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Multiple renal cysts in adult patients could have asymptomatic, benign and a nonprogressive course. However, these cysts could be renal features of a very rare hereditary, progressive syndrome defined as cranioectodermal dysplasia (CED or Sensenbrenner syndrome). Affected patients show dysmorphic features such as craniosynostosis, nail dystrophy, cutaneous dyshydrosis, dry or scaly palmar skin, trichodysplasia, deafness, pectus excavatum, telecanthus, hypertelorism, low set ears, everted lower lip, anteverted nares, short neck and height, joint laxity, inguinal hernia, widely spaced teeth, microdontia, hypodontia in addition to nephronophthisis. We report a 22-year-old male hypertensive patient with multiple renal cysts and dental malformations listed as malocclusion, screwdriver shaped crowns, widely spaced front teeth, microdontia and hyperdontia. Molecular analysis reported missense p.(Ala875Thr) and p.(Lys969Asn) variants in the WDR35 gene. The 1-year follow-up of this case provided the knowledge that angiotensin II receptor blocker drug (olmesartan) reduced the microalbuminuria to normal levels and preserved the renal functions. We suggest interdisciplinary studies, especially intraoral and genetic evaluations for patients with cystic renal diseases. For the first time we report that hyperdontia could be found as a dental feature of CED.

Background: The prevalence of malignancies is higher among kidney transplant recipients (KTR) than general population. Although the incidence of renal cell cancer (RCC) after KTR was reported as 0.6 % with a high mortality rate of 13.9 %, none of the guidelines except the European Best Practice Guideline (EBPG) recommends RCC screening based on cost-effectiveness and lack of solid evidence. The EBPG recommends RCC screening in native kidneys (not the allograft) by ultrasonography (USG) every 1-3 years., Case Description: A 55-year-old male patient who had a kidney transplant from a living donor 14 years before, presented with sudden onset uncontrolled hypertension (180/110 mmHg) and rapid deterioration in graft functions (increase in serum creatinine to two-times baseline). Evaluations revealed RCC in the allograft. Abdominal pain, hematuria, fever, weight loss, flu-like syndrome, recurrent urinary tract infections, weakness, hypertension, and allograft dysfunction are reported as the main complaints and signs of RCC in KTR patients. Our patient's findings were hypertension and allograft dysfunction., Conclusion: It is essential to follow EBPG for KTR and not to forget the annual USG for screening of RCC both in native kidneys and allograft. HIPPOKRATIA 2023, 27 (2):69-71., Competing Interests: All co-authors have agreed with the contents of our manuscript and declare no conflict of interest., (Copyright 2023, Hippokratio General Hospital of Thessaloniki.)

Background: Even though complement 1q nephropathy (C1qN) was first introduced in 1985, this entity is still unknown and recognized by clinicians due to its rare prevalence (0.2 - 2.5 %) and insufficient emphasis. Description of the case: A 50-year-old woman was incidentally found to have non-nephrotic proteinuria with a normal glomerular filtration rate. Renal biopsy revealed C1qN with severe fibrosis. The presence of consanguinity and kidney diseases in family members of the patient led to genetic research, and homogenous mutation of c.991G>T (p.G331C) in the WD-repeat domain 19 (WDR19) gene was found. The same homozygous and heterozygous mutations in the WDR gene were found in the relatives of our patient with kidney diseases. One year of follow-up with methylprednisolone and mycophenolate mofetil treatment resulted in partial remission of the kidney disease. Conclusion: Renal biopsy for patients with non-nephrotic proteinuria without delay is suggested as it might be a surrogate marker of severe injury. Genetic mutations in the WDR19 gene should be searched for C1qN pathogenesis. This is the first adult case report on C1qN from Turkey. [ABSTRACT FROM AUTHOR]

Background: The present study aimed to investigate and compare mental health, health-related quality of life, and sleep levels of patients with various stages of chronic kidney disease (CKD) and undergoing different renal replacement therapies and analyze the factors affecting these parameters. Methods: Overall, 140 patients with a mean age of 43 ± 14 years were recruited into this study. Study groups [controls and patients with CKD undergoing predialysis, hemodialysis (HD), peritoneal dialysis, kidney transplantation (KT)] were evaluated using Short Form Health Survey-36 (SF-36), Kidney Disease Quality of Life-36 (KDQoL-36), Pittsburgh Sleep Quality Index (PSQI), and General Health Questionnaire-12 (GHQ-12). Results: The KT group had the highest scores in physical and mental components of the subscales of SF-36 and KDQoL-36 but the lowest scores in PSQI and GHQ-12, indicating the best results in terms of mental health and quality of life, and sleep. Serum albumin and hemoglobin levels were positively correlated with several subscales of quality of life. Significant negative correlations were observed among PSQI, GHQ-12, and subscale scores of SF-36 and KDQoL-36. The HD group showed significantly lower scores in the subscales of symptoms and burden of kidney disease of KDQoL-36. Conclusion: KDQoL was worse in the HD group and better in the KT group than in other groups. Serum albumin and hemoglobin levels, and Kt/V (dialyzer clearance of urea multiplied by dialysis time and normalized for urea distribution volume) values of patients with CKD exerted a linear and significant effect on the quality of life, which showed a significant positive correlation with the quality of sleep and mental health. In contrast, serum calcium x phosphorus levels showed an inverse correlation with the subscale scores of KDQoL. HIPPOKRATIA 2020, 24(2): 51-58. [ABSTRACT FROM AUTHOR]

Competing Interests: Authors declare no conflict of interest.

Background: Cutaneous diseases are observed with increasing duration and severity of renal disease in patients with chronic kidney disease (CKD). This study aimed to elucidate dermatological manifestations at different stages of CKD and determine their relationship with interleukin 31 (IL-31), a T-cell cytokine that induces severe pruritus, and uridine diphosphate (UDP)-glucose ceramide glucosyltransferase (UGCG), an enzyme that metabolizes ceramide, which plays an important role in moisturizing epidermis., Methods: In this retrospective cohort study 145 patients with a mean age of 46 ± 17 years were categorized into hemodialysis (group 1), peritoneal dialysis (group 2),  kidney transplant (group 3), CKD (group 4), and healthy control (group 5) groups. Serum IL-31 and  UGCG levels were measured using enzyme-linked immunosorbent assay, and clinical dermatologists evaluated dermatological manifestations., Results: In the overall cohort, pruritus was significantly and inversely correlated with glomerular filtration rate and serum hemoglobin and albumin levels (p <0.005). Additionally, pruritus was significantly more frequent in group 2 than in group 5; and significantly less frequent in group 3 than in groups 1, 2, and 4 (p =0.01). In group 4, the patients with longitudinal nail ridges had significantly higher serum IL-31 levels than those without longitudinal nail ridges in their nails (p =0.02). Furthermore, in group 2, the patients with pruritus had significantly lower UGCG levels than those without pruritus (p =0.045)., Conclusion: IL-31 might play a role in the development of longitudinal nail ridges, whereas UGCG might provide protection from pruritus and xerosis in patients with CKD.  HIPPOKRATIA 2019, 23(2): 75-80., Competing Interests: All authors declare that there are no conflicts of interest related to the study., (Copyright 2019, Hippokratio General Hospital of Thessaloniki.)

Competing Interests: None declared by authors.

Background: A chronic HBsAg carrier state, a major cause of viral spread in a community, is one of the consequences of hepatitis B virus (HBV) infection. Although successful immunization programs have been initiated to eliminate the virus, there is still a large number of people with HBV infection worldwide. This study was designed to investigate the effect of indomethacin treatment on HBV markers in humans, in comparison with a control group. Methods: In total, 65 chronic `healthy' HBV carriers were involved in the study. Patients were divided randomly into two groups. Group I (n = 42) received oral indomethacin 75 mg daily for 6 months. Group II (n = 23) acted as control. Patients in both groups were followed up for 6 months, during which laboratory tests, including viral parameters, were performed periodically. Liver biopsy was done in 17 patients (11/42 of the indomethacin group and 6/23 of the control group). Results: All liver biopsies showed grade 0-2 and stage 0-1 HBV in both groups (P > 0.05). HBsAg positivity did not change in any patient in either group. Five patients who had positive HBeAg in group I became negative 4 months later, while patients in group II continued to be positive at 6 months (P < 0.001). Similarly, all patients receiving indomethacin exhibited a total anti-HBeAg immunoglobulin response at 6 months, while the control group remained the same during this period (P < 0.05). HBV DNA, as detected by polymerase chain reaction in 20/22 (91%), was negative in group I at the end of 6 months. No change was observed in group II (P = 0.007). Conclusions: Although no biochemical analyses were performed on prostaglandins in the present study, the results suggest that the prostaglandin pathway may be involved in the pathogenesis of the immune response against HBV, and that the suppression of viral replication is achieved as indicated by the disappearance of HBeAg and HBV DNA in healthy chronic HBV carriers. [ABSTRACT FROM AUTHOR]

Acute kidney injury (AKI) is a major cause of mortality and morbidity in hospitalized patients. Incidence and mortality rates vary from country to country, and according to different in-hospital monitoring units and definitions of AKI. The aim of this study was to determine factors affecting frequency of AKI and mortality in our hospital. We retrospectively evaluated data for 1550 patients diagnosed with AKI and 788 patients meeting the Kidney Disease: Improving Global Outcomes (KDIGO) guideline AKI criteria out of a total of 174 852 patients hospitalized in our institution between January 1, 2007 and December 31, 2012. Staging was performed based on KDIGO Clinical Practice for Acute Kidney Injury and RIFLE (Risk, Injury, Failure, Loss of kidney function and End-stage renal failure). Demographic and biochemical data were recorded and correlations with mortality were assessed. The frequency of AKI in our hospital was 0.9%, with an in-hospital mortality rate of 34.6%. At multivariate analysis, diastolic blood pressure (OR 0.89, 95% CI 0.87-0.92; P < 0.001), monitoring in the intensive care unit (OR 0.18, 95% CI 0.09-0.38; P < 0.001), urine output (OR 4.00, 95% CI 2.03-7.89; P < 0.001), duration of oliguria (OR 1.51, 95% CI 1.34-1.69; P < 0.001), length of hospitalization (OR 0.83, 95% CI 0.79-0.88; P < 0.001), dialysis requirement (OR 2.30, 95% CI 1.12-4.71; P < 0.05), APACHE II score (OR 1.16, 95% CI 1.09-1.24; P < 0.001), and albumin level (OR 0.32, 95% CI 0.21-0.50; P < 0.001) were identified as independent determinants affecting mortality. Frequency of AKI and associated mortality rates in our regional reference hospital were compatible with those in the literature. This study shows that KDIGO criteria are more sensitive in determining AKI. Mortality was not correlated with staging based on RIFLE or KDIGO. Nonetheless, our identification of urine output as one of the independent determinants of mortality suggests that this parameter should be used in assessing the correlation between staging and mortality., (Copyright © 2015 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

In addition to being the main cause of glomerulonephritis in children, poststreptococcal glomerulonephritis (PSGN) has recently been shown in older patients, especially those with malignancy or diabetes mellitus. The pathogenesis of PSGN has been ascribed to activation of complement 3 (C3) of the alternative complement cascade which, along with immunoglobulin (Ig) G and IgM deposits, is observed in renal tissue. Our aim here is to discuss the probable causes of PSGN developing with isolated IgM deposition in a 52-year-old patient with essential thrombocytosis followed-up over the previous 3.5 years. These characteristics make our case the first to be reported in the literature.

Background: The combination antimicrobial therapy consisting of amikacin, polymyxin-B, and sulbactam demonstrated in vitro synergy against multi-drug resistant Acinetobacter baumannii. Objectives: The objectives were to predict drug disposition and extrapolate their efficacy in the blood, lung, heart, muscle and skin tissues using a physiologicallybased pharmacokinetic (PBPK) modeling approach and to evaluate achievement of target pharmacodynamic (PD) indices against A. baumannii. Methods: A PBPK model was initially developed for amikacin, polymyxin-B, and sulbactam in adult subjects, and then scaled to pediatrics, accounting for both renal and non-renal clearances. The simulated plasma and tissue drug exposures were compared to the observed data from humans and rats. Efficacy was inferred using joint probability of target attainment of target PD indices. Results: The simulated plasma drug exposures in adults and pediatrics were within the 0.5 to 2 boundary of the mean fold error for the ratio between simulated and observed means. Simulated drug exposures in blood, skin, lung, and heart were consistent with reported penetration ratio between tissue and plasma drug exposure. In a virtual pediatric population from 2 to <18 years of age using pediatric dosing regimens, the interpretive breakpoints were achieved in 85-90% of the population. Conclusion: The utility of PBPK to predict and simulate the amount of antibacterial drug exposure in tissue is a practical approach to overcome the difficulty of obtaining tissue drug concentrations in pediatric population. As combination therapy, amikacin/polymyxin-B/sulbactam drug concentrations in the tissues exhibited sufficient penetration to combat extremely drug resistant A. baumannii clinical isolates. [ABSTRACT FROM AUTHOR]

One of the major contaminants in aquatic ecosystems is bisphenol A (BPA), a synthetic industrial chemical widely used in the production of epoxy resins and plastics. BPA has negative effects on fish health. Proanthocyanidin, a natural immune stimulant extracted from grape seeds (GR), possesses various biological and antioxidant properties. This study aimed to mitigate the harmful effects of BPA by supplementing the fish diet with GR. Healthy Nile tilapia (120 fish, 14.95±0.10g) were divided into four groups: Group 1 served as the control; group 2 was fed 400mg GR per kg of feed; group 3 was exposed to 3mg of BPA per liter of water without GR supplementation, and group 4 was exposed to 3mg of BPA per liter of water and fed a GR-supplemented diet (400mg GR per kg). After 28 days, blood and tissue samples were collected to assess serum biochemical parameters, cytokines, catalase (CAT) activity, malondialdehyde (MDA) levels, and the histological features of the liver, spleen, and kidney. Co-treatment with GR improved serum protein levels as well as urea and creatinine levels that were previously altered by BPA exposure. BPA exposure elevated serum interleukin (IL)-1β and hepatic MDA levels, which were reduced by GR administration. Additionally, CAT activity decreased in the BPA group but increased in the GR+BPA group. BPA also significantly upregulated the mRNA transcripts of IL-6 and tumor necrosis factor (TNF-α), which may trigger inflammation, while GR significantly downregulated these genes in the BPA-treated group. Histological analysis showed that GR alleviated tissue damage caused by BPA. In conclusion, GR supplementation improved fish biochemical and histological parameters and mitigated the toxic effects of BPA. [ABSTRACT FROM AUTHOR]

Network monitoring systems can struggle to detect the full sequence of actions in a multi-step cyber attack, frequently resulting in multiple alerts (some of which are false positive (FP)) and missed actions. The challenge of easing the job of security analysts by triggering a single and accurate alert per attack requires developing and evaluating advanced event correlation techniques and models that have the potential to devise relationships between the different observed events/alerts. This work introduces a flexible architecture designed for hierarchical and iterative correlation of alerts and events. Its key feature is the sequential correlation of operations targeting specific attack episodes or aspects. This architecture utilizes IDS alerts or similar cybersecurity sensors, storing events and alerts in a non-relational database. Modules designed for knowledge creation then query these stored items to generate meta-alerts, also stored in the database. This approach facilitates creating a more refined knowledge that can be built on top of existing one by creating specialized modules. For illustrative purposes, we make a case study where we use this architectural approach to explore the feasibility of monitoring the progress of attacks of increased complexity by increasing the levels of the hyperalerts defined, including a case of a multi-step attack that adheres to the ATT&CK model. Although the mapping between the observations and the model components (i.e., techniques and tactics) is challenging, we could fully monitor the progress of two attacks and up to 5 out of 6 steps of the most complex attack by building up to three specialized modules. Despite some limitations due to the sensors and attack scenarios tested, the results indicate the architecture's potential for enhancing the detection of complex cyber attacks, offering a promising direction for future cybersecurity research. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the effects of adipocytokines including adiponectin, leptin, resistin, neuropeptide Y and ghrelin in chronic kidney disease (CKD) patients on appearance of protein-energy wasting (PEW). One hundred fifty patients with mean age of 45.4 ± 15.9 years, without active infections or chronic inflammatory conditions were recruited into the study. Study groups were control group (consisting of 30 healthy volunteers with normal kidney functions), hemodialysis group, predialysis group, peritoneal dialysis group and kidney transplant group. Fasting morning serum leptin, ghrelin, acylated ghrelin, neuropeptide Y, adiponectin, resistin levels of all of the groups were measured. Anthropometric and nutritional assessments of all patients were obtained. Diagnosis of PEW was made according to definition recommended by the International Society of Renal Nutrition and Metabolism. Presence of PEW in hemodialysis (23.3%) and peritoneal dialysis (26.7%) groups were significantly higher than those of predialysis (3.3%), and transplantation (0%) groups. Adiponectin and resistin levels in predialysis, peritoneal dialysis and hemodialysis patients were significantly higher than control group (p: 0.0001). This study had given significant positive correlations between presence of PEW and serum resistin (r: 0.267, p: 0.001), and serum adiponectin levels (r: 0.349, p: 0.0001). There were no relationship between presence of PEW and ghrelin, acylated-ghrelin, neuropeptide Y, and leptin levels of the groups. CKD patients except transplant patients had higher adiponectin and resistin levels than control group. PEW was found to be linearly correlated with resistin and adiponectin. High serum resistin and adiponectin levels might have a role in development of PEW among dialysis patients., (© 2013 International Society for Hemodialysis.)

Objective: The aim of this study is to evaluate depression and anxiety scores among chronic kidney disease (CKD) patients and to search the changes of serum concentrations of adipokines with respect to emotional disturbances of CKD patients., Patients and Methods: 150 patients recruited into this cross-sectional analytic study. Study groups were control, hemodialysis, predialysis, peritoneal dialysis and kidney transplantation groups. Fasting morning serum leptin, ghrelin, acylated ghrelin, neuropeptide Y, adiponectin, resistin levels of all of the groups were measured using ELISA (Sandwich) method. A screening interview based on the Structured Clinical Interview for DSM-IV and self-report scales (The Beck Depression [BDI] and The Beck Anxiety Inventory [BAI] and Brief Symptom Inventory [BSI] which is self report scales) were administered and conducted by a trained interviewer., Results: BDI scores were significantly higher in hemodialysis and predialysis groups compared to control group (p = 0.009). Somatization sub scores of BSI were significantly higher in hemodialysis and peritoneal dialysis groups compared to control group (p = 0.041). Also positive symptom distress index scores of BSI were significantly higher in hemodialysis and transplantation groups compared to control group (p = 0.047). BDI score were significantly negatively correlated with duration of education (r = -0.165, p = 0.045), positively correlated with presence of protein energy wasting (r = 0.198, p = 0.016), and resistin levels (r = 0.233, p = 0.004)., Conclusion: CKD patients had higher BDI, BSI-somatization, BSI-positive symptom distress index scores compared to control group. High serum resistin levels, presence of protein energy wasting might have a role in development of depressive disorders of patients with chronic kidney disease.

Hemodialysis (HD) adequacy requires monitoring in line with standards and at appropriate intervals. However, the use of inappropriate or incorrectly applied techniques in the determination of HD adequacy can lead to highly unfortunate results. This study was intended to identify the path to a solution by determining how far HD adequacy in HD centers in our region reflects reality. Three hundred and thirty HD patients from eight centers were included. On the first visit, predialysis and postdialysis blood collection with the centers' own methods being used were observed and errors were recorded. Kt/V1 was calculated from pre- and postdialysis blood specimens taken by the units themselves. On the second visit, one session later, pre- and postdialysis blood samples were collected in line with guidelines by ourselves, the authors, and Kt/V2 was calculated from these samples. The eight units' total Kt/V2 value was significantly lower compared with Kt/V1 (<0.0001). The level of patients in all centers with Kt/V1 <1.2 was 13.5%, and that of patients with Kt/V2 <1.2 was 22.1%. No center, apart from one unit, managed to complete the collection of blood specimens as recommended by the guidelines. With one exception, blood collection for HD adequacy was not performed using proper technique in any center. This simple but easily overlooked situation, HD being regarded as adequate though in fact it is not, may lead to patients not being treated effectively and accurately and to a rise in mortality and morbidity in the long term., (© 2012, Copyright the Authors. Artificial Organs © 2012, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)