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Aim: This study aimed to present the demographic, clinical, and laboratory features of children clinically diagnosed with familial Mediterranean fever (FMF) and to predict more severe mutations by evaluating those findings. Methods: We enrolled cases diagnosed with FMF with a defined variation in at least one allele. The medical charts of the patients were reviewed retrospectively. The patients were grouped as homozygous, compound heterozygous, and simple heterozygous cases, with and without M694V mutation. We compared the data between the subgroups using logistic regression analysis and determined the risk factors for being homozygous or compound heterozygous for M694V. Results: A total of 263 (M/F =109/154) cases were included. The mean age at the onset of symptoms, follow-up duration, and time to diagnosis were 6.75 ± 3.9 (0.25-17) years, 51.78 ± 39.31 (6-166) months, and 9.23 ± 14.44 (1-132) months, respectively. The rates of parental consanguinity, positive family history for FMF, and FMF in a first-degree relative were 15%, 42%, and 31.4% respectively. The most common symptom was abdominal pain (85%). There was no difference between the growth parameters of the cases during the initial and final control periods. The most frequent alleles were M694V, E148Q, and V726A. The most common accompanying disease was IgA vasculitis (20%). Almost 90% of the cases fulfilled all the defined criteria. The rate of patients having a first-degree relative with FMF was higher, Hb values were lower, and the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values were higher during the attack period; the ESR and CRP values were higher in the attack-free period; and Pras disease severity scores were higher in homozygous or compound heterozygous cases carrying M694V. The presence of FMF in a first-degree relative increases the probability of being homozygous and compound heterozygous for M694V by a factor of 2.39; and each 1 unit increase in the Pras score increases this probability by a factor of 1.43. The threshold Pras score for this possibility is 5.5 (AUC = 0.651; 95% CI, 0.545-0.757; P = .006; sensitivity, 65%; specificity, 55%). Conclusion: M694V was the most common and severe mutation in our cohort. The presence of a first-degree relative with FMF and Pras scores >5.5 may predict a homozygous or compound heterozygous mutation for M694V. [ABSTRACT FROM AUTHOR]

Aim: Enuresis (EN) is a common problem in childhood. Family physicians have an important role in revealing children with EN. The aim of this study was to assess the knowledge and attitude of family medicine (FM) residents regarding the diagnosis and treatment of enuresis in children. Materials and Methods: Family medicine residents of the University of Health Sciences, İzmir Tepecik Training and Research Hospital were invited to complete a questionnaire concerning enuresis in children. Those who had completed their paediatrics rotation training were defined as group 1, and those who had not completed their paediatrics rotation as group 2. The responses were compared between the groups. Results: Sixty (88%) of the FM residents agreed to complete the survey. The mean age of the participants was 28.7 (25-35) years, 38 (63%) had completed their paediatrics rotation (group 1). The question about the age of night-time bedwetting was more often answered correctly in group 1 (19/38) (p=0.025). In the question on the symptoms of non-monosymptomatic enuresis; the constipation option was marked as a symptom by only 15 of the participants (25%), with 13 (87%) being in group 1 (p=0.03). The correct response rates to the questions about other symptoms of non-monosymptomatic enuresis, the causes of secondary enuresis, the need for treatment, treatment options and the follow-up of patients with enuresis were similar between groups 1 and 2. Conclusion: As a result of this study, more correct answers, which revealed adequate knowledge and experience regarding enuresis, were obtained from those who had concluded their paediatrics rotation. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Tepecik Education & Research Hospital / İzmir Tepecik Eğitim ve Araştırma Hastanesi Dergisi is the property of Logos Medical Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Current Pediatrics / Guncel Pediatri is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Tepecik Education & Research Hospital / İzmir Tepecik Eğitim ve Araştırma Hastanesi Dergisi is the property of Logos Medical Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Polyarteritis nodosa (PAN) is a necrotizing systemic vasculitis involving the wall of small and medium sized arteries. The histological aspect is defined by the presence of fibrinoid necrosis and an infiltrate rich in neutrophil polynuclears in the artery wall and rare granulomas. CECR1 (Cat Eye Syndrome Chromosome Region 1) gene mutation causing adenosine deaminase 2 (ADA2) deficiency is also associated with systemic inflammation, vasculopathy, and frequently PAN. Strokes, neurological involvement, and gastrointestinal involvement have poor prognosis in the cases with ADA2 deficiency particularly in early stage. In this article, we report a 17-year-old male patient diagnosed with PAN who had severe gastrointestinal system involvement that was resistant to intensive and conventional immunosuppressive treatment and showed a fatal course despite the emergency surgical intervention. After the patient was exitus, he was detected to have a heterozygous mutation (V276A) of familial Mediterranean fever (FMF) and also a homozygous ADA2 mutation. The aim of this article is to highlight that ADA2 deficiency may be present in treatment-resistant PAN cases who apply due to severe systemic involvement. In this case, accompanying FMF mutation was also observed. [ABSTRACT FROM AUTHOR]

Hafnia alvei is a rare bacterium that is generally reported to be an opportunistic infectious agent in adults. There are a limited number of identified pediatric cases in the literature. This article reports H. alvei as the causative agent of urinary tract infection in a healthy 8-year-old girl with no underlying disease. The patient recovered with a 10-day oral trimethoprim/sulfamethoxazole treatment. The aim of this study was to review the characteristics of this rare microorganism, as a causative agent of urinary tract infection, for clinicians. [ABSTRACT FROM AUTHOR]

Copyright of Turkish Nephrology, Dialysis & Transplantation Journal is the property of Turkish Society of Nephrology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Turkish Nephrology, Dialysis & Transplantation Journal is the property of Turkish Society of Nephrology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: The two strongest earthquakes in Turkey for eight decades hit Kahramanmaraş province on February 6, 2023. This study aimed to determine psychiatric complaints, acute stress symptoms, anxiety, depression, and sleep characteristics in children who were treated in a tertiary inpatient pediatric unit after the earthquakes. They were evaluated in the fourth week after the earthquake., Methods: Trauma experiences and acute stress disorders were evaluated by child and adolescent psychiatrists, sleep characteristics were examined by the Child Sleep Habits Questionnaire (CSHQ), and the anxiety and depressive symptoms were investigated using the Revised Child Anxiety and Depression Scale (RCADS). Forty-two patients aged 1-17 were included., Results: Trauma experiences and acute stress disorder symptoms were similar for all age groups examined. There were no significant differences in RCADS or CSHQ scores based on age or gender groups. There were significant differences in RCADS scores when the children were grouped as having parental loss or not and, similarly, there were significant differences in sleep anxiety and resistance to bedtime in CSHQ between these children., Conclusion: This research offered baseline findings about psychological outcomes after these earthquakes in child survivors, and it may provide a further basis to assess the effect of psychological interventions after earthquakes. These results may offer a foundation for suitable psychological interventions for children in developing countries., (© 2025 Japan Pediatric Society.)