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Congenital anomalies and variations should be kept in mind when evaluating diseases in the pediatric patient group. A 12 years old girl presented with findings of acut abdomen. Clinical and laboratory data were consistent with acute pancreatitis. Imaging findings showed a peristaltic cystic tissue with a thick wall and central lumen in the head of the pancreas. In the operation, it was shown that this cystic lesion was associated with the accessory pancreatic duct and had no duodenal connection. In the pathology report, the presence of duodenal intestinal tissue compatible with a duplication cyst located in the head of the pancreas was proven. By sharing this rare case, keeping in mind the underlying congenital anomalies in any pathology in children and emphasizing the importance of ultrasonographic dynamic real-time examination in abdominal imaging.

Background: This study aimed to evaluate electrocardiographic and echocardiographic findings, Holter recordings of the multisystem inflammatory syndrome in children, and to identify prognostic factors for cardiac involvement. Methods: We retrospectively reviewed demographic characteristics, medical data, laboratory findings, electrocardiogram and echocardiographic findings, 24-hour Holter recordings, need for an ICU, and extracorporeal membrane oxygenation in multisystem inflammatory syndrome in children. Acute left ventricular systolic dysfunction was defined as left ventricular ejection fraction (EF) ≤%55 on echocardiography. Results: Sixty-seven children were included in the study. 24-hour Holters were recorded in 61.2% of the patients and 49.2% were normal. On echocardiographic examination, 14.9% of the patients had systolic dysfunction (EF ≤ 55%). While 32.8% of patients had mild mitral regurgitation, 3% had moderate mitral regurgitation, and 6% had mild aortic regurgitation. There was no statistically significant difference in EF values between the group with arrhythmia in Holter and the group with normal Holter results (p ≥ 0.05). B-type natriuretic peptide was positively correlated with C-reactive protein, ferritin, and fibrinogen. Significant effectivity of the B-type natriuretic peptide value was observed in the differentiation of those with EF ≤ and > 55%. Extracorporeal membrane oxygenation support was needed for three (4.5%) patients. One patient who died had systemic juvenile idiopathic arthritis. Conclusions: Neutrophil/lymphocyte ratio, C-reactive protein, D-dimer, ferritin, troponin, and B-type natriuretic peptide were found to be significantly higher in patients with systolic dysfunction. Also, the cut-off value of 1700 pg/ml for B-type natriuretic peptide was significantly effective. These parameters may indicate the severity of the disease but should be supported by prospective studies.

Objectives: In this study, we present our clinical severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) experience in patients with childhood rheumatic disease during novel coronavirus-2019 (COVID-19) pandemic. Patients and methods: A total of 87 patients (50 males, 37 females; median age: 12 years; range, 6.6 to 16 years) suspected of having COVID-19 at our pediatric rheumatology clinic between March 11th and October 15th 2020 were retrospectively analyzed. Demographic and clinical features, treatments, laboratory results, imaging findings, and clinical outcomes of the patients diagnosed with COVID-19 and/or multisystem inflammatory syndrome in children (MIS-C) were retrieved from the medical records. The diagnosis of SARS-CoV-2 infection was made based on the reverse transcriptase-polymerase chain reaction test. Results: The most common rheumatic diseases were juvenile idiopathic arthritis and familial Mediterranean fever (35.6% and 34.5%, respectively). Twenty-six of these patients were treated with biological disease-modifying anti-rheumatic drugs. SARS-CoV-2 infection was tested as positive in 84 (96.5%) patients. Also, 51 (58.6%) patients had an epidemiological contact to a person with COVID-19. Eighteen patients met the clinical criteria and diagnosed with MIS-C. The COVID-19 outbreak also caused exacerbation of systemic disease in 56 children due to medication cessation, postponed drug switch, or recurrent viral infection. Conclusion: Children with rheumatic disease do not appear to present a higher risk of severe COVID-19. The immunosuppressive treatments can be adjusted in case of infection; otherwise, it is not recommended to interrupt the treatments. Physicians should be cautious about the hyperinflammatory syndrome associated with COVID-19 in rheumatic children, which may be severe in this group of patients and may be confused with primary diseases.

Rhinosinusitis is a common infection and may rarely cause severe life-threatening orbital and intracranial complications. In this study, two cases with preseptal cellulitis and meningitis as a complication of rhinosinusitis were presented in the light of the literature. A nine years and two months old girl was admitted with complaints as fever, redness and swelling in the left eye. Physical examination revealed erythema and edema in the left lower and upper eyelids, and the eye movements were painless and normal in all directions. Her systemic examination was normal and there was no sign of meningeal irritation. Magnetic resonance imaging revealed ethmoid, frontal and sphenoid sinusitis and left cerebral hemisphere dural meningeal contrast enhancement. The patient was diagnosed with meningitis after lumbar puncture. After 14 days of appropriate antibiotic treatment, the patient recovered and was discharged. An eight years and five months old boy presented with fever, redness and swelling in the left eye was admitted. There were erythema and edema in the left lower and upper eyelid; the eye movements were painless in all directions and were complete. Systemic examination was normal; there was no sign of meningeal irritation. Pansinusitis and preseptal cellulitis findings were detected on computer tomography. The patient's fever persisted under treatment and erythema and edema of the eye became more evident. Orbital MRI was performed considering the complication and contrast enhancement was observed in the left frontal region. The patient was diagnosed with meningitis after lumbar puncture. After 14 days of appropriate antibiotic treatment, the patient recovered and was discharged. Intracranial complication due to preseptal sinusitis is rare but life-threatening. In these cases, we recommend the use of MRI as the radiological imaging method.

Objectives Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition resulting in excessive response of the immune system after SARS-CoV-2 infection. We report a single-center cohort of children with MIS-C, describing the spectrum of presentation, therapies, clinical course, and short-term outcomes. Methods This is a prospective observational study from to a tertiary pediatric rheumatology center including patients (aged 1 month to 21 years) diagnosed with MIS-C between April 2020-April 2021. Demographic, clinical, laboratory results and follow-up data were collected through the electronic patient record system and analyzed. Results A total of 67 patients with MIS-C were included in the study. Fever was detected in all patients; gastrointestinal system symptoms were found in 67.2% of the patients, rash in 38.8%, conjunctivitis in 31.3%, hypotension in 26.9% myocarditis, and/or pericarditis in 22.4%, respectively. Respiratory symptoms were only in five patients (7.5%). Kawasaki Disease like presentation was found 37.3% of the patients. The mean duration of hospitalization was 11.8 7.07 days. Fifty-seven patients (85%) received intravenous immunoglobulin (IVIG), 45 (67%) received corticosteroids, 17 (25.3%) received anakinra, and one (1.5%) received tocilizumab. Seven of the patients (10.4%) underwent therapeutic plasma exchange (TPE). In 21 (31.3%) patients, a pediatric intensive care unit (PICU) was required in a median of 2 days. The first finding to improve was fever, while the first parameter to decrease was ferritin (median 6.5 days (IQR, 4–11.2 days)). Sixty-five patients were discharged home with a median duration of hospital stay of 10 days (IQR, 7–15 days). Conclusion Patients with MIS-C may have severe cardiac findings and intensive care requirements in admission and hospital follow-up. The vast majority of these findings improve with effective treatment without any sequelae until discharge and in a short time in follow-up. Although the pathogenesis and treatment plan of the disease are partially elucidated, follow-up studies are needed in terms of long-term prognosis and relapse probabilities.

Background:Acute rheumatic fever in childhood continues to cause serious morbidity despite all developments. The objective of this study was to evaluate the clinical and laboratory characteristics of patients with acute rheumatic fever and to determine the frequency of subclinical carditis and the side effects of the drugs used in the treatment.Methods:The data of patients hospitalised between 2008 and 2018 with the diagnosis of acute rheumatic fever were included in the study. The relationship of gender and age with the frequency of major symptoms and the distribution of the drugs used in the treatment and their side effects were evaluated.Results:Medical records of 102 patients with complete data were reviewed. 56.9% of the patients were male and the mean age was 10.7 ± 1.9 years. The most common distribution of complaints found were arthritis (51%), arthralgia (25.5%) and fever (16.7%). 10.8% of all patients (n = 11) were diagnosed subclinical carditis via echocardiographic evaluation. The frequency of carditis was higher in female patients with a borderline statistical significance (p = 0.05). However, there was no statistically significant difference between gender and arthritis (p = 0.22) and carditis (p > 0.05). Anti-congestive therapy was required in 22% and inotropic treatment was needed in 6.1% cases. Toxic hepatitis developed in four cases during the acetylsalicylic acid treatment.Conclusions:In a 10-year period, detection of subclinical carditis in 10.8% cases supported that echocardiography should be performed as a standard method for the diagnosis of acute rheumatic fever. Patients should be followed closely in terms of hepatic toxicity due to acetylsalicylic acid used in the treatment.

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was ? 1.64 (? 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was ?2.81 ([? 3.79] to [? 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis. © 2021, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.

Otoimmun hepatit; nedeni bilinmeyen, kronik, ilerleyici ve enflamatuar bir karaciger hastaligidir. Tani; viral hepatitin dislandigi hastalarda karaciger enzim yuksekligi, serumda dolasan cesitli otoantikorlar, hipergamaglobulinemi ve histopatolojik olarak portal alan ya da cevresinde mononukleer hucre infiltrasyonu ile konulmaktadir. Hastalikta ciddi hiperbilirubinemi oldukca nadir gorulmektedir. Biz bu yazida ilk ve belirgin bulgusu sarilik ve ciddi hiperbilirubinemi olan tip 2 otoimmun hepatitli cocuk hastayi sunuyoruz. Bu olguda sarilik immun baskilayici tedavi ile oldukca basarili sekilde duzelmistir.

Acute hemorrhagic edema of infancy is a leukocytoclastic small vessel vasculitis of young children that is limited to the skin, generally has a benign course without systemic involvement, and does not require treatment. It is characterized by fever, edema of the lower extremities, and wide purpuric rash of the skin. It typically affects infants aged 6-24 months with a history of recent respiratory system illness. An 11-month-old and a 57-month-old cases with acute hemorrhagic edema of infancy who concurrently have a lower respiratory system infection are presented in this case report.

Amac: Zehirlenmeler acil servislerde sik karsilasilan, olume yol acabilen, onlenebilir bir saglik sorunudur. Zehirlenme etkenleri yasa, cinsiyete, ulkelere, bolgelere ve mevsimlere gore degiskenlik gosterebilmektedir. Bu calismada, cocuk acil poliklinigine basvuran zehirlenme vakalarinin epidemiyolojik ve demografik ozelliklerinin belirlenmesi ve koruyucu onlemlerin saptanmasi amaclanmistir. Yontem: Calismamizda 01.06.2017 ve 01.06.2018 tarihleri arasinda Bakirkoy Dr. Sadi Konuk Egitim ve Arastirma Hastanesi Cocuk Acil Poliklinigine basvuran 0-17 yas arasi zehirlenme vakalari geriye donuk incelendi. Bulgular: Calismamiza 196 vaka dâhil edilmistir. Bu vakalarin %54.1’i kiz, %45.9’u erkekti. Vakalarin %81.6’si kaza ile %13,3’u ozkiyim amacli %5,1’i ise terapotik hata nedeniyle meydana gelmisti. Zehirlenme etkenlerinin %61.2’si ilaclar, %38.8’i ilac disi toksinlerden olusmaktaydi. En cok alinan etkenler sirasiyla %21.9’unda analjezik-antipiretik, %18.9’unda koroziv madde, %11.7’sinde koroziv olmayan irritan madde, %7.7’sinde antidepresan, %6.6’sinda vitamin-mineral seklindedir. Alim yolu incelendiginde; %96.9’unun oral, %0.5’inin cilt, %2.6’sinin inhalasyon yolla alindigi gorulmektedir. Cocuklarin %81.6’si cocuk acil unitesinde, %5.1’i ayaktan, %7.1’i cocuk servisinde ve %6.1’i cocuk yogun bakim servisinde takip edilmistir. Vakalarin ortalama gozlem suresi 20.55±19.98 saat iken, ortalama maliyeti 274.19±445.69 TL olarak hesaplanmistir. Sonuc: Saglik personelinin ve ailelerin bilinclendirilmesi, koruyucu onlemler alinmasinda, hastanelerin is yukunun azaltilmasinda ve ulke ekonomisine olumlu katki saglamada, birincil adim olacaktir.