The Journal of Pediatric Academy最新文献

{"title":"The Association of Malignancies with The Clinical Profile of Children with Neurofibromatosis Type 1","authors":"Nihal Şahin, U. Demirsoy, F. Çorapçıoğlu","doi":"10.51271/jpea-2022-170","DOIUrl":"https://doi.org/10.51271/jpea-2022-170","url":null,"abstract":"Neurofibromatosis type 1 (NF-1) is a significant autosomal dominant disorder with a wide spectrum of clinical findings. These signs (Café au lait spots, bone dysplasia, Lisch nodules) usually start to emerge after the first months of life and most are benign in nature. On the other hand, neoplasms (optic glioma, neurofibroma, malignant peripheral nerve sheath tumor, soft tissue sarcoma, leukemia, breast cancer) are a major cause of morbidity and mortality in NF-1 patients. Cancer risk during the lifetime of an NF-1 patient is almost 10 times more than a person without NF-1, but what drives these patients into cancer is still unknown. \u0000This study aims to analyze the possible association of clinical findings with malignancies in children with NF-1. \u0000Medical records of 55 children with NF-1 who were followed up in a tertiary care pediatric oncology clinic between January 2005 and December 2014 were analyzed. We assessed clinical and demographic characteristics of patients, as well as the NF-1 diagnostic criteria, NF-1 related complications, and malignancies. The NF-1 patients without malignancy were classified in Group 1 while patients with malignancy were in Group 2. Logistic regression analysis was used to determine the risk factors of malignancy in NF-1. \u0000The mean age was 7.68 ± 4.65 years. Female sex was dominant in both groups. Café au lait spots were present in all patients. Axillary-inguinal freckling was observed in 76.4% of patients, followed by neurofibromas in 30.9%, Lisch nodules in 29.1%, bone dysplasia in 14.5%, optic gliomas in 23.6%, and a history of first degree relative with NF-1 in 63.6%. Central nervous system (CNS) tumors were present in 40%. Tumors other than CNS tumors were acute myeloid leukemia and schwannoma. None of the diagnostic criteria was a risk factor for predisposing to malignancy by itself. Having >3 criteria was found to be the risk factor for malignancy in NF-1 (OR:5.891, CI 95%: 1.676-20.705, p=0.006). \u0000There are no clearly defined risk factors predicting occurance of malignancies in NF-1 at present. However, we found a higher risk of malignancy association in patients who meet more than 3 diagnostic criteria of NF-1.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"214 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116976573","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bacteriological Profile and Antimicrobial Resistance Pattern Among Healthcare-Associated Infections in a Pediatric Intensive Care Unit","authors":"B. Cetin, A. Şahin, C. A. Kürkcü, Fatma Küçük, P. Sağıroğlu, B. Akyıldız","doi":"10.51271/jpea-2022-189","DOIUrl":"https://doi.org/10.51271/jpea-2022-189","url":null,"abstract":"Healthcare-associated infections (HAIs) are a global public health issue with clinical and socioeconomic consequences. These infections are important indices for the quality of healthcare services which are serious complications that should be addressed in pediatric intensive care units (PICUs). This study aimed to retrospectively examine the bacterial HAIs, the frequency of isolated pathogen microorganisms, the areas of infection, and the antibiotic susceptibility recorded in the surveillance system in our Pediatric Intensive Care Unit for five years between 01.01.2015 and 31.12.2019. Two thousand five hundred forty-five patients were admitted to the pediatric intensive care unit during the study period and treated. One-hundred ninety HAIs were detected in 99 patients. In the study, gram-negative microorganisms were most commonly seen (160/190). Pseudomonas aeruginosa, Acinetobacter baumannii, and Klebsiella spp. were the most common bacteria. Enterococcus spp. and coagulase-negative staphylococci were the most common gram-positive microorganisms. The mortality rate of a bacterial HAI was 40.4%. There was no resistance against vancomycin in Gram-positive microorganisms. The resistance rate against methicillin was 100% in coagulase-negative staphylococci and 50% in S. aureus strains. The cumulative rate of carbapenem resistance was found as 76.1% in Pseudomonas aeruginosa, 45.2% in Klebsiella spp. and 0% in Escherichia coli. In 2019, the resistance rate against colistin in Klebsiella spp. and Pseudomonas aeruginosa were 46.2% (6/13) and 20% (1/5), respectively. The resistance rate against carbapenem and colistin was 81.1% and 0% in Acinetobacter baumannii. It was observed that the use of carbapenem before an infection episode increased significantly, and the rate of carbapenem resistance reached 100% over the years in Pseudomonas aeruginosa and Klebsiella spp. A significant proportion of the isolates were multidrug-resistant strains, significantly threatening survival. Implementation of effective preventive strategies to combat the emergence of antibiotic resistance is urgently needed.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"22 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115421530","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erciyes Clinical Guideline For Multisystem Inflammatory Syndrome In Children (MIS-C) Associated With COVID-19","authors":"B. Cetin, A. Kısaarslan, A. Baykan, B. Akyıldız, H. Poyrazoğlu","doi":"10.51271/jpea-2022-182","DOIUrl":"https://doi.org/10.51271/jpea-2022-182","url":null,"abstract":"Multisystem Inflammatory Syndrome in Children (MIS-C) is an inflammatory response to prior SARS-CoV-2 infection. Clinical features of MIS-C could resemble those seen in other diseases, including Kawasaki Disease, Hemophagocytic Lymphohistiocytosis, and cardiovascular shock. The pathogenesis is unclear; however, it is thought to develop 4 to 6 weeks after infection. This guideline aims to provide a framework for physicians to use to guide the evaluation of patients and management of those diagnosed with MIS-C.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"143 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122915910","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Effect of Vitamin D Administration on Androgen Levels in Addition to Metformin Treatment in Adolescent Girls with Hyperandrogenism","authors":"Esranur Çiğ, M. Atar, O. Pirgon","doi":"10.51271/jpea-2022-162","DOIUrl":"https://doi.org/10.51271/jpea-2022-162","url":null,"abstract":"This retrospective study was planned to examine the relationship between glucose metabolism, androgen and lipid profiles after vitamin D and metformin treatments in adolescent girls with hyperandrogenism. Forty-five adolescent female patients diagnosed with hyperandrogenism were divided into three groups. The first group of patients was given metformin (M), the second group was given metformin and vitamin D drops (MdD), and the third group were those who received oral metformin and vitamin D ampoules (MsD). Biochemical and hormonal parameters at the end of 8 weeks were compared statistically. The vitamin D level was higher in the metformin-vitamin D stoss treated group than metformin-vitamin D drops. There was a positive correlation between vitamin D and SHBG in the metformin group (r =0.65, p<0.01). A significant correlation was observed between triglyceride and insulin in both groups given vitamin D, and there was a decrease in these two values (p<0.05). There was a positive correlation between total testosterone and Alanine transaminase (ALT) in the MsD group (p<0.05). Additionally, a positive correlation was observed between SHBG and HDL-cholesterol in three groups. There was a significant correlation between androgen and lipid parameters in the 8-week metformin and vitamin D treated groups. Long-term studies using high-dose vitamin D are needed to support our results.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"78 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124549766","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Musculoskeletal Complaints: When Should We Consult a Pediatric Rheumatologist?","authors":"H. E. Sönmez, Ş. Karadağ, N. Aktay Ayaz","doi":"10.51271/jpea-2022-152","DOIUrl":"https://doi.org/10.51271/jpea-2022-152","url":null,"abstract":"  \u0000This review aimed to summarize key points that might suggest rheumatologic diseases to physicians dealing with musculoskeletal  (MSK) complaints. Evaluation of a child presenting with MSK findings requires a comprehensive, multidisciplinary, and systematic approach. In children with MSK complaints, detailed anamnesis, appropriate physical examination and joint examination, and the use of correct laboratory tests will be helpful for accurate diagnosis. The algorithm we have suggested for MSK complaints of children will be a guide for the physicians.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"41 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116333914","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The importance of immature granulocyte and immature reticulocyte fraction for the severity of acute bronchiolitis","authors":"M. Dogan, M. A. Öztürk","doi":"10.51271/jpea-2022-153","DOIUrl":"https://doi.org/10.51271/jpea-2022-153","url":null,"abstract":"The immature granulocyte (IG) and immature reticulocyte fraction (IRF) are new analytical parameters of the complete blood count, that have been studied as biomarkers of several inflammatory conditions. Here, our aim is to determine the effectiveness of IG and IRF percentages for the severity of acute bronchiolitis (AB). A single-center, prospective study was performed in patients who were hospitalized for acute bronchiolitis and healthy children were included as a control group. The demographic characteristics, white blood cell (WBC) count, platelet (PLT) count, eosinophil%, IG%, and IRF% values were analysed. Receiver operating characteristics (ROC) analysis was used to compare the diagnostic accuracies and predictive performances. We enrolled 168 infants in the acute bronchiolitis group and 80 in the control group. The Clinical Severity Score (CSS) showed that 48, 93, and 27 patients had mild, moderate, and severe bronchiolitis, respectively. The WBC, PLT, and IRF value were significantly higher in patients (p < 0.001). There was no difference between the patients and control group in terms of IG and eosinophil percentage. Only a positive correlation was observed between the clinical severity of the AB and IRF (p=0.003).The ROC curve analysis indicated that the IRF% cut-off point for predicting severity AB was >12.4, with a sensitivity of 53% and specificity of 88% (Areas under the curves (AUC):0.701,p<0.001). The WBC count, PLT count, and IRF value increased in the AB group. The IRF percentage can be used to predict the clinical severity of AB in children.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"3412 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127503708","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Can Point Shear Wave Elastography be Used as an Indicator of Metabolic Complications in Overweight Children and Adolescents? Evaluation of Subcutaneous Adipose Tissue","authors":"Z. Karaman, N. Hatipoğlu","doi":"10.51271/jpea-2022-168","DOIUrl":"https://doi.org/10.51271/jpea-2022-168","url":null,"abstract":"This study aims to search the association of obesity, metabolic parameters, and abdominal subcutaneous white adipose tissue (scWAT) stiffness in children and adolescents using ultrasound point shear wave elastography (p-SWE). One hundred and forty overweight or obese children referred to as overweight were included in the study group. Thirty-two lean children, referred to as leans, were included in the control group. In all individuals, scWAT shear wave speed (SWS) was measured with p-SWE.  ScWAT stiffness was compared between the two groups. The association of anthropometric, metabolic factors and scWAT stiffness is determined. Weight, body mass index, body mass index-standard deviation score, systolic blood pressure, diastolic blood pressure, alanine aminotransferase, fasting insulin were significantly higher in the overweight group (p<0,05). ScWAT SWS was significantly different between the groups (p=0.006) The median value of scWAT SWS was 1,5 m/s (range; 0.9-3.8), 1.23 m/s (range; 0.7-3.1) for leans and overweight, respectively. In leans, no significant difference was found between boys and girls for scWAT SWS (p=0.094). In overweight, a significant difference was found for scWAT SWS between boys and girls (p=0.022). The scWAT stiffness is lower in overweight than leans. Gender has a pivotal role in scWAT stiffness. If supported with future long-time follow-up studies, p-SWE may be compatible with assessing subcutaneous adipose tissue changes related to obesity and metabolic complications in childhood and adolescence.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"11 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130670326","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of Adolescents with Restless Legs Syndrome: Relation to School Success","authors":"Neslihan Günay, Sevda İsmailoğluları, Esra Özdemir Demirci, F. Kardaş, H. Per","doi":"10.51271/jpea-2021-0133","DOIUrl":"https://doi.org/10.51271/jpea-2021-0133","url":null,"abstract":"Restless Legs Syndrome (RLS), which may be a missed diagnosis in children, is seen at an important frequency. The aim of this study was to investigate the clinical characteristics of patients with RLS and to show the effect of RLS on school success. Forty-three patients with RLS and 43 healthy volunteers were included to the study. Blood samples were taken to measure ferritin. The averages of school exam scores were recorded. The patient group was classified as mild, moderate, severe, and very severe according to the RLS rating scale. The daytime sleepiness was measured by the Epworth Sleepiness Scale (ESS). The\"Turgay DSM-IV-Based Child and Adolescent Disruptive Behavioral Disorders Screening and Rating Scale (T-DSM-IV-S)\" was completed by parents, and patients' inattention, and hyperactivity-impulsivity scores were compared with the control group. According to the RLS rating scale scores 25.6% (n:11) were evaluated as mild, 60.5% (n:26) were moderate and 14% (n:6) were severe. The mean ferritin level was significantly lower in the patient group. The mean score of inattention and hyperactivity-impulsivity in the patient group to be significantly higher than the control. The mathematics and science course mean grades were significantly lower in the patient group than the controls (66.7±17.7 vs 74.2±11.7). ESS scores were found to be significantly higher in the patient group. In this study, RLS has been found associated with ADHD and iron deficiency, similar to previous studies. There are not many studies on the effects of RLS in children on daily life and this study has objectively shown that RLS reduces patients' school achievement","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"18 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126187614","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Postinfectious Bronchiolitis Obliterans","authors":"A. Yılmaz, S. Pekcan","doi":"10.51271/jpea-2021-0137","DOIUrl":"https://doi.org/10.51271/jpea-2021-0137","url":null,"abstract":"Childhood bronchiolitis obliterans (BO) is an uncommon complication that is characterized clinically by persistent and continuous obstructive respiratory symptoms, and has been described secondary to various etiologic factors, including drugs, exposure to toxic fumes, allergic reactions, collagen vascular disease or infections. BO occurs most commonly in children after an episode of acute bronchiolitis and is considered a long-term sequela of viral infection.Postinfectious Bronchiolitis Obliterans (PIBO) is characterized by persistent airway obstruction with functional and radiological evidence of small airway involvement that is in general unresponsive to bronchodilator treatment.Although the condition is relatively rare, and its exact incidence is unknown, it is important to keep it in mind. PIBO is complication of lower respiratory tract epithelial injury, and is often misdiagnosed, delaying recognition and potential treatment. A PIBO diagnosis is usually based on a few factors, including a good medical history, positive clinical findings, and lung function test and imaging results, although biopsy and histopathology remain as the optimum diagnostic approach. There have to date been few studies proposing treatments for the condition, and no accepted protocol exists in literature. There is usually a fixed airway obstruction in PIBO. Various treatment approaches have been extrapolated from studies of post bone marrow transplantations and lung transplant BO. The clinical course is variable, and good supportive therapy is essential, with anti-inflammatory therapy often being employed.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"11 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127020468","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of Clinical and Follow-up Results of Patients with Congenital Nephrotic Syndrome","authors":"H. Nalcacioglu, D. Tekcan, H. G. Önal, Özlem Aydoğ","doi":"10.51271/jpea-2021-0142","DOIUrl":"https://doi.org/10.51271/jpea-2021-0142","url":null,"abstract":"Congenital nephrotic syndrome (CNS) is characterized by severe proteinuria, hypoalbuminemia, and edema within the first three months of life. Congenital nephrotic syndrome can occur due to perinatal infections or mutation of genes encoding structural or regulatory proteins of the glomerular filtration barrier. Treatment includes albumin infusions, nephrectomy, dialysis, and transplantation. In this study, we aimed to evaluate the demographic, clinical, and follow-up results of patients with CNS followed up in our center between 2010 and 2020. Demographic, clinical, laboratory values of 8 patients diagnosed with CNS between 2010 and 2020, kidney biopsy results, genetic examinations, and follow-up results were retrospectively evaluated. A total of 8 patients (4 girls) were included in this study. The median age at diagnosis was 36 days (3 days-8 months) and the follow-up period was 34 months (7-114 months). There was a history of prematurity and consanguinity in 5 patients. Edema was detected at the admission of all patients. Albumin infusion and captopril therapy were started from the diagnosis. No pathology was seen in the tests for perinatal infection, and ultrasonographic examinations were normal. In the genetic analysis, NPHS1 (Nephrin) homozygous mutation was detected in six patients, and coenzyme Q2 mutation was detected in one patient. Peritoneal dialysis treatment was performed in four patients during the follow-up, and unilateral nephrectomy was completed in one patient. During the follow-up, four of eight patients (three due to sepsis while on dialysis, one on the postoperative after the first day of transplantation) died. Three patients are followed up with kidney transplantation and one with supportive treatment. According to our results, most CNS cases are genetic, and nephrin mutation is the most common cause. Management of complications in CNS is crucial for patient surviva","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"387 ","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133621986","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}