using bacteria recognition and quantification performed by the FUS200 analyzer has been observed. After further technical improvements in particle recognition and laboratory personnel training, the FUS200 might show better results.

Soluble Receptor for Advanced Glycation End Products (sRAGE) Level and Its Prognostic Significance in Children with Acute Lymphoblastic Leukemia.

Acute lymphoblastic leukemias are the most common malignancies in childhood. Although its etiology is still unclear, it is thought that disorders in oxidative stress metabolism may contribute to leukemogenesis. Advanced glycation end products (AGEs) are formed as a result of the non-enzymatic binding of sugars to biomolecules. Oxidation reactions are triggered through AGE-Receptor (RAGE) interaction, resulting in the formation of reactive oxygen species. These can play crucial roles in cancer pathogenesis and leukemogenesis. It is thought that sRAGE (soluble RAGE) is the end product of glycation and circulates freely in the circulation by binding to RAGE ligands. We investigate novel leukemia biomarkers and focus on soluble RAGE (sRAGE) for acute lymphoblastic leukemia (ALL) diagnosis and prognosis. Thirty children (1-17 years) diagnosed with ALL were included in the study. Patients were divided into standard, medium, and high risk groups according to the Berlin-Frankfurt-Münster (BFM) treatment protocol. Patients were evaluated twice; at the time of diagnosis and at the sixth month of remission. sRAGE and blood parameters were compared with healthy controls (n = 30, 1-17 years). The sRAGE levels in ALL patients at diagnosis (138.7 ± 177.3 pg/mL) were found to be significantly higher than they were during the sixth month of remission (17.6 ± 21.1 pg/mL) and in healthy controls (22.2 ± 23.7 pg/mL). The cut-off value of the sRAGE level for the diagnosis of ALL was found to be 45 pg/mL in ROC analysis (sensitivity: 73.3%, specificity: 86.7%, AUC: 0.681). At the same time, the sRAGE level was found to be significantly higher in T-ALL patients (490.9 ± 236.9 pg/mL) than in B-ALL patients (84.5 ± 82.7 pg/mL). No significant difference was found in terms of the sRAGE level between standard (45.8± 33.1 pg/mL), medium (212 ± 222.1 pg/mL), and high (143.9 ± 111.5 pg/mL) risk group ALL patients classified according to the BFM protocol. Despite the fact that this was a small, single-center study, our findings highlight the potential use of sRAGE as a biomarker for diagnosing ALL and assessing response to treatment.

Urolithiasis in children; The importance of stone localization in treatment and follow-up.

To evaluate the demographic, etiologic, treatment, and follow-up differences in stones according to their location within the kidney. This retrospective study comprised 337 patients with urolithiasis between 2015 and 2019. Patients were classified into 2 groups according to stone location as lower pole stones (LPS) and upper-middle pole stones (UMPS). The patient's data were recorded at 3-month intervals for one year. One hundred and eighty-three (54.3%) female and 154 (45.7%) men were included in the study. One hundred and twenty-nine (38.3%) of the stones were in the LPS and 208 (61.7%) in the UMPS. UMPS was more common in patients aged > 12 months (p < 0.01). At least one metabolic risk factor was present in 93 (72.1%) patients with LPS and 164 (78.4%) with UMPS. The most common urinary metabolic risk factors were hyperoxaluria (31.8%) in patients with LPS and hypocitraturia (34.1%) in patients with UMPS. ROC analysis results showed that cut-off values of 5.5 mm for LPS and 6.1 mm for UMPS did not provide improve with medical treatment. At the 6- and 12-month follow-ups, the improvement rates were higher in the UMPS group than in the LPS group (p < 0.05). During the follow-up, recurrence was detected in 43 patients: 29% of patients with LPS and 5.8% of patients with UMPS (p < 0.01). Patients with small stones can be followed up. Surgical treatment may be considered for small stones in the LPS. In addition, the risk of recurrence is higher in patients with LPS, and close follow-up is required.

The utility of serum neutrophil gelatinase-associated lipocalin level on predicting autosomal dominant polycystic kidney disease progression.

INTRODUCTION: We focused on neutrophil gelatinase-associated lipocalin (NGAL) and autosomal dominant polycystic kidney disease (ADPKD) progression. METHODS: ADPKD patients with an estimated glomerular filtration rate (eGFR) ≥ 30 mL/min/1.73 m2 were included. Serum NGAL level and NGAL to eGFR ratio (NGR), height-adjusted total kidney volume (hTKV) were assessed initially. Patients were followed-up for 5 years. RESULTS: Sixty one patients were enrolled and initial eGFR was 73.6 (48.9-101.5) ml/min/1.73m2. EGFR declined by 3.7 mL/min/1.73m2 per year. Thirty four patients (55.7%) exhibited rapid progression. Rapid progression group had lower serum NGAL levels (p < 0.001) and higher hTKV (p < 0.001). Lower serum NGAL level was a risk factor for rapid progression (p < 0.001). NGR was not associated with rapid progression. Serum NGAL level was predictive in for rapid progression ROC analysis (cut-off <10.62 ng/mL). CONCLUSION: Relatively lower serum NGAL levels can predict worse outcomes in ADPKD and can provide risk stratification in patients with ADPKD.