{"title":"Gastroenteropancreatic neuroendocrine tumors in children and adolescents","authors":"Ülkü Miray Yıldırım, Dilşad Koca, R. Kebudi","doi":"10.24953/turkjpediatr.2024.4526","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2024.4526","url":null,"abstract":"Background. Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare in children and adolescents. Standard management of these tumors has not been well established due to their rarity in this age group. We aimed to report the clinical and pathological characteristics of patients with this rare disease followed and treated between the years 1993-2022.\u0000Materials and methods. The medical records of patients with GEP-NETs were reviewed.\u0000Results. Fourteen patients (11 girls, 3 boys) were diagnosed with GEP-NET. The median age was 13 (9-18) years. Tumor localization was the appendix in 12, stomach in one and pancreas in one patient. Mesoappendix invasion was detected in four patients two of whom underwent right hemicolectomy (RHC) and lymph node dissection (LND). Of those, one patient had lymph node involvement. The other two had not further operations. Somatostatin was used in one with pancreatic metastatic disease and the other with gastric disease after surgery. No additional treatment was given in other patients. All patients are under follow-up without evidence of disease at a median follow-up of 85 months (7-226 months).\u0000Conclusion. GEP-NETs should be considered in the differential diagnosis of acute appendicitis and in cases with persistent abdominal pain. In children, there is invariably a favorable prognosis, and additional surgical interventions other than simple appendectomies generally do not provide benefits. Mesoappendix invasion may not necessitate RHC and LND.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":" 752","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141669371","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}
Emel Ömercioğlu, Ece Naz, Mert Karakaya, Gökçenur Özdemir, Buse Şencan Karakuş, Şeyma Kılınç, H. C. İskender, Ebru Cihan Çam, Ayşe Mete, H. Çelik, S. Karahan, Elif N. Özmert
{"title":"Has the COVID-19 pandemic negatively impacted children’s development? An assessment of the neurodevelopment of premature babies born during the pandemic","authors":"Emel Ömercioğlu, Ece Naz, Mert Karakaya, Gökçenur Özdemir, Buse Şencan Karakuş, Şeyma Kılınç, H. C. İskender, Ebru Cihan Çam, Ayşe Mete, H. Çelik, S. Karahan, Elif N. Özmert","doi":"10.24953/turkjpediatr.2024.4551","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2024.4551","url":null,"abstract":"Background. Pandemics, such as COVID-19, have the potential to adversely affect children’s development due to a variety of negative factors at the level of children, families, and services. In this study the effect of the pandemic on the cognitive, language and motor development of premature babies who are among the most vulnerable group, were evaluated.\u0000Methods. The study included 236 premature infants who were followed at Hacettepe University Department of Developmental Pediatrics. The Bayley-Third Edition Developmental Assessment (Bayley III) was used to evaluate the neurodevelopment of 152 premature infants from the pre-pandemic group and 84 from the post-pandemic group at the corrected age of 18–24 months. The perinatal and sociodemographic risks were also evaluated.\u0000Results. No difference in Bayley III scores (cognitive, language, and motor) was found between the pre- and post-pandemic groups. Furthermore, the multivariate covariance analysis displayed that regardless of the pandemic, infants with higher maternal education consistently scored higher in the cognitive, language, and motor domains; and the motor area scores of infants with moderate perinatal risk were also significantly higher than infants with high perinatal risk.\u0000Conclusions. It is crucial to monitor the development of vulnerable children who encounter developmental risks, such as premature babies. Fortunately, no significant effect was encountered during the COVID-19 pandemic. However, this does not underweigh the need for close supervision in extraordinary circumstances. Additionally, it should be noted that severe postnatal comorbidities, perinatal risks, and social factors, such as maternal education level, interact to influence the neurodevelopmental outcomes of preterm infants.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"73 S7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141682367","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":"Prevalence of premenstrual syndrome in adolescent girls","authors":"Özlem Akbulut, Laden Jafari, Demet Aygün Arı, Melis Pehlivantürk Kızılkan, O. Derman, Sinem Akgül","doi":"10.24953/turkjpediatr.2024.4669","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2024.4669","url":null,"abstract":"Background. Premenstrual syndrome (PMS) is characterized by physical, cognitive, emotional, and behavioral symptoms that appear during the luteal phase of the menstrual cycle, disappear after menstruation, and are recurrent in every cycle. PMS significantly affects the social and academic lives of adolescents, and historically, it has been neglected by healthcare professionals. We aimed to evaluate the current point prevalence of PMS in Turkish adolescents presented to a tertiary adolescent medicine clinic.\u0000Material and Method. Adolescent girls between the ages of 12 and 18 and who had regular menstrual cycles for at least three months without any mental or chronic illness were assessed. A clinic information form and the ‘Premenstrual Syndrome Scale’ (PMSS) questionnaire were completed. Those with a PMSS total score of more than 50% of the total score (>110 out of 220) were classified as PMS (+). Those classified as PMS were further classified as mild-moderate (score: 110-150) and severe (>150).\u0000Results. The study included 417 adolescents. The point prevalence of PMS was found to be 61.2% (n:255). Of those with PMS, 49.4% had mild-moderate and 50.6% had severe PMS. The mean PMSS score was 154.56 ± 30.43 in the PMS group and 76.17 ± 20.65 in the non-PMS group (p<0.001). The mean age was 15.41 ± 1.3 years in the PMS group and 14.88 ± 1.35 years in the non-PMS group (p=0.029). None of the youth in our study applied to our clinic due to any premenstrual complaints.\u0000Conclusion. PMS is frequently observed in youth, as indicated by our study. Adolescents have little awareness of PMS and their need for healthcare services. During the evaluation of adolescents, it is important for health care providers to acquire knowledge regarding the features of menstrual cycles and conduct a comprehensive psychosocial assessment.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":" 17","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141680773","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":"Severe myxedema coma and pericardial effusion in a child with Down syndrome: the importance of adherence to levothyroxine therapy","authors":"H. Arı, A. Anık, Şule Demir, Serkan Fazlı Çelik","doi":"10.24953/turkjpediatr.2024.4587","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2024.4587","url":null,"abstract":"Background. Myxedema coma is a rare, but life-threatening endocrinological emergency. Myxedema is characterized by altered mental status, and is accompanied by hypotension, bradycardia, hypothermia, bradypnea, hyporeflexia, hyponatremia, and hypoglycemia, all stemming from reduced metabolism due to severe hypothyroidism. Additionally, patients may exhibit signs of low cardiac output, edema in the extremities, peripheral circulatory disturbances, shock, and the development of pericardial and pleural effusions, ultimately leading to confusion and coma. We present a successfully treated case of severe myxedema coma with recurrent pericardial effusion and hypotensive shock. This case is characterized by an unusual clinical presentation and required a distinct treatment strategy highlighting its exceptional rarity.\u0000Case. A 2-year-old boy with Down syndrome presented with recurrent pericardial effusion attributed to medication non-adherence. The critically-ill patient, experiencing a severe cardiogenic shock required mechanical ventilation and inotropic infusions in the pediatric intensive care unit. Elevated thyroid stimulating hormone (TSH), and low free T4 (fT4) and free T3 (fT3) levels prompted consideration of myxedema coma. Upon reviewing the patient’s medical history, it was ascertained that he had an ongoing diagnosis of primary hypothyroidism, and exhibited non-adherence to the prescribed treatment regimen and failed to attend scheduled outpatient clinic appointments for follow-up assessments. The treatment plan, devised by the pediatric endocrinology team, included the peroral administration of L-thyroxine (L-T4) at a dose of 50 micrograms per day. After beginning regular oral L-T4 treatment, a gradual improvement in the patient’s condition was observed. Notably, by the 15th day of oral therapy, the patient had made a full recovery. Contrary to the recommended intravenous treatment for myxedema coma, this patient was successfully treated with oral levothyroxine, due to the unavailability of the parenteral form in Türkiye.\u0000Conclusions. This case report presents an instance of non-adherence to L-T4 therapy, which subsequently progressed to severe myxedema coma. Changes in neurologic status and hemodynamic instability in a patient with a history of hypothyroidism should raise the concern of nonadherence and, though rare, myxedema coma should be in the differential diagnosis.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"50 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141683232","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}
Çağrı Coşkun, A. Varan, N. Kurucu, B. Aydın, U. Özçelik, İnci Yaman Bajin, Eren Müngen, Ebru Yalçın, Diclehan Orhan, B. Oğuz, B. Yalçın, T. Kutluk, M. Haliloğlu
{"title":"Pulmonary involvement in children with Langerhans cell histiocytosis","authors":"Çağrı Coşkun, A. Varan, N. Kurucu, B. Aydın, U. Özçelik, İnci Yaman Bajin, Eren Müngen, Ebru Yalçın, Diclehan Orhan, B. Oğuz, B. Yalçın, T. Kutluk, M. Haliloğlu","doi":"10.24953/turkjpediatr.2024.4515","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2024.4515","url":null,"abstract":"Background. Pulmonary Langerhans cell histiocytosis (pLCH) is a rare disease, mostly a component of multisystemic LCH. We aimed to investigate the clinical features and treatment results in children with pLCH.\u0000Methods. We retrospectively reviewed the clinical, radiological, and treatment data of 37 patients with pLCH, diagnosed from 1974 to 2022.\u0000Results. 10% (n=37) of 367 patients with LCH had lung involvement. The median age was 1.8 years (range: 0.4 & 17.7) with a male-to-female ratio of 2.3. At admission 29.7% (n=11) presented with respiratory symptoms. Imaging showed a spectrum from nodular opacities to multiple cysts. All but one patient had multisystem disease. Twenty-nine received vinblastine-containing therapy. Ten-year event-free (EFS) and overall survival (OS) rates were 47.8% and 63.3%, respectively. In children younger and older than two years of age, the 10-year EFS was 53.3% vs. 40.2% and the 10-year OS was 58.7% vs. 68.8%, respectively. In children with and without risk organ involvement, 10-year EFS was 51.9% vs. 46.3% and 10-year OS was 51.9% vs. 73.7%.\u0000Conclusions. Lung and multisystem involvement are significant concerns in LCH, highlighting the need for careful management to reduce morbidity and mortality.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"7 20","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141698830","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}
Aysu Kahraman, Ayşe Aksoy, Gökçen Öz Tunçer, S. Erdem, Ayşe Livanelioğlu
{"title":"The role of certain perinatal features in the early motor repertoire of infants","authors":"Aysu Kahraman, Ayşe Aksoy, Gökçen Öz Tunçer, S. Erdem, Ayşe Livanelioğlu","doi":"10.24953/turkjpediatr.2024.4513","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2024.4513","url":null,"abstract":"Background. Lower gestational age negatively affects the neurodevelopmental outcomes of infants. Early motor repertoire is a reliable way to predict neurodevelopmental outcomes. This study aimed to determine the correlation between gestational age and early motor repertoire in infants and also the roles of multiple pregnancies, gender, cranial utrasonography (USG) results, and birth weight in this relationship.\u0000Methods. This study included 139 infants, who were video recorded 9-17 weeks post-term. The recordings were evaluated using the Motor Optimality Score-Revised (MOS-R). Structural equation modeling tool was used for the path analysis of the models.\u0000Results. There was a weak positive correlation between gestational age and the MOS-R. In the relationship between gestational age and the MOS-R, multiple pregnancies, gender, and USG outcomes had a moderating effect. While abnormal USG, male gender, and singleton pregnancy increased this correlation to a moderate level, normal USG reduced the strength of the correlation. Female and twin pregnancies were non-significant in the model. Birth weight had a full mediating effect on the relationship between gestational age and the MOS-R.\u0000Conclusions. Infants with younger gestational age or lower birth weight, male infants, and infants with problems on cranial USG may have poorer early motor repertoire.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"10 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141379374","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}
Deniz İlgün Gürel, Hilal Ünsal, Elif Soyak Aytekin, Özge Soyer, Ü. Şahiner, S. Ersoy Evans, B. Sekerel
{"title":"The treatment journey of children with moderate to severe atopic dermatitis in Türkiye: unmet needs","authors":"Deniz İlgün Gürel, Hilal Ünsal, Elif Soyak Aytekin, Özge Soyer, Ü. Şahiner, S. Ersoy Evans, B. Sekerel","doi":"10.24953/turkjpediatr.2024.4569","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2024.4569","url":null,"abstract":"Background. Atopic dermatitis (AD) substantially burdens individuals, families, and healthcare systems. We aimed to document the treatment journey of pediatric patients with moderate-to-severe AD in a referral center based in our country.\u0000Methods. This retrospective study reviewed patients aged 1-18 years diagnosed with AD, seeking systemic treatment recommendations from the “pediatric allergy and dermatology multidisciplinary team meeting”.\u0000Results. Over the 14-month study period, 30 (12.5%) of 240 AD patients were evaluated in the pediatric dermato-allergy team meetings. The median age of the patients was 13.66 years (Q1-Q3: 7.94-17.27), of whom 60% were male. The median annual healthcare visits for AD were 4 (Q1-Q3: 1.00-8.75). Among the study group, 70% were sensitized to aeroallergens, and admission markers included total IgE (median: 1980 IU/mL, Q1-Q3: 794.50-5446), and eosinophil counts (median: 650, Q1-Q3: 275-1275). All patients utilized intermittent and/or continuous topical corticosteroids (CS), with 56.6% employing short-term/long-term topical tacrolimus. Over the past two years, systemic CSs were utilized in 93.3% of the patients, whereas 57.1% received more than one course. Approximately 43.3% of the patients agreed to receive systemic cyclosporine treatment, with only 30.8% benefiting and 3.3% reporting adverse effects (hypertrichosis and cellulitis). Three patients self-funded dupilumab, all benefiting without adverse effects. Omalizumab, mycophenolate mofetil and narrow-band ultraviolet (UV) treatments were used in one patient each, with limited benefit observed. Health insurance did not grant approval for a Janus kinase inhibitor for one patient.\u0000Conclusions. Managing moderate to severe AD is complex and costly, considering disease heterogeneity, comorbidities, care pathways, and health system challenges. Addressing the unmet needs should be a priority in Türkiye’s healthcare systems.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"13 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141383593","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":"A pediatric case with hemolytic uremic syndrome associated with COVID-19, which progressed to end-stage kidney disease","authors":"Serra Sürmeli Döven, Esra Danacı Vatansever, Yasemin Yuyucu Karabulut, Berfin Özgökçe Özmen, Fatma Durak, Ali Delibaş","doi":"10.24953/turkjpediatr.2024.4524","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2024.4524","url":null,"abstract":"Background. Hemolytic uremic syndrome (HUS) is a serious cause of acute kidney injury in children. There is a suggestion that coronavirus disease 2019 (COVID-19) may be a trigger for HUS. In this study, we present a pediatric case diagnosed with HUS associated with COVID-19, which progressed to end-stage kidney disease.\u0000Case. A previously healthy 13-year-old girl with fever and vomiting was referred to our hospital. Laboratory investigations revealed direct Coombs-negative hemolytic anemia, thrombocytopenia and renal impairment accompanied by COVID-19 infection. Although anemia and thrombocytopenia showed improvement on the seventh day after admission, the renal impairment persisted. The histopathological findings of a renal biopsy were compatible with both HUS and COVID-19. One month later, the patient had a recurrence of HUS, again testing positive for COVID-19. Kidney function improved with plasma exchange therapy. Eculizumab treatment was recommenced after COVID-19 PCR became negative. Anemia and thrombocytopenia did not recur with eculizumab, while renal impairment persisted. Eculizumab was discontinued after three months when genetic analysis for HUS was negative. Subsequently, the patient was diagnosed with end-stage kidney disease.\u0000Conclusions. COVID-19 can be associated with HUS relapses, leading to chronic kidney disease. Further studies should investigate the mechanism of HUS associated with COVID-19.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"20 15","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141106603","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}