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Systemic pseudohypoaldosteronism is a rare salt wasting syndrome that is caused by inactivating variants in genes encoding epithelial sodium channel subunites. Hyponatremia, hyperkalemia, metabolic acidosis, increased aldosteron and renin levels are expected findings of this disease. It is difficult to manage this disease due to high dose oral replacement therapy. Furthermore patients with systemic pseudohypoaldosteronism require life-long therapy. Here we report a patient with systemic PHA due to SCNN1B variant whose hyponatremia and hyperkalemia was detected at the 24th hour of life. Hyperkalemia did not improve with conventional treatments and dialysis was required. Also he developed myocarditis and hypertension in follow-up. Difficulties in diagnosis and treatment of this patient were discussed in this report. Also we review the literature on common features of patients with SCNN1B variant.

Background Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease of childhood. The various subtypes of JIA differ in clinical features and treatments. The aim of this study was to analyze the frequency of JIA subtypes, patient demographic and clinical features, as well as the rates of macrophage activation syndrome, uveitis, and remission in Turkish JIA patients treated at a single center, and to compare the findings to those in the literature. Materials and methods The files of all JIA patients treated at our pediatric rheumatology department between January 2017 and January 2019 were retrospectively reviewed. Patient demographic, clinical, and laboratory data were obtained from the patients' files and hospital database. Results The study included 305 patients (180 females) with a mean age at onset of 7.83 ± 4.62 years. Among all the JIA subtypes, the most frequent was oligoarthritis (41.6%), followed by enthesitis-related arthritis (29.2%), rheumatoid factor (RF)-negative polyarthritis (13.4%) systemic arthritis (9.5%), RF-positive polyarthritis (2.6%), psoriatic arthritis (2.0%), and undifferentiated arthritis (1.6%). At the time of data collection 278 patients (91.0%) were in remission, whereas 27 patients (9.0%) had active disease. Macrophage activation syndrome developed in 12 of the 29 (41.0%) systemic arthritis. Uveitis was noted in 32 (10.0%) patients. Biological agents were administered in 142 of the patients. Conclusion The available data indicate that JIA as a whole is a heterogeneous disease with significant variability in course and long-term outcome. As such, each patient should be evaluated according to his/her disease subtype.

Hypomagnesemia is a rare cause of seizures in childhood but should be kept in mind in recurrent and intractable seizures and hypocalcemia in communities where consanguineous marriages are common. Familial hypomagnesemia with secondary hypocalcemia is a rare genetic cause of hypomagnesemia, due to variants in the transient receptor potential melastatin 6 (TRPM6) genes. Here, a three year-old boy with a novel variant in this gene and had difficulties with enteral hypomagnesemia treatment is presented. He had recurrent seizures since two years of age and was diagnosed with epilepsy and treated with multiple antiepileptic drugs. Subsequently, he was diagnosed with rickets due to severe hypocalcemia at another center. The patient was hypotonic and neurodevelopmentally poor. The most prominent laboratory finding was of hypomagnesemia with secondary hypocalcemia. The genetic analysis revealed a novel variant in the TRPM6 gene. After parental treatment of intravenous magnesium (Mg2+) sulfate and calcium, the treatment was switched to enteral Mg2+ medications, due to persistent hypomagnesemia and the gastrointestinal side-effects, different oral preparations were used. The patient was stable on an oral maintenance dose of Mg2+ oxide with borderline blood Mg2+ levels and resolution of hypocalcemia. Hypomagnesemia is one of the causes of hypocalcemia. Enteral replacement is the key treatment but the treatment should be individualized for each patient. Normalization of hypomagnesemia is not always easy and should not be the aim of the treatment.

Involuntary movement disorders are rare in childhood. Hyperkinetic movement disorders including chorea stand as the leading cause. Although Sydenham chorea is the major diagnosis in most children and adolescents, appropriate differential diagnosis is fundamental for a final decision. A detailed and careful history as well as physical examination is the principal proceeding for accurate diagnosis. Herein, we report on an adolescent girl who was admitted to our hospital with chorea and subsequently diagnosed with systemic lupus erythematosus (SLE). Accompanying joint complaints in the patient's history, including growth retardation noticed during a physical examination and bicytopenia recognized in laboratory evaluation, increased the suspicion of SLE rather than Sydenham chorea in the patient. Central nervous system involvement defined as neuropsychiatric lupus presents wide clinical findings varying from stroke and seizures to psychosis and cognitive dysfunction. Although disease activity, persistently positive anticardiolipin antibodies, and lupus anticoagulant positivity are reported to be the most important risk factors in neuropsychiatric lupus, they are not always directly correlated. We present this patient in order to draw attention to the importance of physical examination and history in the differential diagnosis of chorea in childhood.Unwillkürliche Bewegungsstörungen im Kindesalter sind selten. Die häufigste Ursache sind hyperkinetische Bewegungsstörungen, einschließlich Chorea. Obwohl die Sydenham-Chorea bei den meisten Kindern und Jugendlichen die Hauptdiagnose darstellt, ist eine angemessene Differenzialdiagnose für eine endgültige Entscheidung von grundlegender Bedeutung. Eine detaillierte und sorgfältige Anamnese sowie eine körperliche Untersuchung sind die wichtigsten Voraussetzungen für eine genaue Diagnose. In diesem Beitrag berichten wir über ein jugendliches Mädchen, das mit Chorea in unser Krankenhaus eingeliefert wurde und bei dem anschließend ein systemischer Lupus erythematodes (SLE) diagnostiziert wurde. Begleitende Gelenkbeschwerden in der Anamnese, eine bei der körperlichen Untersuchung festgestellte Wachstumsverzögerung und eine bei der Laboruntersuchung festgestellte Biztopenie verstärkten den Verdacht auf SLE und nicht auf eine Sydenham-Chorea bei der Patientin. Eine Beteiligung des zentralen Nervensystems, die als neuropsychiatrischer Lupus definiert wird, weist ein breites klinisches Spektrum auf, das von Schlaganfällen und Krampfanfällen bis hin zu Psychosen und kognitiven Funktionsstörungen reicht. Obwohl die Krankheitsaktivität, anhaltend positive Anticardiolipin-Antikörper und eine Positivität auf Lupus-Antikoagulanzien als die wichtigsten Risikofaktoren für neuropsychiatrischen Lupus gelten, besteht nicht immer eine direkte Korrelation. In diesem Artikel wird dieser Patient vorgestellt, um auf die Bedeutung der körperlichen Untersuchung und der Anamnese bei der Differenzialdiagnose von Chorea im Kindesalter aufmerksam zu machen.

Introduction: There have been several studies investigating the association between Toxoplasma gondii seropositivity and psychiatric disorders although there is insufficient data on causality. Suicide, depression, and anxiety disorders have been especially investigated in this regard. In this study, we aimed to investigate whether there is any causal association between Toxoplasma gondii seropositivity and suicide attempts in adolescents. Methodology: This is a case-control study conducted between January and December 2019. A total of 27 adolescents who had attempted suicide and were aged between 12 and 18 years were included in the study. 26 age and sex ratio matched healthy volunteers were taken as the control group. A possible association between suicide attempts and Toxoplasma gondii serology (IgM and IgG) was investigated.. Results: The suicide attempt group consisted of 17 females and 10 males. The mean age was 15.9 ± 1.4 (13.5-17.9) years. Toxoplasma gondii IgG seropositivity was 3.7% (1/27) in the suicide attempt group and 3.8% (1/26) in the control group. There was no significant association between the suicide attempt group and the control group in terms of the presence of Toxoplasma gondii IgG antibodies (p > 0.05). Conclusion: Our study is one of the few studies examining the association between Toxoplasma gondii seropositivity and suicide attempts in adolescents yet we did not find any significant association. Further evidence is needed to clarify this controversial issue.

The outbreak of coronavirus disease 2019 (COVID-19) and its consequences have led to fear and anxiety among individuals worldwide. The risk of coronavirus transmission frightens people more than any other health problem they face. Parents have concerns about being infected with COVID-19 and delay accessing hospitals even in an emergency which can be very detrimental to child health. Here, in this article, we would like to present eight patients delayed in admission to the hospital to draw attention to the harmful consequences of COVID-19 fear in the community. Although anxiety and fear are encouraging to take necessary precautions, exaggeration of these emotions may cause greater health problems.

Chlamydophila pneumoniae, a common cause of respiratory tract infections, rarely leads to serious conditions. A 13-year-old boy with serologically confirmed C. pneumoniae infection presented with p...

Objective: Increased antibiotic resistance is a significant problem associated with higher morbidity, mortality, and costs in managing urinary tract infections (UTI). This study aims to analyze the antimicrobial resistance profile of uropathogens isolated from children with the first attack of community-acquired UTI.Material and Methods: This retrospective cohort study was conducted between January 2010-December 2016, with the patients aged one month to 18 years diagnosed with the first attack of community-acquired UTI at the pediatric outpatient clinic of a tertiary care hospital. Patients’ age, sex, UTI history, clinical findings at admission, urine analysis, urine culture, and antibiogram results were recorded.Results: A total of 1086 patients and 1086 cultures were included in the study. The male/female ratio was 1/5.3. The mean age of the children was 73.7±47.1 (0.4-215.9) months. 16.0% were aged 1-24 months. E. coli was the most common causative agent found to be 85.1% in frequency. The overall antimicrobial resistance was found to be highest for ampicillin, followed by piperacillin and trimethoprim-sulfamethoxazole (63.5, 41.6 and 38.1%, respectively). Imipenem, amikacin, and tobramycin had the least resistance (0.5, 0.5, and 7.6%, respectively). The overall antimicrobial resistance against amoxicillin-clavulanate, ampicillin-sulbactam, cefuroxime, ceftriaxone was 19.4, 24, 25.9 and 21.1%; respectively.Extended-spectrum beta-lactamase positivity was detected in 5.6% (n=61) of samples.Conclusion: Our study showed significant increase in antimicrobial resistance to the most common antibiotics which are prescribed before urine culture results and antibiotic sensitivities are available. The resistance rates for amoxicillin-clavulanate, ampicillin-sulbactam, cefuroxime, ceftriaxone was almost 20% or above in children with the first UTI attack in an outpatient setting., Amaç: Artan antibiyotik direnci, idrar yolu enfeksiyonlarının (İYE) yönetiminde daha yüksek morbidite, mortalite ve maliyetlerle ilişkili önemli bir sorundur. Bu çalışma, ilk kez toplum kökenli İYE geçiren çocuklardan izole edilen üropatojenlerin antimikrobiyal direnç profilini analiz etmeyi amaçlamaktadır.Gereç ve Yöntemler: Bu retrospektif kohort çalışması, Ocak 2010-Aralık 2016 tarihleri arasında, üçüncü basamak bir çocuk hastanesinin polikliniklerinde ilk toplum kökenli İYE atağı tanısı konan bir ay ile 18 yaş arasındaki hastalarla yapıldı. Hastaların yaşı, cinsiyeti, İYE öyküsü, başvurudaki klinik bulguları, idrar analizi, idrar kültürü ve antibiyogram sonuçları kaydedildi.Bulgular: Çalışmaya toplam 1086 hasta ve 1086 kültür dahil edildi. Erkek/kadın oranı 1/5.3’dü. Çocukların yaş ortalaması 73.7±47.1 (0.4-215.9) aydı. %16’sı 1-24 aylıktı. E. coli sıklık olarak %85.1 ile en sık etken etkendi. Genel antimikrobiyal direncin ampisilin için en yüksek olduğu, ampisilini piperasilin ve trimetoprim-sülfametoksazolün (sırasıyla %63.5, %41.6 ve %38.1) takip ettiği bulundu. İmipenem, amikasin ve tobramisin en düşük dirence sahipti (sırasıyla %0.5, 0.5 ve %7.6). Amoksisilin-klavulanat, ampisilin-sulbaktam, sefuroksim, seftriakson’a karşı genel antimikrobiyal direnç oranı sırasıyla %19.4, 24, %25.9 ve %21.1’di. Örneklerin %5.6’sında (n=61) geniş spektrumlu beta-laktamaz pozitifliği saptandı.Sonuç: Çalışmamız, idrar kültürü ve antibiyotik duyarlılık sonuçları belirlenmeden önce reçete edilen en yaygın antibiyotiklere karşı antimikrobiyal dirençte önemli artış olduğunu göstermiştir. Amoksisilin-klavulanat, ampisilin-sulbaktam, sefuroksim, seftriakson için direnç oranları, ayakta tedavi ortamında ilk İYE atağı olan çocuklarda neredeyse %20 veya üzerindedir.

Background COVID-19 rapidly spread worldwide with high morbidity and mortality. In this study, we aimed to detect parental concerns along with their awareness of and attitudes towards COVID-19 among patients admitted to our inpatient and outpatient clinics. Methods This study was conducted at a children's hospital with 141 parents of children who were patients in the inpatient and outpatient clinics. Parents were asked to complete an anonymous questionnaire and psychiatric scales that included the Beck Depression Inventory, Beck Anxiety Inventory (BAI), and Impact of Events Scale-Revised form (IES-R). Results The study sample consisted of 141 parents, of which 59 were parents of inpatients and 82 were parents of outpatients. The most known COVID-19 symptoms were fever, difficulty in breathing, and cough, respectively. The most preferred precaution was "staying at home." We found a significant positive correlation between the number of precautions and BAI score (R = .169, P = .046). Inpatients' parents IES-R scores were significantly higher than outpatients' parents IES-R scores. Conclusions Parents were found to be aware of the COVID-19 pandemic regardless of education status and family income. Participants' IES-R scores revealed significant differences in terms of COVID-19 impact on psychological health between the parents of inpatient and outpatient children; inpatients' parents were more concerned about COVID-19.

Aim Longitudinally extensive transvers myelitis (LETM) is a rare and disabling condition in childhood. The aim of the present study was to share experiences from our center regarding the treatment features and clinical and radiologic course in our LETM patients in light of the literature data. Material and method The study was designed as cross-sectional and included children who followed for LETM at our pediatric neurology clinic between 2010 and 2019. ATM was diagnosed according to the diagnostic criteria report from the Transverse Myelitis Consortium Working Group. LETM was defined as the presence of spinal cord lesions spanning a length of 3 or more consecutive vertebral segments. The patients’ medical records were examined in terms of demographic characteristics, presenting symptoms, history of infection prior to and during LETM, prodromal history, neurological examination, laboratory and radiological findings, clinical course, and treatment. The Barthel Index was used to assess the physical independence in activities of daily living of patients with LETM who were followed for at least one year. Results A total of 15 (8 girl) patients were included in the study. The patients were between 1 and 17 years of age. Presenting symptoms included inability to walk in 12 patients, incontinence in 9 patients, low back pain in 4 patients, abdominal pain in 2 patients, and inability to use the arms in 2 patients. In Barthel Index assessment of physical independence in activities of daily living, 8 patients were evaluated as completely independent, 3 patients as moderately dependent, and 2 patients as slightly dependent. When the 4 patients with motor area impairment and moderate dependency according to the Barthel Index were examined, it was noted that all of them had been admitted 4 days after the onset of symptoms and that 2 (13.3%) had cervicothoracic involvement and 2 (13.3%) had involvement of the entire cord. Conclusion Shorter delay from symptom onset to initiation of immunomodulatory therapy as well as effective rehabilitation resulted in favorable outcomes, with the most noticeable improvement in the areas of motor function and incontinence.

An overdose of isoniazid (INH) is potentially fatal and attempts at suicide are very rare in children. Three patients aged 14-17 years who were receiving INH for tuberculosis prophylaxis were admitted to the emergency department with generalised tonic-clonic seizures. There was metabolic acidosis and elevated levels of blood creatine kinase, aminotransferases and lactate dehydrogenase following ingestion of excess INH in attempts at suicide. The presumed total amounts of INH ingested were 3 g (40 mg/kg), 9 g (160 mg/kg) and 6 g (100 mg/kg), respectively. They all improved with general supportive measures including airway protection, gastric lavage, activated charcoal administration, sodium bicarbonate infusion, fluid replacement, seizure control and pyridoxine administration. They were discharged without complications. Attempts to commit suicide by excess intake of INH is rare in children but should be considered in the differential diagnosis of acute intractable seizures and metabolic acidosis refractory to conventional anticonvulsant therapy in adolescents.

Autism Spectrum Disorder (ASD) is a neurodevelopmental disorder and pediatricians play a critical role in early diagnosis. This study was conducted to evaluate the knowledge, beliefs, and experiences of pediatric residents and pediatricians about ASD. A 35-item questionnaire consisting of two sections was administered. The diagnostic features section consisted of questions measuring the level of knowledge about diagnostic criteria of ASD. The general knowledge and beliefs section consisted of questions about beliefs and experiences regarding ASD. The study consisted of 101 participants. Mean score obtained by all participants from the diagnostic features section was 5.78 ± 1.65. The mean scores obtained by residents and pediatricians from the diagnostic features section were 5.35 ± 1.5 and 6.31 ± 1.68. Pediatricians obtained higher scores significantly than residents from diagnostic features section (t = -2.99, p = 0.004). The mean scores obtained by residents and pediatricians from section of general knowledge and beliefs were 13.69 ± 3 and 14.48 ± 3.15. This difference was not significant (t = -1.27, p = 0.225). This study suggests that participants' knowledge and awareness about ASD was insufficient and while pediatricians knew more about the diagnostic criteria, their awareness and approach regarding ASD were no different from pediatric residents in general practice.

Background/aim Acute necrotizing encephalopathy is a rare type of acute encephalopathy characterized by multi-ocal brain lesions and associated severe neurological findings and various organ dysfunctions may accompany it. Materials and methods Patients with acute necrotizing encephalopathy of childhood diagnosed by pediatric neurology and pediatric intensive care at Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital between 2007 and 2020 were included in this study. Results Nine patients (six females, three males) with a mean age of 4.05 ± 1.94 years (age range 1–6.5) were included in this study. The interval range between fever and encephalopathy in patients was 1–4 days. Influenza A (3H1N1, one untyped) was detected in four patients, influenza B in three patients, and no cause was found in two patients. Major clinical findings other than febrile encephalopathy in all patients were a hemodynamic shock in seven patients, seizures in six patients, vomiting in five patients, dystonia in three patients, and flaccid paralysis in the upper extremity in one patient. Despite all our treatment approaches, including plasmapheresis, moderate to severe neurological sequelae was observed in all of our patients, who survived even with significant radiological improvement. Three patients for whom we could not perform plasmapheresis died. Conclusion Our study revealed that thalamic involvement increased as the interval shortened, and brainstem involvement increased in patients over four years of age. The presence of persistent vomiting accompanying encephalopathy during the parainfectious period and plasmapheresis treatment being a treatment option that could prevent mortality were cautionary for our study.

Refugee children are defined as an at-risk population as they have a high risk of physical and mental health conditions. While data exist regarding the mental health of refugee children, there are limited data about their medical health issues and mortality. Therefore, this study aimed to analyze the demographic data, clinical results, treatment/management data, and mortality data of hospitalized refugee children. This is a descriptive study that analyzed the demographic data, clinical findings, treatment/management data, and mortality data of 728 refugee children aged between 1 month and 18 years who were hospitalized in a tertiary pediatric hospital between 2013 and 2018. During the 5 year duration of this study (2013-2018), there were 12,031 patients hospitalized in the department of general pediatrics. Of these patients, 728 (6%) were refugee children [median age 1.2 (IQR 4.4) years]. The most frequent ethnic origin was Syrian, followed by Iraqi and Afghan [465 (63.87%); 174 (23.9%), and 39 (5.3%), respectively]. The median duration of hospitalization was 6 (IQR 6) days. Those refugee patients who were hospitalized in the pediatric intensive care unit were significantly younger [median age 3.7 (IQR 9.4) years]. The mortality rate in the department of general pediatrics was 16.4% for refugee patients and 8.6% for non-refugee patients (p = 0.001). A logistic regression model revealed that factors associated with mortality included younger age (OR 1.6; CI 1.2-2.1) and being a refugee (OR 2.1; CI 1.3-3.2). Our study revealed detailed knowledge about demographic, clinical, and mortality data, with the largest known series about refugee children in the literature. The results show that mortality rates are significantly higher in refugee pediatric patients who are hospitalized in Turkey than in non-refugee patients.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation. Secondary HLH syndrome develops as a complication of infection, drugs, rheumatologic conditions, or malignancy. The main objectives of this work were to identify the etiology of secondary HLH and prognostic factors associated with mortality. Patients diagnosed with secondary HLH, between January 2011 and December 2016, were retrospectively included in this study. We analyzed clinical and laboratory findings as well as prognostic factors from 24 pediatric patients diagnosed with secondary HLH. The mean age of patients at the time of diagnosis was 79.9 ± 68.7 months (range: 2-202) and 54.2% of the patients were male. The most frequent HLH-2004 criterion was fever (100%). Underlying triggers of HLH were as follows: 13 (54.1%) infections, juvenile idiopathic arthritis in 5 patients (20.8%), drugs in 3 patients (12.5%), malignancies in 2 (0.8%), Kawasaki disease in 1 (0.4%) patient, and 1 (0.4%) with unknown triggers. The median time of diagnosis was 3 days (1-67 days). Overall, the mortality rate was 20.8%. In our logistic regression model, factors associated with mortality were decreased albumin levels (OR1 = 2.3[1.48-3.43]) and etoposide usage (OR2 = 1.22 [1.14-1.89]). The patient's 30-day survival was inferior among patients whose albumin level was 2 g/dL or less compared to those over 2 g/dL. Increased awareness of the underlying condition is critical in HLH patients. Our study emphasizes the prognostic significance of albumin level.

Argininemia is a rare hereditary disease due to a deficiency of hepatic arginase, which is the last enzyme of the urea cycle and hydrolyzes arginine to ornithine and urea. Herein we report a patient with arginase I (ARG1) deficiency who presented with recurrent nonconvulsive status epilepticus and liver failure. A novel homozygous frameshift mutation c.703_707delGGACTinsAGACTGGACC (p.G235Rfs*20) was detected.

Giriş: Çocukluk çağı zehirlenmeleri çocukluk çağı sağlık sorunları arasında önemli bir yer tutmaktadır. Dünyada 5 yaş altı kazaların %7’sini zehirlenmeler oluşturmaktadır. Gelişmiş ülkelerdeki çocuk ölümlerinin %2’si, gelişmekte olan ülkelerde ise %5’inden fazlası zehirlenmelere bağlı oluşmaktadır. Materyal ve metodlar: Bu çalışma Dr. Sami Ulus Kadın-Doğum Çocuk Sağlığı ve Hastalıkları Eğitim ve Araştırma Hastanesi’ne 01 Ocak 2007- 31 Aralık 2007 tarihleri arasında zehirlenme şikayeti ile başvuran 607 çocuk olgunun verilerinin değerlendirildiği tanımlayıcı bir çalışmadır. Bulgular: Olguların 328’i (%54) erkek, 279’u (%46) kız idi. Olguların yaş ortalaması 4,13±1,53 olarak bulundu. Zehirlenmelerin en sık (n=144, %23,7) görüldüğü yaş grubu 2-3 yaş idi.Zehirlenmelerin en sık görüldüğü ay Mayıs ayı idi. Zehirlenmelerin 580’i (%95,6) kaza, 27’si (%4,4) intihar sebebi ile gerçekleşmişti. Zehirlenme nedenlerine göre sınıflandığında; Olguların 338’ünün (%55,7) tarımsal-endüstriyel ürünlerle, 193’ünün (%31,2) ilaçlar ile, 48’inin (%7,9) besinler ile, 23’ünün (%3,8) karbonmonoksit ile, 2’sinin (%0.32 ) hayvan ısırıkları ile ve 3’ünün (%0,5) bilinmeyen faktörlere bağlı olarak geliştiği saptandı. Santral sinir sistemine etkili ilaçlar ile zehirlenme ilaç zehirlenmeleri içerisinde en sık (%29,5) görülen grup idi. Hastaların %60’ı yatırılarak tedavi edildi. İlaç zehirlenmesi nedeni ile takip edilen bir olgu eksitus ile sonuçlandı. Sonuç: Zehirlenmelerin çoğu küçük yaş grubunda ve erkek çocuklarda kazaen alımlarda görülmektedir. Bu nedenle, en doğru yaklaşım koruyucu tedbirlerin arttırılmasıdır. Temizlik maddeleri ve ilaçlar ile oluşan zehirlenmeler sık görülmektedir. Bu maddelerin ve ilaçların emniyetli yerlerde saklanması, ilaçların ve temizlik maddelerinin kapaklarının çocukların açamayacağı mekanizmalarla üretilmesi, temizlik maddelerinin yiyecek ve içecek kaplarında saklanmaması konusunda ailelere eğitim verilmelidir. Riskli davranış sergileyen adolesanlara psikolojik destek sağlanmalıdır.

We investigated the presence of nutritional rickets in Syrian and Iraqi refugee infants who presented to hospital in Turkey in 2017. 25(OH)D levels were examined in 77 refugee children. Nutritional rickets was diagnosed in 22 (28.5%) children; 11 patients with rickets did not follow up.

Objective: Lane-Hamilton syndrome is a rare disease which is coexistence of celiac disease and idiopathic pulmonary hemorrhage. Treatment is based on gluten free diet and immunosuppressive agents however there are no definitive recommendation for the treatment. Case presentation: A 14 year-old boy patient admitted to emergency department with fatigue and minor hemoptysis for 2 months. His hemoglobin was 3.7 g/dl and 2 units of erithrocyte transfusion were given. He had no fever, abdominal pain, nausea, vomiting, diarrhea. Physical examination was normal. There were bilateral interstitial infiltration in lower zones on chest x-ray and common ground-glass opacities were present on Thorax CT. Acute phase reactants, bleeding parametries, immunoglobulins, autoantibodies, serologic and microbiologic tests for tuberculosis, echocardiography were normal. Hemosiderin laden macrophage was present in fasting gastric lavage. Celiac antibodies were positive, endoscopy was performed and cracked mud appearance was present. Duodenum biopsy was compatible with celiac disease. Gluten free diet and methylprednisolone treatment were started. Hemoptysis resolved and hemoglobin increased to 10 g/dl on the second month of the treatment and after then steroid was tapered by weekly. Discussion: Lane-Hamilton syndrome is very rare and it should be kept in mind in patients with pulmonary hemorrhage even without gastrointestinal symptoms. We gave gluten free diet and steroid to our patient because of active hemoptysis and respiratory symptoms and he was improved with the treatment. Although steroid treatment is controversial in the literature, it should be used in patients with active respiratory symptoms.

Giris ve Amac: Bu calismada 2018 yili kis-ilkbahar-yaz doneminde bogmaca/bogmaca benzeri hastalik on tanisi ile yatirilarak izlenmis hastalarin klinik verilerinin sunulmasi amaclanmistir. Yontem ve Gerec: Ocak 2018-Eylul 2018 tarihleri arasinda Dr. Sami Ulus Kadin-Dogum, Cocuk Sagligi ve Hastaliklari EAH pediatri servislerinde bogmaca, bogmaca benzeri hastalik tanisiyla yatirilan, yasi 4 aydan kucuk olan hastalar retrospektif olarak incelendi. Bulgular: Toplam 97 hasta (51 erkek, 46 kiz) bogmaca/bogmaca benzeri hastalik tanisi ile izlendi. Hastalarin ortanca yasi 2 (min-maks 0,97-3.90) aydi. Vakalarin hepsinde basvuru sirasinda nobetler halinde gelen bogulur tarzda oksuruk, 32 vakada (%33) akcigerde dinleme bulgusu, 5 vakada apne, 36 vakada takipne vardi. 31 hastadan (%32) bogmaca icin nazofaringeal ornekgonderildi. Gonderilen 31 ornekten 12’sinde polimeraz zincir reaksiyonu (PZR) ile B. pertussis geni pozitif olarak saptandi (%39).Yatis suresi ve hastalarin yasi acisindan bogmaca PZR pozitif, bogmaca PZR negatif ve tetkik alinmamis grup arasinda anlamli fark yoktu. Bogmaca PZR pozitif grubunun beyaz kuresi ve lenfosit sayisi diger iki gruba gore anlamli olarak daha yuksekti. Hastalarin 2 tanesi ilk yatis gunlerinden sonra solunum sikintisi nedeniyle yogun bakima sevk edildi. Ikisi de mekanik ventilator destegi aldi ve sifa ile taburcu edildi. Tartisma ve Sonuc: Bogmaca enfeksiyonu erken bebeklik doneminde onemli morbidite ve mortaliteye neden olmaktadir. Maternal asilama ve koza stratejisine yonelik kapsamli calismalar risk altindaki bu bebekleri korumada onemli katkilar saglayabilir.

Objectives To evaluate cochlear functions in patients with Familial Mediterranean Fever in relation to the disease severity score and treatment duration. Methods 50 patients (4–18 years) who had been followed-up with the diagnosis of FMF and regularly receiving appropriate colchicine treatment and 39 healthy controls were included in the study. All the patients and controls were evaluated by audiologic evaluation, including high-frequency pure-tone audiometry and distortion product otoacoustic emission tests (DPOAE). The disease severity was determined by scoring system developed by Pras et al. Results Fifty patients (52% female, 48% male; mean age12.2 ± 4.1 years) and 39 controls (58.9% female, 41.1% male, mean age 11.1 ± 3.4 years) were enrolled the study. The pure tone average of FMF patients was significantly higher than that of the control group at 500, 4000, and 8000 Hz frequencies. The patients' DPOAE signal values at 6 kHz, 8 kHz frequencies and SNR values at 8 kHz were significantly higher than control group. The patients' audiometry and DPOAE results were compared with the disease severity scores. Pure tone average was significantly higher in severe and moderate patient groups compared to the mild patient group at 2000 Hz frequency. DPOAE signal values showed statistically significant differences between the patient severity scores at 1.4 and 2.8 kHz frequencies. The mean colchicine treatment duration was found to be 5.1 ± 3.7 years. There were significant differences at 250 and 500 Hz frequencies when patients' audiometry results were compared with the treatment periods. Conclusions FMF affects cochlear functions particularly at high frequencies.

Amaç:HumanPapilloma Virüs (HPV) serviks kanserinin en sık nedenidir. Serviks kanserini veHPV ilişkili diğer hastalıkları önlemede aşılama birincil olarak etkiliyöntemdir. Bu çalışmanın amacı ebeveynlerin HPV aşısı hakkındaki bilgidüzeyleri ve yaklaşımlarını değerlendirmektir. Gereç ve Yöntemler:Tanımlayıcıolan bu çalışma Aralık 2018-Ocak 2019 tarihlerinde 9-18 yaş arası kız çocuğunasahip 203 ebeveyn üzerinde yapılmıştır. Ebeveynlerin demografik verileri, HPVhakkındaki bilgileri ve HPV aşılamasına ilişkin tutumlarını içeren anketlerdeğerlendirildi. Bulgular:203ebeveynin (% 80 anne ve% 20 baba) yaşortalaması 39,1 ± 6,1 idi. Ebeveynlerin% 70'i HPV aşısını duymamıştı ve% 67'sirahim ağzı kanserinin önlenebilir bir hastalık olduğunu bilmiyordu.Ebeveynlerin% 62'si bilgilendirme sonrasında kızlarına HPV aşısını yaptırmayıkabul etti. Aşının fiyatı ve doz sayısı hakkında bilgilendirme sonrasında,ebeveynlerin% 24'ü 'Devlet tarafından ücretsiz karşılansa dahi yaptırmam'şeklinde cevap verdi. En önemli nedenleri aşı hakkında yeterli bilgiye sahipolmamaları idi (% 69).Sonuç:Çoğu ebeveyn HPV aşısını bilmemektedir ve bilgilendirme sonrasıçocuklarına aşı yaptırmayı kabul etmektedir. Bununla birlikte bazı ebeveynler,ücretsiz karşılanmış olsa dahi çocuklarına aşı yaptırmayı düşünmemektedir.Ailelerin bu konuda daha fazla aydınlatılması için sağlık çalışanlarına büyükgörev düşmektedir.

Chronic recurrent multifocal osteomyelitis is a rare autoinflammatory, immunologic disorder. It may involve recurrent inflammatory bone pain associated with aseptic osteomyelitis. If the diagnosis is delayed, it negatively influences quality of life by leading to persistent symptoms or joint damage. Herein, we report a 16-year-old male patient who presented with left hip and right knee pain lasting for the last two months and was diagnosed as having chronic recurrent multifocal osteomyelitis as a result of further evaluation. Improvement was achieved with prednisolone and methotrexate treatment that was initiated when no response to ibuprofen treatment could be obtained. Chronic recurrent multifocal osteomyelitis must be considered in the differential diagnosis in patients presenting with joint symptoms when clinical and radiologic bone lesions are found, no microorganism growth is observed, and no response to antibiotic treatment is obtained.

Background The aim of this study was to assess the quality of life (QoL) of mothers of children with Down syndrome (DS) and the factors related to it. Methods Seventy-five DS child-mother diads and 54 healthy child-mother diads were included in this study. The brief World Health Organization Quality of Life scale - Turkish version (WHOQOL-BREF-TR), Symptom Check List-90-Revised (SCL-90-TR), Beck Depression Inventory, Beck Anxiety Inventory and Maslach Burnout Inventory (MBI) were completed by mothers to assess maternal QoL, burnout, anxiety, depression and general psychiatric symptoms. Results Mothers of DS children had significantly higher SCL-90 general symptom index scores, Beck depressive symptom scores and higher scores in all domains of the brief World Health Organization Quality of Life scale - (WHOQOL-BREF-TR) except the WHOQOL-BREF-TR-psychological health domain. MBI personal achievement scores were weakly correlated with psychological health in mothers of DS children. There was a reverse correlation between comorbid disorders and the WHOQOL-BREF-TR psychological health domain, and a weak correlation between sex (having a baby girl with DS) and increased WHOQOL-BREF-TR physical health domain score. Number of offspring was negatively correlated with maternal social quality of life. Conclusion Mothers of children with DS are negatively affected in terms of QoL and mental health, even in the early period. It would be useful to systematically screen these mothers and to refer them for appropriate intervention at an early stage.

Objectives Lumbar puncture (LP) is fundamental for diagnosis and treatment; however, some parents do not provide consent for their children to undergo the procedure, which can make diagnosis and determination of the optimal treatment difficult. The present study aimed to describe the level of knowledge and attitudes toward LP of parents whose children were scheduled to undergo the procedure. Methods A prospective cross-sectional descriptive study of a convenience sample of parents of 84 children aged 2 months to 17 years scheduled for LP at a single academic children's hospital between 2015 and 2017. Parents were administered a written survey and interviewed by a physician other than the person who did the LP. Data on parental level of knowledge and attitudes regarding LP, in addition to reasons for refusal, were collected.The parents of 84 patients scheduled for LP due to various indications were administered a face-to-face survey interview. The survey was used to collect parental demographic data, as well as opinions and knowledge about LP and postinterventional complications. Results The mean age of the 84 patients (57% male and 43% female) was 6.4 ± 5.17 years. Lumbar puncture was planned for the presumptive diagnosis of neurological disease in 45.25% of the patients, central nervous system infection in 45.25%, and acute encephalopathy in 9.5%. Among the parents, 65% (n = 55) had no knowledge or attitude about LP prior to the survey interview. The most common parental concern related to LP was paralysis (25%), followed by infertility (2%), mental retardation (1%), and disease progression (1%). Only 4.7% of the parents did not provide consent for their child to undergo LP. Conclusions We found that most parents had little knowledge about LP, and the most common parental concern was paralysis. Despite this, in our study, only 5% of parents did not consent to LP.

Aim: The aim of the present study was to evaluate the etiologies, diagnostic approach, clinical and laboratory data of patients with arthritis in a Turkish tertiary care hospital in children.Patients and Methods: 306 hospitalized children aged between 7months-18 years, diagnosed with arthritis were included in the study between 2008 – 2013. The medical records of patients were reviewed retrospectively.Results: Ofthe patients, 51.6% were female, 48.4% were male. The average age was 118 months.Arthritis was symmetrical type in 29.4% of patients and asymmetrical type in 70.6%.The diagnoses of patients were Acute Rheumatic Fever/Poststreptococcal reactivearthritis (ARF/PSRA) (39.2%), Collagen Tissue Disorders (CTD) (29%), Brucellararthritis (13.4%), reactive arthritis (12.5%), septic arthritis (5.2%) and arthritissecondary to malignancy (0.7%), respectively. Of the patients with CTD; Juvenilidiopathic arthritis (JIA) was the most common in frequency (66.3%). Patellarjoint involvement was the most common in frequency (62.4%). The mean ASO levels was significantly higher inpatients with ARF/PSRA group [p=0.000]. Average leucocyte count of ARF/PSRA groupwas significantly higher than CTD group [p=0.000]. Average neutrophilpercentage was significantly higher in ARF/PSRA group than brucellar arthritisgroup [p=0.000]. The mean duration of diagnosis and therapy was found to besignificantly longer in patients with CTD [p=0.000]. All patients were curedexcept one patient with brucellar arthritis. Conclusion: Arthritis canbe a manifestation of multiple disease processes in children. Therefore, theclinician must consider a broad differential diagnosis. Detail history and physical examination with a clinical follow-up inaddition to useful laboratory testing may helpto establish the cause of arthritis in children., Amaç: Bu çalışmanınamacı üçüncü basamak olan bir sağlık kuruluşunda artrit tanısıyla takip edilen çocukhastaların etyolojisi, tanısal yaklaşımı, klinik ve laboratuvar verilerinindeğerlendirilmesidir.Hastalar veYöntemler: 2008 - 2013 yılları arasında, 7 ay-18 yaş arasında, hastanedeyatarak tedavi gören 306 artrit tanılı çocuk çalışmaya dahil edildi. Hastalarıntıbbi kayıtları retrospektif olarak incelendi.Bulgular:Hastaların % 51,6'sı kız, % 48.4'ü erkekti. Yaş ortalaması 118 ay idi. Hastaların% 29.4'ünde simetrik, % 70.6'sında asimetrik tip artrit tespit edildi. Hastalarıntanıları sırasıyla Akut Romatizmal Ateş/Poststreptokoksik reaktif artrit (ARA/PSRA)(% 39.2), Kollajen doku hastalığı (KDH) (% 29), brusella artriti (% 13.4),reaktif artrit (% 12.5), septik artrit (% 5.2) ve maligniteye bağlı artrit (%0.7) idi. Kollajen doku hastalığı grubunda jüvenil idyopatik artrit (JİA) ensık görülen hastalık idi (% 66.3). Patellar eklem tutulumu en sık tutulaneklemdi (% 62.4). ARA / PSRA grubu olan hastalarda ortalama ASO düzeyleriyüksek bulundu [p = 0.000]. ARA / PSRA grubunun ortalama lökosit sayısı, KDHgrubundan yüksekti [p = 0.000]. ARA / PSRA grubunda ortalama nötrofil yüzdesibrusella artriti grubundan daha yüksekti [p = 0.000]. KDH hastalarında ortalamatanı ve tedavi süresi daha uzun bulundu [p = 0.000]. Brusella artriti tanılı birhasta hariç tüm hastalar sekelsiz iyileşti.Sonuç:Artrit çocuklukçağında sistemik hastalık sürecinin bir bulgusu olabilir. Bu nedenle klinisyeninayırıcı tanı listesi geniş olmalıdır. Kapsamlı öykü ve fizik muayeneye ekolarak seçilecek uygun laboratuvar testleriyle birlikte klinik izlem çocuklukçağında artrit nedenlerini aydınlatmaya yardımcı olabilir.

Colchicine is an anti-inflammatory drug that has a narrow therapeutic index. Poisoning typically shows 3 phases with systemic symptoms. Gastrointestinal symptoms dominate in the first phase. Dermatologic manifestations usually appear, with skin eruptions in the second phase where multiorgan failure occurs and alopecia in the third phase where organ derangements resolve. Alopecia is a cardinal feature of the third phase, but there is no specifically defined eruption for toxication. Here, we report a case of colchicine intoxication in a 16-year-old girl with maculopapular/purpuric rash and alopecia.

The aim of the presentation of this case is to discuss whether there is an association with eosinophilic granulomatosis with polyangiitis (EGPA) and the use of montelukast, and clarithromycin and to discuss a successful treatment course. A 4-year-old girl with a preceding history of asthma attacks and increased eosinophil counts was admitted. She had been using clarithromycin for five days and montelucast for a month. She was eventually diagnosed with EGPA with detailed examination. Clinicians should remember EGPA in children with asthma and hypereosinophilia. Patients receiving leukotriene receptor antagonists and/or macrolides should be monitored for developing a multisystem disease. Treatment with immunosuppressive agents may be required to ensure a good prognosis.

The present study was conducted to determine young children's (aged 1-60 months) exposure to and use of mobile devices. The study included 422 parents of children aged 1-60 months admitted to pediatric outpatient clinics. A questionnaire was administered to the parents via face-to-face interview. Children's overall exposure to mobile devices was 75.6% (n = 319). Of the children, 24.4% (n = 103) had never used a mobile device. Among the children that had used a mobile device, 20.6% (n = 66) were aged between 1 and 12 months; 24.5% (n = 78) were aged between 13 and 24 months. The median age at the first time use of a mobile device was 12 months. The youngest child that used a mobile device was 6 months old. Tablets had a significant difference in the age at first use compared with other mobile media types (P 0.01). Children's overall mobile device ownership was 30.7% (98/319) in frequency. There was a positive relationship between mobile device ownership and age (p 0.001). The most commonly owned mobile device was a "tablet" at a frequency of 68.4% (67/98). The frequency of tablet ownership was inversely associated with household income (P 0.01). Of the children that used mobile devices, 25.7% (82/319) used multiple mobile devices simultaneously. Among 422 children, 15.9% (n = 67) had a tablet in their room. The frequency of tablet use and ownership was inversely related to the mother's educational level (P 0.01). The parents reported that 22.3% (n = 71) never received help while navigating the mobile device. The most frequent activity with mobile devices was watching videos (70.8%, n = 226). Of the parents, 59.6% (190/319) let their children use mobile devices while they are doing daily tasks or domestic chores. Of the parents, 91.5% (n = 386) reported not having been informed by a doctor about the effects of mobile devices on their children.Conclusion: This study demonstrates a high prevalence of exposure to mobile devices in young children. The frequency of tablet use and ownership of offspring was inversely related with maternal educational level and household income. What is Known: • Mobile phones and other similar mobile devices are now embedded in the daily lives of children. • There are limited data about mobile media use in early childhood, and few guidelines on which clinicians can base their recommendations. What is New: • The exposure to mobile devices is high in young children aged 1-60 months. • The frequency of tablet use and ownership of offspring was inversely related to household income and maternal educational level.

Complex regional pain syndrome is a condition of uncertain etiology characterized by spontaneous or stimulus-induced pain that is out of proportion to the inciting event. We report a 14-year-7-month-old girl with swelling of the left hand and wrist, was diagnosed as complex regional pain syndrome. The patient was treated successfully with physical therapy and non-steroidal anti-inflammatory drugs. This condition should be kept in mind in the differential diagnosis of musculoskeletal non-inflammatory and inflammatory pains.

Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem. An axillary lymph node biopsy demonstrated necrotising histiocytic lymphadenitis and KFD was diagnosed. He was discharged 35 days after admission. He was re-admitted 3 weeks later with recurrence of symptoms and headache and was found to have papilloedema of the left eye and auto-immune thyroiditis. Intravenous immunoglobulin (IVIG) 400 mg/kg/day was administered for 5 days. The fever and papilloedema slowly resolved and, subsequently, the thyroiditis, and he has remained well on follow-up. This is the first report of an association of S. enteritidis infection and papilloedema with KFD. IVIG may be required in prolonged or recurrent cases and in those with an auto-immune association.

BACKGROUND The pathogenesis of juvenile idiopathic arthritis (JIA) remains unknown, but imbalance between the oxidant and antioxidant defense systems may play a role. Measuring thiols in plasma provides an indirect indication of antioxidative defense. The aim of the present study was to investigate the association between JIA and dynamic thiol/disulfide homeostatic status. METHODS This case-control study involved 34 JIA patients and 30 age- and gender-matched healthy controls. The patients were divided into subgroups according to Simplified Disease Activity Index (SDAI) score: active, SDAI > 3.3; remission, SDAI ≤ 3.3. RESULTS Native thiol and total thiol were significantly lower in the JIA group than in the control group (P = 0.001). There was no significant difference in the disulfide/native thiol, disulfide/total thiol, and native thiol/total thiol ratios between the JIA and control groups (P > 0.05). Based on SDAI score, 22 JIA patients were in the remission subgroup, and 12 JIA patients were in the active subgroup. Native thiol and total thiol were significantly lower in the active JIA subgroup than in the remission subgroup (P = 0.001), but there were no significant differences in the other parameters. There was no significant difference in thiol and disulfide levels between systemic-onset JIA and other JIA (P > 0.05). CONCLUSIONS Plasma thiol is lower in JIA patients, especially during periods of active disease, than in healthy controls, indicating that low thiol might be an important factor in the etiology of JIA and that antioxidant systems are negatively affected by inflammatory diseases, especially during periods of active disease.

Black tongue is a rare, benign, self-limiting disorder caused by certain conditions and some medications. We report the first case of a child diagnosed with black tongue associated with ranitidine use. We report our case to emphasize the rare side effect of this frequently used drug. Health care professionals should be aware of the likelihood of ranitidine-induced black tongue in clinical practice.

Objective: This study was performed to examine the demographic characteristics, clinical features and diagnostic tests of children with the diagnosis of cystic fibrosis. Material and Methods: Presenting symptoms, complaints on admission, clinical features, and diagnostic tests for 41 children with cystic fibrosis between the age of 2 days and 4 months diagnosed at the Pediatrics clinic of Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital between January 2005 and June 2012 were examined. Results: The mean age at diagnosis was 5.2 months. There was a history of consanguinity in 61.1% of cystic fibrosis patients. Mutation analysis revealed that 75% of the children had a cystic fibrosis mutation. Gastrointestinal complaints were the most common presenting complaint (43.9%). There was no statistically significant difference between demographic characteristics, age at diagnosis, duration of complaints, hospitalization frequency, sweat test values, and mutation analysis between children presenting with gastrointestinal symptoms, respiratory tract symptoms (31.7%) and the Pseudo-Bartter clinical syndrome (24.4%). Conclusion: Cystic fibrosis is a multisystemic disease with findings related to the respiratory system and gastrointestinal system, and patients might present with the Pseudo-Bartter syndrome as well. It should be noted that cystic fibrosis can present with different clinical presentations due to the different genotypic features in our country.

Background To establish sonographic (US) criteria for the diagnosis of internal jugular phlebectasia (IJP) in children and to determine reliable cutoff values of US measurements with high specificity and sensitivity. Methods We used B-mode US for the measurement of internal jugular vein transverse (T) and anteroposterior (AP) diameter, and cross-sectional area (CSA) at rest and during the Valsalva maneuver (VM) in 21 patients, aged 15 months to 16 years, diagnosed with IJP, and compared the results with those of 88 healthy children. Receiver operating characteristics curves were used to determine the optimal cutoff values. Results Patients with IJP had higher T, AP diameters, and CSA at rest and during VM than controls on the same side (p 15 mm as a cutoff point for both sides for the diagnosis of IJP in daily practice. US measurement of the jugular vein diameter might help increasing clinicians' awareness of clinically unrecognized cases of IJP and identifying borderline cases that require follow-up. © 2013 Wiley Periodicals, Inc. J Clin Ultrasound, 41:486–492, 2013