BACKGROUND:Herlyn-Werner-Wunderlich syndrome is a rare congenital urogenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Children usually have progressive pelvic pain after menarche, palpable mass d...

The current study aimed to investigate the clinical, laboratory and radiological findings of the pneumonia cases in children that were confirmed as M.pneumoniae by polymerase chain reaction (PCR) testing and to reveal the factors that can be decisive in the diagnosis. Seventy-seven children were included in this study. The median age of the patients was 31 months (1 month-17 years 4 months). The 63.6% of the patients were younger than five years of age, 53.2% were girls and 46.8% were boys. During the eight-year research period, the frequency of M.pneumoniae in the patients hospitalized with the diagnosis of pneumonia was found to be 3.1%. The rate of M.pneumoniae as the underlying factor of pneumonia was found to be statistically significantly lower in patients aged 0-60 months compared to the patients aged 61-216 months. In patients with M.pneumoniae accompanied by viruses, the age group was more likely to between 0-60 months. The most common symptoms were cough (96.1%) and fever (74%). Physical examinations revealed that 70.1% of the patients had rales, 63.6% had tachypnea, 45.5% had oropharyngeal hyperaemia, 35.1% had subcostal-intercostal retraction, 31.2% had long expiration period, 26% had rhonchus, 24.7% had decrease in breath sounds, 15.6% had cervical lymphadenopathy, 13% had tachycardia, 3.9% had otitis media, 3.9% had tonsil hypertrophy and 2.6% had a maculopapular rash. The rate of hypoxemia was found to be 42.2%. When the physical examination findings of patients with only M.pneumoniae detected in multiplex PCR analysis and those with accompanying viruses in M.pneumoniae were compared, tachypnea, oropharyngeal hyperemia and decreased breath sounds were found to be statistically significantly higher in patients with M.pneumoniae only. Retraction was detected more frequently in patients with accompanying viruses. When the laboratory results of the patients were evaluated according to age, leukocytosis was detected in only 18.2% of the patients, while the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were found to be high in 75% and 85.7% of the patients, respectively. In the multiplex PCR analysis, the CRP values of the patients with only M.pneumoniae were found to be higher than the patients with accompanying viruses. M.pneumoniae was accompanied by viruses at the rate of 40.3%. The most common accompanying viruses were rhinovirus, adenovirus, bocavirus and metapneumovirus. The 55.8% of the patients had lobar-segmental consolidation, 46.8% had parahilar-peribronchial thickening, 18.2% had atelectasis, 11.7% had pleural effusion, 9.1% had increase in reticulonodular density, 6.5% had lymphadenopathy whereas no abnormality was observed in 5.2% of them. No diffuse interstitial involvement was recorded. The CRP value of the patients who had lobar segmental consolidation which was detected through chest X-rays were statistically higher than those without consolidation. In multiplex PCR analysis, the rate of parahilar-peribronchial thickening detected in chest X-ray findings was found to be higher in patients with M.pneumoniae accompanied by viruses compared to those with only M.pneumoniae. The rate of the patients who were given empirical antibiotics against atypical agents was 45.5%. The rate of empirically administered antibiotic treatment for atypical agents after being hospitalization was higher in patients diagnosed with only M.pneumoniae compared to patients with M.pneumoniae and viruses. One patient (1.3%) died. As there are no typical clinical, laboratory or radiological findings specific to M.pneumoniae pneumonia, all of the findings should be assessed as a whole to establish a diagnosis. Besides, for the detection of M.pneumoniae, diagnostic tests which are cost effective, with rapid results and are capable of distinguishing colonisation from active infection should be developed.

Objective: Neurofibromatosis type 1 is a most common neurocutaneous syndrome characterized by multi-system involvement and an increased incidence of both benign and malignant tumors. Its diagnosis and follow-up is important because of increased cancer susceptibility in children. The aim of this study is to review the cranial MRI findings, neoplastic and non-neoplastic lesions of the brain in neurofibromatosis type I in pediatric patients. Material and Methods: 183 patients which diagnosed with neurofibromatosis type 1 and had at least 1 MRI between July 2010 and March 2019 were evaluated. Twelve patients were siblings. Contrasted cranial MRI obtained from 82 female and 101 male patients, between ages 1-18 years (averageage 10) retrospectively scanned thereafter type and locations of lesions evaluated by a 9 years experienced radiologist. Results: Out of 183 patients; 37 had no hamartomatous cranial lesions, 24 had optic gliomas or optic nerve thickening (9 bilaterally, 10 rightside, 5 leftside), 8 had plexiform neurofibromas (head or neck location), 9 had subcutaneous neurofibromas. Additionally 9 had intracranial arachnoid cysts, 6 low grade glial tumor, 1 medulloblastoma, 1 glioblastoma, 1 nodular heterotopia. Two patients had suspected low grade glial tumor and their size and view are stableat follow-up. The most common localization of hamartomatous lesions are the globus pallidus, dentate nucleus, mesencephalon, thalamus and pons respectively. The least common area is putamen. In addition, 3 patients are followed with Noonan Syndrome. In two of these patients, intracranial arachnoid cysts were observed in addition to hamartomatous lesions. Conclusion: Neurofibromatosis type 1 is one of the tumor predisposition syndrome that leads to an increased incidence of malign and benign tumors, which are major cause of mortality and morbidity. Gliomas are the most common intracranial tumors and the second most common tumor type after neurofibromas. Regular follow up is important for early diagnosis and treatment of malignancy. Brain MRI is adequate and necessary imaging modality for the diagnosis and follow up of brain involment in Neurofibromatosis type 1 patients.

Background/Objectives: While ancillary tests for brain death diagnosis are not routinely recommended in guidelines, they may be necessary in specific clinical scenarios. Computed tomography angiography (CTA) is particularly advantageous in pediatric patients due to its noninvasive nature, accessibility, and rapid provision of anatomical information. This study aims to assess the diagnostic sensitivity of a revised venous system (ICV-SPV) utilizing a 4-point scoring system in children clinically diagnosed with brain death. Materials and Methods: A total of 43 pediatric patients clinically diagnosed with brain death who underwent CTA were retrospectively analyzed. Imaging was performed using a standardized brain death protocol. Three distinct 4-point scoring systems (A20-V60, A60-V60, ICV-SPV) were utilized to assess vessel opacification in different imaging phases. To evaluate age-dependent sensitivity, patients were categorized into three age groups: 26 days–1 year, 2–6 years, and 6–18 years. The sensitivity of each 4-point scoring system in diagnosing brain death was calculated for all age groups. Results: The revised venous scoring system (ICV-SPV) demonstrated the highest overall sensitivity in confirming brain death across all age groups, significantly outperforming the reference 4-point scoring systems. Furthermore, the ICV-SPV system exhibited the greatest sensitivity in patients with cranial defects. Conclusions: The revised 4-point venous CTA scoring system, which relies on the absence of ICV and SPV opacification, is a reliable tool for confirming cerebral circulatory arrest in pediatric patients with clinical brain death.

Aile hekimliği tıp disiplinin özelliklerini, ilkelerini ve temel değerlerini detaylarıyla bulabileceğiniz bölümlerin yanında, klinik uygulamalarda izlenecek yollar ve kılavuzların eşlik ettiği güncel bilgileri sizlerle paylaşmayı hedefledik. Aile hekimliğinin, yaş, cinsiyet, hastalık ayırt etmeksizin sunduğu sağlık bakımına ek olarak koruyucu sağlık hizmetlerinin prekonsepsiyonel dönemden bireyin ölümü sonrasındaki aile bireylerinin durumuna kadar geniş konu yelpazesinin varlığı bu kitabın kapsamlı ve güncel olması yönünde bize yol haritası olmuştur. Aile hekimliğinin, kendi eğitim içeriği, araştırmaları, kanıta dayalı ve klinik uygulamaları olan akademik ve bilimsel bir disiplin ve birinci basamağa odaklı bir klinik uzmanlık modeli ile konuların bilimsel ve pragmatik düzenlemesi yapılarak akışı sağlanmış, bilgilerin sistematik anlatımı kitap içerisinde planlanmıştır.