Comparative analysis of scoring systems for predicting mortality in Fournier gangrene: single center, 15 years experience.

To compare 5 published scoring systems (Fournier gangrene severity index [FGSI], Uludag FGSI [UFGSI], age-adjusted Charlson Comorbidity Index [ACCI] and the Quick Sequential Organ Failure Assessment (qSOFA) and the Laboratory Risk Indicator for Necrotizing fasciitis (LRINEC) scores and to evaluate risk factors for outcome prediction in patients with Fournier gangrene (FG). Between 2010 and 2024, 311 patients were included in the study. The data of 276 survivors and 55 non-survivors were compared. Univariate and multivariate analyses were performed to determine which of the 5 scoring systems calculated according to demographic, laboratory and clinical data predicted mortality better. In addition, a cut-off value for these scoring systems was determined by ROC analysis. The mortality rate was 17.6% in FG patients. In all 5 scoring systems, univariate analyses showed higher values in non-survivor patients, while UFGSI and FGSI scoring systems were superior to other scoring systems in multivariate analyses (p < 0.001). ROC analysis using mortality-based sensitivity and specificity revealed that the optimal cut-off values for FGSI, UFGSI, ACCI, SOFA and LRINEC should be equal to or higher than 10, 12, 4, 2 and 8, respectively. UFGSI and FGSI scores had the highest AUC values among all scores. This was followed by ACCI, qSOFAand LRINEC. For a UFGSI score ≥ 12, the sensitivity was 84%, specificity 97%, PPV 85% and NPV 97%. Among the scoring systems used to predict mortality, UFGSI was the most accurate, while LRINEC was the least accurate.

In the Treatment of Lower Pole Kidney Stones Between 1-2 cm in Children, Which is the Best Approach? Retrograde Intrarenal Surgery or Mini Percutaneous Nephrolithotomy.

Objectives: Kidney stones are treated with many methods, but there is no consensus on which method should be preferred for 1-2 cm lower renal stones. In our study, we aimed to investigate the results of mini (Percutaneous Nephrolithotomy) PCNL and (Retrograde Intrarenal Surgery) RIRS in lower renal pole 1-2 cm stones. Methods: Twenty-four mini PCNL and 55 RIRS patients were included in the study. Demographic data of the patients and information about stones on Non-Computed Tomography (NCCT) were recorded. Stone-free status (SFR), need for additional treatment and complications of both methods were compared. Results: Operative time was 55.2±20.8 min in mini PCNL and 70.7±36.5 min in RIRS, which was statistically significantly lower (p=0.002). Length of hospital stay was 2.4±1.5 days in the mini PCNL and 1.3±0.7 days in the RIRS, which was statistically significantly longer (p=0.011). In the postoperative 1st month and 3rd month stone-free rates (SFR) were higher in the mini PCNL group. While the 1st month SFR was 91.6% and 54.5%, the 3rd month stone-free rates were 95.8% vs. 69.1%, respectively (p<0.001). The need for re-treatment was statistically lower in the mini PCNL group (p<0.001). In terms of complications, the incidence of complications was 16.6% (pain in 2 patients, fever in 1 patient, need for blood transfusion in 1 patient) in the mini PCNL group and 21.8% (pain in 2 patients, fever in 8 patients, sepsis in 2 patients) in the RIRS group. There was a significant difference between the two groups (p=0.008). Conclusion: Mini PCNL has a higher SFR, less need for re-treatment and fewer complications.

Allergic reactions to enzyme replacement therapy in children with lysosomal storage diseases and their management.

OBJECTIVES: Human recombinant enzyme replacement therapy, given to compensate for genetic enzyme deficiency in lysosomal storage diseases, delays the progression of the disease and improves the quality of life. However, enzyme replacement therapy may cause hypersensitivity reactions. Within the scope of this research, we aimed to elucidate the frequency and clinical features of hypersensitivity reactions against enzyme replacement therapy in children with lysosomal storage diseases and clarify the management of these reactions. METHODS: Medical records of pediatric patients with lysosomal storage disease and receiving enzyme replacement therapy were retrospectively reviewed, and patients who experienced allergic reactions were included in the study. The demographic characteristics of the patients, their diagnosis, the responsible enzyme, the time at which the reaction started and at what dose, the signs and symptoms associated with the reaction, diagnostic tests, the management of the reaction, and the protocol applied for the maintenance of enzyme replacement therapy after the reaction were recorded. RESULTS: Hypersensitivity reactions developed in 18 of 71 patients (25.3 %) who received enzyme replacement therapy. The most common cutaneous findings were observed. Anaphylaxis developed in 6 of 18 patients. Patients who experienced recurrent hypersensitivity reactions with premedication or a slower infusion rate, those with positive skin test results, and patients who developed anaphylaxis were given enzyme replacement therapy with desensitization. CONCLUSIONS: HSR may develop during enzyme replacement therapy, which are vital in lysosomal storage diseases, and discontinuation of enzyme replacement therapy is a significant loss for patients with metabolic disorders. These reactions can be treated with premedication and long-term infusions, but some patients may require desensitization protocols for continued treatment.

Analyzing learning curve for supine percutaneous nephrolithotomy in urology resident training programme: comparative analysis.

INTRODUCTION: This article attempts to provide a comprehensive review of the learning objectives and importance of the supine percutaneous nephrolithotomy (PCNL) technique. MATERIAL METHOD: We retrospectively reviewed the cases of Supine PCNL between January 2018 and January 2024. We divided the groups into 3: residents between 2 and 3 years (Group 1), residents between 4 and 5 years (Group 2), and endourologist (Group 3). The 2-3-year resident started to perform PCNL for the first time, while the 4-5-year resident started to perform Supine PCNL for the first time while previously performing prone PCNL. RESULTS: Access, fluoroscopy, and operation time were higher in Group 1, shorter in Group 2, and shortest in Group 3 (p < 0.001). Postoperative length of stay and the need for additional treatment were found to be shorter (p < 0.001), and the stone-free rate (SFR) increased (p < 0.001) from Group 1 to Group 3. The highest complication rates were observed in Group 1 (p = 0.002). SFR rate increased as the number of cases increased in Group 1 patients. Success was stable after 46-60 cases in terms of SFR. In Group 2, the SFR rate was stable after 31-45. CASES: The most complications were observed in Group 1 and the least in Group 3. CONCLUSION: In 2-3-year residents, access time and fluoroscopy time decrease with experience. In 4-5-year residents, due to their expertise in prone PCNL, the operation time and fluoroscopy time decrease with the number of cases performed. SFR is higher after 46-60 cases for 2-3-year residents and 31-45 cases for 4-5-year residents.

Patients' perspectives on the use of artificial intelligence and robots in healthcare.

OBJECTIVE: We aimed to evaluate the opinions of individuals aged 18 and above in our country regarding the use of artificial intelligence (AI) and robots in the field of healthcare. BACKGROUND: The growing population and patient load, coupled with increasing data, can expedite the diagnosis and treatment process for patients through faster, easier, and more accurate interpretation of information. METHODS: The study encompasses voluntary participants aged 18 and above, who have either undergone surgery in a hospital or have accompanied a family member during a surgical procedure and possess internet access as well as the capability to participate in online surveys. RESULTS: A total of 725 individuals participated in our study 61% (n=442) of respondents expressed trust in the operation of AI and robots in the hospital setting. 64.1% (n=465) of participants expressed trust in AI's contribution to disease diagnosis and laboratory tests. The confidence in AI's use in radiological examinations and its contribution reached 71.6% (n=519). CONCLUSION: This study demonstrates that the use of AI and robots in healthcare services is accepted by our society and would be appropriate in our society (Tab. 5, Fig. 1, Ref. 24).

Differences in bladder neck angles between female patients with overactive bladders and healthy peers.

AIM: The aim of this study was to compare the differences between angles of bladder neck in girls with overactive bladder and those in healthy ones using transabdominal ultrasonography. MATERIALS AND METHODS: This study consists of 28 girls complicated with overactive bladder (Group I) and 40 healthy girls (Group II). The anteroposterior vesical wall angle (APVA), urethroposterior vesical wall angle (UPVA), urethroanterior vesical wall angle (UAVA), thickness of bladder mucosa, distance of urethral orifices, and distance between ureter and urethra orifice were measured in supine position using transabdominal ultrasonography. The results were compared between the two groups. RESULTS: UAVA in Group I was higher than Group II (135.2 ± 12.2 mm vs. 117.4 ± 14.0 mm; p = 0.009). UPVA was smaller in Group I than Group II (114.6 ± 19.5 mm vs. 135.3 ± 16.5 mm; p = 0.014). The distance between the ureteral orifices was 31.8 ± 8.5 mm in Group I and 17.0 ± 4.1 mm in Group II (p < 0.001). There was no statistically significant difference between groups in terms of APVA, bladder mucosa thickness, and distance between ureter and urethra orifice (p > 0.05). CONCLUSION: Bladder neck dynamics may play an important role in overactive bladder pathophysiology due to differences in UPVA, UAV, and location of ureteral orifices in this patient population.

Impact of COVID-19 pandemic on the circadian rhythm of cesarean section deliveries.

Circadian rhythms synchronize all biological functions, enabling humans to foresee and respond better to periodic environmental changes. The coronavirus disease (COVID-19) lockdown regulations significantly changed the lighting conditions in pregnant women, leading to chronological disruption. This study aimed to investigate the impact of the COVID-19 on the circadian rhythm of cesarean deliveries. We investigated whether the circadian rhythm of cesarean section deliveries changed during the first year of the COVID-19 pandemic at a tertiary hospital in Van Province, eastern Turkey. We analyzed the distribution of birth times for 1476 cesarean deliveries performed between March 01, 2020 and January 20, 2021 (1st year of the COVID-19 pandemic) and compared this information with data from 1194 cesarean deliveries performed during a similar period in the previous year. The primary outcome was the change in the circadian rhythm of cesarean deliveries. Secondary outcomes included cesarean section (CS) delivery rates, indications for CS, 1st and 5th minutes Appearance (skin color), Pulse (heart rate), Grimace (reflex irritability), Activity (muscle tone), and Respiration scores, and anesthesia technique use rates. Regarding the time distribution of CS deliveries in the first year of the COVID-19 pandemic, the maximum number of cesarean deliveries (n = 234, 16%) occurred between 14:00 and 16:00 (P = .112). Cesarean deliveries in pre-COVID-19 group were most frequently performed between 10:00 and 12:00, at a rate of 18% (n = 216) (P = .001). In both groups, the time point at which CS deliveries were the least performed was 04:00 to 06:00, and the rates were different (n = 35, 2% and n = 14, 1%, respectively) (P = .022). A 4-hour phase shift was detected at the peak of the birth time in the first year of the COVID-19 pandemic compared to the previous year. These results suggest that the circadian rhythm of cesarean deliveries is affected by the pandemic.

Genotype and clinical phenotype of children with Marfan syndrome in Southeastern Anatolia.

The cardinal phenotypic hallmarks of Marfan syndrome (MFS) include cardiac, ocular, and skeletal abnormalities. Since the clinical phenotype of MFS is highly heterogeneous, with certain symptoms appearing as children age, the diagnostic process and establishing a genotype-phenotype association in childhood MFS can be challenging. The lack of sufficient childhood studies also makes it difficult to interpret the subject. This study aims to evaluate the relationship between clinical symptoms used as diagnostic criteria and FBN1 variations in children with MFS. This study investigated the relationships between genotypes and phenotypes in 131 children suspected of having Marfan syndrome (MFS). Diagnosis of MFS was made according to the revised Ghent nosology. FBN1 variants were categorized based on exon regions, type of variant, and pathogenicity classes. These FBN1 variants were then correlated with the clinical manifestations including cardiovascular, ocular, facial, and skeletal abnormalities. Out of the children, 43 were diagnosed with MFS. FBN1 variant was identified in 32 (74.4%) of the MFS children. MFS diagnosis could not be made in five (15.6%) FBN1 variant-positive children. The most common cardinal finding is cardiac anomalies n = 38 (88.3%). The most common FBN1 pathogenic variant was c.1786 T > C/p.Cys596Arg n = 4 (12.5%). The distribution of pathogenic variants was as follows: 29 (90.6%) missense, 2 (6.3%) frameshift, and 1 (3.1%) nonsense. The numbers of AD and EL of the variant-positive children were 16 (50%) and 14 (43.7%), respectively. Ocular abnormalities were more common in children with FBN1-positive MFS (p = 0.009). There was no difference in the number of cardiac abnormalities between FBN1-positive and FBN1-negative MFS patients (p = 0.139).   Conclusion: This study examines the relationship between FBN1 variants and clinical features used as diagnostic criteria in MFS children. The findings emphasize the importance of long-term monitoring of heterogeneous clinical phenotypes and bioinformatic reanalysis in determining the genotype-phenotype relationship in children, as MFS symptoms can vary with age. What is Known: • Marfan syndrome has highly variable phenotypic heterogeneity. • The genotype-phenotype relationship in childhood Marfan syndrome is not clear enough due to the variation in the time of onset of the findings. What is New: • This article provides regional data for the field of research on genotype-phenotype relationships in childhood Marfan syndrome. • Long-term follow-up of clinical findings and bioinformatics reanalysis is an important requirement for a well-established genotype-phenotype relationship in childhood Marfan syndrome.

Variation coefficient of stone density and renal cortical thickness: the parameters evaluating non-contrast computed tomography imaging for predict extracorporeal shock wave lithotripsy success.

The stone density (SD) is not the same in all parts of the stone due to the heterogeneous nature of the stone and the shock wave (SW) passes through tissues of many different densities until it reaches the stone. These factors affect the success of Extracorporeal Shock Wave Lithotripsy (ESWL). We aimed to evaluate the effect of the Variation Coefficient of Stone Density (VCSD) and Renal Cortical Tickness (RCT) on the success of ESWL. Between 2020 and 2023, 510 patients who underwent ESWL were divided into 2 groups treatment success (n:304) and treatment failure (n:206). Non-Contrast Computed Tomography (NCCT) imaging values of hydronephrosis degree of the kidney, stone location, stone volume (SV), stone-skin distance (SSD), SD, Standard deviation of Stone Density (SDSD), VCSD, RCT, Soft-Tissue Thickness (STT), Muscle Thickness (MT) were analyzed. VCSD value was obtained by dividing SDSD by SD. Along the SW, tissues were divided into three components: kidney (renal cortex), muscle and other soft tissues. RCT, MT and SSD were measured at three different angles (0°, 45°, and 90°) and these 3 lengths were averaged. In univariate analysis, Body Mass Index (BMI), SV, SD, VCSD, SSD, RCT and STT were demonstrated to affect ESWL success. In multivariate analysis, low BMI, SV, SD, RCT and large VCSD were significant independent predictors of ESWL success. Among these parameters, VCSD had the highest prediction accuracy, followed by SD, SV, RCT and BMI, respectively. This study demonstrated that VCSD value and RCT are predictive parameters in determining the treatment of patients with urinary calculi and selecting suitable ESWL candidates.

Effects of hydronephrosis on the surgical outcomes of fluoroscopically guided supine percutaneous nephrolithotomy.

PURPOSE: To evaluate the effect of hydronephrosis on operation success and the development of complications in supine percutaneous nephrolithotomy (PCNL). METHODS: A total 259 patients were included in the study who underwent supine PCNL in our clinic between September 2019 and November 2023. The patients were divided into four groups: normal, mild, moderate and severe, according to their preoperative hydronephrosis degrees. Demographic data, kidney stone characteristics, clinical aspects, surgical findings, and postoperative complications were compared across groups. RESULTS: In terms of American Society of Anesthesiologists score, stone-free rate, operation time, fluoroscopy time, and Clavien-Dindo classification grades, a statistically significant difference was found between the hydronephrosis groups. The stone-free rate in the normal, mild, moderate, and severe groups was 86.6, 82.5, 76.0, and 61.5, respectively. The severe hydronephrosis group varied statistically substantially from the other hydronephrosis groups in terms of stone-free rate, according to the post-hoc analysis. In terms of Clavien-Dindo classification grades, the severe hydronephrosis group varied statistically significantly from the normal and mild hydronephrosis groups (p values 0.04, 0.02, respectively). In terms of Clavien-Dindo classification grades, no statistically significant difference was seen between the severe and moderate hydronephrosis groups (p = 0.085). CONCLUSION: The findings of this study demonstrated that the existence of hydronephrosis was a predictive factor for the occurrence of complications and decreased the success rate of supine PCNL. Furthermore, univariate and multivariate analyses showed that the presence of hydronephrosis was a predictive factor for PCNL success and the development of complications.

Association of childhood asthma with Gasdermin B (GSDMB) and Oromucoid-like 3 (ORMDL3) genes.

OBJECTIVE: Genome-length association studies have shown that Gasdermin B (GSDMB) and Orosomucoid-like 3 (ORMDL3) genes located on the long arm of chromosome 17 are associated with asthma. In this study, it was aimed to determine the possible relationship between asthma control test (ACT), exercise provocation test (ECT), and fractional nitric oxide (FENO) levels and GSDMB and ORMDL3 gene expressions. METHODS: 59 asthmatic and 38 non-asthmatic children were included in the study. We divided the patient group into two subgroups as mild persistent asthma (29 patients) and moderate persistent asthma (30 patients). ORMDL3, GSDMB gene expression levels, ECT, total IgE levels, and eosinophil counts were measured in all cases. In addition, ACT and FeNO levels were measured in children with asthma. Afterward, the relationship of ORMDL3 and GSDMB gene expression coefficient changes with ECT, ACT, and FeNO was examined. RESULTS: When patients with ACT ≤15 were compared with patients with ACT ≥20, ORMDL3 and GSDMB gene expressions were increased 6.74 and 11.74 times, respectively. Comparing patients with ACT ≥20 and ACT ≤15 in terms of coefficient changes (ΔCq), higher change values were observed for ΔCq ORMDL3 in patients with ACT ≤15 (p=0.015). Similarly, when patients with FENO ≤25 ppb were compared with patients with FENO >25 ppb, ORMDL3 and GSDMB gene expressions were increased by 2.93 and 3.56 times, respectively. When the coefficient changes were compared, no significant difference was found between FENO≤25 and FENO >25 patients. There was a slight negative correlation between ΔCq values and ACT score (p=0.003, r=-0.418 for ORMDL3, and p=0.016, r=-0.345 for GSDMB). In addition, we observed a statistically significant positive correlation between ORMDL3 and GSDMB gene expressions (r=0.80, p<0.001). CONCLUSION: We showed that increased ORMDL3 and GSDMB gene expression levels may be associated with ACT scores, FeNO and ECT in asthma. These findings may encourage future studies with larger numbers of subjects that can use gene expression levels in various asthma phenotypes for prognostic prediction.

Increased Severe Cases and New-Onset Type 1 Diabetes Among Children Presenting With Diabetic Ketoacidosis During First Year of COVID-19 Pandemic in Turkey.

Introduction: There have been some significant changes regarding healthcare utilization during the COVID-19 pandemic. Majority of the reports about the impact of the COVID-19 pandemic on diabetes care are from the first wave of the pandemic. We aim to evaluate the potential effects of the COVID-19 pandemic on the severity of diabetic ketoacidosis (DKA) and new onset Type 1 diabetes presenting with DKA, and also evaluate children with DKA and acute COVID-19 infection. Methods: This is a retrospective multi-center study among 997 children and adolescents with type 1 diabetes who were admitted with DKA to 27 pediatric intensive care units in Turkey between the first year of pandemic and pre-pandemic year. Results: The percentage of children with new-onset Type 1 diabetes presenting with DKA was higher during the COVID-19 pandemic (p < 0.0001). The incidence of severe DKA was also higher during the COVID-19 pandemic (p < 0.0001) and also higher among children with new onset Type 1 diabetes (p < 0.0001). HbA1c levels, duration of insulin infusion, and length of PICU stay were significantly higher/longer during the pandemic period. Eleven patients tested positive for SARS-CoV-2, eight were positive for new onset Type 1 diabetes, and nine tested positive for severe DKA at admission. Discussion: The frequency of new onset of Type 1 diabetes and severe cases among children with DKA during the first year of the COVID-19 pandemic. Furthermore, the cause of the increased severe presentation might be related to restrictions related to the pandemic; however, need to evaluate the potential effects of SARS-CoV-2 on the increased percentage of new onset Type 1 diabetes.

A cross sectional study on airborne inhalable microorganisms, endotoxin, and particles in pigeon coops - Risk assessment of exposure.

Pigeon breeding is associated with symptoms of the airways. The aim of this study is to illuminate the bacteriological and toxicological characteristics of airborne dust in pigeon coops. Airborne dust was sampled in 31 urban pigeon coops with homing and fancy pigeons, and following the dust was characterized. In total 141 different bacterial species were identified using MALDI-TOF MS, and of these 11 species are classified in risk group 2. Of the cultivable bacteria, Staphylococcus equorum was present in the highest concentration. Microorganisms in the dust were able to form biofilm, and the amount correlated positively with the number of bacteria. Next generation sequencing showed 180 genera with Acinetobacter in highest reads. On average 999 ± 225 ZOTUs were observed per sample with a Shannon-Wiener biodiversity index of 6.17 ± 0.24. Of the identified species the following have previously been suggested as causative agents of extrinsic allergic alveolitis: Alcaligenes faecalis, Bacillus subtilis, Pantoea agglomerans, Sphingobacterium spiritivorum, Thermoactinomyces sp., and Streptomyces albus. Staphylococcus was present on particles with sizes between 1.1 and > 7.0 µm with a geometric mean diameter of particles on 4.7 ± 1.1 µm. Concentrations of airborne endotoxin and dust were elevated compared to references, and the geometric mean concentrations were 102 EU/m3 and 1.07 mg dust/m3, respectively. Upon exposure to the airborne dust human granulocytes produced Reactive Oxidative Species during the first 5 min, and then no further reaction was observed. The concentrations of bacteria in general, Staphylococcus spp., and endotoxin and biodiversity were associated significantly with season, temperature and/or relative humidity, but not with type or density of pigeons. The bacterial composition and biodiversity indices were not affected by type of pigeon. In conclusion, the exposure to bacteria and endotoxin in pigeon houses should not be neglected in the evaluation of causative agents of airways symptoms among pigeon breeders.

Effects of severe hydronephrosis on the outcomes of percutaneous nephrolithotomy with one-shot dilation method.

OBJECTIVE: To investigate the effect of the presence of severe hydronephrosis on percutaneous nephrolithotomy (PNL) outcome in patients who underwent PNL operation with one-shot dilatation technique. MATERIALS AND METHODS: Medical data of 989 patients underwent PNL operation in our clinic between 2012 and 2018 were retrospectively reviewed. We included 373 of the patients underwent PNL operation due to renal pelvic stone, who were older than 18 years of age, who did not have any urinary tract abnormality, and had no previous history of open renal stone operation. Patients were divided into two groups according to the presence of severe hydronephrosis and absence of hydronephrosis. These two groups were compared in terms of age, gender, body mass index (BMI), number of stones, stone burden, operation duration, fluoroscopy time, hospitalization time, hemoglobin and creatinine change, complications, and stone free rate. RESULTS: There was no statistically significant difference between the groups in terms of age, gender, BMI, stone number, stone size, stone density, operation duration, fluoroscopy time, and hospitalization duration. The mean change in hemoglobin was 1.5 g/dL in group 1 and 1.1 g/dL in group 2. This difference was statistically significant (p = 0.006). Postoperative blood transfusion was required for 3 patients (2.1%) in group 1 and 12 patients (5.1%) in group 2. This difference was statistically significant (p < 0.001). CONCLUSION: Only the presence of severe hydronephrosis was found to be associated with the change in hemoglobin and postoperative blood transfusion.

An investigation of the effects of amniotic fluid on experimental ischemia/reperfusion damage in rat testes.

INTRODUCTION: Various agents have been tested as preventive treatments for ischemia/reperfusion (IR) damage. In this study, we have investigated for the first time in the literature the efficacy of injection of amniomax (AMX) into testicular parenchyma, which is a commercial medium of rat amniotic fluid, in preventing testicular IR damage related to testicular torsion. OBJECTIVE: This study aims to evaluate whether or not amniomax has an effect on experimental IR damage in rat testes using biochemical and histopathological methods based on data in the literature. Even if testicular torsion is repaired surgically in early term injury because of de IR damage still occurs. Is it possible to reduce the ischemia reperfusion injury with amniotic fluid and increase the success of treatment? STUDY DESIGN: 40 male Wistar albino rats were included. Four groups were formed with 10 rats in each group: Sham, Ischemia/Reperfusion (IR), injection 1 min before detorsion (AMX-BD), injection 1 min after detorsion (AMX-AD). Total Oxidant Status (TOS) and Oxidative Stress Index (OSI) were computed for oxidative stress, and Total Antioxidant Status (TAS) levels were computed for the antioxidant system, for both serum and tissue. Necrosis and microcalcification levels were assessed in the evaluation of testicular histology. P < 0.05 was considered statistically significant. RESULTS: AMX-AD group has low necrosis degree than IR, Mean serum and tissue levels of TAS, TOS, and OSI parameters were respectively determined as; for TAS: 0.64 ± 0.11 and 0.96 ± 0.25 mmol Trolox Equivalent/L; for TOS: 6.71 ± 0.87 and 9.40 ± 1.03 µmol H2O2 equivalent/L; for OSI: 11.94 ± 3.74 and 10.70 ± 4.23 arbitrary unit. DISCUSSION: Our study has investigated for the first time in the literature the efficacy of amniotic fluid in preventing testicular IR damage, and used amniomax (AMX) for this purpose. The limitation of our study may be the small number of rat in the groups. CONCLUSION: We think an injection after detorsion is more favorable considering that the AMX-AD group demonstrated significantly lower levels of TOS in serum and tissue and OSI in serum, and significantly higher serum levels of TAS compared to the AMX-BD group, as well as the fact that the morphological protection effect was only observed for injections performed immediately after detorsion.

Clinical and Hormonal Profiles Correlate With Molecular Characteristics in Patients With 11ß-Hydroxylase Deficiency.

BACKGROUND: Given the rarity of 11ß-hydroxylase deficiency (11ßOHD), there is a paucity of data about the differences in clinical and biochemical characteristics of classic (C-11ßOHD) and nonclassic 11ßOHD (NC-11ßOHD). OBJECTIVE: To characterize a multicenter pediatric cohort with 11ßOHD. METHOD: The clinical and biochemical characteristics were retrospectively retrieved. CYP11B1 gene sequencing was performed. Seventeen plasma steroids were quantified by liquid chromatography-mass spectrometry and compared to that of controls. RESULTS: 102 patients (C-11ßOHD, n = 92; NC-11ßOHD, n = 10) from 76 families (46,XX; n = 53) had biallelic CYP11B1 mutations (novel 9 out of 30). Five 46,XX patients (10%) were raised as males. Nineteen patients (19%) had initially been misdiagnosed with 21-hydroxylase deficiency. Female adult height was 152 cm [-1.85 SD score (SDS)] and male 160.4 cm (-2.56 SDS).None of the NC-11ßOHD girls had ambiguous genitalia (C-11ßOHD 100%), and none of the NC-11ßOHD patients were hypertensive (C-11ßOHD 50%). Compared to NC-11ßOHD, C-11ßOHD patients were diagnosed earlier (1.33 vs 6.9 years; P < 0.0001), had higher bone age-to-chronological age (P = 0.04) and lower adult height (-2.46 vs -1.32 SDS; P = 0.05). The concentrations of 11-oxygenated androgens and 21-deoxycortisol were low in all patients. The baseline ACTH and stimulated cortisol were normal in NC-11ßOHD. Baseline cortisol; cortisone; 11-deoxycortisol; 11-deoxycorticosterone and corticosterone concentrations; and 11-deoxycortisol/cortisol, 11-deoxycorticosterone/cortisol, and androstenedione/cortisol ratios were higher in C-11ßOHD than NC-11ßOHD patients (P < 0.05). The 11-deoxycortisol/cortisol ratio >2.2, <1.5, and <0.1 had 100% specificity to segregate C-11ßOHD, NC-11ßOHD, and control groups. CONCLUSION: NC-11ßOHD can escape from clinical attention due to relatively mild clinical presentation. However, steroid profiles enable the diagnosis, differential diagnosis, and subtyping of 11ßOHD.

Comparação de métodos de tratamento tópico utilizados na epistaxe anterior recorrente: ensaio clínico randomizado / Comparison of topical treatment methods used in recurrent anterior epistaxis: a randomized clinical trial

Resumo Introdução: A epistaxe recorrente é uma doença comumente vista por especialistas em otorrinolaringologia, médicos de emergência e pediatras. O fato de que muitas modalidades de tratamento estejam sendo pesquisadas e nenhum método único de tratamento seja universalmente aceito apoiam ainda mais essa informação. Objetivo: Comparar a eficácia clínica do uso de pomada antisséptica tópica, pomada descongestionante tópica e tratamentos de cauterização química, que são frequentemente usados em epistaxe anterior recorrente, tanto isoladamente como em combinação. Método: Entre agosto de 2017 e fevereiro de 2018, 137 pacientes diagnosticados com epistaxe anterior recorrente foram divididos aleatoriamente em 5 grupos. O grupo I foi tratado com pomada antisséptica tópica, o grupo II com pomada descongestionante tópica, o grupo III foi submetido a cauterização química, o grupo IV foi tratado com pomada antisséptica tópica + cauterização química e o grupo V com pomada descongestionante tópica + tratamento de cauterização química. Todos os pacientes foram contatados por telefone 2 semanas e um mês após o tratamento e perguntados sobre a presença (falha) ou ausência (sucesso) de pelo menos um episódio de epistaxe. Pacientes com comorbidades foram excluídos. O sucesso do tratamento foi analisado estatisticamente. Resultados: Não houve diferença significante (p > 0,05) entre os grupos em relação à taxa de sucesso no 15° dia após o tratamento. Os grupos IV e V tiveram maiores taxas de sucesso no 30° dia após o tratamento em comparação com os grupo I e II (p < 0,05). No grupo III, o sucesso do tratamento no 30° dia não foi diferente dos outros 4 grupos (p > 0,05). Conclusão: Embora o número de pacientes que melhoraram com a cauterização química (grupo III) tenha sido maior em nosso estudo, nenhuma diferença significante foi observada nas modalidades de tratamento único (grupos I - III) no 14° dia e no 30° dia após o tratamento. Embora não tenha sido observada diferença estatisticamente significante entre os tratamentos combinados (grupos IV - V) e os tratamentos simples (grupos I - III) na 2ª semana após o tratamento, os tratamentos combinados foram significantemente mais eficazes no 1° mês.

Exhaled TGF-ß1 levels before and after an exercise challenge in asthmatic and healthy children, and during exacerbation.

BACKGROUND: There is conflicting data regarding the role of transforming growth factor-ß1 (TGF-ß1) in the pathogenesis of airway hyper-reactivity and asthma exacerbation. OBJECTIVE: To investigate the role of exhaled-TGF-ß1 in exercise-induced bronchospasm (EIB) in asthmatic and nonasthmatic healthy children, and in asthma exacerbation and asthma control. METHODS: The exhaled-TGF-ß1 levels of 56 stable asthmatic children and 15 nonasthmatic healthy children were evaluated before and 30 min after an exercise challenge. The exhaled-TGF-ß1 levels of 20 additional children with asthma exacerbation were evaluated. RESULTS: While no significant difference in the exhaled-TGF-ß1 levels was found at the baseline, exhaled-TGF-ß1 levels after the exercise challenge were significantly higher in the non-EIB (n = 31) asthmatics when compared to the asthmatic children with EIB (n = 25) (p = 0.04). Although there was a statistically significant increase in the concentration of the exhaled-TGF-ß1 after the exercise challenge in the non-EIB asthmatics (p = 0.008), the concentration of the TGF-ß1 was not increased after the exercise challenge in EIB + asthmatics. The exhaled-TGF-ß1 was significantly correlated with the ACT score (p = 0.01, r = 0.49) and the baseline FEV1 level (p = 0.02, r = 0.35). The exhaled-TGF-ß1 levels were significantly higher in the stable asthmatic children when compared to the nonasthmatic children (p < 0.0001). There was no significant difference in exhaled-TGF-ß1 levels after the exercise challenge in the nonasthmatics. The exhaled-TGF-ß1 levels were significantly lower in those children with asthma exacerbation when compared to the stable asthmatic children (p = 0.0003). CONCLUSION: Our results suggest that TGF-ß1 may play a role in suppressing airway reactivity and its deficiency is associated with asthma exacerbation.

Relationship between testicular histopathology and the success of testicular sperm extraction in patients with non-mosaic Klinefelter syndrome.

OBJECTIVE: To investigate the effect of testicular histopathology on the success of microscopic testicular sperm extraction (mTESE) and the factors that could predict the success of mTESE in patients with non-mosaic Klinefelter syndrome (KS). MATERIAL AND METHODS: Forty-one KS patients diagnosed with non-obstructive azoospermia (NOA) who had undergone mTESE at our clinic were included in the study. The patients were divided into 5 groups according to the histopathology results: hyalinisation of tubules (HT), sertoli cell only (SCO), early maturation arrest (EMA), late maturation arrest (LMA), and hypospermatogenesis (HS). The groups were compared with regard to age, duration of infertility, hormone profile, testicular volume, and sperm retriveal rate. The clinical features of the patients with mTESE from whom sperm could or could not be obtained were also compared with the aim of investigating the predictive value of testicular histopathology and the other variables for prediction of the success of mTESE. RESULTS: Sperm could be obtained through mTESE in 13 out of 41 patients (31.7%). A statistically significant difference was determined between the groups with regard to the rate of sperm collection. No significant difference was determined between the histopathology groups with regard to the other variables. A statistically significant difference was determined between the groups from whic sperm could be collected or not with regard to age, Johnsen criteria, SCO, EMA and LMA variables. Multi-variate analysis revealed that age and Johnsen score were the independent variables predictive for success of mTESE. CONCLUSION: The present study has revealed that impairment in testicular histopathology negatively affects the success of mTESE and that it is a predictive factor for the success of mTESE in patients with KS. Increased patient age was also determined to negatively affect the success of mTESE and the operation was demonstrated to be more successful before 34 years of age.