True empty follicle syndrome is a subtype of oocyte maturation abnormalities.

Objective: To review the outcomes of in vitro maturation (IVM) and in vitro fertilization (IVF) in women with empty follicle syndrome (EFS). The study evaluated the genetic underpinnings of EFS by analyzing mutations. Materials and Methods: This retrospective case series involving 17 women with EFS over at least 2 IVF cycles was conducted. The study also employed whole-exome sequencing to analyze the genetic mutations. The treatment approaches included letrozole-primed IVM, follicle-stimulating hormone (FSH)-human chorionic gonadotrophin (hCG)-primed IVM, and conventional IVF. Results: The average female age was 31.5±4.6 years, and the duration of infertility was 7.3±3.5 years. Four patients underwent IVF. IVM oocyte collections yielded oocytes in 12 of 13 subjects. Of these, 75% (9/12) yielded MII oocytes after 48 h of IVM media incubation. Six subjects had fertilized embryos, resulting in a 40.9% intracytoplasmic sperm injection (ICSI) fertilization rate (9 embryos/22 MII oocytes). Genetic analysis revealed mutations in seven patients. This study demonstrated the partial efficacy of letrozole-primed IVM plus growth hormone and FSH-hCG primed IVM protocols. No pregnancies or live births were recorded after IVM. One ongoing pregnancy post-IVF and one spontaneous live birth were observed. Conclusion: Inter-cycle variabilities were observed in women with oocyte maturation abnormalities (OMAs). Almost all patients with EFS had oocytes collected during IVM following IVF. These oocytes have limited potential for maturation, fertilization, and live birth, as demonstrated by the low rates observed after IVM culture and ICSI. These conditions are observed in OMAs due to defects in the oocyte machinery. The proposed flowchart provides a comprehensive classification approach for various forms of EFS.

Retrospective evaluation of acute kidney injury in paediatric COVID-19 patients: a tertiary referral hospital experience.

BACKGROUND: Coronavirus disease 2019 (COVID-19) has been recognised as a risk factor for acute kidney injury (AKI). Our aim was to investigate the risk factors contributing to hospitalised and outpatient paediatric COVID-19-associated AKI. METHODS: A retrospective observational study was conducted on patients aged 1 month to 18 years with diagnosed COVID-19-associated AKI applied to a tertiary paediatric referral hospital between March 1, 2020 and March 1, 2022. RESULTS: A total of 6683 patients were evaluated and 486 patients were included in the study. Acute kidney injury was observed in 3.7% of outpatients and 23.9% of hospitalised patients. Multivariate logistic regression analysis showed that, on admission, a history of contact with a COVID-19 positive person (p < 0.001), age below 12 months (p = 0.004), presence of comorbidities (p < 0.001), abdominal pain (p = 0.008), anorexia (p = 0.003), dyspnoea (p = 0.005), higher lactate dehydrogenase values (p = 0.004), neutrophilia (p < 0.001), higher neutrophil-to-lymphocyte ratio (NLR) (p = 0.003), higher white blood cell counts (p = 0.006), elevated C-reactive protein (CRP) levels (p = 0.002), anaemia (p = 0.015), hypoalbuminaemia (p < 0.001), hyperglycaemia (p = 0.006), and presence of proteinuria (p = 0.003) were independent predictors of AKI. Higher rates of hospitalisation (p < 0.001) and admission to the paediatric intensive care unit (PICU) (p < 0.001), longer length of hospitalisation (p < 0.001), and greater need for mechanical ventilation (p < 0.001) were associated with AKI. CONCLUSIONS: This study reveals that not only hospitalised children, but also paediatric patients are at risk for AKI. The presence of comorbidities, abdominal pain, anorexia, dyspnoea, anaemia, inflammation, hypoalbuminaemia, proteinuria and history of contact with a COVID-19 positive person were the main risk factors for AKI. COVID-19-associated AKI was associated with worse outcomes.

Effect of sex on chronic stress induced alterations in hindbrain catecholaminergic system and autonomic dysfunction resulting in gastrointestinal dysmotility.

It has been reported that the clinical symptoms of functional dyspepsia (FD) exacerbate upon stress while the gender-related factors have been incompletely understood. This study aims to investigate the role of sex in chronic heterotypic stress (CHS)-induced autonomic and gastric motor dysfunction. For CHS, the rats were exposed to the combination of different stressors for 7 consecutive days. Subsequently, electrocardiography was recorded in anesthetized rats to evaluate heart rate variability (HRV) for the determination of autonomic outflow and sympathovagal balance. Solid gastric emptying (GE) was measured in control and CHS-loaded male and female rats. The immunoreactivities of catecholaminergic cell marker tyrosine hydroxylase (TH), choline acetyltransferase (ChAT), corticotropin releasing factor (CRF), and estrogen receptor (ER-α/ß) were evaluated in medullary and pontine brainstem sections by immunohistochemistry. Compared with the controls, CHS significantly delayed GE in males but not in females. There was no significant sex-related difference in parasympathetic indicator HF under either control or CHS conditions. Sympathetic indicator LF was significantly higher in control females compared to the males. The higher sympathetic output in females was found to be attenuated upon CHS; in contrast, the elevated sympathetic output was detected in CHS-loaded males. No sex- or stress-related effect was observed on ChAT immunoreactivity in the dorsal motor nucleus of N.vagus (DMV). In males, greater number of TH-ir cells was observed in the caudal locus coeruleus (LC), while they were more densely detected in the rostral LC of females. Regardless of sex, CHS elevated immunoreactivity of TH throughout the LC. Under basal conditions, greater number of TH-ir cells was detected in the rostral ventrolateral medulla (RVLM) of females. In contrast, CHS remarkably increased the number of TH-ir cells in the RVLM of males which was found to be decreased in females. There was no sex-related alteration in TH immunoreactivity in the nucleus tractus solitarius (NTS) of control rats, while CHS affected both sexes in a similar manner. Compared with females, CRF immunoreactivity was prominently observed in control males, while both of which were stimulated by CHS. ER-α/ß was found to be co-expressed with TH in the NTS and LC which exhibit no alteration related to either sex or stress status. These results indicate a sexual dimorphism in the catecholaminergic and the CRF system in brainstem which might be involved in the CHS-induced autonomic and visceral dysfunction occurred in males.

Protective Effects of Adropin in Experimental Subarachnoid Hemorrhage.

PURPOSE: We aimed to investigate early effects of exogenously administered adropin (AD) on neurological function, endothelial nitric oxide synthase (eNOS) expression, nitrite/nitrate levels, oxidative stress, and apoptosis in subarachnoid hemorrhage (SAH). METHODS: Following intracerebroventricular AD administration (10 µg/5 µl at a rate of 1 µl/min) SAH model was carried out in Sprague-Dawley rats by injection of autologous blood into the prechiasmatic cistern. The effects of AD were assessed 24 h following SAH. The modified Garcia score was employed to evaluate functional insufficiencies. Adropin and caspase-3 proteins were measured by ELISA, while nitrite/nitrate levels, total antioxidant capacity (TAC) and reactive oxygen/nitrogen species (ROS/RNS) were assayed by standard kits. eNOS expression and apoptotic neurons were detected by immunohistochemical analysis. RESULTS: The SAH group performed notably lower on the modified Garcia score compared to sham and SAH + AD groups. Adropin administration increased brain eNOS expression, nitrite/nitrate and AD levels compared to SHAM and SAH groups. SAH produced enhanced ROS/RNS generation and reduced antioxidant capacity in the brain. Adropin boosted brain TAC and diminished ROS/RNS production in SAH rats and no considerable change amongst SHAM and SAH + AD groups were detected. Apoptotic cells were notably increased in intensity and number after SAH and were reduced by AD administration. CONCLUSIONS: Adropin increases eNOS expression and reduces neurobehavioral deficits, oxidative stress, and apoptotic cell death in SAH model. Presented results indicate that AD provides protection in early brain injury associated with SAH.

Survival Prediction of Patients after Heart Attack and Breast Cancer Surgery with a Hybrid Model Built with Particle Swarm Optimization, Stacked AutoEncoders, and the Softmax Classifier.

The prediction of patient survival is crucial for guiding the treatment process in healthcare. Healthcare professionals rely on analyzing patients' clinical characteristics and findings to determine treatment plans, making accurate predictions essential for efficient resource utilization and optimal patient support during recovery. In this study, a hybrid architecture combining Stacked AutoEncoders, Particle Swarm Optimization, and the Softmax Classifier was developed for predicting patient survival. The architecture was evaluated using the Haberman's Survival dataset and the Echocardiogram dataset from UCI. The results were compared with several Machine Learning methods, including Decision Trees, K-Nearest Neighbors, Support Vector Machines, Neural Networks, Gradient Boosting, and Gradient Bagging applied to the same datasets. The findings indicate that the proposed architecture outperforms other Machine Learning methods in predicting patient survival for both datasets and surpasses the results reported in the literature for the Haberman's Survival dataset. In the light of the findings obtained, the models obtained with the proposed architecture can be used as a decision support system in determining patient care and applied methods.

The evaluation of depression and anxiety levels of mothers of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome and familial Mediterranean fever.

OBJECTIVE: Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome and familial Mediterranean fever (FMF) are autoinflammatory disorders typically characterized by recurrent fever attacks. These recurrent fever attacks can lead to depression and anxiety in mothers of these patients. This study aimed to compare the depression and anxiety levels in mothers of PFAPA and FMF patients. METHODS: This study is a cross-sectional observational study. 48 mothers of children with FMF and 70 mothers of children with PFAPA participated in the study. Mothers in these two groups were compared in terms of anxiety and depression by using the validated Beck Depression Inventory (BDI) and Beck Anxiety Inventory (BAI). RESULTS: Depression and anxiety scores of mothers were found to be similar in FMF and PFAPA groups. Moderate or high level of anxiety was seen in 32% of mothers of patients with PFAPA and 27% of mothers of patients with FMF. 23% of mothers of patients with PFAPA were evaluated as having moderate or severe depression, and 18% of mothers of patients with FMF were evaluated as having moderate depression. There was no statistically significant difference between the duration, frequency of attacks, recurrent hospitalizations, sociodemographic characteristics, and inventory scores. CONCLUSION: Depression and anxiety scores of mothers with children diagnosed with FMF and PFAPA are similar. These two diseases affect families psychosocially at similar levels. It is important to provide psychosocial support to families.

Effect of Colchicine Treatment on Clinical Course in Children with PFAPA Syndrome.

INTRODUCTION: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is the most common periodic fever condition in children. There is no consensus on treatment to prevent attacks and reduce their frequency. In this study, we aimed to evaluate the effectiveness of colchicine treatment in PFAPA syndrome. In addition, we described the demographic and clinical features of PFAPA patients. MATERIALS AND METHODS: We retrospectively analyzed 58 PFAPA patients who were started on colchicine treatment between January 2017 and January 2022. Demographic data, clinical features, laboratory tests, genetic analysis of MEditerranean FeVer (MEFV) mutations, and autoinflammatory disease activity index (AIDAI) scores of all patients were evaluated. In addition, patients were divided into two groups according to MEFV variants and compared. RESULTS: Attack frequency, duration, and AIDAI scores decreased in all patients after colchicine treatment. Duration of follow-up was 13.53±6.65 months. The median±IQR age at diagnosis was 3.2 (2-5) years. Thirty three (56.9%) patients had heterozygous mutations of MEFV. The most common MEFV variants were M694V (63.6%). There was no significant difference between the two groups in terms of colchicine responses. CONCLUSION: Colchicine treatment is effective and safe in patients with PFAPA who have frequent attacks. No association was established between the presence of heterozygous mutations of MEFV and colchicine response.

The effect of B-Lynch uterine compression suture performed for uterine atony on future menstrual pattern and reproductive outcome.

OBJECTIVES: The effects of B-Lynch (UCS) compression sutures applied in postpartum hemorrhage cases due to uterine atony on menstrual pattern, fertility, obstetric outcomes, dysmenorrhea and dyspareunia were evaluated. MATERIAL AND METHODS: Between January 2012 and March 2017, 77 patients (study group 37, control group 40) diagnosed postpartum hemorrhage in our clinic were included in the study. The long-term results of the patients were evaluated comparatively. RESULTS: In the B-Lynch UCS group, an increase in the postoperative menstrual cycle length and the intensity of dyspareunia measured by the VAS score, and a statistically significant decrease in the duration of menstrual bleeding were observed. In the control group, a decrease in the self-estimated time of postpartum menstrual bleeding and a statistically significant increase in dyspareunia VAS values ​​were observed. There was a statistically significant difference between the groups in terms of menstrual cycle length only after treatment. CONCLUSIONS: B-Lynch UCS can be used effectively and safely in PPH due to uterine atony without causing any additional pathology in menstrual pattern, fertility, dysmenorrhea and dyspareunia complaints other than the length of the menstrual cycle.

Evaluation of immature granulocyte percentage and count in pediatric nephrotic syndrome.

OBJECTIVE: The importance of immature granulocytes (IGs) in most infectious and inflammatory diseases has been highlighted. This study aimed to determine the clinical usability and importance of changes in the peripheral complete blood count profile, including IG percentage (IG%) and IG count (IG#), during the relapse and remission phases in pediatric nephrotic syndrome (NS) patients. METHODS: This retrospective observational study was performed at a tertiary care hospital between February 2020 and August 2022. Demographic characteristics and laboratory parameters were recorded. The IG count and IG% were measured using an automated hematological analyzer. RESULTS: IG% and IG# were both higher during the relapse phase of NS than during the remission phase (0.29% ± 0.14%, versus 0.23% ± 0.14%, p = 0.037 and 0.027 ± 0.015 × 103/µL, versus 0.018 ± 0.014 × 103/µL, p = 0.005, respectively). The neutrophil to lymphocyte ratio (NLR), platelet (PLT), white blood cell (WBC), and neutrophil counts had a strong positive correlation with IG# (r = 0.397, p < 0.001; r = 0.352, p < 0.001; r = 0.622, p < 0.001; r = 0.660, p < 0.001, respectively). The NLR, PLT, WBC, and neutrophil counts had a strong positive correlation with IG% (r = 0.348, p < 0.001; r = 0.187, p = 0.039; r = 0.303, p = 0.001; r = 0.426, p < 0.001, respectively). Receiver operating characteristic curve analysis showed that IG# had the best AUC value of 0.69 (95% CI: 0.58-0.77; p = 0.001) for the relapse phase of NS with a cutoff value of 0.025 × 103/µL (sensitivity: 81.0%, specificity: 78.1%). CONCLUSIONS: It is probable that a high level of immature granulocyte count has a positive correlation for NS relapse in pediatric patients. The IG % and IG# can be used together as biomarkers of inflammation in pediatric NS relapse.

Inflammatory comorbidities in the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG).

AIM: The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases. MATERIALS AND METHODS: Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups. RESULTS: The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis (n = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 (p < 0.05). Fever and abdominal pain were more frequently detected in Group 2 (p < 0.05). FMF patients with concomitant inflammatory diseas more frequently demonstrated colchicine resistance. There were no significant differences in the median attack frequency, chest pain, amyloidosis, erysipelas-like erythema, or family history of FMF between the two patient groups. CONCLUSION: To the best of our knowledge, this is the largest pediatric cohort reviewed to date. FMF patients may have different clinical profiles and colchicine responses if they have with concomitant inflammatory diseases. Key points • FMF is associated with some inflammatory comorbidities diseases. • To the best of our knowledge, this is the largest cohort evlauated pediatric FMF associated inflammatory comorbidities diseases reviewed to date.

A score for predicting colchicine resistance at the time of diagnosis in familial Mediterranean fever: data from the TURPAID registry.

OBJECTIVES: Colchicine forms the mainstay of treatment in FMF. Approximately 5-10% of FMF patients are colchicine resistant and require anti-IL-1 drugs. We aimed to compare the characteristics of colchicine-resistant and colchicine-responsive patients and to develop a score for predicting colchicine resistance at the time of FMF diagnosis. METHODS: FMF patients (0-18 years) enrolled in the Turkish Paediatric Autoinflammatory Diseases (TURPAID) registry were included. The predictive score for colchicine resistance was developed by using univariate/multivariate regression and receiver operating characteristics analyses. RESULTS: A total of 3445 FMF patients [256 (7.4%) colchicine-resistant and 3189 colchicine-responsive) were included (female:male ratio 1.02; median age at diagnosis 67.4 months). Colchicine-resistant patients had longer, more frequent attacks and were younger at symptom onset and diagnosis (P < 0.05). Fever, erysipelas-like erythema, arthralgia, arthritis, myalgia, abdominal pain, diarrhoea, chest pain, comorbidities, parental consanguinity and homozygosity/compound heterozygosity for exon 10 MEFV mutations were significantly more prevalent among colchicine-resistant than colchicine-responsive patients (P < 0.05). Multivariate logistic regression analysis in the training cohort (n = 2684) showed that age at symptom onset, attack frequency, arthritis, chest pain and having two exon 10 mutations were the strongest predictors of colchicine resistance. The score including these items had a sensitivity of 81.3% and a specificity of 49.1%. In the validation cohort (n = 671), its sensitivity was 93.5% and specificity was 53.8%. CONCLUSION: We developed a clinician-friendly and practical predictive score that could help us identify FMF patients with a greater risk of colchicine resistance and tailor disease management individually at the time of diagnosis.

The role of hypothalamic Orexin-A in stress-induced gastric dysmotility: An agonistic interplay with corticotropin releasing factor.

BACKGROUND: Central Orexin-A (OXA) modulates gastrointestinal (GI) functions and stress response. This study aimed to investigate whether OXA and CRF interact at hypothalamic level. METHODS: Solid gastric emptying (GE), fecal output (FO), plasma corticosterone (CORT), and postprandial antro-pyloric motility were assessed in rats that underwent acute restraint stress (ARS) and pretreated with central OX1R and/or CRF receptor antagonists SB-334867 and alpha-helical CRF9,41 . Microdialysis was performed to assess ARS-induced release of OXA and CRF in PVN and LHA, respectively. Immunofluorescence labeling was performed to detect the stress-induced changes in OXA and to assess the hypothalamic distribution of OX1R and CRF1/2 receptors. ARS-induced c-Fos immunoreactivity was evaluated in PVN and LHA of rats received OX1R and CRF receptor antagonists. KEY RESULTS: ARS delayed GE by disturbing the coordination of antro-pyloric contractions while stimulating FO and CORT secretion. ARS-induced alterations in GE, FO, plasma CORT, and antro-pyloric motility were attenuated by OX1R and/or CRF receptor antagonists, however, these changes were completely restored in rats received both antagonists. ARS stimulated release of OXA and CRF which were significantly attenuated by α-CRF9,41 and SB-334867, respectively. The OX1R was detected in CRF-immunoreactive cells, whereas dense expression of CRF2 receptor but not CRF1 was observed in LHA. ARS remarkably increased OXA immunoreactivity in LHA. ARS-induced c-Fos expression in LHA and PVN was abolished by α-CRF9,41 and SB-334867, respectively. CONCLUSIONS & INFERENCES: Our findings suggest a reciprocal contribution of OXA and CRF which seems to be involved in the mediation of stress-induced alterations in neuroendocrine and GI motor functions.

Evaluating the requirement of ultrasonography for children with their first urinary tract infection.

BACKGROUND: Management of urinary tract infection (UTI) in children remains important. It may be the first sign for a possible underlying congenital abnormalities for the kidney and urinary tract (CAKUT). This study examined whether performing renal and bladder ultrasonography (RBUS) only for children who have a pathogen other than E. coli during their first urinary tract infection (UTI), or who experience UTI recurrence, would result in more missed diagnoses of kidney anomalies. METHODS: Patients aged between 2 months and 2 years who were seen in a tertiary pediatric hospital during a 2-year period and diagnosed with UTI were included. RBUS and voiding cystourethrography (VCUG) were performed according to American Academy of Pediatrics (AAP) guidelines. Afterwards, we looked back and evaluated how often we found kidney problems when we only did a RBUS on patients who had an atypical cause of their first UTI or who had multiple UTIs. RESULTS: One hundred and seventy-eight patients who were followed up with UTI were included in this study. The isolated pathogen was E. coli in 104 cases (58.4 %) and atypical in 74 cases (41.6 %). VCUG was conducted on 40 patients, and vesicoureteral reflux (VUR) was discovered in 16 cases and ureteropelvic junction obstruction (UPJO) was discovered in 1 case. A different diagnostic approach that required the presence of an atypical pathogen at the first UTI or a fUTI recurrence to perform the RBUS would have missed just two severe kidney anomalies. It was observed that there could be a decrease of 40.4 % in RBUS and at least 20 % in VCUG. CONCLUSIONS: A diagnostic approach that necessitates the presence of an abnormal pathogen during the initial UTI or a second UTI episode for the RBUS to be carried out would lead to fewer negative ultrasounds with minimal risk of overlooking kidney anomalies.

The regulatory role of central neuropeptide-S in locomotion.

Central exogenous Neuropeptide-S (NPS) was demonstrated to increase locomotor activity (LMA) in rodent studies. NPS receptor (NPSR) is produced in locomotion-related brain regions including basal ganglia while NPS mediates dopaminergic neurotransmission suggesting that endogenous brain NPS is involved in the regulation of locomotion. Aim of the study was to elucidate whether antagonism of NPSR impairs locomotion and to determine the neurochemical profile of NPSR-expressing cells in basal ganglia network. In the rats received intracerebroventricular injection of selective non-peptide NPSR antagonist ML154 (20 nmol/5 µL) or vehicle, in addition to measurement of catalepsy, motor performance, and motor coordination were evaluated by assessment of LMA and RR test, respectively. The immunoreactivities for NPSR, tyrosine hydroxylase (TH), glutamate decarboxylase 67 (GAD67), and choline acetyltransferase (ChAT) were detected by immunofluorescence in frozen sections. Compared to the control rats, total LMA was significantly declined following ML154 administration. The ML154-injected rats were more prone to fall in rotarod (RR) test, while they exhibited remarkably high catalepsy time. The most robust immunoreactivity for NPSR was detected in globus pallidus externa (GPe), while moderate levels of NPSR expression were observed in substantia nigra pars compacta (SNpc) and ventral tegmental area (VTA), but not in striatum. The NPSR-ir cell bodies were found to express GAD67 in GPe and TH in SNpc and VTA, respectively. NPSR expression was detected in SNpc-projecting pallidal cells. The present findings indicate the regulatory role of central endogenous NPS in the control of locomotion. NPSR may be a potential therapeutic target for the treatment of movement disorders.

Complications Related to Childhood Idiopathic Nephrotic Syndrome, Its Treatment and the Associated Risks in Patients.

Aim Nephrotic syndrome is the most common childhood glomerular disorder, but data on the associated complications are limited and predisposing risk factors have not been fully defined. The aim of this study was to evaluate disease- and treatment-related acute and chronic complications in patients with childhood idiopathic nephrotic syndrome (INS), and to identify the risk factors involved in the development of complications. Methods This single-center study was performed at the pediatric nephrology department of a tertiary pediatric hospital in Turkey. The study included 411 patients with a diagnosis of childhood INS, 128 of whom had disease-related and treatment-related complications. Patients diagnosed and followed-up between January 2010 and January 2022 were evaluated retrospectively. Results Complications occurred in 31.1% of the 411 patients. Mean age at the time of diagnosis was 7.54 ± 4.37 years, and the male/female ratio was 0.9:1. Among the patients with complications, 96.9% were disease-related, and 50.8% were treatment-related complications. In older age, high proteinuria level, a low estimated glomerular filtration rate (eGFR) level at diagnosis, and female gender were significant risk factors for complication development (P = 0.000, P = 0.006, P = 0.04, and P = 0.07, respectively). Chronic kidney disease (CKD) developed in 7% of patients and 2.9% of patients had end-stage renal disease (ESRD). Additionally, three of 12 patients with progressive ESRD underwent transplantation. Also the incidence of ESRD was significantly higher in the patients with complications than in those without complications (P < 0.05). Conclusion The present findings suggest that careful monitoring of patients with childhood INS at risk for complications and implementation of personalized treatment programs can improve long-term outcomes, especially in patients that progress to ESRD and are followed-up with dialysis or transplantation as targeted therapy.

How common is remission in rheumatoid factor-positive juvenile idiopathic arthritis patients? The multicenter Pediatric Rheumatology Academy (PeRA) research group experience.

OBJECTIVE: Rheumatoid factor (RF)-positive polyarthritis is the least common type of juvenile idiopathic arthritis (JIA). Functional disability in RF-positive polyarthritis patients is much more severe than in patients with other subtypes; but data on this subtype alone is limited. This study aimed to analyze clinical features, long-term follow-up, treatment response, and remission status in a large multicenter cohort of RF-positive polyarthritis patients. METHODS: This retrospective study included RF-positive polyarthritis patients that were followed up for ≥ 6 months between 2017 and 2022 by the Pediatric Rheumatology Academy (PeRA)-Research Group (RG). Data on patient demographics, clinical and laboratory characteristics were obtained from medical charts. JIA treatments and duration of treatment were also recorded. The patients were divided into 2 groups based on methotrexate (MTX) response, as follows: group 1: MTX responsive, group 2: MTX unresponsive. Clinical and laboratory findings were compared between the 2 groups. RESULTS: The study included 56 (45 female and 11 male) patients. The median age at onset of RF-positive polyarthritis was 13.2 years [(interquartile range) (IQR): 9.0-15.0 years] and the median duration of follow-up was 41.5 months (IQR: 19.5-75.7 months). Symmetrical arthritis affecting the metacarpophalangeal and proximal interphalangeal joints of the hands was commonly observed. Subcutaneous MTX was the preferred initial treatment; however, it was ineffective in 39 (69.6%) of the patients. Of 25 patients followed for 24 months, 56% still had active disease at 24 months. CONCLUSION: During 2 years of treatment, 44% of RF-positive polyarthritis patients have inactive disease, and they should be considered as a distinct and important clinical entity requiring aggressive and early treatment.

Comparison of clinical and laboratory features and treatment responses in patients with clinically amyopathic juvenile dermatomyositis and classical juvenile dermatomyositis.

AIM: The aim of this study was to compare the clinical and laboratory features, treatment choices and responses, and outcomes between patients with clinically amyopathic juvenile dermatomyositis (CAJDM) and classical juvenile dermatomyositis (JDM). METHODS: We retrospectively reviewed the medical records of patients with CAJDM and JDM, and compared the 2 groups' clinical and laboratory data, treatment agents and responses, and outcomes. RESULTS: There were 38 JDM and 12 CAJDM patients, with female dominance. There was a higher delay time in diagnosis for CAJDM (P = 0.000). Compared to other clinical symptoms of JDM, muscle weakness and myalgia were more prominent in JDM than in CAJDM (P = 0.000). The absolute lymphocyte count was lower (P = 0.034) in patients with JDM than in those with CAJDM. Anti-p155/140 (TIF-1) antibody positivity was significantly more common in the CAJDM group (P = 0.000), while anti-NXP2 antibody was more common in the JDM group (P = 0.046). In terms of treatment, pulse corticosteroid usage was more common in patients with JDM than in those with CAJDM (P = 0.000). CONCLUSION: Close clinical follow-ups with effective treatments are important to prevent complications, such as calcinosis and skin ulcers, that may develop in patients with poorly controlled CAJDM. Anti-p155/140 antibodies may be a useful indicator for detecting amyopathic forms of dermatomyositis in children.

Remission rates and risk factors for relapse in pediatric morphea: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG).

AIM: Morphea, also known as localized scleroderma, is an immune-mediated disease and the most common form of scleroderma in children. It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment. MATERIALS AND METHODS: The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups. RESULTS: The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% (n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%. CONCLUSION: In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients. Key points • Most of the pediatric morphea patients respond well to MTX. • Multiple involvement, and bilateral involvement, were more common in relapsed patients than in non-relapsed patients. • Presence of extracutaneous findings in patients increased relapse rate 5.7 times.

Zinc (Zn) and adipose-derived mesenchymal stem cells (AD-MSCs) on MPTP-induced Parkinson's disease model: A comparative evaluation of behavioral and immunohistochemical results.

Parkinson's disease (PD) is characterized by the loss of dopaminergic neurons and sustained neuroinflammation due to microglial activation. Adipose tissue-derived mesenchymal stem cells (AD-MSCs) secrete neuroprotective factors to prevent neuronal damage. Furthermore, Zn regulates stem cell proliferation and differentiation and has immunomodulatory functions. Our in vivo study aimed to investigate whether Zn affects the activities of AD-MSCs in the MPTP-induced mouse model. Male C57BL/6 mice were randomly divided into six groups (n = 6): Control, Zn, PD, PD+Zn, PD+ (AD-MSC), PD+ (AD-MSC)+Zn. MPTP toxin (20 mg/kg) was dissolved in saline and intraperitoneally injected into experimental groups for two days with 12 h intervals. On the 3rd day, AD-MSCs were given to the right lateral ventricle of the PD+ (AD-MSC) and PD+ (AD-MSC)+Zn groups by stereotaxic surgery. Then, ZnSO4H2O was administered intraperitoneally for 4 days at 2 mg/kg. Seven days post MPTP injection, the motor activities of the mouse were evaluated. Then immunohistochemical analyzes were performed in SNpc. Our results showed that motor activity was lower in Group PD. AD-MSC and Zn administration have improved this impairment. MPTP caused a decrease in TH and BDNF expressions in dopaminergic neurons in Group PD. However, TH and BDNF expressions were more intense in the other groups. MCP-1, TGF-ß, and IL-10 expressions increased in administered groups compared to the Group PD. The present study indicates that Zn's individual and combined administration with AD-MSCs reduces neuronal damage in the MPTP-induced mouse model. In addition, anti-inflammatory responses that emerge with Zn and AD-MSCs may have a neuroprotective effect.